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1.
Medicentro (Villa Clara) ; 26(4): 995-1001, oct.-dic. 2022.
Article in Spanish | LILACS | ID: biblio-1405688

ABSTRACT

RESUMEN El insulinoma es un tumor poco frecuente de las células de los islotes pancreáticos, caracterizado por la proliferación de las células betas. Son tumores neuroendocrinos pancreáticos, generalmente benignos y de pequeño tamaño, caracterizados por el síndrome de hipersecreción de insulina con el desarrollo de hipoglucemia. La mayoría de estos tumores son esporádicos, solo alrededor de 15-30 % es hereditario; está asociado a enfermedades como la neoplasia endocrina múltiple tipo 1, o el síndrome de Von Hippel-Lindau. La incidencia de dicho tumor es de 3-10 casos por cada millón de habitantes. La edad media de aparición es entre 40-50 años de edad y es más frecuente en mujeres. El reconocimiento temprano y el diagnóstico adecuado de este tumor pancreático raro, son aspectos importantes pues es potencialmente curable con un tratamiento quirúrgico oportuno.


ABSTRACT Insulinoma is a rare pancreatic islet cell tumor characterized by proliferation of beta cells. They are pancreatic neuroendocrine tumors, generally benign and small in size, characterized by insulin hypersecretion syndrome with the development of hypoglycemia. Most of these tumors are sporadic, only about 15-30% are hereditary; it is associated with diseases such as multiple endocrine neoplasia type 1, or Von Hippel-Lindau syndrome. The incidence of this tumor is 3-10 cases per million inhabitants. The average age of onset is between 40-50 years of age and it is more frequent in women. Early recognition and proper diagnosis of this rare pancreatic tumor are important aspects, as it is potentially curable with timely surgical treatment.


Subject(s)
Adenoma, Islet Cell/surgery , Surgical Procedures, Operative
2.
Rev. guatemalteca cir ; 27(1): 3-9, 2021. ilus, tab
Article in Spanish | LILACS, LIGCSA | ID: biblio-1381549

ABSTRACT

La operación de Whipple es el procedimiento quirúrgico de mayor complejidad en cirugía abdominal, este se realiza en pacientes con tumores de la encrucijada pancreatoduodenal, es la única alternativa para tratamiento curativo en fases tempranas de la enfermedad. Objetivo: Determinar los resultados del procedimiento Whipple, en pacientes intervenidos con tumores de la encrucijada pancreatoduodenal en el Hospital General de Enfermedades del Instituto Guatemalteco de Seguridad Social, en el periodo de enero 2,015 a enero 2,020. Método: Descriptivo, observacional, retrospectivo. Resultados: Se incluyeron 42 procedimientos de Whipple, 29 (69%) casos del género masculino y 13 (31%) para el género femenino. La edad media fue de 61.5 años, el 54% presentaban comorbilidad asociada. El 24% utilizo transfusión transoperatoria de hemoderivados, el tiempo quirúrgico de 5.5 horas. La reintervención fue del 4.7%. Complicaciones postoperatorias tempranas 18%. La histología más común fue el carcinoma de cabeza de páncreas en el 43%. La mortalidad postoperatoria temprana fue del 4.7%. El OR de complicaciones asociadas a comorbilidades fue de 1.7 con un IC 0.3046-7.20 y un valor de P: 0.9251 que no es estadísticamente significativo. Conclusiones: Los tumores pancreatoduodenales en nuestra población se presentan en edades más tempranas a lo reportado. La morbimortalidad es similar a lo reportado en otros estudios a nivel latinoamericano, sin embargo las complicaciones están más elevadas que las mejores series internacionales. No existe asociación entre el riesgo de complicaciones con comorbilidades del paciente. (AU)


Whipple operation is the most complex surgical procedure in abdominal surgery, it's performed in patients with tumors of the pancreaticoduodenal crossroads, it is the only alternative for curative treatment in early stages of the disease. Objective: To determine the results of the Whipple procedure in patients operated on with tumors of the ancreaticoduodenal crossroads at the Hospital General de Enfermedades del IGSS in the period from January 2015 to January 2020. Method: Descriptive, observational, retrospective. Results: fourtytwo Whipple procedures were included, 29 (69%) cases of the male gender and 13 (31%) for the female gender. The mean age was 61.5 years, 54% had associated comorbidity. Twentyfour percent used intraoperative transfusion of blood products and surgical time of 5.5 hours. Reoperation was 4.7% with early postoperative complications of 18%. The most common histology was carcinoma of the head of the pancreas in 43%. Early postoperative mortality was 4.7%. The OR of complications associated with comorbidities was 1.7 with a CI 0.3046-7.20 and a P value: 0.9251, which is not statistically significant. Conclusions: Pancreaticoduodenal tumors in our population present at an earlier age than reported. Morbidity and mortality is similar to that reported in other studies. There is no association between the risk of complications with patient comorbidities. (AU)


