[Brown tumors in chronic hemodialysis patients]. / Les tumeurs brunes chez les hémodialysés chroniques.

PURPOSE: Brown tumors are rare and severe manifestations of secondary hyperparathyroidism. We propose in this study: to define and illustrate brown tumors observed in our hemodialysis center; to show the frequency for 20 years in our center; to identify risk factors compared to the rest of dialysis patients; and finally to offer improved support for reducing the incidence. PATIENTS AND METHODS: We conducted a retrospective and descriptive study, over a period of 20 years (1993-2013), including 311 cumulative patients which are chronic hemodialysis in our unit. RESULTS: Twenty-one patients had brown tumors (6.75%). The average age was 36.1 years and the sex ratio M/F is of 0.6. The average time between the start of hemodialysis and the diagnosis of brown tumor was 87.6 months. Clinical symptoms were dominated by bone pain, found in 76.1% of cases. The most frequent locations were costal (28.5% of cases), while spinal involvement was less frequent (4.76% of cases). The location was multifocal in 57.1% of cases. The mean serum calcium was of 2.08 mmol/L, the serum phosphate of 2.25 mmol/L, alkaline phosphatase of 1709 IU/L and the average value of parathyroid hormone of 1934 pg/mL. Radiography was the key of diagnostic. Resonance magnetic imaging and computed tomography had an interest in the exploration of spinal locations and maxillo-mandibular locations. All patients underwent parathyroidectomy and it was total in one patient. Tumorectomy was necessary in three patients (14.2% of cases). The outcome was favorable in 85.7% of cases. CONCLUSION: Our work relates one of the most important series published of brown tumors and is characterized by the multifocal character of these tumors.

Insight into the pathogenesis and nature of Central Giant Cell Lesions of the Jaws.

Central giant cell lesions of the jaws are not uncommon. While the majority of these represent single, sporadic lesions, histologically identical lesions are seen in association with a number of other bone lesions, as well as in certain syndromes. This manuscript offers a brief update on recent developments in this area that provide new insight into the pathogenesis and nature of Central Giant Cell Lesions of the Jaws.

Giant cell tumor of bone: current treatment options.

OPINION STATEMENT: Giant cell tumor of bone (GCTB) comprises up to 20 % of benign bone tumors in the US. GCTB are typically locally aggressive, but metastasize to the lung in ~5 % of cases. Malignant transformation occurs in a small percentage of cases, usually following radiation therapy. Historically, GCTB have been treated primarily with surgery. When the morbidity of surgery would be excessive, radiation therapy may achieve local control. In most cases the primary driver of the malignant cell appears to be a mutation in H3F3A leading to a substitution of Gly34 to either Trp or Leu in Histone H3.3. This change presumably alters the methylation of the protein, and thus, its effect on gene expression. The malignant stromal cells of GCTB secrete RANKL, which recruits osteoclast precursors to the tumor and stimulates their differentiation to osteoclasts. The elucidation of the biology of GCTB led to trials of the anti-RANKL monoclonal antibody denosumab in this disease, with a clear demonstration of beneficial clinical effect. Surgery remains the primary treatment of localized GCTB. When surgery is not possible or would be associated with excessive morbidity, denosumab is a good treatment option. The optimal length of treatment and schedule of denosumab is unknown, but recurrences after apparent complete responses have been observed after stopping denosumab, and long-term follow-up of denosumab treatment may reveal unrecognized effects. The role of denosumab in the preoperative or adjuvant setting will require clinical trials. In some cases local radiation therapy may be useful, although long term effects should be considered.

Giant cell tumor of bone in a patient with diagnosis of primary hyperparathyroidism: a challenge in differential diagnosis with brown tumor.

