Insulinoma simulando una epilepsia: reporte de caso / Insulinoma simulating epilepsy: case report

Insulinoma es un tumor neuroendocrino que surge de las células ß del páncreas y produce hiperinsulinemia endógena. Son neoplasias raras con una incidencia reportada de 4 casos por millón de habitantes por año. La presentación clínica típicamente cursa con síntomas adrenérgicos y neuroglucopénicos secundarios a hipoglicemia. Requiere estudios de niveles séricos de insulina, pro-insulina y péptido C, además de imágenes diagnosticas que confirmen los hallazgos. La mayoría de los insulinomas son benignos, su sitio primario más común es el páncreas y pueden extirparse quirúrgicamente. Se presenta el caso de un hombre de 36 años con déficit cognitivo leve y episodios de diaforesis con deterioro neurológico hasta convulsiones tónico clónicas generalizadas que curiosamente resolvían con uso doméstico de "panela molida". Se ingresó a urgencias por ataques recurrentes de hipoglicemia severa con requerimiento de altas dosis de dextrosa al 50% por acceso central, hasta confirmación diagnóstica, intervención y resección de tumor neuroendocrino pancreático bien diferenciado (G1 según clasificación OMS) tipo insulinoma en la cola del páncreas.

Insulinoma is a neuroendocrine tumor that arises from the ß cells of the pancreas and produces endogenous hyperinsulinemia. They are rare neoplasms with a reported incidence to 4 cases per million inhabitants per year. The clinical presentation typically presents with adrenergic and neuroglycopenic symptoms secondary to hypoglycemia. It requires studies of serum levels of insulin, pro-insulin and C-peptide, in addition to diagnostic images that confirm the findings. Most insulinomas are benign, their most common primary site is the pancreas, and they can be removed surgically. We present the case of a 36-year-old man with mild cognitive deficits and episodes of diaphoresis with neurological deterioration to generalized clonic tonic seizures that curiously resolved with domestic use of "ground brown sugar". He was admitted to the emergency department due to recurrent attacks of severe hypoglycemia with a high-dose requirement for 50% dextrose through central access, until diagnostic confirmation, intervention, and resection of a welldifferentiated pancreatic neuroendocrine tumor (G1 according to WHO classification) insulinoma in the tail of pancreas.

Anesthesitic management for insulinoma resection in a patient having chronic inflammatory demyelinating neuropathy / Manejo anestésico para resección de insulinoma en un paciente portador de neuropatía desmielinizante crónica

Chronic Inflammatory Demyelinating Polyneuropathy associated with hypoglycemia 2 to insulinoma is unusual, and to our knowledge, very few patients have been reported in literature. Despite varying presentations in these patients, the clinical characteristics are usually the same. The syndrome usually occurs after several episodes of protracted hypoglycemia. The neuropathy is nearly always symmetrical. We report anesthetic management for a young female patient presenting with CIDP & repeated hypoglycemic episodes during a 2-year period scheduled for insulinoma enucleation.

La polineuropatía desmielinizante inflamatoria crónica asociada con hipoglicemia secundaria a insulinoma es inusual y, hasta donde sabemos, muy pocos pacientes han sido reportados en la literatura. A pesar de las diferentes presentaciones en estos pacientes, las características clínicas suelen ser las mismas. El síndrome generalmente ocurre después de varios episodios de hipoglicemia prolongada. La neuropatía es casi siempre simétrica. Presentamos el manejo anestésico para una paciente joven que se presenta con polineuropatía desmielinizante inflamatoria crónica y episodios repetidos de hipoglicemia durante un período de 2 años programado para la enucleación de insulinoma.

Insulinoma pancreático gigante / Giant pancreatic insulinoma

Los insulinomas son los tumores neuroendocrinos pancreáticos más frecuentes y típicamente son benignos y pequeños. Los insulinomas gigantes de páncreas son tumores raros. Reportamos el caso de un paciente varón de 67 años, quien presentaba síntomas y signos de hipoglicemia. Subsecuentes estudios de laboratorio y radiológicos establecieron el diagnóstico de un insulinomade 13,5 cm. Fue sometido a pancreaticoduodenectomía. A pesar del tamaño, en la evaluación patológica no se identificó invasión localni metástasis. Además de la descripción de la presentación clínica y los hallazgos operatorios, se realiza una revisión de las opcionespara la localización y manejo de los insulinomas.

