scholarly journals Osteitis Fibrosa Cystica A Rare Presentation of Primary Hyperparathyroidism

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A207-A208
Author(s):  
Patricia Luengo Pierrard ◽  
Laura M Tortolero Giamate ◽  
Belén Porrero Guerrero ◽  
Joaquín Gómez Ramírez ◽  
Jordi Nuñez Nuñez

Abstract Introduction: Osteitis fibrosa cystica (OFC) is the most serious bone involvement of primary hyperparathyroidism (PHPT), it is characterized by subperiosteal resorption, lytic lesions and the appearance of brown tumors; this is why, in some cases, this condition can easily be mistaken for a malignant neoplasm. Its prevalence in developed countries is only 5%. Clinical Case: We present a 58-year-old woman, with no relevant personal history, who came to the emergency room with pain in her right shoulder after an accidental fall on the bus. The humerus radiograph shows a pathological fracture of the right humerus, with significant osteopenia. In the emergency analysis, serum Calcium 13.3 mg / d), Ionic Calcium 7.03 mg / dL, Phosphorus 2.4 mg / dL, Alkaline Phosphatase 248 U / L and normal kidney function stand out. With a diagnosis of severe hypercalcemia, treatment was started in the emergency room with serum therapy (1000 ml of 0.9% physiological saline in 4 hours) and intravenous diuretic treatment (furosemide 40mg) with a decrease in calcemia to 12.8mg / dL. Later, she was admitted to the Internal Medicine hospital ward to perform a differential diagnosis of hypercalcemia secondary to a primary tumor, Multiple Myeloma or Primary Hyperparathyroidism. The study findings are: Calcium metabolism: PTH 660 pg / ml (12 - 65), 25 Hydroxyvitamin D: 14.00. Thyroid ultrasound: Posterocaudal to right thyroid lobe, an area of ​​echogenicity slightly lower than the thyroid is identified, of dimensions not estimated by endothoracic component, which could correspond to a parathyroid adenoma. Body CT: Neck: Heterogeneous nodule dependent on the posterior region of the right thyroid nodule with endothoracic extension. Skeleton: Lytic lesions with a tumor aspect in the humerus, right scapula and bilateral seventh rib and right pubic branch. Skull: Diffuse increase in bone density of the calvaria, showing multiple punctate lytic lesions with a permeative appearance. Bone densitometry: Femur neck: - <1.5, Lumbar spine: - <3.0 With the diagnosis of PHPT causing osteitis fibrosa cystica, surgical intervention was decided. Under general anesthesia, a selective right approach was performed, finding a large parathyroid adenoma weighing 17 grams. PTH fell to 36 pg / ml after surgery. At 9 months after surgery, the patient presented calcium levels of 9 mg / dl and PTH 146 pg / ml with clear radiological improvement. Discussion: Osteitis fibrosa cystica is rare in our environment, it is often confused with other neoplasms. After parathyroidectomy, patients with PHPT have a marked and sustained recovery from OFC, although in some cases this recovery can only be achieved after several years. We consider this case of interest, since it illustrates the importance of evaluating the study of phospho-calcium metabolism and parathyroid function in all patients with bone lesions to rule out Primary Hyperparathyroidism with OFC.

Orthopedics ◽  
2001 ◽  
Vol 24 (10) ◽  
pp. 1000-1002
Author(s):  
Treyce S Knee ◽  
Almond J Drake ◽  
David Turton ◽  
K M Mohamed Shakir

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.


2021 ◽  
Vol 22 (1) ◽  
Author(s):  
Maya L. Nasser ◽  
Serge Medawar ◽  
Tonine Younan ◽  
Halim Abboud ◽  
Viviane Trak-Smayra

Abstract Background We report a case of osteitis fibrosa cystica, a rare benign resorptive bone lesion caused by hyperparathyroidism, that presented on imaging as an aggressive bone tumor. Case presentation The patient is a 51-year-old male complaining of severe sustained pain of the right hip region. Imaging studies were suspicious for a malignant tumor of the right iliac bone. Biopsy under CT guidance was performed and showed remodeled bone trabeculae with numerous osteoclasts, excluding bone tumor and raising the possibility of osteitis fibrosa cystica. Complementary tests disclosed elevated blood level of parathyroid hormone and a partially cystic enlarged left inferior parathyroid gland consistent with adenoma. After parathyroidectomy, the clinical symptoms were relieved and the radiological findings were significantly improved, which confirmed the diagnosis. Conclusions Metabolic diseases-associated bone lesions should always be considered in the differential diagnosis of bone tumors, to avoid unnecessary surgeries and treatments.


