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Craniopharyngiomas in two consanguineous siblings: case report
A L Boch1, R van Effenterre, M Kujas
1Service de Neurochirurgie, Groupe Hospitalier Pitié-Salpêtrière, Paris, France.
Objective And Importance:
We describe a double case of craniopharyngioma in consanguineous siblings, suggesting the disease is sometimes genetic.
Clinical Presentation:
Two typical adamantine craniopharyngiomas were observed in two consanguineous siblings. The brother and the sister, whose parents were first cousins, developed the tumors at the same age.
Intervention:
The male patient was operated on using a frontopterional approach, and the tumor was completely resected. The patient remained free from recurrence 9 years after surgery. His older sister died after tumor removal was attempted at another institution.
Conclusion:
To our knowledge, such a connection has never been reported in the literature. It suggests that craniopharyngioma, which is usually sporadic, can also be transmitted in an autosomal recessive manner.