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Sample records for histiocytosis orbital lesion

  1. Histiocytosis

    MedlinePlus

    Histiocytosis X; Langerhans cell histiocytosis; Eosinophilic granuloma; Pulmonary histiocytosis X; Nonlipid reticuloendotheliosis; Pulmonary Langerhans cell granulomatosis; Hand-Schuller-Christian disease; Letterer-Siwe disease

  2. Langerhans cell histiocytosis with presentation as orbital disease.

    PubMed

    Bhanage, Ashok B; Katkar, Anand D; Ghate, Prajakta S

    2015-01-01

    Langerhans cell histiocytosis (LCH) is an uncommon multisystem disease with an abnormal polyclonal proliferation of Langerhans cells that invade various organs. In rare instances, the affection of the orbit is the only and the first symptom. We report an unusual case of an 18-month-old male who presented with orbital disease as the first symptom, in the form of chronic presentation of periorbital swelling (2 months duration) with acute inflammation (1-week duration) giving a suspicion of orbital cellulitis. Histopathology after radical excision confirmed the diagnosis of LCH and was advised initial therapy as per Histiocyte Society Evaluation and Treatment Guidelines (2009) but was lost to follow-up only reappearing with progression (multisystem LCH with risk organ involvement) and developed progressive active disease on treatment after 5 weeks. He was treated with salvage therapy for risk patients achieving complete remission. PMID:26167225

  3. Langerhans cell histiocytosis with presentation as orbital disease

    PubMed Central

    Bhanage, Ashok B.; Katkar, Anand D.; Ghate, Prajakta S.

    2015-01-01

    Langerhans cell histiocytosis (LCH) is an uncommon multisystem disease with an abnormal polyclonal proliferation of Langerhans cells that invade various organs. In rare instances, the affection of the orbit is the only and the first symptom. We report an unusual case of an 18-month-old male who presented with orbital disease as the first symptom, in the form of chronic presentation of periorbital swelling (2 months duration) with acute inflammation (1-week duration) giving a suspicion of orbital cellulitis. Histopathology after radical excision confirmed the diagnosis of LCH and was advised initial therapy as per Histiocyte Society Evaluation and Treatment Guidelines (2009) but was lost to follow-up only reappearing with progression (multisystem LCH with risk organ involvement) and developed progressive active disease on treatment after 5 weeks. He was treated with salvage therapy for risk patients achieving complete remission. PMID:26167225

  4. Histiocytosis X revealed by diabetes insipidus and skin lesions.

    PubMed

    El Fekih, Nadia; Kamoun, Inés; Jones, Meriem; Remmeh, Soummeya; Zéglaoui, Faten; Ben Slama, Claude; Fazaa, Bécima

    2013-07-01

    Langerhans cell histiocytosis is part of a larger group of syndromes described as histiocytoses. The disease may involve single or multiple systems including skin and nervous system. Here we report an adult case where Langerhans cell histiocytosis presented with diabetes insipidus and cutaneous ulcers.

  5. Crystal storing histiocytosis presenting as a temporal lobe mass lesion

    PubMed Central

    Johnson, Mahlon; Mazariegos, Juan; Lewis, P. Jeffery; Pomakova, Diana

    2013-01-01

    Background: Crystal storing histiocytosis (CSH) is a disorder characterized by local or diffuse infiltration of histiocytes containing crystalline inclusions most commonly of immunoglobulin light chain. Involvement of the central nervous system is extremely rare. CSH may be misdiagnosed as an infection or tumor. In patients with involvement of other organs, it is frequently associated with lymphoplasmacytic diseases. Case Description: A 20-year-old female was evaluated for 2 weeks of progressively worsening headaches. At presentation, she had no history of fevers but reported a sore throat without cough 3-4 days prior. Her past medical history was unremarkable. She denied intravenous drug use or sexually transmitted diseases but lived with an individual with a history of fungal meningitis. On examination she was afebrile, alert, and oriented with a blood pressure of 110/70 mmHg. She had no adenopathy or neurological deficits. Her white blood cell count was minimally elevated. Magnetic resonance imaging revealed a 3.5 × 1.3 × 1.9 cm contrast enhancing lesion of the left temporal lobe with a mild midline shift. Evaluation by multiple specialists suggested a differential diagnosis of an infectious or neoplastic process. Cultures for infectious agents were negative. The biopsy showed CSH. Postoperatively and at 1 month follow up, she was neurologically intact. Conclusion: Radiographically and intraoperatively, CSH may mimic an infectious process or neoplasm. Its recognition is critical to facilitate appropriate therapy and prompt screening for an occult lymphoplasmacytic neoplasm, plasma cell dyscrasia or other underlying disease. PMID:24032087

  6. Langerhans cell histiocytosis: recurrent lesions affecting mandible in a 10-year-old patient.

    PubMed

    Loducca, S V; Mantesso, A; Araújo, N S; Magalhães, M H

    2001-01-01

    Hand-Schuller-Christian disease is a multifocal variant of eosinophilic granuloma, characterised by the classical triad of bony lesions, exophthalmos and diabetes insipidus. This case relates recurrent Langerhans' cell histiocytosis lesions presented as destruction of periodontal support associated with diabetes in a 10-year-old patient. Medical history suggests that the case represents a case of Hand-Schuller Christian disease.

  7. From the Archives of the AFIP. Pediatric orbit tumors and tumorlike lesions: osseous lesions of the orbit.

    PubMed

    Chung, Ellen M; Murphey, Mark D; Specht, Charles S; Cube, Regino; Smirniotopoulos, James G

    2008-01-01

    Many extraocular masses involving the pediatric orbit have an osseous origin. The most common is the dermoid inclusion cyst; these cystic lesions may contain lipid and are most often found near the zygomaticofrontal suture, adjacent to an indolent-appearing erosion of bone. Some primary bone lesions may involve the orbit, producing a lytic or dense lesion with enlargement of the bone; these lesions include fibrous dysplasia, juvenile ossifying fibroma, and osteosarcoma. Fibrous dysplasia tends to produce a mass of ground-glass appearance with longitudinal osseous expansion, whereas juvenile ossifying fibroma is likely to produce a mixed lytic and sclerotic lesion and focal osseous enlargement. Osteosarcoma causes marked bone destruction and variable osteoid production. Langerhans cell histiocytosis, an idiopathic reticuloendothelial proliferative disorder, tends to involve the bones of the skull, especially the lateral orbital roof; it produces lytic destruction of bone with a sclerotic rim and a large intraorbital soft-tissue mass. Granulocytic sarcoma is a solid tumor that may occur in children with myelogenous leukemia. These tumors tend to arise in the subperiosteum of the lateral orbital wall, although they usually do not disrupt the bone. Finally, the orbit is a common site for bone metastases from neuroblastoma, which cause aggressive periosteal reaction in the orbital roof or lateral wall. The last three conditions are often bilateral. At imaging evaluation, osseous lesions may appear similar to each other and to nonosseous masses of the orbit. Knowledge of the pathologic features of these tumors and how these features are reflected in their imaging appearances may help radiologists differentiate them.

  8. Multisystem Langerhans Cell Histiocytosis in Adults Revealed by Skin Lesions.

    PubMed

    Atarguine, Hanane; Hocar, Ouafa; Oussmane, Samia; Mouafik, Sara Batoul; Hamdaoui, Abderrachid; Hafiane, Hanan; Belbaraka, Rhizlane; Akhdari, Nadia; Amal, Said

    2016-01-01

    A 37-year-old woman with no remarkable medical or family history presented with papules and vesicles on an erythematous background involving the neck, sacrum, and folds (postauricular, axillary, inguinal, and under the breasts) (Figure 1). During the previous year, she was treated with local and systemic antifungals without improvement. Her history included a secondary amenorrhea, polydipsia, and polyuria (6 L/d) that started 2 years prior. Physical examination revealed chronic bilateral purulent otorrhea with thick eardrums. Histologic examination of skin biopsy revealed a highly suggestive appearance of multisystem Langerhans cell histiocytosis (LCH) with immunohistochemistry (anti-PS100 and anti-CD1a), which were positive (Figure 2A and 2B). Pituitary magnetic resonance imaging showed a thickening of the pituitary stalk in relation to a location histiocytic (Figure 3). Bone gaps were objectified on two radiographic tibial diaphyseal. Results from computed tomography (CT) scan showed a magma coelio mesenteric, axillary, and inguinal lymph nodes. PMID:27319965

  9. Relative efficacy of radiographic and radionuclide bone surveys in the detection of the skeletal lesions of histiocytosis X

    SciTech Connect

    Parker, B.R.; Pinckney, L.; Etcubanas, E.

    1980-02-01

    Radionuclide studies have been considered a more sensitive indicator of the presence of the bony lesions of histiocytosis X in children that have radiographic bone surveys. Our results suggest that the opposite is true. Although positive correlation between bone scans and radiographs was found in eight of nine patients, only 35% of the individual lesions visible on radiographs were seen on radionuclide studies. Results of radionuclide studies did not correlate with age or sex of the patient, presence or duration of symptoms, radiographic appearance, or anatomic location of lesions. Our results suggest that the radiographic skeletal survey is more sensitive than radionuclide studies in detecting the skeletal lesions of histiocytosis X.

  10. Irradiation of the hypothalamic-hypophyseal area induces complete regression of mucocutaneous lesions in disseminated histiocytosis X

    SciTech Connect

    Palmieri, G.; Stefani, S.; Gridelli, C.; Conte, A.; Airoma, G.; Contegiacomo, A.; Bianco, A.R.

    1989-07-01

    We report on a 54-year-old woman with disseminated histiocytosis X who had a complete regression of all mucocutaneous lesions within 1 month from the completion of radiation therapy (4500 cGy) to the hypothalamic-hypophyseal (H-H) area. This response lasted 12 months, after which new cutaneous and bone lesions appeared.

  11. Polyclonal T-Cells Express CD1a in Langerhans Cell Histiocytosis (LCH) Lesions

    PubMed Central

    West, Jennifer A.; Olsen, Sharon L.; Mitchell, Jenée M.; Priddle, Ross E.; Luke, Jennifer M.; Åkefeldt, Selma Olsson; Henter, Jan-Inge; Turville, Christopher; Kannourakis, George

    2014-01-01

    Langerhans cell histiocytosis (LCH) is a complex and poorly understood disorder that has characteristics of both inflammatory and neoplastic disease. By using eight-colour flow cytometry, we have identified a previously unreported population of CD1a+/CD3+ T-cells in LCH lesions. The expression of CD1a is regarded as a hallmark of this disease; however, it has always been presumed that it was only expressed by pathogenic Langerhans cells (LCs). We have now detected CD1a expression by a range of T-cell subsets within all of the LCH lesions that were examined, establishing that CD1a expression in these lesions is no longer restricted to pathogenic LCs. The presence of CD1a+ T-cells in all of the LCH lesions that we have studied to date warrants further investigation into their biological function to determine whether these cells are important in the pathogenesis of LCH. PMID:25343480

  12. Combined immunological and histochemical analysis of skin and lymph node lesions in histiocytosis X.

    PubMed Central

    Thomas, J A; Janossy, G; Chilosi, M; Pritchard, J; Pincott, J R

    1982-01-01

    The immunological phenotype of the cells involved in skin and lymph node lesions from two cases of histiocytosis X (H-X) were analysed by immunofluorescence techniques using combinations of heterologous and monoclonal antisera to Ia-like antigen and human cortical thymocyte (HTA-1) determinant. These cells were also characterised by a new technique using simultaneous immunofluorescence and enzyme histochemistry for acid phosphatase (ACPase). The major cell type in the lesions was found to express the same Ia+, HTA-1+ phenotype as normal epidermal Langerhans' cells (LC) and was unreactive for ACPase. Additional cell types included Ia-, HTA-1- multinucleate giant cells and residual lymphoid populations. These findings endorse previous concepts that H-X is a proliferation of abnormal LC and emphasise the heterogeneous nature of the cells involved in the disease. Images PMID:6175664

  13. Sclerosing Lesions of the Orbit: A Review.

    PubMed

    Lokdarshi, Gautam; Pushker, Neelam; Bajaj, Mandeep S

    2015-01-01

    Orbital sclerosing inflammation is a distinct group of pathologies characterized by indolent growth with minimal or no signs of inflammation. However, contrary to earlier classifications, it should not be considered a chronic stage of acute inflammation. Although rare, orbital IgG4-related disease has been associated with systemic sclerosing pseudotumor-like lesions. Possible mechanisms include autoimmune and IgG4 related defective clonal proliferation. Currently, there is no specific treatment protocol for IgG4-related disease although the response to low dose steroid provides a good response as compared to non-IgG4 sclerosing pseudotumor. Specific sclerosing inflammations (e.g. Wegener's disease, sarcoidosis, Sjogren's syndrome) and neoplasms (lymphoma, metastatic breast carcinoma) should be ruled out before considering idiopathic sclerosing inflammation as a diagnosis.

  14. Sclerosing Lesions of the Orbit: A Review

    PubMed Central

    Lokdarshi, Gautam; Pushker, Neelam; Bajaj, Mandeep S.

    2015-01-01

    Orbital sclerosing inflammation is a distinct group of pathologies characterized by indolent growth with minimal or no signs of inflammation. However, contrary to earlier classifications, it should not be considered a chronic stage of acute inflammation. Although rare, orbital IgG4-related disease has been associated with systemic sclerosing pseudotumor-like lesions. Possible mechanisms include autoimmune and IgG4 related defective clonal proliferation. Currently, there is no specific treatment protocol for IgG4-related disease although the response to low dose steroid provides a good response as compared to non-IgG4 sclerosing pseudotumor. Specific sclerosing inflammations (e.g. Wegener's disease, sarcoidosis, Sjogren's syndrome) and neoplasms (lymphoma, metastatic breast carcinoma) should be ruled out before considering idiopathic sclerosing inflammation as a diagnosis. PMID:26692715

  15. Cutaneous histiocytosis syndromes.

    PubMed

    Roper, S S; Spraker, M K

    1985-11-01

    Cutaneous histiocytosis may take two principal forms. It is either a benign proliferative process or a relentless, progressive process with a poor prognosis. In histiocytic medullary reticulosis, histiocytes demonstrate nuclear atypia and the outcome is uniformly fatal. Benign cephalic histiocytosis X causes lesions similar to those of histiocytosis X, but Langerhans' cells are absent. In congenital self-healing histiocytosis X, the Letterer-Siwe-like cutaneous infiltrate contains Langerhans' cells, but the lesions heal spontaneously without treatment. The nodular cutaneous lesions of juvenile xanthogranuloma appear in infancy and resolve without treatment; however, the higher percentage (10%) of associated ocular lesions may lead to glaucoma and blindness. In histiocytosis X, the cutaneous lesions show a marked proliferation of Langerhans' cells, with prognosis dependent on the patient's age and the extent of organ dysfunction. Patients who survive the acute form of the disease may develop diabetes insipidus, growth retardation, pulmonary fibrosis, and biliary cirrhosis. A subtle immunologic defect has been identified in patients with histiocytosis X, yet the pathogenesis of the disease is still speculative. Familial disease occurring in early infancy should be differentiated from complete or partial immunodeficiency syndromes. Guidelines for evaluating patients with cutaneous histiocytosis are reviewed.

  16. Insufficiency of Bone Scintigraphy in Vertebral Lesions of Langerhans Cell Histiocytosis Compared to F-18 Fluorodeoxyglucose Positron Emission Tomography/Computed Tomography and Diagnostic Computed Tomography

    PubMed Central

    Koç, Zehra Pınar; Şimşek, Selçuk; Akarsu, Saadet; Balcı, Tansel Ansal; Onur, Mehmet Ruhi; Kepenek, Ferat

    2015-01-01

    Langerhans cell histiocytosis (LCH) is a benign disorder related to the histiocytes which can infiltrate bone tissue. The most effective method for demonstrating severity of this disease is PET/CT and bone scintigraphy might show bone lesions. We present a seventeen year old male patient with disseminated LCH presented with exophtalmos and having multiple vertebral lesions which were identified by F-18 FDG PET/CT scan and diagnostic CT but not in the bone scintigraphy. PMID:25800594

  17. Insufficiency of bone scintigraphy in vertebral lesions of langerhans cell histiocytosis compared to f-18 fluorodeoxyglucose positron emission tomography/computed tomography and diagnostic computed tomography.

    PubMed

    Koç, Zehra Pınar; Şimşek, Selçuk; Akarsu, Saadet; Balcı, Tansel Ansal; Onur, Mehmet Ruhi; Kepenek, Ferat

    2015-02-01

    Langerhans cell histiocytosis (LCH) is a benign disorder related to the histiocytes which can infiltrate bone tissue. The most effective method for demonstrating severity of this disease is PET/CT and bone scintigraphy might show bone lesions. We present a seventeen year old male patient with disseminated LCH presented with exophtalmos and having multiple vertebral lesions which were identified by F-18 FDG PET/CT scan and diagnostic CT but not in the bone scintigraphy.

  18. Langerhans cell histiocytosis

    PubMed Central

    Aruna, D. R.; Pushpalatha, G.; Galgali, Sushma; Prashanthy

    2011-01-01

    Langerhans cell histiocytosis (LCH) is a group of rare disorders histologically characterized by the proliferation of Langerhans cells. Multiple organs and systems may be involved by the disease. Typically, there is bone involvement and, less frequently, lesions may be found in the lungs, liver, lymph nodes, skin, and mucosa. Oral soft tissue lesions without bone involvement are rare. We present a case of oral lesions associated with LCH in a young woman. PMID:22028518

  19. Extramedullary hematopoietic lesion of the orbit presenting as a ring enhancing lesion.

    PubMed

    Crum, Alison V; Morris, Heather; Hogan, R Nick; Mancini, Ronald

    2013-01-01

    A 47-year-old healthy man presented with symptoms of double vision and drooping of the right upper eyelid. Examination disclosed signs of vertical diplopia and right upper-eyelid ptosis. An MRI delineated a well circumscribed, ring enhancing mass within the right orbit. The mass with peripheral enhancement located within the orbit, generated a broad differential diagnosis. After an excisional biopsy was performed, the mass was revealed to be fibrosis with extramedullary hematopoiesis. Current differential diagnoses of ring enhancing orbital lesions do not include extramedullary hematopoiesis, but we present a unique presentation of extramedullary hematopoiesis presenting as a ring enhancing orbital mass.

  20. Pure cutaneous histiocytosis X of the vulva.

    PubMed

    Rose, P G; Johnston, G C; O'Toole, R V

    1984-10-01

    Histiocytosis X of the vulva is extremely rare. In most reported cases, vulvar disease is associated with multiple organ involvement and systemic disease manifestations aiding in the diagnosis. Reported is a case of histiocytosis X presenting solely with vulvar lesions for eight years' duration. The diagnosis, pathology, and subsequent management are discussed. The literature of histiocytosis X of the vulva is reviewed, and its presentations and treatments summarized. Vulvar lesions unresponsive to therapy warrant biopsy and other causes of histiocytic reactions must be excluded to establish the diagnosis.

  1. Pure cutaneous histiocytosis X.

    PubMed

    Magaña-García, M

    1986-03-01

    A 38-month-old boy presented with nodules in the skin of the genital region present for 2 1/2 years. These later spread to the skin of the trunk, head, and extremities. A complete clinical workup could not reveal involvement in any other organ sites and biopsy of one of the cutaneous lesions was diagnosed as histiocytosis X. Because the child was in generally good condition, no treatment was given. Follow-up revealed that the disease had remained limited to the skin, where 15% of the lesions disappeared spontaneously.

  2. Generalized eruptive histiocytosis mimicking leprosy.

    PubMed

    Sharath Kumar, B C; Nandini, A S; Niveditha, S R; Gopal, M G

    2011-01-01

    Generalized eruptive histiocytosis (GEH) is a rare cutaneous histiocytosis that mainly affects adults and presents with multiple symmetric papules on face, trunk, and proximal extremities. GEH is included in type IIa (histiocytes involving cells of dermal dendrocyte lineage) of histiocytic disorders. Clinical and pathological correlations are required for differentiating GEH from other histiocytic disorders and from lepromatous leprosy which clinically mimic GEH and is prevalent in India. We report a case of a middle-aged woman who presented with generalized asymptomatic papules and nodules and was treated for leprosy but was finally diagnosed to have GEH after clinical, histopathological, and immunohistochemical correlation. Furthermore, the newer lesions also showed features of progressive nodular histiocytosis.

  3. Flow-cytometric DNA content of histiocytosis X (Langerhans cell histiocytosis).

    PubMed Central

    Rabkin, M. S.; Wittwer, C. T.; Kjeldsberg, C. R.; Piepkorn, M. W.

    1988-01-01

    Retrospective DNA-content analysis was performed by flow cytometry on formalin-fixed, paraffin-embedded tissue from 36 patients with histiocytosis X (Langerhans cell histiocytosis). Included were 17 patients with solitary bone lesions, 4 patients with multiple bone lesions, 2 patients with solitary extraosseous lesions, 1 patient with congenital self-healing histiocytosis, and 12 patients with disseminated disease. The diagnosis was in each case verified by review of the clinical history and histopathologic material. None of the cases displayed significant cytologic atypia. DNA content analysis failed to reveal additional G0-G1 peaks or "shoulders" suggestive of aneuploid subpopulations in any case. Full-peak coefficients of variation ranged from 3.8 to 8.0. Our data suggest that despite a prior report of a single aneuploid case of histiocytosis X, DNA content analysis may not be useful in predicting clinical stage and outcome in this disease. Images Figure 1 PMID:3258734

  4. Pericardial Effusion in Langerhans Cell Histiocytosis: A Case Report

    PubMed Central

    Gholami, Narges

    2016-01-01

    Introduction Langerhans cell histiocytosis (LCH) is a proliferative disorder of histiocytes in multiple organs. Langerhans cell histiocytosis involves bones, skin, lung and other organs. Case Presentation This study describes a seven-month-old Iranian girl who presented with skin rash and cervical lymphadenopathy. Langerhans cell histiocytosis was suspected when it was associated with anemia, splenomegaly and lytic bone lesions. A skin biopsy confirmed the diagnosis of Langerhans cell histiocytosis. During hospitalization, the patient looked ill with respiratory distress. A chest X-ray showed a ground glass view, and echocardiography showed moderate pericardial effusion. Conclusions Pericardial effusion was a rare finding in this case of Langerhans cell histiocytosis. Pericardial effusion in Langerhans cell histiocytosis, which is an unusual presentation, should be considered when the patient experiences respiratory distress.

  5. Pericardial Effusion in Langerhans Cell Histiocytosis: A Case Report

    PubMed Central

    Gholami, Narges

    2016-01-01

    Introduction Langerhans cell histiocytosis (LCH) is a proliferative disorder of histiocytes in multiple organs. Langerhans cell histiocytosis involves bones, skin, lung and other organs. Case Presentation This study describes a seven-month-old Iranian girl who presented with skin rash and cervical lymphadenopathy. Langerhans cell histiocytosis was suspected when it was associated with anemia, splenomegaly and lytic bone lesions. A skin biopsy confirmed the diagnosis of Langerhans cell histiocytosis. During hospitalization, the patient looked ill with respiratory distress. A chest X-ray showed a ground glass view, and echocardiography showed moderate pericardial effusion. Conclusions Pericardial effusion was a rare finding in this case of Langerhans cell histiocytosis. Pericardial effusion in Langerhans cell histiocytosis, which is an unusual presentation, should be considered when the patient experiences respiratory distress. PMID:27621925

  6. The histopathology of Langerhans cell histiocytosis.

    PubMed Central

    Favara, B. E.; Jaffe, R.

    1994-01-01

    Selected aspects of the histopathology of Langerhans cell histiocytosis representing diagnostic difficulty and/or controversy are presented with emphasis on the composition of pathological lesions. Lesional cell phenotypes and the factors influencing variations are noted. Features of several skin-based histiocytic disorders, dermatopathic lymphadenopathy and Rosai-Dorfman disease are compared. Associations between Langerhans cell histiocytosis and juvenile xanthogranuloma and malignant disorders are considered. Observations of potential significance in the eventual elucidation of the pathogenesis of these enigmatic diseases are presented. Images Figure 3 Figure 4 PMID:7521200

  7. [Orbit: Part 1: anatomy, imaging procedures and retrobulbar lesions].

    PubMed

    Reith, W; Yilmaz, U

    2015-08-01

    The aim of this 2-part review article on diseases of the orbit is to give the reader an insight into the anatomical structure and an overview of the most important diseases in the area of the eye socket. The main focus is on a description of the imaging procedures and their individual advantages and disadvantages. The most important tumors, trauma and degenerative alterations of the orbit are also described.

  8. Langerhans' cell histiocytosis of the temporal fossa: A case report

    PubMed Central

    LIANG, CHEN; LIANG, QIANLEI; DU, CHANGWANG; ZHANG, XIAODONG; GUO, SHIWEN

    2016-01-01

    Langerhans' cell histiocytosis (LCH) is a rare disease with a wide spectrum of clinical manifestations, varying from an isolated lesion to systemic involvement. The etiology of this disease remains to be elucidated. The present study reports a case of LCH with temporal fossa localization in an 8-year-old male patient, who had exhibited left temporal pain and headache for 1 month. Physical examination revealed slight exophthalmos and conjunctival hemorrhage in the patient's left eye, and non-contrast computed tomography imaging of the head revealed a soft tissue mass with unclear margins located in the left temporal fossa, as well as a wide bony defect. Magnetic resonance imaging revealed a heterogeneously contrast-enhanced mass near the left temporal pole, which eroded into the patient's left orbit and maxillary sinus. The lesion was totally excised and confirmed to be LCH through biopsy. PMID:27073529

  9. Histiocytosis X: an ophthalmological review.

    PubMed Central

    Moore, A T; Pritchard, J; Taylor, D S

    1985-01-01

    Of 76 children with histiocytosis X 18 had orbital involvement, and four developed additional neuro-ophthalmic complications. No instance of intraocular involvement was detected. Among those patients with ophthalmic involvement the main problems were bilateral or unilateral proptosis, ptosis, papilloedema, optic atrophy, and seventh nerve palsy. Only one patient developed a severe visual defect. Management of the ophthalmological complications depends not only on the extent of the orbital disease but also on the degree of systemic involvement. Overall management by a paediatric oncologist is mandatory. Images PMID:3871158

  10. Histiocytosis X: treatment with topical nitrogen mustard.

    PubMed

    Berman, B; Chang, D L; Shupack, J L

    1980-07-01

    The case histories of two elderly patients with cutaneous histiocytosis X treated topically with nitrogen mustard are presented. The cutaneous lesions cleared within 2 to 3 weeks, and remission was maintained with daily topical administration of nitrogen mustard. The clinical impression of improvement was substantiated by light and electron microscopic studies prior to and after therapy.

  11. Langerhans cell histiocytosis of bone: MR imaging.

    PubMed

    George, J C; Buckwalter, K A; Cohen, M D; Edwards, M K; Smith, R R

    1994-01-01

    Magnetic resonance (MR) images of 12 pathologically proven lesions of Langerhans cell histiocytosis (LCH) of bone were reviewed retrospectively. MR identified all lesions, three of which were not identified on plain radiographs. In all cases, MR showed greater abnormality than did plain radiographs. With one exception, all lesions were hypointense on T1-weighted images and hyperintense on T2-weighted images. The lesions and associated soft tissue abnormalities were very conspicuous on short TI inversion sequences and T1-weighted post-contrast images. Follow-up MR studies in two patients after chemotherapy showed decreased size and enhancement of lesions compared with baseline studies.

  12. Chronic dermal sinuses as a manifestation of histiocytosis X.

    PubMed Central

    Sacks, S H; Hall, I; Ragge, N; Pritchard, J

    1986-01-01

    Two young patients presented with generalised lymphadenopathy, otorrhoea, otitis, and rash. Over the next few years chronically discharging sinuses began to form over enlarged nodes and histological appearances were typical of histiocytosis X. In neither case were micro-organisms isolated from the lesions, and in both patients healing occurred with immunosuppressive agents. Chronic dermal sinus formation secondary to lymph node disease has never before been recorded as a manifestation of histiocytosis X. Histiocytosis X should therefore be considered in the differential diagnosis of "suppurative" lymphadenopathy so that appropriate treatment may be given without delay. Images Case 1 PMID:3084014

  13. Orbital tumours and tumour-like lesions: exploring the armamentarium of multiparametric imaging.

    PubMed

    Purohit, Bela S; Vargas, Maria Isabel; Ailianou, Angeliki; Merlini, Laura; Poletti, Pierre-Alexandre; Platon, Alexandra; Delattre, Bénédicte M; Rager, Olivier; Burkhardt, Karim; Becker, Minerva

    2016-02-01

    Although the orbit is a small anatomical space, the wide range of structures present within it are often the site of origin of various tumours and tumour-like conditions, both in adults and children. Cross-sectional imaging is mandatory for the detection, characterization, and mapping of these lesions. This review focuses on multiparametric imaging of orbital tumours. Each tumour is reviewed in relation to its clinical presentation, compartmental location, imaging characteristics, and its histological features. We herein describe orbital tumours as lesions of the globe (retinoblastoma, uveal melanoma), optic nerve sheath complex (meningioma, optic nerve glioma), conal-intraconal compartment (hemangioma), extraconal compartment (dermoid/epidermoid, lacrimal gland tumours, lymphoma, rhabdomysarcoma), and bone and sinus compartment (fibrous dysplasia). Lesions without any typical compartmental localization and those with multi-compartment involvement (veno-lymphatic malformation, plexiform neurofibroma, idiopathic orbital pseudotumour, IgG4 related disease, metastases) are also reviewed. We discuss the role of advanced imaging techniques, such as MR diffusion-weighted imaging (DWI), diffusion tensor imaging, fluoro-2-deoxy-D-glucose positron emission tomography CT (FDG-PET CT), and positron emission tomography MRI (MRI PET) as problem-solving tools in the evaluation of those orbital masses that present with non-specific morphologic imaging findings. Main messages/Teaching points • A compartment-based approach is essential for the diagnosis of orbital tumours. • CT and MRI play a key role in the work-up of orbital tumours. • DWI, PET CT, and MRI PET are complementary tools to solve diagnostic dilemmas. • Awareness of salient imaging pearls and diagnostic pitfalls avoids interpretation errors.

  14. Orbital tumours and tumour-like lesions: exploring the armamentarium of multiparametric imaging.

    PubMed

    Purohit, Bela S; Vargas, Maria Isabel; Ailianou, Angeliki; Merlini, Laura; Poletti, Pierre-Alexandre; Platon, Alexandra; Delattre, Bénédicte M; Rager, Olivier; Burkhardt, Karim; Becker, Minerva

    2016-02-01

    Although the orbit is a small anatomical space, the wide range of structures present within it are often the site of origin of various tumours and tumour-like conditions, both in adults and children. Cross-sectional imaging is mandatory for the detection, characterization, and mapping of these lesions. This review focuses on multiparametric imaging of orbital tumours. Each tumour is reviewed in relation to its clinical presentation, compartmental location, imaging characteristics, and its histological features. We herein describe orbital tumours as lesions of the globe (retinoblastoma, uveal melanoma), optic nerve sheath complex (meningioma, optic nerve glioma), conal-intraconal compartment (hemangioma), extraconal compartment (dermoid/epidermoid, lacrimal gland tumours, lymphoma, rhabdomysarcoma), and bone and sinus compartment (fibrous dysplasia). Lesions without any typical compartmental localization and those with multi-compartment involvement (veno-lymphatic malformation, plexiform neurofibroma, idiopathic orbital pseudotumour, IgG4 related disease, metastases) are also reviewed. We discuss the role of advanced imaging techniques, such as MR diffusion-weighted imaging (DWI), diffusion tensor imaging, fluoro-2-deoxy-D-glucose positron emission tomography CT (FDG-PET CT), and positron emission tomography MRI (MRI PET) as problem-solving tools in the evaluation of those orbital masses that present with non-specific morphologic imaging findings. Main messages/Teaching points • A compartment-based approach is essential for the diagnosis of orbital tumours. • CT and MRI play a key role in the work-up of orbital tumours. • DWI, PET CT, and MRI PET are complementary tools to solve diagnostic dilemmas. • Awareness of salient imaging pearls and diagnostic pitfalls avoids interpretation errors. PMID:26518678

  15. Gastric polyposis caused by multifocal histiocytosis X.

    PubMed Central

    Wada, R; Yagihashi, S; Konta, R; Ueda, T; Izumiyama, T

    1992-01-01

    A rare case of gastric polyposis caused by infiltration of Langerhans' cells is reported. A 53 year old Japanese woman complaining of vague abdominal discomfort, was found at endoscopy to have numerous polyps all over the gastric wall. An endoscopic biopsy specimen showed characteristic infiltration of Langerhans' cells in the lamina propria of the mucosa. Functional abnormalities such as impaired gastric acid secretion or malabsorption were not associated with this lesion and the patient was treated conservatively. During follow up over two years, she had a cutaneous eruption with infiltration of histiocytes and osteolytic lesions in the skull. However, no progressive changes occurred in the stomach. This probably benign self-limiting lesion of gastric histiocytosis X may be one of the manifestations of multifocal histiocytosis X, but its aetiology and appropriate treatment have not yet been determined. Images Figure 1 Figure 2 Figure 3 Figure 4 PMID:1644344

  16. Social attachment in juvenile monkeys with neonatal lesion of the hippocampus, amygdala and orbital frontal cortex.

    PubMed

    Goursaud, Anne-Pierre S; Bachevalier, Jocelyne

    2007-01-10

    Non-human primates, like humans, develop and maintain social relationships and attachments throughout their life. The first and most crucial relationship in a primate life is that with its mother. Yet, in absence of their biological mother, infant primates form attachment to surrogate mothers. Although, this early attachment is critical for the development of normal species-typical social and emotional skills, the neural substrates underlying the formation of social relationships in primates are still unclear. The present study assessed, in infant rhesus monkeys (Macaca mulatta) reared by human caregivers and social interactions with peers, the effects of bilateral neonatal (1-2 weeks of age) ibotenic acid lesions of the amygdala and hippocampus (N=6 in each group), aspiration lesions of the orbital frontal cortex (N=6) or sham lesions (N=5) on the development of a social attachment with the principal human caregiver. A specific preference for the later was assessed at 11 months of age, in a two-choice discrimination task, opposing the principal human caregiver to another familiar human, in a familiar environment. None of the lesions impaired the expression of preferential responses toward the principal human caregiver. Nevertheless, lesions of the orbital frontal cortex led to a weaker preference, suggesting that this structure may play a role in the quality and/or strength of the infant/mother relationships. The present non-human primate findings are discussed in terms of their relevance for autism. PMID:17084912

  17. Behavioral and hormonal reactivity to threat: Effects of selective amygdala, hippocampal or orbital frontal lesions in monkeys

    PubMed Central

    Machado, Christopher J.; Bachevalier, Jocelyne

    2008-01-01

    Summary We compared the effects of bilateral amygdala, hippocampal or orbital frontal cortex lesions on emotional and hormonal reactivity in rhesus monkeys (Macaca mulatta). Experiment 1 measured behavioral reactivity to an unfamiliar human intruder before and after surgery. Animals with amygdala lesions demonstrated decreases in one passive defensive behavior (freezing), whereas animals with hippocampal lesions showed decreases in a more stimulus-directed defensive behavior (tooth grinding). Orbital frontal cortex lesions also reduced these two defensive behaviors, as well as decreased cage-shaking dominance displays. Animals with amygdala, hippocampal or sham lesions also demonstrated increased tension-related behaviors after surgery, but those with orbital frontal lesions did not. Finally, all three lesions diminished the operated animals' ability to modulate tension-related behaviors depending on the magnitude of threat posed by the human intruder. Experiment 2 measured circulating levels of cortisol and testosterone when a subset of these same animals were at rest and following physical restraint, temporary isolation, exposure to threatening objects and social interactions with an unfamiliar conspecific. None of the lesions impacted on testosterone levels in any condition. Amygdala or orbital frontal lesions blunted cortisol reactivity during isolation from peers, but not during any other condition. Hippocampal lesions did not alter circulating levels of cortisol under any conditions. These results indicate that the amygdala, hippocampus and orbital frontal cortex play distinct, yet complimentary roles in coordinating emotional and hormonal reactivity to threat. PMID:18650022

  18. Urticating histiocytosis: a mast cell-rich variant of histiocytosis X.

    PubMed

    Foucar, E; Piette, W W; Tse, D T; Goeken, J; Olmstead, A D

    1986-05-01

    Histiocytosis X and mastocytosis are proliferative processes that may have similar cutaneous manifestations. However, a positive Darier's sign (urtication on stroking of the lesion) is thought to reliably distinguish between these two diseases. We recently studied a 13-year-old girl with a 2-year history of extensive skin lesions and a positive Darier's sign. Routine histopathologic studies revealed a polymorphous cutaneous infiltrate composed of histiocytes, mast cells, eosinophils, and lymphoid cells. Electron microscopic studies demonstrated Langerhans granules in some of the histiocytes, and immunologic studies of frozen tissue showed that a significant subpopulation of the histiocytes marked as Langerhans cells. Giemsa staining of specimens from eight other cases of cutaneous histiocytosis X from our files revealed mast cells in all of the lesions, although none showed the abundance of mast cells present in the case with urtication. Our studies emphasize the often polymorphous nature of the cell population in cutaneous histiocytosis X and demonstrate that confusing clinical findings can result when the mast cell population in histiocytosis X produces urtication.

  19. Langerhans Cell Histiocytosis: Emerging Insights and Clinical Implications.

    PubMed

    Zinn, Daniel J; Chakraborty, Rikhia; Allen, Carl E

    2016-02-01

    Langerhans cell histiocytosis is a disorder characterized by lesions that include CD207+ dendritic cells along with an inflammatory infiltrate. Langerhans cell histiocytosis has a highly variable clinical presentation, ranging from a single lesion to potentially fatal disseminated disease. The uncertainty as to whether Langerhans cell histiocytosis is a reactive or a neoplastic disease has resulted in a long-standing debate on this question, and the limited understanding of the pathogenesis of the disease has impeded clinical improvement for patients. The current standard of care for multisystem Langerhans cell histiocytosis, empirically derived chemotherapy with vinblastine and prednisone, cures fewer than 50% of patients, and optimal therapies for relapse and neurodegenerative disease remain uncertain. Recent research advances support a model in which Langerhans cell histiocytosis arises due to pathologic activation of the mitogen-activated protein kinase (MAPK) pathway in myeloid precursors. Redefinition of Langerhans cell histiocytosis as a myeloid neoplastic disorder driven by hyperactive ERK supports the potential of chemotherapy with efficacy against immature myeloid cells, as well as mutation-specific targeted therapy. PMID:26888790

  20. Dissociable effects of subtotal lesions within the macaque orbital prefrontal cortex on reward-guided behavior.

    PubMed

    Rudebeck, Peter H; Murray, Elisabeth A

    2011-07-20

    The macaque orbital prefrontal cortex (PFo) has been implicated in a wide range of reward-guided behaviors essential for efficient foraging. The PFo, however, is not a homogeneous structure. Two major subregions, distinct by their cytoarchitecture and connections to other brain structures, compose the PFo. One subregion encompasses Walker's areas 11 and 13 and the other centers on Walker's area 14. Although it has been suggested that these subregions play dissociable roles in reward-guided behavior, direct neuropsychological evidence for this hypothesis is limited. To explore the independent contributions of PFo subregions to behavior, we studied rhesus monkeys (Macaca mulatta) with restricted excitotoxic lesions targeting either Walker's areas 11/13 or area 14. The performance of these two groups was compared to that of a group of unoperated controls on a series of reward-based tasks that has been shown to be sensitive to lesions of the PFo as a whole (Walker's areas 11, 13, and 14). Lesions of areas 11/13, but not area 14, disrupted the rapid updating of object value during selective satiation. In contrast, lesions targeting area 14, but not areas 11/13, impaired the ability of monkeys to learn to stop responding to a previously rewarded object. Somewhat surprisingly, neither lesion disrupted performance on a serial object reversal learning task, although aspiration lesions of the entire PFo produce severe deficits on this task. Our data indicate that anatomically defined subregions within macaque PFo make dissociable contributions to reward-guided behavior.

  1. Role of Fine Needle Aspiration Cytology as a Diagnostic Tool in Orbital and Adnexal Lesions

    PubMed Central

    Khan, Lubna; Malukani, Kamal; Malaiya, Siddharth; Yeshwante, Prashant; Ishrat, Saba; Nandedkar, Shirish S.

    2016-01-01

    Purpose: To evaluate the role of fine needle aspiration (FNAC) as a diagnostic tool in cases of orbital and ocular adnexal masses. Cytological findings were correlated with histopathological diagnosis wherever possible. Methods: FNAC was performed in 29 patients of different age groups presenting with orbital and ocular adnexal masses. Patients were evaluated clinically and investigated by non-invasive techniques before fine needle aspiration of the masses. Smears were analyzed by a cytologist in all cases. Further, results of cytology were compared with the histopathological diagnosis. Results: The age of patients ranged from 1 to 68 years (mean: 29.79±19.29). There were 14 males and 15 females with a male to female ratio of 0.93:1. Out of 29 cases, 26 aspirates were cellular. Cellularity was insufficient in three (10.34%) aspirates. Out of 26 cellular aspirates, 11 were non-neoplastic while 15 were neoplastic on cytology. Subsequent histopathologic examination was done in 21/26 cases. Concordance rate of FNAC in orbital and ocular adnexal mass lesions with respect to the precise histologic diagnosis was 90%. Conclusion: When properly used in well-indicated patients (in cases where a diagnosis cannot be made by clinical and imaging findings alone), FNAC of orbital and periorbital lesions is an invaluable and suitable adjunct diagnostic technique that necessitates close cooperation between the ophthalmologist and cytologist. However, nondiagnostic aspirates may sometimes be obtained, and an inconclusive FNAC should not always be ignored. PMID:27621787

  2. Role of Fine Needle Aspiration Cytology as a Diagnostic Tool in Orbital and Adnexal Lesions

    PubMed Central

    Khan, Lubna; Malukani, Kamal; Malaiya, Siddharth; Yeshwante, Prashant; Ishrat, Saba; Nandedkar, Shirish S.

    2016-01-01

    Purpose: To evaluate the role of fine needle aspiration (FNAC) as a diagnostic tool in cases of orbital and ocular adnexal masses. Cytological findings were correlated with histopathological diagnosis wherever possible. Methods: FNAC was performed in 29 patients of different age groups presenting with orbital and ocular adnexal masses. Patients were evaluated clinically and investigated by non-invasive techniques before fine needle aspiration of the masses. Smears were analyzed by a cytologist in all cases. Further, results of cytology were compared with the histopathological diagnosis. Results: The age of patients ranged from 1 to 68 years (mean: 29.79±19.29). There were 14 males and 15 females with a male to female ratio of 0.93:1. Out of 29 cases, 26 aspirates were cellular. Cellularity was insufficient in three (10.34%) aspirates. Out of 26 cellular aspirates, 11 were non-neoplastic while 15 were neoplastic on cytology. Subsequent histopathologic examination was done in 21/26 cases. Concordance rate of FNAC in orbital and ocular adnexal mass lesions with respect to the precise histologic diagnosis was 90%. Conclusion: When properly used in well-indicated patients (in cases where a diagnosis cannot be made by clinical and imaging findings alone), FNAC of orbital and periorbital lesions is an invaluable and suitable adjunct diagnostic technique that necessitates close cooperation between the ophthalmologist and cytologist. However, nondiagnostic aspirates may sometimes be obtained, and an inconclusive FNAC should not always be ignored.

  3. Vulvar Langerhans cell histiocytosis: a case report

    PubMed Central

    Khoummane, Nadia; Guimeya, Cyriane; Lipombi, Dominique; Gielen, François

    2014-01-01

    Langerhans cell histiocytoses (LCH) are a rare group of disorders that comprise a large spectrum of diseases initially known as histiocytosis X. In this case report, we relate a case of LCH affecting the vulva of a 47-year-old female. The patient presented since 3 years with a vulvar lesion characterized by non-healing ulcers and a perineal granuloma on which she underwent surgery. Professionals should keep in mind not to treat straightforwardly lesions of the genital tract as simple sexually transmitted diseases. Chronic, atypical genital lesions seen in women need to be worked up and dealt with accordingly. PMID:25404979

  4. Langerhans Cell Histiocytosis of the Clavicle

    PubMed Central

    Wang, Shaowu; Zhang, Weisheng; Na, Shengbo; Zhang, Lina; Lang, Zhijin

    2014-01-01

    Abstract We report a rare case of solitary Langerhans cell histiocytosis (LCH) involving the clavicle of an adult female. The patient was a 32-year-old female presenting with 1 month history of progressive pain, swelling, and tenderness in the region near the left sternoclavicular joint. Radiograph, computed tomography, and magnetic resonance imaging showed an osteolytic lesion in the clavicle with tumor extension and soft tissue edema. Surgical curettage of the lesion was performed, and the histopathologic diagnosis was LCH. Because of its rarity and possibly variable presentation, LCH should be included and considered in the differential diagnosis when we encounter a clavicle lesion. PMID:25365405

  5. A Rare Case of Erdheim-Chester Disease and Langerhans Cell Histiocytosis Overlap Syndrome

    PubMed Central

    Nabi, Shahzaib; Arshad, Adeel; Jain, Tarun; Virk, Fawad; Gulati, Rohit; Awdish, Rana

    2015-01-01

    A 48-year-old woman with a past medical history of seizures and end-stage renal disease secondary to obstructive uropathy from retroperitoneal fibrosis presented to the emergency department with seizures and altered mental status. A Glasgow Coma Scale of 4 prompted intubation, and she was subsequently admitted to the intensive care unit. Magnetic resonance imaging of the brain performed to elucidate the aetiology of her seizure showed a dural-based mass within the left temporoparietal lobe as well as mass lesions within the orbits. Further imaging showed extensive retroperitoneal fibrosis extending to the mediastinum with involvement of aorta and posterior pleural space. Imaging of the long bones showed bilateral sclerosis and cortical thickening of the diaphyses. Imaging of the maxillofacial structures showed osseous destructive lesions involving the mandible. These clinical and radiological features were consistent with a diagnosis of Erdheim-Chester disease; however, the patient's skin biopsy was consistent with Langerhans cell histiocytosis. PMID:26579323

  6. Langerhans cell histiocytosis with multisystem involvement in an infant: A case report

    PubMed Central

    BI, LINTAO; SUN, BUTONG; LU, ZHENXIA; SHI, ZHANGZHEN; WANG, DAN; ZHU, ZHENXING

    2015-01-01

    Langerhans cell histiocytosis (LCH) is a proliferative disease of histiocyte-like cells, with a wide range of clinical presentations that vary from a solitary lesion to more severe multifocal or disseminated lesions. The disease can affect any age group; however, the peak incidence rate is in infants aged between 1 and 3 years-old. Diagnosis of LCH should be based on the synthetical analysis of clinical presentations, in addition to features of imaging and histopathology. Although certain cases regress spontaneously, other patients require systemic chemotherapy together with the administration of steroids. The present study reports the case of an infant with LDH with multisystem involvement, including that of the bone, skin, orbit, spleen and lungs. The patient received chemotherapy and obtained rapid improvement in the involved systems. A total of 2.5 years after completion of the therapy, the patient still remains in follow-up and no evidence of active disease has been noted. PMID:26136948

  7. A Clinical Update and Radiologic Review of Pediatric Orbital and Ocular Tumors

    PubMed Central

    Rao, Ajay A.; Naheedy, John H.; Chen, James Y.-Y.; Robbins, Shira L.; Ramkumar, Hema L.

    2013-01-01

    While pediatric orbital tumors are most often managed in tertiary care centers, clinicians should be aware of the signs of intraocular and orbital neoplasms. In the pediatric population, a delay in diagnosis of orbital and intraocular lesions, even if benign, can lead to vision loss and deformity. Intraocular lesions reviewed are retinoblastoma, medulloepithelioma, and retinal astrocytic hamartoma. Orbital neoplasms reviewed are rhabdomyosarcoma, neuroblastoma metastases, optic pathway glioma, plexiform neurofibroma, leukemia, lymphoprolipherative disease, orbital inflammatory syndrome, dermoid and epidermoid inclusion cysts, and Langerhans' cell histiocytosis. Vascular lesions reviewed are infantile hemangioma and venous lymphatic malformation. In conjunction with clinical examination, high-resolution ophthalmic imaging and radiologic imaging play an important role in making a diagnosis and differentiating between benign and likely malignant processes. The radiologic imaging characteristics of these lesions will be discussed to facilitate prompt diagnosis and treatment. The current treatment modalities and management of tumors will also be reviewed. PMID:23577029

  8. Combined unilateral lesions of the amygdala and orbital prefrontal cortex impair affective processing in rhesus monkeys.

    PubMed

    Izquierdo, Alicia; Murray, Elisabeth A

    2004-05-01

    The amygdala and orbital prefrontal cortex (PFo) interact as part of a system for affective processing. To assess whether there is a hemispheric functional specialization for the processing of emotion or reward or both in nonhuman primates, rhesus monkeys (Macaca mulatta) with combined lesions of the amygdala and PFo in one hemisphere, either left or right, were compared with unoperated controls on a battery of tasks that tax affective processing, including two tasks that tax reward processing and two that assess emotional reactions. Although the two operated groups did not differ from each other, monkeys with unilateral lesions, left and right, showed altered reward-processing abilities as evidenced by attenuated reinforcer devaluation effects and an impairment in object reversal learning relative to controls. In addition, both operated groups showed blunted emotional reactions to a rubber snake. By contrast, monkeys with unilateral lesions did not differ from controls in their responses to an unfamiliar human (human "intruder"). Although the results provide no support for a hemispheric specialization of function, they yield the novel finding that unilateral lesions of the amygdala-orbitofrontal cortical circuit in monkeys are sufficient to significantly disrupt affective processing. PMID:14711973

  9. Congenital cutaneous histiocytosis in a piglet.

    PubMed

    Hélie, P; Kiupel, M; Drolet, R

    2014-07-01

    A 2-week-old crossbred male piglet with numerous congenital, variably sized macules, plaques, and papules distributed all over the body was submitted for necropsy. Significant gross and histological lesions were restricted to the skin. On light microscopic examination, these cutaneous lesions corresponded to dermal and/or subcutaneous masses composed of spindle-shaped to round cells that multifocally contained hemosiderin; epidermotropism was not observed. Immunohistochemically, the neoplastic cells were strongly positive for CD204; moderately positive for CD163, lysozyme, and vimentin; and negative for Mac 387, α-1-antitrypsin, S-100 protein and E-cadherin; frozen tissues were not available for CD1a and CD11c. Transmission electron microscopic examination of sections from formalin-fixed tissues did not reveal Birbeck's granules. The clinical, morphological, and immunohistochemical results were consistent with a congenital cutaneous histiocytosis of non-Langerhans cell origin. The condition most resembled juvenile xanthogranuloma in humans, a generally skin-limited non-Langerhans histiocytic disorder that can be congenital. Cutaneous and/or systemic histiocytic disorders are well characterized in dogs and have been described in cats, and a case with some similarities to ours has been reported in a neonatal piglet, but this is to our knowledge the first immunohistochemically supported report of histiocytosis in the pig and congenital histiocytosis in animals.

  10. Langerhans Cell Histiocytosis: An Illusion of Hope

    PubMed Central

    Desai, Vela D; Varma, Beena; Sharma, Rajeev

    2013-01-01

    ABSTRACT Introduction: Langerhans cell histiocytosis (LCH) is a rare atypical cellular disorder characterized by clonal proliferation of Langerhans cells leading to myriad clinical presentations and variable outcomes. It usually occurs in children and young adults. It can be present with local and systemic manifestation involving skin, bone, mucosal tissues and internal organs. Aims and objectives: The stomatologist plays an important role in management of the disease by keeping in mind the various oral manifestations of the disease. Case report: Of a child with disseminated LCH with multiorgan involvement who presented with failure to thrive, osteolytic bony lesions and extensive cutaneous eruptions. Conclusion: Early diagnosis and awareness is necessary to treat the patients. How to cite this article: Desai VD, Priyadarshinni SR, Varma B, Sharma R. Langerhans Cell Histiocytosis: An Illusion of Hope. Int J Clin Pediatr Dent 2013;6(1):66-70. PMID:25206193

  11. Benign cephalic histiocytosis.

    PubMed

    Gianotti, F; Caputo, R; Ermacora, E; Gianni, E

    1986-09-01

    Benign cephalic histiocytosis is a self-healing non-X, nonlipid cutaneous histiocytosis of children, characterized by a papular eruption on the head. Mucous membranes and viscera are always spared. In the 13 cases reported herein, the children were otherwise in good general health. The disease appeared during the first three years of life, and spontaneous regression was complete by the age of nine years in the four cases healed to date. The histiocytic infiltrate was localized in the upper and middle dermis and contained no lipids at any stage of evolution. All the histiocytes contained coated vesicles, and 5% to 30% also contained comma-shaped bodies in their cytoplasm.

  12. [Cutaneous histiocytosis X].

    PubMed

    Metz, J; Metz, G; Lechner, W

    1980-09-01

    Histiocytosis X comprises three clinical entities whose common substrate is a localized or systemic proliferation of atypical histiocytes. On the basis of the age of manifestation, acuity of the clinical course and organ involvement Abt-Letterer-Siwe's disease, Hand-Schüller-Christian's disease and eosinophilic granuloma can be differentiated from each other, although transitional varieties of these syndromes are possible. Not infrequently oligosymptomatic forms are misinterpreted, especially when the skin is the only involved organ. In the following case report cutaneous histiocytosis X will be discussed in terms of its clinical expression. Electron-microscopy has proved to be the best methods to make the diagnosis of such atypical cases.

  13. Indeterminate cell histiocytosis that presented clinically as benign cephalic histiocytosis.

    PubMed

    Haimovic, Adele; Chernoff, Karen; Hale, Christopher S; Meehan, Shane A; Schaffer, Julie V

    2014-12-16

    Indeterminate cell histiocytosis (ICH) is a rare, heterogeneous disorder that is characterized by immunophenotypic features of both Langerhans cell histiocytosis (LCH) and non-LCH. We describe a 12-month-old boy with a four-month history of asymptomatic, small, pink-tan papules on his face. Histopathologic evaluation showed a superficial, dermal infiltrate of histiocytes that was positive for S100, CD1a, CD68, and Factor XIIIa. To our knowledge, this represents the first report of the clinical presentation of benign cephalic histiocytosis with immunohistochemical findings of ICH. We review the classification of histiocytic disorders and the clinical and immunohistochemical features of both ICH and benign cephalic histiocytosis.

  14. Benign cephalic histiocytosis

    PubMed Central

    Samson, Joan F.; Libu, Gnanaseelan Kanakamma; Philip, Mariam; Simi, Puthenveedu Salahudeen

    2013-01-01

    A one and a half year old girl born of a non-consanguineous marriage presented with multiple asymptomatic erythematous to hyperpigmented and skin colored papules on both cheeks slowly increasing in number of 1 year duration. On the basis of clinical, histopathological, and immunohistochemistry findings, a diagnosis of benign cephalic histiocytosis was made. PMID:24350010

  15. Benign cephalic histiocytosis.

    PubMed

    Samson, Joan F; Libu, Gnanaseelan Kanakamma; Philip, Mariam; Simi, Puthenveedu Salahudeen

    2013-10-01

    A one and a half year old girl born of a non-consanguineous marriage presented with multiple asymptomatic erythematous to hyperpigmented and skin colored papules on both cheeks slowly increasing in number of 1 year duration. On the basis of clinical, histopathological, and immunohistochemistry findings, a diagnosis of benign cephalic histiocytosis was made.

  16. Lanthanum-Induced Gastrointestinal Histiocytosis

    PubMed Central

    Araya, Hiwot; Longacre, Teri A.; Pasricha, Pankaj J.

    2015-01-01

    A patient with end-stage renal disease (ESRD) on hemodialysis presented with fever, anorexia, and nausea shortly after starting oral lanthanum carbonate for phosphate control. Gastric and duodenal biopsies demonstrated diffuse histiocytosis with intracellular aggregates of basophilic foreign material. Transmission electron microscopy, an underutilized diagnostic test, revealed the nature of the aggregates as heavy metal particles, consistent with lanthanum. Symptoms and histiocytosis improved after discontinuation of lanthanum. Lanthanum may be an underdiagnosed cause of gastrointestinal histiocytosis. PMID:26157959

  17. Opposing effects of amygdala and orbital prefrontal cortex lesions on the extinction of instrumental responding in macaque monkeys.

    PubMed

    Izquierdo, Alicia; Murray, Elisabeth A

    2005-11-01

    Extinction is a well-known behavioural phenomenon that allows organisms to respond flexibly to a changing environment. Although recent work implicates the amygdala and orbital prefrontal cortex (PFo) in extinction of Pavlovian conditioned fear and aversion, much less is known about the neural bases of instrumental extinction. To explore the contribution of the macaque amygdala to flexible responding in the face of changing reward contingency, we tested the effects of selective, excitotoxic lesions of the amygdala on extinction of an instrumental response. For comparison, we evaluated the effects of ablation of PFo on the same task. Amygdala lesions facilitated the extinction of instrumental responses, whereas lesions of PFo had the opposite effect.

  18. Otic Langerhans' Cell Histiocytosis in an Adult: A Case Report and Review of the Literature

    PubMed Central

    Gungadeen, Anil; Kullar, Peter; Yates, Philip

    2013-01-01

    Objective. To present a case of otic Langerhans' cell histiocytosis in an adult. Also included the diagnosis and management of the condition and a review of the relevant literature. Case Report. We report a case of a 41-year-old man with a history of persistent unilateral ear discharge associated with an aural polyp. Radiological imaging showed bony lesions of the skull and a soft-tissue mass within the middle ear. Histological analysis of the polyp demonstrated Langerhans' cell histiocytosis. His otological symptoms were completely resolved with the systemic therapy. Conclusions. Otic Langerhans' cell histiocytosis can present in adults. Persistent ear symptoms along with evidence of soft-tissue masses within the ear and bony lesions of the skull or elsewhere should prompt the otolaryngologists to include Langerhans' cell histiocytosis in their differential diagnosis. Management should be with systemic therapy rather than local surgical treatment. PMID:23762704

  19. Photodynamic therapy for multi-resistant cutaneous Langerhans cell histiocytosis

    PubMed Central

    Failla, Valérie; Wauters, Odile; Caucanas, Marie; Nikkels-Tassoudji, Nazli; Nikkels, Arjen F

    2010-01-01

    Langerhans cell histiocytosis is a rare group of proliferative disorders. Beside cutaneous involvement, other internal organs can be affected. The treatment of cutaneous lesions is difficult and relies on topical corticosteroids, carmustine, nitrogen mustard, and photochemotherapy. Systemic steroids and vinblastine are used for recalcitrant skin lesions. However, some cases fail to respond. An 18-month old boy presented a CD1a+, S100a+ Langerhans cell histocytosis with cutaneous and severe scalp involvement. Topical corticosteroids and nitrogen mustard failed to improve the skin lesions. Systemic corticosteroids and vinblastine improved the truncal involvement but had no effect on the scalp lesions. Methylaminolevulinate (MAL) based photodynamic therapy (PDT) resulted in a significant regression of the scalp lesions. Control histology revealed an almost complete clearance of the tumor infiltrate. Clinical follow-up after six months showed no recurrence. Although spontaneous regression of cutaneous Langerhans cell histiocytosis is observed, the rapid effect of photodynamic therapy after several failures of other treatment suggests that photodynamic therapy was successful. As far as we know this is the first report of photodynamic therapy for refractory skin lesions. Larger series are needed to determine whether photodynamic therapy deserves a place in the treatment of multiresistant cutaneous Langerhans cell histiocytosis. PMID:21139836

  20. Progressive Nodular Histiocytosis Associated with Eale's Disease

    PubMed Central

    Williams, Abhilasha; Thomas, Abraham G; Kwatra, Kanwardeep Singh; Jain, Kunal

    2015-01-01

    Progressive nodular histiocytosis (PNH) is a rare normolipemic macrophage disorder and belongs to a subgroup of non-Langerhans cell histiocytosis (LCHs) which is characterized by a progressive course with no sign of spontaneous resolution but without systemic involvement. We report a 30-year-old gentleman who presented with skin lesions all over the body associated with gradual bilateral painless loss of vision. On examination, approximately 30 to 40, skin-colored, firm, non-tender papules and nodules were noted over the body especially on the face and trunk. A skin biopsy revealed a cellular tumor in the dermis composed of oval to spindle-shaped cells, positive for CD68 but negative for S-100, CD34, CD21, CD35 and HMB45, supporting a diagnosis of spindle cell histiocytic tumor. Ophthalmic examination revealed a generalized arteriolar attenuation in both eyes. He received Tab Imatinib 400 mg OD for 5 months followed by Tab Pazopanib 800 mg OD for 4 months and both the drugs were stopped due to lack of any response in the skin lesions. We report this case due to its rarity, characteristic clinical presentation, and its association with Eale's disease. Primary treatment remains surgical excision of bothersome lesions and optimal systemic treatment is still unknown. PMID:26288410

  1. Congenital cutaneous Langerhans cell histiocytosis and cutaneous mastocytoma in a child.

    PubMed

    Lozano Masdemont, B; Campos Dominguez, M; Gomez-Recuero Munoz, L; Moreno Garcia, B; Parra Blanco, V; Suarez Fernandez, R

    2016-01-01

    Langerhans cell histiocytosis and mastocytoma are clonal disorders of bone-marrow-derived cells, most commonly seen in the pediatric age. Infiltration of mast cells and Langerhans cells in the same lesion has been published before, but, to our knowledge, this is the first time that the occurrence of two mastocytomas and Langerhans cell histiocytosis is reported. It could be hypothesized that both clonal disorders of bone-marrow-derived cells could have a common origin. PMID:27617456

  2. Langerhans cell histiocytosis in monozygotic twins with central diabetes insipidus and hypophyseal masses

    PubMed Central

    Wei, Sung-Tai; Chen, Der-Cherng; Cho, Der-Yang; Lin, Hung-Lin

    2015-01-01

    Langerhans cell histiocytosis (LCH) is a systemic disease mainly affecting children and young adults. It can manifest as single system disorder or multi-system involvement. When the central nervous system is involved, the hypothalamic–pituitary axis is the most common location affected. Herein we report a rare case of Langerhans cell histiocytosis in monozygotic twins both with central diabetes and hypophyseal masses. This is the first report about LCH in monozygotic twins with hypophyseal lesions. PMID:25972939

  3. [Colonic histiocytosis (author's transl)].

    PubMed

    Remmele, W; Endris, R

    1977-02-01

    Macrophages accumulating various substances can be detected in the mucosa of the small and large bowel under physiological and various pathological conditions. Among these the so-called PAS-positive macrophages have attracted much attention in recent times. Abundant occurrence of such cells in the intestinal mucosa has been termed "colonic histiocytosis". The occurrence of PAS-positive macrophages was investigated in 200 unselected and otherwise normal biopsy specimens of rectal mucosa; no correlation was found between the occurrence of these cells on the one hand and any intestinal or extraintestinal disease on the other. PAS-positive macrophages were mostly found close to the surface of the mucosa or to the cryptal epithelium as well as between the crypts. It is suggested to abandon the term "colonic histiocytosis" since it induces a false impression of a disease entity in the clinician (and may be related falsely e.g. to "histiocytosis X", and since the clinician may tend to attribute unnecessary importance to this harmless finding.

  4. Electrolytic lesions of the bilateral ventrolateral orbital cortex inhibit methamphetamine-associated contextual memory formation in rats.

    PubMed

    Zhao, Yan; Liu, Peng; Chu, Zheng; Liu, Fei; Han, Wei; Xun, Xi; Dang, Yong-hui

    2015-10-22

    The memories that are formed between rewarding and drug-associated contextual cues have been suggested to contribute to drug addiction relapse. Recent evidence has indicated that the ventrolateral orbital cortex (VLO) plays important roles in reward-based learning and reversal learning. However, whether the VLO is required for methamphetamine-induced contextual memory formation is not well understood. In the present study, a three-phase methamphetamine-induced conditioned place preference (CPP) model was used to investigate the effects of VLO lesions on the formation of drug-associated contextual memories in rats. We found that the VLO lesions themselves elicited no observable effects on place preferences. However, the VLO lesions delayed the acquisition and extinction phases of CPP without affecting the expression level. Furthermore, the VLO lesions did not have an obvious influence on CPP reinstatement. These results indicate that electrolytic lesions of the bilateral ventrolateral orbital cortex can inhibit the formation of methamphetamine-induced contextual memories in rats. Moreover, VLO may not be critically involved in memory storage and retrieval.

