Mehtap EVRAN, Gamze AKKUŞ, Murat SERT, Tamer TETİKER

Familyal Feokromasitoma Semptomlarıyla Başvuran Nadir Bir Olgu

Feokromasitoma 100.000 kişide bir görülebilen nadir tümörlerden birisidir. Genel olarak adrenal medulladan katekolamin salınımı ile karakterizedir. Kırk altı yaşında erkek hasta baş ağrısı, terleme, çarpıntı şikayeti ile kliniğimize başvurdu. Hastaya feokromasitoma tanısı konuldu. Bu yazıda ailesinde feokromasitomaya bağlı morbidite ve ölüm öyküsü olan bir hasta aktarılmıştır.

A Rare Case Presenting with Symptoms of Familial Pheochromacytoma

Pheochromocytoma is a rare tumor in which seen an incidence of 1 per 100.000 in the general population. Pheochromocytoma is a catecholamine producing neuroendocrine tumor arising from adrenal medulla. A 46-year-old man experienced headache, sweating and palpitation referred to our hospital. Familial pheocromocytoma was detected. We presented this case as whole family members had suffered from morbidities and deaths due to this disorder

Keywords:

Pheocromocytoma, familial disorder, hypertension,

PDF

___

Santos P, Pimenta T, Taveira-Gomes A. Hereditary pheochromocytoma. Int J Surg Pathol. 2014;22:393- 400
Kaltsas GA, Papadogias D, Grossman AB. The clinical presentation (symptoms and signs) of sporadic and familial chromaffin cell tumours (phaeochromocytomas and paragangliomas) Front Horm Res. 2004;31:61-75.
DeLellis R, Lloyd P, Heitz C. Pathology and Genetics of Tumours of Endocrine Organs (IARC WHO Classification of Tumours), IARC Press, 2004 Lyon, France. Martins R,
Paragangliomas/Pheochromocytomas: Oriented Genetic Testing. Int J Endocrinol. 2014; 2014: 794187. doi: 10.1155/2014/794187. Epub 2014 May Bugalho MJ. Clinically
Van Berkel A, Lenders JW, Timmers HJ. Diagnosis of endocrine pheochromocytoma and paraganglioma. Eur J Endocrinol. 2014;170:R109-119. diagnosis of
Neumann HP, Bausch B, McWhinney SR, et al. Germ-line pheochromocytoma N Engl J Med. 2002;346:1459- in nonsyndromic
Plouin PF, Duclos JM, Soppelsa F, et al Factors associated with perioperative morbidity and mortality in patients with pheochromocytoma: analysis of 165 operations at a single center J Clin Endocrinol Metab. 2001;86:1480-6.
Maher ER, Eng. C. The pressure rises: update on the genetics of phaeochromocytoma Hum Mol Genet. 2002;11:2347-54. Ilias I, Pacak K. Current approaches and the recommended localization of pheochromocytoma J Clin Endocrinol Metab. 2004;89:479-91. for diagnostic
Sahdev A, Sohaib A, Monson JP, et al CT and MR imaging of unusual locations of extra-adrenal paragangliomas (pheochromocytomas) Eur Radiol. 2005;15:85-92.
Bauters C, Vantyghem MC, Leteurtre E, et al. Hereditary paragangliomas: a study of five susceptibility genes J Med Genet. 2003;40:e75. and