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1.
J Endocrinol Invest ; 46(4): 805-814, 2023 Apr.
Article in English | MEDLINE | ID: mdl-36323983

ABSTRACT

PURPOSE: To compare the intraoperative and surgical outcomes of normotensive pheochromocytomas and sympathetic paragangliomas (PPGLs), hypertensive PPGLs and non-PPGL adrenal lesions. METHODS: This a retrospective multicenter cohort study of patients with PPGLs from 18 tertiary hospitals. A control group of histologically confirmed adrenocortical adenomas (non-PPGL group) was selected to compare intraoperative and surgical outcomes with of the normotensive PPGLs. RESULTS: Two hundred and ninety-six surgeries performed in 289 patients with PPGLs were included. Before surgery, 209 patients were classified as hypertensive PPGLs (70.6%) and 87 as normotensive PPGLs. A higher proportion of normotensive PPGLs than hypertensive PPGLs did not receive alpha presurgical blockade (P = 0.009). When we only considered those patients who received presurgical alpha blockers (200 hypertensive PPGLs and 76 normotensive PPGLs), hypertensive PPGLs had a threefold higher risk of intraoperative hypertensive crisis (OR 3.0 [95% 1.3-7.0]) and of hypotensive episodes (OR 2.9 [95% CI 1.2-6.7]) than normotensive PPGLs. When we compared normotensive PPGLs (n = 76) and non-PPGLs (n = 58), normotensive PPGLs had a fivefold higher risk of intraoperative complications (OR 5.3 [95% CI 1.9-14.9]) and a six times higher risk of postoperative complications (OR 6.1 [95% CI 1.7-21.6]) than non-PPGLs. CONCLUSION: Although the risk of intraoperative hypertensive and hypotensive episodes in normotensive PPGLs is significantly lower than in hypertensive PPGLs, normotensive PPGLs have a greater risk of intraoperative and postoperative complications than non-PPGL adrenal lesions. Therefore, it is recommended to follow the standard of care for presurgical and anesthetic management of PPGLs also in normotensive PPGLs.


Subject(s)
Adrenal Gland Neoplasms , Hypertension , Paraganglioma , Pheochromocytoma , Humans , Pheochromocytoma/surgery , Pheochromocytoma/pathology , Cohort Studies , Paraganglioma/surgery , Paraganglioma/pathology , Hypertension/epidemiology , Adrenal Gland Neoplasms/surgery , Adrenal Gland Neoplasms/pathology , Treatment Outcome
2.
Clin. transl. oncol. (Print) ; 23(10): 1995-2019, oct. 2021. ilus, tab
Article in English | IBECS | ID: ibc-223371

ABSTRACT

'Pheochromocytomas and paragangliomas (PPGLs) are rare neuroendocrine tumors that arise from chromaffin cells of the adrenal medulla and the sympathetic/parasympathetic neural ganglia, respectively. The heterogeneity in its etiology makes PPGL diagnosis and treatment very complex. The aim of this article was to provide practical clinical guidelines for the diagnosis and treatment of PPGLs from a multidisciplinary perspective, with the involvement of the Spanish Societies of Endocrinology and Nutrition (SEEN), Medical Oncology (SEOM), Medical Radiology (SERAM), Nuclear Medicine and Molecular Imaging (SEMNIM), Otorhinolaryngology (SEORL), Pathology (SEAP), Radiation Oncology (SEOR), Surgery (AEC) and the Spanish National Cancer Research Center (CNIO). We will review the following topics: epidemiology; anatomy, pathology and molecular pathways; clinical presentation; hereditary predisposition syndromes and genetic counseling and testing; diagnostic procedures, including biochemical testing and imaging studies; treatment including catecholamine blockade, surgery, radiotherapy and radiometabolic therapy, systemic therapy, local ablative therapy and supportive care. Finally, we will provide follow-up recommendations (AU)


Subject(s)
Humans , Genetic Counseling/methods , Pheochromocytoma/therapy , Paraganglioma/therapy , Biomarkers, Tumor , Genetic Predisposition to Disease , Study Guides as Topic , Genetic Testing
3.
Clin Transl Oncol ; 23(10): 1995-2019, 2021 Oct.
Article in English | MEDLINE | ID: mdl-33959901

ABSTRACT

Pheochromocytomas and paragangliomas (PPGLs) are rare neuroendocrine tumors that arise from chromaffin cells of the adrenal medulla and the sympathetic/parasympathetic neural ganglia, respectively. The heterogeneity in its etiology makes PPGL diagnosis and treatment very complex. The aim of this article was to provide practical clinical guidelines for the diagnosis and treatment of PPGLs from a multidisciplinary perspective, with the involvement of the Spanish Societies of Endocrinology and Nutrition (SEEN), Medical Oncology (SEOM), Medical Radiology (SERAM), Nuclear Medicine and Molecular Imaging (SEMNIM), Otorhinolaryngology (SEORL), Pathology (SEAP), Radiation Oncology (SEOR), Surgery (AEC) and the Spanish National Cancer Research Center (CNIO). We will review the following topics: epidemiology; anatomy, pathology and molecular pathways; clinical presentation; hereditary predisposition syndromes and genetic counseling and testing; diagnostic procedures, including biochemical testing and imaging studies; treatment including catecholamine blockade, surgery, radiotherapy and radiometabolic therapy, systemic therapy, local ablative therapy and supportive care. Finally, we will provide follow-up recommendations.


