AUTHOR INFORMATION

Section 1 of 12

Authored by Joseph L Lasky III, MD, Pediatric Hematology/Oncology, Assistant Professor of Pediatrics, Mattel Children's Hospital, David Geffen School of Medicine, University of California at Los Angeles

Coauthored by Kathleen Sakamoto, MD, Professor, Department of Pediatrics, Division of Hematology-Oncology and Pathology and Laboratory Medicine, Mattel Children's Hospital, David Geffen School of Medicine, University of California at Los Angeles; Jerry L Barker, Jr, MD, Staff Physician, Clinical Associate Professor of Radiation Oncology, Department of Radiation Oncology, University of Texas Southwestern Moncrief Cancer Center; B-Chen Wen, MD, Professor, Department of Radiology, Division of Radiation Oncology, University of Iowa Hospitals and Clinics

Joseph L Lasky III, MD, is a member of the following medical societies: American Society of Clinical Oncology, American Society of Hematology, American Society of Pediatric Hematology/Oncology, and Western Society for Pediatric Research

Edited by Samuel Gross, MD, Professor Emeritus, Department of Pediatrics, University of Florida, Clinical Professor, Department of Pediatrics, UNC, Adjunct Professor, Department of Pediatrics, Duke University; Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine.com, Inc; Timothy P Cripe, MD, PhD, Associate Professor of Pediatric Hematology/Oncology, University of Cincinnati; Director, Translational Research Trials Office, Department of Pediatrics, Cincinnati Children's Hospital Medical Center; David Pallares, MD, Clinical Assistant Professor, Department of Pediatrics, Division of Allergy and Immunology, University of Louisville; and Robert J Arceci, MD, PhD, King Fahd Professor, Division of Pediatric Oncology, Johns Hopkins University School of Medicine

Author's Email:	Joseph L Lasky III, MD
Editor's Email:	Samuel Gross, MD

eMedicine Journal, January 16 2007, VOLUME 8, Number 1

INTRODUCTION

Section 2 of 12

Background: Craniopharyngiomas are histologically benign neuroepithelial tumors of the CNS that are predominately observed in children aged 5-10 years. These tumors arise from squamous cell embryologic rests found along the path of the primitive adenohypophysis and craniopharyngeal duct. Although histologically benign, these tumors frequently recur after treatment. In addition, because they originate near critical intracranial structures (eg, visual pathways, pituitary gland, hypothalamus), both the tumor and complications of curative therapy can cause significant morbidity. These characteristics have led to various treatment approaches, and disagreement continues regarding optimal treatment in children with this disease. Evidence suggests that adult craniopharyngiomas are histologically and biologically different from pediatric craniopharyngiomas; however, only childhood craniopharyngiomas are discussed in this article.

Pathophysiology: Pediatric craniopharyngiomas are believed to arise from cellular remnants of the Rathke pouch, which is an embryologic structure that forms both the infundibulum and anterior lobe of the pituitary gland. Its path of development extends from the pharynx to the floor of the sella turcica; not surprisingly, these tumors have been identified extensively in suprasellar, parasellar, and ectopic locations. Typically, the tumors arise within the sella or adjacent suprasellar space. Symptoms are caused by mass effects on adjacent normal intracranial structures.

Frequency:

• In the US: Craniopharyngiomas are relatively rare, representing 6-10% of intracranial malignancies in children and adolescents (approximately 2-3 cases per 1,000,000 children). A bimodal distribution peak has been reported, with one peak at age 5-14 years and the other at age 65-74 years. More than 300 cases are reported in the United States per year, and roughly one third of these involve children aged 0-14 years. One half of childhood tumors that occur in the sella-chiasmatic region are craniopharyngiomas.

Mortality/Morbidity: Previous studies have shown relatively good outcomes, with 10-year overall survival rates of 86-100% among patients who underwent gross total resection. Subtotal resection or recurrence treated with surgery and radiation therapy carry 10-year overall survival rates of 57-86%. In recent series, the surgical mortality rate after primary surgical intervention has been reported to be 0-3%. However, the mortality rate after re-resection can be as high as 25%.

