Lesiones óseas multicéntricas por trastorno paratiroideo: Reporte de un caso clínico / Multicentric Bone Lesions Due to Parathyroid Disease: A Case Report

Resumen Introducción: El hiperparatiroidismo (Hpt) es un desorden endócrino que crea condiciones de hipersecreción de la hormona paratiroidea, propicias para el asentamiento de tumores en el tejido óseo. Si bien no es frecuente, los huesos maxilares, pueden estar involucrados en la patogenia de estas lesiones. Pacientes y métodos: En este trabajo, se presenta un caso clínico de una paciente femenina de 56 años de edad, con lesiones óseas multicéntricas maxilares y extramaxilares a predominio de células gigantes, vinculado a un hiperparatiroidismo. Discusión: El diagnóstico de un Tumor Pardo del Hiperparatiroidismo (TPH) es difícil en la clínica estomatológica, donde la metodología de diagnóstico debe reunir criterios serológicos, clínicos e histopatológicos. La multifocalidad de las lesiones, hacen a este caso un verdadero síndrome endócrino, siendo éste el aspecto más importante. Esta entidad, de incumbencia en la Endocrinología y en la Medicina Bucal, debe ser tratada fundamentalmente de forma interdisciplinar para lograr un tratamiento exitoso.

Abstract Introduction: Hyperparathyroidism is an endocrine disorder that could create parathyroid hormone hypersecretion conditions, propitious for bone tumors development. Although is not frequent, maxillary bones may be involved in the pathogenesis of these lesions. PATIENTS AND METHODS: In this case, a 56-year-old female is reported. An Hyperparathyroidism was diagnosed due to multiple maxillary and extra-maxillary multicentric bone lesions, with a predominance of giant cells, linked to her endocrine disorder. Discussion: The diagnosis of a Hyperparathyroidism Brown Tumor must meet serological, clinical and histopathological criteria. The multifocality of the lesions is the most remarkable clinical aspect in this case. For an adequate treatment of this entity is necessary an interdisciplinary and coordinate work between different areas of healthcare physicians and Oral Medicine specialist.

Hypocalcemia development in patients operated for primary hyperparathyroidism: Can it be predicted preoperatively?

ABSTRACT Objective Primary hyperparathyroidism (PHP) is a common endocrine disease, and its most effective treatment is surgery. Postoperative hypocalcemia is a morbidity of parathyroid surgeries, and it may extend hospitalization durations. The purpose of this study is to determine the predictive factors related to the development of hypocalcemia and hungry bone syndrome (HBS) in patients who underwent parathyroidectomy for PHP. Materials and methods Laboratory data comprising parathyroid hormone (PTH), calcium, phosphate, 25-OHD, albumin, magnesium, alkaline phosphatase (ALP), blood urea nitrogen (BUN), and thyroid stimulating hormone (TSH) of the patients were recorded preoperatively, on the 1st and 4th days postoperatively, and in the 6th postoperative month, and their neck ultrasound (US) and bone densitometry data were also recorded. Results Hypocalcemia was seen in 63 patients (38.4%) on the 1st day after parathyroidectomy. Ten patients (6.1%) had permanent hypocalcemia in the 6th month after surgery. Out of the patients who underwent parathyroidectomy for PHP, 22 (13.4%) had HBS. The incidence of postoperative hypocalcemia was higher in patients who underwent parathyroidectomy for PHP, who had parathyroid hyperplasia, and who had osteoporosis. Preoperative PTH, ALP, and BUN values were higher in those patients who developed HBS. Furthermore, HBS was more common in patients who had osteoporosis, who had parathyroid hyperplasia, and who underwent thyroidectomy simultaneously with parathyroidectomy. Conclusions As a result, patients who have the risk factors for development of hypocalcemia and HBS should be monitored more attentively during the perioperative period.

