Composite pheochromocytoma with spindle cell sarcoma — a rare tumor of the adrenal gland: case report

Composite pheochromocytomas with a non-pheochromocytoma component stemming from an embryological origin other than the neural crest comprise a unique entity. We present a patient diagnosed with a composite pheochromocytoma with spindle cell sarcoma, which is, to our knowledge, the first case reported in the current literature. A 45-year-old female patient with elevated blood pressure had undergone adrenalectomy for clinically and radiologically diagnosed pheochromocytoma. Macroscopic examination of the specimen showed an encapsulated mass containing two nodules. One tumor was composed of large polygonal neoplastic cells with round vesicular nuclei and granular cytoplasm forming the characteristic Zellballen pattern consistent with pheochromocytoma, while the other was composed of spindle cells with hyperchromatic nuclei and prominent nucleoli, thus resembling a sarcoma. The patient was discharged on the fourth post-operative day without any complications. Even though for both tumors surgical resection is the main treatment modality, our case report aims to shed light on and discuss the etiology and management of a rarely presented composite pheochromocytoma and spindle cell sarcoma.