Craniopharyngioma Clinical Presentation: History, Physical Examination

Sections

Presentation

History

Craniopharyngioma typically 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 usually 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 related to hypothyroidism (i.e., weight gain, fatigue, cold intolerance, constipation). Almost 25% have associated signs and symptoms of adrenal failure (i.e., orthostatic hypotension, hypoglycemia, hyperkalemia, cardiac arrhythmias, lethargy, confusion, anorexia, nausea, and vomiting), and 20% have diabetes insipidus (i.e. excessive fluid intake and urination). Most young patients present with growth failure and delayed puberty.^[21]

As disease progress, 80% of adults complain of decreased sexual drive, and almost 90% of men complain of impotence, while most women complain of amenorrhea.

Optic pathway dysfunction is noted in 40-70% of patients on presentation. Children rarely become aware of visual problems (only 20-30%) and often present after almost complete visual damage becomes irreversible. The manifestation of optic pathway dysfunction usually varies from papilledema to visual field deficits and even optic nerve atrophy in severe cases.

Other manifestations relate to the various connections of the hypothalamic-pituitary complex and surrounding structures. When the thalamus, hypothalamus and frontal lobes are affected, patients experience endocrine, autonomic, and behavioral problems (i.e. hyperphagia and obesity, psychomotor retardation, emotional immaturity, apathy, short-term memory deficits, incontinence).^[22] Short stature is present in 23-45% of patients, and obesity affects 11-18% of patients.^[23]

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 (i.e., progressive decline of visual acuity, constriction of visual fields). The chiasm stretches and gets displaced away from the optic nerve, which eventually causes vascular deprivation of the nerves and damage to nerve fibers.
Retrochiasmal location - Commonly is associated with hydrocephalus with signs of increased intracranial pressure (i.e. papilledema, horizontal double vision). The tumor compresses the floor of the third ventricle and can lead to obstruction of CSF flow through the Foramen of Monroe.
Intrasellar craniopharyngioma - Usually manifests as headache and/or endocrinopathy.

Physical Examination

Neurologic and general examinations are both indicated.

Neurologic examination

Signs suggestive of increased intracranial pressure—horizontal double vision (unilateral/bilateral) and papilledema (unilateral/bilateral)—should be sought for 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 (i.e., compression) of the optic chiasm and/or tracts. Formal visual field testing by ophthalmology is recommended as part of the initial work up and serial testing can be used in follow up to monitor tumor growth/recurrence.

General examination

Signs and symptoms may be related to various endocrinopathies.

Hypothyroidism

Symptoms of hypothyroidism include the following:

▪ Puffiness and non-pitting edema

▪ Slow return phase of deep tendon reflexes

▪ Hypoventilation and decrease in cardiac output

▪ Pericardial and pleural effusions

▪ Constipation

▪ Anemia – i.e., normochromic normocytic anemia

▪ Decreased mental function

▪ Psychiatric changes

Cortisol-related deficiency

The signs and symptoms of cortisol deficiency include the following:

Hypotension, which is often orthostatic
Gastrointestinal symptoms, which include anorexia, nausea, and vomiting
Weight loss
Hypoglycemia
Lethargy
Confusion
Psychological disturbances, i.e.. psychosis and intolerance to stress

Changes in volume and sodium control

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

Compression of the infundibulum can lead to the common presentation of diabetes insipidus.

Differential Diagnoses

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Media Gallery

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).
T1-weighted MRI with gadolinium in sagittal (A) and coronal (B) views demonstrates the cystic nature of a craniopharyngioma. The calcified component is evident on axial CT imaging (C).
T1-weighted MRI with gadolinium reveals a large cystic craniopharyngioma in sagittal (A), axial (B), and coronal (C) views. There is associated elevation of the optic apparatus and displacement of the pituitary stalk.
Coronal views of T1-weighted MRI for a patient with craniopharyngioma before gross total resection (A) and at postoperative follow-up evaluation (B). There was no sign of tumor recurrence, and the patient was neurologically and endocrinologically intact.

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Author

George I Jallo, MD Professor of Neurosurgery, Pediatrics, and Oncology, Director, Clinical Pediatric Neurosurgery, Department of Neurosurgery, Johns Hopkins University School of Medicine

George I Jallo, MD is a member of the following medical societies: American Association of Neurological Surgeons, American Medical Association, American Society of Pediatric Neurosurgeons

Disclosure: Nothing to disclose.

Coauthor(s)

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: American Academy of Neurological Surgery, American College of Surgeons, North American Skull Base Society, Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

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, United States and Canadian Academy of Pathology

Disclosure: Nothing to disclose.

Morris D Groves, MD, JD Associate Professor, Department of Neuro-Oncology, Division of Cancer Medicine, Associate Clinic Director for the Brain and Spine Center, 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 Bar Association, American Medical Association, European Association of Neuro-Oncology, Harris County Medical Society, Houston Neurological Society, Society for Neuro-Oncology, Texas Medical Association, Texas Neurological Society

Disclosure: Received grant/research funds from Genentech for other; Received honoraria from Genentech for consulting; Received grant/research funds from GlaxoSmithKline for other; Received grant/research funds from AngioChem for other; Received grant/research funds from Pfizer/Celldex Therapeautics for other.

Meleine M Martinez-Sosa, MD Resident Physician, Department of Neurosurgery and Brain Repair, University of South Florida Morsani College of Medicine

Disclosure: Nothing to disclose.

Nir Shimony, MD Neurosurgeon, Pediatric, Epilepsy, NeuroOncology, Neuroscience Researcher, Geisinger Health System, Johns Hopkins University School of Medicine

Nir Shimony, MD is a member of the following medical societies: American Association of Neurological Surgeons, American Epilepsy Society, Congress of Neurological Surgeons, Society for Neuro-Oncology

Disclosure: Nothing to disclose.

Chief Editor

Tarakad S Ramachandran, MBBS, MBA, MPH, FAAN, FACP, FAHA, FRCP, FRCPC, FRS, LRCP, MRCP, MRCS Professor Emeritus of Neurology and Psychiatry, Clinical Professor of Medicine, Clinical Professor of Family Medicine, Clinical Professor of Neurosurgery, State University of New York Upstate Medical University; Neuroscience Director, Department of Neurology, Crouse Irving Memorial Hospital

Tarakad S Ramachandran, MBBS, MBA, MPH, FAAN, FACP, FAHA, FRCP, FRCPC, FRS, LRCP, MRCP, MRCS is a member of the following medical societies: American College of International Physicians, American Heart Association, American Stroke Association, American Academy of Neurology, American Academy of Pain Medicine, American College of Forensic Examiners Institute, National Association of Managed Care Physicians, American College of Physicians, Royal College of Physicians, Royal College of Physicians and Surgeons of Canada, Royal College of Surgeons of England, Royal Society of Medicine

Disclosure: Nothing to disclose.

Additional Contributors

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, Texas Medical Association, Society for Neuro-Oncology, American Medical Association

Disclosure: Nothing to disclose.

Lee S Hwang University of Texas Southwestern Medical School

Disclosure: Nothing to disclose.

Acknowledgements

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

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