Parathyroid adenoma in third pharyngeal pouch cyst as a rare case of primary hyperparathyroidism.

The primitive thymus and inferior parathyroid derive from the third branchial cleft. During embryonic development, these structures descend, reaching their final localisation. Third branchial cleft anomalies present usually as a fistula, abscess or cyst. However, there are no reports on parathyroid adenomas in the literature other than as a morphological possibility. We describe the case of a 47-year-old man, who had been diagnosed with arterial hypertension and who presented with a cervical mass at the edge of the lower third of the sternocleidomastoid muscle. On ultrasonography, the mass had a cystic walled appearance. Laboratory analysis only revealed an intact parathyroid hormone level of 140.5 pg/ml. Sestamibi imaging showed a probable parathyroid adenoma in the anterior mediastinum. During surgery, a tract running from beyond the superior thyroid pedicle to the superior mediastinum was dissected and removed. In the inferior end of the tract, a brown mass was visible. Pathological examination revealed a thymus cyst surrounding a parathyroid adenoma. The primal alteration was the lack of division between the thymus and inferior parathyroid gland, and the prompt prevention of their development. In the case of our patient, a parathyroid adenoma had grown by chance.

Preoperative parathyroid harpoon localisation: a new technique helpful in reoperative patients with persistent hyperparathyroidism.

Surgery for persistent primary hyperparathyroidism remains a major challenge for surgeons and these reoperative procedures require an experienced parathyroid surgeon. The goal of reoperative surgery is to excise the abnormal parathyroid gland(s) and limit exploration to help minimise the potential complications. At least two positive and concordant localising studies should be available before reoperation because the technical difficulties in these cases make an exact localisation necessary before surgery. We describe the placement of a metallic harpoon under ultrasonography guidance as a safe, simple and inexpensive technique for localisation of the enlarged gland prior to conservative surgery.

Castleman's Disease: A Case Report of the Unicentric Type.

Castleman's disease, or angiofollicular lymph node hyperplasia, is a relatively rare disorder characterized by the benign proliferation of lymphoid tissue related to the chronic human herpes virus 8 (HHV-8) infection and the human immunodeficiency virus (HIV). Two clinical entities have been described: a unicentric presentation with the disease confined to a single anatomic lymph node and a multicentric presentation characterized by generalized lymphadenopathy and a more aggressive clinical course. Also, three histopathological subtypes have been described: hyaline-vascular, plasma cell, and a mixed variant. Preoperative diagnosis of hyaline-vascular Castleman's disease is difficult, and the definitive result is based on postoperative pathological findings. The gold standard therapy is the complete surgical excision.

Diagnóstico y tratamiento del insulinoma: nuestra experiencia en 10 casos / Diagnosis and treatment of insulinoma: review of 10 patients

Objetivo Revisión de la presentación clínica, la sensibilidad y la especificidad de las diferentes técnicas de localización utilizadas y de los tratamientos empleados en pacientes con insulinoma. Pacientes y métodos Estudio restrospectivo y descriptivo de pacientes con diagnóstico de insulinoma intervenidos en nuestro centro durante el período de 1992 a 2004. Se evaluaron la edad, el sexo, la clínica, los valores de insulina y de glucosa, los resultados de estudios de localización, la técnica quirúrgica, las características anatomopatológicas y la morbimortalidad. Resultados Se estudió a 10 pacientes, un 60% mujeres, con una edad de 58 ± 14 años. La clínica fue de neuroglucopenia en el 90% y síntomas simpatoadrenérgicos en el 50%. En todos los casos se demostró una relación insulina/glucosa elevada. Las tasas de detección del tumor fueron, para la ecografía transabdominal del 22%, para la tomografía computarizada del 50%, para la resonancia magnética del 33% y para la gammagrafía con octreótido marcado del 25%; con estas técnicas se localizó el 50% de los tumores. La arteriografía con inyección de calcio y la ecografía intraoperatoria identificaron el tumor en todos los casos en los que se realizaron. Dos pacientes tenían insulinomas malignos con metástasis ganglionares. La cirugía fue la enucleación en 5 casos, resección distal del páncreas en 3 y duodenopancreatectomía cefálica en 2. En ningún caso el insulinoma se asoció a neoplasia endocrina múltiple tipo 1. No existió mortalidad postoperatoria. Todos los pacientes estaban asintomáticos, al menos, 6 meses tras la cirugía. Conclusiones La arteriografía con inyección intraarterial de calcio es la prueba de localización preoperatoria más sensible, pero debido a su complejidad debe reservarse para pacientes con insulina sin diagnóstico de localización previo. La alta sensibilidad y el bajo coste de la ecografía intraoperatoria la convierten en una técnica sencilla y muy útil durante el procedimiento quirúrgico, tanto para la localización del tumor como para la valoración de su extensión (AU)

