Nonfunctional Metastatic Parathyroid Carcinoma in the Setting of Multiple Endocrine Neoplasia Type 2A Syndrome.

Parathyroid carcinoma is a very rare malignancy. It has been associated with hyperparathyroidism-jaw tumour syndrome, familial isolated primary hyperparathyroidism, and multiple endocrine neoplasia type 1 (MEN-1) and 2A (MEN-2A) syndromes. We report a 54-year-old man with a MEN-2A which presents with a nonfunctional metastatic parathyroid carcinoma and a pheochromocytoma in the absence of medullary thyroid carcinoma. Only a few cases of parathyroid carcinoma have been reported in the literature associated with this syndrome.

Transmisión materno-fetal del bacilo de Koch en una paciente inmunocompetente admitida en una unidad de terapia intensiva con diagnóstico de shock séptico: primer caso demostrado en Uruguay / Mother-to-child transmission of KochÆs bacillus in an immunocompetent patient admitted in the Intensive Care Unit with a diagnosis of septic shock

La tuberculosis es una patología cuya incidencia mundial se ha incrementado sensiblemente en los últimos años.El cuadro clínico de sepsis severa y shock séptico puede serdesencadenado por cualquier microorganismo. Sin embargo, algunos de ellos, como el complejo micobacterium tuberculosum, solo excepcionalmente lo provocan, por lo cual su diagnóstico constituye un verdadero desafío para el equipo sanitario. Se presenta un caso clínico de una mujer de 23 años de edad,inmunocompetente, que cursando una gestación de 28 semanas ingresó a la unidad de terapia intensiva por un cuadro de insuficiencia respiratoria. Se realizo diagnóstico de shock séptico respiratorio bacteriémico debido a complejo Micobacterium tuberculosum y se confirmó la transmisión materno-fetaldel microorganismo. La enferma falleció a las 48 horas de la admisión con hipoxemia extrema y shock refractario.

Tuberculosis is a pathology that has evidenced a significant global increase in the last years. Severe sepsis and septic shock may be caused by anymicro-organism. However, some of them, such as the Micobacterium tuberculosum complex, only result inthese clinical symptoms exceptionally, and thus diagnosis constitutes a challenge for the health team. The study presents the case of a 23 year old immunocompetent female patient who was admitted into the Intensive Care Unit at 28 week of gestation due to a respiratory failure. The patient was diagnosed with bacteremia and septic shock caused by Micobacterium tuberculosis and mother-to-child transmission was confirmed. The patient died 48 hours after admission withhypoxemia and refractory shock.

A tuberculose é uma patologia cuja incidência mundial vem aumentando nos últimos anos. El quadro clínico de sepse severa e choque séptico pode ser desencadeado por qualquer microorganismo. No entanto alguns deles, como o Complexo Micobacteriumtuberculosum, excepcionalmente o provocam e por isso fazer seu diagnóstico é um verdadeiro desafiopara a equipe de saúde. Apresenta-se o caso clínico de uma mujer de 23 anosde idade, imunocompetente na 28ª semana de gravidez ingressou à Unidade de Terapia Intensiva por um quadro de insuficiência respiratória. Realizou-se diagnóstico de choque séptico respiratório bacteriêmico devidoao Complexo Micobacterium tuberculosis e a transmissão materno-fetal do microorganismo foi confirmada. A paciente faleceu 48 horas depois do ingresso com hipoxemia extrema e choque refratário.

Colloid carcinoma of the pancreas: a distinct pancreatic neoplasm with good prognosis. Report of a case.

CONTEXT: Colloid carcinoma is considered a distinct type of pancreatic neoplasia with specific histopathological and molecular features, and a better prognosis. CASE REPORT: We present the case of a patient with a 15 cm locally invasive colloid carcinoma of the pancreas, in which an aggressive surgical approach achieved no evidence of disease 24 months after surgery. CONCLUSIONS: If an accurate diagnostic approach and surgical resection are performed, the 5-year survival rate can reach 60%. Presence of invasive intraductal papillary mucinous neoplasm has been reported, and this can affect the prognosis. Adjuvant therapy has not demonstrated improvement of survival in surgically-resected patients.