Hiperparatiroidismo primario normocalcémico

Presentamos las características clínicas, bioquímicas y densitométricas de 35 pacientes con hiperparatiroidismo primario (HPP) normocalcémico, que se caracteriza por un nivel elevado de hormona paratiroidea intacta (PTHi) con el calcio sérico y iónico persistentemente normales, una vez descartadas posibles causas de hiperparatiroidismo secundario. Del total, 30 fueron mujeres (90%) y 5 varones (10%). Se seleccionó un grupo control de 55 pacientes con hiperparatiroidismo primario hipercalcémico: 51 mujeres (93%) y 4 varones (7%). El promedio de edad al diagnóstico de HPP normocalcémico fue de 61.4 ± 11.7 años y del HPP hipercalcémico de 56.4 ± 11.3 años. Además de las diferencias esperables de la calcemia, el calcio iónico, el fósforo y la calciuria de 24 horas, no encontramos cambios significativos en el resto de las variables bioquímicas. Tampoco encontramos diferencias en los valores densitométricos, la presencia de osteopenia u osteoporosis y el número de fracturas entre ambos tipos de HPP. Sí hubo una diferencia significativa en la presencia de litiasis renal entre el HPP normocalcémico (11.4%) vs el HPP clásico (49.1%), p < 0.0005, en parte vinculada a la presencia de hipercalciuria en el HPP clásico. Dos de los 35 pacientes con HPP normocalcémico evolucionaron al HPP hipercalcémico durante un seguimiento de 4 años. Nuestros resultados apoyan la hipótesis que el HPP normocalcémico podría ser una forma temprana del HPP clásico, teniendo ambos similares repercusiones clínicas a nivel renal y óseo.

This report shows our conclusions on the clinical, biochemical and densitometry characteristics of 35 normocalcemic primary hyperparathyroidism (PHPT) patients. This condition is defined by a high level of intact parathyroid hormone (iPTHI) with persistently normal serum and ionized calcium in the absence of secondary hyperparathyroidism. Our selection consisted of 30 women (90%) and 5 men (10%). The control group of 55 hypercalcemic patients with primary hyperparathyroidism included 51 women (93%) and 4 men (7%). The average age at diagnosis of normocalcemic PHPT was 61.4 ± 11.7 years and 56.4 ± 11.3 years in hypercalcemic PHPT. Besides the expected differences in serum calcium, ionized calcium, phosphorus and 24 h urinary calcium, we found no significant changes in other biochemical variables, and no differences in densitometry evaluations such as the presence of osteopenia or osteoporosis and the number of fractures in the two types of PHPT. But there was a significant difference in the presence of renal lithiasis between normocalcemic PHPT (11.4%) and clasic PHPT (49.1%) p < 0.0005, to some extent associated to the presence of hypercalciuria in classic PHPT. Two of the 35 patients with normocalcemic PHPT became classic hypercalcemic PHPT over a 4 year follow-up period. Our findings support the hypothesis that the normocalcemic PHPT could be an early stage of the classic PHPT, both having similar clinical effects to metabolic renal and bone levels.

Nontyphoidal Salmonella urinary tract infection in a case of hyperparathyroidism and nephrocalcinosis / Infección de las vías urinaria por Salmonella no tifoidea en un caso de hiperparatiroidismo y nefrocalcinosis

Nontyphoidal Salmonella infections often present with self-limited gastroenteritis. Extraintestinal focal infections are uncommon but have high mortality and morbidity. Urinary tract infection caused by nontyphoidal Salmonella is usually associated with structural abnormalities of the urinary tract. Nephrocalcinosis and nephrolithiasis are the major risk factors. Although primary hyperparathyroidism has been reported to increase the risk of nephrocalcinosis and nephrolithiasis, little is known about the association between hyperparathyroidism and Salmonella urinary tract infection. We report the case of a 37-year old man who had a history of primary hyperparathyroidism and bilateral nephrocalcinosis and who developed urinary tract infection. Salmonella Group D was isolated from his urine specimen. Salmonella should be considered as a possible causality organism in patients with primary hyperparathyroidism and nephrocalcinosis who develop urinary tract infection. These patients need to be aware of the potential risks associated with salmonellosis.