  5. An unusual presentation of Langerhans cell histiocytosis

    PubMed Central

    Agarwal, Palak; Kaushal, Manju

    2014-01-01

    Langerhans cell histiocytosis (LCH) is a relatively rare and unique disease. An incidence of 7.9% in the jaws is reported. We report a case of 9-year-old male child referred to us from dental outpatient department, who presented with a firm swelling in right lower jaw along with bilateral submandibular lymphadenopathy for 1-month. Fine-needle aspiration was done from lytic lesion in the body of mandible and multiple smears were prepared. On the basis of the clinical and cytomorphological findings, a diagnosis of LCH was suggested. The diagnosis was confirmed on histology. Thus, a high possibility of LCH should be considered in children presenting with lytic lesions in head and neck region. PMID:25745295

  6. A Retrospective Analysis of Oral Langerhans Cell Histiocytosis in an Iranian Population: a 20-year Evaluation

    PubMed Central

    Atarbashi Moghadam, Saede; Lotfi, Ali; Piroozhashemi, Batool; Mokhtari, Sepideh

    2015-01-01

    Statement of the Problem Langerhans cell histiocytosis is a rare disease with unknown pathogenesis and is characterized by local or disseminated proliferation of Langerhans cells. There is no previous investigation on prevalence of oral Langerhans cell histiocytosis in Iranian population. Purpose The purpose of this study was to assess the relative frequency of oral Langerhans cell histiocytosis in an Iranian population and to compare the data with previous reports. Materials and Method Pathology files of Oral and Maxillofacial Pathology Department of Dental School of Shahid Beheshti University of Medical Sciences from 1992 to 2012 were searched for cases recorded as oral Langerhans cell histiocytosis. A total number of 20 cases were found and the clinical information of patients was recorded. Results The relative frequency of oral Langerhans cell histiocytosis was 0.34% and the most common location was the posterior mandible. In addition, the mean age of patients was 27 years and there was a definite male predominance. Most lesions were localized and tooth mobility was the most common oral presentation. Conclusion In Iranian population as in many other countries, the relative frequency of oral Langerhans cell histiocytosis is low. Moreover, tooth mobility and periodontal lesions are the frequent early signs of disease. Therefore, in patients with periodontal problems, good oral health, and no response to the treatment; Langerhans cell histiocytosis must be considered. Additionally, although most cases of oral Langerhans cell histiocytosis are localized, systemic involvement must also be considered and dental professionals have an important role in early detection of the disease. PMID:26535408

  7. Langerhans cell histiocytosis: Current concepts in dentistry and case report

    PubMed Central

    Ramos-Gutiérrez, Efraín; Alejo-González, Francisco; Ruiz-Rodríguez, Socorro; Garrocho-Rangel, José-Arturo

    2016-01-01

    Langerhans cell histiocytosis (LCH), which is a rare granulomatous pediatric disease of unknown etiology, is characterized by the idiopathic proliferation and accumulation of abnormal and clonal Langerhans cells or their marrow precursors, resulting in localized, solitary or multiple destructive lesions. These lesions are most commonly eosinophilic granuloma, which are found in craniofacial bone structures such as the skull and mandible, skin and other organs. In children, the disease has a variable initial presentation, and the clinical course, prognosis and survival are unpredictable. The aims of this report were to present an LCH case in a girl aged 2 years, 8 months and her clinicopathological features, to describe the bucodental management provided, and to discuss special dental considerations of this disease. Key words:Children, dental management, histiocytosis, Langerhans cells. PMID:26855698

  8. [Generalized granuloma annulare or diffuse dermal histiocytosis?].

    PubMed

    Kretzschmar, L; Biel, K; Luger, T A; Goerdt, S

    1995-08-01

    Generalized granuloma annulare is a rare variant of granuloma annulare affecting the trunk and extremities with a multitude of lesions. In contrast to localized granuloma annulare, generalized granuloma annulare occurs in older patients, shows a stronger association with diabetes, and is characteristically chronic. Like our 55-year-old patient, most patients present with papules and annular plaques; less often, macular or non-annular lesions may be encountered. Histology often fails to show necrobiotic or necrotic connective tissue changes demarcated by a palisading granuloma. Instead, there are diffuse dermal, band-like or nodular aggregations of histiocytes intermingled with some multinucleated giant cells and a predominantly lymphocytic infiltrate in the periphery. Because of its special characteristics, it has been suggested that generalized granuloma annulare might constitute a separate disease entity and that it should be classed among the primary cutaneous histiocytoses as a diffuse dermal histiocytosis. Using immunohistochemistry to determine the macrophage phenotype of the lesional histiocytes, we have shown that generalized granuloma annulare is not a cutaneous histiocytosis. Neither MS-1 high-molecular-weight protein, a new specific marker for cutaneous non-Langerhans cell histiocytoses, nor CD1a, the well-known marker for Langerhans cells and Langerhans cell histiocytoses, is expressed by the lesional histiocytes of our patient. In contrast, the antigen expression pattern was diagnostic for non-infectious granulomas and was highly similar to that in localized granuloma annulare. In contrast to the successful treatment of localized granuloma annulare reported with intralesional interferon beta-1, systemic treatment with interferon alpha-2b (9 x 10(6) units three times a week) was ineffective.

  9. General Information about Langerhans Cell Histiocytosis (LCH)

    MedlinePlus

    ... Langerhans Cell Histiocytosis Treatment (PDQ®)–Patient Version General Information About Langerhans Cell Histiocytosis (LCH) Go to Health ... the PDQ Pediatric Treatment Editorial Board . Clinical Trial Information A clinical trial is a study to answer ...

  10. Benign isolated histiocytosis mimicking chicken pox in a neonate: report of two cases with ultrastructural study.

    PubMed

    Valderrama, E; Kahn, L B; Festa, R; Lanzkowsky, P

    1985-01-01

    Two cases of benign isolated cutaneous histiocytosis X in newborn infants are reported. Clinically, the lesions mimicked chicken pox, and the histologic findings in skin biopsies were indistinguishable from those described in infantile Letterer-Siwe disease. Electron microscopy showed Langerhans' cells with the classical trilaminar, racket-shaped granules. Our cases a well as 10 of the 21 previously reported cases showed spontaneous resolution of the skin lesions. Since this is a benign spontaneous resolving histiocytosis, we recommend that no therapy be given provided that no other signs of systemic involvement are found.

  11. [Pulmonary Langerhans cell histiocytosis].

    PubMed

    Popper, H H

    2015-09-01

    Pulmonary Langerhans cell histiocytosis is regarded as a reactive proliferation of the dendritic Langerhans cell population stimulated by chronic tobacco-derived plant proteins due to incomplete combustion but can also occur in childhood as a tumor-like systemic disease. Currently, both these forms cannot be morphologically distinguished. In the lungs a nodular proliferation of Langerhans cells occurs in the bronchial mucosa and also peripherally in the alveolar septa with an accompanying infiltration by eosinophilic granulocytes and destruction of the bronchial wall. Langerhans cells can be selectively detected with antibodies against CD1a and langerin. In the reactive isolated pulmonary form, abstinence from tobacco smoking in most patients leads to regression of infiltration and improvement of symptoms. In high-resolution computed tomography (HRCT) the small star-like scars can still be detected even after complete cessation of tobacco smoking.

  12. Two sporadic cases of adult-onset progressive mucinous histiocytosis.

    PubMed

    Young, A; Olivere, J; Yoo, S; Martins, C; Barrett, T

    2006-02-01

    Progressive mucinous histiocytosis is a rare, benign, non-Langerhans' cell histiocytosis limited to the skin. Ten cases--all women--in four families and one sporadic case have been described in the literature. The disorder usually begins in childhood and progresses slowly. We report two sporadic cases of adult-onset progressive mucinous histiocytosis in unrelated African-American women, aged 48 and 55 years, respectively, who developed red-brown and flesh-coloured, asymptomatic papules on the face, the arms and the legs without truncal, mucosal or visceral involvement. The lesions showed no spontaneous regression. Both patients lacked associated systemic symptoms, including polyuria, polydipsia or seizures. There was no underlying hyperlipidaemia, paraproteinaemia or lymphoproliferative disease. No family history of similar lesions could be identified. Light microscopy revealed dermal proliferation of spindle-shaped histiocytes with abundant mucin deposition. Electron microscopy demonstrated a high number of myelin figures or zebra bodies in the cytoplasm of histiocytes. On immunohistochemistry, positive staining with macrophage markers--CD68, HAM56 and lysozyme--and factor XIIIa, a transglutaminase present in dermal dendrocytes, and negative staining with Langerhans' cell markers--CD1a and S100--and CD34, a marker present in dermal dendritic cells derived from uncommitted mesenchymal cells, were observed. PMID:16420313

  13. "Pure" cutaneous histiocytosis-X.

    PubMed

    Wolfson, S L; Botero, F; Hurwitz, S; Pearson, H A

    1981-11-15

    The case histories of two young children who experienced skin rashes involving various areas of the body are reported. The diagnosis of pure cutaneous histiocytosis-X was established after extensive studies revealed no other organ involvement. The patients were treated with oral corticosteroids. Currently, both children are in good health, show no evidence of disease, and have been followed over a four-to-five-year period. Therapy with corticosteroids may not be indicated with pure cutaneous histiocytosis-X unless there is evidence of extracutaneous dissemination or rapid progression of the disease.

  14. Visual loss with Langerhans cell histiocytosis: multifocal central nervous system involvement.

    PubMed

    Job, O M; Schatz, N J; Glaser, J S

    1999-03-01

    A 42-year-old woman with a 6-year history of diabetes insipidus and progressive hypersomnolence presented with visual loss. Neuroimaging showed infiltration in the hypothalamus, the optic nerve, and the chiasm, as well as multiple lesions in other areas of the brain parenchyma. Biopsy showed Langerhans cell histiocytosis. This is an unusual presentation of Langerhans cell histiocytosis, involving the visual pathways without manifestations outside of the central nervous system. The differential diagnosis and the magnetic resonance imaging findings will be discussed. PMID:10098549

  15. Cutaneous histiocytosis in dogs.

    PubMed

    Mays, M B; Bergeron, J A

    1986-02-15

    Multifocal cutaneous histiocytic lesions were recognized in 9 dogs. Clinically, the dogs had multiple erythematous plaques or nodules in the skin (1 to 5 cm diameter). Histologically, the lesions were comprised of dermal or pannicular infiltrates of large histiocytic cells, with varying numbers of other inflammatory cells intermixed. By electron microscopy, the cells resembled those of canine cutaneous histiocytoma. The lesions seemed to wax and wane and appeared in new sites, regardless of treatment. The dogs ranged in age from 2 to 13 years; 7 dogs were under 6 years of age. Both sexes and various breeds were represented. An infectious agent could not be identified.

  16. Langerhans cell histiocytosis - a case report.

    PubMed

    Jeunon, Thiago; Sousa, Maria Auxiliadora Jeunon; Santos-Rodrigues, Nilton; Lopes, Raquel

    2012-01-01

    A 17-year-old male presented for dermatologic consultation with slightly elevated reddish papules covered by yellowish scales in the scalp for the last two years and reddish and indurated ulcers in the perineum lasting six months. Additional complaints included polyuria, polydipsia, delay in the development of secondary sexual characteristics and hearing loss of the right ear secondary to a medium otitis. Lesions from scalp and perineum were sampled for histopathologic examination and revealed a dense cellular infiltrate made up of mononuclear cells with conspicuous eosinophilic cytoplasm and large cleaved vesicular nucleus, some of them with shapes resembling the format of a kidney and others reminiscent of coffee beans. Numerous intermingling eosinophils were present. The diagnosis of Langerhans cell histiocytosis was then rendered and confirmed by positive immunostaining of neo-plastic cells for anti-CD1a and anti-S100 protein antibodies. The work-up revealed diabetes insipidus, hypogonadotropic hypogonadism, hiperprolactenemia, growing-hormone deficiency and thickness of the pituitary stalk. The patient was treated with prednisone and vinblastin based chemotherapy regimen for six months with complete remission, but presented recurrence of some lesions in the scalp, which were handled with topical mustard and corticosteroids. After chemotherapy, the endocrinologic disturbances were corrected with hormonal replacement therapy. The patient is currently in good health with a follow-up of five years. PMID:24765546

  17. Cell(s) of Origin of Langerhans Cell Histiocytosis.

    PubMed

    Collin, Matthew; Bigley, Venetia; McClain, Kenneth L; Allen, Carl E

    2015-10-01

    Langerhans cell histiocytosis (LCH) is heterogeneous disease characterized by common histology of inflammatory lesions containing Langerin(+) (CD207) histiocytes. Emerging data support a model in which MAPK activation in self-renewing hematopoietic progenitors may drive disseminated high-risk disease, whereas MAPK activation in more differentiated committed myeloid populations may induce low-risk LCH. The heterogeneous clinical manifestations with shared histology may represent the final common pathway of an acquired defect of differentiation, initiated at more than one point. Implications of this model include re-definition of LCH as a myeloid neoplasia and re-focusing therapeutic strategies on the cells and lineages of origin. PMID:26461145

  18. Early-onset sarcoidosis mimicking refractory cutaneous histiocytosis.

    PubMed

    Ohga, Shouichi; Ichino, Kiyomi; Urabe, Kazunori; Ishimura, Masataka; Takada, Hidetoshi; Nishikomori, Ryuta; Furue, Masutaka; Hara, Toshiro

    2008-03-01

    A 10-year-old female was diagnosed as having early-onset sarcoidosis (EOS) after a prolonged skin disease. A granuloma emerged on the face at age 2 and massive lesions extended to the rest of the body. Repeated biopsies indicated histiocytic proliferation. At age 7, fever, disseminated macular eruptions, and multinucleated giant cells in the bone marrow prompted vinblastine and prednisolone therapy. Five months after stopping therapy, hypercalcemic crisis occurred along with fever, cytopenias, and interferon-gamma-nemia indicating a macrophage activation syndrome. A biopsy of nodules confirmed the diagnosis of sarcoidosis. The atypical EOS should be differentiated from histiocytosis.

  19. Cell(s) of Origin of Langerhans Cell Histiocytosis.

    PubMed

    Collin, Matthew; Bigley, Venetia; McClain, Kenneth L; Allen, Carl E

    2015-10-01

    Langerhans cell histiocytosis (LCH) is heterogeneous disease characterized by common histology of inflammatory lesions containing Langerin(+) (CD207) histiocytes. Emerging data support a model in which MAPK activation in self-renewing hematopoietic progenitors may drive disseminated high-risk disease, whereas MAPK activation in more differentiated committed myeloid populations may induce low-risk LCH. The heterogeneous clinical manifestations with shared histology may represent the final common pathway of an acquired defect of differentiation, initiated at more than one point. Implications of this model include re-definition of LCH as a myeloid neoplasia and re-focusing therapeutic strategies on the cells and lineages of origin.

  20. Langerhans cell histiocytosis revisited: Case report with review

    PubMed Central

    Kumar, Y. Pavan; Agrawal, Jayshree; Mohanlakshmi, J.; Kumar, P. Suresh

    2015-01-01

    Langerhans cell histiocytosis (LCH) is a group of idiopathic disorders characterized by proliferation of bone marrow derived Langerhans cells and mature eosinophils. Their clinical features simulate common oral findings such as gingival enlargement, oral ulcers, and mobility of teeth, along with nonspecific radiographic features; hence, diagnosing such lesions becomes difficult for the oral physicians. These lesions are commonly seen in childhood; however, we are reporting a case of LCH in 29-year-old adult male. A provisional diagnosis of giant cell granuloma was considered based on history and examination, although the lesion was histologically proven to be LCH and was confirmed with immunohistochemical staining of S100 protein and CD1a antigen. The purpose of this paper is to enhance the understanding of diverse, nonpathognomical oral presentation of LCH that is easily misdiagnosed and overlooked by dentist. PMID:26321851

  1. Langerhans cell histiocytosis in children diagnosed by fine-needle aspiration

    PubMed Central

    Handa, Uma; Kundu, Reetu; Punia, Rajpal Singh; Mohan, Harsh

    2015-01-01

    Background: Langerhans cell histiocytosis (LCH) is a rare intricate pediatric neoplasm with varied clinical manifestations and multiple treatment modalities. Aim: To study the cytological features of LCH and the differential diagnoses on fine-needle aspiration (FNA). Materials and Methods: FNA was performed using a 23-gauge needle fitted to a 10 mL syringe mounted on syringe holder. LCH was diagnosed on FNA smears in seven cases confined to the head and neck region, which included three cases of lymphadenopathy, three cases of scalp swelling, and one case of orbital swelling. Results: The age of the patients ranged from 25 days to 11 years and male-to-female ratio was 1:1.3. Clinically, the diagnoses suggested were tuberculosis, inflammatory lesion, abscess, and malignancy. The cytologic findings included high cellularity, isolated Langerhans cells (LCs) with prominent nuclear indentation, grooves and abundant vacuolated cytoplasm, multinucleated giant cells, eosinophils, and lymphocytes. Areas of necrosis were noted in one case. Histopathology, along with positive S-100 immunohistochemistry, confirmed the diagnosis of LCH. Conclusions: LCH is a rare disease occurring predominantly in children and can be diagnosed with ease on FNA cytology by the presence of characteristic Langerhans cells. The S-100 positivity aids in suggesting a diagnosis of LCH. PMID:26811572

  2. Orbital

    NASA Astrophysics Data System (ADS)

    Hanson, Robert M.

    2003-06-01

    ORBITAL requires the following software, which is available for free download from the Internet: Netscape Navigator, version 4.75 or higher, or Microsoft Internet Explorer, version 5.0 or higher; Chime Plug-in, version compatible with your OS and browser (available from MDL).

  3. Squash smear cytology of Langerhans cell histiocytosis

    PubMed Central

    Nahm, Ji Hae; Yoon, Gun; Do, Sung-Im; Kim, Hyun-Soo

    2015-01-01

    Squash smear cytology of Langerhans cell histiocytosis (LCH) has rarely been reported. We described squash cytological findings of cranial LCH. Additionally, based on recent data that suggests an association of LCH with either viral infection or genetic alteration, we investigated the presence of several viruses or mutation of TP53 and BRAF in LCH tissue samples. Intraoperative squash smears of a small tissue fragment excised from the lesion demonstrated a mixed population of eosinophils, neutrophils, small lymphocytes and a high content of histiocytes. The histiocytes possessed abundant dense cytoplasm with round cell shape and eccentrically located nuclei with fine chromatin, delicate nuclear membranes and prominent nuclear grooves, indentations and pseudoinclusions. The cytologic features were consistent with Langerhans cells (LCs). Subsequent histopathologic examination confirmed the diagnosis of LCH. Immunohistochemically, the LCs were positive for S-100, CD1a and langerin, but negative for adenovirus, CMV, EBV, HHV-8, HPV, HSV, SV 40 and p53. BRAF V600E mutation was absent. Our findings did not support the role of viruses and genetic abnormalities in the pathogenesis of LCH. In summary, the presence of a mixed population of inflammatory cells and a high content of histiocytes with characteristic cytomorphology, along with radiologic evidence and appropriate clinical findings, is highly suggestive of LCH on the intraoperative squash smears. Awareness of characteristic cytological features of LCH is necessary for rapid and accurate diagnosis. Squash smear cytology is a potentially useful tool in the intraoperative diagnosis of LCH. PMID:26339366

  4. Benign cephalic histiocytosis: report of four cases.

    PubMed

    de Luna, M L; Glikin, I; Golberg, J; Stringa, S; Schroh, R; Casas, J

    1989-09-01

    We cared for four patients with benign cephalic histiocytosis, a self-healing non-X, nonlipid cutaneous histiocytosis of children. The age of onset of the disease was 5 to 9 months, with papules and erythematous macules involving the head (mainly the cheeks), and posterior spread to the trunk and limbs in three patients. Microscopic examination of skin biopsies revealed a histiocytic infiltrate in the superficial dermis that was S100 protein-negative by immunoperoxidase (PAP method). One patient showed comma-shaped bodies and desmosomelike junctions on electron microscopy. No Birbeck's granules were present. Benign cephalic histiocytosis is a self-limiting condition that requires no treatment.

  5. Preserved stimulus-reward and reversal learning after selective neonatal orbital frontal areas 11/13 or amygdala lesions in monkeys

    PubMed Central

    Kazama, Andy; Bachevalier, Jocelyne

    2012-01-01

    Neither lesions of orbital frontal (OFC) areas 11/13 nor selective amygdala lesions alter the ability to learn stimulus-reinforcer association and reversal discriminations in adult monkeys. Here, we investigated whether the same conclusion will hold true when the same lesions occur in infancy. Infant rhesus monkeys received sham-operations, neurotoxic amygdala lesions, or aspiration OFC 11/13 lesions at 8–10 days of age and were trained on object discrimination reversal (ODR) tasks. Performance on a single pair (1-pair) ODR was assessed at the age of 3 months and 3 years, and then animals were tested in a 5-pairs ODR task in which they had to concurrently learn and reverse five discrimination problems. The results indicated that the ability to solve a single-pair discrimination problem followed by six reversals appears to be late maturing in monkeys but is spared following selective lesions of either OFC areas 11/13 or amygdala, even with the use of the more challenging 5-object ODR task. Finally, performance in the 1-pair ODR at 3 years was comparable to that following adult-onset lesions, indicating that neither OFC areas 11/13 nor amygdala are critical for the development of reversal learning. PMID:22494813

  6. Langerhans cell histiocytosis of the female genital tract.

    PubMed

    Axiotis, C A; Merino, M J; Duray, P H

    1991-03-15

    Langerhans cell histiocytosis (LCH) of the female genital tract is rare. Four new cases are reported, and there is a review of the 38 cases in the literature. This disease may involve the vulva, vagina, cervix, endometrium, and ovary. Four distinct patient groups, segregated on the basis of initial presentation and subsequent anatomic extent of disease, were categorized as follows: (1) "pure" genital LCH, (2) genital LCH with subsequent multi-organ involvement, (3) oral or cutaneous LCH with subsequent genital and multi-organ involvement, and (4) diabetes insipidus with subsequent genital and multi-organ disease. Although involvement of the genital tract can occur at any age, it is most common in young adulthood. Clinically, LCH may mimic either primary neoplasia or various inflammatory lesions; the major pathologic differential diagnosis is venereal and other inflammatory diseases. The pure genital form may have a distinct nosologic position in the spectrum of LCH similar to the "pure," self-limited cutaneous histiocytosis seen in infants. There is no correlation between histologic findings and the outcome of the genital lesions. There is also no correlation between clinical presentation and/or the extent of involvement and outcome of genital lesions; complete regression, partial improvement, persistent lesions, and recurrences were seen in all four groups of patients. The treatment of genital LCH is not well defined and is highly individualized. Therapy has included surgery, radiation, topical corticosteroids, topical nitrogen mustard, systemic chemotherapy, and combination therapy; mixed results were obtained with all treatment modalities. Although no modality has been shown to yield a superior outcome, complete surgical excision is advocated as initial therapy.

  7. Saving orphans: BRAF targeting of histiocytosis.

    PubMed

    Heaney, Mark L

    2013-02-28

    In this issue of Blood, Haroche and colleagues report significant therapeutic activity of the BRAF inhibitor, vemurafenib, in 3 patients with rare histiocytic conditions, Erdheim-Chester disease and Langerhans cell histiocytosis. PMID:23449613

  8. Genetics Home Reference: Langerhans cell histiocytosis

    MedlinePlus

    ... cell histiocytosis affects the lungs, liver, or blood-forming (hematopoietic) system; damage to these organs and tissues ... occurs when the Langerhans cells crowd out blood-forming cells in the bone marrow, leads to a ...

  9. Unusual cutaneous histiocytosis expressing an intermediate immunophenotype between Langerhans' cells and dermal macrophages.

    PubMed

    Berti, E; Gianotti, R; Alessi, E

    1988-08-01

    Cutaneous histiocytosis was discovered in a 40-year-old man with a slow-growing nodule located on his right arm. Histologic findings showed an epidermotropic infiltrate of histiocytes with folded, irregular nuclei. Immunologically, the cells presented an intermediate phenotype between Langerhans' cells and dermal macrophages. After surgical removal of the lesion, neither a relapse nor visceral involvement was observed during two years of follow-up.

  10. Primary pulmonary histiocytosis X in two patients with Hodgkin's disease

    PubMed Central

    Sajjad, Syed M; Luna, Mario A

    1982-01-01

    The lungs may be involved in patients with histiocytosis X as part of the generalised disease, but histiocytosis X confined to the lungs without extrapulmonary involvement is rare. This report describes two cases of primary pulmonary histiocytosis arising in patients with Hodgkin's disease. It is impossible to state with certainty whether the pulmonary histiocytosis arose as a response to the defects of cell-mediated immunity, or as a consequence of immunosuppression by radiation and chemotherapy. Images PMID:6979116

  11. Langerhans cell histiocytosis in adults: a case report and review of the literature

    PubMed Central

    2016-01-01

    Background Langerhans cell histiocytosis (LCH) is a proliferative disease of histiocyte-like cells that generally affects children. Immunohistochemistry is essential to obtain the correct diagnosis, and treatment protocols are controversial. Objective Langerhans cell histiocytosis (LCH) is easy to be misdiagnosed because of its various clinic features and laboratory results. This research focused on the clinicopathological, histopathological, immunohistochemical and other features of LCH and aimed to analyze LCH clinical features for improving diagnosis and decreasing misdiagnosis rate. Case report A case of rare adult LCH was reported and the clinicopathological features were summarized by literature review. The multifocal form of this case includes diabetes insipidus, exophthalmos and mucocutaneous lesions in axillae and anogenital regions, such as infiltrated nodules, extensive coalescing, scaling, crusted papules and ulcerated plaques. The Langerhans cells diffusely infiltrated in the dermis and the tumor cells were positive for CD1a and S-100 expression. The diagnosis was Langerhans cell histiocytosis based on the pathological and immunohistochemical changes. Conclusion LCH has high rate of misdiagnosis and definitive diagnosis depends on pathological biopsy and X-ray examination. The prognosis is related to the onset age and the quantity of affected organs. Although specific therapeutic approach hasn't been well established, combined chemotherapy for multisystem lesions and surgical operation or radiotherapy for unifocal lesions may improve the therapy. PMID:26942568

  12. Solitary cutaneous histiocytosis with granular cell changes: a morphological variant of reticulohistiocytoma?

    PubMed

    Caltabiano, Rosario; Magro, Gaetano; Vecchio, Giada Maria; Lanzafame, Salvatore

    2010-02-01

    We first report a case of granular cell histiocytosis occurring as a solitary polypoid lesion of the nipple in a 15-year-old girl. Histologically, the lesion was composed of a dermal population of medium- to large-sized, short spindle- to round- to epithelioid-shaped cells with eosinophilic cytoplasm containing numerous and small diastase-resistant periodic acid-Schiff (PAS) positive granules. No associated inflammatory cells were observed. Immunohistochemical studies, revealing immunoreactivity exclusively to vimentin and CD68, were consistent with their histiocytic profile. Based on clinical, morphological and immunohistochemical features, the diagnosis of 'solitary cutaneous histiocytosis with granular cell changes' was proposed. The absence of an inflammatory cell component, such as lymphocytes and leucocytes, along with no history of a previous trauma or medical treatment, suggest that the present lesion could fit into the morphological spectrum of the so-called solitary epithelioid histiocytoma, also known as reticulohistiocytoma. Alternatively, the possibility of a histiocytic reaction to unknown stimuli cannot be completely ruled out. Nevertheless, awareness of solitary cutaneous histiocytosis with granular cell changes is useful to avoid confusion with other dermal tumors, especially 'granular cell tumor' and 'dermal non-neural granular cell tumor'.

  13. The effect of orbital prefrontal cortex lesions on performance on a progressive ratio schedule: implications for models of inter-temporal choice.

    PubMed

    Kheramin, S; Body, S; Herrera, F Miranda; Bradshaw, C M; Szabadi, E; Deakin, J F W; Anderson, I M

    2005-01-01

    In a previous experiment [Kheramin S, Body S, Mobini S, Ho M-Y, Velazquez-Martinez DN, Bradshaw CM, et al. Effects of quinolinic acid-induced lesions of the orbital prefrontal cortex on inter-temporal choice: a quantitative analysis. Psychopharmacology 2002;165: 9-17], destruction of the orbital prefrontal cortex (OPFC) in rats altered choice between two delayed food reinforcers, enhancing preference for the larger reinforcer. Theoretical analysis based on a quantitative model of inter-temporal choice [Ho M-Y, Mobini S, Chiang T-J, Bradshaw CM, Szabadi E. Theory and method in the quantitative analysis of 'impulsive choice' behaviour: implications for psychopharmacology. Psychopharmacology 1999;146:362-72] indicated that the lesion had increased the relative value of the larger of the two reinforcers due to a general reduction of absolute reinforcer value. The present experiment tested this hypothesis using a reinforcement schedule that did not entail either explicit choice or delayed reinforcement. Ten rats received quinolinic acid-induced lesions of the OPFC, and ten rats received sham lesions. The rats were trained under a progressive-ratio schedule of food reinforcement for 60 daily sessions. Response rates in successive ratios were a bitonic (inverted-U) function of ratio size. Analysis of the data using a three-parameter equation derived from a quantitative model of ratio schedule performance [Killeen PR. Mathematical principles of reinforcement. Behav. Brain Sci. 1994;17:105-72] revealed that the parameter specifying hypothetical reinforcer value was significantly lower in the OPFC-lesioned group than in the sham-lesioned group, consistent with the hypothesis that destruction of the OPFC resulted in devaluation of the food reinforcer.

  14. [Langerhans cell histiocytosis in adults].

    PubMed

    Néel, A; Artifoni, M; Donadieu, J; Lorillon, G; Hamidou, M; Tazi, A

    2015-10-01

    Langerhans cell histiocytosis (LCH) is a rare disease characterized by the infiltration of one or more organs by Langerhans cell-like dendritic cells, most often organized in granulomas. The disease has been initially described in children. The clinical picture of LCH is highly variable. Bone, skin, pituitary gland, lung, central nervous system, lymphoid organs are the main organs involved whereas liver and intestinal tract localizations are less frequently encountered. LCH course ranges from a fulminant multisystem disease to spontaneous resolution. Several randomized controlled trials have enable pediatricians to refine the management of children with LCH. Adult LCH has some specific features and poses distinct therapeutic challenges, knowing that data on these patients are limited. Herein, we will provide an overview of current knowledge regarding adult LCH and its management. We will also discuss recent advances in the understanding of the disease, (i.e. the role of BRAF oncogene) that opens the way toward targeted therapies.

  15. [Langerhans cell histiocytosis in adults].

    PubMed

    Néel, A; Artifoni, M; Donadieu, J; Lorillon, G; Hamidou, M; Tazi, A

    2015-10-01

    Langerhans cell histiocytosis (LCH) is a rare disease characterized by the infiltration of one or more organs by Langerhans cell-like dendritic cells, most often organized in granulomas. The disease has been initially described in children. The clinical picture of LCH is highly variable. Bone, skin, pituitary gland, lung, central nervous system, lymphoid organs are the main organs involved whereas liver and intestinal tract localizations are less frequently encountered. LCH course ranges from a fulminant multisystem disease to spontaneous resolution. Several randomized controlled trials have enable pediatricians to refine the management of children with LCH. Adult LCH has some specific features and poses distinct therapeutic challenges, knowing that data on these patients are limited. Herein, we will provide an overview of current knowledge regarding adult LCH and its management. We will also discuss recent advances in the understanding of the disease, (i.e. the role of BRAF oncogene) that opens the way toward targeted therapies. PMID:26150351

  16. Sinus histiocytosis with massive lymphadenopathy.

    PubMed

    Foucar, E; Rosai, J; Dorfman, R F

    1978-12-01

    Sinus histiocytosis with massive lymphadenopathy (SHML) is a newly recognized, distinct, pseudolymphomatous benign entity with very characteristic microscopic features. Most patients are children or young adults with massive painless cervical adenopathy, although other node groups and extranodal sites often are involved. Sixteen patients with SHML involving the upper respiratory tract and/ or salivary gland are presented. Nine of the patients had ear, nose, and throat (ENT) manifestations at the time of presentation. In most cases the ENT involvement resulted in prominent clinical symptoms. Treatment included surgery, antibiotics, irradiation, chemotherapy, and steroids, frequently in combination, but no consistent pattern of response emerged from the study. These 16 patients were very similar clinically to patients with SHML who did not have ENT disease, indicating that extranodal involvement is not associated with more aggressive disease.

  17. The potential cost-effectiveness of the Diamondback 360® Coronary Orbital Atherectomy System for treating de novo, severely calcified coronary lesions: an economic modeling approach

    PubMed Central

    Chambers, Jeffrey; Généreux, Philippe; Lee, Arthur; Lewin, Jack; Young, Christopher; Crittendon, Janna; Mann, Marita; Garrison, Louis P.

    2015-01-01

    Background: Patients who undergo percutaneous coronary intervention (PCI) for severely calcified coronary lesions have long been known to have worse clinical and economic outcomes than patients with no or mildly calcified lesions. We sought to assess the likely cost-effectiveness of using the Diamondback 360® Orbital Atherectomy System (OAS) in the treatment of de novo, severely calcified lesions from a health-system perspective. Methods and results: In the absence of a head-to-head trial and long-term follow up, cost-effectiveness was based on a modeled synthesis of clinical and economic data. A cost-effectiveness model was used to project the likely economic impact. To estimate the net cost impact, the cost of using the OAS technology in elderly (⩾ 65 years) Medicare patients with de novo severely calcified lesions was compared with cost offsets. Elderly OAS patients from the ORBIT II trial (Evaluate the Safety and Efficacy of OAS in Treating Severely Calcified Coronary Lesions) [ClinicalTrials.gov identifier: NCT01092426] were indirectly compared with similar patients using observational data. For the index procedure, the comparison was with Medicare data, and for both revascularization and cardiac death in the following year, the comparison was with a pooled analysis of the Harmonizing Outcomes with Revascularization and Stents in Acute Myocardial Infarction (HORIZONS-AMI)/Acute Catheterization and Urgent Intervention Triage Strategy (ACUITY) trials. After adjusting for differences in age, gender, and comorbidities, the ORBIT II mean index procedure costs were 17% (p < 0.001) lower, approximately US$2700. Estimated mean revascularization costs were lower by US$1240 in the base case. These cost offsets in the first year, on average, fully cover the cost of the device with an additional 1.2% cost savings. Even in the low-value scenario, the use of the OAS is cost-effective with a cost per life-year gained of US$11,895. Conclusions: Based on economic modeling

  18. Langerhans Cell Histiocytosis Followed by Hodgkin Lymphoma: A Case Report

    PubMed Central

    Safaei, Akbar; Bagheri, Mandana; Shahryari, Jahanbanoo; Noori, Sadat; Esmailzade, Elmira

    2015-01-01

    Langerhans cell histiocytosis (LCH) is a rare neoplasm defined as the proliferation of bone marrow langerhans cells, which is a kind of dendritic cells. The major pathological features of LCH are expression of CD1a and S100 as well as Birbeck granules. Its presentation can differ from a mild bone lesion to a multi-systemic evolved malignant neoplasm; however, the latter outcome is almost rare. Thus, LCH is mostly known as a benign neoplasm. In this study, we present a case of LCH followed by Hodgkin lymphoma (HL). Accompaniment of this disease with malignant lymphoma is rare and considered as case report. Several cases in which malignant lymphoma occurred prior to LCH are reported; however, few cases can be found with LCH followed by malignant lymphomas. PMID:25999631

  19. Solitary Extragnathic Langerhans Cell Histiocytosis – A Rare Case

    PubMed Central

    Reddy, Eppalapally Sharath Kumar; Bhavani, Sangala Naga; A, Krishna; Sekhar, Mane Srinivas Muni

    2015-01-01

    Langerhans cell histiocytosis (LCH), mainly affects the skull, vertebrae, ribs and mandible in children and the long bones of adults. Symptoms range from none to pain, swelling and tenderness over the site of the lesion. This disease presents oral manifestations which can sometimes be the first expression of the condition. It occurs in three forms namely eosinophilic granuloma in which isolated or multiple bones are involved, But has a good prognosis whereas other variants Hand-Shuller-Christian disease (chronic dessiminated variant) and Letterer-Siwe disease (acute dessiminated form) have poor prognosis. Occasionally only soft tissues are affected without bony involvement. Males are more commonly affected than females. This article describes a rare variant of eosinophilic granuloma of labial mucosa without bony involvement. PMID:25954715

  20. Langerhans Histiocytosis in a Child – Diagnosed by Oral Manifestations

    PubMed Central

    Golai, Shruti; Patil, Sandya Devi; Kakanur, Madhu; Paul, Santosh

    2015-01-01

    Langerhans Histiocytosis (LCH) is a rare reactive and proliferative disease of histiocytes with unknown etiology, characterized by excessive proliferation of histiocytes called Langerhans cells. It occurs mainly in children but occurrence in adults has also been reported. It manifests as punched out lesions in the skull, maxilla, mandible, sternum and other flat bones and causes rapid resorption of the alveolar bone leading to floating teeth appearance in the radiographs. This disease manifests initially in the oral cavity in most of the cases and can be diagnosed by careful clinical and radiological examination. Here, we present a case of LCH in a child which was diagnosed by a swelling in the mandibular region. PMID:26023652

  1. Long-lasting "christmas tree rash" in an adolescent: isotopic response of indeterminate cell histiocytosis in pityriasis rosea?

    PubMed

    Wollenberg, Andreas; Burgdorf, Walter H C; Schaller, Martin; Sander, Christian

    2002-01-01

    A 13-year-old girl developed a non-pruritic pityriasis rosea-like rash, which did not respond to topical corticosteroids or UV therapy but persisted for 2 years. The lymphohistiocytic infiltrate in the upper dermis showed mononuclear cells immunoreactive with S100, CD68, factor XIIIa and CD1a. Electron microscopic evaluation of these cells demonstrated lamellated dense bodies but no Birbeck granules, lipid vacuoles or cholesterol crystals. Two diagnoses were made: a primarily clinical diagnosis of generalized eruptive histiocytosis and a more cell-biology-based diagnosis of an indeterminate cell histiocytosis. Three years later, the lesions are showing spontaneous resolution, with loss of erythema and flattening. Our patient's indeterminate cells fulfil Rowden's classical definition (dendritically shaped epidermal non-keratinocytes without identifying cytoplasmic features), as well as Zelger's newer definition (cells with features of both macrophages and dendritic cells). A Christmas tree pattern has not been previously described in indeterminate cell histiocytosis. Development of indeterminate cell histiocytosis in the lesions of a healing pityriasis rosea might explain the unusual distribution pattern. The development of a skin disorder at the site of an unrelated, already healed skin disease is known as an isotopic response. Key

  2. Bisphosphonates in Langerhans Cell Histiocytosis: An International Retrospective Case Series

    PubMed Central

    Chellapandian, Deepak; Makras, Polyzois; Kaltsas, Gregory; van den Bos, Cor; Naccache, Lamia; Rampal, Raajit; Carret, Anne-Sophie; Weitzman, Sheila; Egeler, R. Maarten; Abla, Oussama

    2016-01-01

    Background Bone is the most common organ of involvement in patients with Langerhans cell histiocytosis (LCH), which is often painful and associated with significant morbidity from pathological fractures. Current first-line treatments include chemotherapy and steroids that are effective but often associated with adverse effects, whereas the disease may reactivate despite an initial response to first-line agents. Bisphosphonates are osteoclast inhibitors that have shown to be helpful in treating bone lesions of LCH. To date, there are no large international studies to describe their role in treating bone lesions of LCH. Method We conducted a multicenter retrospective review of 13 patients with histologically proven LCH, who had received bisphosphonates either at diagnosis or at disease reactivation. Results Ten patients (77%) had a single system bone disease, and 3 (23%) had bone lesions as part of multisystem disease. Median follow-up time post-bisphosphonate therapy was 4.6 years (range, 0.8 to 8.2 years). Treatment with bisphosphonates was associated with significant pain relief in almost all patients. Twelve (92%) achieved resolution of active bone lesions, and 10 out of them had no active disease for a median of 3.5 years (range, 0.8 to 5 years). One patient did not respond. No major adverse effects were reported in this series. Conclusion Bisphosphonates are well-tolerated drugs that can significantly improve bone pain and induce remission in active bone LCH. Future prospective studies evaluating the role of bisphosphonates in LCH are warranted. PMID:27413525

  3. Neonatal lesions of orbital frontal areas 11/13 in monkeys alter goal-directed behavior but spare fear conditioning and safety signal learning

    PubMed Central

    Kazama, Andy M.; Davis, Michael; Bachevalier, Jocelyne

    2014-01-01

    Recent studies in monkeys have demonstrated that damage to the lateral subfields of orbital frontal cortex (OFC areas 11/13) yields profound changes in flexible modulation of goal-directed behaviors and deficits in fear regulation. Yet, little consideration has been placed on its role in emotional and social development throughout life. The current study investigated the effects of neonatal lesions of the OFC on the flexible modulation of goal-directed behaviors and fear responses in monkeys. Infant monkeys received neonatal lesions of OFC areas 11/13 or sham-lesions during the first post-natal week. Modulation of goal-directed behaviors was measured with a devaluation task at 3–4 and 6–7 years. Modulation of fear reactivity by safety signals was assessed with the AX+/BX− fear-potentiated-startle paradigm at 6–7 years. Similar to adult-onset OFC lesions, selective neonatal lesions of OFC areas 11/13 yielded a failure to modulate behavioral responses guided by changes in reward value, but spared the ability to modulate fear responses in the presence of safety signals. These results suggest that these areas play a critical role in the development of behavioral adaptation during goal-directed behaviors, but not or less so, in the development of the ability to process emotionally salient stimuli and to modulate emotional reactivity using environmental contexts, which could be supported by other OFC subfields, such as the most ventromedial subfields (i.e., areas 14/25). Given similar impaired decision-making abilities and spared modulation of fear after both neonatal lesions of either OFC areas 11 and 13 or amygdala (Kazama et al., 2012; Kazama and Bachevalier, 2013), the present results suggest that interactions between these two neural structures play a critical role in the development of behavioral adaptation; an ability essential for the self-regulation of emotion and behavior that assures the maintenance of successful social relationships. PMID:24624054

  4. Neonatal lesions of orbital frontal areas 11/13 in monkeys alter goal-directed behavior but spare fear conditioning and safety signal learning.

    PubMed

    Kazama, Andy M; Davis, Michael; Bachevalier, Jocelyne

    2014-01-01

    Recent studies in monkeys have demonstrated that damage to the lateral subfields of orbital frontal cortex (OFC areas 11/13) yields profound changes in flexible modulation of goal-directed behaviors and deficits in fear regulation. Yet, little consideration has been placed on its role in emotional and social development throughout life. The current study investigated the effects of neonatal lesions of the OFC on the flexible modulation of goal-directed behaviors and fear responses in monkeys. Infant monkeys received neonatal lesions of OFC areas 11/13 or sham-lesions during the first post-natal week. Modulation of goal-directed behaviors was measured with a devaluation task at 3-4 and 6-7 years. Modulation of fear reactivity by safety signals was assessed with the AX+/BX- fear-potentiated-startle paradigm at 6-7 years. Similar to adult-onset OFC lesions, selective neonatal lesions of OFC areas 11/13 yielded a failure to modulate behavioral responses guided by changes in reward value, but spared the ability to modulate fear responses in the presence of safety signals. These results suggest that these areas play a critical role in the development of behavioral adaptation during goal-directed behaviors, but not or less so, in the development of the ability to process emotionally salient stimuli and to modulate emotional reactivity using environmental contexts, which could be supported by other OFC subfields, such as the most ventromedial subfields (i.e., areas 14/25). Given similar impaired decision-making abilities and spared modulation of fear after both neonatal lesions of either OFC areas 11 and 13 or amygdala (Kazama et al., 2012; Kazama and Bachevalier, 2013), the present results suggest that interactions between these two neural structures play a critical role in the development of behavioral adaptation; an ability essential for the self-regulation of emotion and behavior that assures the maintenance of successful social relationships.

  5. [Neuroimaging of Langerhans cell histiocytosis in the central nervous system of children].

    PubMed

    De La Hoz Polo, M; Rebollo Polo, M; Fons Estupiña, C; Muchart López, J; Cruz Martinez, O

    2015-01-01

    Langerhans cell histiocytosis (LCH) is a rare disease characterized by the accumulation within tissues of anomalous dendritic cells similar to Langerhans cells. The clinical presentation varies, ranging from the appearance of a single bone lesion to multisystemic involvement. Central nervous system (CNS) involvement, manifesting as diabetes insipidus secondary to pituitary involvement, has been known since the original description of the disease. Two types of CNS lesions are currently differentiated. The first, pseudotumoral lesions with infiltration by Langerhans cells, most commonly manifests as pituitary infiltration. The second, described more recently, consists of neurodegenerative lesions of the CNS associated with neurologic deterioration. This second type of lesion constitutes a complication of the disease; however, there is no consensus about the cause of this complication. Our objective was to describe the radiologic manifestations of LCH in the CNS in pediatric patients.

  6. Severe Periodontal Disease Manifested in Chronic Disseminated Type of Langerhans Cell Histiocytosis in a 3-Year Old Child

    PubMed Central

    Srivastava, Vinay Kumar; Bansal, Rajesh; Gupta, Vineeta; Bansal, Manish; Patne, Shashikant

    2014-01-01

    ABSTRACT% Langerhans cell histiocytosis (LCH), previously known as histio-cytosis X, is a rare idiopathic disorder of reticulo-endothelial system with abnormal proliferation of bone marrow derived Langerhans cells along with a variable number of leukocytes, such as eosinophils, neutrophils, lymphocytes and plasma cells. Three years old male child presented with multifocal osteolytic lesions and papulosquamous skin lesions. Clinical and radio-graphic features, such as severe alveolar bone loss, mobility of teeth, precocious eruption of teeth, foating appearance of teeth in orthopantomogram (OPG), osteolytic lesion in skull and cutaneous lesions were highly suggestive of LCH disease. Skin biopsy confirmed a diagnosis of LCH. Induction chemotherapy with oral prednisolone and intravenous vinblastine was started. Child responded well to chemotherapy. The clinical significance of the presented case is to diagnose the case of LCH on the basis of the manifestation of severe periodontal disease as this can be first or only manifestation of LCH. A dentist plays a major role in the multidisciplinary treatment of LCH through routine examination and periodic follow-up. How to cite this article: Bansal M, Srivastava VK, Bansal R, Gupta V, Bansal M, Patne S. Severe Periodontal Disease Manifested in Chronic Disseminated Type of Langerhans Cell Histiocytosis in a 3-Year Old Child. Int J Clin Pediatr Dent 2014;7(3):217-219. PMID:25709306

  7. Selective aspiration or neurotoxic lesions of orbital frontal areas 11 and 13 spared monkeys’ performance on the object discrimination reversal task

    PubMed Central

    Kazama, Andy; Bachevalier, Jocelyne

    2009-01-01

    Damage to the orbital frontal cortex (OFC) has long been associated with reversal learning deficits in several species. In monkeys, this impairment follows lesions that include several OFC subfields. However, the different connectional patterns of OFC subfields together with neuroimaging data in humans have suggested that specific OFC areas play distinctive roles in processing information necessary to guide behavior (Kringelbach and Rolls, 2004; Barbas, 2007; Price, 2007). More specifically, areas 11 and 13 contribute to a sensory network, whereas medial areas 10, 14, and 25 are heavily connected to a visceromotor network. To examine the contribution of areas 11 and 13 to reversal learning, we tested monkeys with selective damage to these two OFC areas on two versions of the ODR task using either 1 or 5 discrimination problems. We compared their performance with that of sham-operated controls and of animals with neurotoxic amygdala lesions, which served as operated controls. Neither damage to areas 11 and 13 nor damage to the amygdala affected performance on the ODR tasks. The results indicate that areas 11 and 13 do not critically contribute to reversal learning and that adjacent damage to OFC subfields (10, 12, 14 and 25) could account for the ODR deficits found in earlier lesion studies. This sparing of reversal learning will be discussed in relation to deficits found in the same animals on tasks that measure behavioral modulation when relative value of affective (positive and negative) stimuli was manipulated. PMID:19261875

  8. Multiple cutaneous histiocytosis in two dogs.

    PubMed

    Thornton, R N; Tisdall, C J

    1988-12-01

    Two cases of canine cutaneous histiocytosis are described. Diagnosis depended on overall consideration of clinical and histopathological features of the disease, as well as its response to anti-inflammatory therapy. No aetiological agent was visible using light and electron microscopy.

  9. Langerhans cell histiocytosis of the sacrum

    PubMed Central

    Hatem, M.A.

    2015-01-01

    Langerhans cell histiocytosis is a rare disease with a wide spectrum of clinical presentations. It is a multisystemic disease with organ system involvement ranging from simple—where it involves only one organ—to widespread progressive disease. Although it can affect any age group, the peak incidence is between 1 and 3 years of age. PMID:27186251

  10. Langerhans Cell Histiocytosis of the Thyroid with Multiple Cervical Lymph Node Involvement Accompanying Metastatic Thyroid Papillary Carcinoma

    PubMed Central

    Ceyran, A. Bahar; Şenol, Serkan; Bayraktar, Barış; Özkanlı, Şeyma; Cinel, Z. Leyla; Aydın, Abdullah

    2014-01-01

    A 37-year-old male case was admitted with goiter. Ultrasonography of thyroid showed a 5 cm cystic nodule in the left lobe with a 1.5 cm solid component. Fine needle aspiration biopsy revealed atypia of undetermined significance or follicular lesion. The patient was operated on. The pathological diagnosis was reported as papillary thyroid carcinoma. The immunohistochemical examination showed multiple foci of Langerhans cell histiocytosis involving both lobes. The patient died due to cardiac arrest with respiratory causes in the early postoperative period. Langerhans cell histiocytosis is a rare primary condition which involves abnormal clonal proliferation of Langerhans cells in various tissues and organs. Thyroid involvement is infrequently seen. Although the etiology is unknown, genetic components may be linked to the disease. It is also associated with a family history of thyroid disease. Papillary thyroid carcinoma is the most common malignant epithelial tumor of the thyroid gland. Langerhans cell histiocytosis presenting with papillary thyroid carcinoma is rare. The privilege of our case is langerhans cell histiocytosis of the thyroid with multiple cervical lymph node involvement accompanying cervical lymph node metastatic thyroid papillary carcinoma. PMID:25349760

  11. Diagnosis and Intralesional Corticotherapy in Oral Ulcers Occurring as the Sole Manifestation of Langerhans Cell Histiocytosis. A Case Report

    PubMed Central

    Gambirazi, Liane; Libório, Tatiana; Nunes, Fábio; Sugaya, Norberto; Migliari, Dante

    2016-01-01

    This article reports a case of oral mucosa lesions as the sole manifestation in Langerhans cell histiocytosis (LCH). This is a very uncommon manifestation of LCH since this disease preferably affects the bones with frequent involvement of the jaws. LCH may also involve other organs, particularly the lungs, liver, lymph nodes, and skin. The highlights of this report are the differential diagnosis, immunohistochemical analysis and, mostly, the therapeutic approach. PMID:27398106

  12. Langerhans Cell Histiocytosis of the Clavicle in an Adult: A Case Report and Review of the Literature

    PubMed Central

    Udaka, Toru; Susa, Michiro; Kikuta, Kazutaka; Nishimoto, Kazumasa; Horiuchi, Keisuke; Sasaki, Aya; Kameyama, Kaori; Nakamura, Masaya; Matsumoto, Morio; Chiba, Kazuhiro; Morioka, Hideo

    2015-01-01

    Langerhans cell histiocytosis (LCH) usually occurs in children under the age of 10 years with a predilection for the skull, spine, rib and humerus. Solitary LCH occurring in an adult clavicle is uncommon with limited reports to date. The lesion in our patient was curetted with the intent to make a diagnosis, which subsequently lead to the remission of the symptom and the disease. At the final follow-up after 1 year, no local recurrence or metastasis is observed. PMID:26600774

  13. Preparatory attention after lesions to the lateral or orbital prefrontal cortex – An event-related potentials study

    PubMed Central

    Funderud, Ingrid; Løvstad, Marianne; Lindgren, Magnus; Endestad, Tor; Due-Tønnessen, Paulina; Meling, Torstein R.; Knight, Robert T.; Solbakk, Anne-Kristin

    2013-01-01

    The prefrontal cortex (PFC) plays a central role in preparatory and anticipatory attentional processes. To investigate whether subregions of the PFC play differential roles in these processes we investigated the effect of focal lesions to either lateral prefrontal (lateral PFC; n=11) or orbitofrontal cortex (OFC; n=13) on the contingent negative variation (CNV), an electrophysiological index of preparatory brain processes. The CNV was studied using a Go/NoGo delayed response task where an auditory S1 signaled whether or not an upcoming visual S2 was a Go or a NoGo stimulus. Neither early (500–1000 ms) nor late (3200–3700 ms) phase Go trial CNV amplitude was reduced for any of the patient groups in comparison to controls. However, the lateral PFC group showed enhanced Go trial early CNV and reduced late CNV Go/NoGo differentiation. These data suggests that normal orienting and evaluation as reflected by the CNV is intact after OFC lesions. The enhanced early CNV after lateral PFC damage may be due to failure in inhibition and the reduced late CNV difference wave confirms a deficit in preparatory attention after damage to this frontal subregion. PMID:23831520

  14. Executive functions after orbital or lateral prefrontal lesions: Neuropsychological profiles and self-reported executive functions in everyday living

    PubMed Central

    LØVSTAD, M.; FUNDERUD, I.; ENDESTAD, T.; DUE-TØNNESSEN, P.; MELING, T. R.; LINDGREN, M.; KNIGHT, R. T.; SOLBAKK, A. K.

    2014-01-01

    Objective This study examined the effects of chronic focal lesions to the lateral prefrontal cortex (LPFC) or orbitofrontal cortex (OFC) on neuropsychological test performance and self-reported executive functioning in everyday living. Methods Fourteen adults with OFC lesions were compared to 10 patients with LPFC injuries and 21 healthy controls. Neuropsychological tests with emphasis on measures of cognitive executive function were administered along with the Behavior Rating Inventory of Executive Functions (BRIEF-A) and a psychiatric screening instrument. Results The LPFC group differed from healthy controls on neuropsychological tests of sustained mental effort, response inhibition, working memory and mental switching, while the BRIEF-A provided more clinically important information on deficits in everyday life in the OFC group compared to the LPFC group. Correlations between neuropsychological test results and BRIEF-A were weak, while the BRIEF-A correlated strongly with emotional distress. Conclusions It was demonstrated that LPFC damage is particularly prone to cause cognitive executive deficit, while OFC injury is more strongly associated with self-reported dysexecutive symptoms in everyday living. The study illustrates the challenge of identifying executive deficit in individual patients and the lack of strong anatomical specificity of the currently employed methods. There is a need for an integrative methodological approach where standard testing batteries are supplemented with neuropsychiatric and frontal-specific rating scales. PMID:22731818

  15. [Secondary biliary cirrhosis in a patient with histiocytosis X].

    PubMed

    Romão, Zita; Pontes, J; Andrade, P; Leitão, M C; Donato, A; Freitas, Diniz

    2002-01-01

    Histiocitose X or Histiocytosis of the Langerhans cells represents a complex spectrum of clinical alterations, resulting from infiltration by anomalous histiocytes of various organs, including the skin, bones, lungs, lymphatic ganglia and liver. Liver disease is rare and the mechanism by which lesions appear is unknown. Cholestasis results from phenomena of sclerosant colangitis, which affects the intrahepatic ducts, or from proliferation of histiocytic cells in the periportal areas. Some patients develop biliar cyrrhosis. The authors present the clinical case of a 62-year-old female patient, hospitalized for chronic cholestasis, diabetes and gallstone in the main bile duct. She had metastatic lesions of the hypophysis and bones, the biopsies of which revealed infiltration by histiocytic cells. Endoscopic Retrograde Colangiopancreatography (E.R.C.P.) revealed dilatation of intrahepatic bile ducts and stenosis of left hepatic duct. A histological study of the hepatic biopsy showed chronic cholestasis and areas of fibrosis, without infiltration by histiocytic granulomas, which were observed in the medular biopsy and in the thyroid nodule cytology. PMID:12025455

  16. Langerhans cell histiocytosis case with dense metaphyseal band sign.

    PubMed

    Kikkawa, Ichiro; Aihara, Toshinori; Morimoto, Akira; Watanabe, Hideaki; Furukawa, Rieko

    2013-02-01

    Eosinophilic granuloma, a type of Langerhans cell histiocytosis, exhibits a classic vertebral collapse, which is called vertebra plana (Calve's disease) and it manifests as a solitary bony lesion. Vertebra plana can cause severe pain in patients. Bisphosphonates (clodronate, pamidronate and zoledronic acid) have been recently used to treat osteolytic bone lesions of LCH. Zoledronic acid has 100 times relative potency that of pamidronate. We report a case of a 10-year-old girl who had zoledronic acid treatment for severe back pain due to vertebra plana. X-ray photographs of the patient's body showed dense metaphyseal band sign, which can be found in lead poisoning, treated leukemia, healing rickets, recovery from scurvy, vitamin D hypervitaminosis, congenital hypothyroidism and hypoparathyroidism. Increased biological potent zoledronic acid deprived her of severe back pain due to vertebra plana and might cause dense metaphyseal band sign of her skeleton. Conclusion; We have cured the severe back pain of a 10-year-old girl case of eosinophilic granuloma with zoledronic acid. After that treatment, X-ray photographs of the patient's body showed dense metaphyseal band sign. There have been few such cases reported until now. PMID:23409985

  17. A Rare Case of Langerhans Cell Histiocytosis of the Skull in an Adult: a Systematic Review

    PubMed Central

    Chiong, Corinna; Jayachandra, Shruti; D. Eslick, Guy; Al-Khawaja, Darweesh; Casikar, Vidyasagar

    2013-01-01

    We report a 41-year old male who presented to the Emergency Department after falling while water-skiing. He had a previous medical history included chronic headaches, which had persisted for the last 2-3 months prior to presentation. Computed tomography of the head showed a small hypersensitivity with a small extra axial collection with a maximum thickness of 1mm. Differential diagnoses included an arachnoid cyst, haemangioma, meningioma or a secondary lesion. A diagnosis of Langerhans Cell Histiocytosis was made based on the histopathology examination and the immunoperoxidase staining. PMID:24179650

  18. Self-regressing S100-negative CD1a-positive cutaneous histiocytosis.

    PubMed

    Tardío, Juan C; Aguado, Marta; Borbujo, Jesús

    2013-06-01

    In the skin, the antigen-presenting cells are mainly represented by Langerhans cells, indeterminate cells, and interstitial dendritic cells, which show distinctive immunophenotype and/or ultrastructure. We report a case of a cutaneous-limited self-regressing histiocytosis with a peculiar immunohistochemical profile (CD1a-positive and S100 protein-negative) that is not observed in any of the known cutaneous antigen-presenting cell or nowadays recognized neoplasm. This lesion is probably related to indeterminate dendritic cell tumors, but very few cases with such immunoprofile have been reported up-to-date, and their exact nosologic position and outcome remain to be clarified.

  19. Langerhans Cell Histiocytosis of the Rib in an Adult: A Case Report

    PubMed Central

    Kim, Sung Hyun; Choi, Moon Young

    2016-01-01

    Single-site, single-system Langerhans cell histiocytosis (LCH) of the rib is one of the rarest causes of bone tumor in adults. Herein, we report a case of a healthy 35-year-old male who presented with upper back pain that was attributed to a solitary osteolytic lesion at the posterolateral aspect of his sixth rib. For diagnostic confirmation and treatment, partial resection of the sixth rib was performed and pathologic finding was consistent with LCH. At the final follow-up after 2 years, no local recurrence or metastasis was observed. PMID:26933424

  20. A case of thymic Langerhans cell histiocytosis with diabetes insipidus as the first presentation.

    PubMed

    Chen, Xiaoyan; Huang, Xiaochun; Qiu, Yuan; Chen, Hanzhang; Fu, Yingyu; Li, Xinchun

    2013-03-01

    Langerhans cell histiocytosis (LCH) is an idiopathic group of reactive proliferative diseases linked to aberrant immunity, pathologically characterized by clonal proliferation of Langerhans cells. LCH rarely involves the thymus. We report a case of thymic LCH with diabetes insipidus as the first presentation, without evidence of myasthenia gravis and without evidenced involvement of the skin, liver, spleen, bones, lungs and superficial lymph nodes. This present case may have important clinical implications. In screening for LCH lesions, attention should be attached to rarely involved sites in addition to commonly involved organs. Follow-up and imageological examination are very important to a final diagnosis.

  1. Oral manifestations of chronic disseminated histiocytosis. A report of 10 cases.

    PubMed

    Mínguez, Ignacio; Mínguez, Juan Manuel; Bonet, Jaime; Peñarrocha, Miguel; Sanchis, José María

    2004-01-01

    Chronic disseminated histiocytosis is a systemic disorder resulting from tumor proliferation of Langerhans-type histiocytic cells. The etiology and pathogenesis are not fully clear, though the clinical manifestations are the result of the accumulation and infiltration of these types of cells in organs and tissues. The present study reports 10 patients (6 boys and 4 girls) with chronic disseminated histiocytosis. The patient age at onset of the disease varied from 4 months to 3.2 years (mean 1.7 years). All patients had oral lesions, and in 5 cases these were the first manifestation of the disease. The most frequent alterations were gingival bleeding (7 cases), aphthae measuring over 1 cm in diameter (6 cases), maxillary osteolytic lesions (6 cases), tooth loss due to expulsive folliculitis (5 cases), oral candidiasis (4 cases), orofacial swelling (3 cases), aphthae measuring under 1 cm in diameter (3 cases), and nonspecific oral pain (2 cases). All the oral lesions disappeared with the treatments prescribed, though some patients developed new outbreaks and exacerbations of the disease.

  2. Indeterminate cell histiocytosis successfully treated with phototherapy

    PubMed Central

    Sotto, Mirian Nacagami; de Campos, Fernando Peixoto Ferraz; Abdo, Andre Neder Ramires; Pereira, Juliana; Sanches, José Antônio; Martins, Jade Cury

    2016-01-01

    First described in 1985, intermediate cell histiocytosis is a rare disorder of the cutaneous dendritic cell group with a varied clinical presentation and evolution. The pathologic substrate is constituted by the proliferation of indeterminate cells (ICs) that are immunophenotypically characterized by the positivity of CD1a, CD68, and faint/focal S100, plus the negativity for CD207 (langerin). The authors present the case of a healthy elderly woman who presented generalized dome-shaped reddish cutaneous nodules over her trunk, neck, face, and extremities over a period of 18 months. A laboratory and imaging work-up ruled out internal involvement. The skin biopsy was consistent with IC histiocytosis. The patient was treated with narrowband ultraviolet B phototherapy, which resulted in an excellent short-term outcome. PMID:27547741

  3. Nail changes in Langerhans cell histiocytosis.

    PubMed

    Jain, S; Sehgal, V N; Bajaj, P

    2000-05-01

    Nail changes in Langerhans cell histiocytosis are distinctly uncommon. Paronychial erythema, swelling and subungual pustules of the fingernails and toenails were cardinal, and were supported by diffuse as well as dense collections of mononuclear Langerhans cells evidenced by microscopic investigation. Oral administration of co-trimoxazole (800 mg sulphamethoxazole + 160 mg trimethoprim) every 12 h, 50 mg/d cyclophosphamide and 80 mg/d predinisolone were the mainstay of treatment, supported by scalp tar shampoo and local betamethasone lotion application.

  4. Cutaneous reactive histiocytosis in dogs: a retrospective evaluation of 32 cases.

    PubMed

    Palmeiro, Brian S; Morris, Daniel O; Goldschmidt, Michael H; Mauldin, Elizabeth A

    2007-10-01

    Thirty-two cases of canine cutaneous histiocytosis were retrospectively evaluated. Median age at onset was 4 years. Lesions included nodules and plaques affecting the head/face, trunk and limbs, and erythema, swelling and depigmentation of the nasal planum/nares. Systemic involvement was not ruled out in all cases. All dogs had complete resolution of dermatological lesions after initial treatment (median 45 days). Initial treatment included prednisone +/- antibiotics (12 of 32 dogs), prednisone and tetracycline/niacinamide (four of 32), prednisone and azathioprine (three of 32), tetracycline/niacinamide +/- vitamin E/essential fatty acids (six of 32), antibiotics +/- antihistamines (three of 32), cyclosporine and ketoconazole (one of 32), topical therapy (two of 32), and no treatment (one of 32). Seventeen dogs received maintenance therapy which consisted of tetracycline/niacinamide +/- vitamin E/essential fatty acids (12 of 17), cyclosporine/ketoconazole (two to three times a week) (two of 17), azathioprine daily (one of 17), prednisone/azathioprine (two times a week) (one of 17), and prednisone daily (one of 17). Median follow up was 25 months. Nine dogs had a recurrence of cutaneous histiocytosis (median days to recurrence 130 days), with seven of nine having more than one recurrence. At study completion, six dogs were deceased (no lesions at the time of death) and 26 of 32 were alive with no lesions. Ten of 26 dogs were on maintenance treatment (eight tetracycline/niacinamide, one azathioprine, one vitamin E). Previous dermatological disease and season had no detectable influence on recurrence. Recurrence was significantly more likely in dogs with nasal planum/nares lesions than dogs without these lesions. Tetracycline/niacinamide was an effective treatment option for dogs in this study population.