Supresión tiroidea en pacientes con hiperparatiroidismo primario: ¿mejora la localización gammagráfica preoperatoria? / Thyroid suppression in patients with primary hyperparathyroidism: does it improve the pre-surgical scintigraphy localisation?

IntroducciónLa gammagrafía con tecnecio-sestamibi (MIBI) es la prueba de elección para la localización de los adenomas en pacientes con hiperparatiroidismo primario (HPTP). En algunos estudios se ha sugerido que el aumento de la sensibilidad de esta prueba podría estar en relación con una disminución de la captación del radiotrazador por parte de la glándula tiroidea. El objetivo de este estudio es analizar nuestra experiencia en pacientes con gammagrafías con MIBI negativas en los que se repitió el estudio tras la supresión de la función tiroidea con tiroxina.Material y métodosEntre enero de 2006 y abril de 2011 se evaluaron de forma prospectiva 17 pacientes con HPTP e imagen gammagráfica con MIBI negativa en los que se repitió dicha prueba tras la administración de tiroxina. Se revisan los datos gammagráficos y la correlación con los hallazgos en la intervención quirúrgica.ResultadosLa media de TSH en el momento de repetir la MIBI fue de 0,12 + 0,1mlU/L. De los 17 pacientes incluidos, en 13 de ellos (76,5%) la gammagrafía bajo supresión de la función tiroidea fue positiva, y en los otros 4 pacientes (23,5%) no se encontró imagen sugestiva de adenoma. En los casos en los que el MIBI fue positivo tras la supresión, el valor predictivo positivo (VPP) fue del 100%.ConclusiónLa supresión de la función tiroidea mediante la administración de tiroxina puede ayudar a mejorar la sensibilidad del MIBI en pacientes con estudios gammagráficos previos negativos y ayudar a tratar de una forma mínimamente invasiva a pacientes con HPTP (AU)

IntroductionScintigraphy with technetium-sestamibi (MIBI) is the test of choice for localising adenomas in patients with primary hyperparathyroidism (PHPT). In some studies it has emerged that the increase in sensitivity of this test could be associated with a decrease in the uptake of the radiotracer by the thyroid gland. The aim of this study is to analyse our experience in patients with a negative scintigraphy with MIBI, and in whom the study was repeated after suppression of thyroid function with thyroxine.Material and methodsA prospective evaluation was performed on 17 patients who, between January 2006 and April 2011, had PHPT and negative imaging using scintigraphy with MIBI and who had the test repeated after the administration of thyroxine. The scintigraphy data and the correlation with the findings in the surgical intervention are reviewed.ResultsThe mean TSH at the time of repeating the MIBI was 0.12+0.1mlU/L. Of the 17 patients included, the scintigraphy under thyroid suppression was positive in 13 of them (76.5%), and in the other 4 (23.5%) patients no image suggestive of adenoma was found. In the cases where the MIBI was positive after suppression, the positive predictive value (PPV) was 100%.ConclusionSuppression of thyroid function by giving thyroxine can help to improve the sensitivity of MIBI in patients with previously negative scintigraphy studies and help in the minimally invasive treatment of patients with PHPT (AU)

[Thyroid suppression in patients with primary hyperparathyroidism: does it improve the pre-surgical scintigraphy localisation?]. / Supresión tiroidea en pacientes con hiperparatiroidismo primario: ¿mejora la localización gammagráfica preoperatoria?

INTRODUCTION: Scintigraphy with technetium-sestamibi (MIBI) is the test of choice for localising adenomas in patients with primary hyperparathyroidism (PHPT). In some studies it has emerged that the increase in sensitivity of this test could be associated with a decrease in the uptake of the radiotracer by the thyroid gland. The aim of this study is to analyse our experience in patients with a negative scintigraphy with MIBI, and in whom the study was repeated after suppression of thyroid function with thyroxine. MATERIAL AND METHODS: A prospective evaluation was performed on 17 patients who, between January 2006 and April 2011, had PHPT and negative imaging using scintigraphy with MIBI and who had the test repeated after the administration of thyroxine. The scintigraphy data and the correlation with the findings in the surgical intervention are reviewed. RESULTS: The mean TSH at the time of repeating the MIBI was 0.12+0.1mlU/L. Of the 17 patients included, the scintigraphy under thyroid suppression was positive in 13 of them (76.5%), and in the other 4 (23.5%) patients no image suggestive of adenoma was found. In the cases where the MIBI was positive after suppression, the positive predictive value (PPV) was 100%. CONCLUSION: Suppression of thyroid function by giving thyroxine can help to improve the sensitivity of MIBI in patients with previously negative scintigraphy studies and help in the minimally invasive treatment of patients with PHPT.

