title: Ewing's sarcoma/primitive neuroectodermal tumor arising from the adrenal gland: a case report and literature review.
abstract: We report a rare case of Ewing's sarcoma (ES)/primitive neuroectodermal tumor (PNET) arising from the adrenal gland. A 17-year-old Japanese woman presented with left upper abdominal pain and high fever. Computed tomography and magnetic resonance imaging revealed a 15 × 10 cm tumor replacing the adrenal gland. Preoperative diagnosis was an adrenocortical carcinoma. Resection of the tumor was performed. We obtained the final diagnosis of ES/PNET by immunohistochemical molecular study with positive staining for the MIC2 gene product (CD99) and a Ewing sarcoma breakpoint region 1 (EWSR1) gene rearrangement. Local recurrence was observed one month after the surgery. The patient was then treated with systemic chemotherapy and localized radiotherapy.
authors.forename: Takeshi
authors.initials: T
authors.lastname: Sasaki
authors.forename: Takehisa
authors.initials: T
authors.lastname: Onishi
authors.forename: Tadashi
authors.initials: T
authors.lastname: Yabana
authors.forename: Akira
authors.initials: A
authors.lastname: Hoshina
mesh_terms: D000071443:12E7 Antigen; D015746:Abdominal Pain / Q000209:etiology; D000293:Adolescent; D000310:Adrenal Gland Neoplasms / Q000150:complications / Q000175:diagnosis* / Q000235:genetics / Q000628:therapy*; D015703:Antigens, CD / Q000235:genetics*; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D002148:Calmodulin-Binding Proteins / Q000235:genetics*; D015815:Cell Adhesion Molecules / Q000235:genetics*; D017024:Chemotherapy, Adjuvant; D003937:Diagnosis, Differential; D005260:Female; D005334:Fever / Q000209:etiology; D015321:Gene Rearrangement; D006801:Humans; D007150:Immunohistochemistry; D008279:Magnetic Resonance Imaging; D009364:Neoplasm Recurrence, Local / Q000175:diagnosis / Q000628:therapy; D018241:Neuroectodermal Tumors, Primitive, Peripheral / Q000150:complications / Q000175:diagnosis / Q000628:therapy; D034802:RNA-Binding Protein EWS; D016601:RNA-Binding Proteins / Q000235:genetics*; D018714:Radiotherapy, Adjuvant; D012512:Sarcoma, Ewing / Q000150:complications / Q000175:diagnosis* / Q000235:genetics / Q000628:therapy*; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
chemical_list: D000071443:12E7 Antigen; D015703:Antigens, CD; C061828:CD99 protein, human; D002148:Calmodulin-Binding Proteins; D015815:Cell Adhesion Molecules; C500986:EWSR1 protein, human; D034802:RNA-Binding Protein EWS; D016601:RNA-Binding Proteins