eMedicine Specialties > Pediatrics: General Medicine > Oncology

Craniopharyngioma: Differential Diagnoses & Workup

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

Differential Diagnoses

Astrocytoma
Ependymoma
Neuroblastoma

Other Problems to Be Considered

Cerebral aneurysm
Meningioma
Oligodendroglioma
Optic pathway gliomas
Pineoblastoma
Pituitary adenoma
Primitive neuroectodermal tumor
Rathke cleft cyst

Workup

Laboratory Studies

The following laboratory measurements are indicated in patients with craniopharyngiomas:

  • Serum electrolytes levels
    • These routine tests 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
    • Obtain these tests preoperatively as a baseline.
    • Obtain tests to allow for perioperative hormone replacement as necessary.

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.
    • 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.
    • Craniopharyngiomas are usually sharply demarcated and smoothly marginated.
    • 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.

Other Tests

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

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.

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.

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.

More on Craniopharyngioma

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