Abstract
Genodermatoses, while rare, are an important and unique aspect of pediatric dermatology. In this chapter, we will introduce genodermatoses that are associated with malignancies and review how they typically present. We will emphasize genodermatoses with onset of cutaneous malignancy in childhood (younger than 21 years of age) and provide a summary of those correlated with internal malignancy. The chapter is divided into four main sections: genodermatoses associated with (1) primary cutaneous malignancies (BCC, SCC, and melanoma), (2) photosensitivity-induced cutaneous malignancies, (3) other causes of secondary cutaneous malignancies, and (4) extracutaneous malignancies. For each main genodermatosis, we will discuss brief background of disease pathogenesis (if known), characteristic skin findings, associated malignancies, and basic management strategies. Awareness and recognition of malignancy-associated genodermatoses are critical for correct and timely diagnosis, as well as implementation of appropriate screening for patients.
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Recommended Reading
Jaju PD, Ransohoff KJ, Tang JY, Sarin KY. Familial skin cancer syndromes: increased risk of nonmelanotic skin cancer and extracutaneous tumors. J Am Acad Dermatol. 2016;74(3):437–51.
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Spitz JL. Chapter 5: Disorders with malignant potential. Genodermatoses: a full-color clinical guide to genetic skin disorders. New York: Williams & Wilkins; 1996. p. 149–67.
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Robinson, S.N., Song, H., Huang, J.T. (2018). Malignancy-Associated Genodermatoses. In: Huang, J., Coughlin, C. (eds) Skin Tumors and Reactions to Cancer Therapy in Children. Springer, Cham. https://doi.org/10.1007/978-3-319-66200-8_5
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