Which genetic condition features lesions associated with a risk of malignancy, particularly CNS tumors?

Von Recklinghausen disease, also known as neurofibromatosis type 1 (NF1), is characterized by the development of neurofibromas, café-au-lait spots, and other lesions that can appear throughout the body. One of the significant concerns associated with NF1 is an increased risk of various tumors, particularly central nervous system (CNS) tumors, such as gliomas and other malignant lesions.

Individuals with NF1 have mutations in the NF1 gene, which plays a crucial role in regulating cell growth and differentiation. The loss of function of this gene leads to the formation of tumors throughout the nervous system and other tissues. This genetic disorder is significant not only for its physical manifestations but also for its association with malignancies, making screening and monitoring for CNS tumors particularly important in affected individuals.

In contrast, other conditions mentioned here do not primarily focus on central nervous system tumors or involve a similar risk profile. For instance, Peutz-Jeghers syndrome leads to gastrointestinal polyps and an increased risk of certain gastrointestinal cancers rather than CNS tumors specifically. Chronic lymphocytic leukemia is a hematological malignancy, and Alport syndrome primarily affects the kidneys and can lead to renal failure but does not prominently feature CNS tumors or associated lesions