RESUMEN
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.
Asunto(s)
Humanos , Masculino , Adolescente , Osteítis Fibrosa Quística/cirugía , Huesos del Metacarpo/cirugía , Osteítis Fibrosa Quística/etiología , Osteítis Fibrosa Quística/diagnóstico por imagen , Trastorno Mineral y Óseo Asociado a la Enfermedad Renal Crónica/complicaciones , Trasplante Óseo , Huesos del Metacarpo/diagnóstico por imagen , Peroné/cirugía , Hiperparatiroidismo Secundario/complicacionesRESUMEN
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.
Asunto(s)
Trastorno Mineral y Óseo Asociado a la Enfermedad Renal Crónica/etiología , Hiperparatiroidismo Secundario/complicaciones , Fallo Renal Crónico/complicaciones , Enfermedades Mandibulares/etiología , Enfermedades Mandibulares/cirugía , Enfermedades Maxilares/etiología , Enfermedades Maxilares/cirugía , Procedimientos Quirúrgicos Orales/métodos , Osteítis Fibrosa Quística/etiología , Osteítis Fibrosa Quística/cirugía , Hueso Paladar/patología , Hueso Paladar/cirugía , Diagnóstico Diferencial , Estética Dental , Femenino , Humanos , Imagenología Tridimensional , Fallo Renal Crónico/cirugía , Trasplante de Riñón , Enfermedades Mandibulares/diagnóstico por imagen , Enfermedades Maxilares/diagnóstico por imagen , Hueso Paladar/diagnóstico por imagen , Colgajos Quirúrgicos , Tomografía Computarizada por Rayos X , Adulto JovenRESUMEN
BACKGROUND: Secondary hyperparathyroidism is a frequent complication of chronic renal failure. The brown tumor is an unusual presentation of fibrous osteitis that represents a serious complication of renal osteodystrophy, affecting predominantly the hands, feet, skull, and facial bones. CASE REPORT: The aim of this paper is to describe the case of a 53-year-old female patient, with renal failure who has been on dialysis for 6 years and developed severe secondary hyperparathyroidism and brown tumor of the maxilla and mandible, confirmed by incisional biopsy. Parathyroidectomy was indicated as a result of rapid growth of the tumor and the maintenance of laboratory findings. Despite the normalization of serum parathyroid hormone and alkaline phosphatase, tumor regression was slow and patient's important functional and esthetic deficits persisted. Excision of the mandible tumor was conservative. Osteoplasty was recommended because during a 5-year follow-up there was regression of the lesion, decreased pain, bleeding, and tooth mobility.
Asunto(s)
Trastorno Mineral y Óseo Asociado a la Enfermedad Renal Crónica/cirugía , Hiperparatiroidismo Secundario/cirugía , Fallo Renal Crónico/complicaciones , Enfermedades Mandibulares/cirugía , Enfermedades Maxilares/cirugía , Osteítis Fibrosa Quística/cirugía , Paratiroidectomía , Asimetría Facial/diagnóstico por imagen , Asimetría Facial/cirugía , Femenino , Estudios de Seguimiento , Humanos , Hiperparatiroidismo Secundario/diagnóstico por imagen , Enfermedades Mandibulares/diagnóstico por imagen , Enfermedades Maxilares/diagnóstico por imagen , Persona de Mediana Edad , Osteítis Fibrosa Quística/diagnóstico por imagen , Tomografía Computarizada por Rayos XRESUMEN
Bone disease in severe primary hyperparathyroidism (PHPT) is described classically as osteitis fibrosa cystica (OFC). Bone pain, skeletal deformities and pathological fractures are features of OFC. Bone mineral density is usually extremely low in OFC, but it is reversible after surgical cure. The signs and symptoms of severe bone disease include bone pain, pathologic fractures, proximal muscle weakness with hyperreflexia. Bone involvement is typically characterized as salt-and-pepper appearance in the skull, bone erosions and bone resorption of the phalanges, brown tumors and cysts. In the radiography, diffuse demineralization is observed, along with pathological fractures, particularly in the long bones of the extremities. In severe, symptomatic PHPT, marked elevation of the serum calcium and PTH concentrations are seen and renal involvement is manifested by nephrolithiasis and nephrocalcinosis. A new technology, recently approved for clinical use in the United States and Europe, is likely to become more widely available because it is an adaptation of the lumbar spine DXA image. Trabecular bone score (TBS) is a gray-level textural analysis that provides an indirect index of trabecular microarchitecture. Newer technologies, such as high-resolution peripheral quantitative computed tomography (HR-pQCT), have provided further understanding of the microstructural skeletal features in PHPT.
