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Parathyromatosis
nephrology image
http://www.kidney-international.org & 2012 International Society of Nephrology
Kidney International (2012) 82, 1140; doi:10.1038/ki.2012.257
Parathyromatosis Tsung-Jui Wu1, Ying-Tang Wang1, Hung Chang2 and Shih-Hua Lin1 1
Division of Nephrology, Department of Internal Medicine, Tri-Service General Hospital, National Defense Medical Center, Taipei, Taiwan and Division of Thoracic Surgery, Department of Surgery, Tri-Service General Hospital, National Defense Medical Center, Taipei, Taiwan
2
Correspondence: Shih-Hua Lin, Division of Nephrology, Department of Internal Medicine, Tri-Service General Hospital, National Defense Medical Center, No. 325, Section 2, Cheng-Kung Road, Neihu 114, Taipei, Taiwan. E-mail: [email protected]
Figure 1 | 99mTc sestamibi scintigraphy demonstrates increased uptake at the left heart border.
A 45-year-old Chinese man on hemodialysis for 20 years was admitted with a 1-year history of progressive bone pain. He received left-sided video-assisted thoracoscopic surgery (VATS) to remove a mediastinal parathyroid adenoma 7 years ago. His serum intact parathyroid hormone (iPTH) concentration incrementally increased to 2500 pg/ml despite aggressive medical therapy over the past year. On examination, it was observed that he was kyphotic. Laboratory studies showed total calcium 2.8 mmol/l, inorganic phosphorus 2.27 mmol/l, and alkaline phosphatase 732 U/l. Singlephoton emission CT 99mTc sestamibi scintigraphy (Figure 1) demonstrated increased uptake at the left heart border and left chest wall in the delayed phase, suggesting functioning parathyroid nodules. Chest computed tomography (Figure 2) identified three well-enhancing pleural nodules at the left cardiophrenic angle and another over the left chest wall. All four pleural parathyroid nodules were successfully removed by VATS and showed typical hyperplastic parathyroid tissue with microfollicular structure and uniform nuclei, confirming the diagnosis of parathyromatosis. Despite symptomatic improvement, a decreased but still abnormally elevated postoperative iPTH level (928 pg/ml) was ascribable to the presence of orthotopic parathyroid glands and residual tiny parathyroid nests spread over the surgical bed. With the use of intravenous 1,25 (OH)2 vitamin D3 at a dose of 2 mg three
1140
Figure 2 | Chest computed tomography scan identifies three well-enhancing pleural nodules at the left cardiophrenic angle.
times weekly and calcium acetate at a dose of 4 gm daily for 3 months, his serum iPTH level was still around 750 pg/ml. Parathyromatosis is characterized by multiple benignappearing and functioning parathyroid nodules and can be classified into two types.1 The less common type 1 refers to ontogenous parathyromatosis along the embryological developmental tract. The more common type 2 refers to postsurgical parathyromatosis due to inadvertent spillage of parathyroid cells during surgical extirpation.2 Only 40% of parathyromatosis can be diagnosed preoperatively because of its scattered distribution and small size of individual cell nests.3 The time from previous parathyroidectomy to the identification of parathyromatosis at reoperation ranges from 5 months to 19 years.4 Parathyromatosis is not uncommon but under diagnosed. This case illustrates the importance of avoiding rupture of parathyroid capsule during the initial parathyroidectomy. 1. 2. 3.
4.
Palmer JA, Brown WA, Kerr WH et al. The surgical aspects of hyperparathyroidism. Arch Surg 1975; 110: 1004–1007. Reddick RL, Costa JC, Marx SJ. Parathyroid hyperplasia and parathyromatosis. Lancet 1977; 1: 549. Matsuoka S, Tominaga Y, Sato T et al. Recurrent renal hyperparathyroidism caused by parathyromatosis. World J Surg 2007; 31: 299–305. Melck AL, Carty SE, Seethala RR et al. Recurrent hyperparathyroidism and forearm parathyromatosis after total parathyroidectomy. Surgery 2010; 148: 867–873.
