Normocalcemic hyperparathyroidism presented with mandibular brown tumor: report of a case

doi:10.1016/j.anl.2004.03.014

Auris Nasus Larynx

Volume 31, Issue 3, September 2004, Pages 299-304

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Abstract

Brown tumor is a rare clinical entity complicating hyperparathyroidism. It may occur in the head and neck, with the mandible being the most frequent site. Hyperparathyroidism is usually associated with hypercalcemia. We report a case of madibular Brown tumor secondary to primary hyperparathyroidism. In this case in spite of hyperparathyroidism and the bony lesion the serum calcium level was within normal range.

The case managed by surgical excision of the mandibular tumor with an en-bloc hemithyroidectomy with inclusion of the diseased parathyroid gland. This case demonstrates that in osteolytic bony lesions a hyperparathyroid complication can be expected even with normal serum calcium level. The presence of normocalcemia in primary hyperparathyroidism should prompt the physician to look for vitamin D deficiency.

Introduction

In 1891, Von Recklinghausen recognized the clinical impact of hyperparathyroidism and described osteitis fibrosa cystica as the pathognomonic bone lesions of this entity. In 1926, Mandl operated a parathyroid tumor in a patient with hypercalcemia and radiological changes of osteitis fibrosa cystica and demonstrated postoperative regression of the bone disease and biochemical abnormalities [1]. Incidence of primary hyperparathyridism has been reported as approximately five per 10000 population per year. Among patients aged more than 60 years, the frequency is even higher, approaching nearly one per 1000 in men and twice that in women [2]. Osteitis fibrosa cystica is classically associated with primary hyperparathyroidism. However, it is an unusual manifestation with involvement in less than 2% of primary hyperparathyroidism cases [3].

The term Brown tumor is derived from the characteristic macroscopic appearance of brownish material within the cystic lesion. This appearance results of blood pigments that are both free and within hemosiderin-laden macrophages. These “tumors” are hypervascular and intensely active on bone scans [4]. Since this lesion is reactive rather than neoplastic, the Brown tumor is not a tumor at all [5]. Although Brown tumor is very unusual entity in ENT practice, it may encountered in head and neck, in which mandible is the most common location and is involved in 4% of patients with osteitis fibrosa cystica [6]. Brown tumor has also been reported in the paranasal sinuses [7].

Brown tumor most commonly presents as a slowly enlarging, painful mass. That can be locally aggressive, without metastatic potential [8]. When present 90% of osteitis fibrosa cystica occurrences in hyperparathyroidism are due to parathyroid adenoma, of the remaining 10% are due to primary hyperplasia of parathyroid gland. We report an unusual case of Brown tumor in the mandible associated with a normal serum calcium level.

Section snippets

Case report

A 62-year-old female patient had been presented with a mass on her mandible for 7 months. Symptoms of the patient appeared 3 years ago as pain on her left leg and walking difficulties. One year later, she recognized a swelling on her left leg. She was admitted to a hospital for further investigation of left midtibial mass (Fig. 1). The bony lesion had been excised and reconstructed by a bone graft. The histopathologic diagnosis was Brown tumor. The patient had no other abnormality on her

Discussion

Early diagnoses and successful treatment regimes of hyperparathyroidism have made clinical evidence of bone disease uncommon. During the last three decades primary hyperparathyroidism has been recognized much more commonly because of the routine determination of serum calcium level by new methods and the objective parathyroid hormone radioimunoassay technique. Hyperparathyroidism is a well-known cause of hypercalcemia, and serum calcium level with musculoskeletal complications should have been

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Cited by (34)