Asunto(s)
Fracturas Óseas/etiología , Hiperparatiroidismo Primario/complicaciones , Osteítis Fibrosa Quística/complicaciones , Biomarcadores/análisis , Densidad Ósea , Enfermedades Óseas/complicaciones , Calcio/sangre , Femenino , Humanos , Hiperparatiroidismo Primario/patología , Riñón/diagnóstico por imagen , Masculino , Osteítis Fibrosa Quística/diagnóstico por imagen , Osteítis Fibrosa Quística/cirugía , Hormona Paratiroidea/sangre , Paratiroidectomía , Radiografía , Cráneo/diagnóstico por imagen , Ultrasonografía , Vitamina D/análogos & derivados , Vitamina D/sangreRESUMEN
Bone disease in severe primary hyperparathyroidism (PHPT) is described classically as osteitis fibrosa cystica (OFC). Bone pain, skeletal deformities and pathological fractures are features of OFC. Bone mineral density is usually extremely low in OFC, but it is reversible after surgical cure. The signs and symptoms of severe bone disease include bone pain, pathologic fractures, proximal muscle weakness with hyperreflexia. Bone involvement is typically characterized as salt-and-pepper appearance in the skull, bone erosions and bone resorption of the phalanges, brown tumors and cysts. In the radiography, diffuse demineralization is observed, along with pathological fractures, particularly in the long bones of the extremities. In severe, symptomatic PHPT, marked elevation of the serum calcium and PTH concentrations are seen and renal involvement is manifested by nephrolithiasis and nephrocalcinosis. A new technology, recently approved for clinical use in the United States and Europe, is likely to become more widely available because it is an adaptation of the lumbar spine DXA image. Trabecular bone score (TBS) is a gray-level textural analysis that provides an indirect index of trabecular microarchitecture. Newer technologies, such as high-resolution peripheral quantitative computed tomography (HR-pQCT), have provided further understanding of the microstructural skeletal features in PHPT.
A doença óssea no hiperparatiroidismo primário grave é representada pela osteíte fibrosa cística (OFC). Dor óssea, deformidades esqueléticas e fraturas patológicas são achados comuns na OFC. A densidade mineral óssea está, usualmente, extremamente diminuída na OFC, mas é reversível após a cura cirúrgica. Os sinais e sintomas da doença óssea grave incluem dor óssea, fraturas patológicas e fraqueza muscular proximal com hiper-reflexia. O comprometimento ósseo é tipicamente caracterizado pela aparência em “sal-e-pimenta” nos ossos do crânio, erosões ósseas e reabsorção das falanges, tumores marrons e cistos. Na radiografia, observam-se desmineralização difusa e fraturas patológicas especialmente nos ossos longos das extremidades. No hiperparatiroidismo primário (HPTP) sintomático grave, as concentrações séricas de cálcio e PTH estão usualmente bem elevadas e o comprometimento renal se caracteriza pela presença de urolitíase e nefrocalcinose. Uma nova tecnologia, recentemente aprovada para uso clínico nos Estados Unidos e na Europa, torna-se provável se difundir rapidamente, pois utiliza as imagens geradas pela densitometria DXA. O escore trabecular ósseo (TBS), obtido por meio da análise do nível da textura cinza das imagens dos corpos vertebrais, fornece informações indiretas sobre a microarquitetura trabecular. Novos métodos, como a tomografia de alta resolução quantitativa periférica computadorizada (HRpqCT), têm proporcionado conhecimentos adicionais sobre os achados da microarquitetura esquelética no HPTP.
