Craniopharyngioma Workup

  • Author: George C Bobustuc, MD; Chief Editor: Tarakad S Ramachandran, MBBS, FRCP(C), FACP   more...
 
Updated: Jan 12, 2012
 

Approach Considerations

The diagnostic evaluation of craniopharyngioma includes precontrast and postcontrast computed tomography (CT) scanning and magnetic resonance imaging (MRI), MR angiography (MRA), complete endocrinologic and neuro-ophthalmologic evaluation with formal visual field documentation, and neuropsychological assessment.

MIB-1 labeling index

The MIB-1 labeling index is a measure of the disease’s proliferative activity; it is determined by using an immunohistochemical method with monoclonal antibody MIB-1 and may be useful for the planning of adjuvant therapy. A small study found that an MIB-1 labeling index of greater than 7% predicted regrowth/recurrence.

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Endocrinologic Studies

These should include baseline serum electrolytes, serum and urine osmolality, thyroid studies, morning and evening cortisol levels, growth hormone levels, and luteinizing and follicle-stimulating hormone levels (in adolescent and adult patients).

Extending the workup for various hypothalamic-releasing factors allows for differentiation between endocrine disorders of pituitary origin and those of hypothalamic origin. It also helps in correlating various neurohormonal deficits with neuropsychological deficits.

In emergency cases, hormonal testing should be limited to diagnosing diabetes insipidus and hypoadrenalism, as both require the initiation of treatment prior to surgery.

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Imaging Studies

Imaging studies can strongly suggest the diagnosis of craniopharyngioma. The radiologic hallmark of a craniopharyngioma is the appearance of a (supra)sellar calcified cyst. About 80-87% of craniopharyngiomas are calcified and 70-75% are cystic. Calcifications are more common in children (90%) than in adults (50%).

CT scanning is the most sensitive method for demonstrating calcifications as high-density areas and has replaced the use of plain radiographs. It is useful in defining calcified and cystic parts. Cyst content usually has the same density as cerebrospinal fluid (CSF); contrast administration better defines the enhancing cyst capsule.

MRI, with its multiplanar capability, is essential for defining the local anatomy and is the most important imaging modality used to plan the surgical approach.

MRA is used for visualizing the major cerebral vessels and their relation to the tumor; it has largely replaced the 4-vessel angiogram.

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Histologic Findings

The histologic spectrum of craniopharyngioma includes 3 main types: adamantinomas, papillary craniopharyngiomas, and mixed tumors.

Adamantinoma

Adamantinomas consist of reticular epithelial masses that resemble the enamel pulp of developing teeth. This is seen predominantly in children. Distinctive features are a palisading basal layer of small cells enclosing a loose, stellate reticular zone, as well as areas of compactly arranged squamous cells. Adamantinomas contain nodules of keratin ("wet" keratin), which are the hallmarks of this tumor subtype. (See the images below.)

The adamantinomatous craniopharyngioma is a histolThe adamantinomatous craniopharyngioma is a histologically complex epithelial lesion with several very distinctive morphologic features (hematoxylin-eosin, x40). Adamantinomatous craniopharyngiomas. Peripheral paAdamantinomatous craniopharyngiomas. Peripheral palisading of the epithelium is a pronounced feature (hematoxylin-eosin, x100). Adamantinomatous craniopharyngiomas. Frequently, tAdamantinomatous craniopharyngiomas. Frequently, the inner epithelium beneath the superficial palisade undergoes hydropic vacuolization and is referred to as the stellate reticulum (hematoxylin-eosin, x100). Adamantinomatous craniopharyngiomas. Another distiAdamantinomatous craniopharyngiomas. Another distinctive feature of the adamantinomatous variant is scattered nodules of keratin. These nodules are referred to as "wet" keratin because of the plump appearance of the keratinocytes; this is in contrast to the flat, flaky keratin seen in epidermoid and dermoid cysts (hematoxylin-eosin, x100). Adamantinomatous craniopharyngiomas. Nodules of "wAdamantinomatous craniopharyngiomas. Nodules of "wet" keratin frequently calcify; in aggregate, this calcification often can be detected on CT scans and is a recognized radiologic feature of craniopharyngiomas (hematoxylin-eosin, x100).