Influencia de la quimioterapia preoperatoria en el parénquima hepático y en la evolución posquirúrgica de pacientes sometidos a hepatectomía por metástasis de carcinoma colorrectal / Influence of pre-surgical chemotherapy on liver parenchyma and post-surgical outcome of patients subjected to hepatectomy due to colorectal carcinoma metastases

Introducción: El objetivo del estudio fue valorar la relación entre la administración preoperatoria de regímenes de quimioterapia basados en irinotecán u oxaliplatino con el desarrollo de enfermedad hepática grasa no alcohólica (EHGNA) o el síndrome de obstrucción sinusoidal (SOS) y la influencia de estas alteraciones histológicas sobre la evolución de los pacientes tras la intervención quirúrgica. Pacientes y método: Estudio prospectivo en el que se incluyeron 45 pacientes sometidos a intervención quirúrgica por metástasis hepáticas de cáncer colorrectal entre mayo de 2005 y julio de 2009. Se recogieron variables demográficas, preoperatorias, de la intervención quirúrgica y de la evolución postoperatoria. Se obtuvo una muestra de la pieza de resección para su análisis histológico siguiendo los parámetros de clasificación de la EHGNA (índice NAS) y del SOS. Resultados: En 22 casos se administró quimioterapia neoadyuvante previa a la resección (grupo de estudio) y 23 pacientes formaban parte del grupo control (no quimioterapia). En 4 de los 7 pacientes (57,2%) en los que se administró de forma preoperatoria irinotecán, se observó esteatohepatitis borderline o diagnóstica (p=0,001). Siete de los 15 pacientes tratados con oxaliplatino (46,7%) desarrollaron un SOS moderado o grave (p=0,002). No hubo diferencias en cuanto a la morbimortalidad en función del grado de EHGNA, pero sí hubo una mayor tasa de complicaciones hepáticas y mayor estancia media en los pacientes con SOS moderado/intenso (p=0,004 y p=0,021 respectivamente). Conclusiones: La administración de irinotecán se relacionó de forma significativa con un aumento en la incidencia de esteatohepatitis, sin que esto aumentara la morbimortalidad. Los pacientes tratados con oxaliplatino tuvieron una mayor incidencia de SOS y existió un aumento de las complicaciones hepáticas y de la estancia media (AU)

Introduction: The aim of the study was to evaluate the relationship between the pre-surgical administration of a chemotherapy regime based on irinotecan or oxaliplatin and the development of non-alcoholic fatty liver disease (NAFLD) or sinusoidal obstruction syndrome (SOS), and the influence of these histological changes on the outcome of patients after surgical intervention. Patients and method: A prospective study which included 45 patients surgically intervened due to colorectal cancer liver metastases between May 2005 and July 2009. Demographic data and the variables before during and after the operation were collected. A specimen of the resection was obtained for histological analysis following the classification parameters of the NAFLD (NASH index) and SOS scale. Results: Neoadjuvant chemotherapy was given before the resection in 22 cases (study group) and 23 patients made up the control group (no chemotherapy). Borderline or diagnostic steatohepatitis was observed in 4 of the 7 patients (57.2%) who were given preoperative irinotecan (P=0.001). Seven of the 15 patients (46.7%) treated with oxaliplatin developed a moderate or severe SOS (P=0.002). There were no differences in morbidity or mortality associated to the NAFLD grade, but there was a higher rate of liver complications and longer mean hospital stay in patients with moderate/severe SOS (P=0.004 and P=0.021, respectively). Conclusions: Treatment with irinotecan was significantly associated with an increase in the incidence of steatohepatitis, but did not increase the morbidity or mortality. Patients treated with oxaliplatin had a higher incidence of SOS, an increase in liver complications and a longer mean hospital stay (AU)

[Influence of pre-surgical chemotherapy on liver parenchyma and post-surgical outcome of patients subjected to hepatectomy due to colorectal carcinoma metastases]. / Influencia de la quimioterapia preoperatoria en el parénquima hepático y en la evolución posquirúrgica de pacientes sometidos a hepatectomía por metástasis de carcinoma colorrectal.

