Abdominal Imaging Findings in Neurocutaneous Syndromes: Looking Below the Diaphragm.

AMERICAN JOURNAL OF ROENTGENOLOGY(2017)

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摘要
OBJECTIVE. Neurocutaneous syndromes are a heterogeneous group of multisystemic disorders. We review the multimodality imaging findings in the disorders with a propensity for intraabdominal disease. We highlight more recently recognized disease patterns and discuss imaging surveillance optimization. CONCLUSION. Knowledge of varied intraabdominal phenotypic expressions can increase diagnostic rates. Limitation of radiation dose must be considered in screening this tumor-prone population. Early detection of neoplastic findings is critical for morbidity and mortality reduction.
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关键词
Birt-Hogg-Dube syndrome,Cowden syndrome,neurocutaneous syndromes,neurofibromatosis type 1,phakomatoses,tuberous sclerosis complex,von Hippel-Lindau syndrome
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