The perivascular epithelioid cell has been proposed to be the unifying proliferating cell type in a number of lesions such as @entity359 , @entity3008 , @entity1358 . With the exception of rare examples of @entity359 , they are non-metastasizing. We report four examples of a new member of this family of @entity1824 that occur in abdominopelvic location and show metastatic properties. The @entity1 , all @entity1 , were aged 19 to 41 years (mean, 32), and presented with a @entity5 mass involving the serosa of the ileum, uterus or pelvic cavity. Morphologically, the @entity5 were composed of sheets of large polygonal cells with glycogen-rich clear or eosinophilic cytoplasm and moderately pleomorphic nuclei, traversed by a delicate vasculature, mimicking @entity1358 . There were areas of coagulative @entity39 and occasional mitotic figures. Intracytoplasmic brown pigment was present in two cases. Spindly cells, smooth muscle and fat were absent. @entity3 was present in all, lymph node @entity3 was documented in two and @entity3 to the ovary was present in one case. Two @entity1 developed widespread metastatic disease after 10 and 28 months from diagnosis. One @entity1 showed the clinical signs of @entity2916 . In spite of the epithelial-like appearance, the @entity5 cells were negative for epithelial markers but were strongly positive with the melanogenesis-related marker HMB45. Another melanogenesis marker ( @entity3059 ) was positive in two cases. Other markers including S-100 protein, @entity2277 , @entity2283 and @entity3507 were negative. Thus, these @entity5 are not readily classifiable in the existing schema of known entities, and show overlapping morpho-phenotypic features of clear cell "sugar" @entity2 . We consider them as @entity242 composed of a pure population of uncommitted perivascular epithelioid cell, that lack modulation toward smooth muscle or adipose cells.

@entity242 of perivascular epithelioid cells. Report of four cases in young XXXX , one with @entity2916 .

@entity1 :: ('9606', 'Species') :: ['women', 'patients', 'patient'] @entity2283 :: ('1674', 'Gene') :: ['muscle-specific actin, desmin'] @entity1824 :: ('MESH:D054973', 'Disease') :: ['perivascular epithelioid cell neoplasms'] @entity39 :: ('MESH:D009336', 'Disease') :: ['necrosis'] @entity5 :: ('MESH:D009369', 'Disease') :: ['tumor', 'tumors'] @entity3507 :: ('1113', 'Gene') :: ['chromogranin A'] @entity242 :: ('MESH:D012509', 'Disease') :: ['sarcomas'] @entity2 :: ('MESH:D008175', 'Disease') :: ['tumor of the lung and epithelioid angiomyolipoma'] @entity1358 :: ('MESH:D002292', 'Disease') :: ['clear cell "sugar" tumor and renal capsuloma', 'clear cell carcinoma'] @entity2916 :: ('MESH:D014402', 'Disease') :: ['tuberous sclerosis'] @entity3008 :: ('MESH:D018192', 'Disease') :: ['lymphangiomyomatosis'] @entity2277 :: ('7431', 'Gene') :: ['vimentin'] @entity3 :: ('MESH:D009362', 'Disease') :: ['metastasis', 'Lymphovascular invasion'] @entity3059 :: ('2315', 'Gene') :: ['MART-1'] @entity359 :: ('MESH:D018207', 'Disease') :: ['angiomyolipoma']
@entity1 :: (9606,Species) :: ['women']
