後腹腔性腺外卵黃囊瘤罕見病例討論併文獻探討
吳翊豪 陳文祥 林政鋒 劉冠麟 林承家 吳俊德 陳鴻毅
基隆長庚紀念醫院 外科部 泌尿科
Retroperitoneal extragonadal yolk sac tumor with bone and lung metastasis: a rare case report and literature review
Yi-Hao Wu, Wen-hsiang Chen, Cheng-Feng Lin, KL Liu, Cheng-Chia Lin, Chun-Te Wu, Hong-Yi Chen
Department of urology, Chang Gung Memorial Hospital, Keelung, Taiwan
Background:
Extragonadal germ cell tumors (EGGCTs) are a heterogeneous group of tumors of neoplastic germ cells arising from extragonadal anatomical locations, without evidence of gonadal primary tumors. They represent only 2– 5% of adult germ cell malignancies. Their histology is identical to germ cell tumors (GCTs) derived from primary gonadal sites; however, their biology, especially primary mediastinal non- seminomatous GCT (PMNSGCT), is substantially different. EGGCTs may localize in almost every structure along the midline of the body, from the brain to the coccyx. However, the most common anatomical locations are represented by the mediastinum, retroperitoneum, and brain, while the pineal gland and sacrococcygeal area are fewer common locations. We reported one young male who suffered from right low limb weakness, where epidural retroperitoneal yolk sac tumor compression.
Case summary: A 30 year-old male without underlying disease suffered from right low limb weakness and incontinence. MRI revealed right paraspinal mass with bone involvement. The biopsy revealed yolk sac tumor. Abdominal CT showed retroperitoneal mass involve right psoas muscle, retrocaval and aortacaval region complicated with bilateral lung metastasis. After chemotherapy, the retroperitoneal mass subsided and lung lesion improved.
Discussion:
EGGCTs represent a rare group of neoplasms, occurring more commonly in the mediastinum and retroperitoneum. The EGGCTs are histologically constituted by the same components observed in the gonadal counterparts, but they are characterized by different biologic behaviors, clinical features and poorer prognoses, mainly when constituted by nonse- monomatous components.