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1.
Rev. argent. dermatol ; 102(4): 11-20, dic. 2021. graf
Article in Spanish | LILACS-Express | LILACS | ID: biblio-1394701

ABSTRACT

RESUMEN 19- Las dermatosis paraneoplásicas son un grupo heterogéneo de manifestaciones cutáneas que tienen fuerte asociación con patología maligna interna. Su patogenia es poco clara y no se conoce su prevalencia exacta. El tratamiento consiste en el abordaje terapéutico de la enfermedad subyacente. La importancia del conocimiento de las mismas radica en la posibilidad de realizar un diagnóstico temprano de una neoplasia. 20- Se presenta el caso clínico de un paciente con dermatosis paraneoplásica asociada a tumor neuroendócrino de páncreas.


ABSTRACT 24- Paraneoplastic dermatoses are an heterogeneous group of cutaneous manifestations that have a strong association with internal malignancy. Their pathogenesis is unclear and prevalence is unknown. Treatment consists of a therapeutic approach to the underlying disease. The importance of recognizingthem lies in the possibility of making an early diagnosis oftheneoplasm. 25- We report theclinical case of a patient with paraneoplastic dermatosis associated with a pancreatic neuroendocrine tumor.

2.
Chinese Journal of Endocrine Surgery ; (6): 322-324, 2021.
Article in Chinese | WPRIM | ID: wpr-907801

ABSTRACT

Because of low incidence, atypical clinical symptom, pancreatic neuroendocrine tumor (pNENs) and autoimmune pancreatitis (AIP) alway have suspected diagnosis and misdiagnosis. This paper aims to improve the diagnosis and treatment of two diseases by a case of pancreatic neuroendocrine tumor with suspected autoimmune pancreatitis.

3.
Chinese Medical Sciences Journal ; (4): 284-294, 2021.
Article in English | WPRIM | ID: wpr-921878

ABSTRACT

Objective To investigate the impact of prior non-pancreatic cancer on the survival outcomes of patients with localized pancreatic neuroendocrine tumors (PanNETs). Methods We reviewed the Surveillance, Epidemiology, and End Results database and selected patients with localized PanNETs diagnosed between 1973 and 2015. We divided the patients into two groups according to the presence or absence of prior non-pancreatic malignancy. Before and after propensity score matching, we compared the clinicopathological characteristics and studied the overall survival and cancer-specific survival. Results A total of 357 (12.9%) of 2778 patients with localized PanNETs had prior cancer. A total of 1211 cases with only a localized PanNET and 133 cases with a localized PanNET and prior cancer had complete data and met the inclusion criteria of the current study. Patients with prior cancer were associated with advanced age (>65 years, 57.9% prior cancer


Subject(s)
Aged , Female , Humans , Male , Multivariate Analysis , Neoplasms, Second Primary , Neuroendocrine Tumors/epidemiology , Pancreatic Neoplasms/epidemiology , Propensity Score
4.
Chinese Medical Sciences Journal ; (4): 218-224, 2021.
Article in English | WPRIM | ID: wpr-921872

ABSTRACT

Objective To establish a nomogram for predicting the distant metastasis risk of pancreatic neuroendocrine tumors (pNETs) in elderly patients. Methods We extracted data of patients with diagnosis of pNETs at age ≥65 years old between 1973 and 2015 from the Surveillance, Epidemiology, and End Results (SEER) database. All eligible patients were divided randomly into a training cohort and validation cohort. Uni- and multivariate logistic regression analyses were performed on the training cohort to identify independent factors for distant metastasis. A nomogram was developed based on the independent risk factors using rms packages of R software, and was validated internally by the training cohort and externally by the validation cohort using C-index and calibration curves. Results A total of 411 elderly patients were identified, of which 260 were assigned to training cohort and 151 to validation cohort. Univariate and multivariate logistic regression analyses indicated the tumor site (body/tail of pancreas: odds ratio [


Subject(s)
Aged , Humans , Neoplasm Staging , Nomograms , Pancreatic Neoplasms , Prognosis , Risk Factors
5.
Chinese Journal of Endocrinology and Metabolism ; (12): 43-50, 2020.
Article in Chinese | WPRIM | ID: wpr-798595

