Hiperparatiroidismo ectópico: Detección de la localización mediastinal / Ectopic hyperparathyroidism. Detection of mediastinal localization

El tejido paratiroideo ectópico es una causa frecuente de recurrencia del hiperparatiroidismo (HPT) siendo de 16% en primarios y 14% en secundarios. La localización intratorácica representa el 20-35%, pero solo un 2% requiere una cirugía torácica. El objetivo fue analizar una cohorte de pacientes con diagnóstico de HPT mediastinal operados en un hospital de alta complejidad de Argentina. Se realizó un estudio retrospectivo de todos los operados por HPT entre enero de 2006 y julio 2019 en ese hospital. Se incluyeron aquellos que requirieron acceso torácico por HPT ectópico. En este período se trataron 728 pacientes con HPT primario y secundario. Seis con primario y 3 con secundario requirieron cirugía torácica. Se realizaron 6 videotoracoscopias (VATS) y 3 esternotomías, sin complicaciones graves. Se utilizó biopsia por congelación en todos y dosaje de paratohormona intraoperatoria (PTHi) en 8 casos, que descendió en promedio 65% respecto al valor basal. Se confirmaron 5 adenomas y 4 hiperplasias. La enfermedad paratiroi dea mediastinal representó el 1.65% (12/728), mientras que recibieron tratamiento quirúrgico en nuestra institución 1.24% (9/728). La biopsia por congelación y el descenso de PTHi resultan útiles para confirmar el foco y eventualmente disminuir el riesgo de recurrencia. La VATS es segura pero depende del entrenamiento y de la disponibilidad en el medio asistencial. Si bien el sestamibi es el método con mayor sensibilidad, se propone el uso de 18F-colina PET/TC ante la sospecha de HPT ectópico. La posibilidad de obtener conclusiones basadas en la evidencia requiere de estudios con mayor número de pacientes.

The ectopic parathyroid tissue is a frequent cause of recurrent hyperparathyroidism (HPT), accounting 16% in primary HPT and 14% in secondary cases. Although intrathoracic ectopic glands represent 25-35% of all ectopic cases, only 2% requires thoracic surgery. The aim of this study is to report a case series of patients with ectopic mediastinal HPT treated by thoracic approach in a private institution in Argentina. This is a retrospective analysis from January 2006 to June 2019. All patients diagnosed with ectopic hyperparathyroidism who required a thoracic surgical approach were included. During this period, 728 patients with primary HPT and secondary HPT were treated. Six with primary HPT and 3 with secondary HPT required a thoracic approach. Six video-assisted thoracoscopy surgeries (VATS) and 3 sternotomies were performed. None of them presented serious posoperative complications. Frozen section biopsy was used in all cases. iPTH was measured in 8 cases, with a mean drop of 65% after 15 minutes. Final pathology reports confirmed 5 adenomas and 4 hyperplasias. Our case series reported an incidence of 1.65% (12/728) mediastinal parathyroids, while 1.24% (9/728) received surgical treatment at our institution. Intraoperative frozen section and PTHi are useful to confirm the diagnosis and to avoid recurrences. Although VATS is a safe and efficient treatment option, it depends on surgical training and availability. In terms of diagnostic imaging resources, sestamibi remains the current gold standard. However, 18F-choline PET/CT may arise as a new diagnostic tool. The possibility of obtaining evidence-based conclusions requires studies with higher number of patients.

Supernumerary parathyroid glands in hyperparathyroidism associated with multiple endocrine neoplasia type 1 / Paratireoides supranumerárias em hiperparatireoidismo primário associado a neoplasia endócrina múltipla tipo 1

OBJECTIVE: To evaluate frequency, anatomic presentation, and quantities of supernumerary parathyroids glands in patients with primary hyperparathyroidism (HPT1) associated with multiple endocrine neoplasia type 1 (MEN1), as well as the importance of thymectomy, and the benefits of localizing examinations for those glands. METHODS: Forty-one patients with hyperparathyroidism associated with MEN1 who underwent parathyroidectomy between 1997 and 2007 were retrospectively studied. The location and number of supernumerary parathyroids were reviewed, as well as whether cervical ultrasound and parathyroid SESTAMIBI scan (MIBI) were useful diagnostic tools. RESULTS: In five patients (12.2%) a supernumerary gland was identified. In three of these cases (40%), the glands were near the thyroid gland and were found during the procedure. None of the imaging examinations were able to detect supernumerary parathyroids. In one case, only the pathologic examination could find a microscopic fifth gland in the thymus. In the last case, the supernumerary gland was resected through a sternotomy after a recurrence of hyperparathyroidism, ten years after the initial four-gland parathyroidectomy without thymectomy. MIBI was capable of detecting this gland, but only in the recurrent setting. Cervical ultrasound did not detect any supernumerary glands. CONCLUSION: The frequency of supernumerary parathyroid gland in the HPT1/MEN1 patients studied (12.2%) was significant. Surgeons should be aware of the need to search for supernumerary glands during neck exploration, besides the thymus. Imaging examinations were not useful in the pre-surgical location of these glands, and one case presented a recurrence of hyperparathyroidism.

