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Composite pheochromocytoma-ganglioneuroma is one of the mixed neuroendocrine-neural tumors composed of pheochromocytoma and other neural crest derivatives. To date, less than 50 cases of composite pheochromocytoma have been reported, and about 70% of the accompanying tumors were ganglioneuromas. Here, we describe six cases of composite pheochromocytoma-ganglioneuromas in five men and one woman, aged 33 to 64. The size of the tumors ranged from 3.0 to 11.0 cm, and four out of the six presented with intermittent onset of hypertension, palpitation, or dizziness. Microscopically, each tumor was composed of large pleomorphic shaped chromaffin cells arranged in the Zellballen patterns characteristic of pheochromocytoma, and they were mixed with clusters of mature ganglion cells and bundles of spindle-shaped Schwann cells characteristic of ganglioneuroma of variable proportions. All were successfully treated laparoscopically, and none were associated with multiple endocrine neoplasm syndrome or neurofibromatosis. Preoperative diagnosis of a composite pheochromocytoma-ganglioneuroma is impossible because of the low incidence rate, and the radiological findings and symptoms are similar to those of typical pheochromocytomas. Although the significance of microscopic detection of the nonpheochromocytoma component from pheochromocytoma has not yet been clarified, microscopic identification of the composite pheochromocytoma-ganglioneuroma is important because cumulative cases are used in an effort to predict the behavior of this composite tumor.

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참고문헌 (18건) : 자료제공( 네이버학술정보 )

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번호 참고문헌 국회도서관 소장유무
1 Menon S, Mahajan P, Desai SB: Composite adrenal medullary tumor: a rare cause of hypertension in a young male. Urol Ann 3:36-38, 2011 미소장
2 Lau SK, Chu PG, Weiss LM: Mixed cortical adenoma and composite pheochromocytoma-ganglioneuroma: an unusual corticomedullary tumor of the adrenal gland. Ann Diagn Pathol 15:185-189, 2011 미소장
3 George DJ, Watermeyer GA, Levin D, Epstein D, Ross IL, Scholz BU, Setshedi M, Locketz M, Dittrich C, Shaw J, Krige JE: Composite adrenal phaeochromocytoma-ganglioneuroma causing watery diarrhoea, hypokalaemia and achlorhydria syndrome. Eur J Gastroenterol Hepatol 22:632-634, 2010 미소장
4 Composite phaeochromocytoma with malignant peripheral nerve sheath tumour and rhabdomyosarcomatous differentiation in a patient without von Recklinghausen disease. 네이버 미소장
5 A Case of a Composite Adrenal Medullary Tumor of Pheochromocytoma and Ganglioneuroma Masquerading as Acute Pancreatitis 소장
6 A case of papillary thyroid cancer and pheochromocytoma with ganglioneuroma 소장
7 Paraganglioma with ganglioneuromatous component located in the posterior mediastinum 네이버 미소장
8 Khan AN, Solomon SS, Childress RD: Composite pheochromocytomaganglioneuroma: a rare experiment of nature. Endocr Pract 16:291-299, 2010 미소장
9 A Compound Adrenal Medullary Tumor (Pheochromocytoma and Ganglioneuroma) and a Cortical Adenoma in the Ipsilateral Adrenal Gland 네이버 미소장
10 Composite phaeochromocytoma-ganglioneuroma, an uncommon entity: report of two cases. 네이버 미소장
11 Onozawa M, Fukuhara T, Minoguchi M, Takahata M, Yamamoto Y, Miyake T, Kanagawa K, Kanda M, Maekawa I: Hypokalemic rhabdomyolysis due to WDHA syndrome caused by VIP-producing composite pheochromocytoma: a case in neurofibromatosis type 1. Jpn J Clin Oncol 35:559-563, 2005 미소장
12 Composite pheochromocytoma associated with multiple endocrine neoplasia type 2B 네이버 미소장
13 Pheochromocytoma-ganglioneuroma of the adrenal. 네이버 미소장
14 Mansmann G, Lau J, Balk E, Rothberg M, Miyachi Y, Bornstein SR: The clinically inapparent adrenal mass: update in diagnosis and management. Endocr Rev 25:309-340, 2004 미소장
15 Lam KY, Lo CY: Composite pheochromocytoma-ganglioneuroma of the adrenal gland: an uncommon entity with distinctive clinicopathologic features. Endocr Pathol 10:343-352, 1999 미소장
16 Lisewski D, Ryan S, Lim EM, Frost F, Nguyen H: Concomitant composite adrenal pheochromocytoma, multipte gastric stromal tumours and pseudohermaphrodism in a patient with von Recklinghausen’s disease. Int Semin Surg Oncol 3:11, 2006 미소장
17 Composite pheochromocytoma: a clinicopathologic and molecular comparison with ordinary pheochromocytoma and neuroblastoma. 네이버 미소장
18 Kim HH, Kim GH, Sung GT: Laparoscopic adrenalectomy for pheochromocytoma: comparison with conventional open adrenalectomy. J Endourol 18:251-255, 2004 미소장