Kidney International (2012) 82, 1140
http://www.kidney-international.org & 2012 International Society of Nephrology
Kidney International (2012) 82, 1140; doi:10.1038/ki.2012.257
Parathyromatosis Tsung-Jui Wu1, Ying-Tang Wang1, Hung Chang2 and Shih-Hua Lin1 1
Division of Nephrology, Department of Internal Medicine, Tri-Service General Hospital, National Defense Medical Center, Taipei, Taiwan and Division of Thoracic Surgery, Department of Surgery, Tri-Service General Hospital, National Defense Medical Center, Taipei, Taiwan
2
Correspondence: Shih-Hua Lin, Division of Nephrology, Department of Internal Medicine, Tri-Service General Hospital, National Defense Medical Center, No. 325, Section 2, Cheng-Kung Road, Neihu 114, Taipei, Taiwan. E-mail: [email protected]
Figure 1 | 99mTc sestamibi scintigraphy demonstrates increased uptake at the left heart border.
A 45-year-old Chinese man on hemodialysis for 20 years was admitted with a 1-year history of progressive bone pain. He received left-sided video-assisted thoracoscopic surgery (VATS) to remove a mediastinal parathyroid adenoma 7 years ago. His serum intact parathyroid hormone (iPTH) concentration incrementally increased to 2500 pg/ml despite aggressive medical therapy over the past year. On examination, it was observed that he was kyphotic. Laboratory studies showed total calcium 2.8 mmol/l, inorganic phosphorus 2.27 mmol/l, and alkaline phosphatase 732 U/l. Singlephoton emission CT 99mTc sestamibi scintigraphy (Figure 1) demonstrated increased uptake at the left heart border and left chest wall in the delayed phase, suggesting functioning parathyroid nodules. Chest computed tomography (Figure 2) identified three well-enhancing pleural nodules at the left cardiophrenic angle and another over the left chest wall. All four pleural parathyroid nodules were successfully removed by VATS and showed typical hyperplastic parathyroid tissue with microfollicular structure and uniform nuclei, confirming the diagnosis of parathyromatosis. Despite symptomatic improvement, a decreased but still abnormally elevated postoperative iPTH level (928 pg/ml) was ascribable to the presence of orthotopic parathyroid glands and residual tiny parathyroid nests spread over the surgical bed. With the use of intravenous 1,25 (OH)2 vitamin D3 at a dose of 2 mg three
1140
Figure 2 | Chest computed tomography scan identifies three well-enhancing pleural nodules at the left cardiophrenic angle.
times weekly and calcium acetate at a dose of 4 gm daily for 3 months, his serum iPTH level was still around 750 pg/ml. Parathyromatosis is characterized by multiple benignappearing and functioning parathyroid nodules and can be classified into two types.1 The less common type 1 refers to ontogenous parathyromatosis along the embryological developmental tract. The more common type 2 refers to postsurgical parathyromatosis due to inadvertent spillage of parathyroid cells during surgical extirpation.2 Only 40% of parathyromatosis can be diagnosed preoperatively because of its scattered distribution and small size of individual cell nests.3 The time from previous parathyroidectomy to the identification of parathyromatosis at reoperation ranges from 5 months to 19 years.4 Parathyromatosis is not uncommon but under diagnosed. This case illustrates the importance of avoiding rupture of parathyroid capsule during the initial parathyroidectomy. 1. 2. 3.
4.
Palmer JA, Brown WA, Kerr WH et al. The surgical aspects of hyperparathyroidism. Arch Surg 1975; 110: 1004–1007. Reddick RL, Costa JC, Marx SJ. Parathyroid hyperplasia and parathyromatosis. Lancet 1977; 1: 549. Matsuoka S, Tominaga Y, Sato T et al. Recurrent renal hyperparathyroidism caused by parathyromatosis. World J Surg 2007; 31: 299–305. Melck AL, Carty SE, Seethala RR et al. Recurrent hyperparathyroidism and forearm parathyromatosis after total parathyroidectomy. Surgery 2010; 148: 867–873.
Kidney International (2012) 82, 1140