Papillary craniopharyngioma

The squamous papillary craniopharyngioma is composed of islands of squamous metaplasia embedded in a connective tissue stroma, with infrequent cystic degeneration and calcification. This subtype rarely is seen in children and does not form keratin nodules. (See the images below.)

Papillary craniopharyngioma. In contrast to the adPapillary craniopharyngioma. In contrast to the adamantinomatous variant, papillary craniopharyngiomas do not show complex heterogeneous architecture but rather are composed of simple squamous epithelium and fibrovascular islands of connective tissue (hematoxylin-eosin, x40). Papillary craniopharyngiomas. Under high power, onPapillary craniopharyngiomas. Under high power, only simple squamous epithelium is seen in a papillary craniopharyngioma. The distinctive peripheral nuclear palisading, internal stellate reticulum, and nodules of "wet" keratin, which typify the adamantinomatous variant, are not seen in the papillary variant (hematoxylin-eosin, x100).

Brain parenchyma

The brain parenchyma that surrounds both variants of craniopharyngioma is typically gliotic and often shows profuse numbers of eosinophilic Rosenthal fibers. These fibers are composed of densely compacted bundles of glial filaments and typically are seen in astrocytic cell processes of neuropils that have been subjected to chronic compression from slowly expanding mass lesions. (See the image below.)

Rosenthal fibers in neuropils surrounding a cranioRosenthal fibers in neuropils surrounding a craniopharyngioma. The brain parenchyma that surrounds both variants of craniopharyngioma is typically gliotic and often shows profuse numbers of eosinophilic Rosenthal fibers. The latter structures are composed of densely compacted bundles of glial filaments and typically are seen in astrocytic cell processes of neuropils that have been subjected to chronic compression from slowly expanding mass lesions. Rosenthal fibers are a characteristic feature of juvenile pilocytic astrocytomas (JPAs), which also may arise in the suprasellar/third ventricular region. Hence, a biopsy that samples only the surrounding neuropil of a craniopharyngioma may yield an erroneous diagnosis of JPA if the pathologist is unaware of the close association of craniopharyngioma with Rosenthal fiber formation (hematoxylin-eosin, x100).
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Contributor Information and Disclosures
Author

George C Bobustuc, MD  Consulting Staff, Department of Neuro-oncology, MD Anderson Cancer Center of Orlando

George C Bobustuc, MD is a member of the following medical societies: American Academy of Neurology, American Medical Association, Society for Neuro-Oncology, and Texas Medical Association

Disclosure: Nothing to disclose.

Coauthor(s)

Morris D Groves, MD, JD  Assistant Professor, Department of Neuro-oncology, The University of Texas MD Anderson Cancer Center

Morris D Groves, MD, JD is a member of the following medical societies: American Academy of Neurology, American Medical Association, and Texas Medical Association

Disclosure: Genentech Grant/research funds Other; Genentech Honoraria Consulting; GlaxoSmithKline Grant/research funds Other; AngioChem Grant/research funds Other; Pfizer/Celldex Therapeautics Grant/research funds Other

Gregory N Fuller, MD, PhD  Professor of Pathology, Chief, Section of Neuropathology, Department of Pathology, Division of Pathology and Laboratory Medicine, The University of Texas MD Anderson Cancer Center

Gregory N Fuller, MD, PhD is a member of the following medical societies: American Association of Neuropathologists, College of American Pathologists, International Academy of Pathology, Society for Neuro-Oncology, and United States and Canadian Academy of Pathology

Disclosure: Nothing to disclose.

