INTERESTING IMAGE
Hyperparathyroidism and Hungry Bone Syndrome Revisited Rajeev Varma, MD, Yeonsoo James Kim, MD, Kareen Garjian, MD, and David Barank, MD Abstract: A 36-year-old woman presented with a calcium level of 15.7 mg/dL (normal, 8.5Y11.0 mg/dL) and an extremely elevated parathyroid hormone level of 1549.6 pg/mL (normal, 10.0Y65.0 pg/ mL). She subsequently underwent a parathyroid scan and ultrasound evaluation, which suggested parathyroid carcinoma. Biopsy of a lytic lesion in her left iliac bone was performed because of concern for metastatic disease but revealed a brown tumor. The patient then underwent parathyroidectomy. The patient’s postsurgical course was complicated by protracted symptomatic hypocalcemia consistent with hungry bone syndrome. Key Words: primary hyperparathyroidism, brown tumor, hungry bone syndrome (Clin Nucl Med 2014;39: 704Y706)
Received for publication June 29, 2011; revision accepted May 8, 2014. From the Department of Radiology HarborYUCLA Medical Center, Torrance, CA. Conflicts of interest and sources of funding: none declared. Reprints: Rajeev Varma, MD, Department of Radiology HarborYUCLA Medical Center, Box 27 Torrance, CA 90502. E-mail: [email protected]. Copyright * 2014 by Lippincott Williams & Wilkins ISSN: 0363-9762/14/3908Y0704
704
www.nuclearmed.com
REFERENCES 1. Ruda JM, Hollenbeak CS, Stack BC Jr. A systematic review of the diagnosis and treatment of primary hyperparathyroidism from 1995 to 2003. Otolaryngol Head Neck Surg. 2005;132:359Y372. 2. Ou HY, Hung CJ, Hsu WH, et al. Variability of clinical presentations in three cases of parathyroid carcinoma. J Formos Med Assoc. 2003;102:266Y269. 3. Pai M, Park CH, Kim BS, et al. Multiple brown tumors in parathyroid carcinoma mimicking metastatic bone disease. Clin Nucl Med. 1997;22:691Y694. 4. Lu G, Shih WJ, Xiu JY. Technetium-99m MIBI uptake in recurrent parathyroid carcinoma and brown tumors. J Nucl Med. 1995;36:811Y813. 5. Gupta A, Horattas MC, Moattari AR, et al. Disseminated brown tumors from hyperparathyroidism masquerading as metastatic cancer: a complication of parathyroid carcinoma. Am Surg. 2001;67:951Y955. 6. Hsieh MC, Ko JY, Eng HL. Pathologic fracture of the distal femur in osteitis fibrosa cystica simulating metastatic disease. Arch Orthop Trauma Surg. 2004; 124:498Y501. 7. Mautalen CA, Gonzalez D, Casco C, et al. Healing of osteitis fibrosa cystica after partial parathyroidectomy despite persistent parathyroid hyperfunction: a case report. Metab Bone Dis Relat Res. 1982;4:187Y191. 8. Chavda DV, Frock JT, Zielinski CM, et al. Reversal of the histology of bone after parathyroidectomy in patients with hyperparathyroidism. J South Orthop Assoc. 1998;7:65Y71. 9. Brasier AR, Nussbaum SR. Hungry bone syndrome: clinical and biochemical predictors of its occurrence after parathyroid surgery. Am J Med. 1988;84: 654Y660. 10. Bhattacharyya A, Buckler JP. Hungry bone syndromeVrevisited. J R Coll Physicians Edinb. 2002;32:83Y86.
Clinical Nuclear Medicine
& Volume 39, Number 8, August 2014
Copyright © 2014 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
Clinical Nuclear Medicine
& Volume 39, Number 8, August 2014
Hyperparathyroidism and Hungry Bone Syndrome
FIGURE 1. A 36-year-old woman presented with calcium level of 15.7 mg/dL (normal, 8.5Y11.0 mg/dL) and an extremely high parathyroid hormone (PTH) level of 1549.6 pg/mL (normal, 10.0Y65.0 pg/ mL). She subsequently underwent a parathyroid scan and ultrasound evaluation that suggested parathyroid carcinoma. A bone scan demonstrated a superscan appearance consistent with metabolic bone disease. There is diffuse, intense uptake most notably in the periorbital region (‘‘lone ranger sign,’’ arrow) and mandible (triangle) as well as markedly decreased activity in the kidneys (asterisk) and bladder (thick arrow).
FIGURE 2. A radiograph demonstrates lytic lesions in the right femoral neck (arrow), right superior (thick arrow) and inferior pubic bones (triangle), and left iliac wing (asterisk) as well as blurred sacroiliac joints (curved arrows) because of bone resorption. * 2014 Lippincott Williams & Wilkins
www.nuclearmed.com
Copyright © 2014 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
705
Clinical Nuclear Medicine
Varma et al
& Volume 39, Number 8, August 2014
FIGURE 3. A CT-guided biopsy of the left iliac bone was performed because of concern for metastatic disease. The pathological diagnosis was returned as a giant cell tumor of hyperparathyroidism, also known as brown tumor. A biopsy specimen showing giant cells (thick arrow) interspersed in the stroma is pictured.