Subject(s)
Humans , Male , Female , Adult , Middle Aged , Aged , Aged, 80 and over , Pancreas/pathology , Pancreaticoduodenectomy/mortality , Adenoma, Islet Cell/surgery , Pancreatitis/surgery , Postoperative Complications/diagnosis
3.
Korean Journal of Pancreas and Biliary Tract ; : 105-110, 2014.
Article in Korean | WPRIM | ID: wpr-121872

ABSTRACT

A blastoma is a type of cancer, which is common in children; it is caused by malignancies derived from in the precursor cells, often called blasts. Examples are nephroblastomas, retinoblastomas, pleuropulmonary blastomas, and pancreatoblastomas. Pancreatoblastomas are extremely rarely in adults. It is difficult preoperatively to distinguish this tumor from other pancreatic tumors including solid and papillary epithelial neoplasm of the pancreas (SPEN), acinar cell carcinoma, islet cell tumor, and ductal adenocarcinoma with cystic degeneration. To our knowledge, this case may be the second report of a pancreatoblastoma occurring in an adult in Korea. We report a case of a pancreatoblastoma that was confirmed by pathology, despite the radiologic finding that assumed it was a SPEN.


Subject(s)
Adult , Child , Humans , Adenocarcinoma , Adenoma, Islet Cell , Carcinoma, Acinar Cell , Korea , Neoplasms, Glandular and Epithelial , Pancreas , Pathology , Retinoblastoma , Wilms Tumor
5.
Chinese Journal of Oncology ; (12): 540-542, 2013.
Article in Chinese | WPRIM | ID: wpr-267503

ABSTRACT

<p><b>OBJECTIVE</b>To investigate the diagnosis and treatment of pancreatic islet cell tumors.</p><p><b>METHODS</b>Fifty-one patients with islet cell tumors treated in our department from January 1991 to April 2011 were included in this study. The data of clinical features, diagnosis and treatment were retrospectively analyzed.</p><p><b>RESULTS</b>Among the 51 cases, 38 cases showed typical Whipple's triad, and the other 13 cases were non-functional islet cell tumors. In these 13 cases, 5 patients had no specific clinical symptoms, and 8 patients had abdominal distending pain. The positive rates of imaging were: B-ultrasound 43.1%, multi-slice spiral CT 69.8%; MRI 62.5%, endoscopic ultrasonography (EUS) 64.7% (11/17), and intraoperative ultrasound (IOUS) 96.3%, the differences among them were statistically significant (P<0.05). All patients underwent surgical treatment. Postoperative pancreatic leakage happened in 6 cases. Finally all the patients recovered after effective external drainage, anti-infection treatment and nutritional support.</p><p><b>CONCLUSIONS</b>Intraoperative ultrasonography (IOUS) has a higher accuracy in the diagnosis of pancreatic islet cell tumors, compared with preoperative B-ultrasonography, CT, MRI, and endoscopic ultrasound (EUS). The most effective treatment of this disease is surgery.</p>


Subject(s)
Adult , Female , Humans , Male , Middle Aged , Adenoma, Islet Cell , Diagnosis , Diagnostic Imaging , General Surgery , Anastomotic Leak , Endosonography , Insulinoma , Diagnosis , Diagnostic Imaging , General Surgery , Magnetic Resonance Imaging , Multidetector Computed Tomography , Pancreatectomy , Pancreatic Neoplasms , Diagnosis , Diagnostic Imaging , General Surgery , Retrospective Studies
6.
Korean Journal of Endocrine Surgery ; : 99-105, 2010.
Article in Korean | WPRIM | ID: wpr-73488