Subject(s)
Adrenal Gland Neoplasms/diagnosis , Adrenal Gland Neoplasms/therapy , Paraganglioma/diagnosis , Paraganglioma/therapy , Pheochromocytoma/diagnosis , Pheochromocytoma/therapy , Adrenal Gland Neoplasms/genetics , Adrenal Gland Neoplasms/pathology , Aftercare , Algorithms , Biomarkers, Tumor/blood , Biomarkers, Tumor/urine , Catecholamines/antagonists & inhibitors , Diagnostic Imaging/methods , Genetic Counseling , Genetic Predisposition to Disease , Genetic Testing , Humans , Neoplasm Staging , Paraganglioma/genetics , Paraganglioma/pathology , Pheochromocytoma/genetics , Pheochromocytoma/pathology , Societies, Medical , Spain/epidemiology , Symptom Assessment/methods
4.
Endocrine ; 73(2): 416-423, 2021 08.
Article in English | MEDLINE | ID: mdl-33880732

ABSTRACT

PURPOSE: To investigate endocrine function changes after non-functioning pituitary adenomas (NFPA) transsphenoidal surgery and to search for predictors of hypopituitarism resolution and development. METHODS: We included 117 patients with NFPA who underwent endoscopic transsphenoidal surgery from 2005 to 2019 by two neurosurgeons. Twenty-one patients were excluded because of previous pituitary surgery or radiotherapy. We assessed symptoms at diagnosis, tumour volume, tumour removal, hormonal status at diagnosis, hormonal outcomes at 2- and 12-month follow-up, and complications. Pituitary stalk and gland MRI status (visible or not) were included, and it theirs association to hormonal function was studied for the first time, to our knowledge. RESULTS: Pituitary gland visualization was more frequent in those patients who showed a smaller number of axes affected at 12 months (p = 0.011). Pituitary stalk status showed no association to hormonal function. The hormonal normalization rate at 12 months was 13%. The endocrine improvement rate at 12 months was 16.7%. Worsening of hormonal function occurred in 19.8% of patients. Younger age was associated to hormonal improvement (p = 0.004). Higher preoperative tumour volume (p = 0.015) and absence of gross total resection (GTR) (p = 0.049) were associated with worsening in at least one hormonal axis after surgery. CONCLUSIONS: Pituitary gland visibility was higher in those patients who showed better hormonal outcomes. Assessment of initial hormonal function and outcome after surgery regarding pituitary stalk status showed no significant association. Higher preoperative tumour volumes and absence of GTR were associated to postoperative endocrine function worsening, while younger age was associated to its improvement.


Subject(s)
Adenoma , Hypopituitarism , Pituitary Neoplasms , Adenoma/diagnostic imaging , Adenoma/surgery , Humans , Hypopituitarism/diagnostic imaging , Hypopituitarism/etiology , Pituitary Gland/diagnostic imaging , Pituitary Gland/surgery , Pituitary Neoplasms/diagnostic imaging , Pituitary Neoplasms/surgery , Retrospective Studies , Treatment Outcome
5.
Rev Neurol ; 71(5): 163-170, 2020 Sep 01.
Article in Spanish | MEDLINE | ID: mdl-32729107

ABSTRACT

INTRODUCTION: Non-functioning pituitary adenomas are the most frequent tumor group in the sellar region. They are usually benign neoplasms diagnosed after visual or hormonal symptoms, although it is not uncommon to detect them as a casual finding. AIM: To analyze the clinical aspects found in this disease and its response after surgical treatment. PATIENTS AND METHODS: In a series of 100 cases, epidemiological, clinical, endocrinological, visual and radiological data were analyzed before and after surgical treatment, as well as the complications related to surgery and long-term follow-up. RESULTS: The most frequent symptom at the time of diagnosis was visual field involvement (62%), and only the 7% of adenomas were a casual finding. The most common hormonal deficit was hypogonadotropic hypogonadism (48%). After surgery, complete recovery of the visual field defect was observed in 54.8% of the patients, only 1% worsening after surgery, and the incidence of diabetes insipidus was 4%. The resection was superior to 95% in 63% of cases, although the percentage of adenomas with invasion of the cavernous sinus in Knosp grades 3 and 4 it was high (45%). CONCLUSIONS: Although the most frequent symptom of non-functioning pituitary adenomas is campimetric involvement, it has an excellent response to surgery if it is performed within the appropriate time. The grade of invasion of the cavernous sinus is the most limiting factor for a complete surgical resection.


