Clinicopathologic analysis of spindle cell/sclerosing XXXX .

@entity1 @entity242 @entity5 @entity786 @entity4316 @entity1891 @entity3 @entity980 @entity236 @entity204
BACKGROUND: Clinical characteristics and optimal treatment strategies for spindle cell/sclerosing @entity980 (ssRMS) have not been well established because of its rarity. PROCEDURE: Retrospective re-evaluation of @entity242 specimens (1997-2014) identified 16 ssRMSs (median age 20 years, range 7-39 years). Clinicopathological features, clinical course, and outcome were analyzed. RESULTS: Primary disease sites were the head and neck (10 cases) and other regions (6 cases). Nine cases were at Intergroup @entity980 Study preoperative stage 3. The @entity5 were >5   cm in 13 cases. Two @entity1 had lymph node @entity3 , but none had distant @entity3 at presentation. At follow-up (median period 39 months, range 4.6-201), seven @entity1 were alive without disease. Among nine @entity1 treated with the @entity1891 , @entity4316 , and @entity786 (VAC) regimen, five responded well, with four surviving free of disease. Among ten @entity1 with recurrent or progressive disease, three experienced local recurrence, four had distant @entity3 , and three had both. None exhibited @entity236 . Eight of the ten @entity1 died in median time from relapse to @entity204 of 18 months (range 11-56). CONCLUSIONS: Although most ssRMSs present as a bulky @entity5 , nodal or distant @entity3 are rare at presentation. ssRMSs initially show good response to VAC, but >50% of @entity5 recur or progress; these data suggest a worse prognosis of ssRMS compared to the pediatric embryonal variant. As relapse typically occurs as local or distant solitary lesion without @entity236 , localized treatment combined with chemotherapy would contribute to improve the prognosis of recurrent ssRMS. Pediatr @entity5    2014 Wiley Periodicals, Inc.
