Literature DB >> 7116263

Gorlin's syndrome, or nevoid basal cell carcinoma syndrome.

P J Fitzpatrick, G A Thompson.   

Abstract

Gorlin's syndrome is a condition inherited in an autosomal dominant fashion. It involves many organs, but principally affects the skin, skeleton, and endocrine and nervous systems. The most common features are multiple nervi and basal cell carcinomas of the skin, benign jaw cysts, dyskeratotic pits in the palms and soles, rib and vertebral abnormalities, brachymetacarpalism, and calcification of the falx cerebri. In 14 patients, 4 of whom belonged to one family, the age at the time of diagnosis ranged from 11 to 63 years. Ten patients are alive, but five are severely disfigured by carcinomas. Two patients died of complications resulting from uncontrolled tumours, and two died of other cancers. New skin tumours constantly develop; small ones can be excised, but large ones require extensive surgery with or without radiotherapy.

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Mesh:

Year:  1982        PMID: 7116263      PMCID: PMC1862096     

Source DB:  PubMed          Journal:  Can Med Assoc J        ISSN: 0008-4409            Impact factor:   8.262


  7 in total

1.  MULTIPLE BASAL-CELL NEVI SYNDROME. AN ANALYSIS OF A SYNDROME CONSISTING OF MULTIPLE NEVOID BASAL-CELL CARCINOMA, JAW CYSTS, SKELETAL ANOMALIES, MEDULLOBLASTOMA, AND HYPORESPONSIVENESS TO PARATHORMONE.

Authors:  R J GORLIN; R A VICKERS; E KELLEN; J J WILLIAMSON
Journal:  Cancer       Date:  1965-01       Impact factor: 6.860

2.  Parathyroid hormone hyporesponsiveness in patients with basal-cell nevi and bone defects.

Authors:  J B BLOCK; W E CLENDENNING
Journal:  N Engl J Med       Date:  1963-05-23       Impact factor: 91.245

3.  Multiple nevoid basal-cell epithelioma, jaw cysts and bifid rib. A syndrome.

Authors:  R J GORLIN; R W GOLTZ
Journal:  N Engl J Med       Date:  1960-05-05       Impact factor: 91.245

4.  The basal-cell nevus: its relationship to multiple cutaneous cancers and associated anomalies of development.

Authors:  J B HOWELL; M R CARO
Journal:  AMA Arch Derm       Date:  1959-01

5.  Nevoid basal cell carcinoma syndrome.

Authors:  R Jackson; S Gardere
Journal:  Can Med Assoc J       Date:  1971-10-23       Impact factor: 8.262

6.  Linkage analysis of the nevoid basal cell carcinoma syndrome.

Authors:  D E Anderson
Journal:  Ann Hum Genet       Date:  1968-10       Impact factor: 1.670

7.  Multiple basal cell naevus syndrome in ancient Egypt.

Authors:  M I Satinoff; C Wells
Journal:  Med Hist       Date:  1969-07       Impact factor: 1.419

  7 in total
  4 in total

1.  Genetic study of a family suffering from Gorlin's syndrome.

Authors:  X X Liu; Z R Yang; T E Mao; S W Qiang
Journal:  J Tongji Med Univ       Date:  1986

2.  Congenital hydrocephalus and the basal cell nevus syndrome.

Authors:  B A Lycka; V R Chichak
Journal:  Can Med Assoc J       Date:  1985-05-01       Impact factor: 8.262

3.  Gorlin's syndrome.

Authors:  J E MacSweeney; A Forbes; A R Manhire; W R Lees
Journal:  J R Soc Med       Date:  1985-03       Impact factor: 5.344

4.  Nevoid Basal Cell Carcinoma Syndrome: A Long-Term Study in a Family.

Authors:  Thiago de Santana Santos; André Vajgel; Paulo Ricardo Saquete Martins-Filho; Almir Walter de Albuquerque Maranhao Filho; Ricardo José De Holanda Vasconcellos; Riedel Frota; José Rodrigues Laureano Filho
Journal:  Craniomaxillofac Trauma Reconstr       Date:  2015-08-03
  4 in total

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