Retiform hemangioendothelioma:a clinicopathologic analysis of 8 cases
10.3760/cma.j.issn.0529-5807.2015.07.013
- VernacularTitle:网状血管内皮瘤八例临床病理学分析
- Author:
Weng I LAO
1
;
Lihua TANG
;
Lin YU
;
Jian WANG
Author Information
1. 复旦大学附属肿瘤医院病理科 复旦大学上海医学院肿瘤学系
- Keywords:
Retiform hemangioendothelioma;
Dabska tumor;
Hobnail hemangioendothelioma;
Immunohistochemistry
- From:
Chinese Journal of Pathology
2015;(7):480-485
- CountryChina
- Language:Chinese
-
Abstract:
Objective To further elucidate the clinical and pathologic features of retiform hemangioendothelioma ( RH) and its clinical spectrum.Methods Eight cases of RH 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 tumors 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 patients presented with a slowly growing cutaneous plaque or subcutaneous nodule , pain of the upper arm, and dull pain and discomfort in the left hypochondrium respectively.Grossly, the tumor 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 Dabska tumor.There was usually marked lymphocytic infiltration in the stroma which was also hyalinized in some cases.One case had regional lymph node metastasis.By immunohistochemistry , all cases consistently expressed endothelial markers , including CD31 ( 8/8 ) , human coagulation factor Ⅷ(5/8), CD34 (5/7) and D2-40 (1/2).Two of six cases with follow-up information (18 -67 months) developed local recurrences , but distant metastasis was not identified.Conclusions RH is a distinctive vascular tumor of adulthood characterized by retiform growth of vessels with striking hobnail endothelium.Although the tumor occurs predominantly in the skin , the long bones and the spleen can be occasionally affected.The presence of Dabska tumor-like areas in RH may suggest a morphologic continuum between these two entities , comprising the family of hobnail hemangioendothelioma.Familiarity with the characteristic features of this vascular tumor of intermediate malignancy will help in the differential diagnosis of vascular neoplasms with hobnail endothelium .
