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1.
Eur J Endocrinol ; 185(4): 577-586, 2021 Sep 01.
Artigo em Inglês | MEDLINE | ID: mdl-34374651

RESUMO

Objective: Insulinomas are rare functional pancreatic neuroendocrine tumours. As previous data on the long-term prognosis of insulinoma patients are scarce, we studied the morbidity and mortality in the Finnish insulinoma cohort. Design: Retrospective cohort study. Methods: Incidence of endocrine, cardiovascular, gastrointestinal and psychiatric disorders, and cancers was compared in all the patients diagnosed with an insulinoma in Finland during 1980-2010 (n = 79, including two patients with multiple endocrine neoplasia type 1 syndrome), vs 316 matched controls, using the Mantel-Haenszel method. Overall survival was analysed with Kaplan-Meier and Cox regression analyses. Results: The median length of follow-up was 10.7 years for the patients and 12.2 years for the controls. The long-term incidence of atrial fibrillation (rate ratio (RR): 2.07 (95% CI: 1.02-4.22)), intestinal obstruction (18.65 (2.09-166.86)), and possibly breast (4.46 (1.29-15.39) and kidney cancers (RR not applicable) was increased among insulinoma patients vs controls, P < 0.05 for all comparisons. Endocrine disorders and pancreatic diseases were more frequent in the patients during the first year after insulinoma diagnosis, but not later on. The survival of patients with a non-metastatic insulinoma (n = 70) was similar to that of controls, but for patients with distant metastases (n = 9), the survival was significantly impaired (median 3.4 years). Conclusions: The long-term prognosis of patients with a non-metastatic insulinoma is similar to the general population, except for an increased incidence of atrial fibrillation, intestinal obstruction, and possibly breast and kidney cancers. These results need to be confirmed in future studies. Metastatic insulinomas entail a markedly decreased survival.


Assuntos
Insulinoma/epidemiologia , Neoplasias Pancreáticas/epidemiologia , Adulto , Idoso , Estudos de Casos e Controles , Estudos de Coortes , Comorbidade , Finlândia/epidemiologia , Seguimentos , História do Século XX , História do Século XXI , Humanos , Incidência , Insulinoma/complicações , Insulinoma/diagnóstico , Insulinoma/mortalidade , Pessoa de Meia-Idade , Morbidade , Neoplasias Pancreáticas/complicações , Neoplasias Pancreáticas/diagnóstico , Neoplasias Pancreáticas/mortalidade , Prognóstico , Estudos Retrospectivos , Análise de Sobrevida , Fatores de Tempo
2.
Ann Endocrinol (Paris) ; 81(2-3): 110-117, 2020 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-32409005

RESUMO

Hypoglycemia is defined by a low blood glucose level associated to clinical symptoms. Hypoglycemia may be related to treatment of diabetes, but also to drugs, alcohol, critical illness, cortisol insufficiency including hypopituitarism, insulinoma, bariatric or gastric surgery, pancreas transplantation or glucagon deficiency, or may be surreptitious. Some hypoglycemic episodes remain unexplained, and genetic, paraneoplastic and immune causes should be considered. Genetic causes may be related to endogenous hyperinsulinism and to inborn errors of metabolism (IEM). Endogenous hyperinsulinism is related to monogenic congenital hyperinsulinism, and especially to mutations of the glucokinase-activating gene or of insulin receptors, both characterised by postprandial hypoglycemia with major hyperinsulinism. In adulthood, IEM-related hypoglycemia can persist in a previously diagnosed childhood disease or may be a presenting sign. It is suggested by systemic involvement (rhabdomyolysis after fasting or exercising, heart disease, hepatomegaly), sometimes associated to a family history of hypoglycemia. The timing of hypoglycemic episodes with respect to the last meal also helps to orientate diagnosis. Fasting hypoglycemia may be related to type 0, I or III glycogen synthesis disorder, fatty acid oxidation or gluconeogenesis disorder. Postprandial hypoglycemia may be related to inherited fructose intolerance. Exercise-induced hyperinsulinism is mainly related to activating mutation of the SLC16A1 gene. Besides exceptional ectopic insulin secretion, paraneoplastic causes involve NICTH (Non-Islet-Cell Tumour Hypoglycemia), caused by Big-IGF2 secretion by a large tumour, with low blood levels of insulin, C-peptide and IGF1. Autoimmune causes involve antibodies against insulin (HIRATA syndrome), especially in case of Graves' disease, or against the insulin receptor. Medical history, timing, and insulin level orientate the diagnosis.