Insulinomas are the most common pancreatic neuroendocrine tumors and are typically small and benign. Giant pancreatic insulinomasare rare pancreatic tumors. We report a 67 year old man who presented with signs and symptoms of hypoglycemia. Subsequentlaboratory and radiologic studies established the diagnosis of a 13.5 cm insulinoma. The patient underwent a pancreatoduodenectomy.Despite the size, neither local invasion nor metastatic disease was identified on pathological evaluation. In addition to describing theclinical presentation and operative findings, localization and management options of insulinomas are reviewed.

[Nesidioblastosis in adults]

Nesidioblastosis is a rare disorder in adults characterized by diffuse proliferation of islet cells. The ideal diagnostic and therapeutic approach is still controversial. The diagnosis in our case was made after distal pancreatectomy as the treatment of insulinoma. Finally, all the remaining pancreatic tissue had to be removed [total pancreatectomy] to control episodes of hypoglycemia

Hipoglicemia em adultos / Hypoglycemia in adults

A hipoglicemia é um evento potencialmente grave, com significativa morbidade. É rara em indivíduos sem diabetes, e pode ocorrer com frequência variável em pacientes com diabetes mellitus, especialmente naqueles que utilizam insulina. Para minimizar esse risco é imprescindível que se individualize o tratamento, estabelecendo metas glicêmicas de acordo com a idade, expectativa de vida, comorbidades e estilo de vida

Hypoglycemia is a potentially serious event with significant morbidity. It is rare in individuals without diabetes and can occur in a quite variable frequency in diabetic patients, especially in those that use insulin. In order to minimize its risk in these groups, it is essential to individualize treatment, establishing glycemic targets according to age, life expectancy, comorbidities and lifestyle

Tratamento cirúrgico dos insulinomas do pâncreas / Surgical treatment of pancreatic insulinomas

RACIONAL - O insulinoma do pâncreas é neoplasia neuroendócrina proveniente das células beta das ilhotas pancreáticas. Apesar de rara, é a neoplasia endócrina pancreática mais comum, com cerca de quatro casos por milhão de pessoas. O tratamento preferencial dos insulinomas é cirúrgico. OBJETIVO - Analisar as características clínicas, epidemiológicas, cirúrgicas e anatomopatológicas dos pacientes tratados na última década em dois serviços. MÉTODOS - Foram revisados retrospectivamente os prontuários dos pacientes submetidos à cirurgia para tratamento de insulinoma no período de 1999 a 2011. Dados demográficos, tipo e duração dos sintomas, associação ou não à síndrome endócrina e exames diagnósticos foram obtidos dos registros de prontuários. Analisaram-se, também, o método cirúrgico, achados intra-operatórios e complicações imediatas e tardias. RESULTADOS - Dezesseis pacientes com diagnóstico de insulinoma foram submetidos ao tratamento cirúrgico. Foram excluídos dois por falta de registros completos no prontuário. Do total, 68,7% eram mulheres. A idade variou de 20 a 60 anos, com média de 39 anos. Apenas um caso era associado à síndrome de neoplasia endócrina múltipla tipo 1. Sintomas neuropsiquiátricos, principalmente a síncope, foram os mais comuns. A duração média das manifestações clínicas até o diagnóstico foi de um ano e meio. Exames de imagem foram utilizados em todos os pacientes com 68,7% de localização pré-operatória do tumor. Todas as operações foram realizadas de forma convencional (aberta), sem uso da laparoscopia. As lesões foram identificadas em todas as porções do pâncreas sendo a maioria na cabeça pancreática. O alívio dos sintomas só não foi obtido em um paciente. Não houve óbito entre os pacientes analisados. CONCLUSÃO - O diagnóstico de insulinoma é frequentemente estabelecido após vários meses do início das manifestações clínicas e o tratamento cirúrgico é curativo na quase totalidade dos pacientes.

