Giant convexity chondroma with meningeal attachment.

PubMed

Feierabend, Denise; Maksoud, Salah; Lawson McLean, Aaron; Koch, Arend; Kalff, Rolf; Walter, Jan

2018-06-01

Intracranial chondroma is a rare and benign tumor with usual onset in young adulthood. The skull base is the most common site of occurrence although, less often, the tumors can appear at the falx cerebri or at the dural convexity. The differentiation of these lesions from meningiomas through imaging is generally difficult. Clinical case presentation and review of the current literature. We report a case of a 25-year-old male patient with a giant convexity chondroma with meningeal attachment in the right frontal lobe that was detected after a first generalized seizure. Based on the putative diagnosis of meningioma, the tumor was completely resected via an osteoplastic parasagittal craniotomy. The postoperative MRI confirmed the complete tumor resection. Histopathological analysis revealed the presence of a chondroma. Intracranial chondromas are a rarity and their preoperative diagnosis based on neuroimaging is difficult. In young patients and those with skeletal disease, the differential diagnosis of a chondroma should be considered. In symptomatic patients, operative resection is sensible. In most cases total removal of the tumor is possible and leads to full recovery. When the finding is merely incidental in older patients, a watchful waiting approach is acceptable, given the benign and slow-growing nature of the lesion. Copyright © 2018 Elsevier B.V. All rights reserved.

Orbital chondroma rodens in a dog.

PubMed

Pletcher, J M; Koch, S A; Stedham, M A

1979-07-15

A chondroma rodens involving the superficial medial aspect of the right orbit was diagnosed in a 9-year-old dog referred because of chronic unilateral epiphora. Examination revealed several ophthalmic abnormalities attributable to a space-occupying mass in the superficial medial aspect of the orbit. The mass was excised; however, regrowths at the primary site necessitated additional surgical interventions. The dog was given radiation therapy, which provided encouraging results. Subtle histologic differences as well as differing epidemiologic features suggest that chondroma rodens is not analogous to the human entity of juvenile aponeurotic fibroma, to which it has been compared in the past.

[Chondroma adjacent to Meckel's cave mimicking a fifth cranial nerve neurinoma. A case report].

PubMed

Narro-Donate, Jose María; Huete-Allut, Antonio; Velasco-Albendea, Francisco J; Escribano-Mesa, Jose A; Mendez-Román, Paddy; Masegosa-González, Jose

2016-01-01

Cranial chondromas are tumours arising from chondrocyte embryonic remnants cells that usually appear in the skull base synchondrosis. In contrast to the rest of the organism, where chondroid tumours are the most common primary bone tumour just behind the haematopoietic lineage ones, they are a rarity at cranial level, with an incidence of less than 1% of intracranial tumours. The case is reported on a 42 year-old male referred to our clinic due to the finding of an extra-axial lesion located close to the Meckel's cave region, with extension to the posterior fossa and brainstem compression after progressive paraparesis of 6 months onset. With the diagnosis of trigeminal schwannoma, a subtotal tumour resection was performed using a combined supra-infratentorial pre-sigmoidal approach. The postoperative histopathology report confirmed the diagnosis of cranial chondroma. Copyright © 2016 Sociedad Española de Neurocirugía. Published by Elsevier España. All rights reserved.

[Incomplete Carney's Triad and arterial hypertension in a young woman].

PubMed

Allievi, Alberto; Araya, Valentina; Calvar, Cecilia; Cimino, Conrado; Delle Piane, Hugo; Diaz, Gabriela; Gianni, Marta; Prudkin, Ludmila

2006-01-01

The case of young woman with arterial hypertension diagnosed two years before, is here presented; she had a ferropenic anemia caused by digestive loss of blood. Multiple gastric tumors and pararenal non functioning paraganglioma were found. No chondromas were detected. An incomplete Carney's Triad was diagnosed. We remark that multiple gastric tumors in a young adult suggest the possibility of gastrointestinal stromal tumors (GIST) Endoscopic biopsy frequently is not effective because these tumors are deep placed in the muscular gastric layers. The importance of specific techniques for a positive diagnosis are emphasized. Continuous follow up is needed because these tumors have uncertain prognosis. Lung chondromas may appear years later after the GIST was removed and might be confused with GIST metastases.

