Hyperparathyroidism jaw tumour syndrome: a pictoral review
نویسندگان
چکیده
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.
منابع مشابه
Hyperparathyroidism-jaw tumour syndrome detected by aggressive generalized osteitis fibrosa cystica.
Severe hyperparathyroidism can affect bone metabolism and be in the origine of multiple brown tumours (generalized osteitis fibrosa cystica). When associated with fibro-ossifying tumours of the jaw, it realizes a rare genetic syndrome referred as Hyperparathyroidism-jaw tumour HPT-JT. We report the case of a patient we treated for HPT-JT, and literature review.
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عنوان ژورنال:
دوره 7 شماره
صفحات -
تاریخ انتشار 2016