Giant cell lesions of bone share similar clinical, radiological, and histological features. The most challenging differential diagnosis is between giant cell tumor (GCT) and brown tumor (BT) secondary to hyperparathyroidism. Differential diagnosis is based on determining serum calcium concentration and other markers of calcium metabolism. The authors present the unusual case of a 37-year-old Caucasian woman affected by a GCT of the proximal left tibia and concomitant asymptomatic primary hyperparathyroidism (PHPT) due to a parathyroid adenoma. The presence of two concurrent diseases complicated diagnosis and relative treatment. The patient was first treated for the adenoma, then after 9 months, she underwent curettage of tibial GCT. Denosumab treatment was administered for 12 months to control a relapse occurring at 15 months post-curettage. At 32-month follow-up from primary tibial surgery, the patient was free from tumor disease. To our knowledge, this is the first case in the literature reporting the concomitant presence of asymptomatic PHPT and GCT. The possibility of concomitant finding these two diseases has to be considered during the decision-making process.

Giant cell tumor of the larynx: a case of malignant sarcomatous transformation.

We report what is to the best of our knowledge the first case of malignant transformation of a giant cell tumor of the larynx. The patient, a 34-year-old man, presented to our tertiary care university teaching hospital where he underwent hemilaryngopharyngectomy with radial forearm free flap reconstruction and 11 of 15 cycles of chemotherapy. He remained disease-free at approximately 6 years and 4 months of follow-up. The patient is decannulated and continues to have a good voice with excellent quality of life to this day. We discuss the patient's clinical course and subsequent treatment within the context of a review of the current literature regarding this disease entity. Our experience demonstrates that giant cell tumor of the larynx may present as a malignant neoplasm without adversely affecting the patient's prognosis when treated aggressively with surgical resection and adjunct chemotherapy.

Malignant giant cell tumor in the posterior fossa of a neonate.

Giant cell tumors (GCTs) of the bone are rare, usually benign but locally aggressive neoplasms that primarily occur in the epiphyses of long bones. They seldom develop in the cranium; when they do, they involve principally the sphenoid and temporal bones. These tumors usually affect young adults, and few reports in children have been published. Primary malignant GCTs of the skull are even more uncommon. The 3 published cases all involved adults over 40 years of age. Herein, the authors present a case of a highly aggressive primary malignant GCT of the posterior fossa in a 5-week old preterm infant. One month after the gross-total resection of the tumor found in the bone, the infant's condition rapidly deteriorated and she died. Magnetic resonance imaging and postmortem examination revealed a tumor larger than it had been before the operation, with expansion toward the brain. To the best of the authors' knowledge, this is the youngest patient reported with a primary malignant GCT of the skull, and actually the first case in a pediatric patient. In addition, the extremely high growth rate of the tumor in the postoperative period renders this case the most aggressive primary malignant GCT of the cranium described so far.

Primary hyperparathyroidism presenting as multiple giant cell lesions.

A case of giant cell lesion as an initial feature of primary hyperparathyroidism is presented. A 55-year-old woman reported with a complaint of swelling and occasional pain in the upper-left posterior region with 2 months' duration. The diagnosis of a giant cell lesion with primary hyperparathyroidism was confirmed on the basis of radiological, biochemical, and histopathological investigative procedures.

Brown tumor with fluid-fluid levels in a patient with primary hyperparathyroidism: radiological findings.

We report a case of a brown tumor with fluid-fluid levels in a patient with primary hyperparathyroidism. A 19-year-old woman presented with a 3-month history of pain in the left pubic region. The laboratory data showed elevated serum calcium and intact parathyroid hormone, confirming the diagnosis of primary hyperparathyroidism. Plain radiography and computed tomography (CT) showed an expansile lytic lesion of the superior ramus of the left pubis. The cortex was thinned. On magnetic resonance (MR) images, the lesion was solid and cystic. The solid area of the lesion showed heterogeneous low to intermediate signal intensity on T1-weighted images and heterogeneous low to high signal intensity on T2-weighted images. The cystic area showed several fluid-fluid levels on T2-weighted images. Dynamic contrast-enhanced MR images after administration of Gd-DTPA showed marked, early enhancement of the solid area of the lesion. A delayed image showed prolonged enhancement of the solid area and enhancement of the septa and walls of the cystic area. Histopathology of a biopsy specimen showed fibroblastic proliferation, abundant giant cells, and focal hemosiderin deposition, which supported the diagnosis of a brown tumor. After removing the parathyroid adenoma, the brown tumor regressed and became sclerotic on radiographs.

