Atlanto-occipital ligament calcification: A novel sign in nevoid basal cell carcinoma syndrome

Rosalia Leonardi, Andrea Santarelli, Ersilia Barbato, Domenico Ciavarella, Susanne Bolouri, Franz Härle, Giuseppe Palazzo, Lorenzo Lo Muzio

Research output: Contribution to journalArticlepeer-review

Abstract

Background: The nevoid basal cell carcinoma syndrome (NBCCS), first described by Gorlin and Goltz in 1960, is a hereditary autosomal dominant disease with high penetrance and variable expressivity. Almost 70% of patients with NBCCS have some degree of craniofacial anomaly. Among these, the presence of ectopic calcification have been reported but Atlanto-occipital ligament calcification has never been described. Therefore this investigation was carried out to determine the prevalence of atlanto-occipital ligament calcification on lateral x-ray of NBCCS patients aiming to assess the effectiveness of this sign in NBCCS diagnosis. Patients and Methods: Lateral and frontal cephalometric radiographs of 18 patients (11 males and 7 females), aged 8-61 years, with the diagnosis of NBCCS were evaluated for the presence of intracranial calcifications (diaphragma sellae and falx cerebri) and or calcification of the atlanto-occipital ligament. Results: A total of 11 patients presented calcification of atlanto-occipital ligament to various degrees and in three cases this represented the only sign of ectopic calcification. When compared to the other two sites of ossification, atlanto-occipital ligament calcification had a similar prevalence. Conclusion: The calcification of the atlanto-occipital ligament should be considered in addition to the other major criteria for NBCCS diagnosis.

Original languageEnglish
Pages (from-to)4265-4267
Number of pages3
JournalAnticancer Research
Volume30
Issue number10
Publication statusPublished - Oct 2010

Keywords

  • Atlanto-occipital ligament
  • Calcification
  • Gorlin
  • NBCCS
  • Ponticulus posterior

ASJC Scopus subject areas

  • Cancer Research
  • Oncology

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