| Literature DB >> 33661338 |
L H Frommherz1, O K Steinlein2, L E French1, E C Sattler3.
Abstract
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. Examples are fibrofolliculoma in Birt-Hogg-Dubé syndrome, epidermal cysts, sebaceous cysts, neurofibroma in Gardner syndrome and sebaceous neoplasms or keratoacanthoma in Muir-Torre syndrome. If a genetic tumor syndrome is suspected, genetic testing and counselling should be performed in the index patient and is also recommended for family members. Affected patients should be offered regular clinical surveillance by the appropriate medical disciplines. Since curative therapy does not exist so far, preventive screening is of great importance.Entities:
Keywords: Birt-Hogg-Dubé syndrome; Carney complex; Cowden syndrome; Hereditary tumor syndrome; Muir-Torre syndrome
Mesh:
Year: 2021 PMID: 33661338 DOI: 10.1007/s00105-021-04779-4
Source DB: PubMed Journal: Hautarzt ISSN: 0017-8470 Impact factor: 0.751