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Imaging of Gorlin-Goltz Syndrome: Series of 2 Cases

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Date 2021 Oct 27
PMID 34703144
Citations 3
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Abstract

Gorlin-Goltz syndrome (GGS) is a rare autosomal dominant disorder with multisystemic involvement. It is characterized by the triad of multiple baso-cellular epitheliomas, odontogenic keratocysts (OKC) in the jaws and skeletal anomalies. Later, it was found that calcification of falx is also highly specific. We present radiological findings in case series of two cases, one with multiple OKC, calcified falx, skin lesions, and fibrous dysplasia of sphenoid and second with multiple OKC, calcified falx, vertebral anomaly and medulloblastoma.

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References
1.
Manfredi M, Vescovi P, Bonanini M, Porter S . Nevoid basal cell carcinoma syndrome: a review of the literature. Int J Oral Maxillofac Surg. 2004; 33(2):117-24. DOI: 10.1054/ijom.2003.0435. View

2.
Matsuzawa N, Nagao T, Shimozato K, Niikawa N, Yoshiura K . Patched homologue 1 mutations in four Japanese families with basal cell nevus syndrome. J Clin Pathol. 2006; 59(10):1084-6. PMC: 1861741. DOI: 10.1136/jcp.2005.029439. View

3.
Doede T, Seidel J, Riede F, Vogt L, Mohr F, Schier F . Occult, life-threatening, cardial tumor in syndactylism in Gorlin Goltz syndrome. J Pediatr Surg. 2004; 39(10):e17-9. DOI: 10.1016/j.jpedsurg.2004.06.042. View

4.
Casaroto A, Loures D, Moreschi E, Veltrini V, Trento C, Gottardo V . Early diagnosis of Gorlin-Goltz syndrome: case report. Head Face Med. 2011; 7:2. PMC: 3035593. DOI: 10.1186/1746-160X-7-2. View

5.
Kimonis V, Mehta S, DiGiovanna J, Bale S, Pastakia B . Radiological features in 82 patients with nevoid basal cell carcinoma (NBCC or Gorlin) syndrome. Genet Med. 2004; 6(6):495-502. DOI: 10.1097/01.gim.0000145045.17711.1c. View