BASAL CELL NEVUS SYNDROME A CASE REPORT

Authors

  • Suphaneewan Jaovisidha Division of Diagnostic Radiology, Department of Radiology, Faculty of Medicine, Ramathibodi Hospital, Mahidol University
  • Pimjai Siriwongpairat Division of Diagnostic Radiology, Department of Radiology, Faculty of Medicine, Ramathibodi Hospital, Mahidol University
  • Jaovisidha S. Division of Diagnostic Radiology, Department of Radiology, Faculty of Medicine, Ramathibodi Hospital, Mahidol University
  • Siriwongpairat P. Division of Diagnostic Radiology, Department of Radiology, Faculty of Medicine, Ramathibodi Hospital, Mahidol University

Abstract

The basal cell nevus syndrome is a multisystem disorder inherited in an autosomal dominant manner with marked penetrance and variable expression. Originally defined as the triad of nevoid basal cell carcinomas, jaw cysts, and skeletal anomalies. The syndrome complex has since been greatly expanded to include a variety of other organ system abnormalities. We recently had the opportunity to discover a family with this syndrome while evaluating a patient with atopic eczema, and describing hypopneumatization of mastoid antrum, as an associated finding in this syndrome which has not been documented before.

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References

Taybi H. Radiology of Syndromes. Chicago: Year Book Medical publishers Inc., 1975 26-27

Taybi H, Ralph SL. Radiology of Syndromes, Metabolic Disorders, and Skeletal Dysplasia. 3rd Ed. Chicago Year Book Medical Publishers Inc., 1990: 335-7

Bare JW, Lebo RV, Epstein EH Jr. Loss of heterozygosity at chromosome 1q22 in basal cell carcinoma and exclusion of the basal cell nevus syndrome gene from this site. Cancer-Res. 1992 Mar 15; 52(6): 1494-8

Battisti C, Palmeri S, Federico A. Oculo-dento-digital syndrome (Gorlin's sysdrome): clinical & genetical report of a new family. Acta Neurol Napoli 1992 Apr; 14(2): 103-10

Chenevix-Trench-G, Wicking C, Berkman J, et al. Further localization of the gene for basal cell carcinoma syndrome (NBCCS) in 15 Australasian families linkage and loss of heterozygosity. Am J Hum Genet 1993 Sep; 53(3): 760-7

Chenevix-Trench-G. Basal cell naevus sysdrome. Med J Just 1992 May 4; 156(9): 671-2

Coffin CM. Congenital cardiac fibroma associated. with Gorlin syndrome. Pediatr Pathol 1992 Mar-Apr; 12(2): 255-62

DiSanto S, Abt AB, Boal, et al. Fetal rhabdomyoma and nevoid basal cell carcinoma syndrome. Pediatr Pathol 1992 May-Jun; 12(3): 441-7

Evans DG, Ladusans EJ, Rimmer S, et al. Complication of the naevoid basal cell carcinoma syndrome : result of a population based study. J Med Genet 1993 Jun; 30 (6): 460-4

Farndon PA, Del-Mastro-RG, Evans DG, et al. Location of gene for Gorlin sysdrome. Lancet 1992 Mar 7; 339 (8793) 581-2

Fryer A. Odontogenic keratocysts do not occur in Noonan syndrome. Clin Dysmorphol Apr; 2 (2): 185-6

Gailani MR, Bale SJ, Leffell DJ, et al. Developmental defects in Gorlin syndrome related to a putative tumour suppressor gene on chromosome 9. Cell 1992 Apr 3; 69 (1): 111-7

Goldstein AM, Bale SJ, peck GL, et al. Sun exposure and basal cell carcinoma syndrome. J Am Acad Dermatol 1993 Jul; 29(1): 34-41

Gorlin RJ. Nevoid Basal-Cell Carcinoma Syndrome. Medicine 1987; 66 (2): 98-113

Horner K, Rushton V. Adontogenic keratocyst in an infant. Br Dent J 1992 Jul 25; 173 (2): 52

Khalique N, Rippin JW. Odontogenic keratocyst in an infant. Br Dent J 1992 Apr 11; 172 (7) 282-3

Kuster W, Happle R. Neurocutaneoius disorders in children. Curr Opin Pediatr 1993 Aug; 5 (4) 436-40

Mathur MN, Thompson JF, O'Brien CJ, et al. Naevoid basal cell carcinoma (Gorlin's) syndrome. Aust NZ J Surg 1993 May; 63(5): 413-5

Meyvisch K, Andre J, Song M, et al. Basal cell nevus syndrome and congenital hydrocephaly. Dermatology 1993; 186 (4): 311-2

Pilcher R. The basal cell naevus syndrome: a case in the Falkland Islands. J R Army Med Corps 1993 Feb; 139 (1): 20-4

Springate JE. The nevoid basal cell carcinoma syndrome. J pediatr Surg 1986 Oct; 21 (10): 908-10

Strange PR, Lang PG Jr. Long-term management of basal cell nevus syndrom with topical tretinoin and 5-fluorouracil. J Am Acad Dermatol 1992 Nov; 27 (5 Pt 2): 842-5

Theiler R, Hubscher E, Wagenhauser Fj, et al. (Diffuse idiopathic skeletal hyperostosis (DISH) and pseudo-coxarthritis following long-term etretinate therapy). Schweiz Med Wochenschr 1993 Apr 10; 123 (14) 649-53

Tokar IP, Fraser MC, Bale SJ. Genodermatoses with profound malignant potential. Semin Oncol Nurs 1992 Nov; 8 (4): 272-80

Tsuji T, Otake N, Nishimura M. Cryosurgery and topical fluorouracil : a treatment method for widespread basal cell epithelioma in basal cell nevus. syndrome. J Dermatol 1993 Aug; 20 (8): 507-13

Ujpal M, Szabo G. (Familial occurrence of Gorlin- Goltz syndrome). Fogorv Sz. 1992 JIul; 85 (7): 209-12

Watson RA, Harper BN. Paratesticular fibrous pseudotumour in a patient with Gorlin's syndrome : nevoid basal cell carcinoma syndrome J Urol 1992 Oct; 148 (4): 1254-5

Yee KC, Tan Cy, Bhatt KB, et all. Sclerotic bone lesions in Gorlin's sysdrome. Br J Radiol 1993 Jan; 66 (781) 77-80

Zarour H, Grobb JJ, Choux R, et al. ( Basal-cell and linear unilateral adnexal harmatoma (or linear uniear unilateral basal-cell nevus) ). Ann Dermatol Venereol 1992; 119 (11) 901-3

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Published

2023-03-30

How to Cite

1.
Jaovisidha S, Siriwongpairat P, S. J, P. S. BASAL CELL NEVUS SYNDROME A CASE REPORT. ASEAN J Radiol [Internet]. 2023 Mar. 30 [cited 2024 Nov. 21];1(1):39-48. Available from: https://asean-journal-radiology.org/index.php/ajr/article/view/221

Issue

Vol. 1 No. 1 (1995): January - April

Section

Original Article

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