Department of Paediatric Haematology and Oncology, Bhaktapur Cancer Hospital, Dudhpati, Bhaktapur, Nepal.
JNMA J Nepal Med Assoc. 2022 Jul 1;60(251):638-640. doi: 10.31729/jnma.7523.
Ewing sarcoma is the second most common malignant bone tumour in children. It rarely originates from extraskeletal soft tissue sites such as the upper thigh, buttocks, upper arm and shoulder. Primary extraosseous Ewing sarcoma located in the pelvic region is rare. We report a 17-year-female who had gradual onset of progressive lower abdominal mass and pain. A computed tomography scan revealed well defined lobulated heterogeneously enhancing lesion noted in the pelvic region measuring approximately 12.9 x 9.8 x 9.3 cm. Incisional biopsy showed a small round blue cell tumour which was strongly positive for Cluster of Differentiation 99, vimentin, Friend Leukaemia Integration 1 with 40% Ki-67. Following treatment with chemotherapy, surgery and radiotherapy, there was complete resolution of the tumour. Although extraosseous Ewing sarcoma is rare, it can occur virtually in any soft tissue site. Therefore, clinicians need to distinguish it from soft tissue sarcoma because rapid progression, early diagnosis and timely treatment are crucial for a favourable prognosis.
Ewing sarcoma; neoadjuvant chemotherapy; radiation therapy.
尤文肉瘤是儿童中第二常见的恶性骨肿瘤。它很少起源于骨骼外的软组织部位，如大腿上部、臀部、上臂和肩部。原发于骨盆的骨外尤文肉瘤非常罕见。我们报告了一名 17 岁女性，她逐渐出现进行性下腹肿块和疼痛。计算机断层扫描显示在盆腔区域有一个界限清楚的分叶状不均匀增强病变，大小约为 12.9 x 9.8 x 9.3 cm。切开活检显示一个小圆蓝细胞肿瘤，CD99、波形蛋白、Friend 白血病整合 1 阳性，Ki-67 为 40%。在接受化疗、手术和放疗后，肿瘤完全消退。尽管骨外尤文肉瘤很少见，但它几乎可以发生在任何软组织部位。因此，临床医生需要将其与软组织肉瘤区分开来，因为快速进展、早期诊断和及时治疗对于良好的预后至关重要。
尤文肉瘤；新辅助化疗；放射治疗。
