Koivusalo Antti I, Rintala Risto J, Pakarinen Mikko P
Children's Hospital, Section of Pediatric Surgery, University of Helsinki, Finland.
Children's Hospital, Section of Pediatric Surgery, University of Helsinki, Finland.
J Pediatr Surg. 2019 Feb;54(2):285-287. doi: 10.1016/j.jpedsurg.2018.10.090. Epub 2018 Nov 7.
We describe a series of three successive patients with perianal rhabdomyosarcoma (PRMS) from 2014 to 2017 managed with combined chemotherapy, radical surgery, and radiotherapy.
Ethical consent was obtained. Data including tumor presentation, treatment, and survival was collected from hospital reports.
Two girls aged 15 and 16 years (patient #1 and #2) and one boy aged five years (patient #3) were referred because of a suspected perianal abscess. MRI showed large perianal tumors from 7 to 12 cm in diameter that surrounded or infiltrated the anal sphincters and were inconsistent with abscess. Tumor biopsies showed RMS of alveolar (#1 and #2) and embryonal (#3) types. Patient #1 had lymph node and bone metastases, patient #2 lymph node metastases, and patient #3 no metastases. Pretreatment staging, IRS Clinical Group, and Risk Groups were: Stage 4, II, high; Stage 3, GII, intermediate; and Stage 3, I, low, respectively. All underwent colostomy before neoadjuvant chemotherapy (CWS-RMS 2009 program). Neoadjuvant chemotherapy failed to clear the tumors from anal sphincters preventing anus-saving surgery, and all patients underwent abdominoperineal excision. All removed specimens had free margins with negative lymph nodes. After adjuvant chemotherapy and local radiation, the patients were tumor free after 48, 13, and 18 months.
In PRMS local surgical control required abdominoperineal excision. Confusion between PRMS and abscess may cause unnecessary delay in management.
IV (Treatment Study, Case Series with no Comparison Group).
我们描述了2014年至2017年间连续收治的3例肛周横纹肌肉瘤（PRMS）患者，采用联合化疗、根治性手术和放疗进行治疗。
获得伦理批准。从医院报告中收集包括肿瘤表现、治疗和生存情况的数据。
两名分别为15岁和16岁的女孩（患者1和患者2）以及一名5岁男孩（患者3）因疑似肛周脓肿前来就诊。磁共振成像显示直径7至12厘米的巨大肛周肿瘤，这些肿瘤包绕或浸润肛门括约肌，与脓肿表现不符。肿瘤活检显示为肺泡型（患者1和患者2）和胚胎型（患者3）横纹肌肉瘤。患者1有淋巴结和骨转移，患者2有淋巴结转移，患者3无转移。治疗前分期、国际横纹肌肉瘤研究组（IRS）临床分组和风险分组分别为：4期，II组，高危；3期，GII组，中危；3期，I组，低危。所有患者在新辅助化疗（CWS-RMS 2009方案）前均接受了结肠造口术。新辅助化疗未能清除肛门括约肌的肿瘤，无法进行保肛手术，所有患者均接受了腹会阴联合切除术。所有切除标本切缘阴性，淋巴结无转移。辅助化疗和局部放疗后，患者分别在48、13和18个月后无瘤生存。
PRMS的局部手术控制需要腹会阴联合切除术。PRMS与脓肿的混淆可能导致治疗的不必要延迟。
IV（治疗研究，无对照组的病例系列）