ABSTRACT

PURPOSE: Insulinoma is a rare disease for which early diagnosis followed by proper surgical management provides a chance for cure. Analyses of clinicopathological features of patients can help optimize the surgical approach in the treatment of insulinoma. METHODS: The records of 13 patients (seven male, six female mean age 44.3 years; age range 17~62 years) who were diagnosed clinically and pathologically with insulinoma and who underwent surgery between March 1997 and April 2007 at the Department of Surgery, Seoul National University Hospital. Hospital in English please were retrospectively examined. RESULTS: All patients had findings compatible with Whipple's triad. Mean fasting blood sugar was 40.5 mg/dl, serum insulin level was 33.5µU/ml, and insulin-to-glucose ratio was 0.6. A prolonged starvation test was performed on six patients. Tumors were localized in 10 patients with a computed tomography (CT) scan and in three patients with CT angiography. Five tumors were located in the pancreas head and uncinate process, five in the body, and four at the body-tail border and tail. Patients underwent resection of tumorby enucleation, distal pancreatectomy, pylorus-preserving pancreaticoduodenectomy, laparoscopic distal pancreatectomy, and duodenum-preserving resection of pancreas head. Four immediate postoperative complications (fluid collection, pancreatic fistula, delayed gastric emptying) occurred. No symptoms or recurrences were apparent during the median 15 month follow-up. CONCLUSION: Insulinoma is difficult to diagnose correctly without a prolonged duration of symptoms. Localization of insulinoma can be aided by a CT scan and/or CT angiography. Less aggressive operative procedures such as simple enucleation might be a sufficient and feasible procedure for curative resection of benign insulinomas.


Subject(s)
Female , Humans , Male , Adenoma, Islet Cell , Angiography , Blood Glucose , Early Diagnosis , Fasting , Follow-Up Studies , Head , Insulin , Insulinoma , Pancreas , Pancreatectomy , Pancreatic Fistula , Pancreaticoduodenectomy , Postoperative Complications , Rare Diseases , Recurrence , Retrospective Studies , Seoul , Starvation , Surgical Procedures, Operative , Tail , Tomography, X-Ray Computed
7.
Journal of the Korean Society of Magnetic Resonance in Medicine ; : 145-150, 2010.
Article in English | WPRIM | ID: wpr-198470

ABSTRACT

We report the case of a 16-year-old boy with a solid pancreatic mass which proved to be a nonfunctioning, malignant pancreatic neuroendocrine tumor (PNET). In pediatric patients, malignant pancreatic tumors are rare, especially malignant PNET. When dynamic contrast enhanced MRI showed a well enhancing solid pancreatic tumor on arterial and delayed phases and combined with malignant features, such as vascular invasion, invasion of adjascent organs, and lymphadenopathy, we should include malignant pancreatic neuroendocrine tumor in the differential diagnosis of childhood pancreatic tumors.


Subject(s)
Adolescent , Child , Humans , Adenoma, Islet Cell , Diagnosis, Differential , Lymphatic Diseases , Neuroectodermal Tumors, Primitive , Neuroendocrine Tumors
8.
Chinese Journal of Surgery ; (12): 326-328, 2009.
Article in Chinese | WPRIM | ID: wpr-238901

ABSTRACT

<p><b>OBJECTIVE</b>To evaluate the methods of diagnosis and surgical treatment for nonfunctional islet cell tumor (NICT).</p><p><b>METHODS</b>Forty-four patients with non-functional islet cell tumor treated at the First Affiliated Hospital of Nanjing Medical University during January 1968 to June 2008 were analyzed retrospectively. There were 9 males and 35 females, aged from 7- to 70-years-old. Clinical manifestation: 15 cases (34.1%) of abdominal masses, 17 patients (38.6%) with epigastric or back pain, 5 cases of jaundice, 5 cases (11.4%) for upper abdominal fullness or vomiting, 10 cases (22.7%) of pancreatic tumor noticed by routine health checkups or imaging examinations. Imaging examination: CT scan, sonography, ERCP, MRI, upper GI series were performed in 33 (75.0%), 16 (36.4%), 6 (13.6%), 2 (4.5%), and 10 cases (22.7%) respectively. Operation methods: 39 patients (88.6%) underwent surgical resection and the other 5 patients did not.</p><p><b></b>RESULTS</p><p><b>COMPLICATIONS</b>pancreatic fistula in 7 patients (15.9%), intra-abdominal bleeding in 4 (9.1%), gastrojejunal anastomosis outlet obstruction in 1 (2.3%), biliary fistula in 2 (4.5%) and incisional infection in 3 (6.8%). Surgery related mortality happened in 2 patients (4.5%), both treated before 1999. Twenty-five patients underwent operation between January 1999 and June 2008 were followed up for 6 to 108 months. All survive except one died 75 months after the surgery for unknown reason.</p><p><b>CONCLUSIONS</b>No specific clinical manifestation is recognized for non-functional islet cell tumor. Spiral CT is an optimal diagnostic method, while surgery is the first choice for treatment. Middle segmental pancreatectomy has become an alternative surgical protocol for NICT.</p>


