Hiperparatiroidismo primario / Primary hyperparathyroidism

Resumen El hiperparatiroidismo primario (HPP) se define como un trastorno endocrinológico caracterizado por hipercalcemia y niveles de hormona paratiroidea (PTH) elevados o inadecuadamente normales resultado de la secreción excesiva de PTH de una o más glándulas paratiroides. Con respecto a la causa, en un 85% se debe a un adenoma paratiroideo único y un 95% de los casos son esporádicos, sin un factor etiológico identificable. La presentación clásica en la mayoría de los casos es de forma asintomática, sin embargo, sus síntomas o signos clásicos incluyen los derivados de la afectación renal, donde la nefrolitiasis es la manifestación clínica más frecuente de la afectación ósea y de la hipercalcemia. El diagnóstico es bioquímico, sin necesidad de estudio imagenológico, el cual se realiza únicamente como pruebas localizadoras prequirúrgicas. El tratamiento definitivo es quirúrgico, siendo la paratiroidectomía con abordaje cervical el procedimiento de elección.

Primary hyperparathyroidism (PHP) is defined as an endocrine disorder characterized by hypercalcemia and elevated or inadequately normal parathyroid hormone (PTH) levels resulting from the excessive secretion of PTH from one or more parathyroid glands. The cause of this disorder is due, in 85%, to a single parathyroid adenoma and most cases (95%) are sporadic, without an identifiable etiologic factor. PHP is asymptomatic in most patients, however, classic symptoms or signs include those derived from renal involvement, where nephrolithiasis is the most frequent clinical manifestation, and those derived from bone affectation and hypercalcemia. The diagnosis is biochemical, without the need for an imaging study, which is performed only as pre-surgical locator tests. The definitive treatment is surgical, being the parathyroidectomy with cervical approach the procedure of choice.

Hiperparatiroidismo primario por carcinoma paratiroideo: caso problema y revisión / Primary hyperparathyroidism by parathyroid cancer: case report and review

El carcinoma paratiroideo (CP) es una neoplasia maligna con una incidencia de 0,015 cada 100.000 habitantes por año. Representa el 1% de los diagnósticos de hiperparatiroidismo primario y se manifiesta entre la 4.a y 5.a década de la vida, con una incidencia similar entre hombres y mujeres. La etiología del CP es incierta, ha sido asociada a formas esporádicas o familiares. Está caracterizado por altos niveles séricos de calcio y PTH y el desafío clínico-quirúrgico es el diagnóstico diferencial con otras entidades benignas como el adenoma o la hiperplasia de paratiroides. Aunque el diagnóstico de certeza es anatomopatológico, la sospecha clínica y el uso de métodos de baja complejidad (ecografía) con operadores avezados permite una correcta localización y abordaje pertinente del paciente para dirigir el tratamiento quirúrgico adecuado (resección en bloque) evitando persistencias y recurrencias de enfermedad. Se presenta el caso clínico de un paciente masculino que ingresa por síndrome de impregnación asociado a hipercalcemia, su abordaje diagnóstico, tratamiento y manejo interdisciplinario con discusión y revisión bibliográfica. (AU)

Parathyroid carcinoma (CP) is a malignant disease with an incidence of 0.015 per 100,000 inhabitants per year. It accounts for 1% of primary hyperparathyroidism diagnoses and occurs between the 4th and 5th decade of life, with a similar incidence between men and women. The etiology of CP is uncertain and has been associated with sporadic or family forms. CP is characterized by high serum calcium and PTH levels and the clinical-surgical challenge is the differential diagnosis with other benign entities such as parathyroid adenoma or hyperplasia. Although the diagnosis of certainty is achieved by pathological anatomy examination, the clinical suspicion and the use of low complexity methods (ultrasound) by experienced operators allows a correct localization and a patient-specific approach to direct the appropriate surgical treatment (block resection), avoiding persistence and recurrences of disease. The clinical case of a male patient admitted for severe hypercalcemia with multiple organ disfunction, the diagnostic approaches, treatment, and interdisciplinary management, together with review and discussion of the current literature are presented. (AU)

Tumor pardo del paladar, presentación atípica de hiperparatiroidismo primario en adolescente: buena evolución y normalización de la masa ósea tras paratiroidectomía / Brown tumor of the palate, atypical presentation of primary hyperparathyroidism in an adolescent: good evolution and bone mass normalization after parathyroidectomy