OBJETIVO: Avaliação da frequência, da localização anatômica e do número de paratireoides extranumerárias em pacientes com hiperparatireoidismo primário (HPT1) associado a neoplasia endócrina múltipla tipo 1(NEM1), além da avaliação da importância da timectomia e da utilidade dos exames radiológicos para localização destes. MÉTODOS: Foram avaliados de forma retrospectiva 41 pacientes portadores de NEM1 com HPT1 submetidos a paratireoidectomia entre 1997 e 2007. O número de glândulas supranumerárias encontradas e a sua localização foram revisados, assim como a utilidade do ultrassom cervical e do SESTAMIBI (MIBI) de paratireoide como ferramentas diagnósticas. RESULTADOS: Em cinco pacientes (12,2%) foram identificadas glândulas supranumerárias. Em três destes (40%), as glândulas estavam próximas à glândula tireoide e foram encontradas durante a exploração cirúrgica. Os exames de imagem não foram úteis para a localização destas glândulas. Em um caso, apenas o exame anatomopatológico foi capaz de encontrar uma glândula extranumerária microscópica localizada no timo. No último caso, uma quinta glândula foi ressecada por meio de esternotomia após a recidiva do hiperparatireoidismo, cerca de 10 anos após a paratireoidectomia realizada sem timectomia na ocasião. Neste caso o MIBI detectou esta paratireoide apenas após a recidiva da doença. Em nenhum dos casos o ultrassom cervical foi capaz de detectar glândulas extranumerárias. CONCLUSÃO: A frequência de paratireoides supranumerárias em nossa casuística foi significativa (12,2%). Durante a exploração cervical, o cirurgião deve estar atento para localizar glândulas extranumerárias além do timo. Exames de imagem não foram úteis na localização préoperatória dessas glândulas, e em um caso houve recidiva do hiperparatireoidismo.

Hiperparatiroidismo / Hyperparathyroidism

El calcio es esencial para varias funciones del cuerpo, incluyendo la contracción muscular, la conducción nerviosa y el funcionamiento apropiado de muchas enzimas. La mayor parte del calcio del cuerpo se almacena en los huesos, pero también se encuentra en las células y en la sangre. El organismo controla con "toda precisión" la cantidad de calcio tanto en la célula como en la sangre. Para mantener este equilibrio entra en competencia la hormona para tiroidea, secretada por las glándulas paratiroidea. La actividad de las glándulas paratiroideas está controlada por el nivel de calcio libre (ionizado) de la sangre. El descenso de los niveles de este calcio estimula la síntesis y secreción de hormonas paratiroideas, si la cantidad de calcio libre en sangre aumenta, estamos frente a una alteración metabólica "La Hipercalcemia" la cual consiste en una concentración elevada de calcio en sangre superior a 10.5 miligramos por decilitro de sangre, la que puede ser causada por aumento en la ingestión de calcio, pero la causa más común de la hipercalcemia es el "Hiperparatiroidismo", el cual se debe a la hipersecreción de hormonas paratiroideas. Esta alteración puede deberse a un adenoma paratiroide (80%). Hiperplasia primaria (15%). Carcinoma paratiroide (5%). Fenómeno secundario típico de los pacientes con insuficiencia renal crónica. El hiperparatiroidismo puede manifestarse en la cavidad bucal en forma de hiperplasia gingival y lesiones osteolíticas de los maxilares (Tumores pardos) que son asintomáticas. El objetivo de este trabajo es realizar una revisión bibliográfica actualizada sobre esta patología y resaltar la importancia que representan las manifestaciones orales de esta patología sistémica para el odontólogo en general, especialmente para el cirujano oral y maxilofacial.

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.

Bone and renal stone disease in patients operated for primary hyperparathyroidism in Pakistan: is the pattern of disease different from the west?