INTRODUCTION: The aim of the study was to evaluate the relationship between the pre-surgical administration of a chemotherapy regime based on irinotecan or oxaliplatin and the development of non-alcoholic fatty liver disease (NAFLD) or sinusoidal obstruction syndrome (SOS), and the influence of these histological changes on the outcome of patients after surgical intervention. PATIENTS AND METHOD: A prospective study which included 45 patients surgically intervened due to colorectal cancer liver metastases between May 2005 and July 2009. Demographic data and the variables before during and after the operation were collected. A specimen of the resection was obtained for histological analysis following the classification parameters of the NAFLD (NASH index) and SOS scale. RESULTS: Neoadjuvant chemotherapy was given before the resection in 22 cases (study group) and 23 patients made up the control group (no chemotherapy). Borderline or diagnostic steatohepatitis was observed in 4 of the 7 patients (57.2%) who were given preoperative irinotecan (P=0.001). Seven of the 15 patients (46.7%) treated with oxaliplatin developed a moderate or severe SOS (P=0.002). There were no differences in morbidity or mortality associated to the NAFLD grade, but there was a higher rate of liver complications and longer mean hospital stay in patients with moderate/severe SOS (P=0.004 and P=0.021, respectively). CONCLUSIONS: Treatment with irinotecan was significantly associated with an increase in the incidence of steatohepatitis, but did not increase the morbidity or mortality. Patients treated with oxaliplatin had a higher incidence of SOS, an increase in liver complications and a longer mean hospital stay.

Metástasis en la glándula tiroidea / Metastasis in the thyroid gland

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Cystic lymphangioma of the lesser omentum in an adult.

Abdominal lymphangiomas are uncommon congenital benign tumors that occur mainly in children. The authors report the case of a 37-year-old woman with a cystic lymphangioma of the lesser omentum. The lesion was removed surgically with a complete resection. The histologic diagnosis was omental lymphangioma. Complete surgical resection with negative surgical margins is the treatment of choice, and the results are excellent. Incomplete resection may lead to recurrence.

[Surgical treatment and localization techniques in primary hyperparathyroidism]. / Tratamiento quirúrgico y técnicas de localización en el hiperparatiroidismo primario.

Parathyroidectomy is the only definitive cure for primary hyperparathyroidism (PHPT). While bilateral neck exploration has been the conventional surgical approach and the mainstay of therapy, recent advances in technology have revolutionized the field, making a focused or minimally invasive approach to parathyroidectomy a reality. This change has taken place because of the development of accurate preoperative localization techniques able to select patients who have single-gland parathyroid disease (single adenoma) and can be managed by a minimally invasive parathyroidectomy. Currently, the most reliable and practical diagnostic procedure is (99m)TC-sestamibi parathyroid scintigraphy, using different protocols depending on the institution's logistics and experience (classical dual-phase, oblique projections, various subtraction techniques and/or single photon-emission computed tomography or SPECT). Ultrasound has emerged as a complementary technique in the preoperative evaluation of PHPT, which can be used when scintigraphy is negative or as a confirmatory test. When these procedures fail to identify the enlarged gland, other non-invasive procedures such as computed tomographic scanning or magnetic resonance imaging are used in selected cases. A variety of surgical techniques have been employed to achieve a safe and effective minimally invasive procedure. These techniques include mini-incision unilateral parathyroid exploration and endoscopic, video-assisted and radio-guided parathyroidectomy. With optimized preoperative mapping, the success rate of these less invasive techniques equals that of the traditional bilateral approach. This review summarizes the imaging techniques and rationale for preoperative localization studies that are used before parathyroidectomy, as well as the current surgical approaches.