El hiperparatiroidismo primario (HPTP) es poco frecuente en niños y adolescentes. Hay escasos datos para el manejo de estos pacientes en pediatría. Las glándulas paratiroideas son glándulas endocrinas que secretan hormona paratiroidea (PTH) y regulan el metabolismo del calcio y del fósforo. La sobreexpresión de PTH se llama hiperparatiroidismo, que se clasifica en primario, secundario y terciario. En los adolescentes, 80 a 92% de los hiperparatiroidismos primarios se deben a adenoma paratiroideo. Presentamos el caso clínico de una adolescente con una primera manifestación atípica de HPTP, la presencia de un tumor pardo del paladar, presentación rara de adenoma paratiroideo, acompañado de hipercalcemia, marcada elevación de PTH y varias lesiones óseas. (AU)

Primary hyperparathyroidism (PHPT) in children and adolescents is uncommon. Guidelines for management in pediatric patients are limited. Parathyroid glands are endocrine glands that secrete parathyroid hormone (PTH) and regulate calciumphosphate metabolism. The overexpression of PTH is called hyperparathyroidism, and is classified as primary, secondary, and tertiary. In adolescents, 80 to 92% of PHPT cases are due to a parathyroid adenoma. We present here a case report of an adolescent with a brown tumor of the palate as the first manifestation of the disease, atypical and rare presentation of parathyroid adenoma in an adolescent. She had hypercalcemia, marked elevation of PTH and bone lesions. (AU)

Utilidad de la 18f-colina pet/tc en hiperparatiroidismo primario persistente o recurrente: experiencia inicial / Usefulness of 18f-choline pet / tc in persistent or recurrent primary hyperparathyroidism: initial experience

El hiperparatiroidismo persistente/recurrente representa un desafío en la localización del tejido paratiroideo hiperfuncionante. En esta subpoblación, los métodos convencionales ofrecen un menor rédito diagnóstico. La 18F-colina PET/TC podría ser una buena alternativa dada su mejor resolución espacial, capacidad de detectar glándulas ectópicas y la conjunción de la imagen molecular y anatómica. Sin embargo, la evidencia en este subgrupo de pacientes es escasa. Objetivo: evaluar la utilidad de la 18F-colina PET/TC como método de localización en el hiperparatiroidismo persistente o recurrente. Materiales y métodos: se analizaron los pacientes con 18F-colina PET/TC para hiperparatiroidismo entre diciembre de 2015 y enero de 2018 en un centro terciario de alto volumen. Se analizaron el número de lesiones, su localización, tamaño y el Standard Uptake Value máximo (SUV max) en las imágenes tempranas y tardías. Se compararon los resultados con los métodos convencionales. Resultados: 7 de 15 pacientes habían sido operados previamente (persistentes/recurrentes). La 18F-colina PET/TC detectó 6/7 casos (83,33%), la ecografía cervical 1/4 (25%) y el SPECT de paratiroides y la resonancia nuclear magnética 2/5 (40%). El SUV max obtenido fue variable, en la mitad de los casos a los 10 minutos y en los restantes a la hora; el tamaño promedio de las lesiones fue 8,61 mm (6-12 mm). Conclusiones: la 18F-colina PET/TC muestra una alta tasa de detección en los pacientes con hiperparatiroidismo persistente/recurrente. La combinación del comportamiento biológico del PET con los hallazgos morfológicos aportados por la TC con contraste endovenoso le ofrecería ventajas sobre otros estudios que podrían posicionarlo como método de primera línea en esta subpoblación. (AU)

Persistent or recurrent hyperparathyroidism represents a challenge regarding the localization of the hyper-functioning parathyroid tissue. In this subpopulation of hyperpharathyroid patients, conventional methods have a low diagnostic yield. The 18F-choline PET /CT could be a good alternative given its better spatial resolution, ability to detect ectopic glands, and the conjunction of the molecular and anatomical image. However, the evidence in this subgroup of patients is limited. Objective: to evaluate the utility of 18F-choline PET/ CT as a localization method in persistent or recurrent hyperparathyroidism. Materials and methods: patients with 18F-choline PET / CT for hyperparathyroidism between December 2015 and January 2018 in a high-volume tertiary center were included. The number of lesions, and their location, size, and maximum Standard Uptake Value (SUV) in the early and late images were analyzed. The results were compared to conventional methods. Results: 7 of 15 patients had been previously operated (persistent/recurrent). 18F-choline PET / CT detected 6/7 cases (83,33%), cervical ultrasound 1/4 (25%) and parathyroid SPECT and magnetic resonance 2/5 (40%). The maximum SUV was variable, one half at 10 minutes and the other half at 60 minutes; the average size of the lesions was 8.61 mm (6-12 mm). Conclusions: 18F-Choline PET / CT shows a high detection rate in patients with persistent / recurrent hyperparathyroidism. The combination of the biological behavior of PET with the morphological findings provided by CT with intravenous contrast would offer advantages over other studies that could position it as a first line method in this subpopulation. (AU)