Assuntos
Hipoglicemia/epidemiologia , Hipoglicemia/etiologia , Adulto , Idade de Início , Antígenos CD/genética , Criança , Complicações do Diabetes/sangue , Complicações do Diabetes/epidemiologia , Jejum/sangue , Humanos , Hiperinsulinismo/sangue , Hiperinsulinismo/complicações , Hiperinsulinismo/epidemiologia , Insulinoma/sangue , Insulinoma/complicações , Insulinoma/epidemiologia , Erros Inatos do Metabolismo/sangue , Erros Inatos do Metabolismo/complicações , Erros Inatos do Metabolismo/epidemiologia , Erros Inatos do Metabolismo/genética , Neoplasias Pancreáticas/sangue , Neoplasias Pancreáticas/complicações , Neoplasias Pancreáticas/epidemiologia , Receptor de Insulina/genética , Fatores de Risco
3.
World J Surg ; 44(7): 2288-2294, 2020 07.
Artigo em Inglês | MEDLINE | ID: mdl-32128613

RESUMO

INTRODUCTION: Due to the rarity of malignant insulinoma, a lack of the literature describing factors affecting outcomes exists. Our aim was to review malignant insulinoma incidence, characteristics and survival trends. METHODS: We identified all patients with malignant insulinoma in the SEER registries from 1973 to 2015. Incidence, neoplasm characteristics and factors affecting cancer-specific survival (CSS) were described. RESULTS: A total of 121 patients were identified. The crude annual overall incidence was low (range 0.0-0.27 cases per million person years). The largest proportion had localized disease (40%), while 16% had regional disease, 39% distant metastatic disease, and stage was unreported in 5%. Most neoplasms were in the body/tail of the pancreas, followed by the head of the pancreas. Grade was reported in 40% of patients; only a single patient reported as having grade IV with the remainder all grades I/II. Surgical resection was performed in 64% of patients. Within surgical patients, the median primary neoplasm size was 1.8 cm. Regional lymph nodes were examined in 57.1% of surgical patients, while 34% of examined nodes were positive. The median CSS was 183 months. On multivariable analysis, surgical resection, male sex and absence of metastatic disease were associated with superior survival. CONCLUSION: While the greatest proportion of patients with malignant insulinoma present with localized disease, regional lymph node involvement was found in 34% of whose nodes were tested. Further studies are needed to assess the role of lymph node dissection in improving survival and preventing recurrence given the observed frequency of lymph node involvement.


Assuntos
Insulinoma , Tumores Neuroendócrinos , Neoplasias Pancreáticas , Humanos , Insulinoma/diagnóstico , Insulinoma/epidemiologia , Insulinoma/patologia , Insulinoma/cirurgia , Excisão de Linfonodo , Metástase Linfática , Tumores Neuroendócrinos/diagnóstico , Tumores Neuroendócrinos/epidemiologia , Tumores Neuroendócrinos/patologia , Tumores Neuroendócrinos/cirurgia , Neoplasias Pancreáticas/diagnóstico , Neoplasias Pancreáticas/epidemiologia , Neoplasias Pancreáticas/patologia , Neoplasias Pancreáticas/cirurgia , Prognóstico , Taxa de Sobrevida
4.
J Diabetes Investig ; 11(3): 554-563, 2020 May.
Artigo em Inglês | MEDLINE | ID: mdl-31742894

