Lucon A M, Falci R, Praxedes J N, Machado M C, Saldanha L B, Machado M M, Arap S
Division of Urology, Department of Surgery, Faculty of Medicine, University of São Paulo, Brazil.
Sao Paulo Med J. 2001 Mar;119(2):86-8. doi: 10.1590/s1516-31802001000200010.
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 long-term survival free of disease.
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.
嗜铬细胞瘤延伸至下腔静脉的情况罕见。多中心肿瘤也很罕见，在高达10%的病例中出现。由于长期无病生存，手术是首选治疗方法。
病例报告。
我们报告一例右侧肾上腺嗜铬细胞瘤延伸至膈上腔静脉的病例，该病例通过胸腹联合剖腹术进行手术切除，无需体外循环。在6年的随访中，在肾下的祖克坎德尔器官中发现了另一例嗜铬细胞瘤。通过正中剖腹术和完全腹膜后解剖对肿瘤进行了完整的手术切除。在这两个病例中，嗜铬细胞瘤的完全切除通过切缘无肿瘤以及术后儿茶酚胺水平正常得以保证。病理研究显示两个标本均为恶性嗜铬细胞瘤，切缘无肿瘤形成。