OBJECTIVE: To document the clinical presentation of primary hyperparathyroidism [PHPT] in a developing country and note differences from the West. SETTING: A tertiary care teaching hospital. METHOD: The records of 37 patients operated for PHPT between January 1986 and December 1997 were reviewed. Symptoms, laboratory parameters and histopathology results were analyzed. Surgery for PHPT accounted for 0.055% of 67,566 operative procedures performed in the Department of Surgery during the 12 year period. The mean age of our patients was 38.4 +/- 13.2 years [range 17 to 73 years]. Ninety% of patients were less than 60 years old and 51% less than 40 years. At presentation, the mean serum parathyroid hormone [sPTH] level was 618 +/- 741% above the upper limit of normal [range 0-2900% using a variety of assays]. A solitary adenoma was present in 86.5%, hyperplasia in 5.4% and carcinoma in 5.4% of patients. There was one [2.7%] negative exploration. Thirty-five% of patients had renal stone disease [StD], 32.4% had bone disease alone [BD] and 27% had both bone abnormality and stones [BStD]. There were neither bone disease nor stones in 5.4% of patients. BD was associated with a statistically non-significantly [p = 0.08] higher alkaline phosphatase level [sALP] as compared to the StD and BStD groups. The mean urinary calcium [Ca] was higher in the BD group [482 +/- 340 mg/24 hours] as compared to StD group [265 +/- 89 mg/24 hours] [p = 0.013]. The post-operative hospital stay was longer in the BD group [14.4 +/- 16 days] as compared to the StD group [6.7 +/- 3.7 days] [p = 0.001]. As compared to reports from the Western world, PHPT is less commonly diagnosed in our country and occurs at a younger age. In the absence of a screening programme, symptomatic disease and bone involvement occur more frequently. The high levels of PTH may indicate long-standing disease in our population, which may account for higher proportion of patients with symptoms. Unexpectedly, patients with bone disease had higher levels of urinary calcium than patients with stone disease

Cuatro casos de cáncer de paratiroides / Four cases of parathyroid carcinoma

El carcinoma de paratiroides es una causa poco frecuente de hiperparatiroidismo primario, con una prevalancia que oscila entre 0.5 y 4 por ciento. Estas neoplasias tienen un curso agresivo por lo que es importante establecer un diagnóstico oportuno y realizar tratamiento quirúrgico precoz. En el Instituto Nacional de la Nutrición en un periodo de siete años, se realizaron 88 cirugías por hiperparatiroidismo primario, encontrándose en cuatro casos un carcinoma de paratiroides (prevalencia del 4.5 por ciento). Presentamos aquí las características clínicas, diagnóstico, tratamiento y evolución de estos pacientes

Surgical management of hyperparathyroidism. A 24 years experience

Our 24 years experience in the surgical treatment of 130 patients with primary hyperparathyroidism was presented. A single adenoma of the parathyroid glands was the predominant lesion. The lower parathyroid glands were more frequently the site of the adenoma, with the right lower gland presenting the highest incidence. The incidence of hyperplasia and carcinoma is similar to the reported literature. The offending glands were identified in the vast majority of the patients. The number of complications is significantly low with a zero surgical mortality. The utilization of preoperative localizing test is not recommended for the initial neck exploration. Satisfactory results can be obtained with a minimal work up

Afecçöes cirurgicas das glandulas paratiroides / Surgical affections of parathyroid glands

O autor faz uma revisao da literatura sobre o hiperparatiroidismo primario, apresenta um caso de carcinoma funcionante da paratiroide inferior direita e descreve a tecnica cirugica

Carcinoma paratiroideo

La incidencia de hiperparatiroidismo causado por carcinoma de paratiroides (CP) ha sido estimada en soló 0.5 a 5 por ciento (1-11). Por esto se ha recomendado informar todos los casos que se detecten con el fin de elaborar un registro internacional que mejore el conocimiento sobre esta rara entidad. En este artículo se presentan dos pacientes con hiperpartiroidismo por CP evaluados por nuestro servicio, y con base en ellos se hace una corta revisión del tema.

Hiperparatiroidismo primario neonatal severo: presentación de un caso y revisión de la literatura / Neonatal severe primary hyperparathyroidism: report of a case and review of the literature

Se presenta el caso de un neonato en quien se diagnostica hiperparatiroidismo primario neonatal severo, basados en la presencia de hipercalcemia severa con hipofosfatemia concomitante y valores elevados de parathormona. Se hace una revisión de la literatura mundial y nacional sobre el tema y se analiza el diagnóstico diferencial de esta rara entidad