A Diagnosis of Normocalcemic Primary Hyperparathyroidism Prompted by “Salt and Pepper” Lesions of the Calvarium
2022, AACE Clinical Case Reports
Citation Excerpt :
Previous reports have described the “salt and pepper” appearance of the calvarium in cases of hypercalcemic primary hyperparathyroidism and secondary hyperparathyroidism but typically not in cases of normocalcemia.11-13 However, 1 case report described a case of mandibular brown tumor (osteitis fibrosa cystica) in a patient who was ultimately found to have normocalcemic hyperparathyroidism but notably in the setting of 25-hydroxyvitamin D deficiency and parathyroid adenoma.14 Although the patient in the abovementioned case report shares similarities with the patient presented here, the crucial difference is that secondary causes of hyperparathyroidism were excluded in the case of our patient.
We report a case of normocalcemic primary hyperparathyroidism, a diagnosis prompted by radiographic “salt and pepper” calvarial lesions, typically described in hypercalcemic primary hyperparathyroidism or secondary hyperparathyroidism.
A 60-year-old woman noticed indentations of her scalp and presented to her primary care provider. Radiography of the calvarium demonstrated granular “salt and pepper” lesions, prompting investigation. The patient was found to have an elevated parathyroid hormone (PTH) level of 79 pg/mL (reference range, 14-54 pg/mL) and a normal albumin-corrected calcium level of 9.8 mg/dL (reference range, 8.6-10.4 mg/dL). She was referred to our endocrine clinic and described having bone aches, fevers, leg cramps, and a remote history of nephrolithiasis. Her physical examination revealed hypertension. Repeat laboratory evaluation confirmed elevated PTH and normal albumin-corrected calcium. Secondary causes of hyperparathyroidism were ruled out. Her 25-hydroxyvitamin D level was 35 ng/mL (reference range, 30-100 ng/mL), with a normal creatinine level (0.73 mg/dL; reference range, 0.5-0.99 mg/dL). The patient underwent ultrasound and sestamibi scintigraphy, with uptake in the right inferior thyroid pole. She was found to have a 6-mm parathyroid adenoma and underwent a targeted parathyroidectomy, with normalization of serum PTH.
Many cases of normocalcemic primary hyperparathyroidism are diagnosed in asymptomatic patients presenting with low bone mass; however, imaging prompted this patient's evaluation. Ultimately, the calvarial lesions were thought secondary to bone resorption from increased osteoclast activity.
This case highlights an atypical presentation of normocalcemic primary hyperparathyroidism in that the evaluation was precipitated by unexpected radiographic evidence of metabolic bone disease, rather than by symptoms or biochemical studies.
Skeletal lesions in primary hyperparathyroidism
2015, American Journal of the Medical Sciences
Citation Excerpt :
Biochemical screening is also important. Serum calcium and PTH should be examined at the same time because normal serum calcium occurs often in PHPT.30,33 A variety of bone imaging methods in combination with parathyroid radiography are also helpful to make the diagnosis.
Osteitis fibrosa cystica (OFC), a relatively rare skeletal disorder caused by excess parathyroid hormone, is often misdiagnosed as a neoplasm. A summary of the diagnostic procedures and treatment protocols, especially the indications for orthopedic surgery, is helpful to avoid overtreatment.
Eight patients from the Orthopedic Department of Qilu Hospital of Shandong University diagnosed with OFC caused by primary hyperparathyroidism were treated, and the clinical manifestations, biochemical and radiography examination findings, surgeries and prognoses were recorded.
All cases (5 female and 3 male) were admitted to our department with the complaint of bone pain (5/8) or fracture after mild trauma (3/8). Biochemical screening revealed hypercalcemia and high parathyroid hormone of varying levels. Two cases were misdiagnosed as primary bone lesion and metastasis. All cases were treated with parathyroidectomy and experienced spontaneous and progressive regression of the boney disease. Four cases underwent orthopedic surgery. Bone biopsies were necessary to exclude malignant tumors, especially with orthopedic procedures.
OFC can easily be misdiagnosed in orthopedic patients because of a lack of radiological and histologic specificity. Reaching the correct diagnosis requires a combination of clinical manifestations, routine biochemical screenings, radiographic examinations of bone and parathyroid and bone biopsy. It is generally acknowledged that parathyroidectomy is effective, but orthopedic surgery is sometimes necessary after a correct diagnosis and parathyroidectomy. However, the indications for orthopedic surgery must be strictly controlled to avoid overtreatment.
Leontiasis ossea: A presentation of hyperparathyroidism in an indigenous australian man secondary to chronic renal failure
2013, Journal of Oral and Maxillofacial Surgery
Maxillary brown tumor as initial presentation of parathyroid adenoma: A case report
2012, Kaohsiung Journal of Medical Sciences
Citation Excerpt :
The first choice in managing brown tumor caused by primary hyperparathyroidism is parathyroidectomy. Most of the bone lesions will regress with time after parathyroidectomy, thus, surgical removal of the brown tumor may not be necessary [2–5]. However, at least two studies have shown that the tumors failed to regress and became even larger after parathyroidectomy.
Brown tumor is a rare late-stage skeletal change caused by long-term stimulation of excess parathyroid hormone. It is not neoplastic, but a reparative cellular process. Common sites of brown tumor are the ribs, clavicle, long bones and pelvic girdle. Solitary maxillary brown tumor as initial presentation of primary hyperparathyroidism is rare; it is often accompanied by brown tumors of the other facial bones. Here, we present the first case of solitary maxillary brown tumor in a 29-year-old ethnic Chinese woman with initial presentation of a large tumor filling the left maxillary sinus. Underlying long-standing primary hyperparathyroidism caused by a large parathyroid adenoma was finally diagnosed. Brown tumor tends to be misdiagnosed as malignancy, and delayed diagnosis of the underlying hyperparathyroidism is common. Our case validates the suggestion that young women have a higher probability of brown tumor. Biopsy of the suspicious bone tumor and blood tests for calcium and parathyroid hormone level are crucial and essential to reach the correct diagnosis. Most brown tumors show spontaneous regression after parathyroidectomy. However, direct excision of the brown tumor may be indicated to avoid the risk of facial deformity and orbital compression at a special anatomical site, as in our case.
Normal ionized serum calcium is a prerequisite for characterization of normocalcemic primary hyperparathyroidism
2012, American Journal of the Medical Sciences
Normocalcemic Parathyroid Adenoma with Brown's Tumor Maxilla: A Rare Case
2023, Iranian Journal of Otorhinolaryngology

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Normocalcemic hyperparathyroidism presented with mandibular brown tumor: report of a case

Abstract

Introduction

Section snippets

Case report

Discussion

Endocrinol. Metab. Clin. North. Am

Am. J. Otolaryngol

Am. J. Med. Sci

Osteitis fibrosa cystica simulating metastatic tumor. An almost-forgotten relationship

Am. J. Clin. Pathol