Brown tumor of the mandible associated with secondary hyperparathyroidism: a case report and review of the literature.

Brown tumors are rare lesions that can develop in persistent cases of hyperparathyroidism (HPT). Therefore, identification of these lesions by diagnostic imaging is important during the follow-up of patients with HPT. This report describes a 45-year-old woman who developed HPT-induced brown tumors that appeared initially as an oral lesion. The diagnosis, treatment, and control of the disease--as well as the histopathological characteristics--are emphasized.

Giant cell tumours of the maxilla and tibia presenting concurrently as an initial manifestation of primary parathyroid adenoma.

A 14 years female of Afghan origin reported with maxillofacial and tibia growths causing progressive deformities since nine months, both were giant cell tumours on histopathology. Serum calcium was normal, but the parathyroid hormone was exaggerated (678 pg/ml). Ultrasound indicated and Tc-99m Setamibi scan confirmed a left lower parathyroid lesion. A 4cm length mass was identified, removed and proved to be a parathyroid adenoma. Two weeks later a subtotal maxillectomy and six weeks later anterior wedge osteotomy of the tibia were carried out. Serum parathyroid hormone level normalized.

Brown tumor of the facial bones: case report and literature review.

Brown tumor, an uncommon focal giant-cell lesion, arises as a direct result of the effect of parathyroid hormone on bone tissue inpatients with hyperparathyroidism. The initial treatment involves the correction of hyperparathyroidism, which usually leads to tumor regression. We report a case of brown tumor of the right nasal fossa in a 71-year-old woman. The tumor had caused nasal obstruction and epistaxis. Laboratory evaluation revealed that the patient had primary hyperparathyroidism. Anatomicopathologic investigation revealed the presence of a giant-cell tumor We performed a partial parathyroidectomy, but the tumor in the right nasal fossa failed to regress. One year later we performed surgical resection of the lesion. The patient recovered uneventfully, and she remained asymptomatic and recurrence-free at the 1-year follow-up. Facial lesions with histologic features of a giant-cell tumor should be evaluated from a systemic standpoint. Hyperparathyroidism should always be investigated by laboratory tests because most affected patients are asymptomatic. Surgical resection of a brown tumor should be considered if the mass does not regress after correction of the inciting hyperparathyroidism or if the patient is highly symptomatic.

Calcaneal brown tumor with primary hyperparathyroidism caused by parathyroid carcinoma: an atypical localization.

Brown tumors are one of the characteristics of primary hyperparathyroidism, although, in some cases, they are noted with secondary hyperparathyroidism as well. The authors present a case of a 50-year-old woman with primary hyperparathyroidism caused by parathyroid carcinoma with an unusual location of a brown tumor in the calcaneus. She first presented with pain and swelling over the heel and ankle, and the diagnosis was suspected by radiographs. Biopsy of the calcaneal lesion confirmed a brown tumor. After the parathyroid lesion was removed surgically, her symptoms were relieved. The calcaneal lesion was treated with immobilization of the foot.

Giant cell tumor and Paget's disease of bone in one family: geographic clustering.

Giant cell tumor is a rare complication of Paget's disease of bone. Typically, this tumor occurs in the case of polyostotic disease and only in pagetic bones. This tumor rarely has been seen in multiple family members who have Paget's disease, although Paget's bone disease clearly has a hereditary component. Our report documents four cases of polyostotic Paget's bone disease complicated by benign giant cell tumor. In two patients, the giant cell tumor also was multifocal. All patients were from one family. They were born in Avellino and reside in Campania, a Southern Italian region. The ancestors of the patients with familial giant cell tumor in Paget's bone disease were born in the same geographic area. These data suggest that a combination of environmental and genetic factors could be responsible for linkage of the patients born in Avellino with this neoplasm that is highly unusual in patients with Paget's disease of bone.