Abstract

AIM OF THE STUDY: To analyze paediatric patients who were diagnosed with intracranial germ cell tumours, to assess their presentation, management details and to understand their overall prognoses in terms of survival. MATERIAL AND METHODS: Of the 22 patients, there were 12 patients with germinoma, 4 patients with immature teratoma and 1 patient with mature teratoma, 1 with embryonal cell carcinoma, and 4 mixed germ cell tumours. The age of presentation ranged from 3 months to 15 years and there was a clear female preponderance. The most common presentation was with raised intracranial pressure and 11of our patients had papilledema at presentation, 1 presented with seizure, 1 with diabetes insipidus and 1 had precocious puberty at the time of presentation. With the exception of 4 infants who were admitted with poor levels of consciousness and who had presumptive diagnoses of teatimes, and where squash preparation provided definite diagnoses at the time of endoscopic third ventriculostomy, serum markers were obtained in all patients, while CSF tumour markers were obtained in only 14 patients (not done in 4 for technical reasons), Alpha-fetoprotein and beta HCG were measured in all cases. Placental alkaline phosphatase (PLAP) was again not available to us. In 12 patients endoscopic third ventriculostomy and biopsy procedures were done. Those with positive tumour markers (3 patients) no histological diagnoses were obtained and underwent direct radiotherapy. RESULTS: Median survival in the teratoma group was 11.6 months. The germinoma and mixed germ cell tumours had better outcome(stable and asymtomatic at 2 years). The child with embryonal carcinoma did not even survive till adjuvant therapy could be started. CONCLUSION: In our scenario we faced some special problems. 1) tumour markers in CSF could not be measured in 4 cases and we had to depend on serum markers in those cases. 2) almost all our patients with severely raised intracranial pressure required immediate CSF diversion procedures and so we could histological samples for squash examination. Limited resources and limited access to high end laboratory facilities provided a challenge for management of these patients.

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