eMedicine Specialties > Neurology > Neuro-oncology
Craniopharyngioma: Differential Diagnoses & Workup
Updated: Sep 4, 2009
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
- Multimedia
Differential Diagnoses
Other Problems to Be Considered
Brainstem syndromes
Increased intracranial pressure
Epidermoid and dermoid tumor
Germ cell tumor
Hypothalamic-optic pathway glioma
Meningioma
Metastasis
Hypothalamic hamartoma
Pituitary tumorInfectious or inflammatory processes
Histiocytosis X
Infundibulitis
Lymphocytic hypophysitis
Sarcoidosis
Syphilis
TuberculosisVascular malformations
Carotid-cavernous fistula
Cavernous sinus hemangioma
Giant suprasellar carotid aneurysmOther congenital defects
Arachnoid cyst
Rathke cleft cyst
Workup
Laboratory Studies
- The diagnostic evaluation for craniopharyngioma includes precontrast and postcontrast CT scans and MRI, magnetic resonance angiography (MRA), complete endocrinologic and neuro-ophthalmologic evaluation with formal visual field documentation, as well as neuropsychological assessment.
- 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 initiation of treatment prior to surgery.
Imaging Studies
- Imaging studies strongly suggest the diagnosis. 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 scan is the most sensitive method to demonstrate calcifications as high-density areas and has replaced the plain radiograph. It is useful in defining both calcified and cystic parts. Cyst content usually has the same density as 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.
Other Tests
- Neuro-ophthalmologic evaluation with formal visual field documentation
- Neuropsychological assessment
Histologic Findings
The histologic spectrum of craniopharyngioma includes 3 main types—adamantinomas, papillary, and mixed.
- Adamantinomas consist of reticular epithelial masses, resembling the enamel pulp of developing teeth. This is seen predominantly in children. A distinctive feature is a palisading basal layer of small cells, which encloses a loose stellate reticular zone, as well as areas of compactly arranged squamous cells. They contain nodules of keratin ("wet" keratin), which are the hallmarks of this tumor subtype (see Media files 1-5).
The adamantinomatous craniopharyngioma is a histologically complex epithelial lesion with several very distinctive morphologic features. Each of these features is shown under higher power in Images 2-5 (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).
- Squamous papillary type 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 Media files 6-7).
Papillary craniopharyngioma. In contrast to the adamantinomatous variant, papillary craniopharyngiomas do not show complex heterogeneous architecture (compare with Image 1) 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).
- The brain parenchyma that surrounds both variants of craniopharyngioma is typically gliotic and often shows profuse numbers of eosinophilic Rosenthal fibers, which 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 Media file 8).
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).
More on Craniopharyngioma |
| Overview: Craniopharyngioma |
 Differential Diagnoses & Workup: Craniopharyngioma |
| Treatment & Medication: Craniopharyngioma |
| Follow-up: Craniopharyngioma |
| Multimedia: Craniopharyngioma |
| References |
| Further Reading |
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Further Reading
Clinical guidelines
Long term follow up of survivors of childhood cancer. A national clinical guideline.
Scottish Intercollegiate Guidelines Network - National Government Agency [Non-U.S.]. 2004 Jan. 33 pages. NGC:003410
Clinical trials
An Investigation of Pituitary Tumors and Related Hypothalmic Disorders
Effect of Diazoxide on the Obesity Secondary to Hypothalamic-Pituitary Lesions
SCRT Vs Conventional RT in Children and Young Adults With Low Grade and Benign Brain Tumours
Related eMedicine topics
Craniopharyngioma (Radiology)
Craniopharyngioma (Pediatrics)
Craniopharyngiomas (Neurosurgery)
Growth Hormone Deficiency
Hypopituitarism
Keywords
adamantinoma, craniopharyngeal duct tumor, Rathke pouch tumor, craniopharyngioma, cystic tumor, Rathke cleft, epithelial-squamous calcified cystic tumor
