Abstract

Introduction: Craniopharyngiomas are benign epithelial intracranial tumour with local aggressive and infiltrative propensity to adjacent structures. Though clinical manifestations are non-specific, but it classically present with features of endocrine dysfunctions, intracranial hypertension and visual deterioration. Malignant transformation of once recognized benign tumour is rare with only few cases reported in literature. However, no case of multiple disseminated malignant transformations has been  reported before. Case presentation: A 17-year-old boy whose initial diagnosis of adamantinimatous craniopharygioma (benign) was made at age two (2) after which he had both cerebrospinal fluid diversion with shunt and stereotactic guided cyst aspiration was presented. After a 15 year of regular follow-up, he presented once with recurrence (huge compressive cystic mass) and was re-operated with histology still remaining benign adamantinomatous craniopharyngioma and
had stereotactic radiosurgery for the peripheral lesions. However, he came again 8 week later with altered level of consciousness and repeated imaging done revealed multiple metastatic disseminated supratentorial and infratentorial  tumours which was confirmed to be malignant transformation of craniopharyngioma which is unlikely to be treatment-induced (radiosurgery) due to latent period of 8 week between radiosurgery and confirmation of  malignancy. Conclusion: Craniopharyngioma, a tumour once thought of as benign tumour only with no malignant potential even after treatment is curiously been accepted gradually to have malignant potential. It is important for all those actively involved in the management of craniopharyngioma to be aware that during course of management that the potential for malignant transformation do exist and exact cause still remain unclear