Malignant Peripheral Nerve Sheath Tumour of the Adrenal Gland: An Unusual Pathology in an Adrenal Incidentaloma

Malignant peripheral nerve sheath tumours (MPNST) are aggressive soft tissue sarcomas. The occurrence of MPNST of the adrenal gland is exceedingly rare with only two other de-novo tumours reported in literature. It has been reported to be associated in some patients with neurofibromatosis type-1 and composite tumours like pheochromocytomas and ganglioneuromas. There are no reports of an MPNST presenting as an adrenal incidentaloma. It is impossible to achieve a pre-operative diagnosis. Diagnosis depends solely on histopathological identification. Loss of H3K27me3 trimethylation can be helpful in distinguishing these tumours from possible mimics and is associated with higher tumour grade and more aggressive clinical behaviour.