scholarly journals MON-347 Osteitis Fibrosa Cystica and Pathological Fractures: The Classic but Neglected Skeletal Manifestation of Primary Hyperparathyroidism

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Sirinart Sirinvaravong ◽  
Nontouch Singsumpun ◽  
Ekasame Vanitcharoenkul ◽  
Aasis Unnanuntana

Abstract Background: Osteitis fibrosa cystica is one of the classic manifestations of primary hyperparathyroidism (PHPT), yet it has become increasingly rare due to early detection of PHPT. Clinical case: A 37-year-old woman was referred to our hospital for fixation of multiple fractures. Before transferring to our hospital, she was admitted to a local hospital due to right distal humerus and left tibial fractures without history of trauma. During the hospitalization, a new fracture at left femur had occurred. Her past medical history was significant for fractures of right shoulder, left elbow and right femur when she fell on the ground 5 years ago and treated by fixation. She had a history of kidney stones in the remote past. She denied family history of calcium or skeletal disorders. Clinical examination revealed gross deformities at right elbow, left thigh and painful swelling of the left tibia. On HEENT examination, there were no palpable neck masses. Upon review of plain radiographs from outside hospital, we found not only fractures but diffuse osteopenia and brown tumors at multiple sites including shaft of right humerus, right proximal radius, left proximal femoral shaft and left tibia. Skull X-ray showed salt-and-pepper appearance. There were prominent subendplate densities at multiple lumbar spines (Rugger-jersey spine). These findings were consistent with osteitis fibrosa cystica which prompted further laboratory investigation for PHPT. The patient had a high corrected serum calcium level of 13.6 (8.6-10.0) mg/dl, low serum phosphate of 2.2 (2.5-4.5) mg/dL, serum creatinine of 1.16 (0.51-0.95) mg/dL, glomerular filtration rate (GFR) of 60.29 ml/min, high serum alkaline phosphatase of 1,482 (35-105) U/L. The serum parathyroid hormone (PTH) level was significantly high at 3,850 (15-65) pg/mL. Vitamin D level was low at 5.98 (≥30) ng/mL. The patient was diagnosed with PHPT. The left lower parathyroid adenoma was identified by 99mTc-Sestamibi scan with single photon emission computed tomography and neck ultrasound. Given severe skeletal manifestations and extremely elevated PTH level, urgent parathyroidectomy was performed. A 2.5-cm parathyroid adenoma was removed which was histologically confirmed by a pathologist. Postoperatively, she developed a hungry bone syndrome which resolved 10 months after. Clinical lesson: In current practice, skeletal manifestation of PHPT is uncommon with a reported incidence of 1.4% in US. Osteitis fibrosa cystica is a radiographic feature of PHPT characterized by demineralized skeleton, salt-and-pepper appearance of the skull, subperiosteal resorption of the phalanges, bone cysts, and brown tumors. Although PHPT has evolved to asymptomatic disease in majority of patients, the classic skeletal involvement should not be overlooked, particularly in young patients who present with multiple pathological fractures.

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Suruchi K Gupta ◽  
Runhua Hou ◽  
Harold Rosen

Abstract Background: Brown tumors are a part of the complex “osteitis fibrosa cystica” which is a diffuse resorptive process of the bone resulting from uncontrolled hyperparathyroidism. Although these brown tumors were fairly common in the past1, the incidence of brown tumors is now extremely rare in the United States due to early diagnosis and treatment of hyperparathyroidism. Here we describe an unusual presentation for osteitis fibrosa cystica. Clinical Case: A 38 year old woman was admitted to the hospital with rapidly growing facial swelling in June 2019. The swelling initially appeared in January 2019 and had rapidly increased in size since April 2019. The mass was malodorous, painful and interfered with oral intake. The patient smoked 1 pack per day for 13 years. A CT scan of the head and neck with contrast showed a 3.6 x 4.5 x 3.3 cm mass destroying the right mandibular body, involving the right platysma muscle and displacing the central and lateral incisor teeth and was concerning for malignancy. The initial impression was that this was likely a squamous cell carcinoma with local and perhaps distant metastases, complicated by humoral hypercalcemia of malignancy and she was admitted for management. Upon admission, her calcium level was 14 mg/dL, albumin 4.1 g/dL, phosphorus 1.4 mg/dL PTH level was 890 pg/mL and vitamin D level was 22 ng/mL. Since the high PTH did not fit with hypercalcemia of malignancy, further evaluation was undertaken. A thyroid ultrasound showed a 4.6 x 1.8 x 1.5 cm isoechoic, heterogeneous lesion in the left lower pole, and Tc-99 sestamibi scan suggested parathyroid origin. The biopsy of the mouth lesion showed fibro histiocytic proliferation with multiple giant cells, negative for malignancy and consistent with a brown tumor. CT scan of the chest, abdomen and pelvis did not reveal any other masses or lytic lesions. . A skeletal survey showed another 2 cm lytic lesion in the proximal left humeral metaphysis which was not biopsied. On parathyroidectomy, the patient was found to have an enlarged left inferior parathyroid gland measuring 3.3 x 3.0 x 0.8 cm weighing 7.093 grams. Intraoperatively PTH level decreased from 890 pg/mL to 69 pg/mL. Her calcium levels returned to normal post-operatively and she was discharged home. Pathologic examination report revealed the mass to be a parathyroid adenoma. The patient reports a reduction in the size of her mandible mass since the surgery. Conclusion: Although uncommon, brown tumors can be seen in uncontrolled primary hyperparathyroidism. The mandible is a common site, though, as in this case, it is less common for it to be the only site affected Reference: 1. Rosenberg, E. H. (1962). Hyperparathyroidism. A review of 220 proved cases with special emphasis on findings in the jaws. Oral Surg Oral Med Oral Pathol Oral Radiol Endod, 15(2), 84–94.