Objective To evaluate clinical features, the sensitivity and specificity of preoperative imaging techniques, and treatment in patients with insulinoma. Patients and methods All patients treated in our institution for surgically proven insulinoma between 1992 and 2004 were retrospectively reviewed. Age, sex, symptoms, insulin and glucose levels, imaging studies, surgical technique, pathological results, morbidity and mortality were analyzed. Results Ten patients with pancreatic insulinomas were included. The mean age was 58 ± 14 years and 60% were women. Clinical findings included neuroglycopenia in 90% and/or sympathoadrenal symptoms in 50%. In all patients, a high insulin/glucose ratio was demonstrated. The detection rates were 22% for transabdominal ultrasonography, 50% for computed tomography (CT), 33% for magnetic resonance imaging and 25% for 111-In-octreotide imaging. Using these techniques, 50% of the tumors were detected. Selective arterial calcium stimulation with hepatic venous sampling and intraoperative ultrasonography identified the tumor in all patients who underwent these techniques. Two patients had malignant insulinomas with nodal metastases. Surgical procedures included enucleation of insulinoma in five patients, partial distal pancreatectomy in three patients and the Whipple procedure in two patients. None of the patients had associated multiple endocrine neoplasia type 1. There was no postoperative mortality. All patients were symptom-free for at least 6 months after surgery. Conclusions Selective arterial calcium stimulation is the most sensitive preoperative test but, due to its complexity, it should be reserved for difficult cases. Because of its high sensitivity and low cost, intraoperative ultrasonography is a very useful intraoperative technique to evaluate localization and/or extension of the insulinoma (AU)

[The use of suprarenal scintigraphy in the differential diagnosis of suprarenal incidentaloma]. / Utilidad de la gammagrafía suprarrenal en el diagnóstico diferencial del incidentaloma suprarrenal.

OBJECTIVES: To assess the usefulness of adrenal scintigraphy for clinical evaluation of adrenal incidentalomas, and its relation with pathological diagnosis and follow-up. PATIENTS AND METHODS: We have studied 46 patients with unilateral adrenal incidentaloma of size between 10 and 100 mm (average 30.5 +/- 19 mm). The lesions were discovered with abdominal computerized tomography in the study of a primary tumor (22%) or in the evaluation of benign pathology (78%). Adrenal scintigraphy assessed uptake in adrenal incidentaloma. Hormonal study included urinary catecholamines, plasma cortisol after dexamethasone, adrenal androgens, and renin and aldosterone in hypertensive patients. Five patients were operated, FNAB was carried out in three patients, and in the rest average follow-up was 29 +/- 21 months. Adrenal incidentaloma was considered nonfunctional if the lesion did not modified its size nor showed analytical alterations along a follow-up higher than 8 months. RESULTS: Of 46 adrenal lesions, seven didn't show uptake (three metastases, one cyst, one adrenal carcinoma, one pheochromocytoma, and one angiomyolipoma), 34 showed excessive uptake (29 nonfunctional adrenal nodules and 5 hyperfunctional adrenal nodules), and five had normal uptake (nonfunctional adrenal nodules). Adrenal scintigraphy was compatible in all cases with cytological study or the response to chemotherapy. Along the follow-up, growth of the lesion was demonstrated in 23%, and reduction or disappearance of the lesion in 11%, with no hormonal significant changes. CONCLUSIONS: Detection of a lesion with no uptake in adrenal scintigraphy forces to carry out complementary explorations in order to rule out malignant pathology. A lesion with excessive uptake is indicative of a benign process and should be assessed with hormonal determinations.