Hiperparatiroidismo persistente debido a paratiromatosis / Persistent hyperparathyroidism due to parathyromatosis

RESUMEN La paratiromatosis se describe como una dolencia en la que existen múltiples nódulos de tejido paratiroideo hiperfuncionante diseminados por cuello y el mediastino, en la cual las pruebas de imagen no son efectivas para localizar las glándulas paratiroides y requiere de la combinación de un tratamiento médico y quirúrgico que en ocasiones se presenta como un desafío. Describimos el caso de una mujer de 61 años con hiperparatiroidismo primario recurrente a la que se le extirpan un total de 9 glándulas paratiroideas en 3 cirugías diferentes, y realizamos revisión de la literatura.

ABSTRACT Parathyromatosis is described as a condition in which there are multiple nodules of hyperfunctioning parathyroid tissue disseminated by the neck and mediastinum. Imaging tests are not effective in locating the parathyroid glands and requires the combination of medical and surgical treatment that sometimes is challenging. We describe the case of a 61-year-old woman with recurrent primary hyperparathyroidism. A total of 9 parathyroid glands where removed in 3 different surgeries. We also reviewed the literature.

Hiperparatiroidismo primario en adolescente. Reporte de caso / Primary hyperparathyroidism in adolescent. Case report

INTRODUCTION: The hypercalcemia is infrequent in pediatrics, its clinical is diverse, and its etiology is determined by age. Among the dependent causes of parathormone (PHT) is the hyperthyroidism, state of hypersecretion of PHT by parathyroid glands (PG). The primary hyperparathyroidism (PHPT) is rare in children, there are 200 reported cases. In older children, the causes for PHPT correspond to parathyroid adenoma, multiglandular disease and parathyroid carcinoma. OBJECTIVE: Report a case of an 11 years old male adolescent. He presents three months symptoms of constipation, anorexia, vomiting and weight loss. Urgent consultation due to an increase of his symptoms, in exams stand out: calcemia 16.67 mg/dl (NV 8.8-10.8 mg/dl), phosphatemia 2.21 mg/dl (NV 4.5-5.5 mg/dl), parathormone (PHT) 308.7 pg/ml (NV 15-68.3 pg/ml), calciuria/creatininuria 0.56 (NV < 0.2). He was hospitalized to manage his severe hypercalcemia, it was indicated hyperhydration, monopotassium phosphate, intravenous hydrocortisone and furosemide. In his study was performed a cervical ultrasound which showed a solid node in the right parathyroid gland, hypoechogenic and scintigram parathyroid compatible with right superior parathyroid adenoma. In the waiting for surgery was necessary the administration of intravenous pamidronate. In the post-operatory, he evolved with hipocalcemia that was corrected with intravenous calcium carbonate, overlapping to oral calcium and calcitriol. CONCLUSION: The PHPT is a pathology of low prevalence in pediatrics, one hundred times less than adults. In the adolescent, the most frequent cause is the parathyroid adenoma. It should be considered as a differential diagnosis in cases of symptomatic hypercalcemia in this age group

Primary Hyperparathyroidism: The Influence of Bone Marrow Adipose Tissue on Bone Loss and of Osteocalcin on Insulin Resistance

OBJECTIVES: Bone marrow adipose tissue has been associated with low bone mineral density. However, no data exist regarding marrow adipose tissue in primary hyperparathyroidism, a disorder associated with bone loss in conditions of high bone turnover. The objective of the present study was to investigate the relationship between marrow adipose tissue, bone mass and parathyroid hormone. The influence of osteocalcin on the homeostasis model assessment of insulin resistance was also evaluated. METHODS: This was a cross-sectional study conducted at a university hospital, involving 18 patients with primary hyperparathyroidism (PHPT) and 21 controls (CG). Bone mass was assessed by dual-energy x-ray absorptiometry and marrow adipose tissue was assessed by 1H magnetic resonance spectroscopy. The biochemical evaluation included the determination of parathyroid hormone, osteocalcin, glucose and insulin levels. RESULTS: A negative association was found between the bone mass at the 1/3 radius and parathyroid hormone levels (r = -0.69; p<0.01). Marrow adipose tissue was not significantly increased in patients (CG = 32.8±11.2% vs PHPT = 38.6±12%). The serum levels of osteocalcin were higher in patients (CG = 8.6±3.6 ng/mL vs PHPT = 36.5±38.4 ng/mL; p<0.005), but no associations were observed between osteocalcin and insulin or between insulin and both marrow adipose tissue and bone mass. CONCLUSION: These results suggest that the increment of adipogenesis in the bone marrow microenvironment under conditions of high bone turnover due to primary hyperparathyroidism is limited. Despite the increased serum levels of osteocalcin due to primary hyperparathyroidism, these patients tend to have impaired insulin sensitivity.

