3.A case of Cushing's syndrome in ACTH-secreting mediastinal paraganglioma.
Hyeong Kwan PARK ; Chang Min PARK ; Kyung Heng KO ; Myung Soo RIM ; Yu Il KIM ; Jun Hwa HWANG ; Sung Chul IM ; Yung Chul KIM ; Kyung Ok PARK
The Korean Journal of Internal Medicine 2000;15(2):142-146
Paragangliomas are unusual neuroendocrine cell tumors arising from paraganglia, of which ACTH-secreting cases in the mediastinum are extremely rare. A 51-year-old woman was admitted for generalized edema and weakness which began 5 months ago. Chest X-ray and CT scan revealed a tumor mass in the anterior mediastinum. The plasma cortisol and ACTH levels were very high. Other sources secreting ACTH, except mediastinal mass, were not found. Surgical excision of mediastinal mass and left supraclavicular lymph node was performed. The postoperative microscopic finding and immunohistochemical staining revealed organoid tumor cell nests (zellballen) and S-100 protein positive sustentacular cells which are characteristics of paraganglioma. This was thus a case of Cushing's syndrome resulting from ectopic ACTH production in anterior mediastinal paraganglioma.
Case Report
;
Corticotropin/secretion+ACo-
;
Cushing Syndrome/etiology+ACo-
;
Female
;
Human
;
Mediastinal Neoplasms/therapy
;
Mediastinal Neoplasms/secretion
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Mediastinal Neoplasms/complications+ACo-
;
Middle Age
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Paraganglioma, Extra-Adrenal/therapy
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Paraganglioma, Extra-Adrenal/secretion
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Paraganglioma, Extra-Adrenal/complications+ACo-
4.A Case of Hemorrhagic Gallbladder Paraganglioma Causing Acute Cholecystitis.
Young Up CHO ; Jang Yong KIM ; Sun Keun CHOI ; Yoon Seok HUR ; Keon Young LEE ; Sei Joong KIM ; Seung Ik AHN ; Kee Chun HONG ; Ze Hong WOO ; Jee Young HAN ; Seok Hwan SHIN
Yonsei Medical Journal 2001;42(3):352-356
Gallbladder paraganglioma is a very rare tumor and so far only a few cases have been reported. Most of these were asymptomatic and were found incidentally during operation. Recently, we experienced a gallbladder paraganglioma that gave rise to hemorrhage, which in turn caused acute cholecystitis. Our case involved a 45 year-old female patient complaining of an intermittent right upper abdominal pain. After a preoperative evaluation, cholecystectomy and lymphadenectomy were performed under the impression of gallbladder cancer with acute cholecystitis. Postoperative pathologic examination revealed a hemorrhagic gallbladder paraganglioma accompanied by acute cholecystitis. Immunohistochemical staining of the chief cells for neuron specific enolase, chromogranin and synaptophysin were positive. Sustentacular cells also stained positively for S100 protein.
Acute Disease
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Case Report
;
Cholecystitis/*etiology
;
Female
;
Gallbladder Neoplasms/*complications/pathology
;
Hemorrhage/*complications
;
Human
;
Immunohistochemistry
;
Middle Age
;
Paraganglioma/*complications/pathology
6.Clinical Presentation and Management of Jugular Foramen Paraganglioma.
Sa Myung CHUNG ; Hyun Su KIM ; Jinsei JUNG ; Ho Ki LEE ; Won Sang LEE
Clinical and Experimental Otorhinolaryngology 2009;2(1):28-32
OBJECTIVES: Jugular foramen paraganglioma is a locally invasive, benign tumor, which grow slowly and causes various symptoms such as pulsatile tinnitus and low cranial nerve palsy. Complete surgical resection is regarded as the ideal management of these tumors. The goal of this study is to identify the clinical characteristics and most effective surgical approach for jugular foramen paraganglioma. METHODS: Retrospective analysis of 9 jugular foramen paraganglioma patients who underwent surgical resection between 1986 and 2005 was performed. Clinical records were reviewed for analysis of initial clinical symptoms and signs, audiological examinations, neurological deficits, radiological features, surgical approaches, extent of resection, treatment outcomes and complications. RESULTS: Most common initial symptom was hoarseness, followed by pulsatile tinnitus. Seven out of 9 patients had at least one low cranial nerve palsy. Seven patients were classified as Fisch Type C tumor and remaining 2 as Fisch Type D tumor on radiologic examination. Total of 11 operations took place in 9 patients. Total resection was achieved in 6 cases, when partial resection was done in 3 cases. Two patients with partial resection received gamma knife radiosurgery (GKS), when remaining 1 case received both GKS and two times of revision operation. No mortality was encountered and there were few postoperative complications. CONCLUSION: Neurologic examination of low cranial nerve palsy is crucial since most patients had at least one low cranial nerve palsy. All tumors were detected in advanced stage due to slow growing nature and lack of symptom. Angiography with embolization is crucial for successful tumor removal without massive bleeding. Infratemporal fossa approach can be considered as a safe, satisfactory approach for removal of jugular foramen paragangliomas. In tumors with intracranial extension, combined approach is recommended in that it provides better surgical view and can maintain the compliance of the patients.
