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阴茎恶性外周神经鞘瘤

Malignant peripheral nerve sheath tumour of penis.

作者信息

Kaur J, Madan R, Singh L, Sharma D N, Julka P K, Rath G K, Roy S

机构信息

Department of Radiation Oncology, Dr BRA Institute Rotary Cancer Hospital, All India Institute of Medical Sciences, New Delhi, India.

出版信息

Andrologia. 2015 Apr;47(3):333-5. doi: 10.1111/and.12267. Epub 2014 Mar 18.

Abstract

Malignant peripheral nerve sheath tumour (MPNST) is a rare variety of soft tissue sarcoma that originates from Schwann cells or pluripotent cells of neural crest origin. They have historically been difficult tumours to diagnose and treat. Surgery is the mainstay of treatment with a goal to achieve negative margins. Despite aggressive surgery and adjuvant therapy, the prognosis of patients with MPNST remains poor. MPNST arising from penis is a very rare entity; thus, it presents a diagnostic and therapeutic challenge. We present a case of penile MPNST in a 38-year-old man in the absence of neurofibromatosis treated with surgery followed by post-operative radiotherapy to a dose of 60 Gray in 30 fractions and adjuvant chemotherapy with ifosfamide and adriamycin.

摘要

恶性外周神经鞘瘤(MPNST)是一种罕见的软组织肉瘤,起源于施万细胞或神经嵴来源的多能细胞。从历史上看,它们一直是难以诊断和治疗的肿瘤。手术是主要的治疗方法,目标是实现切缘阴性。尽管进行了积极的手术和辅助治疗,但MPNST患者的预后仍然很差。阴茎来源的MPNST是一种非常罕见的实体;因此,它带来了诊断和治疗方面的挑战。我们报告一例38岁男性阴茎MPNST病例,该患者无神经纤维瘤病,接受了手术治疗,随后进行术后放疗,剂量为60格雷,分30次给予,以及异环磷酰胺和阿霉素辅助化疗。

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