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.


2010 ◽  
Vol 2 (1) ◽  
pp. 45-50 ◽  
Author(s):  
Roy Phitayakorn ◽  
Christopher R McHenry

ABSTRACT Introduction To report two unusual cases of primary hyperparathyroidism (HPT) that initially manifested with a “ jaw tumor” and to discuss the clinical implications of a giant cell granuloma vs an ossifying fibroma of the jaw. Material and methods The history, physical examination, laboratory values and the imaging and pathologic findings are described in two patients who presented with a “jaw tumor” and were subsequently diagnosed with primary HPT. The diagnosis and management of osteitis fibrosa cystica and HPT-jaw tumor syndrome are reviewed. Results Patient #1 was a 70-year-old male who presented with hypercalcemia, severe jaw pain, and an enlarging mass in his mandible. Biopsy of the mass revealed a giant cell tumor and he was subsequently diagnosed with primary HPT. A sestamibi scan demonstrated a single focus of abnormal radiotracer accumulation, corresponding to a 13,470 mg parathyroid adenoma, which was resected. Postoperatively, the serum calcium normalized and the giant cell granuloma regressed spontaneously. Patient #2 was a 36-year-old male with four incidentally discovered tumors of the mandible and maxilla, who was diagnosed with normocalcemic HPT and vitamin D deficiency. Biopsy of one of the tumors revealed an ossifying fibroma. Bilateral neck exploration revealed a 2480 mg right inferior parathyroid adenoma, which was resected. Postoperative genetic testing revealed an HRPT2 gene mutation. He subsequently underwent resection of an enlarging ossifying fibroma of the mandible with secondary reconstruction. Conclusions A “jaw tumor” in a patient with primary HPT may be a manifestation of osteitis fibrosa cystica or HPT-jaw tumor syndrome underscoring the importance of biopsy and genetic testing for management and follow-up.


2022 ◽  
Author(s):  
Xiaolong Xu ◽  
Cuiping Yang ◽  
Shengjun Lu ◽  
Hong Pei ◽  
Shunguang Chen ◽  
...  

Abstract Background: Osteitis fibrosa cystica is a rare benign, lytic bone lesion attributed to hyperparathyroidism. The high level of parathyroid hormone cause rapid bone loss.Case presentation: The patient is a 50-year-old male complaining of severe sustained pain of the right knee joint. Imaging studies were suspicious for a benign tumor of the right distal femur. Biopsy under CT guidance showed numerous osteoclast aggregation and hemosiderin deposition around the bone trabeculae. Blood tests disclosed significantly elevated parathyroid hormone, serum calcium, serum alkaline phosphatase. Parathyroid ultrasonography and CT scan showed a solid mass in front of the trachea at the thoracic entrance plane. After resection of the mass, the clinical symptoms were relieved and the radiological findings were significantly improved, which further confirmed the diagnosis.Conclusions: Metabolic diseases-associated bone lesions require a comprehensive diagnosis of multiple inspection items.An interprofessional team approach to the diagnosis and treatment of Osteitis fibrosa cystica will provide the best outcome.


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

2011 ◽  
Vol 3 (3) ◽  
pp. 128-130
Author(s):  
Ana Karena Neukirch ◽  
Christian Heckmann ◽  
Norbert Weyerbrock ◽  
Silke Günther ◽  
Cornelia Dotzenrath

ABSTRACT A 56-year-old female patient with the phenotype of Albright's hereditary osteodystrophy (AHO) and pseudohypoparathyroidism 1a (PHP) diagnosed in 1987 was shown to have a heterozygote inactivating mutation on the GNAS1 gene. The patient has been treated with oral calcium and vitamin D since diagnosis of PHP 1a and developed primary hyperparathyroidism (pHPT) in 2009. Ultrasound as well as 99Tcsestamibi could demonstrate a lesion in the right lower position. Intraoperatively, a solitary parathyroid adenoma was found and resected leading to a ‘normalization’ of PTH and calcium. This case highlight is the rare coincidence of PHP, AHO and pHPT.


Sign in / Sign up

Export Citation Format

Share Document