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craniopharyngioma

Etiology: - slow growing, encapsulated squamous cell tumor derived from remnants of Rathke's pouch (craniopharyngeal duct). Epidemiology: 1) most common tumor in the pituitary region in childhood 2) may occur at any age Pathology: 1) 2/3 of the tumors are suprasellar 2) 1/3 originate or extend into the sella turcica 3) most tumors are cystic - brown cholesterol containing fluid ('machinery oil') 4) some are solid or mixed 5) propensity to calcify 6) presumably derived from remnants of embryonic pituitary Microscopic Pathology: 1) adamantinomatous epithelium 2) loose arrangement of squamous cells 3) nodules of keratin 4) fibrosis 5) cholesterol deposits Clinical manifestations: 1) obstructive hydrocephalus 2) hypothalamic syndrome (injury) a) diabetes insipidus b) hyperprolactinemia c) increased appetite, thirst, weight gain, morbid obesity [5] d) temperature dysregulation e) sleep disorder 3) optic chiasmal defects 4) panhypopituitarism Laboratory: - serum sodium: hypontremia (postoperative SIADH) - long-term central diabetes insipidus [5] - also see panhypopituitarism Radiology: 1) plain radiographs: - calcification in or around the sella turcica 2) computed tomography (CT) a) solid, cystic or mixed solid-cystic mass b) calcification 3) magnetic resonance imaging (MRI) a) solid, cystic or mixed solid-cystic mass [2] b) low attenuation values * image [4] Differential diagnosis: - non-functioning pituitary adenoma (macroadenoma) - generally not cystic, located in sella turcica not suprasellar region [5] Management: 1) surgical excision is feasible only for small tumors 2) surgical decompression of larger tumors 3) ventriculoperitoneal shunt for obstructive hydrocephalus 4) post-operative radiation therapy 5) management of endocrine dysfunction

General

brain neoplasm (intracranial neoplasm) neuroectodermal neoplasm

References

  1. Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 197
  2. Medical Knowledge Self Assessment Program (MKSAP) 16, American College of Physicians, Philadelphia 2012
  3. Bobustuc GC and Ramachandran TS eMedicine: Craniopharyngioma http://emedicine.medscape.com/article/1157758-overview
  4. Khanna O, Ghobrial GM, Farrell CJ 10 Brain Lesions to Recognize (MRI images) Medscape. October 25, 2021 https://reference.medscape.com/slideshow/brain-lesions-6013313
  5. NEJM Knowledge+ Endocrinology

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