TITLE: Adenomas hipofisarios no funcionantes: epidemiología, clínica y evolución posquirúrgica.Introducción. Los adenomas hipofisarios no funcionantes son el grupo tumoral más frecuente en la región selar. Suelen ser neoplasias benignas diagnosticadas por síntomas visuales u hormonales, aunque no es infrecuente detectarlos como un hallazgo casual. Objetivo. Analizar los aspectos clínicos hallados en esta enfermedad y su respuesta tras el tratamiento quirúrgico. Pacientes y métodos. En una serie de 100 casos, se analizaron datos epidemiológicos, clínicos, endocrinológicos, visuales y radiológicos antes y después del tratamiento quirúrgico, y se recogen las complicaciones relacionadas con la cirugía y el seguimiento a largo plazo. Resultados. El síntoma más frecuente en el momento del diagnóstico fue la afectación del campo visual (62%), y sólo el 7% de los adenomas se trataba de un hallazgo casual. El déficit hormonal más frecuente era el hipogonadismo hipogonadótropo (48%). Tras la cirugía se observó recuperación completa del defecto campimétrico en el 54,8% de los pacientes, con sólo un 1% de empeoramiento tras la cirugía, y la incidencia de diabetes insípida fue del 4%. La resección fue superior al 95% en el 63% de los casos, a pesar de que el porcentaje de adenomas con invasión del seno cavernoso en grados altos fue elevado (45%). Conclusiones. Aunque el síntoma más frecuente de los adenomas hipofisarios no funcionantes es la afectación campimétrica, ésta tiene una excelente respuesta a la cirugía si se realiza dentro del tiempo adecuado. El grado de invasión del seno cavernoso parece el factor más limitante para una resección quirúrgica completa.


Subject(s)
Pituitary Neoplasms , Aged , Female , Humans , Male , Middle Aged , Pituitary Neoplasms/diagnosis , Pituitary Neoplasms/epidemiology , Pituitary Neoplasms/surgery , Prospective Studies , Retrospective Studies , Treatment Outcome
7.
Pituitary ; 19(2): 127-37, 2016 Apr.
Article in English | MEDLINE | ID: mdl-26553421

ABSTRACT

PURPOSE: To evaluate the long-term safety of Pegvisomant (PEG) in the Spanish cohort of ACROSTUDY. METHODS: As of July 2013, 199 Spanish patients were included in ACROSTUDY, a global non interventional safety PEG surveillance study. Patients were observed for safety, biochemical outcome and magnetic resonance imaging evaluations. RESULTS: PEG was administered during an average period of 6.7 ± 2.1 years and a mean daily dose of 15.5 ± 7.5 mg. 48.2% of patients received PEG monotherapy. 90.9% of patients had received other medical treatment before PEG start. 195 adverse events (AEs) were reported in 88 patients (44.2%), and serious AEs were described in 31 patients (15.6%). There were no cases of liver tests >10 ULN, or permanent liver damage. Tumor size changes were locally reported in 61 cases (33.5%), with increases observed in 11 patients (6%). In acromegalic patients with diabetes mellitus a decrease in fasting serum glucose value was reported, reaching statistical significance after 1 and 4 years of treatment (-24.6 and -25.9 mg/dl, p = 0.04). After 60 months, normal or lower limit of normal (LLN) IGF-I levels were found in 67.9% of patients. 85.5% of patients showed an IGF-I normal or

Subject(s)
Acromegaly/drug therapy , Hormone Antagonists/adverse effects , Human Growth Hormone/analogs & derivatives , Adult , Drug-Related Side Effects and Adverse Reactions/epidemiology , Female , Follow-Up Studies , Hormone Antagonists/therapeutic use , Human Growth Hormone/adverse effects , Human Growth Hormone/therapeutic use , Humans , Magnetic Resonance Imaging , Male , Middle Aged , Product Surveillance, Postmarketing , Registries , Spain/epidemiology , Time Factors
8.
Br J Dermatol ; 174(6): 1370-1374, 2016 Jun.
Article in English | MEDLINE | ID: mdl-26694762

ABSTRACT

Kaposi sarcoma (KS) is an angioproliferative tumour that develops as a result of an infection by human herpesvirus 8, which is considered a necessary cause but not sufficient. Other factors - genetic, immunological and environmental - might play a role in the development of the disease. We report a case of KS secondary to endogenous Cushing syndrome (ECS) due to a pituitary adenoma, an association that has been reported only once. We also conducted a search through the Medline and PubMed databases for cases involving KS and ECS, finding only three additional cases that shared common clinical and prognostic features with ours. ECS might favour the development of KS due to immunosuppression. Dermatologists and other clinicians should be aware of this association, as it might be an underdiagnosed condition. It also has an important impact on the management of KS, and based on this review it relies on a good prognosis when ECS is well controlled.

