Submitted: 25 Dec 2022
Revision: 27 May 2023
Accepted: 11 Jul 2023
ePublished: 29 Dec 2023
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Avicenna J Dent Res. 2023;15(4): 183-188.
doi: 10.34172/ajdr.1675
  Abstract View: 484
  PDF Download: 171

Case Report

Major Manifestations of Gorlin Syndrome in a Patient With Hemophilia A

Elham Hadadian Pour 1 ORCID logo, Amirhosein Haghir 2, Sirous Risbaf Fakour 3* ORCID logo

1 Department of Oral & Maxillofacial Surgery, Zahedan University of Medical Sciences, Zahedan, Iran
2 Department of Neurosurgery, Mashhad University of Medical Sciences, Mashhad, Iran
3 Oral and Dental Disease Research Center, Department of Oral and Maxillofacial Surgery, Zahedan University of Medical sciences, Zahedan, Iran
*Corresponding Author: Sirous Risbaf Fakour, Email: srisbaf@gmail.com


Nevoid basal cell carcinoma syndrome (NBCCS) is a rare autosomal dominantly inherited condition in which constitutive pathway activity and tumor cell proliferation are disturbed due to defects in hedgehog signaling. This study reports an adult case of hemophilia A suspected of having Gorlin syndrome. There is no similar evidence in the literature to describe the association between these disorders. NBCCS is characterized by the development of multiple basal cell carcinomas (BCCs). The case presented in this study had two major manifestations of NBCCS, including the calcification of the falx cerebri and the presence of keratocystic odontogenic tumors (KOTs); however, no minor manifestations of NBCCS were found in this case. On the other hand, given the financial restrictions, it was impossible to perform a genetic examination to confirm the presence of NBCCS.

Please cite this article as follows: Hadadian Pour E, Haghir A, Risbaf Fakour S. Major manifestations of Gorlin syndrome in a patient with hemophilia A. Avicenna J Dent Res. 2023; 15(4):183-188. doi:10.34172/ajdr.1675
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