Atalay İsmail Burak, Togral Guray
Department of Orthopaedics and Traumatology, DR A.Y Ankara Oncology Training and Research Hospital, Ankara, Turkey.
Department of Orthopaedics and Traumatology, DR A.Y Ankara Oncology Training and Research Hospital, Ankara, Turkey.
Acta Orthop Traumatol Turc. 2019 Nov;53(6):507-511. doi: 10.1016/j.aott.2019.08.008. Epub 2019 Sep 11.
Infantile fibrosarcoma is a very rare soft tissue tumor in infants and children most commonly located in extremities. It constitutes less then 1 percent of all childhood cancers. Prognosis and clinical course of it is relatively good compared to adult forms. Local recurrence is common but metastasis is infrequent. In this case report we present infantile fibrosarcoma with relapse and lung metastasis despite neoadjuvant chemotherapy, pelvic reconstruction surgery with wide surgical excision and adjuvant chemotherapy protocol. The patient was a 2-year-old girl at the time of diagnosis, and there was a huge mass in pelvic region. After neoadjuvant chemotherapy, type 1 pelvic resection and pelvic reconstruction with bone cement performed. The patient presented with relapse and lung metastasis 6 months after the surgery. This is the first report of pelvic infantile fibrosarcoma with pelvic resection surgery. This case suggests that these tumors may exhibit unpredictable clinical behavior.
婴儿纤维肉瘤是一种在婴幼儿中非常罕见的软组织肿瘤，最常见于四肢。它占所有儿童癌症的比例不到1%。与成人型相比，其预后和临床病程相对较好。局部复发很常见，但转移很少见。在本病例报告中，我们介绍了一名尽管接受了新辅助化疗、广泛手术切除的盆腔重建手术及辅助化疗方案，但仍出现复发和肺转移的婴儿纤维肉瘤患者。诊断时患者为一名2岁女孩，盆腔有一个巨大肿块。新辅助化疗后，进行了1型盆腔切除术并用骨水泥进行盆腔重建。术后6个月患者出现复发和肺转移。这是首例关于盆腔婴儿纤维肉瘤行盆腔切除手术的报告。该病例表明这些肿瘤可能表现出不可预测的临床行为。