9.
Pituitary ; 16(1): 101-8, 2013 Mar.
Article in English | MEDLINE | ID: mdl-22396133

ABSTRACT

Combination with cabergoline may offer additional benefits to acromegalic patients on pegvisomant monotherapy. We evaluated the safety and efficacy profile of this combination and investigated the determinants of response. An observational, retrospective, cross-sectional study. Fourteen acromegalic patients (9 females), who were partially resistant to somatostatin analogs and on pegvisomant monotherapy. Cabergoline was added because of the presence of persistent mildly increased IGF-I. The mean follow-up time was 18.3 ± 10.4 months. The efficacy and safety profile was assessed. The influence of clinical and biochemical characteristics on treatment efficacy was studied. IGF-I levels returned to normal in 4 patients (28%) at the end of the study. In addition, some decline in IGF-I levels was observed in a further 5 patients. The % IGF-I decreased from 158 ± 64% to 124 ± 44% (p = 0.001). The average change in IGF-I was -18 ± 27% (range -67 to +24%). Lower baseline IGF-I (p = 0.007), female gender (p = 0.013), lower body weight (p = 0.031), and higher prolactin (PRL) levels (p = 0.007) were associated with a better response to combination therapy. There were no significant severe adverse events. Significant tumour shrinkage was observed in 1 patient. Combination therapy with pegvisomant and cabergoline could provide better control of IGF-I in some patients with acromegaly. Baseline IGF-I levels, female gender, body weight, and PRL levels affect the response to this combination therapy.


Subject(s)
Acromegaly/drug therapy , Ergolines/therapeutic use , Human Growth Hormone/analogs & derivatives , Adult , Cabergoline , Drug Therapy, Combination , Female , Human Growth Hormone/therapeutic use , Humans , Male , Middle Aged , Retrospective Studies
11.
J Clin Endocrinol Metab ; 96(2): E251-9, 2011 Feb.
Article in English | MEDLINE | ID: mdl-21068147

ABSTRACT

CONTEXT: There is concern that pegvisomant could be associated with a higher risk of tumor growth. The rate and possible determinants of this tumor growth are unknown. OBJECTIVE: The objective of the study was to investigate the clinical, immunohistological, and molecular factors conditioning tumor growth in patients taking pegvisomant. DESIGN AND SETTING: This was a cross-sectional study performed from 2004 to 2010 in four university hospitals in Spain. PATIENTS: Seventy-five acromegalic patients with active disease resistant to somatostatin analogs treated with pegvisomant were followed up for a mean of 29 ± 20 months. MAIN OUTCOME MEASURES: Magnetic resonance images before initiation of pegvisomant, at 6 months, and then yearly were examined in all patients. Immunohistological and molecular studies were performed in tumors that grew. RESULTS: A significant increase in tumor size was observed in five patients (6.7%). Absence of previous irradiation (P = 0.014) and shorter duration of prepegvisomant somatostatin analog therapy (P < 0.001) were associated with an increased risk of tumor growth. A stepwise multivariate linear regression analysis (R(2) = 0.334, P < 0.001) identified the duration of somatostatin analog therapy prior to pegvisomant (beta = -4.509, P = 0.014) as the only significant predictor of tumor growth. In those tumors that grew, GH expression and insulin receptor expression were higher (P = 0.033 in both cases) than in the control group. CONCLUSIONS: No previous radiotherapy, shorter duration of prepegvisomant somatostatin analog therapy, and higher tumor expression of GH and insulin receptor could be risk factors for tumor growth during pegvisomant therapy.


Subject(s)
Adenoma/drug therapy , Growth Hormone-Secreting Pituitary Adenoma/drug therapy , Human Growth Hormone/analogs & derivatives , Receptors, Somatotropin/antagonists & inhibitors , Acromegaly/diagnostic imaging , Acromegaly/drug therapy , Acromegaly/etiology , Adenoma/genetics , Adenoma/pathology , Adolescent , Adult , Aged , Aged, 80 and over , Combined Modality Therapy , Cross-Sectional Studies , Disease Progression , Female , Growth Hormone-Secreting Pituitary Adenoma/genetics , Growth Hormone-Secreting Pituitary Adenoma/pathology , Human Growth Hormone/metabolism , Human Growth Hormone/therapeutic use , Humans , Immunohistochemistry , Magnetic Resonance Imaging , Male , Middle Aged , Paraffin Embedding , Pituitary Gland/pathology , Pituitary Gland/surgery , Radiography , Regression Analysis , Reverse Transcriptase Polymerase Chain Reaction , Risk Factors , Young Adult
12.
Actas Urol Esp ; 34(2): 181-5, 2010 Feb.
Article in Spanish | MEDLINE | ID: mdl-20403283

ABSTRACT

OBJECTIVE: Adrenal conditions requiring surgery are uncommon and are usually seen in several surgical departments. Our experience in laparoscopic management of adrenal pathology after almost five years of use of laparoscopy for retroperitoneal conditions at our center is reported. MATERIALS AND METHODS: A total of 37 laparoscopic adrenalectomies were performed over 53 months for benign and malignant conditions. The transperitoneal approach was used in most cases (97%) because of the greater surgeon experience with this route. Pregnancy and suspected periadrenal infiltration were considered as absolute contraindications. RESULTS: Mean operating time was 90 minutes, mean intraoperative bleeding 80 ml, and mean hospital stay was 2 days. The main complication was one death. A malignancy was found in 4 patients (10%), while all other patients (90%) had a benign condition, including 8 pheochromocytomas. CONCLUSIONS: Laparoscopy is considered to be the gold standard for benign adrenal conditions. When the malignant mass is a single metastasis from a primary tumor, the laparoscopic approach appears to be reliable. When the malignant lesion is a primary adrenal tumor, laparoscopic management is more controversial, although the results reported by experienced surgeons in their series appear to be adequate.


