[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.
Facon
F
F
Hôpital Nord, Service d'ORL et de chirurgie cervico-faciale, chemin des bourrellys, F-13915 Marseille, France.
Bruzzo
M
M
Figarella-Branger
D
D
Girard
N
N
Chays
A
A
Magnan
J
J
D000230:Adenocarcinoma / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D000293:Adolescent; D003937:Diagnosis, Differential; D004428:Ear Neoplasms / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D004712:Endolymphatic Sac / Q000473:pathology* / Q000601:surgery; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D015837:Vestibular Diseases / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D006623:von Hippel-Lindau Disease / Q000175:diagnosis