  5. Association of both Langerhans cell histiocytosis and Erdheim-Chester disease linked to the BRAFV600E mutation.

    PubMed

    Hervier, Baptiste; Haroche, Julien; Arnaud, Laurent; Charlotte, Frédéric; Donadieu, Jean; Néel, Antoine; Lifermann, François; Villabona, Carles; Graffin, Bruno; Hermine, Olivier; Rigolet, Aude; Roubille, Camille; Hachulla, Eric; Carmoi, Thierry; Bézier, Maud; Meignin, Véronique; Conrad, Marie; Marie, Laurence; Kostrzewa, Elise; Michot, Jean-Marie; Barete, Stéphane; Taly, Valerie; Cury, Karine; Emile, Jean-François; Amoura, Zahir

    2014-08-14

    Histiocytoses are a group of heterogeneous diseases that mostly comprise Langerhans cell histiocytosis (LCH) and non-LCH. The association of LCH with non-LCH is exceptional. We report 23 patients with biopsy-proven LCH associated with Erdheim-Chester disease (ECD) (mixed histiocytosis) and discuss the significance of this association. We compare the clinical phenotypes of these patients with those of 56 patients with isolated LCH and 53 patients with isolated ECD. The average age at diagnosis was 43 years. ECD followed (n = 12) or was diagnosed simultaneously with (n = 11) but never preceded LCH. Although heterogeneous, the phenotype of patients with mixed histiocytosis was closer to that of isolated ECD than to that of isolated LCH (principal component analysis). LCH and ECD improved in response to interferon alpha-2a treatment in only 50% of patients (8 of 16). We found the BRAF(V600E) mutation in 11 (69%) of 16 LCH lesions and in 9 (82%) of 11 ECD lesions. Eight patients had mutations in both ECD and LCH biopsies. Our findings indicate that the association of LCH and ECD is not fortuitous and suggest a link between these diseases involving the BRAF(V600E) mutation.

  6. Association of both Langerhans cell histiocytosis and Erdheim-Chester disease linked to the BRAFV600E mutation.

    PubMed

    Hervier, Baptiste; Haroche, Julien; Arnaud, Laurent; Charlotte, Frédéric; Donadieu, Jean; Néel, Antoine; Lifermann, François; Villabona, Carles; Graffin, Bruno; Hermine, Olivier; Rigolet, Aude; Roubille, Camille; Hachulla, Eric; Carmoi, Thierry; Bézier, Maud; Meignin, Véronique; Conrad, Marie; Marie, Laurence; Kostrzewa, Elise; Michot, Jean-Marie; Barete, Stéphane; Taly, Valerie; Cury, Karine; Emile, Jean-François; Amoura, Zahir

    2014-08-14

    Histiocytoses are a group of heterogeneous diseases that mostly comprise Langerhans cell histiocytosis (LCH) and non-LCH. The association of LCH with non-LCH is exceptional. We report 23 patients with biopsy-proven LCH associated with Erdheim-Chester disease (ECD) (mixed histiocytosis) and discuss the significance of this association. We compare the clinical phenotypes of these patients with those of 56 patients with isolated LCH and 53 patients with isolated ECD. The average age at diagnosis was 43 years. ECD followed (n = 12) or was diagnosed simultaneously with (n = 11) but never preceded LCH. Although heterogeneous, the phenotype of patients with mixed histiocytosis was closer to that of isolated ECD than to that of isolated LCH (principal component analysis). LCH and ECD improved in response to interferon alpha-2a treatment in only 50% of patients (8 of 16). We found the BRAF(V600E) mutation in 11 (69%) of 16 LCH lesions and in 9 (82%) of 11 ECD lesions. Eight patients had mutations in both ECD and LCH biopsies. Our findings indicate that the association of LCH and ECD is not fortuitous and suggest a link between these diseases involving the BRAF(V600E) mutation. PMID:24894769

  7. Pulmonary Langerhans Cell Histiocytosis: Case Series and Literature Review

    PubMed Central

    Wei, Ping; Lu, Hai-Wen; Jiang, Sen; Fan, Li-Chao; Li, Hui-Ping; Xu, Jin-Fu

    2014-01-01

    Abstract Pulmonary Langerhans cell histiocytosis (PLCH) is a rare disease with insidious onset and nonspecific manifestations. The objective of this article was to characterize the clinical manifestations and features of PLCH by retrospectively analyzing clinical data of patients with PLCH in addition to simultaneous review of literature. A retrospective analysis was conducted on clinical data of patients with PLCH (n = 7), whose conditions were diagnosed by biopsy from pulmonary tissue (n = 6) or enlarged lymph nodes in the neck (n = 1) and confirmed by PLCH typical radiological features on computed tomography (CT) scan, between January 2001 and September 2012 at the Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai, China. The review of published reports was made to further emphasize the clinical manifestation and radiological features of PLCH. Long history of cigarette smoking was found in 6 patients. Two patients had recurrent pneumothorax and the other 2 had pulmonary arterial hypertension (World Health Organization group 5 pulmonary hypertension), diagnosed through ultrasonic cardiogram. The nodular shadows were revealed by chest CT scan in 5 patients, cystic shadows in 5 patients, and reticular shadows in 2 patients, as major manifestations, respectively; most of the lesions were located in the middle or upper segments of the lung. The obvious shrank of lesion was found in 1 patient after completely quitting smoking. The pathogenesis of PLCH might be closely associated with smoking. The cystic or nodular lesion was the typical radiological features. Further prospective studies with large sample size are required to further validate the study results and understand the clinical characteristics of PLCH to avoid misdiagnosis. PMID:25415669

  8. A rare case of solitary brain Langerhans cell histiocytosis with intratumoral hemorrhage in a patient affected by Turner syndrome

    PubMed Central

    Granata, Francesca; Morabito, Rosa; Grasso, Giovanni; Alafaci, Elisabetta; Salpietro, Francesco M.; Alafaci, Concetta

    2016-01-01

    Background: Langerhans cell histiocytosis (LCH) is a rare disease involving clonal proliferation of cells with characteristics similar to bone marrow-derived Langerhans cells. The case of a young woman, affected by Turner syndrome and a solitary intraparenchymal LCH associated with an osteolytic lesion of the overlying skull, is presented. Case Description: The patient, with an insidious history of headache and a growing soft mass in the left frontal region, presented with a sudden generalized tonic-clonic epileptic seizure. Neuroradiological investigations showed an osteolytic lesion of the left frontal bone and an underlying brain lesion associated with recent signs of bleeding. The patient was operated on with a complete removal of the lesion. The postoperative course was uneventful. Conclusions: The clinical, neuroradiological, and intraoperative findings are presented, along with a review of the literature. Although rare, LCH should be considered in the differential diagnosis when a scalp lesion occurs with a progressive growing. PMID:27127696

  9. Pulmonary Langerhans Cell Histiocytosis with Lytic Bone Involvement in an Adult Smoker: Regression following Smoking Cessation

    PubMed Central

    Routy, B.; Hoang, J.; Gruber, J.

    2015-01-01

    Langerhans cell histiocytosis (LCH) is a rare myeloid neoplasm characterized by the proliferation and dissemination of histiocytes. These in turn may cause symptoms ranging from isolated, infiltrative lesions to severe multisystem disease. Pulmonary Langerhans cell histiocytosis (PLCH) presents as a localized polyclonal proliferation of Langerhans cells in the lungs causing bilateral cysts and fibrosis. In adults, this rare condition is considered a reactive process associated with cigarette smoking. Recently, clonal proliferation has been reported with the presence of BRAF V600E oncogenic mutation in a subset of PLCH patients. Spontaneous resolution was described; however, based on case series, smoking cessation remains the most effective way to achieve complete remission and prevent long term complications related to tobacco. Herein, we report the case of an adult woman with biopsy-proven PLCH presenting with thoracic (T8) vertebral bone destruction. Both the lung and the bone diseases regressed following smoking cessation, representing a rare case of synchronous disseminated PCLH with bone localization. This observation underscores the contribution of cigarette smoking as a systemic trigger of both pulmonary and extrapulmonary bone lesions. A review of similar cases in the literature is also presented. PMID:25789184

  10. Medication Management of Jaw Lesions for Dental Patients.

    PubMed

    Ogle, Orrett E; Santosh, Arvind Babu Rajendra

    2016-04-01

    Most pathologic lesions of the jaws or of oral mucosa are treated successfully by surgical interventions. For treatment of the central giant cell lesion, aneurysmal bone cysts, histiocytosis of the mandible, hemangioma, odontogenic keratocyst, Paget disease, oral submucous fibrosis, and oral lichen planus, medical management consisting of intralesional injections, sclerosing agents, and systemic bisphosphonates is as successful as surgical procedures with fewer complications. Pharmacology of agents used and protocols are presented. PMID:27040297

  11. Liver involvement of Langerhans’ cell histiocytosis in children

    PubMed Central

    Yi, Xiaoping; Han, Tong; Zai, Hongyan; Long, Xueying; Wang, Xiaoyi; Li, Wenzheng

    2015-01-01

    Objective: Liver involvement is relatively frequent in children with Langerhans cell histiocytosis (LCH). Its features remain poorly defined. Methods: A retrospective study was carried out on 14 hepatic LCH children in our hospital. The Clinicopathological and radiological features of this disease was discussed. Results: The rate of liver involvement in children LCH patients is 51.9%. Majority of the patients were disseminated cases. Hepatomegaly was clinically confirmed in 11 cases (78.6%). Liver function dysfunction was seen in nine (64.3%) children. The association of multi-modal imaging significantly yielded more diagnostic information. There are some imaging characteristics of this disease, CT and MRI could help to assess the staging, extent of the hepatic lesions. We found that liver involvement had a significant impact on survival. Patients treated with systemic chemotherapy earlier from time of diagnosis had a relatively better outcome. Conclusions: The rate of liver involvement in children LCH patients maybe much higher than that of expected. We suggest that clinical and biological liver evaluation and abdominal imaging must be performed regularly onwards to screen every LCH children patient from the time of the initial diagnosis. Patient should be treated with systemic chemotherapy earlier. PMID:26221247

  12. Sinus histiocytosis with massive lymphadenopathy and epidural involvement.

    PubMed

    Haas, R J; Helmig, M S; Meister, P

    1981-01-01

    Sinus histiocytosis with massive lymphadenopathy (SHML) was recognized as a new clinical-pathological entity in 1969. Up to the present 134 cases have been described. The disease is characterized by prominent cervical lymph node enlargment. Microscopic features include marked dilatation of sinuses with intrasinusal histiocytes and lymphophagocytosis. About 70% of the patients reported were affected during the first 2 decades of life. The disease is held to be benign on account of spontaneous resolution in some patients. A follow-up survey of 72 patients showed disappearance of the symptoms 10 years after the original diagnosis in 24 patients. In 42 patients the disease still persisted 6 months to 21 years later. Six patients died, but only one of them as a result of the disease. Extranodal involvement was seen in the orbit, eyelid, respiratory tract, skin, bone, salivary glands, and testis. In two cases, one of which will be reported here, paraparesis resulted from infiltration of the epidural space. Treatment with prednisolone was tried in some cases with excellent results. In our case treatment with prednisolone and vinblastine resulted in the disappearance of the neurological symptoms.

  13. [X-ray diagnosis of histiocytosis X].

    PubMed

    Khomenko, A G; Dmitrieva, L I; Khikkel', Kh G; Stepanian, I E

    1988-01-01

    The results of a dynamic x-ray study of 27 patients suffering from histiocytosis X with lung involvement were analyzed; the study was supplemented by CT in 4 cases. X-ray semiotics of the disease was investigated with relation to its stage. X-ray symptom complexes were defined: interstitial, interstitial-granulomatous, and focal (tumorous). The authors have emphasized the fact that the small focal-cystic and pneumothoracic x-ray variants of the disease, described in literature, are not nosological entities but reflect only its stage and complications.

  14. Solitary Langerhans cell histiocytosis of the hard palate: a diagnostic pitfall

    PubMed Central

    Varsha, Dalal; Kaur, Manveen; Chaudhary, Neena; Siraj, Fouzia

    2016-01-01

    Langerhans cell histiocytosis (LCH) is a relatively rare and unique disease characterized by an abnormal proliferation of immature dendritic cells. It is predominantly seen in children with adults showing less than ten times the incidence compared to childhood. The clinical presentation and organ involvement is highly variable. Oral manifestations generally consist of mucosal ulceration associated with lesions of the underlying bone. Lesions limited to the oral mucosa are rare. We present a case of a 45-year-old male who presented with an ulcer on the hard palate showing histopathologic features of LCH. The present case is a reminder of the possibility of occurrence of this unusual entity in the oral cavity. Appropriate use of immunohistochemistry is advocated to avoid diagnostic pitfalls. PMID:27703428

  15. An unusual form of localized papulonodular cutaneous histiocytosis in a 6-month-old boy.

    PubMed

    van Haselen, C W; Toonstra, J; den Hengst, C W; van Vloten, W A

    1995-09-01

    We report a 6-month-old boy with an unusual form of cutaneous histiocytosis. The lesions were noticed shortly after birth, and there was no evidence of systemic disease. This histiocytic disorder could not be classified according to the Histiocyte Society classification, and was therefore designated an 'unclassified' group II histiocytic disorder. The clinical picture was characterized by dark-red papulonodules with a tendency to coalesce into plaques. Histologically, the infiltrate was characterized by non-epidermotropic histiocytes showing varying degrees of differentiation, eosinophils and lymphocytes, and by the absence of foamy cells and Touton giant cells. As a most conspicuous feature, electron microscopic examination revealed laminated dense bodies, whereas Birbeck granules and comma-shaped bodies were absent. This further distinguished this uncommon variant from the well-known class II histiocytoses. During a 6-month follow-up period all the lesions showed marked regression.

  16. Case of Langerhans Cell Histiocytosis That Mimics Meningioma in CT and MRI

    PubMed Central

    Zhu, Ming; Yu, Bing-Bing; Zhai, Ji-Liang

    2016-01-01

    Langerhans cell histiocytosis (LCH) is a rare disorder histologically characterized by the proliferation of Langerhans cells. Here we present the case of a 13-year-old girl with LCH wherein CT and MRI results led us to an initially incorrect diagnosis of meningioma. The diagnosis was corrected to LCH based on pathology findings. An intracranial mass was found mainly in the dura mater, with thickening of the surrounding dura. It appeared to be growing downward from the calvaria, pressing on underlying brain tissue, and had infiltrated the inner skull, causing a bone defect. The lesion was calcified with the typical dural tail sign. The dural origin of the lesion was verified upon surgical dissection. There are no previous reports in the literature describing LCH of dural origin presenting in young patients with typical dural tail signs and meningioma-like imaging findings. The current case report underscores the need for thorough histological and immunocytochemical examinations in LCH differential diagnosis. PMID:26962425

  17. Eosinophilic granuloma of bone and biochemical demonstration of 49-kDa CD1a molecule expression by Langerhans-cell histiocytosis.

    PubMed

    Cambazard, F; Dezutter-Dambuyant, C; Staquet, M J; Schmitt, D; Thivolet, J

    1991-09-01

    Histiocytic cells infiltrating the lesions in eosinophilic granuloma of bone as well as in cutaneous histiocytosis X were studied using a murine monoclonal antibody (MA) produced with proliferating cells from an eosinophilic granuloma of bone. This MA reacts with Langerhans cells (LC) of normal human skin or mucous membranes and with proliferating cells of eosinophilic granuloma of bone and skin lesions of Letter-Siwe disease, as shown by immunohistochemistry and immunogold labelling. As other murine MA's obtained after immunization with human cortical thymocytes, this MA immunoprecipitates the 49-kDa CD1a antigen found on human LC and thymic-cell surfaces but not its breakdown product after treatment with trypsin, as demonstrated by analysis of immunoelectron labelling, cytofluorometry and gel electrophoresis. This first production of a CD1a MA from an eosinophilic granuloma supports the concept of Langerhans-cell histiocytosis.

  18. Langerhans Cell Histiocytosis in an Adult with Involvement of the Calvarium, Cerebral Cortex and Brainstem: Discussion of Pathophysiology and Rationale for the Use of Intravenous Immune Globulin

    PubMed Central

    Dardis, Christopher; Aung, Thandar; Shapiro, William; Fortune, John; Coons, Stephen

    2015-01-01

    We report a case of Langerhans cell histiocytosis in a 64-year-old male who presented with symptoms and signs of brain involvement, including seizures and hypopituitarism. The diagnosis was confirmed with a biopsy of a lytic skull lesion. The disease affecting the bone showed no sign of progression following a short course of cladribine. Signs of temporal lobe involvement led to an additional biopsy, which showed signs of nonspecific neurodegeneration and which triggered status epilepticus. Lesions noted in the brainstem were typical for the paraneoplastic inflammation reported in this condition. These lesions improved after treatment with cladribine. They remained stable while on treatment with intravenous immune globulin. PMID:25873887

  19. Endocrine changes in histiocytosis of the hypothalamic-pituitary axis.

    PubMed

    Toro Galván, Silvia; Planas Vilaseca, Alejandra; Michalopoulou Alevras, Theodora; Torres Díaz, Alberto; Suárez Balaguer, Javier; Villabona Artero, Carles

    2015-02-01

    Histiocytosis is characterized by proliferation of cells from the mononuclear phagocyte system, and may be divided into Langerhans cell histiocytosis (LCH) and non-Langerhans cell histiocytosis (including Erdheim-Chester disease [ECD]). While diabetes insipidus (DI) is the most common hypothalamic-pituitary consequence, anterior pituitary deficiencies are less known. This study analyzed the frequency and progression of pituitary hormone deficiencies and the radiographic findings in 9 patients (7 with LCH and 2 with ECD) with hypothalamic-pituitary (HP) axis. Eighty-nine percent of patients had DI (62% at diagnosis), and 78% had one or more anterior pituitary deficiencies (71% at diagnosis). HP involvement is relatively common in patients diagnosed with histiocytosis and hormone deficiencies may be present at diagnosis or appear gradually during the course of disease. Regular monitoring of these patients is recommended.

  20. Pulmonary Langerhans Cell Histiocytosis in an Adult Male Presenting with Central Diabetes Insipidus and Diabetes Mellitus: A Case Report

    PubMed Central

    Choi, Yeun Seoung; Lim, Jung Soo; Kwon, Woocheol; Jung, Soon-Hee; Park, Il Hwan; Lee, Myoung Kyu; Lee, Won Yeon; Yong, Suk Joong; Lee, Seok Jeong; Jung, Ye-Ryung; Choi, Jiwon; Choi, Ji Sun; Jeong, Joon Taek; Yoo, Jin Sae

    2015-01-01

    Pulmonary Langerhans cell histiocytosis is an uncommon diffuse cystic lung disease in adults. In rare cases, it can involve extrapulmonary organs and lead to endocrine abnormalities such as central diabetes insipidus. A 42-year-old man presented with polyphagia and polydipsia, as well as a dry cough and dyspnea on exertion. Magnetic resonance imaging of the hypothalamic-pituitary system failed to show the posterior pituitary, which is a typical finding in patients with central diabetes insipidus. This condition was confirmed by a water deprivation test, and the patient was also found to have type 2 diabetes mellitus. Computed tomographic scanning of the lungs revealed multiple, irregularly shaped cystic lesions and small nodules bilaterally, with sparing of the costophrenic angles. Lung biopsy through video-assisted thoracoscopic surgery revealed pulmonary Langerhans cell histiocytosis. On a follow-up visit, only 1 year after the patient had quit smoking, clinical and radiological improvement was significant. Here, we report an uncommon case of pulmonary Langerhans cell histiocytosis that simultaneously presented with diabetes insipidus and diabetes mellitus. PMID:26508947

  1. Pulmonary Langerhans Cell Histiocytosis in an Adult Male Presenting with Central Diabetes Insipidus and Diabetes Mellitus: A Case Report.

    PubMed

    Choi, Yeun Seoung; Lim, Jung Soo; Kwon, Woocheol; Jung, Soon-Hee; Park, Il Hwan; Lee, Myoung Kyu; Lee, Won Yeon; Yong, Suk Joong; Lee, Seok Jeong; Jung, Ye-Ryung; Choi, Jiwon; Choi, Ji Sun; Jeong, Joon Taek; Yoo, Jin Sae; Kim, Sang-Ha

    2015-10-01

    Pulmonary Langerhans cell histiocytosis is an uncommon diffuse cystic lung disease in adults. In rare cases, it can involve extrapulmonary organs and lead to endocrine abnormalities such as central diabetes insipidus. A 42-year-old man presented with polyphagia and polydipsia, as well as a dry cough and dyspnea on exertion. Magnetic resonance imaging of the hypothalamic-pituitary system failed to show the posterior pituitary, which is a typical finding in patients with central diabetes insipidus. This condition was confirmed by a water deprivation test, and the patient was also found to have type 2 diabetes mellitus. Computed tomographic scanning of the lungs revealed multiple, irregularly shaped cystic lesions and small nodules bilaterally, with sparing of the costophrenic angles. Lung biopsy through video-assisted thoracoscopic surgery revealed pulmonary Langerhans cell histiocytosis. On a follow-up visit, only 1 year after the patient had quit smoking, clinical and radiological improvement was significant. Here, we report an uncommon case of pulmonary Langerhans cell histiocytosis that simultaneously presented with diabetes insipidus and diabetes mellitus.

  2. [Xanthomas in a patient with Langerhans cell histiocytosis and liver cirrhosis].

    PubMed

    Calzado, Leticia; Postigo, Concepción; Prado Sánchez-Caminero, M; Sanz, Henar; Guerra, Aurora; Vanaclocha, Francisco; Rodríguez-Peralto, José L

    2005-10-01

    Skin involvement in acute forms of Langerhans cell histiocytosis (LCH) is in the form of erythematous papules, although rare forms of xanthomatous lesions have been described. We present the case of a boy with acute disseminated LCH who, at the age of 16 months, began to experience outbreaks of seborrheic dermatitis-like skin lesions and progressive hepatic dysfunction. The symptoms were complicated by partial central diabetes insipidus and specific pulmonary infiltration by Langerhans cells, which led to fibrosis. During the course of the disease, the patient developed liver cirrhosis, alterations in the lipid profile and disseminated xanthomatous skin lesions, concomitant with the lesions specific to the LCH. Despite successive cycles of chemotherapy, the outcome was the death of the patient after five years, due to his liver disease. Xanthomatous lesions in LCH are typical of the late stages of chronic progressive forms, such as Hand-Schüller-Christian disease. When they appear in acute disseminated forms, there is some controversy over whether they correspond to a progression of the disease towards more chronic forms, or whether they are associated independent lesions, such as in this case.

  3. Histiocytic sarcoma that mimics benign histiocytosis.

    PubMed

    Boisseau-Garsaud, A M; Vergier, B; Beylot-Barry, M; Nastasel-Menini, F; Dubus, P; de Mascarel, A; Eghbali, H; Beylot, C

    1996-06-01

    A 28-year-old man presented with a histiocytic sarcoma of a very uncommon origin, as it had developed for several years like a benign cutaneous histiocytosis resembling generalized eruptive histiocytoma before becoming acute, with nodal and massive pulmonary involvement. Despite various chemotherapies, the patient died within 8 months. Skin biopsies showed histiocytic proliferation in the dermis and node biopsies showed histiocytic proliferation with a sinusoidal pattern. Immunohistochemical analysis, performed on paraffin-embedded sections, demonstrated strong labeling of tumoral cells for CD68 and moderate labeling for CD3 and CD4. CD30 labeling was negative. S-100 protein was positive on a Langerhans' cell reactive subpopulation. Electron microscopy confirmed the histiocytic nature of malignant cells and showed cytoplasmic inclusions such as regularly laminated bodies, dense bodies and pleomorphic inclusions. No Birbeck granules were seen. A gene rearrangement study of T-cell receptor gamma and immunoglobulin heavy chain genes showed a germline configuration. Histiocytic sarcoma is an extremely rare true histiocytic malignancy, the existence of which has been recently debated since it has often been mistaken in the past for large cell lymphomas. Such a deceptive onset as benign cutaneous histiocytosis has not been described in the literature to our knowledge.

  4. The indeterminate cell proliferative disorder: report of a case manifesting as an unusual cutaneous histiocytosis.

    PubMed

    Wood, G S; Hu, C H; Beckstead, J H; Turner, R R; Winkelmann, R K

    1985-11-01

    A patient with an unusual, distinctive cutaneous histiocytosis is described. Extensive morphologic, antigenic, and enzymatic studies indicate that this histiocytosis represents a proliferative disorder of cutaneous indeterminate cells. Features that distinguish this disorder from other histiocytoses are discussed.

  5. [Multifocal Langerhans cell histiocytosis of bone: late revelation in a 76-year-old woman].

    PubMed

    Lahiani, D; Hammami, B K; Maâloul, I; Frikha, M; Baklouti, S; Jlidi, R; Ben Jemaâ, M

    2008-03-01

    Langerhans cell histiocytosis or histiocytosis X has a variable course from a self-limited eosinophilic granuloma to an aggressive disseminated disease. It mainly affects children. We report a 76-year-old woman with multifocal bone histiocytosis X, involving the rachis, an iliac bone and the skull. The diagnosis has been established by histological exam. Outcome was favourable after chemotherapy.

  6. [Sinus histiocytosis with massive lymphadenopathy or the Destombes-Rosai-Dorfman disease in the Ivory Coast].

    PubMed

    Soubeyrand, J; Clerc, M; Rain, J D; Leleu, J P; Niamkey, E; Diallo, D; Raoul, D; Beda, B Y

    1984-04-01

    Sinusal histiocytosis with massive lymphadenopathy is a rare pathological entity since, as of 1983, only 200 cases have been published. Following the seminal description in 1965 by Destombes in Blacks, histological features of this disease were specified by Rosai and Dorfman in 1969 and 1972. We report three cases in young Ivorian subjects. The presenting finding is always chronically enlarged lymph nodes, but extranodal lesions are possible, most commonly involving the eye, salivary glands, upper respiratory tract, skin, bone, testis, and nervous system. Diagnosis can be ascertained only upon histologic examination of lymph node biopsy specimens which shows the three cardinal criteria, i.e. massive sinusal histiocytosis, lymphophagocytosis, and mature plasmocytosis. Although a few fatal cases have been reported, the disease usually runs a benign course, with exacerbations of variable duration. Great caution should therefore be taken in deciding upon management, particularly as regards corticosteroid therapy, radiotherapy or anticancer chemotherapy. Etiopathogeny of the disease is unsettled; there is general agreement as to the existence of a cellular immune dysfunction resulting in the lympho-histiocytic proliferation.

  7. Efficacy of vinblastine in central nervous system Langerhans cell histiocytosis: a nationwide retrospective study

    PubMed Central

    2011-01-01

    Background Vinblastine (VBL) is the standard treatment for systemic Langerhans cell histiocytosis (LCH), but little is known about its efficacy in central nervous system (CNS) mass lesions. Methods A retrospective chart review was conducted. Twenty patients from the French LCH Study Group register met the inclusion criteria. In brief, they had CNS mass lesions, had been treated with VBL, and were evaluable for radiologic response. Results The median age at diagnosis of LCH was 11.5 years (range: 1-50). Intravenous VBL 6 mg/m2 was given in a 6-week induction treatment, followed by a maintenance treatment. The median total duration was 12 months (range: 3-30). Eleven patients received steroids concomitantly. Fifteen patients achieved an objective response; five had a complete response (CR: 25%), ten had a partial response (PR: 50%), four had stable disease (SD: 20%) and one patient progressed (PD: 5%). Of interest, four out of the six patients who received VBL without concomitant steroids achieved an objective response. With a median follow-up of 6.8 years, the 5-year event-free and overall survival was 61% and 84%, respectively. VBL was well-tolerated and there were no patient withdrawals due to adverse events. Conclusion VBL, with or without steroids, could potentially be a useful therapeutic option in LCH with CNS mass lesions, especially for those with inoperable lesions or multiple lesions. Prospective clinical trials are warranted for the evaluation of VBL in this indication. PMID:22151964

  8. Multifocal Langerhans cell histiocytosis in an adult with a pathological fracture of the mandible and spontaneous malunion: A case report

    PubMed Central

    SHI, SAILANG; LIU, YANMING; FU, TAO; LI, XIUZHEN; ZHAO, SHIFANG

    2014-01-01

    Langerhans cell histiocytosis (LCH) is rare in the adult population and even rarer with jaw involvement. The current study presents the case of a 39-year-old male who complained of recurrent pain, swelling of the gingiva and an occasional pus-like discharge in the right mandible for one year. The patient was previously prescribed antibiotics, but this did not resolve the problem. An initial panoramic radiograph showed an osteolytic lesion and bone fracture in the right mandible. Eight months later, a new radiograph showed the spontaneous malunion of the fractured mandible. The patient was eventually diagnosed with Langerhans cell histiocytosis by histopathology and immunohistochemistry. Further lesions were found in the ribs and ilium by nuclear bone scanning. The patient was subsequently treated with systemic chemotherapy, and the lesions are currently effectively being controlled. This study is the first to show that spontaneous intralesional bone regeneration may lead to reunification of the mandible fracture caused by LCH in an adult. PMID:25120660

  9. Early Diagnosis and Monitoring of Neurodegenerative Langerhans Cell Histiocytosis

    PubMed Central

    Mortilla, Marzia; Savelli, Sara; Grisotto, Laura; Di Giacomo, Gianpiero; Romano, Katiuscia; Fonda, Claudio; Biggeri, Annibale; Guerrini, Renzo; Aricò, Maurizio

    2015-01-01

    Background Neurodegenerative Langerhans Cell Histiocytosis (ND-LCH) is a rare, unpredictable consequence that may devastate the quality of life of patients cured from LCH. We prospectively applied a multidisciplinary diagnostic work-up to early identify and follow-up patients with ND-LCH, with the ultimate goal of better determining the appropriate time for starting therapy. Methods We studied 27 children and young adults with either ND-LCH verified by structural magnetic resonance imaging (MRI) (group 1) or specific risk factors for (diabetes insipidus, craniofacial bone lesions), but no evidence of, neurodegenerative MRI changes (group 2). All patients underwent clinical, neurophysiological and MRI studies. Results Seventeen patients had MRI alterations typical for ND-LCH. Nine showed neurological impairment but only three were symptomatic; 11 had abnormal somatosensory evoked potentials (SEPs), and five had abnormal brainstem auditory evoked potentials (BAEPs). MR spectroscopy (MRS) showed reduced cerebellar NAA/Cr ratio in nine patients. SEPs showed sensitivity, specificity, positive predictive value (PPV) and negative predictive value (NPV) for predicting ND-LCH of 70.6% (95%CI, 44.0%-89.7%), 100% (69.2%-100%), 100% (73.5%-100%), and 66.7% (38.4%-88.2%), respectively. Repeated investigations in group 1 revealed increasingly abnormal EP parameters, or neurological examination, or both, in nine of fifteen patients while MRI remained unchanged in all but one patient. Conclusion A targeted MRI study should be performed in all patients with risk factors for ND-LCH for early identification of demyelination. The combined use of SEPs and careful neurological evaluation may represent a valuable, low-cost, well-tolerated and easily available methodology to monitor patients from pre-symptomatic to symptomatic stages. We suggest a multidisciplinary protocol including clinical, MRS, and neurophysiological investigations to identify a population target for future

  10. Cytomegalovirus and Langerhans Cell Histiocytosis: Is There a Link?

    PubMed Central

    Khoddami, Maliheh; Nadji, Seyed-Alireza; Dehghanian, Paria; Vahdatinia, Mahsa; Shamshiri, Ahmad-Reza

    2016-01-01

    Background: Langerhans cell histiocytosis is a rare proliferative histiocytic disease of unknown etiology. Histologically, it is characterized by granuloma-like proliferation of Langerhans-type dendritic cells derived from bone marrow. Many investigators have suggested the possible role of viruses such as Epstein-Barr virus, human herpesvirus-6 (HHV-6), herpes simplex virus (HSV) types 1 and 2, and Cytomegalovirus in the pathogenesis of Langerhans cell histiocytosis. Objectives: In this study, we have investigated the presence of Cytomegalovirus in Langerhans cell histiocytosis in Iranian children. Patients and Methods: In this retrospective study, we have investigated the presence of Cytomegalovirus DNA expression, using paraffin-embedded tissue samples of 30 patients with Langerhans cell histiocytosis and 30 age and site-matched controls by qualitative Polymerase Chain Reaction (PCR) method. Results: No significant difference in prevalence of Cytomegalovirus presence between patients and controls was found. Cytomegalovirus was found by qualitative PCR in only 2 (6.66%) out of 30 patients and in 1 (3.3%) of 30 control samples with a P value of 1 (1.00 > 0.05) using chi-square test with OR: 2.07; 95% CI of OR: 0.18 - 24.15. Conclusions: Our findings do not support the hypothesis of a possible role for Cytomegalovirus in the pathogenesis of Langerhans cell histiocytosis. PMID:27307972

  11. Radiographic and Pathologic Manifestations of Uncommon and Rare Pulmonary Lesions.

    PubMed

    Pfeifer, Kyle; Mian, Ali; Adebowale, Adeniran; Alomari, Ahmed; Kalra, Vivek; Krejci, Elise; Shin, Myung Soo

    2016-05-01

    Pulmonary opacities/nodules are common findings on computed tomography examinations, which may represent an underlying infections or malignancy. However, not every pulmonary nodule or opacity represents malignancy or infection. We present a pictorial essay illustrating common as well as obscure noninfectious, nonmalignant pulmonary lesions. Lesions discussed include organizing pneumonia, Langerhans cell histiocytosis, pulmonary amyloidosis, hyalinizing granuloma, tumourlet (benign localized neuroendocrine cell proliferations), atypical alveolar hyperplasia, inflammatory myofibroblastic tumour, papillary alveolar adenoma, plasma cell granuloma, juvenile xanthogranuloma, and sclerosing hemangiomas. We discuss the clinical presentation, prevalence, radiographic clues, pathology, and diagnostic pitfalls of these rare lesions. PMID:26690551

  12. [Langerhans cell histiocytosis with atypical and early neonatal debut].

    PubMed

    García-Rodríguez, Esther; Bernabeu-Wittel, José; Calderón-López, Gemma; Pavón-Delgado, Antonio

    2016-04-01

    Langerhans cell histiocytosis is a systemic disease associated with the proliferation of this type of cells in tissues. Its prevalence is estimated at 1-9/100 000. Bone is the most frequently affected organ, followed by the skin, lymph nodes, haematopoietic system, pituitary gland, lungs and liver. In the majority of cases, onset occurs during childhood, with peak between one and three years of age, and poor prognosis before two years of age. The haematological forms (pancytopenia) are usually aggressive in infants. We report a case of Langerhans cell histiocytosis with neonatal onset and complex diagnosis: maintained and significant leukocytosis was the predominant data for the first two months of life, so some type of leukemia was considered. However, the most common blood disorder in Langerhans cell histiocytosis is pancytopenia rather than leukocytosis, so that the diagnosis was delayed.

  13. Tertiary lymphoid structures are confined to patients presenting with unifocal Langerhans Cell Histiocytosis.

    PubMed

    Quispel, Willemijn T; Steenwijk, Eline C; van Unen, Vincent; Santos, Susy J; Koens, Lianne; Mebius, Reina; Egeler, R Maarten; van Halteren, Astrid G S

    2016-08-01

    Langerhans cell histiocytosis (LCH) is a neoplastic myeloid disorder with a thus far poorly understood immune component. Tertiary lymphoid structures (TLS) are lymph node-like entities which create an immune-promoting microenvironment at tumor sites. We analyzed the presence and clinical relevance of TLS in n = 104 H&E-stained, therapy-naive LCH lesions of non-lymphoid origin and applied immunohistochemistry to a smaller series. Lymphoid-follicular aggregates were detected in 34/104 (33%) lesions. In line with the lymphocyte recruitment capacity of MECA-79(+) high endothelial venules (HEVs), MECA-79(+)-expressing-LCH lesions (37/77, 48%) contained the most CD3(+) T-lymphocytes (p = 0.003). TLS were identified in 8/15 lesions and contained T-and B-lymphocytes, Follicular Dendritic Cells (FDC), HEVs and the chemokines CXCL13 and CCL21 representing key cellular components and TLS-inducing factors in conventional lymph nodes (LN). Lymphoid-follicular aggregates were most frequently detected in patients presenting with unifocal LCH (24/70, 34%) as compared to patients with poly-ostotic or multi-system LCH (7/30, 23%, p = 0.03). In addition, patients with lymphoid-follicular aggregates-containing lesions had the lowest risk to develop new LCH lesions (p = 0.04). The identification of various stages of TLS formation within LCH lesions may indicate a key role for the immune system in controlling aberrant histiocytes which arise in peripheral tissues.

  14. Tertiary lymphoid structures are confined to patients presenting with unifocal Langerhans Cell Histiocytosis.

    PubMed

    Quispel, Willemijn T; Steenwijk, Eline C; van Unen, Vincent; Santos, Susy J; Koens, Lianne; Mebius, Reina; Egeler, R Maarten; van Halteren, Astrid G S

    2016-08-01

    Langerhans cell histiocytosis (LCH) is a neoplastic myeloid disorder with a thus far poorly understood immune component. Tertiary lymphoid structures (TLS) are lymph node-like entities which create an immune-promoting microenvironment at tumor sites. We analyzed the presence and clinical relevance of TLS in n = 104 H&E-stained, therapy-naive LCH lesions of non-lymphoid origin and applied immunohistochemistry to a smaller series. Lymphoid-follicular aggregates were detected in 34/104 (33%) lesions. In line with the lymphocyte recruitment capacity of MECA-79(+) high endothelial venules (HEVs), MECA-79(+)-expressing-LCH lesions (37/77, 48%) contained the most CD3(+) T-lymphocytes (p = 0.003). TLS were identified in 8/15 lesions and contained T-and B-lymphocytes, Follicular Dendritic Cells (FDC), HEVs and the chemokines CXCL13 and CCL21 representing key cellular components and TLS-inducing factors in conventional lymph nodes (LN). Lymphoid-follicular aggregates were most frequently detected in patients presenting with unifocal LCH (24/70, 34%) as compared to patients with poly-ostotic or multi-system LCH (7/30, 23%, p = 0.03). In addition, patients with lymphoid-follicular aggregates-containing lesions had the lowest risk to develop new LCH lesions (p = 0.04). The identification of various stages of TLS formation within LCH lesions may indicate a key role for the immune system in controlling aberrant histiocytes which arise in peripheral tissues. PMID:27622056

  15. Localized Langerhans cell histiocytosis masquerading as Brodie's abscess in a 2-year-old child: a case report

    PubMed Central

    Chang, Wei-Fang; Hsu, Yi-Chih; Wu, Yi-Der; Kuo, Chun-Lang; Huang, Guo-Shu

    2016-01-01

    Langerhans cell histiocytosis (LCH), formerly known as histiocytosis X, refers to a spectrum of diseases characterized by idiopathic proliferation of histiocytes that produce either focal (localized LCH) or systemic manifestations (Hand-Schüller-Christian disease and Letterer-Siwe disease). Localized LCH accounts for approximately 60-70 % of all LCH cases. Osseous involvement is the most common manifestation and typically involves the flat bones, along with lesions of the skull, pelvis, and ribs. Localized LCH in bone shows a wide spectrum of clinical manifestations and radiologic features that may mimic those of infections as well as benign and malignant tumors. The diagnostic imaging findings of localized LCH are also diverse and challenging. The penumbra sign is a common and characteristic magnetic resonance imaging (MRI) feature of Brodie's abscess, but is rarely seen in localized LCH. In this report, we describe a case of localized LCH misdiagnosed as Brodie's abscess in a 2-year-old child based on clinical symptoms, laboratory findings, and pre-diagnostic MRI findings (penumbra sign). Therefore, the penumbra sign is not sufficient to clearly establish the diagnosis of Brodie's abscess, and the differential diagnosis of localized LCH should be considered when a child with an osteolytic lesion presents with a penumbra sign. PMID:27065773

  16. Localized Langerhans cell histiocytosis masquerading as Brodie's abscess in a 2-year-old child: a case report.

    PubMed

    Chang, Wei-Fang; Hsu, Yi-Chih; Wu, Yi-Der; Kuo, Chun-Lang; Huang, Guo-Shu

    2016-01-01

    Langerhans cell histiocytosis (LCH), formerly known as histiocytosis X, refers to a spectrum of diseases characterized by idiopathic proliferation of histiocytes that produce either focal (localized LCH) or systemic manifestations (Hand-Schüller-Christian disease and Letterer-Siwe disease). Localized LCH accounts for approximately 60-70 % of all LCH cases. Osseous involvement is the most common manifestation and typically involves the flat bones, along with lesions of the skull, pelvis, and ribs. Localized LCH in bone shows a wide spectrum of clinical manifestations and radiologic features that may mimic those of infections as well as benign and malignant tumors. The diagnostic imaging findings of localized LCH are also diverse and challenging. The penumbra sign is a common and characteristic magnetic resonance imaging (MRI) feature of Brodie's abscess, but is rarely seen in localized LCH. In this report, we describe a case of localized LCH misdiagnosed as Brodie's abscess in a 2-year-old child based on clinical symptoms, laboratory findings, and pre-diagnostic MRI findings (penumbra sign). Therefore, the penumbra sign is not sufficient to clearly establish the diagnosis of Brodie's abscess, and the differential diagnosis of localized LCH should be considered when a child with an osteolytic lesion presents with a penumbra sign.

  17. [THE SPONTANEOUS PNEUMOTHORAX INTERPRETATION, DIFFICULTIES DURING HISTIOCYTOSIS X].

    PubMed

    Duzhyi, I D; Holubnychyi, S A; Holubnucha, V N; Pustovoy, I A

    2014-01-01

    Since spontaneous pneumothorax can be a complication, and sometimes a manifestation of significant amount of intrathoracic and extrathoracic diseases, verifying the process can be very difficult. One of these diseases is the local (pulmonary) histiocytosis X. Authors cite the etiological diagnosis experience of spontaneous pneumothorax during current pathology. According to their data, spontaneous pneumothorax on the histiocytosis X background found in 3 patients. Complications developed during the disease later stages, and therefore the verification was complex and based on morphological data, consistent with the clinical and radiographic changes.

  18. The orbits in cancer imaging

    PubMed Central

    Chong, V F H

    2006-01-01

    Primary malignant lesions in the orbit are relatively uncommon. However, the orbits are frequently involved in haematogeneous metastasis or by direct extension from malignancies originating from the adjacent nasal cavity or paranasal sinuses. This paper focuses on the more commonly encountered primary orbital malignancies and the mapping of tumour spread into the orbits. PMID:17114076

  19. Langerhans cell histiocytosis: literature review and descriptive analysis of oral manifestations.

    PubMed

    Madrigal-Martínez-Pereda, Cristina; Guerrero-Rodríguez, Vanesa; Guisado-Moya, Blanca; Meniz-García, Cristina

    2009-05-01

    Langerhans cell histiocytosis (LCH) is a rare disease, of unknown pathogenesis, characterized by intense and abnormal proliferation of bone marrow-derived histiocytes (Langerhans cells). It can present both local and systemic manifestations involving bone, skin and mucosal tissue, and internal organs. Three basic clinical forms develop: Letterer-Siwe disease (subacute or acute disseminated form), Hand-Schüller-Christian disease (disseminated chronic form) and eosinophilic granuloma (localized chronic form). LCH may manifest orally with single or multiple lesions of the alveolar or basal bone, ulcerated mucosal lesions accompanied by adenopathies and/or periodontal lesions, presenting gingival inflammation, bleeding, recession, necrosis, odontalgia, dental hypermobility and premature loss of teeth. The principal differential diagnoses include advanced periodontal disease or a periapical process of dental or periodontal origin. The odontologist plays a vital role in the diagnosis and multidisciplinary treatment of such patients, by performing routine examinations for periodic follow-up of the disease and its possible oral manifestations, bearing in mind that these may be the first or only signs of LCH.

  20. Langerhans cell histiocytosis: literature review and descriptive analysis of oral manifestations.

    PubMed

    Madrigal-Martínez-Pereda, Cristina; Guerrero-Rodríguez, Vanesa; Guisado-Moya, Blanca; Meniz-García, Cristina

    2009-05-01

    Langerhans cell histiocytosis (LCH) is a rare disease, of unknown pathogenesis, characterized by intense and abnormal proliferation of bone marrow-derived histiocytes (Langerhans cells). It can present both local and systemic manifestations involving bone, skin and mucosal tissue, and internal organs. Three basic clinical forms develop: Letterer-Siwe disease (subacute or acute disseminated form), Hand-Schüller-Christian disease (disseminated chronic form) and eosinophilic granuloma (localized chronic form). LCH may manifest orally with single or multiple lesions of the alveolar or basal bone, ulcerated mucosal lesions accompanied by adenopathies and/or periodontal lesions, presenting gingival inflammation, bleeding, recession, necrosis, odontalgia, dental hypermobility and premature loss of teeth. The principal differential diagnoses include advanced periodontal disease or a periapical process of dental or periodontal origin. The odontologist plays a vital role in the diagnosis and multidisciplinary treatment of such patients, by performing routine examinations for periodic follow-up of the disease and its possible oral manifestations, bearing in mind that these may be the first or only signs of LCH. PMID:19218906

  1. Occult Langerhans Cell Histiocytosis Presenting with Papillary Thyroid Carcinoma, a Thickened Pituitary Stalk and Diabetes Insipidus.

    PubMed

    Gordon, Michael S; Gordon, Murray B

    2016-01-01

    Etiologies of a thickened stalk include inflammatory, neoplastic, and idiopathic origins, and the underlying diagnosis may remain occult. We report a patient with a thickened pituitary stalk (TPS) and papillary thyroid carcinoma (PTC) whose diagnosis remained obscure until a skin lesion appeared. The patient presented with PTC, status postthyroidectomy, and I(131) therapy. PTC molecular testing revealed BRAF mutant (V600E, GTC>GAG). She had a 5-year history of polyuria/polydipsia. Overnight dehydration study confirmed diabetes insipidus (DI). MRI revealed TPS with loss of the posterior pituitary bright spot. Evaluation showed hypogonadotropic hypogonadism and low IGF-1. Chest X-ray and ACE levels were normal. Radiographs to evaluate for extrapituitary sites of Langerhans Cell Histiocytosis (LCH) were unremarkable. Germinoma studies were negative: normal serum and CSF beta-hCG, alpha-fetoprotein, and CEA. Three years later, the patient developed vulvar labial lesions followed by inguinal region skin lesions, biopsy of which revealed LCH. Reanalysis of thyroid pathology was consistent with concurrent LCH, PTC, and Hashimoto's thyroiditis within the thyroid. This case illustrates that one must be vigilant for extrapituitary manifestations of systemic diseases to diagnose the etiology of TPS. An activating mutation of the protooncogene BRAF is a potential unifying etiology of both PTC and LCH. PMID:27656301

  2. Occult Langerhans Cell Histiocytosis Presenting with Papillary Thyroid Carcinoma, a Thickened Pituitary Stalk and Diabetes Insipidus

    PubMed Central

    2016-01-01

    Etiologies of a thickened stalk include inflammatory, neoplastic, and idiopathic origins, and the underlying diagnosis may remain occult. We report a patient with a thickened pituitary stalk (TPS) and papillary thyroid carcinoma (PTC) whose diagnosis remained obscure until a skin lesion appeared. The patient presented with PTC, status postthyroidectomy, and I131 therapy. PTC molecular testing revealed BRAF mutant (V600E, GTC>GAG). She had a 5-year history of polyuria/polydipsia. Overnight dehydration study confirmed diabetes insipidus (DI). MRI revealed TPS with loss of the posterior pituitary bright spot. Evaluation showed hypogonadotropic hypogonadism and low IGF-1. Chest X-ray and ACE levels were normal. Radiographs to evaluate for extrapituitary sites of Langerhans Cell Histiocytosis (LCH) were unremarkable. Germinoma studies were negative: normal serum and CSF beta-hCG, alpha-fetoprotein, and CEA. Three years later, the patient developed vulvar labial lesions followed by inguinal region skin lesions, biopsy of which revealed LCH. Reanalysis of thyroid pathology was consistent with concurrent LCH, PTC, and Hashimoto's thyroiditis within the thyroid. This case illustrates that one must be vigilant for extrapituitary manifestations of systemic diseases to diagnose the etiology of TPS. An activating mutation of the protooncogene BRAF is a potential unifying etiology of both PTC and LCH. PMID:27656301

  3. Occult Langerhans Cell Histiocytosis Presenting with Papillary Thyroid Carcinoma, a Thickened Pituitary Stalk and Diabetes Insipidus

    PubMed Central

    2016-01-01

    Etiologies of a thickened stalk include inflammatory, neoplastic, and idiopathic origins, and the underlying diagnosis may remain occult. We report a patient with a thickened pituitary stalk (TPS) and papillary thyroid carcinoma (PTC) whose diagnosis remained obscure until a skin lesion appeared. The patient presented with PTC, status postthyroidectomy, and I131 therapy. PTC molecular testing revealed BRAF mutant (V600E, GTC>GAG). She had a 5-year history of polyuria/polydipsia. Overnight dehydration study confirmed diabetes insipidus (DI). MRI revealed TPS with loss of the posterior pituitary bright spot. Evaluation showed hypogonadotropic hypogonadism and low IGF-1. Chest X-ray and ACE levels were normal. Radiographs to evaluate for extrapituitary sites of Langerhans Cell Histiocytosis (LCH) were unremarkable. Germinoma studies were negative: normal serum and CSF beta-hCG, alpha-fetoprotein, and CEA. Three years later, the patient developed vulvar labial lesions followed by inguinal region skin lesions, biopsy of which revealed LCH. Reanalysis of thyroid pathology was consistent with concurrent LCH, PTC, and Hashimoto's thyroiditis within the thyroid. This case illustrates that one must be vigilant for extrapituitary manifestations of systemic diseases to diagnose the etiology of TPS. An activating mutation of the protooncogene BRAF is a potential unifying etiology of both PTC and LCH.

  4. Strategies for the Prevention of Central Nervous System Complications in Patients with Langerhans Cell Histiocytosis: The Problem of Neurodegenerative Syndrome.

    PubMed

    Imashuku, Shinsaku; Arceci, Robert J

    2015-10-01

    Diseases of the central nervous system (CNS) are common in patients with Langerhans cell histiocytosis (LCH). Besides active LCH lesions, neurodegenerative (ND) lesions of the cerebellum and/or basal ganglia may occur as late sequelae of LCH. While the etiology of this ND disease remains unclear, biomarkers in cerebrospinal fluid (CSF) may reflect the activity of CNS disease in these patients. However, no well-planned CSF studies have yet been performed in patients at high risk for ND-CNS-LCH. Potential parallels with other neuroinflammatory/neurodegenerative disease suggest the utility of examining these other disorders in establishing strategies for the prevention and/or treatment of ND-CNS-LCH.

  5. Langerhans' cell histiocytosis involving posterior elements of the dorsal spine: An unusual cause of extradural spinal mass in an adult.

    PubMed

    Tyagi, Devendra K; Balasubramaniam, Srikant; Savant, Hemant V

    2011-07-01

    Langerhans cell histiocytosis (LCH) is a clonal proliferation of Langerhans cells occurring as an isolated lesion or as part of a systemic proliferation. It is commoner in children younger than 10 years of age with sparing of the posterior elements in more than 95% of cases. We describe a case of LCH in an adult female presenting with paraplegia. MRI revealed a well-defined extradural contrast enhancing mass at D2-D4 vertebral level involving the posterior elements of spine. D2-5 laminectomy with excision of lesion was performed which lead to marked improvement of patients neurological status. Histopathology was suggestive of eosinophilic granuloma. We describe the case, discuss its uniqueness and review the literature on this rare tumor presentation.

  6. Adult Onset of BRAFV600E-Mutated Langerhans Cell Histiocytosis with Cutaneous Involvement Successfully Diagnosed by Immunohistochemical Staining

    PubMed Central

    Tono, Hisayuki; Fujimura, Taku; Kakizaki, Aya; Furudate, Sadanori; Ishibashi, Masaya; Aiba, Setsuya

    2015-01-01

    Langerhans cell histiocytosis (LCH) is characterized by the clonal proliferation of Langerhans cells; it is categorized as a single-system disease with single or multifocal lesions, and as a multi-system disease with or without the risk of organ involvement. Although the skin is not categorized as a risk organ, the precise diagnosis of skin lesions is necessary to determine the protocol for the treatment of LCH. In this report, we describe a 28-year-old Japanese man with adult onset of BRAFV600E-mutated LCH with cutaneous involvement successfully diagnosed by immunohistochemical staining. Our report suggests that immunohistochemical staining for the BRAFV600E gene could be a diagnostic tool to determine the clinical type of LCH. PMID:26500535

  7. Radiotherapy for Langerhans Cell Histiocytosis of Bilateral Eyelids

    PubMed Central

    Bourque, Jean-Marc; Lukovic, Jelena; Dar, A. Rashid

    2016-01-01

    Langerhans cell histiocytosis (LCH) is a rare disorder with numerous clinicopathological variants with differing clinical courses, treatment methods, and prognoses. We report one patient with atypical LCH of the bilateral lower eyelids and subsequent successful treatment with local radiation therapy. PMID:27004151

  8. Juvenile xanthogranuloma developing after treatment of Langerhans cell histiocytosis: case report and literature review

    PubMed Central

    Strehl, Johanna D; Stachel, Klaus-Daniel; Hartmann, Arndt; Agaimy, Abbas

    2012-01-01

    The synchronous or metachronous development of Langerhans cell histiocytosis and non-Langerhans cell histiocytosis in the same patient is rare. To date, only seven cases of xanthogranulomas developing in young patients with a history of Langerhans cell histiocytosis and systemic therapy have been reported in the literature. As of yet, the pathogenesis and the clinical significance of this phenomenon are unclear. We report the case of a 3 year old boy who developed juvenile Xanthogranulomas on the forehead and right upper eye lid 1.5 years after systemic therapy for monosystemic Langerhans cell histiocytosis of the bone and complete disease remission. PMID:22977671

  9. Canine cutaneous histiocytoma is an epidermotropic Langerhans cell histiocytosis that expresses CD1 and specific beta 2-integrin molecules.

    PubMed Central

    Moore, P. F.; Schrenzel, M. D.; Affolter, V. K.; Olivry, T.; Naydan, D.

    1996-01-01

    Canine cutaneous histiocytoma (CCH) is a common, benign neoplasm of the dog. Histiocytomas most commonly occur as solitary lesions that undergo spontaneous regression. The age-specific incidence rate for histiocytomas drops precipitously after 3 years, although histiocytomas occur in dogs of all ages. Langerhans cells (LCs) in humans and dogs express abundant major histocompatibility complex class II molecules and a variety of leukocyte antigens characteristic of dendritic cell differentiation including CD1a, CD1b, CD1c, and CD11c. The immunophenotype of CCH resembled that of cutaneous LCs by virtue of the expression of CD1 molecules (CD1a, -b, and -c), CD11c, and major histocompatibility complex class II. Furthermore, histiocytoma cells had a tropism for epidermis, which was also consistent with an epidermal LC lineage. The expression of adhesion molecules such as CD11b (variable), CD44, CD54 (ICAM-1), and CD49d (VLA-4) in CCH indicated that the infiltrating cells had some of the characteristics of activated LCs, as these molecules are not expressed by normal, resting canine epidermal LCs. CCH did not express Thy-1 or CD4. Thy-1 expression is a characteristic of human and canine dermal dendrocytes, which are perivascular dendritic antigen-presenting cells closely related to epidermal LCs. CD4 expression is prevalent in human LC histiocytosis, and in this respect CCH differed from human LC histiocytosis. Here we demonstrate that CCH is a localized form of self-limiting LC histiocytosis, which predominantly expresses an epidermal LC phenotype. CCH occurs as solitary or, less commonly, as multiple cutaneous nodules or plaques, which rarely may extend beyond the skin to local lymph nodes. Regression of CCH occurs spontaneously in the vast majority of cases in primary and secondary sites, and is mediated by CD8+ alpha beta T cells. The high frequency of CCH within the general canine population offers the potential that the dog may provide an interesting model system to

  10. Role of multidisciplinary approach in a case of Langerhans cell histiocytosis with initial periodontal manifestations

    PubMed Central

    Cisternino, Angelo; Asa’ad, Farah; Fusco, Nicola; Ferrero, Stefano; Rasperini, Giulio

    2015-01-01

    Introduction: Langerhans cell histiocytosis (LCH) is a rare inflammatory myeloid neoplasia of unknown etiology occurring in both children and adults. This condition is characterized by an abnormal proliferation of Langerhans cells that may virtually affect all sites in the human body. Oral manifestations of LCH could be the first clinical sign of disease and its periodontal localization could be easily mistaken for other more common entities, such as chronic periodontitis, aggressive periodontitis, and necrotizing ulcerative periodontitis. Case presentation: A 32-years old female visited a private dental practice with a chief complaint of sensitivity in the mandibular left first molar. Clinical and radiographic examination revealed deep periodontal pocket, recession, furcation involvement, mobility, severe alveolar bone destruction and a diagnosis of aggressive periodontitis was rendered. Multiple tooth extractions were carried out due to progressive periodontal destruction with impaired healing and development of ulcerative lesions. Multidisciplinary investigation demonstrated that the periodontal involvement was a manifestation of an underlying systemic disease. A biopsy of a bone lesion was therefore performed, revealing the presence of multifocal single system LCH. Conclusion: The identification of periodontal LCH is not trivial given that it may clinically resemble other periodontal disease entities. The dentist can be the first health care personnel to unravel the presence of an underlying systemic LCH. PMID:26722570

  11. Adult Multisystem Langerhans Cell Histiocytosis Presenting with Central Diabetes Insipidus Successfully Treated with Chemotherapy

    PubMed Central

    Choi, Jung-Eun; Lee, Hae Ri; Ohn, Jung Hun; Moon, Min Kyong; Park, Juri; Lee, Seong Jin; Choi, Moon-Gi; Yoo, Hyung Joon; Kim, Jung Han

    2014-01-01

    We report the rare case of an adult who was diagnosed with recurrent multisystem Langerhans cell histiocytosis (LCH) involving the pituitary stalk and lung who present with central diabetes insipidus and was successfully treated with systemic steroids and chemotherapy. A 49-year-old man visited our hospital due to symptoms of polydipsia and polyuria that started 1 month prior. Two years prior to presentation, he underwent excision of right 6th and 7th rib lesions for the osteolytic lesion and chest pain, which were later confirmed to be LCH on pathology. After admission, the water deprivation test was done and the result indicated that he had central diabetes insipidus. Sella magnetic resonance imaging showed a mass on the pituitary stalk with loss of normal bright spot at the posterior lobe of the pituitary. Multiple patchy infiltrations were detected in both lung fields by computed tomography (CT). He was diagnosed with recurrent LCH and was subsequently treated with inhaled desmopressin, systemic steroids, vinblastine, and mercaptopurine. The pituitary mass disappeared after two months and both lungs were clear on chest CT after 11 months. Although clinical remission in multisystem LCH in adults is reportedly rare, our case of adult-onset multisystem LCH was treated successfully with systemic chemotherapy using prednisolone, vinblastine, and 6-mercaptopurine, which was well tolerated. PMID:25309800

  12. Clinicopathological pattern of cranial unifocal Langerhans cell histiocytosis: A study at medical college hospital

    PubMed Central

    Bhat, Salma; Nazir, Parvez; Bashir, Humaira; Reshi, Ruby; Sheikh, Sheema; Wani, Rohi

    2015-01-01

    Background: Eosinophilic granuloma (EG) of bone refers to a generally benign form of Langerhans cell histiocytosis localized to the bone. Patients may present with a solitary lesion (monostotic) or multiple sites of involvement (polyostotic). Materials and Methods: This study was done to evaluate the clinicopathological pattern of 6 cases of EGs of the skull diagnosed at a tertiary care hospital. All patients of EG were included with the help of medical records over a 5-year period that is, November 2009 to November 2014. They all had been preoperatively evaluated by skull X-ray and computed tomography. To rule out a multifocal disease scintigraphy was performed in all cases preoperatively. Surgical excision was performed, and EG was diagnosed on histopathology and immunohistochemistry. Results: There was a male predominance. Parietal bone was the most common affected bone. Total excision of the lesion was performed in all cases. No patient received postoperative radiotherapy. The follow-up period ranged from 6 months to 3 years. No tumor recurrence was noted. Conclusion: With an unknown etiology, nonspecific clinical and radiological findings with diagnosis possible only on histopathological examination, EG needs to be considered in the differential diagnosis as a skull mass, especially in children. PMID:26855527

  13. A Case of Langerhans Cell Histiocytosis Manifested as a Suprasellar Mass

    PubMed Central

    Yoon, Ju Young; Park, Byung-Kiu; Yoo, Heon; Lee, Sang Hyun; Hong, Eun Kyung; Park, Weon Seo; Kwon, Young Joo; Yoon, Jong Hyung

    2016-01-01

    Langerhans cell histiocytosis (LCH) has diverse clinical manifestations, including intracranial mass lesions. We report a case of LCH that manifested as a suprasellar mass, and initially misdiagnosed as a germ cell tumor. A 29-year-old woman presented with polyuria, polydipsia and amenorrhea. Laboratory findings revealed hypopituitarism with central diabetes insipidus, and a suprasellar mass and a pineal mass were observed on magnetic resonance imaging. Under the clinical impression of a germ cell tumor, the patient was treated with germ cell tumor chemotherapy (cisplatin and etoposide) and radiation therapy without biopsy. After initial shrinkage of the lesions, further growth of the tumor was observed and a biopsy was performed. The histopathology revealed LCH. After chemotherapy according to the LCH III protocol, the tumor disappeared. She is on regular follow up for 5 years without relapse. The present findings indicate that LCH should be included in the differential diagnosis of a suprasellar mass, even in adults, especially when it manifests with diabetes insipidus. This case also underscores the importance of a histopathologic diagnosis in patients with suprasellar tumors before the initiation of a specific therapy, even if the clinical findings are highly suggestive of a specific diagnosis. PMID:27195259

  14. Histiocytosis X: Characteristics, behavior, and treatments as illustrated in a case series

    PubMed Central

    Kasper, Ekkehard M.; Aguirre-Padilla, David H.; Alter, Raanan Y.; Anderson, Matthew

    2011-01-01

    Background: Langerhans cell histiocytosis (LCH) is a proliferative disorder predominantly found in children. It often presents with pain in calvarium or spine and may cause neuroendocrine symptoms. The gold standard for diagnosing LCH is the detection of Birbeck Granules by EM. Here, we describe two unique presentations of LCH and we review current treatment guidelines. Case Description: The first patient was a 23-year-old man who presented with progressive swelling and redness of the left eye. MRI revealed a left retrobulbar lesion extending into the middle cranial fossa with no signal abnormality in the brain parenchyma. The lesion was resected and pathological analysis revealed LCH. Bone scans were negative and the patient was discharged soon after. He later underwent fractionated radiotherapy (cumulative dose 26 Gy). Follow-up MRIs show no disease at 24 months post-op. The second patient was a 56-year-old man with left frontal skull pain for 5 months. Imaging showed a solitary osteolytic lesion extending into both dura and scalp with no signal abnormality of the parenchyma. Excisional biopsy revealed LCH. Surgery was well tolerated and follow-up imaging shows no recurrence at 24 months post-op. Conclusion: We demonstrate that LCH, though uncommon, must remain on the differential when osteolytic lesions present in the adult. Although LCH often has the clinical and radiographical presentation of an abscess, pathology analysis can successfully diagnose LCH based on markers and morphological characteristics. LCH has an excellent prognosis when treated aggressively with surgical resection and radiotherapy as both of our patients were and are now disease free at 2 year follow-up. PMID:21697965

  15. Congenital "self-healing" Langerhans cell histiocytosis (Hashimoto-Pritzker disease): a report of two cases with the same cutaneous manifestations but different clinical course.

    PubMed

    Mandel, Victor Desmond; Ferrari, Chiara; Cesinaro, Anna Maria; Pellacani, Giovanni; Del Forno, Corrado

    2014-12-01

    Congenital self-healing Langerhans cell histiocytosis or Hashimoto-Pritzker disease is a rare condition present at birth or in the neonatal period characterized by small reddish-brown crusted papulonodular lesions. In most cases these lesions are not accompanied by systemic findings and tend to involute spontaneously within weeks or months, but in other cases there may be extracutaneous involvement and/or recurrence of the disease. This emphasizes that the clinical course is variable and a long-term follow-up is mandatory in order to reveal possible systemic involvement. We describe two cases of congenital self-healing Langerhans cell histiocytosis with widespread and very similar cutaneous manifestations but different clinical course. The first patient had multisystemic disease (with lymph nodes, bones, liver and lungs affected) that required systemic therapy. The second patient had cutaneous and bony lesions that resolved spontaneously. We think that the adjective "self-healing" is misleading and should be abandoned. We stress the importance of a complete systemic evaluation and the necessity of a long-term follow-up.

