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Craniopharyngioma

Donna Marie McMahon, FAAP, DO Reviewed 05/2023
 


BASICS

A rare neoplasm of the CNS (1

DESCRIPTION

  • Benign epithelioid tumor, usually suprasellar, partially calcified, solid, or mixed solid–cystic that arises from remnants of the craniopharyngeal duc...

DIAGNOSIS

May take years of symptoms before diagnosis is made 

HISTORY

  • Eye: decreased visual acuity, visual field defects, bitemporal hemianopia, abnormal pupillary response

  • GI: anorexia, nausea, vomitin...

TREATMENT

Gross-total resection, subtotal resection, endoscopic endonasal surgery, cyst drainage followed by radiation (1

GENERAL MEASURES

  • Avoidance of driving, operating machinery, playing sports, o...

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

Patient Monitoring

  • Postoperative MRI/CT scans as recommended by neurosurgeon

  • Formal visual field/ophthalmologic evaluation and follow up

  • Monitor endocrine function

  • Co...

REFERENCES

1
Madsen PJ, Buch VP, Douglas JE, et al. Endoscopic endonasal resection versus open surgery for pediatric craniopharyngioma: comparison of outcomes and complications. J Neurosurg Pediatr. 20...

ADDITIONAL READING

Nuiijts MA, Veldhuis N, Stegeman I, et al. Visual functions in children with craniopharyngioma at diagnosis: a systematic review. PLoS ONE 2020;15(10):e0240016....

CODES

ICD10

D35.3 Benign neoplasm of craniopharyngeal duct 

SNOMED

  • 189179009 Craniopharyngioma (disorder)

  • 134216001 Adamantinous craniopharyngioma

  • 128782006 Craniopharyngioma, papillary

CLINICAL PEARLS

  • Although considered a “benign” tumor, a craniopharyngioma is associated with significant morbidity and mortality. Management of these tumors requires a multidisciplinary approach.

  • Hormo...

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