Department of Dermatology, Nara Medical University School of Medicine, Kashihara, Japan.
Department of Dermatology, Nara Prefecture General Medical Center, Nara, Japan.
J Cutan Pathol. 2024 Jun;51(6):430-433. doi: 10.1111/cup.14614. Epub 2024 Mar 25.
The histopathologic diagnosis of poorly differentiated cutaneous angiosarcoma can be challenging. We report a case of cutaneous epithelioid angiosarcoma with numerous multinucleated giant cells (MGCs) developing pulmonary metastasis. A 79-year-old man presented with a red-purple plaque on the scalp. A skin biopsy revealed epithelioid cell proliferation, admixed with numerous MGCs, and background hemorrhage. Vascular spaces were focally present and lined by atypical endothelial cells, including MGCs. Immunohistochemically, tumor cells, including MGCs, were positive for CD31, D2-40, and ERG. The patient received radiation therapy and chemotherapy, after which a follow-up CT scan revealed symptomless pneumothorax and pulmonary metastases. The patient received palliative partial lung resection, and the specimen revealed histopathological and immunohistochemical features similar to the primary cutaneous lesion. Our report expands the morphologic spectrum of cutaneous epithelioid angiosarcoma. Cutaneous angiosarcoma is an aggressive neoplasm; thus, awareness of this rare manifestation is important.
低分化皮肤血管肉瘤的组织病理学诊断具有挑战性。我们报告了一例伴大量多核巨细胞（MGC）的皮肤上皮样血管肉瘤发生肺转移。一名 79 岁男性因头皮出现红斑紫斑就诊。皮肤活检显示上皮样细胞增生，伴有大量多核巨细胞和背景出血。血管腔局灶性存在，由包括多核巨细胞在内的非典型内皮细胞排列。免疫组化染色显示，包括多核巨细胞在内的肿瘤细胞 CD31、D2-40 和 ERG 阳性。患者接受了放疗和化疗，随后 CT 扫描显示无症状性气胸和肺转移。患者接受了姑息性部分肺切除术，标本显示与原发性皮肤病变相似的组织病理学和免疫组化特征。我们的报告扩展了皮肤上皮样血管肉瘤的形态学谱。皮肤血管肉瘤是一种侵袭性肿瘤；因此，对这种罕见表现的认识很重要。