  16. Disseminated Langerhans Cell Histiocytosis Presenting as Cholestatic Jaundice

    PubMed Central

    Loizides, Anthony M.; Sachdeva, Soumya; Paul, Premila

    2015-01-01

    Langerhans cell histiocytosis (LCH) is a disorder associated with proliferation of Langerhans cells in various organs. LCH secondary to multisystem involvement can present in a variety of ways. Because of its infiltrative nature, LCH can involve the skin, lymph nodes, the lung or the liver. Jaundice in LCH is a manifestation of liver disease; biliary dilatation secondary to lithiasis or may be due to coexistent Niemann-Pick disease. However, a case of cholestasis has been very rarely described. Cholestasis may result from lymph nodes obstructing the porta hepatis. In this report, we describe a case of type II histiocytosis X with obstructive cholestasis and pulmonary involvement in the form of cysts without significant lymphadenopathy at the porta. PMID:25859497

  17. Malignant histiocytosis in a Bernese mountain dog presenting as a mandibular mass.

    PubMed Central

    Carioto, L

    1997-01-01

    A Bernese mountain dog was evaluated because of a gingival mass, multiple abdominal masses, and a pulmonary mass. Malignant histiocytosis was diagnosed based on cytological examination of splenic and bone marrow aspirates and histological examination of a bone marrow biopsy and the gingival mass. The case demonstrates that malignant histiocytosis is difficult to diagnose due to the variety of histiocytic disorders. PMID:9028594

  18. Malignant histiocytosis: a clinicopathologic study of 18 consecutive cases.

    PubMed

    Rilke, F; Carbone, A; Musumeci, R; Pilotti, S; De Lena, M; Bonadonna, G

    1978-04-30

    The clinical records and histologic material of 18 consecutive patients with malignant histiocytosis were reviewed. The age of the patients ranged from 20 months to 72 years (median 35 years). There were 14 males and 4 females (3.5:1). Lymph node and liver enlargement, fever, and skin nodules were the most common physical findings; and leukocytosis was frequently the most abnormal laboratory test. Seven of 18 patients died, and their survival ranged from 1 to 15 months (median 8 months) after histopathologic diagnosis. The histologic findings on lymph nodes, spleen, liver, bone marrow, and skin were investigated with special reference to both the cellular composition and the pattern of lymph node involvement. Vascular invasion of small perinodal vessels was observed in 4 fatal cases. The absence of capsular invasion and the lack of cohesiveness among atypical proliferating histiocytes of malignant histiocytosis appeared to be inconstant. Sequential lymph node biopsies revealed in later stages the extension of the histiocytic proliferation from the sinuses into the cords and the complete obliteration of the nodal structures. The radiologic investigations yielded numerous pathologic findings that were consistent with the dissemination of the disease. Complete response to initial treatment was achieved in patients that were treated with radiotherapy and/or chemotherapy. Complete response with chemotherapy was achieved only when the treatment included adriamycin. The histologic and clinical features of the present series provide future evidence for the recognition of malignant histiocytosis as a distinct clinical and pathologic entity.

  19. Spontaneous extradural hemorrhage due to Langerhans cell histiocytosis of the skull in a child: A rare presentation

    PubMed Central

    Bakhaidar, Mohamad G.; Alghamdi, Fahad A.; Baeesa, Saleh S.

    2016-01-01

    Eosinophilic granuloma (EG) represents a local form of Langerhans cell histiocytosis that occurs mostly in children. It usually presents with a gradually enlarging painless skull mass, and rarely presents a rapid clinical deterioration. This 7-year-old boy who was diagnosed with EG, based on a magnetic resonance imaging scan, after presenting with a painless right parietal swelling of 7-week duration. Three weeks prior his scheduled surgery, he presented to the emergency department with a 2-day history of sudden increased of the subcutaneous swelling associated with a headache, vomiting, and decreased the level of consciousness; there was no history of trauma. Brain computed tomography revealed a right parietal bone defect with large subgaleal and extradural hematoma. He underwent emergent surgical excision of the skull lesion and evacuation of the hematoma. Histopathological examination confirmed the diagnosis of EG. We aim to raise the awareness of physicians of this rare spontaneous hemorrhagic complication of EG and review the literature. PMID:27195034

  20. A case of invasive Langerhans cell histiocytosis localizing only in the lung and diagnosed as pneumothorax in an adolescent female

    PubMed Central

    Dejima, Hitoshi; Morita, Shigeki; Takahashi, Yusuke; Matsutani, Noriyuki; Iinuma, Hisae; Kondo, Fukuo; Kawamura, Masafumi

    2015-01-01

    In infants, Langerhans cell histiocytosis (LCH) is associated with poor clinical outcomes as Langerhans cells invade and damage multiple organs, a presentation that is different from that in adults. Here, we present a case of a 15-year-old female who visited ourclinic complaining of right chest pain and dyspnea. She was diagnosed with right pneumothorax by chest X-ray. Chest computed tomography showed multiple cystic changes in the bilateral lung. Additionally, bullous lesions occupying the upper lobe and multiple white tiny nodules on the surface of the lung were observed by thoracoscopy. These nodules comprised proliferating atypical CD1a/S-100-positive cells invading the pulmonary parenchyma, leading to the diagnosis of LCH. Because of the extensive invasion into the pulmonary parenchyma, chemotherapy was administered. This case of LCH was unique in that the age of onset was atypical and the tumor cells occupied a single organ, despite their malignant behavior. PMID:26045867

  1. Multifocal Skeletal Tuberculosis Mimicking Langerhans Cell Histiocytosis in a Child: a Case Report With a Long-Term Follow-Up

    PubMed Central

    Haghighatkhah, Hamidreza; Jafroodi, Yousef; Sanei Taheri, Morteza; Pourghorban, Ramin; Sadeghian Dehkordy, Afarin

    2015-01-01

    Introduction: Multifocal skeletal tuberculosis is a rare condition that may masquerade as Langerhans cell histiocytosis, especially in children. Case Presentation: We report a case of multifocal osseous tuberculosis in a 5-year-old female patient admitted to our hospital with a complaint of low back pain but no history of respiratory symptoms or malaise. Radiological findings included vertebra plana and multiple lytic lesions in both the frontal and pelvic bones. An initial diagnosis of Langerhans cell histiocytosis was made based on imaging findings; however, the patient underwent further evaluation for Mycobacterium tuberculosis, and histopathologic findings confirmed the diagnosis of tuberculosis. The patient showed a nearly complete response after receiving a course of anti-tuberculosis drugs. Conclusions: A high index of suspicion is required for the early diagnosis and prompt treatment of patients with osseous tuberculosis. Given the high prevalence of tuberculosis in developing countries, tuberculosis should be considered in the differential diagnosis of multifocal lytic lesions and vertebra plana, especially in children. PMID:26744631

  2. Notch is active in Langerhans cell histiocytosis and confers pathognomonic features on dendritic cells.

    PubMed

    Hutter, Caroline; Kauer, Max; Simonitsch-Klupp, Ingrid; Jug, Gunhild; Schwentner, Raphaela; Leitner, Judith; Bock, Peter; Steinberger, Peter; Bauer, Wolfgang; Carlesso, Nadia; Minkov, Milen; Gadner, Helmut; Stingl, Georg; Kovar, Heinrich; Kriehuber, Ernst

    2012-12-20

    Langerhans cell histiocytosis (LCH) is an enigmatic disease defined by the accumulation of Langerhans cell-like dendritic cells (DCs). In the present study, we demonstrate that LCH cells exhibit a unique transcription profile that separates them not only from plasmacytoid and myeloid DCs, but also from epidermal Langerhans cells, indicating a distinct DC entity. Molecular analysis revealed that isolated and tissue-bound LCH cells selectively express the Notch ligand Jagged 2 (JAG2) and are the only DCs that express both Notch ligand and its receptor. We further show that JAG2 signaling induces key LCH-cell markers in monocyte-derived DCs, suggesting a functional role of Notch signaling in LCH ontogenesis. JAG2 also induced matrix-metalloproteinases 1 and 12, which are highly expressed in LCH and may account for tissue destruction in LCH lesions. This induction was selective for DCs and was not recapitulated in monocytes. The results of the present study suggest that JAG2-mediated Notch activation confers phenotypic and functional aspects of LCH to DCs; therefore, interference with Notch signaling may be an attractive strategy to combat this disease.

  3. Presence of circulating abnormal CD34+ progenitors in adult Langerhans cell histiocytosis

    PubMed Central

    MISERY, L; ROUGIER, N; CRESTANI, B; FAURE, M; CLAUDY, A; SCHMITT, D; VINCENT, C

    1999-01-01

    Langerhans cell histiocytosis (LCH) is related to the proliferation of cells, which are similar to Langerhans cells (LC) but possess many abnormal characteristics. Lesions are widespread and this fact suggests that LCH cells or their precursors are present in the blood of patients. In five adult patients, we have isolated and cultured CD34+ blood progenitors of dendritic cells. We studied their phenotype by flow cytometry and their functional properties in mixed culture with heterologous lymphocytes and with autologous lymphocytes in the presence of tri-nitro-phenyl antigen (TNP). The amount of CD34+ precursors was dramatically higher than controls but a high mortality occurred during the in vitro differentiation. The phenotype of surviving cells was similar to LC phenotype (CD1a+, CD83+, Lag+) but some of them expressed CD2. These cells were able to induce T cell proliferation in mixed culture. They could not initiate primary response to TNP, except in a patient treated with thalidomide. In our hands, these CD34+ cells may be precursors of LCH cells. PMID:10403933

  4. Isolated Langerhans cell histiocytosis of the sublingual gland in an adult

    PubMed Central

    Yang, Shaodong; Chen, Xinming; Zhang, Jiali; Fang, Qiong

    2015-01-01

    Langerhans cell histiocytosis (LCH) is a rare disorder characterized by the proliferation of pathologic Langerhans cells. Its clinical presentation is highly variable, that range from single-system, limited disease to severe, multi-organ disease with high mortality. LCH usually affects children and young adults. The most frequent sites for LCH are the bone, skin, lung, pituitary gland, and lymph nodes. Salivary gland involvement by LCH is extremely rare, and only a few cases of LHC involving the parotid glands have been reported in the English literature. To our knowledge, the involvement of the sublingual gland as a part of single or multisystem LCH has not been previously described. Herein we reported the first case of primary LCH of the sublingual gland. A 40-year-old woman presented with a 2-month history of a painless mass on the right sublingual area. Excision of the lesion including the right sublingual gland was performed. Histopathological diagnosis of LCH was rendered. The patient remains free of symptoms 17 months after surgery. PMID:26722591

  5. Treatment of Langerhans-cell histiocytosis in children. Experience at the Children's Hospital of Nancy.

    PubMed

    Sessa, S; Sommelet, D; Lascombes, P; Prévot, J

    1994-10-01

    Forty children who had Langerhans-cell histiocytosis were followed for an average of six years (range, excluding patients who died of the disease, two to fifteen years). The patients were divided into two diagnostic groups: those who had localized disease (involving one bone or more only) and those who had multifocal disease (an osseous lesion and a soft-tissue mass, a skin rash, diabetes insipidus, or generalized disease). Methods of treatment included curettage, bone-grafting, chemotherapy, local or systemic corticosteroids, and radiotherapy. Nineteen of the thirty patients who had localized disease had a complete response to the therapy, four had a partial response, and seven had no response. Twenty-one of these thirty patients had not had a recurrence by the time of the latest follow-up examination; nine had a local recurrence within four years after the initial therapy but had no additional recurrences after treatment of the local recurrence. No recurrence occurred more than four years after the time that the initial diagnosis had been made. Five of the ten patients who had multifocal disease had a complete response to the therapy, two had a partial response, and three had no response. Six patients had a recurrence; four did not. Two patients died of the disease. As a result of this study, we recommend the avoidance of intensive measures of treatment, if possible, and we advise long-term follow-up of these patients. PMID:7929499

  6. [Langerhans cell histiocytosis presenting as isolated adenitis in an infant: case report].

    PubMed

    Soriano-Ramos, María; Salcedo Lobato, Enrique; Baro Fernández, María; Blázquez-Gamero, Daniel

    2016-08-01

    Langerhans cell histiocytosis in infants is a rare condition, and presentation as an isolated cervical adenitis is exceptional at this age. We describe the case of a 3-month-old female infant presenting with a neck mass in the right mandibular angle with poor response to antibiotic treatment. Fine needle aspiration was performed and confirmed the diagnosis of Langerhans cell histiocytosis with complementary tests showing no features of systemic involvement. Langerhans cell histiocytosis should be considered in the differential diagnosis of subacute neck masses with poor outcome in infants and physicians should consider performing a fine needle aspiration to establish the diagnosis. PMID:27399030

  7. [Langerhans cell histiocytosis presenting as isolated adenitis in an infant: case report].

    PubMed

    Soriano-Ramos, María; Salcedo Lobato, Enrique; Baro Fernández, María; Blázquez-Gamero, Daniel

    2016-08-01

    Langerhans cell histiocytosis in infants is a rare condition, and presentation as an isolated cervical adenitis is exceptional at this age. We describe the case of a 3-month-old female infant presenting with a neck mass in the right mandibular angle with poor response to antibiotic treatment. Fine needle aspiration was performed and confirmed the diagnosis of Langerhans cell histiocytosis with complementary tests showing no features of systemic involvement. Langerhans cell histiocytosis should be considered in the differential diagnosis of subacute neck masses with poor outcome in infants and physicians should consider performing a fine needle aspiration to establish the diagnosis.

  8. Langerhans cell histiocytosis with nail changes and multisystem disease: a case report.

    PubMed

    De Jesus Semblano Bittencourt, Maraya; Moraes Dias, Carolina; Lima Lage, Thaiane; Magno Parijós, Amanda; Brito Mesquita, Letícia; Haber Carvalho, Alessandra

    2016-01-01

    Nail involvement in Langerhans cell histiocytosis is uncommon and is said to indicate a poor prognosis. We describe a 2-year-old boy with onycholysis, subungual hyperkeratosis, and hemorrhages on his fingernails. He also had hepatosplenomegaly and pulmonary involvement. The diagnosis of Langerhans cell histiocytosis was made by histopathologic examination of skin and liver.The role of nail involvement as an unfavorable prognostic sign is still unclear and this paper concludes that nail involvement in Langerhans cell histiocytosis is a possible sign of multisystemic involvement. PMID:27617727

  9. How I treat Langerhans cell histiocytosis

    PubMed Central

    Allen, Carl E.; Ladisch, Stephan

    2015-01-01

    “Langerhans cell histiocytosis” (LCH) describes a spectrum of clinical presentations ranging from a single bone lesion or trivial skin rash to an explosive disseminated disease. Regardless of clinical severity, LCH lesions share the common histology of CD1a+/CD207+ dendritic cells with characteristic morphology among an inflammatory infiltrate. Despite historical uncertainty defining LCH as inflammatory vs neoplastic and incomplete understanding of mechanisms of pathogenesis, clinical outcomes have improved markedly over the past decades through cooperative randomized clinical trials based on empiric therapeutic strategies. Significant advances include recognition of high- and low-risk clinical groups defined by hematopoietic and/or hepatic involvement, and of the importance of optimal intensity and of duration of chemotherapy. Nevertheless, mortality of high-risk patients, disease recurrence, lack of robustly tested salvage strategies, and significant disease morbidity of both high- and low-risk patients remain challenges. Recent discovery of recurrent somatic mutations in mitogen-activated protein kinase pathway genes at critical stages of myeloid hematopoietic differentiation in LCH patients supports redefinition of the disease as a myeloproliferative disorder and provides opportunities to develop novel approaches to diagnosis and therapy. PMID:25827831

  10. Growth hormone replacement in patients with Langerhan's cell histiocytosis

    PubMed Central

    Howell, S; Wilton, P; Shalet, S

    1998-01-01

    OBJECTIVES—To assess the impact of growth hormone on growth and the underlying disease in children with growth hormone deficiency as a result of Langerhan's cell histiocytosis.
STUDY DESIGN—Retrospective analysis of data from the Kabi (Pharmacia & Upjohn) international growth database (KIGS) for 82 children with Langerhan's cell histiocytosis treated with recombinant growth hormone.
RESULTS—At the start of treatment the median (10-90th centile) age was 9.0 (5.2 to 14.7) years, with a median height standard deviation score (SDS) of −2.0 (−3.5 to −0.9). The median pretreatment height velocity (measured in cm/year) was 3.6 (0.9 to 6.4); this increased to 8.8 (3.8 to 12.0) in the first year of treatment with growth hormone, and then remained significantly greater than the pretreatment height velocity at 7.3 (4.4 to 9.7) and 7.1 (4.1 to 9.3) cm/year in the second and third years, respectively. The median height SDS increased from −2.0 to −0.8 (−2.3 to 0.6) by the end of three years of treatment. There was no increase in the recurrence rate of the underlying disease and no adverse event could be directly attributed to growth hormone treatment, apart from one case of benign intracranial hypertension that resolved on stopping treatment with growth hormone.
CONCLUSIONS—Growth hormone replacement treatment for patients with Langerhan's cell histiocytosis with growth hormone deficiency is beneficial and safe.

 PMID:9659097

  11. Pulmonary langerhans cell histiocytosis masquerading as tuberculosis in an infant.

    PubMed

    Senanayake, M P; Mettananda, S; De Silva, M V C

    2011-01-01

    A 4-month-old infant presented with continued fever, unresolving bronchopneumonia and household contact with sputum-smear-positive tuberculosis (TB) and showed marginal improvement on anti-TB chemotherapy. Recurrent pneumothorax prompted the clinical diagnosis of TB to be revised. High-resolution CT scan of the chest and open lung biopsy confirmed the diagnosis of pulmonary Langerhans cell histiocytosis. Treatment with prednisolone and vinblastin resulted in settling of fever and resolution of respiratory symptoms and signs. In communities where the prevalence of TB is high, unusual presentations should prompt consideration of alternative diagnoses. PMID:22041471

  12. Langerhans Cell Histiocytosis with Atypical Intervertebral Disc and Sacroiliac Joint Involvement Mimicking Osteoarticular Tuberculosis in an Adult

    PubMed Central

    Özdemir, Zeynep Maraş; Kahraman, Ayşegül Sağır; Görmeli, Cemile Ayşe; Sevimli, Reşit; Akpolat, Nusret

    2016-01-01

    Background: Langerhans cell histiocytosis (LCH), typically found in children, is a rare single or multisystem disorder with a wide range of clinical and radiological manifestations. Unusual presentations of LCH are occasionally encountered and it may be difficult to distinguish LCH from an infection or a benign or malignant tumor. Results: A 35-year-old female presented with pain in her back and left buttock, malaise, and weight loss, with a duration of several months. Her laboratory test results were within the normal ranges except for the levels of acute phase reactants, which were elevated. Magnetic resonance imaging and computed tomography revealed a unilateral destructive sacroiliac lesion, and multiple vertebral lesions with adjacent discal involvement and extensive soft tissue extensions. She was initially misdiagnosed with multifocal osteoarticular tuberculosis. An open biopsy and joint curettage was performed. Histopathological examination showed that she had LCH. Conclusion: To the best of our knowledge, this is the first case of LCH associated with a destructive unilateral sacroiliac lesion, discal involvement, and involvement of the adjacent vertebrae, in an adult patient; the LCH mimicked osteoarticular tuberculosis. Disease onset in adulthood is rare, and this can potentially delay diagnosis. Familiarity with the imaging features of unusual LCH manifestations is necessary to ensure accurate diagnosis and appropriate treatment. PMID:27761291

  13. Imaging of orbital disorders.

    PubMed

    Cunnane, Mary Beth; Curtin, Hugh David

    2016-01-01

    Diseases of the orbit can be categorized in many ways, but in this chapter we shall group them according to etiology. Inflammatory diseases of the orbits may be infectious or noninfectious. Of the infections, orbital cellulitis is the most common and typically arises as a complication of acute sinusitis. Of the noninfectious, inflammatory conditions, thyroid orbitopathy is the most common and results in enlargement of the extraocular muscles and proliferation of the orbital fat. Idiopathic orbital inflammatory syndrome is another cause of inflammation in the orbit, which may mimic thyroid orbitopathy or even neoplasm, but typically presents with pain. Masses in the orbit may be benign or malignant and the differential diagnosis primarily depends on the location of the mass lesion, and on the age of the patient. Lacrimal gland tumors may be lymphomas or epithelial lesions of salivary origin. Extraocular muscle tumors may represent lymphoma or metastases. Tumors of the intraconal fat are often benign, typically hemangiomas or schwannomas. Finally, globe tumors may be retinoblastomas (in children), or choroidal melanomas or metastases in adults. PMID:27432687

  14. Spontaneous regression of congenital cutaneous histiocytosis X: report of a case with discussion of nosology and pathogenesis.

    PubMed

    Dehner, L P; Bamford, J T; McDonald, E C

    1983-01-01

    Spontaneous regression of histiocytosis X is a rare biologic event as judged by the extensive recorded experience in the literature. We present a case of congenital cutaneous histiocytosis X that resolved without specific therapy in the first week of life. Histologic and ultrastructural studies confirmed the interpretation. This child is an example of so-called pure cutaneous histiocytosis, which, like the other unisystem forms of the disorders, has an excellent prognosis.

  15. Indeterminate cell histiocytosis with naïve cells

    PubMed Central

    Bakry, Ola A.; Samaka, Rehab M.; Kandil, Mona A.; Younes, Sheren F.

    2013-01-01

    Histiocytoses are a heterogeneous group of disorders characterized by proliferation and accumulation of cells of mononuclear-macrophage system and dendritic cells. Histiocytoses are categorized according to the cell of origin into Langerhans cell histiocytosis (LCH), Non Langerhans cell histiocytoses and indeterminate cell histiocytosis (ICH). ICH is an extraordinary rare neoplastic dendritic cell disorder that has poorly understood histogenesis and pathogenesis. It is characterized by a proliferation of dendritic cells, which mimic Langerhans cells immunophenotypically (positive for CD1a and S-100 protein), but lack Birbeck granules characteristic of Langerhans cells. Twenty-four year-old Egyptian male was presented with reddish brown chest wall nodule. Clinical, histopathological, immunohistochemical and ultrastructure features are typical for ICH. He was in a good state without any evidence of recurrence or metastasis after 24 months follow up. Peculiar histopathological features were detected in the present case. Many unidentified cells with Hematoxylin & Eosin Langerhans like features showed negative staining for S-100, CD1a, Langerin and CD68. In absence of cellular atypia and mitosis, the infiltrating cells showed epidermotropism that was reported once in ICH as well as neural and perineural invasion that were not previously reported. Therefore we prefer using a tentatively designated diagnosis; dendritic cell tumor, not otherwise specified or newly proposed diagnosis (Indeterminate cell histocytosis with naïve cells) for the present case. PMID:23772299

  16. A rare occurrence of Langerhans cell histiocytosis in an adult

    PubMed Central

    Shevale, Vruturaj V; Ekta, K; Snehal, T; Geetanjal, M

    2014-01-01

    Langerhans Cell Histiocytosis (LCH) is a disease process characterized by accumulation and infiltration of cells, showing ultrastructural and immunohistochemical similarities to Langerhans’ cell, in the affected tissues. It exhibits extreme clinical heterogeneity. LCH was historically divided into 3 clinical entities based on extent of tissue involvement and severity of presentation. These 3 entities were eosinophilic granuloma, Hand-Schuler-Christian disease, Letterer-Siwe disease. Owing to similarities of their histologic appearance, they were grouped together under the term histiocytosis X. It was recently changed to LCH, emphasizing the primary cell involved in the disease process. LCH is a rare disease with an incidenceestimated to be 4.0 to 5.4 per million population. Males are affected twice as frequently as females. The disease may occur at any age with peak incidence in children aged 1 to 3 years. We describe an unusual case of a 65-year-old man who presented with painless swelling in anterior region of mandible. PMID:25948998

  17. Redefining Langerhans Cell Histiocytosis as a Myeloid Dysplasia and Identifying B | Division of Cancer Prevention

    Cancer.gov

    DESCRIPTION (provided by applicant): Redefining Langerhans Cell Histiocytosis as a Myeloid Dysplasia and Identifying Biomarkers for Early Detection and Risk Assessment. This application addresses Program Announcement PA-09-197: Biomarkers for Early Detection of Hematopoietic Malignancies (R01). The overall aim of this project is to identify novel biomarkers that may be used to diagnose and treat patients with Langerhans Cell Histiocytosis (LCH). LCH occurs with similar frequency as other rare malignancies including Hodgkin's lymphoma and AML. |

  18. [Histiocytosis X. An oligosymptomatic form of Hand-Schüller-Christian disease with a new form and possibilities of diagnosis].

    PubMed

    Eberth-Willershausen, W; Steger, O; Barran, W; Burg, G; Ryckmanns, F; Kaudewitz, P

    1984-10-01

    A patient is reported who had a monosymptomatic, cutaneous form of histiocytosis X. The reactivity of cutaneous histiocytosis-X cells with monoclonal anti-T-6 antibody is discussed as a new diagnostic procedure.

  19. Orbital involvement in multifocal fibrosclerosis.

    PubMed Central

    Aylward, G W; Sullivan, T J; Garner, A; Moseley, I; Wright, J E

    1995-01-01

    Multifocal fibrosclerosis is a condition of unknown aetiology, characterised by fibrous lesions occurring at a variety of sites. Clinical variants include retroperitoneal fibrosis, Riedel's thyroiditis, sclerosing cholangitis, and mediastinal fibrosis. Orbital pseudotumour has been reported as a manifestation of this condition. Three patients with multifocal fibrosclerosis in whom orbital involvement was the dominant feature are described. Images PMID:7703203

  20. Chorioretinal lesions, sea-blue histiocytes and other manifestations in familial chronic granulomatous disease.

    PubMed

    Lischner, H W; Martyn, L J

    1975-01-01

    Several little-emphasized manifestations of familial chronic granulomatous disease are considered: destructive chorioretinal lesions may be as constant as the pigmented histiocytosis seen in reticuloendothelial organs and could be related to a defect in the phagocytic activity of the retinal pigment epithelium; pigmented histiocytes with the staining characteristics of sea-blue histiocytes may be present in the bone marrow; patients may present with lesions resembling eosinophilic granuloma. Also discussed are some observations related to the sequestration of bacteria within phagocytic cells and the use of continuous antimicrobial therapy.

  1. Cluster headache after orbital exenteration.

    PubMed

    Evers, S; Sörös, P; Brilla, R; Gerding, H; Husstedt, I W

    1997-10-01

    A 37-year-old man developed an ipsilateral headache which fulfilled the criteria for cluster headache after orbital extenteration because of a traumatic lesion of the bulb. The headache could be treated successfully by drugs usually applied in the therapy of cluster headache. Six similar cases of cluster headache after orbital exenteration could be identified in the literature suggesting that the eye itself is not necessarily part of the pathogenesis of cluster headache. We hypothesize that orbital exenteration can cause cluster headache by lesions of sympathetic structures. Possibly, these mechanisms are similar to those of sympathetic reflex dystrophy (Sudeck-Leriche syndrome) causing pain of the limbs. PMID:9350391

  2. Solitary pulmonary MALT lymphoma presenting crystal-storing histiocytosis.

    PubMed

    Nagaharu, Keiki; Kageyama, Yuki; Watanabe, Takuya; Yamaguchi, Takanori; Ito, Ryugo; Baba, Youichirou; Masuya, Masahiro; Ohashi, Riuko; Kawakami, Keiki

    2016-08-01

    Crystal-storing histiocytosis (CSH) is characterized by the accumulation of large histiocytes with intracytoplasmic crystallized immunoglobulin and is typically associated with hematological malignancies. A 69-year-old man, who had a history of left nephrectomy and chemotherapy for renal pelvic cancer six years earlier, had received a CT scan every year thereafter and a small nodule was found in the left lower lobe of his lungs two years prior to the current presentation. Because of progression of this pulmonary nodule, he underwent pulmonary lobectomy on suspicion of lung cancer. He was ultimately diagnosed as having CSH accompanied by mucosa-associated lymphoid tissue lymphoma stage IAE. In the absence of further treatment, he has been well with no recurrence of the disease for 10 months postoperatively. Because CSH could reportedly be an initial presentation of hematological malignancies, careful observation and evaluation for the presence of these blood disorders is essential.

  3. Solitary pulmonary MALT lymphoma presenting crystal-storing histiocytosis.

    PubMed

    Nagaharu, Keiki; Kageyama, Yuki; Watanabe, Takuya; Yamaguchi, Takanori; Ito, Ryugo; Baba, Youichirou; Masuya, Masahiro; Ohashi, Riuko; Kawakami, Keiki

    2016-08-01

    Crystal-storing histiocytosis (CSH) is characterized by the accumulation of large histiocytes with intracytoplasmic crystallized immunoglobulin and is typically associated with hematological malignancies. A 69-year-old man, who had a history of left nephrectomy and chemotherapy for renal pelvic cancer six years earlier, had received a CT scan every year thereafter and a small nodule was found in the left lower lobe of his lungs two years prior to the current presentation. Because of progression of this pulmonary nodule, he underwent pulmonary lobectomy on suspicion of lung cancer. He was ultimately diagnosed as having CSH accompanied by mucosa-associated lymphoid tissue lymphoma stage IAE. In the absence of further treatment, he has been well with no recurrence of the disease for 10 months postoperatively. Because CSH could reportedly be an initial presentation of hematological malignancies, careful observation and evaluation for the presence of these blood disorders is essential. PMID:27599420

  4. Severe Langerhans cell histiocytosis in an infant: haemophagocytic syndrome association

    PubMed Central

    Póvoas, Marta Isabel; Luís, Pedro Pereira; Esteves, Isabel; Ferrão, Anabela

    2014-01-01

    Langerhans cell histiocytosis (LCH) is a rare disease of unknown origin with a heterogeneous clinical presentation, varying from benign and self-limited to lethal. It is classified as single or multisystemic, according to the number of organs involved (one or at least two, respectively). Diagnosis can be challenging and is based on the histological and immunophenotypic examination of affected tissues. Secondary haemophagocytic lymphohistiocytosis is rarely reported in association with LCH and may impair its diagnosis. Some authors suggest that the coexistence of the two disorders is more than coincidental. We present a case of multisystem LCH in a 5-month-old infant, with all risk organs involved, in which severity and rapid progression reflect an association with haemophagocytic syndrome. PMID:25336558

  5. Adult Langerhans Cell Histiocytosis with Hepatic and Pulmonary Involvement

    PubMed Central

    Araujo, Bruno; Costa, Francisco; Lopes, Joanne; Castro, Ricardo

    2015-01-01

    Langerhans cell histiocytosis (LCH) is a rare proliferative disorder of Langerhans cells of unknown etiology. It can involve multiple organ systems with different clinical presentation, which complicates the diagnosis. It can range from isolated to multisystem disease with different prognosis. Although common among children, liver involvement is relatively rare in adults and frequently overlooked. Natural history of liver LCH fits into two stages: an early stage with infiltration by histiocytes and a late stage with sclerosis of the biliary tree. Pulmonary findings are more common and include multiple nodules in different stages of cavitation, predominantly in the upper lobes. We present a case of adult LCH with pulmonary and biopsy proven liver involvement with resolution of the hepatic findings after treatment. PMID:25977828

  6. A case of langerhans cell histiocytosis with anal fistula.

    PubMed

    Akbayram, Sinan; Akgun, Cihangir; Ozen, Suleyman; Kaya, Avni; Tuncer, Oguz; Yuca, Sevil Ari; Caksen, Huseyin; Oner, Ahmet Faik

    2009-01-01

    Langerhans cell histiocytosis (LCH) is an uncommon clinically heterogeneous disorder characterized by the proliferation and accumulation of Langerhans cells with local infiltration of tissues and organ destruction. LCH takes many clinical forms, affecting different systems and different sites in the same system with variable outcomes. Bone, skin, lymph node, pituitary, liver, lung, bone marrow and spleen involvement can be seen in patients with LCH. Involvement of the perianal site is rare. In this article, a 16-month-old boy with multiple organ involvement including skin, liver, lung, and bone is presented. Aside from these systemic involvements, he also had a simple anal fistula. According to our best knowledge, this case of LCH with anal fistula is only the second to be reported in childhood. We would like to emphasize that LCH may be associated with anal fistula; therefore, we suggest that patients with LCH should be examined for this condition. PMID:20505285

  7. Central nervous system imaging in childhood Langerhans cell histiocytosis – a reference center analysis

    PubMed Central

    Porto, Luciana; Schöning, Stefan; Hattingen, Elke; Sörensen, Jan; Jurcoane, Alina; Lehrnbecher, Thomas

    2015-01-01

    Background The aim of our study was (1) to describe central nervous system (CNS) manifestations in children with Langerhans cell histiocytosis (LCH) based on images sent to a reference center and meeting minimum requirements and (2) to assess the inter-rater agreement of CNS-MRI results, which represents the overall reproducibility of this investigation. Methods We retrospectively reviewed brain MRI examinations in children with LCH, for which MRI minimum requirements were met. Abnormalities were rated by two experienced neuroradiologists, and the inter-rater agreement was assessed. Results Out of a total of 94 imaging studies, only 31 MRIs met the minimum criteria, which included T2w, FLAIR, T1w images before/after contrast in at least two different section planes, and thin post contrast sagittal slices T1w through the sella. The most common changes were osseous abnormalities, followed by solid enlargement of the pineal gland, thickened enhancing stalk and signal changes of the dentate nucleus. Whereas inter-rater agreement in assessing most of the CNS lesions was relatively high (κ > 0.61), the application of minimum criteria often did not allow to evaluate the posterior pituitary. Conclusions The diversity of radiological protocols from different institutions leads to difficulties in the diagnosis of CNS abnormalities in children with LCH. Although the inter-rater agreement between neuroradiologists was high, not all the LCH manifestations could be completely ruled out when using the minimum criteria. Brain MRIs should therefore follow LCH guideline protocols and include T1 pre-gadolinium sagittal images, and be centrally reviewed in order to improve the comparison of clinical trials. PMID:26401129

  8. [Endoscopic approaches to the orbit].

    PubMed

    Cebula, H; Lahlou, A; De Battista, J C; Debry, C; Froelich, S

    2010-01-01

    During the last decade, the use of endoscopic endonasal approaches to the pituitary has increased considerably. The endoscopic endonasal and transantral approaches offer a minimally invasive alternative to the classic transcranial or transconjunctival approaches to the medial aspect of the orbit. The medial wall of the orbit, the orbital apex, and the optic canal can be exposed through a middle meatal antrostomy, an anterior and posterior ethmoidectomy, and a sphenoidotomy. The inferomedial wall of the orbit can be also perfectly visualized through a sublabial antrostomy or an inferior meatal antrostomy. Several reports have described the use of an endoscopic approach for the resection or the biopsy of lesions located on the medial extraconal aspect of the orbit and orbital apex. However, the resection of intraconal lesions is still limited by inadequate instrumentation. Other indications for the endoscopic approach to the orbit are the decompression of the orbit for Graves' ophthalmopathy and traumatic optic neuropathy. However, the optimal management of traumatic optic neuropathy remains very controversial. Endoscopic endonasal decompression of the optic nerve in case of tumor compression could be a more valid indication in combination with radiation therapy. Finally, the endoscopic transantral treatment of blowout fracture of the floor of the orbit is an interesting option that avoids the eyelid or conjunctive incision of traditional approaches. The collaboration between the neurosurgeon and the ENT surgeon is mandatory and reduces the morbidity of the approach. Progress in instrumentation and optical devices will certainly make this approach promising for intraconal tumor of the orbit.

  9. Vascular Lesions.

    PubMed

    Jahnke, Marla N

    2016-08-01

    Vascular lesions in childhood are comprised of vascular tumors and vascular malformations. Vascular tumors encompass neoplasms of the vascular system, of which infantile hemangiomas (IHs) are the most common. Vascular malformations, on the other hand, consist of lesions due to anomalous development of the vascular system, including the capillary, venous, arterial, and lymphatic systems. Capillary malformations represent the most frequent type of vascular malformation. IHs and vascular malformations tend to follow relatively predictable growth patterns in that IHs grow then involute during early childhood, whereas vascular malformations tend to exhibit little change. Both vascular tumors and vascular malformations can demonstrate a wide range of severity and potential associated complications necessitating specialist intervention when appropriate. Evaluation and treatment of the most common types of vascular lesions are discussed in this article. [Pediatr Ann. 2016;45(8):e299-e305.]. PMID:27517358

  10. Diagnosis of pulmonary histiocytosis X by immunodetection of Langerhans cells in bronchoalveolar lavage fluid.

    PubMed Central

    Chollet, S.; Soler, P.; Dournovo, P.; Richard, M. S.; Ferrans, V. J.; Basset, F.

    1984-01-01

    Based on the finding that Langerhans cells and histiocytosis X cells react with the monoclonal antibody OKT6, raised against a subset of thymocytes, we used this antibody to study the cells collected by bronchoalveolar lavage (BAL) from 131 patients, including 18 with pulmonary histiocytosis X, 43 with pulmonary sarcoidosis, 67 with miscellaneous pulmonary disorders, and 3 controls. Immunofluorescence studies demonstrated the presence of OKT6-reactive cells in all patients with pulmonary histiocytosis X (mean +/- SEM, 5.29% +/- 1.14% of all cells in BAL fluid). Immunoelectron microscopic studies revealed that the cells labeled in these patients (n = 13) contained Langerhans granules. The number of fluorescent cells in the other 113 patients was significantly smaller (mean +/- SEM, 0.20% +/- 0.04% of all cells; P less than 0.001). In the 3 control patients, in the 43 patients with sarcoidosis, and in 61 of the 67 patients with miscellaneous disorders unrelated to histiocytosis X, no cells or less than 1% of the total were labeled; however, in the 6 remaining patients in this miscellaneous group, 1.3 to 2.8% of all cells in BAL were labeled. In 3 of these 6 patients, immunoelectronmicroscopic examination showed that the cells labeled by OKT6 had the general characteristics of Langerhans cells but lacked Langerhans granules. OKT3, OKT4, and OKT8 monoclonal antibodies did not stain histiocytosis X cells in BAL fluid. Images Figure 2 Figure 3 Figure 4 PMID:6372496

  11. Orbit to orbit transportation

    NASA Astrophysics Data System (ADS)

    Bergeron, R. P.

    1980-07-01

    Orbital transfer vehicle propulsion options for SPS include both chemical (COTV) and electrical (EOTV) options. The proposed EOTV construction method is similar to that of the SPS and, by the addition of a transmitting antenna, may serve as a demonstration or precursor satellite option. The results of the studies led to the selection of a single stage COTV for crew and priority cargo transfer. An EOTV concept is favored for cargo transfer because of the more favorable orbital burden factor over chemical systems. The gallium arsenide solar array is favored over the silicon array because of its self annealing characteristics of radiation damage encountered during multiple transitions through the Van Allen radiation belt. Transportation system operations are depicted. A heavy lift launch vehicle (HLLV) delivers cargo and propellants to LEO, which are transferred to a dedicated EOTV by means of an intraorbit transfer vehicle (IOTV) for subsequent transfer to GEO. The space shuttle is used for crew transfer from Earth to LEO. At the LEO base, the crew module is removed from the shuttle cargo bay and mated to a COTV for transfer to GEO. Upon arrival at GEO, the SPS construction cargo is transferred from the EOTV to the SPS construction base by IOTV. Crew consumables and resupply propellants are transported to GEO by the EOTV. Transportation requirements are dominated by the vast quantity of materials to be transported to LEO and GEO.

  12. Cystic and cavitary lung lesions in children: radiologic findings with pathologic correlation.

    PubMed

    Odev, Kemal; Guler, Ibrahim; Altinok, Tamer; Pekcan, Sevgi; Batur, Abdussamed; Ozbiner, Hüseyin

    2013-01-01

    A number of diseases produce focal or multiple thin-walled or thick-walled air- or fluid-containing cysts or cavitary lung lesions in both infants and children. In infants and children, there is a spectrum of focal or multifocal cystic and cavitary lung lesions including congenital lobar emphysema, congenital cystic adenomatoid malformation, pleuropulmonary blastoma, bronchogenic cyst, pulmonary sequestration, Langerhans cell histiocytosis, airway diseases, infectious diseases (bacterial infection, fungal infection, etc.), hydatid cysts, destroid lung, and traumatic pseudocyst. For the evaluation of cystic or cavitary lung lesion in infants and children, imaging plays an important role in accurate early diagnosis and optimal patient management. Therefore, a practical imaging approach based on the most sensitive and least invasive imaging modality in an efficient and cost-effective manner is paramount. We reviewed the conventional radiographs and computed tomography findings of the most common cystic and cavitary lung lesions in infants and children. PMID:24605255

  13. Kepler's Orbit

    NASA Video Gallery

    Kepler does not orbit the Earth, rather it orbits the Sun in concert with the Earth, slowly drifting away from Earth. Every 61 Earth years, Kepler and Earth will pass by each other. Throughout the ...

  14. Atypical radiological manifestations of pulmonary Langerhans cell histiocytosis in a 12-year-old girl.

    PubMed

    Ko, S-M; Choe, B-K; Kim, H-S; Lee, H-J; Kwon, K-Y

    2008-10-01

    Pulmonary Langerhans cell histiocytosis is a rare pulmonary disease that typically affects cigarette smokers from 30-40 years of age onwards. It is very rare in children, especially for those under 15 years of age. We report an atypical radiological manifestation of isolated pulmonary Langerhans cell histiocytosis in a 12-year-old girl that showed multifocal consolidation and multiple small nodules on an initial chest radiograph, and gradual fibrotic change with multiple cysts on follow-up chest radiographs and CT scans.

  15. Generalized eruptive histiocytosis associated with a novel fusion in LMNA-NTRK1.

    PubMed

    Pinney, Sarah S; Jahan-Tigh, Richard R; Chon, Susan

    2016-08-15

    Non-Langerhans cell histiocytosis (NLCH) is a histiocyte disorder comprised of dermal dendritic histiocytes with a characteristic staining pattern. Erdheim-Chester disease (ECD) is a subset of NLCH in which patients experience bone pain with corresponding changes on imaging. In addition, these patients show other evidence of systemic involvement, which can also be identified with imaging. This disease can occasionally present with cutaneous findings. We present a case of generalized eruptive histiocytosis (GEH), misdiagnosed as ECD, found to have an NTRK1 gene rearrangement. This is the first report of an NTRK1 kinase fusion with NLCH. The implication is unclear and further studies are warranted.

  16. Generalized eruptive histiocytosis associated with a novel fusion in LMNA-NTRK1.

    PubMed

    Pinney, Sarah S; Jahan-Tigh, Richard R; Chon, Susan

    2016-01-01

    Non-Langerhans cell histiocytosis (NLCH) is a histiocyte disorder comprised of dermal dendritic histiocytes with a characteristic staining pattern. Erdheim-Chester disease (ECD) is a subset of NLCH in which patients experience bone pain with corresponding changes on imaging. In addition, these patients show other evidence of systemic involvement, which can also be identified with imaging. This disease can occasionally present with cutaneous findings. We present a case of generalized eruptive histiocytosis (GEH), misdiagnosed as ECD, found to have an NTRK1 gene rearrangement. This is the first report of an NTRK1 kinase fusion with NLCH. The implication is unclear and further studies are warranted. PMID:27617936

  17. Chondromyxoid fibroma of the orbit.

    PubMed

    Ditta, Lauren C; Qayyum, Sohail; O'Brien, Thomas F; Choudhri, Asim F; Wilson, Matthew W

    2012-01-01

    A 51-year-old woman with a history of migraine headaches was found to have an incidental right orbital mass on MRI during neurologic evaluation for headaches. The orbital mass was a well-defined, lobulated, intraosseous soft tissue lesion with circumscribed margins. Clinically, there was noted proptosis, tenderness to palpation, and slight limitation to right abduction. An orbitotomy with incisional biopsy revealed a lesion arising within the lateral orbital rim extending to the subperiosteal space. Intraoperative frozen sections indicated a low grade sarcoma, possibly metastatic. The extraosseous component was excised, and the bone was curetted until all visible tumor was removed. A diagnosis of chondromyxoid fibroma was made. The patient did well until 5 months postoperatively, when right-sided proptosis returned due to recurrent tumor. Repeat surgical resection with removal of the lateral orbital rim was performed. Histopathology was consistent with recurrent chondromyxoid fibroma. PMID:22743695

  18. Macrophages in Langerhans cell histiocytosis are differentiated toward M2 phenotype: their possible involvement in pathological processes.

    PubMed

    Ohnishi, Koji; Komohara, Yoshihiro; Sakashita, Naomi; Iyama, Ken-Ichi; Murayama, Toshihiko; Takeya, Motohiro

    2010-01-01

    Although numerous macrophages are found in the lesions of Langerhans cell histiocytosis (LCH), their activation phenotypes and their roles in the disease process have not been clarified. Paraffin-embedded LCH samples were examined on immunohistochemistry and it was found that CD163 can be used to distinguish infiltrated macrophages from neoplastic Langerhans cells (LC). The number of CD163-positve macrophages was positively correlated with the number of multinucleated giant cells (MGC), indicating that most MGC are derived from infiltrated macrophages. A significant number of CD163-positive macrophages were positive for interleukin (IL)-10 and phospho-signal transducer and activator of transcription-3 (pSTAT3), an IL-10-induced signal transduction molecule. This indicates that these macrophages are polarized to anti-inflammatory macrophages of M2 phenotype. Tumor-derived macrophage-colony-stimulating factor (M-CSF) was considered to responsible for inducing M2 differentiation of infiltrated macrophages. The number of CD163-positive macrophages in different cases of LCH varied, and interestingly the density of CD163-positive macrophages was inversely correlated with the Ki-67-positivity of LC. Although the underlying mechanism is not fully elucidated, macrophage-derived IL-10 was considered to be involved in the suppression of tumor cell proliferation via activation of STAT3. PMID:20055949

  19. Langerhans cell histiocytosis in Chinese adults: absence of BRAF mutations and increased FOXP3+ regulatory T cells

    PubMed Central

    Tong, Chunguang; Jia, Xingyuan; Jia, Yanjun; He, Yanling

    2014-01-01

    Langerhans cell histiocytosis (LCH) is a rare disorder characterized by the proliferation of abnormal Langerhans cells. Previous studies mainly focused on children with LCH. However, there is limited information on the clinical and pathological aspects of LCH in adults. Therefore, this study aimed to investigate the clinical and pathological aspects of LCH in Chinese adults. The results showed that the average age of 18 LCH patients was 35.22 ± 16.57 years old. The ratio of male to female was 3.5:1.14 patients (77.8%) had single-system involvement and 4 patients (22.2%) had multi-system diseases. The skin (38.9%) and lungs (44.4%) were the mainly affected organs. No BRAF mutations were detected in the lesions of 18 cases. The number of FOXP3+ Tregs was significantly increased in LCH. In conclusion, clinical features of LCH in adults are distinct from those in children. Adult LCH has a relatively good prognosis and presents as a benign disease. Immune regulation plays an important role in the pathogenesis of adult LCH. PMID:25031736

  20. Langerhans cell histiocytosis is a neoplasm and consequently its recurrence is a relapse: In memory of Bob Arceci.

    PubMed

    Egeler, R Maarten; Katewa, Satyendra; Leenen, Pieter J M; Beverley, Peter; Collin, Matthew; Ginhoux, Florent; Arceci, Robert J; Rollins, Barrett J

    2016-10-01

    Langerhans cell histiocytosis (LCH) remains a poorly understood disorder with heterogeneous clinical presentations characterized by focal or disseminated lesions that contain excessive CD1a+ langerin+ cells with dendritic cell features known as "LCH cells." Two of the major questions investigated over the past century have been (i) the origin of LCH cells and (ii) whether LCH is primarily an immune dysregulatory disorder or a neoplasm. Current opinion is that LCH cells are likely to arise from hematopoietic precursor cells, although the stage of derailment and site of transformation remain unclear and may vary in patients with different extent of disease. Over the years, evidence has provided the view that LCH is a neoplasm. The demonstration of clonality of LCH cells, insufficient evidence alone for neoplasia, is now bolstered by finding driver somatic mutations in BRAF in up to 55% of patients with LCH, and activation of the RAS-RAF-MEK-ERK (where MEK and ERK are mitogen-activated protein kinase and extracellular signal-regulated kinase, respectively) pathway in nearly 100% of patients with LCH. Herein, we review the evidence that recurrent genetic abnormalities characterized by activating oncogenic mutations should satisfy prerequisites for LCH to be called a neoplasm. As a consequence, recurrent episodes of LCH should be considered relapsed disease rather than disease reactivation. Mapping the complete genetic landscape of this intriguing disease will provide additional support for the conclusion that LCH is a neoplasm and is likely to provide more potential opportunities for molecularly targeted therapies. PMID:27314817

  1. Salivary gland manifestations of sinus histiocytosis with massive lymphadenopathy: fine-needle aspiration cytology findings. A case report.

    PubMed

    Panikar, Nirupma; Agarwal, Sarla

    2005-09-01

    Sinus histiocytosis with massive lymphadenopathy (SHML or Rosai-Dorfman disease) is a nonneoplastic, usually self-limiting disease. Alhough it affects all age groups, it is more commonly seen in young males in their first or second decades. The disease primarily manifests as painless lymphadenopathy of cervical region, but other nodal groups and extranodal sites may also be affected. Seldom SHML may involve the salivary glands. We present the cytologic features and differential diagnoses of one such case.A 45-yr-old woman presented with an enlarged submandibular gland on the left side with ipsilateral cervical lymphadenopathy that had been persisting for 1 mo. The gland and the two enlarged nodes measured 2.5 x 2.5 cm(2) each and were firm in consistency. Clinically, tumor of the salivary glands was suspected. Fine-needle aspiration (FNA) smears showed moderate cellularity, with large histiocytes dispersed in the background of intense lymphoplasmacytic infiltrate. These histiocytes showed lymphophagocytosis. Isolated stromal fragments consisting of fibrocytes were seen separate from salivary acinar clusters on repeat aspirations. The diagnosis of SHML involving left submandibular gland and ipsilateral lymph nodes was returned. When analyzed in the context of clinical findings (laboratory data), the cytologic features of SHML involving salivary gland could be differentiated from those of malignancies and other benign lesions, especially Kuttner's tumor of the submandibular gland, which mimics neoplasm clinically.

  2. Tumor pathology of the orbit.

    PubMed

    Héran, F; Bergès, O; Blustajn, J; Boucenna, M; Charbonneau, F; Koskas, P; Lafitte, F; Nau, E; Roux, P; Sadik, J C; Savatovsky, J; Williams, M

    2014-10-01

    The term orbital tumor covers a wide range of benign and malignant diseases affecting specific component of the orbit or developing in contact with them. They are found incidentally or may be investigated as part of the assessment of a systemic disorder or because of orbital signs (exophthalmos, pain, etc.). Computed tomography, MRI and Color Doppler Ultrasound (CDU), play a varying role depending on the clinical presentation and the disease being investigated. This article reflects long experience in a reference center but does not claim to be exhaustive. We have chosen to consider these tumors from the perspective of their usual presentation, emphasizing the most common causes and suggestive radiological and clinical presentations (progressive or sudden-onset exophthalmos, children or adults, lacrimal gland lesions, periorbital lesions and enophthalmos). We will describe in particular muscle involvement (thyrotoxicosis and tumors), vascular lesions (cavernous sinus hemangioma, orbital varix, cystic lymphangioma), childhood lesions and orbital hematomas. We offer straightforward useful protocols for simple investigation and differential diagnosis. Readers who wish to go further to extend their knowledge in this fascinating area can refer to the references in the bibliography.

  3. A case of Langerhans' cell histiocytosis following Hodgkin's disease

    PubMed Central

    LI, XIN; DENG, QI; LI, YU-MING

    2016-01-01

    Langerhans' cell histiocytosis (LCH) is a group of disorders in various tissues characterized by the proliferation of Langerhans cells. It is rarely observed in adults. Langerhans cells are dendritic cells that express cluster of differentiation 1a (CD1a) and S100 protein, and contain Birbeck granules. Its etiopathogenesis remains to be elucidated. One possible etiological cause is a reactive proliferation of Langerhans cells following chemotherapy or radiotherapy for Hodgkin's disease (HD). A number of cases of LCH associated with malignant lymphoma have been reported previously. It may follow after the malignant lymphoma, or occur with it. However, fewer cases have been reported where the LCH followed after HD. In the present case report, a patient was diagnosed with HD following chemotherapy for LCH. As LCH was diagnosed, the patient was treated with a combination of various chemotherapeutic agents in two cycles of cyclophosphamide, vincristine, and prednisolone (COP), and eight cycles of cyclophosphamide, doxorubicin, vincristine, and prednisolone (CHOP). The patient went into a successful clinical remission. One year later, computed tomographic (CT) scans of the thorax and abdomen revealed augmentation of the tumor mass in the mediastinum. An excisional biopsy of the right inguinal lymph node was performed. The patient was diagnosed with nodular sclerosing Hodgkin's disease. Following four cycles of doxorubicin (Adriamycin), bleomycin, vinblastine and dacarbazine (ABVD) chemotherapy, a whole-body positron emission tomographic CT scan revealed a decrease in tumor mass in the mediastinum. At present, the patient remains in treatment, and the prognosis has yet to be fully determined. PMID:27330759

  4. An unusual case of adult disseminated cutaneous Langerhans cell histiocytosis.

    PubMed

    Moravvej, Hamideh; Yousefi, Maryam; Barikbin, Behrooz

    2006-01-01

    Langerhans cell histiocytosis (LCH) represents a group of rare histiocytic syndromes characterized by tissue infiltration with dendritic cells. The management of LCH is difficult because these disorders respond inconsistently to immunosuppressive and chemotherapeutic strategies. We describe a refractory and relapsing case of skin and nail limited LCH in a 27-year-old man. He presented with a 7-year history of an erythematous papular eruption of the scalp, ears, face, trunk, axillae, groins, fingernails, feet, and toenails. Diagnosis of LCH was made based on skin histopathology and immunohistochemical staining. Histological studies of biopsy specimens revealed a dense infiltrate of histiocytic mononuclear cells beneath the epidermis; these cells reacted strongly with anti-S-100 antibodies. In addition, CD1a was positive in most of the infiltrating cells. Extensive investigations failed to detect systemic involvement. The patient's cutaneous eruption did not respond to various therapeutic interventions, including phototherapy with oral psoralen with long-wave UV radiation in the A range (PUVA) and cyclosporine. Marked but temporary clinical improvement was achieved with thalidomide, etoposide with systemic steroid, and total body electron beam radiotherapy. Now the patient is on maintenance therapy with thalidomide and is under acceptable control. PMID:17083893

  5. Langerhans cell histiocytosis of the urinary bladder in a patient with bladder cancer previously treated with intravesical Bacillus Calmette-Guérin therapy.

    PubMed

    Numakura, Satoe; Morikawa, Teppei; Ushiku, Tetsuo; Toyoshima, Toyoaki; Fukayama, Masashi

    2014-02-01

    We report an extremely rare case of Langerhans cell histiocytosis (LCH) of the urinary bladder. A 68-year-old man presented with gross hematuria. Cystoscopy showed multiple papillary tumors in the urinary bladder, and transurethral resection was performed. Pathological diagnosis was high-grade papillary urothelial carcinoma with lamina propria invasion. The patient received six treatments with intravesical Bacillus Calmette-Guérin (BCG) therapy. Seven months after surgery, follow-up cystoscopy showed three elevated lesions in the urinary bladder, two of which were identified histologically as recurrent urothelial carcinoma. Microscopic examination of the lesion at the anterior wall revealed diffuse infiltration of medium to large histiocytoid cells in the lamina propria, many of which had distorted nuclei and nuclear grooves. Dense eosinophilic infiltration was also observed. Immunohistochemically, the histiocytoid cells were diffusely positive for S-100 and CD1a, but negative for cytokeratin AE1/AE3 and melanosome-associated antigen recognized by HMB-45. Based on the histological and immunohistochemical features, we diagnosed the lesion as LCH of the urinary bladder. There was no evidence of recurrence of either bladder cancer or LCH after an 18-month follow-up. To avoid misdiagnosis, urologists and pathologists should be aware that LCH may develop in the urinary bladder after intravesical BCG therapy for bladder cancer.

  6. Pulmonary Langerhans cell histiocytosis and diabetes insipidus in pregnant women: our experience.

    PubMed

    Fuks, Leonardo; Kramer, Mordechai R; Shitrit, David; Raviv, Yael

    2014-04-01

    Pulmonary Langerhans cell histiocytosis (PLCH) occurs predominantly in young adult smokers. Diabetes insipidus occurs in up to 15 % patients with PLCH. Information on PLCH in pregnancy is sparse, especially associated with diabetes insipidus. We report three patients with these conditions and describe the disease history and pregnancy outcomes.

  7. Expansion of Regulatory T Cells in Patients with Langerhans Cell Histiocytosis

    PubMed Central

    Senechal, Brigitte; Elain, Gaelle; Jeziorski, Eric; Grondin, Virginie; Patey-Mariaud de Serre, Natacha; Jaubert, Francis; Beldjord, Kheira; Lellouch, Arielle; Glorion, Christophe; Zerah, Michel; Mary, Pierre; Barkaoui, Mohammed; Emile, Jean Francois; Boccon-Gibod, Liliane; Josset, Patrice; Debré, Marianne; Fischer, Alain; Donadieu, Jean; Geissmann, Frederic

    2007-01-01

    Background Langerhans cell histiocytosis (LCH) is a rare clonal granulomatous disease that affects mainly children. LCH can involve various tissues such as bone, skin, lung, bone marrow, lymph nodes, and the central nervous system, and is frequently responsible for functional sequelae. The pathophysiology of LCH is unclear, but the uncontrolled proliferation of Langerhans cells (LCs) is believed to be the primary event in the formation of granulomas. The present study was designed to further investigate the nature of proliferating cells and the immune mechanisms involved in the LCH granulomas. Methods and Findings Biopsies (n = 24) and/or blood samples (n = 25) from 40 patients aged 0.25 to 13 y (mean 7.8 y), were studied to identify cells that proliferate in blood and granulomas. We found that the proliferating index of LCs was low (∼1.9%), and we did not observe expansion of a monocyte or dendritic cell compartment in patients. We found that LCH lesions were a site of active inflammation, tissue remodeling, and neo-angiogenesis, and the majority of proliferating cells were endothelial cells, fibroblasts, and polyclonal T lymphocytes. Within granulomas, interleukin 10 was abundant, LCs expressed the TNF receptor family member RANK, and CD4+ CD25high FoxP3high regulatory T cells (T-regs) represented 20% of T cells, and were found in close contact with LCs. FoxP3+ T-regs were also expanded compared to controls, in the blood of LCH patients with active disease, among whom seven out of seven tested exhibited an impaired skin delayed-type hypersensitivity response. In contrast, the number of blood T-regs were normal after remission of LCH. Conclusions These findings indicate that LC accumulation in LCH results from survival rather than uncontrolled proliferation, and is associated with the expansion of T-regs. These data suggest that LCs may be involved in the expansion of T-regs in vivo, resulting in the failure of the host immune system to eliminate LCH cells. Thus

  8. The cognitive spectrum in neurodegenerative Langerhans cell histiocytosis.

    PubMed

    Le Guennec, Loïc; Decaix, Caroline; Donadieu, Jean; Santiago-Ribeiro, Maria; Martin-Duverneuil, Nadine; Levy, Richard; Delgadillo, Daniel; Kas, Aurélie; Drier, Aurélie; Magy, Laurent; Bayen, Eleonore; Hoang-Xuan, Khe; Idbaih, Ahmed

    2014-08-01

    Clinical spectrum of cognitive troubles complicating neurodegenerative Langerhans cell histiocytosis (ND-LCH) is poorly known. The aim of this study is to evaluate cognitive functions in ND-LCH. The cognitive functions of a series of eight adult patients (7 males and 1 female; mean age 26 years IQ 25-75; range 20-33) suffering from clinical and/or radiological ND-LCH were evaluated using the following tests: (1) forward/backward digit and spatial span tasks of the WAIS-R scale and the Corsi block task, (2) the French version of the free and cued selective reminding test, (3) verbal fluency tests, (4) the Frontal Assessment Battery (FAB), (5) backward measurement of the verbal and visuospatial memories of the WAIS-R scale, (6) the Rey complex figure test, (7) the trail making tests A and B, (8) digit symbol and symbol search of the WAIS-IV scale, and (9) the Stroop test. Episodic (i.e. autobiographical or personal) memory free recall, categorical verbal fluency, phonological verbal fluency, visuospatial processing skills, attention, speed of processing, and sensitivity to interference were impaired in ND-LCH patients. In contrast, verbal and visuospatial short-term memories (i.e. immediate memories or forward span tasks) were preserved in all patients. Adult ND-LCH patients suffer from a severe but dissociated dysexecutive syndrome, mostly affecting executive strategies and relatively sparing short-term memory. Our study supports the need of assessing executive functions using comprehensive cognitive evaluation in ND-LCH patients for early diagnosis. PMID:24848633

  9. Differentiating skin-limited and multisystem Langerhans cell histiocytosis

    PubMed Central

    Simko, Stephen J.; Garmezy, Benjamin; Abhyankar, Harshal; Lupo, Philip J.; Chakraborty, Rikhia; Lim, Karen Phaik Har; Shih, Albert; Hicks, M. John; Wright, Teresa S.; Levy, Moise L.; McClain, Kenneth L.; Allen, Carl E.

    2014-01-01

    Objective To identify features associated with multisystem involvement and therapeutic failure in patients with skin Langerhans cell histiocytosis (LCH). Study design We reviewed medical records of 71 consecutive LCH patients with skin involvement evaluated at Texas Children’s Hospital and analyzed clinical features, laboratory results, and presence of circulating cells with the BRAF-V600E mutation, with respect to initial staging and clinical outcomes. Results Skin disease in patients older than 18 months at diagnosis was associated with presence of multisystem disease (OR 9.65, 95% CI 1.17–79.4). Forty percent of patients referred for presumed skin-limited LCH had underlying multisystem involvement, half of these with risk-organ involvement. Patients with skin-limited LCH had 3-year progression-free survival (PFS) of 89% after initial therapy, and none developed multisystem disease. Patients with skin/multisystem involvement had 3 year PFS of 44% with vinblastine/prednisone therapy, and risk-organ involvement did not correlate with failure to achieve non-active disease. Circulating cells with BRAF-V600E were detected at higher frequency in multisystem patients (8/11 skin/multisystem, 1/13 skin-limited, P=0.002). Conclusions Skin-limited LCH requires infrequent therapeutic intervention and has lower risk of progression relative to skin plus multisystem LCH. The less aggressive clinical course and lack of circulating cells with BRAF-V600E mutation in skin-limited LCH suggest a different mechanism of disease origin compared with multisystem or risk-organ disease. PMID:25441388

  10. The cognitive spectrum in neurodegenerative Langerhans cell histiocytosis.

    PubMed

    Le Guennec, Loïc; Decaix, Caroline; Donadieu, Jean; Santiago-Ribeiro, Maria; Martin-Duverneuil, Nadine; Levy, Richard; Delgadillo, Daniel; Kas, Aurélie; Drier, Aurélie; Magy, Laurent; Bayen, Eleonore; Hoang-Xuan, Khe; Idbaih, Ahmed

    2014-08-01

    Clinical spectrum of cognitive troubles complicating neurodegenerative Langerhans cell histiocytosis (ND-LCH) is poorly known. The aim of this study is to evaluate cognitive functions in ND-LCH. The cognitive functions of a series of eight adult patients (7 males and 1 female; mean age 26 years IQ 25-75; range 20-33) suffering from clinical and/or radiological ND-LCH were evaluated using the following tests: (1) forward/backward digit and spatial span tasks of the WAIS-R scale and the Corsi block task, (2) the French version of the free and cued selective reminding test, (3) verbal fluency tests, (4) the Frontal Assessment Battery (FAB), (5) backward measurement of the verbal and visuospatial memories of the WAIS-R scale, (6) the Rey complex figure test, (7) the trail making tests A and B, (8) digit symbol and symbol search of the WAIS-IV scale, and (9) the Stroop test. Episodic (i.e. autobiographical or personal) memory free recall, categorical verbal fluency, phonological verbal fluency, visuospatial processing skills, attention, speed of processing, and sensitivity to interference were impaired in ND-LCH patients. In contrast, verbal and visuospatial short-term memories (i.e. immediate memories or forward span tasks) were preserved in all patients. Adult ND-LCH patients suffer from a severe but dissociated dysexecutive syndrome, mostly affecting executive strategies and relatively sparing short-term memory. Our study supports the need of assessing executive functions using comprehensive cognitive evaluation in ND-LCH patients for early diagnosis.