[Benign tumors and pseudotumors of temporo-mandibular joint: radiologic aspects].

PubMed

Izzo, L; Caputo, M; Buffone, A; Casullo, A; Perrone, A; Sassi, S; Impara, L; Luppi, G; Mazza, D; Marini, Marina

2005-01-01

Benign tumors and tumor-like lesions that involve temporo mandibular joint are very rare. Those more frequent are osteochondroma, chondroma, osteoma, pigmented villonodular synovitis and synovial chondromatosis. The Authors report six cases of patients affected by these pathologies in which imaging, such as TC, MRI and/or ortopantomography have been useful to have a diagnosis.

Chondromyxoid Fibroma of the Ethmoid Sinus

DTIC Science & Technology

2011-06-01

and its perceptible recurrence rate. It is for these reasons that CMF should be distinguished from a myxoid variant of chondrosarcoma (which usually...cartilage of the type seen in chondroma or chondrosarcoma and is a focal finding in less than 20% of CMF cases [5]. Mitotic figures are sparse, accounting...CMF from chondrosarcoma [5]. The histologic differential diagnosis of CMF includes, most importantly, myxoid chondrosarcoma and chondro- blastoma [7

Giant Solitary Synovial Osteochondroma of the Subtalar Joint.

PubMed

Lui, Tun Hing

2016-01-01

A rapidly progressing calcified mass was found in the left sinus tarsi in a 12-year-old female after a trivial ankle sprain. The lesion mimicked an aggressive lesion clinically and radiographically. Ultrasound-guided biopsy confirmed the diagnosis of a synovial chondroma. Excision of the tumor and partial synovectomy were performed. The histologic diagnosis was a solitary synovial osteochondroma. The condition had not recurred after a follow-up period of 12 months. Copyright © 2016 American College of Foot and Ankle Surgeons. Published by Elsevier Inc. All rights reserved.

Imaging of rare appendicular non-acral soft-tissue chondromas in adults with histopathologic correlation.

PubMed

Nouh, Mohamed Ragab; Amr, Hanan Abd El-Aziz; Ali, Rola H

2018-06-01

Background Soft-tissue chondroma (STC) is a rare benign soft tissue tumor that arises primarily in acral extra-skeletal locations. Occasionally, STCs may arise in more proximal non-acral locations, accompanied by non-classic features that label them as indeterminate lesions and pose diagnostic challenge for both radiologists and pathologists alike. Purpose To explicate the potential of diagnostic imaging in the identification and characterization of appendicular non-acral STCs with emphasis on their morphologic magnetic resonance imaging (MRI) enhancement. Material and Methods Our clinical database records were searched for patients with histologically proven primary soft-tissue chondroid lesions over a five-year period. Two musculoskeletal (MSK) trained radiologists evaluated the imaging studies and an MSK pathologist revised the pathological findings. Results The study included six cases of appendicular non-acral STCs (mean age = 40.5 years). The mean size of the tumors was 5.6 cm, with four localized to the knee region, one in the thigh, and one in the sternoclavicular region. All cases showed high signal intensity matrix with low-signal intensity septa on T2-weighted MRI and post-contrast marginal/septal enhancement. The lesions were lobulated and lacked host tissue reaction except for one showing subjacent mild soft-tissue edema. Histologically, the cases lacked overt features of malignancy although one was originally misdiagnosed as chondrosarcoma. Conclusion Non-acral STCs are benign cartilaginous tumors that may pose a diagnostic challenge, both radiologically and pathologically. Collaborative imaging and pathologic workup is needed for better characterization of non-aggression of these lesions, and to avoid diagnostic pitfalls and unnecessary radical resections.

Multiple myeloma involving the cricoid cartilage.

PubMed

Floré, B; Hermans, R

2013-01-01

We present the case of a man with dyspnea due to a mass in the cricoid cartilage that turns out to be an extramedullary plasmocytoma. Although the patient has a history of multiple myeloma, the disease only rarely affects the cricoid cartilage. Other subglottic lesions possibly involving the cricoid cartilage are squamous cell carcinoma, chondroma, chondrosarcoma and metastasis. The imaging characteristics suggesting extramedullary plasmocytoma arising from the cricoid consist of thinning and expansion of the cartilage laminae without mucosal lesions nor soft tissue mass adjacent to the cricoid cartilage. The patient was successfully treated with radiation therapy and peroral steroids.

