Department of Pediatric Surgery, Riga Stradins University, Lv-1007 Riga, Latvia.
Department of Pediatric Surgery, Childrens Clinical Univercity Hospital, Lv-1004 Riga, Latvia.
Medicina (Kaunas). 2020 Jul 21;56(7):362. doi: 10.3390/medicina56070362.
Rhabdomyosarcoma (RMS) is a common soft tissue sarcoma in childhood, however, it is very rare in the neonatal period (0.4-2% of cases). This case depicts a boy, who presented with RMS at two weeks of age, but officially diagnosed at the age of three months. MRI and scintigraphy determined a soft tissue tumor in the soleus muscle, while biopsy confirmed embryonal RMS with high mitotic activity (Ki67 (monoclonal antibodies) ~80%). CWS (Cooperative Weichteilsarkom Studiengruppe)-2012 with I2VA (ifosfamide, vincristine, actinomycin) chemotherapy regimen was administered per protocol. Surgical treatment was performed at age of six months and 18 days. The operation consisted of radical tumor resection and total triceps surae with partial fibula resection. Immediate reconstruction of triceps muscle was accomplished using a vascularized functional musculocutaneous vastus lateralis flap. Functional outcome was measured using the Lower Extremity Functional Scale (LEFS) and the Foot and Ankle Outcome Score (FAOS) with the results of 92.5% and 99% respectively.
横纹肌肉瘤(RMS)是儿童期常见的软组织肉瘤,但在新生儿期(0.4-2%的病例)非常罕见。本例描述了一名两周大时出现 RMS 的男孩,但正式诊断为三个月大时。MRI 和闪烁扫描确定了比目鱼肌中的软组织肿瘤,而活检证实了具有高有丝分裂活性的胚胎性 RMS(Ki67(单克隆抗体)~80%)。根据方案给予 CWS(软组织肉瘤协作研究组)-2012 与 I2VA(异环磷酰胺、长春新碱、放线菌素)化疗方案。在六个月零十八天时进行了手术治疗。手术包括根治性肿瘤切除术和三头肌全长切除以及部分腓骨切除。使用带血管的功能性股外侧肌阔筋膜瓣立即重建三头肌。使用下肢功能评分(LEFS)和足踝结局评分(FAOS)测量功能结果,分别为 92.5%和 99%。