Utilidad de la gammagrafía suprarrenal en el diagnóstico diferencial del incidentaloma suprarrenal / Role of adrenal scintigraphy in the management of adrenal incidentaloma

Objetivos. Valorar la utilidad de la gammagrafía suprarrenal en el estudio de los incidentalomas suprarrenales y su relación con el diagnóstico patológico y el seguimiento posterior. Pacientes y métodos. Hemos estudiado 46 pacientes con incidentaloma suprarrenal unilateral de tamaño entre 10 y 100 mm (media: 30,5 ± 19 mm). Las lesiones se localizaron mediante tomografía computarizada abdominal durante el estudio de un tumor primario (22%) o de patología benigna (78%). La gammagrafía suprarrenal valoró la existencia o no de captación en el incidentaloma suprarrenal. El estudio hormonal incluyó catecolaminas urinarias, cortisol plasmático tras dexametasona, andrógenos adrenales y renina y aldosterona en pacientes hipertensos. Cinco pacientes fueron operados, en tres se realizó punción-aspiración con aguja fina y en los restantes seguimiento medio de 29 ± 21 meses. Se consideró incidentaloma suprarrenal no funcionante si la lesión no modificaba su tamaño ni mostraba alteraciones analíticas en el seguimiento superior a 8 meses. Resultados. De las 46 lesiones suprarrenales 7 fueron hipocaptadoras (tres metástasis, un quiste, un carcinoma suprarrenal, un feocromocitoma y un angiomiolipoma), 34 hipercaptadoras (29 nódulos suprarrenales no funcionantes y 5 hiperfuncionantes) y 5 normocaptadoras (nódulos suprarrenales no funcionantes). La gammagrafía suprarrenal fue concordante con estudio citológico o la respuesta a quimioterapia en todos los casos. Durante el seguimiento se demostró un crecimiento de la lesión en el 23% y reducción o desaparición de las lesiones en el 11% y no se encontraron cambios significativos hormonales.Conclusiones. Una lesión hipocaptadora en gammagrafía suprarrenal hace necesaria la realización de exploraciones complementarias para descartar patología maligna. Una lesión hipercaptadora es indicativa de proceso benigno y debe ser valorada con determinaciones hormonales

Objetives. To assess the usefulness of adrenal scintigraphy for clinical evaluation of adrenal incidentalomas, and its relation with pathological diagnosis and follow-up. Patients and methods. We have studied 46 patients with unilateral adrenal incidentaloma of size between 10 and 100 mm (average 30.5 ± 19 mm). The lesions were discovered with abdominal computerized tomography in the study of a primary tumor (22%) or in the evaluation of benign pathology (78%). Adrenal scintigraphy assessed uptake in adrenal incidentaloma. Hormonal study included urinary catecholamines, plasma cortisol after dexamethasone, adrenal androgens, and renin and aldosterone in hypertensive patients. Five patients were operated, FNAB was carried out in three patients, and in the rest average follow-up was 29 ± 21 months. Adrenal incidentaloma was considered nonfunctional if the lesion did not modified its size nor showed analytical alterations along a follow-up higher than 8 months. Results. Of 46 adrenal lesions, seven didn't show uptake (three metastases, one cyst, one adrenal carcinoma, one pheochromocytoma, and one angiomyolipoma), 34 showed excessive uptake (29 nonfunctional adrenal nodules and 5 hyperfunctional adrenal nodules), and five had normal uptake (nonfunctional adrenal nodules). Adrenal scintigraphy was compatible in all cases with cytological study or the response to chemotherapy. Along the follow-up, growth of the lesion was demonstrated in 23%, and reduction or disappearance of the lesion in 11%, with no hormonal significant changes. Conclusions. Detection of a lesion with no uptake in adrenal scintigraphy forces to carry out complementary explorations in order to rule out malignant pathology. A lesion with excessive uptake is indicative of a benign process and should be assessed with hormonal determinations

Hidatidosis suprarrenal primaria / Primary hydatidosis of the adrenal gland

La afección suprarrenal por la hidatidosis es una entidad muy poco frecuente, sobre todo si es primaria. Presentamos dos casos y comentamos las características clínicas, diagnósticas, epidemiológicas y terapéuticas. El caso número 1 es un varón de 55 años diagnosticado de masa adrenal no funcionante que al examen histológico resultó ser una hidatidosis. El caso número 2 es una mujer de 51 años diagnosticada de quiste hidatídico hepático que en el acto quirúrgico se descubrió que era dependiente de la suprarrenal derecha. Ambos casos evolucionaron favorablemente (AU)