RESUMO

AIMS/INTRODUCTION: We aimed to investigate the nationwide incidence, treatment details and outcomes of patients with endogenous hyperinsulinemic hypoglycemia (EHH), including those with transient/persistent congenital hyperinsulinism (CHI), insulinoma, non-insulinoma pancreatogenous hypoglycemia syndrome and insulin autoimmune syndrome (Hirata's disease) in Japan. MATERIALS AND METHODS: A nationwide, questionnaire-based survey was carried out to determine the number of patients with EHH who were treated for hypoglycemia or hypoglycemia-related complications in 2017-2018. The questionnaires were sent to all hospitals in Japan with >300 beds, and with pediatric and/or adult clinics likely managing EHH patients. The secondary questionnaires were sent to obtain the patients' date of birth, sex, age at onset, treatment details and post-treatment outcomes. RESULTS: A total of 447 patients with CHI (197 transient CHI, 225 persistent CHI and 25, unknown histology), 205 with insulinoma (118 benign, 18 malignant and 69 unknown subtype), 111 with non-insulinoma pancreatogenous hypoglycemia syndrome (33 post-gastric surgery HH, 57 postprandial HH, 10 nesidioblastosis and 11 unknown subtype) and 22 with insulin autoimmune syndrome were identified. Novel findings included: (i) marked improvement in the prognosis of persistent CHI over the past 10 years; (ii) male dominance in the incidence of transient CHI; (iii) non-insulinoma pancreatogenous hypoglycemia syndrome emerging as the second most common form of EHH in adults; (iv) frequent association of diabetes mellitus with insulin autoimmune syndrome; and (v) frequent post-treatment residual hypoglycemia and impaired quality of life. CONCLUSIONS: The first nationwide, all age group survey of EHH showed the current status of each type of EHH disorder and the unmet needs of the patients.


Assuntos
Hiperinsulinismo/epidemiologia , Hipoglicemia/epidemiologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , Pré-Escolar , Hiperinsulinismo Congênito/epidemiologia , Feminino , Humanos , Lactente , Recém-Nascido , Insulinoma/epidemiologia , Japão/epidemiologia , Masculino , Pessoa de Meia-Idade , Nesidioblastose/epidemiologia , Inquéritos e Questionários , Adulto Jovem
5.
Vet Q ; 38(1): 53-62, 2018 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-29806550

RESUMO

OBJECTIVES: To determine 1) the sensitivity of contrast-enhanced CT (CECT) for detection of primary canine insulinomas and metastases 2) the sensitivity of CECT to locate canine insulinomas within the pancreas and 3) the CECT attenuation pattern of canine insulinomas and post-contrast phase in which insulinomas have the best visibility. METHODS: A retrospective review was performed of the medical records of 27 canine insulinoma patients. Simultaneous occurrence of blood glucose < 3.5 mmol/L (reference interval: 4.2-5.8 mmol/L) and plasma insulin > 10 mIU/L (reference interval: 1.4-24.5 mIU/L) were considered diagnostic for insulinoma. The dogs had a mean age of 9.0 ± 1.7 (SD) years and comprised 11 males and 17 females. RESULTS: Using CECT-scans, 26/27 insulinomas were successfully detected. However, CECT-scans predicted the correct location of insulinomas within the pancreas in only 14/27 dogs. In 9/13 inaccurately located insulinoma cases, the location error was major. There was no significant difference between triple, double and single-phase CECT-scans with location accuracies of 54%, 50% and 50%, respectively. Also, there was no specific post-contrast phase in which insulinomas could be visualised best. Detection of lymph node metastases with CECT-scans had a sensitivity of 67% (10/15 lymph node metastases). Detection of liver metastases had a sensitivity of 75% (6/8 liver metastases). This study highlights that major location errors mainly occurred if single- or double-phase CECT-scans were used (6/9 cases). CONCLUSION: It is suggested that triple-phase CECT-scans have superior outcome over single- or double-phase CECT-scans in pre-operative imaging of canine insulinomas.


Assuntos
Doenças do Cão/diagnóstico por imagem , Insulinoma/veterinária , Neoplasias Pancreáticas/veterinária , Tomógrafos Computadorizados/veterinária , Animais , Glicemia , Meios de Contraste , Doenças do Cão/epidemiologia , Cães , Feminino , Insulina/sangue , Insulinoma/diagnóstico por imagem , Insulinoma/epidemiologia , Masculino , Neoplasias Pancreáticas/diagnóstico por imagem , Neoplasias Pancreáticas/epidemiologia , Estudos Retrospectivos , Sensibilidade e Especificidade
6.
Duodecim ; 131(17): 1598-604, 2015.
Artigo em Finlandês | MEDLINE | ID: mdl-26548107

RESUMO

BACKGROUND: Insulin-producing neuroendocrine tumours (iNETs) are rare, but their incidence is increasing. We studied the incidence, clinical picture, diagnostics, and treatment of insulinomas diagnosed in 1980 to 2010. METHODS: Retrospective analysis of insulinomas diagnosed in Tampere University Hospital. RESULTS: We found 23 iNET cases corresponding to an incidence of 0.7/million/year. All had neuroglycopenic symptoms and 83% had autonomic ones. The median diagnostic delay (from first symptoms up to diagnosis) was 25 months. Preoperative imaging found the tumor in 87%. Twenty-one out of 22 patients who underwent surgery recovered completely. CONCLUSIONS: Despite improved imaging the diagnostic delay of iNETs remained unchanged. Hypoglycemia and insulinoma should be considered as a cause of unspecific, symptomatic attacks.