BACKGROUND - Insulinoma is a pancreatic neuroendocrine tumor originated from pancreatic islet beta cells. Although rare, is the most common pancreatic endocrine tumor, with about four cases per million people. The preferential treatment of insulinoma is surgical. AIM - To analyze the epidemiological, pathological, clinical and surgical patients treated in the last decade in two surgical services. METHODS - Were retrospectively reviewed the medical records of patients undergoing surgical treatment of insulinoma in the period of 1999 to 2011. Demographic data, type and duration of symptoms, associated or not with endocrine syndrome and diagnostic tests were obtained from medical records. Were analyzed the method of surgery, intraoperative findings and immediate and late complications. RESULTS - Sixteen patients with insulinoma underwent surgical treatment, 68,7% were women. The age ranged from 20 to 60 years, with a mean age of 39 years. Only one case was associated with multiple endocrine neoplasia type 1. Neuropsychiatric manifestations, mainly syncope , were the most prevalent. The average duration of clinical manifestations until the diagnosis was one year and a half. Imaging tests were used in all patients with 68.7% of preoperative tumor localization. All operations were performed in a conventional (open) manner, without use of laparoscopy. The lesions were identified in all portions of the pancreas with the majority in the pancreatic head. Relief of symptoms was not obtained only in one patient. There were no deaths among the patients. CONCLUSION - The diagnosis of insulinoma is often established after several months of the onset of clinical manifestations and surgical treatment is curative in almost all patients.

Surgical treatment of pancreatic endocrine tumors in multiple endocrine neoplasia type 1

Surgical approaches to pancreatic endocrine tumors associated with multiple endocrine neoplasia type 1 may differ greatly from those applied to sporadic pancreatic endocrine tumors. Presurgical diagnosis of multiple endocrine neoplasia type 1 is therefore crucial to plan a proper intervention. Of note, hyperparathyroidism/multiple endocrine neoplasia type 1 should be surgically treated before pancreatic endocrine tumors/multiple endocrine neoplasia type 1 resection, apart from insulinoma. Non-functioning pancreatic endocrine tumors/multiple endocrine neoplasia type 1 >1 cm have a high risk of malignancy and should be treated by a pancreatic resection associated with lymphadenectomy. The vast majority of patients with gastrinoma/multiple endocrine neoplasia type 1 present with tumor lesions at the duodenum, so the surgery of choice is subtotal or total pancreatoduodenectomy followed by regional lymphadenectomy. The usual surgical treatment for insulinoma/multiple endocrine neoplasia type 1 is distal pancreatectomy up to the mesenteric vein with or without spleen preservation, associated with enucleation of tumor lesions in the pancreatic head. Surgical procedures for glucagonomas, somatostatinomas, and vipomas/ multiple endocrine neoplasia type 1 are similar to those applied to sporadic pancreatic endocrine tumors. Some of these surgical strategies for pancreatic endocrine tumors/multiple endocrine neoplasia type 1 still remain controversial as to their proper extension and timing. Furthermore, surgical resection of single hepatic metastasis secondary to pancreatic endocrine tumors/multiple endocrine neoplasia type 1 may be curative and even in multiple liver metastases surgical resection is possible. Hepatic trans-arterial chemo-embolization is usually associated with surgical resection. Liver transplantation may be needed for select cases. Finally, pre-surgical clinical and genetic diagnosis of multiple endocrine neoplasia type 1 syndrome and localization of multiple endocrine neoplasia type 1related tumors are crucial for determining the best surgical strategies in each individual case with pancreatic endocrine tumors.

Utilidad de la ecografía intraoperatoria para la resección de un insulinoma en la cabeza del páncreas / Usefulness of intraoperative echography for resection of a insulinoma located in the head of pancreas

El insulinoma es la neoplasia más frecuente entre los tumores neuroendocrinos del páncreas. En el presente trabajo se describe el cuadro clínico, los exámenes de laboratorio e imaginológicos y los aspectos quirúrgicos y complicaciones de una paciente con diagnóstico clínico de insulinoma, sin evidencias por imágenes preoperatorias. La cirugía se indicó sobre la base de las evidencias clínicas y analíticas de hipoglucemia e hiperinsulinemia, y la realización de una laparotomía exploradora donde no se encontró lesión alguna con la palpación directa del páncreas. Se utilizó la ecografía intraoperatoria y se localizó un nódulo de 0,8 cm de diámetro en la cabeza de este órgano. Tras la enucleación se presentó, como complicación posoperatoria, una fístula pancreática de bajo gasto que remitió espontáneamente. El diagnóstico histológico fue de insulinoma benigno. La paciente presentó reversión clínica de los síntomas, además de la normalización de los valores de glucemia, comparados con los preoperatorios(AU)

