Milton Camille K, Pelargos Panayiotis E, Stetson Nathaniel D, Maldonado-Vital Manuel, Fung Kar-Ming A, Dunn Ian F
Department of Neurosurgery, University of Oklahoma Health Sciences Center, Oklahoma City, OK, USA.
INTEGRIS Spine and Neurological Surgery, Oklahoma City, OK, USA.
Rare Tumors. 2021 Jun 20;13:20363613211026501. doi: 10.1177/20363613211026501. eCollection 2021.
The diagnosis and management of mixed intracranial germ cell tumors may be complicated by the diversity present within this tumor category. Mixed germ cell tumors demonstrate variable natural histories which may be altered by the inclusion of even the most minute immature histological components. We report the case of an 18-year-old male who presented with a 3-month history of progressive headache and nausea leading to lethargy. Imaging revealed a giant pineal region mass extending superiorly from the roof of the fourth ventricle into the lateral ventricle, with resultant obstructive hydrocephalus. No spinal lesions were noted. Following gross total resection, the patient experienced marked improvement. Pathologic analysis identified an uncommon tumor composition: mature teratoma (96%), immature teratoma (2%), and germinoma (2%). Guided by the immature component, chemotherapy and radiation were added post-operatively to provide this patient with the greatest chance of long-term survival. Intracranial pathology, including germ cell tumors, should be included in the differential for any young patient presenting with new and progressive headache and nausea. This case emphasizes the benefit of a multimodal approach to mixed germ cell tumors of the pineal region and the importance of careful pathologic review of all submitted material.
混合性颅内生殖细胞肿瘤的诊断和管理可能因该肿瘤类别中存在的多样性而变得复杂。混合性生殖细胞肿瘤表现出可变的自然病程，即使包含最微小的不成熟组织学成分也可能改变其病程。我们报告了一例18岁男性患者，其有3个月进行性头痛和恶心病史，进而发展为嗜睡。影像学检查显示一个巨大的松果体区肿块，从第四脑室顶部向上延伸至侧脑室，导致梗阻性脑积水。未发现脊柱病变。在进行全切除术后，患者病情明显改善。病理分析确定了一种不常见的肿瘤成分：成熟畸胎瘤（96%）、不成熟畸胎瘤（2%）和生殖细胞瘤（2%）。根据不成熟成分，术后增加了化疗和放疗，以使该患者获得最大的长期生存机会。对于任何出现新发和进行性头痛及恶心的年轻患者，颅内病变（包括生殖细胞肿瘤）都应列入鉴别诊断范围。该病例强调了对松果体区混合性生殖细胞肿瘤采用多模式治疗方法的益处，以及对所有送检材料进行仔细病理检查的重要性。
