CASE OF THE MONTH
Myxoid LiposarcomaThis is the case of a 26 year old woman who presents with a 205 gram, 9.7 x 9 x 5 cm soft tissue mass of the vulva with a smooth, lobulated surface. The cut surface of the mass is tan beige to dusky purple brown with a mucinous component. The diagosis is a MYXOID LIPOSARCOMA with the following findings.
TUMOR SIZE: 9.7 CENTIMETERS
TUMOR GRADE: GRADE 2 (FNCLCC)
ROUND CELL/HYPERCELLULAR COMPONENT: ABSENT
NECROSIS: ABSENT
MITOTIC ACTIVITY: < 1/10 HPF
MARGINS: WIDELY POSITIVE
NOTE: Because of the slightly atypical body site location and relatively young patient age, additional testing was performed. ProPath Laboratories report FISH is positive for the DDIT3 (12Q23) gene translocation consistent with myxoid liposarcoma. Immunohistochemical staining, including positivity for Ny-Eso-1 and lack of reactivity for EMA, Desmin, ER, and CD34 support the diagnosis. A high grade component is absent. Classic pulmonary edema-like changes are present as well as a chicken wire like vasculature. Lipoblasts are focal. A vulvar location is unusual and has only been reported with genetic confirmation in one previous patient in 2015, a 34 year old woman with an approximately 12 cm tumor (Schoolmeester, et. al., International Journal of Gynecologic Pathology).
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