Tratamiento quirúrgico y técnicas de localización en el hiperparatiroidismo primario / Surgical treatment and localization techniques in primary hyperparathyroidism

La paratiroidectomía es el único tratamiento definitivo del hiperparatiroidismo primario (HP). Aunque la exploración cervical bilateral ha sido el tratamiento de elección tradicional de esta enfermedad, avances tecnológicos recientes han revolucionado el campo de la paratiroidectomía y han hecho posible una intervención focalizada o mínimamente invasiva. Este cambio se ha debido, sobre todo, al desarrollo de técnicas de localización preoperatoria precisas capaces de seleccionar a pacientes con enfermedad de una sola glándula paratiroidea (adenoma único) y que pueden tratarse mediante paratiroidectomía mínimamente invasiva (PMI). En la actualidad, el test diagnóstico más fiable y práctico es la gammagrafía paratiroidea con tecnecio 99m sestamibi, con el uso de distintos protocolos, dependiendo de la logística y la experiencia del centro (técnica dual clásica, proyecciones oblicuas, técnicas de sustracción, tomografía computarizada o tomografía computarizada por emisión de fotones simples). La ecografía ha surgido como una técnica complementaria, que puede utilizarse tanto cuando la gammagrafía es negativa, como prueba de confirmación. Cuando estos procedimientos fracasan en identificar una glándula aumentada de tamaño, en casos seleccionados pueden emplearse otros procedimientos no invasivos, como la tomografía computarizada o la resonancia magnética. Para realizar un procedimiento mínimamente invasivo, se han utilizado varias técnicas quirúrgicas, incluida una exploración paratiroidea unilateral con incisión mínima, que es la más utilizada, así como también paratiroidectomía endoscópica, videoasistida y radioguiada. Con una localización preoperatoria óptima, las tasas de curación con procedimientos menos invasivos son similares a las de la exploración bilateral. Este trabajo resume las técnicas diagnósticas de localización previas a la patiroidectomía, así como las técnicas quirúrgicas empleadas en la actualidad (AU)

Parathyroidectomy is the only definitive cure for primary hyperparathyroidism (PHPT). While bilateral neck exploration has been the conventional surgical approach and the mainstay of therapy, recent advances in technology have revolutionized the field, making a focused or minimally invasive approach to parathyroidectomy a reality. This change has taken place because of the development of accurate preoperative localization techniques able to select patients who have single-gland parathyroid disease (single adenoma) and can be managed by a minimally invasive parathyroidectomy. Currently, the most reliable and practical diagnostic procedure is 99mTC-sestamibi parathyroid scintigraphy, using different protocols depending on the institution’s logistics and experience (classical dual-phase, oblique projections, various subtraction techniques and/or single photon-emission computed tomography or SPECT). Ultrasound has emerged as a complementary technique in the preoperative evaluation of PHPT, which can be used when scintigraphy is negative or as a confirmatory test. When these procedures fail to identify the enlarged gland, other non-invasive procedures such as computed tomographic scanning or magnetic resonance imaging are used in selected cases. A variety of surgical techniques have been employed to achieve a safe and effective minimally invasive procedure. These techniques include mini-incision unilateral parathyroid exploration and endoscopic, video-assisted and radio-guided parathyroidectomy. With optimized preoperative mapping, the success rate of these less invasive techniques equals that of the traditional bilateral approach. This review summarizes the imaging techniques and rationale for preoperative localization studies that are used before parathyroidectomy, as well as the current surgical approaches (AU)

Expresión de la enzima óxido nítrico sintetasa inducible en las enfermedades tiroideas autoinmunitarias / Inducible nitric oxide synthase (iNOS) expression in autoimmune thyroid disorders (AITD)