Las infecciones por Salmonella no tifoidea se presentan a menudo con gastroenteritis auto-limitada. Las infecciones extra-intestinales focales son poco frecuentes, pero tienen una alta mortalidad y morbilidad. La infección de las vías urinarias causada por la Salmonella no tifoidea se asocia generalmente a anomalías estructurales de las vías urinarias. La nefrocalcinosis y la nefrolitiasis son los principales factores de riesgo. Aunque se ha reportado que el hiperparatiroidismo primario aumenta el riesgo de la nefrocalcinosis y la nefrolitiasis, poco se sabe sobre la asociación entre el hiperparatiroidismo y la infección de las vías urinarias por Salmonella. Damos a conocer aquí el caso de un hombre de 37 años con una historia de hiperparatiroidismo primario y nefrocalcinosis bilateral, que desarrolló una infección de las vías urinarias. La Salmonella del grupo D fue aislada de su muestra de orina. La Salmonella se debe considerar como un posible organismo de causalidad en pacientes con hiperparatiroidismo primario y nefrocalcinosis que desarrollan infección del tracto urinario. Estos pacientes necesitan tomar conciencia de los riesgos potenciales asociados con la salmonellosis.

Tumores marrons múltiplos em paciente com carcinoma de paratireoide / Multiple brown tumors in patient with parathyroid carcinoma

Os tumores marrons são complicações que entram no amplo espectro clínico de manifestações do hiperparatireoidismo, geralmente surgindo como lesões líticas, únicas, frequentemente associadas, na forma primária, ao adenoma de paratireoide (principal causa) e, na forma secundária, à insuficiência renal crônica. Porém, há relatos de apresentação múltipla. No presente artigo descreve-se um caso incomum de tumor marrom associado à carcinoma de paratireoide, apresentando outra peculiaridade rara, que é a manifestação como lesões múltiplas...

Brown tumors are complications that can be included in the wide clinical spectrum of hyperparathyroidism manifestations, usually emerging as lithic, single lesions that are frequently associated, in the primary form, with parathyroid adenoma (main cause) and, in the secondary form, with chronic kidney insufficiency. There are, however, reports of multiple manifestations. This paper describes an unusual case of brown tumor associated with parathyroid carcinoma, and also reports on the rare manifestation of the tumor with multiple lesions...

Hiperparatiroidismo terciario: a propósito de tres pacientes con complicaciones diferentes / Tertiary hyperparathyroidism: representation of the complications in three different cases

El hiperparatiroidismo terciario ha sido reconocido como una hiperfunción autónoma de las glándulas paratiroides. Cuando esta enfermedad se instaura, la única opción terapéutica efectiva es la paratiroidectomía. Estos pacientes frecuentemente tienen hiperplasia paratiroidea por lo que la exploración cervical debe ser bilateral. Presentación de casos: Presentamos 3 pacientes, cada uno con una complicación diferente a propósito del hiperparatiroidismo terciario. La primera paciente con arteriolopatía cálcico-urémica, la segunda con calcinosis tumoral de la cadera, y la tercera paciente con tumor pardo bimaxilar. A todos los pacientes los tratamos con paratiroidectomía total con auto trasplante de la glándula más sana en el músculo recto anterior del abdomen. El estudio anatomopatológico reveló hiperplasia de las glándulas paratiroideas resecadas en todos los pacientes. La complicación principal de este procedimiento fue la hipocalcemia sintomática, requiriendo infusión de calcio endovenoso. Dos pacientes presentaron síndrome de hueso hambriento, que se resolvió progresivamente y en el seguimiento se observó normocalcemia. Hubo desenlace fatal en 2 pacientes como consecuencia de complicaciones sistémicas. El hiperparatiroidismo terciario es una enfermedad rara que el clínico debe reconocer para tratarla oportunamente. La paratiroidectomía total es terapéuticamente efectiva en esta condición, pero la hipocalcemia profunda posoperatoria es frecuente y necesita de un manejo cuidadoso.

