Ganglioneuromas in Childhood: Hacettepe Experience With 70 Cases


Aydin Goker E. T., YALÇIN B., KARNAK İ., ORHAN D., HALİLOĞLU M., EKİNCİ S., ...Daha Fazla

Journal of Pediatric Surgery, cilt.59, sa.3, ss.483-487, 2024 (SCI-Expanded) identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 59 Sayı: 3
  • Basım Tarihi: 2024
  • Doi Numarası: 10.1016/j.jpedsurg.2023.09.036
  • Dergi Adı: Journal of Pediatric Surgery
  • Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus, CAB Abstracts, Veterinary Science Database
  • Sayfa Sayıları: ss.483-487
  • Anahtar Kelimeler: Ganglioneuroma, Image-defined risk factors, Neuroblastoma, Surgical complication
  • Hacettepe Üniversitesi Adresli: Evet

Özet

Background: Ganglioneuromas (GNs) are rare benign peripheral neuroblastic tumors (PNTs). We shared our institutional experience with childhood GNs. Methods: Records of the children with PNTs between January 1995 and December 2021 were reviewed, and cases with histopathological diagnoses of GN were identified. Clinical, laboratory, radiological, and histopathological findings, image-defined risk factors (IDRFs), procedures, and overall outcomes were recorded. Results: Of 668 cases with PNTs, 70 (10.4%) had GNs. The median age was 7.4 years (range, 2.6–15.7 years) (girls/boys, 41/29). Common presenting complaints were abdominal pain and cough; 33/70 cases (47.1%) were diagnosed incidentally. Primary tumors were in the abdomen in 41/70, the thorax in 25/70, the neck in 3 cases, and the pelvis in one. The median tumor size was 6.5 cm (range, 1.4–17). Fifty cases (71.4%) were staged as INRG-L1; 20 cases with IDRFs (15 single, five >1) were staged as INRG-L2. Complete and partial tumor resections were performed in 58/70 and 6/70 cases, while 6 had no resection. The overall complication rate was 17.1% (11/64). At a median follow-up of 9 years, five were lost to follow-up; 65 were alive. One patient with gross residue underwent total resection due to progression 13 years after the surgery, and one in the unresected group was lost to follow-up. Ten other cases without a complete resection experienced no tumor progression. Conclusions: Ganglioneuromas are benign PNTs, and most are free of IDRFs. Even without complete resection, long-term outcomes are excellent. Guidelines should be devised considering the high surgical complication rates and benign course of GNs. Level of Evidence: Case series, IV.