2012 ◽  
Vol 2012 ◽  
pp. 1-3 ◽  
Author(s):  
Anthony M. Maina ◽  
Harry Kraus

Osteitis Fibrosa Cystica (OFC) is defined as the classic skeletal manifestation of advanced primary hyperparathyroidism. With the increased detection by means of routine calcium screening, the clinical profile of primary hyperparathyroidism in Western countries has shifted from symptomatic disease to one with subtle or no specific symptoms (“asymptomatic” primary hyperparathyroidism). The authors describe a classical feature of advanced primary hyperparathyroidism due to a parathyroid adenoma and its successful treatment.


2013 ◽  
Vol 31 (1) ◽  
pp. 26
Author(s):  
W.Y.M. Abeysekera ◽  
P.M.C. Udaya Kumara ◽  
A.A.W.K. Amarasinghe ◽  
D.M.H. Fernando ◽  
A.B.S. Ananda Perera

Diagnostics ◽  
2021 ◽  
Vol 11 (8) ◽  
pp. 1355
Author(s):  
Adrien Holzgreve ◽  
Matthias P. Fabritius ◽  
Thomas Knösel ◽  
Lena M. Mittlmeier ◽  
Johannes Rübenthaler ◽  
...  

Benign so-called “brown tumors” secondary to hyperparathyroidism are a rare diagnostic pitfall due to their impressively malignant-like character in various imaging modalities. We present the case of a 65-year-old male patient with multiple unclear osteolytic lesions on prior imaging suspicious for metastatic malignant disease. Eventually, findings of 18F-FDG PET/CT staging and 99mTc-MIBI scintigraphy resulted in revision of the initially suspected malignant diagnosis. This case illustrates how molecular imaging findings non-invasively corroborate the correct diagnosis of osteitis fibrosa cystica generalisata with the formation of multiple benign brown tumors.


2016 ◽  
Vol 4 (2) ◽  
pp. 283-286
Author(s):  
Olivera Stojceva-Taneva ◽  
Borjanka Taneva ◽  
Gjulsen Selim

BACKGROUND: Hypercalcemia is a common manifestation in clinical practice and occurs as a result of primary hyperparathyroidism, malignancy, milk-alkali syndrome, hyper or hypothyroidism, sarcoidosis and other known and unknown causes. Patients with milk-alkali syndrome typically are presented with renal failure, hypercalcemia, and metabolic alkalosis caused by the ingestion of calcium and absorbable alkali. This syndrome is caused by high intake of milk and sodium bicarbonate.CASE PRESENTATION: We present a 28-year old male admitted to hospital with a one-month history of nausea, vomiting, epigastric pain, increased blood pressure and worsening of renal function with hypercalcemia. His serum PTH level was almost undetectable; he had mild alkalosis, renal failure with eGFR of 42 ml/min, anemia, hypertension and abnormal ECG with shortened QT interval and ST elevation in V1-V4. He had a positive medical history for calcium-containing antacids intake and after ruling out primary hyperparathyroidism, malignancy, multiple myelomas, sarcoidosis, and thyroid dysfunction, it seemed plausible to diagnose him as having the milk-alkali syndrome.CONCLUSION: Although milk-alkali syndrome currently may be more probably a result of calcium and vitamin D intake in postmenopausal women, or in elderly men with reduced kidney function taking calcium-containing medications, one should not exclude the possibility of its appearance in younger patients taking calcium-containing medications and consider it a serious condition taking into account its possibility of inducing renal insufficiency.


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