Asunto(s)
Femenino , Humanos , Masculino , Fracturas Óseas/etiología , Hiperparatiroidismo Primario/complicaciones , Osteítis Fibrosa Quística/complicaciones , Densidad Ósea , Biomarcadores/análisis , Enfermedades Óseas/complicaciones , Calcio/sangre , Hiperparatiroidismo Primario/patología , Riñón , Osteítis Fibrosa Quística , Osteítis Fibrosa Quística/cirugía , Paratiroidectomía , Hormona Paratiroidea/sangre , Cráneo , Vitamina D/análogos & derivados , Vitamina D/sangreRESUMEN
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.
Asunto(s)
Hiperparatiroidismo Secundario/etiología , Enfermedades Mandibulares/etiología , Osteítis Fibrosa Quística/etiología , Glándulas Paratiroides/anomalías , Granuloma de Células Gigantes/etiología , Granuloma de Células Gigantes/cirugía , Humanos , Hiperparatiroidismo Secundario/cirugía , Masculino , Enfermedades Mandibulares/cirugía , Osteítis Fibrosa Quística/cirugía , Glándulas Paratiroides/diagnóstico por imagen , Glándulas Paratiroides/cirugía , Paratiroidectomía , Complicaciones Posoperatorias , Cintigrafía , Insuficiencia Renal Crónica/complicaciones , Adulto JovenRESUMEN
OBJECTIVE: To assess the effect of bisphosphonates on post-parathyroidectomy hypocalcemia in patients with osteitis fibrosa cystica. METHODS: Review of the medical records of six patients using bisphosphonates preoperatively. RESULTS: Mean age was 35.6 ± 10.5 years; serum calcium = 13.51 + 0.87 mg/dL; iPTH = 1,389 + 609 pg/mL. The mean value of urine deoxypyridinoline (UDPD) of three patients was 131 ± 183 nmol/mmol Cr, and of C-telopeptide (CTX), 2,253 ± 1,587 pg/mL. The mean values of bone densitometry (T score) were as follows: 0.673 ± 0.150 g/cm(2) (-4.42 ± 1.23) in lumbar spine (L2-L4); 0.456 ± 0.149 g/cm(2) (-5.58 ± 1.79) in the femoral neck; and 0.316 ± 0.055 g/cm(2) (-5.85 ± 0.53) in radius 33. Patient 1 received oral alendronate, 30 mg/day for four weeks; his calcium decreased from 14 to 11.6 mg/dL, and his UDPD from 342 to 160 nmol/mmol Cr. Patient 2 received oral alendronate, 20 mg/day for six weeks; his calcium decreased from 14 to 11.0 mg/dL and his UDPD from 28.8 to 14 nmol/mmol Cr. Patient 3 received intravenous pamidronate, 90 mg prior to surgery. Patient 4 received oral alendronate, 140 mg/week for six weeks; her calcium decreased from 13.7 to 12.3 mg/dL and her CTX from 2,160 to 1,340 pg/mL. Patient 5 received oral alendronate, 140 mg/ week for six weeks; her calcium levels dropped from 14.3 to 14.1 mg/dL; her CTX did not change. Patient 6 received ibandronate, 150 mg, ten days prior to surgery; his CTX reduced by 62%. No patient developed severe hypocalcemia in the first postoperative week. One year after surgery, the mean gain in bone mineral density was 40% ± 29% in L2-L4, 86 ± 39% in the femoral neck, and 22% ± 11% in radius 33. CONCLUSION: The preoperative use of bisphosphonates seems to attenuate bone hunger without preventing a significant increase in bone mass in the follow-up of parathyroidectomy.
