Composite phaeochromocytoma with malignant peripheral nerve sheath tumour and rhabdomyosarcomatous differentiation in a patient without von Recklinghausen disease

Abstract

The coexistence of adrenal phaeochromocytoma with non-chromaffin tumours is a rare fascinating occurrence. This category of tumours is subdivided into "composite" and "mixed". The coexistence of adrenal phaeochromocytoma with a malignant Triton tumour does not appear to have been described in the available literature so far. A unique case of composite phaeochromocytoma in a 26-year-old male patient, where the non-chromaffin component was a malignant Triton tumour composed of peripheral nerve sheath tumour and skeletal muscle differentiation, is reported. This admixture was confirmed with immunohistochemical pattern of expression. This is the first case of such a phenomenon in a composite phaeochromocytoma. The present case further widens the histomorphological range of composite phaeochromocytoma of the adrenal gland, which the histopathologist should be aware of. Since the prognosis of composite phaeochromocytoma with malignant nerve sheath tumour would be determined by the nerve sheath component, recognition of this tumour is imperative.

Neurofibromatosis 1 with pheochromocytoma

“…Literature search revealed a single case report from India about the occurrence of pheochromocytoma in a patient with malignant peripheral nerve sheath tumor without NF1. [5] Pheochromocytoma with neurofibromatosis has not been reported earlier from India and hence we report the same in this article.…”

Adrenocortical neoplasia: evolving concepts in tumorigenesis with an emphasis on adrenal cortical carcinoma variants

“…Although melanocytic differentiation, suggestive of a divergent evolutionary pathway, has been reported in carcinosarcomas of other anatomic locations [86–89], pathologists should always keep in mind to first exclude the possibilities of a metastatic melanoma, a collision tumor consisting of an ACC and a primary or metastatic melanoma (not yet reported) and a primary perivascular epithelioid cell tumor [90]. Other differential diagnostic considerations include composite pheochromocytoma with a malignant peripheral nerve sheath tumor component (the latter with or without divergent differentiation into mesenchymal elements such as rhabdomyo-, osteo-, chondro-, angio- and/or fibro-sarcomatous components) [91, 92], metastatic carcinoma with sarcomatous or sarcoma-like areas and metastatic neoplasm with sarcomatoid elements other than carcinomas (melanoma or germ cell tumor), as well as primary retroperitoneal sarcomas [69]. Given that adrenal biphasic tumors, in contrast to those arising in epithelial organs, are unlikely to express cytokeratins while focal smooth muscle differentiation is a common finding, thorough sampling of the resected tumor is of critical importance in order to reveal the co-existent carcinomatous component and subsequently prove the adrenal origin on immunohistochemical grounds [67, 69].…”

Laparoscopically Resected Composite Pheochromocytoma-Ganglioneuroma

“…To date, less than 50 cases of composite pheochromocytoma have been reported [1][2][3][4][5][6], and two Korean cases of composite pheochromocytomas have been reported in the English and Korean literature [5,6]. Here, we report additional six cases of unusual tumor of the adrenal gland and the tumor exhibited a mixed phenotype composed of cells of adrenal medullary and neural lineages.…”

“…Composite pheochromocytoma is a well-defined neoplasm of the medulla and the tumor consists of both endocrine and neural components. Histologically, the endocrine portion is that of a pheochromocytoma, whereas the neural portion has been reported as ganglioneuroma, ganglioneuroblastoma, neuroblastoma, neuroendocrine carcinoma or malignant peripheral nerve sheath tumor [4]. Pheochromocytoma is usually characterized by a catecholaminergic effect with hypertension, whereas ganglioneuroma is a rare mature neuroblastic tumor that is typically nonmetabolically active, and so it is usually asymptomatic [5,6].…”