Pediatric Craniopharyngioma Follow-up

  • Author: Joseph L Lasky III, MD; Chief Editor: Robert J Arceci, MD, PhD   more...
 
Updated: Aug 11, 2010
 

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.

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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
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Complications

Endocrine morbidity

The need for hormone replacement is common (approximately 80%) following therapy for craniopharyngiomas. Individual hormone deficits manifest with rates of 88-100% for GH, 55-88% for ACTH, 39-95% for TSH, 80-95% for FSH/LH, and 25-86% for ADH.

Diabetes insipidus is one of the 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 can be 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).

Consequences of radiation therapy

Although it is now more commonly used in the management of craniopharyngiomas, even for children, late effects of radiation therapy are becoming increasingly recognized.

Endocrinopathies, vasculopathies (as high as 21%), and second malignancies (1.9% at 10 years) are now being seen following radiation therapy for these tumors.[14]

Transformation of low-grade craniopharyngiomas into high-grade epithelial carcinomas has also been observed. Generally this is in association with prior radiation therapy, although 1 of 3 cases from 1 series did not have prior radiation.[15]

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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.

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Patient Education

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

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Contributor Information and Disclosures
Author

Joseph L Lasky III, MD  Clinical Assistant Professor of Pediatrics and Neurosurgery, University of California, Los Angeles, David Geffen School of Medicine; Physician Specialist, Division of Pediatric Hematology/Oncology, Harbor-UCLA Medical Center

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; Co-Associate Program Director of the Signal Transduction Program Area, Jonsson Comprehensive Cancer Center, David Geffen School of Medicine at UCLA and California Nanosystems Institute and Molecular Biology Institute, UCLA

Kathleen M Sakamoto, MD, PhD is a member of the following medical societies: American Society of Hematology, American Society of Pediatric Hematology/Oncology, International Society for Experimental Hematology, 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.

Specialty Editor Board

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.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Pharmacy Editor, eMedicine

Disclosure: Nothing to disclose.

Timothy P Cripe, MD, PhD  Professor of Pediatrics, Division of Hematology/Oncology, Cincinnati Children's Hospital Medical Center; Clinical Director, Musculoskeletal Tumor Program, Co-Medical Director, Office for Clinical and Translational Research, Cincinnati Children's Hospital Medical Center; Director of Pilot and Collaborative Clinical and Translational Studies Core, Center for Clinical and Translational Science and Training, University of Cincinnati College of Medicine

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.

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, Professor of Pediatrics, Oncology and the Cellular and Molecular Medicine Graduate Program, Kimmel Comprehensive Cancer Center at 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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This MRI sequence was obtained following the intravenous administration of gadolinium contrast. Observe the relatively homogeneous and cystic mass arising from the sella turcica and extending superiorly and posteriorly with compression of normal regional structures. Note that the lesion is sharply demarcated and smoothly contoured. This fluid-filled mass is consistent with a typical craniopharyngioma.
This axial CT scan image demonstrates a cystic lesion in the typical location of a craniopharyngioma. Although most of the lesion is fluid filled, a rim of enhancing soft tissue is observed following the administration of intravenous contrast.
 
 
 
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