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Sample records for mediastinal cystic lymphangioma

  1. [Retroperitoneal cystic lymphangioma].

    PubMed

    Waisberg, J; Pezzolo, S; Henrique, A C; Kerr, L M; Speranzini, M B

    1999-01-01

    The lymphangioma is a rare disease, more frequently reported in children and just occasionally in the adult patient. The lymphangioma is considered a benign neoplasm of embryonic origin of the lymphatic vessels. Its habitual location is in the cervical and axillary area; it is rarely found in the abdominal cavity and exceptionally in the retroperitonio. In this latter location, the lesion habitually is asymptomatic. The clinical diagnosis of the retroperitoneal cystic lymphangioma is not often due to its rarity and the absence of clinical expression. The size of the lesion is more important than its location to the symptomatology development. The findings of the abdominal ultrasonography and computerized tomography of the abdomen usually show a cystic lesion and its location. The treatment is surgical and it consists of the resection of the cyst or group of cysts once the liquid accumulation in its interior may be responsible for the development of some important complications of this disease. The cure is obtained when the lesion is completely resected also with the resection of eventual adhesive structures. The relapse may take place when the resection is incomplete. A case of retroperitoneal lymphangioma in a female adult patient as incidental finding of abdominal ultra-sonography is described. It is discussed the clinical picture, the radiologic diagnosis, the treatment and the prognostic of this unusual disease.

  2. Retroperitoneal cystic lymphangioma.

    PubMed

    Nuzzo, G; Lemmo, G; Marrocco-Trischitta, M M; Boldrini, G; Giovannini, I

    1996-03-01

    Two cases of retroperitoneal cystic lymphangioma (CL) are presented; the current literature on this rare, benign neoplasm of the lymphatic system is reviewed. This tumor consists of various numbers of cyst-like cavities filled with a serous, serosanguineous or chylous fluid. The histogenesis of CL is still uncertain. Most commonly CL occurs in the neck and in the axillary region, whereas it is rare in the retroperitoneum. Although retroperitoneal CL is a benign lesion, it may cause significant morbidity due to its large size, and its often invasive character with a strong tendency to secondary infection. The treatment of choice is surgical excision.

  3. Congenital cystic choristoma mimicking cervical lymphangioma.

    PubMed

    Moon, Suk-Bae; Park, Kwi-Won; Yun, Woong-Jae; Patten, Phillip P; Jung, Sung-Eun

    2008-09-01

    Choristoma is a tissue or mass with a normal histology at an abnormal location, and cystic choristoma has rarely been reported in the head and neck region. Cervical cystic masses in neonates are usually diagnosed as cystic lymphangioma. The authors present a case of a congenital laterocervical cystic mass that appeared to be lymphangioma, but which turned out to be a cystic choristoma.

  4. Mesenteric cystic lymphangioma mimicking malignancy.

    PubMed

    Hureibi, Khalid; Sunidar, Osama A

    2014-01-01

    Mesenteric cystic lymphangiomas are benign tumours arising from the mesentery, and have no known aetiology. Patients might be discovered incidentally to have asymptomatic mesenteric cysts, or they can present with symptoms such as pain, nausea and vomiting. A 27-year-old man presented with vague abdominal pain, loss of appetite, postprandial fullness and significant weight loss. There was no lymphadenopathy, and abdominal examination was unremarkable. CT showed a mesenteric mass and a diagnosis of abdominal lymphoma was suggested. There was no evidence of pulmonary tuberculosis on chest X-ray and the purified protein derivative test was negative. On laparotomy, a 5×9×7 cm sessile cyst containing thick white fluid and arising from the ileal mesentery was found and completely removed. Histopathology proved a diagnosis of mesenteric cystic lymphangioma. The patient made uneventful recovery, and was asymptomatic on clinical follow-up after 6 weeks. PMID:25178885

  5. Mesenteric cystic lymphangioma mimicking malignancy

    PubMed Central

    Hureibi, Khalid; Sunidar, Osama A

    2014-01-01

    Mesenteric cystic lymphangiomas are benign tumours arising from the mesentery, and have no known aetiology. Patients might be discovered incidentally to have asymptomatic mesenteric cysts, or they can present with symptoms such as pain, nausea and vomiting. A 27-year-old man presented with vague abdominal pain, loss of appetite, postprandial fullness and significant weight loss. There was no lymphadenopathy, and abdominal examination was unremarkable. CT showed a mesenteric mass and a diagnosis of abdominal lymphoma was suggested. There was no evidence of pulmonary tuberculosis on chest X-ray and the purified protein derivative test was negative. On laparotomy, a 5×9×7 cm sessile cyst containing thick white fluid and arising from the ileal mesentery was found and completely removed. Histopathology proved a diagnosis of mesenteric cystic lymphangioma. The patient made uneventful recovery, and was asymptomatic on clinical follow-up after 6 weeks. PMID:25178885

  6. Laparoscopic observation of retroperitoneal cystic lymphangioma.

    PubMed

    Takeuchi, Y; Fujinami, S; Kitagawa, S; Shiro, T; Fujii, T; Fukui, Y; Kawashima, Y; Kubota, Y; Shiozaki, Y; Inoue, K

    1994-01-01

    A case of retroperitoneal cystic lymphangioma observed laparoscopically is reported. In a 60 year old asymptomatic male patient, an 8 cm multi-loculated cystic lesion was detected incidentally near the splenic hilum with ultrasonography. Endosonography, computed tomography and magnetic resonance imaging revealed the lesion with thin wall and clear fluid. Laparoscopy showed a thin-walled cyst with smooth surface, and straw-coloured clear fluid was observed through the wall. These findings suggested benign aetiology, and seemed to be characteristic of cystic lymphangioma. The tumour was resected, and microscopic examination showed proliferated lymph channels intespersed by lymph follicles. Diagnosis of cystic lymphangioma was established. Laparoscopy seems a useful pre-operative method.

  7. Chronic Low Back Pain due to Retroperitoneal Cystic Lymphangioma.

    PubMed

    Fattahi, Asieh Sadat; Maddah, Godratollah; Motamedolshariati, Mohammad; Ghiasi-Moghadam, Taghi

    2014-03-01

    Abdominal cystic lymphangioma is a rare benign neoplasm. Less than 1% of lymphangiomas is in the retroperitoneum. Lymphangioma is mostly asymptomatic. Chronic symptoms were reported in retroperitoneal type more than others. Acute symptoms due to complications like infection, cyst rupture or hemorrhage may occur. We report an 18-years-old girl with low back pain from 6 months ago with huge pelvic mass and diagnosis of retroperitoneal cystic lymphangioma.

  8. [A case of retroperitoneal cystic lymphangioma].

    PubMed

    Perini, F; Metelli, F; Bazzana, M

    1979-06-30

    A case of retroperitoneal cystic lymphangioma in a 62-yr-old woman presented as symptom-free abdominal swelling. The intraoperative diagnosis was confirmed histologically. Radical management consisted of stripping of the cyst from its adjacent structures. The postoperative course was uneventful.

  9. Cystic retroperitoneal lymphangioma in infants and children.

    PubMed

    Leonidas, J C; Brill, P W; Bhan, I; Smith, T H

    1978-04-01

    Retroperitoneal lymphangiomas are uncommon benign tumors usually presenting in early life. They may cause significant pressure on adjacent structures, most often with superior and medial displacement of the kidney and medial deviation of the ureter. Relative radiolucency on total body opacification during excretory urography, and features of a cystic structure on ultrasonography and computed tomography should further suggest the possibility of a retroperitoneal lymphangioma. Vascular stretching and displacement without neovascularity on angiography and contrast entry into the cysts on lower extremity lymphography are additional features, but such procedures are usually unnecessary.

  10. Mesenteric calcified cystic lymphangioma in an adult patient.

    PubMed

    Albayrak, Yavuz; Albayrak, Fatih; Arslan, Serdar; Calik, Ilknur

    2011-06-01

    Abdominal cystic lymphangiomas are rare congenital benign malformations of the lymphatic system. To the best of our knowledge, only 6 mesenteric calcified cystic lymphangiomas have ever been reported. We herein describe a woman who presented to our hospital with stomachache that had been continuous for approximately 8 months. An abdominal computed tomography showed a cystic lesion. In the exploration, the cyst was totally excised. Based on the histomorphological data, a case of "calcified cystic lymphangioma" was diagnosed. Although mesenteric lymphangiomas are rare, especially in adults, they should be considered as a possible cause of abdominal pain. Treatment is surgical with resection of the mass, sometimes including resection of adjacent bowel.

  11. [Retroperitoneal cystic mesothelioma and lymphangioma].

    PubMed

    Segura Martín, M; Lorenzo Romero, J G; Hernández Millán, I; Pastor Guzmán, J M; Salinas Sánchez, A S; Ruiz Mondéjar, R; Virseda Rodríguez, J A

    1998-03-01

    Retroperitoneal cysts are uncommon entities of difficult diagnosis because of their insidious symptomatology. Urinary apparatus involvement is quite often the mode of presentation and the reason for calling on the urologist. The origin of many of these retroperitoneal cysts remains practically unknown. Surgery with exeresis is the choice management method. Follow-up is necessary for cystic mesothelioma because of the highly frequent relapses. The outlook of hormonal conservative therapy for relapses appears as a future alternative to treatment.

  12. [Retroperitoneal cystic lymphangioma. Preoperative diagnosis. Apropos of a case].

    PubMed

    Guérin, E; Babin, C; Moulle, P; Barret, F

    1987-11-01

    Retroperitoneal cystic lymphangioma is an uncommon tumor. One case is reported, with surgical confirmation. Authors point out the diagnostic value of ultrasonography and computed tomography in the pre-operative diagnosis.

  13. Rare Case of Ovarian Cystic Lymphangioma Managed at Laparoscopy.

    PubMed

    Naik, Sejal Arunbhai

    2011-01-01

    Lymphangiomas are rare, usually benign tumors of the lymphatic system. Lymphangiomas may arise in any part of the body. Lymphangioma of the ovary is very rare tumor, which is usually slow-growing, remains asymptomatic for a long time, and identified incidentally at histopathological examination after excision. It is advisable to excise the lesion with microscopically clear margins. Many times, diagnosis becomes difficult because of confusion with malignant ovarian mass, especially in post-menopausal woman. A 72-year-old post-menopausal woman had a symptomatic pelvic mass. Imaging studies demonstrated a complex right ovarian cyst. Laparoscopic total hysterectomy with bilateral salpingo-oophorectomy was performed successfully. Cystic Lymphangioma should be included in the differential diagnosis of an ovarian cystic mass, and laparoscopic excision may be the method of treatment. PMID:26085753

  14. Rare Case of Ovarian Cystic Lymphangioma Managed at Laparoscopy

    PubMed Central

    Naik, Sejal Arunbhai

    2011-01-01

    Lymphangiomas are rare, usually benign tumors of the lymphatic system. Lymphangiomas may arise in any part of the body. Lymphangioma of the ovary is very rare tumor, which is usually slow-growing, remains asymptomatic for a long time, and identified incidentally at histopathological examination after excision. It is advisable to excise the lesion with microscopically clear margins. Many times, diagnosis becomes difficult because of confusion with malignant ovarian mass, especially in post-menopausal woman. A 72-year-old post-menopausal woman had a symptomatic pelvic mass. Imaging studies demonstrated a complex right ovarian cyst. Laparoscopic total hysterectomy with bilateral salpingo-oophorectomy was performed successfully. Cystic Lymphangioma should be included in the differential diagnosis of an ovarian cystic mass, and laparoscopic excision may be the method of treatment. PMID:26085753

  15. Laparoscopic resection of a retroperitoneal cystic lymphangioma in an adult.

    PubMed

    Kasza, Jason; Brody, Fredrick J; Khambaty, Fatima; Vaziri, Khashayar; Wallace, Brian

    2010-06-01

    Cystic lymphangiomas are rare, benign anomalies of the lymphatic system. More than 95% of cystic lymphangiomas occur in the head, neck, and axilla with only 1% in the retroperitoneum. Most of these cases are diagnosed by the second year of life with only a handful of adult cases. Once a symptomatic cystic lesion of the abdomen or retroperitoneum is diagnosed, treatment usually consists of surgical excision. Traditionally, surgery requires a laparotomy. This paper describes a patient with a retroperitoneal cyst who underwent a successful laparoscopic resection. The etiology and management of adult retroperitoneal cysts are reviewed as well.

  16. Computed tomography of retroperitoneal cystic lymphangiomas.

    PubMed

    Munechika, H; Honda, M; Kushihashi, T; Koizumi, K; Gokan, T

    1987-01-01

    The CT features of retroperitoneal lymphangiomas are unilocular or multilocular cysts in the retroperitoneum and slightly and uniformly thickened wall that enhances following contrast medium administration. Three cases are reported.

  17. Mesenteric cysts and intra-abdominal cystic lymphangiomas.

    PubMed

    Takiff, H; Calabria, R; Yin, L; Stabile, B E

    1985-11-01

    Although mesenteric cysts and intra-abdominal cystic lymphangiomas are uncommon and clinically confusing lesions, histologic and ultrastructural evidence suggests that they are pathologically distinct. Differentiation of these lesions is important since lymphangiomas may follow a proliferative and invasive course. Of 28 cases documented at laparotomy, histologically eight patients (29%) had cystic lymphangiomas and 20 patients (71%) had mesenteric cysts. Lymphangioma was found to be exclusively a disease of childhood and young adulthood (mean age, 10 years); mesenteric cyst was found in all age groups (mean age, 44 years), and two thirds of these patients were over 40 years old. Patients with lymphangiomas more frequently were male (75% vs 30%), symptomatic (88% vs 35%), and had ascites (50% vs 0%) and larger lesions (mean, 8.8 vs 4.7 cm) when compared with patients with mesenteric cysts. Complete excision was possible in all but four patients, with no operative deaths and a postoperative complication rate of 7%. After a mean follow-up period of four years, there were no recurrences among 16 patients who had undergone complete excision.

  18. Diagnosis of cystic lymphangioma of the colon by endoscopic ultrasound: Biopsy is not needed!

    PubMed Central

    Bhutani, Manoop S.; Annangi, Srinadh; Koduru, Pramoda; Aggarwal, Aakash; Suzuki, Rei

    2016-01-01

    Cystic lymphangioma of the colon (CLC) is a rare benign lesion that is usually asymptomatic and found incidentally during colonoscopy. Limitations in the conventional noninvasive diagnostic techniques have led to surgical resection of these lesions for diagnostic confirmation. Classic endoscopic ultrasound (EUS) findings of colonic cystic lymphangioma are submucosal anechoic cystic spaces with septations, intact muscularis propria, and no solid component. Patients who are asymptomatic with lesions having classic appearance as cystic lymphangioma with EUS can be observed without any intervention. We herein report a case of cystic lymphangioma of distal transverse colon in an asymptomatic patient diagnosed noninvasively using 20-MHz miniprobe EUS and managed conservatively without any surgical intervention. PMID:27803907

  19. Infected retroperitoneal cystic lymphangioma masquerading as psoas abscess.

    PubMed

    Pratap, Akshay; Tiwari, Awadhesh; Sah, Birendra Prasad; Sinha, Arvind Kumar; Shakya, Vikal Chandra; Niels, Koopmans G

    2008-01-01

    A 2-year-old male is described who presented with fever, fixed flexion deformity of the right hip and a tender mass in the right iliac fossa. A clinical diagnosis of psoas abscess was made. Abdominal ultrasound was suggestive of an echogenic retroperitoneal cyst but could not accurately determine the nature of the cyst. A contrast CT scan showed a retroperitoneal cyst. At laparotomy, a large retroperitoneal cyst containing turbid fluid was found and was excised completely. Histopathological examination was suggestive of cystic lymphangioma. Clinical presentation of retroperitoneal lymphangioma may be variable and misleading, therefore complex imaging studies are necessary in the evaluation of this condition.

  20. Retroperitoneal cystic lymphangioma in an adult: A case report and review of the literature.

    PubMed

    Bhavsar, Tapan; Saeed-Vafa, Daryoush; Harbison, Sean; Inniss, Susan

    2010-12-15

    Lymphangiomas are rare benign cystic tumors of the lymphatic system. Retroperitoneal lymphangiomas account for 1% of all lymphangiomas, and approximately 186 cases have been reported. They may clinically present as a palpable abdominal mass and can cause diagnostic dilemmas with other retroperitoneal cystic tumors, including those arising from the liver, kidney and pancreas. This report describes the rare case of a cystic retroperitoneal lymphangioma in a 54-year-old male patient. The lymphangioma had progressed to the point of inducing clinical symptoms of abdominal distention, abdominal pain, anorexia, fever, nausea and diarrhea. Radiological imaging revealed a large multiloculated cystic abdominal mass with enhancing septations involving the upper retroperitoneum and extending into the pelvis. Surgical removal of the cyst was accomplished without incident. A benign cystic retroperitoneal lymphangioma was diagnosed on histology and confirmed with immunohistochemical stains.

  1. Retroperitoneal cystic lymphangioma: a rare presentation in childhood, treated laparoscopically.

    PubMed

    Singh, Rashmi R; Govindarajan, Krishna K; Bowen, Claire; Chandran, Harish

    2009-04-01

    Retroperitoneal cystic lymphangioma is a rare benign lesion of childhood. A 15-year-old girl underwent laparoscopic excision of such a cyst. On histopathological examination, the resection was complete. She had an uneventful recovery and remains asymptomatic. We report this in view of the rarity of this condition and also the unusual presentation of this case. Complete surgical excision via laparoscopy is a feasible option.

  2. Identification and surgical management of cystic retroperitoneal lymphangioma in children.

    PubMed

    Waldhausen, J H; Holterman, M J; Tapper, D

    1996-04-01

    Retroperitoneal lymphangiomas are rare, benign cystic structures that are best evaluated with computed tomography and ultrasound. Preoperative diagnosis is often difficult, in part because there is little to distinguish them from other cystic masses and because the lesion is often not considered on the differential diagnosis. Surgery may be required as both a diagnostic and therapeutic measure. The cysts may be asymptomatic for years and then present because of compression of surrounding structures or pain. The treatment is as complete surgical excision as is possible. Bowel cleansing should be done preoperatively. The long-term results are excellent when total excision or near-total excision with marsupialization is accomplished.

  3. [Retroperitoneal cystic lymphangiomas in adults: four case reports and review of the literature].

    PubMed

    Tohmé, Cyril; Ata, Toufic; Ghorra, Claude; Noun, Roger; Abboud, Bassam; Sarkis, Riad

    2008-01-01

    Retroperitoneal location of cystic lymphangiomas in adult patients is rare. Their clinical presentation is not specific. Magnetic resonance imaging is the best radiological exam for the diagnosis. These tumors must be distinguished from mesenteric cysts which are more frequent and can degenerate. The authors report four cases of retroperitoneal cystic lymphangioma with a literature review.

  4. A Case of Laparoscopic Excision of a Huge Retroperitoneal Cystic Lymphangioma

    PubMed Central

    Yagihashi, Yusuke; Kato, Keiji; Nagahama, Kanji; Yamamoto, Masakazu; Kanamaru, Hiroshi

    2011-01-01

    Retroperitoneal cystic lymphangioma is a rare benign tumor. Most patients eventually experience some symptoms that necessitate therapeutic intervention. Excision is the treatment of choice, and some cases of laparoscopic resection have been reported. We report another case of a huge retroperitoneal cystic lymphangioma that was successfully excised laparoscopically with the SAND balloon catheter. Large cystic lymphangioma was downsized by puncturing and aspirated with the SAND balloon catheter. Laparoscopic surgical technique should be considered for treatment of selected cystic lesions of retroperitoneal origin. PMID:22606623

  5. Retroperitoneal cystic lymphangioma: a diagnostic and surgical challenge.

    PubMed

    Gümüştaş, Oguzhan Güven; Sanal, Murat; Güner, Osman; Tümay, Volkan

    2013-01-01

    A lymphangioma is a benign proliferation of lymph vessels, producing fluid-filled cysts that result from a blockage of the lymphatic system. The incidence of abdominal lymphangiomas is unknown; however they account for from 3% to 9.2% of all pediatric lymphangiomas, with retroperitoneal lymphangioma representing less than 1% of abdominal lymphangiomas. Due to rarity, preoperative diagnosis is often difficult.

  6. Cystic lymphangioma of the inguinal and scrotal regions in childhood - report of three cases

    PubMed Central

    Patoulias, I; Prodromou, K; Feidantsis, Th; Kallergis, I; Koutsoumis, G

    2014-01-01

    Backround: Cystic lymphangiomas are congenital lymphatic malformations that most commonly develop in the neck, axilla, mediastinum and retroperitoneum. Inguinal and scrotal lymphangiomas are extremely rare. Cases report: We present the cases of three children with cystic lymphangiomas that were treated in our department during a two year period. The patients were all boys, aged 3.5, 9 and 13 years, and the location of the cystic lymphangioma was the scrotum, the inguinal region and the epididymis respectively. Clinical examination and ultrasonography described the lesions as cystic. Surgical excision of the lesions with a testis-sparing approach was performed in all three cases and histopathology set the diagnosis of cystic lymphangiomas. Complementary imaging of the regions adjacent to the excised lesions, excluded any extension or co-existing lesions. Post-operative period was uneventful and during a follow-up period of six months all patients were well with no signs of recurrence. Conclusions: Scrotal and inguinal cystic lymphangiomas are treated with surgical excision with care to preserve the intra-scrotal structures and the structures of the inguinal canal. Complete excision is necessary to prevent recurrence. Complementary imaging of the adjacent regions is necessary to identify any possible extension or co-existing lesions. PMID:25125963

  7. Percutaneous Sclerotherapy With OK-432 of a Cervicomediastinal Lymphangioma.

    PubMed

    Golinelli, Gloria; Toso, Andrea; Borello, Giovanni; Aluffi, Paolo; Pia, Francesco

    2015-11-01

    The present study reports a case of percutaneous sclerotherapy of a giant cystic cervicomediastinal lymphangioma using OK-432. To the best of our knowledge, percutaneous sclerotherapy of a mediastinal lymphangioma using OK 432 has not previously been reported in the English literature.

  8. Retroperitoneal lymphangioma.

    PubMed

    Cherk, Martin; Nikfarjam, Mehrdad; Christophi, Christopher

    2006-01-01

    Lymphangiomas are rare cystic tumours that may present as cystic masses in the retroperitoneum. Retroperitoneal lymphangiomas account for approximately 1% of all lymphangiomas. Confusion with other cystic tumours of the retroperitoneum including those arising from the liver, kidney and pancreas is common. A case of a retroperitoneal cystic lymphangioma occurring in a 41-year-old woman raising interesting diagnostic and management issues is reported. The patient presented with vague abdominal pain and persistent nausea. Radiological imaging demonstrated a large multiloculated thin-walled cyst involving the upper retroperitoneum. Surgical resection was complete, revealing a benign cavernous lymphangioma. The patient made a complete recovery and was disease-free 30 months postoperatively.

  9. Abdominal cystic lymphangiomas in children: presurgical evaluation with imaging.

    PubMed

    Chateil, J-F; Brun, M; Vergnes, P; Andrieu de Lewis, P; Pérel, Y; Diard, F

    2002-02-01

    Cystic lymphangiomas are benign vascular tumors which are most often seen in young children. They are considered to be congenital malformations stemming from sequestration of lymphatic tissue. The authors report 15 cases of abdominal location and detail the findings of imaging in the etiologic and topographic diagnosis of these lesions. The initial incidents were essentially the discovery of a palpable abdominal mass, and more rarely, an acute gastrointestinal complication. There was also one case of prenatal diagnosis. Plain films provide only indirect signs related to the displacement of neighboring organs. Ultrasonography permits the etiologic diagnosis by showing an often voluminous, septated cyst. The intra- or retroperitoneal location of the lesion is sometimes difficult to determine by sonography, in which case CT scanning is usually adequate. For abdominal locations, percutaneous sclerosis is not available, and surgical removal is the only treatment for this disorder. The topography of the lesion and the involvement of retroperitoneal structures is important to determine.

  10. OK432 (picibanil) efficacy in an adult with cystic cervical lymphangioma. A case report.

    PubMed

    Alonso, Juan; Barbier, Luis; Alvarez, Julio; Romo, Laura; Martín, Jesús C; Arteagoitia, Iciar; Santamaría, Joseba

    2005-01-01

    Cervical cystic lymphangioma (CCL) is a rare and benign tumour involving congenital and cystic abnormalities derived from lymphatic vessels. The most accepted treatment continues to be surgical excision. However, when this infiltrates vital neurovascular neck structures, complete excision is difficult and if only partial, the recurrence rate is very high. The most frequently used alternative treatment is to inject sclerosants into the lesion. The use of these techniques has reported good results in children; however, there are few references thereof with regard to adults. We are reporting on a cervical cystic lymphangioma in a male aged 22, treated with an intra-lesion injection of 20 cc with 0.01 mg/cc dilution of OK-432 (picibanil) in physiological serum. Sole complications were fever and local reaction where the solution was injected. One month after treatment the lymphangioma had totally remitted and sixteen months later continues in remittance.

  11. Laparoscopic excision of an adult retroperitoneal cystic lymphangioma coexisting with an esophageal hiatus hernia.

    PubMed

    Izumi, Daisuke; Toyama, Eiichiro; Shigaki, Hironobu; Iwagami, Shiro; Baba, Yoshifumi; Hayashi, Naoko; Watanabe, Masayuki; Baba, Hideo

    2015-06-01

    Lymphangiomas are rare cystic tumors that may be present in the retroperitoneum. Retroperitoneal lymphangiomas account for approximately 1% of all lymphangiomas. We experienced an adult patient with a retroperitoneal cystic lymphangioma coexisting with an esophageal hiatus hernia. Abdominal ultrasonography showed a cyst in the abdomen of a 76-year-old woman. She was admitted to our clinic because of an enlargement of the cyst and epigastric pain after meals. She had a long history of heartburn after meals and had not undergone any treatment. She had no record of previous illnesses. Computed tomography revealed a single bunch cystic tumor with septations, located from the mediastinum to the retroperitoneal space. The tumor was not enhanced, and there was no solid part. She was diagnosed with a retroperitoneal cyst. Laparoscopic total excision was performed because the cyst was increasing in size and the patient had symptoms. The cyst was successfully removed. Although laparoscopic excision of retroperitoneal cystic lymphangiomas is the treatment of choice, surgical methods should be carefully chosen in selected patients. PMID:25998607

  12. Laparoscopic excision of an adult retroperitoneal cystic lymphangioma coexisting with an esophageal hiatus hernia.

    PubMed

    Izumi, Daisuke; Toyama, Eiichiro; Shigaki, Hironobu; Iwagami, Shiro; Baba, Yoshifumi; Hayashi, Naoko; Watanabe, Masayuki; Baba, Hideo

    2015-06-01

    Lymphangiomas are rare cystic tumors that may be present in the retroperitoneum. Retroperitoneal lymphangiomas account for approximately 1% of all lymphangiomas. We experienced an adult patient with a retroperitoneal cystic lymphangioma coexisting with an esophageal hiatus hernia. Abdominal ultrasonography showed a cyst in the abdomen of a 76-year-old woman. She was admitted to our clinic because of an enlargement of the cyst and epigastric pain after meals. She had a long history of heartburn after meals and had not undergone any treatment. She had no record of previous illnesses. Computed tomography revealed a single bunch cystic tumor with septations, located from the mediastinum to the retroperitoneal space. The tumor was not enhanced, and there was no solid part. She was diagnosed with a retroperitoneal cyst. Laparoscopic total excision was performed because the cyst was increasing in size and the patient had symptoms. The cyst was successfully removed. Although laparoscopic excision of retroperitoneal cystic lymphangiomas is the treatment of choice, surgical methods should be carefully chosen in selected patients.

  13. Retroperitoneal cystic lymphangioma section imaging in two cases, and review of the literature.

    PubMed

    Feldberg, M A; Hendriks, A V; Van Leeuwen, M S; Witkamp, T D; Obertop, H

    1990-03-01

    Two patients with retroperitoneal cystic lymphangioma are reported with a review of the medical literature. In one, the patient was asymptomatic from the lesion but had a computed tomography (CT) and an ultrasound to evaluate the extent of disease with a known carcinoma of the colon. The second patient presented with an acute abdominal pain requiring surgical management. CT on this patient showed multiple cystlike cavities filled with fluid, in a retroperitoneal location. The preoperative diagnosis of retroperitoneal cystic lymphangioma was confirmed at surgery. Magnetic resonance (MR) performed on the second patient gave better delineation of craniocaudal extent and showed one of the cysts to have probable hemorrhage.

  14. Giant cystic lymphangioma originating from the cardia of the stomach: A case report

    PubMed Central

    CHEN, GENG; LIU, MINGQING; MALIK, TAYYAB HAMID; LI, SHOUYING; TANG, YING; XU, HONG

    2016-01-01

    Cystic lymphangiomas are rare benign tumors involving the neck, head, and axilla, and most frequently occur in children before the age of 5 years. In the present study, the case of a giant cystic lymphangioma originating in the cardia of the stomach was reported in an 18-year-old female complaining of abdominal distention. Contrast-enhanced computed tomography and endoscopic ultrasonography revealed a large, multilobulated cystic mass located between the cardia and esophagogastric junction with a diameter of 4.0 cm. The lesion was successfully removed by endoscopic submucosal dissection. Subsequent immunohistochemical analysis of the lymphatic endothelium-specific O-linked sialoglycoprotein D2-40 confirmed the diagnosis of cystic lymphangioma. No complications associated with the tumor dissection occurred, and the patient did not report any further complaints or any signs of recurrence at 6- and 18-month follow-up. The present case demonstrates that a diagnosis of cystic lymphangioma should be considered in non-pediatric patients suffering aspecific abdominal complaints. PMID:27168832

  15. An Interesting Association of Cystic Hygroma of the Neck and Lymphangioma Causing a Paediatric Swollen Tongue.

    PubMed

    Beech, A N; Farrier, J N

    2016-01-01

    Up to 75% of lymphatic malformations occur in the head and neck region. Of these, cystic hygromas and lymphangiomas have been widely reported; however they rarely occur in the same patient. We report the case of a 5-year-old girl who presented to the Department of Paediatrics of a district general hospital with a short history of recurrent, painful swelling of the anterior one-third of her tongue. She was reviewed under the joint care of the Oral and Maxillofacial Surgery and Otolaryngology Teams. Relevant past medical history included a previously excised cystic hygroma from her right neck when she was aged 2 years. Diagnosis of lymphangioma was made and of the potential management options available active monitoring was favoured due to the patient's age. To our knowledge the occurrence of both tongue lymphangioma and cystic hygroma has not been previously reported in a paediatric patient. This case report therefore shows a rare association between a cystic hygroma of the neck and lymphangioma of the tongue. PMID:27069707

  16. Intraabdominal cystic lymphangiomas obscured by marked superimposed reactive changes: clinicopathological analysis of a series.

    PubMed

    Hornick, Jason L; Fletcher, Christopher D M

    2005-04-01

    Cystic (or cavernous) lymphangiomas are uncommon tumors that most often occur in the head and neck, axilla, or groin of young children but are detected occasionally in adulthood at various other anatomic sites. When arising in the abdomen, cystic lymphangiomas may present with acute abdominal pain. We have encountered examples of mesenteric and retroperitoneal cystic lymphangiomas associated with such marked superimposed reactive and inflammatory changes that their lymphatic nature is obscured, a situation that is not widely recognized. To further characterize these lesions, 7 abdominal lymphangiomas associated with florid reactive changes were retrieved from the authors' consultation files. There were 5 female patients and 2 male patients (median age, 42 years; range, 1 month to 51 years). Five cases presented in adulthood. Tumor size ranged from 8 to 20 cm (median, 15 cm). Three tumors arose in the mesentery of the small intestine and 4 arose in the retroperitoneum (one of which also involved the posterior mediastinum). Three patients presented with a short history of abdominal pain. Radiological studies revealed large cystic or solid masses; clinical differential diagnoses included sarcoma (2 cases), enteric duplication cyst (2 cases), and cystic tumor not otherwise specified. Grossly, the tumors were generally multiloculated cystic masses associated with areas of fat necrosis and hemorrhage. The cysts often contained thick, gelatinous, or milky fluid. Histologically, all cases showed extensive areas of granulation tissue, most also including a floridly cellular reactive myofibroblastic proliferation, obscuring the lymphatic nature of the lesion. Two cases contained extensive areas of xanthogranulomatous inflammation. In foci where the underlying lesion could be discerned, the tumors were composed of cystically dilated lymphatic spaces, some of which were partially invested by a layer of smooth muscle and were associated with occasional lymphoid aggregates. The

  17. Cystic lymphangioma of adrenal gland: a clinicopathological study of 3 cases and review of literature

    PubMed Central

    Zhao, Ming; Gu, Qianfeng; Li, Changshui; Yu, Jingjing; Qi, Honggang

    2014-01-01

    Cystic lymphangioma of the adrenal gland is a rare and benign lesion, most often found incidentally during abdominal imaging studies, abdominal surgery or at autopsy. We aimed to retrospectively review all adrenal lymphangioma cases at our hospital, further document their lymphatic origin by immunohistochemical staining and discuss the differential diagnosis with other cystic adrenal gland lesions. A total of 3 adrenal lymphangioma cases were identified. All three patients were men and adults at time of diagnosis aged 41 years, 43 years, and 66 years, respectively. All were incidentally identified during investigating for unrelated reasons, two of which were discovered by routine radiologic check-up while the last one was found during imaging detection of ureteral cancer. The average size of an adrenal lymphangioma lesion was 3.2 cm (range, 2.5-4.6 cm). Histologically, all three cases showed a typical multicystic architecture with dilated spaces lined by flattened, bland, simple lining. The cystic spaces occasionally contained proteinaceous material but lacked red blood cell content. On immunohistochemical stains, D2-40 cytoplasmic staining was positive in all three lesions, whereas AE1/AE3 was negative, thus, confirming their lymphatic nature. PMID:25197378

  18. [The diagnosis and treatment of one huge cystic lymphangioma in etropharyngeal space].

    PubMed

    Zhang, Hua; Song, Xicheng; Jia, Chuanliang

    2016-01-01

    One child was hospitalized because of repeated cough and sputum. The biopsy diagnosis in local hospital was cystic lymphangioma in retropharyngeal space. We carried out transoral incision and drainage by catheter under general anesthesia. Put into the surgical cavity a suction drainage tube, and injected 5 mg dexamethasone and 8mg Bleomycin. He had nasogastric liquid diet after operation. We removed the suction drainage tube two weeks later. No recurrence was found following up over two years. PMID:27192920

  19. Mediastinal Tuberculosis in an Adult Patient with Cystic Fibrosis▿

    PubMed Central

    Morand, Philippe C.; Burgel, Pierre-Régis; Carlotti, Agnès; Desmazes-Dufeu, Nadine; Farhi, David; Martin, Clémence; Kanaan, Reem; Mangialavori, Luigi; Palangié, Estelle; Dusser, Daniel; Poyart, Claire; Hubert, Dominique

    2011-01-01

    Tuberculosis (TB) is rarely observed in cystic fibrosis (CF) patients. We report the first case of mediastinal TB, associated with leg pain and skin rash, in an adult patient with CF, and discuss factors suggestive of TB in the course of CF. PMID:21106788

  20. Laparoscopic resection of a huge retroperitoneal cystic lymphangioma after successful reduction of tumor size with a double balloon catheter

    PubMed Central

    Ishibashi, Yusuke; Tsujimoto, Hironori; Kouzu, Keita; Horiguchi, Hiroyuki; Nomura, Shinsuke; Ito, Nozomi; Kanematsu, Kyohei; Yamazaki, Kenji; Hiraki, Shuichi; Aosasa, Suefumi; Noro, Takuji; Yamamoto, Junji; Hase, Kazuo

    2015-01-01

    Introduction Retroperitoneal cystic lymphangiomas are rare. We report a case of retroperitoneal huge cystic lymphangioma that was successfully aspirated the cyst’s contents with double balloon catheter and excised laparoscopically. Presentation of case A 34-year-old man was admitted to our hospital with low-grade fever and abdominal pain that had lasted for 1 week. Abdominal computed tomography and magnetic resonance imaging showed a fluid-filled multilocular mass measuring 13.5 cm in diameter around the tail of the pancreas, which was diagnosed as a retroperitoneal cystic lymphangioma. We successfully excised the tumor by laparoscopic distal pancreatosplenectomy. We punctured and aspirated the tumor with a double-balloon catheter to decrease the tumor’s size without spilling the tumor content. Cytology showed no malignant cells, and histopathological examination confirmed cystic lymphangioma. No recurrence was noted on radiographic imaging 10 months postoperatively. Discussion and conclusion Laparoscopic treatment for retroperitoneal huge cystic lymphangioma is feasible, and the double balloon catheter is useful for reducing the tumor volume. PMID:25898335

  1. [Mediastinal cystic teratoma. Review of two cases].

    PubMed

    Fuenmayor-M, Carmen Elena; García-R, Maryori; Gómez, Denis; Quintiliani-Gamboa, María; Altamiranda, Cleyzer

    2007-12-01

    Extragonadal teratomas are more frequent in the mediastinum and sacrococcygeal region. In this work, two cases of mature mediastinal teratoma, that simulated infection processes are reported. These cases appeared during the last semester of the year 2000 and represent the casuistry of the University Hospital of The Andes (IAHULA) between 1996 and 2005. Two patients, one ten-year-old and the other eight-month-old were admitted at the hospital with a diagnosis of bronchopneumonia and pulmonary abscess with clinical manifestations of cough, dyspnea and chest pain. During surgery, a multicystic tumor composed of fat, sebaceous and mucinous materials, hair, mineralized elements and blood was found in both cases. A histological examination revealed the presence of neoplastic cells derived from more than one germinal strata. Teratomas are infrequent, but the diagnosis must be considered in children with respiratory symptoms. A full histological examination was required to reach a definitive diagnosis. The histological diagnosis was easily performed by conventional light microscopic examination. No additional techniques were necessary. Surgical resection should thus be the first choice for treatment. Four years later, there is no evidence of tumor recurrence. PMID:18271395

  2. Retroperitoneal lymphangioma: A report of 2 cases and a review of the literature regarding the differential diagnoses of retroperitoneal cystic masses

    PubMed Central

    DI MARCO, MARIACRISTINA; GRASSI, ELISA; VECCHIARELLI, SILVIA; DURANTE, SANDRA; MACCHINI, MARINA; BIASCO, GUIDO

    2016-01-01

    Cystic lymphangioma is a type of benign tumor originating from the lymph vessels. The tumor commonly occurs in childhood, in the head or neck regions, and retroperitoneal localization and presentations in adulthood are rare. Determining a pre-operative diagnosis is often challenging, and in the majority of cases, a diagnosis is only possible subsequent to the histological examination of the surgical specimen. A radical resection is the recommended treatment for cystic lymphangioma, and recurrence is usually due to an incomplete excision of the mass. The present study reports 2 cases of cystic lymphangioma, localized in the pancreatic gland and duodenal wall respectively, which were treated with surgical resection. The study also briefly reviews the literature regarding the differential diagnosis of retroperitoneal cystic masses. PMID:27123082

  3. Cystic lymphangioma-like adenomatoid tumor of the adrenal gland: report of a rare case and review of the literature

    PubMed Central

    Zhao, Ming; Li, Changshui; Zheng, Jiangjiang; Yan, Minghui; Sun, Ke; Wang, Zhaoming

    2013-01-01

    Adenomatoid tumors (AT) are uncommon, benign tumors of mesothelial origination most frequently encountered in the genital tracts of both sexes. Their occurrences in the extragenital sites are much rarer and could elicit a variety of differential diagnosis both clinically and morphologically. With regard to the adrenal gland, to the best knowledge of us, only 31 cases of AT have been reported in the English literature. Several histologic growth patterns have been documented in AT, among which cystic type is the least common one. We herein present a further case of AT arising in the adrenal of a 62-year-old Chinese man with a medical history for systemic hypertensive disease. The tumor was incidentally identified during routine medical examination. An abdomen computed tomography scan revealed a solitary mass in the right adrenal. Grossly, the poorly-circumscribed mass measured 3.0 x 3.0 x 2.0 cm with a cut surface showing a gelatinous texture with numerous tiny cystic structures. Microscopic examination showed an infiltrated lesion with honeycomb appearance mimicking a lymphangioma, which composed predominantly of variably sized and shaped anastomosing small cystic spaces lined by flattened endothelial-like cells, without any epithelioid or signet-ring like components present. Foci of extraadrenal tumor extension, lymphoid aggregates with occasional germinal centre formation, intralesional fat tissue, stromal myoid proliferation and ossification were also observed. Immunohistochemical analyses confirmed the mesothelial differentiation of this tumor and indicated a diagnosis of cystic lymphangiomatoid AT of the adrenal. PMID:23638228

  4. [Thoracic and retroperitoneal cystic lymphangiomyomatosis. Value of lymphography].

    PubMed

    Caron-Poitreau, C; Racineux, J L; Raimbault, J P; Dauver, A

    1979-01-01

    Standard radiological examinations in a woman of 40 years of age with a pneumothorax and chylothorax revealed the presence of a posterior mediastinal mass compressing the oesophagus, and an interstitial syndrome. Retroperitoneal and mediastinal cystic lymphangiomas were found on lymphography. These findings are in accordance with the lymphangiomyomatosis described by Cornog and Enterline in 1966. The similarity between this disease and tuberculous sclerosis is underlined in present-day publications.

  5. [Mediastinitis].

    PubMed

    Martínez Vallina, Primitivo; Espinosa Jiménez, Dionisio; Hernández Pérez, Lucía; Triviño Ramírez, Ana

    2011-01-01

    Mediastinitis is defined as acute or chronic inflammation of the mediastinal structures and generally has a low incidence. The most frequent acute cause is sternotomy following cardiac revascularization surgery with both internal mammary arteries, with an incidence of 0.4% to 5% and a mortality of 16.5% to 47%. The most frequent vector is Staphylococcus aureus. Esophageal perforation, usually iatrogenic, is the second most frequent cause of acute mediastinitis, produced by common oropharyngeal flora, with a mortality rate of 20% to 60%, depending on the time of diagnosis. The third most frequent cause is descending necrotizing mediastinitis, the origin being an odontogenous focus in 60% and beta-hemolytic streptococcus the causative agent in 71.5% of cases. The most accurate diagnostic imaging technique is computed tomography. Treatment is almost always surgical and survival depends on its early performance. The worst postsurgical prognostic factor is septic shock.

  6. Mediastinitis

    MedlinePlus

    ... heart valve surgery, or coronary artery bypass surgery. Did You Know... A child who swallows a button ... treatment for fibrosing mediastinitis. Resources In This Article Did You Know 1 Did You Know... Pleural and ...

  7. Mediastinitis

    MedlinePlus

    ... Trauma Other causes of mediastinitis include: Histoplasmosis Radiation Sarcoidosis Tuberculosis Breathing in anthrax Cancer Risk factors include: ... of breath If you have tuberculosis, histoplasmosis, or sarcoidosis and develop any of these symptoms, contact your ...

  8. Retroperitoneal lymphangioma. A case report.

    PubMed

    Wang, H H; Li, S G; Chang, S Y; Ma, C P

    1989-06-01

    Retroperitoneal cystic lymphangiomas are rare benign tumours. Ultrasonography, computed tomography, lymphography or fine-needle percutaneous aspiration may be used to make the diagnosis pre-operatively. Complete excision of the cyst without rupture is the preferred treatment. However, in complicated cases a conservative surgical approach is mandatory.

  9. Adrenal lymphangioma removed by a retroperitoneoscopic procedure.

    PubMed

    Liu, Ben; Li, Yanyuan; Wang, Shuo

    2013-02-01

    We report a case of an adrenal lymphangioma removed by retroperitoneal laparoscopy. A 45-year-old female was referred to the urological ward for an adrenal mass that was incidentally detected by ultrasound examination one month earlier. An abdominal ultrasonography (US) scan revealed a 3.0 cm anechoic cystic mass, while a computed tomography (CT) scan revealed a 3.0×2.7 cm left adrenal cystic mass, which was suspected to be an adrenal cyst. The patient underwent retroperitoneoscopic removal of the tumor. Pathological evaluation revealed a cystic lymphangioma in the left adrenal gland.

  10. Cases of atypical lymphangiomas in children.

    PubMed

    Minocha, Prashant K; Roop, Lakhan; Persad, Rambachan

    2014-01-01

    Background. Lymphatic malformations or lymphangiomas are rare benign hamartomas that result from maldevelopment of primitive lymphatic sacs. They are most frequently found in the neck and axilla, while intra-abdominal and mediastinal lymphangiomas are uncommon. These are primarily tumours of infancy and childhood and are successfully treated with surgical excision. Summary of Cases. Five cases of lymphangioma comprising three intra-abdominal lymphangiomas and two unilateral axillary lymphangiomas presenting at one institution in Trinidad W.I. between 2005 and 2012 were examined. The presentations, location, workup, treatment, and outcome of these patients were studied. Conclusion. This paper discusses a range of extracervical lymphangioma cases seen at San Fernando General Hospital, Trinidad W.I. We report three intra-abdominal cases and the most common clinical presentations were abdominal pain and distension. Also two axillary cases were reported, which presented as painless axillary masses. The major concerns for excision of axillary lymphangioma by parents and surgeons were cosmesis and feasibility of complete resection without disruption of developing breast tissue and axillary vessels. We believe that ultrasound scan is very good at detection of the lesion, while CT is better at determining tumour content and planning for the operation. It is our opinion that complete surgical excision can be achieved. PMID:25328741

  11. [Abdominal cystic tumor revealing lymphangioleiomyomatosis].

    PubMed

    Barbier, L; Ebbo, M; Andrac-Meyer, L; Schneilitz, N; Le Treut, Y-P; Reynaud-Gaubert, M; Hardwigsen, J

    2009-02-01

    We report the case of a 39 year-old woman with many years of intermittent abdominal pain who was found to have cystic masses evocative of cystic lymphangioma involving the posterior mediastinal and retroperitoneum. Worsening abdominal pain led to a recommendation for laparoscopic unroofing and decompression of the cysts. During the postoperative period, hemorrhagic shock required reintervention with excision of the tumoral mass. Pathologic examination revealed lymphangioleiomyomatosis (LAM). On the 15th postoperative day, the patient developed a chylopneumothorax which required prolonged chest tube drainage. The presence of multiple polycystic lesions in the pulmonary parenchyma supported the diagnosis of diffuse LAM with primary extrapulmonary presentation. This diagnosis should be considered preoperatively since it modifies the treatment: a complete excision of the cystic lesions seems to be necessary in order to prevent bleeding and lymphatic extravasation.

  12. Adrenal lymphangioma masquerading as a pancreatic tail cyst

    PubMed Central

    Jung, Hae Il; Ahn, Taesung; Son, Myoung Won; Kim, Zisun; Bae, Sang Ho; Lee, Moon Soo; Kim, Chang Ho; Cho, Hyon Doek

    2014-01-01

    Cystic lymphangiomas of the adrenal gland are rare. A 79-year-old female presented in the emergency room with epigastric discomfort, and an immovable mass was palpated in her abdomen upon physical examination. Imaging studies revealed a large cystic lesion in the pancreatic tail. The radiologic impression ruled out the possibility of a mucinous cystic neoplasm, or a pseudocyst in the pancreas. The operative findings demonstrated that the cystic mass originated in the left adrenal gland. A laparoscopic excision of the cystic mass was performed, and immunohistochemistry confirmed that this mass was a lymphangioma of the adrenal gland. Several prior reports have suggested that lymphangioma can mimic renal or splenic cysts. However, lymphangioma cases mimicking pancreatic cysts are very rare. PMID:25278717

  13. Parotid lymphangioma associated with facial nerve paralysis.

    PubMed

    Imaizumi, Mitsuyoshi; Tani, Akiko; Ogawa, Hiroshi; Omori, Koichi

    2014-10-01

    Parotid lymphangioma is a relatively rare disease that is usually detected in infancy or early childhood, and which has typical features. Clinical reports of facial nerve paralysis caused by lymphangioma, however, are very rare. Usually, facial nerve paralysis in a child suggests malignancy. Here we report a very rare case of parotid lymphangioma associated with facial nerve paralysis. A 7-year-old boy was admitted to hospital with a rapidly enlarging mass in the left parotid region. Left peripheral-type facial nerve paralysis was also noted. Computed tomography and magnetic resonance imaging also revealed multiple cystic lesions. Open biopsy was undertaken in order to investigate the cause of the facial nerve paralysis. The histopathological findings of the excised tumor were consistent with lymphangioma. Prednisone (40 mg/day) was given in a tapering dose schedule. Facial nerve paralysis was completely cured 1 month after treatment. There has been no recurrent facial nerve paralysis for eight years.

  14. Asymptomatic lymphangioma involving the spleen and retroperitoneum in adults.

    PubMed

    Chung, Sook Hee; Park, Young Sook; Jo, Yun Ju; Kim, Seong Hwan; Jun, Dae Won; Son, Byoung Kwan; Jung, Jun Young; Baek, Dae Hyun; Kim, Dong Hee; Jung, Yoon Young; Lee, Won Mi

    2009-11-28

    Lymphangioma, a benign neoplasm of the lymphatic system, is common in children but rare in adults. Its clinical manifestations include abdominal pain, nausea, vomiting and a palpable mass. However, abdominal sonography or abdominal computed tomography (CT) scan can also incidentally reveal lymphangioma. A larger or symptomatic lymphangioma is treated with total resection to prevent recurrence, infection, torsion and enlargement. Although lymphangioma rarely becomes malignant, its prognosis is generally good. We report a cystic lymphangioma of the spleen and retroperitoneum, which was incidentally found in a 56-year-old man who was hospitalized due to a colon mass. Physical examination showed no specific findings. Abdominal CT revealed a 5.7 cm, non-enhanced multilobulated cystic mass with multiple septa in the spleen and a 10 cm lobulated cystic mass in the paraaortic area. Splenectomy and retroperitoneal resection of the cystic mass were conducted. The endothelium of splenic and retroperitoneal cyst was immunohistochemically stained with D2-40 antibody. The patient was finally diagnosed with splenic cystic and retroperitoneal cavernous lymphangioma.

  15. Adrenal Lymphangioma Masquerading as a Catecholamine Producing Tumor

    PubMed Central

    Hodish, Israel; Schmidt, Lindsay; Moraitis, Andreas G.

    2015-01-01

    Objective. To report the unusual case of an adrenal lymphangioma presenting in a patient with an adrenal cystic lesion and biochemical testing concerning for pheochromocytoma. The pertinent diagnostic and imaging features of adrenal lymphangiomas are reviewed. Methods. We describe a 59-year-old patient who presented with hyperhidrosis and a 2.2 by 2.2 cm left adrenal nodule. Biochemical evaluation revealed elevated plasma-free normetanephrine, urine normetanephrine, urine vanillylmandelic acid, and urine norepinephrine levels. Elevated plasma norepinephrine levels were not suppressed appropriately with clonidine administration. Results. Given persistent concern for pheochromocytoma, the patient underwent adrenalectomy. The final pathology was consistent with adrenal lymphangioma. Conclusions. Lymphangiomas are benign vascular lesions that can very rarely occur in the adrenal gland. Imaging findings are generally consistent with a cyst but are nonspecific. Excluding malignancy in patients presenting with adrenal cysts can be difficult. Despite its benign nature, the diagnosis of adrenal lymphangioma may ultimately require pathology. PMID:26618011

  16. Lymphangioma of the ovary: etiology and management.

    PubMed

    Radhouane, Achour; Mayada, Saadaoui; Khaled, Neji

    2016-08-01

    Lymphangioma of the ovary is an extremely rare lesion. It was first described in 1908 [1] and there is only 19 cases reports in a 50-year literature survey [2]. Lymphangiomas are benign congenital malformations of lymphatic system. They are thought to happen due to obstruction of local lymph flow system and they can occur anywhere in the skin and the mucous membranes. Most common sites are the head and the neck and sometimes they can be found in the intestines, the pancreas and the mesentery. Lymphangioma of the ovary is a very rare tumor. Typically, lymphangiomas are slow-growing tumors that remain asymptomatic for a long time, with the tumor being identified incidentally during histopathological examination after excision. A 36-year-old women who consulted our department for infertility for 2 years. The systematic ultrasound examination reveals a right ovarian mass. The histologic analysis confirmed the diagnosis of cystic lymphangioma of the right ovary. She was planned for total laparoscopic tumorectomy with removal of all ovarian mass. The evolution was good for one year within Clinical and ultrasound monitoring. We report through this case report a review of the literature. PMID:27401696

  17. Fine-needle aspiration cytology of lymphangioma of the parotid gland in an adult.

    PubMed

    Henke, A C; Cooley, M L; Hughes, J H; Timmerman, T G

    2001-02-01

    Lymphangioma or cystic hygroma is an uncommon benign congenital tumor of lymphatics that is seen in children and, rarely, adults. Lymphangioma primarily involving the parotid gland is an extremely uncommon occurrence in adults. We report on the cytologic findings of a parotid lymphangioma in a 34-yr-old man which showed 13 cc of yellow fluid with red blood cells, lymphocytes, and rare fragments of benign-appearing salivary gland epithelium. The differential diagnosis of cystic parotid gland lesions in adults may include Warthin's tumor, lymphoma, benign lymphoepithelial lesions, branchial cleft cysts, chronic sialadenitis, cystic low-grade mucoepidermoid carcinoma, and cystic pleomorphic adenoma. In this case, the fine-needle aspiration findings along with the magnetic resonance imaging (MRI) findings of a multiloculated cystic mass in the parotid gland allowed the diagnosis of lymphangioma.

  18. [A case of retroperitoneal cavernous lymphangioma with the content of chylous fluid].

    PubMed

    Sano, K; Kawasaki, C; Sato, K; Iwasaki, A; Ishizuka, E

    1990-12-01

    We report a case of retroperitoneal lymphangioma in a 26-year-old man whose chief complaint was lumbar discomfort. Ultrasonography revealed pararenal cyst on his left side. At the operation, we discovered a cystic tumor between peritoneum and Gerota's fascia. Pathological study indicated cavernous lymphangioma containing chylous fluid. There has been no recurrence for a year after the operation.

  19. Percutaneous Sclerotherapy Using Acetic Acid After Failure of Alcohol Ablation in an Intra-abdominal Lymphangioma

    SciTech Connect

    Park, Sang Woo Cha, In Ho; Kim, Kyeong Ah; Hong, Suk Joo; Park, Cheol Min; Chung, Hwan Hoon

    2004-09-15

    We report a case of percutaneous sclerotherapy using acetic acid in a 22-year-old woman with an intra-abdominal cystic lymphangioma who was not successfully treated with ethanol despite multiple trials.

  20. Omental lymphangioma in adults-rare presentation report of a case.

    PubMed

    Rao, T Narayana; Parvathi, T; Suvarchala, A

    2012-01-01

    Lymphangioma is an uncommon benign lesion that usually occurs during childhood. Its occurrence in adults is rare. Its presentation in the abdomen is even rare. This case report describes a case of omental lymphangioma presented as retroperitoneal lump. Subsequent imaging, operative, and histological findings revealed omental lymphangioma. Laparotomy done under general anesthesia, a 10 × 12 cm cystic swelling arising from omentum, identified complete excision of the cyst done and send the specimen for histopathological examination. Biopsy report came as omental lymphangioma. Complete surgical excision is the treatment of choice. Prognosis is excellent and recurrence rate is very low if resection is complete. During two years of followup no recurrence was detected. Omental lymphangioma is very rare presentation among abdominal lymphangiomas specifically in adults. Complete excision is the treatment of choice. Long-term followup is required to detect recurrence.

  1. Lymphangioma of vulva

    SciTech Connect

    Young, A.W. Jr.; Wind R.M.; Tovell, H.M.M.

    1980-05-01

    A second case with acquired lymphangioma of the vulva has been presented. The condition was diagnosed 22 years following radiation and lymph-node dissection for carcinoma of the cervix and a long history of recurring erysipelas involving the vulva and lymphedematous lower extremities. The pathogenesis of the lymphangioma and the possibility of a later development of a vascular cancer have been discussed.

  2. [Cystic angiomatosis].

    PubMed

    Fernández Jiménez, I; Alvarez Muñoz, V; Peláez Mata, D; Díaz Blanco, M; Galbe Sada, M; Antuña García, M J

    2000-04-01

    A case of cystic angiomatosis is presented in a patient whose clinical sign were the presence of an inguinoescrotal mass due to a retroperitoneal lymphangioma, visceral splenic lesions and diffuse skeletal lesions. This rare entity is discussed and a brief review of the literature is made.

  3. Retroperitoneal multiple lymphangioma with differential cyst contents causing hydronephrosis and biliary dilatation.

    PubMed

    Adachi, S; Maruyama, T; Suetomi, T; Morishita, Y; Otsuka, M; Todoroki, T; Fukao, K

    2001-01-01

    We report a 40-year-old woman with retroperitoneal multiple cystic lymphangioma. Multiple cystic lesions were found on both sides of the aorta in the retroperitoneal and right pelvic spaces. These thin-walled cysts gradually increased in size leading to left hydronephrosis and dilatation of the common bile duct with cholecystitis, requiring resection. Analysis of cystic contents revealed differences between the cysts. The right cyst contained high concentrations of amylase, 3429 U/L, while the left cyst contained high concentrations of triglyceride, 470 mg/dL. No reports of patients with idiopathic retroperitoneal cystic lymphangioma have described differences in cystic contents. Differences in cystic contents according to tumor location strongly suggest that the cause of retroperitoneal lymphangioma is a developmental malformation in which lymphangiectasia follows the failure of developing lymphatic tissue to establish normal communication with the remainder of the lymphatic system.

  4. Spontaneous regression of a cystic retroperitoneal tumour in young women postpartum. Report of two cases.

    PubMed

    de Zeeuw, Sharonne; Schouten van der Velden, Arjan P; Eggink, Alex J; Strijk, Simon; Wobbes, Theo

    2011-01-01

    Retroperitoneal cystic tumours are rarely found, and of these, the most common lesion is a cystic lymphangioma. We present two postpartum patients with a cystic retroperitoneal tumour which showed spontaneous regression and a review of the literature.

  5. Adrenal lymphangioma: clinicopathologic and immunohistochemical characteristics of a rare lesion.

    PubMed

    Ellis, Carla L; Banerjee, Priya; Carney, Erin; Sharma, Rajni; Netto, George J

    2011-07-01

    Adrenal lymphangiomas, also known as cystic adrenal lymphangiomas, are rare, benign vascular lesions that usually remain asymptomatic throughout life. Although previously adrenal lymphangioma lesions were primarily found at autopsy, they are currently detected during imaging work-up for unrelated causes and are likely to imitate other adrenocortical or adrenal medullary neoplasms. We aimed to retrospectively review all adrenal lymphangioma cases at our hospital and further document their lymphatic origin by immunohistochemical staining. A search of surgical pathology records (1984-2008) was conducted. All hematoxylin and eosin sections were retrieved from archives and reviewed by 2 pathologists in the study. Clinical information was gathered from electronic medical records. Representative paraffin-embedded sections from each case were selected for immunohistochemical analysis using monoclonal antibodies D2-40 and AE1/AE3. A total of 9 adrenal lymphangioma cases were identified (6 women and 3 men). All 9 patients were adults at time of diagnosis with a mean age of 42 years (range, 28-56 years). There were 7 white patients, 1 African American patient, and 1 Asian patient. The average size of an adrenal lymphangioma lesion was 4.9 cm (range, 2.0-13.5 cm). Adrenal lymphangioma was twice more frequently located on the right side (6 right-sided and 3 left-sided). Clinically, 4 (44%) of the 9 lesions presented with abdominal, flank, or back pain. One lymphangioma was found during work-up for labile hypertension. The remaining 4 lesions (44%) were asymptomatic and incidentally found during imaging studies for unrelated causes. Surgical removal was achieved by total adrenalectomy in 8 of the 9 lesions and by partial adrenalectomy in the remaining case. No evidence of recurrence or development of a contralateral lesion was encountered in any of the patients. Histologically, our adrenal lymphangiomas showed a typical multicystic architecture with dilated spaces lined by

  6. Mediastinal hemangioma: Masquerading as pleural effusion.

    PubMed

    Deepak, J; Babu, M Narendra; Gowrishankar, B C; Ramesh, S

    2013-10-01

    We present a rare case of mediastinal hemangioma in a 4-year-old child. The child presented with cough and fever and the chest radiograph revealed right pleural effusion. On further work-up with chest computed tomography mediastinal cystic mass occupying the right hemithorax was identified. The cystic mass was resected completely through a right thoracotomy. Histopathological examination established the final diagnosis of mediastinal capillary hemangioma. PMID:24347874

  7. Cutaneous Lymphangioma circumscriptum - dermoscopic features**

    PubMed Central

    Massa, António Fernandes; Menezes, Nuno; Baptista, Armando; Moreira, Ana Isabel; Ferreira, Eduarda Osório

    2015-01-01

    Lymphangiomas are congenital lymphatic malformations and cutaneous lymphangioma circumscriptum is the most common type. It is clinically characterized by clusters of translucent vesicles and the presence of dermoscopically yellow lacunae surrounded by pale septa, as well as reddish to bluish lacunae. In our case, the recently described hypopyon-like feature manifested, aiding in the sometimes difficult differential diagnosis of cutaneous lymphangioma circumscriptum with vascular lesions, further highlighting the importance of dermoscopy in what can be a diagnostic challenge. PMID:25831002

  8. Cervical Cystic Hygroma in an Adult

    PubMed Central

    Derin, Serhan; Şahan, Murat; Dere, Yelda; Çullu, Neşat; Şahan, Leyla

    2014-01-01

    Cystic hygromas/lymphangiomas are extremely rare malformations in adults. They are usually seen in infants and children under 2 years of age. En bloc resection is difficult due to the adhesive characteristics of the tumors. Inadequate surgical intervention often leads to recurrent disease. We report herein the case of a cystic hygroma/lymphangioma that presented as an uncommon mass on the cervical region in an adult, together with its histopathological, radiologic, and operative features. PMID:25548704

  9. Cervical cystic hygroma in an adult.

    PubMed

    Derin, Serhan; Şahan, Murat; Dere, Yelda; Çullu, Neşat; Şahan, Leyla

    2014-01-01

    Cystic hygromas/lymphangiomas are extremely rare malformations in adults. They are usually seen in infants and children under 2 years of age. En bloc resection is difficult due to the adhesive characteristics of the tumors. Inadequate surgical intervention often leads to recurrent disease. We report herein the case of a cystic hygroma/lymphangioma that presented as an uncommon mass on the cervical region in an adult, together with its histopathological, radiologic, and operative features. PMID:25548704

  10. Lymphangioma of the tongue

    PubMed Central

    Goswami, Mousumi; Singh, Sanjay; Gokkulakrishnan, S; Singh, Amit

    2011-01-01

    Lymphangiomas are developmental malformations and have a marked predilection for the head and neck region. They are benign proliferation of lymphatic vessels and represents hamartomas of malformed lymphatics. The most common location in the mouth is the dorsum of tongue, followed by lips, buccal mucosa, soft palate, and floor of the mouth. In the tongue, they may present as a localized or a diffused growth which may enlarge to cause macroglossia, impaired speech and difficulty in mastication. These tumors are typically present at birth, but may go unnoticed until after dentition erupts or even after puberty. Presenting here is a case of localized lymphangioma of the tongue in a five year old child and its surgical management. PMID:22442618

  11. Primary Mediastinal Classical Hodgkin Lymphoma.

    PubMed

    Piña-Oviedo, Sergio; Moran, Cesar A

    2016-09-01

    Primary mediastinal Classical Hodgkin lymphoma (CHL) is rare. Nodular sclerosis CHL (NS-CHL) is the most common subtype involving the anterior mediastinum and/or mediastinal lymph nodes. Primary thymic CHL is exceedingly rare. The disease typically affects young women and is asymptomatic in 30% to 50% of patients. Common symptoms include fatigue, chest pain, dyspnea and cough, but vary depending on the location and size of the tumor. B-symptoms develop in 30% of cases. By imaging, primary mediastinal CHL presents as mediastinal widening/mediastinal mass that does not invade adjacent organs but may compress vital structures as bulky disease. Histopathology is the gold standard for diagnosis. Primary mediastinal NS-CHL consists of nodules of polymorphous inflammatory cells surrounded by broad fibrous bands extending from a thickened lymph node capsule. The cellular nodules contain variable numbers of large Hodgkin/Reed-Sternberg cells, required for diagnosis. Primary thymic CHL may exhibit prominent cystic changes. The histopathologic recognition of NS-CHL can be challenging in cases with prominent fibrosis, scant cellularity, artifactual cell distortion, or an exuberant granulomatous reaction. The differential diagnosis includes primary mediastinal non-HLs, mediastinal germ cell tumors, thymoma, and metastatic carcinoma or melanoma to the mediastinum. Distinction from primary mediastinal non-HLs is crucial for adequate therapeutic decisions. Approximately 95% of patients with primary mediastinal CHL will be alive and free of disease at 10 years after treatment with short courses of combined chemoradiotherapy. In this review, we discuss the history, classification, epidemiology, clinicoradiologic features, histopathology, immunohistochemistry, differential diagnosis, and treatment of primary mediastinal CHL. PMID:27441757

  12. A Giant Retroperitoneal Lymphangioma: A Case Report

    PubMed Central

    Hubli, Prabhu; Sachin, Basavaraju M

    2016-01-01

    Lymphangioma is a congenital lesion of childhood. They usually present in head and neck region. Intra-abdominal lymphangioma in an adult is a rare lesion which poses diagnostic difficulty. Although asymptomatic, they may present with an acute abdomen. Here, we report a rare case of huge asymptomatic retro-peritoneal lymphangioma who underwent complete surgical excision with an uneventful postoperative period.