Presenting morbidities include the following:

• Visual loss: Anterior extension to the optic chiasm can result in a classic bitemporal hemianopsia, unilateral temporal hemianopsia, papilledema, or unilateral/bilateral decrease in visual acuity. Children are frequently inattentive to visual loss, and formal testing may be required.

• Endocrinologic derangement: This results from direct compression or destruction of the hypothalamus and pituitary stalk, leading to growth hormone deficiency (75%), thyroid-stimulating hormone deficiency (25-64%), adrenocorticotropic hormone deficiency (25-56%), and luteinizing hormone or follicle-stimulating hormone deficiency (40-44%). This can present as clinically significant short stature, hypothyroidism, and other signs of panhypopituitarism.

• Diabetes insipidus: This is reported to occur in 9-17% of patients prior to surgery and in 66-100% of patients postoperatively.

Race: No clear racial predilection has been reported.

Sex: The most recent large series demonstrate equal sex distribution, although a slight male preponderance has been historically reported.

Age: Peak incidence of childhood craniopharyngiomas occurs in individuals aged 5-10 years. Neonatal craniopharyngiomas are rare. Of the more than 300 cases per year in the United States, approximately one third involve children aged 0-14 years.

CLINICAL

Section 3 of 12

History: Craniopharyngiomas produce symptoms by compression of adjacent neural structures. They can become quite large, obstructing cerebral spinal fluid 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:


• • 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 the ventromedial hypothalamus can result in a dysregulation of energy balance and resultant "hypothalamic obesity" upon presentation.
  
  
  
  • Symptoms of growth hormone deficiency (ie, short stature): Growth hormone deficiency is the most common possible endocrinologic disturbance caused by craniopharyngiomas. One series reported that growth failure preceded the diagnosis at a mean of 4 years.
  
  
  
  • Symptoms of hypothyroidism
    • Weight gain

    • Lethargy

    • Fatigue

    • Cold intolerance

    • Dry skin

    • Dry brittle hair

    • Slow teething

    • Anorexia

    • Large tongue

    • Deep voice

    • Myxedema
  
  
  
  • Symptoms of adrenal insufficiency (ie, excessive thirst, frequent urination, fever): Secondary adrenal insufficiency is the second most common endocrinologic disturbance caused by craniopharyngiomas.
  
  
  
• 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.

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
    • 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
    • This 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: 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.

Causes:

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



• Little is also known regarding the genetic basis for craniopharyngiomas. Transformation of normal cells into neoplastic ones likely involves multiple genomic changes, including loss of tumor-suppressor genes, activation of oncogenes, and alterations in DNA repair and methylation mechanisms. Although these events have started to be elucidated for neuroepithelial neoplasms such as gliomas, little progress has been made in understanding these events in craniopharyngiomas. Inconsistent abnormalities in chromosomes 2 and 12 have been identified, as well as overexpression of prostaglandin H synthase-2. The significance of these findings remains to be determined.

DIFFERENTIALS

Section 4 of 12

Astrocytoma
Ependymoma
Neuroblastoma

Other Problems to be Considered:

Cerebral aneurysm
Meningioma
Oligodendroglioma
Optic pathway gliomas
Pineoblastoma
Pituitary adenoma
Primitive neuroectodermal tumor
Rathke cleft cyst

WORKUP

Section 5 of 12

Lab Studies:

• Serum electrolytes levels


• • These routine tests establish readiness for surgery.

  • Importantly, the endocrine dysfunction frequently associated can cause abnormalities in several of these test findings.
  
  
  
• Growth hormone levels, including thyroid-stimulating hormone/thyroid hormone levels, steroid hormone levels (cortisol), follicle-stimulating hormone/luteinizing hormone levels


• • Obtain these tests preoperatively as a baseline.

  • Obtain tests to allow for perioperative hormone replacement as necessary.
  
  
  

Imaging Studies:

• Skull radiography


• • Approximately 85% of craniopharyngiomas have calcifications above or within the pituitary fossa on plain radiographs of the skull.

  • Enlargement of the sella turcica can also be reliably identified.

  • If hydrocephalus is associated with a tumor in a young patient, split sutures may be observed.
  
