Article
Four generations of SDHB-related disease: complexities in management.
Familial cancer - 1 Apr 2017
Srirangalingam U, LeCain M, Tufton N, Akker S A, Drake W M, Metcalfe K
Abstract excerpt
SDHB mutations are linked to the familial paraganglioma syndrome type 4 (PGL4), which is associated with predominantly extra-adrenal disease and has high metastatic rates. Despite the lower penetrance rates in carriers of SDHB mutations compared to mutations in other paraganglioma susceptibility genes, the aggressive behavior of SDHB-linked disease warrants intensive surveillance to identify and resect tumors...
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