Laparoscopically Resected Composite Pheochromocytoma-Ganglioneuroma

Abstract

Composite pheochromocytoma-ganglioneuroma is one of the mixed neuroendocrine-neural tumors composed of pheochromocytoma and other neural crest derivatives. To date, less than 50 cases of composite pheochromocytoma have been reported, and about 70% of the accompanying tumors were ganglioneuromas. Here, we describe six cases of composite pheochromocytoma-ganglioneuromas in five men and one woman, aged 33 to 64. The size of the tumors ranged from 3.0 to 11.0 cm, and four out of the six presented with intermittent onset of hypertension, palpitation, or dizziness. Microscopically, each tumor was composed of large pleomorphic shaped chromaffin cells arranged in the Zellballen patterns characteristic of pheochromocytoma, and they were mixed with clusters of mature ganglion cells and bundles of spindle-shaped Schwann cells characteristic of ganglioneuroma of variable proportions. All were successfully treated laparoscopically, and none were associated with multiple endocrine neoplasm syndrome or neurofibromatosis. Preoperative diagnosis of a composite pheochromocytoma-ganglioneuroma is impossible because of the low incidence rate, and the radiological findings and symptoms are similar to those of typical pheochromocytomas. Although the significance of microscopic detection of the nonpheochromocytoma component from pheochromocytoma has not yet been clarified, microscopic identification of the composite pheochromocytoma-ganglioneuroma is important because cumulative cases are used in an effort to predict the behavior of this composite tumor. (Endocrinol Metab 26:340-344, 2011)

Composite Adrenal Pheochromocytoma-Ganglioneuroma in an Adult Patient

“…The clinical signs and symptoms of tumor are usually similar to those of pure PHEOs (14,18). Clinically, active PHEO may produce the classic symptoms of headache, palpitations, and excessive sweating in 50% of the cases.…”

“…Rarely, watery diarrhea, hypokalemia and achlorhydria (WDHA) syndrome due to excessive VIP secretion can be observed in patients with composite PHEO-GN. These cases are frequently normotensive because of vasodilatory action of VIP and lack typical symptoms such as headaches (14,16,28,29). Composite adrenal PHEO-GN may be incidentally discovered, manifested as catecholamine-induced acute relapsing pancreatitis without hypertension (19).…”

“…On imaging procedures such as CT, MR or 18 F-FDG-PET, composite adrenal PHEO-GNs commonly appear as a well-defined, smooth or lobulated heterogeneous mass with varying admixtures of PHEO and GN components (2,12,14,21). However, approximately one third of all cases show a nonspecific appearance that may overlap with the appearance of adrenocortical carcinoma (14,33).…”

“…Composite adrenal PHEO-GN may be incidentally discovered, manifested as catecholamine-induced acute relapsing pancreatitis without hypertension (19). These composite adrenal tumors may be associated with sporodic tumors or as part of familial tumors such as neurofibromatosis type 1 (NF-1), von Hippel-Lindau disease, MEN 2A and 2B syndromes (14,17,18,20,21,(30)(31)(32). In our case, no features of either NF-1 or MEN or adrenocortical tumor were observed.…”

“…Composite adrenal PHEO-GN is extremely rare. To date, only 46 cases have been reported in the English literature (4,(12)(13)(14)(15)(16)(17)(18)(19)(20)(21)(22)(23)(24)(25). It is difficult to diagnose these tumors precisely as composite adrenal PHEO-GN before surgery.…”