Introducción: Diferentes tipos de células producen óxido nítricomediante la acción de la enzima óxido nítrico sintetasa (NOS). Existen cuatro isoformas de la enzima NOS, la neuronal, la endotelial, la mitocondrial y la inducible (iNOS). La producción de NO puede tener un papel importante en la patogenia de la inflamación y el daño de tejidosq ue no ha sido explorado adecuadamente en la enfermedad tiroideaautoinmunitaria. Objetivo: Estudiar la expresión de iNOS, tanto en proteína como en el ARNm, en las enfermedades tiroideas autoinmunitarias. Pacientes y método: Se evaluó la expresión de iNOS en los tiroides de10 pacientes con enfermedad de Graves (EG), 5 con tiroiditis de Hashimoto (TH) y 10 controles sanos, mediante inmunohistoquímica y transcripción inversa-reacción en cadena de la polimerasa (RT-PCR).Resultados: Se encontró un incremento en la expresión de iNOS en lag lándula tiroides de pacientes con EG y con TH tanto por PCR como mediante inmunohistoquímica. Esta expresión fue mayor en pacientes con EG. Por el contrario, en la glándula tiroides de sujetos sanos no se observó expresión de iNOS. La expresión de iNOS, tanto en EG como en TH, se detectó en células foliculares tiroideas, principalmente en las próximas al infiltrado inflamatorio. Asimismo, se observó expresión de iNOS en células endoteliales y células mononucleares del infiltrado inflamatorio en EG y TH. Conclusiones: La expresión prominente de iNOS en la enfermedad tiroidea autoinmunitaria indica que el NO puede tener un papel importante en la patogenia del fenómeno inflamatorio y el daño del tejido que ocurre en esta afección (AU)

Introduction: Nitric oxide is synthesized by different cell types through the action of the enzyme nitric oxide synthase (NOS).There are four is forms of this enzyme: the neuronal, the endothelial, the mitochondrial, and the inducible (iNOS) forms. Although NO production may play an important role in the pathogenesis of inflammation and tissue damage, its possible role in autoimmune thyroiditis has not been adequately explored. Objective: To study protein and mRNA expression of iNOS in human autoimmune thyroid disorders (AITD).Patients and method: We evaluated the expression of iNOS in thyroid glandspecimens from 10 patients with Graves’ disease (GD), from five patients with Hashimoto’s thyroiditis (HT) and from 10controls by immunohistochemical and reverse transcription-polymerase chain reaction (RT-PCR).Results: Both immunohistochemistry and PCR techniques showed up-regulated expression of iNOS in the thyroid glands of patients with GD and HT. iNOS expression was higher in GD than in HT. In contrast,no iNOS expression was detected in normal thyroid tissue. In both GD and HT, iNOS was detected in thyrocytes, mainly int hose localized in areas close to the inflammatory cell infiltrate. In addition, upregulated expression of iNOS was observed in endothelial cells and thyroidinfiltrating mononuclear cells in both GD and HT. Conclusions: The enhanced expression of iNOS in autoimmune thyroiditis suggests that NO synthesis may play an important role in the inflammatory phenomena and tissue damage observed in this disease (AU)

99MTc-sestamibi as sole technique in selection of primary hyperparathyroidism patients for unilateral neck exploration.

BACKGROUND: Unilateral neck exploration (UNE) is becoming the procedure of choice for treatment of primary hyperparathyroidism (PHPT). The aim of this study was to evaluate the role of (99m)Tc-sestamibi (MIBI) parathyroid scintigraphy as the sole technique in selecting patients for UNE. METHOD: We selected 136 consecutive PHPT patients who had only 1 solitary uptake at a MIBI were for UNE. The technique was a single dual-phase using MIBI and a subtraction technique with (99m)Tc-pertechnetate. Imaging data were correlated with surgical results. RESULTS: In 3 cases, the sestamibi scan was falsely positive, 1 had a contralateral location relative to the uptake, and 2 had multiglandular hyperplasia. Overall, the positive predictive value (PPV) of MIBI for detecting a solitary parathyroid adenoma in patients with 1 solitary uptake was 97.8. Sixteen patients (12%) had evidence of multinodular goiter. Overall, the PPV of MIBI was 98.4% (2 false positive among 120 cases) in patients with no multinodular thyroid disease (MNG) and 93.7% (1 false negative among 16 cases) in patients with MNG. The mean duration of the surgical procedure was 34.17 minutes. Mean hospitalization was 0.6 days. Conversion to bilateral neck exploration was performed in 5 patients. After a period of follow-up of 40 months (range, 6-72 months), the cure rate was 98%. CONCLUSION: Patients with PHPT and unequivocally positive preoperative (99m)Tc-sestamibi can safely be managed with UNE without additional localizing techniques.

Bocio amiloide / Amyloid goiter

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