ABSTRACT

Objective@#To improve the understanding of thyrotropin-secreting adenoma in multiple endocrine neoplasia type 1(MEN1) through analyzing the clinical diagnosis and treatment process, as well as outcomes in one case of this disorder.@*Methods@#The clinical manifestations, biochemical and hormone levels, imaging presentations, medical and surgical treatments, and post-operational pathologic findings in the process of diagnosis and treatment of a patient with thyrotropin-secreting adenoma in MEN1 were analyzed. The next generation sequencing followed by Sanger method was used for analyzing MEN1 and related genes. The results were evaluated with online PolyPhen2 and PROVEAN for variation hazard.@*Results@#One 19-year old male patient was diagnosed with hyperthyroidism due to thyrotoxicosis and high level of thyroid hormones(THs) with measurable TSH(2.78 mIU/L) and negative thyrotropin receptor antibody(TRAb). Meanwhile, primary hyperparathyroidism was suggested by hypercalcemia, hypophosphatemia, and elevated intact parathyroid hormone(PTH) level, all the parameters were returned to normal after surgical resection of the mass which was below the left thyroid lobe indicated by ultrasound and 99mTc scan. Thyrotoxicosis remained in spite of one year treatment with antithyroid drug, thyrotropinoma was then suspected, and subsequent MRI scan found a macroadenoma at right pituitary. TSH and THs returned to normal 1 month after transsphenoidal removal of the adenoma. As expected, immunohistochemical staining revealed TSH positive. In addition, a pancreatic mass was found by both CT and MRI scan, which was considered as a silent neuroendocrine tumor. Gene analysis revealed a missense mutation of MEN1 as c. 415C>T and p. His139Tyr(H139Y), which was predicted highly hazard. Only five cases of thyrotropinoma in MEN1 were previously reported.@*Conclusion@#Thyrotropinoma should be cautiously identified from hyperthyroidism to avoid misdiagnosis and mistreatment, and it should keep in mind that thyrotropinoma may be associated with MEN1 though it would be very rare.

6.
Cancer Research and Treatment ; : 1639-1652, 2019.
Article in English | WPRIM | ID: wpr-763197

ABSTRACT

PURPOSE: The 8th edition of the American Joint Committee on Cancer (AJCC) staging system for pancreatic neuroendocrine tumor (PNET) included several significant changes. We aim to evaluate this staging system compared to the 7th edition AJCC staging system and European Neuroendocrine Tumors Society (ENETS) system. MATERIALS AND METHODS: We used Korean nationwide surgery database (2000-2014). Of 972 patients who had undergone surgery for PNET, excluding patients diagnosed with ENETS/World Health Organization 2010 grade 3 (G3), only 472 patients with accurate stage were included. RESULTS: Poor discrimination in overall survival rate (OSR) was noted between AJCC 8th stage III and IV (p=0.180). The disease-free survival (DFS) curves of 8th AJCC classification were well separated between all stages. Compared with stage I, the hazard ratio of II, III, and IV was 3.808, 13.928, and 30.618, respectively (p=0.007, p < 0.001, and p < 0.001). The curves of OSR and DFS of certain prognostic group in AJCC 7th and ENETS overlapped. In ENETS staging system, no significant difference in DFS between stage IIB versus IIIA (p=0.909) and IIIA versus IIIB (p=0.291). In multivariable analysis, lymphovascular invasion (p=0.002), perineural invasion (p=0.003), and grade (p < 0.001) were identified as independent prognostic factors for DFS. CONCLUSION: This is the first large-scale validation of the AJCC 8th edition staging system for PNET. The revised 8th system provides better discrimination compared to that of the 7th edition and ENETS TNM system. This supports the clinical use of the system.


Subject(s)
Humans , Classification , Discrimination, Psychological , Disease-Free Survival , Joints , Neoplasm Staging , Neuroectodermal Tumors, Primitive , Neuroendocrine Tumors , Pancreas , Survival Rate
7.
Chinese Journal of Digestion ; (12): 545-548, 2019.
Article in Chinese | WPRIM | ID: wpr-756307

