eMedicine Specialties > Pediatrics: General Medicine > Oncology

Craniopharyngioma: Follow-up

Author: Joseph L Lasky III, MD, Clinical Assistant Professor, Department of Pediatrics, Division of Hematology and Oncology, Mattel Children's Hospital UCLA
Coauthor(s): Kathleen M Sakamoto, MD, PhD, Professor and Chief, Division of Hematology-Oncology, Vice-Chair of Research, Mattel Children's Hospital at UCLA; Department of Pathology and Laboratory Medicine, Jonsson Comprehensive Cancer Center, David Geffen School of Medicine at UCLA and California Nanosystems Institute and Molecular Biology, UCLA; Jerry L Barker, Jr, MD, Staff Physician, Clinical Associate Professor of Radiation Oncology, Department of Radiation Oncology, University of Texas Southwestern Moncrief Cancer Center
Contributor Information and Disclosures

Updated: Dec 9, 2008

Follow-up

Further Outpatient Care

  • Endocrine: Close monitoring of growth, pubertal development, and any known hormone abnormalities is essential in patients with craniopharyngioma.
  • Oncologic
    • Routine neuroimaging is the mainstay of follow-up assessment.
    • The expert consensus recommends brain MRI every 3 months for 1 year, followed by MRI every 6 months for 1 year, and then annual MRI until year 5. If they received radiation therapy, an MRI annually or every other year for life is recommended due to the potential risk for second malignancies.
  • Ophthalmologic
    • Routine ophthalmology evaluations are recommended.
    • In particular, new visual field deficits may hallmark disease progression.
  • Neuropsychiatric
    • Periodic formal assessment can be useful when monitoring the long-term sequelae of surgery or radiotherapy.
    • In formal assessment, include comparison with preoperative and postoperative evaluations.

Inpatient & Outpatient Medications

  • Long-term hormone supplementation is the primary medical treatment associated with childhood craniopharyngiomas and includes the following:
    • Intranasal vasopressin
    • Corticosteroids
    • Thyroid hormones
    • Growth hormones
    • Sex hormones

Complications

  • Endocrine morbidity
    • The need for hormone replacement is common (approximately 80%) following therapy for craniopharyngiomas.
    • Diabetes insipidus is one of the least common (6-10%), but most serious, endocrinologic disturbances. Children with diabetes insipidus can become dehydrated during episodes of illness, and their summer activity must frequently be curtailed; in addition, vasopressin administration is expensive.
    • Hypothyroidism, growth hormone deficiencies, steroid hormone deficiencies, and delayed or precocious puberty have also been reported. Pay particular attention to these children when they develop significant systemic illnesses; stress doses of steroids may be required.
    • Severe endocrine abnormalities are associated almost exclusively with radical surgery.
  • Neurologic morbidity: This is also frequently associated with attempts at radical tumor resection, visual loss, and hypothalamic injury (morbid obesity, hypersomnolence), which may develop following therapy for craniopharyngiomas.
  • Neuropsychological morbidity
    • Memory loss, behavior changes, and academic decline can result directly from neurologic damage.
    • These neuropsychological changes can also occur indirectly as a result of other complications of therapy (eg, endocrinologic derangement).

Prognosis

  • Previous studies have shown relatively good outcomes, with 10-year overall survival rates of 86-100% among patients who underwent gross total resection. Subtotal resection or recurrence treated with surgery and radiation therapy carry 10-year overall survival rates of 57-86%. More recently, the surgical mortality rate after primary surgical intervention has been reported to be 1.7-5.4%. However, mortality rate after re-resection can be as high as 25%. Gross total resection carries a greater risk of long-term neurologic and endocrinologic morbidity.

Patient Education

  • For additional information, see the People Living With Cancer article on craniopharyngioma

Miscellaneous

Medicolegal Pitfalls

  • Pediatric tumors are rare, and one of their most common modes of presentation, increased intracranial pressure, can mimic many common childhood infectious diseases. The pediatrician who first observes children with seemingly routine nausea and vomiting should perform a reasonable screening neurologic examination. If papilledema is present in children with nausea, vomiting, and headaches, referral for further neurologic examination or neuroradiologic evaluation is suggested.
 