Association between atypical parathyroid adenoma and neurofibromatosis

Primary hyperparathyroidism is a disease characterized by excessive production of parathyroid hormone (PTH), which is due to a parathyroid adenoma in 85% of cases. An atypical parathyroid adenoma, with some histopathological features of parathyroid carcinoma, may be found in some of the cases, although it may not fulfill all the criteria for this diagnosis. Neurofibromatosis type 1 (NF1) is an autosomal dominant systemic disease that may be associated with hyperparathyroidism. We report here the rare combination of a patient with NF1 and clinical manifestations of hyperparathyroidism due to an atypical parathyroid adenoma.

Macroadenoma cervical palpable: Una presentación muy inusual de hiperparatiroidismo primario / Cervical macroadenoma causing hyperparathyroidism: Report of one case

We report a 59-year-old man with a history of hypertension, recurrent renal stones and a severe hypercalcemia of 14.9 mg/dl with a serum phosphorus of 2.4 mg/dl and a serum albumin of 3.6 g/dl. Physical examination showed a 4 cm left cervical nodule, consistent with the diagnosis of thyroid nodule. Parathyroid hormone (PTH) levels were 844 pg/mL (normal 15-65 pg/ml) and a cervical ultrasound examination disclosed a solid nodule in the lower left lobe of 40 x 30 x 25 mm, adjacent to the thyroid parenchyma. Abdominal ultrasound revealed bilateral renal stones. Parathyroid scintigraphy showed a high uptake of the left lower parathyroid mass and a bone densitometry showed bone density t scores of -1.2 in the spine, -2.0 in the right femoral neck and -3.5 in the distal radius. A review of his medical record revealed the presence of hypercalcemia for at least 4 years. He was admitted for hydration and administration of 4 mg zoledronic acid iv. At 24 hours, serum calcium dropped to 11.0 mg/dl, and a left thyroid lobectomy was performed including the lower left parathyroid gland. The pathology report showed a 22.6 g parathyroid adenoma. Intraoperatory PTH descended > 50%, consistent with successful parathyroidectomy. At 7 days after surgery serum calcium was 8.8 mg/dl, phosphorus 2.1 mg/dl, alkaline phosphatase 166 U/L, albumin 3.9 g/dL, PTH 230 pg/ml and 25-OH vitamin D 12.4 ng/ml. This finding was interpreted as secondary hyperparathyroidism due to vitamin D deficiency and “hungry bone”, being less likely the presence of residual or metastatic parathyroid tissue. A cholecalciferol load was administered, with significant descent of PTH.

Nonadenomatous nonencapsulated thymic parathyroid tissue concomitant with primary hyperparathyroidism due to ectopic parathyroid adenoma / Tecido tímico paratireoidiano não edematoso e não encapsulado concomitante com hiperparatireoidismo devido a adenoma paratireoidiano ectópico

Primary hyperparathyroidism due to ectopic parathyroid adenoma is not infrequent. Primary hyperparathyroidism caused by unusual thymic nonadenomatous nonencapsulated parathyroid tissue has been reported before. Both can cause unsuccessful neck explorations. Here we presented for the first time a patient with hyperparathyroidism due to ectopic parathyroid adenoma concomitant to the presence of thymic nonadenomatous nonencapsulated parathyroid tissue.

O hiperparatireodismo primário devido a adenoma ectópico paratireoidiano não é raro. O hiperparatireodismo primário causado por tecido tímico paratireoidiano não edematoso e não encapsulado incomum já foi relatado anteriormente. Ambos podem levar à exploração cervical malsucedida. Apresentamos aqui, pela primeira vez, uma paciente com hiperparatireoidismo decorrente de um adenoma paratireoidiano concomitante com a presença de tecido tímico paratireoidiano não edematoso e não encapsulado.