FIGURE 4. The patient subsequently underwent surgery for en bloc resection of her left parathyroid, left thyroid and isthmus, and left recurrent laryngeal nerve. Pathology revealed tissue consistent with parathyroid carcinoma. Postoperatively, the patient experienced a prolonged course of hypocalcemia consistent with hungry bone syndrome. This complication required intravenous administration of calcium. She was eventually able to maintain normal calcium levels with oral calcium supplements. A pelvic radiograph taken almost a year after her surgery shows partial resolution of the brown tumors with sclerotic changes in the right pubic symphysis (arrow) and the left iliac wing (thick arrow). This presented case is a rare instance of primary hyperparathyroidism due to parathyroid carcinoma, occurring in less than 1% of primary hyperparathyroidism cases.1,2 Osteitis fibrosa cystica is a term used to denote advanced bony changes of hyperparathyroidism characterized by softened and deformed bones. Cysts that develop in this condition are known as brown tumors.3 Brown tumors are histologically indistinguishable from giant cell tumors and are often referred to as ‘‘giant cell tumors of hyperparathyroidism.’’ Multiple brown tumors from hyperparathyroidism present a diagnostic dilemma as they may be difficult to differentiate from metastatic lesions.3Y6 Parathyroid resection has been associated with rapid healing of the bone lesions of osteitis fibrosa cystica.7,8 Our patient also experienced hungry bone syndrome, which occurs in approximately 12% of primary hyperparathyroidism patients after parathyroid resection.9 It is characterized by significant symptomatic hypocalcemia, low serum phosphate, and increasing serum alkaline phosphatase after parathyroidectomy. The presence of osteitis fibrosa cystica and high preoperative parathyroid hormone levels as seen in our patient are considered predictors of developing hungry bone syndrome. Other risk factors include advanced age, large parathyroid adenoma, and high calcium or alkaline phosphatase levels. This syndrome is a potentially life-threatening complication and requires prolonged treatment with intravenous calcium and vitamin D therapy.10 706
www.nuclearmed.com
* 2014 Lippincott Williams & Wilkins
Copyright © 2014 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
Hyperparathyroidism and Hungry Bone Syndrome Revisited Rajeev Varma, MD, Yeonsoo James Kim, MD, Kareen Garjian, MD, and David Barank, MD Abstract: A 36-year-old woman presented with a calcium level of 15.7 mg/dL (normal, 8.5Y11.0 mg/dL) and an extremely elevated parathyroid hormone level of 1549.6 pg/mL (normal, 10.0Y65.0 pg/ mL). She subsequently underwent a parathyroid scan and ultrasound evaluation, which suggested parathyroid carcinoma. Biopsy of a lytic lesion in her left iliac bone was performed because of concern for metastatic disease but revealed a brown tumor. The patient then underwent parathyroidectomy. The patient’s postsurgical course was complicated by protracted symptomatic hypocalcemia consistent with hungry bone syndrome. Key Words: primary hyperparathyroidism, brown tumor, hungry bone syndrome (Clin Nucl Med 2014;39: 704Y706)
Received for publication June 29, 2011; revision accepted May 8, 2014. From the Department of Radiology HarborYUCLA Medical Center, Torrance, CA. Conflicts of interest and sources of funding: none declared. Reprints: Rajeev Varma, MD, Department of Radiology HarborYUCLA Medical Center, Box 27 Torrance, CA 90502. E-mail: [email protected]. Copyright * 2014 by Lippincott Williams & Wilkins ISSN: 0363-9762/14/3908Y0704
704
www.nuclearmed.com
REFERENCES 1. Ruda JM, Hollenbeak CS, Stack BC Jr. A systematic review of the diagnosis and treatment of primary hyperparathyroidism from 1995 to 2003. Otolaryngol Head Neck Surg. 2005;132:359Y372. 2. Ou HY, Hung CJ, Hsu WH, et al. Variability of clinical presentations in three cases of parathyroid carcinoma. J Formos Med Assoc. 2003;102:266Y269. 3. Pai M, Park CH, Kim BS, et al. Multiple brown tumors in parathyroid carcinoma mimicking metastatic bone disease. Clin Nucl Med. 1997;22:691Y694. 4. Lu G, Shih WJ, Xiu JY. Technetium-99m MIBI uptake in recurrent parathyroid carcinoma and brown tumors. J Nucl Med. 1995;36:811Y813. 5. Gupta A, Horattas MC, Moattari AR, et al. Disseminated brown tumors from hyperparathyroidism masquerading as metastatic cancer: a complication of parathyroid carcinoma. Am Surg. 2001;67:951Y955. 6. Hsieh MC, Ko JY, Eng HL. Pathologic fracture of the distal femur in osteitis fibrosa cystica simulating metastatic disease. Arch Orthop Trauma Surg. 2004; 124:498Y501. 7. Mautalen CA, Gonzalez D, Casco C, et al. Healing of osteitis fibrosa cystica after partial parathyroidectomy despite persistent parathyroid hyperfunction: a case report. Metab Bone Dis Relat Res. 1982;4:187Y191. 8. Chavda DV, Frock JT, Zielinski CM, et al. Reversal of the histology of bone after parathyroidectomy in patients with hyperparathyroidism. J South Orthop Assoc. 1998;7:65Y71. 9. Brasier AR, Nussbaum SR. Hungry bone syndrome: clinical and biochemical predictors of its occurrence after parathyroid surgery. Am J Med. 1988;84: 654Y660. 10. Bhattacharyya A, Buckler JP. Hungry bone syndromeVrevisited. J R Coll Physicians Edinb. 2002;32:83Y86.