  11. Solitary fibrous tumor of the orbit.

    PubMed

    Ing, E B; Kennerdell, J S; Olson, P R; Ogino, S; Rothfus, W E

    1998-01-01

    Solitary fibrous tumor (SFT) of the orbit is a very rare lesion that may be misdiagnosed as fibrous histiocytoma, hemangiopericytoma, or other orbital tumors. We present a 62-year-old man who presented with painless proptosis, 20 years following left eye enucleation for a presumed neurofibroma. On T2-weighted magnetic resonance imaging (MRI), a hypointense tumor almost filled his entire left orbit. There was no intracranial extension. The specimen obtained at orbital exenteration was consistent with the histologic, immunohistochemical, and electron microscopic findings of SFT. The tumor was positive for vimentin and CD34 staining but negative for S-100 protein and epithelial membrane antigen. Only nine other cases of SFT of the orbit have been documented in the literature. Recognition of SFT of the orbit as a distinct pathologic entity and further follow-up of published cases are needed to determine the prognosis of this rare lesion.

  12. Orbital fibrous hamartoma of infancy.

    PubMed

    Choi, Se Hyun; Hwang, Jeong-Min; Kim, Namju

    2016-02-01

    Fibrous hamartoma of infancy is a rare, benign lesion of the superficial soft tissue, typically presenting within the first 2 years of life, and usually located on the upper extremities, axilla, and upper back. Lesions affecting the orbit have not been reported previously. We report the case of a 7-month-old boy with orbital fibrous hamartoma of infancy. He had a right hypertropia and a limitation of infraduction in the right eye. A mass on the right anteroinferior orbit involving the inferior rectus and inferior oblique muscles was seen on magnetic resonance imaging. Hamartoma of infancy was confirmed through pathologic examination. Right hypertropia and limitation of infraduction moderately improved after surgery.

  13. Computed tomography of the eye and orbit

    SciTech Connect

    Hammerschlag, S.B.; Hesselink, J.R.; Weber, A.L.

    1982-01-01

    This book is the product of the evolution of computed tomography (CT) into subspecialization and the need for one source of information for the busy radiologist. The authors have succeeded in providing a readable overview of orbital CT as well as a reference book. The book is divided into seven major catagories of pathology (Neurofibromatosis, Primary Orbital Neoplasms, Secondary and Metastic Tumors of the Orbit, Vascular Disorders, Inflammatory Disease, Occular Lesions, and Trauma) after separate discussions of anatomy and technique.

  14. Orbiter's Skeleton

    NASA Technical Reports Server (NTRS)

    2005-01-01

    The structure of NASA's Mars Reconnaissance Orbiter spacecraft is constructed from composite panels of carbon layers over aluminum honeycomb, lightweight yet strong. This forms a basic structure or skeleton on which the instruments, electronics, propulsion and power systems can be mounted. The propellant tank is contained in the center of the orbiter's structure. This photo was taken at Lockheed Martin Space Systems, Denver, during construction of the spacecraft.

  15. Myths in the Diagnosis and Management of Orbital Tumors

    PubMed Central

    Gündüz, Kaan; Yanık, Özge

    2015-01-01

    Orbital tumors constitute a group of diverse lesions with a low incidence in the population. Tumors affecting the eye and ocular adnexa may also secondarily invade the orbit. Lack of accumulation of a sufficient number of cases with a specific diagnosis at various orbital centers, the paucity of prospective randomized studies, animal model studies, tissue bank, and genetic studies led to the development of various myths regarding the diagnosis and treatment of orbital lesions in the past. These myths continue to influence the diagnosis and treatment of orbital lesions by orbital specialists. This manuscript discusses some of the more common myths through case summaries and a review of the literature. Detailed genotypic analysis and genetic classification will provide further insight into the pathogenesis of many orbital diseases in the future. This will enable targeted treatments even for diseases with the same histopathologic diagnosis. Phenotypic variability within the same disease will be addressed using targeted treatments. PMID:26692710

  16. Cutaneous histiocytosis X. The presence of S-100 protein and its use in diagnosis.

    PubMed

    Rowden, G; Connelly, E M; Winkelmann, R K

    1983-07-01

    The cellular localization of glial S-100 protein was investigated in paraffin-embedded sections of cutaneous histiocytosis X and in a variety of cutaneous infiltrative disorders, including juvenile xanthogranuloma, necrobiotic xanthogranuloma, papular xanthoma, eruptive histiocytoma, and reticulohistiocytosis. Immunoperoxidase staining with a rabbit anti-calf brain-S-100 antibody demonstrated strong and consistent activity in atypical histiocytes in all histiocytosis X specimens. No detectable S-100 protein was demonstrated in either histiocytes or giant cells in the non-X histiocytic disorders investigated. Positive controls were included within both groups of disorders in respect to melanocyte, epidermal Langerhans' cell, and dermal Schwann's cell staining. These findings are interpreted as evidence for diversity in the mononuclear phagocyte system and demonstrate the practicality of such a simple test in diagnostic problems involving infiltrative histiocytic disorders of the skin.

  17. [Elevated gastric lesions].

    PubMed

    de Careaga, B; Villagómez, G; Pabón, J; Calderón, O; Elío, D; Pérez, J; Martínez, M; Patiño, F; Ponce, R; Lora, J

    1986-01-01

    Elevated gastric lesions, represent an important group among gastric pathology. To establish its incidence in our experience, we studied the endoscopic reports of two important hospitals in La Paz city: Instituto de Gastroenterología Boliviano Japonés and Hospital Obrero No. 1. In order to make a good endoscopic diagnosis among different elevated lesions we use some parameters like: location, shape, size, diameter, surface of the lesion and surrounding mucosa and characteristics of the falls. 10.472 endoscopic reports were reviewed, 497 elevated gastric lesions were found, 475 corresponded to mucosal lesions (352 benign lesions and 123 malignant lesions), 11 to submucosal and 11 extragastric lesions.

  18. Laryngeal Langerhans Cell Histiocytosis Presenting with Neck Mass in an Adult Woman

    PubMed Central

    Jahandideh, Hesam; Nasoori, Yasser; Rostami, Sara; Safdarian, Mahdi

    2016-01-01

    Langerhans cell histiocytosis (LCH) is a very rare condition that commonly affects the head and neck region. There are very few cases of isolated laryngeal involvement by LCH, mostly reported in pediatric patients. Here, we report a case of laryngeal LCH in a 62-year-old woman presenting with a neck mass several weeks ago. The clinical and histopathological findings are reported with a brief discussion about the disease. PMID:27127670

  19. Multiple myeloma involving the orbit.

    PubMed

    Fay, A M; Leib, M L; Fountain, K S

    1998-01-01

    Multiple myeloma is a plasma cell malignancy often associated with destructive skeletal lesions. Orbital involvement in multiple myeloma is rare. Risk factors for orbital involvement have not been established, although risk may vary with immunoglobulin subtype. Early detection of orbital plasmacytoma may affect treatment and clinical course. A case is reported of multiple myeloma without elevated serum immunoglobulins that involves the orbit, and the implications of early detection are discussed. The patient was first examined by an ophthalmologist 13 months after multiple myeloma was diagnosed and 5 months after the external appearance of an orbital tumor. Urine protein electrophoresis demonstrated kappa light chains. Hypergammaglobulinemia was not detected. Plain-film roentgenography showed orbital involvement at the time of initial diagnosis. An impressive clinical response to external beam radiation therapy was seen. Attention to immunoprotein characteristics in multiple myeloma may help to identify risk factors for orbital involvement. Early detection may permit safer and equally effective treatment. All patients with multiple myeloma should undergo thorough ophthalmic examination at the time of initial diagnosis.

  20. Orbital Debris

    NASA Technical Reports Server (NTRS)

    Kessler, D. J. (Compiler); Su, S. Y. (Compiler)

    1985-01-01

    Earth orbital debris issues and recommended future activities are discussed. The workshop addressed the areas of environment definition, hazards to spacecraft, and space object management. It concluded that orbital debris is a potential problem for future space operations. However, before recommending any major efforts to control the environment, more data are required. The most significant required data are on the population of debris smaller than 4 cm in diameter. New damage criteria are also required. When these data are obtained, they can be combined with hypervelocity data to evaluate the hazards to future spacecraft. After these hazards are understood, then techniques to control the environment can be evaluated.

  1. Combined Cutaneous Rosai-Dorfman Disease and Localized Cutaneous Langerhans Cell Histiocytosis Within a Single Subcutaneous Nodule

    PubMed Central

    Litzner, Brandon R.; Subtil, Antonio

    2015-01-01

    Abstract: Rosai-Dorfman disease (RDD) is a reactive multisystem histiocytosis that typically presents with cervical lymphadenopathy and systemic symptoms. Cutaneous involvement occurs in approximately 10% of cases, and 3% of cases are limited to the skin without nodal or other extranodal involvement. Langerhans cell histiocytosis (LCH) is a clonal histiocytosis with a wide spectrum of presentations ranging from isolated skin or bone disease to multisystem involvement. Rare case reports have identified concomitant presentation of RDD and LCH; however, most of these reports have involved LCH and RDD occurring concurrently but at separate sites. We present a rare case of concurrent RDD and LCH presenting within a single skin nodule. The patient did not have any evidence of systemic involvement and has remained stable without additional treatment. We also review the literature on this unusual co-presentation and suggest possible underlying mechanisms. Finally, we recommend baseline laboratory and imaging studies and discuss treatment options based on the available evidence. PMID:26588339

  2. Microsurgical anatomy of the orbit: the rule of seven.

    PubMed

    Martins, Carolina; Costa E Silva, Isabel Eugênia; Campero, Alvaro; Yasuda, Alexandre; Aguiar, Luiz Roberto; Tatagiba, Marcos; Rhoton, Albert

    2011-01-01

    The orbits are paired structures, located on the anterior part of the face. Morphologically, each orbit is a four sided pyramid with a posterior apex and anterior base. In the orbit, all openings are arranged around the base, apex or between the orbital walls. An anatomical characteristic of the orbit is that structures are arranged in groups of seven: there are seven bones, seven intraorbital muscles and seven nerves in the orbit. Tumors confined within the periorbita in the anterior two thirds of the orbit can often be approached extracranially, but those located in the apical area, and especially those on the medial side of the optic nerve, often require a transcranial approach. Thus, knowledge of orbital osteology is paramount in adequately choosing and performing an orbital approach. Understanding the critical topographical elements in this area helps to classify an orbital lesion and provides for a solid basis in choosing the most adequate intraorbital route for its treatment. PMID:22567293

  3. [Orbital varices].

    PubMed

    Seceleanu, Andreea; Szabo, I; Călugăru, M; Dudea, S M; Preda, D

    2004-01-01

    The purpose of this study was to point out a case with orbital venous abnormalities at the left eye, associated with varices of the legs. The clinical picture of this case was: intermittent exophthalmos, venous malformations at the level of the lids and episclera, elevated ocular pressure. All this signs reveal an abnormality at the level of venous wall, indicating a constitutional weakness of the venous system. The case was investigated by imagistic methods: ultrasound examination, Doppler -ultrasound and magnetic resonance imaging. According to the facts offered by clinical and imagistic investigation this case can be included into the first type of orbital varices, associated with secondary glaucoma provoked by an elevated episcleral venous pressure. PMID:15598045

  4. Eye and orbit ultrasound

    MedlinePlus

    Echography - eye orbit; Ultrasound - eye orbit; Ocular ultrasonography; Orbital ultrasonography ... eye is numbed with medicine (anesthetic drops). The ultrasound wand (transducer) is placed against the front surface ...

  5. Solitary fibrous tumor of the orbit

    PubMed Central

    Gupta, Sumiti; Verma, Renuka; Sen, Rajeev; Singh, Ishwar; Marwah, Nisha; Kohli, Rachneet

    2016-01-01

    Solitary fibrous tumor (SFT) is a rare spindle-cell neoplasm originating from the mesenchyme. It was originally thought to occur exclusively in the intrathoracic region but has been recently described in extrapleural sites including the orbit. SFT of the orbit is a rare lesion, which can be misdiagnosed as hemangiopericytoma, fibrous histiocytoma, meningioma, or neurofibroma. Immunohistochemistry plays an important role. We report an orbital SFT in a 39-year-old female presented with painless, progressive proptosis, and diminished vision in the right eye for the duration of 6 months. Magnetic resonance imaging demonstrated well-defined enhancing mass lesion. The patient underwent complete tumor removal through a right fronto-orbital approach, and a pathological diagnosis of the solitary fibrous tumor was made. Postoperatively, the patient was symptom-free. Clinical and pathological findings including immunohistochemistry are presented along with a brief discussion of literature. PMID:26889300

  6. Solitary fibrous tumor of the orbit.

    PubMed

    Gupta, Sumiti; Verma, Renuka; Sen, Rajeev; Singh, Ishwar; Marwah, Nisha; Kohli, Rachneet

    2016-01-01

    Solitary fibrous tumor (SFT) is a rare spindle-cell neoplasm originating from the mesenchyme. It was originally thought to occur exclusively in the intrathoracic region but has been recently described in extrapleural sites including the orbit. SFT of the orbit is a rare lesion, which can be misdiagnosed as hemangiopericytoma, fibrous histiocytoma, meningioma, or neurofibroma. Immunohistochemistry plays an important role. We report an orbital SFT in a 39-year-old female presented with painless, progressive proptosis, and diminished vision in the right eye for the duration of 6 months. Magnetic resonance imaging demonstrated well-defined enhancing mass lesion. The patient underwent complete tumor removal through a right fronto-orbital approach, and a pathological diagnosis of the solitary fibrous tumor was made. Postoperatively, the patient was symptom-free. Clinical and pathological findings including immunohistochemistry are presented along with a brief discussion of literature.

  7. Solitary fibrous tumor of the orbit.

    PubMed

    Gupta, Sumiti; Verma, Renuka; Sen, Rajeev; Singh, Ishwar; Marwah, Nisha; Kohli, Rachneet

    2016-01-01

    Solitary fibrous tumor (SFT) is a rare spindle-cell neoplasm originating from the mesenchyme. It was originally thought to occur exclusively in the intrathoracic region but has been recently described in extrapleural sites including the orbit. SFT of the orbit is a rare lesion, which can be misdiagnosed as hemangiopericytoma, fibrous histiocytoma, meningioma, or neurofibroma. Immunohistochemistry plays an important role. We report an orbital SFT in a 39-year-old female presented with painless, progressive proptosis, and diminished vision in the right eye for the duration of 6 months. Magnetic resonance imaging demonstrated well-defined enhancing mass lesion. The patient underwent complete tumor removal through a right fronto-orbital approach, and a pathological diagnosis of the solitary fibrous tumor was made. Postoperatively, the patient was symptom-free. Clinical and pathological findings including immunohistochemistry are presented along with a brief discussion of literature. PMID:26889300

  8. Extra Nodal Rosai-Dorfman Disease (Sinus Histiocytosis with Massive Lymphadenopathy) Presenting as Asymmetric Bilateral Optic Atrophy : An Atypical Ocular Presentation.

    PubMed

    Shukla, Eesha; Nicholson, Anjali; Agrawal, Anamika; Rathod, Darshana

    2016-09-01

    Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy, SHML) is a rare, non-hereditary, benign histiocytic proliferative disorder, presenting as painless bilateral cervical lymphadenopathy, with systemic symptoms. Extra nodal manifestations have been reported in 28-43 % cases with rare ocular involvement. We report a case of a 57 year old female presenting with gradual progressive decrease of vision OU since 8 months associated with epistaxis. Fundus examination revealed established optic atrophy in right eye with features of chronic papilloedema in left eye suggestive of compressive lesion. CT of brain, paranasal sinuses confirmed the presence of homogenously enhancing mass in left ethmoid sinus, left sphenoid sinus extending into suprasellar region. The biopsy of this mass revealed extra nodal SHML with tissue sections being S100 and CD68 positive with emperipolesis noted. Here we describe this atypical ocular presentation of extra nodal SHML to highlight that this rare disease can manifest as an aggressive sight threatening entity, even in older age group. PMID:27091209

  9. Sporadic progressive mucinous histiocytosis in a Mexican patient.

    PubMed

    Narváez-Rosales, Verónica; Sáez-de-Ocariz, Marimar; Toussaint-Caire, Sonia; Ortiz-Hidalgo, Carlos; Espinosa-Rosales, Francisco

    2013-01-01

    A 33 year-old woman presented with numerous 3- to 5-mm red-brown and yellow-brown dome-shaped nodules, primarily located on the scalp, dorsal aspects of the forearms, and lower extremities (Figure 1 and Figure 2). Her lesions started to appear 5 years prior to her consultation with increasing number and without spontaneous regression. Findings from a previous biopsy revealed epithelioid dermatofibroma. The remainder of the physical examination was unremarkable. There were no familial cases of this condition (both the mother and two older sisters were examined).

  10. Benign breast lesions: Ultrasound

    PubMed Central

    Masciadri, N.; Ferranti, C.

    2011-01-01

    Benign breast diseases constitute a heterogeneous group of lesions arising in the mammary epithelium or in other mammary tissues, and they may also be linked to vascular, inflammatory or traumatic pathologies. Most lesions found in women consulting a physician are benign. Ultrasound (US) diagnostic criteria indicating a benign lesion are described as well as US findings in the most frequent benign breast lesions. PMID:23396888

  11. Example based lesion segmentation

    NASA Astrophysics Data System (ADS)

    Roy, Snehashis; He, Qing; Carass, Aaron; Jog, Amod; Cuzzocreo, Jennifer L.; Reich, Daniel S.; Prince, Jerry; Pham, Dzung

    2014-03-01

    Automatic and accurate detection of white matter lesions is a significant step toward understanding the progression of many diseases, like Alzheimer's disease or multiple sclerosis. Multi-modal MR images are often used to segment T2 white matter lesions that can represent regions of demyelination or ischemia. Some automated lesion segmentation methods describe the lesion intensities using generative models, and then classify the lesions with some combination of heuristics and cost minimization. In contrast, we propose a patch-based method, in which lesions are found using examples from an atlas containing multi-modal MR images and corresponding manual delineations of lesions. Patches from subject MR images are matched to patches from the atlas and lesion memberships are found based on patch similarity weights. We experiment on 43 subjects with MS, whose scans show various levels of lesion-load. We demonstrate significant improvement in Dice coefficient and total lesion volume compared to a state of the art model-based lesion segmentation method, indicating more accurate delineation of lesions.

  12. Example Based Lesion Segmentation

    PubMed Central

    Roy, Snehashis; He, Qing; Carass, Aaron; Jog, Amod; Cuzzocreo, Jennifer L.; Reich, Daniel S.; Prince, Jerry; Pham, Dzung

    2016-01-01

    Automatic and accurate detection of white matter lesions is a significant step toward understanding the progression of many diseases, like Alzheimer’s disease or multiple sclerosis. Multi-modal MR images are often used to segment T2 white matter lesions that can represent regions of demyelination or ischemia. Some automated lesion segmentation methods describe the lesion intensities using generative models, and then classify the lesions with some combination of heuristics and cost minimization. In contrast, we propose a patch-based method, in which lesions are found using examples from an atlas containing multi-modal MR images and corresponding manual delineations of lesions. Patches from subject MR images are matched to patches from the atlas and lesion memberships are found based on patch similarity weights. We experiment on 43 subjects with MS, whose scans show various levels of lesion-load. We demonstrate significant improvement in Dice coefficient and total lesion volume compared to a state of the art model-based lesion segmentation method, indicating more accurate delineation of lesions.

  13. Radiosensitive orbital metastasis as presentation of occult colonic adenocarcinoma

    PubMed Central

    Ludmir, Ethan B; McCall, Shannon J; Czito, Brian G; Palta, Manisha

    2014-01-01

    An 82-year-old man presented with progressive right frontal headaches. The patient's history was significant for benign polyps on surveillance colonoscopy 2 years prior, without high-grade dysplasia or carcinoma. MRI revealed an enhancing lesion arising within the superomedial aspect of the right orbit. Lesion biopsy demonstrated histological appearance and immunophenotype suggestive of colonic adenocarcinoma. Staging positron emission tomography/CT showed visceral metastases and diffuse activity in the posterior rectosigmoid, consistent with metastatic colon cancer. Treatment of the orbital lesion with external beam radiotherapy to 30 Gy resulted in significant palliation of the patient's headaches. The patient expired 2 months following treatment completion due to disease progression. Orbital metastasis as the initial presentation of an occult colorectal primary lesion is exceedingly rare, and occurred in this patient despite surveillance colonoscopy. Radiotherapy remains an efficacious modality for treatment of orbital metastases. PMID:25240005

  14. S100-Negative, CD1a-Positive Cutaneous Histiocytosis in a Patient with S100-Positive, CD1a-Positive Pulmonary Histiocytosis.

    PubMed

    Mask-Bull, Lisa; Crowson, Neil A; John, Andrew; Mask, Neal A

    2015-08-01

    In the diagnostic approach to histiocytic proliferations, immunohistochemistry may be a source of both confusion and clarification. We present a case of a 60-year-old man with a generalized pruritic eruption that demonstrated positive staining for CD1a, but negative staining for langerin and S100 protein. This immunophenotype is neither representative nor characteristic of any recognized dendritic cell tumor but has been previously described in 3 cases of skin-limited histiocytosis. However, our patient also demonstrated pulmonary histiocytic infiltrates that were positive for both CD1a and S100 proteins. This differing expression of S100 protein witnessed in 2 separate organ systems affords us insight into the pathophysiology of these histiocytic proliferations.

  15. Orbit analysis

    SciTech Connect

    Michelotti, L.

    1995-01-01

    The past fifteen years have witnessed a remarkable development of methods for analyzing single particle orbit dynamics in accelerators. Unlike their more classic counterparts, which act upon differential equations, these methods proceed by manipulating Poincare maps directly. This attribute makes them well matched for studying accelerators whose physics is most naturally modelled in terms of maps, an observation that has been championed most vigorously by Forest. In the following sections the author sketchs a little background, explains some of the physics underlying these techniques, and discusses the best computing strategy for implementing them in conjunction with modeling accelerators.

  16. [The Langerhans cell histiocytosis with thymic localization as initial and exclusive place].

    PubMed

    Hernández Pérez, J M; Franquet Casas, T; Rodríguez, S; Giménez, A

    2007-10-01

    The Langerhans' cell histiocytosis (LCH), also known as Histiocitosis X it is an illness not very frequent granulomatosus etiology not clarified yet, that it can have different manifestations and localizations, however the thymic localization as initial and exclusive place gives presentation HCL it is quite unusual. The present case is presented a patient that debuted with a clinical unspecific, where the tests give image they put she gives apparent a mass in previous mediastinum and that after the pathologic and immunohistochemical analysis they evidenced a proliferation Langerhans s cells and eosinophils it being positive for CD1a and S-100 confirming the diagnosis of the LCH.

  17. Cutaneous T cell lymphoma mimicking cutaneous histiocytosis: differentiation by flow cytometry.

    PubMed

    Baines, S J; McCormick, D; McInnes, E; Dunn, J K; Dobson, J M; McConnell, I

    2000-07-01

    A two-year-old, neutered female cross-bred labrador had multiple cutaneous nodules, biopsies of which revealed pathological changes consistent with cutaneous histiocytosis. During a period of one month the dog developed multicentric lymphadenopathy, a retrobulbar mass and masses within the quadriceps and cervical muscles. Fine needle aspiration cytology of the cutaneous nodules and lymph nodes and histological examination of the cutaneous nodules and muscle masses suggested the presence of lymphoblastic lymphoma. A definitive diagnosis of CD8+ T cell lymphoma was achieved by immunophenotyping the tumour cells by flow cytometry.

  18. [An unknown cause of deep lymphadenopathies: polyethylene histiocytosis related to a joint prosthesis].

    PubMed

    Bertrand, A F; Bertrand, G; Dauver, N; Richard, M C

    1993-01-01

    A case of deep seated lomboaortic lymph node enlargement, due to a storage hitiocytosis by fragments of polyethylene, draining the site of hip joint prosthesis is reported. These lymph node enlargement, made visible by tomodensitometry, were satellites of an ovarian adenocarcinoma. The artificial hip joint operation had taken place 17 years before and 10 years later the patient underwent a secondary operation to replace the prosthesis of the right hip. To the knowledge of the authors this is the first recorded case of lymph node enlargement due to a storage histiocytosis in response to debris shed from hip prosthesis being made visible by way of a radiological examination.

  19. Contrast-enhanced ultrasound (CEUS) for the study of peripheral lung lesions: a preliminary study.

    PubMed

    Sperandeo, Marco; Sperandeo, Giuseppe; Varriale, Antonio; Filabozzi, Paola; Decuzzi, Marco; Dimitri, Lucia; Vendemiale, Gianluigi

    2006-10-01

    The use of contrast-enhanced ultrasound (CEUS) for the study of peripheral lung lesions has never been systematically investigated. We evaluated the CEUS patterns of 98 peripheral lung lesions before performing US-guided fine-needle biopsies (FNB). The examinations were done with an Esaote Technos MPX scanner in the harmonic mode with a mechanical index of 0.04 or less. Contrast enhancement was achieved with a 4.8 mL bolus of SonoVue (Bracco) administered via an antecubital vein. All FNBs performed under CEUS guidance were adequate for pathologic diagnosis. Seventy-eight lesions were malignant: 33 (41.9%) were adenocarcinomas, 29 (36.5%) were squamous-cell carcinomas, 13 (17.6%) were undifferentiated large-cell carcinomas and the remaining three (4.1%) were small-cell carcinomas. All presented intralesional enhancement consistent with tumor neovascularization. In some cases, there were unenhanced areas consistent with zones of necrosis and these areas were avoided during FNB. The other 20 lesions were benign (four lipomas, two fibrous lung tumor, two noncaseous granulomas, six abscesses, one rheumatoid nodule, one histiocytosis X, one chondroid hamartoma, one sclerosing hemangioma, two sarcoid nodules) and none presented intralesional enhancement. This initial and admittedly limited experience suggests that CEUS may provide diagnostically useful information on peripheral lung lesions and increase the diagnostic yield of transthoracic FNB by reducing the risk of inadequate tissue sampling.

  20. Surgical approach to the superior mid-orbit.

    PubMed

    Krohn-Hansen, Dag; Nicolaissen, Bjørn; Meling, Torstein R; Haaskjold, Erling

    2013-09-01

    Access to the superior mid-orbit is required for procedures on the levator muscle in the correction of upper eyelid ptosis and in surgery aimed at local lesions in this region. The purpose with this human cadaver study was to clarify the anatomical substrate for a surgical approach to the levator muscle and the upper mid-orbit structures, in which the orbital septum and the retroseptal fat pad is not harmed during surgery. Macro-anatomical dissections and histological examinations were performed on five human orbits from three formalin embalmed cadaver heads. It was found that the orbital septum extends posteriorly from its junction with the levator aponeurosis. This posterior continuation of the orbital septum encloses the superior orbital fat pad and separates this from the anterior surface of the levator muscle. In between the orbital septum and the levator, there is a dissection space that provides a minimal invasive access corridor to the structures in the upper mid-orbit.

  1. Successful outcome of Langerhans cell histiocytosis complicated by therapy-related myelodysplasia and acute myeloid leukemia: a case report

    PubMed Central

    Al-Anazi, Khalid A; Alshehri, Abdulrahman; Al-Zahrani, Hazza A; Al-Mohareb, Fahad I; Maghfoor, Irfan; Ajarim, Dahish

    2008-01-01

    Background Various therapeutic options are available for the management of Langerhans cell histiocytosis. However, treatment administered to control this disease may be complicated by acute leukemia. Case presentation A 34 years old male was diagnosed to have Langerhans cell histiocytosis in March 1999. Unfortunately, the cytotoxic chemotherapy and radiotherapy given to control the repeated relapses and exacerbations of the primary disease predisposed him to therapy-induced myelodysplastic syndrome which transformed into acute myeloid leukemia. After achieving complete remission of his leukemia, the patient received an allogeneic hematopoietic stem cell transplant. The allograft was complicated by chronic graft versus host disease that was controlled by various immunosuppressive agents and extracorporal photophoresis. Conclusion Management of complicated cases of histiocytosis requires various therapeutic modalities and a multidisciplinary approach. Having complications of therapy eg myelodysplasia or acute leukemia make the outcome more dismal and the management options limited to aggressive forms of treatment. High dose chemotherapy followed by an allograft may be a curative option not only for therapy-related myelodysplasia/acute leukemia, but also for frequently relapsing and poorly controlled Langerhans cell histiocytosis. PMID:18710527

  2. Orbital Winch

    NASA Technical Reports Server (NTRS)

    Hoyt, Robert (Inventor); Slostad, Jeffrey T. (Inventor); Frank, Scott (Inventor); Barnes, Ian M. (Inventor)

    2016-01-01

    Orbital winch having: lower and upper frames; spool having upper and lower flanges with lower flange attached to lower frame; axial tether guide mounted to upper frame; secondary slewing ring coaxial with spool and rotatably mounted to upper frame, wherein secondary slewing ring's outer surface has gearing; upper tether guide mounted to inner surface of secondary slewing ring; linear translation means having upper end mounted to upper frame and lower end mounted on lower frame; primary slewing ring rotatably mounted within linear translation means allowing translation axially between flanges, wherein primary slewing ring's outer surface has gearing; lower tether guide mounted on primary slewing ring's inner surface; pinion rod having upper end mounted to upper frame and lower end mounted to lower frame, wherein pinion rod's teeth engage primary and secondary slewing rings' outer surface teeth; and tether passing through axial, upper, and lower tether guides and winding around spool.

  3. [An autopsy case of malignant histiocytosis-like disorder following hypersensitive reaction to mosquito bite].

    PubMed

    Matsumoto, S; Baba, K; Koide, O; Nakayama, K

    1989-03-01

    An autopsy case of an 18-year-old Japanese girl with a malignant histiocytosis-like disorder that developed during the course of a hypersensitive reaction to mosquito bite is reported. Episodes of hypersensitive reactions to mosquito bite had been repeated since she was 12 years old and at the age of 18 years she died of acute respiratory failure only 11 days after a mosquito bite. On autopsy, the dermal reaction to the last mosquito bite had already calmed down, and 'poorly differentiated histiocytes', which were presumed from their histochemical characteristics, had remarkably infiltrated into multiple organs, appearing like leukemic infiltration. Only a small number of them were noted in the lymph nodes and the bone marrow. The results of histochemical examination of 'poorly differentiated histiocytes' was as follows: (1) Neither markers for granulocytes (peroxidase and naphthyl AS-D Cl esterase) nor markers for lymphocytes and plasma cells (leukocyte common antigen and immunoglobulins) were detected. (2) Some markers for histiocytes (peanut lectin agglutinin and lysozyme) were positive in some of the proliferated cells. (3) A marker for T-zone histiocyte (s-100 protein) was negative. These results suggested that the proliferated cells included cells of the monocyte-macrophage system. These cells morphologically showed no phagocytic activity and were suggested to be immature histiocytes. Jurco et al. reported (poorly differentiated) malignant histiocytosis consisting of immature histiocytes without phagocytic activity, by using histochemical methods.(ABSTRACT TRUNCATED AT 250 WORDS)

  4. The histiocytosis Erdheim-Chester disease is an inflammatory myeloid neoplasm.

    PubMed

    Haroche, Julien; Cohen-Aubart, Fleur; Charlotte, Frédéric; Maksud, Philippe; Grenier, Philippe A; Cluzel, Philippe; Mathian, Alexis; Emile, Jean-François; Amoura, Zahir

    2015-01-01

    Erdheim-Chester disease (ECD) is a rare, non-Langerhans histiocytosis, characterized by the infiltration of tissues by foamy CD68(+)CD1a(-) histiocytes. (99)Technetium bone scintigraphy revealing almost constant tracer uptake by the long bones is highly suggestive of ECD, and a 'hairy kidney' appearance on abdominal computed tomography scan is observed in about half of all ECD cases. CNS involvement is a strong prognostic factor and independent predictor of death. IFN-α seems to be the best initial treatment for ECD. More than half of all ECD patients carry the BRAF(V600E) mutation. More than 30 patients worldwide harboring this mutation and displaying multisystemic, refractory ECD have been treated with vemurafenib, a BRAF inhibitor, which has proven highly beneficial. Other recurrent mutations of the MAPK and PIK3 pathways (NRAS, PIK3CA) have recently been described. These mutations should lead to a new classification of histiocytic disorders such that Langerhans cell histiocytosis and ECD are classified as inflammatory myeloid neoplasms. PMID:26197238

  5. [Residual left orbital phlebolith. An anatomic-clinical observation].

    PubMed

    D'hermies, F; Hurbli, T; Berges, O; Meyer, A; Morel, X; Lot, G; Cophignon, J; Renard, G

    2000-05-01

    A 29-year-old female patient displayed a dense and hard left orbital mass, inferiorly, 3 years after she was treated through a neurosurgical approach for a left orbital varix. The lesion was hyperdense on CT-scan and was removed through a trans-conjunctival approach, with an excellent result. The histological diagnosis was a phlebolith. PMID:10844320

  6. Treatment Challenges with Benign Bone Tumors of the Orbit

    PubMed Central

    Merritt, Helen; Yin, Vivian T.; Pfeiffer, Margaret L.; Wang, Wei-Lien; Sniegowski, Matthew C.; Esmaeli, Bita

    2015-01-01

    Benign mesenchymal tumors of the craniofacial complex present unique challenges for orbital surgeons because of their potential for orbital compartment syndrome, ocular morbidity, and facial disfigurement and because definitive surgical management may be associated with significant morbidity. While the precise classification of such lesions depends on radiologic as well as histologic evaluations and remains controversial, benign tumors involving the bony walls of the orbit share features of bony expansion, facial deformity, and the potential to cause significant orbital and ophthalmic morbidity. We herein present 2 cases of benign mesenchymal tumors with bony involvement in the orbitofacial region (1 juvenile ossifying fibroma and 1 central giant cell granuloma) and review the current management of similar benign fibro-osseous and reactive bone lesions of the orbit. These rare entities presented share common orbital and ophthalmic manifestations and remain without any effective definitive treatment options. PMID:27171013

  7. Ghost cell lesions

    PubMed Central

    Rajesh, E.; Jimson, Sudha; Masthan, K. M. K.; Balachander, N.

    2015-01-01

    Ghost cells have been a controversy for a long time. Ghost cell is a swollen/enlarged epithelial cell with eosnophilic cytoplasm, but without a nucleus. In routine H and E staining these cells give a shadowy appearance. Hence these cells are also called as shadow cells or translucent cells. The appearance of these cells varies from lesion to lesion involving odontogenic and nonodontogenic lesions. This article review about the origin, nature and significance of ghost cells in different neoplasms. PMID:26015694

  8. Superior oblique muscle paresis and restriction secondary to orbital mucocele.

    PubMed

    Pineles, Stacy L; Velez, Federico G; Elliot, Richard L; Rosenbaum, Arthur L

    2007-02-01

    Mucoceles are chronic cystic lesions of the paranasal sinuses lined by respiratory epithelium. Their extension into the adjacent orbit may result in proptosis, ocular motility disorders, and diplopia. Brown syndrome secondary to extension of a mucocele into the orbit has been reported previously. Superior oblique (SO) muscle weakness, either isolated or in combination with an ipsilateral limitation to elevation in adduction, has not been previously reported in patients with orbital mucocele.

  9. [Orbital leiomyoma. Case report and review of the literature].

    PubMed

    González-Martínez, Emilio; Robla Costales, Javier; Fernández Fernández, Javier; Viñuela Lobo, Jesús; Santamarta, David; Lomas García, Jesús; Iglesias, Elena; Ribas Ariño, Teresa; García-Cosamalón, José

    2014-01-01

    Leiomyoma are slowly growing lesions arising from smooth muscle. Orbital location has been reported in 25 cases. Histological findings and no recurrence after total resection support their benign behaviour. We report an intraconal orbital haemangioleiomyoma in a 55-year-old female treated by total resection through fronto-orbital craniotomy, with no recurrence after 15 months of follow-up. Radiological and pathological features are discussed, emphasising the prognostic role of the surgery.

  10. Preinvasive lesions

    Cancer.gov

    This definition is for allocation of lesions with preinvasive/borderline properties. It is currently aimed at newly identified neoplasms, which may be similar to those described in humans. In mouse pathology, many adenomas may be preinvasive/borderline lesions. However, their inclusion in the preinvasive category can be justified only upon development of better diagnostic criteria.

  11. Imaging Pediatric Vascular Lesions

    PubMed Central

    Nguyen, Tuyet A.; Krakowski, Andrew C.; Naheedy, John H.; Kruk, Peter G.

    2015-01-01

    Vascular anomalies are commonly encountered in pediatric and dermatology practices. Most of these lesions are benign and easy to diagnose based on history and clinical exam alone. However, in some cases the diagnosis may not be clear. This may be of particular concern given that vascular anomalies may occasionally be associated with an underlying syndrome, congenital disease, or serious, life-threatening condition. Defining the type of vascular lesion early and correctly is particularly important to determine the optimal approach to management and treatment of each patient. The care of pediatric patients often requires collaboration from a multitude of specialties including pediatrics, dermatology, plastic surgery, radiology, ophthalmology, and neurology. Although early characterization of vascular lesions is important, consensus guidelines regarding the evaluation and imaging of vascular anomalies does not exist to date. Here, the authors provide an overview of pediatric vascular lesions, current classification systems for characterizing these lesions, the various imaging modalities available, and recommendations for appropriate imaging evaluation. PMID:26705446

  12. Extragastric Dieulafoy's lesion

    PubMed Central

    Gauci, James; Galea, Samuel; Galea, Joseph; Schembri, Mark

    2014-01-01

    A 74-year-old man on warfarin for aortic valve replacement presented with recurrent episodes of melaena. An initial oesophagogastroduodenoscopy (OGD) was normal, as were red cell scanning and colonoscopy. It was a third OGD that revealed the cause of the melaena—a vascular lesion in the duodenum, at the junction between D1 and D2. An extragastric Dieulafoy's lesion was diagnosed, and the lesion was injected with epinephrine and tattooed. Over the following months, episodes of bleeding recurred despite further attempts at injection. Percutaneous radiologically assisted embolisation of the gastroduodenal artery, and eventually duodenotomy and oversuturing of the lesion were performed to no avail. The patient has undergone over 10 endoscopies, and has received over 70 units of packed red cells to date, since his initial presentation 6 years ago. Attempts to stop the bleeding permanently have been difficult, highlighting the complexity of managing such a lesion. PMID:25216921

  13. [Sinus histiocytosis with massive lymphadenopathy with complete occlusion of the superior and inferior vena cava].

    PubMed

    Runde, J; Ebbecke, H; Kurlemann, G; Frosch, M; Schuierer, G

    1997-01-01

    Sinus histiocytosis with massive lymphadenopathy (SHML) is a rare disease of the lymph nodes, still of unknown origin. We are reporting the case of a 16 year old boy with SHML which occurred in 1983. Investigations showed a massive lymphadenopathy of the mediastinal and abdominal nodes, causing displacement and compression of surrounding tissue. The patient further developed a blockage of the vena cava superior and inferior, leading to numerous collateral circulation routes in the upper and lower extremities. The etiology of the venous blockage is still disputed. It is possible that they are the result of compression of the major veins. Alternatively, the cause could lie in the disruption of the coagulation system. Finally and more likely, the problem could be the result of fibrosis developing through the healing process.

  14. Pulmonary Langerhans Cell Histiocytosis and Diabetes Insipidus in a Young Smoker

    PubMed Central

    Earlam, K.; Souza, C. A.; Glikstein, R.; Gomes, M. M.; Pakhalé, S.

    2016-01-01

    Langerhans cell histiocytosis is characterized by the abnormal nodular proliferation of histiocytes in various organ systems. Pulmonary involvement seen in young adults is nearly always seen in the context of past or current cigarette smoking. Although it tends to be a single-system disease, extrapulmonary manifestations involving the skin, bone, and hypothalamic-pituitary-axis are possible. High resolution CT (HRCT) of the thorax findings includes centrilobular nodules and cysts that are bizarre in shape, variable in size, and thin-walled. Often the diagnosis can be made based on the appropriate clinical presentation and typical imaging findings. Treatment includes smoking cessation and the potential use of glucocorticoids or cytotoxic agents depending on the severity of disease and multisystem involvement. PMID:27445532

  15. Dramatic and sustained responsiveness of pulmonary Langerhans cell histiocytosis-associated pulmonary hypertension to vasodilator therapy

    PubMed Central

    May, Adam; Kane, Garvan; Yi, Eunhee; Frantz, Robert; Vassallo, Robert

    2014-01-01

    Pulmonary Langerhans cell histiocytosis (PLCH) is an uncommon diffuse lung disease characterized by the abnormal accumulation of Langerhans' cells around small airways and other distal lung compartments. Although pulmonary hypertension (PH) is a frequent complication of PLCH, the role of advanced PH therapies for PLCH-related PH is not well-established. We describe a PLCH patient with severe, disease-related PH that responded unexpectedly well to advanced PH therapy with sustained improvement over a 10 year follow-up period. This case indicates that PLCH-associated PH may, in certain instances, be highly responsive to advanced PH therapies and emphasizes the importance of trialing these therapies among patients with PLCH-related PH. PMID:26029568

  16. Langerhans Cell Histiocytosis in an Infant Mimicking a Lymphoma at Presentation

    PubMed Central

    Madasu, Anjan; Noor Rana, Asim; Banat, Saleh; Humad, Hani; Mustafa, Rashid; AlJassmi, Abdulrahman Mohd

    2015-01-01

    Langerhans cell histiocytosis (LCH) is a rare disorder characterized by proliferation and accumulation of clonal dendritic cells with varied clinical presentation and an unpredictable course. We report a 5-month-old infant with LCH who presented with severe respiratory distress, a large mediastinal mass, significant generalized lymphadenopathy, and hepatosplenomegaly. Lymphoma, especially T cell lymphoblastic lymphoma, can present with superior mediastinal syndrome needing urgent empirical therapy without biopsy. However, lack of response prompted a biopsy which confirmed it to be a case of LCH and that leads to appropriate therapy and survival. There have been reports of LCH presenting with isolated mediastinal mass or with generalized lymphadenopathy, but the combined presentation of generalized lymphadenopathy with large mediastinal mass, hepatosplenomegaly, and fever in an infant has rarely been reported. Conclusion. LCH should also be considered in the differential diagnosis of an infant presenting with generalized lymphadenopathy, mediastinal mass, hepatosplenomegaly, and fever. PMID:26587301

  17. Pulmonary Langerhans Cell Histiocytosis and Diabetes Insipidus in a Young Smoker.

    PubMed

    Earlam, K; Souza, C A; Glikstein, R; Gomes, M M; Pakhalé, S

    2016-01-01

    Langerhans cell histiocytosis is characterized by the abnormal nodular proliferation of histiocytes in various organ systems. Pulmonary involvement seen in young adults is nearly always seen in the context of past or current cigarette smoking. Although it tends to be a single-system disease, extrapulmonary manifestations involving the skin, bone, and hypothalamic-pituitary-axis are possible. High resolution CT (HRCT) of the thorax findings includes centrilobular nodules and cysts that are bizarre in shape, variable in size, and thin-walled. Often the diagnosis can be made based on the appropriate clinical presentation and typical imaging findings. Treatment includes smoking cessation and the potential use of glucocorticoids or cytotoxic agents depending on the severity of disease and multisystem involvement. PMID:27445532

  18. Multifocal vascular lesions.

    PubMed

    Levin, Laura E; Lauren, Christine T

    2016-03-01

    Multifocal vascular lesions are important to recognize and appropriately diagnose. Generally first noticed on the skin, multifocal vascular lesions may have systemic involvement. Distinguishing among the different types of multifocal vascular lesions is often based on clinical features; however, radiological imaging and/or biopsy are frequently needed to identify distinct features and guide treatment. Knowledge of the systemic associations that can occur with different vascular anomalies may reduce life-threatening complications, such as coagulopathy, bleeding, cardiac compromise, and neurologic sequelae. This review provides a synopsis of the epidemiology, pathogenesis, presentation, workup, and treatment of several well-recognized multifocal vascular tumors and malformations. PMID:27607324

  19. Oral Lesions in Neonates

    PubMed Central

    Rao, Roopa S; Majumdar, Barnali; Jafer, Mohammed; Maralingannavar, Mahesh; Sukumaran, Anil

    2016-01-01

    ABSTRACT Oral lesions in neonates represent a wide range of diseases often creating apprehension and anxiety among parents. Early examination and prompt diagnosis can aid in prudent management and serve as baseline against the future course of the disease. The present review aims to enlist and describe the diagnostic features of commonly encountered oral lesions in neonates. How to cite this article: Patil S, Rao RS, Majumdar B, Jafer M, Maralingannavar M, Sukumaran A. Oral Lesions in Neonates. Int J Clin Pediatr Dent 2016;9(2):131-138. PMID:27365934

  20. Retinal lesions in septicemia.

    PubMed

    Neudorfer, M; Barnea, Y; Geyer, O; Siegman-Igra, Y

    1993-12-15

    We explored the association between septicemia and specific retinal lesions in a prospective controlled study. Hemorrhages, cotton-wool spots, or Roth's spots were found in 24 of 101 septicemic patients (24%), compared to four of 99 age- and gender-matched control patients (4%) (P = .0002). There was no significant association between types of organisms or focus of infection and the presence of specific lesions. Histologic examination of affected eyes disclosed cytoid bodies in the nerve fiber layer without inflammation. A definite association between septicemia and retinal lesions was found and indicates the need for routine ophthalmoscopy in septicemic patients. PMID:8250076

  1. Immature sinus histiocytosis. Light- and electron-microscopic features, immunologic phenotype, and relationship with marginal zone lymphocytes.

    PubMed Central

    van den Oord, J. J.; de Wolf-Peeters, C.; De Vos, R.; Desmet, V. J.

    1985-01-01

    The light-microscopic, ultrastructural, and immunohistochemical features of immature sinus histiocytosis were studied in 10 lymph nodes with the histologic picture of toxoplasmic lymphadenitis and compared with the features of lymphoid cells present in the marginal zone of the splenic white pulp. Areas of immature sinus histiocytosis consisted largely of medium-sized lymphoid cells with markedly irregular nuclei and abundant pale cytoplasm. Using a panel of monoclonal antibodies, the predominating lymphoid cells were found to carry the B-cell phenotype B1+Ba1-sIgM+sIgD-OKIa1+. Admixed were variable numbers of larger, blastic lymphoid cells, small lymphocytes, histiocytic elements, and polymorphonuclear granulocytes. The marginal zone of the splenic white pulp was composed of a similar mixture of cells, and marginal-zone lymphocytes demonstrated an analogous immunohistochemical phenotype. Our results indicate that immature sinus histiocytes are B-lymphoid cells that are closely related to marginal zone lymphocytes. As such, immature sinus histiocytes may have a role similar to that of marginal-zone lymphocytes, which have been claimed to transport antigens or immune complexes toward the follicular center or to serve as precursors of plasma cells. We suggest that immature sinus histiocytosis represents an abnormal expansion of the marginal zone, normally present at the sinusoidal pole of lymphoid follicles. The reason for this marginal-zone hyperplasia, recognized as immature sinus histiocytosis in a variety of reactive lymph node conditions, may be a maturation arrest in the normal development of immature sinus histiocytes into small, sIgM+ sIgD+ lymphocytes. Images Figure 3 Figure 1 Figure 2 Figure 4 Figure 5 Figure 6 Figure 7 Figure 8 Figure 9 Figure 10 Figure 11 Figure 12 Figure 13 Figure 14 PMID:3970140

  2. Skin lesion of blastomycosis

    MedlinePlus

    ... in: Africa Canada Central and southeastern United States India Israel Saudi Arabia A person gets infected by ... is diagnosed by identifying the fungus in a culture taken from a skin lesion. This usually requires ...

  3. Acute nontraumatic liver lesions.

    PubMed

    Caremani, Marcello; Tacconi, Danilo; Lapini, Laura

    2013-11-26

    The principal conditions requiring emergency/urgent intervention in patients with nontraumatic liver lesions are hemorrhage (with or without tumor rupture), rupture of hydatid cysts (with or without infection), complications arising from liver abscesses or congenital liver cysts, rupture related to peliosis hepatis, and in rare cases spontaneous hemorrhage. This article examines each of these conditions, its appearance on ultrasound (the first-line imaging method of choice for assessing any urgent nontraumatic liver lesion) and indications for additional imaging studies.

  4. [Osteoarticular lesions from parachuting].

    PubMed

    Orso, C A; Valbonesi, L; Calabrese, B F; D'Onofrio, S

    1990-01-01

    Based on personal experience gained in a parachuting centre (Pescara Aero-club) from 1975 up to 1988, the authors report their evaluation on chronic and acute osteoarticular lesions. The review of the cases was not based on the incidence of the lesions nor on their characteristics, normally found in common traumatology, but it was related to the dynamics of the trauma during the landing and to painful syndromes following a prolonged parachuting activity.

  5. Theory of satellite orbit-orbit resonance

    NASA Technical Reports Server (NTRS)

    Blitzer, L.; Anderson, J. D.

    1981-01-01

    On the basis of the strong mathematical and physical parallels between orbit-orbit and spin-orbit resonances, the dynamics of mutual orbit perturbations between two satellites about a massive planet are examined, exploiting an approach previously adopted in the study of spin-orbit coupling. Resonances are found to exist when the mean orbital periods are commensurable with respect to some rotating axis, which condition also involves the apsidal and nodal motions of both satellites. In any resonant state the satellites are effectively trapped in separate potential wells, and a single variable is found to describe the simultaneous librations of both satellites. The librations in longitude are 180 deg out-of-phase, with fixed amplitude ratio that depends only on their relative masses and semimajor axes. The theory is applicable to Saturn's resonant pairs Titan-Hyperion and Mimas-Tethys, and in these cases the calculated libration periods are in reasonably good agreement with the observed periods.

  6. Lunar Reconnaissance Orbiter Orbit Determination Accuracy Analysis

    NASA Technical Reports Server (NTRS)

    Slojkowski, Steven E.

    2014-01-01

    Results from operational OD produced by the NASA Goddard Flight Dynamics Facility for the LRO nominal and extended mission are presented. During the LRO nominal mission, when LRO flew in a low circular orbit, orbit determination requirements were met nearly 100% of the time. When the extended mission began, LRO returned to a more elliptical frozen orbit where gravity and other modeling errors caused numerous violations of mission accuracy requirements. Prediction accuracy is particularly challenged during periods when LRO is in full-Sun. A series of improvements to LRO orbit determination are presented, including implementation of new lunar gravity models, improved spacecraft solar radiation pressure modeling using a dynamic multi-plate area model, a shorter orbit determination arc length, and a constrained plane method for estimation. The analysis presented in this paper shows that updated lunar gravity models improved accuracy in the frozen orbit, and a multiplate dynamic area model improves prediction accuracy during full-Sun orbit periods. Implementation of a 36-hour tracking data arc and plane constraints during edge-on orbit geometry also provide benefits. A comparison of the operational solutions to precision orbit determination solutions shows agreement on a 100- to 250-meter level in definitive accuracy.

  7. Computer-assisted orbital surgery.

    PubMed

    Klimek, L; Wenzel, M; Mösges, R

    1993-06-01

    In orbital surgery, deep lesions must be operated on through extremely small approaches. We developed a method (Computer Assisted Surgery or CAS) designed to achieve safer and more precise surgery by providing highly accurate intraoperative information regarding the location of the surgical instrument. The device involved consists of a high-capacity computer that processes computed tomographic (CT) data in real time, connected to a mechanical measuring arm attached to the surgical instrument. From the CT data, the computer calculates a three-dimensional reconstruction and displays the tip of the instrument as a reticle in this model. Using the CAS system, the surgeon is continuously apprised of the precise position of the surgical instrument vis-a-vis all relevant anatomical structures. We have used CAS successfully in 21 cases of orbital surgery. We have found the system especially useful in dealing with deep-seated tumors, either for biopsy or surgery; in performing orbital and optic nerve decompression; in localizing deep-seated foreign bodies; and in all cases of impaired orientation due to severe bleeding, tumor-destroyed landmarks, or preoperated anatomy. Although the number of patients is too small to yield statistically significant data allowing a comparison of patient outcomes with and without using the system, we have just begun a large prospective clinical trial designed to provide such data. PMID:8336894

  8. A multicentre retrospective survey of Langerhans' cell histiocytosis: 348 cases observed between 1983 and 1993. The French Langerhans' Cell Histiocytosis Study Group.

    PubMed Central

    1996-01-01

    In a retrospective study involving 32 haematology/oncology departments in France, 348 cases of Langerhans' cell histiocytosis diagnosed between 1983 and 1993 were collated. The percentage of males was 56.4%. Median age at diagnosis was 30.2 months. The median follow up was 35.5 months. Initially, 108 patients (31%) had isolated unifocal or bifocal bone involvement, 67 (19%) had isolated multifocal bone involvement, 136 (39%) had soft tissue involvement without organ dysfunction, and 37 (11%) had organ dysfunction. Two thirds of the sites of involvement diagnosed throughout the course of the disease were present at diagnosis, while the remaining one third appeared during a relapse. Treatment was tailored to the individual patient and was extremely varied, hampering any comparison of regimens. Vinblastine with or without steroids was the most common regimen when systemic chemotherapy was used for the first episode (246/348). Twenty four of the 216 patients received VP 16 as first line treatment. Two patients with progressive multiorgan relapse, despite the use of several drugs, underwent bone marrow transplantation and are alive and disease free 60 and 22 months later. Altogether 21.9% of patients had sequelae, including diabetes insipidus in 17.5% of cases. The overall survival rate is 91.7% (confidence interval 90.7 to 95%) three years after diagnosis. In the univariate analysis, age less than 1 year, ear, nose, and throat, cutaneous, lymph node, liver, spleen, lung, marrow and intestinal involvement, male sex, progressive episodes, the absence of response, and partial responses, were associated with a poor vital prognosis. In a multivariate analysis of prognostic factors, poor early outcome emerged as the most important parameter, closely linked to other poor outcome features such as young age and organ dysfunction. It identified a small number of patients with a poor initial response to treatment, for whom intensive treatment should be assessed in a phase II

  9. Meniscal Ramp Lesions

    PubMed Central

    Chahla, Jorge; Dean, Chase S.; Moatshe, Gilbert; Mitchell, Justin J.; Cram, Tyler R.; Yacuzzi, Carlos; LaPrade, Robert F.

    2016-01-01

    Meniscal ramp lesions are more frequently associated with anterior cruciate ligament (ACL) injuries than previously recognized. Some authors suggest that this entity results from disruption of the meniscotibial ligaments of the posterior horn of the medial meniscus, whereas others support the idea that it is created by a tear of the peripheral attachment of the posterior horn of the medial meniscus. Magnetic resonance imaging (MRI) scans have been reported to have a low sensitivity, and consequently, ramp lesions often go undiagnosed. Therefore, to rule out a ramp lesion, an arthroscopic evaluation with probing of the posterior horn of the medial meniscus should be performed. Several treatment options have been reported, including nonsurgical management, inside-out meniscal repair, or all-inside meniscal repair. In cases of isolated ramp lesions, a standard meniscal repair rehabilitation protocol should be followed. However, when a concomitant ACL reconstruction (ACLR) is performed, the rehabilitation should follow the designated ACLR postoperative protocol. The purpose of this article was to review the current literature regarding meniscal ramp lesions and summarize the pertinent anatomy, biomechanics, diagnostic strategies, recommended treatment options, and postoperative protocol. PMID:27504467

  10. Monitoring pigmented skin lesions

    NASA Astrophysics Data System (ADS)

    Wallace, Vincent P.; Bamber, Jeffery C.; Ott, Robert J.; Crawford, Diane C.; Mortimer, Peter S.

    2002-06-01

    The rising incidence of skin cancer has led to an increase in the number of patients with skin lesions that require diagnosis, mostly using subjective visual examination. Successful treatment depends on early diagnosis. Unfortunately diagnostic accuracy, even by experts, can be as low as 56%; therefore, an accurate, objective diagnostic aid is greatly needed. Reflectance characteristics of pigmented skin lesions were documented to evaluate their diagnostic potential. Reflectance spectra in the wavelength range 320-1100nm were obtained from 260 lesions. Differences between spectra from benign and malignant lesions were utilized by extracting features with the best discriminating power. Discrimination was evaluated using two techniques: multivariate statistical analysis and artificial neural networks, using histology as the standard. Each technique was tested in a blind study and assessed in terms of its ability to diagnose new cases and compared to the clinical diagnosis. The artificial neural network achieved the best diagnostic performance for discriminating between malignant melanoma and benign nevi, having a sensitivity of 100% and a specificity of 65%. Utilization of visible and infrared techniques for monitoring skin lesions has lead to improvements in diagnostic accuracy. We conclude that these techniques are worthy of further development and evaluation in clinical practice as a screening tool.

  11. Efficient orbit integration by orbital longitude methods

    NASA Astrophysics Data System (ADS)

    Fukushima, Toshio

    Recently we developed a new formulation of numerical integration of orbital motion named manifold correction methods. The main trick is to keep rigorously the consistency of some physical relations such as that of the orbital energy, of the orbital angular momentum, or of the Laplace integral of a binary subsystem. This maintenance is done by applying a sort of correction to the integrated variables at every integration step. Typical methods of correction are certain geometric transformation such as the spatial scaling and the spatial rotation, which are commonly used in the comparison of reference frames, or mathematically-reasonable operations such as the modularization of angle variables into the standard domain [-π, π). The finally-evolved form of the manifold correction methods is the orbital longitude methods, which enable us to conduct an extremely precise integration of orbital motions. In the unperturbed orbits, the integration errors are suppressed at the machine epsilon level for an infinitely long period. In the perturbed cases, on the other hand, the errors initially grow in proportion to the square root of time and then increase more rapidly, the onset time of which depends on the type and the magnitude of perturbations. This feature is also realized for highly eccentric orbits by applying the same idea to the KS-regularization. Expecially the introduction of time element greatly enhances the performance of numerical integration of KS-regularized orbits whether the scaling is applied or not.

  12. Rare Form of Erdheim-Chester Disease Presenting with Isolated Central Skeletal Lesions Treated with a Combination of Alfa-Interferon and Zoledronic Acid

    PubMed Central

    Bulycheva, E. N.; Baykov, V. V.; Zaraĭskiĭ, M. I.; Salogub, G. N.

    2015-01-01

    Erdheim-Chester disease (ECD) represents a clonal non-Langerhans histiocytosis, which manifests under an extensive variety of clinical symptoms. This creates a challenge for the physician, who is required to recognize and diagnose the disease in the early stages. Despite this considerable challenge, in the last decade there has been a dramatic increase in ECD diagnoses, in most part due to an increasing awareness of this rare disorder. Involvement of the axial skeleton is exclusively uncommon with no official recommendations for the treatment of the bone lesions. Here, we present a case report of a young male patient with isolated lesions of the spine, ribs, and pelvis, who was successfully treated with a combination therapy of alfa-interferon and zoledronic acid. PMID:25949835

  13. Intraventricular mass lesions

    SciTech Connect

    Morrison, G.; Sobel, D.F.; Kelley, W.M.; Norman, D.

    1984-11-01

    Determining the precise etiology of an intraventricular mass can be a difficult diagnostic problem. CT and angiographic findings were reviewed in a series of 73 patients who had intraventricular masses. The histologic diagnosis can be suggested preoperatively by an analysis of the frequency of lesions occurring at a given ventricular location, lesion density before and after administration of contrast material, age, and sex of the patient, morphologic appearance of the mass, and presence or absence of hydrocephalus. Angiography is useful when meningioma, choroid plexus papilloma and carcinoma, or arteriovenous malformation are considered.

  14. Lunar orbiting prospector

    NASA Technical Reports Server (NTRS)

    1988-01-01

    One of the prime reasons for establishing a manned lunar presence is the possibility of using the potential lunar resources. The Lunar Orbital Prospector (LOP) is a lunar orbiting platform whose mission is to prospect and explore the Moon from orbit in support of early lunar colonization and exploitation efforts. The LOP mission is divided into three primary phases: transport from Earth to low lunar orbit (LLO), operation in lunar orbit, and platform servicing in lunar orbit. The platform alters its orbit to obtain the desired surface viewing, and the orbit can be changed periodically as needed. After completion of the inital remote sensing mission, more ambitious and/or complicated prospecting and exploration missions can be contemplated. A refueled propulsion module, updated instruments, or additional remote sensing packages can be flown up from the lunar base to the platform.

  15. [Orbital hernias: new views of the pathogenesis, possibilities of correction].

    PubMed

    Lutsevich, E E

    2006-01-01

    The paper deals with the basic pathogenetic aspects of development of orbital hernias--the factors of a tarsoorbital fascial change in the presence of the hereditary syndrome of connective tissue hyperplasticity and elevated intraorbital pressure, which affects the volume of orbital fat. The possibilities of a differential diagnosis of orbital hernias and eyelid edemas are considered. There is a biomechanical association of orbital hernias with acquired age-related enophthalmos. The examples of impairments in the tolerance of the optic nerve and in the development of optic neuropathy in enophthalmos are considered. The fact that there may be tarsoorbital fascial lesions, followed by the development of orbital hernias after parabulbar injections is indicated. The author proposes an operation dealing with the reposition of orbital hernias instead of their resection during blepharoplastic interventions. PMID:17217192

  16. Magnetic resonance imaging of liver lesions: exceptions and atypical lesions.

    PubMed

    van den Bos, Indra C; Hussain, Shahid M; de Man, Robert A; Zondervan, Pieter E; Ijzermans, Jan N M; Preda, A; Krestin, Gabriel P

    2008-01-01

    On state-of-the-art magnetic resonance imaging, most lesions can be detected and characterized with confidence according to well-known criteria. However, atypical characteristics in some common lesions and the incidental encounter with rare lesions may pose diagnostic difficulties. In this article, six challenging hepatic lesions will be discussed and evaluated on the most important magnetic resonance imaging sequences, with histological correlation when available. In addition, the background information concerning these lesions will be described based on the most recent available literature. By reading this article, the reader will be able to (1) categorize the lesion in solid and fluid-containing lesions, based on the T2 signal intensity; and (2) define the benign or malignant nature of the lesion, in relation to the signal intensity and dynamic enhancement pattern, despite the presence of atypical characteristics of some lesions. PMID:18436109

  17. Introducing Earth's Orbital Eccentricity

    ERIC Educational Resources Information Center

    Oostra, Benjamin

    2015-01-01

    Most students know that planetary orbits, including Earth's, are elliptical; that is Kepler's first law, and it is found in many science textbooks. But quite a few are mistaken about the details, thinking that the orbit is very eccentric, or that this effect is somehow responsible for the seasons. In fact, the Earth's orbital eccentricity is…

  18. Five Equivalent d Orbitals

    ERIC Educational Resources Information Center

    Pauling, Linus; McClure, Vance

    1970-01-01

    Amplifies and clarifies a previous paper on pyramidal d orbitals. Discusses two sets of pyramid d orbitals with respect to their maximum bond strength and their symmetry. Authors described the oblate and prolate pentagonal antiprisms arising from the two sets of five equivalent d orbitals. (RR)

  19. SEASAT B orbit synthesis

    NASA Technical Reports Server (NTRS)

    Rea, F. G.; Warmke, J. M.

    1976-01-01

    Addition were made to Battelle's Interactive Graphics Orbit Selection (IGOS) program; IGOS was exercised via telephone lines from JPL, and candidate SEASAT orbits were analyzed by Battelle. The additions to the program enable clear understanding of the implications of a specific orbit to the diverse desires of the SEASAT user community.

  20. Ultrasound features of orbital granular cell tumor.

    PubMed

    Ayres, Bernadete; Miller, Neil R; Eberhart, Charles G; Dibernardo, Cathy W

    2009-01-01

    The authors report the echographic characteristics of a rare orbital granular cell tumor and correlate these findings with histopathology. A 56-year-old woman presented with proptosis. Complete ophthalmic and ultrasound examinations were performed. Ultrasound revealed an oval, well-outlined orbital mass in the intraconal space with low-medium reflectivity and regular internal structure. An orbitotomy with complete excision of the tumor was performed. Histopathologic evaluation showed sheets and nests of cells with abundant eosinophilic and granular cytoplasm in a uniform distribution throughout the lesion. The echographic characteristics correlated well with the morphologic surgical findings and the histologic architecture. This is the first report describing the echographic characteristics of orbital granular cell tumor.

  1. Cemento-ossifying Fibroma Of Paranasal Sinus Presenting Acutely As Orbital Cellulitis.

    PubMed

    Khanna, Maneesh; Buddhavarapu, Shanker Rao; Hussain, Sheik Akbar; Amir, Emran

    2009-01-01

    Fibro-osseous lesions of the face and paranasal sinuses are relatively uncommon. These lesions have overlapping clinical, radiologic and pathologic features causing difficulty in diagnosis. Neoplastic fibro-osseous paranasal sinus lesions can be benign or malignant. The benign fibro-osseous lesions described are: ossifying fibroma (and its histologic variants) and fibrous dysplasia. The variants of ossifying fibroma differ in the nature of calcified material (i.e. cementum versus bone), in the location of the lesion (oral versus paranasal sinus or orbital), other morphologic variations (presence of psammomatoid concretions) and biologic behavior (aggressive versus stable). Presence of cementum or bone classifies the lesion as cementifying fibroma or ossifying fibroma respectively while lesions with mixture of both cementum and bone are called cemento-ossifying fibroma. We describe a case of a young adult male with cemento-ossifying fibroma of paranasal sinus presenting acutely as left orbital cellulitis with proptosis.

