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Gorlin-Goltz Syndrome and Neoplasms: A Case Study

  • Nilza N F Lopes1,*,
  • Eliana M. Caran1
  • Maria Lucia Lee1
  • Nasjla Saba Silva1
  • André Caroli Rocha1
  • Carla R D Macedo1

1Pediatric Oncologist. Pediatric Oncology Institute – GRAACC, Medical School of São Paulo, Federal University of São Paulo, SP, Brazil.

DOI: 10.17796/jcpd.35.2.x01248284w166485 Vol.35,Issue 2,March 2011 pp.203-206

Published: 01 March 2011

*Corresponding Author(s): Nilza N F Lopes E-mail: nnflopes@terra.com.br

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Abstract

Gorlin syndrome is a rare autosomal dominant disorder exhibiting high penetrance and variable expressivity. It is characterized by facial dysmorphism, skeletal anomalies, multiple basal cell carcinomas, odontogenic keratocysts (OKC), palmar and plantar pits, bifid ribs, vertebral anomalies and a variety of other malformations. Various neoplasms, such as medulloblastomas, meningiomas, ovarian and cardiac fibromas are also found in this syndrome. Objective: To describe a twelve-year-old patient with Gorlin-Goltz syndrome, with basal cell carcinomas and promyelocytic leukemia developed after receiving craniospinal radiation for a medulloblastoma. Bifid ribs as well as mandibular and maxillar OKC were also diagnosed. Conclusion: The patient with Gorlin-Goltz syndrome should receive close follow-up for early detection of malformations and malignant neoplasias.

Keywords

Gorlin syndrome, odontogenic keratocysts, basal cell carcinoma, medulloblastoma, acute myeloid leukemia

Cite and Share

Nilza N F Lopes,Eliana M. Caran,Maria Lucia Lee,Nasjla Saba Silva,André Caroli Rocha,Carla R D Macedo. Gorlin-Goltz Syndrome and Neoplasms: A Case Study. Journal of Clinical Pediatric Dentistry. 2011. 35(2);203-206.

URL:

https://www.jocpd.com/articles/10.17796/jcpd.35.2.x01248284w166485

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