Mr. Nucci et Cdm. Fletcher, LIPOSARCOMA (ATYPICAL LIPOMATOUS TUMORS) OF THE VULVA - A CLINICOPATHOLOGICAL STUDY OF 6 CASES, International journal of gynecological pathology, 17(1), 1998, pp. 17-23
Liposarcoma, the most common soft tissue sarcoma, usually arises in th
e limbs, trunk, or abdomen, but very rarely in the vulva. Six cases of
liposarcoma of the vulva are described here. They were characterized
by their occurrence in predominantly middle-aged women (median age 52)
, variable size, and focally infiltrative margin. The preoperative cli
nical diagnosis for all patients was of a benign lesion, with a diagno
sis of lipoma in two. Four of six cases had the usual histologic appea
rance of a well-differentiated liposarcoma/atypical lipomatous tumor w
ith variation in adipocyte size, adipocytic nuclear atypia, and occasi
onal lipoblasts. Two of the six cases had a very unusual histologic ap
pearance, not previously described, with an admixture of neoplastic bl
and spindle and round cells along with adipocytes showing variation in
size as well as numerous mainly bivacuolated lipoblasts. Liposarcomas
of other types were not identified. Follow-up data were available for
five of the six patients and all were treated by excision with no add
itional treatment, Four showed no sign of recurrence at 12, 14, 18, an
d 84 months. One tumor was incompletely excised, regrew over a 10-year
period, was re-excised, and showed no further recurrence 31 months th
ereafter. Liposarcoma can occur in the vulva, is predominantly of the
well-differentiated subtype, can show an unusual histologic appearance
with an admixture of spindle cells and numerous lipoblasts, and seems
to have a behavior similar to these tumors in other anatomic location
s.