Multicentric pheochromocytoma and involvement of the inferior vena cava

Authors

  • Antonio Marmo Lucon Hospital das Clínicas, Faculty of Medicine, Universidade de São Paulo
  • Renato Falci Júnior Hospital das Clínicas, Faculty of Medicine, Universidade de São Paulo
  • José Nery Praxedes Hospital das Clínicas, Faculty of Medicine, Universidade de São Paulo
  • Marcel Cerqueira Cesar Machado Hospital das Clínicas, Faculty of Medicine, Universidade de São Paulo
  • Luis Balthazar Saldanha Hospital das Clínicas, Faculty of Medicine, Universidade de São Paulo
  • Marcelo Marcondes Machado Hospital das Clínicas, Faculty of Medicine, Universidade de São Paulo
  • Sami Arap Hospital das Clínicas, Faculty of Medicine, Universidade de São Paulo

Keywords:

Pheochromocytoma, Retroperitoneal neoplasia, Adrenal tumor, Adrenal surgery, Vena cava

Abstract

CONTEXT: Extension of pheochromocytomas to the inferior vena cava is rare. Multicentric tumors are rare as well, being present in up to 10% of cases. Surgery is the treatment of choice because of the longterm survival free of disease. DESIGN: Case report. CASE REPORT: We report on a case of right adrenal pheochromocytoma with extension to the supra-diaphragmatic vena cava, which underwent surgical excision through thoracophrenic laparotomy without the need for cardiopulmonary bypass. In a 6-year follow-up, another pheochromocytoma was found in the infra-renal Zuckerkandl’s organ. Complete surgical excision of the tumor was performed by a median laparotomy and complete retroperitoneal dissection. In both cases, the total removal of the pheochromocytoma has been guaranteed by having margins free of tumor and a normal post-operative level of catecholamines. The pathological study revealed a malignant pheochromocytoma with margins free of neoplasia in both specimens.

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Author Biographies

Antonio Marmo Lucon, Hospital das Clínicas, Faculty of Medicine, Universidade de São Paulo

MD, PhD. Associate Professor, Division of Urology, Department of Surgery, Faculty of Medicine, University of São Paulo, São Paulo, Brazil.

Renato Falci Júnior, Hospital das Clínicas, Faculty of Medicine, Universidade de São Paulo

MD. Resident in Urology, Division of Urology, Department of Surgery, Faculty of Medicine, University of São Paulo, São Paulo, Brazil.

José Nery Praxedes, Hospital das Clínicas, Faculty of Medicine, Universidade de São Paulo

MD, PhD. Associate Professor, Department of Nephrology, Faculty of Medicine, University of São Paulo, São Paulo, Brazil.

Marcel Cerqueira Cesar Machado, Hospital das Clínicas, Faculty of Medicine, Universidade de São Paulo

MD, PhD. Associate Professor, Division of Gastroenterological Surgery, Department of Surgery, Faculty of Medicine, University of São Paulo, São Paulo, Brazil.

Luis Balthazar Saldanha, Hospital das Clínicas, Faculty of Medicine, Universidade de São Paulo

MD, PhD. Associate Professor, Department of Pathology, Faculty of Medicine, University of São Paulo, São Paulo, Brazil.

Marcelo Marcondes Machado, Hospital das Clínicas, Faculty of Medicine, Universidade de São Paulo

MD, PhD. Full Professor and Head, Department of Nephrology, Faculty of Medicine, University of São Paulo, São Paulo, Brazil.

Sami Arap, Hospital das Clínicas, Faculty of Medicine, Universidade de São Paulo

MD, PhD. Full Professor and Head, Division of Urology, Department of Surgery, Faculty of Medicine, University of São Paulo, São Paulo, Brazil.

References

Korobkin M, Francis IR, Kloos RT, Dunnick NR. The incidental adrenal mass. Radiol Clin North Am 1996;34(5):1037-54.

Bravo EL, Gifford RW Jr. Current concepts – pheochromocy- toma: diagnosis, localization and management. N Engl J Med 1984;311(20):1298-303.

Young JD Jr, Qureshi AS, Connor TB, Wiswell JG. Problem le- sion in adrenal surgery. J Urol 1969;101:233-40.

Rote AR, Flint LD, Ellis FH Jr. Intracaval recurrence of pheochro- mocytoma extending into right atrium. Surgical management us- ing extra corporeal circulation. N Engl J Med 1977;296:1269-71.

Costello P, Clouse ME, Kane RA, Paris A. Problems in the diag- nosis of adrenal tumors. Radiology 1977;125:335-41.

Scott HW, Reynolds V, Green N, Page D, Oates JA, Robertson D. Clinical experience with malignant pheochromocytoma. Surg Gynecol Obstet 1982;154:801-18.

