Craniopharyngiomas in two consanguineous siblings: case report.

Boch, A L; van Effenterre, R; Kujas, M · Neurosurgery · 1997

case_report · Level V

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Abstract

We describe a double case of craniopharyngioma in consanguineous siblings, suggesting the disease is sometimes genetic. 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. 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. 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.

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