  
  
• Head CT scan


• • This may be the only preoperative radiographic study needed because craniopharyngiomas are observed with mixed solid and cystic components, and the solid component is enhanced following the administration of intravenous contrast.

  • CT scan is better than MRI at revealing the common tumor-associated calcifications.

  • Peritumoral edema is rare.

  • Hydrocephalus is identified and readily characterized.
  
  
  
• Brain MRI


• • MRI is better than CT scan at determining the relationship of the tumor to adjacent normal structures.

  • As with CT scan, mixed solid and cystic components are identifiable, and multiple cysts are common.

  • The solid component of the lesion frequently enhances following intravenous contrast administration, and a smooth ring of enhancement of the cyst wall can also be present.

  • Craniopharyngiomas are usually sharply demarcated and smoothly marginated.

  • Distortion or obliteration of the third ventricle is common.

  • Frequent involvement of the optic chiasm is found.

  • Obtaining postoperative imaging within 48 hours after surgery to best distinguish residual tumor from postsurgical changes is important.
  
  
  

Other Tests:

• Preoperative intellectual or psychological assessment may be useful as a baseline examination prior to undertaking curative therapies.

Procedures:

• Angiography


• • Cerebral angiography can be useful in planning the surgical approach, although it has been largely replaced by MRI/magnetic resonance angiography (MRA) in most centers.
  
  
  
  • A vascular blush can be observed, although the tumor is not visible.
  
  
  

Microscopic examination of the solid components reveals an epithelial tumor with angulated columnar cells resting on a collagen basement membrane. Papillary structures are common, and calcification is nearly universal. Large tumors may induce an intense glial reaction and intensely adhere to the underlying normal brain.

Staging: Preoperative and postoperative MRIs of the brain are adequate staging modalities for most children with craniopharyngioma. The postoperative scan is important in assessing residual disease. Neuraxis dissemination does not occur; thus, full spinal evaluation is unnecessary in an asymptomatic patient.

TREATMENT

Section 6 of 12

Medical Care:

• Endocrine complications


• • Long-term hormone supplementation is the primary medical treatment associated with childhood craniopharyngiomas and includes the administration of intranasal vasopressin (desmopressin acetate [DDAVP]), corticosteroids, thyroid hormones, growth hormones, and sex hormones.
  
  
  
  • Perioperative care includes attention to frequently associated multiple hormone deficiencies.
  
  
  
  • Frequently, perioperative corticosteroid administration (stress doses) is required.
  
  
  
• Chemotherapy


• • The use of chemotherapy in the treatment of craniopharyngioma is still under investigation. The use of systemic cytotoxic chemotherapy has not been shown to be of benefit in this disease.

  • Systemic use of the immunostimulatory agent interferon alfa has been attempted in a clinical trial. The rationale for use of this agent was based on the similar epithelial origin of craniopharyngiomas and squamous cell carcinomas, in which interferon alfa has shown some efficacy. In a phase II trial, Jakacki et al (2000) used interferon alfa at a dose of 8 million U/m² administered subcutaneously every day for a 16-week induction period and then the same dose 3 times/wk for 32 additional weeks in 15 patients with recurrent or progressive craniopharyngioma. Although an objective radiographic response was seen in only 3 of 12 patients who were able to be evaluated, the time until radiation therapy was required was delayed in those patients. However, 60% experienced moderately severe toxicities (eg, hepatic, neurologic, cutaneous), but these were all reversible with discontinuation or dose reduction.

  • Another chemotherapeutic modality that has been more thoroughly investigated is the use of intracavitary/intracystic bleomycin, an antibiotic that induces DNA strand breaks and acts as an antineoplastic agent. First reported in 1985, this therapy involves placement of an Ommaya-type catheter into the cyst cavity. After confirming no leakage from the cavity after dye instillation, administration of bleomycin can commence (eg, 5 mg every other day until a total cumulative dose of 30 mg). In their recently reported series, Takahashi et al (2005), reported regression of the cystic cavities in 10 of 11 patients, with minimal adverse effects. Intratumoral therapy has also been performed using interferon alfa, with similar results.

  • One report has described the use of carmustine (BCNU)–impregnated wafers (Gliadel: Guilford Pharmaceuticals, Inc; Baltimore, Md) in a patient with recurrent craniopharyngioma (Laws, 2003).