Tertiary hyperparathyroidism has been recognized as an autonomous hyperfunction of the parathyroid glands. When this disease is established, the only effective therapeutic option is parathyroidectomy. These patients often have parathyroid hyperplasia so that the the neck exploration must be bilateral. Cases presentation: We report 3 patients, each with a different complication as a consequence of tertiary hyperparathyroidism. The first patient with calcic uremic arteriolopathy, the second with tumoral calcinosis of the hip, and the third patient with bimaxillary brown tumor. We treated all this patients with total parathyroidectomy, followed by healthy gland autotransplantation in the anterior rectus abdominal muscle. The anatomopathological study revealed hyperplasia of the resected parathyroid glands, in all the specimens. The main complication of this procedure were the symptomatic hypocalcemia, requiring intravenous calcium infusion. Two patients had the hungry bone syndrome, which was solved progressively. Two parients died due to systemic complications. Tertiary hyperparathyroidism is a rare disease that the clinician should recognize in order to treat it promptly. Total parathyroidectomy is therapeutically effective in this condition, but the postoperative profound hypocalcemia is frequent and needs a careful management.

Tumor marrom bilateral do hiperparatiroidismo primário em mandíbula: relato de caso / Bilateral brown tumor of primary hyperparathyroidism in the mandible: case report

OBJETIVOS: Apresentar um caso de envolvimento mandibular bilateral por tumor marrom do hiperparatireoidismo primário e discutir a diferenciação do tumor marrom do hiperparatireoidismo com a lesão central de células gigantes. DISCUSSÃO E CONCLUSÃO: A diferenciação é estabelecida pela confirmação do distúrbio endócrino, fator determinante no tratamento do tumor. O tratamento foi a resolução da endocrinopatia pela exérese de adenoma nas glândulas paratireoide.

OBJECTIVES: To presente a cased of bilateral mandibular involvement by a brown tumor of hyperparathyreoidism,in which the treatment consisted in resolution of the endocrinopathy by neoplasm exeresis from the parathyroid glands. DISCUSSION AND CONCLUSION: The correct differentiation of the hyperparathyroidism brown tumor from the central giant cell lesion is an essential factor to avoid diagnostic errors and unnecessary treatments.This differentiation is established by the confirmation of endocrinal disturbance, a determinant factor in the treatment of the tumor.

[Brown tumor of mandible in a patient with normocalcemic primary hyperparathyroidism]

Brown tumor or Osteitis Fibrosa Cystica [OFC] is a rare clinical entity complicating hyperparathyroidism. It may occur in the head and neck, with the mandible being the most frequent site. Primary hyperparathyroidism is usually associated with hypercalcemia. We report a 35 years old female with mandibular brown tumor secondary to primary hyperparathyroidism. In this case in spite of primary hyperparathyroidism and the bony lesion the serum calcium level was within normal limit. This case demonstrates that in osteolytic bony lesions a hyperparathyroidism complication can be expected even with normal serum calcium level. The presence of normocalcemia in hyperparathyroidism should prompt the physician to measurement of ionized calcium and look for vitamin D deficiency, magnesium deficiency, impaired renal function, use of interacting drugs and associated disorders. If there weren't these factors, normocalcemic primary hyperparathyroidism should be considered

