Brain, Adamantinomatous craniopharyngioma [LMP36172]

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Diagnostic Modality: 
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Disease Category: 
Neoplasia
Diagnosis: 
Adamantinomatous craniopharyngioma
Species: 
Human
Organ System: 
Central Nervous System
Gender: 
Male
Age: 
22wk fetus
Diagnosis Code: 
ICD-10: D44.4 | ICD-O: 9351/1
Slide Contributor Information
Gao, A., Shannon, P.

Keywords

fetal, congenital brain tumour

Clinical History

Termination of pregnancy of male fetus at 22 weeks’ gestational age due to intracranial mass detected on prenatal ultrasound.

Clinical Discussion

Adamantinomatous craniopharyngiomas are benign tumours of the sellar/suprasellar region occurring in children and middle-aged adults classified as WHO grade I. Exceptionally, fetal or neonatal presentations occur. Microscopically, adamantinomatous craniopharyngiomas are characterized by irregularly arranged squamous epithelium bordered by a palisade of columnar epithelium; loosely cohesive aggregates of squamous cells known as stellate reticulum; and nodules of “wet keratin” consisting of anuclear squamous cells. It is associated with CTNNB1 (beta-catenin) mutations. Although benign, prognosis is dependent on size and extent of surgical resection; in congenital cases, tumours are usually large and carry a poor prognosis.

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