Assuntos
Insulinoma/diagnóstico , Insulinoma/terapia , Neoplasias Pancreáticas/diagnóstico , Neoplasias Pancreáticas/terapia , Feminino , Finlândia/epidemiologia , Hospitais Universitários , Humanos , Incidência , Insulinoma/epidemiologia , Masculino , Neoplasias Pancreáticas/epidemiologia , Estudos Retrospectivos
7.
Endocrinol. nutr. (Ed. impr.) ; 62(7): 306-313, ago.-sept. 2015. ilus, tab
Artigo em Espanhol | IBECS | ID: ibc-143056

RESUMO

OBJETIVO: Analizar las características clínicas, metodología diagnóstica, tratamientos empleados y resultados de los casos de insulinoma diagnosticados y tratados entre 1983-2014 en 4 centros hospitalarios españoles. MÉTODOS: Se incluyeron en el estudio todos los pacientes que tenían demostración histológica de tumor secretor de insulina o criterios diagnósticos bioquímicos y morfológicos de insulinoma. RESULTADOS: Se estudiaron 29 pacientes (23 mujeres [79,3%]; edad media 48,7 ± 17,4 años [intervalo 16-74]). En 26 (89,7%) casos el tumor fue esporádico y en el resto (3 mujeres, 10,3%) se presentó en el contexto de una neoplasia endocrina múltiple tipo 1. Hubo 3 (10,3%) insulinomas múltiples, uno de ellos asociado a neoplasia endocrina múltiple tipo 1, y 2 (6,9%) insulinomas malignos, ambos esporádicos. La mayoría (n = 18, 62,1%) mostró hipoglucemia de ayuno, aproximadamente un tercio (31%) hipoglucemia tanto de ayuno como posprandial y el 6,9% solo hipoglucemia posprandial. El tiempo en alcanzar el nadir de glucosa (37,3 ± 6,5 mg/dl) en la prueba de ayuno fue 9,0 ± 4,4 h, con insulinemia de 25,0 ± 20,3 μU/ml. La TAC abdominal localizó el insulinoma en el 75% de los casos. El 93,1% (n = 27) de los pacientes fue intervenido quirúrgicamente (enucleación, 18 [66,7%] y pancreatectomía parcial, 9 [33,3%] pacientes; tamaño tumor 1,7 ± 0,7 cm). La cirugía consiguió la curación en la mayoría (n = 24, 88,9%) de los pacientes. CONCLUSIÓN: El insulinoma en nuestro medio es un tumor benigno, de pequeño tamaño y solitario, que afecta más a mujeres entre 45-50 años y que se localiza generalmente con TAC abdominal. La cirugía mediante enucleación constituye el método terapéutico más habitual consiguiendo unas altas tasas de curación


OBJECTIVE: To analyze the clinical features, diagnostic procedures, treatment, and clinical outcome of insulinomas diagnosed and treated in the period 1983-2014 in four Spanish hospitals. METHODS: All patients with either biochemical and morphological criteria of insulinoma and/or histological demonstration of insulin-secreting tumor were included. RESULTS: Twenty-nine patients [23 women (79.3%); mean age 48.7 ± 17.4 years (range, 16-74)] were recruited. Twenty-six patients (89.7%) had sporadic tumors, and the rest (3 women, 10.3%) developed in the context of multiple endocrine neoplasia type 1. There were 3 (10.3%) multiple insulinomas, one associated with multiple endocrine neoplasia type 1, and two (6.9%) malignant insulinomas, both sporadic. Most patients (n = 18, 62.1%) had fasting hypoglycemia, about a third (31%) both postprandial and fasting hypoglycemia, and 6.9% postprandial hypoglycemia only. Time to glucose nadir (37.3 ± 6.5 mg/dL) in the fasting test was 9.0 ± 4.4 h, with maximal insulin levels of 25.0 ± 20.3 μU/mL. Abdominal CT detected insulinoma in 75% of patients. Twenty-seven (93.1%) patients underwent surgery [enucleation, 18 (66.7%) and subtotal pancreatectomy, 9 (33.3%); tumor size, 1,7 ± 0,7 cm]. Surgery achieved cure in the majority (n = 24, 88.9%) of patients. CONCLUSION: In our setting, insulinoma is usually a benign, small, and solitary tumor, mainly affecting women aged 45-50 years, and usually localized with abdominal CT. The most commonly used surgical technique is enucleation, which achieves a high cure rate


