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Spontaneous Healing of Osteitis Fibrosa Cystica in Primary Hyperparathyroidism

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Spontaneous Healing of Osteitis Fibrosa Cystica in Primary Hyperparathyroidism 754 Gibbs, Millar, Smith Postgrad Med J: first published as 10.1136/pgmj.72.854.754 on 1 December 1996. Downloaded from Spontaneous healing of osteitis fibrosa cystica in primary hyperparathyroidism CJ Gibbs, JGB Millar, J Smith Summar biochemistry showed hypercalcaemia, hypo- A 24-year-old man with primary hyper- phosphataemia, elevated parathyroid hormone, parathyroidism and osteitis fibrosa cystica but normal alkaline phosphatase (table). developed acute hypocalcaemia. Sponta- Radiographs showed improvement in the neous healing of his bone disease was mandibular translucency and resolution of the confirmed radiographically and by correc- phalangeal tuft resorption and subperiosteal tion of the serum alkaline phosphatase. erosion (figures 1B, 2B). Thallium scan of the Hypercalcaemia associated with a raised neck showed no evidence of parathyroid serum parathyroid hormone recurred 90 activity and neck exploration failed to reveal weeks after the initial presentation. Dur- any parathyroid tissue. Venous sampling ing the fourth neck exploration a para- showed no step-up in parathyroid hormone thyroid adenoma was removed, resulting concentration in the neck or chest. Selective in resolution of his condition. Haemor- angiography suggested a parathyroid adenoma rhagic infarction of an adenoma was the behind the right clavicle but two further most likely cause of the acute hypocalcae- explorations revealed only one normal para- mic episode. thyroid gland. Computed tomography (CT) of the neck showed a low attenuation, non- Keywords: primary hyperparathyroidism, osteitis enhancing mass in the right lower pole of the fibrosa cystica, hypercalcaemia thyroid gland. Ultrasonography confirmed a hypo-echoic mass 1.5 x 0.5 cm in the right lobe of the thyroid. A 24-year-old man presented with a painless Removal of the right lobe of the thyroid swelling of the right mandible, lethargy, during the fourth neck exploration, five years anorexia, thirst and lower backache. Examina- after the first presentation, was successful and tion was normal apart from the mandibular histology confirmed a parathyroid adenoma swelling. Radiographs showed a large translu- 1.4 x 1.0 x 0.8 cm within the excised thyroid cency in the right mandibular body (figure 1A). tissue. The patient became hypocalcaemic and http://pmj.bmj.com/ Subperiosteal erosion and distal phalangeal tuft required maintenance oral calcitriol treatment resorption were present in the hands (figure for hypoparathyroidism, but he remained well 2A). Intravenous urography showed localised two years later on calcitriol 6 jg/day with a medullary sponge kidney of the upper pole of serum calcium in the normal range. the left kidney with nephrocalcinosis. Serum biochemistry showed hypercalcaemia and an Discussion elevated parathyroid hormone concentration on September 30, 2021 by guest. Protected copyright. (table). This is the first well-documented report of a Ten days after the initial consultation the spontaneous cure of osteitis fibrosa cystica in patient was admitted with paraesthesiae in the proven primary hyperparathyroidism. The Department of acute hypocalcaemia that heralded the event Endocrine Medicine, hands, feet and face, and dyspnoea from a Southampton Univer- feeling of pressure on his chest. Serum was most probably due to haemorrhagic sity and St Mary's biochemistry showed hypocalcaemia (table). infarction of a parathyroid adenoma, which Hospital, Portsmouth, He was treated with oral calcium supple- could be termed parathyroid apoplexy. Similar UK ments 1200 mg daily (without vitamin D) and crises are well known in other endocrine CJ Gibbs discharged home. A biopsy of the mandibular adenomata, for example, pituitary apoplexy. JGB Millar swelling one month later showed a 'brown We could not justify urgent surgical explora- Department of tumour' due to hyperparathyroid bone disease. tion to prove the presence of a necrotic Pathology, St Mary's There was fibrous replacement of the marrow parathyroid adenoma and at that date urgent Hospital, Newport, space (osteitis fibrosa cystica). Osteoclasts were CT, ultrasound or magnetic resonance ima- Isle of Wight, UK numerous and osteoblastic new bone forma- ging of the neck to demonstrate a haemor- J Smith tion was increased at the margins of the lesion. rhagic mass were not available at the unit When Correspondence to Dr CJ His backache gradually improved; by 30 where he presented. hyperparathyroid- Gibbs, Department of weeks the serum calcium returned to normal ism recurred and a parathyroid adenoma was General and Endocrine and the serum alkaline fell to just excised, no infarcted parathyroid tissue was Medicine, Greenwich phosphatase District Hospital, Vanbrugh above the upper reference limit (figure 3). He found, but this is not surprising in view of the Hill, Greenwich, London remained well on no treatment until he interval of five years. A previous pathological SE10 9HE, UK presented