Pediatric Craniopharyngioma Workup

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

Laboratory Studies

The following laboratory measurements are indicated in patients with craniopharyngiomas:

Serum electrolytes levels establish readiness for surgery. Importantly, the endocrine dysfunction frequently associated can cause abnormalities in several of these test findings.

Growth hormone levels, including thyroid-stimulating hormone/thyroid hormone levels, steroid hormone levels (cortisol), follicle-stimulating hormone/luteinizing hormone levels should be obtained preoperatively as a baseline. Obtain tests to allow for perioperative hormone replacement as necessary.

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Imaging Studies

Skull radiography

Approximately 85% of craniopharyngiomas have calcifications above or within the pituitary fossa on plain radiographs of the skull.

Enlargement of the sella turcica can also be reliably identified.

If hydrocephalus is associated with a tumor in a young patient, split sutures may be observed.

Head CT scanning

This may be the only preoperative radiographic study needed because craniopharyngiomas are observed with mixed solid and cystic components, and the solid component is enhanced following the administration of intravenous contrast.

CT scanning is better than MRI at revealing the common tumor-associated calcifications.

Peritumoral edema is rare.

Hydrocephalus is identified and readily characterized.

Brain MRI

MRI is better than CT scan at determining the relationship of the tumor to adjacent normal structures.

As with CT scanning, mixed solid and cystic components are identifiable, and multiple cysts are common.

Craniopharyngiomas are usually sharply demarcated and smoothly marginated (see the image below).

This MRI sequence was obtained following the intraThis 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.

The solid component of the lesion frequently enhances following intravenous contrast administration, and a smooth ring of enhancement of the cyst wall can also be present (see the image below).

This axial CT scan image demonstrates a cystic lesThis 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.

Distortion or obliteration of the third ventricle is common.

Frequent involvement of the optic chiasm is found.

Obtaining postoperative imaging within 48 hours after surgery to best distinguish residual tumor from postsurgical changes is important.

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Other Tests

Preoperative intellectual or psychological assessment may be useful as a baseline examination prior to undertaking curative therapies.

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Procedures

Angiography: Cerebral angiography can be useful in planning the surgical approach, although it has been largely replaced by MRI/magnetic resonance angiography (MRA) in most centers. A vascular blush can be observed, although the tumor is not visible.

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Histologic Findings

Craniopharyngiomas can be histologically classified into 3 types: adamantinomatous, papillary, and mixed. The adamantinomatous type is by far the most common in children (92-96%). Grossly, these tumors usually have both solid and cystic components. The fluid within the cysts has been historically described as "crankcase oil" because of its frequently dark and oily intraoperative appearance. Upon microscopic examination, the fluid contains abundant lipids with birefringent cholesterol crystals. Clinically, spillage of the cyst fluid into the subarachnoid space can cause severe chemical arachnoiditis.

Microscopic examination of the solid components reveals an epithelial tumor with angulated columnar cells resting on a collagen basement membrane. Papillary structures are common, and calcification is nearly universal. Large tumors may induce an intense glial reaction and intensely adhere to the underlying normal brain.

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Staging

Preoperative and postoperative MRIs of the brain are adequate staging modalities for most children with craniopharyngioma. The postoperative scan is important in assessing residual disease. Neuraxis dissemination does not occur; thus, full spinal evaluation is unnecessary in an asymptomatic patient.

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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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