Hitchcock M G, Hurt M A, Santa Cruz D J
Pathology Department, Duke University Medical Center, Durham, NC 27710.
J Cutan Pathol. 1994 Jun;21(3):256-62. doi: 10.1111/j.1600-0560.1994.tb00270.x.
The majority of cutaneous angiosarcomas display typical architectural features of irregular anastomosing vascular channels in the dermis and subcutis. Nuclei are usually hyperchromatic and pleomorphic but the volume of cytoplasm of the neoplastic cells is often small. Diagnosis can be made readily on an adequate biopsy. We recently experienced difficulty diagnosing an angiosarcoma composed predominantly of cells with abundant granular cytoplasm. We were able to compare the present case with sections obtained from the only other reported example. The architectural expression of an anastomosing vascular pattern in areas of tumor, combined with the positive staining for Factor VIII-related antigen (FVIIIRAg) and Ulex europaeus agglutinin-1 (UEA1) enabled us to make a diagnosis of angiosarcoma. The tumor failed to stain for the other endothelial markers (CD31 and CD34) which were positive in the original case. A marker for lysosomes (CD68) stained the granules in both cases. The granular cell variant of cutaneous angiosarcoma is very rare. Diagnosis is possible by recognizing the typical anastomosing neoplastic vascular channels at the periphery of the lesion, and by use of a combination of lectin (UEA1) and immunohistochemical (FVIIIRAg, CD34 and CD31) endothelial markers.
大多数皮肤血管肉瘤在真皮和皮下组织中呈现出不规则吻合血管通道的典型结构特征。细胞核通常染色质增多且形态多样，但肿瘤细胞的细胞质体积往往较小。通过充分的活检能够轻易做出诊断。我们最近在诊断一个主要由具有丰富颗粒状细胞质的细胞组成的血管肉瘤时遇到了困难。我们能够将本病例与从唯一另一例已报道病例获取的切片进行比较。肿瘤区域中吻合血管模式的结构表现，结合对VIII因子相关抗原（FVIIIRAg）和荆豆凝集素-1（UEA1）的阳性染色，使我们能够做出血管肉瘤的诊断。该肿瘤对其他内皮标志物（CD31和CD34）未染色，而在原始病例中这些标志物呈阳性。溶酶体标志物（CD68）在两例中均使颗粒染色。皮肤血管肉瘤的颗粒细胞变体非常罕见。通过识别病变周边典型的吻合肿瘤血管通道，并结合使用凝集素（UEA1）和免疫组化（FVIIIRAg、CD34和CD31）内皮标志物，可以做出诊断。
