Optimal surgical treatment and urological outcomes in boys with pelvic and urogenital rhabdomyosarcomas and soft tissue sarcomas.
Optimal surgical treatment and urological outcomes in boys with pelvic and urogenital rhabdomyosarcomas and soft tissue sarcomas.
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患有盆腔和泌尿生殖系统横纹肌肉瘤和软组织肉瘤的男孩的最佳手术治疗和泌尿外科结果。
DOI：
10.1007/s00383-013-3400-y
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发表时间：
2013
影响因子：
1.8
通讯作者：
Yoshida H.Ｈ
中科院分区：
文献类型：
--
作者：
Hishiki T;Saito T;Mitsunaga T;Nakata M;Terui E;Komatsu S;Mise N;Harada K;Iwai J;Higashimoto Y;Okimoto Y;Kakuda H;Ochiai H;Hino M;Homma S;Osa Y;Yoshida H.Ｈ
研究背景男童盆腔软组织肉瘤(STS)常累及泌尿生殖器官。根治性手术的最佳广泛性长期以来一直是一个讨论的问题，因为根治性手术会导致严重的泌尿生殖系统不良反应。我们对两家区域中心医院收治的盆腔STS患者进行了回顾，评估了手术的根治性和膀胱的功能结果。回顾了1997-2012年间收治的9例患者的病历和手术报告(胚胎型横纹肌肉瘤6例，恶性Triton瘤2例，疑似横纹肌肉瘤1例)。7例起源于前列腺，2例起源于腹膜后。平均随访96个月。治疗及转归。所有病例均接受新辅助化疗。反应为PR者4例，SD者2例，PD者2例。根治性手术导致8例大部切除，1例部分切除。3例行膀胱前列腺切除术，2例行前列腺切除术，4例行保留膀胱肿瘤切除术。在最后一次随访中，三人保留了功能性膀胱。结论大多数正在进行的临床试验建议对膀胱/前列腺横纹肌肉瘤进行保守手术，并保留膀胱功能，尤其是对化疗敏感的肿瘤。然而，其他一些团体则主张根治性手术对防止局部复发的重要性。这些报告包括队列中的异质性患者组，因此很难得出结论，哪种方法真正有助于患者更好地生存。未来的研究应该客观地评估膀胱和性功能，以建立可靠的证据，说明不同手术方法的益处和不良反应。这些数据将为优化盆腔横纹肌肉瘤儿童的治疗平衡提供信息。
BackgroundSoft tissue sarcomas (STS) of pelvic origin in boys often involve the urogenital organs. The optimal extensiveness of radical surgery has long been an issue of discussion, since exenterative surgeries result in severe urogenital adverse effects. We conducted a retrospective review of patients with pelvic STS treated in two regional center hospitals and assessed the radicality of surgery and the functional outcome of the bladder.PatientsMedical records and surgical reports of nine cases (embryonal rhabdomyosarcoma 6, malignant triton tumor 2, suspected rhabdomyosarcoma 1) treated within 1997–2012 were reviewed. Site of origin was prostate in seven, retroperitoneal in two. Average follow-up period was 96 months.Treatment and outcomeAll cases were subjected to neoadjuvant chemotherapy. Response was PR in four, SD in two, and PD in two. Radical surgery resulted in gross total resection in eight, and partial resection in one. Three underwent cystoprostatectomy, two cases underwent prostatectomy, and bladder-preserving tumor resection was carried out in four cases. At the last follow-up, three retained a functional bladder. Two required augmentation cystoplasty with intestinal conduits.ConclusionsThe majority of the on-going clinical trials recommend conservative surgery for bladder/prostate rhabdomyosarcoma, and to preserve the bladder function particularly in chemosensitive tumors. Some other groups, however, advocate the importance of radical surgery to prevent local relapse. These reports include heterogenous group of patients in the cohort, and therefore it is difficult to draw a conclusion of which approach truly contributes to the survival of the patients better. Future studies should evaluate bladder and sexual function objectively to establish reliable evidence regarding the benefit and adverse effects of different surgical approaches. These data would be informative to optimize the treatment balance for children with pelvic rhabdomyosarcomas.