Subject(s)
Adrenalectomy/methods , Laparoscopy/methods , Adenoma/surgery , Adrenal Gland Diseases/surgery , Adrenal Gland Neoplasms/secondary , Adrenal Gland Neoplasms/surgery , Adrenalectomy/statistics & numerical data , Adult , Aged , Carcinoma/secondary , Carcinoma/surgery , Contraindications , Cushing Syndrome/surgery , Cysts/surgery , Female , Humans , Hyperaldosteronism/surgery , Laparoscopy/statistics & numerical data , Male , Middle Aged , Myelolipoma/surgery , Pheochromocytoma/surgery , Pregnancy , Pregnancy Complications/surgery , Retrospective Studies
13.
Ann Oncol ; 21(9): 1794-1803, 2010 Sep.
Article in English | MEDLINE | ID: mdl-20139156

ABSTRACT

BACKGROUND: Neuroendocrine tumors (NETs) are an unusual family of neoplasms with a wide and complex spectrum of clinical behavior. Here, we present the first report of a National Cancer Registry of gastroenteropancreatic neuroendocrine tumors from a Southern European country. PATIENTS AND METHODS: Data was provided online at www.retegep.net by participating centers and assessed for internal consistency by external independent reviewers. RESULTS: The study cohort comprised 907 tumors. The most common tumor types were carcinoids (55%), pancreatic nonfunctional tumors (20%), metastatic NETs of unknown primary (9%), insulinomas (8%) and gastrinomas (4%). Forty-four percent presented with distant disease at diagnosis, most often those from small intestine (65%), colon (48%), rectum (40%) and pancreas (38%), being most unusual in appendix primaries (1.3%). Stage at diagnosis varied significantly according to sex, localization of primary tumor, tumor type and grade. Overall 5-year survival was 75.4% (95% confidence interval 71.3% to 79.5%) and was significantly greater in women, younger patients and patients with hormonal syndrome and early stage or lower grade tumors. Prognosis also differed according to tumor type and primary tumor site. However, stage and Ki-67 index were the only independent predictors for survival. CONCLUSION: This national database reveals relevant information regarding epidemiology, current clinical practices and prognosis of NETs in Spain, providing valuable insights that may contribute to understand regional disparities in the incidence, patterns of care and survival of this heterogeneous disease across different continents and countries.


Subject(s)
Delivery of Health Care/standards , Gastrointestinal Neoplasms/diagnosis , Neuroendocrine Tumors/diagnosis , Pancreatic Neoplasms/diagnosis , Adolescent , Adult , Aged , Aged, 80 and over , Child , Female , Gastrointestinal Neoplasms/epidemiology , Gastrointestinal Neoplasms/therapy , Humans , Incidence , Middle Aged , Neoplasm Metastasis , Neoplasm Staging , Neuroendocrine Tumors/epidemiology , Neuroendocrine Tumors/therapy , Pancreatic Neoplasms/epidemiology , Pancreatic Neoplasms/therapy , Prognosis , Registries , Research Report , Spain/epidemiology , Survival Rate , Young Adult
14.
Actas urol. esp ; 34(2): 181-185, feb. 2010. graf, tab, ilus
Article in Spanish | IBECS | ID: ibc-85782

ABSTRACT

Objetivo: la patología adrenal susceptible de indicación quirúrgica es poco frecuente y normalmente se encuentra dividida entre varios servicios quirúrgicos. Presentamos nuestra experiencia en el manejo laparoscópico tras casi 5 años de implantación de la laparoscopia en la patología retroperitoneal en nuestro centro. Material y métodos: en 53 meses se han realizado un total de 37 suprarrenalectomías por patología benigna y maligna. El abordaje más frecuentemente empleado es el transperitoneal (97%) debido a la mayor experiencia del cirujano con esta vía. La paciente embarazada y la sospecha de infiltración periadrenal se han tomado como contraindicaciones absolutas. Resultados: el tiempo quirúrgico medio es de 90 minutos, sangrado intraoperatorio de 80 cc, estancia media postoperatoria de dos días y la principal complicación ha sido un exitus. En4 ocasiones la patología es maligna (10%), el resto (90%) benigna, con 8 feocromocitomas. Conclusiones: la laparoscopia se considera el patrón oro para la patología benigna adrenal. Cuando la lesión es de naturaleza maligna, en caso de ser una metástasis única procedente de otro tumor primario, parece que el abordaje laparoscópico es fiable. Cuando la lesión maligna es primaria adrenal existe más controversia en este tipo de abordaje, si bien es cierto que en series de cirujanos experimentados los resultados parecen adecuados (AU)