  13. A Giant Retroperitoneal Lymphangioma: A Case Report.

    PubMed

    Hubli, Prabhu; Rohith, Muddasetty; Sachin, Basavaraju M

    2016-07-01

    Lymphangioma is a congenital lesion of childhood. They usually present in head and neck region. Intra-abdominal lymphangioma in an adult is a rare lesion which poses diagnostic difficulty. Although asymptomatic, they may present with an acute abdomen. Here, we report a rare case of huge asymptomatic retro-peritoneal lymphangioma who underwent complete surgical excision with an uneventful postoperative period. PMID:27630902

  14. A Giant Retroperitoneal Lymphangioma: A Case Report

    PubMed Central

    Hubli, Prabhu; Sachin, Basavaraju M

    2016-01-01

    Lymphangioma is a congenital lesion of childhood. They usually present in head and neck region. Intra-abdominal lymphangioma in an adult is a rare lesion which poses diagnostic difficulty. Although asymptomatic, they may present with an acute abdomen. Here, we report a rare case of huge asymptomatic retro-peritoneal lymphangioma who underwent complete surgical excision with an uneventful postoperative period. PMID:27630902

  15. A Giant Retroperitoneal Lymphangioma: A Case Report.

    PubMed

    Hubli, Prabhu; Rohith, Muddasetty; Sachin, Basavaraju M

    2016-07-01

    Lymphangioma is a congenital lesion of childhood. They usually present in head and neck region. Intra-abdominal lymphangioma in an adult is a rare lesion which poses diagnostic difficulty. Although asymptomatic, they may present with an acute abdomen. Here, we report a rare case of huge asymptomatic retro-peritoneal lymphangioma who underwent complete surgical excision with an uneventful postoperative period.

  16. Mediastinal tumor

    MedlinePlus

    ... contains the heart, large blood vessels, windpipe (trachea), thymus gland, esophagus, and connective tissues. The mediastinum is divided into three sections: Anterior (front) Middle Posterior (back) Mediastinal ... Cancer Browse the Encyclopedia A.D.A.M., ...

  17. [Chronic back pain resulting from a retroperitoneal lymphangioma].

    PubMed

    Justinger, C; Weinrich, M; Katoh, M; Schilling, M K

    2008-08-01

    A 39-year-old female patient presented with a 3-year history of lower back pain which had not been alleviated by pain treatment combined with physiotherapy. Radiological findings were normal with the exception of a cystic paravertebral tumor in the left retroperitoneum. The cyst was primarily regarded as a coincidental finding because no criteria for malignancy were present. Magnetic resonance tomography, however, the cyst showed a progressive increase in size and the pain remained unchanged so that surgical resection was performed. The histopathological examination showed a lymphangioma and the pain was nearly completely resolved after surgery.

  18. Lymphangioma Presenting as Cardiac Tamponade in a Child.

    PubMed

    Mishra, Prashant; Vaideeswar, Pradeep; Marathe, Supreet P; Aironi, Balaji; Agrawal, Nandkishor B

    2015-07-01

    Lymphangiomas are hamartomatous malformations, which are commonly encountered in children. Intrapericardial lymphangioma is exceptionally rare. We present a case of an intrapericardial lymphangioma in a child presenting with cardiac tamponade.

  19. [Minimally Invasive Thoracoscopic Surgery for Mediastinal Lesions].

    PubMed

    Maeda, Sumiko

    2016-07-01

    This review article describes minimally invasive thoracoscopic surgery for anterior mediastinal lesions. The operative procedures for anterior mediastinal lesions have been changed in a couple of decades from open surgery under median sternotomy to complete thoracoscopic mediastinal surgery with sternal lifting or carbon dioxide insufflation. Carbon dioxide insufflation of the thoracic cavity or the mediastinum is now prevailing to improve the surgical field and facilitate the operative procedures. Surgical indications for complete thoracoscopic mediastinal surgery include benign cystic lesions generally regardless of their size and non-invasive anterior mediastinal tumors usually less than 50~60 mm in the greatest dimension. There are currently three surgical approaches in the complete thoracoscopic surgery for the anterior mediastinal lesions. One is the unilateral or bilateral transthoracic approach. The second is the combination of the subxiphoid and the transthoracic approach. The last is the subxiphoid approach. The selection of the surgical approach depends on the surgeon's preference and experiences. When carbon dioxide insufflation is applied during the operation, following complications may occur;hypercapnia, gas embolism, subcutaneous emphysema, endotracheal tube dislocation due to the mediastinal sift, and hypotention. Special safety considerations are necessary during the complete thoracoscopic mediastinal surgery with carbon dioxide insufflation. PMID:27440034

  20. Pinocchio nasal deformity secondary to lymphangioma circumscriptum.

    PubMed

    Uysal, Afşin; Yildiz, Kaya; Kankaya, Yüksel; Oruç, Melike; Sungur, Nezih; Koçer, Uğur; Ozer, Elif

    2007-11-01

    Pinocchio or Cyrano nasal tip deformity is a rare situation that develops secondary to the soft tissue tumors underneath. In literature, there is only one case reported with Pinocchio nasal deformity secondary to cavernous lymphangioma. In this study, we present a Pinocchio or Cyrano nasal deformity with skin involvement secondary to lymphangioma circumscriptum.

  1. Subserous lymphangioma of the sigmoid colon: an uncommon cause of acute abdomen in pediatric patients

    PubMed Central

    Fernandes, Bianca Furlan; Moraes, Érika Neves de Souza; de Oliveira, Francini Rossetto; Felipe-Silva, Aloísio; Ferreira, Cristiane Rúbia; de Alcântara, Paulo Sérgio Martins; Tokeshi, Flavio; Martinês, João Augusto dos Santos; Ferronato, Ângela Espósito

    2015-01-01

    Lymphangioma is a rare, benign lesion derived from a malformation of the lymphatic system, which is more frequently found in the head, neck, and axilla. However, it may be present anywhere in the body, and the diagnosis involves adults as children with some distinct clinical features among them. In pediatric patients, abdominal cystic lymphangioma occurs mostly in the mesentery presenting abdominal pain, intestinal obstruction, or, more rarely, hemorrhage. The authors report the case of a child with a short-course history of fever, abdominal pain, and constipation. The physical examination disclosed the presence of an abdominal mass and signs of peritoneal irritation. Imaging was consistent with a cystic lesion compressing the sigmoid colon and laterally displacing the remaining loops. Exploratory laparotomy was undertaken, and a sigmoidectomy, followed by Hartman’s colostomy, was performed. Histological examination revealed the nature of the lesion as a cystic lymphangioma. The authors highlight the clinical features of this entity and call attention to this disease in the differential diagnosis of acute abdomen or abdominal pain, mainly in pediatric patients. PMID:26894047

  2. Echocardiographic features of a mediastinal pancreatic pseudocyst.

    PubMed Central

    Aroney, C N; Nicholson, M R; Shevland, J E

    1985-01-01

    Cross sectional echocardiography detected a mediastinal pancreatic pseudocyst which caused extracardiac compression in a 49 year old man. Computed tomography confirmed the presence of a cystic lesion lying behind the heart and extending from the pancreas to above the carina. Surgical decompression resulted in resolution of the clinical and echocardiographic findings. Images PMID:3994874

  3. [Mediastinal lymphomas].

    PubMed

    Rauthe, S; Rosenwald, A

    2016-09-01

    Lymphomas infiltrating the mediastinum are a challenge for the treating physician as well as for the pathological diagnostics. The clinical scenario is often an emergency situation, while the pathologist is usually confronted only with small biopsy samples. Classical Hodgkin's lymphoma is by far the most frequently occurring lymphoma in the mediastinum and predominantly the nodular sclerosis subtype. In small and very sclerotic specimens it can be difficult to morphologically detect Hodgkin and Reed-Sternberg cells and to identify the characteristic phenotype by immunohistochemistry. Primary mediastinal large B‑cell lymphomas should be distinguished from classical Hodgkin's lymphomas as the treatment is different. This is characterized by the detection of sheets of blast cells, which immunohistochemically show a strong B‑cell phenotype (positivity for CD20 and CD79a), while CD30 can also often be expressed. The intimate biological relationship between classical Hodgkin's lymphomas and mediastinal large B‑cell lymphomas is illustrated by the existence of B‑cell lymphomas with intermediate features (so-called mediastinal grey zone lymphomas). It is important to recognize and diagnose these lymphomas as they are associated with a slightly inferior prognosis. Extranodal thymic marginal zone lymphomas of the mucosa-associated lymphoid tissue (MALT) type are a rare form of lymphoma encountered in the mediastinum, which can be associated with autoimmune diseases. T‑lymphoblastic lymphomas and leukemia, which occur predominantly in children and young adults, represent a rapidly growing precursor cell neoplasia and must be distinguished from thymomas in the differential diagnostics as well as from normal and hyperplastic thymus glands. PMID:27507160

  4. Oral lymphangioma: A rare case report

    PubMed Central

    Bhayya, Harsha; Pavani, D.; Avinash Tejasvi, M. L.; Geetha, P.

    2015-01-01

    Lymphangiomas are benign hamartomatous tumors of the lymphatic channels which present as developmental malformations arising from sequestration of lymphatic tissue that do not communicate with the rest of the lymphatic channels. Lymphatic vessels are filled with a clear protein-rich fluid containing few lymph cells. It can also occur in association with hemangioma. The onset of lymphangiomas are either at birth (60% to 70%) or up to two years of age (90%) and rare in adults. Lymphangiomas have marked predilection for the head and neck region (50-70%). The most common location in the mouth is the dorsum of tongue, followed by lips, buccal mucosa, soft palate, and floor of the mouth. On tongue, they may present as a localized or a diffused growth which may enlarge to cause macroglossia, impaired speech and difficulty in mastication. Herewith, we present a rare case of lymphangioma of tongue leading to macroglossia in a 8-year-old boy. PMID:26681873

  5. Conjunctival lymphangioma in a 4-year-old girl revealed tuberous sclerosis complex

    PubMed Central

    Freiberg, Florentina Joyce; Kunstmann, Erdmute; König, Thomas; Matlach, Juliane; Kampik, Daniel

    2016-01-01

    Background: To present a case of conjunctival lymphangioma in a 4-year-old girl with tuberous sclerosis complex. Methods/results: A 4-year-old girl presented with a relapsing cystic lesion of the bulbar conjunctiva in the right eye with string-of-pearl-like dilation of lymphatic vessels and right-sided facial swelling with mild pain. Best-corrected vision was not impaired. Examination of the skin revealed three hypomelanotic macules and a lumbal Shagreen patch. Magnetic resonance imaging (MRI) findings displayed minimal enhancement of buccal fat on the right side. Cranial and orbital MRI showed signal enhancement in the right cortical and subcortical areas. Genetic analysis revealed a heterozygous deletion encompassing exon 1 and 2 of the TSC1 gene (tuberous sclerosis complex 1 gene), confirming the diagnosis of tuberous sclerosis complex. Conclusion: In conjunctival lymphangioma, tuberous sclerosis complex should be considered as the primary disease. PMID:27703871

  6. Lymphangioma of the small bowel mesentery: a case report and review of the literature.

    PubMed

    Suthiwartnarueput, Worapop; Kiatipunsodsai, Siriphut; Kwankua, Amolchaya; Chaumrattanakul, Utairat

    2012-11-21

    Lymphangioma is a rare benign condition characterized by proliferation of lymphatic spaces. It is usually found in the head and neck of affected children. Lymphangioma of the small-bowel mesentery is rare, having been reported for less than 1% of all lymphangiomas. Importantly, it can cause fatal complications such as volvulus or involvement of the main branch of the mesenteric arteries, requiring emergency surgery. Moreover, the gross and histopathologic findings may resemble benign multicystic mesothelioma and lymphangiomyoma. Immunohistochemical study for factor VIII-related antigen, D2-40, calretinin and human melanoma black-45 (HMB-45) are essential for diagnosis. Factor VIII-related antigen and D2-40 are positive in lymphangioma but negative in benign multicystic mesothelioma. HMB-45 shows positive study in the smooth-muscle cells around the lymphatic spaces of the lymphangiomyoma. We report a case of small-bowel volvulus induced by mesenteric lymphangioma in a 2-year-and-9-mo-old boy who presented with rapid abdominal distension and vomiting. The abdominal computed tomography scan showed a multiseptated mass at the right lower quadrant with a whirl-like small-bowel dilatation, suggestive of a mesenteric cyst with midgut volvulus. The intraoperative findings revealed a huge, lobulated, yellowish pink, cystic mass measuring 20 cm × 20 cm × 10 cm, that was originated from the small bowel mesentery with small-bowel volvulus and small-bowel dilatation. Cut surface of the mass revealed multicystic spaces containing a milky white fluid. The patient underwent tumor removal with small-bowel resection and end-to-end anastomosis. Microscopic examination revealed that the cystic walls were lined with flat endothelial cells and comprised of smooth muscle in the walls. The flat endothelial cells were positive for factor VIII-related antigen and D2-40 but negative for calretinin. HMB-45 showed negative study in the smooth-muscle cells around the lymphatic spaces. Thus

  7. [Adrenal cystic masses. Our experience].

    PubMed

    Costantino, V; Petrin, P; Da Lio, C; Zaramella, D; Pedrazzoli, S

    1993-10-01

    Cystic masses of the adrenal gland are clinically and pathologically rare findings and few cases have been reported up to now in the medical literature. In the present work 5 new cases are reported: 3 adrenal pseudocysts, 1 lymphangioma, 1 cystic pheochromocytoma. In 3 cases there were clinical symptoms of retroperitoneal mass (lumbar pain, palpable mass, digestive symptoms); in 3 cases conventional radiology was helpful; ultrasonography was used for diagnosis in 1, CT scan in 2. In the pheochromocytoma case the real nature of the mass was determined through fluid hormone determination after fine needle puncture. All cases were treated by surgery.

  8. [A case of retroperitoneal lymphangioma successfully treated by pancreatoduodenectomy].

    PubMed

    Kurokawa, Y; Kamiya, J; Kirioka, T; Kajita, M; Maeda, M; Nakamura, T; Akiyama, S

    1987-02-01

    A rare case of retroperitoneal lymphangioma in 44-year-old male complaining of high fever and right hypochondralgia is reported. Retroperitoneal malignant tumor was suspected preoperatively, and pancreatoduodenectomy was performed because the tumor infiltrated to duodenum and head of the pancreas. Histopathological examination revealed that the tumor was lymphangioma infiltrating to the duodenum. There are two types of lymphangioma, solitary lymphatic cyst and true proliferating lymphangioma and in this case, the tumor is diagnosed as true proliferating lymphangioma which is cavernous and infiltrating.

  9. Mediastinal mature teratoma in a child- A case report.

    PubMed

    Liew, W X; Lam, H Y; Narasimman, S; Navarasi, S; Mohd Hamzah, K

    2016-02-01

    Mediastinal teratoma is an infrequent germ cell tumour and comprises of 1 to 5% of all mediastinal tumours. We report a case of mediastinal mature teratoma in a 12 year old boy who presented to us with persistent non-productive cough, fever and dyspnoea for the past 7 months. Computed tomographic scan of thorax revealed a large anterior mediastinal mass measuring 11.2x9.9x14cm with calcification within. He subsequently underwent a median sternotomy with left subcostal extension (L-incision) and excision of tumour. Histopathology of the tumour revealed a mature cystic teratoma. We would like to report a case of successful surgical management of a large mediastinal mature teratoma in a child. PMID:27130743

  10. [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.

  11. Lymphangioma communicating with the excretory system.

    PubMed

    Pancione, L; Giacomelli, G; Moroni, M; Taraglio, G; Mecozzi, B

    2008-03-01

    Lymphangioma is a rare benign tumor caused by failure in the development of the lymphatic communicating system. The corresponding nomenclature is confusing. In recent years ''renal lymphangiectasia'' is the preferred name. Although this disease may occur in any site of the body, the neck (75%) and axillary area (20%) are the most common sites, and the kidney is occasionally involved. We report a case of lymphangioma communicating with the urinary system in a 61-year-old man diagnosed by CT scan treated with nephrectomy and histological confirmation.

  12. Klippel-Trenaunay Syndrome with Extensive Lymphangiomas

    PubMed Central

    Mneimneh, Sirin; Tabaja, Ali; Rajab, Mariam

    2015-01-01

    Klippel-Trenaunay syndrome (KTS) is a rare disorder characterized by the triad of vascular malformations, venous varicosities, and bone and soft-tissue hypertrophy. We present a case of Klippel-Trenaunay syndrome with limb hypertrophy, port-wine stains, lymphangiomas, and venous varicosities in the limbs. PMID:26587303

  13. Contemporary mediastinal tuberculosis.

    PubMed Central

    Bloomberg, T J; Dow, C J

    1980-01-01

    Mediastinal lymphadenopathy is a prominent feature of adult tuberculosis in immigrant groups in the United Kingdom. Chest radiography of 95 tuberculous immigrants showed mediastinal gland enlargement in 29 cases, whereas none of the 42 Europeans showed this feature. An analysis has been made of the distribution of the disease in all groups together with a more detailed evaluation of mediastinal involvement in the immigrants. Pericardial effusions were seen in eight patients together with the following three unusual complications: (1) broncho-oesophageal fistula; (2) bronchial erosion; (3) superior mediastinal obstruction. Images PMID:7434292

  14. Traumatic Rupture of A Posterior Mediastinal Teratoma following Motor-Vehicle Accident.

    PubMed

    Bell, Christopher; Domingo, Fernando; Miller, Ashley D; Smith, Jeremiah S; Headrick, James R

    2016-01-01

    We report a case of a posterior mediastinal mature cystic teratoma with rupture secondary to blunt chest trauma in a 20-year-old male involved in a motor-vehicle accident. Initial treatment was guided by Advanced Trauma Life Support and a tube thoracostomy was performed for presumed hemothorax. The heterogeneous collection within the thoracic cavity was discovered to be the result of a ruptured cystic mass. Pathologic findings confirmed the mass consistent with a mature cystic teratoma. As mediastinal teratomas are most commonly described arising from the anterior mediastinum, the posterior location of the teratoma described in this report is exceedingly rare. PMID:27660731

  15. Traumatic Rupture of A Posterior Mediastinal Teratoma following Motor-Vehicle Accident

    PubMed Central

    Bell, Christopher; Domingo, Fernando; Smith, Jeremiah S.; Headrick, James R.

    2016-01-01

    We report a case of a posterior mediastinal mature cystic teratoma with rupture secondary to blunt chest trauma in a 20-year-old male involved in a motor-vehicle accident. Initial treatment was guided by Advanced Trauma Life Support and a tube thoracostomy was performed for presumed hemothorax. The heterogeneous collection within the thoracic cavity was discovered to be the result of a ruptured cystic mass. Pathologic findings confirmed the mass consistent with a mature cystic teratoma. As mediastinal teratomas are most commonly described arising from the anterior mediastinum, the posterior location of the teratoma described in this report is exceedingly rare. PMID:27660731

  16. Cystic Fibrosis

    MedlinePlus

    ... and Diseases > Lung Disease Lookup > Cystic Fibrosis Cystic Fibrosis Cystic Fibrosis (CF) is an inherited disease that ... quality of life has improved. Learn About Cystic Fibrosis Cystic fibrosis is a genetic (inherited) condition that ...

  17. Cystic hygroma

    MedlinePlus

    ... Names Lymphangioma References Richards DS. Ultrasound for pregnancy dating, growth, and the diagnosis of fetal malformations. In: ... the first to achieve this important distinction for online health information and services. Learn more about A. ...

  18. [Plunging and mediastinal goiters].

    PubMed

    Barrault, S; Gandon, J; Le Guillou, C

    1986-01-01

    Of 185 cases of substernal goitre operated upon between 1976 and 1985, four were patients with autonomous mediastinal goitre. Diagnosis was established from results of clinical examination, a scan of mediastinum and radiologie imaging with a cervical and mediastinal CT scan as the investigation of choice. Treatment is exclusively surgical because of the risk of mediastinal compression. Most cases in this series were operated upon through a purely cervical approach, but 22 patients required partial upper sternotomy combined with cervicotomy, this minimal approach route allowing very good exposure of upper mediastinum.

  19. Mediastinal Imaging Pitfalls.

    PubMed

    Lieberman, Sivan; Truong, Mylene T; Marom, Edith M

    2016-06-01

    Potential pitfalls in the interpretation of diseases involving the mediastinum are seen when imaging with computed tomography and [18F]-fluoro-2-deoxy-d-glucose positron emission tomography. These pitfalls can involve any mediastinal structure, including the mediastinal vessels, heart, lymph nodes, thymus, trachea, esophagus, and fat. Misinterpretation of normal variants or benign conditions as pathology can affect staging and alter treatment. After reading this review, the reader should be able to identify common mediastinal imaging pitfalls and apply ancillary measures to confirm the correct diagnosis and thus reach an accurate diagnosis to facilitate correct patient treatment. PMID:27261349

  20. [One Case about Primary Mediastinal Primary Mediastinal Tumor 
with Mediastinal Sarcoma and Literature Review].

    PubMed

    Du, Jinchen; Li, Qingxin; Chao, Dong; Ban, Yulian; Li, Qunqun

    2016-09-20

    Primary mediastinal seminoma is a kind of germ cell malignancy outside the gonads, and it's rarer with sarcoma component. This disease which has no special clinical manifestations and imaging characteristics is difficult to identify with other mediastinal tumors and mediastinal type lung cancer. This paper reported a case of primary mediastinal seminoma with mediastinal sarcoma. Through the analysis of the diagnosis and treatment process in this patient, we will make a comprehensive review of the disease. PMID:27666557

  1. Mediastinal thoracic duct cyst.

    PubMed Central

    Gowar, F J

    1978-01-01

    A case of mediastinal thoracic duct cyst is described; it is believed to be the first to be reported in Britain. Five surgically treated cases have been reported but in none was the diagnosis made before operation. Symptoms are caused by pressure of the cyst on the trachea and oesophagus and my be aggravted by eating a fatty meal. Differential diagnosis from other mediastinal tumours, especially bronchogenic cyst and neurofibroma, could perhaps be established before operation by lymphangiography. Images PMID:746509

  2. [Study of suprarenal cysts. Apropos of a case of cystic lymphagioma of the adrenal glands].

    PubMed

    Boscaino, N; D'Alessandro, G; Romano, C; Bruzzese, E; De Falco, A

    Following a review of the literature on the incidence and characteristics of suprarenal cysts, the case of a young woman who was suffering from bland urinary symptomatology is reported. On the basis of anatomopathological study, the case was classified as a cystic lymphangioma of the suprarenal gland arising on a malformative base.

  3. Acquired progressive lymphangioma of the nipple

    PubMed Central

    Alkhalili, Eyas; Ayoubieh, Houriya; O'Brien, William; Billings, Steven D

    2014-01-01

    A 47-year-old woman presented with left nipple itch and discomfort. On physical examination she was found to have a 7 mm lesion. She underwent bilateral mammography and bilateral breast ultrasound which were normal. A punch biopsy of the lesion was performed in the office and the specimen submitted to pathology. Histopathological examination showed ectatic vascular spaces lined by flattened, cytologically bland endothelial cells dissecting the dermal collagen. Evident lymphatic valves were present within the vascular spaces confirming that the vessels were lymphatic in nature. The diagnosis of acquired progressive lymphangioma (benign lymphangioendothelioma) was rendered. PMID:25246470

  4. Classical Cases of Lymphangioma - As Multiple Vesicular Eruptions.

    PubMed

    Devi, Anju; Narwal, Anjali; Yadav, Achla Bharti; Singh, Virender; Gupta, Ambika

    2016-06-01

    Lymphangiomas are uncommon congenital hamartomas of the lymphatic system, usually diagnosed in infancy and early childhood. They are rarely situated in oral cavity and most commonly identified on the anterior two-thirds of the tongue. Though rarely seen in the oral cavity, lymphangiomas are the entities which should be taken into consideration by the clinician while examining vesicular lesions of the oral cavity. Early recognition is of utmost importance for the initiation of proper treatment and to avoid serious complications. We hereby report two classical cases of lymphangioma of the buccal mucosa with multiple vesicular eruptions, a rare site. PMID:27504428

  5. Classical Cases of Lymphangioma – As Multiple Vesicular Eruptions

    PubMed Central

    Narwal, Anjali; Yadav, Achla Bharti; Singh, Virender; Gupta, Ambika

    2016-01-01

    Lymphangiomas are uncommon congenital hamartomas of the lymphatic system, usually diagnosed in infancy and early childhood. They are rarely situated in oral cavity and most commonly identified on the anterior two-thirds of the tongue. Though rarely seen in the oral cavity, lymphangiomas are the entities which should be taken into consideration by the clinician while examining vesicular lesions of the oral cavity. Early recognition is of utmost importance for the initiation of proper treatment and to avoid serious complications. We hereby report two classical cases of lymphangioma of the buccal mucosa with multiple vesicular eruptions, a rare site. PMID:27504428

  6. [Mediastinal germ cell tumors].

    PubMed

    Bremmer, F; Ströbel, P

    2016-09-01

    The mediastinum is among the most frequent anatomic region in which germ cell tumors (GCT) arise, second only to the gonads. Mediastinal GCT (mGCT) account for 16 % of all mediastinal neoplasms. Although the morphology and (according to all available data) the molecular genetics of mediastinal and gonadal GCT are identical, a number of unique aspects exist. There is a highly relevant bi-modal age distribution. In pre-pubertal children of both sexes, mGCT consist exclusively of teratomas and yolk sac tumors. The prognosis is generally favorable with modern treatment. In post-pubertal adults, virtually all patients with malignant mGCT are males; the prognosis is more guarded and depends (among other factors) on the histological GCT components and is similar to GCT in other organs. So-called somatic type malignancies (i. e. clonally related, non-germ cell neoplasias arising in a GCT) are much more frequent in mGCT than in other organs, and the association between mediastinal yolk sac tumors and hematological malignancies, such as myelodysplasias and leukemias, is unique to mediastinal tumors. The prognosis of GCT with somatic type malignancies is generally dismal. PMID:27491549

  7. Abdominal lymphangioma in children: report of three cases.

    PubMed

    Muramori, Katsumi; Zaizen, Yoshio; Noguchi, Shinichi

    2009-01-01

    Most cases of abdominal lymphangioma are asymptomatic. However, patients may occasionally present with acute abdomen because of an intestinal obstruction or peritonitis caused by infected cysts, hemorrhaging, and/or torsion. These conditions may differ based on the location of the lymphangioma and do not always require emergency surgery. This report presents two cases of abdominal lymphangioma derived from the omentum, which required emergency surgery because of severe abdominal distension and peritonitis caused by torsion, respectively. In contrast, another case of retroperitoneal lymphangioma presented with acute abdomen and underwent elective surgery after conservative therapy for peritonitis. Elective surgery is acceptable if the symptoms are controllable under conservative therapy. However, it is important not to overlook the possible lethal complications such as intestinal and/or urological obstruction, aggressive peritonitis, and torsion of the cyst, which require emergency surgery.

  8. Airway obstruction caused by rapid enlargement of cervical lymphangioma in a five-month-old boy.

    PubMed

    Shimizu, Junji; Taga, Takashi; Kishimoto, Takuma; Ohta, Motoki; Tagawa, Kouji; Kunitsu, Tomoaki; Yamane, Tetsunobu; Tsujita, Yasuyuki; Kubota, Yoshihiro; Eguchi, Yutaka

    2016-09-01

    Cervical lymphangioma can cause airway obstruction secondary to enlargement following infection. Physicians should be aware that the airway obstruction can progress rapidly when patients with cervical lymphangioma have respiratory symptoms. Sclerotherapy for lymphangioma can cause both transient swelling and airway obstruction; thus, prophylactic and elective tracheostomy should be considered. PMID:27648270

  9. [An unusual mediastinal opacity].

    PubMed

    Margery, J; Bâ-Fall, K; Rouquet, P; Rique, M; Lefebvre, N; Chevalier, B; Camara, P; Mbaye, P S; Debonne, J-M; Sané, M

    2006-12-01

    A 36-year old immunocompetent male from Senegal with an uneventful history was admitted for exploration of a bullous collection in the posterior mediastinum. Multifocal tuberculosis was diagnosed. Computed tomography-guided drainage removed 600 cc of caseum. The diagnosis as rupture of intrathoracic Pott's abscess complicated by a probably esophageal fistula. The clinical course was rapidly favorable with later development of mediastinal fibrosis. This uncommon case illustrates the contribution of interventional radiology recently developed in the Principal Hospital in Dakar, Senegal.

  10. Three cases of mediastinal pancreatic pseudocysts

    PubMed Central

    Fujihara, Yoshio; Maeda, Kazunori; Okamoto, Masaru; Yanagitani, Atsushi; Tanaka, Kiwamu; Nakamura, Kazuhiko; Ogawa, Toshihide

    2016-01-01

    A rare complication of acute or chronic pancreatitis is the formation of a mediastinal pancreatic pseudocyst (MPP), which is caused by tracking of pancreatic fluids through anatomical openings of the diaphragm into the mediastinum. Herein, we report the imaging characteristics of three cases of this condition. Our results revealed three features in common: (i) the connection between the mediastinum and the pancreatic cystic lesion; (ii) the presence of pleural effusions; and (iii) imaging findings consistent with chronic pancreatitis, such as pancreatic atrophy and calcifications and dilatation and/or stricture of main pancreatic duct (MPD). Serial diameter changes of the MPD and of the adjacent pseudocysts were necessary for the determination of the therapeutic strategy used in each case. PMID:27330827

  11. Torsion of the Greater Omentum Secondary to Omental Lymphangioma in a Child: A Case Report

    PubMed Central

    Mistry, Kewal Arunkumar; Iyer, Dayashankar

    2015-01-01

    Summary Background Omental cyst and omental torsion both are uncommon but important causes of acute abdomen with a difficult clinical diagnosis due to nonspecific features. Here we report a case of an eight year old child with acute abdominal pain referred for USG and CT scan which revealed two cysts in greater omentum leading to secondary omental torsion. Case Report An eight year old male child presented to casualty with severe pain abdomen since 1 day. There was no history of vomiting or altered bowel habits. The patient was febrile with tachycardia on arrival. On examination rigidity and tenderness all over abdomen were present. Serum amylase was within normal range. USG and CECT abdomen were done subsequently. USG showed two well defined cystic lesions in lower abdomen with presence of some internal echogenic debris and calcified foci in their dependent part. There was also presence of omentum with a whirl of blood vessels seen along anterior abdominal wall leading to these lesions suggesting torsion. On colour Doppler the presence of blood flow within the whirl of vessels was seen. Mild amount of free fluid was also seen in the peritoneal cavity. On CECT abdomen the findings of omental cysts and torsion of greater omentum with free fluid in abdomen were confirmed. The cysts measured 60×55 and 65×55mm on CT. The patient was taken for an emergency laparotomy for indication of acute generalized peritonitis. Two large omental cysts were found in the pelvic cavity along with torsed greater omentum along with 150 ml of hemorrhagic fluid in peritoneal cavity. The cysts and twisted necrotic part of the greater omentum were excised at surgery. No postoperative complications were observed. Histopathologic examination was suggestive of lymphangioma of omentum. Conclusions Lymphangioma of the omentum is an not very uncommon however acute presentation with omental torsion and infarction is an unusual entity. Optimal utilization of preoperative imaging with USG, Doppler

  12. [Multiple retroperitoneal and mediastinal lymph cysts in primary ectatic lymph vessels].

    PubMed

    Graf, D; Pfister, J; Streuli, H K

    1993-09-01

    A unique case of a large retroperitoneal and multiple mediastinal lymphcysts is presented. Cystic abdominal tumors of lymphatic origin are very rare. Usually single cases or small numbers have been reported in the international literature. The aetiology, clinical findings and therapy of these lesions are discussed.

  13. Fibrosing mediastinitis mimicking bronchogenic carcinoma

    PubMed Central

    Bayiz, Hulya; Mutluay, Neslihan; Koyuncu, Adem; Demirag, Funda; Dagli, Gulfidan; Berktas, Bahadir; Berkoglu, Mine

    2013-01-01

    Fibrosing mediastinitis is a rare but benign disorder characterized by an excessive fibrotic reaction in the mediastinum which can result in compromise of airways, great vessels, and other mediastinal structures. In this paper we presented a patient with fibrosing mediastinitis mimicking bronchogenic carcinoma. The patient was a 32-year-old diabetic male admitting with cough and hemoptysis. There was a right hilar mass and multiple mediastinal conglomerated lymph nodes on chest computed tomography. Positron emission tomography with computed tomography (PET/CT) scan demonstrated increased fluorodeoxyglucose (FDG) uptake at the right hilar mass lesion and mediastinal lymph nodes. Fiberoptic bronchoscopy showed mucosal distortion of right upper lobe. Pathologic examination of the mucosal biopsy revealed inflammation. Endobronchial ultrasound guided transbronchial needle and cervical mediastinoscopic lymph node biopsies were undiagnostic. Diagnostic thoracotomy confirmed the diagnosis fibrosing mediastinitis. Administration of six months of systemic corticosteroid and antituberculous therapy was not beneficial. In conclusion, despite being a rare clinical entity, fibrosing mediastinitis should be kept in mind in the differential diagnosis of mediastinal mass lesions of unknown etiology. The diagnosis is exceptionally difficult in the presence of atypical radiological findings. The treatment is particularly challenging without any proven effective therapy. PMID:23372962

  14. Minimally invasive mediastinal surgery

    PubMed Central

    Melfi, Franca M. A.; Mussi, Alfredo

    2016-01-01

    In the past, mediastinal surgery was associated with the necessity of a maximum exposure, which was accomplished through various approaches. In the early 1990s, many surgical fields, including thoracic surgery, observed the development of minimally invasive techniques. These included video-assisted thoracic surgery (VATS), which confers clear advantages over an open approach, such as less trauma, short hospital stay, increased cosmetic results and preservation of lung function. However, VATS is associated with several disadvantages. For this reason, it is not routinely performed for resection of mediastinal mass lesions, especially those located in the anterior mediastinum, a tiny and remote space that contains vital structures at risk of injury. Robotic systems can overcome the limits of VATS, offering three-dimensional (3D) vision and wristed instrumentations, and are being increasingly used. With regards to thymectomy for myasthenia gravis (MG), unilateral and bilateral VATS approaches have demonstrated good long-term neurologic results with low complication rates. Nevertheless, some authors still advocate the necessity of maximum exposure, especially when considering the distribution of normal and ectopic thymic tissue. In recent studies, the robotic approach has shown to provide similar neurological outcomes when compared to transsternal and VATS approaches, and is associated with a low morbidity. Importantly, through a unilateral robotic technique, it is possible to dissect and remove at least the same amount of mediastinal fat tissue. Preliminary results on early-stage thymomatous disease indicated that minimally invasive approaches are safe and feasible, with a low rate of pleural recurrence, underlining the necessity of a “no-touch” technique. However, especially for thymomatous disease characterized by an indolent nature, further studies with long follow-up period are necessary in order to assess oncologic and neurologic results through minimally

  15. Magnetic resonance imaging of cystic pancreatic lesions in adults: an update in current diagnostic features and management.

    PubMed

    Barral, Matthias; Soyer, Philippe; Dohan, Anthony; Laurent, Valérie; Hoeffel, Christine; Fishman, Elliot K; Boudiaf, Mourad

    2014-02-01

    Magnetic resonance (MR) imaging has become a widespread diagnostic solving tool for the detection and characterization of a large range of pancreatic cystic lesions. Benign and malignant cystic lesions of the pancreas including serous microcystic adenoma, mucinous cystic tumor, intraductal papillary mucinous tumor, solid pseudopapillary tumor, and also the less common lesions such as cystic endocrine tumors, cystic metastases, and lymphangiomas have suggestive MR imaging presentation that allows them to be differentiated from each other. Knowledge of MR imaging findings of cystic pancreatic lesions is critical to help suggest the diagnosis and chose the best therapeutic approach. The purpose of this review is to discuss and illustrate MR imaging features that are helpful for pancreatic cystic lesion detection and characterization and to provide an update in current MR imaging diagnostic features and management. PMID:24263208

  16. Mediastinal and retroperitoneal teratoma with focal gastrointestinal adenocarcinoma.

    PubMed

    Chang, Y-L; Wu, C-T; Lee, Y-C

    2006-09-01

    We report an unusual case of gastrointestinal adenocarcinoma arising in a giant posterior mediastinal mature cystic teratoma extending into the retroperitoneum, which was treated by complete excision with a good outcome for more than 2 years. Teratomas with malignant transformation are rare non-germ cell malignant tumors arising from a preexisting mature teratoma. Histological examination revealed that the cyst wall was composed of mature ectodermal, mesodermal, and endodermal elements. Neoplastic glands with a cribriform pattern were found in a small, solid nodule. Strong cytokeratin 20 cytoplasmic immunostaining of the tumor cells supported the diagnosis of gastrointestinal adenocarcinoma. In this report, we describe the potential aggressiveness of a giant mature cystic teratoma with adenocarcinoma and suggest that complete surgical resection without adjuvant chemotherapy be considered as a therapy in the treatment of teratoma with focal malignant transformation.

  17. 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.

  18. Lymphangioma of the Lower Lip Mimicking a Mucocele in Children.

    PubMed

    de Carvalho, Fabrício Kitazono; Pinheiro, Tiago Novaes; da Silva, Raquel Assed Bezerra; de Queiroz, Alexandra Mussolino; da Silva, Lea Assed Bezerra; Nelson-Filho, Paulo

    2015-01-01

    Lymphangioma is a hamartoma resulting from the proliferation of common lymphatic vessels in the head and neck area, rarely appearing in the lower lips. Its clinical presentation is a nodular mass with a pebbly surface and no defined borders formed by a cluster of slow-growing coalescing vesicles. The purpose of this paper is to present two children with a lesion in the lower lip whose clinical characteristics (single papillary lesion with a pediculated base, same color and consistency of the surrounding mucosa, and negative result for diascopy test) suggested an initial diagnosis of mucocele but were later confirmed as cavernous lymphangiomas. The clinical and microscopic characteristics of the lesions are discussed as well as the differential diagnosis and the treatment approach. These cases reinforce the importance of always performing a confirmatory histopathological analysis, even for lesions with typical clinical features. PMID:26349801

  19. An Unusual Origin of Fetal Lymphangioma Filling Right Axilla.

    PubMed

    Ersoy, Ali Ozgur; Oztas, Efser; Saridogan, Erdinc; Ozler, Sibel; Danisman, Nuri

    2016-03-01

    Fetal lymphangioma is a hamartomatous congenital anomaly of the lymphatic system, which is embracing the fetal skin (sometimes mucous membranes) and the subcutaneous tissue. The general consensus is that it occurs as a result of failure in lymphatic drainage. A 36-year-old pregnant woman was referred to our perinatology clinic at 22 weeks' gestation, because of a fetal right-sided axillary mass revealed by ultrasonography. The mass measuring 5x7x7cm in three dimensions had a multilocular structure without colour Doppler flow and well-circumscribed borders. Amniocentesis revealed a normal constitutional karyotyping. Lymphangioma was considered as prediagnosis. A healthy female baby weighing 3470 grams was delivered at term. Neonatal examination and the postnatal MRI confirmed the diagnosis. The baby is still on follow-up with the medical treatment of Sirolimus an anti-proliferative drug, and the mass got smaller significantly in 8 months after delivery. PMID:27134953

  20. Thyroid Storm and Incidental Anterior Mediastinal Teratoma: Coincidence or Correlation?

    PubMed Central

    Wang, Cheng-An; Chen, Wei-Ta; Cheng, Ho-Shun; Chung, Cheng-Chih; Chen, Yu-Ju; Hsieh, Ming-Hsiung

    2013-01-01

    Teratoma is a rare cause of thyrotoxicosis. Among the different types of teratoma, struma ovarii is the main type which contains thyroid tissue. There is no evidence in the literature that would indicate mediasternal teratoma would also lead to thyrotoxicosis or thyroid storm. Herein we report a 37-year-old woman who suffered from palpitation. Her chest X-ray showed a mass lesion at the left hilum, and chest computed tomography scan yielded a suspicion of pericardial cyst. Thereafter, video-assisted thoracoscopic surgery was performed, and thymic cyst was diagnosed during the operation. However, subsequent pathological studies confirmed a diagnosis of mature cystic teratoma. A thyroid function test demonstrating hyperthyroidism was completed prior to the patient’s operation, and thyroid storm was diagnosed by clinical presentation. The patient’s symptoms did not improve after the operation until we added beta blocker and anti-thyroid agents. Therefore, was the presence of thyroid storm and anterior mediastinal teratoma coincident or correlative in this case? The special stain of teratoma tissues did not reveal any thyroid tissues. In conclusion, thyroid storm and anterior mediastinal teratoma in our case occurred coincidentally. However, a survey of possible hyperthyroidism in patients with anterior mediastinal tumor before operation is critical to avoid perioperative complications. PMID:27122746

  1. Lymphangioma of the retroperitoneum: CT and sonographic characteristic.

    PubMed

    Davidson, A J; Hartman, D S

    1990-05-01

    The authors retrospectively evaluated radiologic, clinical, and pathologic findings in 19 cases of lymphangioma of the retroperitoneum. The tumors were judged confined to one compartment of the retroperitoneum in 68% of the cases, whereas in 32% of cases the tumor involved more than one compartment. Abdominal radiography depicted the mass in all cases. Excretory urography demonstrated organ displacement without tumor invasion in all cases. Sonography showed multiloculated fluid in 61% of cases and a unicameral mass in 39% of cases. All but one of the multiloculated lymphangiomas had thick septa. Sonography also depicted the fluid as uncomplicated in 56% of cases. The remainder had debris that sometimes layered in the dependent portion of the cyst. Computed tomography (CT) showed a unicameral mass in 57% and a septated mass in 43% of cases. CT also showed thin, smooth walls in 79% and thick, irregular walls in 21% of cases. At CT the fluid contents were found to be homogeneous and of fluid attenuation in 64% and were complex in 36% of cases. The attenuation of fluid in one case was the same as that of retroperitoneal fat. In two cases the mass contained mural calcification. The most characteristic radiologic finding of lymphangioma of the retroperitoneum is an elongated tumor containing uncomplicated fluid with or without septa. Chyle and mural calcification are very uncommon in this location.

  2. Pulmonary Hypertension Complicating Fibrosing Mediastinitis

    PubMed Central

    Seferian, Andrei; Steriade, Alexandru; Jaïs, Xavier; Planché, Olivier; Savale, Laurent; Parent, Florence; Amar, David; Jovan, Roland; Fadel, Elie; Sitbon, Olivier; Simonneau, Gérald; Humbert, Marc; Montani, David

    2015-01-01

    Abstract Fibrosing mediastinitis is caused by a proliferation of fibrous tissue in the mediastinum with encasement of mediastinal viscera and compression of mediastinal bronchovascular structures. Pulmonary hypertension (PH) is a severe complication of fibrosing mediastinitis caused by extrinsic compression of the pulmonary arteries and/or veins. We have conducted a retrospective observational study reviewing clinical, functional, hemodynamic, radiological characteristics, and outcome of 27 consecutive cases of PH associated with fibrosing mediastinitis diagnosed between 2003 and 2014 at the French Referral Centre for PH. Fourteen men and 13 women with a median age of 60 years (range 18–84) had PH confirmed on right heart catheterization. The causes of fibrosing mediastinitis were sarcoidosis (n = 13), tuberculosis-infection confirmed or suspected (n = 9), mediastinal irradiation (n = 2), and idiopathic (n = 3). Sixteen patients (59%) were in NYHA functional class III and IV. Right heart catheterization confirmed moderate to severe PH with a median mean pulmonary artery pressure of 42 mm Hg (range 27–90) and a median cardiac index of 2.8 L/min/m2 (range 1.6–4.3). Precapillary PH was found in 22 patients, postcapillary PH in 2, and combined postcapillary and precapillary PH in 3. Severe extrinsic compression of pulmonary arteries (>60% reduction in diameter) was evidenced in 2, 8, and 12 patients at the main, lobar, or segmental levels, respectively. Fourteen patients had at least one severe pulmonary venous compression with associated pleural effusion in 6 of them. PAH therapy was initiated in 7 patients and corticosteroid therapy (0.5–1 mg/kg/day) was initiated in 3 patients with sarcoidosis, with 9 other being already on low-dose corticosteroids. At 1-year follow-up, 3 patients had died and among the 21 patients evaluated, 3 deteriorated, 14 were stable, and only 4 patients with sarcoidosis improved (4 receiving corticosteroids and 1

  3. Post extubation negative pressure pulmonary edema due to posterior mediastinal cyst in an infant.

    PubMed

    Dubey, Prakash Kumar

    2014-01-01

    A 3-month-old male child underwent uneventful inguinal herniotomy under general anesthesia. After extubation, airway obstruction followed by pulmonary edema appeared for which the baby was reintubated and ventilated. The baby made a complete recovery and extubated after about 2 h. A post-operative computed tomography scan revealed a posterior mediastinal cystic mass abutting the tracheal bifurcation. Presumably, extrinsic compression by the mass on the tracheal bifurcation led to the development of negative pressure pulmonary edema.

  4. Combined mediastinal and retroperitoneal fibrosis

    PubMed Central

    Salmon, H. W.

    1968-01-01

    A case of combined idiopathic mediastinal fibrosis and retroperitoneal fibrosis is described. It is possibly the twelfth case to be reported during life. A review of the literature reveals the `ubiquity' of localized collagenosis and the trend of opinion as regards aetiology and treatment. Images PMID:5654073

  5. The 'Pinocchio' nasal deformity due to cavernous lymphangioma.

    PubMed

    Hobby, J L; Tiernan, E; Mayou, B J

    1995-09-01

    The 'Pinocchio' or 'Cyrano' nose is a rare condition in which deformity of the nasal tip is produced by an underlying soft tissue tumour. Previously reported cases have been due to either capillary or cavernous haemangiomas (angiolipomas). The deformity is the cause of much teasing in children. There has been debate as to whether surgical intervention is indicated, as a proportion of cases will regress spontaneously. We report a case of 'Pinocchio' nose with a lymphangioma of the nasal tip which is previously undescribed and review the options for management.

  6. The 'Pinocchio' nasal deformity due to cavernous lymphangioma.

    PubMed Central

    Hobby, J L; Tiernan, E; Mayou, B J

    1995-01-01

    The 'Pinocchio' or 'Cyrano' nose is a rare condition in which deformity of the nasal tip is produced by an underlying soft tissue tumour. Previously reported cases have been due to either capillary or cavernous haemangiomas (angiolipomas). The deformity is the cause of much teasing in children. There has been debate as to whether surgical intervention is indicated, as a proportion of cases will regress spontaneously. We report a case of 'Pinocchio' nose with a lymphangioma of the nasal tip which is previously undescribed and review the options for management. Images Figure 1 Figure 2 PMID:7562857

  7. Renal peripelvic lymphatic cysts (lymphangiomas) associated with generalized lymphangiomatosis.

    PubMed

    Younathan, C M; Kaude, J V

    1992-01-01

    Renal peripelvic lymphatic cysts (lymphangiomas) are usually seen as an isolated finding. They appear as multiple mass lesions with low attenuation on computed tomography (CT), and show no contrast enhancement. We present a case of renal peripelvic lymphatic cysts associated with generalized lymphangiomatosis, and examined with magnetic resonance imaging (MRI), CT, and lymphangiography. Multiplanar MRI demonstrated the extent of disease, including retroperitoneal lesions. The lesions had low-signal intensity on T1-weighted and high-signal intensity on T2-weighted images. The diagnosis of generalized lymphangiomatosis was verified by renal sinus cyst puncture, lymphangiography, and tissue biopsy.

  8. Dental care management of a young patient with extensive lymphangioma of the tongue: a case report.

    PubMed

    de Queiroz, Alexandra Mussolino; Silva, Raquel A B; Margato, Loureiro Cristiane; Nelson-Filho, Paulo

    2006-01-01

    Lymphangiomas are benign tumors resulting from a congenital malformation of the lymphatic system. Relatively uncommon, lymphangiomas are usually diagnosed at birth and develop within the first years of life. When these tumors occur in the oral cavity, the tongue is the most frequently affected site. Lymphangioma of the tongue is a common cause of macroglossia in children, which may lead to a dry/cracked tongue with ulcerating secondary infections, difficulty in swallowing and mastication, speech disturbances, exclusive nasal breathing, airway obstruction, mandibular prognathism and other possible deformities of maxillofacial structures. This paper discusses the most relevant features, clinical manifestations, disease-related impairments and treatment options for lymphangioma of the tongue. It presents the case report of a five-year-old child diagnosed with this lesion, including a description of the patient's dental care management.

  9. Lymphangioma of the tongue - a case report and review of literature.

    PubMed

    V, Usha; Sivasankari, T; Jeelani, S; Asokan, G S; Parthiban, J

    2014-09-01

    Lymphangiomas are benign tumours resulting from a congenital malformation of the lymphatic system. They are relatively uncommon and usually diagnosed in infancy and early childhood. Commonly located at head and neck, they rarely occur in the oral cavity. Intraoral lymphangiomas occur more frequently on the dorsum of tongue, followed by palate, buccal mucosa, gingiva, and lips. Lymphangioma of the tongue is a common cause of macroglossia in children associated with difficulty in swallowing and mastication, speech disturbances, airway obstruction, mandibular prognathism, openbite and other possible deformities of maxillofacial structures. We present the case of a 13-year-old female with lymphangioma of tongue. The clinical, radiological, and treatment modalities of this case are discussed. PMID:25386537

  10. Cystic Echinococcosis

    PubMed Central

    Brunetti, Enrico; McCloskey, Cindy

    2015-01-01

    Echinococcosis is one of the 17 neglected tropical diseases (NTDs) recognized by the World Health Organization. The two major species of medical importance are Echinococcus granulosus and Echinococcus multilocularis. E. granulosus affects over 1 million people and is responsible for over $3 billion in expenses every year. In this minireview, we discuss aspects of the epidemiology, clinical manifestations, and diagnosis of cystic echinococcosis or cystic hydatid disease caused by E. granulosus. PMID:26677245

  11. Cystic Echinococcosis.

    PubMed

    Agudelo Higuita, Nelson Iván; Brunetti, Enrico; McCloskey, Cindy

    2016-03-01

    Echinococcosis is one of the 17 neglected tropical diseases (NTDs) recognized by the World Health Organization. The two major species of medical importance are Echinococcus granulosus and Echinococcus multilocularis. E. granulosus affects over 1 million people and is responsible for over $3 billion in expenses every year. In this minireview, we discuss aspects of the epidemiology, clinical manifestations, and diagnosis of cystic echinococcosis or cystic hydatid disease caused by E. granulosus. PMID:26677245

  12. Cystic fibrosis - resources

    MedlinePlus

    Resources - cystic fibrosis ... The following organizations are good resources for information on cystic fibrosis : Cystic Fibrosis Foundation -- www.cff.org March of Dimes -- www.marchofdimes.org/baby/cystic-fibrosis-and- ...

  13. Complete Resolution of Retroperitoneal Lymphangioma with a Single Trial of OK-432 in an Infant.

    PubMed

    Olivieri, Claudio; Nanni, Lorenzo; De Gaetano, Anna Maria; Manganaro, Lucia; Pintus, Claudio

    2016-06-01

    Retroperitoneal lymphangioma is extremely rare. Although these neoplasms are benign, they can grow progressively with subsequent compression and infiltration of the adjacent structures. Surgical excision is demanding when the lesion surrounds vital structures and it is generally fraught with a high recurrence and morbidity rate. We report the case of a huge retroperitoneal lymphangioma in a newborn treated successfully with intracystic injection of OK-432.

  14. Optimal treatment of descending necrotising mediastinitis

    PubMed Central

    Corsten, M. J.; Shamji, F. M.; Odell, P. F.; Frederico, J. A.; Laframboise, G. G.; Reid, K. R.; Vallieres, E.; Matzinger, F.

    1997-01-01

    BACKGROUND: Descending necrotising mediastinitis is caused by downward spread of neck infection and has a high fatality rate of 31%. The seriousness of this infection is caused by the absence of barriers in the contiguous fascial planes of neck and mediastinum. METHODS: The recent successful treatment of seven adult patients with descending necrotising mediastinitis emphasises the importance of optimal early drainage of both neck and mediastinum and prolonged antibiotic therapy. The case is also presented of a child with descending necrotising mediastinitis, demonstrating the rapidity with which the infection can develop and lead to death. Twenty four case reports and 12 series of adult patients with descending necrotising mediastinitis published since 1970 were reviewed with meta-analysis. In each case of confirmed descending necrotising mediastinitis the method of surgical drainage (cervical, mediastinal, or none) and the survival outcome (discharge home or death) were noted. The chi 2 test of statistical significance was used to detect a difference between cases treated with cervical drainage alone and cases where mediastinal drainage was added. RESULTS: Cervical drainage alone was often insufficient to control the infection with a fatality rate of 47% compared with 19% when mediastinal drainage was added (p < 0.05). CONCLUSIONS: Early combined drainage with neck and chest incisions, together with broad spectrum intravenous antibiotics, should be considered standard care for this disease. 


 PMID:9337829

  15. Pitfalls in CT recognition of mediastinal lymphadenopathy

    SciTech Connect

    Glazer, H.S.; Aronberg, D.J.; Sagel, S.S.

    1985-02-01

    Computed tomography (CT) has become the most accurate radiologic technique for the evaluation of mediastinal lymph nodes. Since the introduction of thoracic CT, a variety of anatomic structures, both normal and aberrant, have been described that can be confused with enlarged mediastinal lymph nodes; these represent potential diagnostic pitfalls. This essay illustrates many of these structures and distinguish them from abnormal lymph nodes.

  16. Retroperitoneal cystic masses: CT, clinical, and pathologic findings and literature review.

    PubMed

    Yang, Dal Mo; Jung, Dong Hae; Kim, Hana; Kang, Jee Hee; Kim, Sun Ho; Kim, Ji Hye; Hwang, Hee Young

    2004-01-01

    Cystic lesions of the retroperitoneum can be classified as either neoplastic or nonneoplastic. Neoplastic lesions include cystic lymphangioma, mucinous cystadenoma, cystic teratoma, cystic mesothelioma, müllerian cyst, epidermoid cyst, tailgut cyst, bronchogenic cyst, cystic change in solid neoplasms, pseudomyxoma retroperitonei, and perianal mucinous carcinoma. Nonneoplastic lesions include pancreatic pseudocyst, nonpancreatic pseudocyst, lymphocele, urinoma, and hematoma. Because the clinical implications of and therapeutic strategies for retroperitoneal cystic masses vary depending on the cause, the ability to noninvasively differentiate between masses is important. Although there is substantial overlap of computed tomographic (CT) findings in various retroperitoneal cysts, some CT features, along with clinical characteristics, may suggest a specific diagnosis. CT may provide important information regarding lesion location, size, and shape; the presence and thickness of a wall; the presence of septa, calcifications, or fat; and involvement of adjacent structures. The most important clinical parameters include patient gender, age, symptoms, and clinical history. Familiarity with the CT and clinical features of various retroperitoneal cystic masses facilitates accurate diagnosis and treatment.

  17. Cystic Fibrosis

    MedlinePlus

    Cystic fibrosis (CF) is an inherited disease of the mucus and sweat glands. It affects mostly your lungs, pancreas, liver, intestines, sinuses, and sex organs. CF causes your mucus to be thick and sticky. The mucus clogs the lungs, causing breathing problems and ...

  18. Mediastinal impalement with a fibreglass sheet

    PubMed Central

    Wimalachandra, Welege Samantha Buddhika; Asmat, Atasha

    2014-01-01

    Mediastinal impalement injuries are uncommon and often fatal. There have been very few reported cases of survival following mediastinal impalement. Patients who present with these injuries always undergo operative intervention regardless of their underlying haemodynamic status or associated injuries. We herein present a case of mediastinal impalement injury, where a sheet of fibreglass had fractured the manubrium and entered thffige anterior mediastinum with no associated great vessel injury. The fibreglass sheet was removed via a partial sternotomy and the patient made an uneventful postoperative recovery. PMID:25273944

  19. Primary mediastinal seminoma. [X ray

    SciTech Connect

    Bush, S.E.; Martinez, A.; Bagshaw, M.A.

    1981-10-15

    Primary mediastinal seminoma is a rare germ cell neoplasm histologically identical to testicular seminoma. Thirteen patients have been treated with definitive megavoltage radiotherapy at the Stanford University Medical Center, Division of Radiation Therapy, between 1961 and 1976. Doses ranged from 2500 rads over five weeks to 6000 rads over seven weeks. Actuarial survival at ten years is 69% with relapse-free survival of 54%. No patient receiving greater than 4700 rads to the primary lesion had local or systemic relapse. Patterns of relapse and recommendations for diagnostic evaluation are discussed. Primary megavoltage irradiation to the mediastinum and neck with a dose of 4500 to 5000 rads over five to six weeks is appropriate treatment.

  20. Enlarging mediastinal/hilar lymphadenopathy with calcification.

    PubMed

    Adachi, Takashi; Nakahata, Masashi; Moritani, Suzuko; Iida, Hiroatsu; Ogawa, Kenji

    2016-02-01

    A 77-year-old man was referred to our hospital due to enlarging mediastinal/hilar lymphadenopathy with calcification. Endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) and bone marrow aspiration were performed. Subsequently, monoclonal gammopathy of undetermined significance (MGUS) associated with mediastinal amyloidosis was diagnosed. We hereby report a case in which EBUS-TBNA led to a successful diagnosis of amyloidosis. PMID:26862422

  1. Descending necrotizing mediastinitis associated with Lactobacillus plantarum

    PubMed Central

    2013-01-01

    Background Descending necrotizing mediastinitis (DNM), a severe infection with a high fatality rate, develops in mediastinal spaces due mainly to deep cervical abscesses. The majority of causative microbes of DNM are Streptococci and oral anaerobes. DNM associated with Lactobacillus-infection is rather rare. Case presentation A 69-year-old male with an unremarkable past medical history was referred to our hospital for surgical resection of advanced laryngeal cancer. Full examination revealed a neck abscess and DNM with a background of untreated diabetes mellitus. Initially, he was treated with meropenem. However, Lactobacillus plantarum was isolated from surgical drainage of a mediastinal abscess. Despite using antibiotics capable of eradicating all isolates with susceptibilities not differing significantly from those of the neck and mediastinal abscesses, we attributed DNM to the L. plantarum detected only in the mediastinal abscess. After DNM treatment, he underwent total pharyngolaryngectomy with bilateral neck dissection followed by reconstruction using free jejunum. He was discharged fully recovered. Conclusion We concluded that L. plantarum as the sole cause of the mediastinal abscess in the present case cannot be ruled out. As the number of immunocompromised patients increases, we should be cautious regarding this “familiar” microbe. PMID:23987907

  2. Videothoracoscopy in the treatment of mediastinal cysts

    PubMed Central

    Brzeziński, Daniel; Kozak, Józef

    2014-01-01

    Introduction Progress in the development of surgical techniques has led to the growing use of video-assisted thoracoscopic surgery (VATS) techniques in mediastinal cyst treatment. Aim To present our experience of treating mediastinal cysts with the minimally invasive technique. Material and methods Fifty patients with mediastinal cysts were treated from 2001 to 2011. There were 32 women and 18 men. The age of the patients ranged from 17 to 72, the mean age being 42 years. All patients underwent basic preoperative diagnostic tests of the chest: X-ray, computed tomography (CT), bronchoscopy and spirometry; 4 patients underwent endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) and 3 fine needle aspiration biopsy (FNAB) of the described lesions. Results The VATS was performed in each case. Conversion was carried out due to superior mediastinal location in 10 cases and pleural adhesions in 3 cases. The partial resection of a cyst was performed in 3 patients. One patient was treated conservatively due to heart failure. In that patient the transthoracic needle aspiration of a cyst under ultrasound guidance using alcoholisation with 76% ethanol with a good effect was performed twice. Cyst recurrence was observed in 1 case. Conclusions The surgical access depends on the location of a cyst. The VATS resection of a superior mediastinal cyst is not always feasible. Surgery of mediastinal cysts is both diagnostic and curative. PMID:25337163

  3. Mediastinal lymph node size in lung cancer

    SciTech Connect

    Libshitz, H.I.; McKenna, R.J. Jr.

    1984-10-01

    Using a size criterion of 1 cm or greater as evidence for abnormality, the size of mediastinal lymph nodes identified by computed tomography (CT) was a poor predictor of mediastinal lymph node metastases in a series of 86 patients who had surgery for bronchogenic carcinoma. The surgery included full nodal sampling in all patients. Of the 86 patients, 36 had nodes greater than or equal to 1 cm identified by CT. Of the 21 patients with mediastinal metastases proven at surgery, 14 had nodes greater than or equal to 1 cm (sensitivity = 67%). Of the 65 patients without mediastinal metastases, 22 had nodes greater than or equal to 1 cm. Obstructive pneumonia and/or pulmonary collapse distal to the cancer was present in 39 patients (45%). Obstructive pneumonia and/or pulmonary collapse is a common occurrence in bronchogenic carcinoma, but mediastinal nodes greater than or equal to 1 cm in this circumstance cannot be presumed to represent metastatic disease. Metastatic mediastinal lymph node involvement was related to nodal size also in patients with evidence of prior granulomatous disease and in patients with no putative benign cause for nodes greater than or equal to 1 cm.