  2. Orbit Software Suite

    NASA Technical Reports Server (NTRS)

    Osgood, Cathy; Williams, Kevin; Gentry, Philip; Brownfield, Dana; Hallstrom, John; Stuit, Tim

    2012-01-01

    Orbit Software Suite is used to support a variety of NASA/DM (Dependable Multiprocessor) mission planning and analysis activities on the IPS (Intrusion Prevention System) platform. The suite of Orbit software tools (Orbit Design and Orbit Dynamics) resides on IPS/Linux workstations, and is used to perform mission design and analysis tasks corresponding to trajectory/ launch window, rendezvous, and proximity operations flight segments. A list of tools in Orbit Software Suite represents tool versions established during/after the Equipment Rehost-3 Project.

  3. Lunar Reconnaissance Orbiter Orbit Determination Accuracy Analysis

    NASA Technical Reports Server (NTRS)

    Slojkowski, Steven E.

    2014-01-01

    LRO definitive and predictive accuracy requirements were easily met in the nominal mission orbit, using the LP150Q lunar gravity model. center dot Accuracy of the LP150Q model is poorer in the extended mission elliptical orbit. center dot Later lunar gravity models, in particular GSFC-GRAIL-270, improve OD accuracy in the extended mission. center dot Implementation of a constrained plane when the orbit is within 45 degrees of the Earth-Moon line improves cross-track accuracy. center dot Prediction accuracy is still challenged during full-Sun periods due to coarse spacecraft area modeling - Implementation of a multi-plate area model with definitive attitude input can eliminate prediction violations. - The FDF is evaluating using analytic and predicted attitude modeling to improve full-Sun prediction accuracy. center dot Comparison of FDF ephemeris file to high-precision ephemeris files provides gross confirmation that overlap compares properly assess orbit accuracy.

  4. Efficient orbit integration by orbital longitude methods

    NASA Astrophysics Data System (ADS)

    Fukushima, T.

    2005-09-01

    Triggered by the desire to investigate numerically the planetary precession through a long-term numerical integration of the solar system, we developed a new formulation of numerical integration of orbital motion named manifold correction methods. The main trick is to keep rigorously the consistency of some physical relations such as that of the orbital energy, of the orbital angular momentum, or of the Laplace integral of a binary subsystem. This maintenance is done by applying a sort of correction to the integrated variables at every integration step. Typical methods of correction are certain geometric transformation such as the spatial scaling and the spatial rotation, which are commonly used in the comparison of reference frames, or mathematically-reasonable operations such as the modularization of angle variables into the standard domain [-π,π). The finally-evolved form of the manifold correction methods is the orbital longitude methods, which enable us to conduct an extremely precise integration of orbital motions. In the unperturbed orbits, the integration errors are suppressed at the machine epsilon level for an infinitely long period. In the perturbed cases, on the other hand, the errors initially grow in proportion to the square root of time and then increase more rapidly, the onset time of which depends on the type and the magnitude of perturbations. This feature is also realized for highly eccentric orbits by applying the same idea to the KS-regularization. Especially the introduction of time element greatly enhances the performance of numerical integration of KS-regularized orbits whether the scaling is applied or not.

  5. Langerhans cell histiocytosis: a retrospective analysis in a Korean tertiary hospital from 2003 to 2012.

    PubMed

    Kwon, Soon Hyo; Choi, Jae Woo; Kim, Hyo Jin; Youn, Sang Woong

    2013-10-01

    Epidemiological study of Langerhans cell histiocytosis (LCH) has been limited due to its rarity and multisystemic involvement. The aim of this study was to investigate the epidemiological features of LCH via the clinical data warehouse (CDW). Clinical data of 30 LCH patients from the all departments of a tertiary referral hospital between 2003 and 2012 were analyzed retrospectively by searching the CDW. The male-to-female ratio was 2.8:1. The age of onset ranged 7 days to 57 years with a median of 13 years. Of the patients, 36.7% presented initial symptoms before the age of 10 years. The involved organs at diagnosis were: bone (66.7%), skin (16.7%), lungs (13.3%) and lymph node (3.3%). For all of the 30 cases, there were 31 disease sites because of a single case of multisystemic disease involving both skin and bone. Of the 96.7% of patients with single-system disease, 69.0% had bony involvement. This study elucidated the clinical features of LCH from all the departments of a tertiary hospital via the CDW, which suggests a potential role of the CDW as a new epidemiological approach for rare diseases.

  6. Cladribine and cytarabine in refractory multisystem Langerhans cell histiocytosis: results of an international phase 2 study

    PubMed Central

    Bernard, Frederic; van Noesel, Max; Barkaoui, Mohamed; Bardet, Odile; Mura, Rosella; Arico, Maurizio; Piguet, Christophe; Gandemer, Virginie; Armari Alla, Corinne; Clausen, Niels; Jeziorski, Eric; Lambilliote, Anne; Weitzman, Sheila; Henter, Jan Inge; Van Den Bos, Cor

    2015-01-01

    An international phase 2 study combining cladribine and cytarabine (Ara-C) was initiated for patients with refractory, risk-organ–positive Langerhans cell histiocytosis (LCH) in 2005. The protocol, comprising at least two 5-day courses of Ara-C (1 g/m2 per day) plus cladribine (9 mg/m2 per day) followed by maintenance therapy, was administered to 27 patients (median age at diagnosis, 0.7 years; median follow-up, 5.3 years). At inclusion, all patients were refractory after at least 1 course of vinblastine (VBL) plus corticosteroid, all had liver and spleen involvement, and 25 patients had hematologic cytopenia. After 2 courses, disease status was nonactive (n = 2), better (n = 23), or stable (n = 2), with an overall response rate of 92%. Median disease activity scores decreased from 12 at the start of therapy to 3 after 2 courses (P < .0001). During maintenance therapy, 4 patients experienced reactivation in risk organs. There were 4 deaths; 2 were related to therapy toxicity and 2 were related to reactivation. All patients experienced severe toxicity, with World Health Organization grade 4 hematologic toxicity and 6 documented severe infections. The overall 5-year survival rate was 85% (95% confidence interval, 65.2%-94.2%). Thus, the combination of cladribine/Ara-C is effective therapy for refractory multisystem LCH but is associated with high toxicity. PMID:26194764

  7. Abnormal cell surface antigen expression in individuals with variant CD45 splicing and histiocytosis.

    PubMed

    Boxall, Sally; McCormick, James; Beverley, Peter; Strobel, Stephan; De Filippi, Paola; Dawes, Ritu; Klersy, Catherine; Clementi, Rita; De Juli, Emanuella; Ferster, Aline; Wallace, Diana; Aricò, Maurizio; Danesino, Cezare; Tchilian, Elma

    2004-03-01

    Hemophagocytic lymphohistiocytosis (HLH) and Langerhans cell histiocytosis (LCH) are members of a group of rare heterogenous disorders, the histiocytoses, characterized by uncontrolled accumulation of pleomorphic infiltrates of leukocytes. The etiology of these diseases is mainly unknown. CD45 is a hemopoietic cell specific tyrosine phosphatase essential for antigen receptor mediated signaling in lymphocytes and different patterns of CD45 splicing are associated with distinct functions. Recently a polymorphism (C77G) in exon 4 of CD45 causing abnormal CD45 splicing and a point mutation affecting CD45 dimerization were implicated in multiple sclerosis in humans and lymphoproliferation and autoimmunity in mice respectively. Here we show that two patients with HLH exhibited abnormal CD45 splicing caused by the C77G variant allele, while a further 21 HLH patients have normal CD45. We have also examined 62 LCH patients and found three to have the C77G mutation. Peripheral blood thymus-derived (T) CD8(+) cells from normal individuals carrying the C77G mutation show a significant decrease in the proportion of cells expressing L-selectin and increased frequency of cells with LFA-1(hi) expression. It remains to be established whether C77G is a contributing factor in these histiocytic disorders. PMID:14630980

  8. Haematopoietic Stem Cell Transplantation for Refractory Langerhans Cell Histiocytosis: Outcome by Intensity of Conditioning

    PubMed Central

    Veys, Paul A.; Nanduri, Vasanta; Baker, K. Scott; He, Wensheng; Bandini, Giuseppe; Biondi, Andrea; Dalissier, Arnaud; Davis, Jeffrey H.; Eames, Gretchen M.; Egeler, R. Maarten; Filipovich, Alexandra H.; Fischer, Alain; Jürgens, Herbert; Krance, Robert; Lanino, Edoardo; Leung, Wing H.; Matthes, Susanne; Michel, Gérard; Orchard, Paul J.; Pieczonka, Anna; Ringdén, Olle; Schlegel, Paul G.; Sirvent, Anne; Vettenranta, Kim; Eapen, Mary

    2015-01-01

    Summary Patients with Langerhans cell histiocytosis (LCH) refractory to conventional chemotherapy have a poor outcome. There are currently two promising treatment strategies for high-risk patients: the first involves the combination of 2-chlorodeoxyadenosine and cytarbine; the other approach is allogeneic haematopoietic stem cell transplantation (HSCT). Here we evaluated 87 patients with high-risk LCH who were transplanted between 1990–2013. Prior to the year 2000, most patients underwent HSCT following myeloablative conditioning (MAC): only 5 of 20 patients (25%) survived with a high rate (55%) of transplant-related mortality (TRM). After the year 2000 an increasing number of patients underwent HSCT with reduced intensity conditioning (RIC): 49/67 (73%) patients survived, however, the improved survival was not overtly achieved by the introduction of RIC regimens with similar 3-year probability of survival after MAC (77%) and RIC transplantation (71%). There was no significant difference in TRM by conditioning regimen intensity but relapse rates were higher after RIC compared to MAC regimens (28% vs. 8%, p=0.02), although most patients relapsing after RIC transplantation could be salvaged with further chemotherapy. HSCT may be a curative approach in 3 out of 4 patients with high risk LCH refractory to chemotherapy: the optimal choice of HSCT conditioning remains uncertain. PMID:25817915

  9. A solitary pulmonary ground-glass nodule in adult systemic langerhans’ cell histiocytosis

    PubMed Central

    Zhou, Ying; Li, Yunyuan; Fan, Li; Liu, Shiyuan

    2015-01-01

    During a thoracic computed tomography (CT) scan, a 36-year-old male was diagnosed with a solitary oval pulmonary mixed ground-glass nodule in the right upper lobe of the lung. The edge of the nodule was well-defined, and its largest axial size was approximately 1.1×0.9 cm2. This nodule was slightly lobulated, but not obviously speculated. Solid components, micro-cystic lucency shadow, small high-density rings and tiny vascular branches were all visible in the nodule. During hospitalization, a technetium 99 m methylene diphosphonate (Tc-99 m MDP) bone scan was performed, which showed a skeletal foci with abnormal uptake in the left iliac. A pulmonary lobectomy of the right upper lobe of the lung by video-assisted thoracoscopy was performed. In post-operative pathological photomicrographs, proliferative Langerhans’ cells, eosinophils and lymphocytes were found. Immunohistochemistry showed that the expression of S-100 protein, CD1a, and CD68 antigen all stained positive. Since Langerhans’ cell histiocytosis (LCH) that is also associated with isolated mixed ground-glass nodules is relatively rare, such a multi-systemic LCH case as identified herein, is reported. PMID:26722574

  10. [Diseases of the orbit].

    PubMed

    Lukasik, S; Betkowski, A; Cyran-Rymarz, A; Szuber, D

    1995-01-01

    Diseases of the orbital cavity require more attention because of its specific anatomic structure and placement. Their curing requires cooperation of many medical specialties. Analysis consider orbital fractures, mainly caused by car accidents (69.2%). The next half of them consider inflammatory processes and tumor in equal numbers. Malignant tumors of orbital cavity occur most frequently (48.0%), less frequent are pseudotumors--pseudotumor orbitae (36.0%) and rare--malignant ones (16.0%). Malignant tumors more frequently infiltrate the orbit in neighborhood (63.3%), less frequently they come out from orbit tissue (16.7%). It should be emphasized that the number of orbit inflammations decreases in subsequent years, whereas occurrence of orbit tumors increases. PMID:9454170

  11. [Managing focal incidental renal lesions].

    PubMed

    Nicolau, C; Paño, B; Sebastià, C

    2016-01-01

    Incidental renal lesions are relatively common in daily radiological practice. It is important to know the different diagnostic possibilities for incidentally detected lesions, depending on whether they are cystic or solid. The management of cystic lesions is guided by the Bosniak classification. In solid lesions, the goal is to differentiate between renal cancer and benign tumors such as fat-poor angiomyolipoma and oncocytoma. Radiologists need to know the recommendations for the management of these lesions and the usefulness of the different imaging techniques and interventional procedures in function of the characteristics of the incidental lesion and the patient's life expectancy.

  12. Novel lesion detection aids.

    PubMed

    Neuhaus, K W; Longbottom, C; Ellwood, R; Lussi, A

    2009-01-01

    Several non-invasive and novel aids for the detection of (and in some cases monitoring of) caries lesions have been introduced in the field of 'caries diagnostics' over the last 15 years. This chapter focusses on those available to dentists at the time of writing; continuing research is bound to lead to further developments in the coming years. Laser fluorescence is based on measurements of back-scattered fluorescence of a 655-nm light source. It enhances occlusal and (potentially) approximal lesion detection and enables semi-quantitative caries monitoring. Systematic reviews have identified false-positive results as a limitation. Quantitative light-induced fluorescence is another sensitive method to quantitatively detect and measure mineral loss both in enamel and some dentine lesions; again, the trade-offs with lower specificity when compared with clinical visual detection must be considered. Subtraction radiography is based on the principle of digitally superimposing two radiographs with exactly the same projection geometry. This method is applicable for approximal surfaces and occlusal caries involving dentine but is not yet widely available. Electrical caries measurements gather either site-specific or surface-specific information of teeth and tooth structure. Fixed-frequency devices perform best for occlusal dentine caries but the method has also shown promise for lesions in enamel and other tooth surfaces with multi-frequency approaches. All methods require further research and further validation in well-designed clinical trials. In the future, they could have useful applications in clinical practice as part of a personalized, comprehensive caries management system. PMID:19494675

  13. Long-Term Extracorporeal Membrane Oxygenation as Bridging Strategies to Lung Transplantation in Rapidly Devastating Isolated Langerhans Cell Histiocytosis.

    PubMed

    Sacco, Oliviero; Moscatelli, Andrea; Conte, Massimo; Grasso, Chiara; Magnano, Gian Michele; Sementa, Angela Rita; Martelli, Alberto; Rossi, Giovanni A

    2016-05-01

    Isolated pulmonary involvement in pediatric Langerhans cell histiocytosis (LCH) is extremely rare. While the multisystem-LCH course varies from spontaneous remission to rapid deterioration with lethal outcome, single system involvement is generally associated with favorable prognosis. A child with isolated pulmonary LCH had an extremely rapid progression leading to respiratory failure, despite treatment with prednisone and vinblastine. Since lung hyperinflation and cystic degeneration contraindicated conventional mechanical ventilation, extracorporeal membrane oxygenation (ECMO) was chosen for 50 days as a bridge to lung transplantation. The mechanisms involved in disease progression and the usefulness of long-term ECMO are discussed. PMID:26840616

  14. [Lymph node eosinophilic granuloma. Apropos of 2 cases of Langerhans-cell histiocytosis with isolated lymph node involvement].

    PubMed

    Robert, M; Marty-Double, C

    1996-01-01

    The authors report two cases of isolated lymph node involvement by Langerhans' cell histiocytosis which affected two young children. The histologic aspect reveals that lymph nodes have been modified by a proliferation of large histiocyte-like cells, associated with eosinophils. An immunohistochemical study on paraffin sections and for one case on frozen sections, reveals the usual phenotype of Langerhans' cells: these cells stain positively with S 100 protein and CD1 and are negative for both lysozyme and al antichymotrypsine. After a period of two years for one child and four years for the other, these children are in total remission, one spontaneously, the other after chemotherapy. PMID:9339010

  15. Purely cutaneous Langerhans cell histiocytosis presenting as an ulcer on the chin in an elderly man successfully treated with thalidomide

    PubMed Central

    Subramaniyan, Radhakrishnan; Ramachandran, Rajagopal; Rajangam, Gnanasekaran; Donaparthi, Navya

    2015-01-01

    Langerhans cell histiocytosis (LCH) is a rare, clonal proliferative disorder of Langerhans’ cells of unknown etiology. Although the clinical presentation and therapeutic approach to the disease in children have been well established; limited data is available about the disease in adults. Purely cutaneous involvement of LCH in a man older than 70 years has rarely been described. Herein we report the case of a 71-year-old man with cutaneous LCH confined to the perioral region, scalp, and flexures successfully treated with thalidomide. PMID:26753141

  16. Orbit Determination of the Lunar Reconnaissance Orbiter

    NASA Technical Reports Server (NTRS)

    Mazarico, Erwan; Rowlands, D. D.; Neumann, G. A.; Smith, D. E.; Torrence, M. H.; Lemoine, F. G.; Zuber, M. T.

    2011-01-01

    We present the results on precision orbit determination from the radio science investigation of the Lunar Reconnaissance Orbiter (LRO) spacecraft. We describe the data, modeling and methods used to achieve position knowledge several times better than the required 50-100m (in total position), over the period from 13 July 2009 to 31 January 2011. In addition to the near-continuous radiometric tracking data, we include altimetric data from the Lunar Orbiter Laser Altimeter (LOLA) in the form of crossover measurements, and show that they strongly improve the accuracy of the orbit reconstruction (total position overlap differences decrease from approx.70m to approx.23 m). To refine the spacecraft trajectory further, we develop a lunar gravity field by combining the newly acquired LRO data with the historical data. The reprocessing of the spacecraft trajectory with that model shows significantly increased accuracy (approx.20m with only the radiometric data, and approx.14m with the addition of the altimetric crossovers). LOLA topographic maps and calibration data from the Lunar Reconnaissance Orbiter Camera were used to supplement the results of the overlap analysis and demonstrate the trajectory accuracy.

  17. [Therapy-resistant aggressive cystic lesion of the mandible].

    PubMed

    Ziegler, C M; Bergstrand, S; Lund, J-A; Viset, T

    2011-10-01

    Several extensive surgical interventions of a cystic lesion in the left mandible were followed by recurrences. The lesion extended from the primary mandibular region into the area of pterygopalatine fossa finally infiltrating the orbital region and the skull base. Histological results could never demonstrate a malignancy with certainty. Due to the patient's poor general condition, the refusal for further surgical inventions and due to the malignoma-like growth pattern radiation treatment was performed. However, this had no effect on tumor progression. PMID:21845481

  18. Orbital inflammation: Corticosteroids first.

    PubMed

    Dagi Glass, Lora R; Freitag, Suzanne K

    2016-01-01

    Orbital inflammation is common, and may affect all ages and both genders. By combining a thorough history and physical examination, targeted ancillary laboratory testing and imaging, a presumptive diagnosis can often be made. Nearly all orbital inflammatory pathology can be empirically treated with corticosteroids, thus obviating the need for histopathologic diagnosis prior to initiation of therapy. In addition, corticosteroids may be effective in treating concurrent systemic disease. Unless orbital inflammation responds atypically or incompletely, patients can be spared biopsy.

  19. Magnetospheric Multiscale (MMS) Orbit

    NASA Video Gallery

    This animation shows the orbits of Magnetospheric Multiscale (MMS) mission, a Solar-Terrestrial Probe mission comprising of four identically instrumented spacecraft that will study the Earth's magn...

  20. Orbital Debris: A Chronology

    NASA Technical Reports Server (NTRS)

    Portree, Davis S. F. (Editor); Loftus, Joseph P., Jr. (Editor)

    1999-01-01

    This chronology covers the 37-year history of orbital debris concerns. It tracks orbital debris hazard creation, research, observation, experimentation, management, mitigation, protection, and policy. Included are debris-producing, events; U.N. orbital debris treaties, Space Shuttle and space station orbital debris issues; ASAT tests; milestones in theory and modeling; uncontrolled reentries; detection system development; shielding development; geosynchronous debris issues, including reboost policies: returned surfaces studies, seminar papers reports, conferences, and studies; the increasing effect of space activities on astronomy; and growing international awareness of the near-Earth environment.

  1. Introducing Earth's Orbital Eccentricity

    NASA Astrophysics Data System (ADS)

    Oostra, Benjamin

    2015-12-01

    Most students know that planetary orbits, including Earth's, are elliptical; that is Kepler's first law, and it is found in many science textbooks. But quite a few are mistaken about the details, thinking that the orbit is very eccentric, or that this effect is somehow responsible for the seasons. In fact, the Earth's orbital eccentricity is small, and its only effect on the seasons is their unequal durations. Here I show a pleasant way to guide students to the actual value of Earth's orbital eccentricity, starting from the durations of the four seasons. The date of perihelion is also found.

  2. Family of Orbiters

    NASA Technical Reports Server (NTRS)

    2008-01-01

    This image shows the paths of three spacecraft currently in orbit around Mars, as well as the path by which NASA's Phoenix Mars Lander will approach and land on the planet. The t-shaped crosses show where the orbiters will be when Phoenix enters the atmosphere, while the x-shaped crosses show their location at landing time.

    All three orbiters, NASA's Mars Reconnaissance Orbiter, NASA's Mars Odyssey and the European Space Agency's Mars Express, will be monitoring Phoenix during the final steps of its journey to the Red Planet.

    Phoenix will land just south of Mars's north polar ice cap.

  3. Cutaneous histiocytosis with Langerhans cell features induced by scabies: a case report.

    PubMed

    Talanin, N Y; Smith, S S; Shelley, E D; Moores, W B

    1994-12-01

    An infant with biopsy-proven scabies developed nodular lesions. Histopathology revealed atypical histiocytes with Langerhans cell features. Within six months after treatment all skin lesions gradually disappeared. We suggest that the nodules in scabies can be due to Langerhans cell proliferation.

  4. Cystic Lesions of the Mediastinum.

    PubMed

    Vargas, Daniel; Suby-Long, Thomas; Restrepo, Carlos S

    2016-06-01

    Cystic lesions are commonly seen in the mediastinum, and they may arise from virtually any organ. The vast majority of these lesions are benign and result in no symptoms. When large, cysts may produce symptoms related to compression of adjacent structures. The most common mediastinal cysts are pericardial and foregut duplication cysts. Both computed tomography and magnetic resonance are routinely used to evaluate these lesions. Although computed tomography offers superior spatial resolution, magnetic resonance is useful in differentiating cysts that contain proteinaceous material from solid lesions. Occasionally, cysts arise from solid lesions, such as thymoma or teratoma. Although cysts are alike in appearance, location helps narrowing the differential diagnoses.

  5. Orbital hemangiopericytoma and solitary fibrous tumor: a morphologic continuum.

    PubMed

    Goldsmith, J D; van de Rijn, M; Syed, N

    2001-10-01

    Solitary fibrous tumors (SFT) and hemangiopericytomas (HPC) are soft tissue tumors with known histologic and immunohistochemical overlap. A series of these tumors located in the orbit were analyzed in order to determine whether they could be re-classified based on currently recognized histologic criteria. Ten orbital spindle cell lesions, all of which were positive for CD34 antigen, were examined. Diagnostic criteria for SFT included a cytologically bland spindle cell lesion with variable cellularity and focal dense collagenization with diffuse, strong CD34 reactivity, while the criteria for HPC required a more monotonous cellular proliferation without significant variability in cellularity, a "staghorn" vascular pattern, minimal collagenization, and focal or absent CD34 staining. Tumors with typical histologic and immunohistochemical features of HPC or SFT were diagnosed as HPC and SFT, respectively. Those tumors with histologic or antigenic profiles not classic for HPC or SFT were defined as 'indeterminate.' Three lesions were classified as SFT and 1 tumor was diagnosed as HPC through use of the above-cited histologic criteria. All lesions showed positive staining of tumor cells with CD34 antigen in varying amounts and were negative for cytokeratin AE1-3, epithelial membrane antigen, CD68, and Factor XIIIa. One solitary fibrous tumor focally stained for S-100 protein and 1 hemangiopericytoma was focally positive for HHF-35. Of the 10 analyzed tumors, 6 were classified as 'indeterminate.' Furthermore, 1 lesion whose primary histology was that of an SFT recurred 9 years later with an appearance consistent with an 'indeterminate' lesion. Our results call into question the present histologic separation of HPC and SFT in the orbit. As in other sites, including deep soft tissue, these data suggest that SFT and HPC are 2 lesions whose morphologic features are best interpreted to exist along a continuum, rather than 2 lesions with distinctly defined histopathology.

  6. Lesion mimic mutants

    PubMed Central

    Moeder, Wolfgang

    2008-01-01

    Over the last decade a substantial number of lesion mimic mutants (LMM) have been isolated and a growing number of the genes have been cloned. It is now becoming clear that these mutants are valuable tools to dissect various aspects of programmed cell death (PCD) and pathogen resistance pathways in plants. Together with other forward genetics approaches LMMs shed light on the PCD machinery in plant cells and revealed important roles for sphingolipids, Ca2+ and chloroplast-derived porphyrin-metabolites during cell death development. PMID:19513227

  7. Pulmonary Langerhans Histiocytosis: an uncommon cause of interstitial pneumonia in a patient with Sjögren syndrome.

    PubMed

    González García, Andrés; Callejas Rubio, José Luis; Ríos Fernández, Raquel; Ortego Centeno, Norberto

    2016-03-01

    Sjögren syndrome is a chronic, systemic, and autoimmune disorder that targets exocrine glands by remarkable B cell hyperactivity. Eventually, it is associated with extra-glandular clinical manifestations that affect essentially any organ system, including pulmonary involvement. Interstitial lung disease is one of the most serious pulmonary complications, and the early diagnosis is essential to initiate a prompt therapy. On the other hand, Sjögren syndrome could present concomitantly with several rheumatologic diseases such as systemic lupus erythematosus or rheumatoid arthritis. Pulmonary Langerhans Histiocytosis is a rare clonal proliferative disease characterized by pulmonary involvement by cells phenotypically similar to Langerhans cells. We describe the case of a nonsmoker 62-year-old woman with Sjögren syndrome who presented concomitantly a Pulmonary Langerhans Histiocytosis mimicking a pulmonary complication of its Sjögren. Fortunately, she had a well response to corticosteroids and azathioprine regimen. The aim of the paper is to emphasize the importance of the good differential diagnosis related to the pulmonary involvement. To the best of our knowledge, this is the first description of these two entities in the literature.

  8. Insights into the pathogenesis of Langerhans cell histiocytosis: the development of targeted therapies

    PubMed Central

    Hutter, Caroline; Minkov, Milen

    2016-01-01

    More than a century after its first description, Langerhans cell histiocytosis (LCH) still remains an intriguing disease. Considerable progress in understanding its biology has been achieved recently. Description of the V600E BRAF mutation in samples of LCH tissue in 2010 was followed by description of additional mutations, all leading to constitutive ERK activation. Current experimental data suggest that LCH is a myeloid neoplasia with inflammatory properties, yet the exact pathophysiology remains poorly understood. Disease management paradigms have changed over time, closely reflecting the evolving view of the nature of the disease. The international Histiocyte Society have conducted three prospective clinical studies on multisystem LCH since the early 1990s. The standard frontline therapy for patients with multisystem LCH based on the cumulative knowledge of those trials consists of 6–12 weeks of initial therapy (daily oral steroids and weekly vinblastine injections), followed by pulses of prednisolone/vinblastine every 3 weeks, for a total treatment duration of 12 months. A currently ongoing study (LCH-IV) with a complex design (five interventional and two observational strata) targets further reduction of mortality and morbidity by tailoring treatment intensity depending on expected risk, as well as by exploring treatment regimens for special locations. Current knowledge on LCH pathobiology opens opportunities for improvement in the patient outcome. The activating BRAF and MAP2K1 mutations collectively accounting for about 75% of the LCH population as well as the resulting constitutive activation of downstream ERK offer an opportunity for targeted treatment. Related issues (eg, finding most effective and less toxic drugs or combinations, appropriate dosage, and optimal treatment duration) must be addressed in controlled prospective trials. Additional mechanisms, such as the interactions of the mutated dendritic cell clone with other inflammatory cells and

  9. [Possibilities and limits of paraffin-embedded cell markers in diagnosis of primary cutaneous histiocytosis].

    PubMed

    Fartasch, M; Goerdt, S; Hornstein, O P

    1995-03-01

    To date, the rare primary histiocytoses of the skin are diagnosed definitively on the basis of the clinical symptoms, H&E-stained sections, and demonstration of CD1 positivity in frozen sections and of Birbeck granules on electron microscopy. The improvement and analysis of antibodies with the ability to react in paraffin tissue allow retrospective evaluation and classification of these disorders. The antibodies for S-100-protein, peanut agglutinin (PNA) and PCNA (proliferating cell nuclear antigen) have been advocated for differentiation of the specific cells of Langerhans cell histiocytosis (LCH) from other histiocytic cell systems. To date the non-Langerhans cell histiocytoses (non-LCH) have no common ultrastructural and immunohistochemical characteristics. The infiltrate is made up of multiple cell populations, which are of significance for the cellular pathobiology (subtypes of monocytes/macrophages and dendritic cells). The number and distribution of the different monocyte/macrophages and dendritic cells and their ability to react with immunohistochemical markers in paraffin tissue can be completely different in different clinical entities. The antibodies against factor XIIIa (shown on xanthoma disseminatum) and the monoclonal antibody Ki-M1P (shown on juvenile xanthogranuloma) seem to be valuable in discrimination between LCH and non-LCH. Both markers show a positive staining pattern with the characteristic large macrophages. In juvenile xanthogranuloma, the foam cells and giant cells express Ki-M1P, KP1 and anti-cathepsin B. Other monocyte/macrophage markers with the ability to react in paraffin tissue, such as Mac387, lysozyme, alpha 1-antitrypsin and Leu-M1 (Anti-CD 15), in contrast, did not show a typical staining pattern with the characteristic large macrophages dominating the histological picture.

  10. Detection of Epstein-Barr Virus DNA in Langerhans Cell Histiocytosis

    PubMed Central

    Khoddami, Maliheh; Nadji, Seyed Alireza; Dehghanian, Paria; Vahdatinia, Mahsa; Shamshiri, Ahmad Reza

    2015-01-01

    Background: Langerhans cell histiocytosis (LCH) is a rare histiocytic proliferation of unknown etiology. It is characterized by granuloma-like proliferation of Langerhans-type dendritic cells and mainly affects young children. Although multiple investigators have suggested the possible role of viruses, such as Epstein-Barr virus (EBV), human herpesvirus-6 (HHV-6), Herpes simplex virus (HSV) types 1 and 2, and Cytomegalovirus (CMV) in the pathogenesis of LCH, it remains, however, debated. Objectives: The EBV infection is reported to be associated with LCH. Nevertheless, no report could be found about involved Iranian children in English medical literature. In this study, we investigated the presence of EBV in Iranian children with LCH. Patients and Methods: In this retrospective study, in which we investigated the prevalence of presence of EBV DNA in LCH, using paraffin-embedded tissue samples of 30 patients with LCH and 30 age and tissue-matched controls, who were operated for reasons other than infectious diseases (between the years 2002 and 2012), by real-time polymerase chain reaction (RT-PCR) method, in the department of pediatric pathology. No ethical issues arose in the study, because only the pathology reports were reviewed, retrospectively, and the patients were anonymous. Results: There was a significant difference in prevalence of EBV presence between patients and controls. The EBV was found by RT-PCR in 19 (63.33%) out of 30 patients and only in eight (26.7%) of 30 control samples. The P = 0.004, was calculated using chi-square test (OR: 4.75; 95% CI: 1.58 ‒ 14.25). Conclusions: Our study is the first investigation performed on patients with LCH and its possible association with EBV in Iran. Considering the P = 0.004, which is statistically significant, the findings do support the hypothesis of a possible role for EBV in the pathogenesis of LCH. These results are in accordance with several previous investigations, with positive findings. PMID:26870310

  11. Pulmonary Langerhans cell histiocytosis: analysis of 14 patients and literature review

    PubMed Central

    Li, Cheng-Wei; Li, Man-Hui; Li, Jiang-Xiong; Tao, Ru-Jia; Xu, Jin-Fu

    2016-01-01

    Background Pulmonary Langerhans cell histiocytosis (PLCH) is an orphan disease in respiratory medicine, which most affects adult smokers. The purpose of this article was to discuss the clinical features, especially the radiologic features of PLCH patients during their hospitalization through a retrospective analysis on clinical data. Furthermore, the current literature was also reviewed. Methods Between December 2008 and June 2012, 14 patients with PLCH were assessed at Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai, China. Among these patients, seven patients were diagnosed through tissue biopsy from the lung and one patient from enlarged cervical lymph nodes; the rest of six patients were diagnosed based on the clinical-radiological data. The data consisting of demographics, clinical presentation, smoking habits, pulmonary function tests (PFTs) and radiographic image from the medical records was analyzed retrospectively. Results The average age of patients (11 males and 3 females) was 42.79 (±13.71) years old. All male patients and one female patient had a long smoking history. The common manifestations were cough and exertional dyspnea. Spontaneous pneumothorax was found in three patients. Varieties of pulmonary shadows such as nodular, cystic, patch-like and cord-like were revealed by chest computed tomography (CT) examination. Large Langerhans cells (LCs) were discovered in biopsy tissue by immunohistochemical stains. Conclusions PLCH is still an orphan disease and maybe related to smoking. Clinical symptoms such as cough and exertional dyspnea are non-specific. We shall pay attention to recurrent pneumothorax as clinically it is associated with PLCH. The characteristic radiological manifestation is cystic or nodular shadow in the lungs, which plays crucial roles in diagnosing PLCH. PMID:27293848

  12. Computed tomography of the orbit with special emphasis on coronal sections: Part II. Pathological anatomy.

    PubMed

    Tadmor, R; New, P F

    1978-01-01

    Visualization of orbital soft tissue structures by computed tomography in direct coronal and axial studies is extremely useful in diagnosis. Direct enlargement viewing of scans has disclosed minute anatomical details. This study reviews some of our experiences in the investigation of a variety of lesions within the orbit and attempts, in particular, to illustrate the value of direct coronal studies.

  13. Giant osteoma of the ethmoid sinus with orbital extension: craniofacial approach and orbital reconstruction.

    PubMed

    Sanchez Burgos, R; González Martín-Moro, J; Arias Gallo, J; Carceller Benito, F; Burgueño García, M

    2013-12-01

    Osteomas are the most common fibro-osseous lesions in the paranasal sinus. They are benign tumours characterized by slow growth and are often asymptomatic. Treatment is indicated in sphenoid osteomas that threaten the optic canal or orbital apex and in symptomatic cases. The choice of surgical management depends on the location, size and experience of the surgeon. An open approach allows tumour removal with direct visual control and remains the best option in large tumours, but the continued progression in endoscopic approaches is responsible for new indications in closed techniques. Immediate reconstruction allows aesthetic and functional restoration of neighbouring structures, which should one of the goals in the treatment of this benign entity. We report a case of a giant ethmoid osteoma with orbital invasion treated by a combined open craniofacial approach with reconstruction of the anterior cranial base and orbital walls. The literature is reviewed and aetiopathogenic theories, diagnostic procedures and surgical approaches are discussed.

  14. Analyzing Shuttle Orbiter Trajectories

    NASA Technical Reports Server (NTRS)

    Lear, W. M.

    1986-01-01

    LRBET4 program best-estimated-of-trajectory (BET) calculation for post-flight trajectory analysis of Shuttle orbiter. Produces estimated measurements for comparing predicted and actual trajectory of Earth-orbiting spacecraft. Kalman filter and smoothing filter applied to input data to estimate state vector, reduce noise, and produce BET. LRBET4 written in FORTRAN IV for batch execution.

  15. Titan Orbiter Aerorover Mission

    NASA Technical Reports Server (NTRS)

    Sittler Jr., E. C.; Acuna, M.; Burchell, M. J.; Coates, A.; Farrell, W.; Flasar, M.; Goldstein, B. E.; Gorevan, S.; Hartle, R. E.; Johnson, W. T. K.

    2001-01-01

    We propose a combined Titan orbiter and Titan Aerorover mission with an emphasis on both in situ and remote sensing measurements of Titan's surface, atmosphere, ionosphere, and magnetospheric interaction. The biological aspect of the Titan environment will be emphasized by the mission (i.e., search for organic materials which may include simple organics to 'amono' analogues of amino acids and possibly more complex, lightening detection and infrared, ultraviolet, and charged particle interactions with Titan's surface and atmosphere). An international mission is assumed to control costs. NASA will provide the orbiter, launch vehicle, DSN coverage and operations, while international partners will provide the Aerorover and up to 30% of the cost for the scientific instruments through collaborative efforts. To further reduce costs we propose a single PI for orbiter science instruments and a single PI for Aerorover science instruments. This approach will provide single command/data and power interface between spacecraft and orbiter instruments that will have redundant central DPU and power converter for their instruments. A similar approach could be used for the Aerorover. The mission profile will be constructed to minimize conflicts between Aerorover science, orbiter radar science, orbiter radio science, orbiter imaging science, and orbiter fields and particles (FP) science. Additional information is contained in the original extended abstract.

  16. Orbital Shape Representations.

    ERIC Educational Resources Information Center

    Kikuchi, Osamu; Suzuki, Keizo

    1985-01-01

    Discusses the use of orbital shapes for instructional purposes, emphasizing that differences between polar, contour, and three-dimensional plots must be made clear to students or misconceptions will occur. Also presents three-dimensional contour surfaces for the seven 4f atomic orbitals of hydrogen and discusses their computer generation. (JN)

  17. Ossifying cystic odontogenic and Schneiderian choristoma of the orbit.

    PubMed

    Mudhar, Hardeep Singh; Nurrudin, Murtuza

    2014-02-01

    A 12-year-old girl presented with a left infraorbital lesion, causing upward globe displacement. Imaging confirmed a mass between the globe and the orbital floor. The lesion was removed via a sub-ciliary approach and histology revealed a a mature tooth along with a periodontal ligament, oral-type mucinous glands and bone. Six years later a mass recurred at exactly the same site and on this occasion, revealed cysts containing mucin and lined by Schneiderian type epithelium. A rather complex combination of a tooth, lamellar bone, mucinous oral type glands and Schneiderian cystic epithelium is highly unusual and we have called the lesion "ossifying cystic odontogenic and Schneiderian choristoma of the orbit."

  18. Mars Climate Orbiter

    NASA Technical Reports Server (NTRS)

    1998-01-01

    The purpose of this mission is to study the climate history and the water distribution of Mars. Beautiful panoramic views of the shuttle on the launch pad, engine ignition, Rocket launch, and the separation and burnout of the Solid Rocket Boosters are shown. The footage also includes an animation of the mission. Detailed views of the path that the Orbiter traversed were shown. Once the Orbiter lands on the surface of Mars, it will dig a six to eight inch hole and collect samples from the planets' surface. The animation also included the prospective return of the Orbiter to Earth over the desert of Utah. The remote sensor on the Orbiter helps in finding the exact location of the Orbiter so that scientists may collect the sample and analyze it.

  19. Remote Controlled Orbiter Capability

    NASA Technical Reports Server (NTRS)

    Garske, Michael; delaTorre, Rafael

    2007-01-01

    The Remote Control Orbiter (RCO) capability allows a Space Shuttle Orbiter to perform an unmanned re-entry and landing. This low-cost capability employs existing and newly added functions to perform key activities typically performed by flight crews and controllers during manned re-entries. During an RCO landing attempt, these functions are triggered by automation resident in the on-board computers or uplinked commands from flight controllers on the ground. In order to properly route certain commands to the appropriate hardware, an In-Flight Maintenance (IFM) cable was developed. Currently, the RCO capability is reserved for the scenario where a safe return of the crew from orbit may not be possible. The flight crew would remain in orbit and await a rescue mission. After the crew is rescued, the RCO capability would be used on the unmanned Orbiter in an attempt to salvage this national asset.

  20. CT and orbital ultrasound findings in a case of Castleman disease.

    PubMed

    Brubaker, Jacob W; Harrie, Roger P; Patel, Bhupendra C; Davis, Don K; Mamalis, Nick

    2011-01-01

    A 53-year-old man with a 2-month history of left periorbital swelling was found to have a large solid intraconal mass on CT scan. Orbital ultrasound showed that the lesion had a cavernous pattern of internal reflectivity. Histopathology revealed hyaline-vascular type Castleman disease (CD). This article represents the first reported orbital ultrasound findings in CD. The findings of CT scan and ultrasound may be useful in the preoperative evaluation of orbital hyaline-vascular type CD.

  1. Orbital Causes of Incomitant Strabismus

    PubMed Central

    Lueder, Gregg T.

    2015-01-01

    Strabismus may result from abnormal innervation, structure, or function of the extraocular muscles. Abnormalities of the orbital bones or masses within the orbit may also cause strabismus due to indirect effects on the extraocular muscles. This paper reviews some disorders of the orbit that are associated with strabismus, including craniofacial malformations, orbital masses, trauma, and anomalous orbital structures. PMID:26180465

  2. Imaging findings of vascular lesions in the head and neck.

    PubMed

    Güneyli, Serkan; Ceylan, Naim; Bayraktaroğlu, Selen; Acar, Türker; Savaş, Recep

    2014-01-01

    Vascular lesions of the head and neck include vascular neoplasms, vascular malformations, and hypervascular lesions, derived from nonvascular soft-tissue elements. We retrospectively evaluated magnetic resonance imaging and computed tomography images of vascular lesions located in the head and neck. Twelve patients (seven males, five females) aged 1-68 years (mean age, 35.25 years) were included in this study. Most of the vascular lesions in our study were histologically diagnosed. The lesions were as follows: a hemangioma located in the parotid space (n=1); a hemangioendothelioma located in the parotid space (n=1); a hemangiopericytoma located in the larynx (n=1); a juvenile angiofibroma located in the nasopharynx (n=1); a glomus tumor located in the carotid bifurcation (n=1); venous malformations located in the parapharyngeal space, the pterygoid area, the orbital space, and the larynx (n=4); lymphatic malformations located in the parotid space and the supraclavicular area (n=2); and an arteriovenous malformation located in the infratemporal fossa (n=1). We present rare vascular lesions of the head and neck, which have typical radiological findings.

  3. Imaging findings of vascular lesions in the head and neck

    PubMed Central

    Güneyli, Serkan; Ceylan, Naim; Bayraktaroğlu, Selen; Acar, Türker; Savaş, Recep

    2014-01-01

    Vascular lesions of the head and neck include vascular neoplasms, vascular malformations, and hypervascular lesions, derived from nonvascular soft-tissue elements. We retrospectively evaluated magnetic resonance imaging and computed tomography images of vascular lesions located in the head and neck. Twelve patients (seven males, five females) aged 1–68 years (mean age, 35.25 years) were included in this study. Most of the vascular lesions in our study were histologically diagnosed. The lesions were as follows: a hemangioma located in the parotid space (n=1); a hemangioendothelioma located in the parotid space (n=1); a hemangiopericytoma located in the larynx (n=1); a juvenile angiofibroma located in the nasopharynx (n=1); a glomus tumor located in the carotid bifurcation (n=1); venous malformations located in the parapharyngeal space, the pterygoid area, the orbital space, and the larynx (n=4); lymphatic malformations located in the parotid space and the supraclavicular area (n=2); and an arteriovenous malformation located in the infratemporal fossa (n=1). We present rare vascular lesions of the head and neck, which have typical radiological findings. PMID:25010372

  4. Ethmoid osteoma as a culprit of orbital emphysema: a case report.

    PubMed

    Zhuang, Ai; Li, Yinwei; Lin, Ming; Shi, Wodong; Fan, Xianqun

    2015-05-01

    Orbital emphysema is generally recognized as a complication of orbital fractures involving any paranasal sinuses. The recognition about its etiology has extended beyond sole trauma, but few articles mentioned tumors to be a possible cause.In this case report, we present a patient with orbital emphysema associated with ethmoid osteoma without orbital cellulitis or trauma history. The patient developed sudden proptosis, eyelid swelling, and movement limitation of the left eye, peripheral diplopia, and left periorbital crepitus after a vigorous nose blowing.Complete surgical resection of ethmoid osteoma followed by repair of the orbital medial wall was performed with assistance of combined endoscopy and navigational techniques. Twelve-month follow-up showed no residual lesion or recurrence; the orbital medial wall was accurately repaired with good visual function and facial symmetry.Tumors should be considered for differential diagnosis of orbital emphysema, and combined endoscopy and navigational techniques may improve safety, accuracy, and effectiveness of orbital surgeries.

  5. Respiratory epithelial cysts of the orbit.

    PubMed

    Goh, Rachel L Z; Hardy, Thomas G; Williams, Richard A; McNab, Alan A

    2016-10-01

    To describe post-traumatic and congenital respiratory epithelial cysts in the orbit, which are rare lesions with only 5 and 13 published cases, respectively. We reviewed all cases of respiratory epithelial cysts diagnosed at three institutions (two tertiary referral hospitals, one private clinic) between 1995 and 2015. We describe 10 cases of post-traumatic respiratory epithelial cyst (age range 23 - 82), presenting a mean of 17.4 years after their original trauma; and 3 congenital cases (age range 17-34). All but one case underwent surgical excision of the cyst and its lining, along with any surgical implant within the cyst. Two were recurrent after incomplete excision. Three presented with acute infection within the cyst. Respiratory epithelial orbital cysts are probably commoner than the paucity of published reports would suggest. Post-traumatic cysts often present many years after trauma, and may become secondarily infected. Complete surgical removal is recommended to prevent future recurrence. PMID:27468088

  6. Respiratory epithelial cysts of the orbit.

    PubMed

    Goh, Rachel L Z; Hardy, Thomas G; Williams, Richard A; McNab, Alan A

    2016-10-01

    To describe post-traumatic and congenital respiratory epithelial cysts in the orbit, which are rare lesions with only 5 and 13 published cases, respectively. We reviewed all cases of respiratory epithelial cysts diagnosed at three institutions (two tertiary referral hospitals, one private clinic) between 1995 and 2015. We describe 10 cases of post-traumatic respiratory epithelial cyst (age range 23 - 82), presenting a mean of 17.4 years after their original trauma; and 3 congenital cases (age range 17-34). All but one case underwent surgical excision of the cyst and its lining, along with any surgical implant within the cyst. Two were recurrent after incomplete excision. Three presented with acute infection within the cyst. Respiratory epithelial orbital cysts are probably commoner than the paucity of published reports would suggest. Post-traumatic cysts often present many years after trauma, and may become secondarily infected. Complete surgical removal is recommended to prevent future recurrence.

  7. Thalamic Lesions: A Radiological Review

    PubMed Central

    Renard, Dimitri; Campello, Chantal; Bouly, Stephane; Le Floch, Anne; Thouvenot, Eric; Waconge, Anne; Taieb, Guillaume

    2014-01-01

    Background. Thalamic lesions are seen in a multitude of disorders including vascular diseases, metabolic disorders, inflammatory diseases, trauma, tumours, and infections. In some diseases, thalamic involvement is typical and sometimes isolated, while in other diseases thalamic lesions are observed only occasionally (often in the presence of other typical extrathalamic lesions). Summary. In this review, we will mainly discuss the MRI characteristics of thalamic lesions. Identification of the origin of the thalamic lesion depends on the exact localisation inside the thalamus, the presence of extrathalamic lesions, the signal changes on different MRI sequences, the evolution of the radiological abnormalities over time, the history and clinical state of the patient, and other radiological and nonradiological examinations. PMID:25100900

  8. Visualization of atom's orbits.

    PubMed

    Kim, Byungwhan

    2014-02-01

    High-resolution imaging techniques have been used to obtain views of internal shapes of single atoms or columns of atoms. This review article focuses on the visualization of internal atomic structures such as the configurations of electron orbits confined to atoms. This is accomplished by applying visualization techniques to the reported images of atoms or molecules as well as static and dynamic ions in a plasma. It was found that the photon and electron energies provide macroscopic and microscopic views of the orbit structures of atoms, respectively. The laser-imaged atoms showed a rugged orbit structure, containing alternating dark and bright orbits believed to be the pathways for an externally supplied laser energy and internally excited electron energy, respectively. By contrast, the atoms taken by the electron microscopy provided a structure of fine electron orbits, systematically formed in increasing order of grayscale representing the energy state of an orbit. This structure was identical to those of the plasma ions. The visualized electronic structures played a critical role in clarifying vague postulates made in the Bohr model. Main features proposed in the atomic model are the dynamic orbits absorbing an externally supplied electromagnetic energy, electron emission from them while accompanying light radiation, and frequency of electron waves not light. The light-accompanying electrons and ionic speckles induced by laser light signify that light is composed of electrons and ions.

  9. Harmonically excited orbital variations

    SciTech Connect

    Morgan, T.

    1985-08-06

    Rephrasing the equations of motion for orbital maneuvers in terms of Lagrangian generalized coordinates instead of Newtonian rectangular cartesian coordinates can make certain harmonic terms in the orbital angular momentum vector more readily apparent. In this formulation the equations of motion adopt the form of a damped harmonic oscillator when torques are applied to the orbit in a variationally prescribed manner. The frequencies of the oscillator equation are in some ways unexpected but can nonetheless be exploited through resonant forcing functions to achieve large secular variations in the orbital elements. Two cases are discussed using a circular orbit as the control case: (1) large changes in orbital inclination achieved by harmonic excitation rather than one impulsive velocity change, and (2) periodic and secular changes to the longitude of the ascending node using both stable and unstable excitation strategies. The implications of these equations are also discussed for both artificial satellites and natural satellites. For the former, two utilitarian orbits are suggested, each exploiting a form of harmonic excitation. 5 refs.

  10. Orbit Stabilization of Nanosat

    SciTech Connect

    JOHNSON,DAVID J.

    1999-12-01

    An algorithm is developed to control a pulsed {Delta}V thruster on a small satellite to allow it to fly in formation with a host satellite undergoing time dependent atmospheric drag deceleration. The algorithm uses four short thrusts per orbit to correct for differences in the average radii of the satellites due to differences in drag and one thrust to symmetrize the orbits. The radial difference between the orbits is the only input to the algorithm. The algorithm automatically stabilizes the orbits after ejection and includes provisions to allow azimuthal positional changes by modifying the drag compensation pulses. The algorithm gives radial and azimuthal deadbands of 50 cm and 3 m for a radial measurement accuracy of {+-} 5 cm and {+-} 60% period variation in the drag coefficient of the host. Approaches to further reduce the deadbands are described. The methodology of establishing a stable orbit after ejection is illustrated in an appendix. The results show the optimum ejection angle to minimize stabilization thrust is upward at 86{sup o} from the orbital velocity. At this angle the stabilization velocity that must be supplied by the thruster is half the ejection velocity. An ejection velocity of 0.02 m/sat 86{sup o} gives an azimuthal separation after ejection and orbit stabilization of 187 m. A description of liquid based gas thrusters suitable for the satellite control is included in an appendix.

  11. Orbital exenteration in immunodeficiency virus-infected patients

    PubMed Central

    Giles, Kagmeni; Bilong, Yannick; Arlette, Nomo; Chantal, Nanfack; Lucienne, Bella Assumpta

    2016-01-01

    Background Orbital exenteration (OE) is a disfiguring procedure most commonly performed for locally advanced and potentially life-threatening periorbital malignancies. Methods We retrospectively reviewed records of 11 consecutive HIV patients who underwent OE for invasive orbital malignancy at our institution from January 2005 to December 2015. Patient demographic and clinic data and histopathology of the tumor were analyzed. Results There were eight (72.72%) female and three (27.28%) male participants ranging in age from 31 to 52 years with an mean of 39.4 years. Nine patients had been known to be HIV-positive for at least 2 years, and HIV-positive status was revealed at presentation for two patients. The mean CD4 cell count was 154.4 cells/mm3. Histopathological examination showed invasive orbital squamous cell carcinomas in nine patients (81.81%), achromic orbital melanoma in one patient (9.09%), and adenoid cystic carcinoma in one patient (9.09%). None of the patients underwent primary orbital reconstruction. The mean follow-up time was 3.4 months. Only one patient who underwent adjuvant radiotherapy was seen after 12 months. Conclusion Oculo-orbital malignancies are very aggressive in HIV-positive individuals, especially in untreated patients. Routine screening for suspected ocular surface lesions and early surgical removal of all these lesions could help to avoid the need to perform the radical and disfiguring OE procedure. PMID:27799732

  12. Pigmented Lesion of Buccal Mucosa

    PubMed Central

    Bajpai, Manas; Kumar, Malay; Kumar, Manish; Agarwal, Deshant

    2014-01-01

    Pigmented lesions are commonly found in the mouth. Such lesions represent a variety of clinical entities, ranging from physiologic changes to manifestation of systemic illness and malignant neoplasm. Diagnosis of such lesions requires a proper case history, extraoral and intraoral examination, and, in some cases, biopsy, aspiration cytology, and laboratory investigations. Here we present a case of purple lesion on the buccal mucosa of a 34-year-old male patient which was provisionally diagnosed as mucocele but on the basis of histopathological picture it was finally diagnosed as angiofibroma, and we also discuss the clinical and histopathological differential diagnosis. PMID:25161669

  13. Stress-induced cervical lesions.

    PubMed

    Braem, M; Lambrechts, P; Vanherle, G

    1992-05-01

    The increasing occurrence of dental lesions at the cervical surfaces requires more knowledge of the causes of the process. Acidic and abrasive mechanisms have clearly been documented as causes but the stress theory by Lee and Eakle is still controversial. This report describes several incidences of possible stress-induced lesions according to the characteristics described by Lee and Eakle. The occurrences of subgingival lesions lend credence to the stress-induction theory by exclusion of other superimposing etiologic factors. With the current concepts, a perceptive approach to the treatment of cervical lesions can be executed. PMID:1527763

  14. Orbit Determination Issues for Libration Point Orbits

    NASA Technical Reports Server (NTRS)

    Beckman, Mark; Bauer, Frank (Technical Monitor)

    2002-01-01

    Libration point mission designers require knowledge of orbital accuracy for a variety of analyses including station keeping control strategies, transfer trajectory design, and formation and constellation control. Past publications have detailed orbit determination (OD) results from individual libration point missions. This paper collects both published and unpublished results from four previous libration point missions (ISEE (International Sun-Earth Explorer) -3, SOHO (Solar and Heliospheric Observatory), ACE (Advanced Composition Explorer) and MAP (Microwave Anisotropy Probe)) supported by Goddard Space Flight Center's Guidance, Navigation & Control Center. The results of those missions are presented along with OD issues specific to each mission. All past missions have been limited to ground based tracking through NASA ground sites using standard range and Doppler measurement types. Advanced technology is enabling other OD options including onboard navigation using seaboard attitude sensors and the use of the Very Long Baseline Interferometry (VLBI) measurement Delta Differenced One-Way Range (DDOR). Both options potentially enable missions to reduce coherent dedicated tracking passes while maintaining orbital accuracy. With the increased projected loading of the DSN (Deep Space Network), missions must find alternatives to the standard OD scenario.

  15. Report on orbital debris

    NASA Technical Reports Server (NTRS)

    1989-01-01

    The success of space endeavors depends upon a space environment sufficiently free of debris to enable the safe and dependable operation of spacecraft. An environment overly cluttered with debris would threaten the ability to utilize space for a wide variety of scientific, technological, military, and commercial purposes. Man made space debris (orbital debris) differs from natural meteoroids because it remains in earth orbit during its lifetime and is not transient through the space around the Earth. The orbital debris environment is considered. The space environment is described along with sources of orbital debris. The current national space policy is examined, along with ways to minimize debris generation and ways to survive the debris environment. International efforts, legal issues and commercial regulations are also examined.

  16. Orbiter thermal protection system

    NASA Technical Reports Server (NTRS)

    Dotts, R. L.; Curry, D. M.; Tillian, D. J.

    1985-01-01

    The major material and design challenges associated with the orbiter thermal protection system (TPS), the various TPS materials that are used, the different design approaches associated with each of the materials, and the performance during the flight test program are described. The first five flights of the Orbiter Columbia and the initial flight of the Orbiter Challenger provided the data necessary to verify the TPS thermal performance, structural integrity, and reusability. The flight performance characteristics of each TPS material are discussed, based on postflight inspections and postflight interpretation of the flight instrumentation data. Flights to date indicate that the thermal and structural design requirements for the orbiter TPS are met and that the overall performance is outstanding.

  17. Habitability study shuttle orbiter

    NASA Technical Reports Server (NTRS)

    1972-01-01

    Studies of the habitability of the space shuttle orbiter are briefly summarized. Selected illustrations and descriptions are presented for: crew compartment, hygiene facilities, food system and galley, and storage systems.

  18. ARTEMIS Orbits Magnetic Moon

    NASA Video Gallery

    NASA's THEMIS spacecraft have completed their mission and are still working perfectly, so NASA is re-directing the outermost two spacecraft to special orbits around the Moon. Now called ARTEMIS, th...

  19. Altimetry, Orbits and Tides

    NASA Technical Reports Server (NTRS)

    Colombo, O. L.

    1984-01-01

    The nature of the orbit error and its effect on the sea surface heights calculated with satellite altimetry are explained. The elementary concepts of celestial mechanics required to follow a general discussion of the problem are included. Consideration of errors in the orbits of satellites with precisely repeating ground tracks (SEASAT, TOPEX, ERS-1, POSEIDON, amongst past and future altimeter satellites) are detailed. The theoretical conclusions are illustrated with the numerical results of computer simulations. The nature of the errors in this type of orbits is such that this error can be filtered out by using height differences along repeating (overlapping) passes. This makes them particularly valuable for the study and monitoring of changes in the sea surface, such as tides. Elements of tidal theory, showing how these principles can be combined with those pertinent to the orbit error to make direct maps of the tides using altimetry are presented.

  20. MMS Orbit Animation

    NASA Video Gallery

    This animation shows the orbits of Magnetospheric Multiscale (MMS)mission, a Solar Terrestrial Probes mission comprising of fouridentically instrumented spacecraft that will study the Earth’sm...

  1. B-RAF Mutant Alleles Associated with Langerhans Cell Histiocytosis, a Granulomatous Pediatric Disease

    PubMed Central

    Lu, Hui-chun; Mian, Sophie; Trouillet, Celine; Mufti, Ghulam; Emile, Jean-Francois; Fraternali, Franca; Donadieu, Jean; Geissmann, Frederic

    2012-01-01

    Background Langerhans cell histiocytosis (LCH) features inflammatory granuloma characterised by the presence of CD1a+ dendritic cells or ‘LCH cells’. Badalian-Very et al. recently reported the presence of a canonical V600EB-RAF mutation in 57% of paraffin-embedded biopsies from LCH granuloma. Here we confirm their findings and report the identification of two novel B-RAF mutations detected in LCH patients. Methods and Results Mutations of B-RAF were observed in granuloma samples from 11 out of 16 patients using ‘next generation’ pyrosequencing. In 9 cases the mutation identified was V600EB-RAF. In 2 cases novel polymorphisms were identified. A somatic 600DLATB-RAF insertion mimicked the structural and functional consequences of the V600EB-RAF mutant. It destabilized the inactive conformation of the B-RAF kinase and resulted in increased ERK activation in 293 T cells. The 600DLATB-RAF and V600EB-RAF mutations were found enriched in DNA and mRNA from the CD1a+ fraction of granuloma. They were absent from the blood and monocytes of 58 LCH patients, with a lower threshold of sequencing sensitivity of 1%–2% relative mutation abundance. A novel germ line T599AB-RAF mutant allele was detected in one patient, at a relative mutation abundance close to 50% in the LCH granuloma, blood monocytes and lymphocytes. However, T599AB-RAF did not destabilize the inactive conformation of the B-RAF kinase, and did not induce increased ERK phosphorylation or C-RAF transactivation. Conclusions Our data confirmed presence of the V600EB-RAF mutation in LCH granuloma of some patients, and identify two novel B-RAF mutations. They indicate that V600EB-RAF and 600DLATB-RAF mutations are somatic mutants enriched in LCH CD1a+ cells and absent from the patient blood. Further studies are needed to assess the functional consequences of the germ-line T599AB-RAF allele. PMID:22506009

  2. Langerhans cell histiocytosis in adults. Report from the International Registry of the Histiocyte Society.

    PubMed

    Aricò, M; Girschikofsky, M; Généreau, T; Klersy, C; McClain, K; Grois, N; Emile, J-F; Lukina, E; De Juli, E; Danesino, C

    2003-11-01

    Langerhans cell histiocytosis (LCH), characterised by the infiltration of one or more organs by large mononuclear cells, can develop in persons of any age. Although the features of this disease are well described in children, they remain poorly defined in adults. From January 2000 to June 2001, 274 adults from 13 countries, with biopsy-proven adult LCH, were registered with the International Histiocyte Society Registry. Information was collected about clinical presentation, family history, associated conditions, cigarette smoking and treatment, to assist in future management decisions in patients aged 18 years and older. There were slightly more males than females (143:126), and the mean ages at the onset and diagnosis of disease were 33 years (standard deviation (S.D.) 15 years) and 35 years (S.D. 14 years), respectively. 2 patients had consanguineous parents, and 1 had a family history of LCH; 129 reported smoking (47.1%); 17 (6.2%) had been diagnosed with different types of cancer. Single-system LCH, found in 86 patients (31.4%), included isolated pulmonary involvement in 44 cases; 188 patients (68.6%) had multisystem disease; 81 (29.6%) had diabetes insipidus. Initial treatment consisted of vinblastine administered with or without steroids, to 82 patients (29.9%), including 9 who had received it with etoposide, which was the sole agent given to 19 patients. 236 patients were considered evaluable for survival. At a median follow-up of 28 months from diagnosis, 15 patients (6.4%) had died (death rate, 1.5/100 person years, 95% Confidence Interval (95% CI) 0.9-2.4). The probability of survival at 5 years postdiagnosis was 92.3% (95% CI 85.6-95.9) overall, 100% for patients with single-system disease (n=37), 87.8% (95% CI 54.9-97.2) for isolated pulmonary disease (n=34), and 91.7% (95% CI 83.6-95.9) for multisystem disease (n=163). Survival did not differ significantly among patients with multisystem disease, with or without liver or lung involvement) 5-year

  3. A tapestry of orbits

    SciTech Connect

    King-Hele, D.

    1992-01-01

    In this book, the author describes how orbital research developed to yield a rich harvest of knowledge about the earth and its atmosphere. King-Hele relates a personal account of this research based on analysis of satellite orbits between 1957 and 1990 conducted from the Royal Aircraft Establishment in Farnborough England. The early research methods used before the launch of Sputnik in 1957 are discussed.

  4. Orbits of 6 Binaries

    NASA Astrophysics Data System (ADS)

    Olevic, D.; Cvetkovic, Z.

    In this paper the orbits of binaries WDS 10093+2020 = A 2145, WDS 21074-0814 = BU 368 AB and WDS 22288-0001 = STF 2909 AB are recalculated because of significant deviations of more recent observations from the ephemerides. For binaries WDS 22384-0754 = A 2695, WDS 23474-7118 = FIN 375 Aa and WDS 23578+2508 = McA 76 the orbital elements are calculated for the first time.

  5. The Lunar Orbital Prospector

    NASA Technical Reports Server (NTRS)

    Redd, Frank J.; Cantrell, James N.; Mccurdy, Greg

    1992-01-01

    The establishment of lunar bases will not end the need for remote sensing of the lunar surface by orbiting platforms. Human and robotic surface exploration will necessarily be limited to some proximate distance from the support base. Near real-time, high-resolution, global characterization of the lunar surface by orbiting sensing systems will continue to be essential to the understanding of the Moon's geophysical structure and the location of exploitable minerals and deposits of raw materials. The Lunar Orbital Prospector (LOP) is an orbiting sensing platform capable of supporting a variety of modular sensing packages. Serviced by a lunar-based shuttle, the LOP will permit the exchange of instrument packages to meet evolving mission needs. The ability to recover, modify, and rotate sensing packages allows their reuse in varying combinations. Combining this flexibility with robust orbit modification capabilities and near real-time telemetry links provides considerable system responsiveness. Maintenance and modification of the LOP orbit are accomplished through use of an onboard propulsion system that burns lunar-supplied oxygen and aluminum. The relatively low performance of such a system is more than compensated for by the elimination of the need for Earth-supplied propellants. The LOP concept envisions a continuous expansion of capability through the incorporation of new instrument technologies and the addition of platforms.

