title: Molecular Confirmation of Ewing Sarcoma in an 85-Year-Old Woman.
abstract: We report a case of a Ewing sarcoma/primitive neuroectodermal tumor in an 85-year-old woman who presented with an enlarging circumscribed left flank mass. Magnetic resonance imaging revealed a 3 × 5 × 10 cm heterogeneous mass arising from the 10th rib. Computed tomography demonstrated a small nodule in the right middle lobe and bilateral pleural effusions. The patient underwent computed tomography-guided biopsy followed by open biopsy. The tumor cells were characterized by loosely cohesive sheets of tumor cells with uniform nuclei, and scant, granular, eosinophilic cytoplasm with indistinct cell membranes. Frequent mitoses, apoptosis, and necrosis were present. The cells were positive for CD99 with a strong concentric staining pattern. Epithelial, hematopoietic, and neural markers were all negative. Fluorescence in situ hybridization was performed and demonstrated EWSR1 (22q12) gene rearrangement. Sanger sequencing of the reverse transcriptase polymerase chain reaction product from the patient's tumor demonstrated the EWSR1-FLI1 type 1 fusion. Following diagnosis the patient elected to proceed with localized radiation and declined chemotherapy. She developed progressive lung disease and subsequently died of her disease a year after her initial diagnosis. Ewing sarcoma is predominantly a pediatric disease and uncommon in patients older than 40 years of age. To the best of our knowledge, this is the oldest documented case of Ewing sarcoma, diagnosed using modern molecular techniques.
authors.affiliation: University of Utah, Salt Lake City, UT, USA.
authors.forename: Michael J
authors.initials: MJ
authors.lastname: Monument
authors.affiliation: University of Utah, Salt Lake City, UT, USA ARUP Institute for Clinical and Experimental Pathology, Salt Lake City, UT, USA.
authors.forename: Allie H
authors.initials: AH
authors.lastname: Grossmann
authors.affiliation: ARUP Institute for Clinical and Experimental Pathology, Salt Lake City, UT, USA.
authors.forename: Christine C
authors.initials: CC
authors.lastname: Baker
authors.affiliation: University of Utah, Salt Lake City, UT, USA.
authors.forename: R Lor
authors.initials: RL
authors.lastname: Randall
authors.affiliation: University of Utah, Salt Lake City, UT, USA ARUP Institute for Clinical and Experimental Pathology, Salt Lake City, UT, USA.
authors.forename: Ting
authors.initials: T
authors.lastname: Liu
authors.affiliation: University of Utah, Salt Lake City, UT, USA ARUP Institute for Clinical and Experimental Pathology, Salt Lake City, UT, USA dan.albertson@path.utah.edu.
authors.forename: Daniel J
authors.initials: DJ
authors.lastname: Albertson
mesh_terms: D000369:Aged, 80 and over; D014408:Biomarkers, Tumor / Q000235:genetics*; D002148:Calmodulin-Binding Proteins / Q000235:genetics*; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D008175:Lung Neoplasms / Q000175:diagnosis* / Q000235:genetics; D018193:Neoplasms, Complex and Mixed / Q000175:diagnosis* / Q000235:genetics; D018242:Neuroectodermal Tumors, Primitive / Q000175:diagnosis* / Q000235:genetics; D034802:RNA-Binding Protein EWS; D016601:RNA-Binding Proteins / Q000235:genetics*; D012512:Sarcoma, Ewing / Q000175:diagnosis* / Q000235:genetics
chemical_list: D014408:Biomarkers, Tumor; D002148:Calmodulin-Binding Proteins; C500986:EWSR1 protein, human; D034802:RNA-Binding Protein EWS; D016601:RNA-Binding Proteins
keywords: EWS/FLI-1; Ewing sarcoma; RT-PCR; fluorescent in situ hybridization