Ganglioneuroma presenting with differentiated skeletal metastases. Report of a case

✍ Scribed by James H. Garvin Jr; Ernest E. Lack; William Berenberg; Christopher N. Frantz

• Publisher: John Wiley and Sons
• Year: 1984
• Tongue: English
• Weight: 394 KB
• Volume: 54
• Category: Article
• ISSN: 0008-543X
• DOI: 10.1002/1097-0142(19840715)54:2<357::aid-cncr2820540230>3.0.co;2-4

✦ Synopsis

The authors report the case of a child with retroperitoneal ganglioneuroma and cytodifferentiated skeletal metastases. The primary tumor was surgically resected, and the child is alive and well 2 years later without additional therapy. This rarely documented phenomenon can be explained by spontaneous cytomaturation within both primary and metastatic tumor.

Cancer 54357-360. 1984.

EUR~BLASTOMA is the most common extracranial N malignant solid tumor of childhood, appearing most often during the first 3 years of life. Spontaneous regression of neuroblastoma in young infants is well documented.' In a small number of patients, neuroblastoma differentiates into ganglioneuroma, a tumor with benign histologic appearance and good clinical prognosis. Spontaneous differentiation of metastatic neuroblastoma is extremely rare.* We report a child with intraabdominal ganglioneuroma in whom deposits of differentiated ganglioneuroma were found in bone. The patient was successfully treated by surgery alone. We are unaware of any documented cases of neuroblastoma presenting with fully differentiated skeletal metastases. The possibility of differentiated metastases should be considered in patients with ganglioneuroma and ganglioneuroblastoma even in the absence of symptoms referable to metastatic tumor.

Case Report

A girl aged 4 years 4 months presented with a 4-month history of increasingly severe diarrhea, crampy abdominal pain, and moderate hair loss. On physical examination she was a healthyappearing, normotensive child with a vague fullness in her right