Department of Pathology at the University of Texas, M.D. Anderson Cancer Center, Houston, TX 77030, USA.
Am J Surg Pathol. 2010 Mar;34(3):393-400. doi: 10.1097/PAS.0b013e3181cf7fc1.
Dermatofibrosarcoma protuberans (DFSP) is a low-grade sarcoma seldom seen in the vulva with only 29 cases reported. We present the clinicopathologic and immunohistochemical features of 13 such cases seen in our institution over a period of 29 years (1978 to 2007). Patient age ranged from 23 to 76 years (mean, 46 y). Twelve patients had a vulvar mass. One patient presented with a pigmented skin lesion. Tumor size ranged from 1.2 to 15 cm (median, 4 cm). Microscopically, all the cases showed typical features of DFSP. In 1 case, myxoid changes were also noted; 3 cases showed fibrosarcomatous transformation. Of interest, in 7 of our 13 cases, a variety of diagnoses, such as cellular dermatofibroma, cellular leiomyoma, neurofibroma, low-grade leiomyosarcoma, fibrosarcoma, low-grade malignant schwannoma, desmoplastic melanoma, cellular neurofibroma, and low-grade malignant peripheral nerve sheet tumor were initially considered. All 11 cases tested for CD34 were positive, whereas 7/9 cases, 8/9 cases, and 9/9 cases were positive for PDGFR-alpha, PDGFR-beta, and c-abl, respectively. All patients were initially treated with excisional biopsy, wide local excision, or radical vulvectomy. Local recurrences occurred in 7 cases. One patient also developed distant metastases. All recurrences were treated surgically; 1 patient also received chemotherapy and radiotherapy and another received imatinib (Gleevec). Follow-up data ranging from 2 to 444 months was available for all patients. Nine patients had no evidence of disease, 2 patients were alive with disease, 1 patient had died of disease, and 1 patient had died of other causes. DFSP affects women of a wide age range and has a propensity to recur locally. The frequent expression of PDGFR-alpha, PDGFR-beta, and c-abl in these cases agrees with the findings of other investigators and supports the use of imatinib (Gleevec) in cases that are recurrent or not amenable to surgery.
隆突性皮肤纤维肉瘤（DFSP）是一种少见的外阴低度恶性肉瘤，仅有 29 例报道。我们报道了在过去 29 年（1978 年至 2007 年）中，我院遇到的 13 例此类病例的临床病理和免疫组化特征。患者年龄 23 岁至 76 岁，平均年龄 46 岁。12 例患者有外阴肿块，1 例患者有色素沉着皮损。肿瘤大小 1.2 厘米至 15 厘米，中位数为 4 厘米。所有病例均表现出典型的 DFSP 特征。其中 1 例有黏液样变，3 例有纤维肉瘤样转化。有趣的是，在我们的 13 例病例中，有 7 例最初考虑了多种诊断，如细胞性纤维瘤、细胞性平滑肌瘤、神经纤维瘤、低度恶性平滑肌肉瘤、纤维肉瘤、低度恶性恶性外周神经鞘瘤、促结缔组织增生性黑色素瘤、细胞性神经纤维瘤和低度恶性外周神经鞘瘤。所有 11 例 CD34 检测均为阳性，而 PDGFR-α、PDGFR-β和 c-abl 的阳性率分别为 7/9、8/9 和 9/9。所有患者均行广泛局部切除或根治性外阴切除术，7 例患者出现局部复发。1 例患者发生远处转移。所有复发患者均行手术治疗，1 例患者同时接受化疗和放疗，另 1 例患者接受伊马替尼（格列卫）治疗。所有患者的随访时间为 2 个月至 444 个月。9 例患者无疾病证据，2 例患者带瘤生存，1 例患者死于疾病，1 例患者死于其他原因。DFSP 影响广泛年龄范围的女性，且具有局部复发的倾向。这些病例中 PDGFR-α、PDGFR-β和 c-abl 的频繁表达与其他研究者的发现一致，支持在复发或不可手术的病例中使用伊马替尼（格列卫）。
