Isolated Maxillary Extracranial Metastasis of a Suprasellar Germinoma.

Jun Hyun Lee, Hyeon Jin Park, Sang-Hoon Shin, Joo-Young Kim, So-Hyun Ji, Nayoung Han, Ji-Man Kang
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Abstract

Intracranial germinomas are exquisitely radiosensitive, and a tissue diagnosis obtained by minimal biopsy followed by radiotherapy (with or without chemotherapy) provides excellent long-term control; aggressive resection adds morbidity without oncological benefit. We report a 20-year-old man whose sellar and suprasellar mass was preoperatively interpreted as a pituitary adenoma and was treated by aggressive endoscopic endonasal transsphenoidal resection after an intraoperative frozen section suggested adenoma; final histology demonstrated germinoma. The postoperative course was complicated by a persistent skull-base cerebrospinal fluid (CSF) leak, methicillin-resistant Staphylococcus aureus meningitis/ventriculitis, and CSF dissemination documented by repeatedly positive CSF cytology and elevated CSF β-human chorionic gonadotropin. The initial skull-base reconstruction failed, and the resulting persistent CSF rhinorrhea required revision dural repair on postoperative day (POD) 29; proton craniospinal irradiation was delivered from POD 52. The patient achieved remission but, approximately 21 months after completing radiotherapy, developed a destructive germinoma lesion in the midline maxilla, with no recurrence at the primary site and no leptomeningeal disease at that time. In light of the anterior maxillary location and the relatively long interval after radiotherapy, we interpret the lesion as an isolated extracranial metastasis rather than a proven operative-tract event. The principal lesson is that accurate initial diagnosis and approach selection-measurement of tumor markers and minimal or transventricular biopsy in preference to resection-remain essential in young patients with sellar/suprasellar masses in whom germinoma is a diagnostic possibility.

鞍上生殖细胞瘤的分离性上颌颅外转移。
颅内生殖细胞瘤对放射非常敏感,通过最小的活检和放疗(有或没有化疗)获得的组织诊断提供了良好的长期控制;积极切除增加发病率,但无肿瘤益处。我们报告了一名20岁的男性,其鞍上肿块术前被解释为垂体腺瘤,术中冰冻切片提示腺瘤后,通过积极的鼻内经蝶窦切除术治疗;最终组织学显示为生殖细胞瘤。术后并发颅底脑脊液(CSF)持续泄漏,耐甲氧西林金黄色葡萄球菌脑膜炎/脑室炎,脑脊液播散,脑脊液细胞学反复阳性,脑脊液β-人绒毛膜促性腺激素升高。最初的颅底重建失败,导致持续的脑脊液鼻漏需要在术后第29天(POD)进行再版硬脑膜修复;质子颅脊髓照射由POD 52传送。患者获得缓解,但在完成放疗约21个月后,在上颌中线出现破坏性生殖细胞瘤病变,原发部位未复发,当时无轻脑膜疾病。鉴于上颌前位置和放疗后相对较长的间隔,我们将病变解释为孤立的颅外转移,而不是已证实的手术道事件。主要的教训是,准确的初始诊断和方法选择——肿瘤标志物的测量和最小或经脑室活检优先于切除——对于有鞍/鞍上肿块的年轻患者仍然是必要的,他们有可能诊断为生殖细胞瘤。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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