Aydin Goker Elif Tugce, Yalçın Bilgehan, Karnak İbrahim, Orhan Diclehan, Haliloglu Mithat, Ekinci Saniye, Oguz Berna, Aydin Burca, Kurucu Nilgun, Varan Ali, Kutluk Tezer
Department of Pediatrics, Hacettepe University Faculty of Medicine, Ankara, Turkey.
Department of Pediatric Oncology, Hacettepe University Faculty of Medicine, Ankara, Turkey.
J Pediatr Surg. 2024 Mar;59(3):483-487. doi: 10.1016/j.jpedsurg.2023.09.036. Epub 2023 Sep 27.
Ganglioneuromas (GNs) are rare benign peripheral neuroblastic tumors (PNTs). We shared our institutional experience with childhood GNs.
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.
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.
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.
Case series, IV.
神经节神经瘤（GNs）是罕见的良性外周神经母细胞瘤（PNTs）。我们分享了我们机构关于儿童GNs的经验。
回顾了1995年1月至2021年12月期间患有PNTs的儿童记录，并确定了组织病理学诊断为GN的病例。记录临床、实验室、放射学和组织病理学检查结果、影像界定的危险因素（IDRFs）、手术操作及总体结果。
在668例PNTs病例中，70例（10.4%）为GNs。中位年龄为7.4岁（范围2.6 - 15.7岁）（女孩/男孩，41/29）。常见的就诊主诉为腹痛和咳嗽；33/70例（47.1%）为偶然诊断。原发肿瘤位于腹部41/70例，胸部25/70例，颈部3例，骨盆1例。肿瘤中位大小为6.5cm（范围1.4 - 17cm）。50例（71.4%）分期为INRG-L1；20例有IDRFs（15例单个，5例多个>1）分期为INRG-L2。58/70例和6/70例分别进行了肿瘤完整切除和部分切除，6例未切除。总体并发症发生率为17.1%（11/64）。中位随访9年时，5例失访；65例存活。1例有大体残留的患者术后13年因病情进展接受了全切除，未切除组1例失访。其他10例未完全切除的病例无肿瘤进展。
神经节神经瘤是良性PNTs，大多数无IDRFs。即使未完全切除，长期预后也很好。鉴于GNs手术并发症发生率高且病程良性，应制定相关指南。
病例系列，IV级
