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.

From mild to severe primary hyperparathyroidism: the Brazilian experience / Do hiperparatiroidismo primário leve ao severo: a experiência brasileira

Primary hyperparathyroidism often presents as an asymptomatic disorder. In our institution, routine serum calcium measurements have now been used as part of medical examination for 23 years. Out of 124 patients consecutively seen at our institution, 47 percent presented with no symptoms related to the disease, while 25 percent presented with severe skeletal involvement and osteitis fibrosa cystica, 25 percent with renal stone disease without overt bone involvement, and 2 percent with the typical neuropsychiatric syndrome. This same pattern is seen in the city of São Paulo. In severe disease pathological fractures are frequently seen, especially in long bones of the lower extremities, and also loss of lamina dura of the teeth and salt-and-pepper appearance of the skull. Bone mineral density is extremely low in these patients but usually show remarkable recovery following surgical cure. Serum PTH and bone markers are considerable higher in severely affected patients, who also have a high rate of vitamin D deficiency, and the parathyroid lesion is easier located compared with asymptomatic patients. From pathological specimens 87 percent had histological confirmation of a single adenoma, 6.4 percent multiple gland hyperplasia and 3.8 percent carcinoma.

Na maioria dos relatos da literatura recente, o hiperparatiroidismo primário apresenta-se, com freqüência, na forma assintomática. Em nossa instituição utilizamos a determinação rotineira do cálcio sérico há 23 anos. Em nossa série de 124 casos consecutivos, 45 por cento não apresentavam sintomas relacionados com a doença, 25 por cento tinham envolvimento esquelético intenso com osteíte fibrosa cística, 25 por cento tinham nefrolitíase sem envolvimento ósseo severo, e 2 por cento apresentavam a síndrome neuro-psiquiátrica típica. Esse mesmo padrão tem sido observado na cidade de São Paulo. Na doença severa são freqüentes as fraturas patológicas, especialmente nos ossos longos dos membros inferiores, como também a reabsorção da lâmina dura dos dentes e o aspecto em "sal e pimenta" nas radiografias do crânio. A densidade mineral óssea mostra-se extremamente reduzida nesses pacientes, mas em geral exibe melhora marcante após a cura cirúrgica. O PTH no soro e os marcadores bioquímicos da remodelação óssea estão significativamente mais altos nos pacientes com doença severa, os quais freqüentemente apresentam deficiência de vitamina D e localização mais fácil da lesão paratiroideana, quando comparados aos pacientes assintomáticos. Ao exame anátomo-patológico, 87 por cento tiveram confirmação de adenoma único, 6,4 por cento hiperplasia glandular difusa e 3,8 por cento carcinoma.

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.