Brown tumors of the jaw: an atypical manifestation of primary hyperparathyroidism.

Brown tumors represent the terminal stage of bone remodeling processes in primary hyperparathyroidism. Currently they are rare, and typically affect long bones, pelvis and ribs. Brown tumors may be not included in the initial differential diagnosis of bone disease, especially when they are present in atypical localizations. We reported two cases of oral brown tumors as the initial presentation of primary hyperparathyroidism. In the first case, a 44-year-old woman presented a painful and sessile lesion of 4 × 3 cm over the central body of the mandible which progressively increased in 4-month. The second case involved a 23-year-old woman who was referred with a 3-month history of a painful and ulcerated mass of 2 cm arising from left maxilla, episodes of gingival hemorrhage and difficulty of breathing. Both cases were solitary tumors with no evidence of palpable cervical lymphadenopathy. Incisional biopsy of oral tumors resulted in giant cell and primary hyperparathyroidism was confirmed by laboratory tests. After parathyroidectomy, histology confirmed adenoma in both cases. Although this type of clinical presentation has almost disappeared in the recent decades, brown tumors should be considered in the differential diagnosis of bone oral masses.

Los tumores pardos son raros y, por lo general, afectan huesos largos, pelvis y costillas. Pueden no estar incluidos en el diagnóstico diferencial inicial como manifestación de enfermedad esquelética, especialmente cuando se presentan en localizaciones atípicas. Comunicamos dos casos de tumores pardos orales como presentación inicial de hiperparatiroidismo primario. En el primer caso, una mujer de 44 años presentó una lesión dolorosa y sésil de 4 × 3 cm sobre el cuerpo central de la mandíbula que aumentó de tamaño progresivamente en 4 meses. El segundo caso corresponde a una mujer de 23 años que acudió por presentar una masa dolorosa y ulcerada de 2 cm en maxilar izquierdo de 3 meses de evolución, episodios de hemorragia gingival y dificultad para respirar. Todos fueron tumores solitarios sin evidencia de linfadenopatía cervical palpable. La biopsia incisional de los tumores orales resultó en células gigantes, y las pruebas de laboratorio confirmaron el hiperparatiroidismo primario. Tras la paratiroidectomía, la histología confirmó adenoma en ambos casos. Los tumores pardos representan la etapa terminal de los procesos de remodelación ósea en el hiperparatiroidismo primario. Aunque este tipo de presentación clínica casi ha desaparecido en las últimas décadas, los tumores pardos deben ser considerados en el diagnóstico diferencial de las masas óseas orales.

Manifestations of hyperparathyroidism in the jaws: Concepts, mechanisms, and clinical aspects.

Hyperparathyroidism is one of the most common endocrine disorders worldwide. In countries where routine biochemical screening is not common, symptomatic hyperparathyroidism predominates. Its manifestations include skeletal alterations, calcification of soft tissues, kidney stones, and functional alterations in other systems. Notably, jaw alterations can be the first clinical sign of hyperparathyroidism, including brown tumor, renal osteodystrophy, osteitis fibrosa, and leontiasis ossea, and knowing such conditions is of core importance for the multidisciplinary diagnosis and management of hyperparathyroidism. We aimed to perform a concise review, systematizing the concepts and mechanisms underlying hyperparathyroidism and associated gnathic alterations. In addition, a detailed description of the clinical aspects of the jaw manifestations is presented.

Manejo quirúrgico de tumor pardo en metacarpo. Reporte de caso / Surgical management of Metacarpal brown tumor. A case report

