Komasara Leszek, Stefanowicz Joanna, Bryks-Laszkowska Anna, Gołębiewski Andrzej, Czauderna Piotr
Department of Surgery and Urology for Children and Adolescents, Medical University of Gdansk, Gdansk, Poland.
Department of Pediatrics, Hematology and Oncology, Medical University of Gdansk, Gdansk, Poland.
Int J Urol. 2016 Aug;23(8):679-85. doi: 10.1111/iju.13120. Epub 2016 May 15.
To present versatile surgical reconstructive techniques and their outcomes in pediatric patients with genitourinary rhabdomyosarcoma.
We retrospectively analyzed the oncological and urological outcomes of seven patients treated between 1992 and 2014 according to the Cooperative Weichteilsarkom Studiengruppe protocols. Intergroup Rhabdomyosarcoma Study staging: local, six patients; and IV, one patient.
embryonal, five patients; unclassified, one patient; triton tumor one patient. Surgical treatment included: cystectomy, uterectomy and partial vaginectomy, one patient; radical cystectomy, two patients; cystectomy, one patient; cystectomy with partial prostatectomy, one patient; partial cystectomy, one patient; and partial prostatectomy, one patient.
All patients were alive in complete remission at last follow up. In four cases, ileal conduit with ureteral reimplantation with serous-lined extramural tunnel (Abol-Enein technique) was carried out, which was followed by conversion into ileal continent bladder with continent appendiceal stoma for clean intermittent catheterization in three patients. In one boy, partial cystectomy and continent reconstruction was carried out during a single surgical procedure. One child with incontinent urinary diversion is still awaiting a continence solution. One child after partial prostatectomy is continent without any voiding disturbances.
The timing and extent of radical surgery for treatment of genitourinary rhabdomyosarcoma depend on the local anatomical conditions, and the response to previous chemo- and radiotherapy. Cystectomy followed by various reconstructive techniques still remains an important option in the local treatment.
介绍针对小儿泌尿生殖系统横纹肌肉瘤患者的多种手术重建技术及其效果。
我们根据合作横纹肌肉瘤研究组方案，回顾性分析了1992年至2014年间接受治疗的7例患者的肿瘤学和泌尿学结果。横纹肌肉瘤协作组分期：局部病变6例，IV期1例。
胚胎型5例，未分类1例，蝾螈瘤1例。手术治疗包括：膀胱切除术、子宫切除术和部分阴道切除术1例；根治性膀胱切除术2例；膀胱切除术1例；膀胱切除术加部分前列腺切除术1例；部分膀胱切除术1例；部分前列腺切除术1例。
所有患者在最后一次随访时均存活且处于完全缓解状态。4例患者采用了带输尿管再植的回肠导管术并构建浆膜内衬的壁外隧道（阿博尔-埃奈因技术），其中3例随后转变为带可控阑尾造口的回肠可控膀胱，以便进行清洁间歇性导尿。1名男孩在一次手术中进行了部分膀胱切除术和可控性重建。1名存在尿失禁的尿流改道患儿仍在等待解决控尿问题的方案。1名接受部分前列腺切除术后的患儿控尿良好，无任何排尿障碍。
根治性手术治疗泌尿生殖系统横纹肌肉瘤的时机和范围取决于局部解剖条件以及对先前化疗和放疗的反应。膀胱切除术后采用各种重建技术仍是局部治疗的重要选择。