Franco DeMonte, MD, FRCSC, FACS  Professor of Neurosurgery, Mary Beth Pawelek Chair in Neurosurgery, The University of Texas MD Anderson Cancer Center

Franco DeMonte, MD, FRCSC, FACS is a member of the following medical societies: Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

Chief Editor

Tarakad S Ramachandran, MBBS, FRCP(C), FACP  Professor of Neurology, Clinical Professor of Medicine, Clinical Professor of Family Medicine, Clinical Professor of Neurosurgery, State University of New York Upstate Medical University; Chair, Department of Neurology, Crouse Irving Memorial Hospital

Tarakad S Ramachandran, MBBS, FRCP(C), FACP is a member of the following medical societies: American Academy of Neurology, American Academy of Pain Medicine, American College of Forensic Examiners, American College of International Physicians, American College of Managed Care Medicine, American College of Physicians, American Heart Association, American Stroke Association, Royal College of Physicians, Royal College of Physicians and Surgeons of Canada, Royal College of Surgeons of England, and Royal Society of Medicine

Disclosure: Abbott Labs None None; Teva Marion None None; Boeringer-Ingelheim Honoraria Speaking and teaching

Additional Contributors

Jorge Kattah, MD Head, Program Director, Professor, Department of Neurology, University of Illinois College of Medicine at Peoria

Jorge Kattah, MD is a member of the following medical societies: American Academy of Neurology, American Neurological Association, and New York Academy of Sciences

Disclosure: Biogen Honoraria Consulting; Bayer Corporation Honoraria Consulting

Amy A Pruitt, MD Associate Professor of Neurology, University of Pennsylvania; Attending Neurologist, Hospital of the University of Pennsylvania

Amy A Pruitt, MD is a member of the following medical societies: American Academy of Neurology

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 Reference Salary Employment

References

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The adamantinomatous craniopharyngioma is a histologically complex epithelial lesion with several very distinctive morphologic features (hematoxylin-eosin, x40).
Adamantinomatous craniopharyngiomas. Peripheral palisading of the epithelium is a pronounced feature (hematoxylin-eosin, x100).
Adamantinomatous craniopharyngiomas. Frequently, the inner epithelium beneath the superficial palisade undergoes hydropic vacuolization and is referred to as the stellate reticulum (hematoxylin-eosin, x100).
Adamantinomatous craniopharyngiomas. Another distinctive feature of the adamantinomatous variant is scattered nodules of keratin. These nodules are referred to as "wet" keratin because of the plump appearance of the keratinocytes; this is in contrast to the flat, flaky keratin seen in epidermoid and dermoid cysts (hematoxylin-eosin, x100).
Adamantinomatous craniopharyngiomas. Nodules of "wet" keratin frequently calcify; in aggregate, this calcification often can be detected on CT scans and is a recognized radiologic feature of craniopharyngiomas (hematoxylin-eosin, x100).
Papillary craniopharyngioma. In contrast to the adamantinomatous variant, papillary craniopharyngiomas do not show complex heterogeneous architecture but rather are composed of simple squamous epithelium and fibrovascular islands of connective tissue (hematoxylin-eosin, x40).
Papillary craniopharyngiomas. Under high power, only simple squamous epithelium is seen in a papillary craniopharyngioma. The distinctive peripheral nuclear palisading, internal stellate reticulum, and nodules of "wet" keratin, which typify the adamantinomatous variant, are not seen in the papillary variant (hematoxylin-eosin, x100).
Rosenthal fibers in neuropils surrounding a craniopharyngioma. The brain parenchyma that surrounds both variants of craniopharyngioma is typically gliotic and often shows profuse numbers of eosinophilic Rosenthal fibers. The latter structures are composed of densely compacted bundles of glial filaments and typically are seen in astrocytic cell processes of neuropils that have been subjected to chronic compression from slowly expanding mass lesions. Rosenthal fibers are a characteristic feature of juvenile pilocytic astrocytomas (JPAs), which also may arise in the suprasellar/third ventricular region. Hence, a biopsy that samples only the surrounding neuropil of a craniopharyngioma may yield an erroneous diagnosis of JPA if the pathologist is unaware of the close association of craniopharyngioma with Rosenthal fiber formation (hematoxylin-eosin, x100).
 
 
 
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