Assuntos
Humanos , Insulinoma/epidemiologia , Neoplasia Endócrina Múltipla/epidemiologia , Pancreatectomia , Estudos Retrospectivos , Tomografia Computadorizada por Raios X , Hipoglicemia/epidemiologia , Período Pós-Prandial
8.
Endocrinol Nutr ; 62(7): 306-13, 2015.
Artigo em Inglês, Espanhol | MEDLINE | ID: mdl-26050581

RESUMO

OBJECTIVE: To analyze the clinical features, diagnostic procedures, treatment, and clinical outcome of insulinomas diagnosed and treated in the period 1983-2014 in four Spanish hospitals. METHODS: All patients with either biochemical and morphological criteria of insulinoma and/or histological demonstration of insulin-secreting tumor were included. RESULTS: Twenty-nine patients [23 women (79.3%); mean age 48.7±17.4 years (range, 16-74)] were recruited. Twenty-six patients (89.7%) had sporadic tumors, and the rest (3 women, 10.3%) developed in the context of multiple endocrine neoplasia type 1. There were 3 (10.3%) multiple insulinomas, one associated with multiple endocrine neoplasia type 1, and two (6.9%) malignant insulinomas, both sporadic. Most patients (n=18, 62.1%) had fasting hypoglycemia, about a third (31%) both postprandial and fasting hypoglycemia, and 6.9% postprandial hypoglycemia only. Time to glucose nadir (37.3±6.5mg/dL) in the fasting test was 9.0±4.4h, with maximal insulin levels of 25.0±20.3µU/mL. Abdominal CT detected insulinoma in 75% of patients. Twenty-seven (93.1%) patients underwent surgery [enucleation, 18 (66.7%) and subtotal pancreatectomy, 9 (33.3%); tumor size, 1,7±0,7cm]. Surgery achieved cure in the majority (n=24, 88.9%) of patients. CONCLUSION: In our setting, insulinoma is usually a benign, small, and solitary tumor, mainly affecting women aged 45-50 years, and usually localized with abdominal CT. The most commonly used surgical technique is enucleation, which achieves a high cure rate.


Assuntos
Insulinoma/epidemiologia , Neoplasias Pancreáticas/epidemiologia , Adolescente , Adulto , Idoso , Glicemia/análise , Peptídeo C/sangue , Jejum/sangue , Feminino , Humanos , Hipoglicemia/sangue , Hipoglicemia/etiologia , Insulinoma/complicações , Insulinoma/diagnóstico por imagem , Insulinoma/cirurgia , Masculino , Pessoa de Meia-Idade , Neoplasia Endócrina Múltipla Tipo 1/epidemiologia , Pancreatectomia/métodos , Neoplasias Pancreáticas/complicações , Neoplasias Pancreáticas/diagnóstico por imagem , Neoplasias Pancreáticas/cirurgia , Período Pós-Prandial , Estudos Retrospectivos , Espanha/epidemiologia , Carga Tumoral , Adulto Jovem
10.
J Clin Endocrinol Metab ; 100(4): 1568-77, 2015 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-25594862