Persistent primary hyperparathyroidism: an uncommon location for an ectopic gland - Case report and review / Hiperparatiroidismo primário persistente: glândula ectópica em localização rara - Caso clínico e revisão

Primary hyperparathyroidism (PHPT) is a common endocrine disorder that mainly affects middle-aged women. Patients are usually asymptomatic. The disease might be ascribable to hyperplasia, carcinoma, and single or multiple adenomas. PHPT may be sporadic or familial, the latter comprising multiple endocrine neoplasia type 1 or 2A, familial benign hypocalciuria hypercalcemia, and hyperparathyroidism-jaw tumor syndrome. The most common causes for persistent PHPT are multiglandular disease, and missed abnormal ectopic or orthotopic parathyroid glands. Imaging localization studies should precede a new surgical intervention. Ectopic parathyroid glands are rarely located at the aortopulmonary window. For diagnosis confirmation, 99mTc-sestamibi SPECT/CT seems to be an advantageous test. Another possibility is to perform 99mTc-sestamibi followed by thoracic CT or MRI. Parathyroidectomy may be performed by means of median sternotomy, thoracotomy, or video-assisted thoracoscopy. We describe a case of persistent primary hyperparathyroidism due to the presence of an ectopic parathyroid gland found at the aortopulmonary window. As the investigation necessary to clarify the etiology of recurrent nephrolithiasis proceeded, the diagnosis of PHPT was determined. The patient underwent subtotal parathyroidectomy; nevertheless, PHPT persisted. Genetic syndromes that could account for this condition were excluded. Imaging studies available at that time were not able to locate abnormal glands; moreover, the patient refused to undergo surgical exploration. Later, the patient underwent 99mTc-sestamibi SPECT/CT, which revealed a parathyroid gland at the aortopulmonary window.

O hiperparatiroidismo primário (HPP) é uma endocrinopatia frequente que afeta maioritariamente mulheres de meia-idade e é geralmente assintomática. A doença pode ser atribuível a hiperplasia, carcinoma, adenomas únicos ou múltiplos. O HPP inclui formas esporádicas e familiares. As formas familiares englobam neoplasia endócrina múltipla tipo 1 ou 2A, hipercalcemia hipocalciúrica familiar e síndrome hiperparatiroidismo/tumor mandibular-maxilar. As causas mais frequentes de HPP persistente são a presença de doença multiglandular ou de paratiroide anômala ectópica ou ortotópica não identificada previamente. É recomendável que a localização imagiológica preceda a reintervenção cirúrgica. A janela aortopulmonar é uma localização ectópica rara, sendo o 99mTc-sestamibi SPECT/TC um exame de confirmação vantajoso ou, alternativamente o 99mTc-sestamibi seguido de TC ou RM torácica. A paratiroidectomia pode ser efetuada por meio de esternotomia mediana, toracotomia ou toracoscopia videoassistida. Descrevemos um caso de HPP persistente atribuível à presença de uma glândula paratiroide ectópica localizada à janela aortopulmonar. O diagnóstico de HPP foi estabelecido na sequência da investigação requisitada para esclarecimento etiológico da nefrolitíase recidivante constatada nessa doente. Foi submetida à paratiroidectomia subtotal; não obstante, o HPP persistiu. Excluíram-se síndromes genéticas que pudessem justificar esse quadro clínico. Os exames imagiológicos disponíveis (à época) revelaram-se infrutíferos na detecção de paratiroides anômalas; adicionalmente, a doente recusou exploração cirúrgica. Posteriormente, a doente foi submetida a 99mTc-sestamibi SPECT/TC, que revelou a presença de uma paratiroide na janela aortopulmonar.

Biochemical, bone and renal patterns in hyperparathyroidism associated with multiple endocrine neoplasia type 1

Primary hyperparathyroidism associated with multiple endocrine neoplasia type I (hyperparathyroidism/multiple endocrine neoplasia type 1) differs in many aspects from sporadic hyperparathyroidism, which is the most frequently occurring form of hyperparathyroidism. Bone mineral density has frequently been studied in sporadic hyperparathyroidism but it has very rarely been examined in cases of hyperparathyroidism/multiple endocrine neoplasia type 1. Cortical bone mineral density in hyperparathyroidism/multiple endocrine neoplasia type 1 cases has only recently been examined, and early, severe and frequent bone mineral losses have been documented at this site. Early bone mineral losses are highly prevalent in the trabecular bone of patients with hyperparathyroidism/multiple endocrine neoplasia type 1. In summary, bone mineral disease in multiple endocrine neoplasia type 1related hyperparathyroidism is an early, frequent and severe disturbance, occurring in both the cortical and trabecular bones. In addition, renal complications secondary to sporadic hyperparathyroidism are often studied, but very little work has been done on this issue in hyperparathyroidism/multiple endocrine neoplasia type 1. It has been recently verified that early, frequent, and severe renal lesions occur in patients with hyperparathyroidism/multiple endocrine neoplasia type 1, which may lead to increased morbidity and mortality. In this article we review the few available studies on bone mineral and renal disturbances in the setting of hyperparathyroidism/multiple endocrine neoplasia type 1. We performed a meta-analysis of the available data on bone mineral and renal disease in cases of multiple endocrine neoplasia type 1-related hyperparathyroidism.