Calcifying (juvenile) aponeurotic fibroma of the scalp.

PubMed

Thakur, Jagdeep S; Diwana, Vijay K; Sharma, Sudarshan; Thakur, Anamika

2011-10-01

Calcifying aponeurotic fibroma is a benign tumor with a predilection for distal parts of the extremities; it is very rare in the head and neck region. It commonly affects young patients-hence the term juvenile in the name. It is fibroblastic in origin and considered a cartilage analogue of fibromatosis, but its exact etiology remains unknown. Clinically, this tumor needs to be differentiated from fibromatosis, nodular fasciitis, chondroma, schwannoma, and rheumatoid nodule. A 24-year-old woman presented with swelling in the forehead for the previous 6 months. Wide surgical excision of the lesion was performed, and histopathologic examination revealed a calcifying aponeurotic fibroma. We reviewed the literature on this rare clinical entity and present our hypothesis on its etiology.

68Ga-DOTA-TATE PET/CT for Molecular Imaging of Somatostatin Receptor Expression in Extra-adrenal Paraganglioma in a Case of Complete Carney Triad.

PubMed

Derlin, Thorsten; Hartung, Dagmar; Hueper, Katja

2017-12-01

Carney triad is a very rare syndrome characterized by the synchronous or metachronous occurrence of gastrointestinal stromal tumors, pulmonary chondroma, and extra-adrenal paraganglioma. We present the case of a 36-year-old woman with complete Carney triad who underwent a Ga-DOTA-TATE PET/CT scan for restaging of metastasizing extra-adrenal paraganglioma and for evaluation of targeted radionuclide therapy potential. On the Ga-DOTA-TATE PET scan, increased tracer accumulation was observed in paraganglioma metastases. This case highlights the usefulness of Ga-DOTA-TATE PET/CT for restaging of metastasizing paraganglioma in Carney triad and the option of targeted radionuclide therapy in this entity.

Light up the "no-man's land" on the brain stem.

PubMed

Kawase, T

1995-12-01

The ventral surface of the brain stem is anatomically surrounded by the clivus anteriorly, brain stem posteriorly and by the petrous pyramid and cranial nerves from IIIrd to XIIth laterally in the deep posterior cranial fossa. Neurosurgical extra-axial pathologies arising from the area are aneurysms on the vertebro-basilar artery, benign tumors such as clival meningiomas, chordomas, chondromas, trigeminal neurinomas and prepontine epidermoid tumors. Surgical access to the area had been difficult for long years since the neurosurgery was established, because located deeply in such a surgical blindness, so-called "no-man's land". However, recent technical development of "skull base surgery" is opening new doors to light up the surgical darkness of the "no-man's land". This paper reviews the history, development, technique and future prospect of the skull base surgery to open the "no-man's land".

Synchronous adrenocortical neoplasms, paragangliomas, and pheochromocytomas: syndromic considerations regarding an unusual constellation of endocrine tumors.

PubMed

LeBlanc, Melissa; Tabrizi, Mohsen; Kapsner, Patricia; Hanson, Joshua Anspach

2014-12-01

The most common clinical syndromes presenting with paragangliomas and/or pheochromocytomas as their endocrine components are multiple endocrine neoplasia type 2, neurofibromatosis, Von Hippel-Lindau syndrome, Carney-Stratakis syndrome, Carney triad, and the recently described hereditary paraganglioma syndrome. Only Carney triad is known to also present with adrenocortical adenomas, currently representing the only described syndrome in which all 3 of the aforementioned tumors are found together. In most cases, prototypical lesions of the triad such as gastrointestinal stromal tumor and pulmonary chondromas are also seen. We present a case of a young woman with synchronous paragangliomas, adrenal/extra-adrenal cortical neoplasms, and pheochromocytoma without genetic mutations for multiple endocrine neoplasia 2, Von Hippel-Lindau syndrome, neurofibromatosis, and succinate dehydrogenase. We speculate that this represents a previously undescribed presentation of Carney triad and, at the very least, indicates the need for monitoring for the development of other tumors of the triad. Copyright © 2014 Elsevier Inc. All rights reserved.