Subject(s)
Adolescent , Adult , Aged , Child , Female , Humans , Male , Middle Aged , Young Adult , Adenoma, Islet Cell , Diagnosis , General Surgery , Follow-Up Studies , Pancreatectomy , Methods , Pancreatic Neoplasms , Diagnosis , General Surgery , Prognosis , Retrospective Studies
9.
The Korean Journal of Gastroenterology ; : 171-178, 2008.
Article in Korean | WPRIM | ID: wpr-28354

ABSTRACT

BACKGROUND/AIMS: Since pancreatic endocrine tumors (PET) are rare and heterogeneous diseases, their survival and prognosis are not well known. Due to recent advances in CT/MRI technology, incidentalomas of the pancreas are detected with increasing frequency. This study presents results of clinical manifestations of PET and predictive factors associated with survival. METHODS: From year 1990 through 2006, medical records of 98 patients (56 men, 42 women) who were diagnosed as PET pathologically at Seoul National University Hospital were reviewed retrospectively. RESULTS: Ages ranged from 17 to 76 years (mean 51.6+/-1.3 years) with a mean follow-up of 3.6+/-0.4 years (range 0-10.1 years). Overall 5-year survival rate was 68.1%, and 5-year survival rate of the patients who had distant metastases at initial diagnosis was 43.9%. Functioning tumors [hazard ratio (HR) 0.229, 95% confidence interval (CI) 0.056-0.943, p=0.041] and lymph node or liver metastases (HR 5.537, 95% CI 2.106-14.555, p<0.001) were the significant prognostic factors associated with survival rate. However, tumor size and pathology showed no significant association with survival. CONCLUSIONS: Because small and pathologically benign nature do not predict good prognosis in PET, aggressive treatment such as curative resection would be considered initially even in the case of incidental PET.


Subject(s)
Adolescent , Adult , Aged , Female , Humans , Male , Middle Aged , Adenoma, Islet Cell/diagnosis , Combined Modality Therapy , Follow-Up Studies , Liver Neoplasms/diagnosis , Lymph Nodes/pathology , Multivariate Analysis , Pancreatic Neoplasms/diagnosis , Predictive Value of Tests , Prognosis , Retrospective Studies , Survival Rate , Tomography, X-Ray Computed , Treatment Outcome
10.
Chinese Journal of Surgery ; (12): 1768-1770, 2008.
Article in Chinese | WPRIM | ID: wpr-275952

ABSTRACT

<p><b>OBJECTIVE</b>To summarize the surgical technique and clinical experience of total laparoscopic resection of the pancreatic islet cell tumors.</p><p><b>METHODS</b>From July 2002 to December 2007, 30 cases including 12 males and 18 females were diagnosed as pancreatic islet cell tumor. There were at least one positive imaging examination of each patient preoperatively. The location of the tumors included 4 in proximal pancreas and 26 in distal pancreas.</p><p><b>RESULTS</b>The tumors were successfully found and removed in 28 cases and unsuccessfully located in the other 2 cases. The procedures included local resections in 13 cases and distal pancreatectomies in 15 cases (spleen reserved in 7 cases). The mean operation time was 165 min (range, 65 - 465 min). The mean blood loss was 145 ml (range, 50 - 800 ml). Pancreatic leakage occurred in 3 cases, 2 of which were cured conservatively. And the other one were cured by endoscopic retrograde cannulation of the pancreatic duct. The mean postoperative hospital stay was 5.6 days (range, 2 - 17 d). There were no conversions and death. After follow up of (14.3 +/- 16.7) months (range, 4 - 62 months), there were no recurrences.</p><p><b>CONCLUSION</b>Total laparoscopic resection is a safe and effective method for pancreatic islet cell tumors.</p>


