Aswath et al. 51 CASE IN IMAGES OPEN ACCESS Pimary hyperparathyroidism as central giant cell granuloma of the jaws: Pre and post treatment pattern of clinical and radiographic presentation Nalini Aswath, Pravda Chidambaranathan ABSTRACT Introduction: The parathyroid glands regulate serum calcium and phosphorous levels by its secretion and maintenance, within physiological limits, of its hormone parathormone (PTH). Hyperparathyroidism is a metabolic disorder resulting from excessive secretion of parathyroid hormone. Hyper secretion might occur either due to a primary pathology in the glands (or) due to secondary causes. Central giant cell lesions occur in jaw bones. Case Report: A 20 year old boy reported with complaints of swelling in left maxilla. Intra orally swelling extended from left upper canine till left upper second premolar and caused expansion of the cortical plates. Intra oral radiograph and orthopantomograph showed a poorly defined radiolucent lesion in between left upper canine and left upper 1st premolar with multiple cystic cavities in the region of the symphysis, left body and right angle of mandible. The radiographs of long bones showed osteoporosis. Serum alkaline phosphatase level was raised. Histopathology was reported as central giant cell granuloma. Nuclear scan of parathyroid showed a functioning parathyroid adenoma. The swelling in left maxilla regressed Nalini Aswath 1, Pravda Chidambaranathan 2 Affiliations: 1 Professor & Head of Department-Sree Balaji Dental College and Hospital, Chennai, Tamilnadu, India; 2 Reader-Sathyabama Dental College and Hospital, Chennai Tamil Nadu, India. Corresponding Author: Dr. Nalini Aswath, Mathuram, Plot No:1 61, #5, Murugu Nagar 5 th street, Velachery, Chennai, Tamil Nadu, India - 600042; Ph: +91-044-22591 51 9; Mob: 91-94441 -61 841 ; Fax: +91-044-22460631 ; Email: naliniaswath@gmail.com Received: 20 December 2011 Accepted: 1 2 April 201 2 Published: 01 August 201 2 on its own after the adenoma of the parathyroids was excised. Conclusion: Timely diagnosis of parathyroid adenoma results in total regression of intra oral swelling and further progression of osteoporosis and fractures of long bones can be prevented. Keywords: Central giant cell granuloma, Brown tumor, Hyperparathyroidism ********* Aswath N, Chidambaranathan P. Pimary hyperparathyroidism as central giant cell granuloma of the jaws: Pre and post treatment pattern of clinical and radiographic presentation. International Journal of Case Reports and Images 2012;3(8):51 56. ********* doi:10.5348/ijcri 2012 08 167 CII 14 INTRODUCTION The parathyroid glands regulate serum calcium and phosphorous levels by secretion and maintenance, within physiological limits, of its hormone parathormone (PTH). Secretion of PTH is mainly controlled through interaction of calcium with calcium sensitive receptors on the membrane of the parathyroid cells. Hyperparathyroidism is a syndrome of hypercalcemia resulting from excessive secretion of parathyroid hormone. A case of central giant cell granuloma in left maxilla due to adenoma of parathyroid gland is presented. CASE REPORT A 20 year old boy reported with complaints of swelling in the left upper jaw (Figure 1). The swelling
Aswath et al. 52 was initially smaller six months ago and gradually increased in size. It was asymptomatic since onset and had not regressed in size at any time. There was no history of discharge from the swelling. No history of paresthesia or fever. The past medical history and surgical histories were unremarkable. Patient was moderately built, well nourished, calm and cooperative. His growth appeared to be retarded to his age. His vital signs were stable. A single left submandibular lymph node was enlarged, palpable, nontender, measuring 1 cm/1 cm in size, firm in consistency and mobile. Extra oral examination revealed a solitary swelling, with well defined margins, measuring 3 cm/3 cm in diameter in the region of the left maxilla (Figure 2). It was irregular in shape, with a smooth surface extending anteriorly till nasolabial fold, posteriorly till the lateral margin of the eye, superiorly till the infra orbital margin, and inferiorly in line with angle of the mouth. It was nontender, hard in consistency. The skin over the swelling appeared normal. There was no local rise in temperature. The swelling was not mobile and it was fixed to the underlying structure. Intra oral examination (Figure 3) revealed a swelling extending from left upper central incisor to left upper second premolar. There was expansion of the buccal and palatal cortical plates. Margins were well defined. It was firm to hard in consistency, nontender. Mucosa over the swelling was normal. It measured 1.5 