Craniopharyngioma and Cushing disease: case report.
Craniopharyngioma is a common sellar region tumor occurring in children. It usually manifests as endocrinological deficits such as short stature, delayed puberty, and obesity. Patients with craniopharyngioma commonly present with visual deficits and hydrocephalus. The authors present the case of a child who presented with short stature and clinical evidence of Cushing disease (CD) associated with a suprasellar tumor. The patient underwent insertion of an Ommaya reservoir into the tumor's cystic portion. High adrenocorticotropic hormone (ACTH) levels were demonstrated within the cyst's fluid and in the serum. After adequate decompression of the tumor, the patient underwent total resection. The tumor pathology was compatible with an adamantinomatous craniopharyngioma and immunohistochemical studies failed to show staining for ACTH. Panhypopituitarism developed postoperatively in the patient and he received hormone substitution therapy with final adequate height and normal-high weight. The neurosurgical implications of CD along with a possible mechanism for this patient's presentation are discussed in detail on the basis of the pertinent literature.
Caceres
Adrian
A
Neurosurgery Division, Children's Memorial Hospital, Chicago, Illinois 60614, USA.
Reitman
Aaron J
AJ
Tomita
Tadanori
T
D000293:Adolescent; D000324:Adrenocorticotropic Hormone / Q000097:blood; D000328:Adult; D020863:Central Nervous System Cysts / Q000175:diagnosis / Q000209:etiology / Q000601:surgery*; D002648:Child; D003397:Craniopharyngioma / Q000150:complications / Q000175:diagnosis / Q000601:surgery*; D020420:Cyst Fluid / Q000737:chemistry; D003937:Diagnosis, Differential; D004393:Dwarfism, Pituitary / Q000209:etiology; D005500:Follow-Up Studies; D006423:Hemianopsia / Q000209:etiology; D006801:Humans; D007016:Hypophysectomy; D007018:Hypopituitarism / Q000209:etiology; D008279:Magnetic Resonance Imaging; D008297:Male; D009765:Obesity / Q000209:etiology; D047748:Pituitary ACTH Hypersecretion / Q000175:diagnosis / Q000209:etiology / Q000601:surgery*; D010911:Pituitary Neoplasms / Q000150:complications / Q000175:diagnosis / Q000601:surgery*; D011183:Postoperative Complications / Q000209:etiology; D014057:Tomography, X-Ray Computed
D000324:Adrenocorticotropic Hormone