Composite tumor with pheochromocytoma and immature neuroblastoma: report of two cases with cytogenetic analysis and discussion of current terminology

Pheochromocytoma, paraganglioma, and neuroblastoma are all derived from sympathetic nervous tissues and can exhibit a spectrum of morphology. The literature includes reports of tumors with morphologic features of both pheochromocytoma and neuroblastoma within the same tumor. The term “composite pheochromocytoma” has been used to describe these tumors as well as pheochromocytomas with other neural crest derivatives, such as malignant peripheral nerve sheath tumor and neuroendocrine carcinoma. “Composite pheochromocytoma” is rare, and there is a paucity of information in the literature regarding its biological behavior, clinical outcome, and molecular profile. The most commonly reported cases include components of pheochromocytoma and ganglioneuroma [ 1 ]. However, there are only few reports of tumors with components of pheochromocytoma and stroma-poor neuroblastoma (Table 1 ) [ 2 , 3 , 4 , 5 , 6 , 7 ]. We report two distinct cases of composite tumors of the adrenal gland consisting of pheochromocytoma and neuroblastoma.