Cormenzana Carpio María, Nehme Álvarez Daniel, Hernández Marqúes Carmen, Pérez Martínez Antonio, Lassaletta Atienza Alvaro, Madero López Luis
Centro de Salud Lucero, Madrid, España.
Servicio de Neuropediatría, Hospital de General de Villalba, Collado Villalba, Madrid, España.
An Pediatr (Barc). 2017 Jan;86(1):20-27. doi: 10.1016/j.anpedi.2016.03.005. Epub 2016 Apr 8.
Intracranial germ cell tumours are rare in children. They are a heterogeneous group of neoplasms that show different clinical manifestations despite having a common origin.
A retrospective analysis was carried out on the epidemiological and histological characteristics, clinical manifestations, and outcomes of 20 patients diagnosed with intracranial germ cell tumours in the Niño Jesús Children's Hospital of Madrid from 1994-2014.
A total of 20 patients were identified: 14 boys and 6 girls. The mean age was 11.1 years (range 2-18 years). Histological confirmation of the diagnosis was obtained in 95% of the patients. Of the 20 patients, 14 were pure germinoma (70%) and 6 non-seminomatous germ cell tumours (30%). The most frequent locations were pineal (45%) and suprasellar (45%). The most frequent clinical symptoms in pineal tumours at diagnosis were headache and vomiting (77.77%), followed by visual disturbances (44.4%). In suprasellar tumours it was polydipsia and polyuria (100%). At diagnosis, 90% of the patients received radiotherapy, and 55% received chemotherapy combined with radiotherapy. There was a relapse in 4 patients (20%), and 3 of them died. Overall survival was 80%; 85.7% for pure germinomas and 60% for non-seminomatous germ cell tumours.
The most common histological subtype was pure germinoma. Germ cell tumours include heterogeneous disease entities that have a variable prognosis. Thus, an accurate diagnosis is vital for patient counselling and treatment planning.
颅内生殖细胞肿瘤在儿童中较为罕见。它们是一组异质性肿瘤，尽管起源相同，但临床表现各异。
对1994年至2014年在马德里尼诺·耶稣儿童医院确诊为颅内生殖细胞肿瘤的20例患者的流行病学、组织学特征、临床表现及预后进行回顾性分析。
共纳入20例患者，其中14例为男性，6例为女性。平均年龄为11.1岁（范围2至18岁）。95%的患者获得了诊断的组织学证实。20例患者中，14例为纯生殖细胞瘤（70%），6例为非精原细胞性生殖细胞肿瘤（30%）。最常见的部位是松果体区（45%）和鞍上区（45%）。松果体区肿瘤诊断时最常见的临床症状是头痛和呕吐（77.77%），其次是视力障碍（44.4%）。鞍上区肿瘤则是烦渴和多尿（100%）。诊断时，90%的患者接受了放疗，55%的患者接受了化疗联合放疗。4例患者（20%）出现复发，其中3例死亡。总生存率为80%；纯生殖细胞瘤为85.7%，非精原细胞性生殖细胞肿瘤为60%。
最常见的组织学亚型是纯生殖细胞瘤。生殖细胞肿瘤包括预后各异的异质性疾病实体。因此，准确的诊断对于患者咨询和治疗计划至关重要。
