Pediatric Craniopharyngioma Clinical Presentation

  • Author: Joseph L Lasky III, MD; Chief Editor: Robert J Arceci, MD, PhD   more...
 
Updated: Aug 11, 2010
 

History

Craniopharyngiomas produce symptoms by compression of adjacent neural structures. They can become quite large, obstructing cerebral spinal fluid (CSF) pathways (ie, third ventricle, Monro foramen) and causing hydrocephalus and increased intracranial pressure that leads to headaches, nausea, and projectile vomiting.

Symptoms at presentation may include the following:

  • Headache: Headaches occur in 60-80% of children with craniopharyngioma at presentation and are usually a symptom of increased intracranial pressure or hydrocephalus.
  • Vomiting: Classic projectile vomiting (frequently without nausea) accompanies headaches as a sign of increased intracranial pressure and is reported in 35-70% of children with these tumors at presentation.
  • Vision loss: As mentioned above (see Mortality/Morbidity), children are frequently unaware of significant vision loss; nevertheless, this symptom reportedly occurs in 20-60% of pediatric patients with craniopharyngioma at presentation. Classically, vision loss starts with a superior temporal field cut. However, the eccentric growth of these tumors can result in varying patterns and severity of vision loss, including decreased acuity, diplopia, blurred vision, and subjective visual field deficits.

The following symptoms related to endocrine dysfunction may be present prior to therapy[2] :

  • Diencephalic syndrome: This term is used to describe emaciated hyperactive children who occasionally present with unusual eye movements and even blindness; these symptoms result from extrinsic compression of the hypothalamus. Conversely, damage to or invasion of the ventromedial hypothalamus can result in a dysregulation of energy balance and resultant obesity upon presentation.
  • Symptoms of growth hormone deficiency (ie, short stature): Growth hormone deficiency is the most common possible endocrinologic disturbance caused by craniopharyngiomas (35-95%). One series reported that growth failure preceded the diagnosis at a mean of 4 years.
  • Symptoms of hypothyroidism (present in 21-42% of cases)
    • Weight gain
    • Lethargy
    • Fatigue
    • Cold intolerance
    • Dry skin
    • Dry brittle hair
    • Slow teething
    • Anorexia
    • Large tongue
    • Deep voice
    • Myxedema
  • Symptoms of adrenal insufficiency: Secondary adrenal insufficiency (ACTH deficiency) is the second most common endocrinologic disturbance caused by craniopharyngiomas (21-62% of cases).
  • Symptoms of leuteinizing hormone/follicle-stimulating hormone deficiency: Gonadotropin deficiency is the most common presenting symptom of craniopharyngioma in adults (38-82% of cases). As many as 100% of presenting adolescents may have complaints of delayed puberty.[6]

Mental status changes occur in as many as 25% of adults but are rare in children. Temporal lobe involvement can result in seizures, although this is rare.

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Physical

Focus physical examination on the identification of neurologic and endocrine derangements.

Papilledema

Papilledema occurs in 25-40% of children and results from increased intracranial pressure.

Visual field deficits

Formal testing is generally required to identify visual field deficits in children, which likely explains the wide reported range (10-95%) of patients with craniopharyngioma.

Given the typical proximity of the tumor to the optic nerves, optic chiasm, and anterior optic tracts, the common discovery of visual fields defects at presentation is not surprising.

See-saw nystagmus

Although often referred to as a classic physical examination finding among children with parasellar tumors, the literature reports an incidence rate of less than 10%.

Cranial nerve palsy

With the notable exception of the optic nerves, cranial nerve palsies are relatively rare, with a reported incidence rate of 8% for children at time of diagnosis.

Endocrine effects

Short stature or growth retardation is the most common endocrine derangement associated with this tumor. Growth retardation (as documented on formal pediatric growth charts) is reported in 86% of patients with craniopharyngioma at presentation.

Obesity and weight gain is the third most common endocrine abnormality associated with craniopharyngiomas. Hypothyroidism, growth hormone deficiency, and direct hypothalamic injury can contribute to obesity and weight gain. Obesity and weight gain are reported in 20% of presenting patients.

Hypothyroidism can manifest as weight gain, dry skin, brittle hair, and bradycardia.

Precocious or delayed puberty

Precocious or delayed puberty is the fourth most common endocrine derangement associated with craniopharyngiomas and is present at diagnosis in 10-15% of patients. This is the most common presenting sign in adolescents.

Intellectual or emotional disturbance and somnolence

These signs are most likely the result of either hydrocephalus or thyroid dysfunction.

