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Published on: July 5, 2021
Congenital craniopharyngioma: a case report and literature review
Farida Chentli1, Faiza Belhimer, Farid Kessaci
1Department of Endocrine and Metabolic Diseases, Bab El Oued University Hospital, Algiers, Algeria. endofarida@hotmail.fr
Insights
Craniopharyngiomas, rare congenital tumors, can be life-threatening even if undiagnosed early. This case highlights a severe, rapidly progressing tumor in an infant, emphasizing the need for vigilant prenatal monitoring.
Area of Science:
- Pediatric Oncology
- Neuroscience
- Developmental Biology
Background:
- Craniopharyngiomas are congenital tumors rarely diagnosed prenatally.
- Large tumors can be debilitating and life-threatening, as illustrated by this case.
Observation:
- A 21-month-old girl presented with a diagnosed craniopharyngioma at 5 months due to neurological complications.
- Imaging revealed a large (5 cm), complex supra sellar mass causing hydrocephalus and brainstem displacement.
- Despite interventions, the patient experienced blindness, hemiparesis, seizures, and died at age 2.
Findings:
- A large head circumference at birth, significant tumor burden, blindness, and hemiparesis by 5 months were noted.
- These clinical signs suggested an antenatal origin for the craniopharyngioma.
Implications:
- This case underscores the potential for severe, undetected congenital craniopharyngiomas.
- Inadequate gestational monitoring may lead to delayed diagnosis and poor outcomes for life-threatening pediatric tumors.
Introduction:
Although craniopharyngiomas are congenital tumors, they are rarely diagnosed in the prenatal or early neonatal period. Sometimes they are very large, debilitating and life-threatening, as in the case discussed here.
Observation:
A 21-month-old girl was referred for craniopharyngioma that had been diagnosed at 5 months because of neurologic complications. The pregnancy and birth were considered to be unremarkable. The birth weight was 4 kg, length 53 cm, head circumference 38 cm (n=35±1). Brain CT revealed active hydrocephalus caused by a very large solid, cystic and calcified supra sellar mass. MRI confirmed the process measuring 5 cm in height, multidirectional, diving into pituitary sella, displacing the brainstem. After several resections and reservoir drainage, the patient was blind, hemiparetic and she convulsed frequently. The patient died when she was 2.
Conclusion:
The large head circumference at birth, the size of the tumor, blindness, and hemiparesis observed before the age of 5 months suggested an antenatal process unnoticed because of inadequate gestational monitoring.
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