Summary
Definition
History and exam
Key diagnostic factors
- visual loss
- macrocephaly and hydrocephalus
- faltering growth
Other diagnostic factors
- symptoms of hypogonadotropic hypogonadism (amenorrhea, erectile dysfunction)
- headache
- symptoms of intracranial hypertension (nausea, vomiting, decreased sensorium, diplopia)
- galactorrhea
- optic atrophy
- polyuria/polydipsia
Risk factors
- age 5 to 14 years
- age 50 to 70 years
Diagnostic tests
1st tests to order
- ophthalmologic evaluation; computerized visual-field examination
- MRI brain (contrast-enhanced)
- CT brain (contrast-enhanced)
- serum prolactin
- serum insulin-like growth factor 1
- growth hormone (GH) stimulation test
- serum luteinizing hormone
- serum follicle-stimulating hormone
- morning serum testosterone
- serum thyroid-stimulating hormone (TSH) and free thyroxine (FT4)
- morning serum cortisol and adrenocorticotropic hormone (ACTH)
- serum electrolytes
- urine and serum osmolality
- plain x-rays for bone age
Tests to consider
- adrenocorticotropic hormone (ACTH) stimulation test
- tissue histology
Treatment algorithm
at initial treatment
post initial treatment
Contributors
Authors
Olabisi Sanusi, MD
Assistant Professor (Skull Base and Open cerebrovascular)
Departments of Neurosurgery, Otolaryngology
Director of Diversity, Equity, and Inclusion
Co-director Skull base fellowship
Oregon Health & Science University
Portland
OR
Declarações
OS has given lectures related to Craniopharyngioma at the Congress of Neurological Surgeons meeting 2023, and will be giving another at the National Association of Skull Base Surgeons Meeting 2025.
Agradecimentos
Dr Olabisi Sanusi would like to gratefully acknowledge Dr Marc C. Chamberlain and Dr Daniel L. Silbergeld, the previous contributors to this topic.
Declarações
MCC and DLS declare that they have no competing interests.
Revisores
Edwin S. Kulubya Jr., MD, MBA
Neurosurgical Research Fellow
UC Davis Medical Center
Sacramento
CA
Declarações
EK declares that he has no competing interests.
Ramez Kirollos, MBChB, FRCS(Ed), FRCS(Eng), MD, FRCS(SN)
Consultant Neurosurgeon
Addenbrooke's Hospital
Cambridge University Hospitals NHS Foundation Trust
Cambridge
UK
Declarações
RK declares that he has no competing interests.
Keyoumars Ashkan, BA, BSc, MB BCh, MRCP, FRCS, FRCPS, FRCS(SN), MD
Consultant Neurosurgeon and Lead of Neuro-Oncology
King's College Hospital
London
UK
Declarações
KA declares that he has no competing interests.
Créditos aos pareceristas
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Declarações
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Referências
Principais artigos
Louis DN, Perry A, Wesseling P, et al. The 2021 WHO classification of tumors of the central nervous system: a summary. Neuro Oncol. 2021 Aug 2;23(8):1231-51.Texto completo Resumo
Karavitaki N, Cudlip S, Adams CB, et al. Craniopharyngiomas. Endocr Rev. 2006 Jun;27(4):371-97. Resumo
Gan HW, Morillon P, Albanese A, et al. National UK guidelines for the management of paediatric craniopharyngioma. Lancet Diabetes Endocrinol. 2023 Sep;11(9):694-706. Resumo
Hoffman LM, Jaimes C, Mankad K, et al. Response assessment in pediatric craniopharyngioma: recommendations from the Response Assessment in Pediatric Neuro-Oncology (RAPNO) Working Group. Neuro Oncol. 2023 Feb 14;25(2):224-33.Texto completo Resumo
Artigos de referência
Uma lista completa das fontes referenciadas neste tópico está disponível para os usuários com acesso total ao BMJ Best Practice.

Diagnósticos diferenciais
- Pituitary adenoma
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Mais Diagnósticos diferenciaisDiretrizes
- Response assessment in pediatric craniopharyngioma: recommendations from the Response Assessment in Pediatric Neuro-Oncology (RAPNO) Working Group
- National UK guidelines for the management of paediatric craniopharyngioma
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