Nevoid Basal Cell Carcinoma Syndrome (Gorlin Syndrome) in Children

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  •   S. Binsheikhan

  •   S. Mittal

  •   M. Al Abadie

Abstract


Introduction: Gorlin syndrome or nevoid basal cell carcinoma syndrome (NBCCS) is a rare genetic disorder characterised by development of multiple basal cell carcinomas (BCC) at a young age.


Case report: A 7 year female child presented with MULTIPLE skin growths on the neck, face and upper chest for 3 years, with prominent forehead and mild non-scarring alopecia. She also had a history of medulloblastoma treated 3 years ago. There was no significant family history. Biopsy from one of the lesions showed basal cell carcinoma (BCC).




Discussion: Nevoid basal cell carcinoma syndrome (NBCCS) is an autosomal dominantly inherited disorder caused by mutations in the tumour suppressor patched 1 (PTCH-1) gene. Patients present with both cutaneous and extra-cutaneous manifestations. Multiple basal cell carcinomas (BCCs) are one of the most frequent cutaneous manifestations, occurring on both photo-exposed and non-exposed areas. The commonest extra-cutaneous tumours are medulloblastomas, which are often the first presentation of the disease. There are multiple but no established treatment modalities for the disease.



Keywords: Gorlin syndrome, medulloblastoma, nevoid basal cell carcinoma syndrome

References

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How to Cite
Binsheikhan, S., Mittal, S., & Al Abadie, M. (2021). Nevoid Basal Cell Carcinoma Syndrome (Gorlin Syndrome) in Children. European Journal of Medical and Health Sciences, 3(5), 15–17. https://doi.org/10.24018/ejmed.2021.3.5.888