cm/2 cm in diameter. The left upper canine and first premolar were decayed and the vitality test showed a delayed response. Based on the history and clinical findings a provisional diagnosis of adenomatoid odontogenic tumor and a differential diagnosis of central giant cell granuloma, ameloblastoma, fibro osseous lesion were given. Intra oral radiographs (Figure 4). Showed the presence of a poorly defined radiolucent lesion in between the left upper canine and left upper 1st premolar with divergence of roots of left upper 1st premolar. Orthopantomograph (Figure 5) showed the presence of a poorly defined radiolucent lesion in between the left upper canine and left upper 1st premolar with divergence of the roots of the left upper 1st premolar and well defined cystic cavities in the region of symphysis, left body and right angle of mandible. Radiographs of long bones, chest, skull and ultrasonography of abdomen were done. Radiographs of long bones (Figure 6) were suggestive of osteoporosis. No significant findings were revealed in ultrasonography of abdomen. Biochemical investigations revealed the following: Serum calcium: 9.4 mg%, Serum phosphorus: 3.1 mg%, serum alkaline phosphatise: 2559 mg/ml. Incisional biopsy was done and histopathological examination showed presence of mature bundles of connective tissue with plump fibroblasts, interspersed with numerous multinucleated giant cells. Overlying epithelium appeared hyperplastic, with few bundles of blood vessels. The lesion was suggestive of central giant cell granuloma (Figure 7). Thereafter the patient was referred to an endocrinologist to rule out hyperparathyroidism. After general examination of the patient by the endocrinologist, serum parathyroid estimation was done and was found to be elevated. Nuclear study of the parathyroid glands (Figure 8) was done with Tc 99m MIBI injected intravenously and it showed the presence of a functioning parathyroid lesion in the region of the lower pole of the left lobe of thyroid. An incisional biopsy was taken from the left. Inferior parathyroid gland which was suggestive of parathyroid adenoma. The tumor was surgically excised and post operatively the patient was treated with IV calcium and calcium supplements, and multivitamin tablets. The patient was periodically reviewed there after at an interval of three months. The serum calcium, alkaline phosphatase, serum phosphorus returned to their normal limits. The central giant cell granuloma in the region of the left maxilla regressed on its own (Figure 9, 10). Intraoral radiographs, Occlusal X ray (Figure 11), OPG (Figure 12), were taken which showed disappearance of the previous lesion. The multiple cystic cavities that were noticed in the mandible too disappeared. Figure 1: Photograph of face shows swelling in the left maxilla.
Aswath et al. 53 Figure 4: Intraoral radiograph shows poorly defined radiolucent lesion between canine and second premolar and divergence of root of first premolar. Figure 2: Facial profile shows swelling in the left maxilla. Figure 5: Orthopantomogram shows poorly defined radiolucent lesion between canine and second premolar in left maxilla and well defined cystic cavities in symphysis at left body and right angle of mandible. Figure 3: Intraoral photograph shows swelling in alveolar mucosa extending from the central incisor to second premolar. DISCUSSION Hyperparathyroidism is a metabolic bone disease. Primary Hyperparathyroidism is a disease, in which the parathyroid gland secretes excessive quantities of parathormone (PTH), due to increased activity of the gland due to (1) hyperplasia of the gland, (2) adenoma of the gland, (3) functional carcinoma of the parathyroid. Secondary hyperparathyroidism is secondary to chronic renal failure, rickets and osteomalacia [1]. Figure 6: Radiographs of long bones shows reduced density of bones suggestive of osteoporosis. Hyperparathyroidism is a relatively rare disease which is three times more common in women than men. It usually occurs in middle age, but might occur occasionally in children or in later life. Clinically, pathologic
