Literature DB >> 33661338

[Cancer-associated genodermatoses].

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


  2 in total

Review 1.  Birt-Hogg-Dubé syndrome: an underdiagnosed genetic tumor syndrome.

Authors:  Ortrud K Steinlein; Birgit Ertl-Wagner; Thomas Ruzicka; Elke C Sattler
Journal:  J Dtsch Dermatol Ges       Date:  2018-03       Impact factor: 5.584

2.  Muir-Torre syndrome: a rare but important disorder.

Authors:  Holly H Hare; Neetu Mahendraker; Sandhya Sarwate; Krishnarao Tangella
Journal:  Cutis       Date:  2008-10
  2 in total

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