El tumor pardo, también conocido como osteoclastoma ó como osteítis fibrosa quística, es un tumor lítico, que se presenta en hiperparatiroidismo (primario, secundario y terciario), aunque su presentación habitual es altamente invasiva, no tiene potencial de malignidad. Los tumores pardos en la mano son muy poco frecuentes y existen solo algunos reportes de casos. Presentamos un paciente masculino de 18 años con una tumoración dura, no móvil, adherida a planos profundos en región dorsal de la mano derecha sobre el cuarto metacarpiano, que además limita la flexión y extensión del cuarto dedo sin alterar su función neurovascular. El paciente fue sometido a resección de la tumoración que involucraba por completo al cuarto metacarpiano derecho, además se realizó un abordaje lateral directo en miembro pelvico izquierdo para tomar un injerto autólogo de peroné no vascularizado. Es importante la detección temprana de este tipo de tumores y se debe dar un adecuado seguimiento, ya que, al progresar, generan una destrucción ósea importante y el tratamiento se vuelve de mayor complejidad. En etapas tempranas, el manejo agresivo con resección y aporte óseo puede evitar secuelas funcionales. El uso de injerto no vascularizado de peroné de seis centímetros para la sustitución del cuarto metacarpiano por osteolísis secundaria a un tumor pardo es una alternativa adecuada de tratamiento que permite la preservación estético funcional de la mano.

The brown tumour, also known as osteoclastoma, or as osteitis fibrosa cystica, is a lytic tumour, which occurs in hyperparathyroidism (primary, secondary, and tertiary), although its usual presentation is highly invasive, has no potential for malignancy. Brown tumours of the hand are sporadic, and there are only few case reports. The case is presented of an 18-year-old male patient with a solid, non-mobile tumour, adhered to deep planes, in the dorsal region of the right hand over the fourth metacarpal. This also limited the flexion and extension of the fourth finger, but did not show alterations in the neurovascular function of the finger. The patient underwent a tumour resection that completely involved the right fourth metacarpal. A direct lateral approach was made in the left pelvic limb to perform a non-vascularised autologous fibular graft. Early detection of this type of tumour is important, and an adequate follow-up must be carried out, since when they progress, they generate significant bone destruction and the treatment becomes more complex. In early stages, aggressive management of resection and bone support can prevent functional sequelae.

Expansive renal osteitis fibrosa: a case report.

Hyperparathyroidism (HPT) is an endocrine metabolic disorder characterized by increased secretion of parathyroid hormone. Untreated secondary HPT leads to renal osteodystrophy (ROD). Facial skeletal abnormalities in patients with ROD are rare. The purpose of this paper is to report a conservative surgical approach of exuberant osteitis fibrosa lesions in patient with chronic kidney disease. A 24-year-old female was referred to maxillofacial surgery department with giants ROD affecting palate, maxilla, and mandible, resulting in esthetic and functional impairment. The pathogeneses and multidisciplinary management of ROD are discussed with a brief literature review. Eight years after the conservative treatment of exuberant jaw lesions, no noticeable bone changes were observed in the patient. A multidisciplinary therapy is essential for correct diagnosis of ROD and optimal multimodality treatment. The conservative management was an efficient alternative for the success of the case reported.

Unusually rapid growth of brown tumour in the mandible after parathyroidectomy associated with the presence of a supernumerary parathyroid gland.

The aim of this study is to report the case of a quick growing brown tumour in the jaw after a parathyroidectomy due to the presence of a rare fifth parathyroid gland. The patient had chronic renal disease and the diagnosis was tertiary hyperparathyroidism. Thirty days after the parathyroidectomy, the patient returned with a significant increase in the tumour size. The suspicion of a supernumerary gland was confirmed by parathyroid scintigraphy. The treatment of brown tumour is dependent on the treatment of the hyperparathyroidism. However, curettage should be considered if a large lesion is disturbing mastication. In conclusion, this case should attract the attention of general practitioner dentists, since they may be the first professionals who have contact with the patient with a brown tumour in the jaws. Likewise, this case emphasises the importance of knowing the type of hyperparathyroidism involved to allow for effective treatment planning.

Prevalence of radiological findings among cases of severe secondary hyperparathyroidism.

