title: An unusual association of malignant gastrointestinal neuroectodermal tumor (clear cell sarcoma-like) and Ewing sarcoma.
abstract: Very recently a new designation of "Malignant Neuroectodermal Gastrointestinal Tumor" has been proposed for an aggressive form of neuroectodermal tumor with features similar to that of Clear Cell Sarcoma of Soft Tissue, however without a melanocytic differentiation. Also known as "clear cell sarcoma-like tumors of the gastrointestinal tract", these tumors show some features strongly suggesting an origin from a gastrointestinal neuroectodermal precursor cell unable to differentiate along the melanocytic lineage. They occur mainly in young and middle-aged adults, and have a poor prognosis with a high rate of liver and lymphnode metastases. Histologically they are composed of epithelioid or oval-to spindle cells with a sheet-like or nested pattern of growth, strongly positive for neural markers (S-100, SOX10, and vimentin) and negative for the melanocytic ones. EWSR1 gene rearrangements including EWSR1-ATF1 or EWSR1-CREB1 GENE fusions are typically assessed in these tumors. Here we report a case of malignant neuroectodermal gastrointestinal tumor which immunophenotypically unusually expressed FLI-1, occurring in a 29-year-old man with a previous medical history of Ewing sarcoma. We finally suggest that this case might be a further evidence of a link between these two entities.
authors.affiliation: Dip. Scienze Biomorfologiche e Chirurgiche, Università Federico II Napoli, Italy. Electronic address: insabato@unina.it.
authors.forename: Luigi
authors.initials: L
authors.lastname: Insabato
authors.affiliation: Dip. Scienze Biomorfologiche e Chirurgiche, Università Federico II Napoli, Italy.
authors.forename: Elia
authors.initials: E
authors.lastname: Guadagno
authors.affiliation: Dip. Scienze Biomorfologiche e Chirurgiche, Università Federico II Napoli, Italy.
authors.forename: Valentina
authors.initials: V
authors.lastname: Natella
authors.affiliation: Dip. Scienze Biomorfologiche e Chirurgiche, Università Federico II Napoli, Italy.
authors.forename: Anna
authors.initials: A
authors.lastname: Somma
authors.affiliation: Department of Pathology, University Hospital of Basel, Switzerland.
authors.forename: Michel
authors.initials: M
authors.lastname: Bihl
authors.affiliation: SSD Oncologia medica Sarcomi dei Tessuti molli e delle Ossa, Istituto Tumori, Fondazione G. Pascale, Naples, Italy.
authors.forename: Antonio
authors.initials: A
authors.lastname: Pizzolorusso
authors.affiliation: Dip. Scienze Biomorfologiche e Chirurgiche, Università Federico II Napoli, Italy.
authors.forename: Pier Paolo
authors.initials: PP
authors.lastname: Mainenti
authors.affiliation: SSD Oncologia medica Sarcomi dei Tessuti molli e delle Ossa, Istituto Tumori, Fondazione G. Pascale, Naples, Italy.
authors.forename: Gaetano
authors.initials: G
authors.lastname: Apice
authors.affiliation: Department of Pathology, University Hospital of Basel, Switzerland.
authors.forename: Luigi
authors.initials: L
authors.lastname: Tornillo
mesh_terms: D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D014408:Biomarkers, Tumor / Q000032:analysis; D001706:Biopsy; D001859:Bone Neoplasms / Q000737:chemistry / Q000473:pathology*; D017024:Chemotherapy, Adjuvant; D005743:Gastrectomy; D006801:Humans; D007150:Immunohistochemistry; D017404:In Situ Hybridization, Fluorescence; D008279:Magnetic Resonance Imaging; D008297:Male; D016609:Neoplasms, Second Primary / Q000737:chemistry / Q000235:genetics / Q000473:pathology* / Q000628:therapy; D018242:Neuroectodermal Tumors, Primitive / Q000737:chemistry / Q000235:genetics / Q000473:pathology* / Q000628:therapy; D016133:Polymerase Chain Reaction; D018227:Sarcoma, Clear Cell / Q000737:chemistry / Q000235:genetics / Q000473:pathology* / Q000628:therapy; D012512:Sarcoma, Ewing / Q000737:chemistry / Q000473:pathology*; D013274:Stomach Neoplasms / Q000737:chemistry / Q000235:genetics / Q000473:pathology* / Q000628:therapy; D016896:Treatment Outcome
chemical_list: D014408:Biomarkers, Tumor
keywords: Clear cell sarcoma-like gastric tumor