title: Superficial Ewing's sarcoma family of tumors: a clinicopathological study with differential diagnoses.
abstract: BACKGROUND
  Superficial/cutaneous Ewing's sarcoma family of tumors (ESFT) are rare and have a relatively favorable prognosis compared with deep-seated tumors. The aim of the present study is to describe the clinicopathological characteristics of six genetically confirmed ESFT presenting a superficial location.
  
  
  METHODS
  Clinical data, radiology, histopathology, immunohistochemistry, molecular study [reverse transcriptase-polymerase chain reaction (RT-PCR)/fluorescence in situ hybridization], treatment and follow-up data were retrieved.
  
  
  RESULTS
  Locations included fingers (2), back (1), neck (1), thigh (1) and subcutaneous breast (1). Two tumors showed conventional morphology, one consisted of primitive neuroectodermal tumor and three tumors showed atypical vascular morphology with hemosiderin deposition and pigmentation. All cases showed CD99, FLI-1, HNK-1 and CAV-1 positivity. RT-PCR revealed the EWS/Fli1 gene fusion in all cases. Treatment was by wide excision in all cases; one received chemotherapy (CT) and one CT and radiotherapy. Available follow-up revealed the following: two patients with metastasis and death at 5 months and 2 years and one local recurrence at 18 years.
  
  
  CONCLUSIONS
  Superficial ESFT appears to have a relatively favorable prognosis but further studies with additional series, a larger number of cases and more extensive follow-up are necessary to confirm this statement.
authors.affiliation: Pathology Department, Medical School, Valencia University, Valencia, Spain.
authors.forename: Isidro
authors.initials: I
authors.lastname: Machado
authors.forename: Beatriz
authors.initials: B
authors.lastname: Llombart
authors.forename: Silvia
authors.initials: S
authors.lastname: Calabuig-Fariñas
authors.forename: Antonio
authors.initials: A
authors.lastname: Llombart-Bosch
mesh_terms: D000328:Adult; D000368:Aged; D014408:Biomarkers, Tumor / Q000378:metabolism; D015266:Carcinoma, Merkel Cell / Q000175:diagnosis; D003131:Combined Modality Therapy; D004273:DNA, Neoplasm / Q000032:analysis; D003937:Diagnosis, Differential; D005260:Female; D015870:Gene Expression; D006801:Humans; D017404:In Situ Hybridization, Fluorescence; D017728:Lymphoma, Large-Cell, Anaplastic / Q000175:diagnosis; D008297:Male; D008545:Melanoma / Q000175:diagnosis; D008875:Middle Aged; D015514:Oncogene Proteins, Fusion / Q000235:genetics / Q000378:metabolism; D011379:Prognosis; D051789:Proto-Oncogene Protein c-fli-1 / Q000235:genetics / Q000378:metabolism; D034802:RNA-Binding Protein EWS / Q000235:genetics / Q000378:metabolism; D012512:Sarcoma, Ewing / Q000235:genetics / Q000556:secondary* / Q000628:therapy; D012878:Skin Neoplasms / Q000175:diagnosis / Q000556:secondary; D012983:Soft Tissue Neoplasms / Q000235:genetics / Q000473:pathology* / Q000628:therapy; D055815:Young Adult
chemical_list: D014408:Biomarkers, Tumor; D004273:DNA, Neoplasm; C106031:EWS-FLI fusion protein; D015514:Oncogene Proteins, Fusion; D051789:Proto-Oncogene Protein c-fli-1; D034802:RNA-Binding Protein EWS