  4. The use of the carbon dioxide laser in head and neck lymphangioma.

    PubMed

    White, B; Adkins, W Y

    1986-01-01

    The carbon dioxide laser has been used to treat various lesions of the head and neck, ranging from carcinomas to hemangiomas, and even including tatoos. A search of the literature does not reveal any reports of the carbon dioxide laser being used to treat lymphangioma. This report discusses the efficacy of treating lymphangioma of the air and food passages with the carbon dioxide laser, and presents three patients who have been treated in this fashion-two for palliation and one for cure. PMID:2426548

  5. Lymphangioma of the Ileocecal Valve Clinically Masquerading as a Submucosal Small Intestinal Gist: Report of a Case and Literature Review

    PubMed Central

    Al-Obeed, Omar A.; Abdulla, Maha-Hamadien

    2014-01-01

    Lymphangiomas are rare tumors affecting the gastrointestinal tract, and may be seen in the bowel, gall bladder, and pancreas. They resemble hemangiomas, but consist of spaces of variable sizes containing lymph. In this report, we describe the case of a 53-year-old male who presented with abdominal pain and constipation. Computerized tomography (CT) scan showed a polypoidal lesion at the ileocecal valve which was thought to be a gastrointestinal stromal tumor. Resected specimen did, however, show a lymphangioma. We also describe the clinicopathologic features of gastrointestinal lymphangiomas with a literature review. PMID:25038213

  6. Spontaneous Retropharyngeal and Mediastinal Emphysema.

    PubMed

    Cho, Do-Yeon; Aaron, Geoffrey P; Shepard, Kimberly G

    2016-06-01

    A 14-year-old girl with no significant medical history presented at Emergency Department with sore throat and odynophagia after one episode of nonviolent coughing. She denied any respiratory distress, voice change, foreign body ingestion, retching, substance abuse, dental procedures, or trauma. She was afebrile with normal oxygen saturation and physical examination including the head and neck was unremarkable with the exception of bilateral neck crepitus without tenderness on palpation. Fiberoptic laryngoscopy revealed a patent laryngeal airway with normal vocal fold movement. Lateral neck X-ray demonstrated a linear air-column in the retropharyngeal space and computed tomography confirmed emphysema involving the retropharyngeal space and mediastinum with no evidence of fluid collection or abscess formation. Spontaneous retropharyngeal and mediastinal emphysema are clinical entities where free air is present within the confines of retropharyngeal space and mediastinum without obvious cause. It is benign and self-limited in nature and allows for conservative management. This case is presented with a review of literature. PMID:27090269

  7. [Prognosis of childhood mediastinal lymphoma].

    PubMed

    Okamura, J; Ikuno, Y; Sakata, N; Kai, T; Tasaka, H

    1990-11-01

    Between 1973 and 1989, 16 children with non-Hodgkins lymphoma (NHL) with a mediastinal mass (MM) were treated at our institution with multi-agent chemotherapy and radiotherapy. They also received central nervous system (CNS) prophylaxis including intrathecal methotrexate administration (14 cases) and cranial irradiation (7 cases). Twelve were boys and 4 girls. Median age was 11 +/- 3. One patient died of air way obstruction one day after admission. Fourteen of 15 patients entered into complete remission (CR) and one patient partial remission. Five remains in CR 7 to 175 months after diagnosis (median 76 months). Nine patients relapsed in the bone marrow (3 cases), CNS (3), testicles (1), neck lymph node (1) and bones plus kidneys (1). Of these, 7 patients died within 13 months after initial relapse. The disease free survival (DFS) and overall survival of all patients were 27% and 33%, respectively. Analysis of the prognostic factors among patients with MM+ -NHL revealed that the serum LDH level below 1,000IU/l was a good prognostic sign. Other factors such as age, stage, initial WBC count, size of MM and response of the MM to the initial treatment did not correlate with DFS. Because of its rarity and the poor treatment result, we need more aggressive treatment program by a multiinstitutional study for MM+ -NHL. PMID:2287064

  8. A case of HPV and acquired genital lymphangioma: over-lapping clinical features.

    PubMed

    Cestaro, Giovanni; De Rosa, Michele; Gentile, Maurizio; Massa, Salvatore

    2015-03-25

    Lymphatic malformation or lymphangioma is a benign proliferation of the lymphatics accounting for 4% of all vascular malformations and 26% of all benign vascular tumors. There are several reports about genital lymphangiomas mimicking venereal lesions, such as genital warts. Hereby we described a case of a 24 year old man affected by multiple vesicles and warts in genital area. All hematological and biochemical parameters, Human Immunodeficiency Virus (HIV) and Treponema Pallidum tests, C1-Inhibitor and C1-Q values were within limits. An accurate fulguration and wide excision of bigger lesions were performed. Histological examination showed numerous dilated lymphatic vessels in the superficial dermis with infiltration of inflammatory cells, that is a histopathological picture compatible with genital lymphangioma. Considering our clinical suspicion of condylomatosis, HPV (Human Papilloma Virus) Polimerase Chain Reaction (PCR) Genotyping, named INNOLiPA test, was performed, that revealed a genital infection by HPV - genotype 6. We think that our case can be considered an example of HPV infection and acquired genital lymphangioma overlap clinical syndrome. The patient presented any lesions one year after the procedure at follow-up examination.

  9. Isolation and characterization of lymphatic endothelial cells from human glossal lymphangioma.

    PubMed

    You, Lanying; Wu, Mingfu; Chen, Ying; Xu, Gang; Wei, Juncheng; Li, Qiong; Song, Anping; Zhao, Liangpin; Li, Shuang; Han, Zhiqiang; Zhou, Jianfeng; Lu, Yunpin; Wang, Shixuan; Ma, Ding; Meng, Li

    2010-01-01

    Abnormal lymphangiogenesis is associated with several diseases such as tumor metastasis and lymphangioma. Human lymphangioma originated from the transformation of lymphatic endothelium is a benign malformation of lymphatic vessels and its pathogenesis has up to date not been illuminated and its cell model has also not been established. An optimized method was used to isolate lymphatic endothelial cells from human glossal lymphangioma (GL-LECs) and GL-LECs were further primarily cultured and expanded. GL-LECs were of typical cobblestone appearance when they reached confluence. The weible-palade body was observed in the GL-LECs cytoplasm. Almost all GL-LECs were strongly positive for specific lymphatic markers FLT-4, LYVE-1 and prox-1 by immunocytochemistry. Furthermore, three-dimension tube-like capillaries of GL-LECs resembled the lymphatic system in vivo, and the GL-LECs spheroids sprouted radically out to form three-dimensional buds when embedded in the cultured BME. These results indicated that high purity GL-LECs were successfully isolated and expanded. They had the abilities of tube formation and differentiation in vitro, which provide a favorable cell model for further uncovering the pathogenesis of human lymphangiomas.

  10. Jejunal lymphangioma: an unusual cause of intussusception in an adult patient.

    PubMed

    Limaiem, F; Khalfallah, T; Marsaoui, L; Bouraoui, S; Lahmar, A; Mzabi, S

    2015-03-01

    Adult intussusception is a relatively rare clinical entity. Almost 90% of cases of intussusception in adults are secondary to a pathologic condition that serves as a lead point. Lymphangioma of the small bowel is an unusual tumour that has been rarely reported to cause intussusception. In this paper, we present a rare case of adult intussusception due to jejunal lymphangioma. A 22-year-old female patient with a medical history significant for anaemia presented with intermittent colicky abdominal pain, diarrhoea and oedema of the inferior limbs for the past three months. Ultrasonography and CT scan revealed a typical target sign with dilated intestinal loops. At laparotomy, a jejuno-jejunal intussusception was found. Partial resection of the jejunum was performed. Macroscopic examination of the surgical specimen revealed a pedunculated polyp measuring 2 cm in diameter. Histological sections of the polyp revealed in the lamina propria and submucosal layer of the jejunum several markedly dilated thin-walled lymphatic spaces lined with single layers of flat endothelial cells. The final pathologic diagnosis was submucosal lymphangioma. This case report indicates that intussusception, although rare in adults, should be considered in the differential diagnosis of abdominal pain. Moreover, it should be taken into consideration that lymphangioma is one of the possible lesions that can cause intussusception.

  11. [The diagnostics and treatment of the postoperative mediastinitis].

    PubMed

    Kokhan, E P; Aleksandrov, A S

    2011-01-01

    Treatment results of 48 patients with mediastinitis after the open cardiac surgery were analyzed. The main mediastinitis risk factors were: the chronic obstructive lung desease, diabetes mekkitus, severe cardiac insufficiency, prolonged operation, the use of artificial blood circulation and sterna instsbility. Major infective agents of mediastinitis were the Gram-positive cocci. The treatment options varied from the prolonged antibiotic therapy to plastic reconstructive surgery, which were determined by the severity of mediastinitis. PMID:22413155

  12. Primary mesenteric lymphangioma in a young adult with intestinal malrotation and ‘counter-clockwise barber pole sign’

    PubMed Central

    Singh, Neha; Singh, Ragini; Maheswari, Udbhav; Aga, Pallavi

    2013-01-01

    Mesenteric lymphangiomas are uncommon benign lesions that usually occur in isolation but rarely may be associated with gastrointestinal (GI) malrotation. Malrotation may cause chronic and recurrent volvulus leading to chronic venous congestion and lymphatic engorgement. Interference with lymphatic drainage may result in formation of a lymphangioma or a chylous mesenteric cyst. In our case, mesenteric lymphangioma was present with partial malrotation of small and large bowel. There was no radiological and surgical evidence of volvulus, favouring the hypothesis that lymphangioma may be a primary congenital association of GI malrotation. Another interesting finding was the counter-clockwise twisting of the superior mesenteric vein around the superior mesenteric artery which has been reported only seven times in the literature. PMID:23761495

  13. Mediastinal syndrome: A report of three cases

    PubMed Central

    Zardi, Enrico Maria; Pipita, Maria Elena; Afeltra, Antonella

    2016-01-01

    Mediastinal syndromes are a group of disorders involving the anatomical structures of the mediastinum. An increase in the incidence of this syndrome has been observed following the widespread use of diagnostic imaging. In the present study, three different cases of mediastinal syndrome, one of which was complicated by superior vena cava syndrome, are discussed. The treatment in the three cases was dependent on the etiology. While a patient with goiter experienced resolution of the symptoms following thyroidectomy, and a patient with lymphoma experienced beneficial effects following medical treatment, the patient with lymph node metastasis caused by a gastric tumor succumbed to the disease. In conclusion, the present case reports demonstrated that mediastinal syndrome is a life threatening condition whose prognosis depends on the underlying cause. PMID:27698718

  14. Mediastinal syndrome: A report of three cases

    PubMed Central

    Zardi, Enrico Maria; Pipita, Maria Elena; Afeltra, Antonella

    2016-01-01

    Mediastinal syndromes are a group of disorders involving the anatomical structures of the mediastinum. An increase in the incidence of this syndrome has been observed following the widespread use of diagnostic imaging. In the present study, three different cases of mediastinal syndrome, one of which was complicated by superior vena cava syndrome, are discussed. The treatment in the three cases was dependent on the etiology. While a patient with goiter experienced resolution of the symptoms following thyroidectomy, and a patient with lymphoma experienced beneficial effects following medical treatment, the patient with lymph node metastasis caused by a gastric tumor succumbed to the disease. In conclusion, the present case reports demonstrated that mediastinal syndrome is a life threatening condition whose prognosis depends on the underlying cause.

  15. Descending Necrotizing Mediastinitis Treated with Tooth Extractions following Mediastinal and Cervical Drainage

    PubMed Central

    Fukuchi, Minoru; Suzuki, Okihide; Nasu, Daisuke; Koizumi, Kazue; Muta, Yu; Imaizumi, Hideko; Ishiguro, Toru; Kumagai, Youichi; Ishibashi, Keiichiro; Mochiki, Erito; Ishida, Hideyuki

    2015-01-01

    Descending necrotizing mediastinitis (DNM) is a rare condition in which oropharyngeal infection spreads to the mediastinum via the cervical fascia. Delayed diagnosis and surgery result in a high mortality rate among patients with DNM. We present a case of DNM resulting from odontogenic infection treated successfully with tooth extraction following mediastinal and cervical drainage. A 43-year-old, previously healthy Japanese man was admitted to our hospital for treatment of acute mediastinitis. Computed tomography revealed gas collection around the mid-thoracic esophagus and bilateral pleural effusion. We performed mediastinal drainage via right thoracotomy. Cervicotomy was performed on postoperative day 14 to drain a residual cervical abscess. The patient required the extraction of ten teeth over three procedures to address primary odontogenic infection before his fever resolved on postoperative day 40. Prompt diagnosis, aggressive drainage and removal of the source of infection can improve survival among patients with this life-threatening disease. PMID:26600767

  16. [Descendending necrotizing mediastinitis single center experience].

    PubMed

    Kovacić, Ivan; Kovacić, Marijan

    2014-01-01

    The descending necrotizing mediastinitis is a rare but life-threatening inflammation, and occurs as a complication of deep inflammation of the neck. The mortality rate is still high by 40% despite the use of a variety of potent antimicrobial drugs. We describe 7 patients with the descending necrotizing mediastinitis treated in our hospital during the last 12 years. The primary site of infection in 5 patients were tonsils and pharynx, and in the other two patients odontogenic inflammation of the lower molars. Most of the patients belonged to the risk groups (diabetes mellitus, alcoholism), the average age of 60.4 years. After the diagnosis with computed tomography (CT), we surgically intervened in all patients. Deep neck infections are treated with aggressive surgical cervicotomy and high quality mediastinal drainage was performed with transcervical approach in all patients. Perioperative tracheotomy (n=3) was performed for the upper airway edema and postoperative tracheostomy for extended intubation (n = 1).Only in one case, we subsequently conducted a secondary surgical procedure, lateral thoracotomy because of pleural decortication. All patients were successfully cured with an average length of hospitalization was 24.6 days. For successful treatment of the descending necrotizing mediastinitis diagnosis must be set as early as possible and with the use of computed tomography scanning. Treatment requires the simultaneous application of potent antimicrobial drugs, aggressive surgical debridement of the neck and high-quality drainage of the mediastinum, which can be achieved through the transcervical approach.

  17. Primary mediastinal lymphoma: diagnosis and treatment options.

    PubMed

    Martelli, Maurizio; Di Rocco, Alice; Russo, Eleonora; Perrone, Salvatore; Foà, Robin

    2015-04-01

    Primary mediastinal large B-cell lymphoma (PMBCL) is a unique B-cell lymphoma variant that arises from a putative thymic medulla B cell. It constitutes 2-4% of non-Hodgkin lymphomas and occurs most frequently in young females. PMBCL is characterized by a diffuse proliferation of medium-to-large B cells associated with sclerosis. Molecular analysis shows that PMBCL is a distinct entity compared to other types of diffuse large B-cell lymphomas. PMBCL is characterized by a locally invasive anterior mediastinal bulky mass. The combination of rituximab with CHOP/CHOP-like regimens followed by mediastinal radiation therapy (RT) is associated with a 5-year progression-free survival of 75-85%. However, the role of consolidation RT still remains uncertain. More intensive regimens, such as DA-EPOCH-R without mediastinal RT, have shown very promising results. The conclusive role of PET-CT scan requires prospective studies and there is hope that this may allow to de-escalate RT and accordingly yield reliable prognostic information.

  18. Intralobar pulmonary sequestration and mediastinal bronchogenic cyst.

    PubMed Central

    Grewal, R. G.; Yip, C. K.

    1994-01-01

    A patient with a bronchogenic cyst and intralobar pulmonary sequestration is presented. Chest radiography showed a posterior mediastinal mass and a computed tomographic scan of the chest revealed, in addition, an abnormality suggestive of pulmonary sequestration. This was confirmed by an aortogram. It is important to be aware of the coexistence of these anomalies to make a diagnosis preoperatively. Images PMID:8016803

  19. What Causes Cystic Fibrosis?

    MedlinePlus

    ... What Causes Cystic Fibrosis? A defect in the CFTR gene causes cystic fibrosis (CF). This gene makes ... and very salty sweat. Research suggests that the CFTR protein also affects the body in other ways. ...

  20. Learning about Cystic Fibrosis

    MedlinePlus

    ... order to digest food. Cystic Fibrosis: A Single Gene Disease Mutations in a single gene - the Cystic ... the defective gene, or correcting the defective protein. Gene Therapy Research Offers Promise of a Cure for ...

  1. Living with Cystic Fibrosis

    MedlinePlus

    ... from the NHLBI on Twitter. Living With Cystic Fibrosis If you or your child has cystic fibrosis (CF), you should learn as much as you ... about CF Care Centers, go to the Cystic Fibrosis Foundation's Care Center Network Web page. It's standard ...

  2. Intestinal T-cell lymphoma associated with celiac disease masked by cavernous lymphangioma.

    PubMed

    Valentí, V; Echeveste, J I; Hernández Lizóain, J L; Martínez Regueira, F; Beunza, J J; Spiteri, N R; Olea, J; Gil, A; Cervera, M; Sola, J; Cienfuegos, J A

    2003-09-01

    We present the case of a patient admitted to our emergency ward with a clinical setting of acute abdominal pain and a history of cavernous lymphangioma, diagnosed in another center by exploratory lapartomy. The patient presented complete analysis including serology tests, as well as an abdominal CT scan that revealed multiple large size retroperitoneal cysts. In view of the clinical symptomatology and results of the tests, a second CT scan was carried out upon admission. As a result of the findings obtained, a second exploratory laparotomy was carried out in which intestinal resection of the perforated jejunal loop and largest cysts was performed. Pathological anatomy diagnosed an intestinal lymphoma associated with enteropathy and abdominal cysts compatible with cavernous lymphangioma. In this work we describe both pathologies, the most characteristic aspects are analyzed and the etiology and possible relation between both entities is discussed.

  3. Radiotherapy of unicentric mediastinal Castleman's disease

    PubMed Central

    Li, Yue-Min; Liu, Peng-Hui; Zhang, Yu-Hai; Xia, Huo-Sheng; Li, Liang-Liang; Qu, Yi-Mei; Wu, Yong; Han, Shou-Yun; Liao, Guo-Qing; Pu, Yong-Dong

    2011-01-01

    Castleman's disease is a slowly progressive and rare lymphoproliferative disorder. Here, we report a 55-year-old woman with superior mediastinal Castleman's disease being misdiagnosed for a long term. We found a 4.3 cm mass localized in the superior mediastinum accompanied with severe clinical symptoms. The patient underwent an exploratory laparotomy, but the mass failed to be totally excised. Pathologic examination revealed a mediastinal mass of Castleman's disease. After radiotherapy of 30 Gy by 15 fractions, the patient no longer presented previous symptoms. At 3 months after radiotherapy of 60 Gy by 30 fractions, Computed tomography of the chest showed significantly smaller mass, indicating partial remission. Upon a 10-month follow-up, the patient was alive and free of symptoms. PMID:21527068

  4. DESCENDING NECROTIZING MEDIASTINITIS SECONDARY TO RETROPHARYNGEAL ABSCESS.

    PubMed

    Kovacić, Marijan; Kovacić, Ivan; Dželalija, Boris

    2015-12-01

    Descending necrotizing mediastinitis secondary to a nontraumatic retropharyngeal abscess is very rare. This form of mediastinitis in the era of potent antibiotics often ends up with lethal outcome. It usually occurs in immunocompromised patients and requires intensive multidisciplinary treatment approach. We report a case of nontraumatic retropharyngeal abscess complicated by descending necrotizing mediastinitis in a 70-year-old man with insulin dependent diabetes mellitus. The patient was admitted to our hospital after clinical and radiological diagnosis of retropharyngeal abscess. During treatment for retropharyngeal abscess with antibiotic therapy and transoral incision, the patient showed mild clinical improvement but his condition suddenly aggravated on day 4 of hospital stay. He had high fever, chest pain with tachypnea, tachycardia, hypotension, and showed signs of occasional disorientation. Emergency computed tomography (CT) scan of the neck and thorax showed inflammation in the retropharyngeal space, as well as thickening of the upper posterior mediastinum fascia with the presence of air. Emergency surgery including cervicotomy and drainage of the retropharyngeal space and posterior mediastinum was performed. The patient promptly recovered with improvement of the clinical status and laboratory findings. After 16 days of treatment he was discharged from the hospital in good condition. Descending necrotizing mediastinitis can be a serious and life threatening complication of deep neck infection if the diagnosis is not quickly established. Besides inevitable application of antimicrobial drugs, good drainage of the mediastinum is necessary. We believe that transcervical approach can achieve high-quality drainage of the upper mediastinum, especially if it is done timely as in this case. Its efficacy can be verified by intensive monitoring of the patient clinical condition, by CT scan of the thorax, and by laboratory tests. In the case of inefficacy of this type

  5. DESCENDING NECROTIZING MEDIASTINITIS SECONDARY TO RETROPHARYNGEAL ABSCESS.

    PubMed

    Kovacić, Marijan; Kovacić, Ivan; Dželalija, Boris

    2015-12-01

    Descending necrotizing mediastinitis secondary to a nontraumatic retropharyngeal abscess is very rare. This form of mediastinitis in the era of potent antibiotics often ends up with lethal outcome. It usually occurs in immunocompromised patients and requires intensive multidisciplinary treatment approach. We report a case of nontraumatic retropharyngeal abscess complicated by descending necrotizing mediastinitis in a 70-year-old man with insulin dependent diabetes mellitus. The patient was admitted to our hospital after clinical and radiological diagnosis of retropharyngeal abscess. During treatment for retropharyngeal abscess with antibiotic therapy and transoral incision, the patient showed mild clinical improvement but his condition suddenly aggravated on day 4 of hospital stay. He had high fever, chest pain with tachypnea, tachycardia, hypotension, and showed signs of occasional disorientation. Emergency computed tomography (CT) scan of the neck and thorax showed inflammation in the retropharyngeal space, as well as thickening of the upper posterior mediastinum fascia with the presence of air. Emergency surgery including cervicotomy and drainage of the retropharyngeal space and posterior mediastinum was performed. The patient promptly recovered with improvement of the clinical status and laboratory findings. After 16 days of treatment he was discharged from the hospital in good condition. Descending necrotizing mediastinitis can be a serious and life threatening complication of deep neck infection if the diagnosis is not quickly established. Besides inevitable application of antimicrobial drugs, good drainage of the mediastinum is necessary. We believe that transcervical approach can achieve high-quality drainage of the upper mediastinum, especially if it is done timely as in this case. Its efficacy can be verified by intensive monitoring of the patient clinical condition, by CT scan of the thorax, and by laboratory tests. In the case of inefficacy of this type

  6. Mediastinal paraganglioma causing spinal cord compression.

    PubMed Central

    Reyes, M G; Fresco, R; Bruetman, M E

    1977-01-01

    An invasive paraganglioma of the posterior mediastinum caused spinal cord compression in a 31 year old women. Electron microscopic examination of the paraganglioma invading the epidural space revealed numerous dense-cored granules in the cytoplasm of the tumour cells. We are reporting this case to present the ultrastructure of mediastinal paraganglioma, and to call attention to an unusual cause of spinal cord compression. Images PMID:886352

  7. Sick sinus syndrome as a complication of mediastinal radiation therapy

    SciTech Connect

    Pohjola-Sintonen, S.; Toetterman, K.J.K.; Kupari, M. )

    1990-06-01

    A 33-year-old man who had received mediastinal radiation therapy for Hodgkin's disease 12 years earlier developed a symptomatic sick sinus syndrome requiring the implantation of a permanent pacemaker. The sick sinus syndrome and a finding of an occult constrictive pericarditis were considered to be due to the previous mediastinal irradiation. A ventricular pacemaker was chosen because mediastinal radiotherapy also increases the risk of developing atrioventricular conduction defects.

  8. Report: workshop on mediastinal grey zone lymphoma.

    PubMed

    Poppema, Sibrand; Kluiver, Joost L; Atayar, Cigdem; van den Berg, Anke; Rosenwald, Andreas; Hummel, Michael; Lenze, Dido; Lammert, Hetty; Stein, Harald; Joos, Stephan; Barth, Thomas; Dyer, Martin; Lichter, Peter; Klein, Uwe; Cattoretti, Giorgio; Gloghini, Annunziata; Tu, Yuhai; Stolovitzky, Gustavo A; Califano, Andrea; Carbone, Antonino; Dalla-Favera, Ricardo; Melzner, Ingo; Bucur, Alexandra J; Brüderlein, Silke; Dorsch, Karola; Hasel, Cornelia; Barth, Thomas F E; Leithäuser, Frank; Möller, Peter

    2005-07-01

    There are several indications that classical Hodgkin lymphoma (cHL) and at least a proportion of cases of Primary Mediastinal B cell Lymphoma (PMBL) are derived from B cells at similar stages of differentiation and share common pathogenic mechanisms. The first indication was the existence of mediastinal grey zone lymphomas as identified in the 4th International Symposium on HL, with clinical, histological and immunohistochemical features intermediate between cHL and PMBL. Second, both tumor types resemble a cell that is developmentally situated in-between the germinal center reaction and a plasma cell. Third, cHL and PMBL were found to have similar gene expression profiles, including the lack of immunoglobulin expression and low levels of B cell receptor signalling molecules, and the secretion of molecules like the chemokine TARC and the prominent expression of IL-13 receptors. Fourth, both entities were found to have common genomic aberrancies, notably in 2p15 and 9p24, the sites of the REL oncogene and the tyrosine kinase gene JAK2, respectively. Further comparison of both lymphoma types may provide further insight in the pathogenic mechanisms and allow the design of diagnostic algorithms to sort out the small number of so-called mediastinal grey zone lymphomas, that appear to be intermediate between PMBL and cHL. PMID:16007868

  9. Primary mediastinal B-cell lymphoma and mediastinal gray zone lymphoma: do they require a unique therapeutic approach?

    PubMed Central

    Dunleavy, Kieron

    2015-01-01

    Primary mediastinal B-cell lymphoma (PMBL) is a subtype of diffuse large B-cell lymphoma (DLBCL) that is putatively derived from a thymic B cell. Accounting for up to 10% of cases of DLBCL, this subtype predominantly affects women in the third and fourth decades of life. Its clinical and molecular characteristics are distinct from other subtypes of DLBCL and, in fact, closely resemble those of nodular sclerosing Hodgkin lymphoma (NSHL). Recently, mediastinal lymphomas with features intermediate between PMBL and NSHL, called mediastinal gray-zone lymphomas, have been described. The optimal management of PMBL is controversial, and most standard approaches include a combination of immunochemotherapy and mediastinal radiation. Recently, the recognition that mediastinal radiation is associated with significant long-term toxicities has led to the development of novel approaches for PMBL that have shown excellent efficacy and challenge the need for routine mediastinal radiation. PMID:25499450

  10. Video Assisted Thoracoscopic Surgery (VATS) for Excision of an Ectopic Anterior Mediastinal Intra-Thymic Parathyroid Adenoma

    PubMed Central

    Naik, Dukhabandhu; Jebasingh, Kumaradoss Felix; Ramprasath; Roy, Gnanamuthu Birla

    2016-01-01

    Ectopic anterior mediastinal parathyroid adenoma is a rare cause of Primary Hyperparathyroidism (PHPT). Imaging studies such as Technetium-99m (99mTc) sestamibi parathyroid scan along with a Single Photon Emission Computerized Tomogram (SPECT), and contrast enhanced Computerized Tomogram (CT) of the neck and thorax can precisely localize the ectopic mediastinal parathyroid adenoma. We report a 40-year-old gentleman who presented with persistent pain in the right shoulder following trivial trauma. His biochemical investigations showed an elevated serum calcium of 11.6mg% (Normal: 8.3-10.4 mg%) along with an elevated iPTH of 1443 pg/ml (normal: 8-70 pg/ml) which were suggestive of primary hyperparathyroidism. The localization studies revealed an ectopic cystic parathyroid adenoma in the anterior mediastinum that was not accessible from the neck. He underwent a Video Assisted Thoracoscopic (VAT) excision procedure with normalization of serum calcium and an uncomplicated recovery. The VAT approach is a successful minimally invasive technique for mediastinal parathyroidectomy. PMID:27504353

  11. Video Assisted Thoracoscopic Surgery (VATS) for Excision of an Ectopic Anterior Mediastinal Intra-Thymic Parathyroid Adenoma.

    PubMed

    Naik, Dukhabandhu; Jebasingh, Kumaradoss Felix; Ramprasath; Roy, Gnanamuthu Birla; Paul, Mazhuvanchary Jacob

    2016-06-01

    Ectopic anterior mediastinal parathyroid adenoma is a rare cause of Primary Hyperparathyroidism (PHPT). Imaging studies such as Technetium-99m ((99m)Tc) sestamibi parathyroid scan along with a Single Photon Emission Computerized Tomogram (SPECT), and contrast enhanced Computerized Tomogram (CT) of the neck and thorax can precisely localize the ectopic mediastinal parathyroid adenoma. We report a 40-year-old gentleman who presented with persistent pain in the right shoulder following trivial trauma. His biochemical investigations showed an elevated serum calcium of 11.6mg% (Normal: 8.3-10.4 mg%) along with an elevated iPTH of 1443 pg/ml (normal: 8-70 pg/ml) which were suggestive of primary hyperparathyroidism. The localization studies revealed an ectopic cystic parathyroid adenoma in the anterior mediastinum that was not accessible from the neck. He underwent a Video Assisted Thoracoscopic (VAT) excision procedure with normalization of serum calcium and an uncomplicated recovery. The VAT approach is a successful minimally invasive technique for mediastinal parathyroidectomy. PMID:27504353

  12. How Is Cystic Fibrosis Diagnosed?

    MedlinePlus

    ... from the NHLBI on Twitter. How Is Cystic Fibrosis Diagnosed? Doctors diagnose cystic fibrosis (CF) based on ... to see whether the baby has CF. Cystic Fibrosis Carrier Testing People who have one normal CFTR ...

  13. Pathological assessment of mediastinal lymph nodes in lung cancer: implications for non-invasive mediastinal staging.

    PubMed Central

    Kerr, K M; Lamb, D; Wathen, C G; Walker, W S; Douglas, N J

    1992-01-01

    BACKGROUND: The use of computed tomography in mediastinal staging of lung cancer relies on the premiss that malignant lymph nodes are larger than benign ones. This hypothesis was tested by linking node size and presence or absence of malignancy and looking at factors possibly influencing the size of benign nodes. METHODS: All accessible mediastinal lymph nodes were taken from 56 consecutive patients with lung cancer who underwent thoracotomy. Nodes were measured and histologically examined. Resected cancer bearing lung from 44 of these patients was assessed for degree of acute and chronic inflammation. RESULTS: Lymph node size was not significantly related to the presence of metastatic disease, 58% of malignant and 43% of benign lymph nodes measuring over 15 mm. Similarly, there was no statistically significant relation between size of lymph nodes and the likelihood of malignancy, 20% of lymph nodes of 10 mm or more but also 15% of those less than 10 mm being malignant. Thresholds of 15 and 20 mm showed similar results. The maximum size of benign lymph nodes was significantly greater in those patients with histological evidence of acute pulmonary inflammation than in those without. CONCLUSIONS: The study shows that in patients with lung cancer (1) malignant mediastinal lymph nodes are not larger than benign nodes; (2) small mediastinal lymph nodes are not infrequently malignant; and (3) benign adenopathy is more common in patients with acute pulmonary inflammation. Images PMID:1609375

  14. Tuberculosis-associated Fibrosing Mediastinitis: Case Report and Literature Review.

    PubMed

    Tan, Ronald; Martires, Joanne; Kamangar, Nader

    2016-01-01

    Fibrosing mediastinitis is a rare condition defined by the presence of fibrotic mediastinal infiltrates that obliterate normal fat planes. It is a late complication of a previous granulomatous infection, such as histoplasmosis or tuberculosis (TB). Due to its rarity, fibrosing mediastinitis is often under-recognized, and the clinical presentation is variable and dependent on the extent of infiltration or encasement of structures within the mediastinum. We present a case of fibrosing mediastinitis in a man with a prior history of TB, who presented with progressive dyspnea and was found to have chronic mediastinal soft tissue opacities and pulmonary hypertension. His diagnosis was delayed due to the lack of recognition of this clinical/radiographic entity. Fibrosing mediastinitis is a rare entity usually caused by granulomatous disease. Most cases develop as a late complication of histoplasmosis or TB. The presence of calcified mediastinal soft tissue infiltrates on advanced chest imaging can be diagnostic of fibrosing mediastinitis in patients with a prior history of a granulomatous infection once active processes such as malignancy are excluded. PMID:27625898

  15. Tuberculosis-associated Fibrosing Mediastinitis: Case Report and Literature Review

    PubMed Central

    Tan, Ronald; Martires, Joanne; Kamangar, Nader

    2016-01-01

    Fibrosing mediastinitis is a rare condition defined by the presence of fibrotic mediastinal infiltrates that obliterate normal fat planes. It is a late complication of a previous granulomatous infection, such as histoplasmosis or tuberculosis (TB). Due to its rarity, fibrosing mediastinitis is often under-recognized, and the clinical presentation is variable and dependent on the extent of infiltration or encasement of structures within the mediastinum. We present a case of fibrosing mediastinitis in a man with a prior history of TB, who presented with progressive dyspnea and was found to have chronic mediastinal soft tissue opacities and pulmonary hypertension. His diagnosis was delayed due to the lack of recognition of this clinical/radiographic entity. Fibrosing mediastinitis is a rare entity usually caused by granulomatous disease. Most cases develop as a late complication of histoplasmosis or TB. The presence of calcified mediastinal soft tissue infiltrates on advanced chest imaging can be diagnostic of fibrosing mediastinitis in patients with a prior history of a granulomatous infection once active processes such as malignancy are excluded. PMID:27625898

  16. Descending Mediastinitis in Epstein-Barr Virus Infection

    PubMed Central

    van Driel, E. M.; Janssen, M. J. F. M.

    2015-01-01

    Our case report describes a previously healthy 34-year-old male who develops a descending mediastinitis as a complication of an Epstein-Barr virus (EBV) infection. The mediastinitis was suspected to have developed by a breakthrough of a peritonsillar abscess through the space between the alar and prevertebral space. PMID:25740774

  17. Pediatric Mediastinal Tumors and Tumor-Like Lesions.

    PubMed

    Singh, Achint K; Sargar, Kiran; Restrepo, Carlos S

    2016-06-01

    This article reviews the imaging findings of pediatric mediastinal tumors and tumor-like lesions. The classification of the mediastinum is discussed with normal imaging appearance of the thymus in pediatric age group followed by a discussion on multiple mediastinal lesions in different compartments with emphasis on their imaging characteristics.

  18. Tuberculosis-associated Fibrosing Mediastinitis: Case Report and Literature Review

    PubMed Central

    Tan, Ronald; Martires, Joanne; Kamangar, Nader

    2016-01-01

    Fibrosing mediastinitis is a rare condition defined by the presence of fibrotic mediastinal infiltrates that obliterate normal fat planes. It is a late complication of a previous granulomatous infection, such as histoplasmosis or tuberculosis (TB). Due to its rarity, fibrosing mediastinitis is often under-recognized, and the clinical presentation is variable and dependent on the extent of infiltration or encasement of structures within the mediastinum. We present a case of fibrosing mediastinitis in a man with a prior history of TB, who presented with progressive dyspnea and was found to have chronic mediastinal soft tissue opacities and pulmonary hypertension. His diagnosis was delayed due to the lack of recognition of this clinical/radiographic entity. Fibrosing mediastinitis is a rare entity usually caused by granulomatous disease. Most cases develop as a late complication of histoplasmosis or TB. The presence of calcified mediastinal soft tissue infiltrates on advanced chest imaging can be diagnostic of fibrosing mediastinitis in patients with a prior history of a granulomatous infection once active processes such as malignancy are excluded.

  19. [X-ray computed tomography in the diagnosis of mediastinitis].

    PubMed

    Abakumov, M M; Barmina, T G; Ishmukhametov, A I

    2001-01-01

    Results of X-ray computed tomography (RCT) use in 21 patients treated for acute mediastinitis in N.V. Sklifosovsky Emergency Care Research Institute have been analyzed retrospectively. RCT proved able to defect accurate location, spreading and sizes of inflammatory disorders in the mediastinum are demonstrated. Semiotics of mediastinitis depending on location, spreading and phase of inflammatory process is presented.

  20. Mediastinal paragangliomas related to SDHx gene mutations

    PubMed Central

    Ćwikła, Jarosław; Prejbisz, Aleksander; Kwiatek, Paweł; Szperl, Małgorzata; Michalski, Wojciech; Wyrwicz, Lucjan; Kuśmierczyk, Mariusz; Januszewicz, Andrzej; Maciejczyk, Anna; Roszczynko, Marta; Pęczkowska, Mariola

    2016-01-01

    Introduction Paragangliomas (PGLs) related to hereditary syndromes are rare mediastinal tumors. Paragangliomas are caused by mutations in genes encoding subunits of succinate dehydrogenase enzyme (SDH). Aim To evaluate clinical, anatomical and functional characteristics of mediastinal paragangliomas related to SDHx gene mutations. Material and methods Retrospective analysis of 75 patients with confirmed SDHx gene mutations (24 patients with SDHB, 5 SDHC, 46 with SDHD mutations) was performed. Patients underwent evaluation using computed tomography (CT), somatostatin receptor scintigraphy (SRS) (99mTc-[HYNIC,Tyr3]-octreotide), 123I mIBG scintigraphy and urinary excretion of total methoxycatecholamines. Results Out of 75 patients, 16 (21%) patients (1 SDHB, 15 SDHD mutations) had 17 PGLs localized in the mediastinum. Fourteen PGLs were localized in the middle mediastinum (intrapericardial) and 3 PGLs in the posterior mediastinum. The median diameter of paragangliomas measured on the axial slice was 24.3 mm (interquartile range (IQR): 14.7–36.6), and the median volume was 2.78 ml (IQR: 0.87–16.16). Twelve out of 16 patients (75%) underwent SRS, and 11 of them (92.3%) had pathological uptake of the radiotracer. Eleven (68.75%) out of 16 patients underwent 123 I mIBG, with only 3 positive results. Symptoms of catecholamine excretion were observed in 3 patients with PGLs localized in the posterior mediastinum. All PGLs were benign except in 1 patient with the SDHB mutation and PGL detected in the posterior mediastinum, who had a metastatic disease. Conclusions Most mediastinal paragangliomas were related to SDHD gene mutations. They were asymptomatic, localized in the medial mediastinum, intrapericardially. PMID:27785149

  1. [A case of mediastinal cavernous hemangioma].

    PubMed

    Maebeya, S; Nishimura, O; Yokoi, H; Shimizu, T; Yoshimasu, T; Naito, Y

    1990-03-01

    A 6-year-old boy had an abnormal shadow on the chest X-ray film. It showed a tumor shadow with calcification on the right hilum. The plain CT scan showed an anterior mediastinal mass and its density was similar to that of large vessels. On the angio CT scan the lesion displayed a much lower enhancement than large vessels. The tumor was resected completely by median sternotomy. It was 5.6 X 3.6 X 3.0 cm in size and contained a phlebolith 5 mm in diameter. The histological diagnosis was cavernous hemangioma. PMID:2348129

  2. Mediastinal angiomatosis: a rare case report

    PubMed Central

    Pak, Min Gyoung; Choi, Phil Jo; Choi, Won Suk; Kang, Eun Ju

    2015-01-01

    Angiomatosis refers to a rare condition of large hamartomatous vascular lesions that extensively affect a region of the body or several different tissue types in a contiguous way. Several cases have been reported in the mediastinum. We experienced a histologically proven case of mediastinal angiomatosis in a 56-year-old woman that was incidentally detected as multiple conglomerated masses mimicking metastatic lymph nodes on chest radiography. Despite its rareness, our case emphasizes that pathologists and radiologists need to be aware of the rare diagnosis of angiomatosis in the mediastinum. PMID:26793386

  3. Mediastinal bronchogenic cyst mimicking congenital lobar emphysema.

    PubMed

    Arun, Sumitha; Kumar, Manish; Ross, Benjamin Jeyanth

    2016-01-01

    Bronchogenic cyst (BC) is a rare congenital malformation of the lung. Most patients remain asymptomatic until adulthood while some are symptomatic in the first few years of life. However, symptoms in newborn period are rare. We report a case of a 3-day-old preterm baby with respiratory distress diagnosed as congenital lobar emphysema on chest X-ray. A CT scan revealed a mediastinal cyst causing obstructive lobar emphysema. The cyst was excised and pathological examination was suggestive of BC. PMID:27609589

  4. [Giant cystic lymphangioma breast. Report of a case with 20-year follow-up and review of the literature].

    PubMed

    Vargas-Hernández, Víctor Manuel; Tovar-Rodríguez, José María; Moreno-Eutimio, Mario Adán; Acosta-Altamirano, Gustavo

    2014-01-01

    Antecedentes: los linfangiomas son malformaciones congénitas o adquiridas (secundarias a traumatismos, infecciones o neoplasias) de la glándula mamaria sumamente raras. Estas lesiones tienden a infiltrar los tejidos circundantes y la degeneración maligna es excepcional. Su manifestación clínica es en tumores benignos y de crecimiento lento. Se diagnostica clínicamente y por estudios de imagen. El tumor se extirpa por razones estéticas y para establecer el diagnóstico diferencial con otras lesiones más comunes. Caso clínico: paciente femenina de 45 años de edad, con incremento progresivo del tamaño de la mama izquierda, quizá originado por un traumatismo y no tiene otros sintomas. El diagnóstico histológico definitivo fue: linfangioma quístico gigante de la glándula mamaria izquierda. Discusión: los linfangiomas son lesiones extremadamente raras en la glándula mamaria, de comportamiento agresivo local y benignas. El tejido linfático anormal posee cierta capacidad de proliferar y de acumular grandes cantidades de líquido, lo que representa su apariencia quística, como sucedió en el caso aquí reportado. La extirpación quirúrgica local es el tratamiento indicado. Conclusión: este primer caso de linfangioma quístico gigante de mama reportado en México permite corroborar la benignidad de la lesión.

  5. Posterior mediastinal mass diagnosed as schwanomma with concomittant tuberculosis

    PubMed Central

    Tripathi, Surya Kant; Mishra, Ashwini Kumar; Verma, Ajay Kumar; Prakash, Ved

    2014-01-01

    A 21-year-old non-smoker, non-hypertensive male without diabetes was referred to our pulmonary medicine department with suspected malignant intrathoracic mass. The clinicoradiological evaluation revealed that it could be a posterior mediastinal mass. The same diagnosis was confirmed on performing CT and MRI. Benign posterior mediastinal schwanomma was suspected as it is the most common posterior mediastinal mass. It was completely resected. Histopathological examination confirmed the same. The mass was also sent for culture for mycobacterium which came out to be positive. The patient recovered from the surgery and postoperative X-ray showed complete clearance. He was treated with antitubercular treatment and responded very well. PMID:25246469

  6. Human mediastinal adipose tissue displays certain characteristics of brown fat

    PubMed Central

    Cheung, L; Gertow, J; Werngren, O; Folkersen, L; Petrovic, N; Nedergaard, J; Franco-Cereceda, A; Eriksson, P; Fisher, R M

    2013-01-01

    Background: The amount of intra-thoracic fat, of which mediastinal adipose tissue comprises the major depot, is related to various cardiometabolic risk factors. Autopsy and imaging studies indicate that the mediastinal depot in adult humans could contain brown adipose tissue (BAT). To gain a better understanding of this intra-thoracic fat depot, we examined possible BAT characteristics of human mediastinal in comparison with subcutaneous adipose tissue. Materials and methods: Adipose tissue biopsies from thoracic subcutaneous and mediastinal depots were obtained during open-heart surgery from 33 subjects (26 male, 63.7±13.8 years, body mass index 29.3±5.1 kg m−2). Microarray analysis was performed on 10 patients and genes of interest confirmed by quantitative PCR (qPCR) in samples from another group of 23 patients. Adipocyte size was determined and uncoupling protein 1 (UCP1) protein expression investigated with immunohistochemistry. Results: The microarray data showed that a number of BAT-specific genes had significantly higher expression in the mediastinal depot than in the subcutaneous depot. Higher expression of UCP1 (24-fold, P<0.001) and PPARGC1A (1.7-fold, P=0.0047), and lower expression of SHOX2 (0.12-fold, P<0.001) and HOXC8 (0.14-fold, P<0.001) in the mediastinal depot was confirmed by qPCR. Gene set enrichment analysis identified two gene sets related to mitochondria, which were significantly more highly expressed in the mediastinal than in the subcutaneous depot (P<0.01). No significant changes in UCP1 gene expression were observed in the subcutaneous or mediastinal depots following lowering of body temperature during surgery. UCP1 messenger RNA levels in the mediastinal depot were lower than those in murine BAT and white adipose tissue. In some mediastinal adipose tissue biopsies, a small number of multilocular adipocytes that stained positively for UCP1 were observed. Adipocytes were significantly smaller in the mediastinal than the

  7. Management of large mediastinal masses: surgical and anesthesiological considerations

    PubMed Central

    van Boven, Wim Jan P.; Annema, Jouke T.; Eberl, Susanne; Klomp, Houke M.; de Mol, Bas A.J.M.

    2016-01-01

    Large mediastinal masses are rare, and encompass a wide variety of diseases. Regardless of the diagnosis, all large mediastinal masses may cause compression or invasion of vital structures, resulting in respiratory insufficiency or hemodynamic decompensation. Detailed preoperative preparation is a prerequisite for favorable surgical outcomes and should include preoperative multimodality imaging, with emphasis on vascular anatomy and invasive characteristics of the tumor. A multidisciplinary team should decide whether neoadjuvant therapy can be beneficial. Furthermore, the anesthesiologist has to evaluate the risk of intraoperative mediastinal mass syndrome (MMS). With adequate preoperative team planning, a safe anesthesiological and surgical strategy can be accomplished. PMID:27076967

  8. Asymptomatic peritoneal carcinomatosis originating from benign cystic peritoneal mesothelioma

    PubMed Central

    Iacoponi, S; Calleja, J; Hernandez, G; de la Cuesta, R Sainz

    2015-01-01

    Benign multicystic mesothelioma is a rare tumour that originates from the abdominal peritoneum with a predisposition to the pelvic peritoneum. It typically affects women of reproductive age. There have been less than 200 cases of this rare neoplasia reported to date. We present the case of a 35-year-old woman who was referred to our centre because of the detection of a peritoneal carcinomatosis during a gynaecological exam. A diagnostic laparoscopy was performed. The findings included multiple cysts appearing as ‘a bunch of grapes’ occupying the omentum. Biopsies were taken during the surgery and the results showed benign multicystic peritoneal mesothelioma. Benign multicystic mesothelioma can simulate other conditions, such as malignant ovarian tumours or cystic lymphangioma. It is often diagnosed accidentally during surgery performed for another reason. The diagnosis is interoperative, observing multicystic structures grouped as a ‘bunch of grapes’ containing clear fluid with thin walls made of connective tissue. Immunohistochemistry confirmed mesothelial origin. Surgery is considered the treatment of choice and is based on the removal of the cysts from the abdominal cavity. Hyperthermic intraperitoneal chemotherapy can be considered as a primary treatment in patients with recurrences or even as a part of primary treatment associated with surgery. Survival at 5 years is 100% and invasive or malignant progression is extraordinary. The treatment approach should be multidisciplinary, and the patient should be referred to a referral centre. PMID:26715942

  9. [Primary mediastinal B-cell lymphoma].

    PubMed

    Coso, D; Rey, J; Bouabdallah, R

    2010-02-01

    Primary mediastinal B-cell lymphoma (PMBL) is a clinicopathological entity among the world health organization classification of lymphoid neoplasms. PMBL often concerns young adults, and the disease remains a localized disease in the majority of cases. The outcome of patients with PMBL is variable and unlike diffuse large cell lymphomas, the international prognostic index seems to be less applicable to such disease. The combination of rituximab and chemotherapy is the gold standard treatment of patients with good prognosis features and allows high cure rates. However, high-dose chemotherapy supported by peripheral blood stem cell support is often warranted in poor-prognosis patients. The use of positrons emission tomography examination is more and more used in such situations to select the best therapeutic strategy. PMID:20207294

  10. Glioblastoma arising within a mediastinal mature teratoma.

    PubMed

    Liu, Liping; Jentoft, Mark E; Boland, Jennifer M

    2016-10-01

    Herein we present the case of a 42-year-old man who presented with an anterior mediastinal mass, which was found to represent a mature teratoma. Within it, there was a secondary somatic malignant glial neoplasm with mitotic activity and necrosis, compatible with glioblastoma. He experienced early local recurrence and lymph node metastasis, but is alive and well 3 1/2 years after diagnosis. Neither the teratoma nor the glioblastoma components had abnormalities of chromosome 12, which may implicate that this teratoma was more closely related to those arising along the midline of infants and children (type I germ cell tumor) than to the typically malignant testicular examples, which often contain mixed germ cell elements (type II germ cell tumor). PMID:27327191

  11. Cystic Adenomatoid Odontogenic Tumor

    PubMed Central

    Grover, Sonal; Rahim, Ahmed Mujib Bangalore; Parakkat, Nithin Kavassery; Kapoor, Shekhar; Mittal, Kumud; Sharma, Bhushan; Shivappa, Anil Bangalore

    2015-01-01

    Adenomatoid Odontogenic Tumor (AOT) is a well-established benign epithelial lesion of odontogenic origin. Rightfully called “the master of disguise,” this lesion has been known for its varied clinical and histoarchitectural patterns. Not only does AOT predominantly present radiologically as a unilocular cystic lesion enclosing the unerupted tooth (which is commonly mistaken as a dentigerous cyst) but the lesion also presents rarely with a cystic component histopathologically. We present one such unusual case of cystic AOT associated with an impacted canine, mimicking a dentigerous cyst. The present case aims to highlight the difference between cystic AOT and dentigerous cyst radiographically. The exact histogenesis of AOT and its variants still remains obscure. An attempt has been made to hypothesize the new school of thought regarding the origin of AOT. PMID:26579317

  12. Diabetes in cystic fibrosis.

    PubMed

    Bridges, Nicola

    2013-05-01

    Cystic fibrosis related diabetes (CFRD) is a common complication of cystic fibrosis, caused by a fall in insulin secretion with age in individuals with pancreatic insufficiency. CFRD is associated with worse clinical status and increased mortality. Treatment of CFRD with insulin results in sustained improvements in lung function and nutrition. While clinical experience with insulin treatment in CF has increased, the selection of who to treat and glycaemic targets remain unclear.

  13. Troubleshooting thoracoscopic anterior mediastinal surgery: lessons learned from thoracoscopic lobectomy

    PubMed Central

    Hirji, Sameer A.; Balderson, Scott S.; Berry, Mark F.

    2015-01-01

    Video-assisted thoracoscopic surgery (VATS) lobectomy is safe, oncologically effective, and increasingly utilized for lung cancer resection. Lessons from VATS lobectomy experience can guide the use of a VATS approach to resect mediastinal masses. Exposure and dissection when using VATS to resect anterior mediastinal masses has unique challenges. Several maneuvers acquired from experience with VATS lobectomy can reduce the technical difficulty and often prevent conversion to an open approach. In this troubleshooting guide, we offer ‘tips’ to both avoid and manage numerous intra-operative technical difficulties that commonly arise during VATS anterior mediastinal procedures. Avoiding an open approach may improve outcomes, although conversion for safety or complete resection can be necessary. Techniques and experiences derived from VATS lobectomy can facilitate VATS resection of mediastinal masses. PMID:26693151

  14. A patient with combined mediastinal, mesenteric and retroperitoneal fibrosis.

    PubMed

    Graal, M B; Lustermans, F A

    1994-06-01

    We report a patient with combined mediastinal, mesenteric and retroperitoneal fibrosis who first presented with signs of a superior vena cava syndrome. She was successfully treated with corticosteroids. The aetiology, clinical picture, and possible therapy of idiopathic fibrosclerosis are discussed.

  15. Pancreatic Cystic Neoplasms

    PubMed Central

    Limaiem, Faten; Khalfallah, Tahar; Farhat, Leila Ben; Bouraoui, Saâdia; Lahmar, Ahlem; Mzabi, Sabeh

    2014-01-01

    Background: Cystic neoplasms of the pancreas are rare and constitute approximately 0.5% of all pancreatic neoplasms. Aims: The study was to describe clinicopathological features of pancreatic cystic tumors. Patients and Methods: In our retrospective study, we reviewed 10 cases of pancreatic cystic neoplasms that were diagnosed at the pathology department of Mongi Slim hospital over a 14-year period (2000-2013). We adopted the latest World Health Organization (WHO) classification (2010) in grouping all tumors. Results: There were one male and nine female patients (sex ratio M/F = 1:9) aged between 21 and 68 years (mean = 37.5 years). The most common clinical presentation was epigastric and abdominal pain (n = 6) followed by vomiting (n = 3). Abdominal computed tomography (CT) scan disclosed a cystic lesion of the pancreas ranging in size between 2 and 10 cm (mean = 6.75 cm). All patients underwent surgical treatment. Histopathological examination of the surgical specimen established the diagnosis of solid pseudopapillary neoplasm (n = 2), serous cystic neoplasm (n = 2), mucinous cystadenoma (n = 4), mucinous cystadenocarcinoma (n = 1), and intraductal papillary mucinous neoplasm with invasive carcinoma (n = 1). Conclusion: Better understanding of pancreatic cystic neoplasms is essential for clinicians to make accurate diagnosis and to provide the best management for patients. PMID:25210676

  16. Cystic Fibrosis (CF) Respiratory Screen: Sputum

    MedlinePlus

    ... Cystic Fibrosis (CF) Chloride Sweat Test Lungs and Respiratory System Cystic Fibrosis: Diet and Nutrition Cystic Fibrosis Cystic Fibrosis: Diet and Nutrition Lungs and Respiratory System Contact Us Print Resources Send to a friend ...

  17. Correlative Imaging in a Patient with Cystic Thymoma: CT, MR and PET/CT Comparison

    PubMed Central

    Romeo, Valeria; Esposito, Alfredo; Maurea, Simone; Camera, Luigi; Mainenti, Pier Paolo; Palmieri, Giovannella; Buonerba, Carlo; Salvatore, Marco

    2015-01-01

    Summary Background Cystic thymoma is a rare variant of thymic neoplasm characterized by almost complete cystic degeneration with mixed internal structure. We describe a case of a 60 year-old woman with a cystic thymoma studied with advanced tomographic imaging stydies. CT, MRI and PET/CT with 18F-FDG were performed; volumetric CT and MRI images provided better anatomic evaluation for pre-operative assessment, while PET/CT was helpful for lesion characterization based on 18F-FDG uptake. Although imaging studies are mandatory for pre-operative evaluation of cystic thymoma, final diagnosis still remains surgical. Case Report A 60-year-old woman with recent chest pain and no history of previous disease was admitted to our departement to investigate the result of a previous chest X-ray that showed bilateral mediastinal enlargement; for this purpose, enhanced chest CT scan was performed using a 64-rows scanner (Toshiba, Aquilion 64, Japan) before and after intravenous bolus administration of iodinated non ionic contrast agent; CT images demonstrated the presence of a large mediastinal mass (11×8 cm) located in the anterior mediastinum who extended from the anonymous vein to the cardio-phrenic space, compressing the left atrium and causing medium lobe atelectasis; bilateral pleural effusion was also present. Conclusions In conclusion, correlative imaging plays a foundamental role for the diagnostic evaluation of patient with cystic thymoma. In particular, volumetric CT and MRI studies can provide better anatomic informations regarding internal structure and local tumor spread for pre-operative assessment. Conversely, metabolic imaging using 18F-FDG PET/CT is helpful for lesion characterization differentiating benign from malignant lesion on the basis of intense tracer uptake. The role of PET/MRI is still under investigation. However, final diagnosis still remains surgical even though imaging studies are mandatory for pre-operative patient management. PMID:25593635

  18. Fluorine-18 Fluorodeoxyglucose Positron Emission Tomography/Computed Tomography Findings of Post Traumatic Lymphangioma in a Young Adult Male

    PubMed Central

    Kwon, Sang Don; Chun, Kyung Ah; Kong, Eun Jung; Cho, Ihn Ho

    2016-01-01

    The authors report the case of a 34-year-old male, who underwent a fluorine-18 fluoro deoxyglucose (18F-FDG) positron emission tomography/computed tomography (PET/CT) scan 7 years after trauma for the evaluation of multifocal masses in the right iliac and right inguinal areas. CT findings showed multifocal low density masses and 18F-FDG PET revealed slightly increased uptake (maximum standardized uptake value [SUVmax] 3.1). These findings did not exclude the possibility of a benign or malignant lesion. To achieve differential diagnosis, partial surgical excision was performed and a pathologic examination subsequently revealed lymphangioma. Here, the authors describe the 18F-FDG PET/CT findings of a rare case of lymphangioma resulting from trauma. PMID:27699163

  19. Fluorine-18 Fluorodeoxyglucose Positron Emission Tomography/Computed Tomography Findings of Post Traumatic Lymphangioma in a Young Adult Male

    PubMed Central

    Kwon, Sang Don; Chun, Kyung Ah; Kong, Eun Jung; Cho, Ihn Ho

    2016-01-01

    The authors report the case of a 34-year-old male, who underwent a fluorine-18 fluoro deoxyglucose (18F-FDG) positron emission tomography/computed tomography (PET/CT) scan 7 years after trauma for the evaluation of multifocal masses in the right iliac and right inguinal areas. CT findings showed multifocal low density masses and 18F-FDG PET revealed slightly increased uptake (maximum standardized uptake value [SUVmax] 3.1). These findings did not exclude the possibility of a benign or malignant lesion. To achieve differential diagnosis, partial surgical excision was performed and a pathologic examination subsequently revealed lymphangioma. Here, the authors describe the 18F-FDG PET/CT findings of a rare case of lymphangioma resulting from trauma.

  20. Immature mediastinal teratoma with unusual histopathology

    PubMed Central

    Mustafa, Osama M.; Mohammed, Shamayel F.; Aljubran, Ali; Saleh, Waleed N.

    2016-01-01

    Abstract Germ cell tumors (GCTs) represent a well-recognized group of heterogeneous neoplasms with diverse clinical, histopathological, diagnostic, and prognostic characteristics. We present a rare case of a locally aggressive, chemotherapy-resistant immature mediastinal teratoma with a peculiar histological finding of a multilineage somatic-type malignant degeneration. A 21-year-old male patient presented with a 3-week history of persistent, blood-tinged productive cough and shortness of breath. A contrast-enhanced computed tomography (CT) scan of the chest showed a heterogeneous mass occupying the right hemithorax and abutting on adjacent structures. CT-guided biopsy was consistent with immature teratoma. Combination chemotherapy with bleomycin, etoposide, and cisplatin was initiated, albeit without success; the mass showed interval progression in size, and surgical resection through clamshell incision was performed. Histological assessment of the resected mass confirmed the diagnosis of immature teratoma and revealed an extensive multilineage malignant differentiation into sarcomatous, carcinomatous, and melanomatous components. The patient underwent an uneventful recovery but presented 2 months later with extensive liver and bone melanomatous metastases. In this report, relevant findings from the literature are also highlighted. Despite being exceptionally rare, such tumors carry poor prognosis. Understanding the clinicopathological characteristics and biological behavior of such tumors may provide an insight into interventions tailored to improve the otherwise dismal disease outlook. PMID:27367976

  1. Primary Mediastinal B-Cell Lymphoma

    PubMed Central

    Pileri, Stefano A.; Gaidano, Gianluca; Zinzani, Pier Luigi; Falini, Brunangelo; Gaulard, Philippe; Zucca, Emanuele; Pieri, Federica; Berra, Eva; Sabattini, Elena; Ascani, Stefano; Piccioli, Milena; Johnson, Peter W. M.; Giardini, Roberto; Pescarmona, Edoardo; Novero, Domenico; Piccaluga, Pier Paolo; Marafioti, Teresa; Alonso, Miguel A.; Cavalli, Franco

    2003-01-01

    Although primary mediastinal (thymic) large B-cell lymphoma has been primarily studied, its precise phenotype, molecular characteristics, and histogenesis are still a matter of debate. The International Extranodal Lymphoma Study Group collected 137 such cases for extensive pathological review. Histologically, the lymphomatous growth was predominantly diffuse with fibrosis that induced compartmentalized cell aggregation. It consisted of large cells with varying degrees of nuclear polymorphism and clear to basophilic cytoplasm. On immunohistochemistry, the following phenotype was observed: CD45+, CD20+, CD79a+, PAX5/BSAP+, BOB.1+, Oct-2+, PU.1+, Bcl-2+, CD30+, HLA-DR+, MAL protein+/−, Bcl-6+/−, MUM1/IRF4+/−, CD10−/+, CD21−, CD15−, CD138−, CD68−, and CD3−. Immunoglobulins were negative both at immunohistochemistry and in situ hybridization. Molecular analysis, performed in 45 cases, showed novel findings. More than half of the cases displayed BCL-6 gene mutations, which usually occurred along with functioning somatic IgVH gene mutations and Bcl-6 and/or MUM1/IRF4 expression. The present study supports the concept that a sizable fraction of cases of this lymphoma are from activated germinal center or postgerminal center cells. However, it differs from other aggressive B-cell lymphomas in that it shows defective immunoglobulin production despite the expression of OCT-2, BOB.1, and PU.1 transcription factors and the lack of IgVH gene crippling mutations. PMID:12507907

  2. Effective treatment for malignant mediastinal teratoma.

    PubMed

    Parker, D; Holford, C P; Begent, R H; Newlands, E S; Rustin, G J; Makey, A R; Bagshawe, K D

    1983-12-01

    Primary malignant mediastinal teratoma is a rare tumour previously regarded as inevitably fatal. In a series of eight male patients with a mean age of 24 years five remain alive and well. All patients showed raised serum concentrations of human chorionic gonadotrophin or alpha fetoprotein. The patients were treated with intermittent combination chemotherapy that included cisplatin. Six patients responded to chemotherapy with a fall in human chorionic gonadotrophin or alpha fetoprotein to near normal levels and they then had radical excision of the remaining tumour. Living malignant tumour was found in four of the specimens and these patients received postoperative chemotherapy. One patient died after eight months and the remaining five patients are alive and well 13-136 months after the start of treatment. The two patients who did not undergo surgery died at one month and 15 months. Intermittent combination chemotherapy and carefully timed radical excision of these tumours would appear to have produced better results than have been reported in other series.

  3. Effective treatment for malignant mediastinal teratoma.

    PubMed Central

    Parker, D; Holford, C P; Begent, R H; Newlands, E S; Rustin, G J; Makey, A R; Bagshawe, K D

    1983-01-01

    Primary malignant mediastinal teratoma is a rare tumour previously regarded as inevitably fatal. In a series of eight male patients with a mean age of 24 years five remain alive and well. All patients showed raised serum concentrations of human chorionic gonadotrophin or alpha fetoprotein. The patients were treated with intermittent combination chemotherapy that included cisplatin. Six patients responded to chemotherapy with a fall in human chorionic gonadotrophin or alpha fetoprotein to near normal levels and they then had radical excision of the remaining tumour. Living malignant tumour was found in four of the specimens and these patients received postoperative chemotherapy. One patient died after eight months and the remaining five patients are alive and well 13-136 months after the start of treatment. The two patients who did not undergo surgery died at one month and 15 months. Intermittent combination chemotherapy and carefully timed radical excision of these tumours would appear to have produced better results than have been reported in other series. Images PMID:6198739

  4. [Atypical intraperitoneal cystic masses].

    PubMed

    Domínguez-Pérez, S T; Baeza-Herrera, C; Villalobos-Castillejos, A; González-Mateos, T; Aguilar-Venegas, M

    2010-01-01

    Omental, mesenteric and retroperitoneal cystic masses are very rare in pediatric population. They usually present as asymptomatic abdominal tumors and only occasionally a preoperative diagnosis is made. We presented 4 cases presented to our hospital during a 2-year period. All patients were male with an age ranged from 5 months to 7 years. Three patients had previous diagnosis of cystic mass by abdominal ultrasound. Three patients presented with acute abdomen and one patient with bowel obstruction. All patients underwent successful resection of the mass with no perioperative mortality.

  5. Isolated mediastinal necrotizing granulomatous lymphadenopathy due to cat-scratch disease.

    PubMed

    Lovis, A; Clerc, O; Lazor, R; Jaton, K; Greub, G

    2014-02-01

    We report a patient suffering from cat-scratch disease limited to mediastinal lymphadenitis. Although rare, cat-scratch disease should be considered in the differential diagnosis of mediastinal lymphadenitis, especially when patients were exposed to cats.