RESUMO

CONTEXT: Multiple endocrine neoplasia Type-1 (MEN1) in young patients is only described by case reports. OBJECTIVE: To improve the knowledge of MEN1 natural history before 21 years old. METHODS: Obtain a description of the first symptoms occurring before 21 years old (clinical symptoms, biological or imaging abnormalities), surgical outcomes related to MEN1 Neuro Endocrine Tumors (NETs) occurring in a group of 160 patients extracted from the "Groupe d'étude des Tumeurs Endocrines" MEN1 cohort. RESULTS: The first symptoms were related to hyperparathyroidism in 122 cases (75%), pituitary adenoma in 55 cases (34%), nonsecreting pancreatic tumor (NSPT) in 14 cases (9%), insulinoma in 20 cases (12%), gastrinoma in three cases (2%), malignant adrenal tumors in 2 cases (1%), and malignant thymic-NET in one case (1%). Hyperparathyrodism was the first lesion in 90 cases (56%). The first symptoms occurred before 10 years old in 22 cases (14%) and before 5 years old in five cases (3%). Surgery was performed before age 21 in 66 patients (41%) with a total of 74 operations: pituitary adenoma (n = 9, 16%), hyperparathyroidism (n = 38, 31%), gastrinoma (n = 1, 33%), NSPT (n = 5, 36%), and all cases of insulinoma, adrenal tumors, and thymic-NET. One patient died before age 21 due to a thymic-NET. Overall, lesions were malignant in four cases. CONCLUSIONS: Various MEN1 lesions occurred frequently before 21 years old, but mainly after 10 years of age. Rare, aggressive tumors may develop at any age. Hyperparathyroidism was the most frequently encountered lesion but was not always the first biological or clinical abnormality to appear during the course of MEN1.


Assuntos
Neoplasia Endócrina Múltipla Tipo 1/epidemiologia , Adenoma/diagnóstico , Adenoma/epidemiologia , Adolescente , Neoplasias das Glândulas Suprarrenais/diagnóstico , Neoplasias das Glândulas Suprarrenais/epidemiologia , Adulto , Idade de Início , Criança , Pré-Escolar , Estudos de Coortes , Feminino , França/epidemiologia , Humanos , Lactente , Insulinoma/diagnóstico , Insulinoma/epidemiologia , Masculino , Neoplasia Endócrina Múltipla Tipo 1/diagnóstico , Tumores Neuroendócrinos/diagnóstico , Tumores Neuroendócrinos/epidemiologia , Neoplasias Pancreáticas/diagnóstico , Neoplasias Pancreáticas/epidemiologia , Neoplasias Hipofisárias/diagnóstico , Neoplasias Hipofisárias/epidemiologia , Adulto Jovem
11.
Klin Med (Mosk) ; 92(2): 65-70, 2014.
Artigo em Russo | MEDLINE | ID: mdl-25269185

RESUMO

The incidence of insulinoma, an insulin-producing tumour arising from pancreatic beta-cells and responsible for the development of fasting hypoglycemia, in the general population is 1-4 per 1,000,000 yearly, mostly at the age of 25-55 yr. Malignization of this neoplasm occurs in 10-15% of the cases. One third of the tumours produce metastases. The most characteristic clinical manifestation of insulinoma is the Whipple's triad, with episodes of fasting hypoglycemia (below 2.8 mmol/l) correctable by intravenous glucose injection or intake of sugar. The authors report a case of intravital diagnosis of malignant metastasizing insulinoma in a 82 year old woman with type 2 diabetes mellitus. A review of relevant literature is presented.


Assuntos
Diabetes Mellitus Tipo 2/patologia , Insulinoma/patologia , Insulinoma/secundário , Neoplasias Pancreáticas/patologia , Idoso de 80 Anos ou mais , Comorbidade , Diabetes Mellitus Tipo 2/epidemiologia , Evolução Fatal , Feminino , Humanos , Hipoglicemia/complicações , Hipoglicemia/epidemiologia , Hipoglicemia/patologia , Insulinoma/epidemiologia , Neoplasias Pancreáticas/complicações , Neoplasias Pancreáticas/epidemiologia
12.
Vojnosanit Pregl ; 71(3): 293-7, 2014 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-24697017