  • Given the morbidity of repeated surgeries for recurrent craniopharyngiomas after radiation therapy has failed, further research into other therapies for these locally invasive tumors is desperately needed.
  
  
  

Surgical Care: Treatment options include radical surgery, conservative surgery with postoperative radiotherapy, and palliative surgery. Although reports suggest management of craniopharyngiomas with limited surgery or conventional external-beam radiotherapy alone, these methods are not widely used. Newer management options such as stereotactic radiosurgery or radiotherapy are promising but remain largely experimental.

• Radical surgery


• • Historically, initial management of craniopharyngiomas has been surgical. Unfortunately, true complete resection of these tumors is challenging, even for experienced neurosurgeons who operate on several children with craniopharyngiomas each year.

  • Frequently, these tumors densely adhere to the optic chiasm, pituitary stalk, and internal carotid artery, and they often invade the region of the third ventricle; therefore, not surprisingly, radical surgery frequently causes significant morbidity including panhypopituitarism, neurologic deficits (cranial nerve palsies, hemiparesis, aphasia), and visual field deficits or blindness.
  
  
  
  • The postoperative mortality rate currently ranges from 0-20%, with the most recent series reporting rates of approximately 5-10%.

  • Perhaps most disheartening, gross total resection does not prevent recurrence. Following radical resection, local relapses are described in 0-60% of patients.

  • One series reported that complete excision was achieved in only 63% of patients treated with radical surgery, and one half of the tumors believed to be completely excised subsequently recurred (Wen, 1989).
  
  
  
• Conservative surgery alone: Morbidity and mortality associated with radical surgery led neurosurgeons to attempt lesser resections; unfortunately, limited surgery alone resulted in worse local control (75-90% local progression is reported) and even greater morbidity because of the need for repeated resections for recurrences.



• Conservative surgery with postoperative radiotherapy


• • Because limited surgery does not prevent recurrences and radical surgery carries unacceptable morbidity and mortality, postoperative external-beam radiotherapy has been added to limited surgery in an effort to improve local control.

  • The literature seems to support this approach, with a reported long-term control of approximately 80-95% at 5-20 years and a low risk of long-term morbidity.

  • Risk of parenchymal brain injury or second malignancy caused by radiation therapy is estimated to be less than 1-2%.

  • Some advocate postoperative radiation even after gross total resections, particularly if residual calcifications are noted on postoperative imaging studies (this carries a poor prognosis).
  
  
  
  • In general, radiotherapy is administered using field arrangements similar to those used for pituitary adenomas (>2 fields, narrow margin around gross tumor volume). A dose response for craniopharyngiomas has been reported; thus, the total tumor dose is generally 5000-5500 cGy in 25-30 fractions.

  • Children younger than 3 years may not be candidates for such radiotherapy because they can develop unusually severe long-term adverse effects.
  
  
  
• Palliative surgery



• Conservative management with limited surgery and external-beam radiotherapy requires close monitoring of the neurologic status during treatment.



• During or soon after radiotherapy, craniopharyngiomas can undergo cystic degeneration, which can lead to obstruction of cerebrospinal fluid outflow or compression of the optic apparatus, with potentially devastating consequences.

• Fortunately, early recognition and appropriate surgical treatment followed by conventional full-dose radiotherapy are associated with good long-term outcome.

Consultations:

• Obtain consultations from the following:


• • Pediatric neurosurgeon
  
  
  
  • Radiation oncologist
  
  
  
  • Pediatric endocrinologist
  
  
  
  • Pediatric hematologist or oncologist
  
  
  

Diet:

• Attention to special neurologic and endocrinologic concerns is prudent. Weight gain can be dramatic and a significant long-term problem. Nutritional consultation can be helpful.

MEDICATION

Section 7 of 12

Drug therapy currently is not usually a component of standard care for this condition. However, altered CNS hormone regulation may result following treatment with surgery and radiation, requiring long-term hormonal therapy. Endocrinologic consultation is recommended.

Drug Category: Chemotherapeutic agents -- Immunostimulatory therapies with interferon and intracystic/intratumoral injection of chemotherapeutic agents (eg, bleomycin) are occasionally used in cases of recurrent disease.