The insulinoma is the more frequent neoplasms among the neuroendocrine tumors of pancreas. The aim of present paper is to describe the clinical picture, laboratory and imaging examinations and the surgical features as well as the complications in a patient diagnosed with insulinoma without evidences by preoperative images. Surgery was prescribed on the base of clinical and analytic evidences of hypoglycemia and hyperinsulinism and the carrying out of an exploratory laparatomy without any lesion with direct palpation of pancreas. Intraoperative echography was used to locate a 0,8 cm nodule in the head of pancreas. After enucleation the only postoperative complication was a low-flow pancreatic fistula with a spontaneous remission. The histological diagnosis was a benign insulinoma. Patient had a clinical reversion of symptoms in addition to normalization of glycemia values, compared to those intraoperative(AU)

Insulinoma: diagnóstico y tratamiento tras 5 años de manejo psiquiátrico / Insulinoma: diagnosis and treatment after 5 years of psychiatric management

El insulinoma es un tumor endocrinológico derivado de las células beta del páncreas, de baja frecuencia y de mayor prevalencia en la 5a década de la vida. Son pequeños, benignos y únicos, en la mayoría de los casos. Presentamos un caso, diagnosticado en el Servicio de Medicina Interna del Hospital Regional de Talca, en una paciente de 38 años de edad con antecedentes de depresión de 5 años de evolución en tratamiento farmacológico, que ingresó para estudio de hipoglicemias de larga data, persistentes, de ayuno. Se objetivaron hipoglicemias de ayuno e insulinemia inapropiadamente alta. TAC abdomen con contraste demostró masa hipervascular en relación al páncreas. Se realizó resección quirúrgica del tumor y el estudio inmunohistoquímico confirmó insulinoma. La evolución postoperatoria fue satisfactoria, con normalización de glicemias y notable mejoría de su cuadro depresivo.

Hepatectomía izquierda por metástasis insulino-secretantes, a los 15 años de la resección de un insulinoma / Left hepatectomy associated with liver metastases, 15 years after resection of a pancreatic insulinoma

La insulinoma se origina en las células de los islotes pancreáticos. Es un tumor poco común que ocurre en aproximadamente 1 a 6 personas por millones de habitantes por año. Predomina en el sexo femenino, entre los 40 y 60 años de edad. Se estima que aproximadamente entre el 6 al 10% son neoplasias malignas. El objetivo del trabajo es la presentación de un paciente con recurrencia del cuadro clínico (hipoglucemia, hiperinsulinismo) asociado a metástasis hepáticas 15 años después de la resección de un insulinoma pancreático...

Insulinoma is a neoplasm that arises from the pancreatic insulin-producing cells. It is a rare tumor that occurs in approximately 1 to 6 persons per million of population per year. Predominates in females, between 40 and 60 years of age. It is estimated that approximately 6 to 10% are malignant. Objective: the presentation of a patient with recurrence of clinical symptoms (hypoglycemia, hyperinsulinism) associated with liver metastases 15 years after resection of a pancretic insulinoma..

Acidose metabólica por hiperglicemia transitória pós enucleação de insulinoma pancreático / Metabolic acidosis secondary to transient hyperglycemia after pancreatic insulinoma enucleation

INTRODUÇÃO: Insulinoma é a neoplasia endócrina mais frequente dos tumores funcionantes do pâncreas. Origina-se a partir das células beta das ilhotas de Langerhans e caracteriza-se pela produção excessiva de insulina, com consequente hipoglicemia. O tratamento de escolha é a remoção cirúrgica da neoplasia. O presente relato tem como objetivo apresentar uma complicação metabólica pouco observada.RELATO DO CASO: Homem de 41 anos de idade há dois anos com tonturas, visão turva e convulsões. Os sintomas estavam bem relacionados com períodos prolongados de jejum e melhoravam com as refeições, e durante um dos episódios foi constatada a presença de hipoglicemia, melhorando os sintomas imediatamente após administração de glicose endovenosa. A pesquisa glicêmica revelou intensa hipoglicemia. Ultrassonografia, tomografia computadorizada e ressonância magnética de abdome não revelaram nenhum tipo de alterações no pâncreas. Com a hipótese diagnóstica de hiperglicemia orgânica por provável insulinoma, o paciente foi submetido à enucleação da lesão. No 5º dia do pós-operatório surgiu fístula pancreática e acidose metabólica com resolução satisfatória. O laudo histopatológico mostrou tumor endócrino de pâncreas de 1,5 cm.CONCLUSÃO: Todo paciente submetido à ressecção de insulinoma pancreático deve realizar o pós-operatório imediato em unidades de terapia intensiva, monitorando de forma rigorosa os níveis de glicemia como prevenção de acidose metabólica.

