Cancer-associated genodermatoses and familial cancer syndromes with cutaneous manifestations.

Clin Dermatol

GeneDx, Inc., Rockville, Maryland 20830, USA.

Published: September 2001

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http://dx.doi.org/10.1016/s0738-081x(01)00175-4DOI Listing

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Article Synopsis
  • Skin cancers often relate to various genodermatoses, but current knowledge primarily reflects presentations in White patients, lacking sufficient focus on skin-of-color (SOC) individuals.
  • The study analyzed 381 articles involving 578 SOC patients to uncover notable differences in the manifestation of skin cancer-related findings and age of onset based on genodermatoses.
  • Findings indicated SOC patients may display fewer classic signs of conditions like Gorlin syndrome and experience varying onset ages for skin cancers, highlighting the need for improved awareness to enhance early diagnosis and treatment.
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[Cancer-associated genodermatoses].

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Hereditary tumor syndromes are characterized by a familial occurrence of tumors/cancer. A hereditary tumor syndrome should be suspected if a familial occurrence of cancer is seen and/or persons at younger age are affected. Some of the currently known tumor syndromes are associated with specific skin symptoms that can aid the physician in establishing the correct diagnosis.

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Skin cancer has become the most common type of cancer worldwide as a result of environmental exposure and medical treatments. A small group of patients are genetically predisposed to skin cancer and this article is intended as a diagnostic tool when encountering patients with multiple skin cancer lesions. The disorders are described with clinical characteristics, genetics and management.

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PDT is widely applied for the treatment of non-melanoma skin cancer pre-malignant and malignant lesions (actinic keratosis, basal cell carcinoma and squamous cell carcinoma). In photodynamic therapy (PDT) the interaction of a photosensitizer (PS), light and oxygen leads to the formation of reactive oxygen species (ROS) and thus the selective tumor cells eradication. Xeroderma pigmentosum (XP) and Gorlin-Goltz Syndrome (GS) patients are at high risk of developing skin cancer in sun-exposed areas.

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Acquired segmental neuromas.

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Department of Dermatology, School of Medicine, University of Puerto Rico Medical Sciences Campus, San Juan, Puerto Rico.

Neuromas are benign hyperplastic proliferations of normal peripheral nerve components. These can be associated with some genodermatoses, namely Cowden syndrome and multiple endocrine neoplasia (MEN) 2B, especially when numerous lesions are present. Given the potential for malignancy (such as breast cancer, pheochromocytoma, and thyroid cancer) associated with these syndromes, other features indicative of either syndrome should be evaluated in patients presenting with multiple cutaneous neuromas.

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