RESUMO

BACKGROUND/AIM: Insulinomas are rare benign tumors in the most cases and the most frequent endocrine tumors of the pancreas. A wide spectrum of clinical manifestations in patients with insulinoma is the reason for difficult recognition of the disease with a long period of time between the onset of symptoms and the diagnosis. Diagnostic procedures include Whipple's triad, 72-hour fast test and topographic assessment. The only currative therapy for patients with insulinoma is operative treatment. METHODS: This retrospective study included 42 patients with diagnosis of insulinoma treated in our institution in a 60-year period. In all the patients a demographic and clinical data, types of biochemical methods for diagnosis, and diagnostic procedures for insulinoma localization were analyzed. Tumor size and localization, surgical procedures, postoperative complications and outcome were assessed. RESULTS: A study included 42 patients, 29 women and 13 men. The median age at diagnosis was 43 years. Median time between the onset of symptoms and diagnosis was 3 years. The most common clinical symptoms and signs were disturbance of consciousness and abnormal behavior in 73%, confusion and convulsions in 61% of patients. The diagnosis of insulinoma was estimated by Whipple's triad and 72-hour fast test in 14 patients. Determination of insulinoma localization was assessed by angiography in 16 (36%) of the patients, by ultrasound (US) in 3 of 16 (18.8%) patients, by abdominal computed tomography (CT) in 8 of 18 (44.5%) patients, and magnetic resonance imaging (MRI) in 2 of 8 (25%) patients. Insulinoma was found in 13 of 13 (100%) patients by arterial stimulation with venous sampling (ASVS) and in 13 of 14 (93%) patients by endoscopic ultrasound (EUS). Of the 42 patients, 38 (90.5%) underwent operative procedure. Minimal resection was performed in 28 (73.6%) of the patients [tumor enucleation in 27 (71%) and central pancreatectomy in one (2.6%) of the patients], and the major resection was performed in 9 (23.6%) of the operated patients [distal splenopancreatectomy in 8 (21%) and pancreaticoduodenectomy in one (2.6%) patient]. The overall mortality rate in postoperative period was 2.6% (one patient). CONCLUSION: A combination of ASVS and EUS as diagnostic procedures ensures high accuracy for preoperative determination of insulinoma localization. Minimal resection such as enucleation shoud be performed whenever it is possible.


Assuntos
Academias e Institutos , Insulinoma/cirurgia , Medicina Militar , Pancreatectomia/métodos , Neoplasias Pancreáticas/cirurgia , Adolescente , Adulto , Idoso , Angiografia , Criança , Feminino , Humanos , Incidência , Insulinoma/diagnóstico , Insulinoma/epidemiologia , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Pancreatectomia/estatística & dados numéricos , Neoplasias Pancreáticas/diagnóstico , Estudos Retrospectivos , Sérvia/epidemiologia , Tomografia Computadorizada por Raios X , Resultado do Tratamento , Adulto Jovem
14.
Hepatobiliary Pancreat Dis Int ; 12(3): 324-8, 2013 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-23742779

RESUMO

BACKGROUND: The etiology of insulinoma is poorly understood. Few studies investigated the possible roles of environmental factors and lifestyle in the pathogenesis of insulinoma. The aim of this study is to identify risk factors associated with occurrence of insulinoma in the Chinese population. METHODS: This study consisted of 196 patients with insulinoma and 233 controls. Demographic information of the patients and controls and risk factors of the disease were analyzed. Univariate and unconditional multivariable logistic regression analyses were made to estimate odds ratios (ORs) and possible risk factors. RESULTS: Approximately 68.88% (135/196) of the patients were from rural areas in contrast to 10.30% (24/233) of the controls (P<0.0001). This difference was confirmed by the multivariate analysis (OR=4.950; 95% CI: 2.928-8.370). Family history of pancreatic endocrine tumor (OR=16.754; 95% CI: 2.125-132.057) and other cancers (OR=2.360; 95% CI: 1.052-5.291) was also related to a high-risk population of insulinoma. CONCLUSION: Rural residents or people who have a family history of pancreatic endocrine tumor and other cancers are a high-risk population of insulinoma.


Assuntos
Insulinoma/epidemiologia , Neoplasias Pancreáticas/epidemiologia , Adolescente , Adulto , Idoso , Distribuição de Qui-Quadrado , China/epidemiologia , Feminino , Predisposição Genética para Doença , Humanos , Insulinoma/genética , Estilo de Vida , Modelos Logísticos , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Razão de Chances , Neoplasias Pancreáticas/genética , Linhagem , Características de Residência , Estudos Retrospectivos , Fatores de Risco , Saúde da População Rural , Saúde da População Urbana , Adulto Jovem
17.
Endocr J ; 59(10): 859-66, 2012.
Artigo em Inglês | MEDLINE | ID: mdl-22785103