Drug Name	Bleomycin (Blenoxane) -- Peptide antibiotic that acts to inhibit DNA, RNA, and protein synthesis through the induction of DNA strand breaks.
Pediatric Dose	Various protocols exist for intracystic administration; one example is as follows: 5 mg in 5 mL 0.9% NaCl instilled into the Ommaya reservoir qod until a cumulative dose of 30 mg is achieved
Contraindications	Documented hypersensitivity
Interactions	Coadministration with cimetidine may increase toxicity; coadministration with etoposide may cause severe hepatic dysfunction, hyperbilirubinemia ascites, or thrombocytopenia
Pregnancy	D - Unsafe in pregnancy
Precautions	The most serious complications are pulmonary fibrosis and impaired lung function; other adverse effects (typically seen with systemic administration) include fever, rash, hyperpigmentation, alopecia, Raynaud phenomenon, or ototoxicity With intracystic therapy, the only adverse effect observed frequently was headache following instillation (however, chemical meningitis can result if leakage occurs)

Drug Name	Interferon alfa2-a (Roferon-A) -- Naturally occurring interferon alpha is normally secreted by B cells and T cells in response to viral or bacterial infection. The manufactured form (ie, interferon alfa) is a protein product manufactured using recombinant DNA technology. Mechanism of antitumor activity is not clearly understood; however, direct antiproliferative effects against malignant cells and modulation of host immune response may play important roles.
Pediatric Dose	Limited data exist Induction: 8 million units/m2 SC qd for 16 wk Maintenance: 8 million U/m2/d SC 3 times/wk for 32 more wk following induction completion
Contraindications	Documented hypersensitivity; avoid in patients who have anaphylactic sensitivity to mouse immunoglobulin (IgG), egg protein, or neomycin; autoimmune hepatitis
Interactions	Theophylline may increase interferon alpha toxicity; cimetidine may increase antitumor effects; zidovudine and vinblastine may increase toxicity of interferon alfa
Pregnancy	C - Safety for use during pregnancy has not been established.
Precautions	Caution in brain metastases, severe hepatic or renal insufficiencies, seizure disorders, multiple sclerosis, or compromised CNS; may cause fever, neutropenia, elevated levels of hepatic transaminases, fatigue, rash, or insomnia; depression and suicidal ideation may be side effects of treatment

FOLLOW-UP

Section 8 of 12

Further Outpatient Care:

• Endocrine: Close monitoring of growth, pubertal development, and any known hormone abnormalities is essential.



• Oncologic


• • Routine neuroimaging is the mainstay of follow-up assessment.
  
  
  
  • Obtain brain MRI every 3 months for 1 year, followed by every 6 months for 1 year, and then annually until year 5.
  
  
  
• Ophthalmologic


• • Routine ophthalmology evaluations are recommended.
  
  
  
  • In particular, new visual field deficits may hallmark disease progression.
  
  
  
• Neuropsychiatric


• • Periodic formal assessment can be useful when monitoring the long-term sequelae of surgery or radiotherapy.
  
  
  
  • In formal assessment, include comparison with preoperative and postoperative evaluations.
  
  
  

In/Out Patient Meds:

• Long-term hormone supplementation is the primary medical treatment associated with childhood craniopharyngiomas and includes the following:


• • Intranasal vasopressin
  
  
  
  • Corticosteroids
  
  
  
  • Thyroid hormones
  
  
  
  • Growth hormones
  
  
  
  • Sex hormones
  
  
  

Complications:

• Endocrine morbidity


• • The need for hormone replacement is common (approximately 80%) following therapy for craniopharyngiomas.
  
  
  
  • Diabetes insipidus is one of the least common (6-10%), but most serious, endocrinologic disturbances. Children with diabetes insipidus can become dehydrated during episodes of illness, and their summer activity must frequently be curtailed; in addition, vasopressin administration is expensive.
  
  
  
  • Hypothyroidism, growth hormone deficiencies, steroid hormone deficiencies, and delayed or precocious puberty have also been reported. Pay particular attention to these children when they develop significant systemic illnesses; stress doses of steroids may be required.
  