Subject(s)
Adult , Aged , Female , Humans , Male , Middle Aged , Adenoma, Islet Cell , General Surgery , Follow-Up Studies , Laparoscopy , Pancreatectomy , Methods , Pancreatic Neoplasms , General Surgery , Retrospective Studies , Treatment Outcome
11.
Chinese Journal of Oncology ; (12): 457-460, 2007.
Article in Chinese | WPRIM | ID: wpr-298576

ABSTRACT

<p><b>OBJECTIVE</b>To analyze the clinical and pathological features in order to investigate appropriate way of diagnosis and treatment for non-functional islet cell tumors of the pancreas (NFICT).</p><p><b>METHODS</b>The data and experience of surgically treated 43 patients with pathologically confirmed NFICT over the last 30 years were retrospectively reviewed. The survival rate was estimated using Kaplan-Meier method and the potential risk factors affecting survival were compared with Log rank test.</p><p><b>RESULTS</b>There were 7 males and 36 females in this series with a mean age of 31.6 years ranged from 8 to 67 years. Twenty-eight patients were diagnosed as having non-functional islet cell carcinomas of the pancreas (NFICC) and 15 patients benign islet cell tumors. The most common symptoms in NFICT were abdominal pain 55.8%, nausea and/or vomiting (32.6%), fatigue (25.6%) and abdominal mass (23.3%). Preoperatively, all of those were found to have a mass in their pancrease by ultrasonic and computed tomography examination, with 21 in the head, 10 in the body and 6 in the tail of the pancreas. Multicemtric tumor were found in one patient. Thirty-nine of these 43 patients (90.7%) underwent surgical resection, with a curative resection in 30 (69.8%) and palliative in 9 (20.9%). The resectability and curative resection rate in 28 patients with nonfunctioning islet cell carcinomas of the pancreas was 78.6% and 60.7%, respectively. None of the 15 patients with benign nonfunctioning islet cell tumor of the pancreas died of this disease. While the overall cumulative 5- and 10-year survival rate in 28 patients with non-functional islet cell carcinomas of the pancreas was only 58.1% and 29.0%, respectively. Curative resection, female, younger than 30 years old and mass diameter < 10 cm were found to be positive prognostic factors. But multivariate Cox regression analysis indicated that radical resection was the only independent prognostic factor (P = 0.007).</p><p><b>CONCLUSION</b>Nonfunctioning islet cell tumor of the pancreas is frequently found in young female. Surgical resection, especially curative resection can achieve satisfactory long-term survival.</p>


Subject(s)
Adolescent , Adult , Aged , Child , Female , Humans , Male , Middle Aged , Adenoma, Islet Cell , Diagnosis , Therapeutics , Antineoplastic Combined Chemotherapy Protocols , Therapeutic Uses , Carcinoma, Islet Cell , Diagnosis , Therapeutics , Chemotherapy, Adjuvant , Methods , Combined Modality Therapy , Doxorubicin , Therapeutic Uses , Fluorouracil , Therapeutic Uses , Kaplan-Meier Estimate , Mitomycin , Therapeutic Uses , Multivariate Analysis , Pancreatic Neoplasms , Diagnosis , Therapeutics , Pancreaticoduodenectomy , Methods , Proportional Hazards Models , Regression Analysis , Retrospective Studies
12.
Korean Journal of Hepato-Biliary-Pancreatic Surgery ; : 41-46, 2006.
Article in Korean | WPRIM | ID: wpr-112619

ABSTRACT

Neuroendocrine pancreatic tumors (NPTs) arise from the pancreatic islet cells and belong to the amine and precursor uptake and decarboxylation (APUD) system. These tumors are rare and account for only 1% to 5% of pancreatic tumor. The pancreas is an extremely uncommon site of neoplasia in children and adolescents. For this reason, our understanding of these tumors is still quite limited. Although the complete surgical resection is the key to successful management of all malignant adolescence pancreatic tumors, the information on the possible role of chemotherapy and radiation in recurrent, unresectable, or metastatic cases is purely anecdotal. The 17-year-old man transferred to our hospital with abnormal ultrasonographic findings. Result of abdominal ultrasonographic examination showed a mass in the upper abdomen. He presented with 6months history of intermittent abdominal pain and vomiting and diarrhea. A computed tomography (CT) scan and magnetic resonance showed a 4.5 x 6 cm mass in the head of the pancreas. An ultrasound-guided core biopsy confirmed an pancreatoblastoma or pancreas islet cell tumor. On operative findings, there was locally advanced, unresectable tumor within the pancreatic head. We report a 17-years-old man patient with non-functional panceratic islet cell carcinoma.


