Familial pheochromocytoma

Abstract 

Objectives

To identify disease characteristics, operative outcome, and prognosis in patients with familial pheochromocytoma.

Methods

Records of 29 patients with familial pheochromocytoma were retrospectively analyzed. Patients' age, gender, presenting symptoms and clinical signs, chemical and radiologic findings, and associated conditions were recorded.

Results

Of the 29 patients, 25 were adults and 4 were children. Age ranged from 18 to 52 years (mean age 30.8 years). Twenty patients were females. Of the 29 patients, 26 had adrenal tumors, 2 had extra-adrenal tumors, and 1 had a combined adrenal and extra-adrenal tumor. The patients included 17 with multiple endocrine neoplasia (MEN), 8 with von Hippel Lindeau disease (vHLD), and 4 with Von Recklinghausen disease (vRD). Two patients had malignant pheochromocytoma. All except one patient with MEN had thyroid carcinoma. The two patients with malignant pheochromocytoma had MEN II. All patients were managed by either open or laparoscopic adrenalectomy or excision of extra-adrenal tumors. There was no perioperative mortality.

Conclusions

Associated conditions in patients with familial varieties are often the dominant or initial presentation. Presence of other manifestations of familial pheochromocytoma significantly influenced the clinical course and prognosis. However, it had no bearing on operative outcome of these patients.

Keywords: Adrenal, Pheochromocytoma, Hypertension, Multiple endocrine neoplasia, Neuroectodermal syndromes