Damavandy Ali Alexander, Anatelli Florencia, Skelsey Maral Kibarian
*Department of Dermatology, Georgetown University Hospital, Washington Hospital Center, Washington, DC; and †Mid-Atlantic Pathology Services, Inc, Sterling, VA.
Am J Dermatopathol. 2016 May;38(5):384-7. doi: 10.1097/DAD.0000000000000481.
Malignant glomus tumor is an exceedingly rare vascular malignancy of unclear etiology, which is believed to arise de novo or from malignant transformation of a benign glomus tumor. The authors present a case of an elderly woman evaluated for a painful 2.5-cm purple-brown tumor on the right forearm that grew rapidly within a long-standing, seemingly benign lesion. An excisional biopsy revealed a multilobular dermal tumor composed of areas of benign-appearing glomus tumor cells interspersed with areas of dense, pleomorphic cells arranged in sheets and fascicles with an elevated mitotic rate. The diagnosis of malignant glomus tumor was made on the basis of the lesion's nuclear atypia and elevated mitotic rate. This case demonstrates malignant transformation of a presumably benign glomus tumor precursor lesion highlighting this rarely reported phenomenon. The authors present this case to raise awareness of this vascular malignancy that carries a significant risk of metastasis.
恶性血管球瘤是一种病因不明的极为罕见的血管恶性肿瘤，被认为是原发性的，或由良性血管球瘤恶变而来。作者报告了一例老年女性病例，该患者右前臂有一个2.5厘米大小的紫褐色疼痛性肿瘤，在一个长期存在的看似良性的病变内迅速生长。切除活检显示为多叶性真皮肿瘤，由看似良性的血管球瘤细胞区域与密集的多形性细胞区域交错组成，这些多形性细胞呈片状和束状排列，有较高的有丝分裂率。根据病变的核异型性和较高的有丝分裂率，诊断为恶性血管球瘤。该病例显示了一个可能为良性的血管球瘤前驱病变发生了恶变，突出了这一鲜有报道的现象。作者展示此病例以提高对这种有显著转移风险的血管恶性肿瘤的认识。
