Composite pheochromocytoma-ganglioneuroma of the adrenal gland: A case report with immunohistochemical study

Abstract

Composite tumors of the adrenal medulla consisting of pheochromocytoma and ganglioneuroma are rare tumors accounting for less than 3% of all sympathoadrenal tumors. These tumors display more than one line of differentiation in which normal and neoplastic chromaffin cells are capable of differentiating into ganglion cells under the influence of nerve growth factors. To the best of our knowledge, we report the second case with a composite tumor of the adrenal medulla in a normotensive patient from India.

Pheochromocytoma and paraganglioma in patients with neurofibromatosis type 1

“…These tumours are quite rare and show features of PHEO/PGL combined with neurogenic features, such as Schwann cells embedded in ganglion cells as seen in ganglioneuromas. These tumours typically occur in the adrenal gland (50‐70 reported cases) but can rarely be found in the retroperitoneum (5 case reports), as in our case . There is a known association between composite PHEO–ganglioneuroma tumours and NF1, but no case reports have shown an extra‐adrenal composite tumour in the setting of NF1 …”

Laparoscopic resection of primary adrenal ganglioneuroma: A case report and review of the literature

“…A diagnosis of GN depends upon whether or not the tumor is composed of ganglion cells (19). Immunohistochemical results, including S100, CD56, synaptophysin and NSE positivity, are also important for forming a diagnosis (20).…”

“…A pathological investigation is often a conclusive diagnostic determinant used to differentiate between these tumors. The gross and histopathological morphological appearance and immunohistochemical features differ significantly due to the different origins of the tumors (20).…”

Hypertensive retinopathy as the initial presentation of neuroblastoma

“…One other possible explanation for this child's presentation of neuroblastoma with hypertensive retinopathy is the possibility that this child harbored a mixed tumor containing neuroblastoma and pheochromocytoma cells, a very rare occurrence. 13 This tumor was extremely large and despite multiple biopsies, there is the small possibility that a focus of pheochromocytoma tumor cells may have been missed.…”