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.
MeSH terms
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12E7 Antigen
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Abdominal Pain / etiology
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Adolescent
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Adrenal Gland Neoplasms / complications
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Adrenal Gland Neoplasms / diagnosis*
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Adrenal Gland Neoplasms / genetics
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Adrenal Gland Neoplasms / therapy*
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Antigens, CD / genetics*
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Antineoplastic Combined Chemotherapy Protocols / therapeutic use
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Calmodulin-Binding Proteins / genetics*
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Cell Adhesion Molecules / genetics*
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Chemotherapy, Adjuvant
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Diagnosis, Differential
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Female
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Fever / etiology
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Gene Rearrangement*
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Humans
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Immunohistochemistry
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Magnetic Resonance Imaging
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Neoplasm Recurrence, Local / diagnosis
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Neoplasm Recurrence, Local / therapy
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Neuroectodermal Tumors, Primitive, Peripheral / complications
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Neuroectodermal Tumors, Primitive, Peripheral / diagnosis
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Neuroectodermal Tumors, Primitive, Peripheral / therapy
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RNA-Binding Protein EWS
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RNA-Binding Proteins / genetics*
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Radiotherapy, Adjuvant
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Sarcoma, Ewing / complications
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Sarcoma, Ewing / diagnosis*
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Sarcoma, Ewing / genetics
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Sarcoma, Ewing / therapy*
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Tomography, X-Ray Computed
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Treatment Outcome
Substances
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12E7 Antigen
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Antigens, CD
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CD99 protein, human
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Calmodulin-Binding Proteins
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Cell Adhesion Molecules
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EWSR1 protein, human
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RNA-Binding Protein EWS
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RNA-Binding Proteins