Lipoblastoma-like Tumor of the Vulva: Further Characterization in 8 New Cases

Lipoblastoma-like tumor of the vulva (LLTV) is an exceptionally rare adipocytic mesenchymal tumor with only 4 cases reported previously. The aim of this study is to help characterize this tumor type. Eight cases of LLTV were identified in the consult files of one of the authors (C.D.M.F.). Clinical data and follow-up information were obtained from the referring pathologists. Detailed clinical information is available in all 8 cases. Patient age ranged from 17 to 46 years (median 27 y). Lesions presented as a vulvar mass with a variable growth rate, sometimes being painful. The most common preoperative clinical diagnosis was a Bartholin gland abnormality (4). The size of the mass ranged from 3.5 to 15 cm (median 5.6 cm). The lesions were described as grossly myxoid, mucoid, or gelatinous (4), well defined (4), and lobulated (3). None of the lesions exhibited necrosis. Histologically, LLTVs were lobulated and composed of variable proportions of mature adipocytes, bland univacuolated and bivacuolated lipoblasts, and spindle cells with short stubby nuclei in a diffusely myxoid background with prominent branching vessels. Nuclear atypia was minimal. No necrosis and only rare mitotic activity was identified. Only 1 of 7 tumors (in a 26-y-old patient) was positive for PLAG1 and retinoblastoma (Rb). Rb was lost, and PLAG1 was not expressed in all other tumors. HMGA2 was negative in 6 of 6 cases tested. Tumors were negative for S100 (5/5), MDM2, and CDK4 (5/6; 1 with just scattered ce...
Source: The American Journal of Surgical Pathology - Category: Pathology Tags: Original Articles Source Type: research