ABSTRACT
Endolymphatic sac tumors (ELSTs) are rare neuroectodermal neoplasms arising within
the posterior petrous bone. We present a case of a 21-year-old man who presented with
a 6-month history of intermittent morning headaches, fatigue, diplopia, and gait ataxia.
Imaging and surgical pathology identified an adenocarcinoma of the endolymphatic sac
compressing the cerebellum and brain stem. The tumor and multiple metastases were
treated with surgery, radiation, and radiosurgery. Following insertion of a ventriculoperitoneal
shunt for hydrocephalus, he developed symptomatic tension pneumocephalus secondary
to radionecrosis of his petrous bone, requiring flap reconstruction and use of a programmable
shunt valve complemented by hyperbaric oxygen (HBO) therapy. We document here a young
patient with a rare adenocarcinoma of the endolymphatic sac. This case is unique for
its initial presentation without any vestibuloauditory symptoms. Metastatic spread
of ELSTs is also rare. While osteoradionecrosis (ORN) of the temporal bone has been
reported previously in patients with nasopharyngeal carcinoma, this is the first time
it has been presented in the context of an ELST. Tension pneumocephalus is a rare
complication of skull base ORN. This is the first reported use of a programmable shunt
valve and HBO therapy in the management of tension pneumocephalus.
KEYWORDS
Endolymphatic sac tumor - osteoradionecrosis - hyperbaric oxygen therapy - tension
pneumocephalus
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Daipayan Guha
Toronto Western Hospital, 399 Bathurst Street
4W-439, Toronto, ON M5T 2S8
Email: deep.guha@gmail.com