CONTEXT AND OBJECTIVE: Patients with end stage renal disease (ESRD) and secondary hyperparathyroidism (HPT2) are prone to develop heterotopic calcifications and severe bone disease. Determination of the sites most commonly affected would decrease costs and patients' exposure to X-ray radiation. The aim here was to determine which skeletal sites produce most radiographic findings, in order to evaluate hemodialysis patients with HPT2, and to describe the most prevalent radiographic findings. DESIGN AND SETTING: This study was cross-sectional, conducted in one center, the Hospital Universitário Clementino Fraga Filho (HUCFF), in Rio de Janeiro, Brazil. METHODS: Whole-body radiographs were obtained from 73 chronic hemodialysis patients with indications for parathyroidectomy due to severe HPT2. The regions studied were the skull, hands, wrists, clavicles, thoracic and lumbar column, long bones and pelvis. All the radiographs were analyzed by the same two radiologists, with great experience in bone disease interpretation. RESULTS: The most common abnormality was subperiosteal bone resorption, mostly at the phalanges and distal clavicles (94% of patients, each). 'Rugger jersey spine' sign was found in 27%. Pathological fractures and deformities were seen in 27% and 33%, respectively. Calcifications were presented in 80%, mostly at the forearm fistula (42%), abdominal aorta and lower limb arteries (35% each). Brown tumors were present in 37% of the patients, mostly on the face and lower limbs (9% each). CONCLUSION: The greatest prevalence of bone findings were found on radiographs of the hands, wrists, lateral view of the thoracic and lumbar columns and femurs. The most prevalent findings were bone resorption and ectopic calcifications.

Prevalence of radiological findings among cases of severe secondary hyperparathyroidism / Prevalência de achados radiológicos em pacientes com grave hiperparatireoidismo secundário

CONTEXT AND OBJECTIVE: Patients with end stage renal disease (ESRD) and secondary hyperparathyroidism (HPT2) are prone to develop heterotopic calcifications and severe bone disease. Determination of the sites most commonly affected would decrease costs and patients' exposure to X-ray radiation. The aim here was to determine which skeletal sites produce most radiographic findings, in order to evaluate hemodialysis patients with HPT2, and to describe the most prevalent radiographic findings. DESIGN AND SETTING: This study was cross-sectional, conducted in one center, the Hospital Universitário Clementino Fraga Filho (HUCFF), in Rio de Janeiro, Brazil. METHODS: Whole-body radiographs were obtained from 73 chronic hemodialysis patients with indications for parathyroidectomy due to severe HPT2. The regions studied were the skull, hands, wrists, clavicles, thoracic and lumbar column, long bones and pelvis. All the radiographs were analyzed by the same two radiologists, with great experience in bone disease interpretation. RESULTS: The most common abnormality was subperiosteal bone resorption, mostly at the phalanges and distal clavicles (94 percent of patients, each). "Rugger jersey spine" sign was found in 27 percent. Pathological fractures and deformities were seen in 27 percent and 33 percent, respectively. Calcifications were presented in 80 percent, mostly at the forearm fistula (42 percent), abdominal aorta and lower limb arteries (35 percent each). Brown tumors were present in 37 percent of the patients, mostly on the face and lower limbs (9 percent each). CONCLUSION: The greatest prevalence of bone findings were found on radiographs of the hands, wrists, lateral view of the thoracic and lumbar columns and femurs. The most prevalent findings were bone resorption and ectopic calcifications.

