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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http://dx.doi.org/10.2214/AJR.17.18404DOI Listing

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