INTRODUCTION: The insulinoma is the most frequent endocrine neoplasm among the functional pancreatic tumors. It originates in the beta cells of the islets of Langerhans and is characterized by the oversecretion of insulin, leading to hypoglycemia. The treatment of choice is the surgical excision of the tumor. The aim of the present report is to describe a rarely observed metabolic complication.CASE REPORT: The case is presented of a 41-year-old man with a 2-year history of dizziness, blurred vision and seizures. The symptoms were closely related to prolonged fasting and improved with eating; hypoglycemia was found during one of the episodes. Symptoms were relieved immediately after intravenous administration of glucose. Blood glucose workup showed severe hypoglycemia. Abdominal ultrasonography, computed tomography and magnetic resonance imaging did not show any alteration in the pancreas. With the diagnostic hypothesis of organic hypoglycemia from a likely insulinoma, the patient underwent the enucleation of the lesion. On the 5th postoperative day, a pancreatic fistula appeared, as well as metabolic acidosis which resolved satisfactorily. The histopathological report showed a 1.5-cm endocrine pancreatic tumor.CONCLUSION: Every patient submitted to pancreatic insulinoma resection should stay in an intensive care unit during the immediate postoperative period and their glycemic levels must be monitored closely to prevent metabolic acidosis.

Insulinoma tem ocorrência incomum na faixa etária pediátrica: relato de um caso / Insulinoma occurence has been uncommon during childhood: report of a case

Os autores apresentam os achados clínicos, laboratoriais e radiológicos de um paciente de 14 anos. Os testes obtidos após jejum de 18 horas revelaram resultados de glicemia, insulinemia e pepitídeo-C compatíveis com a suspeita de diagnóstico de insulinoma...

Authors report a case of a 14 ys.o patient and his clinical, laboratory and image findings. Blood samples drawn during 18 hours fasting revealed glucose, insulin, and c-peptide serum levels matching the suspect of insulinoma...

Tratamento cirúrgico videolaparoscópico de insulinomas utilizando ultra-sonografia intra-operatória / Laparoscopic surgical treatment of insulinomas with the use of intraoperative ultrasonography

RACIONAL: O insulinoma é o tumor pancreático mais freqüente (50 por cento a 60 por cento), com incidência de 4/1 milhão de pessoas, mais comum em mulheres (2:1) e comportamento benigno quando seu diâmetro for inferior a 2 cm. O diagnóstico é clínico-laboratorial e a cirurgia é o único tratamento com potencial curativo. OBJETIVOS: Relatar a aplicabilidade da videolaparoscopia com ultra-sonografia intra-operatória em cinco casos diagnosticados e tratados cirurgicamente. MÉTODOS: Foram estudados três homens e duas mulheres, idade variando de 20 e 53 anos, com quadro clínico compatível com insulinoma, sendo proposta, após comprovação de imagem, enucleação da lesão por videolaparoscopia, com ultra-sonografia intra-operatória. RESULTADOS: Apresentaram boa evolução pós-operatória e remissão completa da hipoglicemia relacionada ao tumor, com um caso evoluindo com fístula pancreática e outro com pseudocisto pancreático. CONCLUSÃO: O tratamento cirúrgico dos insulinomas é factível e exeqüível por videolaparoscopia com ultra-sonografia intra-operatória e completa ressecção das lesões.

BACKGROUND: Insulinoma are insulin productive tumors originated from the pancreatic beta cells with an incidence of 4/1 million persons. It is more prevalent between the 5th and 6th decade, in women (2:1) and from the endocrine pancreatic tumor is the more frequent (50 percent to 60 percent). Insulinoma behave as a benign tumor when the diameter is inferior to 2 cm. The diagnosis is mainly clinical and laboratorial. Surgery is the unique treatment with potential cure. AIM: The present observation reports the use of simultaneous laparoscopic resection associated to intraoperative ultrasonography. METHODS: Five patients were studied (3 man and 2 women) with age from 20 to 53 years old, clinically diagnosed with insulinoma. After image work out it was proposed nuclear resection of the insulinoma by laparoscopic technique associated to intraoperative ultrasonography. RESULTS: The patients had a complete remission of tumor related hypoglycemia and one patient developed a pancreatic fistula and other a pancreatic pseudocist with good postoperative resolution. CONCLUSIONS: The videolaparoscopic approach for the surgical treatment of insulinoma is feasible. The intraoperative ultrasonography helps to identify the lesions and check their complete resection with low complication rate.