RESUMO

More than 50% of patients with multiple endocrine neoplasia type 1 (MEN1) develop gastroenteropancreatic neuroendocrine tumors (GEPNETs), and insulinoma is the second most common functioning GEPNET. Compared to other functioning and nonfunctioning GEPNETs in MEN1, insulinoma is considered to develop at a younger age. To clarify the clinical features of insulinoma developed in Japanese patients with MEN1, a recently constructed database of Japanese MEN1 patients was analyzed. Among 560 registered cases, insulinoma was seen in 69 patients and information on age at diagnosis was available for 54 patients. Tumors predominantly occurred in the body and tail of the pancreas. The mean age at diagnosis of insulinoma (34.8 ± 16.7 yrs) was significantly younger than that of gastrinoma (50.6 ± 14.3 yrs) and nonfunctioning tumor (44.7 ± 13.3 yrs) in patients with MEN1. Patients diagnosed as having insulinoma during middle-age (30 - 49 yrs) tended to have a long period from appearance of hypoglycemic symptoms to diagnosis of the tumor. Of note, 13 patients (24%) were diagnosed as having insulinoma before 20 yrs of age. Such young onset was not seen in other GEPNETs. Since the development of GEPNETs during adolescence is quite rare, insulinoma diagnosed before 20 yrs strongly suggests the presence of MEN1 and warrants further investigation, including MEN1 genetic testing. Also, clinicians should be aware that insulinoma can often be missed in middle-aged patients.


Assuntos
Insulinoma/epidemiologia , Neoplasia Endócrina Múltipla Tipo 1/complicações , Neoplasia Endócrina Múltipla Tipo 1/patologia , Adolescente , Adulto , Idade de Início , Idoso , Criança , Bases de Dados Factuais , Feminino , Humanos , Insulinoma/etiologia , Insulinoma/patologia , Japão/epidemiologia , Masculino , Pessoa de Meia-Idade , Neoplasia Endócrina Múltipla Tipo 1/epidemiologia , Neoplasia Endócrina Múltipla Tipo 1/genética , Tumores Neuroendócrinos/genética , Neoplasias Pancreáticas/genética , Neoplasias Pancreáticas/patologia
20.
J Surg Oncol ; 103(2): 169-74, 2011 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-21259252

RESUMO

BACKGROUND: Insulinoma is rare tumor with an incidence of approximately four cases per million per year. There are few large sample, single-center series that focus on the surgical management strategy of insulinomas. PATIENTS AND METHODS: Medical records of patients diagnosed as insulinoma from 1990 to 2010 in Peking Union Medical College Hospital were reviewed retrospectively. Clinical data were collected and statistically analyzed. RESULTS: A total of 328 patients were diagnosed with insulinomas; 292 of them underwent 320 operations, which included 46 laparoscopic surgeries. Tumor enucleation was the most common operative procedure. Multiple tumors were found in 30 cases; 17 cases were multiple endocrine neoplasia-1 syndrome. Thirteen patients with malignant insulinomas underwent tumor resection. Pancreatic fistula (PF) was the most frequent complication, and the incidence of clinical PFs (Grades B and C) was 14.4%. There was no significant statistical difference between open and laparoscopic surgery in blood loss, operative time, and complications. Metachronous tumors were noted in 11 patients. CONCLUSION: Surgery is the best treatment of choice for insulinoma patients. Surgical approach depends on tumor size, location, and its pathological characters. Laparoscopic management of insulinomas is feasible and safe for tumors located in the body or tail of the pancreas. Open surgery combined with intraoperative ultrasonography is recommended to avoid omission of lesions in patients with multiple insulinomas. An aggressive surgical approach is indicated for malignant insulinoma patients.


Assuntos
Insulinoma/cirurgia , Pancreatectomia/métodos , Neoplasias Pancreáticas/cirurgia , Adolescente , Adulto , Idoso , Causalidade , Criança , Comorbidade , Feminino , Humanos , Incidência , Insulinoma/diagnóstico por imagem , Insulinoma/epidemiologia , Laparoscopia/métodos , Tempo de Internação , Masculino , Pessoa de Meia-Idade , Monitorização Intraoperatória/métodos , Fístula Pancreática/epidemiologia , Neoplasias Pancreáticas/diagnóstico por imagem , Neoplasias Pancreáticas/epidemiologia , Complicações Pós-Operatórias/epidemiologia , Estudos Retrospectivos , Cirurgia Assistida por Computador/métodos , Resultado do Tratamento , Ultrassonografia , Adulto Jovem
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