ABSTRACT

Objective To analyze the relationship between the changes of fasting plasma level of leptin and obesity in patients with insulinoma before operation.Methods From January 2003 to May 2008,40 patients with insulinoma diagnosed at Peking Union Medical College Hospital were selected.Preoperative fasting plasma samples of them were collected.From January 2003 to May 2008,the plasma samples of 28 volunteers matched with age,gender and body weight matched with the patients were collected as the controls.All the subjects were divided into overweight-obesity group and normal weight group according to their body mass index (BMI).Plasma levels of leptin of all the subjects were measured by enzyme linked immunosorbent assay (ELISA).The Mann-Whitney U test and the correlation coefficient test were used for statistical analysis.Results The plasma leptin level of patients with insulinoma was 0.35 ng/mL (0.25 ng/mL to 1.13 ng/mL),which was higher than that of the control group (0.29 ng/mL,0.25 ng/mL to 1.15 ng/mL),and the difference was statistically significant (U =324.50,P =0.003).In the normal-weight group,the plasma leptin level of the patients with insulinoma was 0.35 ng/mL (0.27 ng/mL to 0.62 ng/mL),which was higher than that of the control group (0.28 ng/mL,0.25 ng/mL to 0.37 ng/mL),and the difference was statistically significant (U =28.000,P =0.001).While in the overweight-obesity group,the plasma leptin levels of the patients with insulinoma and the controls were 0.35 ng/mL (0.25 ng/mL to 1.13 ng/mL) and 0.34 ng/mL (0.26 ng/mL to 1.15 ng/mL),respectively,and the difference was not statistically significant (U =153.500,P =0.525).Plasma leptin levels in both the patients with insulinoma and the controls,were correlated with BMI (r =0.355,P =0.025;r =0.571,P =0.001,respectively).Conclusion Preoperative fasting plasma level of leptin increase in patients with insulinoma which is correlated with BMI.

8.
Tianjin Medical Journal ; (12): 755-757, 2018.
Article in Chinese | WPRIM | ID: wpr-810871

ABSTRACT

@#Non-functional pancreatic neuroendocrine tumors (NF-PNETs) are rare in clinic. In this paper, three cases of typical NF-PNETs patients with pathological diagnosis in Tianjin First Central Hospital from July 2012 to February 2017 were analyzed retrospectively. The clinical manifestations, pathological and immunohistochemical features, diagnosis and treatment and prognosis of NF-PNETs were discussed.

9.
Chinese Journal of Digestion ; (12): 187-191, 2018.
Article in Chinese | WPRIM | ID: wpr-711587

ABSTRACT

Objective To investigate the clinical features and prognostic factors of small (maximum diameter≤ 2.0 cm) non-functional pancreatic neuroendocrine tumors (NF-PNET).Methods From January 2002 to April 2017,the clinical data of 18 patients with small NF-PNET were retrospectively analyzed.The prognosis and clinical pathological features were investigated.According to the prognosis,the patients were divided into death group (four cases) and disease-free survival group (14 cases).Fisher's exact test and Mann-Whitney rank inspection were performed for statistical analysis.Results All 18 patients (nine males,nine females) aged 23 to 80 years old,median age 52.5 years old received radical resection.The median follow-up time was 54 months.Four patients died during the follow-up.There was no statistically significant difference in median age (61.0 years,32.0 to 80.0 years vs 49.0 years,23.0 to 72.0 years) and median tumor maximum diameters (1.9 cm,0.8 to 2.0 cm vs 1.5 cm,0.8 to 2.0 cm) between the death group and disease-free survival group (Z=-1.223 and-0.752,P=0.233 and 0.505).Compared with that of disease-free survival group,tumor differentiation degree of the death group was lower (poor/high differentiation,2/2 vs 0/14),and the difference was statistically significant (Fisher's exact test,P=0.039).However,there was no significant difference in histological grades (G1/G2/G3,1/1/2 vs 9/5/0;Fisher's exact test,P=0.057).The distribution of tumor in death group and disease-free survival group was similar.The proportion of patients with tumor at pancreatic head was 2/4 and 6/14 of the death group and disease free survival group,respectively.The surgical methods of the death group included pancreaticoduodenectomy (two cases),distal pancreatectomy (one case),and total pancreatectomy (one case),and there was no statistically significant difference in rates of surgical methods between the two groups (Fisher's exact test,P=0.260).Conclusion Age,tumor maximum diameter,degree of differentiation,histological grade and surgical methods may be correlated with poor prognosis of small NF PNET.

