Muir-Torre syndrome is a predictor of gastric cancer (clinical observation)

Abstract

Muir-Torre syndrome is a hereditary autosomal dominant syndrome that belongs to HNPCC (Lynch syndrome), being its subspecies. The disease is associated with a high risk of developing colorectal cancer, as well as other cancers of the gastrointestinal tract, genitourinary tract and skin lesions in the form of tumors of the sebaceous glands and keratoacanthus. Visceral cancer in the syndrome is characterized by multiple localization and a low degree of malignancy. Mutations in the genes MSH2 and MLH1 - loci 2p22–p21 and 3p21.3 in the DNA repair system were detected in patients with Muir-Torre syndrome, which leads to its microsatellite instability. Skin tumors may precede visceral cancer, may develop in parallel with the malignant process of internal organs. The importance of timely diagnosis of skin neoplasms (sebaceous gland adenomas and keratoacanthus) as a marker of Muir-Torre syndrome is emphasized. The article presents a clinical case of Muir-Torre syndrome in a 50-year-old patient, which was manifested by a combination of gastric cancer, multiple adenomas of the sebaceous glands and solitary keratoacanthoma. The first manifestations of the syndrome were adenomas of the sebaceous glands of the facial skin, mistakenly regarded by dermatologists as a papular form of rosacea. After 4 years, the patient underwent surgery for a keratoacanthoma of the shin, a couple of months later, the patient was diagnosed with stage IIA gastric cancer (T3N0M0), a ring-cell variant. Skin rashes were regarded as hereditary paraneoplastic Muir–Torre syndrome. In order to detect oncological diseases early, it is necessary to improve the qualifications of doctors in the clinic and diagnosis of hereditary paraneoplastic dermatoses.