Acknowledgments

The authors and editors of eMedicine gratefully acknowledge the contributions of previous author B-Chen Wen, MD to the development and writing of this article.



More on Craniopharyngioma

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Differential Diagnoses & Workup: Craniopharyngioma
Treatment & Medication: Craniopharyngioma
Follow-up: Craniopharyngioma
Multimedia: Craniopharyngioma
References
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Further Reading

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Keywords

craniopharyngioma, adamantinoma, pituitary adamantinoma, ameloblastoma, suprasellar cyst, craniopharyngeal duct tumor, Rathke pouch tumor, Rathke's pouch tumor, Erdheim tumor, visual loss, blindness, growth hormone deficiency, thyroid-stimulating hormone deficiency, adrenocorticotropic hormone deficiency, luteinizing hormone deficiency, follicle-stimulating hormone deficiency, short stature, hypothyroidism, panhypopituitarism, diabetes insipidus, hydrocephalus, intracranial pressure, Diencephalic syndrome, adrenal insufficiency, papilledema, cranial nerve palsy

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Contributor Information and Disclosures

Author

Joseph L Lasky III, MD, Clinical Assistant Professor, Department of Pediatrics, Division of Hematology and Oncology, Mattel Children's Hospital UCLA
Joseph L Lasky III, MD is a member of the following medical societies: American Association for Cancer Research, American Society of Clinical Oncology, American Society of Pediatric Hematology/Oncology, Children's Oncology Group, and Society for Neuro-Oncology
Disclosure: Nothing to disclose.

Coauthor(s)

Kathleen M Sakamoto, MD, PhD, Professor and Chief, Division of Hematology-Oncology, Vice-Chair of Research, Mattel Children's Hospital at UCLA; Department of Pathology and Laboratory Medicine, Jonsson Comprehensive Cancer Center, David Geffen School of Medicine at UCLA and California Nanosystems Institute and Molecular Biology, UCLA
Kathleen M Sakamoto, MD, PhD is a member of the following medical societies: American Society of Hematology, American Society of Pediatric Hematology/Oncology, New York Academy of Sciences, Society for Pediatric Research, and Western Society for Pediatric Research
Disclosure: Nothing to disclose.

Jerry L Barker, Jr, MD, Staff Physician, Clinical Associate Professor of Radiation Oncology, Department of Radiation Oncology, University of Texas Southwestern Moncrief Cancer Center
Jerry L Barker, Jr, MD is a member of the following medical societies: American Society for Therapeutic Radiology and Oncology
Disclosure: Nothing to disclose.

Medical Editor

Samuel Gross, MD, Professor Emeritus, Department of Pediatrics, University of Florida; Clinical Professor, Department of Pediatrics, University of North Carolina; Adjunct Professor, Department of Pediatrics, Duke University
Samuel Gross, MD is a member of the following medical societies: American Association for Cancer Research, American Society for Blood and Marrow Transplantation, American Society of Clinical Oncology, American Society of Hematology, and Society for Pediatric Research
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

Timothy P Cripe, MD, PhD, Professor of Pediatric Hematology/Oncology, University of Cincinnati; Director, Translational Research Trials Office, Department of Pediatrics, Cincinnati Children's Hospital Medical Center
Timothy P Cripe, MD, PhD is a member of the following medical societies: American Association for the Advancement of Science, American Pediatric Society, American Society of Hematology, American Society of Pediatric Hematology/Oncology, and Society for Pediatric Research
Disclosure: Nothing to disclose.

CME Editor

David Pallares, MD, Clinical Assistant Professor, Department of Pediatrics, Division of Allergy and Immunology, University of Louisville
David Pallares, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology
Disclosure: Nothing to disclose.

Chief Editor

Robert J Arceci, MD, PhD, King Fahd Professor of Pediatric Oncology, Department of Oncology, Division of Pediatric Oncology, Johns Hopkins University School of Medicine
Robert J Arceci, MD, PhD is a member of the following medical societies: American Association for Cancer Research, American Association for the Advancement of Science, American Pediatric Society, American Society of Hematology, and American Society of Pediatric Hematology/Oncology
Disclosure: Nothing to disclose.

 
 
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