Vulvar Ganglioneuroma in a Prepubertal 3 Year Old with an Extra Genital Concomitant Tumor

Author Type(s)

Faculty, Resident/Fellow

Document Type

Abstract

Publication Date

2022

DOI

10.1016/j.jpag.2022.01.121

Journal Title

Journal of Pediatric and Adolescent Gynecology

Department

Obstetrics and Gynecology

Abstract

Background: Ganglioneuromas are rare, benign forms of peripheral nerve tumors. Peripheral nerve tumors are derived from immature sympathetic neuroblasts and are typically found in the locations of migrating neural crest cells. Common locations include the posterior mediastinum, retroperitoneal spaces, adrenal gland and neck. They occur more commonly in older children, and present more often as a single foci rather than in multiple locations. We described a unique presentation of vulvar ganglioneuroma in a 3 year old female patient with a concomitant extragenital ganglioneuroma. This report focuses attention on the unusual primary location of this neural tumor in an atypical age group and therapeutic considerations. Case: The patient is a 3 year old female patient who presented in 2021 with a firm oval shaped 3.5 by 3.0 cm mobile lump in the right labia majora/mons pubis region that did not self resolve. Several weeks later, a small left flank lesion was noted by the mother that was tender to palpation. Both lesions were just under the dermis. The patient presented with no other obvious symptoms. Ultrasound of lesion suggested neurofibroma. Dermatology located two cafe au lait spots, and eye exam was negative for lisch nodules. Both masses were excised to verify pathology and avoid future complications of enlargement, pain, and a rare chance of malignant transformation. Histopathological assessment revealed that both tumors were well differentiated, benign ganglioneuromas. Currently she is complaining of a new lesion under the skin at the base of her neck. Comments: Multiple peripheral nerve tumors are more often associated with metastatic rather than benign disease. This unique location has only been documented in one other case, as a ganglioneuroma on the external genitalia. Surgical resection is effective, but can involve multiple resections, and does not prevent the possible development of more lesions. An NF1 genetic mutation has been associated with ganglioneuromas but is very rare. Our objective is to show that very young patients can have ganglioneuromas in rare locations, that multiple tumors can still represent benign pathology, and that ganglioneuroma belongs in the differential of a vulvar mass.

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