  6. Disseminated necrotic mediastinitis spread from odontogenic abscess: our experience

    PubMed Central

    Filiaci, Fabio; Riccardi, Emiliano; Mitro, Valeria; Piombino, Pasquale; Rinna, Claudio; Agrillo, Alessandro; Ungari, Claudio

    2015-01-01

    Summary Aims Deep neck infections are rare but potentially fatal complication of pulpal abscess of the teeth. If an infection can progress rapidly from a toothache to a life threatening infection, then it is critical that dentists be able to recognize the danger signs and identify the patients who are at risk. Mediastinitis is a severe inflammatory process involving the connective tissues that fills the intracellular spaces and surrounds the organs in the middle of the chest. This pathology has both an acute and a chronic form and, in most cases, it has an infectious etiology. This study want to expose the experience acquired in the Oral and Maxillo-facial Sciences Department, Policlinico Umberto I, “Sapienza” University of Rome, regarding two clinical cases of disseminated necrotizing mediastinitis starting from an odontogenic abscess. Methods We report two clinical cases of disseminated necrotic mediastinitis with two different medical and surgical approaches. The radiographic and photographic documentation of the patients was collected in the pre-and post-operatively. All patients underwent a CT scan and MRI. Results Mediastinitis can result from a serious odontogenic abscess, and the extent of its inflammation process must be never underestimated. Dental surgeons play a key role as a correct diagnosis can prevent further increasing of the inflammation process. Conclusions A late diagnosis and an inadequate draining represent the major causes of the elevated mortality rate of disseminated necrotizing mediastinitis. PMID:26330907

  7. Evaluation of magnetic resonance sequences in imaging mediastinal tumors

    SciTech Connect

    Webb, W.R.; Gamsu, G.; Stark, D.D.; Moon, K.L. Jr.; Moore, E.H.

    1984-10-01

    Ten patients having a mediastinal tumor were studied with magnetic resonance imaging (MRI) using from two to four imaging sequences. Seven had bronchial carcinoma and three had benign lesions. The sequences included the spin-echo technique with repetition time (TR) values of 0.5, 1.0, and 2.0 sec and echo time (TE) values of 28 and 56 msec, and the inversion-recovery technique. The signal-intensity ratios of the mediastinal mass and mediastinal fat, which are a measure of image contrast, were compared for the different imaging sequences. Also signal-to-noise ratios were measured relative to both mediastinal fat and mediastinal mass. With spin-echo imaging, decreasing the TR value resulted in an increase in mass/fat contrast in all patients, making the masses easier to detect, but this also resulted in decreased signal-to-noise ratios. Inversion-recovery imaging with the sequence used resulted in a greatly increased mass/fat contrast, because of a relative decrease in signal from the mass. Spin-echo imaging with both short and long TR values provides good tissue contrast and good signal-to-noise ratios.

  8. Concurrent cranial mediastinal Blastomyces granuloma and carcinoma with cranial vena caval syndrome in a dog

    PubMed Central

    Evans, Natashia A.; Viviano, Katrina R.

    2015-01-01

    This report describes an unusual progression of blastomycosis in a dog with concurrent mediastinal carcinoma. The dog was evaluated for respiratory distress. Diagnostic results revealed chylothorax and a cranial vena caval thrombus. Histopathology of the cranial mediastinal mass diagnosed mediastinal carcinoma and fungal granuloma. Intercurrent disease may complicate the clinical presentation and clinical course of blastomycosis. PMID:26538674

  9. [US-guided transhepatic drainage of a mediastinal abscess].

    PubMed

    Simón-Yarza, I; Viteri-Ramírez, G; García-Lallana, A; Benito, A

    2014-01-01

    Anastomoses often leak after esophageal surgery; if they are not detected in time, leaks can give rise to complications like fluid collections, superinfections, and mediastinitis. Although these complications usually require surgical treatment, different series of patients successfully treated with conservative or minimally invasive approaches have been reported. We present the case of a patient who developed a mediastinal abscess after epiphrenic diverticulectomy. We treated the abscess with US-guided percutaneous transhepatic drainage to avoid surgical reintervention. Although other cases of a transhepatic approach to thoracic lesions have been reported, to our knowledge this is the first report of this technique in mediastinal abscesses. We recommend that transhepatic drainage be considered a therapeutic option in the management of these complications. PMID:22464798

  10. Aspergillus Mediastinitis after Orthotopic Heart Transplantation: A Case Report.

    PubMed

    El-Sayed Ahmed, Magdy M; Almanfi, Abdelkader; Aftab, Muhammad; Singh, Steve K; Mallidi, Hari R; Frazier, O H

    2015-10-01

    A 55-year-old woman was admitted for orthotopic heart transplantation. Her medical history was notable for multiple cardiovascular problems, including ischemic cardiomyopathy that necessitated circulatory support with a left ventricular assist device. Five weeks after undergoing orthotopic heart transplantation, she developed Aspergillus calidoustus mediastinitis, for which she underwent a prolonged course of antifungal treatment that comprised (in sequence) posaconazole for 11 days, voriconazole for 10 days, and amphotericin B for 42 days. During this period, she also underwent repeated mediastinal drainage and sternal débridement, followed by sternal wiring and coverage with bilateral pectoralis advancement flaps. Four months postoperatively, she was discharged from the hospital with a successfully controlled infection and a healed sternum. To our knowledge, only 3 previous cases of Aspergillus mediastinitis after orthotopic heart transplantation have been reported in the literature, none of which was Aspergillus calidoustus.

  11. Craniocervical necrotizing fasciitis of odontogenic origin with mediastinal extension.

    PubMed

    Edwards, John D; Sadeghi, Nader; Najam, Farzad; Margolis, Mark

    2004-08-01

    We review an interesting case of craniocervical necrotizing fasciitis with thoracic extension in an immunocompetent 44-year-old man. The patient underwent aggressive medical and surgical management during a long hospitalization. Multiple surgical debridements, including transcervical mediastinal debridement, and eventually a thoracotomy for mediastinal abscess were required. The patient eventually recovered, and 3 months later he showed no sign of complications or recurrence. Craniocervical necrotizing fasciitis is a fulminant soft-tissue infection, usually of odontogenic origin, that requires prompt identification and treatment to ensure survival. Broad-spectrum intravenous antibiotics, aggressive surgical debridement and wound care, hyperbaric oxygen, and good intensive care are the mainstays of treatment.

  12. Idiopathic azygos vein aneurysm: a rare cause of mediastinal mass

    PubMed Central

    Gallego, M.; Mirapeix, R.; Castaner, E.; Domingo, C.; Mata, J.; Marin, A.

    1999-01-01

    Venous aneurysm of the azygos arch is a very rare cause of mediastinal mass and is usually an incidental finding on chest radiography. Nowadays the diagnosis is made by non-invasive tests such as thoracic CT scanning and/or magnetic resonance imaging. The case is described of an asymptomatic woman in whom a mediastinal mass due to an azygos vein aneurysm was diagnosed by non-invasive procedures, the aetiology of which, in all probability, was idiopathic.

 PMID:10377213

  13. Video-assisted thoracoscopic surgery for complex mediastinal mass resections

    PubMed Central

    DeMarchi, Lorenzo; Emerson, Dominic A.; Holzner, Matthew L.

    2015-01-01

    Minimally invasive surgery has changed the way operative procedures are performed in many specialties. As surgeons have become progressively facile with these techniques, the opportunities to use them have expanded. In thoracic surgery, many surgeons now use minimally invasive techniques to resect small, uncomplicated pathologies of the mediastinum as well as to perform thymectomy for myasthenia gravis. Experience with these techniques has allowed new knowledge to be gained and expansion of the use of these techniques for more complicated mediastinal pathology. This keynote address will outline the instrumentation and techniques that we have adopted over a decade of using these techniques for more complicated mediastinal pathology. PMID:26693146

  14. Cervicothoracic cystic dysraphism.

    PubMed

    Valeur, Natalie S; Iyer, Ramesh S; Ishak, Gisele E

    2016-09-01

    Cystic dysraphism of the cervical and upper thoracic spine is very rare. It differs from the much more common lumbosacral dysraphism in appearance and structure, and usually portends a better prognosis due to lack of functional neurological tissue in the dysraphic sac and absent or less severe intracranial anomalies. There is ambiguity in the literature regarding terminology because of the paucity of cases. We present cases of the most common type of cervicothoracic cystic dysraphism and emphasize differences from lumbosacral myelomeningocele. Patient outcome depends on the presence of associated anomalies and whether complete surgical resection is performed. Imaging plays a critical role in surgical planning, screening the central nervous system for additional anomalies, and in the postoperative setting for evaluation of retethering. PMID:27147079

  15. Cystic pulmonary hydatidosis

    PubMed Central

    Sarkar, Malay; Pathania, Rajnish; Jhobta, Anupam; Thakur, Babu Ram; Chopra, Rajesh

    2016-01-01

    Cystic echinococcosis (CE) is a zoonotic parasitic disease caused by the larval stages of the cestode Echinococcus granulosus. Worldwide, pulmonary hydatid cyst is a significant problem medically, socially, and economically. Surgery is the definitive therapy of pulmonary hydatidosis. Benzimidazoles may be considered in patients with a surgical contraindication. This review will focus on pathogenesis, lifecycle, clinical features, and management of pulmonary hydatid disease. PMID:27051107

  16. Unusual metastases of thyroid cancer to mediastinal blood vessels.

    PubMed

    Ma, Hong Yun; Moadel, Renee; Freeman, Leonard M

    2015-01-01

    Poorly differentiated thyroid cancer is a rare thyroid cancer, accounts for approximately 5% of all thyroid cancer cases, and is associated with a poor prognosis. It commonly metastasizes to regional lymph nodes, lung, and bones. We present a patient with poorly differentiated thyroid cancer with unusual extensive spread to mediastinal blood vessels.

  17. A prospective study of mediastinal gray-zone lymphoma

    PubMed Central

    Pittaluga, Stefania; Nicolae, Alina; Camphausen, Kevin; Shovlin, Margaret; Steinberg, Seth M.; Roschewski, Mark; Staudt, Louis M.; Jaffe, Elaine S.; Dunleavy, Kieron

    2014-01-01

    Mediastinal B-cell lymphomas present in the mediastinum and are most frequent in young patients. Nodular sclerosis Hodgkin lymphoma (NSHL) and primary mediastinal B-cell lymphoma (PMBL) are the common types, whereas mediastinal gray-zone lymphoma (MGZL) is extremely rare and has pathological features intermediate between NSHL and PMBL. The indeterminate pathobiology of MGZL has led to uncertainty regarding therapeutic strategy, and its clinical characteristics and treatment have not been characterized. We conducted a prospective study of infusional dose-adjusted etoposide, doxorubicin, and cyclophosphamide with vincristine, prednisone, and rituximab (DA-EPOCH-R) and filgrastim in untreated MGZL. We analyzed biomarkers of outcome and compared their clinical and biological characteristics to PMBL. Twenty-four MGZL patients had a median age of 33 years (range, 14 to 59 years), and 46% had mediastinal masses ≥10 cm. At 59 months median follow-up, the event-free survival and overall survival were 62% and 74%, respectively. The serum absolute lymphocyte count, the presence of tumor-infiltrating dendritic cells, CD15 expression on the malignant cells, and tumor morphology were biomarkers of outcome in MGZL. Compared with PMBL, MGZL patients were more likely to be male, express CD15, have lower expression of CD20, and have a worse outcome. DA-EPOCH-R alone is effective in MGZL. The trial was registered at ClinicalTrials.gov (NCT00001337). PMID:25024303

  18. A prospective study of mediastinal gray-zone lymphoma.

    PubMed

    Wilson, Wyndham H; Pittaluga, Stefania; Nicolae, Alina; Camphausen, Kevin; Shovlin, Margaret; Steinberg, Seth M; Roschewski, Mark; Staudt, Louis M; Jaffe, Elaine S; Dunleavy, Kieron

    2014-09-01

    Mediastinal B-cell lymphomas present in the mediastinum and are most frequent in young patients. Nodular sclerosis Hodgkin lymphoma (NSHL) and primary mediastinal B-cell lymphoma (PMBL) are the common types, whereas mediastinal gray-zone lymphoma (MGZL) is extremely rare and has pathological features intermediate between NSHL and PMBL. The indeterminate pathobiology of MGZL has led to uncertainty regarding therapeutic strategy, and its clinical characteristics and treatment have not been characterized. We conducted a prospective study of infusional dose-adjusted etoposide, doxorubicin, and cyclophosphamide with vincristine, prednisone, and rituximab (DA-EPOCH-R) and filgrastim in untreated MGZL. We analyzed biomarkers of outcome and compared their clinical and biological characteristics to PMBL. Twenty-four MGZL patients had a median age of 33 years (range, 14 to 59 years), and 46% had mediastinal masses ≥10 cm. At 59 months median follow-up, the event-free survival and overall survival were 62% and 74%, respectively. The serum absolute lymphocyte count, the presence of tumor-infiltrating dendritic cells, CD15 expression on the malignant cells, and tumor morphology were biomarkers of outcome in MGZL. Compared with PMBL, MGZL patients were more likely to be male, express CD15, have lower expression of CD20, and have a worse outcome. DA-EPOCH-R alone is effective in MGZL. The trial was registered at ClinicalTrials.gov (NCT00001337). PMID:25024303

  19. Invasive Esophageal Candidiasis with Chronic Mediastinal Abscess and Fatal Pneumomediastinum

    PubMed Central

    Aghdam, Mohammad Reza F.; Sund, Ståle

    2016-01-01

    Patient: Male, 68 Final Diagnosis: Invasive esophageal candiasis Symptoms: Chest discomfort Medication: — Clinical Procedure: — Specialty: Infectious Diseases Objective: Unusual clinical course Background: Invasive candidiasis is a potential problem for patients receiving long-term immunosuppressive treatment. Psoriatic arthritis is one of many chronic diseases that can be successfully treated with immunosuppressive drugs, in spite of a documented and accepted risk for infectious complications. Critical awareness of possible infection must be part of the surveillance of such patients. Case Report: This is the case of a 68-year-old Norwegian male, treated with long-term immunosuppression for psoriatic arthritis, hospitalized with acute subcutaneous and mediastinal emphysema of unknown cause. He died of acute respiratory failure with circulatory collapse shortly after admission. The autopsy revealed mediastinal and subcutaneous emphysema and a mediastinal abscess containing Candida with probable entrance from the esophagus. Conclusions: We consider invasive candidiasis of the esophagus to be the cause of both the chronic abscess and the acute mediastinal emphysema. This case illustrates the importance of awareness of invasive candidiasis as a possible complication in a patient with long-term immunosuppression. PMID:27389822

  20. Picibanil (OK-432) in the treatment of head and neck lymphangiomas in children

    PubMed Central

    Rebuffini, Elena; Zuccarino, Luca; Grecchi, Emma; Carinci, Francesco; Merulla, Vittorio Emanuele

    2012-01-01

    Background: Picibanil (OK-432) is a lyophilized mixture of group A Streptococcus pyogenes with antineoplastic activity. Because of its capacity to produce a selective fibrosis of lymphangiomas (LMs), it has been approved by Japanese administration in 1995 for the treatment of LMs. Materials and Methods: We treated 15 children (age range: 6-60 months) affected by head and neck macrocystic LMs with intracystic injections (single dose of 0.2 mL) of Picibanil (1-3 injections). Results: Complete disappearance of the lesion was noticed in eight (53.33%) cases, a marked (>50%) reduction of LMs was found five (33.33%) cases, while a moderate (<50%) response was recorded in two (13.33%) cases. Picibanil side effects included fever, local inflammation, and transitory increase of blood platelets’ concentration; a single case of anemia was resolved with concentrated red blood cells transfusion. Conclusions: Intracystic injection of Picibanil is an effective and safe treatment for macrocystic LMs in pediatric patients and may represent the treatment of choice in such cases, especially where surgical excision is associated with the risk of functional/cosmetic side effects. PMID:23814582

  1. Molecular Diagnosis of Cystic Fibrosis.

    PubMed

    Deignan, Joshua L; Grody, Wayne W

    2016-01-01

    This unit describes a recommended approach to identifying causal genetic variants in an individual suspected of having cystic fibrosis. An introduction to the genetics and clinical presentation of cystic fibrosis is initially presented, followed by a description of the two main strategies used in the molecular diagnosis of cystic fibrosis: (1) an initial targeted variant panel used to detect only the most common cystic fibrosis-causing variants in the CFTR gene, and (2) sequencing of the entire coding region of the CFTR gene to detect additional rare causal CFTR variants. Finally, the unit concludes with a discussion regarding the analytic and clinical validity of these approaches.

  2. Cystic Fibrosis Therapeutics

    PubMed Central

    Ramsey, Bonnie W.

    2013-01-01

    A great deal of excitement and hope has followed the successful trials and US Food and Drug Administration approval of the drug ivacaftor (Kalydeco), the first therapy available that targets the underlying defect that causes cystic fibrosis (CF). Although this drug has currently demonstrated a clinical benefit for a small minority of the CF population, the developmental pathway established by ivacaftor paves the way for other CF transmembrane conductance regulator (CFTR) modulators that may benefit many more patients. In addition to investigating CFTR modulators, researchers are actively developing numerous other innovative CF therapies. In this review, we use the catalog of treatments currently under evaluation with the support of the Cystic Fibrosis Foundation, known as the Cystic Fibrosis Foundation Therapeutics Pipeline, as a platform to discuss the variety of candidate treatments for CF lung disease that promise to improve CF care. Many of these approaches target the individual components of the relentless cycle of airway obstruction, inflammation, and infection characteristic of lung disease in CF, whereas others are aimed directly at the gene defect, or the resulting dysfunctional protein, that instigates this cycle. We discuss how new findings from the laboratory have informed not only the development of novel therapeutics, but also the rationales for their use and the outcomes used to measure their effects. By reviewing the breadth of candidate treatments currently in development, as well as the recent progress in CF therapies reflected by the evolution of the therapeutics pipeline over the past few years, we hope to build upon the optimism and anticipation generated by the recent success of Kalydeco. PMID:23276843

  3. Ultrasound techniques in the evaluation of the mediastinum, part 2: mediastinal lymph node anatomy and diagnostic reach of ultrasound techniques, clinical work up of neoplastic and inflammatory mediastinal lymphadenopathy using ultrasound techniques and how to learn mediastinal endosonography.

    PubMed

    Jenssen, Christian; Annema, Jouke Tabe; Clementsen, Paul; Cui, Xin-Wu; Borst, Mathias Maximilian; Dietrich, Christoph Frank

    2015-10-01

    Ultrasound imaging has gained importance in pulmonary medicine over the last decades including conventional transcutaneous ultrasound (TUS), endoscopic ultrasound (EUS), and endobronchial ultrasound (EBUS). Mediastinal lymph node (MLN) staging affects the management of patients with both operable and inoperable lung cancer (e.g., surgery vs. combined chemoradiation therapy). Tissue sampling is often indicated for accurate nodal staging. Recent international lung cancer staging guidelines clearly state that endosonography should be the initial tissue sampling test over surgical staging. Mediastinal nodes can be sampled from the airways [endobronchial ultrasound combined with transbronchial needle aspiration (EBUS-TBNA)] or the esophagus [endoscopic ultrasound fine needle aspiration (EUS-FNA)]. EBUS and EUS have a complementary diagnostic yield and in combination virtually all MLNs can be biopsied. Additionally endosonography has an excellent yield in assessing granulomas in patients suspected of sarcoidosis. The aim of this review in two integrative parts is to discuss the current role and future perspectives of all ultrasound techniques available for the evaluation of mediastinal lymphadenopathy and mediastinal staging of lung cancer. A specific emphasis will be on learning mediastinal endosonography. Part 1 deals with an introduction into ultrasound techniques, MLN anatomy and diagnostic reach of ultrasound techniques and part 2 with the clinical work up of neoplastic and inflammatory mediastinal lymphadenopathy using ultrasound techniques and how to learn mediastinal endosonography.

  4. Primary mediastinal choriocarcinoma with brain metastasis in a female patient.

    PubMed

    Kuno, I; Matsumoto, Y; Kasai, M; Fukuda, T; Hashiguchi, Y; Ichimura, T; Yasui, T; Sumi, T

    2016-01-01

    Nongestational choriocarcinoma is very rare and carries a poor prognosis in female patients. In this report, the authors present a case of nongestational choriocarcinoma with brain metastasis in a female. A 58-year-old female with intermittent back pain was referred to a private hospital. On examination, a mediastinal tumor and a pancreatic tumor were detected. Endoscopic ultrasound-guided fine needle aspiration biopsy of the tumor was performed for histological evaluation. Pathological diagnosis was difficult because only a small amount of tissue was collected. Head MRI showed multiple metastatic tumors in the brain. The patient was diagnosed with primary mediastinal choriocarcinoma with brain metastasis. She was treated with one course of an etoposide, methotrexate, dactinomycin, cyclophosphamide, and vincristine regimen, but her general condition gradually deteriorated, and she died on day 41. Nongestational choriocarcinoma is drug resistant, whereas gestational choriocarcinoma has better chemotherapeutic sensitivity. PMID:27172760

  5. Thoracoscopic resection of functional posterior mediastinal paraganglioma: a case report

    PubMed Central

    Ma, Lin; Mei, Jianong

    2014-01-01

    A 48-year-old man with posterior mediastinal mass was diagnosed as functional mediastinal paraganglioma during surgical exploration via open thoracotomy in another hospital. The operation was terminated because of severe hypertension when touching the tumor. He was transferred to our center later. After systemic evaluation, the patient was medicated with oral alpha- and beta-blockades, as well as intravenous fluid resuscitation for two weeks. His blood pressure became stable and a second operation was planned. The tumor was removed completely via the thoracoscopic approach, and was finally confirmed as functional paraganglioma by immunohistochemistry. The patient recovered uneventfully after surgery, with no recurrence during one year follow-up visit. PMID:25589992

  6. Perforation of esophagus and subsequent mediastinitis following mussel shell ingestion

    PubMed Central

    Park, Il Hwan; Lim, Hyun Kyo; Song, Seung Woo

    2016-01-01

    Esophageal perforation is a very rare occurrence because accidental swallowing of foreign bodies is uncommon in adults. Thus, perforation due to swallowing of a foreign body and subsequent development of mediastinitis is rarely encountered by physicians. We experienced such a case and described an adult male patient who had perforated esophagus after accidentally swallowing a mussel shell. The patient visited our emergency department complaining of painful dysphagia for 4 days. A review of history revealed that he consumed a spicy seafood noodle soup containing mussel shells 4 days ago. Computed tomography (CT) of the chest showed the foreign body in the esophagus and pneumomediastinum was identified. We removed the mussel shell fragment using rigid esophagoscopy; explo-thoracotomy, debridement of mediastinal abscess and irrigation were performed. PMID:27621902

  7. [Malignant mediastinal lesions with invasion to the superior vena cava].

    PubMed

    Kitada, M; Kubo, Y; Hirata, S; Yatsuyanagi, E; Nosaka, T; Sugimoto, S; Moriyama, H; Koshigo, S; Muraki, S; Sasajima, T

    1995-11-01

    Four patients underwent resection of mediastinal malignant tumors with invasion to the superior vena cava. Two patients had invasive thymoma, one seminoma, and one metastatic mediastinal lymph nodes of unknown origin. Prior to resection of the tumor, an ePTFE graft was anastomosed between the innominate vein and the right atrium to maintain the venous drainage from the brain and the upper extremities. In two patients, the superior vena cava was reconstructed by patch angioplasty after the tumor with a part of the vena cava was safely resected. One patient died of acute respiratory failure, but the other three are alive and well without any evidence of graft obstruction. This safe and useful method in order to prevent cerebral congestion during and after resection of the tumor.

  8. [Mediastinal fibrosis associated with Riedel's thyroiditis. Apropos of a case].

    PubMed

    Sembach, N; Benhamou, D; Girault, C; Testard, J; Ozenne, G; Muir, J F

    1995-01-01

    The authors describe a case of mediastinal fibrosis in a 53 year old woman which presented with a superior venacaval syndrome ten years after the diagnosis of a Riedel's thyroiditis. The clinical and laboratory evidence did not reveal anything to suggest other fibrosing disorders (such as retroperitoneal fibrosis, extra-hepatic biliary fibrosis or orbital fibrosis) which could be associated either simultaneously or successively with this multi-focal fibrosis. The physiology of this disorder currently remains imprecise. A current hypothesis is that there is an immunological reaction in the periarterial area leading to lipid components make atheromatous plaques. The therapeutic means are limited and depend on the localisation of the fibrous tissue and of their functional repercussions. Currently, the benefit of corticosteroids on the mediastinal fibrotic lesions has not been demonstrated. PMID:7899673

  9. [Mediastinitis and bilateral pleural effusion of pancreatic origin (author's transl)].

    PubMed

    Holzer, R; Pesendorfer, F X; Pridun, N

    1982-01-01

    A case report is presented of a patient admitted to a thoracic specialist unit on account of predominantly pulmonary symptomatology. There was a left-sided haemorrhagic pulmonary effusion, which rapidly returned after tapping. The patient then developed a high fever and shock. Mediastinal widening was noted on X-ray, as well as a right-sided pleural effusion. Repeated tapping of the pleural effusions and drainage of a mediastinal abscess, which was diagnosed on mediastinoscopy as well as the administration of antibiotics led to an improvement in the patient's previously poor general condition. Sonography and subsequent endoscopic retrograde pancreatography were instrumental in eventually diagnosing chronic recurrent pancreatitis with cyst formation and retroperitoneal perforation into the mediastinum and pleural space. Laparotomy with removal of the pancreatic cysts and abdominal drainage led to complete recovery. The pathogenetic processes involved in the formation of internal fistulae, diagnostic problems and therapeutic management are discussed.

  10. Giant Mediastinal Germ Cell Tumour: An Enigma of Surgical Consideration

    PubMed Central

    Ali, Nurayub Mohd; Azizan, Nornazirah; Zakaria, Andee Dzulkarnaen; Rahman, Mohd Ramzisham Abdul

    2016-01-01

    We present a case of 16-year-old male, who was referred from private centre for dyspnoea, fatigue, and orthopnea. The chest radiograph revealed complete opacification of left chest which was confirmed by computed tomography as a large left mediastinal mass measuring 14 × 15 × 18 cm. The diagnostic needle core biopsy revealed mixed germ cell tumour with possible combination of embryonal carcinoma, yolk sac, and teratoma. After 4 cycles of neoadjuvant BEP regime, there was initial response of tumour markers but not tumour bulk. Instead of classic median sternotomy or clamshell incision, posterolateral approach with piecemeal manner was chosen. Histology confirmed mixed germ cell tumour with residual teratomatous component without yolk sac or embryonal carcinoma component. Weighing 3.5 kg, it is one of the largest mediastinal germ cell tumours ever reported. We describe this rare and gigantic intrathoracic tumour and discuss the spectrum of surgical approach and treatment of this exceptional tumour. PMID:27807495

  11. Paravertebral mediastinal Mullerian cyst resected by video assisted thoracoscopic surgery.

    PubMed

    Chon, Soon-Ho; Im, Ui Jae; Song, Dong Seop

    2015-03-01

    Ever since Hattori et al. had described the mediastinal Mullerian cyst in 2005 there has been several new cases described in the literature. We report a 51-year-old woman with an incidentally found 2 cm × 3 cm mass in her left paravertebral mediastinum. She underwent thoracoscopic removal with the impression of a neurogenic tumor and was unexpectedly found with a ciliated cyst of Mullerian origin.

  12. An unusual case of non-traumatic pneumococcal mediastinal abscess.

    PubMed

    Lotan, C; Boneh, A; Tamir, I; Goitein, K J

    1985-01-01

    A 16-month old baby developed severe respiratory failure because of acute laryngitis and required mechanical ventilation. Intubation was complicated by aspiration and development of chemical pneumonia. Following 4 days of treatment the child was successfully extubated. Thirty six hours after extubation the patient again developed respiratory failure and on chest X-ray pneumomediastinum was seen and later evidence of a mediastinal abscess. Conservative treatment, with antibiotics, effected complete cure.

  13. Giant Chordoma of the Upper Thoracic Spine with Mediastinal Involvement: A Surgical Challenge

    PubMed Central

    Rena, Ottavio; Allegra, Giuliano; Casadio, Caterina; Turello, Davide

    2014-01-01

    Thoracic chordomas are very rare malignant tumours originating from notochordal remnants. These tumours develop within a vertebral body and enlarge involving the mediastinal compartment. Because of their slow-growing attitude, they become symptomatic only when they invade or compress the spinal cord and/or mediastinal organs. We present a rare case of a thoracic spine chordoma presenting with increasing paraparesis with a huge mediastinal component which was surgically debulked to decompress the spinal cord and medistinal organs. PMID:24967050

  14. Mediastinal Mass with Hyper-eosinophilia in a Young Boy -A Diagnostic Dilemma

    PubMed Central

    Prem, Shruti; Jacob, Priya Mary; Nair, Rekha Appukuttan

    2016-01-01

    Mediastinal masses in children comprises of a heterogeneous group of tumours. In such cases, biopsy and histological analysis are mandatory for planning of treatment. We have reported an unusual aetiology for a mediastinal mass in a young boy presenting with features of Superior Vena Caval Obstruction (SVCO) who also had marked blood and marrow eosinophilia mimicking Chronic Eosinophilic Leukaemia (CEL). We have also discussed the differential diagnoses of mediastinal tumours with hyper-eosinophilia and possible therapeutic implications.

  15. Mediastinal Mass with Hyper-eosinophilia in a Young Boy -A Diagnostic Dilemma.

    PubMed

    Parambil, Abdul Shahid Poovathum; Prem, Shruti; Jacob, Priya Mary; Nair, Rekha Appukuttan

    2016-07-01

    Mediastinal masses in children comprises of a heterogeneous group of tumours. In such cases, biopsy and histological analysis are mandatory for planning of treatment. We have reported an unusual aetiology for a mediastinal mass in a young boy presenting with features of Superior Vena Caval Obstruction (SVCO) who also had marked blood and marrow eosinophilia mimicking Chronic Eosinophilic Leukaemia (CEL). We have also discussed the differential diagnoses of mediastinal tumours with hyper-eosinophilia and possible therapeutic implications.

  16. Mediastinal Mass with Hyper-eosinophilia in a Young Boy -A Diagnostic Dilemma

    PubMed Central

    Prem, Shruti; Jacob, Priya Mary; Nair, Rekha Appukuttan

    2016-01-01

    Mediastinal masses in children comprises of a heterogeneous group of tumours. In such cases, biopsy and histological analysis are mandatory for planning of treatment. We have reported an unusual aetiology for a mediastinal mass in a young boy presenting with features of Superior Vena Caval Obstruction (SVCO) who also had marked blood and marrow eosinophilia mimicking Chronic Eosinophilic Leukaemia (CEL). We have also discussed the differential diagnoses of mediastinal tumours with hyper-eosinophilia and possible therapeutic implications. PMID:27630938

  17. Complete transthoracic resection of giant posterior mediastinal goiter: case report and review of surgical strategies

    PubMed Central

    Zhao, Honglin; Ren, Dian; Liu, Yi; Li, Xin; Wu, Yi; Chen, Gang; Chen, Jun

    2016-01-01

    Intrathoracic goiters generally occupy anterior mediastinum, rarely involving the posterior mediastinal space. Reported herein is a 54-year-old female with a giant posterior mediastinal mass that was successfully resected via right posterolateral thoracotomy. The final pathologic diagnosis was giant posterior mediastinal goiter. This patient has done well postoperatively, with no evidence of local recurrence at 12-month follow-up. Related surgical strategies in past publications are summarized. PMID:27217766

  18. Mediastinal Mass with Hyper-eosinophilia in a Young Boy -A Diagnostic Dilemma.

    PubMed

    Parambil, Abdul Shahid Poovathum; Prem, Shruti; Jacob, Priya Mary; Nair, Rekha Appukuttan

    2016-07-01

    Mediastinal masses in children comprises of a heterogeneous group of tumours. In such cases, biopsy and histological analysis are mandatory for planning of treatment. We have reported an unusual aetiology for a mediastinal mass in a young boy presenting with features of Superior Vena Caval Obstruction (SVCO) who also had marked blood and marrow eosinophilia mimicking Chronic Eosinophilic Leukaemia (CEL). We have also discussed the differential diagnoses of mediastinal tumours with hyper-eosinophilia and possible therapeutic implications. PMID:27630938

  19. Supradiaphragmatic Hodgkin's disease: significance of large mediastinal masses

    SciTech Connect

    Prosnitz, L.R.; Curtis, A.M.; Knowlton, A.H.; Peters, L.M.; Farber, L.R.

    1980-07-01

    In order to assess the significance of large mediastinal masses in patients with Hodgkin's disease, we analyzed all patients with pathological stage (PS) IA or IIA disease evaluated and treated at Yale between 1969 and 1978. There were 131 such patients treated initially with radical radiotherapy only, combination chemotherapy being reserved for those who failed radiation. Actuarial 5 and 10 year survivals were 95%. The presence of a mediastinal mass regardless of size did not affect survival. Relapse-free survival was 77% at 5 years, 74% at 10 years in the entire group. Patients with any mediastinal involvement had a 65% relapse-free survival, 72% if the mass was < 33% of transverse chest diameter, 55% if the mass was > 33%. These differences are suggestive of a greater tendency of such patients to fail radiotherapy but the differences were not statistically significant. Patients who did fail radiotherapy were for the most part successfully retreated with combined modality therapy (chemotherapy and radiation), accounting for the most part successfully retreated with combined modality therapy (chemotherapy and radiation), accounting for the overall survival of 95%. Only 6 patients died of causes related to Hodgkin's disease and 2 of these deaths were related to combined modality therapy complications. Because of the serious potential long term consequences of combined modality treatment, it should be used with great caution and on an individual basis only in PSIA and IIA patients.

  20. Mediastinal Hibernoma: A Rare Case with Radiologic-Pathologic Correlation

    PubMed Central

    Dasyam, Anil; Then, Matthew; Varma, Kavita; Borhani, Amir A.

    2016-01-01

    Hibernomas, especially located in the mediastinum, are extremely rare benign tumors, which are important to consider in the differential diagnosis of a heterogeneously enhancing mass with areas of fat attenuation on imaging of an often incidentally discovered mass. Other common possibilities in the differential include malignant tumors, such as liposarcoma, hence histopathology is usually required to confirm the diagnosis. Hibernomas often follow the distribution of sites of persistence of brown fat in adults, and intrathoracic locations are unusual. We present a very rare case of a mediastinal hibernoma in a 53-year-old woman. She presented to the emergency department with severe, progressive right neck and shoulder pain with radiation down her arm and was found to have a right apical posterior mediastinal mass on imaging. Initial radiographs of the shoulder showed a soft tissue mass within the apical right hemithorax. Further imaging with CT revealed a well circumscribed, heterogeneously enhancing mass with areas of fat attenuation. Pathology confirmed the diagnosis of mediastinal hibernoma, and the mass was completely excised. Fourteen months after surgery, the patient had a normal chest radiograph, and thirty-two months after surgery, she remains asymptomatic. PMID:27651970

  1. Tuberculous constrictive pericarditis complicated with tuberculous mediastinitis - case report.

    PubMed

    Man, Milena Adina; NiŢu, Mimi Floarea; Strâmbu, Lelia; Florescu, Cristina; Streba, Costin Teodor; Trofor, Antigona Carmen

    2016-01-01

    Constrictive pericarditis is a rare and severe disease. A 37-year-old patient was admitted in the hospital for dyspnea, precordial pain, right-sided cardiac failure. Chest X-ray showed cardiac enlargement and an opacity suggestive for pleural effusion. Echocardiography revealed an adhesive-effusive-constrictive pericarditis, a very thickened pericardium and bilateral pleural effusion. After a pericardiectomy done to restore cardiac compensation and to identify etiological factors, a tuberculous pericarditis (TBP) was diagnosed. After surgery and starting anti-TB treatment, the patient presented altered clinical status, dyspnea, dry cough, fever and delayed callus formation at sternum level. Thoracic scan revealed mediastinal air collections, pericarditis and pleurisy. Thus, the TBP diagnosis was extended to mediastinal TB and anti-TB therapy was continued. After four months of treatment, another thoracic scan showed disappearance of the mediastinal air-leakage bubbles, multiple new micronodules in both lungs and lymph nodes of up to 15 mm; also increasing pericardial and pleural effusions. This case was interpreted as a TB treatment failure situation. A retreatment regimen was started, resulting in a slow favorable outcome. Pericardial TB is a rare condition, usually with delayed diagnosis and poor treatment benefits. Whenever possible, earlier diagnostic can contribute to better management of these cases.

  2. Tuberculous constrictive pericarditis complicated with tuberculous mediastinitis - case report.

    PubMed

    Man, Milena Adina; NiŢu, Mimi Floarea; Strâmbu, Lelia; Florescu, Cristina; Streba, Costin Teodor; Trofor, Antigona Carmen

    2016-01-01

    Constrictive pericarditis is a rare and severe disease. A 37-year-old patient was admitted in the hospital for dyspnea, precordial pain, right-sided cardiac failure. Chest X-ray showed cardiac enlargement and an opacity suggestive for pleural effusion. Echocardiography revealed an adhesive-effusive-constrictive pericarditis, a very thickened pericardium and bilateral pleural effusion. After a pericardiectomy done to restore cardiac compensation and to identify etiological factors, a tuberculous pericarditis (TBP) was diagnosed. After surgery and starting anti-TB treatment, the patient presented altered clinical status, dyspnea, dry cough, fever and delayed callus formation at sternum level. Thoracic scan revealed mediastinal air collections, pericarditis and pleurisy. Thus, the TBP diagnosis was extended to mediastinal TB and anti-TB therapy was continued. After four months of treatment, another thoracic scan showed disappearance of the mediastinal air-leakage bubbles, multiple new micronodules in both lungs and lymph nodes of up to 15 mm; also increasing pericardial and pleural effusions. This case was interpreted as a TB treatment failure situation. A retreatment regimen was started, resulting in a slow favorable outcome. Pericardial TB is a rare condition, usually with delayed diagnosis and poor treatment benefits. Whenever possible, earlier diagnostic can contribute to better management of these cases. PMID:27151714

  3. Mediastinal Hibernoma: A Rare Case with Radiologic-Pathologic Correlation.

    PubMed

    Darke, Maxine; Dasyam, Anil; Then, Matthew; Varma, Kavita; Borhani, Amir A; Varma, Rakesh

    2016-01-01

    Hibernomas, especially located in the mediastinum, are extremely rare benign tumors, which are important to consider in the differential diagnosis of a heterogeneously enhancing mass with areas of fat attenuation on imaging of an often incidentally discovered mass. Other common possibilities in the differential include malignant tumors, such as liposarcoma, hence histopathology is usually required to confirm the diagnosis. Hibernomas often follow the distribution of sites of persistence of brown fat in adults, and intrathoracic locations are unusual. We present a very rare case of a mediastinal hibernoma in a 53-year-old woman. She presented to the emergency department with severe, progressive right neck and shoulder pain with radiation down her arm and was found to have a right apical posterior mediastinal mass on imaging. Initial radiographs of the shoulder showed a soft tissue mass within the apical right hemithorax. Further imaging with CT revealed a well circumscribed, heterogeneously enhancing mass with areas of fat attenuation. Pathology confirmed the diagnosis of mediastinal hibernoma, and the mass was completely excised. Fourteen months after surgery, the patient had a normal chest radiograph, and thirty-two months after surgery, she remains asymptomatic. PMID:27651970

  4. CT of chronic infiltrative lung disease: Prevalence of mediastinal lymphadenopathy

    SciTech Connect

    Niimi, Hiroshi; Kang, Eun-Young; Kwong, S.

    1996-03-01

    Our goal was to determine the prevalence of mediastinal lymph node enlargement at CT in patients with diffuse infiltrative lung disease. The study was retrospective and included 175 consecutive patients with diffuse infiltrative lung diseases. Diagnoses included idiopathic pulmonary fibrosis (IPF) (n = 61), usual interstitial pneumonia associated with collagen vascular disease (CVD) (n = 20), idiopathic bronchiolitis obliterans organizing pneumonia (BOOP) (n = 22), extrinsic allergic alveolitis (EAA) (n = 17), and sarcoidosis (n = 55). Fifty-eight age-matched patients with CT of the chest performed for unrelated conditions served as controls. The presence, number, and sites of enlarged nodes (short axis {ge}10 mm in diameter) were recorded. Enlarged mediastinal nodes were present in 118 of 175 patients (67%) with infiltrative lung disease and 3 of 58 controls (5%) (p < 0.001). The prevalence of enlarged nodes was 84% (46 of 55) in sarcoidosis, 67% (41 of 61) in IPF, 70% (14 of 20) in CVD, 53% (9 of 17) in EAA, and 36% (8 of 22) in BOOP. The mean number of enlarged nodes was higher in sarcoidosis (mean 3.2) than in the other infiltrative diseases (mean 1.2) (p < 0.001). Enlarged nodes were most commonly present in station 10R, followed by 7, 4R, and 5. Patients with infiltrative lung disease frequently have enlarged mediastinal lymph nodes. However, in diseases other than sarcoid, usually only one or two nodes are enlarged and their maximal short axis diameter is <15 mm. 11 refs., 2 figs., 1 tab.

  5. Nutritional Issues in Cystic Fibrosis.

    PubMed

    Solomon, Missale; Bozic, Molly; Mascarenhas, Maria R

    2016-03-01

    The importance of maintaining adequate nutrition in patients with cystic fibrosis has been well known for the past 3 decades. Achieving normal growth and maintaining optimal nutrition is associated with improved lung function. Comprehensive and consistent nutritional assessments at regular intervals can identify those at risk of nutritional failure and uncover micronutrient deficiencies contributing to malnutrition. Management of malnutrition in cystic fibrosis should follow a stepwise approach to determine the causes and comorbidities and to develop a nutritional plan. Nutritional management is crucial at every stage in a person's life with cystic fibrosis and remains a cornerstone of management.

  6. Pneumothorax in cystic fibrosis

    PubMed Central

    Kioumis, Ioannis P.; Zarogoulidis, Konstantinos; Huang, Haidong; Li, Qiang; Dryllis, Georgios; Pitsiou, Georgia; Machairiotis, Nikolaos; Katsikogiannis, Nikolaos; Papaiwannou, Antonis; Lampaki, Sofia; Porpodis, Konstantinos; Zaric, Bojan; Branislav, Perin; Mpoukovinas, Ioannis; Lazaridis, George

    2014-01-01

    Pneumothorax is recognized as a common and life-threatening complication in cystic fibrosis (CF) patients, especially in those who are infected with P. aeruginosa, B. cepacia or Aspergillus, need enteral feeding, are diagnosed as suffering from allergic bronchopulmonary aspergillosis (ABPA), developed massive hemoptysis, and their respiratory function is seriously compromised. Structural impairment and altered airflow dynamics in the lungs of CF patients are considered as the main predisposing factors, but also inhaled medications and non-invasive positive pressure ventilation (NIPPV) could increase the risk of pneumothorax. Clinical presentation could range from dramatic to very mild. Management of spontaneous pneumothorax occurring to patients with CF is essentially similar to that for non-CF patients. Therapeutic options include intercostal tube drainage, video-assisted thoracoscopic surgery (VATS), and medical or surgical pleurodesis. Pneumothorax increases both short- and long-term morbidity and mortality in CF patients and causes significant deterioration of their quality of life. PMID:25337406

  7. Genetics Home Reference: cystic fibrosis

    MedlinePlus

    ... protects the linings of the airways, digestive system, reproductive system, and other organs and tissues. In people with ... experience health problems affecting the respiratory, digestive, and reproductive systems. Most men with cystic fibrosis have congenital bilateral ...

  8. 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

  9. Nasal Methicillin-Resistant S. Aureus is a Major Risk for Mediastinitis in Pediatric Cardiac Surgery

    PubMed Central

    2015-01-01

    Background: Mediastinitis caused by methicillin-resistant Staphylococcus aureus (MRSA) is a serious complication after pediatric cardiac surgery. An outbreak of surgical site infections (SSIs) provided the motivation to implement SSI prevention measures in our institution. Methods: Subjects comprised 174 pediatric patients who underwent open-heart surgery after undergoing preoperative nasal culture screening. The incidence of SSIs and mediastinitis was compared between an early group, who underwent surgery before SSI measures (Group E, n = 73), and a recent group, who underwent surgery after these measures (Group R, n = 101), and factors contributing to the occurrence of mediastinitis were investigated. Results: The incidence of both SSIs and Mediastinitis has significantly decreased after SSI measures. With regard to factors that significantly affected mediastinitis, preoperative factors were “duration of preoperative hospitalization” and “preoperative MRSA colonization,” intraoperative factors were “Aristotle basic complexity score,” “operation time,” “cardiopulmonary bypass circuit volume” and “lowest rectal temperature.” And postoperative factor was “blood transfusion volume.” Patients whose preoperative nasal cultures were MRSA-positive suggested higher risk of MRSA mediastinitis. Conclusions: SSI prevention measures significantly reduced the occurrence of SSIs and mediastinitis. Preoperative MRSA colonization should be a serious risk factor for mediastinitis following pediatric cardiac surgeries. PMID:25641035

  10. Pre-operative mediastinal evaluation in primary bronchial carcinoma--a review of staging investigations.

    PubMed Central

    Elliott, J. A.

    1984-01-01

    A review of staging investigations in the preoperative evaluation of mediastinal involvement in primary bronchial carcinoma is presented. The following conclusions are offered as guidelines for the use of mediastinal staging procedures in clinical practice: Surgical staging methods have the over-riding advantage of superior specificity over indirect imaging techniques. Where 67Ga-imaging or CT scanning are not available, routine pre-operative mediastinoscopy or, when appropriate, mediastinotomy will identify most patients with non-resectable disease but this approach entails a high proportion of true negative examinations. Radioisotope ventilation and perfusion lung imaging has no place in the pre-operative staging of lung cancer. Where the techniques are available, 67Ga-imaging and CT scanning have a use in selecting patients for mediastinal exploration. A negative mediastinal 67Ga scan or a negative CT examination suggest that mediastinal exploration will be unrewarding in the vast majority of cases and may be omitted prior to thoracotomy. A positive mediastinal 67Ga scan or the demonstration of abnormal mediastinal nodes by CT is an indication for mediastinal exploration which, if negative should be followed by thoracotomy. PMID:6369288

  11. [Acute suppurative mediastinitis in heart surgery performed under extracorporeal circulation].

    PubMed

    Amrein, C; Guibourt, P; Garcia, A; Benizri, A; Trinquier, R; Vaudron, E; Gomez, F; Deloche, A; Carpentier, A; Passelecq, J

    1984-10-01

    The incidence of acute suppurative mediastinitis after open heart cardiac surgery in a 5 year retrospective study was found to be 1.56%. The commonest causal agent was the DNAse + coagulase + staphylococcus (68.8%). All cases were treated with bactericidal antibiotics and local measures (dosed chest irrigation-drainage in 1 or 2 stages). Adult men, early and/or late reoperation, immediate postoperative complications (cardiovascular collapse, haemorrhage) were positive risk factors. On the other hand, the type of underlying cardiac disease, the duration of surgery and bypass time, emergency surgery, and protocols of prophylactic antibiotic therapy did not seem to influence this risk. No sources of exogenous contamination, no notions of epidemics or periodicity were discovered in this study. The clinical course may be complicated, especially by renal failure. Thirty-two of the 109 patients studied died. However, the prognosis has improved in the last 5 years in relation to technical advances in intensive care and, above all, to adoption of 1 stage dosed chest irrigation-drainage: the mortality rate has fallen from 37.2% in the first period to 20% more recently. When a cure is obtained, it is complete and there are no sequellae. Nevertheless, irrespective of the adoption of draconian measures of asepsis and protocols of prophylactic antibiotic therapy, the risk of acute postoperative mediastinitis persists. This suggests that the efficacy of the patient's local (and general) anti-infective immunity defenses may vary in the presence of constant mediastinal bacterial contamination despite aleatory preventive measures, due to the remenance of the skin flora.

  12. Mediastinal large-cell lymphoma with sclerosis (MLCLS).

    PubMed Central

    Rohatiner, A. Z.; Whelan, J. S.; Ganjoo, R. K.; Norton, A. J.; Wilson, A.; Lister, T. A.

    1994-01-01

    In a retrospective analysis encompassing a 14 year period (1978-92), 22 patients (age range 19-71, median 30 years) were identified as having mediastinal large-cell lymphoma with sclerosis on the basis of clinical and pathological features. At presentation, 15/22 had 'bulky' disease and 11/22 had evidence of superior vena caval obstruction. Thirteen patients had stage II disease (6,II; 7,IIE), nine presented with stage IV disease. Complete remission (CR) was achieved in only 4/22 patients with the initial adriamycin-containing regimen. 'Good partial remission' (no clinical evidence of disease, minimal abnormalities of uncertain significance on radiological investigation) was achieved in a further seven patients and 'poor partial remission' (a reduction in measurable disease > 50%) in four, giving an overall response rate of 15/22 (68%). One patient died within 48 h of arrival at the hospital; 16 of the 17 remaining patients in whom anything less than CR was achieved subsequently received additional, alternative treatment (one chemotherapy, six mediastinal radiotherapy, nine both treatment modalities) but in only 2/16 did this result in any further degree of response. With a median follow-up of 5 1/2 years, 10/22 patients remain well without progression between 6 months and 14 years (5/6 in whom CR was eventually achieved and 5/11 in whom only partial remission was ever documented). The seven patients in whom the initial treatment demonstrably failed have all died. These results suggest that a proportion of patients with this rare subtype of high-grade B-cell lymphoma may be cured by chemotherapy alone and that the presence of a residual mediastinal mass after treatment does not necessarily imply treatment failure. However, patients in whom the initial chemotherapy fails have a very grave prognosis. PMID:8123496

  13. Surgical debridement, vacuum therapy and pectoralis plasty in poststernotomy mediastinitis.

    PubMed

    Ennker, I C; Pietrowski, D; Vöhringer, L; Kojcici, B; Albert, A; Vogt, P M; Ennker, J

    2009-11-01

    In cardiac surgery poststernotomy mediastinitis continues to be a serious cause of morbidity and mortality. We report our experience with vacuum-assisted closure (VAC) therapy followed by reconstruction with M. pectoralis muscle flaps as treatment for deep sternal wound infections. Our group performed a retrospective analysis of 3630 consecutive cardiac surgical patients using median sternotomy from 11/2004 to 11/2007. After removing sternal wires, necrotic debris and potentially infective material, restabilisation of the sternum was performed and VAC therapy was employed. Wound closure and subsequent reconstruction were performed using a bilateral pectoralis muscle plasty. Of the analysed patients 16 female and 29 male patients suffered from deep sternal wound infections and were treated with VAC. The most common risk factors were diabetes mellitus odds ratio (OR 3.5), chronic obstructive pulmonary disease (COPD) (OR 2.9), use of bilateral mammarian artery (OR 2.0) and obesity (1.8). The median age of patients with deep sternal infections was similar to control patients. Staphylococcus epidermis was the most common pathogen (37.8%) followed by Enterococcus faecilis (22.2%) and Staphylococcus aureus (17.8). In 22.2% no pathogen could be detected. The 30 day mortality was 0%, the in-hospital mortality was 15.6%. The results of our studies demonstrate that vacuum therapy in conjunction with early and aggressive debridement is an effective strategy for treating poststernotomy mediastinitis. We consider pectoralis major muscle flap reconstruction as a safe technique and regard it as the primary choice for wound closure in poststernotomy mediastinitis. PMID:18996074

  14. [Early diagnosis of a newborn with a mediastinal mass].

    PubMed

    Flores-Hernández, Salomón Sergio; Ahumada Mendoza, Héctor; Santana-Montero, Blanca Lilia; González Flores, María de Lourdes

    2005-01-01

    Neuroblastoma is an embryonal tumour that evolves from the neural crest cell. This neoplasm may arise at any site in the sympathetic nervous system, including the brain, the cervical region, the posterior mediastinum, the para-aortic sympathetic ganglia, the pelvis, and the adrenal medulla. The clinical presentation in neonatal age is rare and the differential diagnosis includes congenital lung malformations, pneumoniae, atelectasia, etc. The case of a newborn with a mediastinal neuroblastoma is presented. This case illustrates how a patient with an X-ray image compatible with a thoracic tumor should be studied. PMID:16381510

  15. A Three-Dimensional Mediastinal Model Created with Rapid Prototyping in a Patient with Ectopic Thymoma

    PubMed Central

    Nakada, Takeo; Inagaki, Takuya

    2014-01-01

    Preoperative three-dimensional (3D) imaging of a mediastinal tumor using two-dimensional (2D) axial computed tomography is sometimes difficult, and an unexpected appearance of the tumor may be encountered during surgery. In order to evaluate the preoperative feasibility of a 3D mediastinal model that used the rapid prototyping technique, we created a model and report its results. The 2D image showed some of the relationship between the tumor and the pericardium, but the 3D mediastinal model that was created using the rapid prototyping technique showed the 3D lesion in the outer side of the extrapericardium. The patient underwent a thoracoscopic resection of the tumor, and the pathological examination showed a rare middle mediastinal ectopic thymoma. We believe that the construction of mediastinal models is useful for thoracoscopic surgery and other complicated surgeries of the chest diseases. PMID:24633133

  16. Mucolytics in cystic fibrosis.

    PubMed

    Henke, Markus O; Ratjen, Felix

    2007-03-01

    Mucus accumulation in the lower airways is a key feature of cystic fibrosis (CF) lung disease. The major component of mucus in CF is not mucin derived from mucus producing cells but rather pus that includes viscous material such as polymerized DNA derived from degraded neutrophils. This has important implications for mucolytic therapy aiming to improve mucus clearance from the airways, since degradation of mucin may not be a suitable treatment strategy. In addition, thinning of secretions may not always be beneficial, since it may negatively affect certain aspects of mucus transport such as cough clearance. While inhaled N-acetylcysteine has been used as a mucolytic drug in CF for decades, there is little evidence that it has any beneficial effect. Dornase alfa has been shown to reduce pulmonary exacerbations and improve lung function and is currently the only mucolytic agent with proven efficacy in CF. Newer agents targeting other components of CF mucus, such as filamentous actin, are currently in development. Ultimately, drugs that are mucokinetic, which preserve viscoelasticity, rather than mucolytic may prove to be beneficial for CF lung disease in the future. PMID:17419975

  17. Cystic Fibrosis Sinusitis.

    PubMed

    Le, Christopher; McCrary, Hilary C; Chang, Eugene

    2016-01-01

    Cystic fibrosis (CF) is an autosomal recessive genetic disorder caused by mutations in the CF transmembrane conductance regulator gene(CFTR) resulting in impaired ion transport. Nearly all people with CF will develop chronic rhino-sinusitis (CRS) and present with the characteristic viscous mucus, impaired mucociliary clearance and chronic inflammation/infection of the sinonasal cavity. While some individuals with CF can appear relatively asymptomatic in terms of their sinus disease, commonly reported symptoms include anosmia, headache, facial pain, nasal obstruction, chronic congestion and nasal discharge. Nasal endoscopy typically reveals mucosal edema, purulent discharge and nasal polyposis. Computed tomography (CT) imaging classically demonstrates the distinguishing findings of sinus hypoplasia or aplasia with generalized opacification, medial bulging of the lateral sinonasal sidewall and a demineralized uncinate process. Current treatment for CF sinusitis includes the use of hypertonic saline, topical and systemic steroids, antibiotics and endoscopic surgery. Research investigating novel therapies designed at targeting the primary defect of CF is showing promise for reversal of CF sinus disease, in addition to potential for disease prevention. PMID:27466844

  18. PULMONARY CYSTIC ECHINOCOCCOSIS

    PubMed Central

    Santivanez, Saul; Garcia, Hector H.

    2012-01-01

    Pulmonary cystic echinococosis, a zoonosis caused by the larvae of the dog tapeworm Echinococcus granulosus, is considered as a major public health problem in those countries where dogs are used to care for large herds because of the incapacitating effects produced in affected population. The ratio lung:liver involvement is higher in children than in adults. A higher proportion of lung cases are discovered incidentally on a routine x-ray evaluation; the majority of infected people remain asymptomatic until the cyst enlarges sufficiently to cause symptoms. The majority of symptoms are caused by mass effect from the cyst volume; the presence of complications caused by cysts broke changes the clinical presentation; the principal complication is cyst rupture, producing cough, chest pain, hemoptysis, or vomica. Diagnosis is obtained by imaging evaluation (Chest X-ray or CT scan), supported by serology in the majority of cases. Surgery is the main therapeutic approach, having as principal objective, the removal of the parasite, preventing intraoperative dissemination; the use of pre surgical chemotherapy reduces the chances of seeding and recurrence; treatment using benzimidazoles is the preferred treatment when surgery is not available, or complete removal is not feasible PMID:20216420

  19. 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.

  20. Mucinous Cystic Neoplasms of Pancreas

    PubMed Central

    Naveed, Shah; Qari, Hasina; Banday, Tanveer; Altaf, Asma; Para, Mah

    2014-01-01

    The purpose of this study was to investigate the actual management of mucinous cystic neoplasm (MCN) of the pancreas. A systematic review was performed in December 2009 by consulting PubMed MEDLINE for publications and matching the key words “pancreatic mucinous cystic neoplasm”, “pancreatic mucinous cystic tumor”, “pancreatic mucinous cystic mass”, “pancreatic cyst” and “pancreatic cystic neoplasm” to identify English language articles describing the diagnosis and treatment of the MCN of the pancreas. In total, 16,322 references ranging from January 1969 to December 2009 were analyzed and 77 articles were identified. No articles published before 1996 were selected because MCNs were not previously considered to be a completely autonomous disease. Definition, epidemiology, anatomopathological findings, clinical presentation, preoperative evaluation, treatment and prognosis were reviewed. MCNs are pancreatic mucin-producing cysts with a distinctive ovarian-type stroma localized in the body-tail of the gland and occurring in middle-aged females. The majority of MCNs are slow growing and asymptomatic. The prevalence of invasive carcinoma varies between 6% and 55%. Preoperative diagnosis depends on a combination of clinical features, tumor markers, computed tomography (CT), magnetic resonance imaging, endoscopic ultrasound with cyst fluid analysis and positron emission tomography-CT. Surgery is indicated for all MCNs.

  1. Using Cystic Fibrosis Therapies for Non-Cystic Fibrosis Bronchiectasis.

    PubMed

    ElMaraachli, Wael; Conrad, Douglas J; Wang, Angela C C

    2016-03-01

    Non-cystic fibrosis bronchiectasis (NCFB) is an increasingly prevalent disease that places a significant burden on patients and health systems globally. Although many of the therapies used to treat NCFB were originally developed as cystic fibrosis (CF) therapies, not all of them have been demonstrated to be efficacious in NCFB and some may even be harmful. This article explores the evidence for which therapeutic strategies used to treat CF have been translated into the care of NCFB. The conclusion is that therapies for adult NCFB cannot be simply extrapolated from CF clinical trials, and in some instances, doing so may actually result in harm.

  2. Acute neck cellulitis and mediastinitis complicating a continuous interscalene block.

    PubMed

    Capdevila, Xavier; Jaber, Samir; Pesonen, Pertti; Borgeat, Alain; Eledjam, Jean-Jacques

    2008-10-01

    We report a case of acute neck cellulitis and mediastinitis complicating a continuous interscalene brachial plexus block. A 61-yr-old man was scheduled for an elective arthroscopic right shoulder rotator cuff repair. A continuous interscalene block was done preoperatively and 20 mL of 0.5% bupivacaine and 20 mL of 2% mepivacaine were injected through the catheter. Postoperative analgesia was provided by a continuous infusion of bupivacaine, 0.25% at 5 mL/h for 39 h using a 240-mL elastomeric disposable pump. The day after surgery, the patient complained of neck pain. The analgesic block was not fully effective. He was discharged home. Three days later, the patient was readmitted with neck edema and erythema, fever and fatigue. Neck ultrasonography and computed tomographic scan revealed an abscess of the interscalene and sternocleidomastiod muscles and cellulitis, as well as acute mediastinitis. Two blood cultures and surgical samples were positive for Staphylococcus aureus. The infection was treated with surgery, the site was surgically debrided, and a 2-mo course of vancomycin, imipenem, and oxacilline. The technique of drawing local anesthetic from the bottle and filling the elastomeric pump was the most likely cause of infection. This case emphasizes the importance of strict aseptic conditions during puncture, catheter insertion, and management of the local anesthetic infusate. PMID:18806062

  3. The anaesthetic management of children with anterior mediastinal masses.

    PubMed

    Hack, H A; Wright, N B; Wynn, R F

    2008-08-01

    Children with anterior mediastinal masses may experience serious complications during general anaesthesia. We retrospectively surveyed the records of children with an anterior mediastinal mass who had been admitted to our hospital over a 7 year period. The presence of pre-operative symptoms or signs, findings of any special investigations performed and the anaesthetic outcome were noted. All radiological investigations were studied and tracheal compression measured. The majority of patients presented with severe clinical signs. There was a poor relationship between clinical signs and size of tumour or tracheal compression on CT scan. Corticosteroids were used prior to diagnosis in 33% of patients, all of whom were considered high risk. A clear diagnosis was made in 95% of these patients. The overall complication rate was 20% and 5% of patients had a serious complication related to anaesthesia. Stridor was the only sign that predicted an anaesthetic complication. Peri-operative respiratory complications were confined to patients with an isolated tracheal cross-sectional area less than 30% normal or less than 70% and associated with bronchial compression.

  4. Belatacept and mediastinal histoplasmosis in a kidney transplant patient

    PubMed Central

    Trimarchi, Hernán; Rengel, Tatiana; Andrews, José; Paulero, Matías; Iotti, Alejandro; Forastiero, Agustina; Lombi, Fernando; Pomeranz, Vanesa; Forrester, Mariano; Iriarte, Romina; Agorio, Iris

    2016-01-01

    Background: In transplantation immunosuppression enhances the appearance of opportunist infections. An ideal balance between the prevention of rejection, the lowest risk of infections and the highest rates of graft survival is a continuous challenge. Lower doses of immunosuppression may diminish the risk of infections, metabolic and hemodynamic complications or even of malignancy, but may expose patients to episodes of acute rejection. New drugs are being developed to improve graft survival at the lowest risk of side effects. Belatacept has recently been introduced in kidney transplantation to inhibit the co-ligand signal of T cell stimulation. It is a drug with a safe profile, is well-tolerated and appears to improve long-term survival of kidney grafts. However, there may be an increase in opportunistic infections which may be facilitated by T cell depression, as Aspergillus sp., Cryptococcus neoformans or tuberculosis. Case Presentation: We describe a 59-year-old female who developed fever, clinical wasting and a mediastinal mass 31 months after receiving a living non-related kidney transplant while on belatacept therapy. A mediastinal node biopsy disclosed the presence of Histoplasma capsulatum. Infection successfully resolved after appropriate antifungal treatment. Conclusions: To our knowledge, this is the first reported case of Histoplasma capsulatum in a kidney transplanted patient on belatacept therapy PMID:27152295

  5. A Case of Bilateral Cystic Partially Differentiated Nephroblastoma vs Cystic Wilms' Tumor: Highlighting a Diagnostic Dilemma.

    PubMed

    Stout, Thomas E; Au, Jason K; Hicks, J M; Gargollo, Patricio C

    2016-06-01

    Cystic partially differentiated nephroblastoma (CPDN) is a rare multicystic renal tumor along the spectrum of cystic nephroma and cystic Wilms' tumor. There have only been two previously reported cases of bilateral CPDN in the literature. We present here a case of bilateral CPDN vs cystic Wilms' tumor treated with neoadjuvant and adjuvant chemotherapy in addition to a bilateral partial nephrectomy. We also review the relevant literature regarding CPDN in an effort to aid in diagnosis and management of these rare cystic renal tumors.

  6. [Cystic cancer of the kidney].

    PubMed

    el Moussaoui, A; Dakir, M; Sarf, I; Aboutaeib, R; el Mrini, M; Benjelloun, S

    1997-01-01

    Cystic renal cancer is uncommon and raises real preoperative diagnostic problems, requiring the use of medical imaging, and sometimes even surgery. The authors report 3 cases of cystic renal cancer in 2 men and 1 woman, aged 87, 67 and 20 years, respectively. Three patients presented with the urological triad (haematuria, pain and lumbar mass). Ultrasonography suggested the diagnosis of cystic cancer in all 3 cases. Computed tomography was performed in 2 patients and more precisely confirmed the ultrasound findings. Selective arteriography, performed in one patient, confirmed the hypothesis of malignancy. Surgical exploration resulted in radical total nephrectomy. Histology confirmed the diagnosis of adenocarcinoma. The course was favourable in 2 cases after a follow-up of 4 years. One patient presented a local recurrence with pulmonary metastases 6 months after the operation. A review of the literature illustrates the diagnostic difficulties of this form of renal cancer.

