颅内原发无性细胞瘤伴继发性闭经1例

One case of primary intracranial dysgerminoma with secondary amenorrhea

颅内原发性无性细胞瘤是一种罕见的肿瘤,主要发生在青少年,好发于松果体区和蝶鞍区,其临床表现主要与肿瘤位置、大小相关。大多数生殖细胞瘤对放疗和化疗非常敏感,早期治疗对减少并发症和病死率至关重要。由于其发病率较低,因此目前国内外针对颅内原发无性细胞瘤的诊治还没有一个统一的标准。本文报道一例颅内原发性无形细胞瘤,为20岁女性,因停经9个月入院,行神经内镜下肿瘤切除术,术后病理证实为无性细胞瘤。术后第6周开始“全脑+全脊髓”普通放射治疗,DT 30 Gy×15次(5次/周);随后,开始“全脑加量”普通放射治疗,DT 24 Gy×12次(5次/周)。病人入院诊断垂体功能减退症后,规律口服醋酸泼尼松及左甲状腺素钠替代治疗,放射治疗完成后3个月,MRI显示鞍区未见明显结节、肿块影,但月经尚未恢复。这提示颅内原发性无性细胞瘤病人早期正确诊治对后期激素紊乱的治疗非常重要。

Primary intracranial dysgerminoma(PID)is a rare tumor,mainly occurring in adolescents,which is usually found in the pineal region and sella region.The clinical manifestations of PID are mainly related to their location and size.Most PIDs are very sensitive to radiation and chemotherapy,and early treatment is essential to reduce morbidity and mortality.Due to the low incidence of this disease,there is no unified standard for the diagnosis and treatment of PID.In this paper,we reported a 20-year old female patient with PID who was admitted to our hospital because of secondary menstrual cessation for 9 months.After admission,neuroendoscopic resection was performed.The patient was confirmed as PID by postoperative pathological examination.The patient was treated with"whole brain+whole spinal cord"general radiotherapy starting from the 6th week after surgery,DT 30Gy×15 times(5 times/week);followed by a"whole brain dose"of ordinary radiation therapy,DT 24 Gy x 12 times(5 times/week).The patient was admitted to hospital and diagnosed with hypopituitarism.After diagnosis,she was treated with prednisone acetate and levothyroxine sodium replacement regularly.Three months after the completion of radiotherapy,MRI showed no obvious nodules or masses in the sella area,but her menstruation did not recovered.This suggests that early correct diagnosis and treatment of patients with PID is very important for the treatment of hormone disorders in the later period.