Bizueto-Rosas H, Salazar-Reyes A, Sánchez-Fabela C, Serrato-Auld R, Muñoz-Paredes J, Hernández-Pérez NA

Language: Spanish
References: 26
Page: 132-138
PDF size: 274.00 Kb.

ABSTRACT

The paragangliomic system constituted by the adrenal medulla, carotid, aortic, and Zuckerkandl bodies and ganglion remains the sympathetic chain. It’s formed by cell migration from the neural crest. The functional tumor of chromaffin different from adrenal is called extra-adrenal pheochromocytoma and paraganglioma to nonfunctioning tumor. According to the above, the body of Zuckerkandl, miscalled paraganglioma is a extraadrenal pheochromocytoma; it may appear in the context of familiar syndrome. We present the case of a pheochromocytoma of Zuckerkandl body located between the aorta and vena cava in a 20-year-old woman, with poorly controlled hypertension, subject to excision after alpha and beta-blockers treatment. Currently asymptomatic without antihypertensive drugs. The Zuckerkandl body pheochromocytoma must be suspected in the finding of a retroperitoneal mass in the medial line with signs and symptoms of catecholaminergic activity. A MIBG-I131 gammagraphy must be performed before and after surgery to confirm the diagnosis and discard distant tumors. The surgical treatment is indicated and must be done with administration of alpha-blocker with or without beta blocker administration.

REFERENCES

1. Giménez-Bachs JM, Salinas-Sánchez AS, Lorenzo- Romero JG, Segura-Martín M, Hernández-Millán I, Barba- Romero MA, et al. Feocromocitoma del órgano de Zuckerkandl. A propósito de un caso. Actas Urol Esp 2002; 26(5): 372-6.

2. Benn DE, Gimenez-Roqueplo AP, Reilly JR, Bertherat J, Burgess J, Byth K, et al. Clinical presentation and penetrance of pheochromocytoma/paraganglioma syndromes. J Clin Endocrinol Metab 2006; 91: 827-36.

3. Karagiannis A, Mikhailidis DP, Athyros VG, Harsoulis F. Pheochromocytoma: An update on genetics and management. Endocr Relat Cancer 2007; 14: 935-56.

4. Tischler AS, Kimura N, McNicol AM. Pathology of pheochromocytoma and extra-adrenal paraganglioma. Ann N Y Acad Sci 2006; 1073: 557-70.

5. McNicol AM. Differential diagnosis of pheochromocytomas and paragangliomas. Endocr Pathol 2001; 12: 407-15.

6. Cadena M, Vergara A, Olarte A, Ospina-González D. Paraganglioma del órgano de Zuckerkandl. Revisión de tema y presentación de caso. Rev Colomb Cir 2010; 25: 309-22.

7. Lee JA, Duh QY. Sporadic Paraganglioma. World J Surg 2008; 32(5): 683-7.

8. Whalen RK, Althausen AF, Daniels GH. Extra-adrenal pheochromocitoma. J Urol 1992; 147(1): 1-10.

9. Plouin PF, Giménez-Roqueplo AP. Pheochromocytomas and secreting paragangliomas. Orphanet J Rare Dis 2006; 8(1): 49.

10. Clarke MR, Weyant RJ, Watson CG, Carty SE. Prognostic markers in pheochromocytoma. Hum Pathol 1998; 29(5): 522-6.

11. Barfield R, Hill Da, Hoffer FA, Tekautz T, Spunt SL. Retroperitoneal paraganglioma. Med Pediatr Oncol 2002; 39: 120-4.

12. Alp H, Orgak Z, Salman B. A patient with extra-adrenal retroperitoneal paraganglioma and review of the literature. J Pediatr Endocrinal Metab 1998; 11(6): 763-5.

13. McNicol AM. Histopathology and immunohistochemistry of adrenal medullary tumors and paragangliomas. Endocr Pathol 2006; 17: 329-36.

14. Glenn F, Gray GF. Functional tumors of the organ of Zuckerkandl. Ann Surg 1976; 183: 578-86.

15. Arroyo-Martínez L, Álvarez-Pertuz H, Acuña-Calvo J, Montoya- Calles J. Paraganglioma funcional extra-adrenal. Acta Med Costarric 2006; 48: 39-42.

16. Gutiérrez-Carreño AR, Sánchez-Fabela C, Lizola-Margolis RI, Sigler-Morales L, Mendieta-Hernández M. Paraganglioma del órgano de Zuckerkandl e hipertensión arterial. Caso clínico. Rev Mex Angiol 2012; 40(4): 139-43.

17. Mittendorf EA, Evans DB, Lee JE, Perrier ND. Pheochromocytoma: Advances in genetics, diagnosis, localization, and treatment. Hematol Oncol Clin North Am 2007; 21: 509-25.

18. Janetschek G, Finkenstedt G, Gasser R, Waibel UG, Peschel R, Bartsch G, et al. Laparoscopic surgery for pheochromocytoma: Adrenalectomy, partial resection, excision of paragangliomas. J Urol 1998; 160: 330-4.

19. Havekes B, Lai EW, Corssmit EP, Romijn JA, Timmers HJ, Pacak K. Detection and treatment of pheochromocytomas and paragangliomas: Current standing of MIBG scintigraphy and future role of PET imaging. Q J Nucl Med Mol Imaging 2008; 52: 419-29.

20. Sainz-Esteban A. Aportación del SPECT/CT a la Gammagrafía Convencional en el diagnóstico de los Tumores Neuroendocrinos. Valladolid. Tesis doctoral. España: Universidad de Valladolid; 2012. Disponible en: http://uvadoc.uva.es/handle/ 10324/41.

21. Kercher KW, Novitsky YW, Park A, Matthews BD, Litwin DE, Heniford BT. Laparoscopic curative resection of pheochromocytomas. Ann Surg 2005; 241: 919-26.

22. Eisenhofer G, Bornstein SR, Brouwers FM, Cheung NK, Dahia PL, de Krijger RR, et al. Malignant pheochromocytoma: Current status and initiatives for future progress. Endocr Relat Cancer 2004; 11: 423-36.

23. Misra MC, Bhattacharjee HK, Hemal AK, Bansal VK. Laparoscopic management of rare retroperitoneal tumors. Surg Laparosc Endosc Percutan Tech 2010; 20: 17-22. 138 Bizueto-Rosas H y cols. Paraganglioma del órgano de Zuckerkandl. Rev Mex Angiol 2014; 42(3): 132-138

24. López M, Águila P, González E. Anestesia en el feocromocitoma: presentación de un paciente. Revista Cubana de Anestesiología y Reanimación 2004; 2: 45-8.

25. Lenders JW, Eisenhofer G, Mannelli M, Pacak K. Phaeochromocytoma. Lancet 2005; 366: 665-75.

26. Cascon A, Pita G, Burnichon N, Landa I, López-Jiménez E, Montero-Conde C, et al. Genetics of pheochromocytoma and paraganglioma in Spanish patients. J Clin Endocrinol Metab 2009; 94: 1701-5.