Intracranial germ cell tumours: A 21-year review

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• 作者：Marí ; a Cormenzana Carpio^a ; ^{maria.cormenzana@gmail.com} ; Daniel Nehme &Aacute ; lvarez^b ; Carmen Hern&aacute ; ndez Marqú ; es^c ; Antonio Pé ; rez Martí ; nez^d ; Alvaro Lassaletta Atienza^c ; Luis Madero Ló ; pez^c
• 关键词：Germ cell tumour ; Intracranial ; Children ; Pure germinoma ; Non-seminomatous
• 刊名：Anales de Pediatría (English Edition)
• 出版年：2017
• 出版时间：January 2017
• 年：2017
• 卷：86
• 期：1
• 页码：20-27
• 全文大小：666 K
• 卷排序：86

文摘

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.Patients and methodsA 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 to 2014.ResultsA 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.ConclusionsThe 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.