Aswath et al. 54 Figure 7: Photomicrograph shows connective tissue with plump fibroblasts, multinucleated giant cells and blood vessels (H & E, X400). Figure 9: Postoperative photograph shows disappearance of swelling in the region of the left maxilla. Figure 8: Nuclear scan shows functioning parathyroid lesion in the region of the lower pole of the left lobe of thyroid. fractures may be the first symptom of the disease, although bone pain and joint stiffness are frequently heard early symptoms. Urinary tract stone is also a significant early finding [2]. Intraorally, the first sign of the disease may be a giant cell tumor (Brown tumor) or a cyst of the jaw. Brown tumor represents a giant cell reparative reaction. The loss of phosphorous and calcium results in generalized osteoporosis with attempts to repair the bone by new bone formation. The new bone may be resorbed and the resorption may lead to pseudocyst formation. Then the proliferation of granulation tissue from the cystic cavity occurs. As the area of bone resorption undergoes replacement by fibrous tissue, hemorrhage may occur. Small multinucleated giant cells may appear in an attempt to remove the blood. Hemosiderin in blood gives the lesion a brown color. Malocclusion caused due to sudden drifting of teeth may be the first sign of the disease [3 7, 8, 11, 12 14]. Radiographs show the presence of small or large sharply defined radiolucencies suggestive of cysts in the Figure 10: Postoperative intraoral view shows regression of intra oral swelling. maxilla or mandible. Some lesions show the classical ground glass appearance. The lamina dura around the teeth may be partially lost. Multifocal involvement of other bones in the body might also be seen [8, 11, 12]. The serum calcium is raised as high as 12 to 20 mg per 100 ml, while the serum phosphorus is lowered to 2 mg or less per 100 ml. If bone lesions are present serum alkaline phosphatase and serum parathormone levels
Aswath et al. 55 regress if the pathology in the parathyroid gland is corrected. ********* Figure 11: Postoperative intraoral occlusal and periapical radiographs showing disappearance of the radiolucent lesion between canine and second premolar. Author Contributions Nalini Aswath Substantial contributions to conception and design, Acquisition of data, Analysis and interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published Pravda Chidambaranathan Analysis and interpretation of data, Revising it critically for important intellectual content, Final approval of the version to be published Guarantor The corresponding author is the guarantor of submission. Conflict of Interest Authors declare no conflict of interest. Copyright Nalini Aswath et al. 2012; This article is distributed under the terms of Creative Commons Attribution 3.0 License which permits unrestricted use, distribution and reproduction in any means provided the original authors and original publisher are properly credited. (Please see /copyright policy.php for more information.) Figure 12: Postoperative orthopantomogram shows disappearance of radiolucent lesion in the left maxilla, symphysis, left body and right angle of mandible. are usually, raised, and urinary output of calcium is considerably increased [11, 12]. Histologically, multinucleated osteoclast like giant cells bone lesions show lying in hemorrhagic fibrous tissue. Deposits of hemosiderin may be seen. Multiple cystic cavities are noted [5, 6]. CONCLUSION To conclude hyperparathyroidism is a metabolic bone disorder that might occur either due to hypertrophy (or) adenoma of the parathyroid gland, that manifests intraorally as a central gint cell granuloma. Timely diagnosis, and treatment of the parathyroid lesion, results in total regression of the intraoral lesion on its own [10]. Further progression of osteoporosis and pathological fractures can also be prevented. Therefore if any case of central giant cell granuloma involving the jaw bones is reported, it is mandatory to rule out hyperparathyroidism. There is no need to surgically excise the lesion as the lesion will REFERENCES 1. Neville BW, Damm DD, Allen CM, Bouquot JE. Oral and maxillofacial pathology. Philadelphia, W. B. Saunders 1995. 2. Sapp JP, Eversole LR, Wysocki GP. Contemporary oral and maxillofacial pathology. Mosby; St. Louis 1997. 3. Odell EW, Morgan PR. Biopsy pathology of the oral tissues. London; Chapman & hall Medical 1998. 4. Anderson L, Fejerskov O, Philipsen HP. Oral giant cell granulomas: a clinical and histological study of 129 new cases. Acta Pathol Microbiol Scand 1973;81:6. 5. Ficarra G, Kaban LB, Hansen LS. Giant cell lesions of the jaws: a clinicopathologic cytometric study. Oral Surg Oral Med Oral Pathol 1987;64:44 9. 6. Auclair PJ, Cuenin P, Kratochvil FJ, Slater LJ, Ellis GL. A clinical and histomorphologic comparison of the central giant cell granuloma and the giant cell tumor. Oral Surg Oral Med Oral Pathol 1988;66:197 208. 7. Eisenbud L, Stern M, Rothberg M, Sachs SA. Central giant cell granuloma of the jaws: experience in the management of thirty seven cases. J Oral Maxillofac Surg 1988;46:37. 8. Whitaker SB, Waldron CA. Central giant cell lesions of the jaws: a clinical, radiologic and histopathologic study. Oral Surg Oral Med Oral Pathol 1993;75:199.
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