Objective: Adrenal conditions requiring surgery are uncommon and are usually seen in several surgical departments. Our experience in laparoscopic management of adrenal pathology after almost five years of use of laparoscopy for retroperitoneal conditions a tour center is reported. Materials and methods: A total of 37 laparoscopic adrenalectomies were performed over 53months for benign and malignant conditions. The transperitoneal approach was used in most cases (97%) because of the greater surgeon experience with this route. Pregnancy and suspected periadrenal infiltration were considered as absolute contraindications. Results: Mean operating time was 90 minutes, mean intraoperative bleeding 80 ml, and mean hospital stay was 2 days. The main complication was one death. A malignancy was found in 4 patients (10%), while all other patients (90%) had a benign condition, including8 pheochromocytomas. Conclusions: Laparoscopy is considered to be the gold standard for benign adrenal conditions. When the malignant mass is a single metastasis from a primary tumor, the laparoscopic approach appears to be reliable. When the malignant lesion is a primary adrenal tumor, laparoscopic management is more controversial, although the results reported by experienced surgeons in their series appear to be adequate (AU)


Subject(s)
Humans , Male , Female , Aged , Adrenalectomy , Adrenalectomy/methods , Laparoscopy/methods , Pheochromocytoma/pathology , Pheochromocytoma/surgery , Postoperative Complications/mortality , Blood Loss, Surgical/statistics & numerical data , Referral and Consultation/statistics & numerical data , Cushing Syndrome/therapy
15.
Rev Port Cardiol ; 29(12): 1873-7, 2010 Dec.
Article in English | MEDLINE | ID: mdl-21428142

ABSTRACT

Surgical treatment of pheochromocytoma is associated with high hemodynamic risk, which is even higher in patients with complex congenital heart disease. Nowadays, patients with cyanotic congenital heart disease are living longer and an increased incidence of pheochromocytoma has been reported in this population. We demonstrate the feasibility and importance of minimally invasive surgery in the management of pheochromocytoma in a 45-year-old woman with complex congenital heart disease and Eisenmenger's syndrome. A successful laparoscopic resection of the tumor was performed in association with multidisciplinary management during hospitalization.


Subject(s)
Adrenal Gland Neoplasms/complications , Eisenmenger Complex/complications , Pheochromocytoma/complications , Adrenal Gland Neoplasms/surgery , Eisenmenger Complex/surgery , Female , Humans , Middle Aged , Pheochromocytoma/surgery
16.
Horm Metab Res ; 41(9): 672-5, 2009 Sep.
Article in English | MEDLINE | ID: mdl-19343621

ABSTRACT

Hereditary susceptibility to pheochromocytoma (PCC) and paraganglioma (PGL) represents a very complex genetic scenario. It has been reported that the absence of familial antecedents of the disease does not preclude the existence of a mutation affecting any of the five major susceptibility genes. In fact, 11-24% of apparently sporadic cases (without familial or syndromic antecedents) harbor an unexpected germline mutation, but we do not know what is happening in "truly apparently" sporadic patients (i.e., apparently sporadic cases diagnosed with only one tumor). In the present study, we have analyzed 135 apparently sporadic patients developing a single tumor for the five major susceptibility genes: VHL, RET, SDHB, SDHC, and SDHD. Fourteen percent of cases were found to harbor a germline mutation, and only 2.2% of patients were older than 45 years at onset. By taking into account the tumor location and a threshold age at onset of 45 years, we propose a rational scheme for genetic testing. Analyzing VHL and RET genes would be recommended only in young patients developing a single PCC. On the other hand, genetic testing of SDHD should be done in all patients developing an extra-adrenal tumor before the age of 45, and SDHC could be the responsible gene in cases developing a single head and neck tumor, independently of age. Finally, the analysis of SDHB should always be performed because of its association to malignancy and the low penetrance of mutations affecting this gene.


Subject(s)
Adrenal Gland Neoplasms/genetics , Genetic Testing , Paraganglioma/genetics , Pheochromocytoma/genetics , Adolescent , Adrenal Gland Neoplasms/diagnosis , Adult , Aged , Female , Germ-Line Mutation , Humans , Male , Middle Aged , Paraganglioma/diagnosis , Pheochromocytoma/diagnosis , Proto-Oncogene Proteins c-ret/genetics , Succinate Dehydrogenase/genetics , Von Hippel-Lindau Tumor Suppressor Protein/genetics , Young Adult
17.
Av. diabetol ; 22(3): 223-227, jul.-sept. 2006. ilus, tab
Article in Es | IBECS | ID: ibc-050117

