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Neuroradiology Case of the Week

Case 347

September 2008

Hisao Nakamura, MD

Clinical Presentation: Patient is an 8-year-old child who presented with history of headache, nausea and vomiting. MRI of the brain is requested to evaluate for the cause of headache.

Imaging Findings: Suprasellar cystic multilobulated mass - craniopharyngioma is most likely.

Diagnosis: Adamantinous craniopharyngioma

Discussion: Differential diagnosis includes craniopharyngioma and hypothalamic glioma. Considering the fact that the supersellar mass shows mild enhancement, craniopharyngioma is more likely. Pathology demonstrates adamantinous craniopharyngioma. Craniopharyngiomas derive from remnants of the craniopharyngeal duct. They may arise anywhere from the infundibular stalk from the floor of the third ventricle to the pituitary gland. The prevalence peaks between 10 to 14 years, with a second peak occurring in the fourth to sixth decades of life. They are divided histologically into two types: adamantinous (pediatric) and papillary (adult) type. Pediatric type such as this case appears on MR imaging as predominantly multicystic masses. The cystic areas may be iso, hyper, or hypointense relative to brain tissue with T1-weighted images. The short T1 relaxation times are the result of very high protein content. With T2-weighted images, both cystic and solid components tend to have high signal intensity. The solid portions enhance heterogeneously after the contrast. The thin walls of the cystic areas nearly always enhance. It has been postulated that lobulated craniopharyngiomas with large, hyperintense cysts on T1-weighted MR images are adamantinous, whereas the smaller, round, primary solid craniopharyngiomas with hypointense cyst on T1-weighted images have papillary histologic features.

References:

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2. Sartoretti-Schefer S, Wichmann W, Aguzzi A, Valavanis A. MR differentiation of adamantinous and squamous-papillary craniopharyngiomas. AJNR Am J Neuroradiol. 1997 Jan;18(1):77-87. [PubMed]
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