Leake J F, Buscema J, Cho K R, Currie J L
Department of Gynecology and Obstetrics, Johns Hopkins Hospital, Baltimore, Maryland 21205.
Gynecol Oncol. 1991 Jun;41(3):245-9. doi: 10.1016/0090-8258(91)90317-x.
Dermatofibrosarcoma protuberans of the vulva is an uncommon low-grade sarcoma of dermal origin. Although wide local excision is the treatment of choice, microscopic tumor projections beyond the central tumor nodule explain the tumors' propensity for local recurrence. Frozen sections of margins may be useful to ensure complete resection. The following report contributes two additional patients with this uncommon neoplasm. Notably, one of these two had a fibrosarcomatous area within the dermatofibrosarcoma protuberans. This is the second reported case of a fibrosarcoma arising in a dermatofibrosarcoma protuberans of the vulva.
外阴隆突性皮肤纤维肉瘤是一种罕见的起源于真皮的低度恶性肉瘤。尽管广泛局部切除是首选治疗方法，但显微镜下肿瘤超出中央肿瘤结节的情况解释了肿瘤局部复发的倾向。切缘冰冻切片可能有助于确保完全切除。以下报告新增了两名患有这种罕见肿瘤的患者。值得注意的是，这两名患者中有一名在外阴隆突性皮肤纤维肉瘤内存在纤维肉瘤区域。这是外阴隆突性皮肤纤维肉瘤中发生纤维肉瘤的第二例报告病例。
