Clinical management of infantile fibrosarcoma: a retrospective single-institution review.
Clinical management of infantile fibrosarcoma: a retrospective single-institution review.
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DOI：
10.1007/s00383-013-3326-4
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发表时间：
2013-07
影响因子：
1.8
通讯作者：
Rao BN
中科院分区：
文献类型：
--
作者：
Parida L;Fernandez-Pineda I;Uffman JK;Davidoff AM;Krasin MJ;Pappo A;Rao BN
婴儿纤维肉瘤是一种少见的软组织肉瘤。在这里我们回顾了我们治疗这种肿瘤的经验。我们回顾了1980-2009年间在圣犹大儿童研究医院接受治疗的骨盆综合征患者的记录。我们确认了15名患者，8名女孩和7名男孩；13名白人和2名黑人。确诊时的中位年龄为3个月。原发部位：小腿3例，胸壁2例，足部2例，舌头、枕区、腋窝、肩旁、手臂、前臂、腹膜后、大腿各1例。所有患者均行手术切除，11例行一期手术，4例行延期手术。并发症包括失去胫后神经和动脉、腋静脉、二头肌、胸大肌、胆囊和横窦/乙状窦。8人接受了化疗，3人接受了放射治疗。7例局部复发，3例肺转移。中位随访时间为65个月。在审查时，12名患者还活着，3名患者已经死亡。所有死亡病例均发生在确诊时年龄大于1岁的中轴线原发部位的患者。非毁损性手术应是主要的治疗方法。当一期切除不可行时，应进行新辅助化疗。手术切缘呈阳性的患者应接受辅助化疗。对于不可能完全切除的轴位原发部位，应进行放射治疗。
Infantile fibrosarcoma (IFS) is an uncommon soft-tissue sarcoma. Here we review our experience treating this tumor. We retrospectively reviewed records of patients with IFS treated at St. Jude Children’s Research Hospital between 1980 and 2009. We identified 15 patients, 8 girls and 7 boys; 13 white and 2 black. Median age at diagnosis was 3 months. Primary sites included the leg (n = 3), chest wall (n = 2), foot (n = 2), and one each in the tongue, occipital region, axilla, parascapular region, arm, forearm, retroperitoneum, and thigh. All patients underwent resection; 11 upfront surgery, and 4 delayed. Complications included loss of the posterior tibial nerve and artery, axillary vein, biceps, pectoralis major, gallbladder, and transverse/sigmoid sinus. Eight received chemotherapy and three radiotherapy. Seven experienced local recurrence and three lung metastasis. Median follow-up was 65 months. At the time of the review, 12 patients were alive and 3 had died. All deaths were in patients older than 1 year at diagnosis with an axial primary site. Non-mutilating surgery should be the primary treatment for IFS. Neoadjuvant chemotherapy is indicated when upfront resection is unfeasible. Patients with positive surgical margins should receive adjuvant chemotherapy. Radiotherapy is indicated for axial primary sites where complete resection is impossible.