Absceso hepático piogénico como complicación de la enfermedad de Crohn / Pyogenic liver abcess as a complication of Chron´s disease

El absceso hepático piogénico es una complicación infrecuente de la enfermedad de Crohn que presenta una alta mortalidad si el diagnóstico y tratamiento no se realizan de forma temprana. Los abscesos abdominales, fístulas o tratamiento con esteroides son factores predisponentes en la patogenia de esta entidad. Presentamos el caso de un paciente con enfermedad de Crohn y abscesos hepáticos múltiples en el lóbulo hepático derecho sin factores predisponentes claros. El diagnóstico de absceso hepático fue establecido por TAC y el paciente fue tratado con drenaje percutáneo. Es importante considerar esta rara complicación debido a que el diagnóstico es difícil de hacer y se requiere un alto grado de sospecha. Una vez sospechado, hay que realizar un estudio exhaustivo e instituir el tratamiento adecuado. La mayoría de los pacientes con absceso hepático pueden ser manejados con éxito por drenaje percutáneo combinado con tratamiento antibiótico si el diagnóstico se ha realizado antes de que se haya producido necrosis extensa (AU)

Characterization of pathogenic and prognostic factors of nonalcoholic steatohepatitis associated with obesity.

BACKGROUND/AIMS: Nonalcoholic steatohepatitis is an emerging clinical problem among the obese population. However, risk factors of progression to advanced forms of liver disease in this particular group of patients remain to be defined. METHODS: The demographics and clinical and histologic features of 46 obese patients were evaluated. The intrahepatic immunological phenotype was assessed in all liver biopsy samples by immunohistochemistry. RESULTS: Histologic findings of nonalcoholic steatohepatitis were observed in 69.5% of the obese population studied and significant fibrosis was evident in 41% of patients with nonalcoholic steatohepatitis. Age (p=0.003), degree of steatosis (p=0.000002), and grade of inflammation (p=0000) at liver biopsy were independent variables positively associated with fibrosis. Intrahepatic expression levels of several immunologic markers of inflammation as well as nitric oxide derivatives were significantly higher in the severe forms of nonalcoholic steatohepatitis than in the mildest forms. CONCLUSIONS: Obese persons with higher age, with greater degrees of hepatic steatosis, and specially those with increased grades of intrahepatic inflammation have the greatest risk for progression to fibrotic liver disease. An oxidative stress-triggered intrahepatic inflammatory response appears to be important in the pathogenesis of nonalcoholic steatohepatitis in obesity.

Altered regulation of calcium channels and exocytosis in single human pheochromocytoma cells.

We established primary cultures of human pheochromocytoma chromaffin cells. We then tried to find what mechanism of their secretory apparatus could be altered to produce the massive release of catecholamines into the circulation and the subsequent hypertensive crisis observed in patients suffering this type of tumor. Their whole-cell Ca2+ channel currents could be pharmacologically separated into components similar to those found in normal human adrenal chromaffin cells: 20% L-type, 30% N-type, and 50% P/Q-type Ca2+ channels. However, modulation of the channels by exogenous or endogenous ATP and opioids, via a G-protein membrane-delimited pathway, was deeply altered; some cells having no modulation or very little modulation alternated with others having normal modulation. This may be the cause of the uncontrolled secretory response, measured amperometrically at the single-cell level. Some cells secreted for long time periods and were insensitive to nifedipine (L-type channel blocker) or to omega-conotoxin MVIIC (N/P/Q-type channel blocker), while others were highly sensitive to nifedipine and partially sensitive to omega-conotoxin MVIIC. Alteration of the autocrine/paracrine modulation of Ca2+ channels may lead to indiscriminate Ca2+ entry and exacerbate catecholamine release responses in human pheochromocytoma cells.

Ambulatory surgery for groin hernia: the Gilbert repair.

