Surgery for Craniopharyngiomas Workup

  • Author: Lawrence S Chin, MD, FACS; Chief Editor: Allen R Wyler, MD   more...
 
Updated: Oct 29, 2010
 

Laboratory Studies

Endocrine assessment

A full pituitary endocrine workup is usually mandatory. This includes evaluating the following:

  • Adrenocorticotropic hormone (ACTH or corticotropin)
  • Growth hormone (GH) and insulin growth factor (IGF-1)
  • Cortisol
  • Prolactin
  • Luteinizing hormone (LH)
  • Follicle-stimulating hormone (FSH)
  • Thyrotropin (ie, thyroid-stimulating hormone [TSH])
  • Triiodothyronine (T3)
  • Thyroxine (T4).

Any abnormalities should be corrected preoperatively but, at the very least, low cortisol levels and diabetes insipidus should be treated prior to any surgical procedure.

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Imaging Studies

CT and MRI are the complementary examinations of choice. Today, the best imaging tool is MRI without and with contrast enhancement.[7, 8]

CT scan

Craniopharyngiomas can vary greatly in size, from a few millimeters to greater than 5 cm. On CT scan, 90% are at least partially cystic, 90% have calcifications, and 90% have nodular or rim enhancement. These tumors are heterogeneous, with the cystic component being hypodense on CT scan and the solid component being isodense or slightly hyperdense with variable enhancement with contrast.

MRI

An MRI with and without contrast is the preferred sequence of choice. On T1-weighted images, the cystic component is often hyperintense and the solid component is isointense, with enhancement of the rim or tumor nodule. On T2-weighted images, the cystic component is hyperintense, as is the solid component.

In the immediate postoperative period (within the first 48 h) a contrast-enhanced MRI is usually performed to determine whether residual tumor is present, as well as to establish a baseline for future follow-up.[7]

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Other Tests

Ophthalmology assessment

Visual acuity and visual field assessment is required to delineate any deficit, including papilledema.

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Histologic Findings

Craniopharyngiomas are usually composed of both solid and cystic components. Cyst walls may vary from thin membranes to thick, tough structures that may be hard and rigid because of calcifications. The interior is lined with stratified squamous epithelium with pearly keratin formations. The outside layer is columnar epithelium on a collagenous basement membrane. Calcifications are common; 90% of tumors in children have calcifications, as do 40% of these tumors in adults. Inside the tumor, fibrous tissue, necrotic debris, cholesterol clefts, and keratin pearls are commonly found.

The following 3 histologic phenotypes are seen in craniopharyngioma:

  • Adamantinomatous tumors (seen predominantly in children) resemble enamel-forming oropharyngeal neoplasms. The classic appearance is that of a cystic tumor, usually with a solid component. The cyst may contain fluid that can vary in color, but it usually has a tan appearance that is classically described as resembling “motor oil.” Extensive fibrosis and inflammation have also been observed, which result in dense adhesions between the mass and vasculature, a phenomenon that further contributes to the difficulty in resecting craniopharyngiomas.
  • Squamous papillary tumors (seen predominantly in adults) generally involve only a solid component, which is typically seen without calcifications. It is frequently located in the third ventricle and is usually more encapsulated than the other types and, thus, more easily resectable.
  • Mixed tumors are a combination of the adamantinomatous and papillary forms.

Craniopharyngiomas are known to not undergo malignant degeneration and are usually well defined.[9] However, at the tumor margins, the epithelial fronds tend to penetrate deep into the brain tissue. This may cause a glial reaction in the surrounding brain, making complete resection difficult and possibly predisposing this tissue to traction injuries, particularly in the hypothalamus.

The characteristic location of these tumors in the sellar and parasellar region, together with the different histologic subtypes, allows for the above theories that may explain the origin of these tumors.[10]

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Contributor Information and Disclosures
Author

Lawrence S Chin, MD, FACS  Professor and Chairman, Department of Neurosurgery, Boston University School of Medicine; Neurosurgeon-in-Chief, Boston Medical Center

Lawrence S Chin, MD, FACS is a member of the following medical societies: Alpha Omega Alpha, American Association for Cancer Research, American Association for the Advancement of Science, American Association of Neurological Surgeons, American College of Surgeons, Children's Oncology Group, Congress of Neurological Surgeons, Phi Beta Kappa, and Society for Neuro-Oncology

Disclosure: Nothing to disclose.

Coauthor(s)

Mayur Jayarao, MD  Fellow, Department of Neurosurgery, Boston Medical Center

Mayur Jayarao, MD is a member of the following medical societies: American College of Surgeons, American Medical Association, and Medical Council of India

Disclosure: Nothing to disclose.

Specialty Editor Board

Paul L Penar, MD, FACS  Professor, Department of Surgery, Division of Neurosurgery, Director, Functional Neurosurgery and Radiosurgery Programs, University of Vermont College of Medicine

Paul L Penar, MD, FACS is a member of the following medical societies: Alpha Omega Alpha, American Association of Neurological Surgeons, Congress of Neurological Surgeons, and World Society for Stereotactic and Functional Neurosurgery

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Ryszard M Pluta, MD, PhD  Associate Professor, Neurosurgical Department Medical Research Center, Polish Academy of Sciences at Warsaw, Poland; Clinical Staff Scientist, Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health (NIH); Fishbein Fellow, JAMA, Chicago ,IL

Ryszard M Pluta, MD, PhD is a member of the following medical societies: Congress of Neurological Surgeons and Polish Society of Neurosurgeons

Disclosure: Nothing to disclose.

Paolo Zamboni, MD  Professor of Surgery, Chief of Day Surgery Unit, Chair of Vascular Diseases Center, University of Ferrara, Italy

Paolo Zamboni, MD is a member of the following medical societies: American Venous Forum and New York Academy of Sciences

Disclosure: Nothing to disclose.

Chief Editor

Allen R Wyler, MD  Former Medical Director, Northstar Neuroscience, Inc

Allen R Wyler, MD is a member of the following medical societies: American Academy of Neurological and Orthopaedic Surgeons, American Association of Neurological Surgeons, and Society of Neurological Surgeons

Disclosure: Nothing to disclose.

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Coronal MRI shows a craniopharyngioma in the suprasellar space that causes compression of the optic nerves and chiasm.
Sagittal MRI shows a cystic craniopharyngioma in the suprasellar space with extension into the third ventricle.
Axial MRI shows a craniopharyngioma cyst that contains proteinaceous fluid in the third ventricle. The cyst fluid appears hyperintense.
Dissection of craniopharyngioma cyst with aspiration.
 
 
 
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