title: Extragonadal malignant germ cell tumors: a clinicopathological and immunohistochemical analysis of 48 cases at a single Chinese institution.
abstract: Primary extragonadal malignant germ cell tumors (EMGCTs) are rare and characterized by the location in the midline of the body, including mediastinum, CNS, retroperitoneum and coccyx. EMGCTs present with different clinical and biologic characteristics in different tumor locations. Accurately diagnosing MEGCTs would be very difficult by performing on HE staining alone, and requires immunohistochemical verification. This study was to investigate the biological feature of EMGCTs and diagnostic value of immunohistochemical markers OCT3/4, CD117, PLAP, AFP, β-HCG and CD30 in EMGCTs. A retrospective study was performed on 48 patients with EMGCTs. EMGCTs were found to occur predominantly in males, especially for mediastinal MGCTs. The tumor locations included mediastinum, CNS and retroperitoneum. The mediastinum and CNS were the most common sites of EMGCTs. Seminoma/germinomas (64.6%) was the most common histological subtypes of EMGCTs. Chest pain, dyspnea, cough and fever were the most common clinical presentations in mediastinal MGCTs. Headache, visual disturbances, endocrine abnormalities, and signs of increased intracranial pressure were common clinical symptoms in CNS MGCTs. Abdominal mass with or without pain, backache and weight loss were common clinical presentations in retroperitoneal MGCTs. PLAP, CD117 and OCT3/4 were highly expressed in seminomas/gernimomas. CD30, EMA and CK AE1/3 staining were positive in embryonal carcinoma. AFP and β-HCG positive staining are characteristic in yolk sac tumors and choriocarcinoma, respectively. Patients with seminomas/germinomas had a better prognosis than those with NS/G-GCTs. Our finding suggests that the accurate diagnosis of EMGCTs is critical not only for predicting the tumor progression but also for patient management. Immunohistochemical markers have become an important tool in the diagnosis and differential diagnosis of EMGCTs.
authors.affiliation: Shanghai Key Laboratory for Assisted Reproduction and Reproductive Genetics, Center for Reproductive Medicine, Ren Ji Hospital, School of Medicine, Shanghai Jiao Tong University 845 Lingshan Road, Shanghai 200135, China.
authors.forename: Yuping
authors.initials: Y
authors.lastname: Gao
authors.affiliation: Department of Neurosurgery, Ren Ji Hospital, School of Medicine, Shanghai Jiao Tong University 160 Pujian Road, Shanghai 200127, China.
authors.forename: Jiyao
authors.initials: J
authors.lastname: Jiang
authors.affiliation: Department of Pathology, Ren Ji Hospital, School of Medicine, Shanghai Jiao Tong University 160 Pujian Road, Shanghai 200127, China.
authors.forename: Qiang
authors.initials: Q
authors.lastname: Liu
mesh_terms: D000293:Adolescent; D000328:Adult; D014408:Biomarkers, Tumor / Q000032:analysis*; D001706:Biopsy; D016543:Central Nervous System Neoplasms / Q000737:chemistry* / Q000150:complications / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D002681:China; D003050:Coccyx / Q000737:chemistry* / Q000473:pathology; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008479:Mediastinal Neoplasms / Q000737:chemistry* / Q000150:complications / Q000473:pathology; D008875:Middle Aged; D009373:Neoplasms, Germ Cell and Embryonal / Q000737:chemistry* / Q000150:complications / Q000473:pathology; D011237:Predictive Value of Tests; D011379:Prognosis; D012186:Retroperitoneal Neoplasms / Q000737:chemistry* / Q000150:complications / Q000473:pathology; D012189:Retrospective Studies; D013125:Spinal Neoplasms / Q000737:chemistry* / Q000150:complications / Q000473:pathology; D055815:Young Adult
chemical_list: D014408:Biomarkers, Tumor
keywords: Extragonada malignant germ cell tumors; diagnosis; immunohistochemistry; prognosis