Craniopharyngioma Clinical Presentation

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

History

Craniopharyngioma usually is a slow-growing tumor. Symptoms frequently develop insidiously and usually become obvious only after the tumor attains a diameter of about 3cm. The time interval between the onset of symptoms and diagnosis ranges from 1-2 years.

The most common presenting symptoms are headache (55-86%), endocrine dysfunction (66-90%), and visual disturbances (37-68%). Headache is slowly progressive, dull, continuous, and positional; it becomes severe in most patients when endocrine symptoms become obvious.

On presentation, 40% of patients have symptoms of hypothyroidism (eg, weight gain, fatigue, cold intolerance, constipation). Almost 25% have associated signs and symptoms of adrenal failure (eg, orthostatic hypotension, hypoglycemia, hyperkalemia, cardiac arrhythmias, lethargy, confusion, anorexia, nausea and vomiting), and 20% have diabetes insipidus (eg, excessive fluid intake and urination). Most young patients present with growth failure and delayed puberty.[17]

Eighty percent of adults complain of decreased sexual drive, and almost 90% of men complain of impotence, while most women complain of amenorrhea.

Optic pathway dysfunction on presentation is noted in 40-70% of patients. Children rarely become aware of visual problems (only 20-30%) and often present after almost complete visual damage has taken place.

Other manifestations relate to the various connections of the hypothalamic-pituitary complex and surrounding structures. The thalamus and frontal lobes present with corresponding endocrine, autonomic, and behavioral problems (eg, hyperphagia and obesity, psychomotor retardation, emotional immaturity, apathy, short-term memory deficits, incontinence).[18] Short stature is present in 23-45% of patients, and obesity, in 11-18% of patients.

The following relationships are seen between the anatomic location of the craniopharyngioma and major clinical syndromes:

  • Prechiasmal localization - Typically results in associated findings of optic atrophy (eg, progressive decline of visual acuity, constriction of visual fields)
  • Retrochiasmal location - Commonly is associated with hydrocephalus with signs of increased intracranial pressure (eg, papilledema, horizontal double vision)
  • Intrasellar craniopharyngioma - Usually manifests with headache and endocrinopathy
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Physical Examination

Neurologic and general examinations are indicated.

Neurologic examination

Signs suggestive of increased intracranial pressure—horizontal double vision (unilateral/bilateral) and papilledema (unilateral/bilateral)—should be sought in any patient suspected of having an intracranial mass.

Visual field examination may reveal various patterns of visual loss (most frequently bitemporal hemianopsia) suggestive of involvement (ie, compression) of the optic chiasma and/or tracts. Visual fields should be tested further with formal testing.

General examination

This may reveal signs relating to different endocrinopathies

Hypothyroidism

Symptoms of hypothyroidism include the following:

  • Puffiness and nonpitting edema
  • Slow return phase of deep tendon reflexes
  • Long-standing effects on organ systems
  • Hypoventilation and decrease in cardiac output
  • Pericardial and pleural effusions
  • Constipation
  • Anemia - Ie, normochromic normocytic
  • Decreased mental function
  • Psychiatric changes

Adrenal insufficiency

Cortisol deficiency results in hypotension, which is often orthostatic. Gastrointestinal symptoms include anorexia, nausea, and vomiting; other signs and symptoms include weight loss, hypoglycemia, lethargy, confusion, psychosis, and intolerance to stress.

The signs and symptoms of aldosterone deficiency include the following:

  • Hypovolemia
  • Decreased cardiac output
  • Decreased renal blood flow with azotemia
  • Fatigue
  • Weight loss
  • Cardiac arrhythmias due to hyperkalemia
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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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