10.
China Oncology ; (12): 321-325, 2017.
Article in Chinese | WPRIM | ID: wpr-618818

ABSTRACT

Pancreatic neuroendocrine tumor is a common pancreatic tumor with high heterogeneity and multiple management modalities. A standard and practical staging system for pancreatic neuroendocrine tumors will be beneficial to clinical management and research. At present, there are two staging systems (ENETS and AJCC). Both of them have shortcomings which limit their clinical application. In addition, the coexistence of two staging systems is confusing to clinicians. We proposed a modified ENETS staging system by keeping the ENETS TNM definition and adopting the AJCC staging definition. The modified staging system can successfully distinguish patients with different prognosis and is helpful in establishing clinical standard. This study has been published in Journal of Clinical Oncology (JCO) and was selected as 2017 Best of JCO: Gastrointestinal edition. This paper was aimed to interpret the modified staging system in clinical practice.

11.
Clinical Endoscopy ; : 126-137, 2017.
Article in English | WPRIM | ID: wpr-195337

ABSTRACT

Development and use of linear-array echoendoscope and endoscopic ultrasound-guided fine needle aspiration (EUS-FNA) have made endoscopic ultrasound (EUS) more of an interventional procedure than a purely diagnostic procedure. This is a literature review of previously published clinical studies on EUS-guided direct intervention for solid pancreatic tumors, including EUS-guided fine needle injection (EUS-FNI) of antitumor agents, EUS-guided fiducial marker placement, EUS-guided brachytherapy and EUS-guided tumor ablation.


Subject(s)
Antineoplastic Agents , Brachytherapy , Endoscopic Ultrasound-Guided Fine Needle Aspiration , Endosonography , Fiducial Markers , Needles , Pancreatic Neoplasms , Ultrasonography
12.
Clinical Endoscopy ; : 537-545, 2017.
Article in English | WPRIM | ID: wpr-58942

ABSTRACT

Pancreatic neuroendocrine tumors (PNETs) are relatively rare; however, the incidence has increased over the last few decades. They are classified as functional or non-functional tumors according to the presence of associated clinical symptoms. The majority are non-functional tumors. For classification and staging, the World Health Organization 2010 classification system is the most commonly accepted. Chromogranin A is the most sensitive marker but has insufficient specificity. In general, PNETs are hypervascular tumors, and multiphasic contrast-enhanced computed tomography is considered the first choice for imaging study. Multiphasic magnetic resonance imaging can detect PNETs smaller than 2 cm and small liver metastasis compared with other modalities. Somatostatin receptor scintigraphy is often used in cases where functional PNETs are suspected. Positron emission tomography (PET) scan with 18F-fluorodeoxyglucose cannot visualize PNETs, but PET with 68-Ga DOTATATE can. Endoscopic ultrasonography can characterize smaller PNETs using contrast and confirm histology through fine needle aspiration or biopsy. In this article, we review the characteristics of grading systems and diagnostic modalities commonly used for PNETs.


Subject(s)
Biopsy , Biopsy, Fine-Needle , Chromogranin A , Classification , Diagnosis , Endosonography , Incidence , Liver , Magnetic Resonance Imaging , Neoplasm Metastasis , Neuroectodermal Tumors, Primitive , Neuroendocrine Tumors , Positron-Emission Tomography , Radionuclide Imaging , Receptors, Somatostatin , Sensitivity and Specificity , World Health Organization
13.
Clinical Endoscopy ; : 546-551, 2017.
Article in English | WPRIM | ID: wpr-58941

ABSTRACT

Pancreatic neuroendocrine tumors (PNETs) are increasingly being detected, though usually as incidental findings. Majority of the PNETs are non-functional and surgical resection is the standard of care for most of them. However, in patients with small PNETs localized within the pancreas, who are unfit or unwilling for surgery, alternate methods of treatment are needed. Direct methods of ablation of PNETs, using either ethanol injection or radiofrequency ablation (RFA), are emerging as effective methods. The limited literature available as case reports or case series on endoscopic ultrasound (EUS)-guided local ablation using either ethanol or RFA has demonstrated safety and efficacy along with short- to medium-term sustained relief. Long-term benefits with these local ablative therapies are awaited. Comparative studies are needed to show which of these two competing technologies is superior. Finally, comparative trials of EUS-guided ablation with surgical resection in terms of efficacy and safety will ensure their place in the management algorithm.


