Successful penile reconstruction after multimodal therapy in patients with primitive neuroectodermal tumor originating from the penis

Successful penile reconstruction after multimodal therapy in patients with primitive neuroectodermal tumor originating from the penis
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DOI:
10.1111/iju.12363
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发表时间:
2014-06-01
影响因子:
2.6
通讯作者:
Nonomura, Katsuya
Nonomura, Katsuya
中科院分区:
医学3区
文献类型:
--
作者:
Akino, Tomoshige;Shinohara, Nobuo;Nonomura, Katsuya

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我们在此报告一例罕见的起源于阴茎的原始神经外胚层肿瘤。一名16岁的男性青少年表现为疼痛的阴茎肿胀。病理、免疫组织化学和细胞遗传学检查证实为原始神经外胚层肿瘤。全切除术后,患者接受了以异环磷酰胺为基础的辅助化疗,为期48周。作为一系列治疗,患者在完成辅助化疗后接受了阴茎重建术。患者在阴茎重建手术后的7年中没有表现出任何复发的迹象,排尿功能完全正常。多模式治疗效果良好,包括积极切除局部控制,强化辅助化疗,阴茎重建术获得外观和功能上的成功。对于患有阴茎原发恶性肿瘤的儿童,也可以选择类似的治疗方法。
We herein present an extremely rare case of primitive neuroectodermal tumor originating in the penis. A 16-year-old male adolescent presented with painful penile swelling. Pathological, immunohistochemical and cytogenetical examinations of the specimens obtained from total penectomy confirmed the diagnosis of primitive neuroectodermal tumor. After total penectomy, the patient received adjuvant chemotherapy with ifosfamide-based regimen for 48 weeks. As a series of therapies, the patient underwent penile reconstruction surgery after completing adjuvant chemotherapy. The patient has not shown any evidence of recurrence for the 7 years after penile reconstruction surgery, and voiding function is completely normal. A favorable outcome was observed by multimodal therapy including aggressive resection for local control, intensive adjuvant chemotherapy, and penile reconstruction with cosmetic and functional success. Similar therapeutic approaches might be selected for children with primary malignant tumors of the penis.