CONTEXTO E OBJETIVO: Pacientes com hiperparatireoidismo secundário (HPT2) à insuficiência renal crônica são propensos a desenvolver calcificações ectópicas e grave doença óssea. A determinação dos sítios mais revelantes pode diminuir o custo e a exposição do paciente a radiação desnecessária. O objetivo foi determinar quais locais radiológicos apresentam mais achados radiográficos para avaliar o HPT2 em pacientes em hemodiálise, assim como os achados mais prevalentes. TIPO DE ESTUDO E LOCAL: Estudo transversal, realizado no Hospital Universitário Clementino Fraga Filho (HUCFF), no Rio de Janeiro, Brasil. MÉTODOS: Radiografias de corpo inteiro foram obtidas de 73 pacientes em hemodiálise crônica que tiveram indicação de paratireoidectomia devido a HPT2 grave. As regiões estudadas foram crânio, mãos e punhos, clavículas, coluna torácica e lombar, ossos longos e pélvis. Todas as imagens foram analisadas pelos mesmos dois radiologistas, com grande experiência na interpretação de doenças do osso. RESULTADOS: A alteração mais comum foi reabsorção óssea subperiosteal, principalmente nas falanges distais e clavículas (ambos em 94 por cento de pacientes). Sinal de "rugger jersey" foi descoberto em 27 por cento. Fratura patológica e deformidades foram visualizadas em 27 por cento e 33 por cento, respectivamente. As calcificações foram encontradas em 80 por cento dos pacientes, principalmente na fístula de antebraço (42 por cento), aorta abdominal e artérias dos membros inferiores (ambos 35 por cento). Tumores marrons estavam presentes em 37 por cento dos pacientes, principalmente na face e nos membros inferiores (ambos 9 por cento). CONCLUSÃO: As radiografias com achados mais prevalentes foram mãos e punhos, radiografia lateral da coluna torácica e lombar e fêmur. Os achados mais prevalentes foram reabsorção óssea e calcificação ectópica.

[Brown tumor as the initial manifestation of primary hyperparathyroidism]. / Tumor pardo como manifestación inicial de hiperparatiroidismo primario.

Brown tumor is a localized form of osteitis fibrosa cystica, being part of the hyperparathyroid bone disease. It rarely is the first manifestation of hyperparathyroidism, since nowadays, the diagnosis is made at an asymptomatic or minimally symptomatic stage. We present a case of a left superior maxillar brown tumor as the first manifestation of primary hyperparathyroidism due to a parathyroid adenoma. A parathyroidectomy was performed, and there was a regression of the bone lesion, without the need of performing other local surgical procedures.

Evolution of maxillofacial brown tumors after parathyroidectomy in primary hyperparathyroidism.

BACKGROUND: Brown tumor occasionally affects the facial bones. Clinically, these lesions can be mistaken for a neoplasm. Opinions are divided on the course of management of the bony lesions once parathyroidectomy has been carried out. METHODS: We treated 22 patients with primary hyperparathyroidism and osteitis fibrosa cystica and observed their clinical and biochemical recovery. RESULTS: Fifteen patients (68.2%) had brown tumors in mandible, and 7 (31.8%) in maxilla. After parathyroidectomy, 21 patients had normal total serum calcium values. All brown tumors presented a spontaneous progressive regression; in 18 cases, regression was total, with a mean time period of 10 months. Two patients had partial regression after nearly 2 years. Another 2 patients were lost to follow-up. CONCLUSIONS: After successful parathyroid surgery, the bony lesions tended to regress spontaneously, either partially or completely. However, if the lesion is disfiguring or symptomatic, surgical excision may be indicated.

Tumor pardo como manifestación inicial de hiperparatiroidismo primario / Brown tumor as the initial manifestation of primary hyperparathyroidism

El tumor pardo es una forma localizada de osteítis fibrosa quística, parte del compromiso óseo por hiperparatiroidismo. Como primera expresión de hiperparatiroidismo es infrecuente, debido a que actualmente éste se diagnostica en estadios asintomáticos o mínimamente sintomáticos. Presentamos el caso de una paciente con un tumor pardo localizado en el maxilar superior izquierdo, como primera manifestación de hiperparatiroidismo primario causado por un adenoma paratiroideo. Posterior a la realización de una paratiroidectomía el tumor evolucionó con franca regresión, sin necesidad de ningún otro procedimiento quirúrgico local.

Brown tumor is a localized form of osteitis fibrosa cystica, being part of the hyperparathyroid bone disease. It rarely is the first manifestation of hyperparathyroidism, since nowadays, the diagnosis is made at an asymptomatic or minimally symptomatic stage. We present a case of a left superior maxillar brown tumor as the first manifestation of primary hyperparathyroidism due to a parathyroid adenoma. A parathyroidectomy was performed, and there was a regression of the bone lesion, without the need of performing other local surgical procedures.

Highly aggressive brown tumor in the jaw associated with tertiary hyperparathyroidism.

