Çocukluk Çağında Ganglionöromlar

Abstract

Ganglioneuromas (GNs) are rare benign peripheral neuroblastic tumors (PNTs). The primary treatment modality is surgical resection. We aimed to review our institutional experience with childhood GNs. Hospital files of the children with PNTs diagnosed between 1995 and 2021 were reviewed and cases with the histopathological diagnosis of GN were identified. Data concerning demographic characteristics, clinical, laboratory, and radiological findings, image-defined risk factors (IDRF), INRG stages, diagnostic and surgical procedures, histopathological findings, and overall outcome were recorded. Of 668 cases with PNTs, 70 (10.4%) had GNs. The median age was 7.4 years (range, 2.6-15.7 years; 16/70 <5 years) (females/males, 41/29). Common presenting complaints were abdominal pain and cough; 27/70 cases (38.6%) were diagnosed incidentally. Primary tumor sites were the abdomen in 41/70 (58.6%) , the thorax in 25/70 (35.7%), the neck in 3 cases; the pelvis in one. Urinary HVA and VMA were elevated in 8 of 63 cases (12.7%). The median tumor size was 6.5 cm (range, 1.4-17). Fifty cases (71.4%) with no IDRF were staged as INRG-L1; 20 cases with IDRF(s) (15 single, five >1) were staged as INRG-L2. Complete and partial tumor resections were performed in 58/70 and 6/70 cases (8.6%), respectively (6 had no resection). The overall complication rate was 17.1% (11/64) (Horner syndrome in 3, renal atrophy in 2, leg hypoesthesia in 2). At a median follow-up of 9 years (range, 0-27), 5 cases were lost to follow-up; 65 were alive. One patient with a gross tumor residue underwent total resection due to tumor progression 13 years after the initial surgery. Eleven other cases with gross residual tumors experienced no tumor progression in the follow-up. GNs are benign PNTs and most are free of IDRFs. Even with incomplete or partial resection, long-term outcomes are excellent. A multidisciplinary approach is necessary, and the decision of tumor resection should be made meticulously for each case, though may not be mandatory in every instance.