title: Multiple Ewing Sarcoma/Primitive Neuroectodermal Tumors in the Mediastinum: A Case Report and Literature Review.
abstract: Ewing sarcoma/primitive neuroectodermal tumors (ES/PNET) are high-grade malignant neoplasms. These malignancies present very rare tumors of thoracopulmonary area and even rarer in the mediastinum. In our knowledge, ES/PNET presented with multiple mediastinal masses has not been reported previously. We experienced a case of a 42-year-old man presented with gradual onset of left-side pleuritic chest pain. A contrast-enhanced chest computed tomography (CT) scan showed separate 2 large heterogeneously enhancing masses in each anterior and middle mediastinum of the left hemithorax. Positron emission tomography-computed tomography (PET-CT) scan revealed high fluorodeoxyglucose (FDG) uptake in the mediastinal masses. After surgical excision for the mediastinal masses, both of the masses were diagnosed as the ES/PNET group of tumors on the histopathologic examination. The patient refused postoperative adjuvant chemotherapy and came back with local tumor recurrence and distant metastasis on 4-month follow-up after surgical resection. We report this uncommon form of ES/PNET. We are to raise awareness that this rare malignancy should be considered as a differential diagnosis of the malignant mediastinal tumors and which can be manifested as multiple masses in a patient. Understanding this rare entity of extra-skeletal ES/PNET and characteristic imaging findings can help radiologists and clinicians to approach proper diagnosis and better management for this highly malignant tumor.
authors.affiliation: From the Department of Radiology (SHB, JHH, BDN); Department of Cardiothoracic Surgery (HJK); Department of Respiratory and Allergy Medicine (K-UK); and Department of Pathology (DWK, IHC), Soonchunhyang University Seoul Hospital, Seoul, Korea.
authors.forename: Sung Hwan
authors.initials: SH
authors.lastname: Bae
authors.forename: Jung Hwa
authors.initials: JH
authors.lastname: Hwang
authors.forename: Bo
authors.initials: B
authors.lastname: Da Nam
authors.forename: Hyun Jo
authors.initials: HJ
authors.lastname: Kim
authors.forename: Ki-Up
authors.initials: KU
authors.lastname: Kim
authors.forename: Dong Won
authors.initials: DW
authors.lastname: Kim
authors.forename: In Ho
authors.initials: IH
authors.lastname: Choi
mesh_terms: D000328:Adult; D006801:Humans; D008297:Male; D008479:Mediastinal Neoplasms / Q000175:diagnosis*; D018241:Neuroectodermal Tumors, Primitive, Peripheral / Q000175:diagnosis*; D012512:Sarcoma, Ewing / Q000175:diagnosis*