Primary Hyperparathyroidism with Rare Presentation As Multiple

Clinics and Practice 2012; volume 2:e48 Primary hyperparathyroidism extremely rare in normocalcemic hyper- parathyroid patients.5 Correspondence: Tapan Nagpal, Department of with rare presentation ENT, S.B.K.S. Medical institute and research cen- as multiple brown tumours ter, Waghodia, Vadodara, India. E-mail: [email protected] Smit Doshi,1 Arti Vadi,1 Amit Mahajan,2 Case Report Key words: hyperparathyroidism, brown tumour, 1 Tapan Nagpal osteitis fibrosa cystica, parathyroidectomy. 1Department of ENT and 2Department of A 30-year-old female presented with multi- Oral and Maxillofacial Surgery, S.B.K.S. ple swellings over different areas of body since Conflict of interests: the authors declare no 3 months, which followed after pregnancy. potential conflict of interests. Medical institute and research center, There was no other associated complaints or Waghodia, Vadodara, India any significant family history. On examination Received for publication: 18 January 2012. Revision received: 23 February 2012. there were multiple bony swellings present as Accepted for publication: 1 March 2012. follows: i) hard and mildly tender swelling in Abstract the right side body of mandible; ii) hard, non- This work is licensed under a Creative Commons tender swelling present in left frontal region; Attribution NonCommercial 3.0 License (CC BY- iii) hard and nontender swelling present below NC 3.0). We present a case of primary hyperparathy- the left knee joint. Radiological evaluation of roidism with an uncommon presentation as the above areas revealed an osteolytic lesion at ©Copyright S. Doshi et al., 2012 multiple brown tumours, which may easily be all sites (Figures 1, 2, 3). Her serological inves- Licensee PAGEPress, Italy mistaken for a primary bone neoplasm. A brief Clinics and Practice 2012; 2:e48 tigation - serum alkaline phosphate - 1080 U/L doi:10.4081/cp.2012.e48 literature review and its clinical and surgical (normal 245-770 U/L) was also suggestive of management are also discussed here. increased osteoclastic activity. Serum calcium was 10.2 mg/dL (normal range- 9 to 11 mg/dL) and serum phosphorous was 2.1 mg/dL (nor- that may occur after removal of the parathyroid mal range 2 to 4 mg/dL) raising the possibility only Introduction tumour due to suppression of normal parathy- of hyperparathyroidism, which was confirmed roid activity by the tumour. The parathyroid by a markedly elevated assay of serum PTH assay done post-operatively was now within Hyperparathyroidism (HPT) is a disease in [>1900 pg/mL (normal: 11.1-79.5 pg/mL)]. All which there may be a complex of biochemical, use normal range (23.5 pg/mL). Post-operatively in other routine blood investigations were nor- the third week the patient required a single anatomic and clinical abnormalities resulting mal. Ultrasono graphy neck (Figure 4) revealed parenteral supplementation of calcium glu- from increased secretion of parathyroid hor- presence of hypo echoic lesion of size conate and also magnesium sulphate to correct mone (PTH). Hyperparathyroidism may be pri- 21 10 11 mm on posterior-inferior aspect of ¥ ¥ the hypocalcaemia and hypomagnesia that mary due to idiopathic hyperplasia, which left lobe of thyroid gland (clinical examination developed due to inadequate oral intake and involves all four glands, or parathyroid adeno- of neck did not reveal any abnormal swelling or depleted body stores of calcium and magne- ma, which is isolated involving only one gland, fullness). sium due to previously long standing hyper- or as a part of Multiple Endocrine Neoplasia A whole body computerized scans for com- parathyroid disease. The patient was coun- Syndrome1 with resultant hypercalcemia. plete skeletal survey revealed multiple lytic seled regarding long-term oral calcium and Secondary hyperparathyroidism1 is usually expansile lesions involving whole skeleton and magnesium supplementation post-operatively secondary to calcium malabsorption and renal increase in size of parathyroid glands on left to re-build the depleted body stores. calcium loss due to chronic renal failure. side (Figure 5). The typical salt and pepper Tertiary hyperparathyroidism1 is due to appearance on skull radiology and periarticu- acquired abnormalities of the parathyroid lar osteopenia on radiology of the hands was glands in long standing cases of hyperparathy- not very obvious. roidism and chronic renal failure due to defi- The patient was operated under general Discussion cient response to parathyroid hormone at the anaesthesia with Inj. Methylene Blue being level of the receptor in kidney Non-commercial(renal resist- given at the dose of 7.5 mg/kg body weight as For unknown reasons hyperparathyroidism ance). A fourth type occurs due to ectopic an intravenous infusion in 5% Dextrose 