Enlarging head circumference

This finding is highly suggestive of an intracranial mass or hydrocephalus, particularly when paired with papilledema.

Ataxia

This is another sign of increased intracranial pressure or hydrocephalus, which is present in 5-10% of patients at initial evaluation.

Seizures

These are rarely described as a presenting feature.

Focal motor weakness

This is also rarely described as a presenting feature.

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Causes

No known environmental or infectious causes predispose to the development of craniopharyngiomas.

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Contributor Information and Disclosures
Author

Joseph L Lasky III, MD  Clinical Assistant Professor of Pediatrics and Neurosurgery, University of California, Los Angeles, David Geffen School of Medicine; Physician Specialist, Division of Pediatric Hematology/Oncology, Harbor-UCLA Medical Center

Joseph L Lasky III, MD is a member of the following medical societies: American Association for Cancer Research, American Society of Clinical Oncology, American Society of Pediatric Hematology/Oncology, Children's Oncology Group, and Society for Neuro-Oncology

Disclosure: Nothing to disclose.

Coauthor(s)

Kathleen M Sakamoto, MD, PhD  Professor and Chief, Division of Hematology-Oncology, Vice-Chair of Research, Mattel Children's Hospital at UCLA; Co-Associate Program Director of the Signal Transduction Program Area, Jonsson Comprehensive Cancer Center, David Geffen School of Medicine at UCLA and California Nanosystems Institute and Molecular Biology Institute, UCLA

Kathleen M Sakamoto, MD, PhD is a member of the following medical societies: American Society of Hematology, American Society of Pediatric Hematology/Oncology, International Society for Experimental Hematology, Society for Pediatric Research, and Western Society for Pediatric Research

Disclosure: Nothing to disclose.

Jerry L Barker, Jr, MD  Staff Physician, Clinical Associate Professor of Radiation Oncology, Department of Radiation Oncology, University of Texas Southwestern Moncrief Cancer Center

Jerry L Barker, Jr, MD is a member of the following medical societies: American Society for Therapeutic Radiology and Oncology

Disclosure: Nothing to disclose.

Specialty Editor Board

Samuel Gross, MD  Professor Emeritus, Department of Pediatrics, University of Florida; Clinical Professor, Department of Pediatrics, University of North Carolina; Adjunct Professor, Department of Pediatrics, Duke University

Samuel Gross, MD is a member of the following medical societies: American Association for Cancer Research, American Society for Blood and Marrow Transplantation, American Society of Clinical Oncology, American Society of Hematology, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Pharmacy Editor, eMedicine

Disclosure: Nothing to disclose.

Timothy P Cripe, MD, PhD  Professor of Pediatrics, Division of Hematology/Oncology, Cincinnati Children's Hospital Medical Center; Clinical Director, Musculoskeletal Tumor Program, Co-Medical Director, Office for Clinical and Translational Research, Cincinnati Children's Hospital Medical Center; Director of Pilot and Collaborative Clinical and Translational Studies Core, Center for Clinical and Translational Science and Training, University of Cincinnati College of Medicine

Timothy P Cripe, MD, PhD is a member of the following medical societies: American Association for the Advancement of Science, American Pediatric Society, American Society of Hematology, American Society of Pediatric Hematology/Oncology, and Society for Pediatric Research

Disclosure: Nothing to disclose.

David Pallares, MD  Clinical Assistant Professor, Department of Pediatrics, Division of Allergy and Immunology, University of Louisville

David Pallares, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology

Disclosure: Nothing to disclose.

Chief Editor

Robert J Arceci, MD, PhD  King Fahd Professor of Pediatric Oncology, Professor of Pediatrics, Oncology and the Cellular and Molecular Medicine Graduate Program, Kimmel Comprehensive Cancer Center at Johns Hopkins University School of Medicine

Robert J Arceci, MD, PhD is a member of the following medical societies: American Association for Cancer Research, American Association for the Advancement of Science, American Pediatric Society, American Society of Hematology, and American Society of Pediatric Hematology/Oncology

Disclosure: Nothing to disclose.

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This MRI sequence was obtained following the intravenous administration of gadolinium contrast. Observe the relatively homogeneous and cystic mass arising from the sella turcica and extending superiorly and posteriorly with compression of normal regional structures. Note that the lesion is sharply demarcated and smoothly contoured. This fluid-filled mass is consistent with a typical craniopharyngioma.
This axial CT scan image demonstrates a cystic lesion in the typical location of a craniopharyngioma. Although most of the lesion is fluid filled, a rim of enhancing soft tissue is observed following the administration of intravenous contrast.
 
 
 
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