PEDIATRIC RADIOLOGICAL CASE Craniopharyngioma

Craniopharyngioma
Alexandra N Dominianni, Richard B Towbin, MD; Carrie M Schaefer, MD; Alexander J Towbin, MD; David J Aria, MD

Figure 1. Head CT demonstrates a round, hypodense suprasellar lesion with comma-

Case Summary shaped calcifications, which are larger on the left than on the right. The lesion exerts
minimal mass effect on the posterior aspect of the anterior interhemispheric fissure
A previously healthy 13-year-old and the anterior midbrain.
presented with headaches, visual im-
pairment, and new onset of seizures.

Imaging Findings
Head CT (Figure 1) demonstrated
a large, hypodense, suprasellar mass.
The lesion had a large, rounded
hypodense component that exerted
mass effect on adjacent structures,
including the posterior aspect of
the anterior interhemispheric
fissure and the midbrain. The lesion
was bracketed by comma-shaped
calcifications laterally. Brain MRI
(Figure 2) revealed a large suprasel-
lar mass consisting of cystic and
solid components. The solid tissue in
the rim of the lesion enhanced after
contrast injection.

Diagnosis
Craniopharyngioma. The differ-
ential diagnosis of an intrasellar/
suprasellar mass in childhood
includes craniopharyngioma, Rathke
cleft cyst, pituitary macroadenoma,
and intracranial teratoma. Langer-
hans cell histiocytosis, lymphocytic

Affiliations: University of Arizona College of Medicine—

Phoenix (Ms Dominianni); Phoenix Children’s Hospital
(Drs R Towbin, Schaefer, Aria); Cincinnati Children’s Hospital
and Cincinnati College of Medicine (Dr A Towbin)

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 Craniopharyngioma PEDIATRIC RADIOLOGICAL CASE

Figure 2. (A) Sagittal T1 image demonstrates a large, heterogeneous lesion originating in the sella turcica
and extending cephalad to compress and upwardly displace the fornix and third ventricle. The mass has
a hypointense component in the sella inferiorly and a mildly hyperintense larger component superiorly.
(B) Sagittal T1 contrast-enhanced image shows peripheral enhancement of the tumor. (C) Sagittal T2
image shows the T2 hyperintense inferior cystic component, and the superior, solid component, which is
slightly hypointense compared to the cystic portion.

A C

hypophysitis, and sarcoidosis may Headaches, caused by increased Children with large tumors can thus
involve the pituitary infundibulum. intracranial pressure, are present present with cognitive and function-
in 60-75% of patients and are one al deterioration typically manifest-
of the most common symptoms at ed as poor school performance or
Discussion
presentation.2 Visual deficits are the psychomotor decline.2 Importantly,
Craniopharyngiomas are rare second-most common presenting craniopharyngiomas are slowly
tumors, with an incidence of 0.13- symptoms, occurring in more than growing tumors. Thus, symptom
0.18 cases per 100,000 in the US1 and 50% of patients. Children will often onset can be insidious, leading to
a bimodal age distribution. Peak report being unable to see the board delayed diagnosis. Presentation
incidence in children ranges from at school or the television at home.2 also tends to vary by age — younger
6 to 15 years of age, and in adults Endocrine abnormalities are pres- children manifest with symptomatol-
over 40 years. Craniopharyngiomas ent in 20 to 50 % of patients and can ogy of elevated intracranial pressure,
account for 6 to 9% of all pediatric include diabetes insipidus, obesity, whereas adolescents present more
brain tumors.1 Given their typical lo- growth retardation, and delayed commonly with hypopituitarism and
cations within the sella turcica with puberty related to compression visual deficits.
suprasellar extension, these tumors and/or invasion of the hypothalamic- Imaging is critical in evaluating
are especially challenging to resect pituitary stalk.2 As the tumor grows, craniopharyngiomas, specifically
and can cause significant neurologi- it can cause frontal lobe compression with regard to tumor location and
cal complications. and/or obstructive hydrocephalus. adjacent structure involvement.

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PEDIATRIC RADIOLOGICAL CASE Craniopharyngioma

These factors are important in determin- subtypes exhibit distinctly differ- resected without neurovascular injury
ing the best course of treatment. Tumor ent pathogenesis. and visual impairment. Limited
involvement of and/or proximity to the Childhood craniopharyngio- surgery and radiation therapy are
frontal and temporal lobes, ventricles, mas are nearly exclusively aCPs. appropriate for most patients includ-
optic nerves and chiasm, hypothala- These are hypothesized to develop ing those for whom radical surgery
mus, pituitary gland, circle of Willis, from neoplastic transformation of might be considered. The concept of
and brainstem all impact the treatment embryonic epithelial remnants of limited surgery and radiation therapy
plan.2 While MRI is generally accepted the Rathke pouch or the craniopha- involves the use of surgery to alleviate
as the best imaging modality to visualize ryngeal duct, the embryonic origin and prevent symptoms, improve a pa-
crucial information about craniopharyn- giving rise to the anterior pituitary tient’s ability to tolerate and complete
giomas, CT may be additive. as it ascends from the pharynx into irradiation, and potentially enhance
On CT, the tumor can have hetero- the future sella turcica region during or optimize dose delivery6.
geneous density with cystic and solid embryogenesis.5 In contrast, pCPs
components. Typically, the cystic com- are seen almost exclusively in adults,
Conclusion
ponents are dominant. Approximately presenting as solid tumors with little
90% of lesions contain calcifications mineralization.2 They are thought to Craniopharyngioma is the most
located peripherally. Secondary skull result from metaplasia of differenti- common non-glial intracranial
base changes such as enlargement of the ated pituitary cells. tumor of childhood. Because of the
sella turcica or erosion of the dorsum Craniopharyngiomas are generally tumor’s slow growth, the onset of
sellae can be seen.3 benign and rarely undergo malig- symptoms can be insidious. Patients
The MRI appearance may vary nant transformation.2 Both subtypes typically present with headaches,
depending on the cystic component. are considered by the World Health visual field defects, or endocrine
On T1-weighted sequences, the cystic Organization to be grade 1 tumors. anomalies. On imaging, the mass
components may be isointense to hyper- Nevertheless, their proximity to vital has variable cystic and solid areas.
intense to brain. Hyperintensity, when neurovascular structures causes Peripheral calcifications are often
present, results from the high protein symptoms that not only can lower present. One of the primary goals
content of the cyst fluid. On T2-weighted quality of life but can also be fatal. of imaging is to determine disease
sequences, the lesion has mixed hyperin- Complete surgical resection can be extent and tumor relationship to
tense signal. The solid portions of the tu- curative; however, treatment often vital neurovascular structures. While
mor enhance with injection of contrast.3 also incorporates irradiation. surgical resection and/or radiation
Magnetic resonance angiography may Surgical resection can be via a therapy can be curative, the central
be valuable to evaluate tumor proximity craniotomy or endoscopically. Given location in the brain of these tumors
to the internal carotid arteries and adja- the significant morbidity associated poses risks of long-term morbidity.
cent portions of the circle of Willis.3 with open approaches, the endo-
Magnetic resonance spectroscopy (MRS) scopic, endonasal/transsphenoi-
References
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