Shankar Gowri, Konamme Vinay Kumar
Department of Pediatric Surgery, Indira Gandhi Institute of Child Health, Bengaluru, Karnataka, India.
J Indian Assoc Pediatr Surg. 2022 Nov-Dec;27(6):771-774. doi: 10.4103/jiaps.jiaps_46_22. Epub 2022 Nov 14.
Nonrhabdomyosarcoma soft-tissue sarcomas (NRSTSs) are a heterogeneous group of neoplasms of presumed mesenchymal origin. The precise diagnosis of the type of NRSTS tumor is complex. Among them, Ewing's sarcoma is very rare. We report a case of abdominal wall Ewing's sarcoma in an 8-year-old child. There are very few cases on pediatric abdominal wall Ewing's sarcoma reported. An 8-year-old female child presented with a progressively increasing swelling in the left iliac fossa for 3 months. On evaluation it was found to have a 4 cm × 2.87 cm × 4.1 cm circumscribed, heterogeneous exophytic mass arising from oblique muscles in the left lower abdominal wall with no intraperitoneal extension. On trucut biopsy and karyotyping, it was diagnosed to be an extraskeletal Ewing's sarcoma (EES) of the abdominal wall. Due to the proximity to the pelvic bone and the possible large size, the child was given six cycles of VAEC-IE as neoadjuvant chemotherapy to downsize the tumor. Following chemotherapy, wide excision of the tumor was done requiring partial resection of the external oblique, internal oblique, transverse abdominis, and leaving the transversalis fascia intact. The defect was closed using a polypropylene mesh. Histopathology showed tumor-free margins, with minimal histological tumor response to chemotherapy. Hence, the child underwent radiotherapy (45 Gy in 25 fractions). Follow-up fluorodeoxyglucose-positron emission tomography-computed tomography showed no recurrent lesion. There was no recurrence on 18-month follow-up. Extraskeletal Ewing's sarcoma is a rare tumor arising from the abdominal wall. The evaluation and management are similar to EES. Treatment is multimodal; however, surgery is the mainstay. Wide excision with tumor negative margin has a good outcome.
非横纹肌肉瘤软组织肉瘤（NRSTSs）是一组起源于间充质的异质性肿瘤。准确诊断NRSTS肿瘤类型很复杂。其中，尤因肉瘤非常罕见。我们报告一例8岁儿童腹壁尤因肉瘤病例。小儿腹壁尤因肉瘤的报道病例极少。一名8岁女童左髂窝渐进性肿胀3个月。经评估，发现左下腹壁斜肌有一个4 cm×2.87 cm×4.1 cm的边界清楚、异质性外生性肿块，无腹腔内延伸。经粗针活检和核型分析，诊断为腹壁骨外尤因肉瘤（EES）。由于靠近骨盆且肿瘤可能较大，该患儿接受了6个周期的VAEC-IE新辅助化疗以缩小肿瘤大小。化疗后，对肿瘤进行了广泛切除，需要部分切除腹外斜肌、腹内斜肌、腹横肌，保留腹横筋膜完整。缺损用聚丙烯网片修补。组织病理学显示切缘无肿瘤，化疗后的组织学肿瘤反应最小。因此，该患儿接受了放疗（25次分割，共45 Gy）。随访氟脱氧葡萄糖正电子发射断层扫描计算机断层扫描显示无复发病变。18个月随访无复发。骨外尤因肉瘤是一种起源于腹壁的罕见肿瘤。评估和治疗与EES相似。治疗是多模式的；然而，手术是主要治疗方法。切缘阴性的广泛切除预后良好。