Localización de insulinoma pancreático con ultrasonografía laparoscópica / Location of insulinoma pancreatic with laparoscopic ultrasonography

Se presenta el caso de una paciente de 51 años con cuadro clínico de hipoglicemia por un probable insulinoma pancreático. Los exámenes de laboratorio confirmaron la sospecha de insulinoma, pero los estudios por imágenes no pudieron demostrar el tumor. Bajo estas circunstancias, la paciente tuvo abordaje laparoscópico y con la ayuda del ultrasonido intraoperatorio se localizó el tumor en el proceso uncinado del páncreas y, finalmente, se procedió a la enucleación laparoscópica.

The case of a 51-year-old woman with a clinical history of hipoglicemia causedby a presumed pancreatic insulinoma is reported. Laboratory tests pointed outfor a insulinoma, but imaginologic studies could not locate the tumor. Under this circumstances, the patient was laparoscopically approached. By means of the ultrasononography device the tumor was located at the uncinate process of the pancreas and, eventually treated by laparoscopic enucleation.

Insulinoma induced hypoglycaemia in a Jamaican patient / Hipoglicemia inducida por insulinoma en un paciente de Jamaica

Herein reported is the case of a young woman who had hyperinsulinaemic hypoglycaemia which was biochemically consistent with an insulinoma. Initial imaging was negative and definitive treatment was delayed until repeat imaging localized the tumour several years later. This case demonstrates the importance of clinical judgment and biochemical testing in the diagnosis of insulinoma despite negative imaging.

Aquí se reporta el caso de una joven que presentó hipoglicemia hiperinsulinémica, bioquímicamente concordante con un insulinoma. La imagen inicial fue negativa y el tratamiento fue retardado hasta que mediante la repetición de la técnica de imaginología años más tarde localizó el tumor. Este caso demuestra la importancia de juicio clínico y las pruebas bioquímicas en el diagnóstico del insulinoma, especialmente frente a la obtención de una imagen negativa.

Experiencia clinica sobre 37 casos de insulinoma / Clinical experience in 37 cases of insulinoma

El insulinoma es el tumor neuroendocrino pancreático más frecuente. Se manifiesta por signos adrenérgicos y de neuroglucopenia. Su diagnóstico se confirma documentando la existencia de hipoglucemia junto a una inapropiada secreción de insulina. Desde 1988 fueron estudiados 23 mujeres (48 ± 18 años) y 14 varones (45 ± 19 años) con diagnóstico de insulinoma. La evolución de la enfermedad hasta el diagnósticofue de 2.8 ± 2.1 años. Veintisiete pacientes (73%) presentaron principalmente síntomas de neuroglucopenia, y el 27% refirió síntomas adrenérgicos. El laboratorio mostró glucemia en ayunas 32.4 ± 8.7 mg/dl, insulina (RIA) 38.2 ± 39.7 μU/ml (n=11), insulina (quimioluminiscencia) 23.8 ± 18.1 μU/ml (n=26), péptido C1.15 ± 1.6 nmol/l (n=14). El test de ayuno prolongado fue diagnóstico a las 9.0 ± 5.2 horas (n=21). La localizaciónpreoperatoria fue posible en el 73% por imágenes, arteriografía con estimulación de calcio y/o ecografía intraoperatoria. Once casos fueron operados por laparoscopia, y el resto por vía convencional. El diagnósticofue confirmado por histología e inmunohistoquímica. Veintidos pacientes (61.1%) presentaron insulinomas únicos(16 en cuerpo y cola, 6 en la cabeza y proceso uncinado), 6 eran portadores de insulinomas múltiples, 5 deinsulinomas malignos, 1 de nesidioblastosis del adulto y en 2 casos los hallazgos fueron incaracterísticos. En 3 pacientes se halló un glucagonoma asociado (1 de ellos con NEM1). Una paciente no fue operada recibiendotratamiento con verapamilo, con buena respuesta clínica. El seguimiento postquirúrgico fue de 60.4 ± 59.9 meses