Total parathyroidectomy in a large cohort of cases with hyperparathyroidism associated with multiple endocrine neoplasia type 1: experience from a single academic center

Most cases of sporadic primary hyperparathyroidism present disturbances in a single parathyroid gland and the surgery of choice is adenomectomy. Conversely, hyperparathyroidism associated with multiple endocrine neoplasia type 1 (hyperparathyroidism/multiple endocrine neoplasia type 1) is an asynchronic, asymmetrical multiglandular disease and it is surgically approached by either subtotal parathyroidectomy or total parathyroidectomy followed by parathyroid auto-implant to the forearm. In skilful hands, the efficacy of both approaches is similar and both should be complemented by prophylactic thymectomy. In a single academic center, 83 cases of hyperparathyroidism/ multiple endocrine neoplasia type 1 were operated on from 1987 to 2010 and our first surgical choice was total parathyroidectomy followed by parathyroid auto-implant to the non-dominant forearm and, since 1997, associated transcervical thymectomy to prevent thymic carcinoid. Overall, 40% of patients were given calcium replacement (mean intake 1.6 g/day) during the first months after surgery, and this fell to 28% in patients with longer follow-up. These findings indicate that several months may be needed in order to achieve a proper secretion by the parathyroid auto-implant. Hyperparathyroidism recurrence was observed in up to 15% of cases several years after the initial surgery. Thus, long-term follow-up is recommended for such cases. We conclude that, despite a tendency to subtotal parathyroidectomy worldwide, total parathyroidectomy followed by parathyroid auto-implant is a valid surgical option to treat hyperparathyroidism/multiple endocrine neoplasia type 1. Larger comparative systematic studies are needed to define the best surgical approach to hyperparathyroidism/multiple endocrine neoplasia type 1.

Intervención farmacológica con calcitonina en gramagrafía con 99mTc-Sestamibi de dos fases para localización de adenoma y/o hiperplasia de paratiroides / Pharmacologic intervention with calcitonin in 99mTc-Sestamibi scintigraphy for patients with primary hyperparathyroidism

Introducción. En el diagnóstico de enfermedad paratiroidea, persiste la necesidad de contar con un procedimiento eficiente que contribuya a tomar la mejor decisión respecto al manejo del paciente; con este propósito se desarrolló un estudio retrospectivo para evaluar el rendimiento de la gamagrafía con 99mTc-Sestamibi luego de la intervención farmacológica con calcitonina en pacientes con diagnóstico de hiperparatiroidismo primario. Método Se incluyeron pacientes con diagnóstico de hiperparatiroidismo primario que fueron evaluados de forma consecutiva con gamagrafía de paratiroides utilizando el protocolo habitual de 99mTc-Sestamibi en dos fases, y el estudio de 99mTc-Sestamibi-calcitonina, con seguimiento mínimo de 6 meses post gamagrafía. Se calculó la sensibilidad (SENS), especificidad (ESP) y exactitud (EXAC) para cada protocolo, entre otros valores estadísticos. Resultados. Los pacientes que fueron sometidos a gamagrafía basal y post-calcitonina fueron 42, de los cuales 35 tuvieron seguimiento completo: a) Estudio Basal: SENS 65,6 por ciento, ESP 100 por ciento, EXAC 68,5 por ciento. Al diferenciarlos por patologías: para adenoma, SENS 53,8 por ciento, ESP 54,5 por ciento; para hiperplasia, SENS 25 por ciento, ESP 90 por ciento. b) Estudio Post-Calcitonina: SENS 92,3 por ciento, ESP 43,5 por ciento, EXAC 94,3 por ciento. Para adenoma, SENS 92,3 por ciento, ESP 45,5 por ciento; para hiperplasia, SENS 25 por ciento, ESP 77,4 por ciento. Conclusiones. Nuestros resultados utilizando el protocolo con calcitonina demuestran aumento de la sensibilidad global, ayuda a clasificar mejor los pacientes con enfermedad paratiroidea e incrementa la exactitud diagnóstica.