  6. The Lunar Orbital Prospector

    NASA Astrophysics Data System (ADS)

    Redd, Frank J.; Cantrell, James N.; McCurdy, Greg

    1992-09-01

    The establishment of lunar bases will not end the need for remote sensing of the lunar surface by orbiting platforms. Human and robotic surface exploration will necessarily be limited to some proximate distance from the support base. Near real-time, high-resolution, global characterization of the lunar surface by orbiting sensing systems will continue to be essential to the understanding of the Moon's geophysical structure and the location of exploitable minerals and deposits of raw materials. The Lunar Orbital Prospector (LOP) is an orbiting sensing platform capable of supporting a variety of modular sensing packages. Serviced by a lunar-based shuttle, the LOP will permit the exchange of instrument packages to meet evolving mission needs. The ability to recover, modify, and rotate sensing packages allows their reuse in varying combinations. Combining this flexibility with robust orbit modification capabilities and near real-time telemetry links provides considerable system responsiveness. Maintenance and modification of the LOP orbit are accomplished through use of an onboard propulsion system that burns lunar-supplied oxygen and aluminum. The relatively low performance of such a system is more than compensated for by the elimination of the need for Earth-supplied propellants. The LOP concept envisions a continuous expansion of capability through the incorporation of new instrument technologies and the addition of platforms.

  7. [The focal renal lesions].

    PubMed

    Tuma, Jan

    2013-06-01

    The focal renal lesions are altogether common. Most frequently are found Columna Bertini hypertrophies (so called pseudotumors) and simple renal cysts. The role of sonography in the practice is to distinguish pseudotumors from real renal tumors, and simple renal cysts from complex cysts. The differentiation of complex renal cysts is possible with the help of the CEUS (= contrast enhanced ultrasound) and other imaging modalities such as CT or MRI. In these cases, the CEUS imaging agent has clear advantages over CT and MRI, because it is composed of gas bubbles, which are only slightly smaller than red blood cells and remains exclusively intravascularly while the CT and MRI contrast agents diffuse into the interstitial space without any real perfusion. The real tumors can be differentiated from certain focal non-tumorous changes based on the ultrasound and clinic. The further differentiation of individual kidney tumors and metastases using ultrasound, MRI, CT and CEUS is only partly possible. In all uncertain or unclear cases, therefore, an open or ultrasound-guided biopsy is useful.

  8. Orbital cavernous hemangiomas: ultrasound and magnetic resonance imaging evaluation.

    PubMed

    Diamantopoulou, A; Damianidis, Ch; Kyriakou, V; Kotziamani, N; Emmanouilidou, M; Goutsaridou, F; Tsitouridis, I

    2010-03-01

    Cavernous hemangioma is the most common intraorbital lesion in adults. The aim of our study was to evaluate the magnetic resonance imaging (MRI) and ultrasound (US) characteristics of cavernous hemangioma and their role in the differential diagnosis of orbital tumors. Eight patients with orbital cavernous hemangiomas, five women and three men with a mean age of 48 years were examined in a period of six years. All patients underwent MRI examination and four patients were also evaluated by US. In all cases MRI depicted a well-defined intraconal tumor. The lesions were homogeneous, isointense to muscle on T1-weighted sequence and hyperintense to muscle on T2-weighted sequence in six patients. In one patient the mass was isointense on T1WI with heterogeneous signal intensity on T2WI and in one patient the lesion had heterogeneous signal intensity on both T1- and T2-weighted sequences. After intravenous contrast medium administration, the tumors showed initial inhomogeneous enhancement with progressive accumulation of contrast material on delayed images in seven patients and initial homogeneous enhancement in one patient. On ultrasonography, the orbital masses appeared slightly hyperechoic, heterogeneous with small areas of slow blood flow. The analysis of imaging characteristics of a well-defined intraconal lesion in an adult patient with painless progressive proptosis can be highly suggestive of the diagnosis of cavernous hemangioma.

  9. Characterization of recovery and neuropsychological consequences of orbitofrontal lesion: A case study.

    PubMed

    Fisher, T; Shamay-Tsoory, Simone G; Eran, A; Aharon-Peretz, J

    2011-06-01

    We present a case study of a patient with acquired prefrontal lesion involving mainly ventromedial and orbital structures (VM-PFD). The patient showed behavioral and emotional disturbances one year after the injury. In a follow-up examination seven years later, we evaluated her performance in tasks found theoretically to be sensitive to orbital and medial lesions. In contrast to our hypothesis, her performance was in the normal range. We suggest that a possible explanation for her magnificent recovery may include a high cognitive reserve and the specific characteristics of her injury. PMID:21667397

  10. Overall view of the Orbiter Servicing Structure within the Orbiter ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    Overall view of the Orbiter Servicing Structure within the Orbiter Processing Facility at Kennedy Space Center. Can you see any hint of the Orbiter Discovery? It is in there. - Space Transportation System, Orbiter Discovery (OV-103), Lyndon B. Johnson Space Center, 2101 NASA Parkway, Houston, Harris County, TX

  11. Biphasic solitary fibrous tumor of the orbit with distant metastases.

    PubMed

    Parrozzani, Raffaele; Fusetti, Stefano; Montesco, Cristina; Favero, Vittorio; Midena, Edoardo

    2013-12-01

    The aim of this study is to report the first case of biphasic solitary fibrous tumor (SFT) of the orbit with documented histological transformation and metastatic diffusion. We describe a case of a 23-year-old Caucasian man with recurrent SFT of the right orbit with intracranial invasion. The patient underwent surgical tumor removal via a right fronto-orbital approach. Histopathological examination showed a biphasic tumor pattern with both spindle cell and epithelioid components. The histopathological re-evaluation of previously removed lesions (1999 and 2004) confirmed the diagnosis of SFT, without any evidence of epithelioid component at that time. The patient developed local recurrence and systemic metastases (occipital foramen and clivus, paravertebral muscles and peritoneum) three years after surgery. We are unaware of previous reports of biphasic solitary fibrous tumor of the orbit with documented histological transformation and metastatic diffusion.

  12. Orbitomaxillary mass after repair of the orbital floor.

    PubMed

    Verma, Shreya; Garg, Ankit; Nastri, Alf

    2014-12-01

    A 48-year-old man presented with an orbitomaxillary mass 31 years after repair of the orbital floor. He gave a history of progressive diplopia and paraesthesiae of the right infraorbital nerve. He also noted improvement in his long-standing post-traumatic enophthalmos. Imaging showed a large orbitomaxillary cystic mass, which was thought to be secondary to a silicone implant. The implant and the cystic mass were removed, and the orbital floor was reconstructed with titanium mesh. Histological examination confirmed an inclusion cyst. Maxillary antral lesions can present with symptoms such as sinusitis, paraesthesiae, diplopia, and orofacial pain, and they may arise from the lining of the sinus, or from surrounding structures such as the orbit, nose, or maxilla. This was a late complication of silicone elastomeric implants, and there are alternative treatments for defects of the orbital floor. PMID:25305794

  13. A Case Report: The Diagnosis and Therapeutic Evaluation for a Rare Disease of Langerhans Cell Histiocytosis Involving Thyroid

    PubMed Central

    Cai, Ye-Feng; Wang, Qing-Xuan; Ni, Chun-Jue; Dong, Si-Yang; Lv, Lin; Li, Quan; Chen, En-Dong; Zhang, Xiao-Hua

    2015-01-01

    Abstract Langerhans cell histiocytosis (LCH) involving the thyroid gland is extremely rare. Currently, the diagnosis and therapeutic evaluation for LCH involving thyroid is a challenge. We reported a rare case of LCH involving thyroid, presenting as painless thyroid goiters, and successfully performed positron emission tomography/computed tomography (PET/CT) to make an accurate diagnosis and therapeutic evaluation for LCH. Although the histology or cytology is the golden standard for the diagnosis of LCH involving thyroid, the PET/CT should be keep in mind when LCH involving thyroid with inconclusive cytologic results. During the treatment of LCH, PET/CT can be performed to assess the therapeutic effect and select the most effective and reliable treatment for LCH. PMID:26554785

  14. Total pleurectomy as the surgical treatment for recurrent secondary spontaneous pneumothorax in a child with severe pulmonary Langerhans cells histiocytosis.

    PubMed

    Abdul Aziz, Dayang Anita; Abdul Rahman, Nur Afdzillah; Tang, Swee Fong; Abdul Latif, Hasniah; Zaki, Faizah Mohd; Annuar, Zulfiqar Mohd; Alias, Hamidah; Abdul Latiff, Zarina

    2011-12-01

    Pulmonary Langerhans cell histiocytosis (LCH) in children is more extensive and is a rare cause of spontaneous secondary pneumothorax (SSP) which tends to be recurrent and refractory to conventional treatment. Its occurrence in paediatric patients posed great challenge to the choice of surgical management. Surgery in the form of pleurodesis is only considered if SSP does not improve after chemotherapy and after considering all relevant risk and benefits of surgery to patients. Chemical pleurodesis will not give the expected effect to eradicate SSP in this patient. Therefore mechanical pleurodesis is the treatment of choice. There are various techniques to perform mechanical pleurodesis; from pleural abrasion to pleurectomy. In the authors' experience, bilateral total pleurectomy provided the best outcome for this 9-year-old patient with persistent respiratory distress from SSP due to extensive pulmonary LCH.

  15. Granulomatous cheilitis with intralymphatic histiocytosis possibly associated with calcium deposition caused by chronic inflammation owing to dental metals and periodontitis.

    PubMed

    Kano, Shinya; Kurimoto, Shingo; Ito, Ayako; Yoshida, Yuichi; Yamamoto, Osamu

    2015-01-01

    Granulomatous cheilitis (GC) is an uncommon disease and the pathogenesis of GC has not been clear. Here, we present two cases of GC associated with calcium deposition caused by chronic inflammation owing to dental metal allergy and periodontitis. Histopathologically, intralymphatic histiocytosis (IH) in addition to non-caseating granulomas was seen in both cases. The results of a patch test were positive for some dental metals. Qualitative analyses by energy-dispersive X-ray spectroscopy detected calcium from biopsy specimens. We considered that partial destruction of teeth by chronic inflammation induced reactive proliferation of histiocytes, resulting in IH. Our study suggests that GC and IH is a consequence of chronic inflammatory diseases caused by dental metals and periodontitis. Dental care is necessary for treatment of GC.

  16. [Orbital complications of sinusitis].

    PubMed

    Šuchaň, M; Horňák, M; Kaliarik, L; Krempaská, S; Koštialová, T; Kovaľ, J

    2014-12-01

    Orbital complications categorised by Chandler are emergency. They need early diagnosis and agresive treatment. Stage and origin of orbital complications are identified by rhinoendoscopy, ophtalmologic examination and CT of orbite and paranasal sinuses. Periorbital cellulitis and early stage of orbital cellulitis can be treated conservatively with i. v. antibiotics. Monitoring of laboratory parameters and ophtalmologic symptoms is mandatory. Lack of improvement or worsening of symptoms within 24-48 hours and advanced stages of orbital complications are indicated for surgery. The purpose of the study is to evaluate epidemiology, clinical features and management of sinogenic orbital complications. Retrospective data of 8 patients with suspicion of orbital complication admited to hospital from 2008 to 2013 were evaluated. Patients were analyzed in terms of gender, age, CT findings, microbiology, clinical features, stage and treatment. Male and female were afected in rate 1,66:1. Most of patients were young adult in 3rd. and 4th. decade of life (62,5 %). Acute and chronic sinusitis were cause of orbital complication in the same rate. The most common origin of orbital complication was ethmoiditis (62,5 %), than maxillary (25 %) and frontal (12,5 %) sinusitis. Polysinusitis with affection of ethmoidal, maxillary and frontal sinuses (75 %) was usual CT finding. Staphylococcus epidermidis and Staphylococcus aureus were etiological agens in half of cases. Periorbital oedema (100 %), proptosis, chemosis (50 %), diplopia and glaucoma (12,5 %) were observed. Based on examinations, diagnosis of periorbital oedema/preseptal cellulitis was made in 3 (37,5 %), orbital cellulitis in 3 (37,5 %) and subperiosteal abscess in 2 cases (25 %). All patients underwent combined therapy - i. v. antibiotics and surgery within 24 hours. Eradication of disease from ostiomeatal complex (OMC), drainage of affected sinuses and drainage of subperiosteal abscess were done via fuctional endonasal

  17. Mars Geoscience Orbiter and Lunar Geoscience Orbiter

    NASA Technical Reports Server (NTRS)

    Fuldner, W. V.; Kaskiewicz, P. F.

    1983-01-01

    The feasibility of using the AE/DE Earth orbiting spacecraft design for the LGO and/or MGO missions was determined. Configurations were developed and subsystems analysis was carried out to optimize the suitability of the spacecraft to the missions. The primary conclusion is that the basic AE/DE spacecraft can readily be applied to the LGO mission with relatively minor, low risk modifications. The MGO mission poses a somewhat more complex problem, primarily due to the overall maneuvering hydrazine budget and power requirements of the sensors and their desired duty cycle. These considerations dictate a modification (scaling up) of the structure to support mission requirements.

  18. Elliptical Orbit Performance Computer Program

    NASA Technical Reports Server (NTRS)

    Myler, T.

    1984-01-01

    Elliptical Orbit Performance (ELOPE) computer program for analyzing orbital performance of space boosters uses orbit insertion data obtained from trajectory simulation to generate parametric data on apogee and perigee altitudes as function of payload data. Data used to generate presentation plots that display elliptical orbit performance capability of space booster.

  19. Orbital Fluid Resupply Assessment

    NASA Technical Reports Server (NTRS)

    Eberhardt, Ralph N.

    1989-01-01

    Orbital fluid resupply can significantly increase the cost-effectiveness and operational flexibility of spacecraft, satellites, and orbiting platforms and observatories. Reusable tankers are currently being designed for transporting fluids to space. A number of options exist for transporting the fluids and propellant to the space-based user systems. The fluids can be transported to space either in the Shuttle cargo bay or using expendable launch vehicles (ELVs). Resupply can thus be accomplished either from the Shuttle bay, or the tanker can be removed from the Shuttle bay or launched on an ELV and attached to a carrier such as the Orbital Maneuvering Vehicle (OMV) or Orbital Transfer Vehicle (OTV) for transport to the user to be serviced. A third option involves locating the tanker at the space station or an unmanned platform as a quasi-permanent servicing facility or depot which returns to the ground for recycling once its tanks are depleted. Current modular tanker designs for monopropellants, bipropellants, and water for space station propulsion are discussed. Superfluid helium tankers are addressed, including trade-offs in tanker sizes, shapes to fit the range of ELVs currently available, and boil-off losses associated with longer-term (greater than 6-month) space-basing. It is concluded that the mixed fleet approach to on-orbit consumables resupply offers significant advantages to the overall logistics requirements.

  20. Orbital spacecraft resupply technology

    NASA Technical Reports Server (NTRS)

    Eberhardt, R. N.; Tracey, T. R.; Bailey, W. J.

    1986-01-01

    The resupplying of orbital spacecraft using the Space Shuttle, Orbital Maneuvering Vehicle, Orbital Transfer Vehicle or a depot supply at a Space Station is studied. The governing factor in fluid resupply designs is the system size with respect to fluid resupply quantities. Spacecraft propellant management for tankage via diaphragm or surface tension configurations is examined. The capabilities, operation, and application of adiabatic ullage compression, ullage exchange, vent/fill/repressurize, and drain/vent/no-vent fill/repressurize, which are proposed transfer methods for spacecraft utilizing tankage configurations, are described. Selection of the appropriate resupply method is dependent on the spacecraft design features. Hydrazine adiabatic compression/detonation, liquid-free vapor venting to prevent freezing, and a method for no-vent liquid filling are analyzed. Various procedures for accurate measurements of propellant mass in low gravity are evaluated; a system of flowmeters with a PVT system was selected as the pressurant solubility and quantity gaging technique. Monopropellant and bipropellant orbital spacecraft consumable resupply system tanks which resupply 3000 lb of hydrazine and 7000 lb of MMH/NTO to spacecraft on orbit are presented.

  1. Mars Telecommunications Orbiter, Artist's Concept

    NASA Technical Reports Server (NTRS)

    2005-01-01

    This illustration depicts a concept for NASA's Mars Telecommunications Orbiter in flight around Mars. The orbiter is in development to be the first spacecraft with a primary function of providing communication links while orbiting a foreign planet. The project's plans call for launch in September 2009, arrival at Mars in August 2010 and a mission of six to 10 years while in orbit. Mars Telecommunication Orbiter would serve as the Mars hub for an interplanetery Internet, greatly increasing the information payoff from other future Mars missions. The mission is designed to orbit Mars more than 10 times farther from the planet than orbiters dedicated primarily to science. The high-orbit design minimizes the time that Mars itself blocks the orbiter from communicating with Earth and maximizes the time that the orbiter is above the horizon -- thus capable of communications relay -- for rovers and stationary landers on Mars' surface.

  2. Unusual case of traumatic neuroma of the orbit.

    PubMed

    Ulivieri, Simone; Muscas, Giovanni; Miracco, Clelia; Oliveri, Giuseppe; Galluzzi, Paolo; Giorgio, Antonio

    2016-01-01

    Traumatic or amputation neuromas are neoformations developing after damage to a peripheral nerve. They are not proper tumors but rather a reactive process or a frustrated attempt of nerve regeneration. Traumatic neuromas are potentially found in every sensory peripheral nerve and often at the site of past surgical intervention, including orbital surgery. A 29-year-old Northern African migrant presented progressive exophthalmos and progressive loss of acuity in left eye, which had started about 6 months before after a cranio-facial trauma caused by a violent assault. MRI of the orbits showed a massive intra-orbital, intra-conical lesion, clearly compressing and dislocating the optic nerve and extending posteriorly to the orbital apex. Surgery was performed through lateral approach of Kroenlein and led to complete excision of the lesion. Histology revealed fibrotic, adipose and striated muscle tissues, a disordered, non-neoplastic overgrowth of small and large fascicles of nerves, inflammatory infiltrates, and fibrosis with sparse calcifications were diffusely observed in a background of fat, scar and striated muscle tissued. Patient was discharged on the fifth day in good health condition, without deficit of eye motion but without recovery of visual acuity. In conclusion, this case demonstrates that traumatic neuromas may arise in the orbit in patients with minor direct trauma to nerves and without previous surgical treatment.

  3. Voxelwise Bayesian Lesion Deficit Analysis

    PubMed Central

    Chen, Rong; Hillis, Argye E.; Pawlak, Mikolaj; Herskovits, Edward H

    2008-01-01

    Relating cognitive deficits to the presence of lesions has been an important means of delineating structure-function associations in the human brain. We propose a voxel-based Bayesian method for lesion-deficit analysis, which identifies complex linear or nonlinear associations among brain-lesion locations, and neurological status. We validated this method using a simulated data set, and we applied this algorithm to data obtained from an acute-stroke study to identify associations among voxels with infarct or hypoperfusion, and impaired word reading. We found that a distributed region involving Brodmann areas (BA) 22, 37, 39, and 40 was implicated in word reading. PMID:18328733

  4. Nerve lesioning with direct current

    NASA Astrophysics Data System (ADS)

    Ravid, E. Natalie; Shi Gan, Liu; Todd, Kathryn; Prochazka, Arthur

    2011-02-01

    Spastic hypertonus (muscle over-activity due to exaggerated stretch reflexes) often develops in people with stroke, cerebral palsy, multiple sclerosis and spinal cord injury. Lesioning of nerves, e.g. with phenol or botulinum toxin is widely performed to reduce spastic hypertonus. We have explored the use of direct electrical current (DC) to lesion peripheral nerves. In a series of animal experiments, DC reduced muscle force by controlled amounts and the reduction could last several months. We conclude that in some cases controlled DC lesioning may provide an effective alternative to the less controllable molecular treatments available today.

  5. No Carious Cervical Lesions: Abfraction

    PubMed Central

    Shetty, Sumanth M; Shetty, Rashmi G; Mattigatti, Sudha; Managoli, Noopur A; Rairam, Surabhi G; Patil, Ashwini M

    2013-01-01

    Abfraction or Theory of Abfraction is a theory explaining the non-carious cervical lesions (NCCL). It suggests that they are caused by flexural forces, usually from cyclic loading; the enamel, especially at the cementoenamel junction (CEJ), undergoes this pattern of destruction by separating the enamel rods. Clinical aspect importance of these ineart lesions are at most important to be detected for early intervention and treatment modalities as options during the progression of the disease. How to cite this article: Shetty SM, Shetty RG, Mattigatti S, Managoli NA, Rairam SG, Patil AM. No Carious Cervical Lesions: Abfraction. J Int Oral Health 2013; 5(5):142-5. PMID:24324319

  6. Deceleration Orbit Improvements

    SciTech Connect

    Church, M.

    1991-04-26

    During the accelerator studies period of 12/90-1/91 much study time was dedicated to improving the E760 deceleration ramps. 4 general goals were in mind: (1) Reduce the relative orbit deviations from the nominal reference orbit as much as possible. This reduces the potential error in the orbit length calculation - which is the primary source of error in the beam energy calculation. (2) Maximize the transverse apertures. This minimizes beam loss during deceleration and during accidental beam blow-ups. (3) Measure and correct lattice parameters. Knowledge of {gamma}{sub T}, {eta}, Q{sub h}, Q{sub v}, and the dispersion in the straight sections allows for a more accurate energy calculation and reliable SYNCH calculations. (4) Minimize the coupling. This allows one to discern between horizontal and vertical tunes.

  7. Spiral Orbit Tribometer

    NASA Technical Reports Server (NTRS)

    Pepper, Stephen V.; Jones, William R., Jr.; Kingsbury, Edward; Jansen, Mark J.

    2007-01-01

    The spiral orbit tribometer (SOT) bridges the gap between full-scale life testing and typically unrealistic accelerated life testing of ball-bearing lubricants in conjunction with bearing ball and race materials. The SOT operates under realistic conditions and quickly produces results, thereby providing information that can guide the selection of lubricant, ball, and race materials early in a design process. The SOT is based upon a simplified, retainerless thrust bearing comprising one ball between flat races (see figure). The SOT measures lubricant consumption and degradation rates and friction coefficients in boundary lubricated rolling and pivoting contacts. The ball is pressed between the lower and upper races with a controlled force and the lower plate is rotated. The combination of load and rotation causes the ball to move in a nearly circular orbit that is, more precisely, an opening spiral. The spiral s pitch is directly related to the friction coefficient. At the end of the orbit, the ball contacts the guide plate, restoring the orbit to its original radius. The orbit is repeatable throughout the entire test. A force transducer, mounted in-line with the guide plate, measures the force between the ball and the guide plate, which directly relates to the friction coefficient. The SOT, shown in the figure, can operate in under ultra-high vacuum (10(exp -9) Torr) or in a variety of gases at atmospheric pressure. The load force can be adjusted between 45 and 450 N. By varying the load force and ball diameter, mean Hertzian stresses between 0.5 and 5.0 GPa can be obtained. The ball s orbital speed range is between 1 and 100 rpm.

  8. Mars Orbiter Laser Altimeter

    NASA Technical Reports Server (NTRS)

    Zuber, Maria T.

    1997-01-01

    The objective of this study was to support the rebuild and implementation of the Mars Orbiter Laser Altimeter (MOLA) investigation and to perform scientific analysis of current Mars data relevant to the investigation. The instrument is part of the payload of the NASA Mars Global Surveyor (MGS) mission. The instrument is a rebuild of the Mars Observer Laser Altimeter that was originally flown on the ill-fated Mars Observer mission. The instrument is currently in orbit around Mars and has so far returned remarkable data.

  9. Pediatric Orbital Fractures

    PubMed Central

    Oppenheimer, Adam J.; Monson, Laura A.; Buchman, Steven R.

    2013-01-01

    It is wise to recall the dictum “children are not small adults” when managing pediatric orbital fractures. In a child, the craniofacial skeleton undergoes significant changes in size, shape, and proportion as it grows into maturity. Accordingly, the craniomaxillofacial surgeon must select an appropriate treatment strategy that considers both the nature of the injury and the child's stage of growth. The following review will discuss the management of pediatric orbital fractures, with an emphasis on clinically oriented anatomy and development. PMID:24436730

  10. Electrocautery for Precancerous Anal Lesions

    Cancer.gov

    Results from a randomized clinical trial conducted in Amsterdam suggest that electrocautery is better than topical imiquimod or fluorouracil at treating potentially precancerous anal lesions in HIV-positive men who have sex with men.

  11. Gram stain of skin lesion

    MedlinePlus

    ... may be done along with this test. Other studies are often done on a skin sample to determine if cancer is present. Viral skin lesions like herpes simplex are examined by other tests or a viral culture.

  12. Osteochondral Lesions of Major Joints

    PubMed Central

    Durur-Subasi, Irmak; Durur-Karakaya, Afak; Yildirim, Omer Selim

    2015-01-01

    This paper provides information about osteochondral lesions (OCL) and example cases of OCL occurring in major joints, some of which are rarely seen. This simple tutorial is presented in question and answer format. PMID:26180500

  13. Orbital invasion by an intracranial chordoma.

    PubMed

    Ferry, A P; Haddad, H M; Goldman, J L

    1981-07-01

    A 26-year-old woman experienced bilateral hearing loss, progressive nasal obstruction, and rhinorrhea. Examination disclosed a retropharyngeal mass. A needle biopsy specimen of the mass showed that it was a chordoma. The patient underwent surgery to remove the mass and received a postoperative course of radioactive cobalt. She did well for 18 months, at which time proptosis gradually developed in her right eye. Although the optic nerve heads and visual fields appeared normal, roentgenograms showed a large lesion involving the anterior and middle cranial fossae and destruction of the right posterior ethmoid sinus and right superior orbital fissure. Shortly after completing a course of methotrexate therapy (total dose, 89 mg), the patient experienced sudden pain and visual loss in her right eye. Surgical decompression of the orbit failed to restore light perception. The following year, her left eye became involved. A transfrontal craniotomy and extradural orbital decompression provided only temporary improvement. Four months later, left lateral rectus muscle palsy developed and her visual acuity decreased to 6/60 (20/200). Radiation therapy (400 rads per week; total dose, 3,200 rads) and treatment with methotrexate, vincristine sulfate, and prednisone did not improve her condition. At the time of her death, six years after the first symptom appeared, the patient was blind in both eyes, almost completely deaf, and suffered from severe dysphagia. PMID:7258280

  14. The calculation of orbital positioning using standard orbital parameters.

    NASA Astrophysics Data System (ADS)

    Pritchard, W.

    1999-08-01

    Practical difficulties arise solving the deceptively simple Kepler's equation. Kepler's equation can be solved easily using the method Newton developed for doing so. The authors recommend that this method be used in any general approach to orbital calculations. Another practical point to be reckoned with, is the variation in true orbital parameters. It is important to note that inclined orbits, eccentric orbits, and low orbits all suffer from rapid changes in their parameters. They can be ignored only for simple calculations for just a few orbits. Any calculation covering a longer period of time must take these changes into account.

  15. ARTEMIS Lunar Orbit Insertion and Science Orbit Design Through 2013

    NASA Technical Reports Server (NTRS)

    Broschart, Stephen B.; Sweetser, Theodore H.; Angelopoulos, Vassilis; Folta, David; Woodard, Mark

    2015-01-01

    As of late-July 2011, the ARTEMIS mission is transferring two spacecraft from Lissajous orbits around Earth-Moon Lagrange Point #1 into highly-eccentric lunar science orbits. This paper presents the trajectory design for the transfer from Lissajous orbit to lunar orbit insertion, the period reduction maneuvers, and the science orbits through 2013. The design accommodates large perturbations from Earth's gravity and restrictive spacecraft capabilities to enable opportunities for a range of heliophysics and planetary science measurements. The process used to design the highly-eccentric ARTEMIS science orbits is outlined. The approach may inform the design of future planetary moon missions.

  16. Asterixis in focal brain lesions.

    PubMed

    Degos, J D; Verroust, J; Bouchareine, A; Serdaru, M; Barbizet, J

    1979-11-01

    Asterixis was observed in 20 cases of focal brain lesions. Metabolic or toxic factors were excluded. An electromyogram study of asterixis was carried out in nine cases to establish the diagnosis. The site of the focal lesion was either parietal or mesencephalic and was always contralateral to the asterixis. "Focal asterixis" could result from a dysfunction of the sensorimotor integration in the parietal lobe and the midbrain.

  17. Benign Pediatric Salivary Gland Lesions.

    PubMed

    Carlson, Eric R; Ord, Robert A

    2016-02-01

    Salivary gland lesions are rare in pediatric patients. In addition, the types of salivary gland tumors are different in their distribution in specific sites in the major and minor salivary glands in children compared with adults. This article reviews benign neoplastic and nonneoplastic salivary gland disorders in pediatric patients to help clinicians to develop an orderly differential diagnosis that will lead to expedient treatment of pediatric patients with salivary gland lesions.

  18. Endoscopic Management of Dieulafoy's Lesion

    PubMed Central

    Jeon, Hye Kyung

    2015-01-01

    A Dieulafoy's lesion is a vascular abnormality consisting of a large caliber-persistent tortuous submucosal artery. A small mucosal defect with the eruption of this protruding vessel can cause bleeding. In fact, a Dieulafoy's lesion is a relatively rare but potentially life-threatening condition. It accounts for 1% to 2% of cases of acute gastrointestinal bleeding. Although there is no consensus on the treatment of Dieulafoy's lesions; treatment options depend on the mode of presentation, site of the lesion, and available expertise. Endoscopic therapy is usually successful in achieving primary hemostasis, with hemostasis success rates reaching 75% to 100%. Although various therapeutic endoscopic methods are used to control bleeding in Dieulafoy's lesions, the best method for endoscopic intervention is not clear. Combination endoscopic therapy is known to be superior to monotherapy because of a lower rate of recurrent bleeding. In addition, mechanical therapies including hemostatic clipping and endoscopic band ligation are more effective and successful in controlling bleeding than other endoscopic methods. Advances in endoscopic techniques have reduced mortality in patients with Dieulafoy's lesion-from 80% to 8%-and consequently, the need for surgical intervention has been reduced. Currently, surgical intervention is used for cases that fail therapeutic endoscopic or angiographic interventions. PMID:25844338

  19. DNA lesions: A thermodynamic perspective

    SciTech Connect

    Plum, G.E.; Breslauer, K.J.

    1994-12-31

    The studies described in this paper are part of an overall program project entitled {open_quotes}The Chemistry and Biology of Exocyclic DNA Adducts and Oxidative DNA Damage.{close_quotes}. Initially, all the project leaders discuss and agree on biologically interesting lesions to target for study. Then begins the process of developing the chemistry required to synthesize modified nucleosides that either correspond to or model the damage sites of interest. Such modified nucleotides then are incorporated into oligonucleotides that are hybridized to their complements, thereby forming lesion-containing duplex structures. In any given duplex, the identity of the lesion-opposing nucleoside on the complementary strand is systematically altered, thereby allowing us to evaluate the impact on duplex properties of the identity of the base opposite the lesion. For comparative purposes, the undamaged parent Watson-Crick duplex also is synthesized. Such families of DNA duplexes are then sent for independent physiochemical characterizations. Armed with an extensive body of biophysical data, one then searches for correlations between the physiochemical influences of the lesions on duplex properties and the biological consequences of each lesion. At this stage, our approach is highly empirical. Ultimately, we hope that our studies will reveal correlations between physiochemical properties and biological consequences such that we will develop predictive powers and gain insight into the mechanisms of recognition, repair, and mutagenesis.

  20. Simulation of spiculated breast lesions

    NASA Astrophysics Data System (ADS)

    Elangovan, Premkumar; Alrehily, Faisal; Pinto, R. Ferrari; Rashidnasab, Alaleh; Dance, David R.; Young, Kenneth C.; Wells, Kevin

    2016-03-01

    Virtual clinical trials are a promising new approach increasingly used for the evaluation and comparison of breast imaging modalities. A key component in such an assessment paradigm is the use of simulated pathology, in particular, simulation of lesions. Breast mass lesions can be generally classified into two categories based on their appearance; nonspiculated masses and spiculated masses. In our previous work, we have successfully simulated non-spiculated masses using a fractal growth process known as diffusion limited aggregation. In this new work, we have extended the DLA model to simulate spiculated lesions by using features extracted from patient DBT images containing spiculated lesions. The features extracted included spicule length, width, curvature and distribution. This information was used to simulate realistic looking spicules which were attached to the surface of a DLA mass to produce a spiculated mass. A batch of simulated spiculated masses was inserted into normal patient images and presented to an experienced radiologist for review. The study yielded promising results with the radiologist rating 60% of simulated lesions in 2D and 50% of simulated lesions in DBT as realistic.

  1. Europa Orbiter Exploration Strategies

    NASA Technical Reports Server (NTRS)

    Johnson, T. V.

    2001-01-01

    The Europa Orbiter mission is planned as the next stage of Europa exploration. Its primary goals are to search for definitive evidence of a subsurface ocean, to characterize the ice crust and ice/water interface, and to prepare for future surface/sub-surface missions. Additional information is contained in the original extended abstract.

  2. Sedna Orbit Animation

    NASA Technical Reports Server (NTRS)

    2004-01-01

    This animation shows the location of the newly discovered planet-like object, dubbed 'Sedna,' in relation to the rest of the solar system. Starting at the inner solar system, which includes the orbits of Mercury, Venus, Earth, and Mars (all in yellow), the view pulls away through the asteroid belt and the orbits of the outer planets beyond (green). Pluto and the distant Kuiper Belt objects are seen next until finally Sedna comes into view. As the field widens the full orbit of Sedna can be seen along with its current location. Sedna is nearing its closest approach to the Sun; its 10,000 year orbit typically takes it to far greater distances. Moving past Sedna, what was previously thought to be the inner edge of the Oort cloud appears. The Oort cloud is a spherical distribution of cold, icy bodies lying at the limits of the Sun's gravitational pull. Sedna's presence suggests that this Oort cloud is much closer than scientists believed.

  3. Global orbit corrections

    SciTech Connect

    Symon, K.

    1987-11-01

    There are various reasons for preferring local (e.g., three bump) orbit correction methods to global corrections. One is the difficulty of solving the mN equations for the required mN correcting bumps, where N is the number of superperiods and m is the number of bumps per superperiod. The latter is not a valid reason for avoiding global corrections, since, we can take advantage of the superperiod symmetry to reduce the mN simultaneous equations to N separate problems, each involving only m simultaneous equations. Previously, I have shown how to solve the general problem when the machine contains unknown magnet errors of known probability distribution; we made measurements of known precision of the orbit displacements at a set of points, and we wish to apply correcting bumps to minimize the weighted rms orbit deviations. In this report, we will consider two simpler problems, using similar methods. We consider the case when we make M beam position measurements per superperiod, and we wish to apply an equal number M of orbit correcting bumps to reduce the measured position errors to zero. We also consider the problem when the number of correcting bumps is less than the number of measurements, and we wish to minimize the weighted rms position errors. We will see that the latter problem involves solving equations of a different form, but involving the same matrices as the former problem.

  4. A Neptune Orbiter Mission

    NASA Technical Reports Server (NTRS)

    Wallace, R. A.; Spilker, T. R.

    1998-01-01

    This paper describes the results of new analyses and mission/system designs for a low cost Neptune Orbiter mission. Science and measurement objectives, instrumentation, and mission/system design options are described and reflect an aggressive approach to the application of new advanced technologies expected to be available and developed over the next five to ten years.

  5. Mars Climate Orbiter

    NASA Technical Reports Server (NTRS)

    1998-01-01

    The Mars Surveyor '98 Climate Orbiter is shown here during acoustic tests that simulate launch conditions. The orbiter was to conduct a two year primary mission to profile the Martian atmosphere and map the surface. To carry out these scientific objectives, the spacecraft carried a rebuilt version of the pressure modulated infrared radiometer, lost with the Mars Observer spacecraft, and a miniaturized dual camera system the size of a pair of binoculars, provided by Malin Space Science Systems, Inc., San Diego, California. During its primary mission, the orbiter was to monitor Mars atmosphere and surface globally on a daily basis for one Martian year (two Earth years), observing the appearance and movement of atmospheric dust and water vapor, as well as characterizing seasonal changes of the planet's surface. Imaging of the surface morphology would also provide important clues about the planet's climate in its early history. The mission was part of NASA's Mars Surveyor program, a sustained program of robotic exploration of the red planet, managed by the Jet Propulsion Laboratory for NASA's Office of Space Science, Washington, DC. Lockheed Martin Astronautics was NASA's industrial partner in the mission. Unfortunately, Mars Climate Orbiter burned up in the Martian atmosphere on September 23, 1999, due to a metric conversion error that caused the spacecraft to be off course.

  6. Spin-orbit photonics

    NASA Astrophysics Data System (ADS)

    Cardano, Filippo; Marrucci, Lorenzo

    2015-12-01

    Spin-orbit optical phenomena involve the interaction of the photon spin with the light wave propagation and spatial distribution, mediated by suitable optical media. Here we present a short overview of the emerging photonic applications that rely on such effects.

  7. Orbital Fluid Transfer System

    NASA Technical Reports Server (NTRS)

    Johnston, A. S., (Nick); Ryder, Mel; Tyler, Tony R.

    1998-01-01

    An automated fluid and power interface system needs to be developed for future space missions which require on orbit consumable replenishment. Current method of fluid transfer require manned vehicles and extravehicular activity. Currently the US does not have an automated capability for consumable transfer on-orbit. This technology would benefit both Space Station and long duration satellites. In order to provide this technology the Automated Fluid Interface System (AFIS) was developed. The AFIS project was an advanced development program aimed at developing a prototype satellite servicer for future space operations. This mechanism could transfer propellants, cryogens, fluids, gasses, electrical power, and communications from a tanker unit to the orbiting satellite. The development of this unit was a cooperative effort between Marshall Space Flight Center in Huntsville, Alabama, and Moog, Inc. in East Aurora, New York. An engineering model was built and underwent substantial development testing at Marshall Space Flight Center (MSFC). While the AFIS is not suitable for spaceflight, testing and evaluation of the AFIS provided significant experience which would be beneficial in building a flight unit. The lessons learned from testing the AFIS provided the foundation for the next generation fluid transfer mechanism, the Orbital Fluid Transfer System (OFTS). The OFTS project was a study contract with MSFC and Moog, Inc. The OFTS was designed for the International Space Station (ISS), but its flexible design could used for long duration satellite missions and other applications. The OFTS was designed to be used after docking. The primary function was to transfer bipropellants and high pressure gases. The other items addressed by this task included propellant storage, hardware integration, safety and control system issues. A new concept for high pressure couplings was also developed. The results of the AFIS testing provided an excellent basis for the OFTS design. The OFTS

  8. [Orbital decompression for Graves' ophthalmopathy].

    PubMed

    Boulétreau, P; Breton, P; Freidel, M

    2005-04-01

    Graves' ophthalmopathy is a complex orbital condition with a controversial pathogenesis. It is the clinical expression of a discordance between the inextensible orbit and hypertrophic muscular and fatty elements within the orbit responding to immunological stimulation. The relationship between the orbital and its content can be improved by surgical expansion which increases the useful volume of the orbit. This procedure can be combined with lipectomy to decrease the volume of the orbital contents. We briefly recall the history of surgical decompression techniques and present our experience with Graves' ophthalmopathy patients.

  9. Occult orbital neuroblastoma detected after administration of an antitumor vaccine.

    PubMed

    Wilson, Matthew W; Moshfeghi, Darius M; Haik, Barrett G; Haight, Ann E; Hill, D Ashley; Davidoff, Andrew M; Rousseau, Raphael F; Bowman, Laura C

    2003-01-01

    A 6-year-old girl with neuroblastoma developed swelling and erythema of her right upper eyelid following administration of an interleukin-2 and lymphotactin gene-modified allogeneic neuroblastoma cell vaccine. Computed tomography demonstrated a cystic lesion in the subperiosteal space. A biopsy of the mass showed necrotic neuroblastoma with minimal associated inflammation. To our knowledge, this case represents the first description of occult orbital metastases in a patient with neuroblastoma detected after administration of an antitumor vaccine.

  10. Prefrontal and Executive Attention Network Lesions and the Development of Attention-Deficit/hyperactivity Symptomatology.

    ERIC Educational Resources Information Center

    Max, Jeffrey E.; Manes, Facundo F.; Robertson, Brigitte A.M.; Mathews, Katherine; Fox, Peter T.; Lancaster, Jack

    2005-01-01

    Objective: To investigate the association between focal stroke lesions of Posner's executive attention network and a specific region of interest in the frontal lobes (orbital frontal and mesial frontal) and either attention-deficit/hyperactivity disorder (ADHD) or traits of the disorder (ADHD symptomatology). Method: Twenty-nine children with…

  11. Unusual sclerosing orbital pseudotumor infiltrating orbits and maxillofacial regions.

    PubMed

    Toprak, Huseyin; Aralaşmak, Ayşe; Yılmaz, Temel Fatih; Ozdemir, Huseyin

    2014-01-01

    Idiopathic orbital pseudotumor (IOP) is a benign inflammatory condition of the orbit without identifiable local or systemic causes. Bilateral massive orbital involvement and extraorbital extension of the IOP is very rare. We present an unusual case of IOP with bilateral massive orbital infiltration extending into maxillofacial regions and discuss its distinctive magnetic resonance imaging (MRI) features that help to exclude other entities during differential diagnoses.

  12. Unusual Sclerosing Orbital Pseudotumor Infiltrating Orbits and Maxillofacial Regions

    PubMed Central

    Toprak, Huseyin; Aralaşmak, Ayşe; Yılmaz, Temel Fatih; Ozdemir, Huseyin

    2014-01-01

    Idiopathic orbital pseudotumor (IOP) is a benign inflammatory condition of the orbit without identifiable local or systemic causes. Bilateral massive orbital involvement and extraorbital extension of the IOP is very rare. We present an unusual case of IOP with bilateral massive orbital infiltration extending into maxillofacial regions and discuss its distinctive magnetic resonance imaging (MRI) features that help to exclude other entities during differential diagnoses. PMID:24991481

  13. Comparison of Low Earth Orbit and Geosynchronous Earth Orbits

    NASA Technical Reports Server (NTRS)

    Drummond, J. E.

    1980-01-01

    The technological, environmental, social, and political ramifications of low Earth orbits as compared to geosynchronous Earth orbits for the solar power satellite (SPS) are assessed. The capital cost of the transmitting facilities is dependent on the areas of the antenna and rectenna relative to the requirement of high efficiency power transmission. The salient features of a low orbit Earth orbits are discussed in terms of cost reduction efforts.

  14. Shuttle on-orbit rendezvous targeting: Circular orbits

    NASA Technical Reports Server (NTRS)

    Bentley, E. L.

    1972-01-01

    The strategy and logic used in a space shuttle on-orbit rendezvous targeting program are described. The program generates ascent targeting conditions for boost to insertion into an intermediate parking orbit, and generates on-orbit targeting and timeline bases for each maneuver to effect rendezvous with a space station. Time of launch is determined so as to eliminate any plane change, and all work was performed for a near-circular space station orbit.

  15. Close up view of the Orbiter Discovery in the Orbiter ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    Close up view of the Orbiter Discovery in the Orbiter Processing Facility at Kennedy Space Center. The view is a detail of the aft, starboard landing gear and a general view of the Thermal Protection System tiles around the landing-gear housing. - Space Transportation System, Orbiter Discovery (OV-103), Lyndon B. Johnson Space Center, 2101 NASA Parkway, Houston, Harris County, TX

  16. Helioseismology with Solar Orbiter

    NASA Astrophysics Data System (ADS)

    Löptien, Björn; Birch, Aaron C.; Gizon, Laurent; Schou, Jesper; Appourchaux, Thierry; Blanco Rodríguez, Julián; Cally, Paul S.; Dominguez-Tagle, Carlos; Gandorfer, Achim; Hill, Frank; Hirzberger, Johann; Scherrer, Philip H.; Solanki, Sami K.

    2015-12-01

    The Solar Orbiter mission, to be launched in July 2017, will carry a suite of remote sensing and in-situ instruments, including the Polarimetric and Helioseismic Imager (PHI). PHI will deliver high-cadence images of the Sun in intensity and Doppler velocity suitable for carrying out novel helioseismic studies. The orbit of the Solar Orbiter spacecraft will reach a solar latitude of up to 21∘ (up to 34∘ by the end of the extended mission) and thus will enable the first local helioseismology studies of the polar regions. Here we consider an array of science objectives to be addressed by helioseismology within the baseline telemetry allocation (51 Gbit per orbit, current baseline) and within the science observing windows (baseline 3×10 days per orbit). A particularly important objective is the measurement of large-scale flows at high latitudes (rotation and meridional flow), which are largely unknown but play an important role in flux transport dynamos. For both helioseismology and feature tracking methods convection is a source of noise in the measurement of longitudinally averaged large-scale flows, which decreases as T -1/2 where T is the total duration of the observations. Therefore, the detection of small amplitude signals (e.g., meridional circulation, flows in the deep solar interior) requires long observation times. As an example, one hundred days of observations at lower spatial resolution would provide a noise level of about three m/s on the meridional flow at 80∘ latitude. Longer time-series are also needed to study temporal variations with the solar cycle. The full range of Earth-Sun-spacecraft angles provided by the orbit will enable helioseismology from two vantage points by combining PHI with another instrument: stereoscopic helioseismology will allow the study of the deep solar interior and a better understanding of the physics of solar oscillations in both quiet Sun and sunspots. We have used a model of the PHI instrument to study its

  17. SPECS: Orbital debris removal

    NASA Technical Reports Server (NTRS)

    1991-01-01

    The debris problem has reached a stage at which the risk to satellites and spacecraft has become substantial in low Earth orbit (LEO). This research discovered that small particles posed little threat to spacecraft because shielding can effectively prevent these particles from damaging the spacecraft. The research also showed that, even though collision with a large piece of debris could destroy the spacecraft, the large pieces of debris pose little danger because they can be tracked and the spacecraft can be maneuvered away from these pieces. Additionally, there are many current designs to capture and remove large debris particles from the space environment. From this analysis, it was decided to concentrate on the removal of medium-sized orbital debris, that is, those pieces ranging from 1 cm to 50 cm in size. The current design incorporates a transfer vehicle and a netting vehicle to capture the medium-sized debris. The system is based near an operational space station located at 28.5 deg inclination and 400 km altitude. The system uses ground-based tracking to determine the location of a satellite breakup or debris cloud. These data are uploaded to the transfer vehicle, which proceeds to rendezvous with the debris at a lower altitude parking orbit. Next, the netting vehicle is deployed, tracks the targeted debris, and captures it. After expending the available nets, the netting vehicle returns to the transfer vehicle for a new netting module and continues to capture more debris in the target area. Once all the netting modules are expended, the transfer vehicle returns to the space station's orbit where it is resupplied with new netting modules from a space shuttle load. The new modules are launched by the shuttle from the ground and the expended modules are taken back to Earth for removal of the captured debris, refueling, and repacking of the nets. Once the netting modules are refurbished, they are taken back into orbit for reuse. In a typical mission, the

  18. SPECS: Orbital debris removal

    NASA Astrophysics Data System (ADS)

    The debris problem has reached a stage at which the risk to satellites and spacecraft has become substantial in low Earth orbit (LEO). This research discovered that small particles posed little threat to spacecraft because shielding can effectively prevent these particles from damaging the spacecraft. The research also showed that, even though collision with a large piece of debris could destroy the spacecraft, the large pieces of debris pose little danger because they can be tracked and the spacecraft can be maneuvered away from these pieces. Additionally, there are many current designs to capture and remove large debris particles from the space environment. From this analysis, it was decided to concentrate on the removal of medium-sized orbital debris, that is, those pieces ranging from 1 cm to 50 cm in size. The current design incorporates a transfer vehicle and a netting vehicle to capture the medium-sized debris. The system is based near an operational space station located at 28.5 deg inclination and 400 km altitude. The system uses ground-based tracking to determine the location of a satellite breakup or debris cloud. These data are uploaded to the transfer vehicle, which proceeds to rendezvous with the debris at a lower altitude parking orbit. Next, the netting vehicle is deployed, tracks the targeted debris, and captures it. After expending the available nets, the netting vehicle returns to the transfer vehicle for a new netting module and continues to capture more debris in the target area. Once all the netting modules are expended, the transfer vehicle returns to the space station's orbit where it is resupplied with new netting modules from a space shuttle load. The new modules are launched by the shuttle from the ground and the expended modules are taken back to Earth for removal of the captured debris, refueling, and repacking of the nets. Once the netting modules are refurbished, they are taken back into orbit for reuse. In a typical mission, the

  19. LRO Enters Lunar Orbit (Highlights)

    NASA Video Gallery

    After a four and a half day journey from the Earth, the Lunar Reconnaissance Orbiter, or LRO, successfully entered orbit around the moon. Engineers at NASA's Goddard Space Flight Center in Greenbel...

  20. Lunar Reconnaissance Orbiter Mission Highlights

    NASA Video Gallery

    Since launch on June 18, 2009 as a precursor mission, the Lunar Reconnaissance Orbiter (LRO) has remained in orbit around the moon, collecting vast amounts of science data in support of NASA's expl...

  1. The Orbital Acceleration Research Experiment

    NASA Astrophysics Data System (ADS)

    Blanchard, R. C.; Hendrix, M. K.; Fox, J. C.; Thomas, D. J.; Nicholson, J.

    The hardware and software of NASA's proposed Orbital Acceleration Research Experiment (OARE) are described. The OARE is to provide aerodynamic acceleration measurements along the Orbiter's principal axis in the free-molecular flow-flight regime at orbital attitude and in the transition regime during reentry. Models considering the effects of electromagnetic effects, solar radiation pressure, orbiter mass attraction, gravity gradient, orbital centripetal acceleration, out-of-orbital-plane effects, orbiter angular velocity, structural noise, mass expulsion signal sources, crew motion, and bias on acceleration are examined. The experiment contains an electrostatically balanced cylindrical proofmass accelerometer sensor with three orthogonal sensing axis outputs. The components and functions of the experimental calibration system and signal processor and control subsystem are analyzed. The development of the OARE software is discussed. The experimental equipment will be enclosed in a cover assembly that will be mounted in the Orbiter close to the center of gravity.

  2. The Orbital Acceleration Research Experiment

    NASA Technical Reports Server (NTRS)

    Blanchard, R. C.; Hendrix, M. K.; Fox, J. C.; Thomas, D. J.; Nicholson, J.

    1986-01-01

    The hardware and software of NASA's proposed Orbital Acceleration Research Experiment (OARE) are described. The OARE is to provide aerodynamic acceleration measurements along the Orbiter's principal axis in the free-molecular flow-flight regime at orbital attitude and in the transition regime during reentry. Models considering the effects of electromagnetic effects, solar radiation pressure, orbiter mass attraction, gravity gradient, orbital centripetal acceleration, out-of-orbital-plane effects, orbiter angular velocity, structural noise, mass expulsion signal sources, crew motion, and bias on acceleration are examined. The experiment contains an electrostatically balanced cylindrical proofmass accelerometer sensor with three orthogonal sensing axis outputs. The components and functions of the experimental calibration system and signal processor and control subsystem are analyzed. The development of the OARE software is discussed. The experimental equipment will be enclosed in a cover assembly that will be mounted in the Orbiter close to the center of gravity.

  3. Skin lesions in returning travellers.

    PubMed

    Korzeniewski, Krzysztof; Juszczak, Dariusz; Jerzemowski, Janusz

    2015-01-01

    Skin lesions, apart from diarrhoeas, fever of unknown origin, and respiratory tract infections belong to the most frequent medical problems in travellers returned from tropical and subtropical destinations, accounting more than 10% of reported cases. Most dermatoses have their clinical onset during travel, although some of them can occur after return. Travel-related dermatological problems can have a wide spectrum of clinical picture, from macular, popular or nodular rash, linear and migratory lesions, to plaques, vesicles, bullae, erosions or ulcers. Skin conditions in returning travellers may be of infectious and non-infectious aetiologies. Infectious lesions may be originally tropical (e.g. dengue, chikungunya, schistosomiasis, leishmaniasis, myiasis, tungiasis, loiasis), although the majority are cosmopolitan (arthropod bites, sunburns, allergic rashes). The evaluation of skin lesions depends on many factors, including immune status of patients, use of medicines, exposure on health hazards (fauna, flora, risky behaviours), as well as the time, duration and location of travel. As the number of travellers to tropical and subtropical destinations has been continuously rising, the number of skin illnesses has also been increasing. This means that specialists in travel medicine need to extend their knowledge of epidemiology, clinical features and diagnosis of travel-related health problems including skin lesions in returning travellers. PMID:26394319

  4. Skin lesions in returning travellers.

    PubMed

    Korzeniewski, Krzysztof; Juszczak, Dariusz; Jerzemowski, Janusz

    2015-01-01

    Skin lesions, apart from diarrhoeas, fever of unknown origin, and respiratory tract infections belong to the most frequent medical problems in travellers returned from tropical and subtropical destinations, accounting more than 10% of reported cases. Most dermatoses have their clinical onset during travel, although some of them can occur after return. Travel-related dermatological problems can have a wide spectrum of clinical picture, from macular, popular or nodular rash, linear and migratory lesions, to plaques, vesicles, bullae, erosions or ulcers. Skin conditions in returning travellers may be of infectious and non-infectious aetiologies. Infectious lesions may be originally tropical (e.g. dengue, chikungunya, schistosomiasis, leishmaniasis, myiasis, tungiasis, loiasis), although the majority are cosmopolitan (arthropod bites, sunburns, allergic rashes). The evaluation of skin lesions depends on many factors, including immune status of patients, use of medicines, exposure on health hazards (fauna, flora, risky behaviours), as well as the time, duration and location of travel. As the number of travellers to tropical and subtropical destinations has been continuously rising, the number of skin illnesses has also been increasing. This means that specialists in travel medicine need to extend their knowledge of epidemiology, clinical features and diagnosis of travel-related health problems including skin lesions in returning travellers.

  5. Orbitals and orbital energies in DFT and TDDFT

    NASA Astrophysics Data System (ADS)

    Baerends, Evert Jan

    The status and meaning of orbitals and orbital energies in the Kohn-Sham one-electron model of DFT has been controversial, in contrast to Hartree-Fock orbitals and orbital energies. We will argue the opposite: the exact Kohn-Sham orbitals of DFT are ''better'' than HF orbitals and their orbital energies are much closer to ionization energies than HF orbital energies are. This follows from the relation between the KS potential and the wavefunction, which can be cast in the form vs =vc , kin +vH +vxchole +vresp, where each term depends on the KS orbitals and the wavefunction (the one- or two-particle density matrices). The response potential vresp (r) = ∑ j ∞|/dj(r) | 2 ρ (r) Ij - ∑ i H|/ψs , i(r) | 2 ρ (r) (-ɛi) (dj is the Dyson orbital corresponding to ion state ΨjN - 1 , ψs , i is a Kohn-Sham orbital) enables the connection between ionization energies Ii and orbital energies ɛi to be made. For virtual orbitals and orbital energies similar statements can be made: the shapes and energies of the (exact) KS orbitals are much more realistic than those of the Hartree-Fock model or hybrid functionals. The HOMO-LUMO gap in molecules is very close to the optical gap, and very different from the fundamental gap. In solids the situation is very different, which is the well-known ''KS gap problem''. Again the response potential vresp (a good approximation to it) helps to solve this problem, affording a straigtforward correction method of the KS gap to the fundamental gap.

  6. Multifocal fibrosclerosis associated with suprasellar and macular lesions.

    PubMed Central

    Brazier, D J; Sanders, M D

    1983-01-01

    Multifocal fibrosclerosis is a term used to denote a combination of similar fibrous disorders occurring at different anatomical sites and including idiopathic mediastinal and retroperitoneal fibrosis, sclerosing cholangitis, Riedel's thyroiditis, and orbital pseudotumour. This paper reports a patient, known to have retroperitoneal and testicular fibrosis, who suffered visual loss resulting from marked changes in the macular region of one eye and suprasellar extension of a mass in the pituitary fossa. These lesions appear to represent very uncommon manifestations of multifocal fibrosclerosis. Images PMID:6838800

  7. Adult orbital trapdoor fracture.

    PubMed

    Kum, Clarissa; McCulley, Timothy J; Yoon, Michael K; Hwang, Thomas N

    2009-01-01

    Trapdoor fractures occur almost exclusively in the pediatric population. The authors describe an adult with an entrapped inferior rectus muscle sheath in a trapdoor fracture. A 37-year-old man presented with persistent diplopia 3 weeks after blunt right orbital trauma. The only abnormal findings on clinical examination were limited vertical ductions. No bony defect or displacement was evident on CT. However, several small pockets of air were visible adjacent to the inferior rectus muscle. On surgical exploration, a linear nondisplaced orbital floor fracture was confirmed, and the entrapped inferior rectus muscle was released. One month postoperatively, extraocular motility had improved with no diplopia in primary or reading positions. This case demonstrates that trapdoor fractures can occur in adults and should be considered when suggestive findings are encountered. Clinicians should be aware of this because timely diagnosis and treatment might achieve more favorable outcomes.

  8. Mercury orbiter transport study

    NASA Technical Reports Server (NTRS)

    Friedlander, A. L.; Feingold, H.

    1977-01-01

    A data base and comparative performance analyses of alternative flight mode options for delivering a range of payload masses to Mercury orbit are provided. Launch opportunities over the period 1980-2000 are considered. Extensive data trades are developed for the ballistic flight mode option utilizing one or more swingbys of Venus. Advanced transport options studied include solar electric propulsion and solar sailing. Results show the significant performance tradeoffs among such key parameters as trip time, payload mass, propulsion system mass, orbit size, launch year sensitivity and relative cost-effectiveness. Handbook-type presentation formats, particularly in the case of ballistic mode data, provide planetary program planners with an easily used source of reference information essential in the preliminary steps of mission selection and planning.

  9. Constant attitude orbit transfer

    NASA Astrophysics Data System (ADS)

    Cress, Peter; Evans, Michael

    A two-impulse orbital transfer technique is described in which the spacecraft attitude remains constant for both burns, eliminating the need for attitude maneuvers between the burns. This can lead to significant savings in vehicle weight, cost and complexity. Analysis is provided for a restricted class of applications of this transfer between circular orbits. For those transfers with a plane change less than 30 deg, the total velocity cost of the maneuver is less than twelve percent greater than that of an optimum plane split Hohmann transfer. While this maneuver does not minimize velocity requirement, it does provide a means of achieving necessary transfer while substantially reducing the cost and complexity of the spacecraft.

  10. Small Mercury Relativity Orbiter

    NASA Technical Reports Server (NTRS)

    Bender, Peter L.; Vincent, Mark A.

    1989-01-01

    The accuracy of solar system tests of gravitational theory could be very much improved by range and Doppler measurements to a Small Mercury Relativity Orbiter. A nearly circular orbit at roughly 2400 km altitude is assumed in order to minimize problems with orbit determination and thermal radiation from the surface. The spacecraft is spin-stabilized and has a 30 cm diameter de-spun antenna. With K-band and X-band ranging systems using a 50 MHz offset sidetone at K-band, a range accuracy of 3 cm appears to be realistically achievable. The estimated spacecraft mass is 50 kg. A consider-covariance analysis was performed to determine how well the Earth-Mercury distance as a function of time could be determined with such a Relativity Orbiter. The minimum data set is assumed to be 40 independent 8-hour arcs of tracking data at selected times during a two year period. The gravity field of Mercury up through degree and order 10 is solved for, along with the initial conditions for each arc and the Earth-Mercury distance at the center of each arc. The considered parameters include the gravity field parameters of degree 11 and 12 plus the tracking station coordinates, the tropospheric delay, and two parameters in a crude radiation pressure model. The conclusion is that the Earth-Mercury distance can be determined to 6 cm accuracy or better. From a modified worst-case analysis, this would lead to roughly 2 orders of magnitude improvement in the knowledge of the precession of perihelion, the relativistic time delay, and the possible change in the gravitational constant with time.

  11. Spatial orbital tether constructions

    NASA Astrophysics Data System (ADS)

    Kogan, A. Yu.

    2016-09-01

    This paper is concerned with the problem of shape-retaining spatial tether configurations in a circular Keplerian orbit. Sufficient conditions of shape retention are described, which are imposed on the geometry of the structure, distribution of mass in the nodes, and parameters of rotation. The paper also mentions classes of structures with different properties of symmetry and motion, as well as specific examples of shaperetaining structures.

  12. 'Spider' in Earth Orbit

    NASA Technical Reports Server (NTRS)

    1969-01-01

    View of the Apollo 9 Lunar Module 'Spider' in a lunar landing configuration photographed by Command Module pilot David Scott inside the Command/Service Module 'Gumdrop' on the fifth day of the Apollo 9 earth-orbital mission. The landing gear on 'Spider' has been deployed. lunar surface probes (sensors) extend out from the landing gear foot pads. Inside the 'Spider' were astronauts James A. McDivitt, Apollo 9 Commander; and Russell L. Schweickart, Lunar Module pilot.

  13. Spectrophotovoltaic orbital power generation

    NASA Technical Reports Server (NTRS)

    Onffroy, J. R.

    1980-01-01

    The feasibilty of a spectrophotovoltaic orbital power generation system that optically concentrates solar energy is demonstrated. A dichroic beam-splitting mirror is used to divide the solar spectrum into two wavebands. Absorption of these wavebands by GaAs and Si solar cell arrays with matched energy bandgaps increases the cell efficiency while decreasing the amount of heat that must be rejected. The projected cost per peak watt if this system is $2.50/W sub p.

  14. Circular-Orbit Maintenance Strategies for Primitive Body Orbiters

    NASA Technical Reports Server (NTRS)

    Wallace, Mark S.; Broschart, Stephen

    2013-01-01

    For missions to smaller primitive bodies, solar radiation pressure (SRP) is a significant perturbation to Keplerian dynamics. For most orbits, SRP drives large oscillations in orbit eccentricity, which leads to large perturbations from the irregular gravity field at periapsis. Ultimately, chaotic motion results that often escapes or impacts that body. This paper presents an orbit maintenance strategy to keep the orbit eccentricity small, thus avoiding the destabilizing secondary interaction with the gravity field. An estimate of the frequency and magnitude of the required maneuvers as a function of the orbit and body parameters is derived from the analytic perturbation equations.

  15. An Orbit Plan toward AKATSUKI Venus Reencounter and Orbit Injection

    NASA Technical Reports Server (NTRS)

    Kawakatsu, Yasuhiro; Campagnola, Stefano; Hirose, Chikako; Ishii, Nobuaki

    2012-01-01

    On December 7, 2010, AKATSUKI, the Japanese Venus explorer reached its destination and tried to inject itself into Venus orbit. However, due to a malfunction of the propulsion system, the maneuver was interrupted and AKATSUKI again escaped out from the Venus into an interplanetary orbit. Telemetry data from AKATSUKI suggests the possibility to perform orbit maneuvers to reencounter the Venus and retry Venus orbit injection. Reported in this paper is an orbit plan investigated under this situation. The latest results reflecting the maneuvers conducted in the autumn 2011 is introduced as well.

  16. Radiation Propulsion For Maintaining Orbits

    NASA Technical Reports Server (NTRS)

    Richter, Robert

    1995-01-01

    Brief report proposes radiative propulsion systems for maintaining precise orbits of spacecraft. Radiation from electrical heaters directed outward by paraboloidal reflectors to produce small forces to oppose uncontrolled drag and solar-radiative forces perturbing orbits. Minimizes or eliminates need to fire rocket thrusters to correct orbits.

  17. Orbiter KU-band transmitter

    NASA Technical Reports Server (NTRS)

    Halterman, R.

    1976-01-01

    The design, build, and test of an engineering breadboard Ku band quadraphase shift keyed and wideband frequency modulated transmitter are described. This orbiter Ku band transmitter drawer is to simulate the orbiter transmitter and meet the functional requirements of the orbiter communication link.

  18. What is a MISR orbit?

    Atmospheric Science Data Center

    2014-12-08

    ... platform that carries MISR and other scientific instruments flies at an altitude of 705 km above sea level on a sun-synchronous orbit. It ... completes an orbit and initiates the next one, it actually flies over quite different regions. The orbit number thus also indicates the ...

  19. Orbiter Autoland reliability analysis

    NASA Technical Reports Server (NTRS)

    Welch, D. Phillip

    1993-01-01

    The Space Shuttle Orbiter is the only space reentry vehicle in which the crew is seated upright. This position presents some physiological effects requiring countermeasures to prevent a crewmember from becoming incapacitated. This also introduces a potential need for automated vehicle landing capability. Autoland is a primary procedure that was identified as a requirement for landing following and extended duration orbiter mission. This report documents the results of the reliability analysis performed on the hardware required for an automated landing. A reliability block diagram was used to evaluate system reliability. The analysis considers the manual and automated landing modes currently available on the Orbiter. (Autoland is presently a backup system only.) Results of this study indicate a +/- 36 percent probability of successfully extending a nominal mission to 30 days. Enough variations were evaluated to verify that the reliability could be altered with missions planning and procedures. If the crew is modeled as being fully capable after 30 days, the probability of a successful manual landing is comparable to that of Autoland because much of the hardware is used for both manual and automated landing modes. The analysis indicates that the reliability for the manual mode is limited by the hardware and depends greatly on crew capability. Crew capability for a successful landing after 30 days has not been determined yet.

