Análisis de falsos negativos en la cintigrafía SPECT de paratiroides con sestamibi en pacientes con hiperparatiroidismo primario sometidos a cirugía entre 2008-2015 en hospital universitario / False negative spect parathyroid scintigraphy with sestamibi in patients with primary hyperparathyroidism

Background: 99mTc-sestamibi parathyroid SPECT scintigraphy is a useful tool in the pre-operative study of hyperparathyroidism. False negatives (FN) have been reported in 5.7-14% of the examinations. Aim: To characterize 99mTc-sestamibi FN in cases referred for primary hyperparathyroidism (PHP) to a university hospital. Material and Methods: Descriptive retrospective analysis. We included patients with PHP, studied with SPECT scintigraphy, operated at our center between 2008 and 2015. Clinical and surgical data were recorded; biopsies of the FN were blindly reviewed by one pathologist. Results: One hundred twenty one scintigraphies fulfilled the inclusion criteria. Seven (5.8%) were negative and 114 positive. There was no difference in age, sex and PTH levels between FN and true positive scintigraphies. At surgery, one FN case had two hyperplasic glands and two cases had ectopic glands. Pathology reported adenoma in three cases, hyperplasia in three and carcinoma in one. The largest diameter of the lesion was lower in FN (1.3 and 2.1 cm respectively, p = 0.02) and the proportion of adenomas was higher in true positive cases (29% and 75% respectively; p < 0.01). The interval between scintigraphy and parathyroidectomy was greater in FN with a median of 92 days (range 20 days-3.2 years, p < 0.01). The percentage of oxyphilic cells observed was similar in both groups. Conclusions: FN parathyroid SPECT scintigraphies in PHP are uncommon. They corresponded to lesions under the equipment's resolution limit and resulted in longer time lags between scintigraphy and surgery.

Hiperparatiroidismo primario: manejo quirúrgico / Primary hyperparathyroidism: surgical treatment

Background: Primary hyperparathyroidism (HPTP) is the most common cause of hypercalcaemia in the ambulatory setting. This condition affects between 0.1 -2 percent of the population. Surgery is the only curative treatment. Objective: The aim of the study is to present our 10-year surgical experience. Methods: Analytical-descriptive study. We included all patients diagnosed with HPTP treated with surgery in our hospital between June 2003 and June 2013. Results: 173 patients underwent surgery for HPTP. The mean age was 57.5 +/- 13.5 years. Asymptomatic in 98 cases (56.6 percent). Resection the abnormal parathyroid glands was performed in all cases with intraoperative PTH monitoring. In 167 patients (96.5 percent) intraoperative PTH declined ≥ 50 percent. The median follow-up was 61 months (1-117 months). Cure was achieved in 169 patients (97.7 percent). Four cases (2.3 percent) had recurrence. Conclusions: Surgery is effective and safe for the treatment of HPTP. A decline in intraoperative PTH > 50 percent predicts cure in 97.7 percent of cases.

Introducción: El hiperparatiroidismo primario (HPTP), es una entidad frecuente que afecta entre el 0,1 y 2 por ciento de la población. La cirugía es el único tratamiento curativo. Objetivo: Evaluar la eficacia y riesgos del tratamiento quirúrgico del HPTP. Material y Métodos: Estudio analítico-descriptivo. Se incluyó a todos los pacientes con diagnóstico de HPTP referidos para cirugía al Hospital Clínico de la Pontificia Universidad Católica de Chile entre junio de 2003 y junio de 2013. Resultados: 173 pacientes fueron operados en nuestra institución por HPTP. El promedio de edad de los pacientes fue de 57,5 +/- 13,5 años. La forma de presentación fue asintomática en 98 casos (56,6 por ciento) y sintomática en 75 casos (43,4 por ciento). Todos los pacientes fueron tratados con resección de la o las glándulas hiperfuncionantes con medición de PTH intraoperatoria. En 167 pacientes (96,5 por ciento) se logró caída de PTH intraoperatoria > 50 por ciento. Catorce pacientes (8,1 por ciento) presentaron complicaciones post operatorias. La mediana de hospitalización fue de 2 días (1 a 23 días). La mediana de seguimiento fue de 61 meses (6 a 117 meses). Se logró curación en 169 pacientes (97,7 por ciento). Cuatro casos (2,3 por ciento) presentaron recidiva. Conclusiones: La cirugía es eficaz y segura para el tratamiento del hiperparatiroidismo primario. Una caída de PTH intraoperatoria > 50 por ciento del valor basal predice curación en 97,7 por ciento de los casos.

Bone disease in primary hyperparathyroidism / Doença óssea no hiperparatiroidismo primário

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.

