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ODP362 An Unexpected Finding of Adamantinomatous Type Craniopharyngioma in the Late Seventh Decade of Life: a case report

BACKGROUND: Craniopharyngioma is a rare solid, mixed solid, and benign cystic neoplasm derived from remnants of Rathke's pouch from the sellar region and its surroundings parasellar area. It mainly has two major histopathological subtypes, Adamantinomatous and Papillary. The adamantinomatous su...

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Detalles Bibliográficos
Autores principales: Ahmed, Ammar, Biswas, Suman, Tan, Zi, Jahshan, Bishara, Meedani, Hassan E
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Oxford University Press 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9625398/
http://dx.doi.org/10.1210/jendso/bvac150.1070
Descripción
Sumario:BACKGROUND: Craniopharyngioma is a rare solid, mixed solid, and benign cystic neoplasm derived from remnants of Rathke's pouch from the sellar region and its surroundings parasellar area. It mainly has two major histopathological subtypes, Adamantinomatous and Papillary. The adamantinomatous subtype is more common in children, and the papillary subtype is more common in adults. Clinical case: A 66-year-old male with no known endocrinological disorder presented with progressive fatigue, decreased oral intake, lightheadedness, nausea, visual changes, low libido, myalgia, nausea, vomiting, polyurea, headache, visual field defects, and seizure-like activities requiring hospital admission. The endocrinological workup revealed a deranged HPA axis (panhypopituitarism). MRI revealed 3 cm macroadenoma within the pituitary gland. An endonasal transsphenoidal resection of pituitary mass resection with abdominal fat graft was done as definitive treatment along with correction of the multiple endocrinological derangements. The surgical tissue biopsy revealed a rare with adamantinomatous type Craniopharyngioma WHO Grade I. He was discharged on testosterone supplements, hydrocortisone, levothyroxine, and Desmopressin with significant improvement in his symptoms. CONCLUSION: Craniopharyngioma (CP) is a rare benign intracranial mass (incidence 0.5-2/100000) that can present with extensive endocrinological dysfunction. However, due to the lack of patient-level data and reporting of adult-onset craniopharyngiomas, it can be easily missed during the initial presentation. Not considering craniopharyngioma in differential diagnoses may result in delayed diagnosis and adverse outcomes. Although the papillary CP type is more common among adults, occasionally, adamantinomatous type CP may occur in this group of patients. Presentation: No date and time listed