Cranio-orbital primary intraosseous haemangioma.

PubMed

Gupta, T; Rose, G E; Manisali, M; Minhas, P; Uddin, J M; Verity, D H

2013-11-01

Primary intraosseous haemangioma (IOH) is a rare benign neoplasm presenting in the fourth and fifth decades of life. The spine and skull are the most commonly involved, orbital involvement is extremely rare. We describe six patients with cranio-orbital IOH, the largest case series to date. Retrospective review of six patients with histologically confirmed primary IOH involving the orbit. Clinical characteristics, imaging features, approach to management, and histopathological findings are described. Five patients were male with a median age of 56. Pain and diplopia were the most common presenting features. A characteristic 'honeycomb' pattern on CT imaging was demonstrated in three of the cases. Complete surgical excision was performed in all cases with presurgical embolisation carried out in one case. In all the cases, histological studies identified cavernous vascular spaces within the bony tissue. These channels were lined by single layer of cytologically normal endothelial cells. IOCH of the cranio-orbital region is rare; in the absence of typical imaging features, the differential diagnosis includes chondroma, chondrosarcoma, bony metastasis, and lymphoma. Surgical excision may be necessary to exclude more sinister pathology. Intraoperative haemorrhage can be severe and may be reduced by preoperative embolisation.

Carcinogenesis of PIK3CA

PubMed Central

2013-01-01

PIK3CA is the most frequently mutated oncogene in human cancers. PIK3CA is phosphatidylinositol-4,5-bisphosphate 3-kinase, catalytic subunit alpha. It controls cell growth, proliferation, motility, survival, differentiation and intracellular trafficking. In most of human cancer alteration occurred frequently in the alpha isoform of phosphatidylinositol 3 kinase. PIK3CA mutations were most frequent in endometrial, ovarian, colorectal, breast, cervical, squamous cell cancer of the head and neck, chondroma, thyroid carcinoma and in cancer family syndrome. Inhibition of PI3K signaling can diminish cell proliferation, and in some circumstances, promote cell death. Consequently, components of this pathway present attractive targets for cancer therapeutics. A number of PI3K pathway inhibitors have been developed and used. PI3K inhibitors (both pan-PI3K and isoform-specific PI3K inhibitors), dual PI3K-mTOR inhibitors that are catalytic site inhibitors of the p110 isoforms and mTOR (the kinase component of both mTORC1 and mTORC2), mTOR catalytic site inhibitors, and AKT inhibitors are the most advanced in the clinic. They are approved for the treatment of several carcinomas. PMID:23768168

Cranio-orbital primary intraosseous haemangioma

PubMed Central

Gupta, T; Rose, G E; Manisali, M; Minhas, P; Uddin, J M; Verity, D H

2013-01-01

Purpose Primary intraosseous haemangioma (IOH) is a rare benign neoplasm presenting in the fourth and fifth decades of life. The spine and skull are the most commonly involved, orbital involvement is extremely rare. We describe six patients with cranio-orbital IOH, the largest case series to date. Patients and methods Retrospective review of six patients with histologically confirmed primary IOH involving the orbit. Clinical characteristics, imaging features, approach to management, and histopathological findings are described. Results Five patients were male with a median age of 56. Pain and diplopia were the most common presenting features. A characteristic ‘honeycomb' pattern on CT imaging was demonstrated in three of the cases. Complete surgical excision was performed in all cases with presurgical embolisation carried out in one case. In all the cases, histological studies identified cavernous vascular spaces within the bony tissue. These channels were lined by single layer of cytologically normal endothelial cells. Discussion IOCH of the cranio-orbital region is rare; in the absence of typical imaging features, the differential diagnosis includes chondroma, chondrosarcoma, bony metastasis, and lymphoma. Surgical excision may be necessary to exclude more sinister pathology. Intraoperative haemorrhage can be severe and may be reduced by preoperative embolisation. PMID:23989119

Management of craniofacial chondroid tumors.

