XXXX : a Multi-Institutional Study of 42 Cases in Japan.

@entity5 @entity3293 @entity1
BACKGROUND: @entity3293 ( @entity3293 ) is a rare @entity5 . Despite a consensus for the distinct clinicopathologic entity of @entity3293 , its clinical features remain controversial. In addition, most studies have contained a small number of @entity1 who underwent definitive surgical treatment. METHODS: Forty-two cases of @entity3293 , which had been identified from files of eight affiliated hospitals and confirmed for histologic diagnosis at the Pathology Center, were analyzed for histologic grade, demographics, treatments, outcomes, and prognostic factors. The average follow-up period was 7.4 years. RESULTS: Included in the study were 20 @entity1 and 22 @entity1 with a mean age at diagnosis of 52.1 years. The @entity5 were located mainly in the lower extremities (69%). Thirty-three @entity5 (79%) were classified as Grade 1 and nine as Grade 2 according to the modified French System. Overall survival was 100% at 5 years and 88% at 10 years. Disease-free survival was 45% at 5 years and 36% at 10 years. Inadequate initial surgery was defined as a significant risk factor for local recurrence by univariate analysis of all 42 @entity1 but not by the analysis of those 30 @entity1 who had undergone wide @entity5 excision or amputation. Wide excision led to the recurrence rate of 14%. CONCLUSIONS: These findings supported the role of wide excision in the local control of @entity3293 , irrespective of the previous excision procedure or recurrence. The protracted clinical course of the @entity5 and the presence of @entity1 who had distant metastasis develop after definitive surgery of the @entity5 represented @entity3293 as intermediate @entity5 .
