Presacral ganglioneuroma in an adult. Report of a clinical case

Abstract

Abstract: 

Ganglioneuromas are rare tumors of the sympathetic nervous system, developed from cells of the neural crest. They are the most differentiated within the group of peripheral neuroblastic tumors. They occur more frequently in children, and their most common locations are the posterior mediastinum, retroperitoneum, and adrenal gland. Pelvic ganglioneuromas are very rare, only a few cases have been reported in the world (less than 30). Our objective is to present the case of an adult man with a presacral ganglioneuroma, as this location is extremely infrequent.

We report the case of a 41-year-old patient who consulted at Elpidio Torres Hospital for weight loss of unknown cause over the prior two years, without other accompanying symptoms. Multiple studies were performed, showing a soft tissue mass in front of the sacrum on the abdominal CT scan. These images were complemented with an MRI, which reported a space-occupying lesion adjacent to the sciatic notch, with multilobed edges, heterogeneous and with intense enhancement.

A multidisciplinary surgery was performed, including the General Surgery, Coloproctology and Neurosurgery services. They requested an intraoperative pathological study of the tumor, which was reported as a spindle cell lesion of probable neural lineage. Then, they proceeded to excise approximately 70% of the tumor, with referral of the surgical piece to Pathology for its study.

Macroscopically, the tissue fragments measured overall 7x6x0.6 cm, were whitish and elastic in consistency. Microscopically, the neoplasm consisted of a proliferation of fused cells, with elongated nuclei and ill-defined eosinophilic cytoplasm, arranged in interlocking bundles and fascicles. In addition, the presence of mature ganglion cells was highlighted. Immunohistochemical techniques showed diffuse positivity for S100.

The patient evolved favorably after surgery, without recurrence of the lesion so far.

We conclude that ganglioneuromas should be considered as a differential diagnosis in patients presenting a pelvic mass. Its behavior is benign, being surgical removal the solution in most cases.