PubMed

Cherekaev, Vasily A; Golbin, Denis A; Gasparyan, Tigran G; Shishkina, Lyudmila V; Tsukanova, Tatiana V

2015-01-01

Craniofacial chondroid tumors (CFCTs) constitute less than 1% of all intracranial mass lesions. No protocol for evaluation and management of CFCTs is developed at the moment. We analyzed 51 patients with CFCTs operated on in Burdenko Neurosurgical Institute from 1980 until 2012, which included chondroma (15), chondroblastoma (3), chondromyxoid fibroma (11), and chondrosarcoma (22). Age varied from 2 to 76 years (mean, 40 y); the series included 23 women and 28 men. All tumors were divided into 4 groups: midline unilateral (8),midline bilateral (21), anterolateral (19), and lateral (3). This division was based on differences in surgical approaches (P = 0.009). All patients underwent surgical treatment. Complete removal was achieved in 20; subtotal, in 21; and partial, in 10. Two patients died, and early complications were observed in 10 cases. Early outcomes correlated with the benign nature of the tumors (P = 0.002). Follow-up data were available in 22 patients. Fifteen of 51 patients were reoperated on because of recurrence (a total of 43 reoperations were performed). The mean recurrence-free period was 45 months. In 3 patients, the tumor metastasized, and malignant transformation was observed in 3 cases. Sixteen patients received postoperative radiation therapy. Delayed sequelae occurred in 5 observations, and 5 patients died during long-term follow-up. Three-year survival in benign and malignant tumors was 87.5% and 55.6%, respectively, and 5-year survival was 83.3% and 40.0%, respectively. Surgical resection is the mainstay in treatment of both benign and malignant craniofacial tumors, and adjuvant radiation therapy is mandatory in malignant lesions; however, it should be avoided in benign lesions.

Fine needle aspiration cytology in lesions of the nose, nasal cavity and paranasal sinuses.

PubMed

Gupta, Nalini; Kaur, Jasleen; Srinivasan, Radhika; Das, Ashim; Mohindra, Satyawati; Rajwanshi, Arvind; Nijhawan, Raje

2011-01-01

To assess the spectrum of lesions in the sinonasal region diagnosed on FNAC. This is a retrospective audit of sinonasal lesions diagnosed on FNAC over a period of 12 years (1998-2009). Out of a total of 79,851 FNACs, 158 (0.2%) were from the sinonasal region. FNAC was non-diagnostic in 20 (12.6%) cases. Infective/inflammatory lesions comprised of 30 (19%) cases including non-specific inflammation (19), fungal infection (7), tuberculosis (2), actinomycosis (1) and filariasis (1). Benign cysts (24; 15.2%) included epidermal inclusion cysts, mucocele and aneurysmal bone cyst. Benign bone tumors (4) comprised of giant cell tumor, fibrous dysplasia, chondroma, and osteoblastoma. Other benign tumors included lipoma (6), hemangioma (5), schwannoma (2), meningioma (1), pleomorphic adenoma (1), sebaceous adenoma (1) and other skin adnexal tumors (3). Malignant epithelial tumors (24; 15.2%) included squamous cell carcinoma (10), basal cell carcinoma (5), poorly differentiated carcinoma (4) and metastatic carcinoma (5). Two cases of chordoma and one case each of dermatofibrosarcoma pertuberance and hemangiopericytoma were seen. Sarcomas included sarcoma, not otherwise specified (4), rhabdomyosarcoma (3), osteosarcoma (2), chondrosarcoma (2), leiomyosarcoma (1), malignant fibrous histiocytoma (1), fibrosarcoma (1) and malignant peripheral nerve sheath tumor (1). There were cases of malignant small round cell tumor (11), non-Hodgkin lymphoma (3), plasmacytoma (2) and malignant melanoma (2). A variety of non-neoplastic and neoplastic conditions can involve the sinonasal region. FNAC is a reliable diagnostic procedure in a good number of cases, especially in the light of clinico-radiological data. Copyright © 2011 S. Karger AG, Basel.