The purpose of this paper is to describe the case of a 12-year-old patient with end-stage chronic renal failure. The patient presented with an osteolytic lesion in the mandible with expansion of the buccal, lingual, and occlusal cortical bone, as well as dislocation of the teeth in the area. The calcium, creatinine, and parathormone (PTH) contents of the blood were elevated. A histopathological examination of the jaw lesion revealed the presence of a brown tumor lesion, which is associated with hyperparathyroidism (HPT). An adenoma was found in the upper left parathyroid, a finding compatible with the diagnosis of tertiary HPT. In spite of the continuous ambulatory peritoneal dialysis instituted, the osteolytic lesion kept on growing. A conservative treatment employing an association of intralesional corticosteroid and salmon calcitonin (inhaled) was carried out. After 14 months of therapy, a reduction in size and complete calcification of the lesion were achieved. Aesthetic osteoplasty of the jaw was then performed.

[Brown tumor in hyperparathyroidism secondary to chronic renal failure]. / Tumor pardo en el hiperparatiroidismo secundario a insuficiencia renal cronica.

Brown tumor (BT) is an uncommon condition that represents the terminal stage of the cystic osteitis fibrosa and have been increasingly reported in hyperparathyroidism secondary to renal failure, due to the increase of survival in patient with hemodialysis. The fine needle aspiration diagnosis is of great importance in the recognition of the BT, although it can be difficult to distinguish it of lesions as the aneurysmal bone cyst and giant-cell tumor. We describe the case of 20-year-old female with chronic renal failure undergoing hemodialysis during six years. Both x-rays and computer tomography revealed a tumor in head of right humerus and lytic images in scapula of the same side, clavicles and ribs. The patient was subjected to a fine needle aspiration biopsy of the tumor of humerus head and the sample was processed with the habitual technique of inclusion in paraffin and stained with hematoxilina and eosina. Histological preparations showed several multinucleate giant cells and spindly or fibrillary cells, feature that was pointed out as compatible, in a context of secondary hyperparathyroidism to chronic renal failure, with a BT. We consider that the radiological and tomographyc finds, besides the history of chronic renal failure with a long history of hemodialysis, were enough to link, with great approach, the histopathology with the diagnosis of BT.

Tumor pardo en el hiperparatiroidismo secundario a insuficiencia renal cronica / Brown tumor in hyperparathyroidism secondary to chronic renal failure

Brown tumor (BT) is an uncommon condition that represents the terminal stage of the cystic osteitis fibrosa and have been increasingly reported in hyperparathyroidism secondary to renal failure, due to the increase of survival in patient with hemodialysis. The fine needle aspiration diagnosis is of great importance in the recognition of the BT, although it can be difficult to distinguish it of lesions as the aneurysmal bone cyst and giant-cell tumor. We describe the case of 20-year-old female with chronic renal failure undergoing hemodialysis during six years. Both x-rays and computer tomography revealed a tumor in head of right humerus and lytic images in scapula of the same side, clavicles and ribs. The patient was subjected to a fine needle aspiration biopsy of the tumor of humerus head and the sample was processed with the habitual technique of inclusion in paraffin and stained with hematoxilina and eosina. Histological preparations showed several multinucleate giant cells and spindly or fibrillary cells, feature that was pointed out as compatible, in a context of secondary hyperparathyroidism to chronic renal failure, with a BT. We consider that the radiological and tomographyc finds, besides the history of chronic renal failure with a long history of hemodialysis, were enough to link, with great approach, the histopathology with the diagnosis of BT.(AU)

