Vulvar Lipoblastoma-Like Tumor

**Semantic type:** Neoplastic Process

**Definition:** A benign, well-defined, lobulated, myxoid or gelatinous tumor that arises from the vulva. It shares morphological features with lipoblastoma, myxoid liposarcoma, and spindle cell lipoma. It is characterized by a lobulated growth and is composed of mature adipocytes, lipoblasts, and spindle cells without atypia in a myxoid matrix. DDIT3 and PLAG1 gene rearrangements seen in lipoblastoma and myxoid liposarcoma are not present in this tumor. It can recur infrequently and there are no reports of metastasis.

**Synonyms:** - Vulvar Lipoblastoma-Like Neoplasm