Behranwala K A, Clark M A, Thomas J M
Sarcoma and Melanoma Unit, Royal Marsden NHS Trust, London, UK.
Eur J Surg Oncol. 2002 Jun;28(4):437-42. doi: 10.1053/ejso.2002.1267.
Primary perineal tumours in adults are rare entities that have been infrequently reported (one clinical series of nine patients with sarcoma and isolated case reports). We present our experience of perineal tumours better to characterize and define the natural history of this condition.
Perineal tumours occurring in adults (>18 years) from January 1990 were identified from the Royal Marsden Hospital's Sarcoma Unit prospective database.
Nineteen perineal soft-tissue tumours (12 malignant and seven benign) were evaluated and treated at the Royal Marsden Hospital during this period. Liposarcoma (n=3) and aggressive angiomyxoma (n=4) were the most frequent histological subtypes. Three malignant and four benign tumours were larger than 10 cm. Most tumours were located deep to the deep fascia. Local recurrence in those with sarcoma occurred in one of five patients with negative microscopic margins and in one of two patients who had positive microscopic margins. One patient each with aggressive angiomyxoma and fibromatosis recurred locally.
Aggressive treatment in the form of wide local excision is associated with fewer local recurrences in adults with primary perineal soft-tissue tumours. The aim of surgical treatment should be to obtain negative resection margins without causing disturbance to urinary or anorectal function.
成人原发性会阴肿瘤是罕见的实体瘤，鲜有报道（一组9例肉瘤患者的临床系列及个别病例报告）。我们介绍我们治疗会阴肿瘤的经验，以便更好地描述和界定这种疾病的自然史。
从皇家马斯登医院肉瘤科前瞻性数据库中确定1990年1月以来发生在成人（>18岁）的会阴肿瘤。
在此期间，皇家马斯登医院评估并治疗了19例会阴软组织肿瘤（12例恶性，7例良性）。脂肪肉瘤（n=3）和侵袭性血管黏液瘤（n=4）是最常见的组织学亚型。3例恶性肿瘤和4例良性肿瘤直径大于10 cm。大多数肿瘤位于深筋膜深层。肉瘤患者中，5例镜下切缘阴性者中有1例局部复发，2例镜下切缘阳性者中有1例局部复发。侵袭性血管黏液瘤和纤维瘤病各有1例患者局部复发。
对于成人原发性会阴软组织肿瘤，广泛局部切除这种积极的治疗方式可减少局部复发。手术治疗的目的应是获得阴性切缘，同时不影响泌尿或肛门直肠功能。