  20. Oral Lesions and Lymphoproliferative Disorders

    PubMed Central

    Castellarin, P.; Pozzato, G.; Tirelli, G.; Di Lenarda, R.; Biasotto, M.

    2010-01-01

    Lymphoproliferative disorders are heterogeneous malignancy characterized by the expansion of a lymphoid clone more or less differentiated. At the level of the oral cavity, the lymphoproliferative disorder can occur in various ways, most commonly as lymphoid lesions with extranodal externalization, but sometimes, oral lesions may represent a localization of a disease spread. With regard to the primary localizations of lymphoproliferative disorders, a careful examination of the head and neck, oral, and oropharyngeal area is necessary in order to identify suspicious lesions, and their early detection results in a better prognosis for the patient. Numerous complications have been described and frequently found at oral level, due to pathology or different therapeutic strategies. These complications require precise diagnosis and measures to oral health care. In all this, oral pathologists, as well as dental practitioners, have a central role in the treatment and long-term monitoring of these patients. PMID:20871659

  1. Benign lymphoepithelial lesion and malignancy.

    PubMed

    Hordijk, G J; Meyer, C J

    1981-01-01

    Whereas most patients with benign lymphoepithelial lesion suffer from the involvement of a major salivary gland a number evolve into a clinical form of Sjögren's syndrome or Mikulicz's disease. In a small number development of malignant lymphomas, especially non-Hodgkin's lymphomas, have been described. Therefore, regular follow-up and appropriate histological evaluation of suspected areas in all patients with a benign lymphoepithelial lesion is indicated. Histologically, diagnosis of a non-Hodgkin's lymphoma may be difficult. Demonstration of a cell pattern, monoclonal for cytoplasmic Ig by means of the immunoperoxidase technique may facilitate the diagnosis. In this report we present the history of two cases out of our series with benign lymphoepithelial lesion who developed a non-Hodgkin's lymphoma.

  2. Sino-orbital osteoma with osteoblastoma-like features: case reports.

    PubMed

    Yazici, Zeynep; Yazici, Bulent; Yalcinkaya, Ulviye; Gokalp, Gokhan

    2012-07-01

    Most of the orbital osteomas arise from the adjacent paranasal sinuses. Some of them may contain osteoblastoma-like areas and may be misdiagnosed as osteoblastoma, both radiologically as well as histopathologically. Sino-orbital osteomas with osteoblastoma-like features show a typical radiological appearance. They have a distinct zonal pattern, in which less dense osteoblastoma-like areas are located at the base of the lesion and dense mature bone is located at the periphery. These broad-based bone lesions also have a tendency for extracavitary polypoid growths from the paranasal sinus into the adjacent orbit. We report here the CT and MR imaging findings of three cases with sino-orbital osteoma with osteoblastoma-like features.

  3. Frozen Orbital Plane Solutions for Satellites in Nearly Circular Orbit

    NASA Astrophysics Data System (ADS)

    Ulivieri, Carlo; Circi, Christian; Ortore, Emiliano; Bunkheila, Federico; Todino, Francesco

    2013-08-01

    This paper deals with the determination of the initial conditions (right ascension of the ascending node and inclination) that minimize the orbital plane variation for nearly circular orbits with a semimajor axis between 3 and 10 Earth radii. An analysis of two-line elements over the last 40 years for mid-, geostationary-, and high-Earth orbits has shown, for initially quasi-circular orbits, low eccentricity variations up to the geostationary altitude. This result makes the application of mathematical models based on satellite circular orbits advantageous for a fast prediction of long-term temporal evolution of the orbital plane. To this purpose, a previous model considering the combined effect due to the Earth's oblateness, moon, and sun (both in circular orbit) has been improved in terms of required computational time and accuracy. The eccentricity of the sun and moon and the equinoctial precession have been taken into account. Resonance phenomena with the lunar plane motion have been found in mid-Earth orbit. Dynamical properties concerning the precession motions of the orbital pole have been investigated, and frozen solutions for geosynchronous and navigation satellites have been proposed. Finally, an accurate model validation has also been carried out by comparing the obtained results with two-line elements of abandoned geostationary-Earth orbit and mid-Earth orbit satellites.

  4. Renal lesions of nondomestic felids.

    PubMed

    Newkirk, K M; Newman, S J; White, L A; Rohrbach, B W; Ramsay, E C

    2011-05-01

    To comprehensively evaluate the occurrence of renal lesions in a variety of nondomestic felids, necropsy cases from 1978 to 2008 were reviewed from a municipal zoo and a large cat sanctuary for those in which the kidneys were examined histologically. Seventy exotic felids were identified (25 tigers, 18 lions, 6 cougars, 5 leopards, 3 snow leopards, 3 clouded leopards, 3 Canadian lynx, 2 ocelots, 2 bobcats, 2 cheetahs, 1 jaguar), and their histologic renal lesions were evaluated and compared. The most common lesion was tubulointerstitial nephritis (TIN); 36 of 70 (51%) cats were affected to some degree. Lymphocytic interstitial nephritis was the most common lesion in the tigers (9 of 25, 36%) and was rarely seen in other species. Although the renal pelvis was not available for all cats, 28 of 47 (60%) had some degree of lymphocytic pyelitis. There was no significant association between the presence of pyelitis and that of TIN. Only 1 cat had pyelonephritis. Renal papillary necrosis was present in 13 of 70 (19%) cats and was significantly associated with historical nonsteroidal anti-inflammatory drug treatment (odds ratio, 7.1; 95% confidence interval, 1.9 to 26.8). Only 1 cat (lion) had amyloid accumulation, and it was restricted to the corticomedullary junction. Primary glomerular lesions were absent in all cats. Intraepithelial pigment was identified in many of the cats but was not correlated with severity of TIN. Despite several previous reports describing primary glomerular disease or renal amyloidosis in exotic felids, these lesions were rare to absent in this population. PMID:20876911

  5. Intensified and prolonged therapy comprising cytarabine, vincristine and prednisolone improves outcome in patients with multisystem Langerhans cell histiocytosis: results of the Japan Langerhans Cell Histiocytosis Study Group-02 Protocol Study.

    PubMed

    Morimoto, Akira; Shioda, Yoko; Imamura, Toshihiko; Kudo, Kazuko; Kawaguchi, Hiroshi; Sakashita, Kazuo; Yasui, Masahiro; Koga, Yuhki; Kobayashi, Ryoji; Ishii, Eiichi; Fujimoto, Junichiro; Horibe, Keizo; Bessho, Fumio; Tsunematsu, Yukiko; Imashuku, Shinsaku

    2016-07-01

    The JLSG-96 study reported very low mortality rates for children newly diagnosed with multifocal Langerhans cell histiocytosis (LCH). The JLSG-02 study was performed to further improve the prognosis from 2002 to 2009. The present study compared the therapeutic results of these two studies in terms of multisystem disease. All patients were treated with 6 weeks of the Induction A regimen, comprising cytarabine, vincristine and prednisolone, followed by maintenance therapy. Poor responders to Induction A were switched to Induction B. JLSG-02 has been revised from JLSG-96 in the following respects: prednisolone dosage during Induction A increased; duration of maintenance therapy extended from 24 to 48 weeks; cyclosporine introduced to Induction B for progressive disease. One hundred forty-seven children with multisystem LCH were evaluated. Of these, 84 were positive for risk of organ involvement (RO) and 63 were RO-negative. At the 6-week point, 76.2 % of RO+ and 93.7 % of RO- patients responded to Induction A. Five-year event-free survival (EFS) was 46.2 % [95 % confidence (CI), 35.5-56.9] for RO+ and 69.7 % (58.4-81.1) for RO-, which was significantly superior to that in JLSG-96 [26.8 % (13.3-40.4) and 38.9 % (16.4-61.4), respectively]. The intensified induction and prolonged maintenance regimens in JLSG-02 improved EFS in patients with multisystem LCH. PMID:27040279

  6. Intensified and prolonged therapy comprising cytarabine, vincristine and prednisolone improves outcome in patients with multisystem Langerhans cell histiocytosis: results of the Japan Langerhans Cell Histiocytosis Study Group-02 Protocol Study.

    PubMed

    Morimoto, Akira; Shioda, Yoko; Imamura, Toshihiko; Kudo, Kazuko; Kawaguchi, Hiroshi; Sakashita, Kazuo; Yasui, Masahiro; Koga, Yuhki; Kobayashi, Ryoji; Ishii, Eiichi; Fujimoto, Junichiro; Horibe, Keizo; Bessho, Fumio; Tsunematsu, Yukiko; Imashuku, Shinsaku

    2016-07-01

    The JLSG-96 study reported very low mortality rates for children newly diagnosed with multifocal Langerhans cell histiocytosis (LCH). The JLSG-02 study was performed to further improve the prognosis from 2002 to 2009. The present study compared the therapeutic results of these two studies in terms of multisystem disease. All patients were treated with 6 weeks of the Induction A regimen, comprising cytarabine, vincristine and prednisolone, followed by maintenance therapy. Poor responders to Induction A were switched to Induction B. JLSG-02 has been revised from JLSG-96 in the following respects: prednisolone dosage during Induction A increased; duration of maintenance therapy extended from 24 to 48 weeks; cyclosporine introduced to Induction B for progressive disease. One hundred forty-seven children with multisystem LCH were evaluated. Of these, 84 were positive for risk of organ involvement (RO) and 63 were RO-negative. At the 6-week point, 76.2 % of RO+ and 93.7 % of RO- patients responded to Induction A. Five-year event-free survival (EFS) was 46.2 % [95 % confidence (CI), 35.5-56.9] for RO+ and 69.7 % (58.4-81.1) for RO-, which was significantly superior to that in JLSG-96 [26.8 % (13.3-40.4) and 38.9 % (16.4-61.4), respectively]. The intensified induction and prolonged maintenance regimens in JLSG-02 improved EFS in patients with multisystem LCH.

  7. Can Small Lesions Induce Language Reorganization as Large Lesions Do?

    ERIC Educational Resources Information Center

    Maestu, Fernando; Saldana, Cristobal; Amo, Carlos; Gonzalez-Hidalgo, Mercedes; Fernandez, Alberto; Fernandez, Santiago; Mata, Pedro; Papanicolaou, Andrew; Ortiz, Tomas

    2004-01-01

    Shift of the cortical mechanisms of language from the usually dominant left to the non-dominant right hemisphere has been demonstrated in the presence of large brain lesions. Here, we report a similar phenomenon in a patient with a cavernoma over the anterolateral superior temporal gyrus associated with epilepsy. Language mapping was performed by…

  8. Painful ophthalmoplegia secondary to a mucocele involving the sella turcica, superior orbital fissure, and sphenoid sinus.

    PubMed

    Clarke, H; Clarke, V; Gill, J; St John, A; Lashley, M

    1992-03-01

    A case of painful ophthalmoplegia associated with an extensive lesion involving the sella turcica, superior orbital fissure, and sphenoid sinus in a 57-year-old man is reported. Even though nasal and ocular symptoms and signs represent the usual features of sphenoidal mucoceles, extension to the intracranial cavity as seen in this lesion is rare. Surgical exploration via a sublabial, transseptal approach revealed a mucocele of the sphenoid sinus. This case exhibited extensive and aggressive behavior simulating a malignant neoplasm.

  9. Cutaneous lesions of the nose

    PubMed Central

    2010-01-01

    Skin diseases on the nose are seen in a variety of medical disciplines. Dermatologists, otorhinolaryngologists, general practitioners and general plastic and dermatologic surgeons are regularly consulted regarding cutaneous lesions on the nose. This article is the second part of a review series dealing with cutaneous lesions on the head and face, which are frequently seen in daily practice by a dermatologic surgeon. In this review, we focus on those skin diseases on the nose where surgery or laser therapy is considered a possible treatment option or that can be surgically evaluated. PMID:20525327

  10. Apraxia in deep cerebral lesions.

    PubMed Central

    Agostoni, E; Coletti, A; Orlando, G; Tredici, G

    1983-01-01

    In a series of 50 patients with cerebrovascular lesions (demonstrated with CT scan), seven patients had lesions located in the basal ganglia and/or thalamus. All these seven patients were apractic. Ideomotor apraxia was present in all patients; five also had constructional apraxia, and one had bucco-facial apraxia. None of the patients had utilisation apraxia. These observations indicated that apraxia is not only a "high cerebral (cortical) function", but may depend also on the integrity of subcortical circuits and structures. PMID:6619888

  11. Pediatric Knee Osteochondritis Dissecans Lesions.

    PubMed

    Cruz, Aristides I; Shea, Kevin G; Ganley, Theodore J

    2016-10-01

    Osteochondritis dissecans (OCD) can cause knee pain and dysfunction in children. The etiology of OCD remains unclear; theories on causes include inflammation, ischemia, ossification abnormalities, genetic factors, and repetitive microtrauma. Most OCD lesions in skeletally immature patients will heal with nonoperative treatment. The success of nonoperative treatment decreases once patients reach skeletal maturity. The goals of surgical treatment include maintenance of articular cartilage congruity, rigid fixation of unstable fragments, and repair of osteochondral defects with cells or tissues that can adequately replace lost or deficient cartilage. Unsalvageable OCD lesions can be treated with various surgical techniques. PMID:27637663

  12. Bilateral orbital metastases from breast cancer: a case report of successful palliation using stereotactic radiotherapy.

    PubMed

    Kim, Jin Ho; Choi, Sang Yul; Cho, Chul Koo; Yang, Kwang Mo; Noh, Woo Chul; Kim, Mi-Sook

    2011-01-01

    Of ophthalmic involvement from metastatic breast cancer, extraocular/intraorbital metastases are extremely rare. External beam radiotherapy has been a mainstay palliation for symptomatic orbital metastases. We present a case of bilateral orbital metastases from breast cancer successfully treated with stereotactic radiotherapy (SRT). A 38-year-old woman presented with decreased vision in the right eye for 3 weeks. Eight months previously, she underwent whole-brain radiotherapy for multiple brain metastases from breast cancer. Visual acuity was hand motion, and the eyelid closed incompletely in the affected eye. Computed tomography scans showed a 3-cm extraconal mass in the right orbit. She underwent temporary tarsorrhaphy followed by SRT. A total dose of 39 Gy was delivered to the right orbital mass in three daily fractions. Four months later, her visual function was normal in both eyes and the right orbital mass disappeared. A new lesion was detected in the left orbit. She underwent SRT for the left orbital lesion using the same dose-fractionation schedule. No radiation-related toxicities were observed. She died 19 months after the first SRT. Our case suggests that SRT may be an effective and safe treatment option in patients with orbital metastases from breast cancer. PMID:21999613

  13. Global Orbit Feedback in RHIC

    SciTech Connect

    Minty, M.; Hulsart, R.; Marusic, A.; Michnoff, R.; Ptitsyn, V.; Robert-Demolaize, G.; Satogata, T.

    2010-05-23

    For improved reproducibility of good operating conditions and ramp commissioning efficiency, new dual-plane slow orbit feedback during the energy ramp was implemented during run-10 in the Relativistic Heavy Ion Collider (RHIC). The orbit feedback is based on steering the measured orbit, after subtraction of the dispersive component, to either a design orbit or to a previously saved reference orbit. Using multiple correctors and beam position monitors, an SVD-based algorithm is used for determination of the applied corrections. The online model is used as a basis for matrix computations. In this report we describe the feedback design, review the changes made to realize its implementation, and assess system performance.

  14. Orbital maneuvers and space rendezvous

    NASA Astrophysics Data System (ADS)

    Butikov, Eugene I.

    2015-12-01

    Several possibilities of launching a space vehicle from the orbital station are considered and compared. Orbital maneuvers discussed in the paper can be useful in designing a trajectory for a specific space mission. The relative motion of orbiting bodies is investigated on examples of spacecraft rendezvous with the space station that stays in a circular orbit around the Earth. An elementary approach is illustrated by an accompanying simulation computer program and supported by a mathematical treatment based on fundamental laws of physics and conservation laws. Material is appropriate for engineers and other personnel involved in space exploration, undergraduate and graduate students studying classical physics and orbital mechanics.

  15. Lunar Prospector Orbit Determination Results

    NASA Technical Reports Server (NTRS)

    Beckman, Mark; Concha, Marco

    1998-01-01

    The orbit support for Lunar Prospector (LP) consists of three main areas: (1) cislunar orbit determination, (2) rapid maneuver assessment using Doppler residuals, and (3) routine mapping orbit determination. The cislunar phase consisted of two trajectory correction maneuvers during the translunar cruise followed by three lunar orbit insertion burns. This paper will detail the cislunar orbit determination accuracy and the real-time assessment of the cislunar trajectory correction and lunar orbit insertion maneuvers. The non-spherical gravity model of the Moon is the primary influence on the mapping orbit determination accuracy. During the first two months of the mission, the GLGM-2 lunar potential model was used. After one month in the mapping orbit, a new potential model was developed that incorporated LP Doppler data. This paper will compare and contrast the mapping orbit determination accuracy using these two models. LP orbit support also includes a new enhancement - a web page to disseminate all definitive and predictive trajectory and mission planning information. The web site provides definitive mapping orbit ephemerides including moon latitude and longitude, and four week predictive products including: ephemeris, moon latitude/longitude, earth shadow, moon shadow, and ground station view periods. This paper will discuss the specifics of this web site.

  16. Imaging inflammatory acne: lesion detection and tracking

    NASA Astrophysics Data System (ADS)

    Cula, Gabriela O.; Bargo, Paulo R.; Kollias, Nikiforos

    2010-02-01

    It is known that effectiveness of acne treatment increases when the lesions are detected earlier, before they could progress into mature wound-like lesions, which lead to scarring and discoloration. However, little is known about the evolution of acne from early signs until after the lesion heals. In this work we computationally characterize the evolution of inflammatory acne lesions, based on analyzing cross-polarized images that document acne-prone facial skin over time. Taking skin images over time, and being able to follow skin features in these images present serious challenges, due to change in the appearance of skin, difficulty in repositioning the subject, involuntary movement such as breathing. A computational technique for automatic detection of lesions by separating the background normal skin from the acne lesions, based on fitting Gaussian distributions to the intensity histograms, is presented. In order to track and quantify the evolution of lesions, in terms of the degree of progress or regress, we designed a study to capture facial skin images from an acne-prone young individual, followed over the course of 3 different time points. Based on the behavior of the lesions between two consecutive time points, the automatically detected lesions are classified in four categories: new lesions, resolved lesions (i.e. lesions that disappear completely), lesions that are progressing, and lesions that are regressing (i.e. lesions in the process of healing). The classification our methods achieve correlates well with visual inspection of a trained human grader.

  17. Orbital Debris: A Policy Perspective

    NASA Technical Reports Server (NTRS)

    Johnson, Nicholas L.

    2007-01-01

    A viewgraph presentation describing orbital debris from a policy perspective is shown. The contents include: 1) Voyage through near-Earth Space-animation; 2) What is Orbital Debris?; 3) Orbital Debris Detectors and Damage Potential; 4) Hubble Space Telescope; 5) Mir Space Station Solar Array; 6) International Space Station; 7) Space Shuttle; 8) Satellite Explosions; 9) Satellite Collisions; 10) NASA Orbital Debris Mitigation Guidelines; 11) International Space Station Jettison Policy; 12) Controlled/Uncontrolled Satellite Reentries; 13) Return of Space Objects; 14) Orbital Debris and U.S. National Space Policy; 15) U.S Government Policy Strategy; 16) Bankruptcy of the Iridium Satellite System; 17) Inter-Agency Space Debris Coordination Committee (IADC); 18) Orbital Debris at the United Nations; 19) Chinese Anti-satellite System; 20) Future Evolution of Satellite Population; and 21) Challenge of Orbital Debris

  18. Pseudo-Peritoneal Carcinomatosis Presentation of a Crystal-Storing Histiocytosis With an Unmutated Monoclonal κ Light Chain

    PubMed Central

    Aline-Fardin, Aude; Bender, Sebastien; Fabiani, Bettina; Buob, David; Brahimi, Said; Verpont, Marie Christine; Mothy, Mohamad; Ronco, Pierre; Boffa, Jean Jacques; Aucouturier, Pierre; Garderet, Laurent

    2015-01-01

    Abstract Crystal-storing histiocytosis (CSH) is a rare complication of monoclonal gammopathies caused by accumulation of crystalline material inside macrophages, and it may result in a variety of clinical manifestations depending on the involved organs. Although immunoglobulin κ light chains (LCs) seem to be the most frequent pathogenic component, very few molecular data are currently available. A 69-year-old man presented with a very poor performance status. Remarkable features were mesenteric lymph node enlargement and proteinuria, including a monoclonal κ LC. Light and electron microscopy studies revealed the presence of crystals within macrophages in the lymph nodes, bone marrow, and kidney, leading to the diagnosis of CSH. The pathogenic κ LC variable domain sequence was identical to the germline Vk3-20∗01/Jk2∗01 gene segments, without any somatic mutation, suggesting an extra-follicular B cell proliferation. The patient was successfully treated with 4 cycles of bortezomib and dexamethasone. After a 12-month follow-up, he remains in hematological and renal remission. CSH may present as pseudo-peritoneal carcinomatosis and relate to a monoclonal κ LC encoded by an unmutated gene. Bortezomib-based therapy proved efficacious in this case. PMID:26266355

  19. Pseudo-Peritoneal Carcinomatosis Presentation of a Crystal-Storing Histiocytosis With an Unmutated Monoclonal κ Light Chain.

    PubMed

    Aline-Fardin, Aude; Bender, Sebastien; Fabiani, Bettina; Buob, David; Brahimi, Said; Verpont, Marie Christine; Mothy, Mohamad; Ronco, Pierre; Boffa, Jean Jacques; Aucouturier, Pierre; Garderet, Laurent

    2015-08-01

    Crystal-storing histiocytosis (CSH) is a rare complication of monoclonal gammopathies caused by accumulation of crystalline material inside macrophages, and it may result in a variety of clinical manifestations depending on the involved organs. Although immunoglobulin κ light chains (LCs) seem to be the most frequent pathogenic component, very few molecular data are currently available.A 69-year-old man presented with a very poor performance status. Remarkable features were mesenteric lymph node enlargement and proteinuria, including a monoclonal κ LC. Light and electron microscopy studies revealed the presence of crystals within macrophages in the lymph nodes, bone marrow, and kidney, leading to the diagnosis of CSH. The pathogenic κ LC variable domain sequence was identical to the germline Vk3-2001/Jk201 gene segments, without any somatic mutation, suggesting an extra-follicular B cell proliferation.The patient was successfully treated with 4 cycles of bortezomib and dexamethasone. After a 12-month follow-up, he remains in hematological and renal remission.CSH may present as pseudo-peritoneal carcinomatosis and relate to a monoclonal κ LC encoded by an unmutated gene. Bortezomib-based therapy proved efficacious in this case.

  20. Management of adult patients with Langerhans cell histiocytosis: recommendations from an expert panel on behalf of Euro-Histio-Net.

    PubMed

    Girschikofsky, Michael; Arico, Maurizio; Castillo, Diego; Chu, Anthony; Doberauer, Claus; Fichter, Joachim; Haroche, Julien; Kaltsas, Gregory A; Makras, Polyzois; Marzano, Angelo V; de Menthon, Mathilde; Micke, Oliver; Passoni, Emanuela; Seegenschmiedt, Heinrich M; Tazi, Abdellatif; McClain, Kenneth L

    2013-01-01

    Langerhans Cell Histiocytosis (LCH) is an orphan disease of clonal dendritic cells which may affect any organ of the body. Most of the knowledge about the diagnosis and therapy is based on pedriatic studies. Adult LCH patients are often evaluated by physicians who focus on only the most obviously affected organ without sufficient evaluation of other systems, resulting in patients being underdiagnosed and/or incompletely staged. Furthermore they may be treated with pediatric-based therapies which are less effective and sometimes more toxic for adults. The published literature on adult LCH cases lacks a comprehensive discussion on the differences between pediatric and adult patients and there are no recommendations for evaluation and comparative therapies. In order to fill this void, a number of experts in this field cooperated to develop the first recommendations for management of adult patients with LCH. Key questions were selected according to the clinical relevance focusing on diagnostic work up, therapy, and follow up. Based on the available literature up to December 2012, recommendations were established, drafts were commented by the entire group, and redrafted by the executive editor. The quality of evidence of the recommendations is predominantly attributed to the level of expert opinion. Final agreement was by consensus. PMID:23672541

  1. Management of adult patients with Langerhans cell histiocytosis: recommendations from an expert panel on behalf of Euro-Histio-Net

    PubMed Central

    2013-01-01

    Langerhans Cell Histiocytosis (LCH) is an orphan disease of clonal dendritic cells which may affect any organ of the body. Most of the knowledge about the diagnosis and therapy is based on pedriatic studies. Adult LCH patients are often evaluated by physicians who focus on only the most obviously affected organ without sufficient evaluation of other systems, resulting in patients being underdiagnosed and/or incompletely staged. Furthermore they may be treated with pediatric-based therapies which are less effective and sometimes more toxic for adults. The published literature on adult LCH cases lacks a comprehensive discussion on the differences between pediatric and adult patients and there are no recommendations for evaluation and comparative therapies. In order to fill this void, a number of experts in this field cooperated to develop the first recommendations for management of adult patients with LCH. Key questions were selected according to the clinical relevance focusing on diagnostic work up, therapy, and follow up. Based on the available literature up to December 2012, recommendations were established, drafts were commented by the entire group, and redrafted by the executive editor. The quality of evidence of the recommendations is predominantly attributed to the level of expert opinion. Final agreement was by consensus. PMID:23672541

  2. Long-term improvement during tadalafil therapy in a patient with pulmonary hypertension secondary to pulmonary Langerhans cell histiocytosis.

    PubMed

    Nemoto, Kenji; Oh-Ishi, Shuji; Inui, Toshihide; Nakazawa, Mariko; Hyodo, Kentaro; Nakajima, Masayuki; Kanazawa, Jun; Miura, Yukiko; Takaku, Takio; Minami, Yuko; Hayashihara, Kenji; Saito, Takefumi; Kawabata, Yoshinori

    2016-01-01

    Pulmonary arterial hypertension (PAH) secondary to pulmonary Langerhans cell histiocytosis (PLCH) is known to be a relatively common complication and is associated with a poor prognosis. However, the optimal therapeutic approach for these cases remains to be established. A 57-year-old man visited our hospital because of a progressive dry cough. A thoracic computed tomography examination showed a combination of diffuse thick-walled cysts and reticulonodular shadows that were predominant in bilateral upper lobes of the lungs. He was diagnosed as having PLCH based on the results of video-assisted thoracoscopic lung biopsies. During a 3-year clinical course, his condition deteriorated despite smoking cessation. A systemic evaluation demonstrated precapillary PAH caused by PLCH (PAH-PLCH), and treatment with tadalafil, a phosphodiesterase-5 inhibitor, was started. During a 50-month period of treatment with tadalafil, improvements in his dyspnea, 6-min walking distance, and hemodynamics were maintained without either overt hypoxemia or pulmonary edema. We considered that tadalafil therapy may be a useful option in the treatment of patients with PAH-PLCH. PMID:27330952

  3. Coincident expression of the chemokine receptors CCR6 and CCR7 by pathologic Langerhans cells in Langerhans cell histiocytosis.

    PubMed

    Fleming, Mark D; Pinkus, Jack L; Fournier, Marcia V; Alexander, Sarah W; Tam, Carmen; Loda, Massimo; Sallan, Stephen E; Nichols, Kim E; Carpentieri, David F; Pinkus, Geraldine S; Rollins, Barrett J

    2003-04-01

    It has been suggested that a switch in chemokine receptor expression underlies Langerhans cell migration from skin to lymphoid tissue. Activated cells are thought to down-regulate CCR6, whose ligand macrophage inflammatory protein-3 alpha (MIP-3 alpha)/CCL20 is expressed in skin, and up-regulate CCR7, whose ligands are in lymphoid tissues. In Langerhans cell histiocytosis (LCH), pathologic Langerhans cells (LCs) accumulate in several tissues, including skin, bone, and lymphoid organs. We have examined 24 LCH cases and find that pathologic LCs expressed CCR6 and CCR7 coincidentally in all cases. Furthermore, MIP-3 alpha/CCL20 is expressed by keratinocytes in involved skin and by macrophages and osteoblasts in involved bone. Expression of CCR6 by pathologic LCs may contribute to their accumulation in nonlymphoid organs such as skin and bone, whereas CCR7 expression may direct them to lymphoid tissue. Histiocytes in Rosai-Dorfman disease and hemophagocytic syndrome also coexpressed CCR6 and CCR7, suggesting that this may be a general attribute of abnormal histiocytes.

  4. Outcome of pediatric patients with Langerhans cell histiocytosis treated with 2 chlorodeoxyadenosine: a nationwide survey in Japan.

    PubMed

    Imamura, Toshihiko; Sato, Takashi; Shiota, Yoko; Kanegane, Hirokazu; Kudo, Kazuko; Nakagawa, Shinichirou; Nakadate, Hisaya; Tauchi, Hisamichi; Kamizono, Junji; Morimoto, Akira

    2010-05-01

    The aim of this study was to assess the outcome of treatment with 2-chlorodeoxyadenosine (2-CdA) in pediatric patients with Langerhans cell histiocytosis (LCH) in Japan. We retrospectively identified 17 pediatric LCH patients treated with 2-CdA. All patients were refractory or reactivated cases who had been initially treated according to the JLSG-02 protocol of the Japan LCH study group. At initiation of 2-CdA therapy, 4 patients had primary refractory multisystem (MS) disease with risk organ (RO) involvement (MS+), 9 patients had reactivated MS disease [5 MS+ and 4 without RO involvement (MS-)], and the remaining 4 patients had refractory/reactivated multifocal bone disease (MFB). Treatment with 2-CdA (4-9 mg/m(2)/day) was administered on 2-5 consecutive days and repeated every 3-4 weeks for a period that ranged from 2 to 12 months. Four primary refractory patients were treated with 2-CdA combined with high dose of cytarabine. In MS+ patients, response to treatment was observed in 5 of the 9 patients. In MS-/MFB patients, 5 of the 8 patients showed response to treatment. In the patients who were primary refractory or had reactivation during initial chemotherapy, 4 of 10 patients showed good response. On the other hand, in the patients having reactivation while off therapy, 6 of 7 patients showed good response. These findings suggest that 2-CdA is effective for reactivated LCH while off therapy.

  5. Secondary Sea-Blue Histiocytosis in a Patient with Transfusion Dependent HbE-Beta Thalassaemia and Osteosarcoma.

    PubMed

    Saad Eldeen Bakheet, Omayma; Yusof, Nurasyikin; Raja Zahratul, Azma; Ithnin, Azlin; Abdul Aziz, Suria; Alias, Hamidah

    2016-06-01

    Secondary sea-blue histiocytosis occurs more frequently than the primary form and occurs consequent to a wide range of metabolic and haematologic disorders including thalassaemia. We report an 18-year-old Chinese boy with transfusion-dependent HbE-beta thalassaemia who complained of pain and swelling at the left iliac crest region for 2 months duration. Physical examination revealed pallor with hepatosplenomegaly. Local examination revealed a huge swelling 12 cm × 12 cm in diameter, firm in consistency and tender. Histopathological examination of the mass revealed an osteosarcoma. His bone marrow aspirate showed numerous sea-blue histiocytes, the cytoplasm of which was closely packed with fine granules that stained blue with May-Grunwald-Giemsa. The nuclei were centrally located in some cells and displaced towards the periphery in other cells. There was no malignant cell infiltration in the marrow. The case is reported due to the co-incidental dual pathology in our patient (HbE-beta thalassaemia and osteosarcoma) and the unusual bone marrow finding of numerous sea-blue histiocytes. PMID:27408409

  6. Cystic Lesions in Autoimmune Pancreatitis.

    PubMed

    Gompertz, Macarena; Morales, Claudia; Aldana, Hernán; Castillo, Jaime; Berger, Zoltán

    2015-01-01

    Autoimmune pancreatitis (AIP) can be chronic or recurrent, but frequently completely reversible after steroid treatment. A cystic lesion in AIP is a rare finding, and it can mimic a pancreatic cystic neoplasm. Difficulties in an exact diagnosis interfere with treatment, and surgery cannot be avoided in some cases. We report the history of a 63-year-old male presenting with jaundice and pruritus. AIP was confirmed by imaging and elevated IgG4 blood levels, and the patient completely recovered after corticosteroid therapy. One year later, he presented with a recurrent episode of AIP with elevated IgG4 levels, accompanied by the appearance of multiple intrapancreatic cystic lesions. All but 1 of these cysts disappeared after steroid treatment, but the remaining cyst in the pancreatic head was even somewhat larger 1 year later. Pancreatoduodenectomy was finally performed. Histology showed the wall of the cystic lesion to be fibrotic; the surrounding pancreatic tissue presented fibrosis, atrophy and lymphoplasmacytic infiltration by IgG4-positive cells, without malignant elements. Our case illustrates the rare possibility that cystic lesions can be part of AIP. These pseudocysts appear in the pancreatic segments involved in the autoimmune disease and can be a consequence of the local inflammation or related to ductal strictures. Steroid treatment should be initiated, after which these cysts can completely disappear with recovery from AIP. Surgical intervention may be necessary in some exceptional cases.

  7. Cystic lesions of the pancreas

    PubMed Central

    Karoumpalis, Ioannis; Christodoulou, Dimitrios K.

    2016-01-01

    Different types of benign or malignant cystic lesions can be observed in the pancreas. Pancreatic cystic lesions are classified under pathology terms into simple retention cysts, pseudocysts and cystic neoplasms. Mucinous cystic neoplasm is a frequent type of cystic neoplasm and has a malignant potential. Serous cystadenoma follows in frequency and is usually benign. Intraductal papillary mucinous neoplasms are the most commonly resected cystic pancreatic neoplasms characterized by dilated segments of the main pancreatic duct and/or side branches, the wall of which is covered by mucus secreting cells. These neoplasms can occupy the pancreatic head or any part of the organ. Solid pseudopapillary tumor is rare, has a low tendency for malignancy, and is usually located in the pancreatic body or tail. Endoscopic ultrasound with the use of fine-needle aspiration and cytology permits discrimination of those lesions. In this review, the main characteristics of those lesions are presented, as well as recommendations regarding their follow up and management according to recent guidelines. PMID:27065727

  8. Devic disease with brainstem lesions.

    PubMed

    Chalumeau-Lemoine, Ludivine; Chretien, Fabrice; Gaëlle Si Larbi, Anne; Brugieres, Pierre; Gray, Françoise; Brun-Buisson, Christian; Creange, Alain

    2006-04-01

    We describe a patient who suffered from an unusually severe form of neuromyelitis optica with a hyperacute time-course evolution requiring mechanical ventilation within 3 days. The patient died after 72 days and autopsy showed major spinal cord, optic nerve, and brainstem necrosis, and multifocal necrotic lesions on the cerebellum and cerebral white matter. PMID:16606774

  9. Galactic Habitable Orbits

    NASA Astrophysics Data System (ADS)

    Rahimi, A.; Mao, S.; Kawata, D.

    2014-03-01

    The fossil record shows that the Earth has experienced several mass extinctions over the past 500 million years1, and it has been suggested that there is a periodicity in extinction events on timescales of tens1 and/or hundreds of millions of years. Various hypotheses have been proposed to explain the cause of the mass extinctions, including the suggestion that the Earth's ozone layer may have been destroyed by intense radiation from a nearby supernovae2- 3, exposing the Earth's surface to damaging UV radiation. Recent observations of cores taken from the ocean floor revealed atoms of a very rare isotope of iron (60Fe) believed to have arrived on Earth around 2 million years ago as fallout from a nearby supernovae4. Astronomical evidence for that past supernovae was recently found in the debris of a young cluster of massive stars5, by tracing its past orbit, putting it at the right place at the right time to explain the mild extinction event. Here we report new high-resolution (both in space and time) N-body chemodynamical simulations (carried out with our novel code GCD+6) of the evolution of a model Milky Way Galaxy, tracing the orbit of èsun-like' stars over a 500 million year period, checking the proximity to supernovae throughout the history of the orbit and comparing the times when this occurs with past mass extinctions on Earth. We additionally explain the important effects of the spiral arm pattern, radial migration of stars and Galactic chemistry on habitability.

  10. SLAP lesions: a treatment algorithm.

    PubMed

    Brockmeyer, Matthias; Tompkins, Marc; Kohn, Dieter M; Lorbach, Olaf

    2016-02-01

    Tears of the superior labrum involving the biceps anchor are a common entity, especially in athletes, and may highly impair shoulder function. If conservative treatment fails, successful arthroscopic repair of symptomatic SLAP lesions has been described in the literature particularly for young athletes. However, the results in throwing athletes are less successful with a significant amount of patients who will not regain their pre-injury level of performance. The clinical results of SLAP repairs in middle-aged and older patients are mixed, with worse results and higher revision rates as compared to younger patients. In this population, tenotomy or tenodesis of the biceps tendon is a viable alternative to SLAP repairs in order to improve clinical outcomes. The present article introduces a treatment algorithm for SLAP lesions based upon the recent literature as well as the authors' clinical experience. The type of lesion, age of patient, concomitant lesions, and functional requirements, as well as sport activity level of the patient, need to be considered. Moreover, normal variations and degenerative changes in the SLAP complex have to be distinguished from "true" SLAP lesions in order to improve results and avoid overtreatment. The suggestion for a treatment algorithm includes: type I: conservative treatment or arthroscopic debridement, type II: SLAP repair or biceps tenotomy/tenodesis, type III: resection of the instable bucket-handle tear, type IV: SLAP repair (biceps tenotomy/tenodesis if >50 % of biceps tendon is affected), type V: Bankart repair and SLAP repair, type VI: resection of the flap and SLAP repair, and type VII: refixation of the anterosuperior labrum and SLAP repair.

  11. Quark Orbital Angular Momentum

    NASA Astrophysics Data System (ADS)

    Burkardt, Matthias

    2016-06-01

    Generalized parton distributions provide information on the distribution of quarks in impact parameter space. For transversely polarized nucleons, these impact parameter distributions are transversely distorted and this deviation from axial symmetry leads on average to a net transverse force from the spectators on the active quark in a DIS experiment. This force when acting along the whole trajectory of the active quark leads to transverse single-spin asymmetries. For a longitudinally polarized nucleon target, the transverse force implies a torque acting on the quark orbital angular momentum (OAM). The resulting change in OAM as the quark leaves the target equals the difference between the Jaffe-Manohar and Ji OAMs.

  12. Orbiter door closure tools

    NASA Technical Reports Server (NTRS)

    Acres, W. R.

    1980-01-01

    Safe reentry of the shuttle orbiter requires that the payload bay doors be closed and securely latched. Since a malfunction in the door drive or bulkhead latch systems could make safe reentry impossible, the requirement to provide tools to manually close and secure the doors was implemented. The tools would disconnect a disabled door or latch closure system and close and secure the doors if the normal system failed. The tools required to perform these tasks have evolved into a set that consists of a tubing cutter, a winch, a latching tool, and a bolt extractor. The design, fabrication, and performance tests of each tool are described.

  13. Counter-Orbitals: Another Class of Co-Orbitals

    NASA Astrophysics Data System (ADS)

    Dobrovolskis, Anthony R.

    2012-10-01

    Co-orbital companions share the same orbital period and semi-major axis about a primary (star or planet). Heretofore there have been three recognized classes of co-orbitals: (1) Trojans librate in tadpole-shaped orbits about the equilateral Lagrange points L4 and L5, 60 degrees ahead of or behind the secondary (planet or satellite). (2) Horse-shoe companions librate about both L4 and L5, as well as the L3 Lagrange point diametrically opposite the secondary. (3) ``Quasi-satellites'' appear to be in distant retrograde orbits about the secondary, but actually are in prograde orbits about the primary with the same period as the secondary. Quasi-satellite orbits lie outside the secondary's Hill sphere, and enclose both L1 and L2, and sometimes L4 and L5 as well. In addition, some asteroids and comets are found in hybrid orbits which alternate among the above three classes, or combine some of their features. New research now reveals a fourth class of co-orbitals, which does not appear to be known before, and may be called ``counter-orbitals''. Imagine reversing the inertial velocity of a distant quasi-satellite. Then it remains in orbit about the primary, with the same period, semi-major axis, eccentricity, and orbital plane, although retrograde. But instead of remaining relatively close to the secondary, now it passes the secondary twice per orbit, near periapsis and apoapsis. The attractive impulses at these conjunctions tend to stabilize this arrangement. Numerical simulations of the general three-body problem verify that counter-orbitals can persist for over 10,000 orbits, with small vertical excursions, but a wide range of eccentricities and mass ratios. For example, Charon can maintain counter-orbital companions at least up to 3 percent of its own mass, in eccentric orbits extending from about 7050 km out to 41700 km from the center of Pluto. This may present a collision hazard to the New Horizons spacecraft.

  14. Langerhans Cell Histiocytosis (LCH): Guidelines for Diagnosis, Clinical Work-Up, and Treatment for Patients Till the Age of 18 Years

    PubMed Central

    Haupt, Riccardo; Minkov, Milen; Astigarraga, Itziar; Schäfer, Eva; Nanduri, Vasanta; Jubran, Rima; Egeler, R Maarten; Janka, Gritta; Micic, Dragan; Rodriguez-Galindo, Carlos; Van Gool, Stefaan; Visser, Johannes; Weitzman, Sheila; Donadieu, Jean

    2013-01-01

    These guidelines for the management of patients up to 18 years with Langerhans cell histiocytosis (LCH) have been set up by a group of experts involved in the Euro Histio Net project who participated in national or international studies and in peer reviewed publications. Existing guidelines were reviewed and changed where new evidence was available in the literature up to 2012. Data and publications have been ranked according to evidence based medicine and when there was a lack of published data, consensus between experts was sought. Guidelines for diagnosis, initial clinical work-up, and treatment and long-term follow-up of LCH patients are presented. PMID:23109216

  15. Orbital construction demonstration study

    NASA Technical Reports Server (NTRS)

    1976-01-01

    A conceptual design and program plan for an Orbital Construction Demonstration Article (OCDA) was developed that can be used for evaluating and establishing practical large structural assembly operations. A flight plan for initial placement and continued utility is presented as a basic for an entirely new shuttle payload line-item having great future potential benefit for space applications. The OCDA is a three-axis stabilized platform in low-earth orbit with many structural nodals for mounting large construction and fabrication equipments. This equipment would be used to explore methods for constructing the large structures for future missions. The OCDA would be supported at regular intervals by the shuttle. Construction experiments and consumables resupply are performed during shuttle visit periods. A 250 kw solar array provides sufficient power to support the shuttle while attached to the OCDA and to run construction experiments at the same time. Wide band communications with a Telemetry and Data Relay Satellite compatible high gain antenna can be used between shuttle revisits to perform remote controlled, TV assisted construction experiments.

  16. Orbit selection for a Mars geoscience/climatology orbiter

    NASA Technical Reports Server (NTRS)

    Uphoff, C.

    1984-01-01

    This paper is a presentation of recent work to provide orbit design and selection criteria for a close, nearly polar, nearly circular orbit of Mars. The main aspects of the work are the evaluation of atmospheric drag for altitude selection, the orbit evolution for variations in periapsis altitude, and the interactions of those factors with the science objectives of the MGCO mission. A dynamic model of the Mars atmosphere is available from parallel efforts and the latest estimates of the upper atmospheric density and its time history are incorporated into the analysis to provide a final orbit that satisfies planetary quarantine requirements.

  17. General view of the Orbiter Discovery in the Orbiter Processing ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    General view of the Orbiter Discovery in the Orbiter Processing Facility at Kennedy Space Center showing the payload bay doors open exposing the heat-dissipating radiator panels located on the inside of the payload bay doors. Also in the view is the boom portion of the boom sensor system deployed as part of the return to flight procedures after STS-107 to inspect the orbiter's thermal protection system. The Remote Manipulator System, the "Canadarm", and the airlock are seen in the background of the image. - Space Transportation System, Orbiter Discovery (OV-103), Lyndon B. Johnson Space Center, 2101 NASA Parkway, Houston, Harris County, TX

  18. Primary osteoma of the orbit with atypical facial pain: case report and literature review.

    PubMed

    Kayaci, Selim; Kanat, Ayhan; Gucer, Hasan; Seckin, Hakan

    2012-01-01

    Osteoma is a benign, slowly growing tumor that mainly occurs in the bones and cavities of the middle third of the face, representing the most frequent benign tumor of the paranasal sinuses. It rarely originates primarily from the orbit. Most of these lesions develop in the fourth to fifth decades of life, and are more commonly encountered in males. In the English literature, there are so far three reported cases of primary osteoma of the orbit that originated from the sphenoid bone. Here we present another case of a primary osteoma of the orbit presenting with atypical facial pain and discuss the relevant literature.

  19. Orbital Metastasis of Breast Cancer Mimicking Invasive Fungal Rhinosinusitis.

    PubMed

    Tabai, Mayara; Hazboun, Igor Moreira; Sakuma, Emerson Taro Inoue; Sampaio, Marcelo Hamilton; Sakano, Eulalia

    2016-01-01

    Introduction. A range of traumatic, vascular, inflammatory, infectious, and neoplastic processes can affect the orbit and its structures. In the area of otolaryngology, the rhino-orbital-cerebral involvement of invasive fungal rhinosinusitis can affect the orbit, which may look like initially a rhinosinusitis or even mimic malignancy. Case Presentation. Female patient, 32 years old, with headache and ocular proptosis. She was using prednisone in immunosuppressive doses for a year and had breast cancer treated three years earlier. The initial CT scan showed opacification of the sphenoid and ethmoid sinuses, left intraorbital involvement and contrast impregnation in the cavernous sinus. The biopsy resulted positive for invasive ductal carcinoma of the breast. Discussion. The initial CT scan of our patient showed both signs of early changes of invasive fungal rhinosinusitis (IFR) and possible metastatic involvement. The intracranial extension and ocular involvement are usually the most common signs of IFR (first hypothesis). Among metastases at the orbit and the eye, breast and lung carcinomas are the most frequent. Conclusion. Although several studies on the differential diagnosis of orbital lesions exist, especially when it concerns the involvement of the nasal cavity, the diagnosis by imaging is still a challenge. PMID:27563478

  20. General anaesthetic considerations for haemostasis in orbital surgery.

    PubMed

    Sia, David Ik Tuo; Chalmers, Alison; Singh, Varjeet; Malhotra, Raman; Selva, Dinesh

    2014-02-01

    Orbital surgery is often conducted in areas with limited exposure where vital structures are tightly crowded together. A bloodless field is paramount in orbital surgery for the proper identification of normal and pathologic tissue and even minimal bleeding can obscure the surgical field, making surgery more difficult and increasing the risk of complications. Surgery for highly vascular orbital lesions is an additional situation where maintaining an adequate surgical field is often challenging but paramount. The role of the anaesthetist in controlling surgical blood loss has been increasingly recognized in the last few decades. Various techniques including hypotensive anaesthesia have been described, but the control of intraoperative bleeding does not rely on a single particular technique, but a series of well-designed interventions that result in optimal conditions. An understanding of the anaesthetic considerations pertinent to haemostasis is invaluable for oculoplastic surgeons. Additionally, with the growing use of endonasal approaches to medial wall decompression and accessing the medial orbit, it has become increasingly important that orbital surgeons understand the anaesthetic requirements of their colleagues in other disciplines.

  1. Orbital Metastasis of Breast Cancer Mimicking Invasive Fungal Rhinosinusitis

    PubMed Central

    Hazboun, Igor Moreira; Sakuma, Emerson Taro Inoue; Sampaio, Marcelo Hamilton

    2016-01-01

    Introduction. A range of traumatic, vascular, inflammatory, infectious, and neoplastic processes can affect the orbit and its structures. In the area of otolaryngology, the rhino-orbital-cerebral involvement of invasive fungal rhinosinusitis can affect the orbit, which may look like initially a rhinosinusitis or even mimic malignancy. Case Presentation. Female patient, 32 years old, with headache and ocular proptosis. She was using prednisone in immunosuppressive doses for a year and had breast cancer treated three years earlier. The initial CT scan showed opacification of the sphenoid and ethmoid sinuses, left intraorbital involvement and contrast impregnation in the cavernous sinus. The biopsy resulted positive for invasive ductal carcinoma of the breast. Discussion. The initial CT scan of our patient showed both signs of early changes of invasive fungal rhinosinusitis (IFR) and possible metastatic involvement. The intracranial extension and ocular involvement are usually the most common signs of IFR (first hypothesis). Among metastases at the orbit and the eye, breast and lung carcinomas are the most frequent. Conclusion. Although several studies on the differential diagnosis of orbital lesions exist, especially when it concerns the involvement of the nasal cavity, the diagnosis by imaging is still a challenge. PMID:27563478

  2. Neptune's story. [Triton's orbit perturbation

    NASA Technical Reports Server (NTRS)

    Goldreich, P.; Murray, N.; Longaretti, P. Y.; Banfield, D.

    1989-01-01

    It is conjectured that Triton was captured from a heliocentric orbit as the result of a collision with what was then one of Neptune's regular satellites. The immediate post-capture orbit was highly eccentric. Dissipation due to tides raised by Neptune in Triton caused Triton's orbit to evolve to its present state in less than one billion years. For much of this time Triton was almost entirely molten. While its orbit was evolving, Triton cannibalized most of the regular satellites of Neptune and also perturbed Nereid, thus accounting for that satellite's highly eccentric and inclined orbit. The only regular satellites of Neptune that survived were those that formed well within 5 Neptune radii, and they move on inclined orbits as the result of chaotic perturbations forced by Triton.

  3. Precise Orbit Determination for ALOS

    NASA Technical Reports Server (NTRS)

    Nakamura, Ryo; Nakamura, Shinichi; Kudo, Nobuo; Katagiri, Seiji

    2007-01-01

    The Advanced Land Observing Satellite (ALOS) has been developed to contribute to the fields of mapping, precise regional land coverage observation, disaster monitoring, and resource surveying. Because the mounted sensors need high geometrical accuracy, precise orbit determination for ALOS is essential for satisfying the mission objectives. So ALOS mounts a GPS receiver and a Laser Reflector (LR) for Satellite Laser Ranging (SLR). This paper deals with the precise orbit determination experiments for ALOS using Global and High Accuracy Trajectory determination System (GUTS) and the evaluation of the orbit determination accuracy by SLR data. The results show that, even though the GPS receiver loses lock of GPS signals more frequently than expected, GPS-based orbit is consistent with SLR-based orbit. And considering the 1 sigma error, orbit determination accuracy of a few decimeters (peak-to-peak) was achieved.

  4. Orbit accuracy assessment for Seasat

    NASA Technical Reports Server (NTRS)

    Schutz, B. E.; Tapley, B. D.

    1980-01-01

    Laser range measurements are used to determine the orbit of Seasat during the period from July 28, 1978, to Aug. 14, 1978, and the influence of the gravity field, atmospheric drag, and solar radiation pressure on the orbit accuracy is investigated. It is noted that for the orbits of three-day duration, little distinction can be made between the influence of different atmospheric models. It is found that the special Seasat gravity field PGS-S3 is most consistent with the data for three-day orbits, but an unmodeled systematic effect in radiation pressure is noted. For orbits of 18-day duration, little distinction can be made between the results derived from the PGS gravity fields. It is also found that the geomagnetic field is an influential factor in the atmospheric modeling during this time period. Seasat altimeter measurements are used to determine the accuracy of the altimeter measurement time tag and to evaluate the orbital accuracy.

  5. [Cystic testicular lesions in infancy].

    PubMed

    Calleja Escudero, J; Pascual Samaniego, M; Garrido Redondo, M; Matas Gómez, V; Fernández Domínguez, L; Fernández del Busto, E

    2004-09-01

    The present article reports a case 11 month-old infant with a right intratesticular cyst. We analyze the etiology, differential diagnosis and management off all cystic lesions of the pediatric testis. Patient age at presentation, examination features, tumor markers and sonographic appearance may assist in making a presumptive and occasionally definitive diagnosis preoperatively. The differential diagnosis include intratesticular simple cyst, epidermoid cyst, tunica albuginea cyst, testicular teratoma, juvenil granulosa cell tumor-gonadal stromal tumor, cystic dysplasia of the rete testis, cystic lymphangioma, and testicular torsion. Usually enucleation is the best treatment. A thorough understanding of potentially cystic testis lesions in children leads to the best management choices and often to preservation of a substantial portion of the affected testis.

  6. [Echographic diagnosis in orbital mucoceles].

    PubMed

    Rochels, R; Geyer, G; Bleier, R

    1985-04-01

    Sonography in 68 patients with orbital mucoceles revealed the following, pathognomonical A-scan criteria: Regular structure, low to extremely low reflectivity, sharply outlined borders, bony defects, missing or minimal compressibility and typical location in the supero-nasal anterior orbit. The echographical findings of "mucoceles" secondary to paranasal tumours are specially emphasised. Echography proves to be a very useful method in the detection and differentiation of orbital complications in paranasal diseases.

  7. Primary liposarcoma of the orbit.

    PubMed

    Khurana, Saurbhi; Gupta, Anoop K; Sen, Seema; Kashyap, Seema

    2014-01-01

    Liposarcoma is the most common sarcoma in adults, but is an extremely rare tumor of the orbit. We report 4 cases of primary orbital liposarcoma treated at our center. All cases had tumor localized to the orbit and underwent exenteration followed by radiotherapy in one patient. None of the patients had a recurrence until a follow-up of 1-5 years (mean: 4 years).

  8. Automatic segmentation of psoriasis lesions

    NASA Astrophysics Data System (ADS)

    Ning, Yang; Shi, Chenbo; Wang, Li; Shu, Chang

    2014-10-01

    The automatic segmentation of psoriatic lesions is widely researched these years. It is an important step in Computer-aid methods of calculating PASI for estimation of lesions. Currently those algorithms can only handle single erythema or only deal with scaling segmentation. In practice, scaling and erythema are often mixed together. In order to get the segmentation of lesions area - this paper proposes an algorithm based on Random forests with color and texture features. The algorithm has three steps. The first step, the polarized light is applied based on the skin's Tyndall-effect in the imaging to eliminate the reflection and Lab color space are used for fitting the human perception. The second step, sliding window and its sub windows are used to get textural feature and color feature. In this step, a feature of image roughness has been defined, so that scaling can be easily separated from normal skin. In the end, Random forests will be used to ensure the generalization ability of the algorithm. This algorithm can give reliable segmentation results even the image has different lighting conditions, skin types. In the data set offered by Union Hospital, more than 90% images can be segmented accurately.

  9. Imaging of skull base lesions.

    PubMed

    Kelly, Hillary R; Curtin, Hugh D

    2016-01-01

    Skull base imaging requires a thorough knowledge of the complex anatomy of this region, including the numerous fissures and foramina and the major neurovascular structures that traverse them. Computed tomography (CT) and magnetic resonance imaging (MRI) play complementary roles in imaging of the skull base. MR is the preferred modality for evaluation of the soft tissues, the cranial nerves, and the medullary spaces of bone, while CT is preferred for demonstrating thin cortical bone structure. The anatomic location and origin of a lesion as well as the specific CT and MR findings can often narrow the differential diagnosis to a short list of possibilities. However, the primary role of the imaging specialist in evaluating the skull base is usually to define the extent of the lesion and determine its relationship to vital neurovascular structures. Technologic advances in imaging and radiation therapy, as well as surgical technique, have allowed for more aggressive approaches and improved outcomes, further emphasizing the importance of precise preoperative mapping of skull base lesions via imaging. Tumors arising from and affecting the cranial nerves at the skull base are considered here. PMID:27432686

  10. Imaging of skull base lesions.

    PubMed

    Kelly, Hillary R; Curtin, Hugh D

    2016-01-01

    Skull base imaging requires a thorough knowledge of the complex anatomy of this region, including the numerous fissures and foramina and the major neurovascular structures that traverse them. Computed tomography (CT) and magnetic resonance imaging (MRI) play complementary roles in imaging of the skull base. MR is the preferred modality for evaluation of the soft tissues, the cranial nerves, and the medullary spaces of bone, while CT is preferred for demonstrating thin cortical bone structure. The anatomic location and origin of a lesion as well as the specific CT and MR findings can often narrow the differential diagnosis to a short list of possibilities. However, the primary role of the imaging specialist in evaluating the skull base is usually to define the extent of the lesion and determine its relationship to vital neurovascular structures. Technologic advances in imaging and radiation therapy, as well as surgical technique, have allowed for more aggressive approaches and improved outcomes, further emphasizing the importance of precise preoperative mapping of skull base lesions via imaging. Tumors arising from and affecting the cranial nerves at the skull base are considered here.

  11. Orbital molecules in electronic materials

    SciTech Connect

    Attfield, J. Paul

    2015-04-01

    Orbital molecules are made up of coupled orbital states on several metal ions within an orbitally ordered (and sometimes also charge-ordered) solid such as a transition metal oxide. Spin-singlet dimers are known in many materials, but recent discoveries of more exotic species such as 18-electron heptamers in AlV{sub 2}O{sub 4} and magnetic 3-atom trimerons in magnetite (Fe{sub 3}O{sub 4}) have shown that orbital molecules constitute a general new class of quantum electronic states in solids.

  12. Orbiter emergency crew escape system

    NASA Technical Reports Server (NTRS)

    Lofland, W. W.

    1980-01-01

    Two conventional ejection seats were incorporated into the first two orbiter vehicles to provide the crew with emergency ejection capability during the flight test programs. To avoid extensive development and test costs, existing ejection seats were selected and minimum modifications were made to accommodate the orbiter application. The new components and modifications were qualified at the component level, and a minimum sled test program was conducted to verify the orbiter installation and validate the six degree-of-freedom analysis. The system performance was certified and the orbital flight test capability was established by analysis.

  13. Imaging of the Postoperative Orbit.

    PubMed

    Learned, Kim O; Nasseri, Farbod; Mohan, Suyash

    2015-08-01

    Imaging evaluation of the postoperative orbit remains challenging even for the expert neuroradiologist. This article provides a simplified framework for understanding the complex postoperative appearances of the orbit, in an attempt to enhance the diagnostic accuracy of postoperative computed tomography and MR imaging of the orbit. Readers are familiarized with the normal appearances of common eye procedures and orbit reconstructions to help avoid interpretative pitfalls. Also reviewed are imaging features of common surgical complications, and evaluation of residual/recurrent neoplasm in the setting of oncologic imaging surveillance.

  14. Orbiter utilization as an ACRV

    NASA Technical Reports Server (NTRS)

    Cruz, Jonathan N.; Heck, Michael L.; Kumar, Renjith R.; Mazanek, Daniel D.; Troutman, Patrick A.

    1990-01-01

    Assuming that a Shuttle Orbiter could be qualified to serve long duration missions attached to Space Station Freedom in the capacity as an Assured Crew Return Vehicle (ACRV), a study was conducted to identify and examine candidate attach locations. Baseline, modified hardware, and new hardware design configurations were considered. Dual simultaneous Orbiter docking accommodation were required. Resulting flight characteristics analyzed included torque equilibrium attitude (TEA), microgravity environment, attitude controllability, and reboost fuel requirements. The baseline Station could not accommodate two Orbiters. Modified hardware configurations analyzed had large TEA's. The utilization of an oblique docking mechanism best accommodated an Orbiter as an ACRV.

  15. OSO-6 Orbiting Solar Observatory

    NASA Technical Reports Server (NTRS)

    1972-01-01

    The description, development history, test history, and orbital performance analysis of the OSO-6 Orbiting Solar Observatory are presented. The OSO-6 Orbiting Solar Observatory was the sixth flight model of a series of scientific spacecraft designed to provide a stable platform for experiments engaged in the collection of solar and celestial radiation data. The design objective was 180 days of orbital operation. The OSO-6 has telemetered an enormous amount of very useful experiment and housekeeping data to GSFC ground stations. Observatory operation during the two-year reporting period was very successful except for some experiment instrument problems.

  16. Neurophysiology and neuroanatomy of smooth pursuit: lesion studies.

    PubMed

    Sharpe, James A

    2008-12-01

    Smooth pursuit impairment is recognized clinically by the presence of saccadic tracking of a small object and quantified by reduction in pursuit gain, the ratio of smooth eye movement velocity to the velocity of a foveal target. Correlation of the site of brain lesions, identified by imaging or neuropathological examination, with defective smooth pursuit determines brain structures that are necessary for smooth pursuit. Paretic, low gain, pursuit occurs toward the side of lesions at the junction of the parietal, occipital and temporal lobes (area V5), the frontal eye field and their subcortical projections, including the posterior limb of the internal capsule, the midbrain and the basal pontine nuclei. Paresis of ipsiversive pursuit also results from damage to the ventral paraflocculus and caudal vermis of the cerebellum. Paresis of contraversive pursuit is a feature of damage to the lateral medulla. Retinotopic pursuit paresis consists of low gain pursuit in the visual hemifield contralateral to damage to the optic radiation, striate cortex or area V5. Craniotopic paresis of smooth pursuit consists of impaired smooth eye movement generation contralateral to the orbital midposition after acute unilateral frontal or parietal lobe damage. Omnidirectional saccadic pursuit is a most sensitive sign of bilateral or diffuse cerebral, cerebellar or brainstem disease. The anatomical and physiological bases of defective smooth pursuit are discussed here in the context of the effects of lesion in the human brain.

  17. Geology orbiter comparison study

    NASA Technical Reports Server (NTRS)

    Cutts, J. A. J.; Blasius, K. R.; Davis, D. R.; Pang, K. D.; Shreve, D. C.

    1977-01-01

    Instrument requirements of planetary geology orbiters were examined with the objective of determining the feasibility of applying standard instrument designs to a host of terrestrial targets. Within the basic discipline area of geochemistry, gamma-ray, X-ray fluorescence, and atomic spectroscopy remote sensing techniques were considered. Within the discipline area of geophysics, the complementary techniques of gravimetry and radar were studied. Experiments using these techniques were analyzed for comparison at the Moon, Mercury, Mars and the Galilean satellites. On the basis of these comparative assessments, the adaptability of each sensing technique was judged as a basic technique for many targets, as a single instrument applied to many targets, as a single instrument used in different mission modes, and as an instrument capability for nongeoscience objectives.

  18. Exploratory orbit analysis

    SciTech Connect

    Michelotti, L.

    1989-03-01

    Unlike the other documents in these proceedings, this paper is neither a scientific nor a technical report. It is, rather, a short personal essay which attempts to describe an Exploratory Orbit Analysis (EOA) environment. Analyzing the behavior of a four or six dimensional nonlinear dynamical system is at least as difficult as analyzing events in high-energy collisions; the consequences of doing it badly, or slowly, would be at least as devastating; and yet the level of effort and expenditure invested in the latter, the very attention paid to it by physicists at large, must be two orders of magnitude greater than that given to the former. It is difficult to choose the model which best explains the behavior of a physical device if one does not first understand the behavior of the available models. The time is ripe for the development of a functioning EOA environment, which I will try to describe in this paper to help us achieve this goal.

  19. Orbital angular momentum microlaser.

    PubMed

    Miao, Pei; Zhang, Zhifeng; Sun, Jingbo; Walasik, Wiktor; Longhi, Stefano; Litchinitser, Natalia M; Feng, Liang

    2016-07-29

    Structured light provides an additional degree of freedom for modern optics and practical applications. The effective generation of orbital angular momentum (OAM) lasing, especially at a micro- and nanoscale, could address the growing demand for information capacity. By exploiting the emerging non-Hermitian photonics design at an exceptional point, we demonstrate a microring laser producing a single-mode OAM vortex lasing with the ability to precisely define the topological charge of the OAM mode. The polarization associated with OAM lasing can be further manipulated on demand, creating a radially polarized vortex emission. Our OAM microlaser could find applications in the next generation of integrated optoelectronic devices for optical communications in both quantum and classical regimes.

  20. Orbital angular momentum microlaser

    NASA Astrophysics Data System (ADS)

    Miao, Pei; Zhang, Zhifeng; Sun, Jingbo; Walasik, Wiktor; Longhi, Stefano; Litchinitser, Natalia M.; Feng, Liang

    2016-07-01

    Structured light provides an additional degree of freedom for modern optics and practical applications. The effective generation of orbital angular momentum (OAM) lasing, especially at a micro- and nanoscale, could address the growing demand for information capacity. By exploiting the emerging non-Hermitian photonics design at an exceptional point, we demonstrate a microring laser producing a single-mode OAM vortex lasing with the ability to precisely define the topological charge of the OAM mode. The polarization associated with OAM lasing can be further manipulated on demand, creating a radially polarized vortex emission. Our OAM microlaser could find applications in the next generation of integrated optoelectronic devices for optical communications in both quantum and classical regimes.