Epithelioid hemangioendothelioma of the forearm with radius involvement. Case report.docVIP

GhermanandFodorDiagnosticPathology2011,6:120 /content/6/1/120 CASE REPORT OpenAccess Epithelioidhemangioendotheliomaofthe forearmwithradiusinvolvement.Casereport ClaudiaDGherman1andDanielaFodor2* Abstract Epithelioidhemangioendothelioma(EHE)isarare,well-differentiatedendothelialtumorwithintermediate malignancywhichdevelopsmorefrequentlyfromtheperipheralveins,generallyinthelowerlimb.BoneEHE compriseslessthan1%oftheboneneoplasms.Wepresentthecaseofayoungman,24-year-old,withEHEofthe forearmwithsecondaryinvolvementofthedistalradius.Thelocationandtheextensionofthetumoralloweda wideexcision,withoutthereconstructionofradius,followedbyadjuvantradiotherapy,withasubsequentfavorable evolution.Basedontheclinical,radiographic,andpathologicalfeaturesoftheEHEreview,weconcludedthatitis difficulttoadoptastandardizedtherapeuticapproachduetotheextremelylowincidenceofthebone involvementinEHEandthevariabletendenciestowardsmalignancyofthistumor.Toourknowledgethisisthe thirdcaseofEHEwiththeinvolvementoftheradius. Keywords:vasculartumor,intermediate-malignanttumor,secondaryboneinvolvement Background Casepresentation Epithelioidhemangioendothelioma(EHE)isarare,well- Clinical summary differentiated endothelial tumor with a wide spectrum A 24-year-old, right-handed male, machinist, with no ofbehavior. EHE,also knownas:lowdegree anaplastic pathological personal or family history, was admitted angiosarcoma,cellularhemangioma,histiocytoidheman- to evaluate a right forearm mass that has slowly giomaorhemangioendothelioma,wasconsideredaterm increased in the past 4 years. The patient described meanttodescribetumorsthatrangebetweenhemangio- vague forearm pain, weakness and an oval mass (of masandangiosarcomas. It represents 1% of all vascular neoplasms and it is was found on palpation of the extensor part of the locallyaggressive[1]. forearm. No numbness or functional impairment of approximately 7/4 cm) of hard-elastic consistency that Generally sp