CUREPATH (Chirec institute- Brussels, CHU Tivoli - La Louvière), Rue de Borfilet, 12A, 6040 Jumet, Charleroi, Belgium.
Pathology Department, Erasme University Hospital, Université Libre de Bruxelles, CP 610, Route de Lennik, 808, 1070, Brussels, Belgium.
Diagn Pathol. 2020 Mar 14;15(1):24. doi: 10.1186/s13000-020-00945-9.
Malignant eccrine spiradenoma is one of the rarest sweat-gland tumors. Here, we describe a rare case of low grade malignant eccrine spiradenoma located at the vulva.
The vulvar lesion was described as a mass measured 3.5 cm and located in the dermis and subcutis with no attachment to the epidermis. The neoplasm was arranged in ragged sheets or solid nodules sometimes with focal necrosis. The tumor cells had hyperchromatism, pleomorphism, and prominent nucleoli with high mitotic index and KI-67 estimated at 70-80%.
It's only the fifth case of malignant eccrine spiradenoma localized at the vulva. This is the first time that an HPV genotyping was made in this type of lesion with no HPV found while the p16 expression was diffuse. Moreover, it's the first time that a p53 mutation is detected by sequencing in this location.
恶性小汗腺螺旋腺瘤是最罕见的汗腺肿瘤之一。在这里，我们描述了一例罕见的位于外阴的低度恶性小汗腺螺旋腺瘤。
外阴病变表现为一个 3.5 厘米的肿块，位于真皮和皮下组织，与表皮无附着。肿瘤排列呈参差不齐的片状或实性结节，有时伴有局灶性坏死。肿瘤细胞有明显的核异质，多形性，核仁明显，有较高的有丝分裂指数和 KI-67，估计为 70-80%。
这是第五例位于外阴的恶性小汗腺螺旋腺瘤。这是第一次在外阴这种病变中进行 HPV 基因分型，未发现 HPV，而 p16 表达弥漫。此外，这也是首次在外阴部位检测到 p53 突变。