Clinical Nuclear Medicine
& Volume 39, Number 8, August 2014
Copyright © 2014 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
Clinical Nuclear Medicine
& Volume 39, Number 8, August 2014
Hyperparathyroidism and Hungry Bone Syndrome
FIGURE 1. A 36-year-old woman presented with calcium level of 15.7 mg/dL (normal, 8.5Y11.0 mg/dL) and an extremely high parathyroid hormone (PTH) level of 1549.6 pg/mL (normal, 10.0Y65.0 pg/ mL). She subsequently underwent a parathyroid scan and ultrasound evaluation that suggested parathyroid carcinoma. A bone scan demonstrated a superscan appearance consistent with metabolic bone disease. There is diffuse, intense uptake most notably in the periorbital region (‘‘lone ranger sign,’’ arrow) and mandible (triangle) as well as markedly decreased activity in the kidneys (asterisk) and bladder (thick arrow).
FIGURE 2. A radiograph demonstrates lytic lesions in the right femoral neck (arrow), right superior (thick arrow) and inferior pubic bones (triangle), and left iliac wing (asterisk) as well as blurred sacroiliac joints (curved arrows) because of bone resorption. * 2014 Lippincott Williams & Wilkins
www.nuclearmed.com
Copyright © 2014 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
705
Clinical Nuclear Medicine
Varma et al
& Volume 39, Number 8, August 2014
FIGURE 3. A CT-guided biopsy of the left iliac bone was performed because of concern for metastatic disease. The pathological diagnosis was returned as a giant cell tumor of hyperparathyroidism, also known as brown tumor. A biopsy specimen showing giant cells (thick arrow) interspersed in the stroma is pictured.
FIGURE 4. The patient subsequently underwent surgery for en bloc resection of her left parathyroid, left thyroid and isthmus, and left recurrent laryngeal nerve. Pathology revealed tissue consistent with parathyroid carcinoma. Postoperatively, the patient experienced a prolonged course of hypocalcemia consistent with hungry bone syndrome. This complication required intravenous administration of calcium. She was eventually able to maintain normal calcium levels with oral calcium supplements. A pelvic radiograph taken almost a year after her surgery shows partial resolution of the brown tumors with sclerotic changes in the right pubic symphysis (arrow) and the left iliac wing (thick arrow). This presented case is a rare instance of primary hyperparathyroidism due to parathyroid carcinoma, occurring in less than 1% of primary hyperparathyroidism cases.1,2 Osteitis fibrosa cystica is a term used to denote advanced bony changes of hyperparathyroidism characterized by softened and deformed bones. Cysts that develop in this condition are known as brown tumors.3 Brown tumors are histologically indistinguishable from giant cell tumors and are often referred to as ‘‘giant cell tumors of hyperparathyroidism.’’ Multiple brown tumors from hyperparathyroidism present a diagnostic dilemma as they may be difficult to differentiate from metastatic lesions.3Y6 Parathyroid resection has been associated with rapid healing of the bone lesions of osteitis fibrosa cystica.7,8 Our patient also experienced hungry bone syndrome, which occurs in approximately 12% of primary hyperparathyroidism patients after parathyroid resection.9 It is characterized by significant symptomatic hypocalcemia, low serum phosphate, and increasing serum alkaline phosphatase after parathyroidectomy. The presence of osteitis fibrosa cystica and high preoperative parathyroid hormone levels as seen in our patient are considered predictors of developing hungry bone syndrome. Other risk factors include advanced age, large parathyroid adenoma, and high calcium or alkaline phosphatase levels. This syndrome is a potentially life-threatening complication and requires prolonged treatment with intravenous calcium and vitamin D therapy.10 706
www.nuclearmed.com
* 2014 Lippincott Williams & Wilkins
Copyright © 2014 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
