Isolated, giant cerebellopontine angle craniopharyngioma in a patient with Gardner syndrome: case report.
OBJECTIVE
We report the case of a 29-year-old man with Gardner syndrome and an isolated, giant cerebellopontine angle craniopharyngioma. Our description of this patient is only the second case report of a craniopharyngioma arising primarily in the cerebellopontine angle.


METHODS
The patient presented with a 1-year history of progressive neurological impairment and headache. On the basis of the patient's history of multiple dermal fibromas, a cranial osteoma, familial adenomatous polyposis (FAP), a total abdominal colectomy, and an adenoma of the ampulla of Vater, we diagnosed the patients condition as Gardner syndrome.


METHODS
Magnetic resonance imaging showed a large cerebellopontine angle tumor, which was removed through a suboccipital retromastoid craniotomy. The pathological features were those of an adamantinomatous craniopharyngioma. The patient has done well postoperatively and has no new neurological deficits. A careful retrospective review of the preoperative imaging shows that this tumor was located exclusively in the posterior fossa and was not an extension of a sellar, suprasellar, or clival craniopharyngioma.


CONCLUSIONS
We present the second reported case of FAP and craniopharyngioma. There is no known genetic link between FAP and craniopharyngioma. Now that the patient has manifested a primary tumor of the central nervous system with FAP, it is unclear whether he should be classified as having Turcot syndrome. For this patient, we recommended vigilant follow-up imaging and forgoing external beam radiotherapy unless there is a documented recurrence of his craniopharyngioma.
Department of Neurologic Surgery, Mayo Clinic, Rochester, Minnesota 55905, USA. Link.Michael@mayo.edu
Michael J
MJ
Link
Colin L W
CL
Driscoll
Caterina
C
Giannini
D000328:Adult; D002528:Cerebellar Neoplasms / Q000175:diagnosis / Q000473:pathology / Q000601:surgery*; D002530:Cerebellopontine Angle / Q000473:pathology / Q000601:surgery*; D003082:Colectomy; D003397:Craniopharyngioma / Q000175:diagnosis / Q000473:pathology / Q000601:surgery*; D003937:Diagnosis, Differential; D005500:Follow-Up Studies; D005736:Gardner Syndrome / Q000175:diagnosis / Q000473:pathology / Q000601:surgery*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D009460:Neurologic Examination; D010911:Pituitary Neoplasms / Q000175:diagnosis / Q000473:pathology / Q000601:surgery*; D011183:Postoperative Complications / Q000175:diagnosis / Q000473:pathology / Q000601:surgery; D012086:Reoperation