Hyperparathyroidism jaw tumour syndrome: a pictoral review

被引:17
作者
du Preez H. [1 ]
Adams A. [1 ]
Richards P. [1 ]
Whitley S. [2 ]
机构
[1] Department of Radiology, Barts and The Royal London Hospitals, Barts Health NHS, London
[2] Department of Oral and Maxillofacial surgery, Barts and The Royal London Hospitals, Barts Health NHS, London
关键词
Fibroma; Hyperparathyroidism; Jaw neoplasms; Ossifying; Parathyroid neoplasms; Primary;
D O I
10.1007/s13244-016-0519-0
中图分类号
学科分类号
摘要
Abstract: 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. © 2016, The Author(s).
引用
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页码:793 / 800
页数:7
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