Adenocarcinoma of the endolymphatic sac in children. A case report

We report a case of an adenocarcinoma of the endolymphatic sac in a 13 years old child, which was fortuitously discovered at the time of radiological investigation of growth delay. Arising from the end of the endolymphatic sac, this rare tumour shares a similar clinic expression with other cerebellopontine angle pathologies. Radiological investigation reveals a moth eaten appearance to the posterior temporal bone and specific bony inclusions. With MRI there is an intense and/or intermediate signal on T1, a hyper signal on T2 and T1 enhancement with injection of gadolinium. Treatment is surgical and local recurrences are not uncommon. After histological confirmation, a search for Von Hippel-Lindau disease is mandatory because this tumour is not exceptional among this subset of patients. Diagnosis and therapeutic criteria will be highlighted by our case and a review of the literature.