Subject(s)
Adolescent , Child , Humans , Abdomen , Abdominal Pain , Adenoma, Islet Cell , Biopsy , Carcinoma, Islet Cell , Decarboxylation , Diarrhea , Drug Therapy , Head , Islets of Langerhans , Pancreas , Vomiting
13.
Journal of the Korean Surgical Society ; : 69-72, 2006.
Article in English | WPRIM | ID: wpr-58822

ABSTRACT

Adenosquamous carcinoma of the pancreas is a rare tumor; its clinical features and radiologic findings are not well known, so the definite preoperative diagnosis of this tumor is quite difficult. We report a case of adenosquamous carcinoma of the pancreas. A 47-year-old woman was admitted on April 2005 to our hospital with a complaint of left upper quadrant pain. The serum CA 19-9 and CEA level were increased up to 4019.08 U/ml and 13.13 ng/ml, respectively. An abdominal computed tomographic (CT) revealed a 10x9 cm sized well-defined homogenous lobulated mass in the pancreas tail. Under the impression of solid-pseudopapillary neoplasm (SPN) or nonfunctioning islet cell tumor, distal pancreatectomy and splenectomy with wedge resection of stomach were performed. The specimen consisted of a huge pancreatic mass that was attached to the posterior wall of stomach. The cut surface of pancreatic mass revealed a huge whitish infiltrative mass. Microscopically, glandular elements of ductal adenocarcinoma were admixed with solid squamoid complexes or squamous cell carcinoma components. Although the origin of the adenosquamous carcinoma has not been well proven, the histopathological findings of our case support the theory of squamous metaplasia.


Subject(s)
Female , Humans , Middle Aged , Adenocarcinoma , Adenoma, Islet Cell , Carcinoma, Adenosquamous , Carcinoma, Squamous Cell , Diagnosis , Metaplasia , Pancreas , Pancreatectomy , Splenectomy , Stomach
14.
Arq. bras. endocrinol. metab ; 49(5): 735-746, out. 2005. tab
Article in Portuguese | LILACS | ID: lil-419975

ABSTRACT

As síndromes de neoplasias endócrinas múltiplas (NEM) incluem as do tipo 1 (MEN 1) e 2 (MEN 2), a síndrome de von Hippel-Lindau, neurofibromatose tipo 1 e o complexo de Carney. Estas são síndromes genéticas complexas decorrentes de ativação ou inativação de diferentes tipos de genes envolvidos na regulação da proliferação celular. Nesta revisão, discutiremos as manifestações clínicas e o acompanhamento da MEN 1, assim como o rastreamento genético de potenciais portadores de alterações no gene MEN 1. A MEN 1 inclui o desenvolvimento de hiperparatiroidismo primário multifocal, tumores de ilhotas pancreáticas e adenomas de hipófise. Além disso, alguns pacientes podem apresentar manifestações cutâneas como angiofibromas e colagenomas e ainda podem desenvolver outras neoplasias como tumores carcinóides, tumores de tiróide, adenomas de adrenal, lipomas, feocromocitomas e meningiomas. A MEN 1 é uma síndrome hereditária, transmitida de forma autossômica dominante e causada por mutação inativadora do gene MEN 1. O gene MEN 1 codifica uma proteína denominada "menin", que é um gene supressor tumoral. Vários estudos demonstraram sua importância na regulação da proliferação celular e confirmaram seu papel na patogênese da MEN 1. A identificação do gene MEN 1 e sua análise genética resultaram na possibilidade de monitoração de pacientes que ainda não apresentam manifestações clínicas associadas a esta síndrome e diagnóstico precoce e tratamento dos pacientes afetados. Tais medidas poderão implicar em sobrevida maior para estes pacientes. Estudos adicionais visando uma melhor compreensão da função e dos mecanismos de sinalização da proteína "menin" poderão propiciar alternativas terapêuticas para os pacientes que evoluem com malignização de tumores relacionados à MEN 1, podendo resultar em maior sobrevida.