Insulinoma is the most frequent pancreatic islet cell tumor. Clinical manifestations include adrenergic and neuroglycopenic symptoms. Diagnosis isestablished through demonstration of inappropriately elevated insulin serum concentrations in the presence ofhypoglycemia. The aim of this study is to show our experience in the management of insulinoma. Since 1988,23 women (48 ± 18 years) and 14 men (45 ± 19 years) were studied. Seventy three percent of them sufferedmainly from neuroglucopenic symptoms while 27% referred adrenergic signs. Mean duration of symptoms beforediagnosis was 2.7 ± 2 years. Mean fasting serum glucose was 32.4 ± 8.7 mg/dl, insulin 38.2 ± 39.7 μU/ml(RIA, n= 11) or 23.8 ± 18.1 μU/ml (chemoluminescence, n=26) and C-peptide 1.15 ± 1.60 nmol/l (n=14). Twenty one patients developed clinical and/or biochemical hypoglycaemia within 9.0 ± 5.2 hours of supervised fast. Preoperative localization was performed in 73% by imaging techniques, arterial calcium stimulation and/or intraoperative ultrasonography and palpation by the surgeon. Thirty six patients were operated on by conventional surgery in 25, or laparoscopic approach in 11 cases. In 22 patients, a solitary tumor was excised (61.1%). Six cases presented multiple insulinomas. Five patients had malignant insulinomas. In one case, a pattern of nesidioblastosis was found and 2 patients presented unspecific findings. In 3 patients another tumour(glucagonoma) was found (1 of them with MEN 1). One patient was treated with verapamil with good clinicalresponse. Mean postoperative follow up was 60.4 ± 59.9 months

Surgical Experiences of Functioning Neuroendocrine Neoplasm of the Pancreas

We present our surgical experiences with functioning neuroendocrine neoplasms of the pancreas to define its natural history, and to suggest its proper management. From June 1990 to June 2005, patients with diagnosis of functioning neuroendocrine (islet cell) neoplasms of the pancreas were retrospectively reviewed. Fourteen patients (5 men and 9 women) with a median age of 49 years (range, 12 - 68 years) were identified. Twelve patients (86%) had insulinoma, two (14%) had gastrinoma. One (7%) with pancreatic insulinoma was multiple endocrine neoplasia type 1. Intraoperative ultrasound scan (sensitivity, 83%) was the most powerful modality for tumor localization. Fifteen neoplasms with median tumor size 1 cm (range 0-3 cm) were resected. Four insulinomas (26.7%) were located in the head of the pancreas and 5 (36%), in the tail. Another 5 (36%) insulinomas and 1 (7%) gastrinoma were located around the neck area near the SMV or PV. Eleven patients (79%) underwent enucleation, and 2 patients (14%), distal pancreatectomy with splenectomy. 100% of patients with functioning neuroendocrine neoplasms of the pancreas have survived. The overall disease free 10-year survival was found to be about 81%. Exact localization of tumor by intraoperative ultrasound and surgical removal are promising for good prognosis.

Surgical Experiences of Functioning Neuroendocrine Neoplasm of the Pancreas

We present our surgical experiences with functioning neuroendocrine neoplasms of the pancreas to define its natural history, and to suggest its proper management. From June 1990 to June 2005, patients with diagnosis of functioning neuroendocrine (islet cell) neoplasms of the pancreas were retrospectively reviewed. Fourteen patients (5 men and 9 women) with a median age of 49 years (range, 12 - 68 years) were identified. Twelve patients (86%) had insulinoma, two (14%) had gastrinoma. One (7%) with pancreatic insulinoma was multiple endocrine neoplasia type 1. Intraoperative ultrasound scan (sensitivity, 83%) was the most powerful modality for tumor localization. Fifteen neoplasms with median tumor size 1 cm (range 0-3 cm) were resected. Four insulinomas (26.7%) were located in the head of the pancreas and 5 (36%), in the tail. Another 5 (36%) insulinomas and 1 (7%) gastrinoma were located around the neck area near the SMV or PV. Eleven patients (79%) underwent enucleation, and 2 patients (14%), distal pancreatectomy with splenectomy. 100% of patients with functioning neuroendocrine neoplasms of the pancreas have survived. The overall disease free 10-year survival was found to be about 81%. Exact localization of tumor by intraoperative ultrasound and surgical removal are promising for good prognosis.

[Pancreatic insulinoma: diagnostic approach and therapeutic modalities]

The insulinoma is the most frequent neuroendocrine tumor of the pancreas. It is often a benign tumor but can expose to major hypoglycemia. The positive diagnosis is based on biological dosages. The topographic diagnosis can turn out difficult imposing the appeal to invasive explorations. The treatment is surgical and benefits at present from the contribution of the coeliochirurgie