El tumor pardo (TP) es una condición infrecuente que representa la fase terminal de la osteítis fibrosa quistica, habiéndose incrementado los reportes de tumores pardos en hiperparatiroidismos secundarios a falla renal. debido al aumento de supervivencia en pacientes dializados. El diagnóstico por aspiración con aguja fina es de gran importancia en el reconocimiento del TP. aunque puede ser dificil distinguirlo de lesiones como el quiste óseo aneurismático y el tumor de células gigantes. Presentamos el caso de una mujer de 20 años con insuficiencia renal crónica dializada durante seis años. que mostraba, con la tomografia computada y radiografia, un tumor en cabeza de húmero derecho e imágenes osteolíticas en homóplato homolateral, clavícula y costillas. Se realizó una biopsia por punción de la tumor ación de cabeza de húmero y la muestra fue procesada con la técnica habitual de inclusión en parafina y tinción con hematoxilina y eosina. Los preparados histológicos mostraron tejido constituido por abundantes células fusiformes y numerosas células gigantes multinucleadas, cuadro histopatológico que se señaló como compatible, en un contexto de hiperparatiroidismo secundario a insuficiencia renal crónica, con un TP.(AU)

Tumor pardo en el hiperparatiroidismo secundario a insuficiencia renal cronica / Brown tumor in hyperparathyroidism secondary to chronic renal failure

Brown tumor (BT) is an uncommon condition that represents the terminal stage of the cystic osteitis fibrosa and have been increasingly reported in hyperparathyroidism secondary to renal failure, due to the increase of survival in patient with hemodialysis. The fine needle aspiration diagnosis is of great importance in the recognition of the BT, although it can be difficult to distinguish it of lesions as the aneurysmal bone cyst and giant-cell tumor. We describe the case of 20-year-old female with chronic renal failure undergoing hemodialysis during six years. Both x-rays and computer tomography revealed a tumor in head of right humerus and lytic images in scapula of the same side, clavicles and ribs. The patient was subjected to a fine needle aspiration biopsy of the tumor of humerus head and the sample was processed with the habitual technique of inclusion in paraffin and stained with hematoxilina and eosina. Histological preparations showed several multinucleate giant cells and spindly or fibrillary cells, feature that was pointed out as compatible, in a context of secondary hyperparathyroidism to chronic renal failure, with a BT. We consider that the radiological and tomographyc finds, besides the history of chronic renal failure with a long history of hemodialysis, were enough to link, with great approach, the histopathology with the diagnosis of BT.

El tumor pardo (TP) es una condición infrecuente que representa la fase terminal de la osteítis fibrosa quistica, habiéndose incrementado los reportes de tumores pardos en hiperparatiroidismos secundarios a falla renal. debido al aumento de supervivencia en pacientes dializados. El diagnóstico por aspiración con aguja fina es de gran importancia en el reconocimiento del TP. aunque puede ser dificil distinguirlo de lesiones como el quiste óseo aneurismático y el tumor de células gigantes. Presentamos el caso de una mujer de 20 años con insuficiencia renal crónica dializada durante seis años. que mostraba, con la tomografia computada y radiografia, un tumor en cabeza de húmero derecho e imágenes osteolíticas en homóplato homolateral, clavícula y costillas. Se realizó una biopsia por punción de la tumor ación de cabeza de húmero y la muestra fue procesada con la técnica habitual de inclusión en parafina y tinción con hematoxilina y eosina. Los preparados histológicos mostraron tejido constituido por abundantes células fusiformes y numerosas células gigantes multinucleadas, cuadro histopatológico que se señaló como compatible, en un contexto de hiperparatiroidismo secundario a insuficiencia renal crónica, con un TP.

Fracturas patológicas debidas a hiperparatiroidismo primario / Pathological fractures due to primary hyperparathyroidism

El hiperparatiroidismo primario actualmente es diagnostico en etapa más precoz predominando la presentación asintomática. Las formas sintomáticas son variadas destacando entre ellas el compromiso renal y óseo. Este último puede abarcar desde alteraciones radiológicas mínimas, hasta el grado máximo de compromiso representado por la osteitis fibroquística. Esta es actualmente una manifestación poco frecuente del hiperparatiroidismo primario, pudiendo en raras ocasiones complicarse con fracturas patológicas. Debido a la rareza de este cuadro se presentan dos casos de pacientes con fracturas patológicas debidas a hiperparatiroidismo primario y se discuten algunos aspectos de esta rara e interesante enfermedad