Asunto(s)
Difosfonatos/uso terapéutico , Hiperparatiroidismo Primario/cirugía , Hipocalcemia/etiología , Hipocalcemia/prevención & control , Osteítis Fibrosa Quística/cirugía , Paratiroidectomía/efectos adversos , Adulto , Femenino , Humanos , Masculino , Persona de Mediana EdadRESUMEN
OBJETIVO: Avaliar o efeito dos bisfosfonatos na hipocalcemia pós-paratireoidectomia em pacientes com osteíte fibrosa cística. MÉTODOS: Foram revisados os prontuários de seis pacientes que fizeram uso pré-operatório de bisfosfonatos. RESULTADOS: A idade média foi de 35,6 ± 10,5 anos; cálcio sérico 13,51 ± 0,87 mg/dL; PTHi 1.389 ± 609 pg/mL. O valor médio de deoxipiridinolina urinária (UDPD) de três pacientes foi de 131 ± 183 nmol/mmol Cr; e do C-telopeptídeo (CTX) de 2.253 ± 1.587 pg/mL. Densitometria óssea (escore T) obteve média de 0,673 ± 0,150 g/cm² (-4,42 ± 1,23) em coluna lombar (CL); 0,456 ± 0,149 g/cm² (-5,58 ± 1,79) em colo de fêmur (CF) e 0,316 ± 0,055 g/cm² (-5,85 ± 0,53) em rádio 33 (RD). Um paciente recebeu alendronato oral 30 mg/dia por 4 semanas; o cálcio diminuiu de 14 para 11,6 mg/dL e UDPD de 342 para 160 nmol/mmol Cr. Outro usou alendronato oral 20 mg/dia por 6 semanas; o cálcio baixou de 14 para 11,0 mg/dL e UDPD de 28,8 para 14 nmol/mmol Cr. Um paciente recebeu pamidronato 90 mg endovenoso antes da cirurgia. Um paciente usou alendronato oral 140 mg/semana por 6 semanas; o cálcio diminuiu de 13,7 para 12,3 mg/dL e o CTX de 2.160 para 1.340 pg/mL. Outro usou alendronato VO 140 mg/semana por 6 semanas; o cálcio baixou de 14,3 para 14,1 mg/dL; o CTX não reduziu. Um paciente fez ibandronato 150 mg 10 dias antes da cirurgia; o CTX caiu em 62 por cento. Nenhum paciente desenvolveu hipocalcemia grave na primeira semana do pós-operatório. Um ano após a cirurgia, houve aumento de 40 ± 29 por cento em CL, 86 ± 39 por cento em CF e 22 ± 11 por cento em RD. CONCLUSÃO: O uso pré-operatório de bisfosfonatos parece atenuar a fome óssea sem impedir o marcante aumento de massa óssea no seguimento da paratireoidectomia.
OBJECTIVE: To assess the effect of bisphosphonates on post-parathyroidectomy hypocalcemia in patients with osteitis fibrosa cystica. METHODS: Review of the medical records of six patients using bisphosphonates preoperatively. RESULTS: Mean age was 35.6 ± 10.5 years; serum calcium = 13.51 + 0.87 mg/dL; iPTH = 1,389 + 609 pg/mL. The mean value of urine deoxypyridinoline (UDPD) of three patients was 131 ± 183 nmol/mmol Cr, and of C-telopeptide (CTX), 2,253 ± 1,587 pg/mL. The mean values of bone densitometry (T score) were as follows: 0.673 ± 0.150 g/cm² (-4.42 ± 1.23) in lumbar spine (L2-L4); 0.456 ± 0.149 g/cm² (-5.58 ± 1.79) in the femoral neck; and 0.316 ± 0.055 g/cm² (-5.85 ± 0.53) in radius 33. Patient 1 received oral alendronate, 30 mg/day for four weeks; his calcium decreased from 14 to 11.6 mg/dL, and his UDPD from 342 to 160 nmol/mmol Cr. Patient 2 received oral alendronate, 20 mg/day for six weeks; his calcium decreased from 14 to 11.0 mg/dL and his UDPD from 28.8 to 14 nmol/mmol Cr. Patient 3 received intravenous pamidronate, 90 mg prior to surgery. Patient 4 received oral alendronate, 140 mg/week for six weeks; her calcium decreased from 13.7 to 12.3 mg/dL and her CTX from 2,160 to 1,340 pg/mL. Patient 5 received oral alendronate, 140 mg/ week for six weeks; her calcium levels dropped from 14.3 to 14.1 mg/dL; her CTX did not change. Patient 6 received ibandronate, 150 mg, ten days prior to surgery; his CTX reduced by 62 percent. No patient developed severe hypocalcemia in the first postoperative week. One year after surgery, the mean gain in bone mineral density was 40 percent ± 29 percent in L2-L4, 86 ± 39 percent in the femoral neck, and 22 percent ± 11 percent in radius 33. CONCLUSION: The preoperative use of bisphosphonates seems to attenuate bone hunger without preventing a significant increase in bone mass in the follow-up of parathyroidectomy.