Composite pheochromocytoma of the adrenal gland: a case series

Abstract

BackgroundComposite pheochromocytoma is a rare pathological condition characterized by elements of both pheochromocytoma and neurogenic tumors. However, detailed clinical outcomes of this tumor have not been fully shown. From 2007 to 2013, we experienced three cases of adrenal composite pheochromocytoma. In this report, we investigate the clinicopathological features of these three cases of composite pheochromocytoma and compare them with previously reported cases.Case presentationsCases 1 and 2 were a 29-year-old Japanese woman and a 59-year-old Japanese man, respectively. They underwent laparoscopic left adrenalectomy, and pathological examination revealed composite pheochromocytoma–ganglioneuroma. Case 3 was a 53-year-old Japanese man who had been receiving hemodialysis for 17 years. He underwent laparoscopic right adrenalectomy, and pathological examination revealed composite pheochromocytoma–ganglioneuroblastoma. Although the Ki67-positive rates varied from 1.0 to 6.2% among the three cases, no clinical recurrences occurred. Despite the relatively high rate of Ki67 positivity, complete tumor resection resulted in favorable clinical outcomes.ConclusionWe experienced three cases of adrenal composite pheochromocytoma. Although the clinical findings and treatment outcomes of composite pheochromocytoma were similar to those of ordinary pheochromocytoma, further studies of the biological behavior and genetic profiles of composite pheochromocytoma are necessary to achieve a better understanding of this tumor.

A rare case of Ganglioneuroblastoma Encapsulated in Pheochromocytoma

“…Composite pheochromocytoma-ganglioneuroblastoma is an extremely rare tumor (1,(6)(7)(8)(9)(10)(11)(12), given this unusual association that sparked interest in reporting the case; we confirm its importance to be source of studies and possible new treatments.…”

Rare adrenal ganglioneuroma encasing the inferior vena cava

“…A review of the literature identified fewer than 100 reported cases of adrenal ganglioneuroma in the last 20 years, many of which are found incidentally on imaging, or with non‐specific symptoms of abdominal discomfort, elevated blood pressure or gastrointestinal symptoms. In a small percentage of these cases, the lesions were found to be composite pheochromocytoma–ganglioneuromas, which occasionally present with symptoms related to hormone secretion . We have also identified a similar case of a retroperitoneal extra‐adrenal ganglioneuroma involving multiple vessels that was also excised successfully with preservation of neighbouring anatomical structures due to a plane of cleavage being easily found between the tumour pseudocapsule and adjacent structures …”

A Composite Pheochromocytoma-ganglioneuroma: A Case Report

“…To date, less than 50 cases of composite pheochromocytoma have been reported [7,8,9]. Preoperative detection of a composite pheochromocytoma ganglioneuroma is difficult because of the low incidence of ganglioneuroma and because the symptoms are not different from those of typical pheochromocytomas [10].…”