ABSTRACT

Introducción: El riesgo cardiovascular del individuo diabético se duplica en pacientes fumadores respecto a los no fumadores. Además, el tabaquismo aumenta el riesgo de aparición y deterioro de complicaciones microvasculares. El objetivo del estudio fue conocer la prevalencia del tabaquismo y algunas características del hábito de fumar en la población diabética de nuestro medio. Pacientes y métodos: Se incluyeron en el estudio 806 individuos diabéticos. Los pacientes se clasificaron en: diabetes tipo 1 (n= 70), tipo 2 (n= 674), gestacional (n= 36) y otras diabetes (n= 26). Además, se consideró el lugar de residencia de los pacientes (urbano/rural). En los pacientes que fumaban, se determinó el grado de dependencia al tabaco mediante el test de Fagerström. Resultados: Ciento diez pacientes (13,6%) fumaban, mientras que los 696 restantes (86,4%) no fumaban. Se encontraron diferencias significativas en el porcentaje de fumadores en el grupo de diabetes tipo 1 (38,6%) respecto a los grupos de tipo 2 (10,5%) y gestacional (11,1%). También se encontraron diferencias en el porcentaje de varones fumadores (24,1%) respecto al de mujeres fumadoras (6,8%), así como en el de fumadores que residían en área urbana (18,1%) respecto a los que vivían en el medio rural (11,6%). En el grupo de fumadores, un 33,3% tenía una alta dependencia del tabaco, mientras que el 48,5% presentaba una dependencia moderada y el 18,2% restante una baja dependencia. Conclusiones: Estos resultados demuestran que un porcentaje considerable de individuos diabéticos fuma, en especial en los grupos de diabetes mellitus tipo 1 y otras diabetes. El sexo masculino, junto con la residencia en área urbana, constituyen factores que predisponen al hábito de fumar. El hecho de que sólo uno de cada tres diabéticos fumadores tenga una alta dependencia a la nicotina sugiere que los programas encaminados a la deshabituación tabáquica podrían tener un notable éxito


Introduction: Cardiovascular risk in individuals with diabetes is two-fold higher among smokers as compared to non-smokers. Moreover, smoking increases the risk of the development and deterioration of microvascular complications. The aim of the present study was to determine the prevalence of cigarette smoking and several characteristics of the smoking habit among diabetics in our patient population. Patients and methods: Eight hundred and six diabetic subjects were included in the study. The patients were classified as: type 1 (n= 70), type 2 (n= 674), gestational (n= 36) and other variants (n= 26). Moreover, the place of residence of the patients (urbal/rural) was considered. In the group of smokers, the degree of nicotine dependence was determined by means of the Fagerström test. Results: One hundred and ten patients (13.6%) were smokers, while the remaining 696 (86.4%) were non-smokers. Significant differences were observed in the percentage of smokers among the patients with type 1 diabetes (38.6%) with respect to those with type 2 (10.5%) and gestational (11.1%) diabetes. Moreover, statistically significant differences were observed between the percentage of male smokers (24.1%) as compared to female smokers (6.8%) and between the percentage of urban smokers (18.1%) as compared to those living in rural areas (11.6%). Among the smokers, 33.3% had a strong dependence on nicotine, while 48.5% had a moderate dependence and the remaining 18.2% had a slight dependence. Conclusions: These results show that a substantial percentage of individuals with diabetes are smokers, especially in the group of patients with type 1 diabetes and other variants. Male sex and an urban setting are factors that predispose to the smoking habit. The finding that only a third of the diabetic smokers were strongly dependent on nicotine suggests that the programs focusing on smoking cessation could be highly successful


Subject(s)
Humans , Diabetes Mellitus/complications , Tobacco Use Disorder/epidemiology , Cardiovascular Diseases/epidemiology , Epidemiology, Descriptive , Sex Factors , Risk Factors , Age Factors , Health Surveys
18.
Av. diabetol ; 20(4): 219-223, oct. 2004. tab, graf
Article in Es | IBECS | ID: ibc-37634

ABSTRACT

Introducción: La diabetes mellitus se asocia a un elevado riesgo cardiovascular. El adecuado control glucémico y lipídico reduce el riesgo de complicaciones cardiovasculares. El objetivo del presente estudio fue conocer el grado de control glucémico y lipídico en un grupo de pacientes con diabetes mellitus tipo 2 y antecedentes de cardiopatía isquémica. Material y métodos: Se incluyeron en el estudio 99 pacientes diagnosticados de diabetes mellitus tipo 2 con antecedentes de cardiopatía isquémica. En todos ellos se determinó el control glucémico: glucemia plasmática en ayunas y HbA1C y de los parámetros lipídicos: LDL-colesterol (cLDL), HDLcolesterol (cHDL), colesterol no-HDL y triglicéridos (TG). Se consideró un adecuado control glucémico cuando los niveles de glucemia plasmática y de HbA1C eran inferiores a 110 mg/dl y 7,0 por ciento, respectivamente. Por su parte, se consideró un adecuado control lipídico cuando los valores de eLDL, colesterol no-HDL y TG eran inferiores a 100 mg/dl, 130 mg/dl y 150 mg/dl, respectivamente; mientras que los de cHDL eran superiores a 40 y 50 mg/dl en varones y mujeres, respectivamente. Resultados: Los valores medios de HbAIC y glucemia plasmática en ayunas fueron de 8,3ñ1,4 por ciento y de 187,6ñ61,0 mg/dl, respectivamente. Los valores medios de cLDL, cHDL, Colesterol no-HDL y TG fueron de 109,7ñ30,9 mg/dl, 50,0ñ14,5 mg/dl, 137,4ñ36,5 mg/dl y de 141,5ñ94,2 mg/dl, respectivamente. El porcentaje de individuos que presentaba un buen control de la glucemia plasmática en ayunas y de HbA1C fue del 8,1 por ciento y del 20,2 por ciento, respectivamente. Un adecuado control de cLDL, cHDL, Colesterol no-HDL y TG fue alcanzado por el 41,8 por ciento, 55,6 por ciento, 5I,52 por ciento y 69,7 por ciento de los pacientes, respectivamente. Conclusiones: Estos resultados demuestran que el control glucémico en este grupo de pacientes es deficiente. Sin embargo, el control de parámetros lipídicos, aunque no es óptimo, si que se acerca a los valores de referencia establecidos en los consensos internacionales (AU)


