Endocrine Abstracts (2013) 32 P717 | DOI: 10.1530/endoabs.32.P717

Craniopharyngioma -- a diagnosis not to be missed

Joana Menezes Nunes, Elisabete Rodrigues, Sérgio Salvador, António Cerejo, Ricardo Reis, Sérgio Silva, Luís Augusto, Marcos Guimarães, Rui Vaz & Davide Carvalho

Centro Hospitalar São João, Porto, Portugal.

Introduction: Craniopharyngiomas are intracranial tumors that develop from Rathke’s pouch rests of epithelium. They have a bimodal age distribution, with peak incidence at childhood and in the adult/elderly, although in our series we have more patients in a middle peak. Usually they are benign but responsible for significant morbidity, particularly when located near critical structures such as optic chiasm, pituitary gland and hypothalamus, and thus might cause visual, neurological and endocrine deficits.

Case report: A 24-year-old male, asymptomatic until January/2011, when started complaining of progressive visual impairment of the right eye without other neuro-ophthalmological changes neither hormonal dysfunction symptoms. In July/2011, he reported amaurosis of the right eye. The hormonal evaluation revealed only subclinical hypothyroidism, and he was started on levothyroxine therapy (50 μg/day). Pituitary MRI showed a sellar and supra-sellar cystic lesion with carotid involvement. This lesion was not completely excised, because of its adherence to internal carotid. Histology revealed craniopharyngioma adamantinomatous type. The patient was discharged with diagnosis of hypothyroidism and central diabetes insipidus, on levothyroxine supplementation and desmopressin therapy (0.2 mg/day). The post-operative endocrinological revaluation showed panhypopituitarism, requiring adjustment of levothyroxine besides testosterone and prednisone supplementation. The pituitary follow-up MRI showed enlargement of the suprasellar cistern and sella turcica, probably related to sequelae of surgery, tumor-adjacent residues and optic chiasm with scoop bottom and with discreet focus of the contrast on the inferior right location. Clinically the patient maintains visual impairment of the right eye (<1/10), relative afferent pupillary defect, and pallor of optic disc in ophthalmoscopy, without other neurological or endocrine symptoms.

Conclusion: Craniopharyngiomas are rare tumors, often with suprasellar extension. Early diagnosis and treatment require a high diagnostic accuracy when dealing with visual impairment, neurological and hormonal symptoms. They tend to invade locally and relapse after treatment, requiring a long follow-up.

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