Ann Thorac Surg 2002;73:1651-1653
© 2002 The Society of Thoracic Surgeons
Case report
Brown tumor of the sternum
Chiung-Lun Kao, MD*a,
Jen-Ping Chang, MDa,
Jui-Wei Lin, MDa,
Cheng-Ching Lin, MDa
a Departments of Thoracic and Cardiovascular Surgery and Pathology, Chang Gung Memorial Hospital at Kaohsiung, Taiwan, Republic of China
Accepted for publication September 3, 2001.
* Address reprint requests to Dr Kao, Department of Thoracic and Cardiovascular Surgery, Chang Gung Memorial Hospital at Kaohsiung, 123 Ta-Pei Rd, Niao Sung Hsiang, Kaohsiung Hsien, Taiwan, Republic of China
e-mail: sa11421{at}adm.cgmh.org.tw
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Abstract
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The skeletal changes of severe hyperparathyroidism, known as osteitis fibrosa cystica, are now rarely encountered, because hyperparathyroidism is currently being diagnosed and treated at an early stage. Herein, a case of brown tumor of the sternum is reported; our report adds histologic data on this type of tumor to the literature.
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Introduction
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The bone disease osteitis fibrosa cystica was first described by von Recklinghausen in 1891, but the etiologic link between this disease and parathyroid neoplasms was not established until 1925, when the Viennese surgeon Mandl observed clinical improvement in a 39-year-old man with severe bone disease after removal of a parathyroid adenoma [1]. The skeletal involvement is caused by a generalized increase in osteoclastic bone resorption, accompanied by fibrovascular marrow replacement and increased osteoblastic activity. Aggregates of osteoclasts, reactive giant cells, and hemorrhagic debris occasionally form a mass known as a brown tumor. We describe a case involving a 20-year-old woman with ectopic parathyroid adenoma who had a brown tumor of the sternum. Our report provides histologic data on this case of brown tumor of the sternum.
A 20-year-old woman was referred to our department for excision of an intrathoracic ectopic parathyroid adenoma. She had suffered from arthralgia for 2 years. She also experienced increasing soreness in both thighs, constipation, fatigue, and weight loss for 1 year before onset of arthralgia. She developed progressive proximal muscle weakness of both lower extremities and eventually could not walk. On physical examination, she was bedridden, with limitation of both hip and knee joints. The motor strength of the lower extremities was III/V bilaterally, and the sensation was normal. The deep tendon reflexes were normal. Laboratory studies showed serum calcium 12.8 mg/dL, phosphorus 1.2 mg/dL, alkaline phosphatase 3,960 U/L, and parathyroid hormone 886 pg/mL. A screening for multiple endocrine neoplasia type I was arranged including serum prolactin and gastrin, and the data were within normal limits. The 24-hour urine vanillylmandelic acid level was 6.7 mg/day. Hypercalcemia and high serum parathyroid hormone level led to the diagnosis of hyperparathyroidism. A series of roentgenograms revealed generalized osteoporotic change of the skeleton. Ultrasonography and parathyroid scintigraphic studies of the neck area failed to demonstrate an enlarged parathyroid lesion. However, the chest thallium 201 and technetium 99 sestamibi imaging clearly disclosed a vivid focal uptake in the superior mediastinum, suggesting ectopic parathyroid adenoma. Chest computed tomographic scan confirmed a mass in the anterior mediastinum adhering to the sternum and a cystic lesion of the sternum (Fig 1).
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Fig 1. Chest computed tomographic scan showing a cystic lesion (arrow) in the sternum and a mass in the region of the thymus.
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Surgical exploration was performed through a midline sternotomy. A 1-cm x 2-cm soft tissue mass in the sternomanubrium junction was noted (Fig 2),
and the pathologic examination revealed abundant osteoclast-type multinucleated giant cells scattered in the sea of the spindle-shaped stromal cells (Fig 3),
which is the typical finding of brown tumor. A 2.6-cm x 1.5-cm x 1.5-cm parathyroid adenoma in the right lobe of the thymus was identified. The tumor was removed along with thymectomy. Postoperatively, hungry bone syndrome developed in the patient, requiring a large amount of calcium and phosphorus supply. The postoperative course was complicated by pathologic fractures of bilateral femoral necks, which were treated with closed reduction and internal fixation. The patient was discharged, and her condition remained improved at 6-year follow-up on postoperative day 54.
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Fig 3. Photomicrograph of the mass demonstrating numerous multinucleated osteoblasts ("giant cells") admixed with stromal cells and matrix. (Hematoxylin and eosin; x40.)
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Comment
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Primary hyperparathyroidism is a disorder characterized by excessive production of parathyroid hormone and hypercalcemia. Primary hyperparathyroidism due to parathyroid adenoma is an adult endocrine condition. Approximately 5% of the parathyroid adenomas are found in the mediastinum, and 95% of these adenomas are located within the thymus [2].
The skeletal involvement in primary hyperparathyroidism secondary to parathyroid adenoma is extremely rare. The radiologic signs of primary hyperparathyroidism are well known. Subperiosteal resorption of the digits, skull, and long bones, diffuse osteopenia, and brown tumors describe the bone disease of primary hyperparathyroidism: "osteitic fibrosa cystica." Few patients diagnosed after introduction of multichannel autoanalyzers have the symptoms of classic primary hyperparathyroidism [3]. Clinically evident bone disease, which used to affect approximately 15% of the patient population, is now most uncommon. Although the bone involvement is decreasing, the incidence of primary hyperparathyroidism has increased [4].
Ultrasonography and thallium/technetium scanning are best for ectopic lesions in and around the thyroid gland, whereas computed tomographic scanning and magnetic resonance imaging are more effective in detecting ectopic glands in the anterior mediastinum and tracheoesophageal groove. Technetium 99m sestamibi imaging can improve sensitivity and ability to demonstrate multiple hyperplastic and ectopic glands and may become the agent of choice for imaging parathyroid tumors [5].
Surgical intervention is a safe and effective treatment for parathyroid adenoma. More than 85% of patients with parathyroid adenoma have a long-term cure after surgical removal of a single adenoma [6]. Following a successful operation for primary hyperparathyroidism, bone mass generally improves by as much as 6% to 10% in the first year [1]. Control of the hyperparathyroidism allows the bony changes to regress significantly or disappear completely [7].
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References
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Bringhurst F.R., Demay M.B., Kronenberg H.M. Hormones and disorders of mineral metabolism. In: Wilson J.D., Foster D.W., Kronenberg H.M., Larsen P.R., eds. Williams textbook of endocrinology, 9th ed. Philadelphia: WB Saunders, 1998:1155-1210.
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Taillefer R., Boucher Y., Potvin C., Lambert R. Detection and localization of parathyroid adenomas in patients with hyperparathyroidism using a single radionuclide imaging procedure with technetinum-99m-sestamibi (double-phase study). J Nucl Med 1992;33:1801-1807.[Abstract/Free Full Text]
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Rosenberg A. Bones, joints, and soft tissue tumors. In: Cotran R.S., Kumar V., Collins T., eds. Robbins pathologic basis of disease, 6th ed. Philadelphia: WB Saunders, 1999:1215-1268.
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Abstract
Introduction
