[ XXXX : a clinicopathologic analysis of 8 cases].

@entity1 @entity2801 @entity5 @entity2279 @entity1350 @entity3 @entity158 @entity2280 @entity8146
OBJECTIVE: To further elucidate the clinical and pathologic features of @entity8146 ( @entity8146 ) and its clinical spectrum. METHODS: Eight cases of @entity8146 were reviewed. The clinicopathologic profiles, immunophenotypes and outcome data were investigated. RESULTS: All 8 cases occurred in females with a mean age at presentation of 40 years (range, 13-69 years). Five @entity5 arose in the skin of the head and neck region and lower extremities, two in the long bones and one in the spleen. Clinically, the @entity1 presented with a slowly growing cutaneous plaque or subcutaneous nodule, @entity158 of the upper arm, and dull @entity158 and discomfort in the left hypochondrium respectively. Grossly, the @entity5 appeared as a non-encapsulated gray-yellowish to tan-brown mass with a mean diameter of 2.6 cm (range, 0.8-5.0 cm). On histology, it was composed of delicate branches of elongated vessels lined by a layer of hobnail or matchstick endothelium, exhibiting a retiform pattern with close resemblance of the normal rete testis. Cords or solid nests of epithelioid cells were noted adjacent to the well-formed vessels. In three cases, dilated vascular spaces with formation of intravascular papillary tufts were observed, features overlapping with @entity5 . There was usually marked lymphocytic infiltration in the stroma which was also hyalinized in some cases. One case had regional lymph node @entity3 . By immunohistochemistry, all cases consistently expressed endothelial markers, including @entity2279 (8/8), @entity1 @entity2801 (5/8), @entity2280 (5/7) and D2-40 (1/2). Two of six cases with follow-up information (18-67 months) developed local recurrences, but distant @entity3 was not identified. CONCLUSIONS: @entity8146 is a distinctive @entity5 of adulthood characterized by retiform growth of vessels with striking hobnail endothelium. Although the @entity5 occurs predominantly in the skin, the long bones and the spleen can be occasionally affected. The presence of @entity5 in @entity8146 may suggest a morphologic continuum between these two entities, comprising the family of @entity8146 . Familiarity with the characteristic features of this @entity5 of intermediate malignancy will help in the differential diagnosis of @entity1350 with hobnail endothelium.
