Adrenal Primary Ganglioneuroblastoma Presenting in Adulthood: A Case Report

Abstract

Introduction

Ganglioneuroblastoma is a rare neuroblastic tumor derived from embryonic neural crest cells, composed of mature gangliocytes and immature neuroblasts. Although most neuroblastic tumors present in children and commonly involve the adrenal glands, adrenal ganglioneuroblastoma is rare in adults, with only 25 previously reported cases.

Case Presentation

A 20-year-old female presented with epigastric pain, and imaging revealed a 13 cm left adrenal mass with calcification and heterogeneous enhancement. Endocrinological tests were normal, except for a mildly elevated urinary metanephrine level. Open adrenalectomy was performed, and histopathological examination confirmed a mixed-type adrenal ganglioneuroblastoma with low proliferative activity (Ki-67, < 1%). Adjuvant therapy was not administered. The patient has remained recurrence-free for 39 months post-operatively.

Conclusion

We report a rare case of primary adrenal ganglioneuroblastoma in an adult, emphasizing its diagnostic challenges, histopathological characteristics, and favorable post-operative course.