  
  
  • Severe endocrine abnormalities are associated almost exclusively with radical surgery.
  
  
  
• Neurologic morbidity: This is also frequently associated with attempts at radical tumor resection, visual loss, and hypothalamic injury (morbid obesity, hypersomnolence), which may develop following therapy for craniopharyngiomas.



• Neuropsychological morbidity


• • Memory loss, behavior changes, and academic decline can result directly from neurologic damage.
  
  
  
  • These neuropsychological changes can also occur indirectly as a result of other complications of therapy (eg, endocrinologic derangement).
  
  
  

Prognosis:

• Previous studies have shown relatively good outcomes, with 10-year overall survival rates of 86-100% among patients who underwent gross total resection. Subtotal resection or recurrence treated with surgery and radiation therapy carry 10-year overall survival rates of 57-86%. In recent series, the surgical mortality rate after primary surgical intervention has been reported to be 0-3%. However, mortality rate after re-resection can be as high as 25%. Gross total resection carries a greater risk of long-term neurologic and endocrinologic morbidity.

Patient Education:

• For additional information, see the People Living With Cancer article on craniopharyngioma

MISCELLANEOUS

Section 9 of 12

Medical/Legal Pitfalls:

• Pediatric tumors are rare, and one of their most common modes of presentation, increased intracranial pressure, can mimic many common childhood infectious diseases. The pediatrician who first observes children with seemingly routine nausea and vomiting should perform a reasonable screening neurologic examination. If papilledema is present in children with nausea, vomiting, and headaches, referral for further neurologic examination or neuroradiologic evaluation is suggested.

TEST QUESTIONS

Section 10 of 12

CME Question 1: Which of the following complications usually does not develop in individuals with craniopharyngiomas?

A: Visual field deficits
B: Hemiparesis
C: Hypothyroidism
D: Growth hormone deficiency
E: Diabetes insipidus

The correct answer is B: Hemiparesis is an uncommon long-term complication of therapy for craniopharyngiomas. Endocrine and visual disturbances are far more common, given the usual proximity of the tumor to visual pathways and the hypothalamic-pituitary axis.

CME Question 2: Which of the following is usually not considered an accepted component of the management of pediatric craniopharyngiomas?

A: Observation with serial neuroimaging to monitor for progression
B: Radical tumor resection
C: Conservative surgery with postoperative radiotherapy
D: Endocrinologic evaluation with hormonal supplementation
E: Palliative surgery (eg, shunting for hydrocephalus)

The correct answer is A: Serial observation of these tumors is generally considered inappropriate because they carry a significant risk of local morbidity, including blindness and endocrine derangement; in addition, these tumors, are readily curable, particularly when diagnosed early.

Pearl Question 1 (T/F): Craniopharyngiomas are most commonly located in the posterior fossa.

The correct answer is False: Craniopharyngiomas are usually located in the parasellar region.

Pearl Question 2 (T/F): Craniopharyngiomas are most commonly observed in children aged 5-10 years.

The correct answer is True: Craniopharyngiomas are most commonly observed in children aged 5-10 years, although they can present at virtually any age. Interestingly, these tumors are very rare in neonates.

Pearl Question 3 (T/F): The most common imaging studies used to diagnose or evaluate craniopharyngiomas include pneumoencephalogram and myelogram.

The correct answer is False: The most common imaging studies used to diagnose or evaluate craniopharyngiomas include skull radiography, head CT scan, and brain MRI.

Pearl Question 4 (T/F): Using postoperative radiotherapy as opposed to radical surgery in patients with craniopharyngiomas has no advantage.

The correct answer is False: Conservative surgery with postoperative radiotherapy avoids the significant risk of morbidity and mortality associated with radical surgery.

PICTURES

Section 11 of 12

Caption: Picture 1. 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.
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Picture Type: MRI
Caption: Picture 2. 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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Picture Type: CT

BIBLIOGRAPHY

Section 12 of 12

• Allen ED, Byrd SE, Darling CF, et al: The clinical and radiological evaluation of primary brain tumors in children, Part I: Clinical evaluation. J Natl Med Assoc 1993 Jun; 85(6): 445-51[Medline].
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