).Calcium metabolism is primarily regulated by parathyroid hormone (PTH), a polypeptide secreted by the parathyroid glands in response to a decreased serum level of ionized calcium. 1 This peptide prevents hypocalcemia by acting on multiple target organs. It increases bone turnover, releasing calcium into the circulation; increases the tubular resorption of calcium and phosphate excretion in the kidneys; and enhances the activity of renal α-1-hydroxylase, responsible for activating the vitamin D, which induces the calcium uptake in the intestine. 2 Three categories of Hyperparathyroidism (HPT) are recognized. The primary HPT is due to hypersecretion of PTH by one or more parathyroid glands, as a result of benign parathyroid adenoma (85%), primary parathyroid hyperplasia (10-20%) and, more rarely, is attributed to parathyroid carcinoma (1%). 2 The secondary HPT is due mainly to chronic renal insufficiency, and most of the patients develop the disease at the time that hemodialysis starts. 3 In this condition, abnormalities in the renal tubular absorption of phosphate results in reduced phosphate excretion, and renal synthesis of vitamin D is also impaired. The association of hyperphosphatemia and reduced vitamin D levels result in hypocalcemia and, consequently, oversecretion of PTH, resulting in parathyroid hyperplasia. 4 After restoration of renal disease by transplantation, the hyperplastic glands should return to normal functional states. However, in a small percentage of patients it does not occur, resulting in the rarest form of HPT, the tertiary HPT. 3 The primary HPT is usually a sporadic disorder, with a prevalence of 1 to 2% and, in less than 10% of the cases, it is part of hereditary familial syndromes, namely, multiple endocrine neoplasia type 1 (MEN1) and type 2A (MEN2A); HTP-jaw tumor syndrome (HTP-JT); familial hypocalciuric hypercalcemia (FHH); and familial isolated HTP (FIHP). 2,5,6 These hypercalcemic diseases are usually associated with abnormal expression of an oncogene, which can transform a normal cell into a tumor cell, or due to loss of a tumor suppressor gene, also referred to as recessive oncogenes or antioncogenes, that normally inhibit cell proliferation. 7 The aim of this study is to report a clinical case of ossifying fibroma in the mandible associated with HPT-JT syndrome, which was characterized clinically and radiographically, treated with surgical resection as well as mandibular reconstruction in a second surgical intervention, followed by implant placement.
Keywords► hyperparathyroidism ► primary ► hypercalcemia
AbstractThe purpose of this article is to report a case of ossifying fibroma in the mandible associated with hyperparathyroid-jaw tumor syndrome, in a 46-year-old Caucasian female, surgically resected and reconstructed with iliac crest bone, followed by implant placement. This is a rare syndrome with an autosomal dominant pattern, with the development of primary hyperparathyroidism mainly due to parathyroid adenomas. Fibro-osseous lesions in the jaws can be present and ca...