title: Ewing sarcoma family of tumors in unusual sites: confirmation by rt-PCR.
abstract: Ewing sarcoma family tumors originating in the palate or adrenal gland are extremely rare and may cause difficulty in diagnosis. More common tumors primary to these sites need to be excluded before one arrives at the correct diagnosis. We have recently diagnosed 2 such cases. The 1st case was that of a 24-year-old woman who presented with a swelling in the right side of the hard palate. The 2nd case was diagnosed in a 28-year-old woman who presented with a mass in the right adrenal gland. In both cases, the diagnosis of Ewing sarcoma family of tumors was confirmed by immunohistochemical studies and reverse transcriptase-polymerase chain reaction (RT-PCR). The hard palate case is the 1st and the adrenal gland the 3rd case of Ewing sarcoma family of tumors arising in these sites, in which the diagnosis was confirmed by RT-PCR and/or cytogenetics. Accurate diagnosis of Ewing sarcoma family of tumors is crucial for the management of patients, and when found in such rare locations, diagnosis should be supported by immunohistochemical and/or molecular genetic studies.
authors.affiliation: Laboratory of Pathology, National Cancer Institute, National Institutes of Health, Bethesda, MD 20892, USA.
authors.forename: Atif A
authors.initials: AA
authors.lastname: Ahmed
authors.forename: Victor E
authors.initials: VE
authors.lastname: Nava
authors.forename: Thu
authors.initials: T
authors.lastname: Pham
authors.forename: Jeffery K
authors.initials: JK
authors.lastname: Taubenberger
authors.forename: Jack H
authors.initials: JH
authors.lastname: Lichy
authors.forename: Lynn
authors.initials: L
authors.lastname: Sorbara
authors.forename: Mark
authors.initials: M
authors.lastname: Raffeld
authors.forename: Crystal L
authors.initials: CL
authors.lastname: Mackall
authors.forename: Maria
authors.initials: M
authors.lastname: Tsokos
mesh_terms: D000310:Adrenal Gland Neoplasms / Q000235:genetics / Q000473:pathology* / Q000601:surgery; D000328:Adult; D014408:Biomarkers, Tumor / Q000032:analysis; D018076:DNA, Complementary / Q000032:analysis; D005260:Female; D006801:Humans; D007150:Immunohistochemistry / Q000379:methods; D008279:Magnetic Resonance Imaging; D015514:Oncogene Proteins, Fusion / Q000032:analysis; D010157:Palatal Neoplasms / Q000235:genetics / Q000473:pathology* / Q000601:surgery; D021362:Palate, Hard / Q000473:pathology* / Q000601:surgery; D011247:Pregnancy; D051789:Proto-Oncogene Protein c-fli-1 / Q000032:analysis; D012334:RNA, Neoplasm / Q000032:analysis; D034802:RNA-Binding Protein EWS; D020133:Reverse Transcriptase Polymerase Chain Reaction; D012512:Sarcoma, Ewing / Q000235:genetics / Q000473:pathology* / Q000601:surgery
chemical_list: D014408:Biomarkers, Tumor; D018076:DNA, Complementary; C106031:EWS-FLI fusion protein; D015514:Oncogene Proteins, Fusion; D051789:Proto-Oncogene Protein c-fli-1; D012334:RNA, Neoplasm; D034802:RNA-Binding Protein EWS