Subject(s)
Humans , Multiple Endocrine Neoplasia Type 1 , Mutation/genetics , Proto-Oncogene Proteins/genetics , Adenoma, Islet Cell/diagnosis , Adenoma, Islet Cell/genetics , Adenoma, Islet Cell/therapy , Genetic Testing , Hyperparathyroidism, Primary/diagnosis , Hyperparathyroidism, Primary/genetics , Hyperparathyroidism, Primary/therapy , Multiple Endocrine Neoplasia Type 1/diagnosis , Multiple Endocrine Neoplasia Type 1/genetics , Multiple Endocrine Neoplasia Type 1/therapy , Pancreatic Neoplasms/diagnosis , Pancreatic Neoplasms/genetics , Pancreatic Neoplasms/therapy , Pituitary Neoplasms/diagnosis , Pituitary Neoplasms/genetics , Pituitary Neoplasms/therapy
15.
Article in English | IMSEAR | ID: sea-65267

ABSTRACT

A 7-year-old boy with mixed exocrine-endocrine pancreatic cancer is presented. This may be the second reported case of such a tumor in childhood.


Subject(s)
Adenoma, Islet Cell/epidemiology , Carcinoma, Acinar Cell/epidemiology , Child , Humans , Male , Pancreatic Neoplasms/epidemiology
16.
Journal of the Korean Surgical Society ; : 482-484, 2005.
Article in Korean | WPRIM | ID: wpr-90625

ABSTRACT

A 66-year old presented with hypercalcemia with a 6 month duration. She was a MEN-I patient with a pancreas islet cell tumor and a pituitary adenoma. The clinical examination revealed no mass lesion of the neck. The serum calcium was elevated. Parathyroid scintigraphy showed a high uptake of 4 parathyroid glands that were compatible with hyperparathyroidism. During the operation, five parathyroid glands were identified, two at the right side and three at the left. All five glands were found to have hyperplasia by the pathologist. She underwent subtotal parathyroidectomy. She was discharged on the 8th post operative day and has been followed up without any evidence of recurrence for 36 months.


Subject(s)
Aged , Humans , Adenoma, Islet Cell , Calcium , Hypercalcemia , Hyperparathyroidism , Hyperplasia , Multiple Endocrine Neoplasia Type 1 , Neck , Pancreas , Parathyroid Glands , Parathyroidectomy , Pituitary Neoplasms , Radionuclide Imaging , Recurrence
17.
Journal of the Korean Radiological Society ; : 395-399, 2005.
Article in English | WPRIM | ID: wpr-176367

ABSTRACT

It is difficult to distinguish an islet tumor originating from heterotopic pancreas tissue from the other submucosal tumors. Although the malignant transformation of a heterotopic pancreas, including islet cell tumor, is extremely rare, it remains an important consideration in the differential diagnosis of duodenal submucosal masses. We have demonstrated the radiologic appearance and the clinical-pathologic findings of a highlighted, rare case of islet cell tumor arising from a heterotopic pancreas in the duodenal wall.


Subject(s)
Adenoma, Islet Cell , Diagnosis, Differential , Duodenum , Islets of Langerhans , Pancreas
18.
Korean Journal of Anesthesiology ; : 112-116, 2005.
Article in Korean | WPRIM | ID: wpr-187602

ABSTRACT

Insulinomas are the most common type of islet cell tumor. Generally, these tumors are benign (90%), intrapancreatic, solitary, and small. Moreover, hyperinsulinemia in infants and children can result in permanent damage to the central nervous system. Thus, early diagnosis and treatment are important. The principal challenge during anesthesia is the avoidance of hypoglycemia, which may occur during tumor manipulation, though hyperglycemia may follow after successful surgical removal of the tumor. Because symptoms of hypoglycemia (systemic hypertension, tachycardia, diaphoresis) may be masked during anesthesia, it is important to monitor blood glucose levels frequently during the perioperative period. Here we report up on the case of a 3 year old female with insulinoma. We monitored blood glucose levels intermittently (about every 15 minutes) using a glucometer (Medisense Optium, Abbott, USA). No hypoglycemic episode occurred during anesthesia. We report this clinical experience and review anesthetic choices and the management of this patient.