The aim of this study was to evaluate the results of the Gilbert repair for primary treatment of indirect inguinal hernias performed as day cases. From September 1996 to September 1998, 145 patients who were admitted for ambulatory surgery underwent Gilbert tension-free repair for treatment of unilateral inguinal hernia. Sex, age, the American Society of Anaesthesiologists (ASA) preoperative assessment score, type of anaesthesia, operating time, postoperative recovery, postoperative pain, morbidity, mortality, recurrence, return to work and the normal daily activities were assessed. The mean follow-up was 21 months (range 12-36). Gilbert's classification, type 2 and 3 hernias were the most common. Spinal anaesthesia was used in 73% of patients. Mortality was zero. Four patients developed postoperative haematomas, two urinary retention, three seromas, and two wound infections. During the follow-up period, only two recurrences of hernia were noted (1.4%). In conclusion, these data show that Gilbert repair is a safe operation, which is simple to learn. It can be performed on an outpatient basis, with a low complication rate, a low level of pain and a short recovery period. Although it seems to have a low risk of recurrence, a long-term follow-up is needed.

[Pyogenic liver abscess as a complication of Crohn's disease]. / Absceso hepático piogénico como complicación de la enfermedad de Crohn.

Pyogenic liver abscess in patients with Crohn's disease is not common, but the mortality has been reported to be high if diagnosis and treatment is delayed. Intra-abdominal abscesses, fistulous disease, and steroid therapy have all been reported to be important predisposing factors in the pathogenesis of this entity. We present a patient with Crohn's disease in whom multiple abscesses were encountered in the right lobe of the liver. The diagnosis of liver abscess was established by abdominal computed tomography and the patient was treated by percutaneous catheter drainage. Awareness of this rare complication is important because diagnosis is difficult to make and a high index of suspicion is required. Once suspected, aggressive diagnostic workup and treatment is indicated. Most patients with liver abscess can be successfully managed by percutaneous catheter drainage combined with antibiotic therapy if it is diagnosed before extensive necrosis has occurred.

Human adrenal chromaffin cell calcium channels: drastic current facilitation in cell clusters, but not in isolated cells.

Human adrenal medullary chromaffin cells were prepared and cultured from a cystic tumoral adrenal gland whose medullary tissue was unaffected. Adrenaline-containing and noradrenaline-containing cells were identified using a confocal fluorescence microscope and antibodies against dopamine beta-hydroxylase (DBH) and phenylethanolamine N-methyltransferase (PNMT). Current/voltage (I/V) curves performed with the voltage-clamped cells bathed in 10 mM Ba2+ (holding potential, Vh=-80 mV) revealed the presence of only high-threshold voltage-dependent Ca2+ channels; T-type Ca2+ channels were not seen. By using supramaximal concentrations of selective Ca2+ channel blockers, the whole-cell IBa could be fractionated into various subcomponents. Thus, IBa had a 25% fraction sensitive to 1 microM nifedipine (L-type channels), 21% sensitive to 1 microM omega-conotoxin GVIA (N-type channels), and 60% sensitive to 2 microM omega-agatoxin IVA (P/Q-type channels). The activation of IBa was considerably slowed down, and the peak current was inhibited upon superfusion with 10 microM ATP. The slow activation and peak current blockade were reversed by strong depolarizing pre-pulses to +100 mV (facilitation). A drastic facilitation of IBa was also observed in voltage-clamped human chromaffin cell surrounded by other unclamped cells; in contrast, in voltage-clamped cells not immersed in a cell cluster, facilitation was scarce. So, facilitation of Ca2+ channels in a voltage-clamped cell seems to depend upon the exocytotic activity of neighbouring unclamped cells, which is markedly increased by Ba2+. It is concluded that human adrenal chromaffin cells mostly express P/Q-types of voltage-dependent Ca2+ channels (60%). L-Type channels and N-type channels are also expressed, but to a considerably minor extent (around 20% each). This dominance of P/Q-type channels in human chromaffin cells clearly contrasts with the relative proportion of each channel type expressed by chromaffin cells of five other animal species studied previously, where the P/Q-type channels accounted for 5-50%. The results also provide strong support for the hypothesis that Ca2+ channels of human chromaffin cells are regulated in an autocrine/paracrine fashion by materials co-secreted with the catecholamines, i.e. ATP and opiates.

Up-regulated beta1-integrin expression in autoimmune thyroid disorders.