Introduction. There is a need to improve the methods for the diagnosis of parathyroid disease allowing to make the most appropriate therapeutic decision. With this purpose, we performed a retrospective study in order to evaluate the diagnostic accuracy of 99mTc-Sestamibi scintigraphy after pharmacologic intervention with calcitonin in patients with primary hyperparathyroidism. Methods. Patients with diagnosis of primary hyperparathyroidism were included, who were consecutively evaluated with parathyroid scintigraphy using the standard double-phase 99mTc-Sestamibi protocol and a study with 99mTc-Sestamibi-calcitonin, having received a follow-up of at least 6 months after the nuclear study. Sensitivity (SENS), specificity (SPE) and accuracy (ACC) were calculated for each protocol, among other statistical parameters. Results. Fourty-two patients were submitted for basal and post-calcitonin scintigraphy, of which 35 had a complete follow-up. a) Basal study: SENS 65,6 percent, SPE 100 percent, ACC 68,5 percent. After classification according to pathology: for adenoma, SENS 53,8 percent, SPE 54,5 percent; for hyperplasia, SENS 25 percent, SPE 90 percent. b) Post-calcitonin study: SENS 92,3 percent, SPE 43,5 percent, ACC 94,3 percent. For adenoma, SENS 92,3 percent, SPE 45,5 percent; for hyperplasia, SENS 25 percent, SPE 77,4 percent. Conclusions Our results using calcitonin protocol demonstrate an increase in global sensitivity, helping to better classify patients with parathyroid disease by improving diagnostic accuracy.

Múltiples adenomas paratiroideos ectópicos mediastinal como causa de hipercalcemia e insuficiencia renal / Multiple mediastinal ectopic parathyroid adenomas as the cause of hypercalcemia and renal failure

Se presenta la historia clínica de una paciente de 66 años de edad con antecedentes de buena salud, que acude a consulta por decaimiento, pérdida del apetito, cifras elevadas de tensión arterial y pérdida de la elasticidad de la piel de ambos miembros inferiores. En los estudios complementarios se detectó una hipercalcemia severa, aumento de las cifras de creatinina de forma progresiva y cifras elevadas de paratohormona. Se le diagnosticó una masa en el cuello y múltiples adenomas paratiroideos mediastinales por ganmagrafía paratiroidea con methoxy-isobutyl-isonitrite 99 mtc y angiotomografía de mediastino. Se presenta este caso por lo inusual que resulta encontrar el hiperparatiroidismo primario como causa de insuficiencia renal rápidamente progresiva sin la presencia de nefropatía obstructiva por litiasis. Se demuestra que este diagnóstico solo es posible mediante la utilización de técnicas radiológicas combinadas.

The medical history of a 66 years-old patient with a history of good health condition, who went to see the doctor because she felt exhausted, had lost appetite, her blood pressure figures had increased and her lower limbs had lost their elasticity. The supplementary exams showed severe hypercalcemia, progressive increase of creatinine values and high paratohormone figures. The diagnosis was a mass in her neck and multiple mediastinal parathyroid adenomas, detected by parathyroid gammagraphy with methoxy-isobutyl isonitrite 99 mtc and angiotomography of the mediastinum. This case was presented in this paper because it is not common to find primary hyperparathyroidism as the cause of rapidly progressive renal failure without obstructive nephropathy due to lithiasis. It was demonstrated that this kind of diagnosis can only be made if combined radiological techniques are used.

Primary hyperparathyroidism due to cystic parathyroid adenoma: a case report.

Raw Cystic parathyroid adenoma is a rare cause of primary hyperparathyroidism. The authors report one case of cystic parathyroid adenoma, who presented with progressive right hip pain for one year. The patient had severe hypercalcemia at the first presentation and was misdiagnosed as having metastatic cancer at first. An iliac bone biopsy was performed and showed a giant cell tumor. Parathyroid hormone level was evaluated later and was found to be high, 1,555 pg/ml (15-65 pg/ml). An MRI study of the neck was done and revealed a cystic mass 38 x 36 x 40 mm in diameter just below the left lower pole of the thyroid gland. Tc-99m MIBI scan demonstrated increase and retention of radioactivity uptake at the same area. Hyperfunctioning parathyroid gland was considered. Parathyroidectomy was done and histopathology revealed cystic parathyroid adenoma. Serum calcium was normal and hip pain was markedly improved after the surgery.

