title: [An unusual adrenal tumor: Ewing tumor].
abstract: We report the case of a voluminous tumor of the adrenal diagnosed in a young pregnant woman at 26(th) week of amenorrhea. Morphologically, a soft white tumor with haemorragic areas was observed, made of sheets of monomorphous, medium sized, spindle-shaped to polygonal, with high mitotic activity. Tumorous cells expressed cytokeratins AE1/AE3, EMA, and CD99 (expression of vimentin is not relevant). Contemplated diagnoses included poorly differentiated synovialosarcoma, sarcomatoid carcinoma and Ewing tumor. Thanks to molecular biology, showing the specific transcript of Ewing/peripheral primitive neuroectodermal tumor (pPNET) EWS/FLI1, the diagnosis of this atypical tumor in an unusual location was performed. Indeed, 75% of Ewing tumors involve bones (especially, the diaphysis of long bones) and 20 to 25% soft tissues. Primitive visceral involvement is rare; less than 10 cases of adrenal involvement have been reported. The hypothesis that Ewing cell's origin is a mesenchymal stem cell, which may derive from neural crest cell, could explain the uncommon adrenal involvement. Diagnosis of Ewing tumor is based on pathologic and molecular findings, especially in atypical cases.
authors.affiliation: Service d'anatomie et cytologie pathologiques, hôpital Cochin, Paris, France.
authors.forename: Julie
authors.initials: J
authors.lastname: Gonin
authors.forename: Frédérique
authors.initials: F
authors.lastname: Larousserie
authors.forename: Bertrand
authors.initials: B
authors.lastname: Dousset
authors.forename: Julien
authors.initials: J
authors.lastname: Rousseau
authors.forename: Olivier
authors.initials: O
authors.lastname: Delattre
authors.forename: Charlotte
authors.initials: C
authors.lastname: Waintrop
authors.forename: Vassili
authors.initials: V
authors.lastname: Tsatsaris
authors.forename: Jean-Yves
authors.initials: JY
authors.lastname: Pierga
authors.forename: Marie-Cécile
authors.initials: MC
authors.lastname: Vacher-Lavenu
authors.forename: Frédérique
authors.initials: F
authors.lastname: Tissier
mesh_terms: D000310:Adrenal Gland Neoplasms / Q000737:chemistry / Q000150:complications / Q000175:diagnosis / Q000473:pathology* / Q000628:therapy; D000315:Adrenalectomy; D000328:Adult; D014408:Biomarkers, Tumor / Q000032:analysis; D002277:Carcinoma / Q000175:diagnosis; D002471:Cell Transformation, Neoplastic; D002585:Cesarean Section; D017024:Chemotherapy, Adjuvant; D003131:Combined Modality Therapy; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D008197:Lymph Node Excision; D059630:Mesenchymal Stem Cells / Q000473:pathology; D018242:Neuroectodermal Tumors, Primitive / Q000737:chemistry / Q000150:complications / Q000175:diagnosis / Q000473:pathology* / Q000628:therapy; D015514:Oncogene Proteins, Fusion / Q000032:analysis; D009928:Organ Specificity; D010673:Pheochromocytoma / Q000175:diagnosis; D011225:Pre-Eclampsia; D011247:Pregnancy; D011252:Pregnancy Complications, Neoplastic / Q000175:diagnosis / Q000473:pathology* / Q000628:therapy; D051789:Proto-Oncogene Protein c-fli-1 / Q000032:analysis; D034802:RNA-Binding Protein EWS / Q000032:analysis; D018714:Radiotherapy, Adjuvant; D012512:Sarcoma, Ewing / Q000737:chemistry / Q000150:complications / Q000175:diagnosis / Q000473:pathology* / Q000628:therapy; D013584:Sarcoma, Synovial / Q000175:diagnosis
chemical_list: D014408:Biomarkers, Tumor; C106031:EWS-FLI fusion protein; D015514:Oncogene Proteins, Fusion; D051789:Proto-Oncogene Protein c-fli-1; D034802:RNA-Binding Protein EWS