Basal Cell Nevus Syndrome Showing Several Histologic Types of Basal Cell Carcinoma
Annals of Dermatology
; : S36-S40, 2011.
Article
in En
| WPRIM
| ID: wpr-190418
Responsible library:
WPRO
ABSTRACT
Basal cell nevus syndrome (BCNS), or Gorlin Syndrome, is an autosomal dominant disorder, characterized by multiple developmental abnormalities and associated with germline mutations in the PTCH gene. Patients show multiple and early onset basal cell carcinomas (BCCs) in skin, odontogeniccysts in the jaw, pits on palms and soles, medulloblastoma, hypertelorism, and calcification of the falx cerebri. Clinical features of BCCs in these patients are indistinguishable from ordinary BCCs. However, some patients show variable histologic findings in subtypes of BCCs, and only one case associated with several histologic types of BCCs in the syndrome has been reported in Korea. We present a case of BCNS characterized by multiple BCCs, odontogenic keratocysts, multiple palmar pits, and calcified falx cerebri. Histopathologic findings of BCCs showed several patterns, which were nodular, superficial, and pigmented types.
Key words
Full text:
1
Database:
WPRIM
Main subject:
Skin
/
Carcinoma, Basal Cell
/
Basal Cell Nevus Syndrome
/
Odontogenic Cysts
/
Germ-Line Mutation
/
Hypertelorism
/
Jaw
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Korea
/
Medulloblastoma
/
Neoplasms by Histologic Type
Limits:
Humans
Country/Region as subject:
Asia
Language:
En
Journal:
Annals of Dermatology
Year:
2011
Document type:
Article