Correlación entre el nivel preoperatorio de paratohormona y el volumen y peso del adenoma de glándula paratiroides extirpado, en pacientes con hiperparatiroidismo primario mejorados / Correlation of preoperative parathormone level with estimated volume and weight of the parathyroid adenoma in patientes with primary hyperparathyroidism

Introducción: El hiperparatiroidismo primario (HPT1°) es la causa más frecuente de hipercalcemia en la población ambulatoria general. Así mismo, es la indicación más frecuente de cirugía paratiroídea, habitualmente causado por la presencia de un adenoma único, que se asocia a un elevado nivel de paratohormona (PTH). Objetivo: Determinar si existe una relación matemática positiva entre el nivel de PTH preoperatoria y el volumen y peso del adenoma extirpado. Material y Método: Se revisaron retrospectivamente los resultados de las biopsias y PTH preoperatoria de 71 pacientes evaluables con diagnóstico de HPT1° por adenoma único, mejorados. Resultados: Se obtuvo una correlación matemática positiva, aunque débil, entre peso, volumen y PTH preoperatoria. La correlación matemática fue positiva y fuerte entre peso y volumen. Conclusiones: Existe una relación positiva débil entre el peso y volumen y los niveles preoperatorios de PTH. Estos aspectos estudiados son útiles pero especialmente en relación con otros hallazgos pre e intraoperatorios.

Background: Primary hyperparathyroidism is caused mostly by a single adenoma and it is the most frequent cause of hypercalcemia in outpatients. Hyperplasia of the four glands and cancer are the other ethiological diagnosis. It has been postulated that the bigger and heavier an adenoma is the higher the preoperative PTH level. Objective: To evaluate a mathematical relation between adenoma estimated volume and weight and preoperative parathormone level (prPTH). Method: Retrospective analysis of 71 evaluable treated patients biopsies and preoperative PTH level. Results: A mathematical positive and weak correlation between volume, weight and preoperative PTH was obtained. This correlation was positive and strong between volumen and weight. Conclusions: In this group we found a positive and weak mathematical relation between weight, volume and preoperative PTH levels. These aspects alone are useful but only in combination with others for the correct interpretation of pre and intraoperative findings.

Hiperparatiroidismo primario y cáncer de paratiroides: caso clínico / Primary hyperparathyroidism and parathyroid cancer: report of one case

We report a 51 years old female operated for a primary hyperparathyroidism that relapsed in two occasions. After the first and second operation, the pathological study of the excised glands disclosed an adenoma. After the second relapse, a parathyroid gland in the right lateral and upper cervical region, located with technetium (Tc)-99m Sestamibi scintigraphy, was excised. The pathological study of the surgical piece disclosed a parathyroid carcinoma without local lymph node involvement. A postoperative 18F-fluorodexyglucose positron emission tomography did not show tumor dissemination.

Presentamos un caso de sexo femenino, operada por hiperparatiroidismo primario, con 2 recidivas posteriores. En las 2 primeras oportunidades, el diagnóstico histológico fue compatible con adenoma. La última intervención se debió realizar por una glándula ubicada, de acuerdo al Sestamibi Tc-99m, en la región cervical lateral alta derecha. Se realizó exploración supraomohiodea, efectuando la extirpación de la glándula y adenopatías peritumorales. La biopsia definitiva informó carcinoma de paratiroides y ganglios linfáticos negativos para neoplasia. Se realizó estudio de diseminación y PET- FDG de control en el postoperatorio alejado, sin nuevos hallazgos. Entre la primera y la segunda intervención, se realizó una tiroidectomía total por un bocio uninodular, cuya biopsia informó 3 paratiroides normotípicas, intratiroideas. En suma, se lograron identificar histológicamente 6 glándulas paratiroides.

Primary hyperparathyroidism and incidental multifocal metastatic papillary thyroid carcinoma in a man / Hiperparatiroidismo primário e carcinoma papilífero metastático, multifocal e incidental da tiroide em paciente do sexo masculino

Co-existence of primary hyperparathyroidism (PHPT) and non-medullary thyroid carcinoma has been previously reported in sporadic case reports and some surgical series, but the majority of cases concerned women with occult papillary carcinomas without cervical lymph node involvement. We present a 71-year man with PHPT and multinodular goiter who was subjected to surgery for single parathyroid adenoma and was found to have synchronous multifocal papillary thyroid carcinoma (PTC) with cervical lymph node involvement. Review of the literature retrieved only a few similar cases. Ultrasonography of both thyroid and parathyroid glands might be considered in patients with hyperparathyroidism. Given that there are no specific guidelines for the management of patients with synchronous PHPT and PTC, they should be managed like the cases of single PTC.

A coexistência de hiperparatiroidismo primário (HPTP) e de carcinoma da tiroide não medular foi descrita anteriormente em relatos de caso esporádicos e em algumas séries cirúrgicas. Entretanto, a maioria dos casos se referiu a mulheres com carcinoma papilífero oculto sem o comprometimento de linfonodos cervicais. Apresentamos um paciente de 71 anos de idade do sexo masculino com HPTP e bócio multinodular que foi submetido a uma cirurgia para adenoma solitário da paratiroide e foi encontrado carcinoma papilífero da tiroide (PTC) multifocal com comprometimento de linfonodos cervicais. Uma revisão da literatura resultou em apenas alguns casos similares. A realização de uma ultrassonografia das glândulas tiroide e paratiroide pode ser considerada em pacientes com hiperparatiroidismo. Como não existe diretriz específica para o tratamento de pacientes com concomitância de HPTP e PTC, eles devem ser tratados como um caso isolado de PTC.

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.