  7. Acquired cystic kidney disease.

    PubMed

    Levine, E

    1996-09-01

    ACKD is characterized by the development of many fluid-filled renal cysts and sometimes neoplasms in the kidneys of individuals with chronic renal failure but without a history of hereditary cystic disease. The condition is seen mainly in dialysis patients, but often begins in patients with ESRD before dialysis is started. Most patients with ACKD are asymptomatic, but the disorder may be associated with such serious complications as retroperitoneal hemorrhage and metastatic renal cell carcinoma. The diagnosis of ACKD and its complications is best achieved by CT scanning, although US and MR imaging may be useful in evaluation, particularly in patients not treated with dialysis. Cyst hemorrhage is common in ACKD and may cause flank pain and hematuria. Hemorrhagic cysts may be recognized by their CT scan, sonographic, or MR imaging features. Hemorrhagic cysts may rupture into the perinephric space causing large perinephric hematomas. These can usually be treated-conservatively. Patients with ACKD, particularly those treated with dialysis, have an increased risk of renal cell carcinoma. Renal cell carcinoma may also develop in the native kidneys of renal transplant recipients with good graft function many years after transplantation. Annual imaging of the native kidneys of all dialysis patients or of transplant recipients for the development of carcinoma is not justified, however, because it has not been shown to have a significant effect on patient outcome. Screening may, however, be useful in selected dialysis patients with good general medical condition and who have known risk factors for renal cell carcinoma including prolonged dialysis, large kidneys, ACKD, and male gender. Screening of the native kidneys of transplant recipients may be performed when they are referred for US evaluation of the renal allograft.

  8. Smell in cystic fibrosis.

    PubMed

    Lindig, J; Steger, C; Beiersdorf, N; Michl, R; Beck, J F; Hummel, T; Mainz, J G

    2013-03-01

    In cystic fibrosis (CF), the most frequent life threatening inherited disease in Caucasians, sinonasal mucosa is regularly affected by defective mucociliary clearance. This facilitates pathogen colonization into CF airways and causes frequent symptoms of rhinosinusitis, including an impaired sense of smell. Despite probable effects on nutrition and overall health, CF-rhinosinusitis is little understood: CF-associated smelling deficiencies reported in literature vary between 12 and 71 %. The aim of this study was to assess olfactory and gustatory function in relation to sinonasal symptoms and sinonasal colonization, and lung function and nutrition. Thirty-five CF patients of different ages were compared to 35 age-matched healthy controls. Olfactory function was assessed by 'Sniffin'Sticks', gustatory qualities by "Taste-strips", and symptoms by sino-nasal outcome test 20 (SNOT-20). Normosmia was found in 62.8 % of healthy controls but only in 28.6 % of CF patients. In contrast the majority of CF patients exhibited a smell loss; almost 62.9 % of them were hyposmic, and 8.6 % functionally anosmic. Importantly, reduced olfactory function only affected odor thresholds, which were significantly increased in CF, not odor identification. This suggests that the olfactory dysfunction in CF results from the olfactory periphery due to either problems in conduction and/or a functional lesion due to the inflammatory process. SNOT-20 scores increased continuously from normosmic to hyposmic and anosmic CF patients (means 7.2/11.1/28.3 points). Neither sinonasal pathogen colonization, gender, pulmonary function, nor allergy or sinonasal surgery appeared to have significant effects on olfactory function and taste. Olfactory disorders are considerably more frequent in CF patients than in age-matched healthy controls. Assessing these parameters within CF-routine care should be considered because of their importance to nutrition and, thus, overall therapy outcome.

  9. [Historical compilation of cystic fibrosis].

    PubMed

    Navarro, Salvador

    2016-01-01

    Cystic fibrosis is the most common life-shortening recessively inherited disorder in the Caucasian population. The genetic mutation that most frequently provokes cystic fibrosis (ΔF508) appeared at least 53,000years ago. For many centuries, the disease was thought to be related to witchcraft and the "evil eye" and it was only in 1938 that Dorothy H. Andersen characterized this disorder and suspected its genetic origin. The present article reviews the pathological discoveries and diagnostic and therapeutic advances made in the last 75 years. The review ends with some considerations for the future.

  10. Newborn intraabdominal cystic lymphatic malformations.

    PubMed

    Lin, J I; Fisher, J; Caty, M G

    2000-08-01

    Cystic lymphatic malformations are rare causes of abdominal masses in the newborn. Also known as mesenteric, omental, or retroperitoneal cysts, they can present in a variety of ways including, intestinal obstruction, volvulus, nonspecific abdominal pain, intracystic hemorrhage, or as an asymptomatic abdominal mass. Abdominal ultrasound scan provides a definitive diagnosis in most suspected cases. Complete resection is possible in most patients except those with extensive retroperitoneal involvement. Recurrence is unusual when complete resection is accomplished. Because most case series with complete data suggest that these entities are lymphatic malformations, the authors suggest the more specific term, cystic lymphatic malformations, be used to describe these lesions.

  11. [Historical compilation of cystic fibrosis].

    PubMed

    Navarro, Salvador

    2016-01-01

    Cystic fibrosis is the most common life-shortening recessively inherited disorder in the Caucasian population. The genetic mutation that most frequently provokes cystic fibrosis (ΔF508) appeared at least 53,000years ago. For many centuries, the disease was thought to be related to witchcraft and the "evil eye" and it was only in 1938 that Dorothy H. Andersen characterized this disorder and suspected its genetic origin. The present article reviews the pathological discoveries and diagnostic and therapeutic advances made in the last 75 years. The review ends with some considerations for the future. PMID:26070393

  12. Mediastinal solitary fibrous tumor with right diaphragm invasion: report of a case.

    PubMed

    Xue, Xingyang; Chen, Jun; Ma, Wei; Zhu, Daxing; Zhang, Weiguo; Chen, Gang; Wei, Sen; Zhou, Qinghua

    2009-01-01

    Mediastinal solitary fibrous tumors (SFTs) are rarely found in adults and there are few reports describing primary mediastinal SFT invading the diaphragm. We report the case of a 47-year-old woman with a large right inferior mediastinal SFT. Magnetic resonance imaging showed the tumor invading the right lower lobe of the lung and the right hemidiaphragm, with displacement of the inferior vena cava (IVC) and right lobe of the liver. Angiogram showed IVC stenosis. To our knowledge, this is the first report of complete resection of the tumor combined with right lower lobectomy of the lung and partial resection and reconstruction of the right diaphragm with a Dacron flap.

  13. [Mediastinal lymph node carcinoma of unknown primary site; report of a case].

    PubMed

    Miura, K; Yoshizawa, K; Tamaki, M; Okumura, K; Furukita, Y

    2009-03-01

    A 44-year-old woman was admitted to our hospital because of mediastinal mass. Serum levels of carcinoembryonic antigen (CEA) were found to be elevated. No preoperative examination could detect the primary lesion. The tumor was resected through right thoracotomy. Histological examination revealed poorly differentiated adenocarcinoma. She was diagonosed as metastatic mediastinal lymph node carcinoma of unknown primary site. She received radiotherapy and chemotherapy and is desease free 29 months after operation. Good results may be obtained by multimodality therapies for cancer in mediastinal lymph node of unknown primary site. PMID:19280962

  14. Anesthetic management of a large mediastinal mass for tracheal stent placement.

    PubMed

    Rajagopalan, Suman; Harbott, Mark; Ortiz, Jaime; Bandi, Venkata

    2016-01-01

    The anesthetic management of patients with large mediastinal masses can be complicated due to the pressure effects of the mass on the airway or major vessels. We present the successful anesthetic management of a 64-year-old female with a large mediastinal mass that encroached on the great vessels and compressed the trachea. A tracheal stent was placed to relieve the tracheal compression under general anesthesia. Spontaneous ventilation was maintained during the perioperative period with the use of a classic laryngeal mask airway. We discuss the utility of laryngeal mask airway for anesthetic management of tracheal stenting in patients with mediastinal masses.

  15. Preoperative Embolization of a Posterior Mediastinal Lipid-Poor Angiolipoma Mimicking a Paravertebral Nerve Sheath Tumor.

    PubMed

    Gorospe, Luis; García-Poza, Javier; González-Gordaliza, María Cristina; Cabañero-Sánchez, Alberto; Muñoz-Molina, Gemma María; Saldaña-Garrido, David

    2015-08-01

    Mediastinal angiolipomas are extremely rare tumors within the thorax, and only 6 cases have been previously reported in the literature. We describe the case of a lipid-poor angiolipoma within the posterior mediastinum of a 63-year-old man who complained of chest pain. Interestingly, initial imaging of the posterior mediastinal mass of our patient suggested a nerve sheath tumor. A specimen from a percutaneous transthoracic core needle biopsy confirmed an angiolipoma. We decided to preoperatively embolize the posterior mediastinal mass to reduce intraoperative bleeding and to facilitate the excision of the tumor.

  16. Cystic pineocytoma--case report.

    PubMed

    Momozaki, N; Ikezaki, K; Abe, M; Fukui, M; Fujii, K; Kishikawa, T

    1992-03-01

    Pineocytoma and pineoblastoma, originating from pineal parenchyma, are rare and usually solid. An unusual case of totally cystic pineocytoma in a 37-year-old female is reported. The tumor showed neuronal differentiation and had a good outcome. Prominent calcification associated with pineocytoma and pineoblastoma is an useful finding to differentiate these from benign pineal cysts. PMID:1377802

  17. Cystic mesothelioma of the peritoneum.

    PubMed

    O'Neil, J D; Ros, P R; Storm, B L; Buck, J L; Wilkinson, E J

    1989-02-01

    Cystic mesothelioma (CM) of the peritoneum is a rare, benign neoplasm that occurs predominantly in women and tends to recur locally. It has received little attention (to our knowledge, a single case report) in the radiology literature. Five cases of CM are presented. Computed tomography (CT) was performed in three cases, ultrasound (US) in four cases, and magnetic resonance (MR) imaging in one case. Twenty-eight cases reported in the literature are reviewed for comparison. CM shows a clear predilection for the surfaces of the pelvic viscera but is seen in other areas of the peritoneum and retroperitoneum. The neoplasm was intraperitoneal in three cases and primarily retroperitoneal in the other two cases. In all CT and US studies performed, a single large, multilocular cystic mass was demonstrated. MR imaging, performed in one case, showed that the lesion had signal characteristics typical of a watery collection low in solute concentration. It is concluded that CM of the peritoneum has a nonspecific multilocular cystic appearance on images, which does not permit it to be differentiated from other cystic lesions.

  18. Video-assisted thoracic surgery compared with posterolateral thoracotomy for mediastinal bronchogenic cysts in adult patients

    PubMed Central

    Guo, Chenglin; Mei, Jiandong; Liu, Chengwu; Deng, Senyi; Pu, Qiang; Lin, Feng

    2016-01-01

    Background Mediastinal bronchogenic cyst (MBC) is the most common primary cystic lesion of the mediastinum. This study aimed to investigate the efficacy and safety of video-assisted thoracic surgery (VATS) compared with posterolateral thoracotomy (PLT) for the treatment of MBCs in a large series. Methods Patients with MBCs who underwent surgical resection between August 2005 and December 2015 were identified from the electronic database of the Department of Thoracic Surgery, West China Hospital. The patient demographic characteristics, intraoperative findings, postoperative outcomes and follow-up information were reviewed and analyzed. Results A total of 99 patients underwent cystectomy were enrolled for the present study. Of those patients, 65 underwent VATS cystectomy (VATS group) and 34 underwent PLT cystectomy (PLT group) during the same period. The VATS group had shorter operative time than the PLT group (108.77±47.81 vs. 144.62±55.16, P=0.001), less intraoperative blood loss (median 20 vs. 100 mL, P<0.001), and less pleural drainage of the first three days after surgery (median 240 vs. 400 mL, P=0.002). In addition, the length of postoperative hospital stay and duration of chest drainage for the VATS group was also shorter than those of the PLT group (4.94±2.01 vs. 8.64±5.52 days, P=0.001; 2.52±1.29 vs. 3.71±1.55 days, P<0.001, respectively). No statistical significance was revealed among the two groups with regard to the maximum diameter of the cysts, pleural atresia, incomplete resection, surgery-related complications, duration of intensive care unit stay, and postoperative complications. Conclusions Both VATS and PLT are reliable approaches for the surgical resection of MBCs. The VATS approach is superior to PLT with shorter operative time, shorter duration of chest drainage, shorter postoperative hospital stay, less intraoperative blood loss, and less pleural drainage of the first three days after surgery. We conclude that VATS should be the

  19. Mediastinitis by Actinomyces meyeri after oesophageal stent placement.

    PubMed

    Branquinho, Diogo Ferreira; Andrade, Daniel Ramos; Almeida, Nuno; Sofia, Carlos

    2014-06-05

    Actinomyces meyeri is a Gram-positive anaerobic forming bacterium of the genus Actinomyces, part of the oral cavity's flora, and its classification remains an unresolved issue. It is an extremely rare cause of disease, occurring in middle-aged immunocompetent patients and frequently misdiagnosed as malignancy or lung abscess. A 56-year-old man diagnosed with oesophageal squamous cell carcinoma had an endoscopically placed stent to palliate his dysphagia. Two weeks later he presented with thoracalgia and fever, interpreted as a common lung infection. Owing to lack of improvement, additional examinations were undertaken revealing mediastinum involvement. Unlike the good prognosis usually associated with this infection, the patient eventually died, reflecting the aggressive nature of his underlying condition. To our knowledge, this is the first report of mediastinitis by A. meyeri, supporting the described propensity of this agent to disseminate, particularly to the thoracic cavity, although probably in this case with an iatrogenic contribution.

  20. [Mediastinal tuberculous adenopathies in Djibouti. Report of 159 cases].

    PubMed

    Auregan, G; Ali, B; Chakib, S; Etienne, B; Migueres, J

    1990-01-01

    One hundred and fifty-nine cases of confirmed mediastinal tuberculous adenopathy were analysed retrospectively. This series, which is the first reported in French-speaking Africa, shows that the disease is not uncommon there, and not restricted to black Africans living in Europe. The clinical symptoms are not specific. Radiography is more interesting, and the extreme scarcity of bacillys-containing expectoration encourages endoscopy. The finding of a fistula at fibroscopy confirms the diagnosis. This easy examination is particularly useful since M. tuberculosis can be grown in cultures from ground bronchial fistula biopsies in 86% of the cases. Associated tuberculous lesions in other sites are very frequent (45%), as is extension of the disease from lymph nodes to lung tissue (79%). This clinical form of tuberculosis can be treated with modern short course chemotherapy which is remarkably effective. PMID:2075364

  1. Propolis-induced descending necrotizing mediastinitis and aspiration pneumonia.

    PubMed

    Wu, Jeng-Yuan; Hsu, Nan-Yung

    2013-04-01

    Propolis is a resinous substance collected by bees as a sealant for their hives. It is also used in traditional medicine as an antioxidant and antiinflammatory agent to treat ulcers, superficial burns, and microbial diseases. In this report, a 40-year-old woman who took liquid propolis for relief of her common cold experienced severe sore throat, dysphagia, and easy choking followed by fever and chills. Descending necrotizing mediastinitis and concomitant aspiration pneumonia were evident on the image studies. We performed video-assisted thoracoscopic surgery to achieve immediate and adequate drainage, and the patient resumed normal deglutition 2 months later. Early diagnosis and prompt video-assisted thoracoscopic surgery intervention are paramount to manage this life-threatening situation.

  2. [Kodamaea ohmeri infection in a newborn with a mediastinal mass].

    PubMed

    Alvarado Socarras, Jorge; Rojas Torres, Juan P; Vargas Soler, José A; Guerrero, Carlos

    2016-10-01

    Invasive fungal infections are a considerable cause of morbidity, mortality, increased hospital stay durations, and high health care costs, during neonatal period. In this period, the premature infants are the most affected. Candida species are the leading cause of invasive fungal infections. The majority of neonatal Candida infections are caused by C. albicans, C. parapsilosis, C. glabrata and C. tropicalis, although other fungus species are being reported. One such emerging pathogen is K. ohmeri. This organism has been reported as a pathogen in the neonatal period, principally in premature infants. The risk factors associated with fungal infection are central line, immunosuppression, prolonged hospital stay, endotracheal intubation and exposure to antibiotics. We present a term baby with a mediastinal mass, who required several procedures, as pericardiocentesis, central catheters, mechanical ventilation, antibiotics. During his evolution, he presented infection by K. ohmeri. The baby was treated with amphotericin B, with satisfactory clinical course. PMID:27606654

  3. [Kodamaea ohmeri infection in a newborn with a mediastinal mass].

    PubMed

    Alvarado Socarras, Jorge; Rojas Torres, Juan P; Vargas Soler, José A; Guerrero, Carlos

    2016-10-01

    Invasive fungal infections are a considerable cause of morbidity, mortality, increased hospital stay durations, and high health care costs, during neonatal period. In this period, the premature infants are the most affected. Candida species are the leading cause of invasive fungal infections. The majority of neonatal Candida infections are caused by C. albicans, C. parapsilosis, C. glabrata and C. tropicalis, although other fungus species are being reported. One such emerging pathogen is K. ohmeri. This organism has been reported as a pathogen in the neonatal period, principally in premature infants. The risk factors associated with fungal infection are central line, immunosuppression, prolonged hospital stay, endotracheal intubation and exposure to antibiotics. We present a term baby with a mediastinal mass, who required several procedures, as pericardiocentesis, central catheters, mechanical ventilation, antibiotics. During his evolution, he presented infection by K. ohmeri. The baby was treated with amphotericin B, with satisfactory clinical course.

  4. [Therapeutic update in cystic fibrosis].

    PubMed

    Durupt, S; Nove Josserand, R; Durieu, I

    2014-06-01

    We present the recent therapeutic advances in the cystic fibrosis care. It concerns improvements in symptomatic treatment with the development of dry powder inhaled antibiotics that improved quality of life, and innovative treatments namely the modulators of the cystic fibrosis transmembrane protein conductance regulator (CFTR), molecules which act specifically at the level of the defective mechanisms implied in the disease. The life expectancy of cystic fibrosis patients born after 2000, is estimated now to be about 50 years. This improvement of survival was obtained with the organization of the care within the specialized centers for cystic fibrosis (Centre de ressource et de compétences de la mucoviscidose) and remains still based on heavy symptomatic treatments. Dry powder inhaled antibiotics constitute a significant time saving for patients to whom all the care can achieve two hours daily. Since 2012, the modulators of CFTR, molecules allowing a pharmacological approach targeted according to the type of the mutations, allows a more specific approach of the disease. Ivacaftor (Kalydeco(®)) which potentialises the function of the CFTR protein expressed on the cellular surface is now available for patients with the G551D mutation. Lumacaftor is going to be tested in association with ivacaftor in patients with the F508del mutation, that is present in at least 75% of the patients. The ataluren which allows the production of a functional protein CFTR in patients with a no sense mutation is the third representing of this new therapeutic class. We presently have numerous symptomatic treatments for the cystic fibrosis care. The development of CFTR modulators, today available to a restricted number of patients treated with ivacaftor represents a very promising therapeutic avenue. It will represent probably the first step to a personalized treatment according to CFTR genotype.

  5. [Mediastinal seminoma. Is it an entity to be treated differently?].

    PubMed

    Ruffié, P; Droz, J P

    1992-01-01

    The evolution of the therapeutic concepts of non seminomatous tumours (T) has chemotherapy as the preponderant treatment, and has also led to a different approach for mediastinal seminomas. In effect the traditional attitude for as complete an excision as possible followed by radiotherapy is in the process of changing. Certainly in 20% of cases a complete excision is possible. Post operative radiotherapy (30-50 grays) does not seem to change the survival and may be questioned in the management of invasive T. The radical debulking surgery of varying degrees no longer has a place. The standard treatment of inoperable T has remained for some time radiotherapy (35-40 grays). The survival at 5 years for these patients is around 60%, very close to the stage IIB seminoma of the testicles. If the seminoma seems to be radiosensitive it is also chemo-sensitive with an RC less than 80% in advance seminomas and of 90% in mediastinal seminomas. Thus there appears to be a contradictory debate; in the absence of precise staging of "thymic type" tumour and of randomised trials made difficult by the small number of cases one can envisage the following option: In the case of an inoperable T radiotherapy alone, excepting in the very bulky forms (diameter greater than 50% of the chest) or metastases where primary chemotherapy (CT) (4 cycles of VP16-platine) has a place: The treatment of the residual mass after CT depends on the size of the latter: less than 3 or equal to 3 cm by complementary treatment and greater than 3 cm by secondary surgery and if the seminoma is active complementary.(ABSTRACT TRUNCATED AT 250 WORDS)

  6. Tracheal varices caused by mediastinal compression of a large intrathoracic goiter: report of a case

    PubMed Central

    LUCCHINI, R.; SANTOPRETE, S.; TRIOLA, R.; POLISTENA, A.; MONACELLI, M.; AVENIA, S.; SANGUINETTI, A.; PUMA, F.; AVENIA, N.

    2015-01-01

    Introduction Tracheal varices are a rare condition but they can be an important source of massive or recurrent haemoptysis. Usually they are related to increased pressure in the pulmonary veins. Mediastinal goiter is often associated to compressive effects on the surrounding structures, including mediastinal vessels with potential superior vena cava syndrome. Case report We describe a case, not previously reported in literature, of mediastinal goiter with hemoptysis as first clinical manifestation. Bleeding was attributed to a superior vena cava syndrome associated to a tracheal fragile mucosa with an easily bleeding intramural nodule which was diagnosed as tracheal varices after total thyroidectomy. The nodule in fact disappeared together with the venous hypertensive signs after venous decompression of the mediastinum. Conclusions Compressive symptoms including tracheal varices, related to mediastinal goiter, can be treated surgically by total thyroidectomy via cervicotomy and when required with associated median sternotomy. PMID:25827666

  7. A rare presentation of a huge mature mediastinal teratoma with right lung cavitation

    PubMed Central

    Pattnaik, Manoj Kumar; Majhi, Paresh Chandra; Nayak, Anil Kumar; Senapati, Debadutta

    2014-01-01

    A school-going child presented with fever and productive cough for a short period, which after laboratory and radiological survey was diagnosed as mediastinal teratoma with lung cavitation. Preoperatively the exact cause of lung pathology could not be established, although more common causes prevalent in this zone such as, tuberculosis and lung abscess were excluded. Surgical treatment was planned and excision of the mediastinal mass with segmentectomy of the right-upper lobe carried out through median sternotomy. Mature teratoma is the most common primary germ cell tumour of the mediastinum accounting for 60–70% of all mediastinal germ cell tumours. On very rare occasions it involves the adjacent lung, usually the left lung, producing secondary changes inviting suspicion of a separate lung pathology. Here we present a rare case of a huge mature mediastinal teratoma with secondary right lung cavitation. PMID:24842359

  8. The history of the management of sternal osteomyelitis and mediastinitis - from Hippocrates until today.

    PubMed

    Ennker, Ina Carolin; Ennker, Jürgen C

    2014-01-01

    Even during the time of Hippocrates, Galen and their colleagues recognized mediastinal affections. However, they were not considered with the surgical treatment. First progress in the treatment options of this severe disease, still denoted as 'terra incognita', over to today's gold standard are pictured. The mediastinitis-registry which was founded by the German Society for Thoracic and Cardiovascular Surgery (DGTHG) in 2011 and the recent establishment of the study group to adopt a guideline 'diagnosis and therapy of postoperative mediastinitis/sternal osteomyelitis following cardiac surgery' are attempts to a standardization of the treatment. Substantial advancement in the treatment of postoperative mediastinitis could be achieved in the past. The mortality dropped as low as less than 10%. With these implementations more benefit for the patients' outcome can be expected. PMID:26504718

  9. Ultrasound-guided biopsy as a diagnostic aid in three horses with a cranial mediastinal lymphosarcoma.

    PubMed

    De Clercq, D; van Loon, G; Lefère, L; Deprez, P

    2004-06-01

    An ultrasound examination of the thorax of three horses which were performing poorly or had mild signs of colic showed that they had a cranial mediastinal mass and a pleural effusion. A cytological examination of the pleural fluid showed that it did not contain neoplastic cells. A histological examination of an ultrasound-guided core biopsy of the cranial mediastinal mass showed that in each of the three horses it was a lymphosarcoma. PMID:15214516

  10. Disseminated cryptococcosis presenting as mediastinal and hilar lymphadenopathy in an immunocompetent patient

    PubMed Central

    Oki, Masahide; Saka, Hideo; Kajikawa, Shigehisa; Murakami, Ayuka; Ishida, Akane

    2016-01-01

    Abstract We herein report a rare case of disseminated cryptococcosis presenting as mediastinal and hilar lymphadenopathy in a young immunocompetent man. A previously healthy 26‐year‐old man presented with persistent headache and nonproductive cough. Chest computed tomography indicated mediastinal and hilar lymphadenopathy. Cryptococcal lymphadenitis and meningitis was confirmed by endobronchial ultrasound‐guided transbronchial needle aspiration and central spinal fluid examination, respectively. He received liposomal amphotericin B and flucytosine followed by fluconazole and finally improved. PMID:27512567

  11. Cystic fibrosis from the gastroenterologist's perspective.

    PubMed

    Ooi, Chee Y; Durie, Peter R

    2016-03-01

    Cystic fibrosis is a life-limiting, recessive disease caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. Increased survival outcomes and the multisystem nature of the disease, including the involvement of hepatobiliary and gastrointestinal tracts, now require the need for more extensive knowledge and expertise in cystic fibrosis among gastroenterologists. Manifestations are either a direct consequence of the primary defect in cystic fibrosis or a secondary complication of the disease or therapy. Adult patients with cystic fibrosis also have an increased risk of malignancy in the gastrointestinal and pancreatico-biliary tracts compared with the general population. Novel treatments that target the basic defects in the CFTR protein have emerged, but to date not much is known about their effects on the gastrointestinal and hepatobiliary systems. The introduction of such therapies has provided new opportunities for the application of intestinal endpoints in clinical trials and the understanding of underlying disease mechanisms that affect the gut in cystic fibrosis.

  12. Biomarkers in Paediatric Cystic Fibrosis Lung Disease.

    PubMed

    Ramsey, Kathryn A; Schultz, André; Stick, Stephen M

    2015-09-01

    Biomarkers in cystic fibrosis are used i. for the measurement of cystic fibrosis transmembrane regulator function in order to diagnose cystic fibrosis, and ii. to assess aspects of lung disease severity (e.g. inflammation, infection). Effective biomarkers can aid disease monitoring and contribute to the development of new therapies. The tests of cystic fibrosis transmembrane regulator function each have unique strengths and weaknesses, and biomarkers of inflammation, infection and tissue destruction have the potential to enhance the management of cystic fibrosis through the early detection of disease processes. The development of biomarkers of cystic fibrosis lung disease, in particular airway inflammation and infection, is influenced by the challenges of obtaining relevant samples from infants and children for whom early detection and treatment of disease might have the greatest long term benefits.

  13. Pharmacogenetics of cystic fibrosis treatment.

    PubMed

    Carter, Suzanne C; McKone, Edward F

    2016-08-01

    Cystic fibrosis (CF) is genetic autosomal recessive disease caused by reduced or absent function of CFTR protein. Treatments for patients with CF have primarily focused on the downstream end-organ consequences of defective CFTR. Since the discovery of the CFTR gene that causes CF in 1989 there have been tremendous advances in our understanding of the genetics and pathophysiology of CF. This has recently led to the development of new CFTR mutation-specific targeted therapies for select patients with CF. This review will discuss the characteristics of the CFTR gene, the CFTR mutations that cause CF and the new mutation specific pharmacological treatments including gene therapy that are contributing to the dawning of a new era in cystic fibrosis care. PMID:27490265

  14. Non-cystic fibrosis bronchiectasis.

    PubMed

    Al Subie, Haya; Fitzgerald, Dominic A

    2012-05-01

    Non-cystic fibrosis (CF) bronchiectasis is said to be a declining problem in developed countries, although its prevalence in indigenous communities in Australia and New Zealand is among the highest reported in the world. Early childhood pneumonia and underlying conditions such as immunodeficiency and primary ciliary dyskinesia need to be considered in the aetiology. A high-resolution computerised tomography scan is the key investigation in children with a chronic wet cough in whom bronchiectasis is suspected. Regardless of the cause, the treatment of bronchiectasis is centred upon facilitating the clearance of airway secretions and the treatment of pulmonary exacerbations. This review aims to provide general paediatricians with an update on the presenting features, investigation and management of non-cystic fibrosis bronchiectasis. PMID:21040075

  15. Adenoid cystic carcinoma of trachea.

    PubMed

    Vigg, Ajit; Mantri, Sumant; Vigg, Avanti; Vigg, Arul

    2004-01-01

    A 20-year-old male, presented with cough, haemoptysis, breathlessness and wheezing for the past one month. Contrast enhanced computerised tomographic (CECT), scan of chest and fibreoptic bronchoscopy revealed an endotracheal mass that on histopathological examination showed adenoid cystic carcinoma of trachea. Magnetic resonance imaging (MRI) scan of chest confirmed involvement of adjacent prevertebral, para-oesophageal and subcarinal lymph nodes rendering the tumour inoperable. PMID:15515830

  16. Non-cystic fibrosis bronchiectasis.

    PubMed

    McShane, Pamela J; Naureckas, Edward T; Tino, Gregory; Strek, Mary E

    2013-09-15

    There is renewed interest in non-cystic fibrosis bronchiectasis, which is a cause of significant morbidity in adults and can be diagnosed by high-resolution chest computed tomography scan. No longer mainly a complication after pulmonary infection with Mycobacterium tuberculosis, diverse disease processes and mechanisms have been demonstrated to result in the chronic cough, purulent sputum production, and airway dilation that characterize this disease. Improved understanding of the role of mucus stasis in causing bacterial colonization has led to increased emphasis on the use of therapies that enhance airway clearance. Inhalational antibiotics reduce the bacterial burden associated with a worse outcome. Low-dose, chronic macrolide therapy has been shown to decrease exacerbation frequency and airway inflammation. For the first time, a number of therapies for non-cystic fibrosis bronchiectasis are undergoing testing in clinical research trials designed specifically for this population. This concise clinical review focuses on the major etiologies, diagnostic testing, microbiology, and management of patients with adult non-cystic fibrosis bronchiectasis. Systematic evaluation identifies a specific cause in the majority of patients and may affect subsequent treatment. We outline current therapies and review the data that support their use. PMID:23898922

  17. Echocardiographic Manifestation of Esophagitis Mimicking a Posterior Mediastinal Mass

    PubMed Central

    Swarup, Supreeya; Kantamneni, Sowmya; Kabir, Sarah; Zeltser, Roman; Makaryus, Amgad N

    2014-01-01

    Incidental extracardiac findings (ECFs) are commonly noted on cardiac imaging. The majority of the ECFs are noticed on computed tomography (CT), cardiac magnetic resonance scanning, and myocardial perfusion imaging. Although transthoracic echocardiography (TTE) is a widely used cardiac modality, there is scarcity of data describing ECF on TTE. ECFs have the potential to alter patient management. We present a rare case of a cystic mass seen in the posterior mediastinum on TTE, which led to further evaluation and diagnosis of esophagitis with ulceration. PMID:25780343

  18. [Ectopic mediastinal parathyroid tumor resected by video-assisted thoracic surgery with intraoperative methylene blue infusion; report of a case].

    PubMed

    Okagawa, Takehiko; Hiramatsu, Yoshinori

    2014-03-01

    We report a surgical case of ectopic mediastinal parathyroid tumor resected by video-assisted thoracic surgery with intraoperative methylene blue infusion. It is often difficult to detect ectopic mediastinal parathyroid tumor during the operation because the tumor is soft, small and buried under mediastinal tissue. After methylene blue 4 mg/kg intravenously administration, the tumor was gradually dyed blue and easily detected and resected by video-assisted thoracic surgery. It is useful of methylene blue for detection of ectopic mediastinal parathyroid tumor.

  19. Mediastinal radiation and adverse outcomes after heart transplantation.

    PubMed

    Uriel, Nir; Vainrib, Alan; Jorde, Ulrich P; Cotarlan, Vlad; Farr, Maryjane; Cheema, Faisal H; Naka, Yoshifuma; Mancini, Donna; Colombo, Paolo C

    2010-03-01

    Orthotopic heart transplantation (OHT) may represent the only treatment option for patients with end-stage cardiovascular disease due to mediastinal radiation therapy (MRT). The primary aim of this study was to evaluate the safety and efficacy of OHT in this patient population. We conducted a retrospective, single-center cohort study of patients with MRT-associated cardiovascular disease who underwent OHT between January 1987 and September 2008. Nine patients (3 men), aged 46 +/- 11 years at the time of their OHT, were identified. Time from MRT to OHT was 26 +/- 11 years. Lymphoma was the indication for MRT in all patients. Five patients had non-ischemic dilated cardiomyopathy, 2 had ischemic cardiomyopathy and 2 had constrictive pericarditis. Three patients expired in the peri-operative period, whereas another patient died 3 years post-transplant from lung carcinoma. Two additional patients developed a secondary malignancy post-transplant. Five patients are still alive at a mean follow-up of 10 +/- 8 years. Early survival rate is poor in patients who undergo OHT for MRT-associated end-stage cardiovascular disease. In addition, long-term follow-up shows an elevated incidence of malignancies. Our results raise concern about the safety and efficacy of performing OHT in patients with MRT-associated cardiovascular disease.

  20. POMB/ACE chemotherapy for mediastinal germ cell tumours.

    PubMed

    Bower, M; Brock, C; Holden, L; Nelstrop, A; Makey, A R; Rustin, G J; Newlands, E S

    1997-05-01

    Mediastinal germ cell tumours (MGCT) are rare and most published series reflect the experiences of individual institutions over many years. Since 1979, we have treated 16 men (12 non-seminomatous germ cell tumours and 4 seminomas) with newly diagnosed primary MGCT with POMB/ACE chemotherapy and elective surgical resection of residual masses. This approach yielded complete remissions in 15/16 (94%) patients. The median follow-up was 6.0 years and no relapses occurred more than 2 years after treatment. The 5 year overall survival in the non-seminomatous germ cell tumours (NSGCT) is 73% (95% confidence interval 43-90%). One patient with NSGCT developed drug-resistant disease and died without achieving remission and 2 patients died of relapsed disease. In addition, 4 patients with bulky and/or metastatic seminoma were treated with POMB/ACE. One died of treatment-related neutropenic sepsis in complete remission and one died of relapsed disease. Finally, 4 patients (2 NSGCT and 2 seminomas) referred at relapse were treated with POMB/ACE and one was successfully salvaged. The combination of POMB/ACE chemotherapy and surgery is effective management for MGCT producing high long-term survival rates.

  1. A combination therapy for cystic fibrosis.

    PubMed

    Brodsky, Jeffrey L; Frizzell, Raymond A

    2015-09-24

    The most prevalent form of cystic fibrosis arises from an amino acid deletion in the cystic fibrosis transmembrane conductance regulator, CFTR. A recently approved treatment for individuals homozygous for this mutation combines a chemical corrector, which helps CFTR fold, and a potentiator that increases CFTR channel activity. PMID:26406363

  2. A combination therapy for cystic fibrosis.

    PubMed

    Brodsky, Jeffrey L; Frizzell, Raymond A

    2015-09-24

    The most prevalent form of cystic fibrosis arises from an amino acid deletion in the cystic fibrosis transmembrane conductance regulator, CFTR. A recently approved treatment for individuals homozygous for this mutation combines a chemical corrector, which helps CFTR fold, and a potentiator that increases CFTR channel activity.

  3. Psychosocial impact of cystic fibrosis in adolescence.

    PubMed

    Harrop, Michele

    2007-12-01

    Over 8,000 children, young people and adults in the UK are affected by cystic fibrosis and although no cure exists, comprehensive therapy started early and administered consistently delays disease progression. This article explores three aspects of the psychosocial effects of cystic fibrosis on the adolescent/young adult: the effect on the family, the effect on relationships and adherence to treatment. Much of the early research on the psychosocial impact of cystic fibrosis on the adolescent and the family presented a dismal picture of dysfunction. More recent studies indicate that cystic fibrosis patients generally lead active, age-appropriate social lives, that good information and support can reduce negative effects on families and that treatment regimes continue to cause difficulties for young people. Further research is needed into the psychosocial impact of cystic fibrosis on the increasing numbers of young people and adults with the disease.

  4. Endocrine Disorders in Cystic Fibrosis.

    PubMed

    Blackman, Scott M; Tangpricha, Vin

    2016-08-01

    Cystic fibrosis is frequently complicated by endocrine disorders. Diabetes can be expected to affect most with CF and pancreatic insufficiency and varies widely in age of onset, but early identification and treatment improve morbidity and mortality. Short stature can be exacerbated by relative delay of puberty and by use of inhaled corticosteroids. Bone disease in CF causes fragility fractures and should be assessed by monitoring bone mineral density and optimizing vitamin D status. Detecting and managing endocrine complications in CF can reduce morbidity and mortality in CF. These complications can be expected to become more common as the CF population ages. PMID:27469183

  5. An unusual retroperitoneal cystic tumor.

    PubMed

    Salinas Sánchez, A S; Lorenzo Romero, J G; Segura Martín, M; Hernández Millán, I R; Pastor Guzmán, J M; Virseda Rodríguez, J A

    2000-01-01

    This report describes a case of textiloma (term given to an inflammatory swelling caused by a retained textile foreign body) in a 60-year-old patient who had undergone surgery for a perforated gastroduodenal ulcer 17 years earlier. On examination the presence of a large mass was detected on the left hypochondria. A CT scan confirmed a cystic tumor with images suggesting detritus or necrosis in its lower part, as well as calcifications. Transperitoneal midline laparotomy was performed with extension by thoracophrenolaparotomy. Splenectomy was necessary. Macroscopic examination showed a spherical mass. After opening the tumor, retained surgical gauze was found. The histopathological diagnosis was granulomatosis reaction to a foreign body.

  6. Fulminant mediastinitis due to extended-spectrum beta-lactamase-producing Klebsiella pneumoniae: atypical presentation and spreading following cardiac surgery†

    PubMed Central

    Valenzuela, Horacio; Carrascal, Yolanda; Maroto, Laura; Arce, Nuria

    2013-01-01

    Mediastinitis due to Klebsiella pneumoniae, related to thoracic wall contamination after cardiac surgery, has rarely been described. We aim to report a case of fulminant mediastinitis due to extended-spectrum beta-lactamase-producing K. pneumoniae, secondary to a disseminated concomitant pulmonary infection. The patient remained pauci-symptomatic until clinical manifestations of sepsis acutely appeared. PMID:23416348

  7. Molecular characterization of primary mediastinal B cell lymphoma.

    PubMed

    Tsang, P; Cesarman, E; Chadburn, A; Liu, Y F; Knowles, D M

    1996-06-01

    Primary mediastinal B cell lymphoma (PMBL) is a diffuse large B cell lymphoma (DLCL) postulated to arise from noncirculating thymic B lymphocytes. Because of its distinctive clinical and morphological features and putative unique cellular origin, PMBL is generally considered a distinct clinicopathological entity. Little is known, however, about the molecular characteristics of PMBL. Therefore, we analyzed 16 PMBLs for molecular alterations involving the bcl-1, bcl-2, bcl-6, c-myc, H-ras, K-ras, N-ras, and p53 genes and for Epstein-Barr virus infection, which are commonly involved in lymphoid neoplasia. Employing a combination of Southern blotting and/or polymerase chain reaction and single-strand conformation polymorphism assays, we detected genetic alterations in 7 of the 16 (44%) PMBLs. Whereas the bcl-6 gene is rearranged in up to 45% of DLCLs, rearrangement of the bcl-6 gene was detected in only 1 of these 16 (6%) PMBLS. Point mutations of the 5' noncoding region of the c-myc gene were demonstrated in 3 other cases (19%), although c-myc gene rearrangements were not seen by Southern blotting. Missense point mutations of the p53 gene were identified in 3 additional PMBLs (19%). Alterations of the bcl-1, bcl-2, or ras genes and evidence of Epstein-Barr virus infection were not observed. In conclusion, a variety of molecular lesions occur in PMBLs and may be involved in their pathogenesis. This molecular genetic pattern bears little resemblance to that known for other B cell malignancies, including DLCL. In particular, the infrequent occurrence of bcl-6 gene rearrangement in PMBLs distinguishes them from other DLCLs of B cell origin, suggesting that PMBLs do not represent a distinct subtype of DLCL. PMID:8669486

  8. Molecular characterization of primary mediastinal B cell lymphoma.

    PubMed Central

    Tsang, P.; Cesarman, E.; Chadburn, A.; Liu, Y. F.; Knowles, D. M.

    1996-01-01

    Primary mediastinal B cell lymphoma (PMBL) is a diffuse large B cell lymphoma (DLCL) postulated to arise from noncirculating thymic B lymphocytes. Because of its distinctive clinical and morphological features and putative unique cellular origin, PMBL is generally considered a distinct clinicopathological entity. Little is known, however, about the molecular characteristics of PMBL. Therefore, we analyzed 16 PMBLs for molecular alterations involving the bcl-1, bcl-2, bcl-6, c-myc, H-ras, K-ras, N-ras, and p53 genes and for Epstein-Barr virus infection, which are commonly involved in lymphoid neoplasia. Employing a combination of Southern blotting and/or polymerase chain reaction and single-strand conformation polymorphism assays, we detected genetic alterations in 7 of the 16 (44%) PMBLs. Whereas the bcl-6 gene is rearranged in up to 45% of DLCLs, rearrangement of the bcl-6 gene was detected in only 1 of these 16 (6%) PMBLS. Point mutations of the 5' noncoding region of the c-myc gene were demonstrated in 3 other cases (19%), although c-myc gene rearrangements were not seen by Southern blotting. Missense point mutations of the p53 gene were identified in 3 additional PMBLs (19%). Alterations of the bcl-1, bcl-2, or ras genes and evidence of Epstein-Barr virus infection were not observed. In conclusion, a variety of molecular lesions occur in PMBLs and may be involved in their pathogenesis. This molecular genetic pattern bears little resemblance to that known for other B cell malignancies, including DLCL. In particular, the infrequent occurrence of bcl-6 gene rearrangement in PMBLs distinguishes them from other DLCLs of B cell origin, suggesting that PMBLs do not represent a distinct subtype of DLCL. Images Figure 1 Figure 2 Figure 3 Figure 4 PMID:8669486

  9. Mediastinal Abscess Following Endobronchial Ultrasound Transbronchial Needle Aspiration in a Patient With Sarcoidosis.

    PubMed

    McGovern Murphy, Frederic; Grondin-Beaudoin, Brian; Poulin, Yannick; Boileau, Robert; Dumoulin, Elaine

    2015-10-01

    Endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) is a minimally invasive procedure with a low rate of complications. It is used in the diagnosis of malignant and benign disease such as sarcoidosis. We report a case a 42-year-old man who had undergone EBUS-TBNA for diagnosis of mediastinal and hilar lymph node enlargement. Sarcoidosis was diagnosed on cytologic examination. Three weeks after the procedure, he developed a mediastinal abscess secondary to EBUS-TBNA. Sarcoidosis may be a risk factor for mediastinal infection complication. A local immune defect related to sarcoidosis may explain this risk. Our case underlines the importance of considering and recognizing this complication, and its possibility should be taken into account when undertaking the procedure for benign disease.

  10. Sonographic findings in horses with mediastinal lymphosarcoma: 13 cases (1985-1992).

    PubMed

    Garber, J L; Reef, V B; Reimer, J M

    1994-11-15

    Sonographic findings correlated with necropsy findings in 8 of 9 horses in which the cranial portion of the mediastinum was evaluated by use of both methods. Cranial mediastinal masses were imaged as multilobular and homogeneously hypoechoic; a complex echogenic pattern was observed with necrosis within the mass. Pleural effusion was a common finding in horses with mediastinal lymphosarcoma. Cytologic evaluation of pleural fluid samples was useful in diagnosis of lymphosarcoma in 10 of 12 horses that had thoracentesis. Sonographic examination of the thorax and cranial portion of the mediastinum can aid in the diagnosis of mediastinal lymphosarcoma in horses. Such examination should be performed in horses with clinical signs of cranial vena cava obstruction in which pleural effusion is detected, or when thoracic lymphosarcoma is suspected. PMID:7698925

  11. From cytology to histology: diagnosis of a relapsed mediastinal lymphoma by endobronchial ultrasound transbronchial histological needle.

    PubMed

    Ariza-Prota, Miguel Angel; Bango Álvarez, Antonio; Pérez, Liliana; Pando-Sandoval, Ana; Fuentes, Nelson; Casan, Pere

    2015-06-01

    Endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) is highly accurate in diagnosing mediastinal lymphadenopathies of lung cancer and benign disorders, with the advantage that it is a minimally invasive technique, unlike open surgery and mediastinoscopy. However, the diagnostic accuracy of EBUS-TBNA for the diagnosis of lymphoma in patients with mediastinal lymphadenopathy is not well defined. The lack of tissue architecture obtained by cytological needles decreases the diagnostic accuracy for diagnosis and subtyping of de novo and relapsed mediastinal lymphomas. We present the first described case in the literature of an anaplastic large cell lymphoma relapsed, diagnosed on tissue fragments obtained by EBUS-TBNA with the particularity of using a histological needle. PMID:26090115

  12. Mediastinal mature teratoma with rupture into pleural cavity due to blunt trauma.

    PubMed

    Miyazawa, Masahisa; Yoshida, Kazuo; Komatsu, Kazunori; Kobayashi, Nobutaka; Haba, Yoshiaki

    2012-03-01

    We report a rare case of mediastinal mature teratoma with rupture due to blunt trauma. A 15-year-old boy had received a strong head-butt in the left upper chest wall and was admitted with the sudden onset of left-sided severe chest pain and dyspnea. Chest computed tomography (CT) scan on admission revealed a heterogeneous mass lesion in the anterior mediastinum. The contrast-enhanced CT scans obtained 2 days after admission showed not only mediastinal mass lesion but also left pleural effusion. On the radiologic finding at 5 months later, the lesion became larger and was thought to be a typical mediastinal mature teratoma. The patient underwent extirpation of the tumor. Microscopically, the tumor was typically composed of adult-type tissues and was confirmed to be mature teratoma.

  13. Hodgkin’s disease presenting as discharging neck sinuses and a mediastinal mass

    PubMed Central

    Zolotar, Meira; Olaleye, Oladejo; Sherif, Ali; Howe, Rachael; Mathews, John

    2011-01-01

    A 23-year-old Asian lady presented with a hard indurated midline neck swelling of 2 months duration without any upper aerodigestive tract or systemic symptoms of note. Her inflammatory markers were elevated and she was commenced on antibiotics. Ultrasound scan and fine needle aspiration cytology (FNAC) were inconclusive. A CT scan showed an ill-defined soft tissue mass anterior to and not well demarcated from the thyroid, and contiguous with a superior and anterior mediastinal mass. Incisional biopsy revealed necrosis and pockets of purulent material. Microbiology suggested a chronic pyogenic abscess negative for acid fast bacilli. She was treated with antituberculous therapy without resolution. She developed a discharging lateral neck mass with progressive increase of the mediastinal mass. She subsequently required a neck exploration and mediastinoscopy. Repeat mediastinal biopsies confirmed the diagnosis of Hodgkin’s disease and no organisms on culture. She was commenced on chemotherapeutic treatment with a satisfactory outcome. PMID:22689859

  14. The surprising outcome of a giant primary mediastinal synovial sarcoma treated with neoadjuvant chemotherapy

    PubMed Central

    Balieiro, Marcos Alexandre; Costa, Bruno Pinheiro; Veras, Gustavo Perissé Moreira; Perelson, Paulo Sergio; Acatauassú Nunes, Rodolfo; Saito, Eduardo Haruo

    2013-01-01

    There are only a few cases of primary mediastinal synovial sarcoma in the literature. Normally, they do not respond well to chemotherapy. In our case, a 30-year-old patient was admitted due to thoracic pain, dyspnea, orthopnea, cough, hoarseness and weight loss over a 3-month period as well as a dramatic worsening a week before the admission. A chest radiography showed a completely white left hemithorax and contralateral mediastinal shift; in addition, a chest tomography revealed a giant heterogeneous mediastinal mass, lung atelectasia and a small pleural effusion. The patient was submitted to Chamberlain procedure (biopsy) under local anesthesia and the diagnosis of a synovial sarcoma was obtained after immunohistochemical analysis. Due to his poor general condition, he received chemotherapy first, with a dramatic response, after what, the mass that had been reduced was removed surgically. After a 5-year- follow-up period there are no signs of disease recurrence. PMID:23372956

  15. The surprising outcome of a giant primary mediastinal synovial sarcoma treated with neoadjuvant chemotherapy.

    PubMed

    Balieiro, Marcos Alexandre; Lopes, Agnaldo José; Costa, Bruno Pinheiro; Veras, Gustavo Perissé Moreira; Perelson, Paulo Sergio; Acatauassú Nunes, Rodolfo; Saito, Eduardo Haruo

    2013-02-01

    There are only a few cases of primary mediastinal synovial sarcoma in the literature. Normally, they do not respond well to chemotherapy. In our case, a 30-year-old patient was admitted due to thoracic pain, dyspnea, orthopnea, cough, hoarseness and weight loss over a 3-month period as well as a dramatic worsening a week before the admission. A chest radiography showed a completely white left hemithorax and contralateral mediastinal shift; in addition, a chest tomography revealed a giant heterogeneous mediastinal mass, lung atelectasia and a small pleural effusion. The patient was submitted to Chamberlain procedure (biopsy) under local anesthesia and the diagnosis of a synovial sarcoma was obtained after immunohistochemical analysis. Due to his poor general condition, he received chemotherapy first, with a dramatic response, after what, the mass that had been reduced was removed surgically. After a 5-year- follow-up period there are no signs of disease recurrence. PMID:23372956

  16. Giant Right Coronary Artery Aneurysm Mimicking a Mediastinal Cyst With Compression Effects: A Case Report

    PubMed Central

    Dadkhah Tirani, Heidar; Aghajanzadeh, Manouchehr; Pourbahador, Reza; Hassanzadeh, Rasool; Ebrahimi, Hannan

    2016-01-01

    Introduction Giant coronary artery aneurysm is an extremely rare form of coronary artery disease. The most common cause of coronary artery aneurysms is atherosclerosis. Although it is usually asymptomatic, it may have various clinical presentations, including angina, myocardial infarction or sudden death. Case Presentation A 32-year-old woman presented with edema of the upper and lower limbs, palpitation, and chest pain, and was diagnosed with a giant right coronary artery aneurysm that had initially mimicked a mediastinal cyst. Although computed tomography (CT) suggested a mediastinal cyst, trans-thoracic echocardiography revealed an extra pericardial cyst. The definitive diagnosis of right coronary artery aneurysm was made based on CT angiography and coronary angiography findings. As treatment, aneurysmectomy was performed, and she was discharged on the sixth postoperative day with good general health condition. Conclusions Coronary artery aneurysm should be a differential diagnosis in cases of mediastinal cyst and mass lesion. PMID:27800453

  17. Mediastinal lymph node metastasis of renal cell carcinoma: A case report

    PubMed Central

    MIYAZAKI, KUNIHIKO; SATO, SHINYA; KODAMA, TAKAHIDE; KURISHIMA, KOICHI; SATOH, HIROAKI; HIZAWA, NOBUYUKI

    2016-01-01

    Renal cell carcinoma (RCC) may metastasize to mediastinal lymph nodes without any abdominal lymph node involvement. The present study describes an autopsy-proven case of RCC presenting with a large mediastinal mass; the case had been previously misdiagnosed as small cell lung carcinoma due to imaging analysis results, an elevated serum level of neuron-specific enolase and the presence of small atypical cells with a high nuclear/cytoplasmic ratio. Despite RCC occurrence being rare, it should be considered in the differential diagnosis, particularly when a mass located in the kidneys presents with metastases to the mediastinal lymph nodes, even if there is no involvement of the abdominal lymph nodes and the primary lesion is of a small size. PMID:26893788

  18. Cystic Fibrosis: Brazilian ENT Experience

    PubMed Central

    Sih, Tania; Godinho, Ricardo; Franco, Leticia Paiva; Piltcher, Otávio

    2012-01-01

    Most published studies about Cystic Fibrosis (CF) are European or North American. There are still few publications about the characteristics of fibrocystic populations in developing countries. The incidence of cystic fibrosis (CF) in Brazil varies among different regions (1 : 10,000 in Minas Gerais, 1 : 9,500 in Paraná, 1 : 8,700 in Santa Catarina, and 1 : 1600 in Rio Grande do Sul). The prevalence of the DF508 mutation also varies according to population: 33% in Sao Paulo, 49% in Rio Grande do Sul, 27% in Santa Catarina, and 52% in Minas Gerais. Cough and nasal obstruction are the most common symptoms. The variation in nasal polyposis prevalence may be explained by population genotypic characteristics in a country that spans a continent. Findings on nasal endoscopy and computed tomography (CT) have better correlation than do this information compared with surgical and clinical history. Microbiologic studies suggest a high level of early contamination of the airways. Sensorineural hearing loss (SNHL) occurs in these patients as a result of ototoxic antibiotics. The data compiled in this paper is useful, but also lead to the general agreement that more research would be welcome due to the unique characteristics of this country. PMID:22611403

  19. Aspergillus infections in cystic fibrosis.

    PubMed

    King, Jill; Brunel, Shan F; Warris, Adilia

    2016-07-01

    Patients with cystic fibrosis (CF) suffer from chronic lung infection and airway inflammation. Respiratory failure secondary to chronic or recurrent infection remains the commonest cause of death and accounts for over 90% of mortality. Bacteria as Staphylococcus aureus, Pseudomonas aeruginosa and Burkholderia cepacia complex have been regarded the main CF pathogens and their role in progressive lung decline has been studied extensively. Little attention has been paid to the role of Aspergillus spp. and other filamentous fungi in the pathogenesis of non-ABPA (allergic bronchopulmonary aspergillosis) respiratory disease in CF, despite their frequent recovery in respiratory samples. It has become more apparent however, that Aspergillus spp. may play an important role in chronic lung disease in CF. Research delineating the underlying mechanisms of Aspergillus persistence and infection in the CF lung and its link to lung deterioration is lacking. This review summarizes the Aspergillus disease phenotypes observed in CF, discusses the role of CFTR (cystic fibrosis transmembrane conductance regulator)-protein in innate immune responses and new treatment modalities. PMID:27177733

  20. Post-surgical mediastinitis due to carbapenem-resistant Enterobacteriaceae: Clinical, epidemiological and survival characteristics.

    PubMed

    Abboud, C S; Monteiro, J; Stryjewski, M E; Zandonadi, E C; Barbosa, V; Dantas, D; Sousa, E E; Fonseca, M J; Jacobs, D M; Pignatari, A C; Kiffer, C; Rao, G G

    2016-05-01

    Invasive infections due to carbapenem-resistant Enterobacteriaceae (CRE), including polymyxin-resistant (PR-CRE) strains, are being increasingly reported. However, there is a lack of clinical data for several life-threatening infections. Here we describe a cohort of patients with post-surgical mediastinitis due to CRE, including PR-CRE. This study was a retrospective cohort design at a single cardiology centre. Patients with mediastinitis due to CRE were identified and were investigated for clinically relevant variables. Infecting isolates were studied using molecular techniques. Patients infected with polymyxin-susceptible CRE (PS-CRE) strains were compared with those infected with PR-CRE strains. In total, 33 patients with CRE mediastinitis were studied, including 15 patients (45%) with PR-CRE. The majority (61%) were previously colonised. All infecting isolates carried blaKPC genes. Baseline characteristics of patients with PR-CRE mediastinitis were comparable with those with PS-CRE mediastinitis. Of the patients studied, 70% received at least one agent considered active in vitro and most patients received at least three concomitant antibiotics. Carbapenem plus polymyxin B was the most common antibiotic combination (73%). Over 90% of patients underwent surgical debridement. Overall, in-hospital mortality was 33% and tended to be higher in patients infected with PR-CRE (17% vs. 53%; P=0.06). In conclusion, mediastinitis due to CRE, including PR-CRE, can become a significant challenge in centres with CRE and a high cardiac surgery volume. Despite complex antibiotic treatments and aggressive surgical procedures, these patients have a high mortality, particularly those infected with PR-CRE.

  1. Feline mediastinal lymphoma: a retrospective study of signalment, retroviral status, response to chemotherapy and prognostic indicators.

    PubMed

    Fabrizio, Francesca; Calam, Amy E; Dobson, Jane M; Middleton, Stephanie A; Murphy, Sue; Taylor, Samantha S; Schwartz, Anita; Stell, Anneliese J

    2014-08-01

    Historically, feline mediastinal lymphoma has been associated with young age, positive feline leukaemia virus (FeLV) status, Siamese breed and short survival times. Recent studies following widespread FeLV vaccination in the UK are lacking. The aim of this retrospective multi-institutional study was to re-evaluate the signalment, retroviral status, response to chemotherapy, survival and prognostic indicators in feline mediastinal lymphoma cases in the post-vaccination era. Records of cats with clinical signs associated with a mediastinal mass and cytologically/histologically confirmed lymphoma were reviewed from five UK referral centres (1998-2010). Treatment response, survival and prognostic indicators were assessed in treated cats with follow-up data. Fifty-five cases were reviewed. The median age was 3 years (range, 0.5-12 years); 12 cats (21.8%) were Siamese; and the male to female ratio was 3.2:1.0. Five cats were FeLV-positive and two were feline immunodeficiency-positive. Chemotherapy response and survival was evaluated in 38 cats. Overall response was 94.7%; complete (CR) and partial response (PR) rates did not differ significantly between protocols: COP (cyclophosphamide, vincristine, prednisone) (n = 26, CR 61.5%, PR 34.0%); Madison-Wisconsin (MW) (n = 12, CR 66.7%, PR 25.0%). Overall median survival was 373 days (range, 20-2015 days) (COP 484 days [range, 20-980 days]; MW 211 days [range, 24-2015 days] [P = 0.892]). Cats achieving CR survived longer (980 days vs 42 days for PR; P = 0.032). Age, breed, sex, location (mediastinal vs mediastinal plus other sites), retroviral status and glucocorticoid pretreatment did not affect response or survival. Feline mediastinal lymphoma cases frequently responded to chemotherapy with durable survival times, particularly in cats achieving CR. The prevalence of FeLV-antigenaemic cats was low; males and young Siamese cats appeared to be over-represented.

  2. Identification of Primary Mediastinal Large B-cell Lymphoma at Nonmediastinal Sites by Gene Expression Profiling.

    PubMed

    Yuan, Ji; Wright, George; Rosenwald, Andreas; Steidl, Christian; Gascoyne, Randy D; Connors, Joseph M; Mottok, Anja; Weisenburger, Dennis D; Greiner, Timothy C; Fu, Kai; Smith, Lynette; Rimsza, Lisa M; Jaffe, Elaine S; Campo, Elias; Martinez, Antonio; Delabie, Jan; Braziel, Rita M; Cook, James R; Ott, German; Vose, Julie M; Staudt, Louis M; Chan, Wing C

    2015-10-01

    Mediastinal involvement is considered essential for the diagnosis of primary mediastinal large B-cell lymphoma (PMBL). However, we have observed cases of diffuse large B-cell lymphoma (DLBCL) with features of PMBL but without detectable mediastinal involvement. The goal was to assess our previously established gene expression profiling (GEP) signature for PMBL in classifying these cases. In a large series of DLBCL cases, we identified 24 cases with a GEP signature of PMBL, including 9 cases with a submission diagnosis of DLBCL consistent with PMBL (G-PMBL-P) and 15 cases with a submission diagnosis of DLBCL. The pathology reviewers agreed with the diagnosis in the 9 G-PMBL-P cases. Among the other 15 DLBCL cases, 11 were considered to be PMBL or DLBCL consistent with PMBL, 3 were considered to be DLBCL, and 1 case was a gray-zone lymphoma with features intermediate between DLBCL and classical Hodgkin lymphoma. All 9 G-PMBL-P and 9 of the 15 DLBCL cases (G-PMBL-M) had demonstrated mediastinal involvement at presentation. Interestingly, 6 of the 15 DLBCL cases (G-PMBL-NM) had no clinical or radiologic evidence of mediastinal involvement. The 3 subgroups of PMBL had otherwise similar clinical characteristics, and there were no significant differences in overall survival. Genetic alterations of CIITA and PDL1/2 were detected in 26% and 40% of cases, respectively, including 1 G-PMBL-NM case with gain of PDL1/2. In conclusion, PMBL can present as a nonmediastinal tumor without evidence of mediastinal involvement, and GEP offers a more precise diagnosis of PMBL. PMID:26135560

  3. Post-surgical mediastinitis due to carbapenem-resistant Enterobacteriaceae: Clinical, epidemiological and survival characteristics.