Parathyroid double adenoma associated with papillary carcinoma of thyroid

It has been reported that thyroid disease is common in patients with hyperparathyroidism, and vice versa. Parathyroid adenoma is the most common cause of primary hyperparathyroidism [PHPT]. Hypercalcemia is an important laboratory finding in the diagnosis of hyperparathyroidism. There have been sporadic reports of the coexistence of hyperparathyroidism and non-medulary carcinoma, but the association of parathyroid adenoma and papillary carcinoma of the thyroid is not well known. This is a rare report of the coexistent occurrence of papillary carcinoma of thyroid and double adenoma of parathyroid. The case is a 52-year-old woman with a 10-year history of pain in lower extremities, with initial diagnosis of osteoporosis and no clinical improvement despite using drugs. The patient was diagnosed as a case of hyperparathyroidism, using elevated concentration of calcium and PTH and metabolic bone disease [according to the isotope bone scan]. In addition to parathyroid adenomas, a firm nodule in the thyroid was detected during the surgery. Therefore, lobectomy as well as hyperparathyroidectomy was performed. In pathologic examination, parathyroid double adenoma and papillary carcinoma of the thyroid were revealed

Radiographic signals detection of systemic disease: orthopantomographic radiography

For the purposes of this report, systemic disease will be interpreted as conditions that are spread out within the body rather than localized strictly to the tissues of the oral cavity. Since it would take many volumes to review all such conditions, the intent of the authors is to review a few examples of conditions where initial panoramic radiographic findings suggested widespread disease of significance enough to affect the quality of life and longevity of the patient.

Para los propósitos de este informe, enfermedad sistémica puede ser interpretado como las condiciones que se producen dentro del cuerpo, más que estrictamente en los tejidos de la cavidad oral. Aunque habría que ocupar muchos volúmenes para revisar todas las condiciones, la intención de los autores sólo fue revisar algunos ejemplos de éstas, en las que la radiografía panorámica inicial, indica los resultados de la enfermedad generalizada, dada la suficiente importancia al afectar la calidad de vida y la longevidad del paciente.

Atypical Radiological Manifestation of Pulmonary Metastatic Calcification

Metastatic pulmonary calcification refers to calcium deposition in the normal pulmonary parenchyma and this deposition is secondary to abnormal calcium metabolism. The most common radiologic manifestation consists of poorly-defined nodular opacities that are mainly seen in the upper lung zone. We present here a case of metastatic pulmonary calcification that manifested as atypical, dense, calcium deposition in airspaces within the previously existing consolidation in the bilateral lower lobes, and this process was accelerated by pneumonia-complicated sepsis in a patient with hypercalcemia that was due to hyperparathyroidism.

The calcium-sensing receptor and related diseases / O receptor sensor de cálcio e doenças associadas

The calcium-sensing receptor (CASR) adjusts the extracellular calcium set point regulating PTH secretion and renal calcium excretion. The receptor is expressed in several tissues and is also involved in other cellular functions such as proliferation, differentiation and other hormonal secretion. High extracellular calcium levels activate the receptor resulting in modulation of several signaling pathways depending on the target tissues. Mutations in the CASR gene can result in gain or loss of receptor function. Gain of function mutations are associated to Autossomal dominant hypocalcemia and Bartter syndrome type V, while loss of function mutations are associated to Familial hypocalciuric hypercalcemia and Neonatal severe hyperparathyroidism. More than one hundred mutations were described in this gene. In addition to calcium, the receptor also interacts with several ions and polyamines. The CASR is a potential therapeutic target to treatment of diseases including hyperparathyroidism and osteoporosis, since its interaction with pharmacological compounds results in modulation of PTH secretion.

O receptor sensor de cálcio (CASR) ajusta o set point do cálcio extracelular através da regulação da secreção de PTH e da excreção renal de cálcio. O receptor é expresso em diversos tecidos e também está envolvido em outras funções celulares como proliferação, diferenciação e secreção de outros hormônios. Concentrações altas de cálcio extracelular ativam o receptor resultando em modulação de inúmeras vias de sinais intracelulares dependendo do tecido-alvo. Mutações no gene do CASR podem resultar em ganho ou perda de função do receptor. Mutações com ganho de função são associadas à Hipocalcemia autossômica dominante e à Síndrome de Bartter tipo V, enquanto que mutações com perda de função são associadas à Hipercalcemia hipocalciúrica familiar e ao Hiperparatireoidismo neonatal grave. Mais de cem mutações foram descritas neste gene. Além do cálcio, o receptor também interage com inúmeros íons e poliaminas. CASR é um alvo terapêutico potencial para tratamento de doenças incluindo hiperparatireoidismo e osteoporose, pois a sua interação com compostos farmacológicos resulta em modulação da secreção de PTH.

