Niedt G W, Greco M A, Wieczorek R, Blanc W A, Knowles D M
Department of Pathology, New York University School of Medicine, New York 10016.
Pediatr Pathol. 1989;9(5):567-75. doi: 10.3109/15513818909026915.
We describe two children with vascular neoplasms that resembled Kaposi's sarcoma in places. Both presented with intraabdominal masses and severe thrombocytopenia. At autopsy the tumors extensively infiltrated the peritoneum and retroperitoneum and surrounded or invaded numerous organs including the kidneys, pancreas, adrenal glands, gastrointestinal tract, mesentery, and lymph nodes in both cases, and spleen or bone marrow in one case each. The neoplasms were histologically identical and displayed two patterns: dilated vascular spaces (angiomatous areas) lined by flat endothelial-like cells and areas of spindle cells forming slitlike vascular spaces similar to those described in Kaposi's sarcoma. Tumor cells in both cases expressed markers for endothelial cells. The clinical and histologic character of these neoplasms differentiates them from Kaposi's sarcoma, hemangioendothelioma, and from conventional juvenile hemangioma.
我们描述了两名患有血管肿瘤的儿童，这些肿瘤在某些部位类似于卡波西肉瘤。两人均表现为腹腔内肿块和严重血小板减少症。尸检时，肿瘤广泛浸润腹膜和腹膜后，在两例中均包围或侵犯了许多器官，包括肾脏、胰腺、肾上腺、胃肠道、肠系膜和淋巴结，在其中一例中还侵犯了脾脏或骨髓。肿瘤在组织学上相同，呈现出两种模式：由扁平内皮样细胞内衬的扩张血管腔隙（血管瘤样区域）和梭形细胞形成类似卡波西肉瘤中描述的裂隙状血管腔隙的区域。两例肿瘤细胞均表达内皮细胞标志物。这些肿瘤的临床和组织学特征将它们与卡波西肉瘤、血管内皮瘤以及传统的幼年血管瘤区分开来。