Subject(s)
Aged , Female , Male , Middle Aged , Humans , Myocardial Ischemia/etiology , Diabetes Mellitus, Type 2/complications , Blood Glucose/analysis , Lipids/blood , Cholesterol/blood , Triglycerides/blood , Glycated Hemoglobin/analysis , Hyperlipidemias/complications
19.
Endocrinol. nutr. (Ed. impr.) ; 49(7): 222-226, ago. 2002. TAB
Article in Es | IBECS | ID: ibc-15394

ABSTRACT

Introducción. La calcitonina es un marcador tumoral específico y sensible del carcinoma medular de tiroides (CMT). El objetivo de este estudio fue determinar la utilidad de la determinación sistemática de calcitonina en la enfermedad nodular tiroidea para mejorar el diagnóstico preoperatorio de CMT. Pacientes y métodos. Desde agosto de 1994 hasta enero de 2002 hemos estudiado a 907 pacientes con enfermedad nodular tiroidea. Los pacientes se agruparon en 7 categorías de diagnóstico clínico: bocio multinodular no tóxico (45,6 por ciento), bocio uninodular no tóxico (29,1 por ciento), bocio multinodular tóxico (10,0 por ciento), nódulo autónomo hiperfuncionante (4,5 por ciento), tiroiditis de Hashimoto nodular (6,4 por ciento), enfermedad de Graves nodular (3,1 por ciento) y tiroiditis subaguda nodular (1,3 por ciento). Resultados. Seis pacientes (0,66 por ciento) presentaron concentraciones de calcitonina elevadas. En todos ellos el diagnóstico clínico fue de bocio uninodular no tóxico. Cuatro de ellos tenían calcitoninas muy elevadas, mientras que en los dos casos restantes la elevación de calcitonina fue leve. El estudio citológico de los 4 casos con una notable elevación de calcitonina fue leve. El estudio citológico de los 4 casos con una notable elevación de calcitonina fue compatible en dos de ellos con CMT mientras que, en los dos restantes, fue sospechoso de malignidad. En los 2 casos con leve elevación de calcitonina, los hallazgos citológicos fueron de benignidad. Los 6 pacientes fueron intervenidos. En los 4 casos con una notable elevación de calcitonina el resultado anatomopatológico fue de CMT, mientras que en los 2 casos con leve elevación de calcitonina el diagnóstico fue de hiperplasia nodular. Conclusiones. La determinación sistemática de calcitonina permitió el diagnóstico de 4 casos de CMT. En los pacientes con clara elevación de calcitonina puede asegurarse que el proceso corresponde a un CMT; sin embargo, en los casos con leve elevación de calcitonina es necesario apoyarse en otros datos clínicos y exploraciones complementarias antes de decidir el tratamiento quirúrgico, ya que el proceso puede no corresponder a un CMT. Dado que todos los casos con calcitonina elevada tenían un diagnóstico clínico de bocio uninodular no tóxico, creemos que la determinación de calcitonina debe realizarse sistemáticamente en los pacientes con este diagnóstico clínico (AU)


Subject(s)
Adolescent , Adult , Aged , Female , Male , Middle Aged , Humans , Thyroid Nodule/metabolism , Calcitonin/blood , Carcinoma, Medullary/diagnosis , Prospective Studies , Biomarkers, Tumor/analysis
20.
Nutr Hosp ; 17(1): 46-7, 2002.
Article in Spanish | MEDLINE | ID: mdl-11939129

ABSTRACT

Anorexia nervosa is a frequent condition of malnutrition in industrialized countries. The most frequent causes of death in patients with anorexia nervosa are suicide and sudden death generally in relationship with cardiovascular abnormalities. We report a case of a 42-year-old woman with anorexia nervosa who was admitted to our hospital because of severe weight loss. Her electrocardiogram showed a severe sinus bradycardia of 27 b/min.


Subject(s)
Anorexia Nervosa/complications , Bradycardia/etiology , Adult , Female , Humans
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