Endocrine Abstracts

Introduction: Composite pheochromocytoma is a rare tumor composed histologically of pheochromocytoma and other neurogenic tumor components such as neuroblastoma, ganglioneuroblastoma, ganglioneuroma, peripheral nerve sheath tumor, or other types of neuroendocrine carcinoma. The frequency of composite adrenal tumors reportedly ranges from <3% of all adrenal gland neoplasms to 1–9% of pheochromocytomas. The clinicopathological diagnosis of composite pheochromocytoma is, at times, a clinical dilemma because it is not known whether the nonpheochromocytoma component has any therapeutic and/or prognostic implications as compared to the standard pheochromocytoma.

Case report: A 82-year-old male presented with gastrointestinal complaints (abdominal pain and diarrhea) for 2 months along with facial flushing, sweating and 10 kg weight loss. He had a history of mild hypertension with orthostatic hypotension. Hemogram reports revealed anemia. Abdominal ultrasonography and computed tomography showed the presence of a large heterogeneous mass 118 × 135 × 108 mm with well-defined boundaries in the left suprarenal region. In view of adrenal tumors, 24-hr urinary metanephrine was further evaluated, with high levels 1674,17 mg/24-hrs (reference range, 25–312), plasma free metanephrines levels: 707,5 ng/l (reference range, 7,9-88,7), cortisol level was 1,76 ug/dl after overnight dexamethasone suppression test showed normal suppression, plasma level of chromogranin A was 746,3 ng/ml (reference range <76,3). Left adrenalectomy was performed. The anesthetic team confronted with intraoperative complications: arrhythmia. Postoperative period was uneventful and plasma levels of free metanephrines and normetanephrines normalized: 74,8pg/ml (reference range, <65), respectively148,1 pg/ml (reference range, <196). Pathological diagnosis was composite pheochromocytoma / ganglioneuroma.

Conclusions: Composite pheochromocytomas are rare catecholamine-producing tumor which has the propensity to large size which is unlikely when it is classical pheochromocytoma. Clinical manifestations unique to the tumor are occasional and atypical and non-specific symptomatology and its association with autoimmune disorders. A multidisciplinary approach involving anesthesia, endocrinology, and surgical expertise is the gold standard in maximizing patient care.