    PubMed

    Abboud, C S; Monteiro, J; Stryjewski, M E; Zandonadi, E C; Barbosa, V; Dantas, D; Sousa, E E; Fonseca, M J; Jacobs, D M; Pignatari, A C; Kiffer, C; Rao, G G

    2016-05-01

    Invasive infections due to carbapenem-resistant Enterobacteriaceae (CRE), including polymyxin-resistant (PR-CRE) strains, are being increasingly reported. However, there is a lack of clinical data for several life-threatening infections. Here we describe a cohort of patients with post-surgical mediastinitis due to CRE, including PR-CRE. This study was a retrospective cohort design at a single cardiology centre. Patients with mediastinitis due to CRE were identified and were investigated for clinically relevant variables. Infecting isolates were studied using molecular techniques. Patients infected with polymyxin-susceptible CRE (PS-CRE) strains were compared with those infected with PR-CRE strains. In total, 33 patients with CRE mediastinitis were studied, including 15 patients (45%) with PR-CRE. The majority (61%) were previously colonised. All infecting isolates carried blaKPC genes. Baseline characteristics of patients with PR-CRE mediastinitis were comparable with those with PS-CRE mediastinitis. Of the patients studied, 70% received at least one agent considered active in vitro and most patients received at least three concomitant antibiotics. Carbapenem plus polymyxin B was the most common antibiotic combination (73%). Over 90% of patients underwent surgical debridement. Overall, in-hospital mortality was 33% and tended to be higher in patients infected with PR-CRE (17% vs. 53%; P=0.06). In conclusion, mediastinitis due to CRE, including PR-CRE, can become a significant challenge in centres with CRE and a high cardiac surgery volume. Despite complex antibiotic treatments and aggressive surgical procedures, these patients have a high mortality, particularly those infected with PR-CRE. PMID:27155944

  4. Management of descending necrotizing mediastinitis complicated with empyema by video-assisted thoracoscopic surgery.

    PubMed

    Chen, I-Chen; Hsu, Jong-Hau; Dai, Zen-Kong

    2016-05-01

    Descending necrotizing mediastinitis (DNM) is an uncommon disease that can cause significant morbidity and mortality. Herein, we report a 17-month-old girl of DNM with an initial normal chest radiograph followed by rapid clinical deterioration during a period of 4 days, from deep neck infection to mediastinitis and then complicated empyema. She was managed successfully with intravenous antibiotics and surgical debridement by video-assisted thoracic surgery (VATS) and cervical drainage. Early recognition and meticulous intensive care of patients with DNM are important to minimize morbidity and mortality. VATS is an effective and minimally invasive therapeutic strategy in children with DNM. PMID:26716737

  5. Emergent Airway Management of an Uncooperative Child with a Large Retropharyngeal and Posterior Mediastinal Abscess.

    PubMed

    Diep, Jack; Kam, David; Kuenzler, Keith A; Arthur, Jill F

    2016-02-01

    Retropharyngeal abscesses are deep neck space infections that can lead to life-threatening airway emergencies and other catastrophic complications. Retropharyngeal abscesses demand prompt diagnosis and early establishment of a definitive airway when there is airway compromise. This can be difficult in an uncooperative patient. We present the case of a 12-year-old girl with mediastinitis and tracheal compression and anterior displacement from a large retropharyngeal and posterior mediastinal abscess secondary to traumatic esophageal perforation, who received successful awake nasal fiberoptic intubation. Anesthesiologists must be prepared for airway emergencies in uncooperative patients, especially children, but there is controversy concerning the use of sedation. PMID:26599735

  6. An uncommon presentation of Kikuchi-Fujimoto disease as mediastinal lymphadenopathy.

    PubMed

    Hino, Haruaki; Nishimura, Takashi; Nitadori, Jun-Ichi; Miyakoshi, Shigesaburo; Seki, Atsuko; Arai, Tomio; Nakajima, Jun

    2016-05-01

    We experienced an uncommon presentation of Kikuchi-Fujimoto disease (KFD) with sole mediastinal lymphadenopathy in senior age, which was histologically diagnosed by thoracoscopic biopsy leading to appropriate therapy. A 69-year-old man was admitted due to intermittent high fever, general malaise, and appetite loss lasting over 3 months along with 10-kg weight loss in 6 months. Chest computed tomography (CT) showed isolated mediastinal lymphadenopathy, and malignant diseases including malignant lymphoma or metastatic carcinoma, tuberculous lymphadenitis, and sarcoidosis were considered. Therefore thoracoscopic biopsy should be performed for the correct diagnosis, even in uncommon portion of lymph node swelling and age for KFD. PMID:27162693

  7. Anesthetic management of superior vena cava syndrome due to anterior mediastinal mass

    PubMed Central

    Chaudhary, Kapil; Gupta, Anshu; Wadhawan, Sonia; Jain, Divya; Bhadoria, Poonam

    2012-01-01

    Anesthetic management of superior vena cava syndrome carries a possible risk of life-threatening complications such as cardiovascular collapse and complete airway obstruction during anesthesia. Superior vena cava syndrome results from the enlargement of a mediastinal mass and consequent compression of mediastinal structures resulting in impaired blood flow from superior vena cava to the right atrium and venous congestion of face and upper extremity. We report the successful anesthetic management of a 42-year-old man with superior vena cava syndrome posted for cervical lymph node biopsy. PMID:22557753

  8. Candida famata mediastinitis. A rare complication of open heart surgery. Case report and brief review.

    PubMed

    Sanchez Betancourt, Alfredo Alonso; Sibaja Alvarez, Pablo; Camacho, Rolando Arguedas; Guevara Espinoza, Edward

    2016-01-01

    Candida mediastinitis is a rare complication of open heart surgery with high mortality and morbidity usually associated with C. albicans. We are reporting the case of a 57 year old male who after having a triple coronary artery bypass graft procedure, had mediastinitis caused by Candida famata, a yeast, that had only been reported once before as the causal agent of this condition. It is of vital importance, that future cases be reported, due to the fact that both reported cases have led to patient demise. PMID:27419075

  9. Treatment of thoracic hemorrhage due to rupture of traumatic mediastinal hematoma.

    PubMed

    Yu, Hui-Jie; Zhang, Ling-Fang; Cao, Wei-Zhong

    2016-01-01

    Patients in traffic accidents are usually presented with pain and bleeding due to fractures or soft tissue injury. On some occasions, more severe complications may be triggered by the trauma. A review of the published English language literature reveals no survival case once the traumatic mediastinal hematoma is ruptured. In our case, a 54-year-old man suffering motorcycle accident was admitted to emergency department. Computed tomography scan revealed subdural hematoma combined with posterior mediastinal hematoma. The patient was saved and discharged with a satisfactory outcome. Here we hope to share our treatment experience in dealing with the patient with severe multiple trauma. PMID:27033275

  10. Giant cystic pheochromocytoma: A silent entity

    PubMed Central

    Gupta, Amit; Bains, Lovenish; Agarwal, Manish Kumar; Gupta, Renu

    2016-01-01

    Pheochromocytoma is a catecholamine secreting tumor that originate from chromaffin cells. Usually, it is solid neoplasm of the adrenal medulla, however cystic pheochromocytoma is a rare neuro-endocrine tumour that is frequently asymptomatic and often diagnosed incidentally on imaging or intra-operatively. Only a few cases of cystic pheochromocytomas have been reported in the world literature. We present a case of giant cystic pheochromocytoma in a 65 years old lady who presented with a large retroperitoneal lump, which is probably the world's third largest pheochromocytoma as per the available indexed literature. PMID:27453669

  11. Giant cystic pheochromocytoma: A silent entity.

    PubMed

    Gupta, Amit; Bains, Lovenish; Agarwal, Manish Kumar; Gupta, Renu

    2016-01-01

    Pheochromocytoma is a catecholamine secreting tumor that originate from chromaffin cells. Usually, it is solid neoplasm of the adrenal medulla, however cystic pheochromocytoma is a rare neuro-endocrine tumour that is frequently asymptomatic and often diagnosed incidentally on imaging or intra-operatively. Only a few cases of cystic pheochromocytomas have been reported in the world literature. We present a case of giant cystic pheochromocytoma in a 65 years old lady who presented with a large retroperitoneal lump, which is probably the world's third largest pheochromocytoma as per the available indexed literature.

  12. Heart involvement in cystic fibrosis: A specific cystic fibrosis-related myocardial changes?

    PubMed

    Labombarda, Fabien; Saloux, Eric; Brouard, Jacques; Bergot, Emmanuel; Milliez, Paul

    2016-09-01

    Cystic fibrosis is a complex multi-systemic chronic disease characterized by progressive organ dysfunction with development of fibrosis, possibly affecting the heart. Over the last four decades pathological, experimental, and clinical evidence points towards the existence of a specific myocardial involvement in cystic fibrosis. Multi-modality cardiac imaging, especially recent echocardiographic techniques, evidenced diastolic and/or systolic ventricular dysfunction in cystic fibrosis leading to the concept of a cystic fibrosis-related cardiomyopathy. Hypoxemia and inflammation are among the most important factors for heart involvement in cystic fibrosis. Cystic Fibrosis Transmembrane Regulator was found to be involved in the regulation of cardiomyocyte contraction and may also account for cystic fibrosis-related myocardial dysfunction. This review, mainly focused on echocardiographic studies, seeks to synthesize the existing literature for and against the existence of heart involvement in cystic fibrosis, its mechanisms and prognostic implications. Careful investigation of the heart function may be helpful for risk stratification and therapeutic decisions in patients with cystic fibrosis.

  13. Heart involvement in cystic fibrosis: A specific cystic fibrosis-related myocardial changes?

    PubMed

    Labombarda, Fabien; Saloux, Eric; Brouard, Jacques; Bergot, Emmanuel; Milliez, Paul

    2016-09-01

    Cystic fibrosis is a complex multi-systemic chronic disease characterized by progressive organ dysfunction with development of fibrosis, possibly affecting the heart. Over the last four decades pathological, experimental, and clinical evidence points towards the existence of a specific myocardial involvement in cystic fibrosis. Multi-modality cardiac imaging, especially recent echocardiographic techniques, evidenced diastolic and/or systolic ventricular dysfunction in cystic fibrosis leading to the concept of a cystic fibrosis-related cardiomyopathy. Hypoxemia and inflammation are among the most important factors for heart involvement in cystic fibrosis. Cystic Fibrosis Transmembrane Regulator was found to be involved in the regulation of cardiomyocyte contraction and may also account for cystic fibrosis-related myocardial dysfunction. This review, mainly focused on echocardiographic studies, seeks to synthesize the existing literature for and against the existence of heart involvement in cystic fibrosis, its mechanisms and prognostic implications. Careful investigation of the heart function may be helpful for risk stratification and therapeutic decisions in patients with cystic fibrosis. PMID:27578468

  14. Pathobiology of primary mediastinal B-cell lymphoma.

    PubMed

    Pileri, Stefano A; Zinzani, Pier L; Gaidano, Gianluca; Falini, Brunangelo; Gaulard, Philippe; Zucca, Emanuele; Sabattini, Elena; Ascani, Stefano; Rossi, Maura; Cavalli, Franco

    2003-01-01

    Controversy still exists over the response to therapy and prognosis of patients with primary mediastinal B-cell lymphoma (PMBL). Recent data from the International Extranodal Lymphoma Study Group (IELSG) suggest that a MACOP-B (methotrexate, adriamycin, cyclophosphamide, vincristine, prednisone, bleomycin) chemotherapy regimen followed by radiotherapy may be a better induction strategy than other previously used treatments. Although the pathobiology of PMBL has been widely studied, its precise histology, phenotype, and molecular characteristics are still not clear. To date, phenotypic analysis has revealed the following phenotype: positivity for CD45 and CD20, but negativity for CD3, CD10, CD21, Class I/II major histocompatibility antigens, and a variety of other immunohistochemical markers. CD79a is generally detected, despite an absence of surface immunoglobulins (Igs). CD30 staining is observed in most cases, but is weaker and less homogeneous than in classic Hodgkin's lymphoma or anaplastic large cell lymphoma. BCL-2 protein is usually expressed but there are few data describing the expression of MUM1/IRF4, PAX5/BSAP, BCL-6, or the B-cell transcription factors BOB.1, Oct-2, and PU.1. Cytogenetic studies reveal gains in segments of chromosome 9p, including amplification of the REL proto-oncogene and the tyrosine kinase gene JAK2. Other molecular findings include: C-myc mutations or rearrangements, p53 mutations, IgV(H), gene mutations, and bcl-2 and mal over-expression. bcl-6 mutations and bcl-2 gene rearrangements are generally absent, suggesting that PMBL is of pre-germinal center (GC) origin. However, two recent reports show isotype-switched Ig genes with a high frequency of somatic hypermutations as well as variants in the 5' noncoding region of the bcl-6 gene. The IELSG collected 137 PMBL cases for extensive pathologic review. Histologically, the lymphomatous growth was predominantly diffuse with sclerosis that induced compartmentalized cell aggregation. It

  15. Primary mediastinal large B-cell lymphoma, classic Hodgkin lymphoma presenting in the mediastinum, and mediastinal gray zone lymphoma: what is the oncologist to do?

    PubMed

    Grant, Cliona; Dunleavy, Kieron; Eberle, Franziska C; Pittaluga, Stefania; Wilson, Wyndham H; Jaffe, Elaine S

    2011-09-01

    In recent years, an overlap in biologic and clinical features has been identified between classic Hodgkin lymphoma (CHL) and primary mediastinal large B-cell lymphoma (PMBL). Further strengthening this relationship is the identification of lymphomas with clinical and morphologic features transitional between the two, known as gray zone lymphomas (GZL). However, this diagnostic gray zone is not just of theoretical interest: it presents a practical problem, as the treatment approaches for CHL traditionally differ from those for aggressive B-cell lymphomas. This article reviews the treatment approach for mediastinal lymphomas, including CHL of the nodular sclerosis subtype (CHL-NS), PMBL, and mediastinal GZL. Though several trials have evaluated different regimens with or without radiation in PMBL and CHL-NS, there is a lack of prospective experience in treating GZL because of the rarity of these tumors. Historical data indicate that they have done poorly with traditional approaches developed for the treatment of either CHL or diffuse large B-cell lymphoma. PMID:21590365

  16. Chronic pancreatitis and cystic fibrosis

    PubMed Central

    Witt, H

    2003-01-01

    Recent discoveries of trypsinogen and trypsin inhibitor mutations in patients with chronic pancreatitis (CP) support the hypothesis that an inappropriate activation of pancreatic zymogens to active enzymes within the pancreatic parenchyma starts the inflammatory process. Current data suggest that CP may be inherited dominant, recessive, or complex as a result of mutations in the above mentioned or yet unidentified genes. Evaluation of patients with CP should include genetic testing. Cystic fibrosis (CF) is an autosomal recessive inherited disorder caused by mutations in the CF transmembrane conductance regulator (CFTR) gene and is characterised by pancreatic insufficiency and chronic bronchopulmonary infection. The progression and severity of pulmonary disease differs considerably between people with identical CFTR mutations and does not seem to correlate with the type or class of the CFTR mutation. The identification of further disease modifying genetic factors will increase the pathophysiological understanding and may help to identify new therapeutic targets. PMID:12651880

  17. Cystic echinococcosis in southern Brazil.

    PubMed

    de la Rue, Mario L

    2008-01-01

    Cystic echinococcosis (CE) is very common in the southern part of Rio Grande do Sul State where sheep and cattle raising is the most important economical activity. Prevalence in intermediate hosts is well known due to reports in slaughterhouses while human reports are of discussed value. This is due to underreporting of cases and few epidemiological studies make it difficult to assess the situation of echinococcosis in the population. Whereas cattle infection rate is more or less stable around 12% of the slaughter animals, in sheep there has been an increasing number in the last five years. Some efforts have been done to control the zoonosis but no effective results were obtained so far. Probably educational efforts to change human practices (feeding dogs with raw viscera), periodic treatment of dogs with praziquantel and joined actions with slaughterhouses could bring more attention to improve some control measures.

  18. Radiological description of cystic pancreatic tumors.

    PubMed

    Rodríguez Torres, C; Larrosa López, R

    2016-01-01

    Although most cystic pancreatic lesions are pseudocysts, it is important to do a thorough differential diagnosis with true cystic tumors because cystic tumors are potentially malignant. Sometimes computed tomography and magnetic resonance imaging cannot establish the definitive diagnosis, making it necessary to perform other imaging tests such as endoscopic ultrasound, which in addition to morphological information, can also enable cytologic and biochemical analysis of the lesion through puncture and aspiration of its contents. Combining all these findings nearly always provides enough diagnostic information to allow the appropriate approach in each case. This article describes the specific morphological characteristics for each cystic pancreatic tumor on computed tomography, magnetic resonance imaging, and endoscopic ultrasound and reviews the guidelines for managing these types of lesions.

  19. Thoracoscopic resection with intraoperative use of methylene blue to localize mediastinal parathyroid adenomas.

    PubMed

    Adachi, Yoshin; Nakamura, Hiroshige; Taniguchi, Yuji; Miwa, Ken; Fujioka, Shinji; Haruki, Tomohiro

    2012-03-01

    We report a case of thoracoscopic resection of mediastinal parathyroid adenomas using methylene blue to localize the tumors during the operation. After methylene blue 4 mg/kg was injected intravenously, we easily identified methylene blue-stained parathyroid glands and successfully resected them with sufficient surgical margins. The use of methylene blue for detection of parathyroid adenoma is a useful technique.

  20. Accurate localization of supernumerary mediastinal parathyroid adenomas by a combination of structural and functional imaging.

    PubMed

    Mackie, G C; Schlicht, S M

    2004-09-01

    Reoperation for refractory or recurrent hyperparathyroidism following parathyroidectomy carries the potential for increased morbidity and the possibility of failure to localize and remove the lesion intraoperatively. Reported herein are three cases demonstrating the combined use of sestamibi scintigraphy, CT and MR for accurate localization of mediastinal parathyroid adenomas.

  1. The flow and composition of lymph from the caudal mediastinal lymph node of sheep.

    PubMed Central

    Spencer, J; Hall, J G

    1984-01-01

    By cannulating the efferent duct of the caudal mediastinal lymph node in sheep, lymph from the lower respiratory tract was collected under physiological conditions for several days. In 18 such preparations the flow rate varied from 4 to 12 ml/hr between individuals and the lymphocyte count between 4000 and 117,000/mm3. The protein content of the lymph plasma averaged nearly 60% of that of the blood, and this indication of the high permeability of the capillary bed of the lungs was confirmed by measuring the time taken for intravenous doses of 125I-albumin to equilibrate between the blood and mediastinal lymph plasma. The concentration of immunoglobulin A was higher in the mediastinal lymph than in blood serum, while the reverse was true of the concentrations of IgG1, IgG2, and IgM. This evidence for the local production of IgA by the intra thoracic lymphoid tissue was supported by the demonstration by immunoperoxidase techniques of IgA-containing plasma cells in sections cut from the caudal mediastinal nodes, and of IgA-containing immunoblasts in the lymph. Images Figure 3 PMID:6715022

  2. Benign Mature Mediastinal Dysembryoma with Pulmonary Extension Revealed by Recurrent Hemoptysis in a Young Woman

    SciTech Connect

    Filaire, M.; Michel-Letonturier, M.; Garcier, J. M.; Escande, G.; Boyer, L.

    2006-06-15

    We report one case of mature mediastinal teratoma with pulmonary extension surgically diagnosed in a 22-year-old woman complaining of recurrent hemoptyses for which no etiological explanation could be found. Thoracic surgery was only decided on after three embolizations proved ineffective.

  3. Cranial vena cava syndrome secondary to cryptococcal mediastinal granuloma in a cat

    PubMed Central

    Letendre, Jo-Annie; Boysen, Søren

    2015-01-01

    The successful management of cranial vena cava syndrome with suspected secondary chylothorax due to mediastinal cryptococcal granuloma in a 4-year-old male domestic shorthair cat is described. Treatment included long-term antifungal medication, short-term corticosteroids, intermittent thoracocentesis, rutin, octreotide, and enalapril. PMID:25829555

  4. A Case of Descending Necrotizing MediastinitisPenetrating to the Esophagus

    PubMed Central

    Inaba, Yuichiro; Tokano, Hisashi; Ohtsu, Atsushi; Kitamura, Ken

    2010-01-01

    Here, we present the case of a 78-year-old man with a deep neck infection that caused descending necrotizing mediastinitis that extended from the pharynx to the stomach and was accompanied by two large esophageal fistulas and multiple gastric ulcers. We believe that the series of lesions were the signs of a hidden carcinoma. PMID:25648975

  5. Precision Genomic Medicine in Cystic Fibrosis.

    PubMed

    Chang, Eugene H; Zabner, Joseph

    2015-10-01

    The successful application of precision genomic medicine requires an understanding of how a person's genome can influence his or her disease phenotype and how medical therapies can provide personalized therapy to one's genotype. In this review, we highlight advances in precision genomic medicine in cystic fibrosis (CF), a classic autosomal recessive genetic disorder. We discuss genotype-phenotype correlations in CF, genetic and environmental modifiers of disease, and pharmacogenetic therapies that target specific genetic mutations thereby addressing the primary defect of cystic fibrosis.

  6. [Cystic degeneration of autonomous adenomas (author's transl)].

    PubMed

    Galvan, G; Pohl, G B

    1976-01-01

    Follow-up examinations in four patients with autonomous adenomas showed cystic degeneration in the autonomous adenomas 20 to 45 months after the first examination, confirmed by fine needle biopsy. Clinical improvement occurred three times with scintigraphic compensation, decompensation occurred once without clinical deterioration. In particular cases a therapeutic policy of wait and see is justified in patients with autonomous adenomas because they may remain clinically inconspicuous for a long time; on the other hand there is a possibility of a cystic degeneration.

  7. Dose-Adjusted EPOCH-Rituximab Therapy in Primary Mediastinal B-Cell Lymphoma

    PubMed Central

    Dunleavy, Kieron; Pittaluga, Stefania; Maeda, Lauren S.; Advani, Ranjana; Chen, Clara C.; Hessler, Julie; Steinberg, Seth M.; Grant, Cliona; Wright, George; Varma, Gaurav; Staudt, Louis M.; Jaffe, Elaine S.; Wilson, Wyndham H.

    2015-01-01

    BACKGROUND Primary mediastinal B-cell lymphoma is a distinct subtype of diffuse large-B-cell lymphoma that is closely related to nodular sclerosing Hodgkin’s lymphoma. Patients are usually young and present with large mediastinal masses. There is no standard treatment, but the inadequacy of immunochemotherapy alone has resulted in routine consolidation with mediastinal radiotherapy, which has potentially serious late effects. We aimed to develop a strategy that improves the rate of cure and obviates the need for radiotherapy. METHODS We conducted a single-group, phase 2, prospective study of infusional dose-adjusted etoposide, doxorubicin, and cyclophosphamide with vincristine, prednisone, and rituximab (DA-EPOCH-R) and filgrastim without radiotherapy in 51 patients with untreated primary mediastinal B-cell lymphoma. We used results from a retrospective study of DA-EPOCH-R from another center to independently verify the outcomes. RESULTS The patients had a median age of 30 years (range, 19 to 52) and a median tumor diameter of 11 cm; 59% were women. During a median of 5 years of follow-up, the event-free survival rate was 93%, and the overall survival rate was 97%. Among the 16 patients who were involved in the retrospective analysis at another center, over a median of 3 years of follow-up, the event-free survival rate was 100%, and no patients received radiotherapy. No late morbidity or cardiac toxic effects were found in any patients. After follow-up ranging from 10 months to 14 years, all but 2 of the 51 patients (4%) who received DA-EPOCH-R alone were in complete remission. The 2 remaining patients received radiotherapy and were disease-free at follow-up. CONCLUSIONS Therapy with DA-EPOCH-R obviated the need for radiotherapy in patients with primary mediastinal B-cell lymphoma. (Funded by the National Cancer Institute; ClinicalTrials.gov number, NCT00001337.) PMID:23574119

  8. Mediastinal transposition of the omentum reduces infection severity and pharmacy cost for patients undergoing esophagectomy

    PubMed Central

    Ye, Peng; Cao, Jin-Lin; Li, Qiu-Yuan; Wang, Zhi-Tian; Yang, Yun-Hai; Lv, Wang

    2016-01-01

    Background The greater omentum has been found to be immunologically competent in protecting abdominal organs from inflammation. Anastomotic omentoplasty has been used and proven effective in preventing anastomotic leaks after an esophagectomy. However, pulmonary complications are still a substantial problem after an esophagectomy. This study investigated the benefits of mediastinal transposition of the omentum, a modification of the conventional omental wrapping technique, in controlling overall postoperative intrathoracic complications. Methods From January 2010 to March 2015, 208 consecutive patients receiving an open Ivor-Lewis esophagectomy at our institution were retrospectively reviewed. One hundred twenty-one patients with omentum mediastinal transposition were assigned to the transposition group and 87 patients without omental transposition were placed in the non-transposition group. The patients’ demographics, postoperative short-term outcomes, and in-hospital cost were documented and analyzed. Results Mediastinal transposition of the omentum led to a shorter postoperative hospital stay (14 vs. 16 d, P=0.038) and a lower intrathoracic infection rate (30.6% vs. 48.3%, P=0.009). Intrathoracic infection was milder in the transposition group (P=0.005), though a non-significant was found in overall complications (P=0.071). The multivariate logistic regression analyses identified omentum mediastinal transposition (P=0.007, OR=0.415) as an independent protective factor for postoperative intrathoracic infection. The total in-hospital cost was comparable in both groups (P>0.05), whereas the pharmacy cost was lower in the transposition group than in the non-transposition group (¥21,668 vs. ¥27,012, P=0.010). Conclusions Mediastinal transposition of the omentum decreases the rate and severity of postoperative intrathoracic infection following an open Ivor-Lewis esophagectomy. This result in decreased pharmacy costs, rather than resulting in an increased economic

  9. Anterior mediastinal fat in Behçet's disease: qualitative and quantitative CT analysis.

    PubMed

    Lee, Sang Yub; Lee, Jongmin; Lee, Hui Joong; Choi, Sun Ju; Hahm, Myong Hun; Yoon, Sung Won

    2013-12-01

    The fat-rich anterior mediastinum could be a sensitive window for monitoring minute changes in vascularity induced by systemic vasculitis. To evaluate this hypothesis, an analysis of anterior mediastinal fat in patients with Behçet's disease and a control group was conducted. This study included 43 patients diagnosed with Behçet's disease within the last 11 years who underwent CT scan; 55 patients were selected as a control population. Mediastinal fat was classified according to CT morphology. Comparison of serum inflammatory markers was performed for evaluation of disease activity according to morphologic types, and average Hounsfield unit of the anterior mediastinum was measured. Significantly higher mean CT attenuation was observed in the Behçet's disease group, compared with the control group (-48.5 ± 33.5 vs. -67.7 ± 18.7, respectively, P < 0.05). Mediastinal fat types were classified as follows: pure fatty tissue (2 vs. 31 % [Behçet's disease vs. control group]), diffuse soft tissue infiltration (16 vs. 29 %), tubular structures (21 vs. 4 %), mixed infiltration with tubular structures (42 vs. 15 %), and evident thymic tissue (19 vs. 22 %). The value for mean mediastinal attenuation was significantly higher in the group with a high level of C-reactive protein than in the normal level group. The mean CT attenuation of anterior mediastinal fat is significantly higher in the Behçet's disease group, compared with the normal group. Although pathologic confirmation is needed, the cause is postulated to be either inflammatory neovascularization or minimal thymic hyperplasia induced by Behçet's disease.

  10. Feasibility of thoracoscopic approach for retrosternal goitre (posterior mediastinal goitre): Personal experiences of 11 cases

    PubMed Central

    Bhargav, Panchangam Ramakanth; Amar, Vennapusa; Mahilvayganan, Sabaretnam; Nanganandadevi, Vimala

    2016-01-01

    INTRODUCTION: Posterior mediastinal goitres constitute of a unique surgical thyroid disorder that requires expert management. Occasionally, they require thoracic approach for the completion of thyroidectomy. In this paper, we describe the feasibility and utility of a novel thoracoscopic approach for such goitres. MATERIALS AND METHODS: This is a retrospective study conducted at a tertiary care endocrine surgery department in South India over a period of 5 years from January 2010 to December 2014. We developed a novel thoracoscopic technique for posterior mediastinal goitres instead of a more morbid thoracotomy or sternotomy. All the clinical, investigative, operative, pathological and follow-up data were collected from our prospectively filled database. Statistical analysis was done with SPSS 15.0 version. Descriptive analysis was done. OPERATIVE TECHNIQUE OF THORACOSCOPIC THYROIDECTOMY: Single lumen endotracheal tube (SLETT) was used of anaesthetic intubation and general inhalational anaesthesia. Operative decubitus was supine with extension and abduction of the ipsilateral arm. Access to mediastinum was obtained by two working ports in the third and fifth intercostal spaces. Mediastinal extension was dissected thoracoscopically and delivered cervically. RESULTS: Out of 1,446 surgical goitres operated during the study period, 72 (5%) had retrosternal goitre. Also, 27/72 (37.5%) cases had posterior mediastinal extension (PME), out of which 11 cases required thoracic approach. We utilised thoracoscopic technique for these 11 cases. The post-operative course was uneventful with no major morbidity. There was one case of recurrent laryngeal nerve (RLN) injury and hoarseness of voice in the third case. Histopathologies in 10 cases were benign, out of which two had subclinical hyperthyroidism. One case had multifocal papillary microcarcinoma. CONCLUSIONS: We opine that novel thoracoscopic technique is a feasibly optimal approach for posterior mediastinal goitre

  11. Surgical outcome in cystic vestibular schwannomas

    PubMed Central

    Nair, Suresh; Baldawa, Sachin S.; Gopalakrishnan, Chittur Viswanathan; Menon, Girish; Vikas, Vazhayil; Sudhir, Jayanand B.

    2016-01-01

    Background: Cystic vestibular schwannomas (VS) form a rare subgroup that differs from the solid variant clinically, radiologically, and histopathologically. These tumors also vary in their surgical outcome and carry a different risk of post-operative complications. We analyzed our series of 64 patients with cystic VS and discuss the technical difficulties related to total excision of these tumors and focus on complication avoidance. Materials and Methods: A retrospective review of cystic VS surgically managed over a span of 11 years. The case records were evaluated to record the clinical symptoms and signs, imaging findings, surgical procedure, complications, and follow-up data. Post-operative facial nerve palsy was analyzed with respect to tumor size and tumor type. Results: Progressive hearing impairment was the most common initial symptom (76.6%). Atypical initial symptoms were present in 15 patients (23.4%). Preoperatively, 78% patients had good facial nerve function (HB grade 1, 2) and 22% had intermediate (HB grade 3, 4) to poor (HB grade 5 and 6) function. Mean tumor size was 4.1 cm. Complete tumor removal was achieved in 53 patients (83%). The facial nerve was anatomically intact but thinned out after tumor excision in 38 patients (59.4%). Ninety percent patients had either intermediate or poor facial nerve function at follow-up. Poor facial nerve outcome was associated with giant tumors and peripherally located, thin-walled cystic tumors. Conclusion: Resection of cystic VS is complicated by peritumoral adhesions of the capsule to the nerve. Extensive manipulation of the nerve in order to dissect the tumor–nerve barrier results in worse facial nerve outcome. The outcome is worse in peripherally located, thin-walled cystic VS as compared to centrally located, thick-walled cystic tumors. Subtotal excision may be justified, especially in tumors with dense adhesion of the cyst wall to the facial nerve in order to preserve nerve integrity. PMID:27366248

  12. Molecular epidemiology of cystic echinococcosis.

    PubMed

    McManus, D P; Thompson, R C A

    2003-01-01

    Echinococcus granulosus exhibits substantial genetic diversity that has important implications for the design and development of vaccines, diagnostic reagents and drugs effective against this parasite. DNA approaches that have been used for accurate identification of these genetic variants are presented here as is a description of their application in molecular epidemiological surveys of cystic echinococcosis in different geographical settings and host assemblages. The recent publication of the complete sequences of the mitochondrial (mt) genomes of the horse and sheep strains of E. granulosus and of E. multilocularis, and the availability of mt DNA sequences for a number of other E. granulosus genotypes, has provided additional genetic information that can be used for more in depth strain characterization and taxonomic studies of these parasites. This very rich sequence information has provided a solid molecular basis, along with a range of different biological, epidemiological, biochemical and other molecular-genetic criteria, for revising the taxonomy of the genus Echinococcus. This has been a controversial issue for some time. Furthermore, the accumulating genetic data may allow insight to several other unresolved questions such as confirming the occurrence and precise nature of the E. granulosus G9 genotype and its reservoir in Poland, whether it is present elsewhere, why the camel strain (G6 genotype) appears to affect humans in certain geographical areas but not others, more precise delineation of the host and geographic ranges of the genotypes characterised to date, and whether additional genotypes of E. granulosus remain to be identified. PMID:15042999

  13. Respiratory Conditions Update: Cystic Fibrosis.

    PubMed

    Pritchard, Lyle L

    2016-09-01

    Cystic fibrosis (CF) is an autosomal recessive genetic disease that occurs in approximately 1 in 2,500 white live births. It is less common in nonwhite individuals. A dysfunctional epithelial chloride channel leads to excessively thick mucus affecting multiple organ systems. Common issues include mucous plugging of the airway, lung inflammation, chronic pulmonary infections, intestinal malabsorption, and malnutrition. Universal screening of newborns for CF is recommended in many countries. CF can be diagnosed based on clinical evidence of disease along with genetic testing or other laboratory evidence of chloride channel dysfunction. Pulmonary system dysfunction causes the most morbidity and mortality. Pulmonary function testing is the primary modality used to monitor CF progression. Therapies include chest physiotherapy, mucolytics, antibiotics, anti-inflammatory drugs, targeted therapies, and vaccines. Dysfunction of the exocrine pancreas and gastrointestinal tract leads to malabsorption, malnutrition, and intestinal obstruction. Nutrition should be optimized with adequate calories, pancreatic enzymes, and appropriate dietary supplements. Complications, including acute pulmonary exacerbations, gastrointestinal conditions, chronic rhinosinusitis, CF-related diabetes, osteoporosis, infertility, and psychosocial issues, must be managed. At the appropriate time, lung transplantation and end-of-life issues must be addressed. PMID:27576234

  14. Lung Transplantation for Cystic Fibrosis

    PubMed Central

    Adler, Frederick R.; Aurora, Paul; Barker, David H.; Barr, Mark L.; Blackwell, Laura S.; Bosma, Otto H.; Brown, Samuel; Cox, D. R.; Jensen, Judy L.; Kurland, Geoffrey; Nossent, George D.; Quittner, Alexandra L.; Robinson, Walter M.; Romero, Sandy L.; Spencer, Helen; Sweet, Stuart C.; van der Bij, Wim; Vermeulen, J.; Verschuuren, Erik A. M.; Vrijlandt, Elianne J. L. E.; Walsh, William; Woo, Marlyn S.; Liou, Theodore G.

    2009-01-01

    Lung transplantation is a complex, high-risk, potentially life-saving therapy for the end-stage lung disease of cystic fibrosis (CF). The decision to pursue transplantation involves comparing the likelihood of survival with and without transplantation as well as assessing the effect of wait-listing and transplantation on the patient's quality of life. Although recent population-based analyses of the US lung allocation system for the CF population have raised controversies about the survival benefits of transplantation, studies from the United Kingdom and Canada have suggested a definite survival advantage for those receiving transplants. In response to these and other controversies, leaders in transplantation and CF met together in Lansdowne, Virginia, to consider the state of the art in lung transplantation for CF in an international context, focusing on advances in surgical technique, measurement of outcomes, use of prognostic criteria, variations in local control over listing, and prioritization among the United States, Canada, the United Kingdom, and The Netherlands, patient adherence before and after transplantation and other issues in the broader context of lung transplantation. Finally, the conference members carefully considered how efforts to improve outcomes for lung transplantation for CF lung disease might best be studied. This Roundtable seeks to communicate the substance of our discussions. PMID:20008865

  15. Evaluation of hepatic cystic lesions

    PubMed Central

    Lantinga, Marten A; Gevers, Tom JG; Drenth, Joost PH

    2013-01-01

    Hepatic cysts are increasingly found as a mere coincidence on abdominal imaging techniques, such as ultrasonography (USG), computed tomography (CT) and magnetic resonance imaging (MRI). These cysts often present a diagnostic challenge. Therefore, we performed a review of the recent literature and developed an evidence-based diagnostic algorithm to guide clinicians in characterising these lesions. Simple cysts are the most common cystic liver disease, and diagnosis is based on typical USG characteristics. Serodiagnostic tests and microbubble contrast-enhanced ultrasound (CEUS) are invaluable in differentiating complicated cysts, echinococcosis and cystadenoma/cystadenocarcinoma when USG, CT and MRI show ambiguous findings. Therefore, serodiagnostic tests and CEUS reduce the need for invasive procedures. Polycystic liver disease (PLD) is arbitrarily defined as the presence of > 20 liver cysts and can present as two distinct genetic disorders: autosomal dominant polycystic kidney disease (ADPKD) and autosomal dominant polycystic liver disease (PCLD). Although genetic testing for ADPKD and PCLD is possible, it is rarely performed because it does not affect the therapeutic management of PLD. USG screening of the liver and both kidneys combined with extensive family history taking are the cornerstone of diagnostic decision making in PLD. In conclusion, an amalgamation of these recent advances results in a diagnostic algorithm that facilitates evidence-based clinical decision making. PMID:23801855

  16. [Azithromycin therapy in cystic fibrosis].

    PubMed

    Máiz Carro, Luis; Cantón Moreno, Rafael

    2004-03-01

    Progressive lung disease, caused by chronic endobronchial colonization, is the major cause of morbidity and mortality in patients with cystic fibrosis (CF). Several pathogens, including Staphylococcus aureus and Pseudomonas aeruginosa are responsible for this effect. The steadily improving prognosis of CF has been attributed to the use of antibiotics with activity against these organisms. Despite a significant increase in the amount of published material demonstrating the potential role of macrolide antibiotics as antiinflammatory agents and their effects on bacterial virulence, their mechanism of action in CF patients is still unknown. Although there is a limited number of clinical trials assessing the efficacy and safety of azithromycin (AZM) in CF, increasing evidence suggests that 3 to 6-month AZM treatment in CF patients is safe and well tolerated. This treatment results in clinical improvement, decreasing the number of pulmonary exacerbations and increasing pulmonary function. Therefore, chronic treatment with AZM should be considered in CF patients added to conventional therapy. Clinical experience with macrolides other than AZM in CF patients is very limited. PMID:15030744

  17. CXCR4+ granulocytes reflect fungal cystic fibrosis lung disease.

    PubMed

    Carevic, Melanie; Singh, Anurag; Rieber, Nikolaus; Eickmeier, Olaf; Griese, Matthias; Hector, Andreas; Hartl, Dominik

    2015-08-01

    Cystic fibrosis airways are frequently colonised with fungi. However, the interaction of these fungi with immune cells and the clinical relevance in cystic fibrosis lung disease are incompletely understood.We characterised granulocytes in airway fluids and peripheral blood from cystic fibrosis patients with and without fungal colonisation, non-cystic fibrosis disease controls and healthy control subjects cross-sectionally and longitudinally and correlated these findings with lung function parameters.Cystic fibrosis patients with chronic fungal colonisation by Aspergillus fumigatus were characterised by an accumulation of a distinct granulocyte subset, expressing the HIV coreceptor CXCR4. Percentages of airway CXCR4(+) granulocytes correlated with lung disease severity in patients with cystic fibrosis.These studies demonstrate that chronic fungal colonisation with A. fumigatus in cystic fibrosis patients is associated with CXCR4(+) airway granulocytes, which may serve as a potential biomarker and therapeutic target in fungal cystic fibrosis lung disease. PMID:25929952

  18. Genetics Home Reference: medullary cystic kidney disease type 1

    MedlinePlus

    ... disease type 1 medullary cystic kidney disease type 1 Enable Javascript to view the expand/collapse boxes. ... Close All Description Medullary cystic kidney disease type 1 (MCKD1) is an inherited condition that affects the ...

  19. Rehabilitation with Cystic Fibrosis: From Utopia to Reality.

    ERIC Educational Resources Information Center

    Goldberg, Richard T.; And Others

    1980-01-01

    The paper dispels some of the myths regarding cystic fibrosis (a genetic metabolism disorder), provides information on the latest developments in rehabilitation, summarizes research in the field, and projects future needs of the patient with cystic fibrosis. (SBH)

  20. CXCR4+ granulocytes reflect fungal cystic fibrosis lung disease.

    PubMed

    Carevic, Melanie; Singh, Anurag; Rieber, Nikolaus; Eickmeier, Olaf; Griese, Matthias; Hector, Andreas; Hartl, Dominik

    2015-08-01

    Cystic fibrosis airways are frequently colonised with fungi. However, the interaction of these fungi with immune cells and the clinical relevance in cystic fibrosis lung disease are incompletely understood.We characterised granulocytes in airway fluids and peripheral blood from cystic fibrosis patients with and without fungal colonisation, non-cystic fibrosis disease controls and healthy control subjects cross-sectionally and longitudinally and correlated these findings with lung function parameters.Cystic fibrosis patients with chronic fungal colonisation by Aspergillus fumigatus were characterised by an accumulation of a distinct granulocyte subset, expressing the HIV coreceptor CXCR4. Percentages of airway CXCR4(+) granulocytes correlated with lung disease severity in patients with cystic fibrosis.These studies demonstrate that chronic fungal colonisation with A. fumigatus in cystic fibrosis patients is associated with CXCR4(+) airway granulocytes, which may serve as a potential biomarker and therapeutic target in fungal cystic fibrosis lung disease.

  1. Living with Cystic Fibrosis: A Guide for the Young Adult.

    ERIC Educational Resources Information Center

    Cystic Fibrosis Foundation, Atlanta, GA.

    Intended for the young adult with cystic fibrosis, the booklet provides information on dealing with problems and on advances in treatment and detection related to the disease. Addressed are the following topics: description of cystic fibrosis; inheritance of cystic fibrosis; early diagnosis; friends, careers, and other matters; treatment;…

  2. 78 FR 26681 - Medical Criteria for Evaluating Cystic Fibrosis

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-05-07

    ... ADMINISTRATION RIN 0960-AF58 Medical Criteria for Evaluating Cystic Fibrosis AGENCY: Social Security....04 to evaluate claims involving cystic fibrosis in adults and children under titles II and XVI of the... information on the disability program. 2. Information for individuals with cystic fibrosis who apply...

  3. Premalignant cystic neoplasms of the pancreas.

    PubMed

    Dudeja, Vikas; Allen, Peter J

    2015-02-01

    Due to increasing utilization of cross-sectional imaging, asymptomatic pancreatic cysts are frequently being diagnosed. Many of these cysts have premalignant potential and offer a unique opportunity for cancer prevention. Mucinous cystic neoplasm and intraductal papillary mucinous neoplasm are the major premalignant cystic neoplasms of pancreas. The prediction of the risk of malignancy (incidental and future risk of malignant transformation) and balancing the risks of watchful waiting with that of operative management with associated mortality and morbidity is the key to the management of these lesions. We review the literature that has contributed to the development of our approach to the management of these cystic neoplasms. We provide an overview of the key features used in diagnosis and in predicting malignancy. Particular attention is given to the natural history and management decision making.

  4. Do brine shrimp diagnose cystic fibrosis?

    PubMed

    Hodes, M E; Thomas, J; Morgan, S; Merritt, A D

    1975-11-01

    The nauplii of the brine shrimp Artemia salina are dependent upon the function of their salt gland to maintain osmotic pressure within narrow limits. A number of drugs interfere with this function and are lethal to the nauplii. Saliva and serum from normal persons, patients with cystic fibrosis, and obligate heterozygotes were tested for lethal effect against brine shrimp nauplii. At salt concentrations between 100 mM and 2.5 no difference was found among the phenotypes. At lower concentrations a difference was noted occasionally between some normal subjects and some individuals carrying one or two genes for cystic fibrosis. Data from an independent series of experiments indicate that the naupliar deaths result from distorted ratios of Na+/K+ and not from a specific gene product. No difference was noted in the O2 uptake of nauplii treated with saliva or serum obtained from normal subjects, patients with cystic fibrosis, or obligate heterozygotes. PMID:1187245

  5. Florid cystic endosalpingiosis of the uterus

    PubMed Central

    Heatley, M; Russell, P

    2001-01-01

    A 73 year old woman presented with a right sided adnexal cystic mass. At laparotomy, this proved to be a benign serous ovarian cyst and an aggregation of thin walled subserosal and soft tissue cysts and spongy nodules up to 16 mm in diameter involving the side wall of the uterus and adjacent parametrium. These were removed by total abdominal hysterectomy and bilateral salpingo-oophorectomy. Histologically, the cystic spaces and smaller acini were lined by benign tubo-endometrioid epithelium, with smaller areas typical of serous differentiation and rare microfoci of endocervical-type mucinous epithelium. These features indicated multidirectional Mullerian differentiation in a process that, overall, was consistent with so called florid cystic endosalpingiosis. This lesion is to be distinguished from other benign conditions including multicystic mesothelioma, endometriosis, endocervicosis, florid deep glands of the uterine cervix, and deep Nabothian cysts of the uterine cervix. Key Words: uterus • endosalpingiosis • cysts PMID:11328842

  6. Mediastinal Yolk Sac Tumor Producing Protein Induced by Vitamin K Absence or Antagonist-II.

    PubMed

    Akutsu, Noriyuki; Adachi, Yasushi; Isosaka, Mai; Mita, Hiroaki; Takagi, Hideyasu; Sasaki, Shigeru; Yamamoto, Hiroyuki; Arimura, Yoshiaki; Ishii, Yoshifumi; Masumori, Naoya; Endo, Takao; Shinomura, Yasuhisa

    2015-01-01

    Extragonadal yolk sac tumors (YSTs) are rare. We herein report the case of a 66-year-old man with mediastinal, lung and liver tumors. The largest mass was located in the liver and contained a high concentration of protein induced by vitamin K absence or antagonist-II (PIVKA-II) and alpha-fetoprotein. Therefore, the lesion was difficult to distinguish from hepatocellular carcinoma. Finally, YST was diagnosed based on the results of a liver biopsy. Although chemotherapy was effective, the patient died of respiratory failure. The autopsy revealed primary mediastinal YST. In the current report, we describe this case of PIVKA-II-producing YST and review previous cases of PIVKA-II-producing tumors other than hepatoma.

  7. [Analysis of mediastinal lymphadenopathy in sarcoidosis with transesophageal ultrasonic endoscopy; influences on cardiovascular system].

    PubMed

    Dambara, T; Ueki, J; Aiba, M; Tamaki, S; Saito, H; Matsuda, K; Nukiwa, T; Kira, S

    1989-01-01

    We studied influences of mediastinal lymphadenopathy on cardiovascular system in nine cases of sarcoidosis with transesophageal ultrasonic endoscopy. Chest x-ray films revealed bilateral hilar lymphadenopathy in all cases, whose diagnoses were made histologically with biopsies of peripheral lung through flexible fiber optic bronchoscope and/or of lymph nodes. These findings were typical in this disease. Many compartments of cardiovascular system in mediastinal and hilar regions were visible with this technique in all subjects. And enlarged lymph nodes changed dimensions in these compartments, especially in main pulmonary arteries, left atria, pulmonary veins and aorta. Furthermore, these phenomena were seen in azygos vein and superior caval vein in some cases. It is suggested that these lymph nodes may influence hemodynamics in above-mentioned compartments of cardiovascular system, if this phenomenon deteriorates more progressively. PMID:2657139

  8. Facial, Cervical, and Mediastinal Emphysema of the Clarinet Player: Case Report

    PubMed Central

    Biçer, Yusuf Özgür; Kesgin, Selcan; Tezcan, Erkan; Köybaşı, Serap

    2014-01-01

    Background: Cervicofacial emphysema may arise due to the leakage of air from a defect in the aerodigestive tract to the fascial layers of neck and face. Rarely, it may be caused by insufflation of air through the Stensen’s duct. Case Report: We present a case with diffuse facial, cervical and mediastinal emphysema due to playing a wind instrument immediately after a facial trauma. There was no mucosal defect or laceration noticed by examination which could explain the origin of the emphysema. Despite the widespread cervicofacial emphysema with mediastinal involvement, the patient significantly improved within 48 hours without any intervention. Conclusion: Even though cervicofacial emphysema ameliorates spontaneously, increased care must be taken, especially when there is pneumomediastinum and/or pneumothorax. PMID:25667794

  9. Late cardiac effect of radiation therapy on a young woman with mediastinal Hodgkin's lymphoma.

    PubMed

    Kodama, Ken; Takami, Hiroshi; Izumi, Masahiro; Hiramoto, Yoshimune; Yoshida, Kyotaro; Nishioka, Kiyonori; Higashiyama, Masahiko

    2016-01-01

    We experienced an excision of the mediastinal tumor located in left anterior mediastinum found in a 21-year-old woman. The histologic diagnosis of Hodgkin's lymphoma was obtained. After operation, she was treated with mantle field radiotherapy (total 42 Gy) followed by chemotherapy. At 43-year old, she developed constrictive pericarditis and heart failure resulted in the late cardiac effects of mediastinal irradiation. Despite pericardiectomy followed by medical treatment, her cardiac symptoms gradually worsened and she died at 51-year old. At autopsy, her heart was smaller than normal person. The residual pericardium and the surface of the myocardium had fibrous degeneration with tight adhesion and thickness that were dominant at the right atrium and ventricle. Likewise, we demonstrated that pericardiectomy scarcely led to a favorable outcome in this patient. These facts might be based on the radiation-associated cardiac effects involving any components of the cardiac structure. PMID:24852447

  10. A Case of Mediastinal Granular Cell Tumor with Horner’s Syndrome

    PubMed Central

    Shikatani, Yoshinobu; Sakao, Nobuhiko; Yukumi, Shungo; Shigematsu, Hisayuki; Kitazawa, Sohei; Sano, Yoshifumi

    2015-01-01

    Granular cell tumor (GCT) is found in various organs but is rare in the mediastinum. We report a case of mediastinal GCT in a 19-year-old woman who presented with left ptosis and miosis. CT and MRI revealed a 29-mm well-circumscribed tumor located close to the first thoracic vertebra with features suggesting a neurogenic tumor. The tumor was completely excised using single-port video-assisted thoracoscopic surgery. Histopathological and immunohistochemical analysis revealed that the tumor was a benign GCT. Postoperatively, left ptosis and miosis had improved slightly. To our knowledge, this is the first report regarding mediastinal GCT presenting with preoperative Horner’s syndrome. PMID:26041255

  11. Best approach for posterior mediastinal goiter removal: transcervical incision and lateral thoracotomy.

    PubMed

    Ojanguren Arranz, Amaya; Baena Fustegueras, Juan Antonio; Ros López, Susana; Santamaría Gómez, Maite; Ojanguren Arranz, Iñigo; Olsina Kissle, Jorge Juan

    2014-06-01

    Surgical removal of intrathoracic goiter can be performed by a cervical approach in the majority of patients. Review of literature shows that experienced surgeons need to perform an extracervical approach in 2-3% of cases. In spite of surgical management of substernal goiter is well defined, there is little available information about surgical approach of intrathoracic goiters extending beyond the aortic arch into the posterior mediastinum. We report two cases and propose combination of cervical incision and muscle-sparing lateral thoracotomy for posterior mediastinal goiter removal. In such cases, we do not favour sternotomy as posterior mediastinum is inaccessible due to the presence of heart and great vessels anterior to the thyroidal mass that would lead to perform a perilous blind dissection. Based in our experience, transcervical and thoracotomy approach is indicated for a complete and safe posterior mediastinal goiter removal.

  12. Diverticula in association with the proximal left subclavian artery: a cause of mediastinal mass lesions.

    PubMed

    Stanford, W; Knauf, D G; Hudson, R E; Tucker, A R

    1979-01-01

    The case reports of 5 patients with an aortic arch diverticulum associated with the proximal left subclavian artery are presented. In 3 patients, the left subclavian artery arose from a diverticulum off a right aortic arch and traversed the retroesophageal area to the opposite arm. In 1 patient, the left subclavian artery arose from a diverticulum off a left aortic arch; this represents the second instance of this anomaly to be reported in the literature. In 1 patient, the left subclavian artery was not attached to the aorta, and a diverticulum was present in the expected area of attachment. Confusion of these anomalies with solid mediastinal mass lesions and with aortic aneurysms may occur. Treatment is predicted on symptomatology, and resection is usually not indicated. It is necessary to remain cognizant of aortic diverticula and to differentiate them from other mediastinal mass lesions.

  13. Mediastinal Yolk Sac Tumor Producing Protein Induced by Vitamin K Absence or Antagonist-II.

    PubMed

    Akutsu, Noriyuki; Adachi, Yasushi; Isosaka, Mai; Mita, Hiroaki; Takagi, Hideyasu; Sasaki, Shigeru; Yamamoto, Hiroyuki; Arimura, Yoshiaki; Ishii, Yoshifumi; Masumori, Naoya; Endo, Takao; Shinomura, Yasuhisa

    2015-01-01

    Extragonadal yolk sac tumors (YSTs) are rare. We herein report the case of a 66-year-old man with mediastinal, lung and liver tumors. The largest mass was located in the liver and contained a high concentration of protein induced by vitamin K absence or antagonist-II (PIVKA-II) and alpha-fetoprotein. Therefore, the lesion was difficult to distinguish from hepatocellular carcinoma. Finally, YST was diagnosed based on the results of a liver biopsy. Although chemotherapy was effective, the patient died of respiratory failure. The autopsy revealed primary mediastinal YST. In the current report, we describe this case of PIVKA-II-producing YST and review previous cases of PIVKA-II-producing tumors other than hepatoma. PMID:26073245

  14. Solitary mediastinal lymph node recurrence after curative resection of colon cancer.

    PubMed

    Matsuda, Yasuhiro; Yano, Masahiko; Miyoshi, Norikatsu; Noura, Shingo; Ohue, Masayuki; Sugimura, Keijiro; Motoori, Masaaki; Kishi, Kentaro; Fujiwara, Yoshiyuki; Gotoh, Kunihito; Marubashi, Shigeru; Akita, Hirofumi; Takahashi, Hidenori; Sakon, Masato

    2014-08-27

    We report two cases of solitary mediastinal lymph node recurrence after colon cancer resection. Both cases had para-aortic lymph node metastasis at the time of initial surgery and received adjuvant chemotherapy for 4 years in case 1 and 18 mo in case 2. The time to recurrence was more than 8 years in both cases. After resection of the recurrent tumor, the patient is doing well with no recurrence for 6 years in case 1 and 4 mo in case 2. Patients should be followed up after colon cancer surgery considering the possibility of solitary mediastinal lymph node recurrence if they had para-aortic node metastasis at the time of initial surgery. PMID:25161766

  15. A 70-Year-Old Man With Large Cervical and Mediastinal Lymphadenopathies

    PubMed Central

    Narechania, Shraddha; Valent, Jason; Farver, Carol

    2015-01-01

    We present a case of a 70-year-old man with enlarged mediastinal and cervical lymph nodes that provided interesting radiologic and pathologic observations. The 70-year-old black man was found to have enlarged mediastinal lymph nodes. He had symptoms of atypical chest pain and generalized weakness for 2 weeks prior to the diagnosis. He denied shortness of breath, fever, chills, or night sweats. He was treated for hypertension and onychomycosis. Basic laboratory findings were within normal limits. Pulmonary function tests at the time of presentation showed FEV1, FVC, and FEV1/FVC ratio of 123% predicted, 133% predicted, and 0.7, respectively. Meanwhile, total lung capacity and carbon monoxide diffusing capacity were 103% and 107% predicted, respectively. Two weeks before he presented to our institution, the patient underwent bronchoscopy with transbronchial biopsies of the right lower lobe and endobronchial ultrasound-guided transbronchial needle aspiration of the right hilar lymph nodes. PMID:26149568

  16. A fatal case of descending necrotizing mediastinitis as a complication of odontogenic infection. A case report

    PubMed Central

    Lewandowski, Bogumił; Wołek, Wojciech; Jednakiewicz, Mariusz; Nicpoń, Jakub

    2014-01-01

    This paper presents the case of a 26-year-old female patient in whom descending necrotizing mediastinitis (DNM) developed as a complication of an odontogenic purulent infection of the mouth. Despite the efforts of a multidisciplinary treatment team, the patient died with symptoms of septic shock and multiple organ failure. According to the literature, and as confirmed by our own observations, successful treatment requires early tomographic diagnosis, radical surgery, combination antibiotic therapy, and intensive care. PMID:26336443

  17. [Anterior Mediastinal Bronchogenic Cyst Associated with paroxysmal supraventricular tachycardia ; Report of a Case].

    PubMed

    Mega, Seiji

    2015-09-01

    We experienced a rare case of anterior mediastinal bronchogenic cyst. A 55-year-old female was admitted to our hospital because of paroxysmal supraventricular tachycardia (PSVT) and an abnormal shadow on the chest computed tomography. She had a 5.5 cm tumor at anterior mediastinum. The tumor was surgically removed completely by video assisted thoracoscopic surgery, and the diagnosis of bronchogenic cyst was established pathologically. After surgery, PSVT has disappeared.

  18. [Aspergillus fumigatus mediastinitis in an immunocompetent pediatric patient after heart surgery].

    PubMed

    Acuña, Mirta; Farfán, Felipe; Cofré, Fernanda; Benadof, Dona

    2016-02-01

    Postsurgical aspergillosis occurs primarily in immunocompetent patients whose main predisposing factor is the loss of skin and mucosal integrity during surgery. Local infection tends to be destructive and refractory to treatment and relapses are common. It is important to consider aspergillosis in the differential diagnosis of slowly progressive and destructive surgical site infections with negative bacterial cultures. We present the case of a child who developed Aspergillus fumigatus mediastinitis months after heart surgery.

  19. Incidentally Detected Mediastinal Vascular Anatomical Variants on PET-CT: A Pictorial Essay.

    PubMed

    Paidipati, Kathyayini Gopalkrishna Murthy; Kashyap, Raghava; Tadimeti, Hima; Peyyeti, Mahidhar

    2015-01-01

    Positron emission tomography with computed tomography (PET-CT) is an integral part of oncology practice in the current-day scenario. The hybrid anatomical and metabolic imaging information in this imaging modality provides access to a wealth of incidental findings which can have implications in the management of the patient, especially when surgical options are being considered. In this pictorial essay, we present incidentally detected mediastinal vascular anomalies on PET-CT, along with a review of associated literature.

  20. Colon cancer metastasis to mediastinal lymph nodes without liver or lung involvement: A case report.

    PubMed

    El-Halabi, Mustapha M; Chaaban, Said A; Meouchy, Joseph; Page, Seth; Salyers, William J

    2014-11-01

    Colon cancer is the second most common type of cancer in females and the third in males, worldwide. The most common sites of colon cancer metastasis are the regional lymph nodes, liver, lung, bone and brain. In this study, an extremely rare case of colon adenocarcinoma with extensive metastasis to the mediastinal lymph nodes without any other organ involvement is presented. A 44-year-old Caucasian male presented with abdominal pain, a change in bowel habits, melena and weight loss. Colonoscopy revealed a large friable, ulcerated, circumferential mass in the ascending colon. Biopsies were consistent with the diagnosis of invasive moderately differentiated adenocarcinoma. Subsequently, right colon resection was performed, and pathological analysis revealed moderately differentiated adenocarcinoma of the right colon with extensive regional lymph node involvement. Computed tomography (CT) scans of the chest, abdomen and pelvis were performed preoperatively as part of routine staging for colon cancer. No liver or lung pathology was identified; however, multiple pathologically enlarged mediastinal lymph nodes were observed. Endoscopic ultrasound with fine needle aspiration of the largest mediastinal lymph node, which measured 5.2×3.5 cm on CT scans, was performed. The pathology was again consistent with the diagnosis of metastatic colorectal primary adenocarcinoma. At present, no optimum treatment has been identified for metastatic colon cancer to the mediastinal lymph nodes. The patient in the current case received chemotherapy with folinic acid, fluorouracil and oxaliplatin (FOLFOX), as well as with bevacizumab. Initial follow-up CT scans of the chest revealed a positive response to treatment. Physicians, in particular, radiologists, must consider the mediastinum during the first evaluation and further follow-up of patients with colorectal carcinoma even in the absence of metastasis. PMID:25289100

  1. Clinical Significance of Serum Biomarkers in Pediatric Solid Mediastinal and Abdominal Tumors

    PubMed Central

    Sandoval, John A.; Malkas, Linda H.; Hickey, Robert J.

    2012-01-01

    Childhood cancer is the leading cause of death by disease among U.S. children between infancy and age 15. Despite successes in treating solid tumors such as Wilms tumor, disappointments in the outcomes of high-risk solid tumors like neuroblastoma have precipitated efforts towards the early and accurate detection of these malignancies. This review summarizes available solid tumor serum biomarkers with a special focus on mediastinal and abdominal cancers in children. PMID:22312308

  2. Thoracoscopic resection of middle mediastinal noninvasive thymoma: report of a case.

    PubMed

    Nakamura, Hiroshige; Adachi, Yoshin; Fujioka, Shinji; Miwa, Ken; Haruki, Tomohiro; Taniguchi, Yuji

    2007-01-01

    We performed thoracoscopic resection of a middle mediastinal noninvasive thymoma in a 69-year-old woman. Chest computed tomography on admission showed a tumor, 75 x 48 x 32 mm in size, and pathological examination revealed a spindle-cell, noninvasive thymoma, of type A according to the World Health Organization classification, and stage I according to the Masaoka staging system. Thymomas are prone to ectopic occurrence, and should be considered in the differential diagnosis of middle mediastinum tumors. PMID:17713734

  3. Concurrent Myelomatous Pleural Effusion and Extramedullary Mediastinal Involvement as an Initial Manifestation of Multiple Myeloma.

    PubMed

    Williams, George; Kadaria, Dipen; Sodhi, Amik

    2016-01-01

    BACKGROUND Myelomatous pleural effusion (MPE) is a rare occurrence in patients with multiple myeloma (MM). Fewer than 20 cases of MPE have been reported as an initial manifestation of MM. Extramedullary plasmacytoma (EMP) occurs in fewer than 5% patients with MM, and mediastinal EMP is even rarer, with only about 80 cases reported in the literature. We present a case study involving a patient with concurrent MPE and mediastinal EMP as an initial manifestation of MM. CASE REPORT The patient was a 74-year-old nonsmoking female with a 3-month history of exertional dyspnea and back pain. On exam, the patient was afebrile (temperature 37.2°C), blood pressure was 160/74 mm Hg, heart rate was 92 bpm, respiratory rate was 22/min, and oxygen saturation was 87% on room air. Patient was in mild distress and had decreased breath sounds over right lung fields about halfway up with dullness to percussion. Computed tomography of the chest showed a moderate-sized right pleural effusion and an anterior mediastinal mass. Thoracentesis showed a lymphocyte-predominant exudate. Cytology showed numerous plasma cells including immature forms. Stains for CD138 were positive, confirming plasma cell origin of cells. The anterior mediastinal mass was also biopsied and showed diffuse infiltrate of lymphocytes with plasma cell features that were also positive for CD138. Systemic protein electrophoresis showed a monoclonal immunoglobulin G kappa spike, and bone marrow biopsy was consistent with MM. CONCLUSIONS MPE and EMP are extremely rare manifestations in MM. In addition, it is extremely rare for these to be the presenting features of MM. We report concurrently occurring MPE and EMP in a patient as her initial manifestation of MM. PMID:27396960

  4. Incidentally Detected Mediastinal Vascular Anatomical Variants on PET-CT: A Pictorial Essay

    PubMed Central

    Paidipati, Kathyayini Gopalkrishna Murthy; Kashyap, Raghava; Tadimeti, Hima; Peyyeti, Mahidhar

    2015-01-01

    Positron emission tomography with computed tomography (PET–CT) is an integral part of oncology practice in the current-day scenario. The hybrid anatomical and metabolic imaging information in this imaging modality provides access to a wealth of incidental findings which can have implications in the management of the patient, especially when surgical options are being considered. In this pictorial essay, we present incidentally detected mediastinal vascular anomalies on PET–CT, along with a review of associated literature. PMID:25883859

  5. Distinguishing benign mediastinal masses from malignancy in a histoplasmosis-endemic region

    PubMed Central

    Naeem, Fouzia; Metzger, Monika L.; Arnold, Sandra R.; Adderson, Elisabeth E.

    2015-01-01

    Objective To describe the characteristics of benign and malignant mediastinal masses, which may predict their etiology and facilitate the safe and timely management of patients, especially those residing in histoplasmosis-endemic regions. Study design We conducted a retrospective review of the health records of 131 patients aged <19 years who were referred to two tertiary care children's hospitals from 2005-2010 for the evaluation of mediastinal masses. Results Most patients (79%) had benign masses, including 98 with confirmed or suspected histoplasmosis. Overall, patients with benign etiologies were younger, more likely to be African American, more likely to complain of cough and to have pulmonary nodules by chest computed tomographs than patients with cancer. Patients with malignant disease were more likely to complain of malaise and to have neck swelling, abnormal extrathoracic lymphadenopathy, lymphopenia, anterior mediastinal involvement and/or pleural effusion. Positive histoplasmosis serologic tests were specific but insensitive for a benign etiology. No single clinical, laboratory or radiologic feature was sufficiently sensitive and specific to distinguish between benign and malignant masses. For cancer, however, the presence of lymphopenia, anterior mediastinal involvement or enlarged cervical lymph nodes on computerized tomography had a sensitivity of 93%, specificity of 95%, positive predictive value of 86%, and negative predictive value of 97% for cancer. Sixty-four patients (49%) underwent invasive testing, including 37 (36%) of patients with benign masses. Conclusions Patients in this series who had involvement of the anterior mediastinum, lymphopenia or enlarged cervical lymph nodes had a high likelihood of cancer. Expectant management of patients lacking these characteristics may be safe and reduce unnecessary invasive testing. PMID:26009018

  6. Concurrent Myelomatous Pleural Effusion and Extramedullary Mediastinal Involvement as an Initial Manifestation of Multiple Myeloma

    PubMed Central

    Williams, George; Kadaria, Dipen; Sodhi, Amik

    2016-01-01

    Patient: Female, 72 Final Diagnosis: Myelomatous pleural effusion Symptoms: Dyspnea Medication: — Clinical Procedure: Thoracentesis Specialty: Pulmonology Objective: Rare disease Background: Myelomatous pleural effusion (MPE) is a rare occurrence in patients with multiple myeloma (MM). Fewer than 20 cases of MPE have been reported as an initial manifestation of MM. Extramedullary plasmacytoma (EMP) occurs in fewer than 5% patients with MM, and mediastinal EMP is even rarer, with only about 80 cases reported in the literature. We present a case study involving a patient with concurrent MPE and mediastinal EMP as an initial manifestation of MM. Case Report: The patient was a 74-year-old nonsmoking female with a 3-month history of exertional dyspnea and back pain. On exam, the patient was afebrile (temperature 37.2°C), blood pressure was 160/74 mm Hg, heart rate was 92 bpm, respiratory rate was 22/min, and oxygen saturation was 87% on room air. Patient was in mild distress and had decreased breath sounds over right lung fields about halfway up with dullness to percussion. Computed tomography of the chest showed a moderate-sized right pleural effusion and an anterior mediastinal mass. Thoracentesis showed a lymphocyte-predominant exudate. Cytology showed numerous plasma cells including immature forms. Stains for CD138 were positive, confirming plasma cell origin of cells. The anterior mediastinal mass was also biopsied and showed diffuse infiltrate of lymphocytes with plasma cell features that were also positive for CD138. Systemic protein electrophoresis showed a monoclonal immunoglobulin G kappa spike, and bone marrow biopsy was consistent with MM. Conclusions: MPE and EMP are extremely rare manifestations in MM. In addition, it is extremely rare for these to be the presenting features of MM. We report concurrently occurring MPE and EMP in a patient as her initial manifestation of MM. PMID:27396960

  7. Genetics of Cystic Fibrosis: Clinical Implications.

    PubMed

    Egan, Marie E

    2016-03-01

    Cystic fibrosis (CF) is a common life-shortening autosomal recessive genetic disorder caused by mutations in the gene that encodes for the cystic fibrosis transmembrane conductance regulator protein (CFTR). Almost 2000 variants in the CFTR gene have been identified. The mutational classes are based on the functional consequences on CFTR. New therapies are being developed to target mutant CFTR and restore CFTR function. Understanding specific CF genotypes is essential for providing state-of-the art care to patients. In addition to the variation in CFTR genotype, there are several modifier genes that contribute to the respiratory phenotype.