Lymphocytic infiltration of the thyroid gland in autoimmune thyroid disorders requires, as a first step, their attachment to endothelial cells (EC) and, subsequently, interaction with thyrocytes and extracellular matrix proteins. Recent studies have focused on the pathophysiologic role of beta1-integrins as adhesion receptors for extracellular matrix proteins and as cell-to-cell adhesion receptors. In this study, we examine by flow cytometry and immunohistochemical techniques the differences in expression of beta1-integrins in thyrocytes and EC between normal thyroids and thyroid glands from patients with Graves' disease (GD) and Hashimoto's thyroiditis (HT). Remarkably, we found an up-regulated de novo expression of very late antigen (VLA)-alpha6 subunit in thyrocytes in close proximity to lymphocyte infiltrates in GD and HT thyroid glands, with no reactivity in control thyroids. Moreover, interferon-gamma (IFN-gamma), tumour necrosis factor-alpha (TNF-alpha) and IL-1beta produced a significant enhancement of VLA-alpha6 expression in vitro in thyrocytes in culture. In addition, an up-regulated expression of VLA-alpha5 and beta1 subunits was found in thyrocytes from GD and HT glands, specifically in those areas more severely inflamed. VLA-alpha2 was basally expressed in middle size and large vessels in control glands, with an increased expression in vessels of all sizes in HT and GD glands. Dendritic cells in thyroid lymphoid follicles were also positive for VLA-beta1, alpha2 and alpha6 subunits. These results indicate the existence of an up-regulatory process in the expression of beta1-integrins, particularly the alpha6 subunit, in several cell types from inflamed GD and HT thyroid glands, suggesting that these integrins could play a relevant role in localizing and perpetuating the autoimmune response in the thyroid gland in autoimmune thyroid disorders.

Expression of vascular adhesion molecules on human endothelia in autoimmune thyroid disorders.

Cellular activation and expression of certain adhesion molecules within vascular endothelium is a critical event in leucocyte recruitment and emigration. A wide array of different adhesion receptors has been identified to mediate the interaction between endothelial cells (EC) and leucocyte subpopulations. In this study, the tissue expression of E-selectin, P-selectin, CD31, and endoglin endothelial cell adhesion molecules was studied on thyroid tissue from patients with Graves' disease (GD) and Hashimoto's thyroiditis (HT). We found an up-regulated expression of E-selectin in EC in GD and HT thyroids, specifically in those areas more severely inflamed, with no reactivity in control thyroids. P-selectin was basally expressed in postcapillary venules in control glands, with an increased expression in HT and GD glands. On the other hand, increased CD31 expression was found on perifollicular, small and large venule EC from GD and HT glands, that correlated with the severity of mononuclear infiltration. In addition, CD31 expression was observed in some intrathyroidal macrophages and T cells in close proximity to CD31+ EC. Furthermore, a markedly enhanced expression of endoglin, a transforming growth factor-beta binding protein, was mainly located on perifollicular EC and EC from small venules as well as in adjacent macrophages from GD and HT thyroid glands. This enhanced expression of E- and P-selectins, CD31 and endoglin by thyroid EC in GD and HT may reflect their ability to regulate leucocyte trafficking and activation.

[Carcinoma of the esophagus: risk factors of morbimortality following esophagectomy]. / Carcinoma de esófago: factores de riesgo en la morbimortalidad tras esofaguectomía.

OBJECTIVE: To identify which perioperative risk factors are associated to postoperative morbidity and mortality in patients with carcinoma of the esophagus. DESIGN: We analyzed 8 perioperative risk factors: age, preoperative hospital stay, intraoperative blood transfusions, tumor stage, histology, grade of tumor differentiation, prior respiratory disease, surgical treatment. PATIENTS: Forty-six patients with esophageal carcinoma who underwent surgical resection. RESULTS: 1) Morbidity: There was statistical significance between the increase in postoperative morbidity and age > 75 years (p < 0.001), advanced stage of disease (p < 0.001), preoperative hospital stay higher than 20 days (p < 0.05) and severe respiratory disease (p < 0.05). 2) Mortality: There was a statistically significant increase in postoperative mortality in patients with preoperative hospital stay higher than 20 days and in patients > 75 years. CONCLUSIONS: The incidence of postoperative morbidity and mortality in esophageal cancer varies according to age, prior respiratory disease, tumor stage and preoperative hospital stay.