Context: Patients with pheochromocytoma (PH) or paraganglioma (PGL) may suffer from tumor persistence or recurrence after resection of the primary tumor. Malignancy and genetic determinants account for the vast majority of these cases, but tumor recurrence or persistence may also arise from tumor spillage during primary resection, followed by peritoneal implantation. We report here five such cases.
Materials and methods: Thirty-nine of the 181 patients referred to our unit for a PH experienced tumor persistence or recurrence as a result of malignant disease (n = 12), new PGL in a context of genetic determinants (n = 18) or incomplete primary surgical resection (n = 4). Another five patients presenting with adrenal PH could not be categorized into these three groups.
Results and discussion: All five patients (age range 45-63 years) presented evidence of tumor capsule rupture documented upon macroscopic examination or in the surgical report. Initial diagnostic examinations provided no evidence of malignancy. All had a period of apparent remission, lasting from 24 to 106 months. The principal site of recurrence was invariably the peritoneum or the retroperitoneum. Two patients suffered solid organ metastasis, involving the liver (n = 2), bones (n = 1), and lung (n = 1). Therapeutic management involved a combination of (131)I-metaiodobenzylguanidine therapy and surgery. Two patients died due to tumor progression. One patient experienced tumor progression despite surgery. Two patients are currently in a satisfactory condition.
Conclusion: Tumor rupture during surgical resection, with subsequent peritoneal and retroperitoneal dissemination, is a potentially lethal complication of primary pheochromocytoma resection. Even in cases of apparently benign disease, it may lead to peritoneal carcinomatosis and metastatic disease. Complete primary surgery is, therefore, crucial for a good prognosis in PH patients. Furthermore, in cases of tumor rupture, careful follow-up is mandatory, because recurrences may occur after long periods of apparent remission.