  8. Diagnosis of Adult Patients with Cystic Fibrosis.

    PubMed

    Nick, Jerry A; Nichols, David P

    2016-03-01

    The diagnosis of cystic fibrosis (CF) is being made with increasing frequency in adults. Patients with CF diagnosed in adulthood typically present with respiratory complaints, and often have recurrent or chronic airway infection. At the time of initial presentation individuals may appear to have clinical manifestation limited to a single organ, but with subclinical involvement of the respiratory tract. Adult-diagnosed patients have a good response to CF center care, and newly available cystic fibrosis transmembrane receptor-modulating therapies are promising for the treatment of residual function mutation, thus increasing the importance of the diagnosis in adults with unexplained bronchiectasis.

  9. Cystic dilation of the ventriculus terminalis

    PubMed Central

    Kawanishi, Masahiro; Tanaka, Hidekazu; Yokoyama, Kunio; Yamada, Makoto

    2016-01-01

    The ventriculus terminalis (VT) is a virtual cavity of the conus medullaris that appears during embryonic life. We presented a case with the cystic dilation of the ventriculus terminalis (CDVT) in a symptomatic patient. A 66-year-old female suffered from disturbance while walking evolving for the past 2 years. An MR image revealed a cystic dilatation of ventriculus terminalis. The patient experienced marked improvement of lower extremity strength by a fenestration of cyst and cyst-subarachnoid shunt. Treatment for asymptomatic patients seems to be the best conducted conservatively, whereas patients with focal neurological deficits seem to be best handled surgically. PMID:27695242

  10. Cystic dilation of the ventriculus terminalis

    PubMed Central

    Kawanishi, Masahiro; Tanaka, Hidekazu; Yokoyama, Kunio; Yamada, Makoto

    2016-01-01

    The ventriculus terminalis (VT) is a virtual cavity of the conus medullaris that appears during embryonic life. We presented a case with the cystic dilation of the ventriculus terminalis (CDVT) in a symptomatic patient. A 66-year-old female suffered from disturbance while walking evolving for the past 2 years. An MR image revealed a cystic dilatation of ventriculus terminalis. The patient experienced marked improvement of lower extremity strength by a fenestration of cyst and cyst-subarachnoid shunt. Treatment for asymptomatic patients seems to be the best conducted conservatively, whereas patients with focal neurological deficits seem to be best handled surgically.

  11. Idiopathic cystic artery aneurysm complicated with hemobilia.

    PubMed

    Anand, Utpal; Thakur, Sanjeev Kumar; Kumar, Sanjay; Jha, Achyutanand; Prakash, Vijay

    2011-01-01

    Aneurysm of the cystic artery is not common, and it is a rare cause of hemobilia. Most of reported cases are pseudoaneurysms resulting from either an inflammatory process in the abdomen or abdominal trauma. We report a healthy individual who developed hemobilia associated with cystic artery aneurysm. The patient was managed with cholecystectomy and concomitant aneurysm repair. Visceral artery aneurysms are rare and can rupture with potentially grave outcome due to excessive bleeding. Angiographic embolization is a common method of treatment for visceral artery aneurysms. Open cholecystectomy and aneurysm repair was performed in our patient due to radiological evidence of associated cholecystitis.

  12. Cystic renal cell carcinoma and acquired renal cystic disease associated with consumption of chaparral tea: a case report.

    PubMed

    Smith, A Y; Feddersen, R M; Gardner, K D; Davis, C J

    1994-12-01

    Nordihydroguaiaretic acid is an antioxidant used experimentally to induce cystic renal disease in rats. It may be extracted from the leaves of the creosote bush, which are consumed as chaparral tea in the southwestern United States. We report a case of cystic renal disease and cystic adenocarcinoma of the kidney associated with a history of protracted consumption of chaparral tea.

  13. Rationale for and Preliminary Results of Proton Beam Therapy for Mediastinal Lymphoma

    SciTech Connect

    Li Jing; Dabaja, Bouthaina; Reed, Valerie; Allen, Pamela K.; Cai, Haihong; Amin, Mayankkumar V.; Garcia, John A.; Cox, James D.

    2011-09-01

    Purpose: To evaluate the potential of three-dimensional proton beam therapy (3D-PBT) for reducing doses to normal structures in patients with mediastinal lymphomas compared with conventional photon radiation therapy (RT). Methods and Materials: We treated 10 consecutive patients with mediastinal masses from lymphomas with 3D-PBT between July 2007 and February 2009 to 30.6-50.4 cobalt-Gray equivalents (CGE). Of those patients, 7 had primary refractory or recurrent disease, and 8 had Hodgkin lymphoma. Dosimetric endpoints were compared with those from conventional RT plans. Results: PBT delivered lower mean doses to the lung (6.2 vs. 9.5 Gy), esophagus (9.5 vs. 22.3 Gy), and heart (8.8 vs. 17.7 Gy) but not the breasts (5.9 vs. 6.1 Gy) than did conventional RT. Percentages of lung, esophagus, heart, and coronary artery (particularly the left anterior descending artery) volumes receiving radiation were consistently lower in the 3D-PBT plans over a wide range of radiation doses. Of the 7 patients who had residual disease on positron emission tomography before PBT, 6 (86%) showed a complete metabolic response. Conclusions: In patients with mediastinal lymphomas, 3D-PBT produced significantly lower doses to the lung, esophagus, heart, and coronary arteries than did the current conventional RT. These lower doses would be expected to reduce the risk of late toxicities in these major organs.

  14. An unusual recurrent bilateral posterior mediastinal goiter after subtotal thyroidectomy: Case report

    PubMed Central

    Aziret, Mehmet; Topçuoğlu, Mehmet Şah; Özçelik, Cemal; Özkaya, Muharrem

    2014-01-01

    INTRODUCTION Surgical treatment of benign thyroid diseases need to be followed up closely, since recurrent thyroid nodules can be seen after subtotal thyroidectomy. Intrathoracic goiter (ITG) occurs in 10–30% of patients following subtotal thyroidectomy. In general these goiters are benign, having a malignant rate of only 2–22%. ITG grows slowly but steadily and in its process of development, it narrows the thoracic inlet by compressing the surrounding structures. Most of these can not located in the anterior mediastinum, others located in posterior retrovascular area. Bilateral posterior retrovascular goiters are very rare. PRESENTATION OF CASE We report a case involving a 61-year-old woman with history of gradual-onset dyspnea who was referred to us for evaluation of a large mediastinal mass. She had undergone bilateral thyroid lobectomy for a cervical goiter 10 years ago. The mass was removed successfully via median sternotomy without complication. The patient recovered well and was discharged in 1 week. DISCUSSION Most anterior mediastinal goiters can be resected through a transcervical approach, but if those extending beyond the aortic arch into the posterior mediastinum are better dealt with by sternotomy or lateral thoracotomy. CONCLUSION Bilateral recurrent posterior mediastinal and retrovascular large goiters are better resected via sternotomy rather than lateral thoracotomy. The reason for that are the possibility of injury to large vascular structures and the difficulty of their management through lateral thoracotomy when cardiopulmonary bypass needed. PMID:24973529

  15. An anterior mediastinal lesion in TAFRO syndrome showing complete remission after glucocorticoid and tocilizumab therapy.

    PubMed

    Sakashita, Kentaro; Murata, Kengo; Inagaki, Yuji; Oota, Souichi; Takamori, Mikio

    2016-09-01

    Thrombocytopenia (T), anasarca (A), myelofibrosis (F), renal dysfunction (R), and organomegaly (O) (TAFRO) syndrome is a variant of multicentric Castleman's disease. We describe here a 57-year-old man who presented with persistent fever, pleural effusion, and ascites. He was negative for human immunodeficiency virus and human herpes virus-8. A computed tomography scan showed an anterior mediastinal mass and small inguinal lymphadenopathy. Although a biopsy of the anterior mediastinum showed fatty tissue infiltrated with CD20 (+) and CD45RO (+) lymphocytes, a biopsy of the left inguinal lymph node revealed a hyaline vascular type of Castleman's disease. He subsequently developed severe thrombocytopenia and renal dysfunction. In addition, his bone marrow biopsy showed myelofibrosis. TAFRO syndrome was diagnosed based on the lymph node pathology and the characteristic manifestations of the syndrome. Tocilizumab and glucocorticoid therapy achieved complete remission and regression of the mediastinal mass. To our knowledge, this is the first report of TAFRO syndrome accompanied by an anterior mediastinal mass, which responded very well to therapy. PMID:27516889

  16. Diagnostic yield of endobronchial ultrasound-guided transbronchial needle aspiration for mediastinal staging in lung cancer*

    PubMed Central

    Fernández-Bussy, Sebastián; Labarca, Gonzalo; Canals, Sofia; Caviedes, Iván; Folch, Erik; Majid, Adnan

    2015-01-01

    OBJECTIVE: Endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) is a minimally invasive diagnostic test with a high diagnostic yield for suspicious central pulmonary lesions and for mediastinal lymph node staging. The main objective of this study was to describe the diagnostic yield of EBUS-TBNA for mediastinal lymph node staging in patients with suspected lung cancer. METHODS: Prospective study of patients undergoing EBUS-TBNA for diagnosis. Patients ≥ 18 years of age were recruited between July of 2010 and August of 2013. We recorded demographic variables, radiological characteristics provided by axial CT of the chest, location of the lesion in the mediastinum as per the International Association for the Study of Lung Cancer classification, and definitive diagnostic result (EBUS with a diagnostic biopsy or a definitive diagnostic method). RESULTS: Our analysis included 354 biopsies, from 145 patients. Of those 145 patients, 54.48% were male. The mean age was 63.75 years. The mean lymph node size was 15.03 mm, and 90 lymph nodes were smaller than 10.0 mm. The EBUS-TBNA method showed a sensitivity of 91.17%, a specificity of 100.0%, and a negative predictive value of 92.9%. The most common histological diagnosis was adenocarcinoma. CONCLUSIONS: EBUS-TBNA is a diagnostic tool that yields satisfactory results in the staging of neoplastic mediastinal lesions. PMID:26176519

  17. Total mechanical stapled oesophagogastric anastomosis on the neck in oesophageal cancer - prevention of postoperative mediastinal complications.

    PubMed

    Zieliński, Jacek; Jaworski, Radosław; Irga-Jaworska, Ninela; Haponiuk, Ireneusz; Jaśkiewicz, Janusz

    2015-12-01

    Oesophagogastric anastomosis after oesophagus resection is commonly performed on the neck. Even though a few different techniques of oesophagogastric anastomosis have been previously detailed, both manual and mechanical procedures have been burdened with leakages and strictures. Our simple technique of oesophagogastric anastomosis is a modification of mechanical anastomosis with the use of a circular stapler in order to prevent postoperative leak and concomitant mediastinal complications. Since 2008, we have performed nine oesophagogastric anastomoses following oesophagus resection. The mean age of the operated patients was 54 years. There was no mortality among the operated patients in the early post-operative period. The mean follow-up period for the patients operated on in our department was 17 months until the time of the analysis. None of the patients showed any leakage or stricture, and no mediastinal complications were reported in the group. Following our own experience, mechanical anastomosis with the use of a circular stapler seems to decrease the time of the operation as well as significantly reducing the incidence of leakages from the anastomosis. This type of anastomosis may decrease the number of postoperative strictures and the most dangerous mediastinal infections.

  18. A Case of Nocardia farcinica Pneumonia and Mediastinitis in an Immunocompetent Patient

    PubMed Central

    Kim, Jinyoung; Kang, Minkyu; Kim, Juri; Jung, Sohee; Park, Junhung; Lee, Dongkyu

    2016-01-01

    Nocardia species are aerobic, gram-positive pathogens found worldwide in soil. Nocardia is considered an opportunistic pathogen, and its infection mostly occurs in immunocompromised patients. We report a case of Nocardia farcinica induced mediastinitis and pneumonia that occurred in a 64-year-old male patient who had no significant medical history except for hypertension. He visited another hospital with a complaint of dyspnea and left chest wall pain. The symptoms arose 7 days ago without any trauma and they worsened. A mediastinal mass was found on computed tomography scan. After being transferred to our hospital for further evaluation, he was diagnosed with mediastinitis and pneumonia. As N. farcinica was found to be the causative organism by 16S rRNA sequencing, proper antibiotic therapy including trimethoprim/sulfamethoxazole was initiated immediately. After this, the patient improved and he was discharged. If an infection has a disseminating course, nocardiosis cannot be excluded even in immunocompetent patients. Once the diagnosis is established, prompt antibiotic therapy should be performed based on the severity. PMID:27066088

  19. Complete atrioventricular block following mediastinal irradiation: A report of six cases

    SciTech Connect

    Slama, M.S.; Le Guludec, D.; Sebag, C.; Leenhardt, A.R.; Davy, J.M.; Pellerin, D.E.; Drieu, L.H.; Victor, J.; Brechenmacher, C.; Motte, G. )

    1991-07-01

    Complete atrioventricular block (AVB) following radiotherapy has been reported rarely, usually after high dose mediastinal irradiation for Hodgkin's disease or lung or breast carcinoma. The authors report six new cases of episodic complete infranodal AVB, requiring permanent pacemaker implantation. The mean age was 48-years old (ranging from 25-60) at the first Adams Stokes attack, mean delay was 12 years after irradiation (10-18), and mean radiation dose was 5,200 rads (4,000-6,500). All patients had abnormal interval electrocardiograms (right bundle branch block in two, left bundle branch block in three, alternating left and right bundle branch block in one). Electrocardiograms during the episode of AVB or Holter recordings were consistent with infranodal block in all patients; electrophysiological study performed in five patients confirmed infranodal AVB in four, and one was normal. Pericardial disease was constant, which included pericardial constriction in four patients. Two patients died after failure of pericardiectomy to improve congestive heart failure, due to epicardial, myocardial, and endocardial involvement. Noncardiac mediastinal lesions were present in four cases. Since this delayed complication may occur in patients of such age that the relation between the AVB and the chest irradiation is questionable, they propose the following etiologic criteria; high radiation dose (over 4,000 rads); delay of 10 years or more; abnormal interval tracings; pericardial involvement; and associated cardiac or mediastinal radiation-induced lesions.

  20. Proposal for a recovery prediction method for patients affected by acute mediastinitis

    PubMed Central

    2012-01-01

    Background An attempt to find a prediction method of death risk in patients affected by acute mediastinitis. There is not such a tool described in available literature for that serious disease. Methods The study comprised 44 consecutive cases of acute mediastinitis. General anamnesis and biochemical data were included. Factor analysis was used to extract the risk characteristic for the patients. The most valuable results were obtained for 8 parameters which were selected for further statistical analysis (all collected during few hours after admission). Three factors reached Eigenvalue >1. Clinical explanations of these combined statistical factors are: Factor1 - proteinic status (serum total protein, albumin, and hemoglobin level), Factor2 - inflammatory status (white blood cells, CRP, procalcitonin), and Factor3 - general risk (age, number of coexisting diseases). Threshold values of prediction factors were estimated by means of statistical analysis (factor analysis, Statgraphics Centurion XVI). Results The final prediction result for the patients is constructed as simultaneous evaluation of all factor scores. High probability of death should be predicted if factor 1 value decreases with simultaneous increase of factors 2 and 3. The diagnostic power of the proposed method was revealed to be high [sensitivity =90%, specificity =64%], for Factor1 [SNC = 87%, SPC = 79%]; for Factor2 [SNC = 87%, SPC = 50%] and for Factor3 [SNC = 73%, SPC = 71%]. Conclusion The proposed prediction method seems a useful emergency signal during acute mediastinitis control in affected patients. PMID:22574625

  1. Gray zone lymphoma: better treated like hodgkin lymphoma or mediastinal large B-cell lymphoma?

    PubMed

    Dunleavy, Kieron; Grant, Cliona; Eberle, Franziska C; Pittaluga, Stefania; Jaffe, Elaine S; Wilson, Wyndham H

    2012-09-01

    Although primary mediastinal large B-cell lymphoma (PMBL) and classic Hodgkin lymphoma of the nodular sclerosis type (CHL-NS) are distinct diseases, they share several clinical characteristics and biologic features. Given that, it is not surprising that there exist mediastinal lymphomas that do not fit well into either category but have clinical and morphologic features overlapping and transitional between PMBL and CHL-NS. The term mediastinal gray zone lymphoma (MGZL) has been used for these tumors, which are included in the World Health Organization classification as "B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and classic Hodgkin lymphoma." Although several studies have evaluated different therapeutic strategies in PMBL and CHL-NS, there is a paucity of prospective experience treating MGZL, given its rarity and relatively recent recognition. Historically, diseases that today would be categorized as MGZL were probably called "anaplastic large-cell lymphoma Hodgkin-like," and their outcome with standard approaches was poor, with short overall survivals. In this review-following a discussion of the biology and clinical features of MGZL, and how they compare to PMBL and CHL-NS-we outline how the treatment of PMBL and CHL-NS has evolved in recent years, and how we believe MGZL should be approached therapeutically. PMID:22833351

  2. Treatment outcomes of postoperative mediastinitis in cardiac surgery; negative pressure wound therapy versus conventional treatment

    PubMed Central

    2012-01-01

    Background The aim of the present study is to compare negative pressure wound therapy versus conventional treatment outcomes at postoperative mediastinitis after cardiac surgery. Methods Between January 2000 and December 2011, after 9972 sternotomies, postoperative mediastinitis was diagnosed in 90 patients. The treatment modalities divided the patients into two groups: group 1 patients (n = 47) were initially treated with the negative pressure wound therapy and group 2 patients (n = 43) were underwent conventional treatment protocols. The outcomes were investigated with Kaplan-Meier method, log-rank test, Student’s test and Fisher’s exact test. Results The 90-days mortality was found significantly lower in the negative pressure wound group than in the conventionally treated group. Overall survival was significantly better in the negative pressure wound group than in the conventionally treated group. Conclusion Negative pressure wound therapy is safe and reliable option in mediastinitis after cardiac surgery, with excellent survival and low failure rate when compared with conventional treatments. PMID:22784512

  3. Primary malignant mediastinal germ cell tumours: improved prognosis with platinum-based chemotherapy and surgery.

    PubMed Central

    Childs, W. J.; Goldstraw, P.; Nicholls, J. E.; Dearnaley, D. P.; Horwich, A.

    1993-01-01

    A retrospective analysis was performed of 18 patients with primary malignant germ cell tumours of the mediastinum treated with platinum-based chemotherapy between 1977 and 1990. All seven patients with pure seminoma were treated initially with chemotherapy and four of these patients received additional mediastinal radiotherapy. Only one patient relapsed; his initial therapy had included radiotherapy and single-agent carboplatin and he was successfully salvaged with combination chemotherapy. With a follow-up of 11 to 117 months (median 41 months) all seven patients with seminoma remain alive and disease free giving an overall survival of 100%. Eleven patients had malignant non seminoma; following chemotherapy eight of these had elective surgical resection of residual mediastinal masses. Complete remission was achieved in nine (82%) patients, however, one of these patients died from bleomycin pneumonitis. With a follow-up of 12 to 113 months (median 55 months) eight of 11 (73%) patients with malignant mediastinal teratoma remain alive and disease free. PMID:8494705

  4. Detection of cystic structures using pulsed ultrasonically induced resonant cavitation

    NASA Technical Reports Server (NTRS)

    Bar-Cohen, Yoseph (Inventor); Kovach, John S. (Inventor)

    2002-01-01

    Apparatus and method for early detection of cystic structures indicative of ovarian and breast cancers uses ultrasonic wave energy at a unique resonance frequency for inducing cavitation in cystic fluid characteristic of cystic structures in the ovaries associated with ovarian cancer, and in cystic structures in the breast associated with breast cancer. Induced cavitation bubbles in the cystic fluid implode, creating implosion waves which are detected by ultrasonic receiving transducers attached to the abdomen of the patient. Triangulation of the ultrasonic receiving transducers enables the received signals to be processed and analyzed to identify the location and structure of the cyst.

  5. Mediastinal ectopic benign colloid goitre detected using iodine-131 whole body scintigraphy and single-photon emission computed tomography-computed tomography.

    PubMed

    Kamaleshwaran, Koramadai Karuppusamy; Rajan, Firoz; Asokumar, Premkumar; Mohanan, Vyshak; Shinto, Ajit Sugunan

    2015-01-01

    Ectopic thyroid tissue primarily occurs along the course of the embryologic migration of the thyroid gland. Mediastinal thyroid may be differentiated into primary and secondary form. Primary mediastinal goiters are quite rare, occurring in <1% of all goiters. We present findings of 29-year-old female, case of papillary carcinoma of the thyroid who underwent iodine-131 whole body scintigraphy after total thyroidectomy showed a primary mediastinal mass lesion along with residual thyroid. She underwent mediastinal mass excision through sternotomy and biopsy confirmed as ectopic nodular colloid goiter with no malignancy.

  6. Mediastinal ectopic benign colloid goitre detected using iodine-131 whole body scintigraphy and single-photon emission computed tomography-computed tomography

    PubMed Central

    Kamaleshwaran, Koramadai Karuppusamy; Rajan, Firoz; Asokumar, Premkumar; Mohanan, Vyshak; Shinto, Ajit Sugunan

    2015-01-01

    Ectopic thyroid tissue primarily occurs along the course of the embryologic migration of the thyroid gland. Mediastinal thyroid may be differentiated into primary and secondary form. Primary mediastinal goiters are quite rare, occurring in <1% of all goiters. We present findings of 29-year-old female, case of papillary carcinoma of the thyroid who underwent iodine-131 whole body scintigraphy after total thyroidectomy showed a primary mediastinal mass lesion along with residual thyroid. She underwent mediastinal mass excision through sternotomy and biopsy confirmed as ectopic nodular colloid goiter with no malignancy. PMID:25829745

  7. Zinc supplementation in children with cystic fibrosis

    Technology Transfer Automated Retrieval System (TEKTRAN)

    Cystic fibrosis (CF) leads to malabsorption of macro- and micronutrients. Symptomatic zinc deficiency has been reported in CF but little is known about zinc homeostasis in children with CF. Zinc supplementation (Zn suppl) is increasingly common in children with CF but it is not without theoretcial r...

  8. Nutritional assessment in children with cystic fibrosis

    Technology Transfer Automated Retrieval System (TEKTRAN)

    Optimal nutrition, including consuming 35–40% of calories (kcal) as fat, is a vital part of the management of cystic fibrosis (CF), and involves accurate assessment of dietary intake. We compared 3 methods of nutritional assessment in 8– to 14-year-old children (n=20) with CF: 1) a 24-h Dietary Reca...

  9. Cystic lesion around the hip joint

    PubMed Central

    Yukata, Kiminori; Nakai, Sho; Goto, Tomohiro; Ikeda, Yuichi; Shimaoka, Yasunori; Yamanaka, Issei; Sairyo, Koichi; Hamawaki, Jun-ichi

    2015-01-01

    This article presents a narrative review of cystic lesions around the hip and primarily consists of 5 sections: Radiological examination, prevalence, pathogenesis, symptoms, and treatment. Cystic lesions around the hip are usually asymptomatic but may be observed incidentally on imaging examinations, such as computed tomography and magnetic resonance imaging. Some cysts may enlarge because of various pathological factors, such as trauma, osteoarthritis, rheumatoid arthritis, or total hip arthroplasty (THA), and may become symptomatic because of compression of surrounding structures, including the femoral, obturator, or sciatic nerves, external iliac or common femoral artery, femoral or external iliac vein, sigmoid colon, cecum, small bowel, ureters, and bladder. Treatment for symptomatic cystic lesions around the hip joint includes rest, nonsteroidal anti-inflammatory drug administration, needle aspiration, and surgical excision. Furthermore, when these cysts are associated with osteoarthritis, rheumatoid arthritis, and THA, primary or revision THA surgery will be necessary concurrent with cyst excision. Knowledge of the characteristic clinical appearance of cystic masses around the hip will be useful for determining specific diagnoses and treatments. PMID:26495246

  10. Intra-mandibular adenoid cystic carcinoma.

    PubMed

    Bouaichi, A; Aimad-Eddine, S; Mommers, X-A; Ella, B; Zwetyenga, N

    2014-04-01

    Intra-mandibular localization of adenoid cystic carcinoma is rare. This tumor is characterized by progressive local, regional, and distant aggressiveness. We reviewed the latest data on this rare type of cancer with a small number of reported cases, alack of consensus for its treatment, and its bad prognosis.

  11. A cystic fibrosis handbook for teachers.

    PubMed

    Ryan, L L; Williams, J K

    1996-01-01

    The purposes of this project were to (1) develop a handbook on cystic fibrosis for elementary school teachers and to (2) pilot this handbook with a group of teachers and school nurses. The project used a descriptive survey design in which parents, teachers, and school nurses of 14 elementary-age children with cystic fibrosis were recruited from one cystic fibrosis clinic. Interest in using the handbook with their child's teachers was elicited from parents; also, interest in using the handbook was obtained by open-ended questions in a mailed survey sent to teachers and school nurses. Levels of teacher and school nurse knowledge were measured with a true/false pretest and posttest instrument. All parents expressed a desire to use the handbook with their child's teachers. Sixty-seven percent of the teachers and 89% of the school nurses returned the survey, and all endorsed the use of the handbook in their classrooms or schools. Comparison of the pretest and posttest scores from the teachers revealed an increase in teachers' knowledge. Scores on pretest and posttest measures from school nurses were high at each testing time. Results support the use of a printed handbook to promote knowledge of cystic fibrosis in teachers and to support communication among nurses, parents, and teachers.

  12. Diabetes mellitus in patients with cystic fibrosis.

    PubMed

    Alves, Crésio de Aragão Dantas; Aguiar, Renata Arruti; Alves, Ana Cláudia S; Santana, Maria Angélica

    2007-01-01

    Cystic fibrosis-related diabetes (CFRD) is the principal extra-pulmonary complication of cystic fibrosis, occurring in 15-30% of adult cystic fibrosis patients. The number of cystic fibrosis patients who develop diabetes is increasing in parallel with increases in life expectancy. The aim of this study was to review the physiopathology, clinical presentation, diagnosis and treatment of CFRD. A bibliographic search of the Medline and Latin American and Caribbean Health Sciences Literature databases was made. Articles were selected from among those published in the last twenty years. Insulin deficiency, caused by reduced beta-cell mass, is the main etiologic mechanism, although insulin resistance also plays a role. Presenting features of type 1 and type 2 diabetes, CFRD typically affects individuals of approximately 20 years of age. It can also be accompanied by fasting, non-fasting or intermittent hyperglycemia. Glucose intolerance is associated with worsening of nutritional status, increased morbidity, decreased survival and reduced pulmonary function. Microvascular complications are always present, although macrovascular complications are rarely seen. An oral glucose tolerance test is recommended annually for patients > or = 10 years of age and for any patients presenting unexplained weight loss or symptoms of diabetes. Patients hospitalized with severe diseases should also be screened. If fasting hyperglycemia persists for more than 48 h, insulin therapy is recommended. Insulin administration remains the treatment of choice for diabetes and fasting hyperglycemia. Calories should not be restricted, and patients with CFRD should be managed by a multidisciplinary team. PMID:17724542

  13. Nutrient Status of Adults with Cystic Fibrosis

    PubMed Central

    GORDON, CATHERINE M.; ANDERSON, ELLEN J.; HERLYN, KAREN; HUBBARD, JANE L.; PIZZO, ANGELA; GELBARD, RONDI; LAPEY, ALLEN; MERKEL, PETER A.

    2011-01-01

    Nutrition is thought to influence disease status in patients with cystic fibrosis (CF). This cross-sectional study sought to evaluate nutrient intake and anthropometric data from 64 adult outpatients with cystic fibrosis. Nutrient intake from food and supplements was compared with the Dietary Reference Intakes for 16 nutrients and outcomes influenced by nutritional status. Attention was given to vitamin D and calcium given potential skeletal implications due to cystic fibrosis. Measurements included weight, height, body composition, pulmonary function, and serum metabolic parameters. Participants were interviewed about dietary intake, supplement use, pulmonary function, sunlight exposure, and pain. The participants’ mean body mass index (±standard deviation) was 21.8±4.9 and pulmonary function tests were normal. Seventy-eight percent used pancreatic enzyme replacement for malabsorption. Vitamin D deficiency [25-hydroxyvitamin D (25OHD)<37.5 nmol/L] was common: 25 (39%) were deficient despite adequate vitamin D intake. Lipid profiles were normal in the majority, even though total and saturated fat consumption represented 33.0% and 16.8% of energy intake, respectively. Reported protein intake represented 16.9% of total energy intake (range 10%–25%). For several nutrients, including vitamin D and calcium, intake from food and supplements in many participants exceeded recommended Tolerable Upper Intake Levels. Among adults with cystic fibrosis, vitamin D deficiency was common despite reported adequate intake, and lipid profiles were normal despite a relatively high fat intake. Mean protein consumption was adequate, but the range of intake was concerning, as both inadequate or excessive intake may have deleterious skeletal effects. These findings call into question the applicability of established nutrient thresholds for patients with cystic fibrosis. PMID:18060897

  14. Systematic Endobronchial Ultrasound-guided Mediastinal Staging Versus Positron Emission Tomography for Comprehensive Mediastinal Staging in NSCLC Before Radical Radiotherapy of Non-small Cell Lung Cancer: A Pilot Study.

    PubMed

    Steinfort, Daniel P; Siva, Shankar; Leong, Tracy L; Rose, Morgan; Herath, Dishan; Antippa, Phillip; Ball, David L; Irving, Louis B

    2016-02-01

    Despite known limitations of positron emission tomography (PET) for mediastinal staging of non-small cell lung cancer (NSCLC), radiation treatment fields are generally based on PET-identified disease extent. However, no studies have examined the accuracy of FDG-PET/CT on a per-node basis in patients being considered for curative-intent radiotherapy in NSCLC.In a prospective trial, patients with NSCLC being considered for definitive thoracic radiotherapy (± systemic chemotherapy) underwent minimally invasive systematic mediastinal evaluation with endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) following noninvasive staging with integrated PET-CT.Thirty patients underwent EBUS-TBNA, with TBNA performed from a mean 2.5 lymph node (LN) stations per patient (median 3, range 1-5). Discordant findings between PET-CT and EBUS-TBNA were observed in 10 patients (33%, 95% CI 19%-51%). PET-occult LN metastases were demonstrated by EBUS in 4 patients, whereas a lesser extent of mediastinal involvement, compared with FDG-PET, was demonstrated by EBUS in 6 patients, including 2 patients downstaged from cN3 to pN2. LNs upstaged by EBUS were significantly smaller than nodes downstaged by EBUS, 7.5 mm (range 7-9) versus 12 mm (range 6-21), P = 0.005.A significant proportion of patients considered for definitive radiotherapy (+/-chemotherapy) undergoing systematic mediastinal evaluation with EBUS-TBNA in this study have an extent of mediastinal NSCLC involvement discordant with that indicated by PET-CT. Systematic EBUS-TBNA may aid in defining the extent of mediastinal involvement in NSCLC patients undergoing radiotherapy. Systematic EBUS-TBNA has the potential to contribute significantly to radiotherapy planning and delivery, by either identifying occult nodal metastases, or demonstrating FDG-avid LNs to be disease-free. PMID:26937894

  15. Response of the mediastinal and thoracic viscera of the dog to intraoperative radiation therapy (IORT)

    SciTech Connect

    Barnes, M.; Pass, H.; DeLuca, A.; Tochner, Z.; Potter, D.; Terrill, R.; Sindelar, W.F.; Kinsella, T.J.

    1987-03-01

    IORT may be a potentially useful adjunctive treatment combined with surgery and/or external beam irradiation in treating locally advanced lung and esophageal tumors. To begin investigation of this modality, the tolerance of intact mediastinal structures to IORT was studied using adult American Foxhounds (wt. 25-30 kg). Groups of six animals received IORT to doses of 20, 30, or 40 Gy to two separate intrathoracic ports, using 9 MeV electrons to treat a portion of the collapsed right upper lobe, and 12 MeV electrons to treat the mediastinal structures. A group of three dogs received thoracotomy with sham irradiation. Two dogs from each treatment dose group, as well as one sham-irradiated control, were sacrificed electively at 1, 3, and 12 months following IORT. There were no acute nor late IORT related mortalities. Post-operative weight loss was minimal (average 4.5% of pre-operative weight) for all dogs. Serial esophagrams showed no inflammation or ulceration. No cardiac nor pulmonary changes were noted clinically. At autopsy, the irradiated lung showed evidence of acute pneumonitis at 1 month with progressive fibrosis at 3 months and 1 year. Esophageal reactions were minimal, with only two dogs (one 30 Gy and one 40 Gy) demonstrating histologically confirmed esophagitis at 1 month. Tracheal changes were minimal. Cardiac damage was evident in the right atrial tissues. In several dogs, this cardiac damage ranged from myocardial vascular changes to frank ischemic necrosis noted at 1 and 3 months, and dense fibrosis at 1 year. The phrenic nerves showed normal function, but had evidence of perineural fibrosis. The large vessels demonstrated only mild histologic evidence of irradiation. The results of this large animal study suggest that intact mediastinal structures will tolerate small volume IORT to doses of 20 Gy without significant clinical sequellae. (Abstract Truncated)

  16. Primary mediastinal large B-cell lymphoma: a single-center experience in Korea

    PubMed Central

    Ahn, Heui June; Yoon, Dok-Hyun; Kim, Shin; Lee, Kyoungmin; Kang, EunHee; Huh, Jooryung; Park, Chan-Sik

    2014-01-01

    Background Primary mediastinal large B-cell lymphoma (PMBL) is a distinct subtype of non-Hodgkin lymphoma, which has no consensus for its ideal treatment or prognosis. Methods We reviewed the clinicopathologic features and clinical outcomes of 25 PMBL cases diagnosed at a single institution between 1993 and 2009 and compared them with 588 cases of non-mediastinal, diffuse large B-cell lymphoma (DLBCL, control group) diagnosed during the same period. Results Thirteen (52.0%) PMBL patients had Ann Arbor stage III or IV disease, and 10 (40.0%) had B symptoms. Thirteen (52%) PMBL patients were classified as high-intermediate/high-risk according to the International Prognostic Index. There was a significant prevalence of young (median: 31 years; range, 15-78 years; P<0.001), female (68%; P=0.014) patients in the PMBL group compared to the control group (median: 56 years; range, 15-85 years; 43.2% female). Bulky disease and elevated levels of lactate dehydrogenase (LDH) were more frequent in the PMBL group (P<0.001 and P=0.003, respectively). Nineteen (76%) PBML patients achieved complete remission, and 18 were alive at the last follow-up (median: 43 months; range, 1-92 months). There was no difference in the 3-year, overall survival rate (72%, 95% confidence interval [CI]: 54.0-83.0 versus 70.1%, 95% CI, 109.0-126.0; P=0.686) between PMBL and control patients, respectively. Conclusion Compared to patients with non-mediastinal DLBCL, Korean patients with PMBL are predominantly young women with bulky disease and high LDH levels but with no significant difference in survival. PMID:24724065

  17. Cardiac Magnetic Resonance Imaging Findings in 20-year Survivors of Mediastinal Radiotherapy for Hodgkin's Disease

    SciTech Connect

    Machann, Wolfram; Beer, Meinrad; Breunig, Margret; Stoerk, Stefan; Angermann, Christiane; Seufert, Ines; Schwab, Franz; Koelbl, Oliver; Flentje, Michael; Vordermark, Dirk

    2011-03-15

    Purpose: The recognition of the true prevalence of cardiac toxicity after mediastinal radiotherapy requires very long follow-up and a precise diagnostic procedure. Cardiac magnetic resonance imaging (MRI) permits excellent quantification of cardiac function and identification of localized myocardial defects and has now been applied to a group of 20-year Hodgkin's disease survivors. Methods and materials: Of 143 patients treated with anterior mediastinal radiotherapy (cobalt-60, median prescribed dose 40 Gy) for Hodgkin's disease between 1978 and 1985, all 53 survivors were invited for cardiac MRI. Of those, 36 patients (68%) presented for MRI, and in 31 patients (58%) MRI could be performed 20-28 years (median, 24) after radiotherapy. The following sequences were acquired on a 1.5-T MRI: transversal T1-weighted TSE and T2-weighted half-fourier acquisition single-shot turbo-spin-echo sequences, a steady-state free precession (SSFP) cine sequence in the short heart axis and in the four-chamber view, SSFP perfusion sequences under rest and adenosine stress, and a SSFP inversion recovery sequence for late enhancement. The MRI findings were correlated with previously reconstructed doses to cardiac structures. Results: Clinical characteristics and reconstructed doses were not significantly different between survivors undergoing and not undergoing MRI. Pathologic findings were reduced left ventricular function (ejection fraction <55%) in 7 (23%) patients, hemodynamically relevant valvular dysfunction in 13 (42%), late myocardial enhancement in 9 (29%), and any perfusion deficit in 21 (68%). An association of regional pathologic changes and reconstructed dose to cardiac structures could not be established. Conclusions: In 20-year survivors of Hodgkin's disease, cardiac MRI detects pathologic findings in approximately 70% of patients. Cardiac MRI has a potential role in cardiac imaging of Hodgkin's disease patients after mediastinal radiotherapy.

  18. Anesthetic management for anterior mediastinal mass and mediastinoscopy: a case study.

    PubMed

    McKeown, T J

    1991-08-01

    A 27-year-old white male was scheduled for a mediastinoscopy of an anterior mediastinal mass. The patient was induced with thiopental and succinylcholine, in anticipation of possible difficulty managing the airway. There was no distortion of the airway, and he was easily intubated with a No. 8.5 anode tube and given 20 mg of atracurium. The extent of tumor growth was greater than expected and, as a result, biopsies of the neck were taken without the need for mediastinoscopy. The atracurium was reversed with atropine and edrophonium. Although respirations were being assisted, the SaO2 decreased significantly, and end-tidal CO2 was greatly increased. When the drapes were removed, the patient was found to be cyanotic, with vein distention in the neck and upper extremities. All anesthetic agents were discontinued, the patient was hyperventilated with 100% oxygen, 100 mg of lidocaine was given for coughing and breath-holding, and the patient was placed in the reverse Trendelenburg position. The SaO2 then increased, and the end-tidal CO2 decreased. Respirations were spontaneous, and the patient could be extubated. The probable cause of this episode was obstruction of the superior vena cava by the anterior mediastinal mass. When the muscle relaxant was reversed, the increase in intrathoracic pressure caused the mass to compress the superior vena cava. The compression was released by placing the patient in a reverse Trendelenburg position, which caused the mass to shift. In addition to superior vena cava compression, other complications of anterior mediastinal masses include airway obstruction, distortion of anatomy, impaired cerebral circulation and myasthenic syndrome.(ABSTRACT TRUNCATED AT 250 WORDS)

  19. Motion Analysis of 100 Mediastinal Lymph Nodes: Potential Pitfalls in Treatment Planning and Adaptive Strategies

    SciTech Connect

    Pantarotto, Jason R.; Piet, Anna H.M.; Vincent, Andrew; Soernsen de Koste, John R. van; Senan, Suresh

    2009-07-15

    Purpose: The motion of mediastinal lymph nodes may undermine local control with involved-field radiotherapy. We studied patterns of nodal and tumor motion in 41 patients with lung cancer. Methods and Materials: Four-dimensional (4D) computed tomography planning scans were retrospectively evaluated to identify patients with clearly visible mediastinal lymph nodes. One hundred nodes from 14 patients with Stage I and 27 patients with Stage III were manually contoured in all 4D computed tomography respiratory phases. Motion was derived from changes in the nodal center-of-mass position. Primary tumors were also delineated in all phases for 16 patients with Stage III disease. Statistical analysis included a multivariate mixed-effects model of grouped data. Results: Average 3D nodal motion during quiet breathing was 0.68 cm (range, 0.17-1.64 cm); 77% moved greater than 0.5 cm, and 10% moved greater than 1.0 cm. Motion was greatest in the lower mediastinum (p = 0.002), and nodes measuring 2 cm or greater in diameter showed motion similar to that in smaller nodes. In 11 of 16 patients studied, at least one node moved more than the corresponding primary tumor. No association between 3D primary tumor motion and nodal motion was observed. For mobile primary tumors, phase offsets between the primary tumor and nodes of two or more and three or more phases were observed for 33% and 12% of nodes, respectively. Conclusions: Mediastinal nodal motion is common, with phase offsets seen between the primary tumor and different nodes in the same patient. Patient-specific information is needed to ensure geometric coverage, and adaptive strategies based solely on the primary tumor may be misleading.

  20. Giant pulmonary bulla with mediastinal shift in a 12 1/2 year old girl.

    PubMed

    Fatimi, Saulat Hasnain; Jafferani, Asif; Ashfaq, Awais

    2012-05-01

    Pulmonary bulla in children represent interesting entities. Mostly congenital bronchopulmonary foregut malformations and acquired cysts like pneumatocoeles have also been described. We present a case of a 12 1/2 years old girl with acute onset respiratory distress symptoms harbouring a huge pulmonary cyst exhibiting mass effects, resulting in mediastinal deviation. Following initial workup, cyst excision was carried out which revealed presence of fungal hyphae that was susceptible to Fluconazole therapy post operatively. The case points out how a pulmonary cyst can present in older children with symptoms of respiratory distress and mass effects.

  1. Excision of middle mediastinal parathyroid adenoma by videoscopic assisted mediastinoscopy (VAM)

    PubMed Central

    McMullen, Todd; Bédard, Eric L. R.

    2016-01-01

    We report a case involving a female patient with primary hyperparathyroidism arising from a middle mediastinal parathyroid adenoma. Initial diagnosis was made based on a history of polyuria and recurrent renal colic. At the age of 77 she underwent videoscopic assisted mediastinoscopy (VAM) for resection of the adenoma which was localized using single-photon emission computed tomography (SPECT/CT). This case illustrates the importance for accurate pre-operative localization and intra-operative monitoring to ensure complete surgical removal in order to improve postoperative normocalcemia success rates. PMID:27747020

  2. Mediastinal teratoma in a free-ranging American black bear (Ursus americanus).

    PubMed

    Munk, Brandon A; Turner, J Chris; Keel, M Kevin

    2013-12-01

    A 2.75-yr-old female American black bear (Ursus americanus) was found emaciated and unable to rise. It was euthanized due to the perceived poor prognosis. An approximately 18 x 8 x 10-cm, multiloculated, well-demarcated mass that extended from the bifurcation of the trachea, laterally displacing the lung lobes and caudally displacing the heart. The mass contained fibrous connective tissue, cartilage, bone, nervous tissue, smooth muscle, and a variety of epithelial structures. A mediastinal teratoma was diagnosed based on the microscopic features of the neoplasm. PMID:24450083

  3. Persistent mediastinal and axillary lymph node tuberculosis in a renal transplant patient with successful outcome.

    PubMed

    Oguz, Y; Yilmaz, M I; Eyileten, T; Caglar, K; Yenicesu, M; Kaya, A; Tasar, M; Saglam, M; Doganci, L; Gulec, B; Oner, K; Oktenli, C; Vural, A

    2006-06-01

    Tuberculosis is an opportunistic infection that carries substantial morbidity and mortality in renal transplant recipients. We report here about a 21 year-old man with a living related renal transplant from his mother who developed persistent extra-pulmonary tuberculosis. The disease showed aggressive invasion to the axillary and mediastinal regions with abscess formations, despite standard antituberculosis treatment. During the course of the disease, immunosuppressive therapy was stopped, and the patient received extraordinary doses of multiple antituberculosis drugs. The patient then showed an uneventful course with good clinical and radiological responses.

  4. Progressive Dyspnea in a 40-Year-Old Man Caused by Giant Mediastinal Thymolipoma

    PubMed Central

    Rad, Kamelia

    2016-01-01

    Thymolipomas are rare tumors of the anterior mediastinum containing both thymic stroma and an abundance of fat. We present a 40-year-old man with no underlying disease who presented with cough and progressive dyspnea. Clinical evaluation revealed a giant mass of lipid density filling almost the entire left hemithorax with mediastinal shift. Total excision of the 40 × 33 × 8 cm mass weighing 4 kg was performed via a left thoracotomy and the histopathologic diagnosis of the mass was reported as thymolipoma. The patient remains alive and disease-free, twelve months after the intervention. PMID:27293949

  5. Multiple Ascending Aortic Mural Thrombi and Acute Necrotizing Mediastinitis Secondary to Acute Pancreatitis

    PubMed Central

    Chong, Byung Kwon; Yun, Jae Kwang; Kim, Joon Bum; Park, Do Hyun

    2016-01-01

    The formation of aortic thrombi is an extremely rare complication of acute pancreatitis. Here we report a case of acute pancreatitis complicated by a paraesophageal pseudocyst, necrotizing mediastinitis, and the formation of multiple thrombi in the ascending aorta. The patient was successfully treated by surgical therapy, which included extensive debridement of the mediastinum and removal of the aortic thrombi under cardiopulmonary bypass. Although esophageal resection was not carried out concomitantly, the lesions were resolved and the patient remained free of complications over 2 years of follow-up care. PMID:27734004

  6. Diffuse Cystic Lung Diseases: Diagnostic Considerations.

    PubMed

    Xu, Kai-Feng; Feng, Ruie; Cui, Han; Tian, Xinlun; Wang, Hanping; Zhao, Jing; Huang, Hui; Zhang, Weihong; Lo, Bee Hong

    2016-06-01

    Diffuse cystic lung disease (DCLD) is a group of heterogeneous diseases that present as diffuse cystic changes in the lung on computed tomography of the chest. Most DCLD diseases are rare, although they might resemble common diseases such as emphysema and bronchiectasis. Main causes of DCLD include lymphangioleiomyomatosis, Birt-Hogg-Dubé syndrome, pulmonary Langerhans cell histiocytosis, lymphoid interstitial pneumonia, amyloidosis, light-chain deposition disease, Sjögren syndrome, and primary or metastatic neoplasm. We discuss clinical factors that are helpful in the differential diagnosis of DCLDsuch as sex and age, symptoms and signs, extrapulmonary presentations, cigarette smoking, and family history. Investigations for DCLD include high-resolution computed tomography, biochemical and histopathological studies, genetic tests, pulmonary function tests, and bronchoscopic and video-assisted thoracoscopic biopsies. A proposed diagnostic algorithm would enhance ease of diagnosing most cases of DCLD. PMID:27231867

  7. Diffuse Cystic Lung Disease. Part II.

    PubMed

    Gupta, Nishant; Vassallo, Robert; Wikenheiser-Brokamp, Kathryn A; McCormack, Francis X

    2015-07-01

    The diffuse cystic lung diseases have a broad differential diagnosis. A wide variety of pathophysiological processes spanning the spectrum from airway obstruction to lung remodeling can lead to multifocal cyst development in the lung. Although lymphangioleiomyomatosis and pulmonary Langerhans cell histiocytosis are perhaps more frequently seen in the clinic, disorders such as Birt-Hogg-Dubé syndrome, lymphocytic interstitial pneumonia, follicular bronchiolitis, and light-chain deposition disease are increasingly being recognized. Obtaining an accurate diagnosis can be challenging, and management approaches are highly disease dependent. Unique imaging features, genetic tests, serum studies, and clinical features provide invaluable clues that help clinicians distinguish among the various etiologies, but biopsy is often required for definitive diagnosis. In part II of this review, we present an overview of the diffuse cystic lung diseases caused by lymphoproliferative disorders, genetic mutations, or aberrant lung development and provide an approach to aid in their diagnosis and management.

  8. Cystic fibrosis and physiological responses to exercise.

    PubMed

    Williams, Craig A; Saynor, Zoe L; Tomlinson, Owen W; Barker, Alan R

    2014-12-01

    Cardiopulmonary exercise testing is underutilized within the clinical management of patients with cystic fibrosis. But within the last 5 years, there has been considerable interest in its implementation, which has included deliberations by the European Cystic Fibrosis Society about incorporating this method within the clinical assessment of patients. This review examines the current use of cardiopulmonary exercise testing in assessing the extent and cause(s) of exercise limitation from a pediatric perspective. Examples of the measured parameters and their interpretation are provided. Critical synthesis of recent work in the oxygen uptake (VO2) kinetics response to and following exercise is also discussed, and although identified more as a research tool, its utilization advances researchers understanding of the cardiovascular, respiratory and muscular limitations to exercise tolerance. Finally, exercise and its application in therapeutic interventions are highlighted and a number of recommendations made about the utility of exercise prescription.

  9. Calcium-mediated mechanisms of cystic expansion

    PubMed Central

    Abdul-Majeed, Shakila; Nauli, Surya M.

    2010-01-01

    In this review, we will discuss several well-accepted signaling pathways toward calcium-mediated mechanisms of cystic expansion. The second messenger calcium ion has contributed to a vast diversity of signal transduction pathways. We will dissect calcium signaling as a possible mechanism that contributes to renal cyst formation. Because cytosolic calcium also regulates an array of signaling pathways, we will first discuss cilia-induced calcium fluxes, followed by Wnt signaling that has attributed to much-discussed planar cell polarity. We will then look at the relationship between cytosolic calcium and cAMP as one of the most important aspects of cyst progression. The signaling of cAMP on MAPK and mTOR will also be discussed. We infer that while cilia-induced calcium fluxes may be the initial signaling messenger for various cellular pathways, no single signaling mediator or pathway is implicated exclusively in the progression of the cystic expansion. PMID:20932898

  10. Acquired Cystic Fibrosis Transmembrane Conductance Regulator Deficiency.

    PubMed

    Cho, Do-Yeon; Woodworth, Bradford A

    2016-01-01

    In the genetic airway disease cystic fibrosis (CF), deficiency or dysfunction of the cystic fibrosis membrane conductance regulator (CFTR) alters anion transport in respiratory epithelium and consequently disrupts mucociliary clearance. An enriched understanding of the role of CFTR in the maintenance of normal epithelial function has revealed that mild and variable CFTR mutations play a causative role in a number of diseases not classically associated with CF. Furthermore, recent evidence indicates that acquired defects in wild-type CFTR protein processing, endocytic recycling and function can contribute to the pathogenesis of airway diseases, such as chronic obstructive pulmonary disease. In this chapter, we discuss emerging findings implicating acquired CFTR dysfunction in the pathogenesis of chronic rhinosinusitis and propose a new and leading edge approach to future CRS therapy using CFTR potentiators. PMID:27466849

  11. Inflammation and its genesis in cystic fibrosis.

    PubMed

    Nichols, David P; Chmiel, James F

    2015-10-01

    The host inflammatory response in cystic fibrosis (CF) lung disease has long been recognized as a central pathological feature and an important therapeutic target. Indeed, many believe that bronchiectasis results largely from the oxidative and proteolytic damage comprised within an exuberant airway inflammatory response that is dominated by neutrophils. In this review, we address the longstanding argument of whether or not the inflammatory response is directly attributable to impairment of the cystic fibrosis transmembrane conductance regulator or only secondary to airway obstruction and chronic bacterial infection and challenge the importance of this distinction in the context of therapy. We also review the centrality of neutrophils in CF lung pathophysiology and highlight more recent data that suggest the importance of other cell types and signaling beyond NF-κB activation. We discuss how protease and redox imbalance are critical factors in CF airway inflammation and end by reviewing some of the more promising therapeutic approaches now under development.

  12. Cystic fibrosis and physiological responses to exercise.

    PubMed

    Williams, Craig A; Saynor, Zoe L; Tomlinson, Owen W; Barker, Alan R

    2014-12-01

    Cardiopulmonary exercise testing is underutilized within the clinical management of patients with cystic fibrosis. But within the last 5 years, there has been considerable interest in its implementation, which has included deliberations by the European Cystic Fibrosis Society about incorporating this method within the clinical assessment of patients. This review examines the current use of cardiopulmonary exercise testing in assessing the extent and cause(s) of exercise limitation from a pediatric perspective. Examples of the measured parameters and their interpretation are provided. Critical synthesis of recent work in the oxygen uptake (VO2) kinetics response to and following exercise is also discussed, and although identified more as a research tool, its utilization advances researchers understanding of the cardiovascular, respiratory and muscular limitations to exercise tolerance. Finally, exercise and its application in therapeutic interventions are highlighted and a number of recommendations made about the utility of exercise prescription. PMID:25395018

  13. [Dry powder inhalers in cystic fibrosis].

    PubMed

    Steinkamp, G

    2014-06-01

    Inhaled medications play an important role in the daily treatment of patients with cystic fibrosis (CF). The classic route of administration was nebulisation via jet nebulisers. Respiratory delivery of fluid particles should loosen the viscid respiratory secretions, making airway clearance via cough or physiotherapy more efficient. Until recently, only jet nebulisers allowed to administer high doses of aerosolised antipseudomonal antibiotics. Powder inhalers for the treatment of cystic fibrosis have recently been made available. The newly developed powders and inhalers differ considerably from conventional dry powder inhalers used for the treatment of chronic obstructive airway disease. The present article will review two inhaled antibiotics, i. e. tobramycin and colistin, and the hyperosmotic agent mannitol, which increases the hydration of the airways. Topics are particle engineering, efficacy and tolerability results from clinical trials, as well as functional and practical aspects related to these new drugs. PMID:24664997

  14. Parotid Cystic Lesion in Amelanotic Malignant Melanoma.

    PubMed

    Santos, Vitorino Modesto Dos; Gondim Neto, Manoel da Costa; de Melo, Tereza Rodrigues de Carvalho Vieira; Motta, Iara Machado

    2016-09-01

    A 60-year Brazilian woman, presented with an enlarged lymph node in the neck for one year, and a superficial nonulcerated lesion was observed in the scalp. Fine needle aspiration and biopsy of the lymph node revealed amelanocytic metastasis, and immunohistochemistry study showed Melan-A/ Mart-1 antigen (clone A103 and S-100 protein). The entire suspected area of the scalp was further resected and an amelanotic melanoma without angiolymphatic invasion was diagnosed. Ultrasonography and PET-computed tomography showed hypermetabolic cystic area in the right parotid. Furthermore, aspiration biopsy and surgical samples from parotid cyst confirmed the malignant amelanotic melanoma. Cystic metastases are scarcely reported in parotid gland, and can pose diagnostic challenges. PMID:27671185

  15. Lung Cancer Detected 5 Years after Resection of Cancer of Unknown Primary in a Mediastinal Lymph Node: A Case Report and Review of Relevant Cases from the Literature

    PubMed Central

    Arakaki, Kazunari; Taira, Naohiro; Furugen, Tomonori; Ichi, Takaharu; Yohena, Tomofumi; Kawabata, Tsutomu

    2015-01-01

    We report the rare and interesting case of a primary lung cancer detected 5 years after cancer of unknown primary (CUP) of a mediastinal lymph node (LN) was resected. A 40-year-old male was diagnosed with adenocarcinoma of unknown primary in a mediastinal lymph node after resection of the mediastinal tumor. Five years after resection of the CUP in mediastinal LN, a small, abnormal nodular shadow in left upper lobe was detected by chest CT. This pulmonary tumor was diagnosed as a lung adenocarcinoma. The pathological and immunohistological findings of the resected pulmonary tumor resembled those of the LN resected 5 years before. We speculated that the pulmonary lesion represented primary lung cancer that enlarged later than the metastatic mediastinal LN. This case illustrates the importance of careful observation and long-term follow-up in patients treated for CUP of a thoracic LN. PMID:26328596

  16. Treatment of stage i and ii mediastinal Hodgkin disease: a comparison of involved fields, extended fields, and involved fields followed by MOPP in patients stage by laparotomy

    SciTech Connect

    Hagemeister, F.B.; Fuller, L.M.; Sullivan, J.A.; North, L.; Velasquez, W.; Conrad, F.G.; McLaughlin, P.; Butter, J.J.; Shullenberger, C.C.

    1981-12-01

    Three treatment programs for Stage I and II mediastinal Hodgkin disease (established by laparotomy) were compared. Involved-field radiotherapy + MOPP gave a disease-free survival rate of 97%, significantly different from 62% and 55% for involved and extended fields, respectively. Corresponding survival figures of 97%, 88%, and 84% were not signiticantly different statistically due to salvage with radiotherapy and/or chemotherapy. Among patients given radiotherapy alone, the survival figure of 94% for limited mediastinal disease was significantly better than 63% for extensive mediastinal and hilar disease; corresponding disease-free figures of 72% and 35% were also significantly different. Constitutional symptoms were an important prognostic factor in disease-free survival following the use of involved fields; hilar disease was important only with large mediastinal masses. Most relapses were intrathoracic; MOPP alone salvaged only 47%. Treatment of State I and II Hodgkin disease should be based on symptoms, extent of mediastinal disease, and hilar involvement.

  17. [Perinephric liposarcoma mimicking cystic renal tumor].

    PubMed

    Horiguchi, Akio; Oyama, Masafumi

    2002-03-01

    Liposarcoma is one of the most common primary retroperitoneal neoplasms, and the perinephric region is a frequent location for them. Liposarcomas show a variety of radiographic features in terms of histological types and tumor sizes, so the specific diagnosis of liposarcoma is often difficult. We present a unique case of perinephric dedifferentiated liposarcoma mimicking cystic renal tumor. A 71-year-old man presented himself at our hospital with a palpable mass in his upper right abdomen. Abdominal computerized tomography (CT) revealed a well-defined cystic mass at the lower pole of the right kidney that contained heterogeneous solid components and small foci of fat. There were no signs of lymphadenopathy or tumor thrombus in the renal vein. Metastatic evaluation by chest x-ray and bone scan was negative. The probable diagnosis was cystic renal cell carcinoma or atypical angiomyolipoma. Because we could not exclude the possibility of cystic malignancy, a right radical nephrectomy was performed. Grossly, the tumor was predominantly encapsulated by a unilocular fibrous capsule and was filled with bloody fluid and debris. The anterior portion of the tumor was composed of various-sized soft and rubbery masses covered with necrotic tissue. The histological diagnosis was dedifferentiated liposarcoma arising in the perinephric retroperitoneum with extensive necrosis, and the cyst wall was composed of a necrotic tumor with a well differentiated liposarcoma and a fibrous capsule. Although the tumor widely covered the right kidney, there was no microscopic invasion of the kidney. No signs of tumor recurrence were noted six months after the operation.

  18. [New prospects in cystic fibrosis treatment].

    PubMed

    Prados, C; Serrano, S; Alvarez-Sala, R; Villamor, J

    1997-04-01

    Only a few years ago, cystic fibrosis (CF) was considered the most frequent genetic disease in childhood, although survival has increased considerably in recent years owing to improved treatment. We discuss treatments that are still relevant as well as others that are under investigation now, aiming for better understanding of the disease and the therapies that have improved quality of life for CF patients. PMID:9280562

  19. Progress in therapies for cystic fibrosis.

    PubMed

    De Boeck, Kris; Amaral, Margarida D

    2016-08-01

    Standard follow-up and symptomatic treatment have allowed most patients with cystic fibrosis to live to young adulthood. However, many patients still die prematurely from respiratory insufficiency. Hence, further investigations to improve these therapies are important and might have relevance for other diseases-eg, exploring how to increase airway hydration, how to safely downscale the increased inflammatory response in the lung, and how to better combat lung infections associated with cystic fibrosis. In parallel, development of modulators that target the underlying dysfunction in the cystic fibrosis transmembrane conductance regulator (CFTR) is fast moving forward. Existing treatments are specific to certain mutations, or mutation class, in CFTR. An effective, although not yet entirely corrective, treatment is available for patients with class III mutations, and a treatment with modest effectiveness is available for patients who are homozygous for Phe508del, albeit at a very high cost. Corrective treatments that are non-specific to mutation class and thus applicable to all patients-eg, gene therapy, cell-based therapies, and activation of alternative ion channels that bypass CFTR-are being explored, but they are still in early stages of development. In view of the large number of patients with very rare mutations, a plan to advance personalised biomarkers to predict treatment effect is also being investigated and validated. PMID:27053340

  20. [Molecular epidemiology of cystic fibrosis in Tunisia].

    PubMed

    Messaoud, T; Bel Haj Fredj, S; Bibi, A; Elion, J; Férec, C; Fattoum, S

    2005-01-01

    Cystic fibrosis is the most frequent autosomal recessive genetic disease in North European population. This pathology seems to not be rare in Tunisia. On another hand, development of molecular biology techniques has largely contributed to implement the study of the different mutations in the CFTR gene where over 1,300 mutations were reported. Herein, we describe the strategy used to detect molecular defects responsible of cystic fibrosis on 390 children (383 families) in Tunisian population. Several techniques were performed for genotype diagnosis: DNA extraction was from peripheral blood. Polymerase chain reaction (PCR) and polyacylamide gel electrophoresis, and reverse dot blot procedures were used to detect known point mutations. Denaturant gradient gel electrophoresis (DGGE) were used in a next step searching for the unknown point mutations that are later identified by automated sequencing on ABIprism 310. This strategy allowed us to detect 17 different mutations located on the different exons of the CFTR gene. The most frequent was the F508del (50.74%) followed by three other mutations (G542X, W1282X and N1303K) known to be common in the Mediterranean area. For mutations (T665S, 2766 del8, F1166C, L1043R) were exclusively found, up to now, in the Tunisian population. Our results permitted to establish cystic fibrosis mutations and their distribution in Tunisia and to implement an appropriate prevention program of these diseases through the genetic council and prenatal diagnosis.

  1. Different manifestations of calcifying cystic odontogenic tumor.

    PubMed

    Utumi, Estevam Rubens; Pedron, Irineu Gregnanin; da Silva, Leopoldo Penteado Nucci; Machado, Gustavo Grothe; Rocha, André Caroli

    2012-01-01

    The calcifying cystic odontogenic tumor normally presents as a painless, slow-growing mass, involving both maxilla and mandible, primarily the anterior segment (incisor/canine area). It generally affects young adults in the third to fourth decades, with no gender predilection. Computerized tomography images revealed important characteristics that were not detected by panoramic radiography, such as fenestration, calcification and tooth-like structures. The typical microscopic feature of this lesion is the presence of variable amounts of aberrant epithelial cells, without nuclei, which are named "ghost cells". In addition, dysplastic dentine can be found and occasionally the cyst can be associated with an area of dental hard tissue formation resembling an odontoma. The treatment for calcifying cystic odontogenic tumor involves simple enucleation and curettage. The purpose of this article is to present two different manifestation of calcifying cystic odontogenic tumor in which computerized tomography, associated to clinical features, served as an important tool for diagnosis, adequate surgical planning and follow-up of patients.