1 h tends to present either with bone disease or hyperparathyroidism in patient with malignan- prior to surgery (Methylene Blue is preferen- with renal stones but never both.1 It is more cy. The classical brown tumour of hyper- tially concentrated in the parathyroid which common in the female gender with the major- parathyroidism is an expansile osteolytic helps in easy localization). The left inferior ity of cases in the third to fifth decade1 as in lesion resulting from excess osteoclast activity parathyroid was found enlarged consistent our case. which can localize anywhere in the skeleton, with the Ultrasonography findings located Skeletal involvement in classical primary the preferential locations being the head along the branch of left inferior thyroid artery hyperparathyroidism reflects a striking bones (particularly the mandible),2 and the (Figure 6). It was carefully dissected from the increase in osteoclastic bone resorption and is ends of long bones and ribs.3 However it has left recurrent laryngeal nerve. Histo-pathology accompanied by a cellular repair process that become increasingly rare to find multiple focal revealed it to be a parathyroid chief cell adeno- results in the accumulation of fibrillar stroma areas of demineralization of the skull; or ma. Post-operatively both vocal cords were and connective tissue cells along with multin- brown tumour (osteitis fibrosa cystica) as part mobile and patient had an uneventful recovery. ucleated giant cells and spicules of osteoid of the classic manifestation of the hyper- The patient was given a slow intravenous infu- deposits. Thus the classical brown tumor of parathyroid bone disease and it may be mistak- sion of calcium gluconate after 24 h postopera- hyperparathyroidism is a collection of osteo- en for primary skeletal neoplasms. Brown tively and then put on oral calcium with active clasts mixed with innocuous spindle cells and tumors are found in primary hyperparathy- Vitamin D3 (calcitriol) to tide over the tempo- poorly mineralized woven bone.6 Even though roidism but uncommon in secondary HPT4 and rary hypocalcemia with the consequent tetany the brown tumor can localize anywhere in the [Clinics and Practice 2012; 2:e48] [page 113] Case Report skeleton, the preferential locations are the head bones (particularly the mandible),2 and the ends of long bones and ribs.3 The rarity of multiple such lesions led us primarily to the differential diagnosis of fibrous dysplasia or less commonly a metastatic lesion. However the characteristic feature as post-operatively observed in our case was the haemorrhage and haemosiderin deposits7 which impart the brownish colour and hence the term. Treatment of brown tumour is dependent on the treatment of hyperparathyroidism. A majority of these lesions may disappear with the removal of the parathyroid pathology. It’s Figure 1. Orthopantomogram showing Figure 4. Ultrasonography colored Dop - generally accepted that treatment of brown multilocular radiolucent lesion (arrow) pler of neck shows presence of hypo echoic tumor should start with treatment of underly- present in right side of mandible involving lesion at inferior pole of left thyroid gland. ing HPT and if persisted after this primary body. Typical loss of lamina dura was not very evident. treatment, enucleation and curettage should be added especially if the lesion results in some functional problems.8 Resection is car- ried out in the majority of cases only to achieve a definitive diagnosis. Primary hyperparathyroidism can be divided pathologically into adenomas (85%), hyperplasia (15%) and carcinomas (<1%).1 Histolo - gically distinction between adenomas and only hyperplasia is virtually impossible with an increased cell to fat ratio in both. Adenomas involve only one gland as in our case (left inferior parathyroid). Double adenomas are rare use (5% cases).1 The major areas of debate sur- rounding primary hyperparathyroidism include: i) differentiation between adenomas and hyperplasia; ii) whether medical therapy in mild cases of primary hyperthyroidism is appropriate; iii) value of preoperative localization studies; iv) unilateral versus bilateral Figure 2. X-ray skull anterior/posterior Figure 5. Computerized scan neck (axial neck dissection as a surgical approach. view showing osteolytic lesion in left view) showing (white arrow) parathyroid frontal region (arrow). Melton9 concluded that long term medical adenoma on left side posterior to thyroid gland. therapy with calcitonin and bisphosphonates to reduce calcium level was costly and possible only in few cases of mild primary hyperparathyroidism with reversal of symptoms and bone density loss. 90-95% of adenomas can be found at neck exploration.10 Hence preoperative localization techniques like

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