Russinovich NAE, Recio MG, Tishler JM, Zornes SL, Luna RF. Intracaval extension of pheochromocytoma: ultrasonographic dem- onstration. Can Assoc Radiol J 1982;33:53-5.

Hoffman JC, Weiner SN, Koenigsberg M, Morehouse H, Smith T. Pheochromocytoma invasion of the inferior vena cava: sonographic evaluation. Radiology 1983;149:793-5.

Smith EJ, McPherson GAD, Lynn J. Inferior vena cava involve- ment by a pheochromocytoma. Br J Surg 1987;74:597.

Dicke TE, Henry ML, Minton JP. Intracaval extension of pheo- chromocytoma simulating pulmonary embolism. J Surg Oncol 1987;34:160-4.

Levine E, de Vries P, Wetzel LH. MR imaging of inferior vena cava recurrence of extra-adrenal pheochromocytoma: a case re- port. J Comput Assist Tomogr 1987;11:717-8.

Novick AC, Kaye MC, Cosgrove DM, et al. Experience with car- diopulmonary bypass and deep hypothermic circulatory arrest in the management of retroperitoneal tumors with large vena cava thrombi. Ann Surg 1990;212(4):472-6.

Dumm CW, Snyder WH, Ring WS, Latson TW. Pheochromocy- toma with extension into the inferior vena cava: a case report. Surgery 1992;111(4):472-4.

Boneschi M, Miani S, Erba M, Giuffrida GF, Giordanengo F. Malignant neoplasms invading into the inferior vena cava. Minerv Cardioangiol 1995;43(3):91-5.

Raghavan R, Ince PG, Walls TJ, Gholkar A, Dark JH, Foster JB. Malignant cerebrovascular thromboembolization by pheochromo- cytoma. Clin Neuropathol 1995;14(2):69-71.

Rotker J, Oberpenning F, Scheld HH, Hertle L, Knichwitz G, Hammel D. Pheochromocytomas with extension into central vas- cular structures. Ann Thorac Surg 1996;61(1):222-4.

Lau TN, Goddard P, Vaidya M, Calloway M, Bullimore J. In- volvement of the inferior vena cava by adrenal pheochromocy- toma: MRI findings. Br J Radiol 1997;70:303-5.

Melicow MM. One hundred cases of pheochromocytoma (107 tumors) at the Columbia Presbyterian Medical Center, 1926-1976. Cancer 1997;40:1987-2004.

Arroja JM, Gudinchet F, Maeder P, Fournier D. Phéochromocytome multiple familial: démonstration échographique de multiples localisations surrénaliennes, coeliaques et vésicales chez un enfant. Schweiz Rundsch Med Prax 1995;84(43):1231-4.

Hedican SP, Marshall FF. Adrenocortical carcinoma with intracaval extension. J Urol 1997;158(6):2056-61.

Ciancio G, Hawke C, Soloway M. The use of liver transplanta- tion technique to aid in the surgical management of urological tumors. J Urol 2000;164:665-72.

Dunnick NR, Dompman JL, Geelhoed GW. Intravenous exten- sion of endocrine tumors. A J R 1980;135:471-6.

Meyers MA, King MC. Unusual radiological features of pheo- chromocytoma. Clin Radiol 1969;20:52-6.

Davis P. Malignant pheochromocytoma with functioning metastases. Lancet 1955;269:274-5.

Mahoney EM, Harrison JH. Malignant pheochromocytoma: clini- cal course and treatment. J Urol 1977;18(2):225-9.

Delarue NC, Morrow JD, Kerr JH, Colapinto RF. Pheochromo- cytoma in the modern context. Can J Surg 1978;21(5):387-94.

van Heerden JA, Roland CF, Carney JA, Sheps SG, Grant CS. Long-term evaluation following resection of apparently benign pheochromocytoma(s)/ paraganglioma(s). World J Surg 1990;14(3):325-9.

Ulchaker JC, Goldfarb DA, Bravo EL, Novick AC. Successful outcomes in pheochromocytoma surgery in the modern era. J Urol 1999;161(3):764-7.

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Published

2001-03-03

How to Cite

1.
Lucon AM, Falci Júnior R, Praxedes JN, Machado MCC, Saldanha LB, Machado MM, Arap S. Multicentric pheochromocytoma and involvement of the inferior vena cava. Sao Paulo Med J [Internet]. 2001 Mar. 3 [cited 2025 Mar. 9];119(2):86-8. Available from: https://periodicosapm.emnuvens.com.br/spmj/article/view/2755

Issue

Vol. 119 No. 2 (2001)

Section

Case Report

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