  2. Downsized Contegra graft as a right ventricle-to-pulmonary artery conduit in the setting of mediastinitis.

    PubMed

    MacIver, Robroy H; Permut, Lester C; McMullan, David M

    2013-09-01

    Polytetrafluoroethylene (PTFE) grafts are commonly used for construction of the right ventricle-to-pulmonary artery conduit in the modified Norwood procedure. Dehiscence of a PTFE conduit in the setting of purulent mediastinitis presents a challenging clinical problem because of limited availability of appropriately sized replacement vascular homografts. The Contegra bovine jugular vein graft is an alternative to placing another PTFE graft in an infected space when a homograft of appropriate size is not available. We describe the use of a downsized Contegra conduit to replace an infected PTFE right ventricle-to-pulmonary artery graft in a neonate with life-threatening purulent mediastinitis. PMID:23992706

  3. Mediastinal irradiation in a patient affected by lung carcinoma after heart transplantation: Helical tomotherapy versus three dimensional conformal radiotherapy

    PubMed Central

    Iorio, Vincenzo; Cammarota, Fabrizio; Toledo, Diego; Senese, Rossana; Francomacaro, Ferdinando; Muto, Matteo; Muto, Paolo

    2016-01-01

    Abstract Patients who have undergone solid organ transplants are known to have an increased risk of neoplasia compared with the general population. We report our experience using mediastinal irradiation with helical tomotherapy versus three‐dimensional conformal radiation therapy to treat a patient with lung carcinoma 15 years after heart transplantation. Our dosimetric evaluation showed no particular difference between the techniques, with the exception of some organs. Mediastinal irradiation after heart transplantation is feasible and should be considered after evaluation of the risk. Conformal radiotherapy or intensity‐modulated radiotherapy appears to be the appropriate treatment in heart‐transplanted oncologic patients. PMID:27148425

  4. Mediastinal irradiation in a patient affected by lung carcinoma after heart transplantation: Helical tomotherapy versus three dimensional conformal radiotherapy.

    PubMed

    Giugliano, Francesca M; Iorio, Vincenzo; Cammarota, Fabrizio; Toledo, Diego; Senese, Rossana; Francomacaro, Ferdinando; Muto, Matteo; Muto, Paolo

    2016-04-26

    Patients who have undergone solid organ transplants are known to have an increased risk of neoplasia compared with the general population. We report our experience using mediastinal irradiation with helical tomotherapy versus three-dimensional conformal radiation therapy to treat a patient with lung carcinoma 15 years after heart transplantation. Our dosimetric evaluation showed no particular difference between the techniques, with the exception of some organs. Mediastinal irradiation after heart transplantation is feasible and should be considered after evaluation of the risk. Conformal radiotherapy or intensity-modulated radiotherapy appears to be the appropriate treatment in heart-transplanted oncologic patients.

  5. Giant cystic leiomyoma of the uterus occupying the retroperitoneal space.

    PubMed

    Funaki, Kaoru; Fukunishi, Hidenobu; Tsuji, Yoshihiko; Maeda, Tetsuo; Takahashi, Takuya

    2013-12-01

    A 31-year-old nulliparous woman visited our hospital complaining of abdominal distension. Abdominal ultrasonography and computed tomography revealed a 40 × 40 × 30-cm, multilocular cystic mass extending from the upper abdomen to the pelvis. Magnetic resonance imaging (MRI) revealed a cystic tumor that was hypointense on T1-weighted images and was heterogeneously hyperintense on T2-weighted images. The final diagnosis was an 8 kg leiomyoma with cystic degeneration. Uterine leiomyomas are common benign tumors in females of reproductive age. However, subserosal leiomyomas with complete cystic degeneration of the retroperitoneal space are rare, and they are difficult to accurately diagnosis without pathological examination.

  6. Chloride and potassium channels in cystic fibrosis airway epithelia

    NASA Astrophysics Data System (ADS)

    Welsh, Michael J.; Liedtke, Carole M.

    1986-07-01

    Cystic fibrosis, the most common lethal genetic disease in Caucasians, is characterized by a decreased permeability in sweat gland duct and airway epithelia. In sweat duct epithelium, a decreased Cl- permeability accounts for the abnormally increased salt content of sweat1. In airway epithelia a decreased Cl- permeability, and possibly increased sodium absorption, may account for the abnormal respiratory tract fluid2,3. The Cl- impermeability has been localized to the apical membrane of cystic fibrosis airway epithelial cells4. The finding that hormonally regulated Cl- channels make the apical membrane Cl- permeable in normal airway epithelial cells5 suggested abnormal Cl- channel function in cystic fibrosis. Here we report that excised, cell-free patches of membrane from cystic fibrosis epithelial cells contain Cl- channels that have the same conductive properties as Cl- channels from normal cells. However, Cl- channels from cystic fibrosis cells did not open when they were attached to the cell. These findings suggest defective regulation of Cl- channels in cystic fibrosis epithelia; to begin to address this issue, we performed two studies. First, we found that isoprenaline, which stimulates Cl- secretion, increases cellular levels of cyclic AMP in a similar manner in cystic fibrosis and non-cystic fibrosis epithelial cells. Second, we show that adrenergic agonists open calcium-activated potassium channels, indirectly suggesting that calcium-dependent stimulus-response coupling is intact in cystic fibrosis. These data suggest defective regulation of Cl- channels at a site distal to cAMP accumulation.

  7. Cystic trophoblastic tumor: a nonaggressive lesion in postchemotherapy resections of patients with testicular germ cell tumors.

    PubMed

    Ulbright, Thomas M; Henley, John D; Cummings, Oscar W; Foster, Richard S; Cheng, Liang

    2004-09-01

    Cystic trophoblastic tumor (CTT) is an uncommon lesion that is usually seen after chemotherapy in patients with testicular germ cell tumors. Its clinical significance has not been well studied. We identified 17 patients with CTT in retroperitoneal lymph node dissections (RPLNDs) after cisplatin-based chemotherapy for testicular germ cell tumors. None had other forms of persistent germ cell tumor except for teratoma, and no patient received additional chemotherapy after RPLND. At the time of RPLND, 7 patients were known to have had normal serum levels of beta-subunit of human chorionic gonadotropin (beta-hCG), whereas 5 had relatively mild elevations (1.6-165 mIU/mL, median, 8.0 mIU/mL). The CTTs consisted of circumscribed, small cysts, usually multifocal, lined by mostly mononucleated trophoblast cells with abundant eosinophilic cytoplasm, often with smudged nuclei and showing only infrequent mitotic figures. Although the epithelial lining was often stratified to several layers in thickness or formed intracystic papillary tufts, solid proliferations of trophoblast cells within the stroma were absent, as were clearly biphasic admixtures of mononucleated and multinucleated trophoblast cells. The cysts were either empty or contained fibrinoid material and were set in a hypocellular, fibrous stroma with adjacent teratoma. Stains for hCG highlighted rare cells. On follow-up of 15 patients, 11 were disease free (mean, 80 months). Three recurred with serum alpha-fetoprotein elevations at 25, 31, and 107 months, respectively, and one with beta-hCG elevation at 2 months. The latter patient, however, also had unresected mediastinal tumor postchemotherapy. We conclude that the finding of CTT in postchemotherapy resections does not warrant additional chemotherapy. Its clinical significance appears similar to that of residual teratoma.

  8. Magnetic resonance imaging of mediastinal and hilar masses: comparison with CT

    SciTech Connect

    Levitt, R.G.; Glazer, H.S.; Roper, C.L.; Lee, J.K.T.; Murphy, W.A.

    1985-07-01

    Magnetic resonance imaging (MRI) was compared to computed tomography (CT) of the mediastinum and/or hila in 37 patients with bronchogenic carcinoma (35 unresectable for cure) and 11 patients with other masses. Spin-echo pulse sequences using a short pulse repetition rate (TR) and short echo delay (TE) were most helpful for detection of abnormal soft-tissue mediastinal and hilar masses. The accuracy of MRI and CT in staging bronchogenic carcinoma for curative resectability/nonresectability was comparable. Several pitfalls in MRI evaluation of the mediastinum were identified. By MRI the esophagus may be misinterpreted as an enlarged retrotracheal lymph node unless serial scans are studied. Small adjacent lymph nodes shown individually by CT may appear as a single enlarged lymph node by MRI due to partial-volume averaging. Because of the requirement for patient selection and the identified pitfalls of MRI, CT remains the radiologic procedure of choice in the staging of patients with bronchogenic carcinoma and the evaluation of other mediastinal and hilar masses at present. However, because of the ability to show blood vessels without an intravascular contrast agent, MRI is useful in evaluating patients with potential contrast allergy and solving diagnostic problems not solved by CT.

  9. An unusual mediastinal parathyroid carcinoma coproducing PTH and PTHrP: A case report

    PubMed Central

    CAO, CHUANGJIE; DOU, CHENGYUN; CHEN, FUQIN; WANG, YAN; ZHANG, XIAOLI; LAI, HONG

    2016-01-01

    Parathyroid carcinoma (PTCA) is a rare disease, and ectopic PTCA is particularly rare. Parathyroid hormone-related protein (PTHrP) expression in PTCA has not been previously described in the relevant literature to the best of our knowledge. The present study reports a unique case with a mediastinal parathyroid carcinoma producing parathyroid hormone (PTH) and PTHrP. A 53-year-old man presented with hyperparathyroidism symptoms, including fatigue, chest pain, dizziness, muscular soreness, polyuria, night sweats and renal stones. However, neck ultrasound revealed no significantly abnormal thyroid or parathyroid nodules. Tc99m methoxyisobutylisonitrile (Tc99m-MIBI) scintigraphy scanning indicated an ectopic mediastinal parathyroid adenoma. Histopathological examination revealed PTCA, and the tumor tissue was coproducing PTH and PTHrP. The patient underwent successful surgical operation. Serum calcium and PTH levels remained within normal ranges, and there was no tumor recurrence observed at a 3-year follow-up appointment. Although rare, ectopic parathyroid glands may lead to malignant disease. Clinical symptoms, biochemical tests, ultrasound and Tc99m-MIBI scintigraphy scanning may assist with the diagnosis of this disease. Hypersecretion of PTHrP and PTH contributed collaboratively to the pathogenesis of hypercalcemia due to PTCA. Complete surgical resection with microscopically negative margins is the recommended treatment for PTCA and offers the best chance of a cure. PMID:27313750

  10. Fatal massive hemorrhage caused by nasogastric tube misplacement in a patient with mediastinitis.

    PubMed

    Wu, Pei-Yu; Kang, Ting-Jui; Hui, Chung-Kun; Hung, Ming-Hui; Sun, Wei-Zen; Chan, Wei-Hung

    2006-01-01

    Nasogastric tube insertion is a routine procedure in medical care. However, misplacement of the tube can cause a variety of complications, which can be life threatening in some instances. We report a case of fatal hemorrhagic shock immediately after nasogastric tube insertion in a patient undergoing debridement by video-assisted thoracoscopic surgery for mediastinitis. Emergency endoscopy showed that the bleeding came from the nasogastric tube which had perforated the esophagus and possibly tore an intrathoracic large vessel. The nasogastric tube insertion was considered to have directly produced the perforation because no esophageal perforation had been found on preoperative endoscopy. Factors contributing to the risk of esophageal perforation in this case included coexisting mediastinitis, surgical manipulation, endotracheal intubation, inability to cooperate during general anesthesia, and repetitive advancement of the nasogastric tube. Prompt clamping of the nasogastric tube or delayed insertion after failed attempts might have improved the outcome. This report illustrates the complication of massive bleeding that can occur immediately after misplaced insertion of a nasogastric tube. Extraordinary care should be taken to avoid misplacement of the nasogastric tube during insertion.

  11. Bilateral Breast Enlargement: An Unusual Presentation of Superior Vena Cava Obstruction in a Hemodialysis Patient with Fibrosing Mediastinitis

    SciTech Connect

    Goo, Dong Erk Kim, Yong Jae; Choi, Deuk Lin; Kwon, Kui Hyang; Yang, Seung Boo

    2011-02-15

    A 67-year-old woman with end-stage renal disease presented with profound edema of both breasts. The presence of a patent hemodialysis basilic transposition fistula and superior vena cava obstruction (SVC), due to fibrosing mediastinitis, was demonstrated by the use of fistulography. Endovascular treatment with a balloon and stent caused immediate resolution of the breast edema.

  12. Clinical evolution of cystic teratoma during treatment with combination chemotherapy.

    PubMed

    Taylor, H G; Tell, D T; Skoog, S J; McLeod, D G

    1986-09-01

    Progressive, cystic tumor enlargement in the abdomen developed in a patient with teratocarcinoma during treatment with systemic chemotherapy. Tumor markers were elevated in the cyst fluid and negative in serum. Further, the patient underwent a successful surgical debulking of large amounts of cystic teratoma.

  13. Pseudomonal infection in cystic fibrosis: the battle continues.

    PubMed

    Elkin, Sarah; Geddes, Duncan

    2003-12-01

    Pseudomonas aeruginosa lung infection is the major cause of morbidity and mortality in patients with cystic fibrosis. Infection usually begins in childhood and is responsible for respiratory failure and death in most patients with cystic fibrosis. The organism triggers an exuberant chronic inflammatory reaction which damages the airways and leads to progressive loss of lung function. Over the last decade significant advances have been made in the understanding of the pathophysiology of cystic fibrosis airways disease. These should assist the development of new and better therapies to treat this pathogen. This review provides an overview of pseudomonal infection in cystic fibrosis, including mechanisms by which the bacteria may colonize the cystic fibrosis airway, persistence of pseudomonal infection and the biofilm mode of growth. Available treatments and possible novel approaches to therapy will be discussed. PMID:15482158

  14. New insights into cystic fibrosis-related diabetes in children.

    PubMed

    Ode, Katie L; Moran, Antoinette

    2013-09-01

    Cystic fibrosis-related diabetes (CFRD) is the most common complication of cystic fibrosis. It is associated with significantly increased morbidity and mortality in adults and children. Adolescents with cystic fibrosis have a much higher prevalence of diabetes than any other similar age population. Glucose abnormalities that precede diabetes are even more common, especially in children younger than 10 years. The pathophysiology of glucose metabolic abnormalities is poorly understood, but insulin insufficiency is clearly the main component. Findings from animal studies have provided insight into the pathophysiology of CFRD, and imply that carbohydrate metabolic abnormalities might begin at much younger ages than was previously thought in patients with cystic fibrosis, and might be related to the basic cystic fibrosis chloride channel defect. In this Review we explore present knowledge of CFRD in children and adolescents, and new data that indicate that the pathophysiology of CFRD begins in very young patients.

  15. Cystic Fibrosis Diagnosis and Newborn Screening.

    PubMed

    Rosenfeld, Margaret; Sontag, Marci K; Ren, Clement L

    2016-08-01

    The diagnosis of cystic fibrosis (CF) has evolved over the past decade as newborn screening has become universal in the United States and elsewhere. The heterogeneity of phenotypes associated with CF transmembrane conductance regulator (CFTR) dysfunction and mutations in the CFTR gene has become clearer, ranging from classic pancreatic-insufficient CF to manifestations in only 1 organ system to indeterminate diagnoses identified by newborn screening. The tools available for diagnosis have also expanded. This article reviews the newest diagnostic criteria for CF, newborn screening, prenatal screening and diagnosis, and indeterminate diagnoses in newborn-screened infants and symptomatic adults. PMID:27469178

  16. The Changing Microbial Epidemiology in Cystic Fibrosis

    PubMed Central

    LiPuma, John J.

    2010-01-01

    Summary: Infection of the airways remains the primary cause of morbidity and mortality in persons with cystic fibrosis (CF). This review describes salient features of the epidemiologies of microbial species that are involved in respiratory tract infection in CF. The apparently expanding spectrum of species causing infection in CF and recent changes in the incidences and prevalences of infection due to specific bacterial, fungal, and viral species are described. The challenges inherent in tracking and interpreting rates of infection in this patient population are discussed. PMID:20375354

  17. Cystic fibrosis diagnosed in an elderly man.

    PubMed

    Gilljam, Marita; Björck, Eva

    2004-01-01

    Although most patients with cystic fibrosis (CF) are diagnosed in early childhood, the diagnosis may be delayed for patients with mild symptoms or single-organ disease. We describe a man with known infertility and a history of productive cough diagnosed with CF at 61 years of age. The patient carries two mutations and one variant in the CF transmembrane conductance regulator gene: 394delTT, P99L and R75Q. During a 15-year follow-up time he has developed significant pulmonary disease.

  18. Nutrition and Growth in Cystic Fibrosis.

    PubMed

    Lusman, Sarah; Sullivan, Jillian

    2016-08-01

    Close attention to nutrition and growth is essential in caring for children with cystic fibrosis (CF). Growth and nutritional status should be monitored as part of routine CF care. Children with CF should achieve growth and nutritional status comparable with that of well-nourished children without CF. Children with CF are at risk for nutritional deficiencies. Optimal nutritional and growth status may be difficult to attain in this population given risk of insufficient caloric intake and likelihood of increased caloric expenditure. Various methods to attain optimal nutritional status may be used, including oral supplementation, behavioral treatment, pharmacotherapy, and enteral nutrition. PMID:27469181

  19. Congenital cystic adenomatoid malformation type I

    PubMed Central

    Ribeiro, Frederico Becker; Schultz, Regina

    2015-01-01

    Congenital cystic adenomatoid malformation (CCAM) is an hamartomatous congenital pulmonary airway malformation with incidence ranging between 1:10,000 and 1:35,000 newborns. Currently CCAM is classified into five groups according to clinical and pathological features. The clinical outcome varies depending on the subtype and the extent of involvement. The authors report the case of a premature male newborn with the prenatal diagnosis of CCAM Type 1 associated with cardiac right axis deviation, who died 67 hours after birth due to respiratory failure. In addition to the autopsy report of this rare entity, the authors present its classification and prognosis. PMID:26558243

  20. [Acquired cystic renal disease. Association with hypernephroma].

    PubMed

    Comesaña, E; Pesqueira, D; Tardáguila, F; De la Fuente, A; Antón, I; Vidal, L; Zungri, E

    1992-02-01

    Emergence of multiple bilateral renal cysts observed in patients undergoing periodic haemodialysis is 40%. The pathology, known as Acquired Cystic Renal Disease (A.C.R.D.) presents a high association to renal cancer. Two cases of A.C.R.D. and their association with hypernephroma, one resulting in secondary retroperitoneal haemorrhage and the other in intracystic haemorrhage, are presented. Forms and diagnosis are analyzed, insisting upon the need of monitoring the patients in haemodialysis from the point of view of tumour emergence.

  1. Retroperitoneal cystic neuroendocrine tumor. A case report.

    PubMed

    Scapinello, A; D'Amore, E S; Cavazzana, A O; Gramegna, V; Ninfo, V

    1995-10-01

    A 21 cm retroperitoneal cystic mass was excised from a 71 year old woman. The cyst was filled with a hemorrhagic fluid and contained a 5 cm parietal hemorrhagic nodule. On histology, the nodule was composed of a uniform population of round cells arranged in trabeculae and nests. The neoplastic cells were immunoreactive to cytokeratin, EMA, NSE, chromogranin A, pancreatic polypeptide (PP) and Gastrin (G). Ultrastructural observation of neurosecretory granules confirmed the neuroendocrine nature of the tumor. No other lesions were detected and a diagnosis of primary epithelial neuroendocrine tumor was rendered. The histogenesis of the tumor including the possibility of a paraganglionic origin is discussed.

  2. [Nutrition, cystic fibrosis and the digestive tract].

    PubMed

    Olveira, Gabriel; Olveira, Casilda

    2008-05-01

    The prevalence of hyponutrition in cystic fibrosis is high although it may vary according to the different studies. Detection of hyponutrition should be done by combining different methods, depending on their availability. However, the simplest and most validated criterion is to measure at each visit the weight (and height in children) in order to calculate the body mass index and categorizing hyponutrition according to absolute criteria: in adults < 18.5 kg/m(2), and in children as percentiles of the body mass index. Worsening of the nutritional status is directly related with the decrease in lung function parameters and it has been proposed as a morbidity (and even mortality) predictive factor in people with cystic fibrosis, independently of the level of pulmonary dysfunction. Exocrine pancreatic insufficiency is present is approximately 70-90% of the patients with cystic fibrosis and the genotype-phenotype correlation is high. Most of the patients with exocrine pancreatic insufficiency tolerate a high-fat diet provided that they are treated with pancreatic enzymes at appropriate doses. The prevalence of diabetes increases with age, reaching up 40% of the cases in patients older than 30 years. Clinical liver involvement is less prevalent (it approximately affects 1/3 of the patients). Other intestinal complications such as meconial ileus, gastroesophageal reflux, obstruction of the distal intestine, or fibrosing colon disease may also condition malnourishment. In patients with cystic fibrosis, a usual high-fat diet providing 120%-150% of the recommended calories is advised. If the nutritional goals are not achieved or maintained with diet modifications, artificial supplements may be added, although the recommendation for their use has not been endorsed by solid scientific evidences. The most frequently used preparations usually are polymeric or hypercaloric. The indications for enteral (through a tube, especially gastrostomy) or parenteral nutritional support are

  3. Genetic alterations in primary mediastinal B-cell lymphoma: an update.

    PubMed

    Scarpa, A; Moore, P S; Rigaud, G; Menestrina, F

    2001-03-01

    Primary mediastinal B-cell lymphoma (PMBL) is a distinct clinical entity among non-Hodgkin's lymphoma. The malignancy has received little attention from a standpoint of basic research due in part to its rarity. However, based on recent studies consistent trends are beginning to emerge regarding the molecular and chromosomal alterations commonly observed in this disease. By both CGH and AP-PCR, genetic gains involving chromosomes 2, 5, 7, 9p, 12, and Xq are among the most frequently observed events. From a molecular standpoint, alterations in the c-myc, p16(INK4) and p53 genes have been observed in up to 30% of cases. This information along with the well-established histological, immunological, and clinical features should convince the few remaining disbelievers that PMBL is a distinct pathological entity among non-Hodgkin's lymphomas. PMID:11342356

  4. [Transmanubrial Osteomuscular-sparing Approach for Resection of Superior Mediastinal Schwannoma].

    PubMed

    Aragaki, Masato; Iimura, Yasuyuki; Sato, Nagato; Fukuda, Naoya; Miyazaki, Dai; Umemoto, Kazufumi; Yoshida, Yusuke; Hasegawa, Naoto

    2016-08-01

    Various approaches can be applied to resect superior mediastinal tumor. It is important to choose the procedure according to the location, size, and characteristics of the tumor in order to perform safe procedure surgery because of distinctive anatomy of this site. We hereby report on a case of Schwannoma of the superior mediastinum resected by the transmanubrial approach. A 67-year-old woman was referred to our department to examine an abnormal chest shadow found at a regular health checkup. Computed tomography revealed a tumor 58×52 mm in size extending from the left supraclavicular fossa to the upper border of the aortic arch. The surgery was performed under the diagnosis of neurogenic tumor using the transmanubrial approach. The tumor was resected safely and the pathological diagnosis was a Schwannoma. Transmanubrial approach was found to be quite useful in securing an adequate visual field and enabling the safe separation of blood vessels and nerves from the tumor. PMID:27476565

  5. [Video-assisted thoracic surgery, lung transplantation and mediastinitis: major issues in thoracic surgery in 2010].

    PubMed

    Borro, José M; Moreno, Ramón; Gómez, Ana; Duque, José Luis

    2011-01-01

    We reviewed the major issues in thoracic surgery relating to the advances made in our specialty in 2010. To do this, the 43(rd) Congress of the Spanish Society of Pneumology and Thoracic Surgery held in La Coruña and the articles published in the Society's journal, Archivos de Bronconeumología, were reviewed. The main areas of interest were related to the development of video-assisted thoracic surgery, lung transplantation and descending mediastinitis. The new tumor-node-metastasis (TNM) classification (7(th) edition), presented last year, was still a topical issue this year. The First Forum of Thoracic Surgeons and the Update in Thoracic Surgery together with the Nurses' Area have constituted an excellent teaching program. PMID:21300211

  6. Cystic fibrosis chronic rhinosinusitis: A comprehensive review

    PubMed Central

    Chaaban, Mohamad R.; Kejner, Alexandra; Rowe, Steven M.

    2013-01-01

    Background: Advances in the care of patients with cystic fibrosis (CF) have improved pulmonary outcomes and survival. In addition, rapid developments regarding the underlying genetic and molecular basis of the disease have led to numerous novel targets for treatment. However, clinical and basic scientific research focusing on therapeutic strategies for CF-associated chronic rhinosinusitis (CRS) lags behind the evidence-based approaches currently used for pulmonary disease. Methods: This review evaluates the available literature and provides an update concerning the pathophysiology, current treatment approaches, and future pharmaceutical tactics in the management of CRS in patients with CF. Results: Optimal medical and surgical strategies for CF CRS are lacking because of a dearth of well-performed clinical trials. Medical and surgical interventions are supported primarily by level 2 or 3 evidence and are aimed at improving clearance of mucus, infection, and inflammation. A number of novel therapeutics that target the basic defect in the cystic fibrosis transmembrane conductance regulator channel are currently under investigation. Ivacaftor, a corrector of the G551D mutation, was recently approved by the Food and Drug Administration. However, sinonasal outcomes using this and other novel drugs are pending. Conclusion: CRS is a lifelong disease in CF patients that can lead to substantial morbidity and decreased quality of life. A multidisciplinary approach will be necessary to develop consistent and evidence-based treatment paradigms. PMID:24119602

  7. Cystic Fibrosis Transmembrane Conductance Regulator (CFTR)

    PubMed Central

    Corradi, Valentina; Vergani, Paola; Tieleman, D. Peter

    2015-01-01

    The cystic fibrosis transmembrane conductance regulator (CFTR) is a member of the ATP-binding cassette (ABC) transporter superfamily. CFTR controls the flow of anions through the apical membrane of epithelia. Dysfunctional CFTR causes the common lethal genetic disease cystic fibrosis. Transitions between open and closed states of CFTR are regulated by ATP binding and hydrolysis on the cytosolic nucleotide binding domains, which are coupled with the transmembrane (TM) domains forming the pathway for anion permeation. Lack of structural data hampers a global understanding of CFTR and thus the development of “rational” approaches directly targeting defective CFTR. In this work, we explored possible conformational states of the CFTR gating cycle by means of homology modeling. As templates, we used structures of homologous ABC transporters, namely TM(287–288), ABC-B10, McjD, and Sav1866. In the light of published experimental results, structural analysis of the transmembrane cavity suggests that the TM(287–288)-based CFTR model could correspond to a commonly occupied closed state, whereas the McjD-based model could represent an open state. The models capture the important role played by Phe-337 as a filter/gating residue and provide structural information on the conformational transition from closed to open channel. PMID:26229102

  8. Cystic echinococcosis is an occupational disease?

    PubMed

    Youssefi, Mohammad Reza; Mirshafiei, Sepide; Moshfegh, Zeinab; Soleymani, Norredin; Rahimi, Mohammad Taghi

    2016-09-01

    Cystic echinococcosis (CE) is considered as an occupational infection and among different careers greengroceries have a close contact with raw vegetables which can transmit eggs of Echinococcus granulosus. Therefore, the objective of this survey was to determine the seroprevalence of CE among greengroceries using ELISA test in Mazandaran and Gilan Provinces, northern Iran. Overall, 160 serum samples (60 male and 100 female) were collected from greengroceries in Mazandaran and Gilan Provinces, 2012. Sera were surveyed employing ELISA assay for diagnosis of CE. In addition, before sampling, a questionnaire was filled out for each subject. Seropositivity was 2.4 % (four cases) and females 1.8 % (three cases) were more positive compared to males 0.8 % (one case). Age group of 40-60 years encompassed the highest rate of positivity (1.8 %) and the least rate was in 20-40 years old age group (0 %). Besides, 1.8 % of seropositive subjects were dog owner, unaware of the disease and lives in rural areas. In conclusion, seroprevalence of cystic hydatid disease among greengroceries is relatively high in Mazandaran and Gilan Provinces. More continuous serological researches and preventive measures should be taken into consideration owing to the significance of the disease. PMID:27605752

  9. Targeting ion channels in cystic fibrosis.

    PubMed

    Mall, Marcus A; Galietta, Luis J V

    2015-09-01

    Mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene cause a characteristic defect in epithelial ion transport that plays a central role in the pathogenesis of cystic fibrosis (CF). Hence, pharmacological correction of this ion transport defect by targeting of mutant CFTR, or alternative ion channels that may compensate for CFTR dysfunction, has long been considered as an attractive approach to a causal therapy of this life-limiting disease. The recent introduction of the CFTR potentiator ivacaftor into the therapy of a subgroup of patients with specific CFTR mutations was a major milestone and enormous stimulus for seeking effective ion transport modulators for all patients with CF. In this review, we discuss recent breakthroughs and setbacks with CFTR modulators designed to rescue mutant CFTR including the common mutation F508del. Further, we examine the alternative chloride channels TMEM16A and SLC26A9, as well as the epithelial sodium channel ENaC as alternative targets in CF lung disease, which remains the major cause of morbidity and mortality in patients with CF. Finally, we will focus on the hurdles that still need to be overcome to make effective ion transport modulation therapies available for all patients with CF irrespective of their CFTR genotype.

  10. CFTR protein repair therapy in cystic fibrosis.

    PubMed

    Quintana-Gallego, Esther; Delgado-Pecellín, Isabel; Calero Acuña, Carmen

    2014-04-01

    Cystic fibrosis is a single gene, autosomal recessive disorder, in which more than 1,900 mutations grouped into 6 classes have been described. It is an example a disease that could be well placed to benefit from personalised medicine. There are currently 2 very different approaches that aim to correct the basic defect: gene therapy, aimed at correcting the genetic alteration, and therapy aimed at correcting the defect in the CFTR protein. The latter is beginning to show promising results, with several molecules under development. Ataluren (PTC124) is a molecule designed to make the ribosomes become less sensitive to the premature stop codons responsible for class i mutations. Lumacaftor (VX-809) is a CFTR corrector directed at class ii mutations, among which Phe508del is the most frequent, with encouraging results. Ivacaftor (VX-770) is a potentiator, the only one marketed to date, which has shown good efficacy for the class iii mutation Gly551Asp in children over the age of 6 and adults. These drugs, or a combination of them, are currently undergoing various clinical trials for other less common genetic mutations. In the last 5 years, CFTR has been designated as a therapeutic target. Ivacaftor is the first drug to treat the basic defect in cystic fibrosis, but only provides a response in a small number of patients. New drugs capable of restoring the CFTR protein damaged by the most common mutations are required.

  11. [CYSTIC FIBROSIS: CARE OF THE LUNG DISEASE].

    PubMed

    Hubert, Dominique

    2015-10-01

    (Rh-DNase) and/or hydration (hypertonic saline) nebulisations, Moreover, treatment with inhaled antibiotics is indicated (tobramycin, colistine or aztreonam lysine) for chronic lung infection with Pseudomonas aeruginosa (PA). The treatment regimen also includes bronchodilators for bronchospasms and azithromycin. Regular physical activity is recommended. A treatment potentiating the CFTR protein, ivacaftor, is now indicated for patients with a class 3 mutation. Initial bronchial infection with PA must be treated as soon as possible in order to eradicate the pathogen. Pulmonary exacerbations require antibiotic courses, either orally or intravenously for PA infection. Complications require hospitalisation, with thoracic chest tube placement for a pneumothorax or bronchial artery embolisation for massive hemoptysis. Oxygen therapy and non-invasive ventilation with a nasal mask become necessary when respiratory insufficiency progresses, justifying the initiation of the lung transplant process. Lung disease affects the prognosis of cystic fibrosis, therefore its management in cystic fibrosis centres is of utmost importance. Maintenance treatment mainly relies on daily chest physiotherapy, which can be facilitated by mucolytic PMID:26749716

  12. Stereotaxic intracavitary irradiation for cystic craniopharyngiomas

    SciTech Connect

    Pollack, I.F.; Lunsford, L.D.; Slamovits, T.L.; Gumerman, L.W.; Levine, G.; Robinson, A.G.

    1988-02-01

    Stereotaxic intracavitary irradiation with instillation of phosphorus-32 (/sup 32/P) colloidal chromic phosphate was performed in nine patients with cystic craniopharyngiomas. Serial neurological, ophthalmological, neuroendocrinological, and radiological examinations were performed before and after treatment. Dosimetry was determined based on a computerized tomography (CT) estimation of tumor volume, and was calculated to provide a tumoricidal dose (200 to 300 Gy) to the cyst wall. The follow-up period ranged from 14 to 45 months (mean 27 months). After treatment, all nine patients showed improvement of symptoms and radiological evidence of cyst regression. Because of an expanding solid component producing recurrent symptoms, one patient required a craniotomy 14 months after isotope instillation. Three of five patients with impaired visual acuity before surgery had significant improvement in acuity after treatment. Preoperative visual field defects in eight patients improved in four after /sup 32/P therapy. Of seven patients with preoperative endocrine abnormalities, one individual showed almost complete normalization and another had improvement in endocrine function. Patients who exhibited residual neuroendocrine function before isotope instillation developed no significant deterioration in endocrine status during the follow-up period. The findings suggest that stereotaxic intracavitary irradiation is a safe and effective treatment which should be considered as the initial surgery for cystic craniopharyngiomas.

  13. CFTR protein repair therapy in cystic fibrosis.

    PubMed

    Quintana-Gallego, Esther; Delgado-Pecellín, Isabel; Calero Acuña, Carmen

    2014-04-01

    Cystic fibrosis is a single gene, autosomal recessive disorder, in which more than 1,900 mutations grouped into 6 classes have been described. It is an example a disease that could be well placed to benefit from personalised medicine. There are currently 2 very different approaches that aim to correct the basic defect: gene therapy, aimed at correcting the genetic alteration, and therapy aimed at correcting the defect in the CFTR protein. The latter is beginning to show promising results, with several molecules under development. Ataluren (PTC124) is a molecule designed to make the ribosomes become less sensitive to the premature stop codons responsible for class i mutations. Lumacaftor (VX-809) is a CFTR corrector directed at class ii mutations, among which Phe508del is the most frequent, with encouraging results. Ivacaftor (VX-770) is a potentiator, the only one marketed to date, which has shown good efficacy for the class iii mutation Gly551Asp in children over the age of 6 and adults. These drugs, or a combination of them, are currently undergoing various clinical trials for other less common genetic mutations. In the last 5 years, CFTR has been designated as a therapeutic target. Ivacaftor is the first drug to treat the basic defect in cystic fibrosis, but only provides a response in a small number of patients. New drugs capable of restoring the CFTR protein damaged by the most common mutations are required. PMID:24095197

  14. Randomized Clinical Trial to Assess the Efficacy of Radiotherapy in Primary Mediastinal Large B-Lymphoma

    SciTech Connect

    Aviles, Agustin; Neri, Natividad; Fernandez, Raul; Huerta-Guzman, Judith; Nambo, Maria J.

    2012-07-15

    Purpose: We developed a controlled clinical trial to assess the efficacy and toxicity of adjuvant-involved field radiotherapy (IFRT) in patients with primary mediastinal B-cell lymphoma that achieved complete response after the patients were treated with cyclophosphamide, doxorubicin, vincristine, prednisone, and rituximab (R-CHOP-14). Methods and Materials: Between January 2001 and June 2004, 124 consecutive patients who were in complete remission after dose dense chemotherapy and rituximab administration (R-CHOP14) were randomly assigned to received IFRT (30 Gy). Sixty-three patients received IFR, and 61 patients did not (control group). Results: The study aimed to include 182 patients in each arm but was closed prematurely because in a security analysis (June 2004), progression and early relapse were more frequent in patients that did not received IFRT. Patients were followed until March 2009, at which point actuarial curves at 10 years showed that progression free-survival was 72% in patients who received IFR and 20% in the control group (p < 0.001), overall survival was 72% and 31%, respectively (p < 0.001). Acute toxicity was mild and well tolerated. Discussion: Adjuvant radiotherapy to sites of bulky disease was the only difference to have an improvement in outcome in our patients; the use of rituximab during induction did not improve complete response rates and did affect overall survival; patients who received rituximab but not IFRT had a worse prognosis. Conclusions: The use of IFRT in patients with primary mediastinal B-cell lymphoma who achieved complete response remain as the best treatment available, even in patients that received rituximab during induction.

  15. Is post-sternotomy mediastinitis still devastating after the advent of negative-pressure wound therapy?

    PubMed

    De Feo, Marisa; Della Corte, Alessandro; Vicchio, Mariano; Pirozzi, Francesco; Nappi, Gianantonio; Cotrufo, Maurizio

    2011-01-01

    In this study, we reviewed a 15-year experience with the treatment of a severe sequela of cardiac surgery: post-sternotomy mediastinitis. We compared the outcomes of conventional treatment with those of negative-pressure wound therapy, focusing on mortality rate, sternal reinfection, and length of hospital stay.We reviewed data on 157 consecutive patients who were treated at our institution from 1995 through 2010 for post-sternotomy mediastinitis after cardiac surgery. Of these patients, 74 had undergone extensive wound débridement followed by negative-pressure wound therapy, and 83 had undergone conventional treatment, including primary wound reopening, débridement, closed-chest irrigation without rewiring, topical application of granulated sugar for recurrent cases, and final plastic reconstruction with pectoral muscle flap in most cases.The 2 study groups were homogeneous in terms of preoperative data and operative variables (the primary cardiac surgery was predominantly coronary artery bypass grafting). Negative-pressure wound therapy was associated with lower early mortality rates (1.4% vs 3.6%; P = 0.35) and significantly lower reinfection rates (1.4% vs 16.9%; P = 0.001). Significantly shorter hospital stays were also observed with negative pressure in comparison with conventional treatment (mean durations, 27.3 ± 9 vs 30.5 ± 3 d; P = 0.02), consequent to the accelerated process of wound healing with negative-pressure therapy.Lower mortality and reinfection rates and shorter hospital stays can result from using negative pressure rather than conventional treatment. Therefore, negative-pressure wound therapy is advisable as first-choice therapy for deep sternal wound infection after cardiac surgery.

  16. [Paraneoplastic limbic encephalitis with positive anti-RI antibodies and mediastinal seminoma].

    PubMed

    Launay, M; Bozzolo, E; Venissac, N; Delmont, E; Fredenrich, A; Thomas, P

    2008-01-01

    We report the case of a 49-year-old man who was admitted for progressive behaviorial disorders with frontal elements. There was no sensorial nor motor deficiency. Clinical examination revealed android obesity, cutaneous and mucous paleness, pubic and axillary depilation and gynecomastia. Encephalic MRI found a lesion of the left amygdalian region with high T2 intensity and low T1 intensity associated with gadolinium-enhancement. Cerebrospinal fluid analysis showed a lymphocytic meningitis. Panhypopituitarism was found on the endocrine investigations. Anti-RI antibodies were positive, leading to the diagnosis of paraneoplastic limbic encephalitis. The CT-scan showed a node of the lower part of the thymic area. Surgical resection revealed an ectopic mediastinal seminoma. The evolution consisted of paraneoplastic fever and crossed-syndrome with right hemiparesia and left common oculomotor nerve paralysis. Treatment was completed by two cycles of carboplatin, corticosteroids and substitutive opotherapy. Paraneoplastic fever disappeared, but behavioral disorders and palsy remain unchanged. The patient died two years later in a bedridden state. This case of paraneoplastic limbic encephalitis associated with positive anti-RI antibodies and mediastinal seminoma is exceptional and has not to our knowledge been described in the literature. Cancers usually associated with anti-RI antibody are breast and lung cancer. Paraneoplastic limbic encephalitis is not the classical clinical presentation, which usually is brainstem encephalitis. Hypothalamic involvement, uncommon in paraneoplastic limbic encephalitis is mainly associated with positive antineuronal anti-Ma2 antibodies. Finally, the gadolinium enhancement on encephalic MRI is unusual in paraneoplastic limbic encephalitis. PMID:18565362

  17. Pancreatic pseudocyst or a cystic tumor of the pancreas?

    PubMed Central

    Rabie, Mohammad Ezzedien; El Hakeem, Ismail; Al Skaini, Mohammad Saad; El Hadad, Ahmad; Jamil, Salim; Tahir Shah, Mian; Obaid, Mahmoud

    2014-01-01

    Pancreatic pseudocysts are the most common cystic lesions of the pancreas and may complicate acute pancreatitis, chronic pancreatitis, or pancreatic trauma. While the majority of acute pseudocysts resolve spontaneously, few may require drainage. On the other hand, pancreatic cystic tumors, which usually require extirpation, may disguise as pseudocysts. Hence, the distinction between the two entities is crucial for a successful outcome. We conducted this study to highlight the fundamental differences between pancreatic pseudocysts and cystic tumors so that relevant management plans can be devised. We reviewed the data of patients with pancreatic cystic lesions that underwent intervention between June 2007 and December 2010 in our hospital. We identified 9 patients (5 males and 4 females) with a median age of 40 years (range, 30–70 years). Five patients had pseudocysts, 2 had cystic tumors, and 2 had diseases of undetermined pathology. Pancreatic pseudocysts were treated by pseudocystogastrostomy in 2 cases and percutaneous drainage in 3 cases. One case recurred after percutaneous drainage and required pseudocystogastrostomy. The true pancreatic cysts were serous cystadenoma, which was treated by distal pancreatectomy, and mucinous cystadenocarcinoma, which was initially treated by drainage, like a pseudocyst, and then by distal pancreatectomy when its true nature was revealed. We conclude that every effort should be exerted to distinguish between pancreatic pseudocysts and cystic tumors of the pancreas to avoid the serious misjudgement of draining rather than extirpating a pancreatic cystic tumor. Additionally, percutaneous drainage of a pancreatic pseudocyst is a useful adjunct that may substitute for surgical drainage. PMID:23958054

  18. A Case of a Methicillin-Resistant Staphylococcus Aureus Retropharyngeal Abscess With Mediastinal Extension in an 11-Month-Old Girl.

    PubMed

    Shanti, Rabie M; Hastings, Mary L; Patel, Stavan; Yeoh, Melvyn S

    2016-04-01

    We present the case of an 11-month-old girl with Mycoplasma pneumoniae-associated pneumonia who was subsequently diagnosed with a methicillin-resistant Staphylococcus aureus retropharyngeal abscess with mediastinal extension. PMID:26541226

  19. Cystic Fibrosis Transmembrane Conductance Regulator. Implications in Cystic Fibrosis and Chronic Obstructive Pulmonary Disease.

    PubMed

    Cantin, André M

    2016-04-01

    Cystic fibrosis (CF) and chronic obstructive pulmonary disease (COPD) have traditionally been viewed as two distinct entities of unrelated origins. However, molecular, cellular, and clinical studies have revealed that cystic fibrosis transmembrane conductance regulator (CFTR) protein dysfunction is common to both conditions, one (CF) being defined genetically and the other (COPD) as an acquired CFTR deficiency. Multiple molecular mechanisms of cigarette smoke-induced CFTR dysfunction have been reported. More importantly, considerable evidence of cigarette smoke-induced CFTR dysfunction in several respiratory and nonrespiratory tissues have been confirmed, making CFTR a target that cannot be overlooked in our quest to understand COPD and improve therapies for individuals affected by this disease. This review summarizes the molecular, cellular, and clinical evidence that CFTR dysfunction is induced by cigarette smoke exposure both in vitro and in vivo, and explores how this may contribute to the development of COPD. PMID:27115950

  20. Late presentation of congenital cystic adenomatoid malformation of the lung

    SciTech Connect

    Hulnick, D.H.; Naidich, D.P.; McCauley, D.I.; Feiner, H.D.; Avitabile, A.M.; Greco, M.A.; Genieser, N.B.

    1984-06-01

    Although most often recognized in neonates and young children, congenital cystic adenomatoid malformation of the lung (CCAM) occasionally appears in later years. Three patients, aged 35, 24, and 7 years, are reported. Chest radiographs in each case suggested a localized patchy density, a cystic mass, or a multicystic mass, but computed tomography (CT) best demonstrated the cystic and solid components while ruling out bronchiectasis or major bronchial obstruction. Bronchography contributed no further diagnostic information compared with CT. Each patient underwent lobectomy. Histologically, the characteristic overgrowth of bronchiolar elements replacing normal parenchymal architecture was accompanied by some superimposed inflammatory change.

  1. Remnant cystic duct adenocarcinoma presenting as gastric outlet obstruction

    PubMed Central

    Lo, Samuel Tsoon Wuan; Cheng, Yue; Cheung, Frances; Tang, Chung Ngai

    2016-01-01

    Only a few case reports of remnant cystic duct carcinoma exist. The presented case of remnant cystic duct carcinoma with invasion to pylorus and bulbus of duodenum leading to gastric outlet obstruction was the first of its kind. We reviewed all cases of remnant cystic duct carcinoma that we found in the literature and summarized its definition, presentation, extent of invasion and clinical outcome after operation. The diagnosis can be difficult due to the rarity of disease, locally advanced nature of disease and distorted postoperative anatomy. A high index of suspicion can increase the likelihood of a preoperative diagnosis. PMID:27154747

  2. Flow in Idealised Compliant Human Cystic Duct Models

    NASA Astrophysics Data System (ADS)

    Al-Atabi, M.; Chin, S. B.; Beck, S.; Luo, X. Y.

    The functions of the cystic duct and the role of its complicated geometry (in particular the valves of Heister), in the delivery of bile flow have always been a subject of speculation. This paper reports on an experimental investigation of the flow in idealised, compliant cystic duct models. The valves of Heister were idealised using eight semi-circular alternating baffles fitted inside the compliant tubes. These tubes were arranged in configurations representing the anatomical configurations of real cystic ducts. Models both with and without baffles were tested for comparison. The results show that the valves of Heister seem to play a role in facilitating both the filling and emptying of the gallbladder.

  3. Cystic change in primary paediatric optic nerve sheath meningioma.

    PubMed

    Narayan, Daniel; Rajak, Saul; Patel, Sandy; Selva, Dinesh

    2016-08-01

    Primary optic nerve sheath meningiomas (PONSM) are rare in children. Cystic meningiomas are an uncommon subgroup of meningiomas. We report a case of paediatric PONSM managed using observation alone that underwent cystic change and radiological regression. A 5-year-old girl presented with visual impairment and proptosis. Magnetic resonance (MR) imaging demonstrated a PONSM. The patient was left untreated and followed up with regular MR imaging. Repeat imaging at 16 years of age showed the tumour had started to develop cystic change. Repeat imaging at 21 years of age showed the tumour had decreased in size. PMID:27310300

  4. Mediastinal Packing for Intractable Coagulopathy in Acute Aortic Dissection (Types 1 and 2 DeBakey): A Life-Saving Technique—Report of Experiences

    PubMed Central

    Moeinipour, Aliasghar; Fathi, Mehdi; Sepehri Shamloo, Alireza; Amini, Shahram; Hoseinikhah, Hamid; Kianinejad, Akram

    2015-01-01

    Nonsurgical bleeding after complex thoracic aortic procedures (such as aortic dissection and aortic aneurysm) is a great challenge for cardiac surgeons because of severe coagulopathy, exsanguinous bleeding, and inevitable death. Temporary mediastinal packing (with sponge) in such cases is the only life-saving technique with good result in most cases. Herein, we presented three cases with acute aortic dissection with intractable bleeding that was successfully managed with mediastinal packing. PMID:26435855

  5. Influenza vaccination in children with cystic fibrosis.

    PubMed

    Patria, Maria Francesca; Longhi, Benedetta; Esposito, Susanna

    2013-04-01

    Cystic fibrosis (CF) is an inherited autosomal recessive disease characterized by progressive pulmonary damage and respiratory failure. It is known that bacterial infections play a critical role in the development of significant lung damage, whereas the role of respiratory viruses in CF pulmonary exacerbations and the relationship between viral infections and the progression of lung damage are uncertain. Health authorities throughout the world recommend influenza vaccination for CF patients. The aim of this review is to analyze the impact of seasonal and pandemic influenza on CF patients and data concerning influenza vaccination in order to assess the current situation and identify areas for future study. As data are limited, further well-constructed clinical studies of the effectiveness of influenza vaccination on the main clinical outcome measures of pulmonary function and nutritional status in patients with CF are required.

  6. Control programme for cystic echinococcosis in Uruguay

    PubMed Central

    Irabedra, Pilar; Ferreira, Ciro; Sayes, Julio; Elola, Susana; Rodríguez, Miriam; Morel, Noelia; Segura, Sebastian; dos Santos, Estela; Guisantes, Jorge A

    2016-01-01

    Cystic echinococcosis is a highly endemic parasitic zoonosis that is present in the Southern Cone countries of America. For several decades, various prevention and control programmes have been implemented in different countries and regions, with varying results. In Uruguay, a new control programme was implemented in 2006 that employed new strategies for canine diagnosis and treatment, dog population control, diagnosis in humans, epidemiological surveillance, and health education, including community participation. The control programme in Uruguay addresses the control and surveillance of the disease from a holistic perspective based on Primary Health Care, which has strengthened the community’s participation in developing and coordinating activities in an interdisciplinary manner. Similarly, the control programme that is currently implemented is based on a risk-focused approach. The surveillance and control measures were focused on small villages and extremely poor urban areas. In this study, the strategies used and the results obtained from 2008-2013 are analysed and discussed. PMID:27223652

  7. Poly Cystic Ovarian Syndrome: An Updated Overview

    PubMed Central

    El Hayek, Samer; Bitar, Lynn; Hamdar, Layal H.; Mirza, Fadi G.; Daoud, Georges

    2016-01-01

    Poly Cystic Ovarian Syndrome (PCOS) is one of the most common metabolic and reproductive disorders among women of reproductive age. Women suffering from PCOS present with a constellation of symptoms associated with menstrual dysfunction and androgen excess, which significantly impacts their quality of life. They may be at increased risk of multiple morbidities, including obesity, insulin resistance, type II diabetes mellitus, cardiovascular disease (CVD), infertility, cancer, and psychological disorders. This review summarizes what the literature has so far provided from guidelines to diagnosis of PCOS. It will also present a general overview about the morbidities associated with this disease, specifically with its more severe classic form. Finally, the review will stress on the various aspects of treatment and screening recommendations currently used in the management of this condition. PMID:27092084

  8. Scoliosis in cystic fibrosis - an appraisal

    SciTech Connect

    Paling, M.R.; Spasovsky-Chernick, M.

    1982-03-01

    An unusually high prevalence (10%) of scoliosis is described in a series of 151 patients aged four years and older with cystic fibrosis. The scolioses were of the late onset (juvenile and adolescent) type, being typically thoracic with the curve convex to the right, although there was no significant preference for either sex. No direct relationship was found between the spinal curvature and the severity or distribution of the lung disease, although the worse scolioses tended to occur in patients with relatively severe pulmonary involvement. There was no evidence of metabolic bone disease as a predisposing cause. Some indication of a familial tendency towards scoliosis was apparent, and a genetic or constitutional basis is postulated with an unknown precipitating factor.

  9. Retroperitoneal bronchogenic cyst mimicking pancreatic cystic lesion.

    PubMed

    Wang, Shin-E; Tsai, Yi-Fang; Su, Cheng-Hsi; Shyr, Yi-Ming; Lee, Rheun-Chuan; Tsai, Wan-Chen; Li, Fen-Yau; Chen, Tien-Hua; Wu, Chew-Wun; Lui, Wing-Yiu

    2006-11-01

    Retroperitoneal bronchogenic cyst is detected extremely rarely and often masquerades as other diseases. Here, we report 2 cases of retroperitoneal bronchogenic cyst mimicking pancreatic mucinous tumor. Histologically, both cysts were composed of ciliated respiratory-like epithelium with abundant mucin content, smooth muscle bundles and mature cartilage, compatible with the diagnosis of retroperitoneal bronchogenic cyst. In addition to these 2 cases, another 42 retroperitoneal bronchogenic cysts reported in the English literature were collected for review and analysis. Twelve (28%) were located over the peripancreatic area. Just over half (51%) of them were asymptomatic. No accurate preoperative diagnosis could be made for any of the lesions. About a third (33.3%) of the peripancreatic retroperitoneal bronchogenic cysts masqueraded as pancreatic cystic lesions.

  10. Chronic Rhinosinusitis in Patients with Cystic Fibrosis.

    PubMed

    Hamilos, Daniel L

    2016-01-01

    Chronic rhinosinusitis (CRS) is highly prevalent in patients with cystic fibrosis (CF) and accounts for significant morbidity and contribution to CF lung disease. Mutations of the cystic fibrosis transmembrane regulator gene occur with increased prevalence in patients with CRS without CF, suggesting some contribution to CRS pathophysiology. Nasal polyps (NPs) occur with increased prevalence in patients with CF of all ages and have a more neutrophilic appearance with fewer eosinophils and increased submucosal glandular elements in comparison to NPs from patients without CF. Mainstays of medical treatment include isotonic saline irrigations and topical intranasal glucocorticoids, with some evidence that topical intranasal glucocorticoids reduce NP size. Although inhaled hypertonic saline (7%) has been widely studied as a mucolytic agent for CF lung disease, there are no reports of its use in CF CRS. Mucolytics have also not been studied as a treatment for CRS in CF, and most evidence does not support their use for CF lung disease. Nasally nebulized dornase alfa (recombinant human deoxyribonuclease) following sinus surgery shows promise for treatment. Other unproven therapies include addition of baby shampoo to isotonic saline to potentially thin mucus and help prevent biofilm formation. There are no data to support the use of low-dose oral macrolide antibiotics or the use of prophylactic oral antibiotics for CRS in patients with CF. However, there is some support for the use of topical antibiotics, including colistimethate sodium or tobramycin, administered as a sinus irrigation or antral lavage in patients following sinus surgery when susceptible bacteria are cultured. Key components of CF sinus surgical management include extensive surgery to ensure that the maxillary, frontal, sphenoid, and ethmoid sinuses are all widely opened with smoothing of bony overhangs to prevent mucus retention and bacterial recolonization, postoperative meticulous daily nasal irrigations

  11. Cystic fibrosis mutations in Costa Rica.

    PubMed

    Venegas, Patricia B; Novak, Jessica M; Oscar, Castro A; Sánchez, Félix L; Gutiérrez, Inés G; Rivera, Julio M; Salas, Jorge P; Montero, Jenny F; Grody, Wayne W

    2003-04-01

    Using polymerase chain reaction amplification of DNA in dried blood spots and a nonisotopic reverse dot blot hybridization method, we performed molecular genetic analysis for 6 and for 16 of the most common mutations of the cystic fibrosis transmembrane conductance regulator gene (CFTR) in 24 unrelated Costa Rican individuals with cystic fibrosis (CF). While many countries and ethnic groups have been surveyed for CF mutations since the cloning of CFTR, Costa Rica has not heretofore been studied. Moreover, Costa Rica represents an especially intriguing population because of its mixed European-African-Amerindian origins and the existence of a detailed historical record of the founding Spanish families. Thus, such a study may reveal not only the population frequencies of various mutant alleles in this country, but also something about their geographic migrations and ethnic founder effects. The most common CF mutation in Caucasians, deltaF508, was found in only 11 (23%) of the CF chromosomes studied, while the G542X mutation, relatively rare in the general population but more common in southern Europe, was observed in 12 (25%). None of the other mutations tested was found in any of the subjects. We failed to detect the second mutant allele in 17 subjects and could not detect either allele in 4 subjects. The high prevalence of the G542X mutation in our cohort, which exceeds that of both the general Caucasian population and the American Hispanic population, reflects the strong genetic influence of the original Spanish founding families of Costa Rica. These results highlight important differences in Costa Rican CF genotypes as compared both to other North American and European populations and to American Hispanics, raising important implications about isolated founder effects and strategies for population screening in that country.

  12. Initial experience with real-time elastography using an ultrasound bronchoscope for the evaluation of mediastinal lymph nodes.

    PubMed

    Andreo García, Felipe; Centeno Clemente, Carmen Ángela; Sanz Santos, José; Barturen Barroso, Ángel; Hernández Gallego, Alba; Ruiz Manzano, Juan

    2015-02-01

    Real-time elastography performed during endoscopic ultrasonography is a relatively new method for characterizing tissue stiffness, and has been used successfully as a predictor of malignancy in mediastinal lymph nodes. This case report describes our practical experience with this technique using an ultrasound bronchoscope to examine mediastinal lymph nodes. We present a case of sectorial endobronchial ultrasound and the first published case of endoscopic ultrasound elastography using ultrasound bronchoscope in two patients with non-small cell lung carcinoma. Qualitative tissue color pattern was obtained in both cases and correlated with pathological evaluation. The initial feasibility results are promising and suggest that ultrasound bronchoscopy techniques, such as guided nodal staging, merit additional studies. It may be important to categorize the risk of malignancy to facilitate sampling decisions. PMID:25443586

  13. The inflatable thymus herniation of the normal mediastinal thymus: A case report and review of the literature.

    PubMed

    Stuut, Marijn; van Zwieten, Gusta; Straetmans, Jos M; Lacko, Martin; Stumpel, Constance T R M

    2016-04-01

    Anterior neck masses in young children can be a diagnostic challenge for otolaryngologists and radiologists. We present a rare case of herniation of normal mediastinal thymus in a four-year-old girl. Additional medical features as an inguinal hernia and trochlear nerve paresis raised the question whether there is a causal relationship or an association. A connective tissue disorder could not be diagnosed as possible causal factor to the abnormal movement of the mediastinal thymus. Awareness and recognition of this benign phenomenon is important in order to avoid unnecessary biopsy or surgery. Diagnosis can be confirmed by ultrasonography. Magnetic Resonance Imaging might be valuable in order to obtain more information about the extension of the mass. PMID:26968057

  14. Occult Mediastinal Great Vessel Trauma: The Value of Aortography Performed During Angiographic Screening for Blunt Cervical Vascular Trauma

    SciTech Connect

    Ray, Charles E. Bauer, Jason R.; Cothren, C. Clay; Turner, James H.; Moore, Ernest E.

    2005-05-15

    Purpose. To determine the value of aortography in the assessment of occult aortic and great vessel injuries when routinely performed during screening angiography for blunt cerebrovascular injury (BCVI). Methods. One hundred and one consecutive patients who received both aortography and screening four-vessel angiography over 4 years were identified retrospectively. Angiograms for these patients were evaluated, and the incidence of occult mediastinal vascular injury was determined. Results. Of the 101 patients, 6 (6%) had angiographically documented traumatic aortic injuries. Of these 6 patients, one injury (17%) was unsuspected prior to angiography. Four of the 6 (67%) also had BCVI. One additional patient also had an injury to a branch of the subclavian artery. Conclusion. Routine aortography during screening angiography for BCVI is not warranted due to the low incidence (1%) of occult mediastinal arterial injury. However, in the setting of a BCVI screening study and no CT scan of the chest, aortography may be advantageous.

  15. Initial experience with real-time elastography using an ultrasound bronchoscope for the evaluation of mediastinal lymph nodes.

    PubMed

    Andreo García, Felipe; Centeno Clemente, Carmen Ángela; Sanz Santos, José; Barturen Barroso, Ángel; Hernández Gallego, Alba; Ruiz Manzano, Juan

    2015-02-01

    Real-time elastography performed during endoscopic ultrasonography is a relatively new method for characterizing tissue stiffness, and has been used successfully as a predictor of malignancy in mediastinal lymph nodes. This case report describes our practical experience with this technique using an ultrasound bronchoscope to examine mediastinal lymph nodes. We present a case of sectorial endobronchial ultrasound and the first published case of endoscopic ultrasound elastography using ultrasound bronchoscope in two patients with non-small cell lung carcinoma. Qualitative tissue color pattern was obtained in both cases and correlated with pathological evaluation. The initial feasibility results are promising and suggest that ultrasound bronchoscopy techniques, such as guided nodal staging, merit additional studies. It may be important to categorize the risk of malignancy to facilitate sampling decisions.

  16. Concurrent Nontuberculous Mycobacteria Infection and High-Grade Anterior Mediastinal Extraskeletal Osteosarcoma (ESOS): Is There a Connection?

    PubMed

    Faz, Gabriel T; Eltorky, Mahmoud; Karnath, Bernard

    2016-01-01

    BACKGROUND Extraskeletal osteosarcomas (ESOS) of the mediastinum are extremely rare and may present with concurrent nontuberculous mycobacteria infection. CASE REPORT We present the second documented case of high-grade anterior mediastinal extraskeletal osteosarcoma in a 59-year-old man with a history of treated, latent tuberculosis (TB). Sputum samples grew Mycoplasma avium complex and Mycobacterium fortuitum. Imaging showed a right-sided 7.6 cm mass with compression of the main bronchus. Subsequent biopsy with vimentin staining established the diagnosis of ESOS. Due to the patient's rapidly declining performance status, he was not deemed a candidate for surgery or chemotherapy. He subsequently expired within one month of presentation. CONCLUSIONS We present a unique case of high-grade anterior mediastinum ESOS and a review of the literature regarding all documented cases of ESOS to date. We suggest there is a possible link between mediastinal masses and nontuberculous mycobacteria infection. PMID:27539718

  17. [Mature cystic teratoma of the ovary with a small ganglioneuroma].

    PubMed

    Marucci, G; Collina, G

    2006-02-01

    A case of ganglioneuroma arising within a cystic mature teratoma of the ovary in a 34-year-old woman is reported. Patient underwent right adnexectomy. The ovary was completely replaced by a bilocular cystic lesion, measuring 8 cm in diameter and filled with adipose tissue and pilosebaceous material. Microscopically the cyst was composed by a mature cystic teratoma containing skin with dermal appendages, fatty tissue and bronchial epithelium. Furthermore a nodule (0.5 cm in size) composed of mature ganglion cells, axons and Schwann cells, was identified. Ganglion cells were positive for NSE and synaptophysin, while Schwann cells stained positively with S100 protein and GFAP. To the best of our knowledgment this is the first reported cases of ganglioneuroma arisen within a cystic mature teratoma of the ovary.

  18. Hydrator Therapies for Chronic Bronchitis. Lessons from Cystic Fibrosis.

    PubMed

    Bennett, William D; Henderson, Ashley G; Donaldson, Scott H

    2016-04-01

    Patients with the chronic bronchitis form of chronic obstructive pulmonary disease and cystic fibrosis share similar clinical features, including mucus obstruction of airways and the development of chronic/recurrent airways infections that often manifest as disease exacerbations. There is growing evidence that these diseases may have parallels in disease pathogenesis as well, including cystic fibrosis transmembrane conductance regulator dysfunction, mucus dehydration, and defective mucociliary clearance. As progress is made in the development of therapies that target the basic defects that lead to cystic fibrosis lung disease, it is possible that similar approaches could also benefit patients with chronic bronchitis. A deeper understanding of how tobacco smoke and other triggers of chronic bronchitis actually lead to disease, and exploration of the concept that therapies that restore cystic fibrosis transmembrane conductance regulator function, mucus hydration, and/or mucociliary clearance may benefit patients with chronic bronchitis, hold the prospect of significant progress in treating this prevalent disease. PMID:27115955

  19. [A method for the combined treatment of chronic cystic sinusitis].

    PubMed

    Svatko, L G; Krasnozhen, V N; Pokrovskaia, E M

    2008-01-01

    A pathogenetically substantiated method is proposed for the combined treatment of chronic cystic sinusitis that includes sparing surgical intervention and postoperative treatment with ximedone, a regenerator drug with immunotropic activity. PMID:19156109

  20. Florid Cystic Endosalpingiosis (Müllerianosis) in Pregnancy

    PubMed Central

    Montero-Balaguer, Beatriz; Desantes-Real, Domingo; Perales-Marín, Alfredo

    2016-01-01

    Cystic endosalpingiosis refers to the existence of heterotopic cystic müllerian tissue resembling structures of the fallopian tubes. We report a case of florid cystic endosalpingiosis discovered in a pregnant woman during a scheduled cesarean section and review the current knowledge of this disease. A 30-year-old woman with a twin pregnancy attended the hospital day unit at term. The first twin was in a breech presentation and a cesarean section was scheduled. During the procedure the uterine fundus and part of the body were seen completely seeded with multitude of cyst-like structures resembling hydatids of Morgagni. The immunohistochemistry analysis showed a positive expression for PAX8 (Box-8), CK7, and estrogen and progesterone receptors. The lesions did not disappear after pregnancy. Cystic endosalpingiosis should be always borne in mind, even in pregnancy, when it comes to making the differential diagnosis of a pelvic or systemic multicystic mass. PMID:27668111