[Recklinghausen's osteitis fibrosa cystica].
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BACKGROUND: After parathyroidectomy, recovery of osteitis fibrosa cystica, which continues to dominate presentation of primary hyperparathyroidism in India has not been documented objectively. METHODS: We followed up clinical recovery, biochemic markers of bone turnover, bone mineral density, and skeletal radiology in 51 patients with primary hyperparathyroidism and osteitis fibrosa cystica for 9 to 124 months (median, 32 months). RESULTS: After parathyroidectomy, 46 patients had hypocalcemia. During postoperative week 1, bone pain improved in 71%. During 3 months, appendicular fractures healed in all 33 such patients, and 6 of 7 patients who were bedridden could walk. Mean bone mineral density increments (percent change/y) seen at various sites at 1 week, 3, 6, and 12 months were distal forearm--37, 28, 23, 21; lumbar spine--165, 104, 101, 106; and total hip--168, 157, 166, 133. Follow-up radiographs demonstrated prompt recovery though disorderly remineralization. Brown tumors and fractures showed hyperdensities within 3 months. Brown tumors regressed partially in 6 of 27 patients after 6 months. CONCLUSIONS: After parathyroidectomy, patients with primary hyperparathyroidism have early, marked, and sustained recovery of osteitis fibrosa cystica. Early (1 week) bone mineral density increments of > 100%/y hint at the skeleton's ability to promptly restore itself. Densitometric recovery is prompt at cancellous (lumbar spine), but not at cortical (forearm) bone sites. Contour defects and bony tumors persist, and may need corrective osteotomies.
The cytology findings of a fine needle aspiration biopsy from osteitis fibrosa cystica (brown tumor) of the rib in a patient with primary hyperparathyroidism due to parathyroid carcinoma are discussed. Many multinucleated osteoclast-type giant cells, characteristic of either osteitis fibrosa cystica or benign giant cell tumor of bone, were noted. There were also spindly or fibrillary cells with single, ovoid nuclei, probably of stromal origin.
A marked direct suppression of primary hyperparathyroidism with osteitis fibrosa cystica has been achieved by the intravenous administration of 1,25-dihydroxycholecalciferol [1,25(OH)2D]. In a recent survey of 306 patients with primary hyperparathyroidism (PHPT), we hypothesized that the far higher degree of parathyroid hormone (PTH) hypersecretion in PHPT with osteitis fibrosa cystica than in PHPT without overt bone disease might be due to the absence of suppression of hormonal hypersecretion by the low-to-normal circulating 1,25(OH)2D reflecting a relative vitamin D deficiency. To test this hypothesis, a patient having hypercalcemic PHPT with florid osteitis fibrosa cystica and normal serum 1,25(OH)2D was given increasing daily doses of intravenous calcitriol (0.5-2 micrograms) for several days. Doubling the level of circulating 1,25(OH)2D from 48 to 100-114 pg/ml was accompanied by a marked decline (46%) in serum iPTH(1-84), without a change in the serum calcium concentration. A lowered set point of parathyroid cells for calcium, and a diminished maximum secretory rate of PTH, may contribute to the marked suppression of PHPT.
A case of osteitis fibrosa cystica or brown tumor of bone in a patient presenting with acute spinal cord compression that was suggested initially by needle aspiration biopsy of the spine is described. Following the aspiration biopsy, excision of vertebral lesions, cord decompression, and spinal fusion were successfully performed. A parathyroid adenoma was subsequently identified and also resected. Along with the diagnosis of malignancy, the presence of hyperparathyroidism with osteitis fibrosa cystica should be considered in a patient presenting with lytic lesions in bone, especially if they are associated with hypercalcemia. Serum parathormone level determination is usually diagnostic of hyperparathyroidism, but this test has a 7-10-day turnaround time. Preoperative needle aspiration biopsy is a safe and rapid method of diagnosing osteitis fibrosa cystica and may be of critical importance in a patient with acute and progressive symptoms such as cord compression.
We present the case of a woman with classical osteitis fibrosa cystica generalisata von Recklinghausen, caused by a single adenoma of the left inferior parathyroid gland. After six months with increasing bone pain, pathological fractures and cystic lesions radiologically, hyperparathyroidism was diagnosed by persistent elevated levels of serum calcium, and elevated levels of PTH. Surgical extirpation of the parathyroid adenoma caused a period with severe hypocalcemia followed by complete clinical restitution. The diagnosis should be considered in any case of persistent bone pain and uncharacteristic fatigue.
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A 24-year-old man with primary hyperparathyroidism and osteitis fibrosa cystica developed acute hypocalcaemia. Spontaneous healing of his bone disease was confirmed radiographically and by correction of the serum alkaline phosphatase. Hypercalcaemia associated with a raised serum parathyroid hormone recurred 90 weeks after the initial presentation. During the fourth neck exploration a parathyroid adenoma was removed, resulting in resolution of his condition. Haemorrhagic infarction of an adenoma was the most likely cause of the acute hypocalcaemic episode.
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Primary hyperparathyroidism is usually associated with normal or elevated serum 1,25-dihydroxyvitamin D [1,25(OH)2D] levels. We report a 63-year-old patient with extreme hypercalcemia (ionized serum calcium, 2.51 mmol/l; normal range, 1.19-1.36), very high serum concentrations of intact immunoreactive parathyroid hormone (iPTH) (145 pmol/l; normal range, 1-6.8), radiological lesions of osteitis fibrosa cystica, only mildly impaired renal function (creatinine clearance, 69 ml/min/m2) and very low serum levels of 1,25(OH)2D (28.8 pmol/l; normal range, 72-120). Presurgery normalization of the calcemia with normal saline, salmon calcitonin and pamidronate caused an increase in 1,25(OH)2D serum concentration to 228.3 pmol/l. A negative correlation could be established between ionized calcium and 1,25(OH)2D levels during that period (r2 = 0.80, P < 0.04). While serum calcium decreased with treatment, serum iPTH also decreased to 48.6 pmol/l, suggesting some 1,25(OH)2D inhibition of parathyroid adenoma function. Serum alkaline phosphatase also rose from 309 to 390 units/l (normal range, 25-97), suggesting the beginning of resolution of her osteitis fibrosa cystica prior to surgery. Surgical removal of a parathyroid adenoma was associated with a decrease in serum calcium and iPTH levels. To our surprise, the hypocalcemia could be managed easily with 1500 mg of oral calcium carbonate daily, even if the hungry bone disease became more active with an increase in alkaline phosphatase to 486 units/l. This was explained by the very high levels of serum 1,25(OH)2D (> 200 pmol/l) which prevailed in the postsurgery period and were probably related to decreased bone resorption and increased bone formation. This case illustrates that normalizing serum calcium prior to surgery in patients with primary hyperparathyroidism and osteitis fibrosa cystica can be highly beneficial.
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In hyperparathyroidism, skeletal changes occur in 13 per cent of severe and long-standing cases (Smith, 1979). These skeletal changes vary from generalized demineralization of bone in early cases to resorption of bone marrow and replacement by fibrous tissue with cystic changes, the latter being termed osteitis fibrosa cystica or brown tumour. These cystic tumours are rare and usually occur in the long bones. Osteitis fibrosa cystica rarely presents in the maxilla as the initial symptom of hyperparathyroidism.
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A case of primary hyperparathyroidism with advanced osteitis fibrosa cystica but without any subperiosteal phalangeal bone resorption is described. A review of this unusual radiological feature is presented. High-detail magnification radiography (microradiography) is advocated for the early diagnosis of bony defects in hyperparathyroidism.