Endocrine Abstracts

Introduction: Retroperitoneal ganglioneuromas (RGN’s) are rare benign tumors originating from the retroperitoneal sympathetic ganglia. They are usually sporadic; though an association with neurofibromatosis type II and multiple endocrine neoplasia (MEN) IIB has been reported. RGN’s are usually asymptomatic. Occasionally they may show symptoms due to local pressure effect or rarely are hormonally active, releasing peptides such as vasoactive intestinal peptide, somatostatins or androgen hormone and present with adrenergic symptoms, such as diarrhea, sweating and hypertension, virilism and hypokalemia.

Case report: A 16-year-old female patient, with no significant past medical history, who presented with lower back pain. MRI revealed a well circumscribed solid mass in the left retroperitoneal site adjacent to the left adrenal gland, the psoas muscle and the head of the pancreas, measuring 6×5.4x3 cm. The tumor was resected, and the histopathological examination confirmed the diagnosis of ganglioneuroma characterized by the presence of fascicles of spindle cells of Schwannian origin (S100+ and NF+) mixed with scattered and small clusters of ganglion cells (Synaptophysin+, S-100+, NF+). Desmin, smooth-muscle actin and MelanA were negative. Ki-67 labeling index was low (~1-2%).

Conclusion: Due to lack of specific characteristics RGNs are quite difficult to differentiate from other tumors of the area. The prognosis for a RGN following complete surgical resection is good. Their close relationship with surrounding vital organs makes surgical excision of these tumors challenging and may act as a limiting factor in complete surgical resection. Also, since they may release catecholaminergic peptides, surgeons should be aware of the possibility of hypertensive crisis during the surgery. Regular and periodic long-term follow-up is recommended.