again with the original symptoms study has shown that severe acute hypocalcae- Accepted 24 January 1996 90 weeks after the first consultation. Serum mia in a patient with a suspected parathyroid Spontaneous cure of osteitis fibrosa cystica 755 Figure 1 Radiographs of the mandible on : (A) Postgrad Med J: first published as 10.1136/pgmj.72.854.754 on 1 December 1996. Downloaded from presentation, (B) 10 months laters and (C) five years later, showing spon- taneous resolution of os- teitis fibrosa cystica. Note the translucency due to a brown tumour eroding the cusps of the adjacent molar (A). Healing is ap- parent in (B) with new.... bone formation within the.............. scleroris is apparenlt in the : posteriorinfeto nCthehalflesionfoof the____.h brown tumour. A dense .d sclerotic rim is extending _ |y0 !i!11Al' mag nsi ......... margins~~~~~~~~~~~~~~~ http://pmj.bmj.com/ on September 30, 2021 by guest. Protected copyright. Figure 2 Radiographs of the hand showing distal phalangeal tuft resorption and subperiosteal resorp- tion along the radial bor- ders of the phalanges (A) and spontaneous resolu- tion after two years (B) A~~B 756 Gibbs, Milar, Smith Table Serum and urine biochemistry in a patient with a parathyroid adenoma to show spontaneous changes over 90 weeks Postgrad Med J: first published as 10.1136/pgmj.72.854.754 on 1 December 1996. Downloaded from Weeks: 1 2 3 12 30 90 Normal range Serum calcium (adjusted) (mmol/1) 3.94 3.71 1.78 2.05 2.23 3.25 2.20-2.60 Serum phosphate (mmoIl) 0.94 0.85 0.59 1.07 1.37 0.56 0.80-1.5 Serum magnesium (mmolI) - 0.94 0.74 - 0.67 0.84 0.7-1.2 Serum alkaline phosphatase (IU/1) 993 1041 925 633 298 269 80-280 Serum urea (mmoIl) 7.0 7.3 8.6 6.1 7.0 5.1 2.0-6.5 Serum creatinine (mmoIl) - 121 - - - 120 60-130 Serum N-terminal parathyroid hormone (ng/ml) - 3.4 0.7 - - - <0.6 Serum intact parathyroid hormone (pmolI) - - - - - 19.3 0.8-8.5 Serum 250H-cholecalciferol (ng/ml) - - 8.0 - 13.8 - 8-50 Urine calcium excretion (mmol/24 h) - - 0.2 - - 4.6 1.5-7.5 Urine phosphate excretion (mmolV24 h) - - 3.4 - - 23 16-32 Urine creatinine excretion (mmolI24 h) - - 4.6 - - 11.4 9-17 Figure 3 Serum cal- 40 Summarynearning points cium and serum alkaline phosphatase in a patient * after parathyroidectomy hungry bone disease with a parathyroid adeno- 3- 5 may occur, causing prolonged hypocalcaemia ma followed for 250 0 while the skeleton takes up mineral to fill weeks. The first hypocal- E resorption cavities caemic episode was spon- E 3.0 * brown tumours heal by dense sclerosis over 12 taneous, the second was E months after parathyroidectomy */ E The heightened activity ofthe osteoclasts up to ID2.0 the time of involution of the adenoma would lead to numerous resorption cavities. The time taken for osteoblasts to fill these cavities with 1.5 new bone is three to four months in normal 0 50 100 150 200 250 bone, but may be longer in osteitis fibrosa Weeks cystica despite the high bone turnover. The bone formation marker, alkaline phosphatase, 1200 took over 30 weeks to return to normal in our case. The mandibular defect healed as would 1000M be expected for a brown tumour which 4) C,) typically undergoes dense sclerosis within 12 http://pmj.bmj.com/ 4 800 months,5 in contrast to bone cysts which do not did not occur, C,) remineralise. Complete sclerosis 0- probably because of recurrent hyperparathy- a 600 roidism after 30 weeks. a) c After parathyroidectomy the skeleton's avid 400- requirement for calcium and phosphate to mineralise new bone (hungry bone disease) E 200 _ may last for months.6 The prolonged hypocal- on September 30, 2021 by guest. Protected copyright. caemia in our patient was probably due to this, compounded by suppression of the remaining parathyroid glands by previous chronic hyper- 0 50 100 150 200 250 calcaemia,7 as demonstrated by an inadequate Weeks parathyroid hormone response (0.7 ng/ml) during the hypocalcaemic crisis. There was difficulty in localising the adeno- tumour was related to recent infarction of an ma because of its size and position. Small and adenoma.' The recurrence of a parathyroid ectopic parathyroid glands are poorly visua- adenoma can be explained by regrowth ofnests lised by thallium subtraction imaging because ofviable chiefcells within a necrosed adenoma, of the low energy of the emitted photon.8 as these have been reported in a patient whose Finally, hypocalcaemia in a patient with parathyroid adenoma infarcted after calcitonin known primary hyperparathyroidism should treatment.2 Histological proof of necrosis in a lead one to consider acute pancreatitis and parathyroid adenoma has also been described vitamin D deficiency, which are known com- in a patient who had a parathyroidectomy soon plications of primary hyperparathyroidism and after spontaneous resolution of hypercalcae- more common causes of hypocalcaemia than mia.)3 necrosis of an adenoma.9 The lack of synchronicity of the activity of osteitis fibrosa cystica with the degree of hypercalcaemia is interesting, but consistent We acknowledge the assistance given by Professor JHL with the Frost model of bone remodelling.4 O'Riordan, Mr EJG Milroy and Dr HWP Rooke.
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