Angiography
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Compliance
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Cranial Nerve Diseases
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Hemorrhage
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Hoarseness
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Humans
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Hypogonadism
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Mitochondrial Diseases
;
Neurologic Examination
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Ophthalmoplegia
;
Paraganglioma
;
Postoperative Complications
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Radiosurgery
;
Retrospective Studies
;
Tinnitus
7.Carney Triad in an Adult with Aggressive Behavior: The First Case in Korea.
Hye Jong SONG ; Kyoung Mee KIM ; Dong Il CHOI ; Cheol Keun PARK
Yonsei Medical Journal 2009;50(5):709-712
Carney triad is a rare syndrome of unknown etiology characterized by having at least two out of three following neoplasms: gastrointestinal stromal tumor, pulmonary chondroma and extra-adrenal paraganglioma. About 100 cases have been reported worldwide. We report a case of Carney triad in a 42-year-old woman presented with a gastrointestinal stromal tumor in the stomach and a malignant functioning paraganglioma in the retroperitoneum that was fatal five years after diagnosis. The gastrointestinal stromal tumor was diagnosed as intermediate-risk of aggressive behavior and diffusely positive for c-kit whereas the retroperitoneal paraganglioma was negative for c-kit. Genetic analyses showed no mutations of KIT, PDGFRA, SDHB, SDHC, and SDHD genes in both tumors. To our best knowledge, this is the first case of Carney triad in Korea.
Adult
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Carney Complex/*chemically induced/complications
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Female
;
Gastrointestinal Stromal Tumors/*diagnosis/pathology
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Humans
;
Korea
;
Paraganglioma/*diagnosis/pathology
;
Retroperitoneal Neoplasms/*diagnosis/pathology
;
Stomach Neoplasms/*diagnosis/pathology
8.Granular cell tumor of appendix: report of a case.
Zongyuan YE ; Xiushan ZHANG ; Yulan ZHANG ; Junning YAN
Chinese Journal of Pathology 2014;43(4):276-277
Adult
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Antigens, CD
;
metabolism
;
Antigens, Differentiation, Myelomonocytic
;
metabolism
;
Appendectomy
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Appendiceal Neoplasms
;
complications
;
metabolism
;
pathology
;
surgery
;
Appendicitis
;
etiology
;
surgery
;
Diagnosis, Differential
;
Female
;
Granular Cell Tumor
;
complications
;
metabolism
;
pathology
;
surgery
;
Humans
;
Paraganglioma
;
metabolism
;
pathology
;
Phosphopyruvate Hydratase
;
metabolism
;
S100 Proteins
;
metabolism
9.Clinicopathological features and molecular genetic analysis of endolymphatic sac tumor: report of 2 cases.
Qiu RAO ; Xiao-jun ZHOU ; Xing-zao JIN ; Heng-hui MA ; Hang-bo ZHOU ; Zhen-feng LU
Chinese Journal of Pathology 2010;39(6):412-413
Adenoma
;
metabolism
;
pathology
;
Adult
;
Carcinoma, Renal Cell
;
metabolism
;
pathology
;
Diagnosis, Differential
;
Ear Neoplasms
;
complications
;
genetics
;
metabolism
;
pathology
;
surgery
;
Endolymphatic Sac
;
Female
;
Follow-Up Studies
;
Humans
;
Kidney Neoplasms
;
metabolism
;
pathology
;
Middle Aged
;
Paraganglioma
;
metabolism
;
pathology
;
Point Mutation
;
Von Hippel-Lindau Tumor Suppressor Protein
;
genetics
;
von Hippel-Lindau Disease
;
complications
;
genetics
;
metabolism