Oxyphilic cell adenoma of parathyroid resulting in primary hyperparathyroidism and osteitis fibrosa cystica--a case report.

Adenomas of the parathyroid gland, the majority of which are of the solitary chief cell type, are the most frequent cause of primary hyperparathyroidism (pHPT). Parathyroid adenomas composed predominantly or exclusively of oxyphil cells are rare and most oxyphil cell adenomas of this organ remain clinically silent. We present here a case of hyperfunctioning oxyphil cell adenoma of the parathyroid gland resulting in pHPT, osteitis fibrosa cystica and simultaneous bilateral fractures upper shafts of femora.

Postural shortening due to primary hyperparathyroidism caused by parathyroid adenoma.

Osteoporosis can be primary or secondary. Secondary osteoporosis is the result of an underlying disease such as an endocrine abnormality, and an example of such is primary hyperparathyroidism. The most common cause of primary hyperparathyroidism is parathyroid gland adenoma. The diagnosis of primary hyperparathyroidism is based on the following biochemical examinations: parathyroid hormone, serum calcium, creatinine clearance, 24 hour urinary calcium, and another examination such as parathyroid gland scan. This is a rare case of an adult man who presented with a chief complaint of decreasing body height, back pain, difficulty in taking deep breaths and difficulty in his activities. The patient was diagnosed with primary hyperparathyroidism caused by parathyroid gland adenoma. His complaint was reduced after parathyroidectomy. His new complaint was that his tooth can be pulled out easily. We found high levels of parathyroid hormone and low levels of serum calcium caused by secondary hyperparathyroidism.

Adenoma paratiroideo ectopico mediastinal: diagnostico por sonda de deteccion gamma y reseccion por videotoracoscopia / Ectopic mediastinal parathyroid adenoma: detection with a radioisotopic probe and resolution with videothoracoscopy

Presentamos el caso de una mujer de 66 años con diagnóstico de hiperparatiroidismo primariopor adenoma paratiroideo ectópico mediastinal realizado por gammagrafía paratiroidea con 99mtcmetoxi-isobutil-isonitrito (Tc99-MIBI). Fue intervenida exitosamente mediante la técnica de videotoracoscopia.Durante el procedimiento se utilizó la medición de parathormona y a los 10 minutos de extirpado el adenoma seobtuvo un descenso mayor del 50% del basal. La sonda de detección gamma no fue efectiva in situ por la superposicióndel radioisótopo con el miocardio pero confirmó la radioactividad dentro del quirófano una vez extirpadoel adenoma. La paciente normalizó los valores de calcemia y parathormona en el postoperatorio y permaneciónormocalcémica luego de 9 meses de seguimiento. En nuestro caso, la localización prequirúrgica y elmonitoreo de parathormona intraoperatoria fueron de gran utilidad para el éxito quirúrgico, la sonda detectora marcada con Tc99-MIBI no fue efectiva in situ pero confirmó la radioactividad una vez extirpado el adenoma. El tratamiento quirúrgico endoscópico por videotoracoscopia fue bien tolerado, menos doloroso que la toracotomía y acortó el tiempo de internación

We report a 66 years old woman with a diagnosis of primary Hyperparathyroidism. Localization to mediastinum was obtained with parathyroid scintigraphy using 99mtc-methoxyisobutyl-isonitrite (Tc99-MIBI). The patient was successfully operated upon by means of a videothora-coscopicapproach. During the procedure serum parathormone was measured before and 10 minutes after adenomectomy,showing a more than 50% reduction from the basal level. An attempt to detect the precise site of the adenomawith a Tc99-MIBI probe was unsuccessful because of its proximity to the myocardium, but radioactivity was confirmed on the surgical specimen after resection. The patient’s calcemia and parathormone levels became normalduring the postoperative course and she remains normocalcemic 9 months after the procedure. In our case,preoperative localization and intraoperative parathormone measurements were both very useful for confirming surgical success; the intraoperative localization with a radioactive probe was not useful, but radioactivity was confirmedafter resection on the surgical specimen. The endoscopic surgical procedure with videothoracoscopy waswell tolerated, less painful than a thoracotomy, and it shortened the hospitalization period

An unusual presentation of parathyroid adenoma--a case report.

A case of intrathyroid parathyroid adenoma which clinically and cytologically mimicked thyroid tumor is presented. The patient was normocalcemic inspite of clinical and radiological features suggestive of hyperparathyroid bone disease. The adenoma showed an area of infarction presumably accounting for the normocalcemic primary hyperparathyroidism. The case is reported for its unusual presentation.