Adenoma de paratireóide diagnosticado por punção aspirativa por agulha fina: relato de caso / Parathyroid adenoma diagnosed by fine needle aspiration cytology: a case report

Os autores relatam o caso de uma paciente com hiperparatireoidismo primário causado por adenoma de paratireóide diagnosticado através de punção aspirativa por agulha fina (PAAF) no pré-operatório. A paratireóide aumentada foi visualizada pela ultra-sonografia (US) e submetida à PAAF, que foi compatível com adenoma de paratireóide. Realizou-se a remoção cirúrgica do adenoma e o histopatológico resultou concordante com a citologia. Este relato destaca a importância da localização e do diagnóstico citológico pré-operatório das lesões de paratireóide para proporcionar uma abordagem cirúrgica menos agressiva.

The authors report a patients case with primary hyperparathyroidism caused by parathyroid adenoma that was diagnosed by fine needle aspiration cytology (FNAC) in the preoperative. The increased parathyroid was visualized by the ultrasonography and submitted to FNAC that was compatible with parathyroid adenoma. The surgical removal of adenoma was accomplished and the histopathologic was concordant with the cytology. This report detaches the importance of the location and preoperative cytological diagnosis of the parathyroid lesions in providing a less aggressive surgical approach.

Hyperfunctioning intrathyroidal parathyroid carcinoma

Intrathyroidal parathyroid carcinoma is an exceedingly rare cause of primary hyperparathyroidism with difficulties in the diagnosis and management. We report a case of hypercalcemia from intrathyroidal parathyroid carcinoma in a 63-year-old Saudi female. She was diagnosed 2 years earlier with osteoporosis in a primary care clinic and was on alendronate since then. This year she was noted to have hypercalcemia, but in retrospect she had more than 10 years history of multiple medical problems related to hypercalcemia. Parathyroid 99mTc-SestaMIBI scintigraphy revealed parathyroid adenoma in the left inferior parathyroid gland. She had successful video-assisted parathyroidectomy that relieved most of her symptoms. The extreme rarity of such a case, the interesting clinical presentation and review of the literature are discussed

Hiperparatireoidismo primário associado a epifisiólise de cabeca do fêmur em adolescente / Primary hyperparathyroidism associated to slipped capital femoral epiphysis in a teenager

O hiperparatireoidismo primário (HPP) é uma doenca incomum na infância e na adolescência. A associacão de HPP e epifisiólise de cabeca do fêmur é rara, tendo sido descritos apenas quatro casos na literatura. Relatamos um caso de HPP por adenoma de paratireóide, com dores e inúmeras deformidades ósseas, associado a epifisiólise de cabeca do fêmur em um jovem de 18 anos. A análise laboratorial mostrou cálcio de 13,7mg/dL, paratormônio de 1.524pg/mL e fosfatase alcalina de 3.449U/L. As deformidades foram decorrentes de diagnóstico tardio e possivelmente pelo fato de a doenca ter ocorrido na fase de estirão puberal. A combinacão de vários fatores metabólicos e mecânicos provavelmente contribuiu para esta associacão. O paciente foi submetido à remocão cirúrgica do adenoma, que costuma ser acompanhada por resolucão pronta da epifisiólise. Desta forma, foi optado por conduta expectante quanto à epifisiólise da cabeca do fêmur, em consonância com a literatura. Houve melhora das dores ósseas e normalizacão dos níveis de cálcio e de paratormônio.