PARAGANGLIOMA OF THE ORGAN OF ZUCKERKANDL : REPORT OF A CASE

Öz

Tumors of the paraganglionic system will arise; these are termed paragangliomas, as they may be both physiologically and histopathologically similar to pheochromocytomas of the adrenal gland. Extra-adrenal paragangliomas make up approximately ten per cent of all paragangliomas, with those occuning in the organ of Zuckerkandl being the most common We describe a case of paraganglioma in a 51-year-old woman with hypertension being present for 32 years. She suffered from abdominal mass, nausea, dizziness, emesis, headache, excessive sweating, palpitations, and back pain. An abdominal mass was diagnosed as a functional extra-abdominal paraganglioma by diagnostic imaging and biochemical tests. The mass was totally excised and her symptoms healed after the operation. Symptoms of tumors of the organ of Zuckerkandl are secondary to synthesis and release of excess catecholamines. This is a rare case of functional paraganglioma arising from the organs of Zuckerkandl with successful surgical removal and amelioration of hypertension.

Anahtar Kelimeler

• Organ of Zuckerkandl,Paraganglioma

ZUCKERKANDL ORGANI PARAGANGLiOMASI: VAKA TAKDiMi

Öz

Retroperitoneal paragangliomalar; aorta cevresinde sempatik zincirle birlikte dagilnus olan ozellesrnis noral krest hucrelerinden kaynaklanrnaktadrr. Bu makalede 32 yillik hipertansiyon hikayesi olan 52 yasmda bir bayan hasta paraganglioma vakasi olarak takdim edilmektedir. Hastada kannda kitle, bulanti, kusma, bas agnsi, bas donrnesi, asm terleme, carpmn ve sut agnsr gibi sikayetler mevcuttu. Yapilan radyolojik ve biyokimyasal incelemeler, kanndaki kitlenin aktif fonksiyon gosteren ekstraabdominal bir paraganglioma oldugunu gosterdi. Kitlenin tamarru eksize edildi ve ameliyat sonrasi hastanm sernptomlan normale dondu.

Anahtar Kelimeler

• Paraganglioma,Zuckerkandl Organı

Kaynakça

1. [1] Holden, A., 1995, Non-functional malignant extra-adrenal retroperitoneal paraganglioma. Australas Radiol, 39:392-395.
2. [2] Erlandson, RA., Nesland, J.M, 1994, Tumors of the endocrine/neuroendocrine system: an overview. Ultrastruct Pathol,18:149-170.
3. [3] Koch, e.A., Vortmeyer, A.a., Huang, S.e., Alesci, S., Zhuang, Z., Pacak, K., 2001, Genetic aspects of pheochromocytoma. Endocr Regul, 35:43-52.
4. [4] Marx, S., Spiegel, A.M., Skarulis, M.e., Doppmann, J.L., Collins, F.S., Liotta, L.A., 1998, Multiple endocrine neoplasia type 1: clinical and genetic topics. Ann Intern Med, 129:484- 494.
5. [5] Sutton, M.G., Sheps, S.G., Lie, IT., 1981, Prevalence of clinically unsuspected pheochromocytoma: review of a 50-year autopsy series. Mayo Clin Proc, 56:354-360.
6. [6] Manger, W.M., Gifford, RW., 1982, Hypertension secondary to pheochromocytoma. Bull N Y Acad Med, 58:139-158.
7. [7] Davidson, D.F., 1987, Urinary free catecholamines--diagnostic application of an HPLC technique to the investigation of neural crest tumours. Ann Clin Biochem, 24: 494-499.
8. [8] Geelhoed, G.W., 1980, CAT scans and catecholamines. Surgery, 87:719-720.

9. [9] Salanitri, J., Smith, P., Schlicht, S., 2001, Multifocal malignant extra adrenal paragangliomas of the Organ of Zuckerkandl and urinary bladder. Australas Radiol,45:229-232.
10. [10] Quint, L.E., Glazer, G.M., Francis, I.R, Shapiro, B., Chenevert, T.L., Pheochromocytoma and paraganglioma: comparison of MR imaging with CT and 1-131 MIBG scintigraphy. Radiology, 165:89-93.