Arab Maliheh, Faghih Nafiseh, Asghari Mahsa, Agha Majidi Mona, Ghavami Behnaz, Noori Ardebili Shahla
Department of Gyneco-Oncology, Imam Hossein Medical Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
Department of Gyneco-Oncology Taleghani Medical Center, Shahid Beheshti University of Medical Sciences and Health Services, Tehran, Iran.
Caspian J Intern Med. 2023 Summer;14(3):572-576. doi: 10.22088/cjim.14.3.572.
Dermatofibrosarcoma protuberans (DFSP) is a soft tissue tumor that originates from deep layers of the dermis and mainly is common in young adults to middle age. This tumor is rare in the vulva.
A 53-year-old multiparous menopause female had complained of asymptomatic swelling of the right labia major. Dermatofibrosarcoma protuberans was reported in primary tumor resection. Six months later, rapid growing mass recurred in the vulva. The patient underwent radical Vulvectomy and resection of the margin of about 2-3 cm along with bilateral Inguinofemoral lymphadenectomy. The margins of the mass were negative in the frozen section. Microscopic examination revealed that hypercellular neoplasm in dermis comprising monomorphic spindle cells with high mitotic activity, some hyperchromatic nuclei arranged in palisading fashion. Microscopic and IHC study confirmed the conversion of dermatofibrosarcoma protuberans to fibrosarcoma.
This case was presented due to the rarity of dermatofibrosarcoma protuberance in the vulva and pathologic conversion to fibrosarcoma.
隆突性皮肤纤维肉瘤（DFSP）是一种起源于真皮深层的软组织肿瘤，主要见于青壮年。该肿瘤在外阴罕见。
一名53岁多产绝经女性主诉右侧大阴唇无症状性肿胀。原发性肿瘤切除术后报告为隆突性皮肤纤维肉瘤。6个月后，外阴出现快速生长的肿块复发。患者接受了根治性外阴切除术，切除约2 - 3厘米的边缘组织，并进行双侧腹股沟股淋巴结切除术。肿块边缘在冰冻切片中为阴性。显微镜检查显示真皮内细胞增多的肿瘤，由具有高有丝分裂活性的单形性梭形细胞组成，并可见一些呈栅栏状排列的深染细胞核。显微镜和免疫组化研究证实隆突性皮肤纤维肉瘤已转变为纤维肉瘤。
本病例报告是由于隆突性皮肤纤维肉瘤在外阴罕见且发生了病理转变为纤维肉瘤。