Subject(s)
Child , Child, Preschool , Female , Humans , Infant , Adenoma, Islet Cell , Anesthesia , Blood Glucose , Central Nervous System , Early Diagnosis , Hyperglycemia , Hyperinsulinism , Hypertension , Hypoglycemia , Insulinoma , Isoflurane , Masks , Pancreas , Perioperative Period , Tachycardia
19.
Korean Journal of Medicine ; : 428-433, 2005.
Article in Korean | WPRIM | ID: wpr-66018

ABSTRACT

Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant tumor syndrome. Thymic carcinoid tumors in MEN1 are not common and their natural history is little known. But development of thymic carcinoid tumors is important because in 1993, they were identified as a frequent case of death. There has not been a report of case in Korea so far. We encountered a case of thymic carcinoid in MEN1. A 42 year old man was referred presenting with diabetes of 12 years duration. Abnormal findings in his blood chemistry were hypercalcemia and hyperprolactinemia. 99mTc- sestamibi scintigraphy showed parathyroid adenoma and hyperplasia. Sella MRI showed pituitary macroadenoma. Abnormal CT scan demonstrated multiple pancreas islet cell tumors, bilateral adrenal tumor and thymoma. Subtotal parathyroidectomy with thymectomy was perfomed and thymic carcinoid was confirmed. This is the first report of thymic carcinoid with MEN1 in Korea.


Subject(s)
Adult , Humans , Adenoma, Islet Cell , Carcinoid Tumor , Chemistry , Hypercalcemia , Hyperplasia , Hyperprolactinemia , Korea , Magnetic Resonance Imaging , Multiple Endocrine Neoplasia Type 1 , Multiple Endocrine Neoplasia , Natural History , Pancreas , Parathyroid Neoplasms , Parathyroidectomy , Radionuclide Imaging , Thymectomy , Thymoma , Tomography, X-Ray Computed
20.
Bol. Asoc. Méd. P. R ; 96(1): 33-38, Jan.-Feb. 2004.
Article in English | LILACS | ID: lil-411111

ABSTRACT

Insulinoma is the most common endocrine tumor of the pancreas. Over 90 of the insulinomas are benign and single, and can be cured by simple excision. Depending on the location, insulinomas can be enucleated, might require partial or distal pancreatectomy or pancreaticoduodenectomy. Five cases with insulinoma successfully treated by surgical intervention, two by enuclation, two by distal pancreatectomy and splenectomy, and preservation of the spleen have been summarized. The management of insulinoma involves the diagnosis, localization of the tumor and treatment. Insulinomas are rare tumors of the pancreas. Nevertheless, it is the most common endocrine tumor of the pancreas. Specifically arising from the beta cells, of the islets of Langerham, that produce insulin (fig.1). Its incidence is one in 250,000 inhabitants. It can be seen at any age, but is more frequent in females between 4 and 82 years of age, with a mean of 45.5 years. Insulinomas are evenly distributed between the head, body and tail of the pancreas. Over 90 are benign and single and can be cured by simple excision. Depending on the location insulinomas can be enucleated, might require partial or distal pancreatectomy or a pancreaticoduodenectomy. Ten percent could be malignant when metastasis to peripancreatic lymph nodes or to the liver is detected. The course of the patient with malignant insulinoma is an indolent one. The release of insulin leads to fasting hypoglycemia producing confusion, loss of consciousness, coma or convulsions. The hypoglycemia in turn can induce the release of cathecolamines producing tachycardia, tremulousness and diaphoresis. The Whipple's triad must be present for the diagnosis of insulinoma; symptoms of hypoglycemia, glucose level below 50 mgs/dl and relief of symptoms by the administration of glucose. In large series the interval between the onset of symptoms and a definitive diagnosis of insulinoma was 37 months, with a range of 0 to 14 years


Subject(s)
Humans , Male , Female , Child, Preschool , Child , Adolescent , Adult , Middle Aged , Insulinoma/surgery , Pancreatic Neoplasms/surgery , Pancreatectomy , Adenoma, Islet Cell , Postoperative Complications/etiology , Diabetes Mellitus, Type 2 , Hyperinsulinism/etiology , Hypoglycemia/etiology , Insulinoma/complications , Insulinoma/diagnosis , Insulinoma/epidemiology , Pancreatic Neoplasms/complications , Pancreatic Neoplasms/diagnosis , Pancreatic Neoplasms/epidemiology , Pancreaticoduodenectomy , Pancreatic Pseudocyst/etiology , Splenectomy
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