The role of metabolic enzymes in mesenchymal tumors and tumor syndromes: genetics, pathology, and molecular mechanisms.

PubMed

Schaefer, Inga-Marie; Hornick, Jason L; Bovée, Judith V M G

2018-04-01

The discovery of mutations in genes encoding the metabolic enzymes isocitrate dehydrogenase (IDH), succinate dehydrogenase (SDH), and fumarate hydratase (FH) has expanded our understanding not only of altered metabolic pathways but also epigenetic dysregulation in cancer. IDH1/2 mutations occur in enchondromas and chondrosarcomas in patients with the non-hereditary enchondromatosis syndromes Ollier disease and Maffucci syndrome and in sporadic tumors. IDH1/2 mutations result in excess production of the oncometabolite (D)-2-hydroxyglutarate. In contrast, SDH and FH act as tumor suppressors and genomic inactivation results in succinate and fumarate accumulation, respectively. SDH deficiency may result from germline SDHA, SDHB, SDHC, or SDHD mutations and is found in autosomal-dominant familial paraganglioma/pheochromocytoma and Carney-Stratakis syndrome, describing the combination of paraganglioma and gastrointestinal stromal tumor (GIST). In contrast, patients with the non-hereditary Carney triad, including paraganglioma, GIST, and pulmonary chondroma, usually lack germline SDH mutations and instead show epigenetic SDH complex inactivation through SDHC promoter methylation. Inactivating FH germline mutations are found in patients with hereditary leiomyomatosis and renal cell cancer (HLRCC) syndrome comprising benign cutaneous/uterine leiomyomas and renal cell carcinoma. Mutant IDH, SDH, and FH share common inhibition of α-ketoglutarate-dependent oxygenases such as the TET family of 5-methylcytosine hydroxylases preventing DNA demethylation, and Jumonji domain histone demethylases increasing histone methylation, which together inhibit cell differentiation. Ongoing studies aim to better characterize these complex alterations in cancer, the different clinical phenotypes, and variable penetrance of inherited and sporadic cancer predisposition syndromes. A better understanding of the roles of metabolic enzymes in cancer may foster the development of therapies that specifically target functional alterations in tumor cells in the future. Here, the physiologic functions of these metabolic enzymes, the mutational spectrum, and associated functional alterations will be discussed, with a focus on mesenchymal tumor predisposition syndromes.

[Epidemiology of lung tumors].

PubMed

Ott, S; Geiser, T

2012-07-01

Approximately one out of 500 chest radiographs shows the incidental finding of a solitary pulmonary nodule and almost one half of these pulmonary lesions are caused by a tumor. Unfortunately, only 2% to 5% of all lung tumors are of benign origin, e. g. lipoma, fibroma, hamartoma, and chondroma, and the majority are malignant neoplasms, most commonly primary lung cancer followed by metastases of extrapulmonary primary carcinomas. Thus, a careful diagnostic work up of solitary pulmonary nodules, including histological diagnosis, is mandatory for an adequate management and treatment of patients with pulmonary lesions. Despite all recent improvements of treatment modalities, lung cancer continues to be a major cause of morbidity and mortality among malignant diseases worldwide. The prognosis of affected patients is still very poor and a 5-years survival rate of only 14% makes lung cancer the number one cause of death due to cancer in Switzerland. Active and passive tobacco smoking are by far the best known risk factor for the development of lung cancer, but there are severe other probably less known factors that may increase the individual risk for malignant neoplasms of the lung. These risk factors include e. g. exposure to natural ionic radiation, consisting of terrestrial radiation and indoor radiation caused by radon gas, exposure to respirable dust and Diesel engine emissions, asbestos, and polycyclic aromatic hydrocarbons. In the majority of cases, the latency between exposure and development of cancer is years to decades and the person concerned was occupationally exposed. Therefore, a detailed evaluation of a patient's medical and occupational history is needed. Due to its poor prognosis, prevention and early diagnosis of lung cancer is crucial to improve our patients' outcome. Good knowledge of epidemiology and aetiology of pulmonary tumors is the key to preventive measures and identification of individuals at increased risk for lung cancer. An overview will be provided on the epidemiology of lung tumors and predominantly preventable risk factors for lung cancer.