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Hyperparathyroidism jaw tumour syndrome: a pictoral review. | LitMetric

Hyperparathyroidism jaw tumour syndrome: a pictoral review.

Insights Imaging

Department of Oral and Maxillofacial surgery, Barts and The Royal London Hospitals, Barts Health NHS, London, UK.

Published: December 2016

AI Article Synopsis

  • Hyperparathyroidism jaw tumour syndrome (HPT-JT) is a rare genetic disorder characterized by jaw tumors, parathyroid issues, and other tumors like renal and uterine cancers.
  • Patients usually show symptoms of jaw tumors first, necessitating awareness for proper diagnosis and treatment.
  • Management includes treating hyperparathyroidism and screening family members for risk, emphasizing the importance of early detection and intervention.

Article Abstract

Unlabelled: Hyperparathyroidism jaw tumour syndrome is a rare autosomal dominant inherited endocrine neoplasia syndrome, which predisposes carriers to develop a triad of multiple ossifying fibromas of the maxilla and mandible, parathyroid adenomas and carcinomas (with consequent hyperparathyroidism) as well as renal and uterine tumours. The prevalence of this condition is unknown. Patients typically present initially with symptoms and signs of a jaw tumour. A high index of suspicion is required for the underlying diagnosis to be recognised, enabling appropriate management of jaw lesions, treatment of hyperparathyroidism, if present, as well as early detection of malignant disease and screening of family members.

Teaching Points: • HPT-JT is a rare autosomal dominant inherited endocrine neoplasia syndrome. • HPT-JT causes facial disfigurement, morbidity secondary to hyperparathyroidism and malignancy. • Patients can present with ossifying fibromas of the jaw, hypercalcaemia or malignancy. • A high index of suspicion is required for the underlying diagnosis to be recognised. • Management involves screening of family members.

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Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5110477PMC
http://dx.doi.org/10.1007/s13244-016-0519-0DOI Listing

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