Kieu Hung Dinh, Van Dang Duong, Le Tam Duc
Department of Neurosurgery and Spine Surgery, Hanoi Medical University Hospital, Hanoi, Vietnam.
Department of Pathology, Hanoi Medical University Hospital, Hanoi, Vietnam.
Surg Neurol Int. 2021 Nov 2;12:549. doi: 10.25259/SNI_663_2021. eCollection 2021.
The primary cerebellar germinoma is exceptional and difficult to diagnose preoperatively. Its recurrence at the middle cranial fossa after complete response to radiotherapy is unique and associated with a poor prognosis. This article aims to report the successful management of the late recurrence of primary cerebellar germinoma at an unusual site after 4 years of complete response to radiotherapy.
A 22-year-old male was admitted to our hospital with complaints of severe headache and loss of balance. Brain magnetic resonance imaging (MRI) showed a triventricular hydrocephalus due to a 45x50mm cerebellar mass. Our preliminary diagnosis was medulloblastoma. First, we placed a ventriculoperitoneal shunt with the medium-pressure valve, and then we used midline suboccipital craniotomy to remove the tumor completely. The histopathology was germinoma. The patient received 24 Gy craniospinal irradiation (CSI) with a 16 Gy boost to the primary site and had an MRI follow-up every six months. After a 4-year follow-up, he complained of recurrent severe headaches. The brain MRI illustrated a 62 × 61 mm temporal mass. We extirpated this tumor, and histopathology again revealed germinoma. After that, the patient received induction radiotherapy. The 1-year postoperative MRI showed no tumor remnant. At the time of writing, the patient had no headache and no neurological deficits.
Regular follow-ups with routine neuroaxis MRI should be recommended to detect recurrence early for all patients with intracranial germinomas. Surgical resection, if possible, and subsequent CSI are the most effective salvage treatment for recurrent germinoma.
原发性小脑生殖细胞瘤极为罕见，术前诊断困难。其在放疗完全缓解后于中颅窝复发的情况独特，且预后不良。本文旨在报告一例原发性小脑生殖细胞瘤在放疗完全缓解4年后于不寻常部位出现晚期复发的成功治疗案例。
一名22岁男性因严重头痛和平衡失调入院。脑部磁共振成像（MRI）显示，一个45×50mm的小脑肿块导致三脑室脑积水。我们初步诊断为髓母细胞瘤。首先，我们置入了带中压阀门的脑室腹腔分流管，然后采用枕下中线开颅术完全切除肿瘤。组织病理学检查为生殖细胞瘤。患者接受了24Gy的全脑全脊髓照射（CSI），原发部位追加16Gy照射，并每六个月进行一次MRI随访。经过4年随访，他再次出现严重头痛。脑部MRI显示颞叶有一个62×61mm的肿块。我们切除了该肿瘤，组织病理学检查再次显示为生殖细胞瘤。此后，患者接受了诱导放疗。术后1年的MRI显示无肿瘤残留。撰写本文时，患者无头痛症状，也无神经功能缺损。
对于所有颅内生殖细胞瘤患者，应建议定期进行常规神经轴MRI随访以早期发现复发。如有可能，手术切除及后续的CSI是复发性生殖细胞瘤最有效的挽救治疗方法。
