title: Sclerosing epithelioid fibrosarcoma of the thigh: report of two cases with synchronous bone metastases.
abstract: We report two cases of sclerosing epithelioid fibrosarcoma occurring in the deep soft tissue of the thigh, confirmed by molecular analysis and associated with bone metastases in the lumbar vertebrae and the iliac wing at the time of diagnosis. Synchronous bone metastases of sclerosing epithelioid fibrosarcoma are extremely difficult to diagnose because clinical and radiological features are not specific. In addition, the range of differential diagnoses is very wide, including metastatic carcinoma and osteosarcoma. At present, all but three published cases of sclerosing epithelioid fibrosarcoma with bone metastases showed bone metastases during follow-up. We confirm in our two cases that the distinct pattern of immunohistochemical staining for MUC4, associated with the absence of staining for both SATB2, a marker of osteoblastic differentiation, and pan-cytokeratin, allows differentiating between sclerosing epithelioid fibrosarcoma and metastatic carcinoma or osteosarcoma.
authors.affiliation: Department of Pathology, Rizzoli Institute, Via di Barbiano 1/10, 40136, Bologna, Italy, alberto.righi@ior.it.
authors.forename: A
authors.initials: A
authors.lastname: Righi
authors.forename: M
authors.initials: M
authors.lastname: Gambarotti
authors.forename: M
authors.initials: M
authors.lastname: Manfrini
authors.forename: S
authors.initials: S
authors.lastname: Benini
authors.forename: G
authors.initials: G
authors.lastname: Gamberi
authors.forename: S
authors.initials: S
authors.lastname: Cocchi
authors.forename: R
authors.initials: R
authors.lastname: Casadei
authors.forename: P
authors.initials: P
authors.lastname: Picci
authors.forename: D
authors.initials: D
authors.lastname: Vanel
authors.forename: A P
authors.initials: AP
authors.lastname: Dei Tos
mesh_terms: D000328:Adult; D014408:Biomarkers, Tumor / Q000032:analysis; D001859:Bone Neoplasms / Q000556:secondary*; D005260:Female; D005354:Fibrosarcoma / Q000556:secondary*; D006801:Humans; D007150:Immunohistochemistry; D017404:In Situ Hybridization, Fluorescence; D036961:Matrix Attachment Region Binding Proteins / Q000032:analysis / Q000096:biosynthesis; D055264:Mucin-4 / Q000032:analysis / Q000096:biosynthesis; D020133:Reverse Transcriptase Polymerase Chain Reaction; D012598:Sclerosis / Q000473:pathology; D012983:Soft Tissue Neoplasms / Q000473:pathology*; D013848:Thigh; D014157:Transcription Factors / Q000032:analysis / Q000096:biosynthesis
chemical_list: D014408:Biomarkers, Tumor; C524658:MUC4 protein, human; D036961:Matrix Attachment Region Binding Proteins; D055264:Mucin-4; C521297:SATB2 protein, human; D014157:Transcription Factors