Subject(s)
Humans , Catheter Ablation , Ethanol , Incidental Findings , Needles , Neuroectodermal Tumors, Primitive , Neuroendocrine Tumors , Pancreas , Standard of Care , Ultrasonography
14.
Gastrointestinal Intervention ; : 177-182, 2016.
Article in English | WPRIM | ID: wpr-184919

ABSTRACT

Accurate diagnosis of pancreatic solid lesions is often difficult using conventional imaging modalities. With the recent introduction of contrast-enhanced harmonic endoscopic ultrasound (CEH-EUS), it is now possible to evaluate the microvascular environment and dynamic enhancement of a variety of pancreatic lesions. With CEH-EUS, three patterns of pancreatic lesion enhancement compared with the normal pancreatic tissue (fast, simultaneous, or slow), two washout patterns (fast or slow) and two distribution patterns (homogeneous, inhomogeneous) can be described. By evaluating the microvasculature, enhancement speed, and washout pattern, CEH-EUS may help to differentiate pancreatic adenocarcinoma from other masses and differentiate between pancreatic neuroendocrine tumor (pNET) and inflammatory masses. The finding of a hyperenhancing lesion on CEH–EUS, both with homogeneous and inhomogeneous patterns, was a strong predictor of histology different from adenocarcinoma (94% positive predictive value). pNET was the most common hyperenhancing lesions overall. Although CEH-EUS is useful for ruling out pancreatic ductal adenocarcinoma, making the differential diagnosis between pNETs and pseudotumoral pancreatic masses is difficult because both may share an isovascular or hypervascular appearance. Currently the interpretation of CEH-EUS findings is examiner-dependent. In the future, digital image analysis by image-processing techniques should allow more objective interpretation.


Subject(s)
Adenocarcinoma , Diagnosis , Diagnosis, Differential , Microvessels , Neuroectodermal Tumors, Primitive , Neuroendocrine Tumors , Pancreatic Ducts , Ultrasonography
15.
Cambios rev. méd ; 14(24): 71-73, abr. 2015.
Article in Spanish | LILACS | ID: biblio-1008012

ABSTRACT

Introducción: los insulinomas son tumores neuroendocrinos pancreáticos secretores de insulina, la incidencia en la población en general estimada es de 1 a 4 casos en un millón de habitantes por año. Los insulinomas constituyen el 60% de los tumores de páncreas, de los cuales el 90% son benignos y únicos; el 10% son múltiples, la mayoría asociados a MEN 1 (Neoplasia Endocrina Múltiple) y del 5 al 10% son malignas. La posibilidad de recidiva obliga a un diagnóstico y seguimiento a largo plazo. La característica clínica del insulinoma es la hipoglucemia en ayunas, con signos y síntomas de neuroglucopenia, que pueden estar precedidos o no por manifestaciones adrenérgicas. El tratamiento de elección es el quirúrgico; en etapas irresecables o avanzadas se proponen tratamientos paliativos o sintomáticos con quimioterapia o procedimientos mediante radiología intervencionista con pobres respuestas. Se ha demostrado que con nuevos tratamientos con inhibidores de la tirosina kinasa e inhibidores de la vía de la rapamicina en tumores neuroendocrinos pancráticos bien diferenciados las respuestas tanto en sobrevida libre de progresión así como sobrevida global mejoran importantemente. Caso: presentamos el caso de un hombre de 44 años, con diagnóstico de insulinoma y MEN 1, debutó con convulsiones tónico clónicas generalizadas tratado con pancreatectomia parcial, siete años más tarde, tras haber permanecido asintomático nuevo episodio de convulsiones, documentándose hipoglicemias severas y múltiples metástasis hepáticas, sometido a una pancreatoduodenectomia y como tratamiento sintomático recibió corticoides en altas dosis, diazóxido, octreotide mensual y dos líneas de quimioterapia, además de embolización de las metástasis hepáticas de mayor tamaño y por progresión de enfermedad se inició inhibidores de tirosin-kinasa por un período de tres meses y el paciente fallece.


Introduction: the Insulinomas are pancreatic neuroendocrine tumors secreting insulin. The incidence in the general population is estimated 1-4 per 1 000 000 yearly Insulinoma accounts for 60% of pancreatic tumors, of which 90% are benign and unique, 10% are multiple, mostly associated with MEN 1 (Multiple Endocrine Neoplasia), and 5-10% is malignant. The possibility of recurrence requires diagnosis and long-term monitoring. The clinical characteristic of insulinoma is fasting hypoglycemia, with signs and symptoms of neuroglucopenia, which may be preceded or not by adrenergic manifestations. Surgery is the treatment of choice, in irrsecable; and in advanced stages the proposed palliatives or symptomatic treatments are with chemotherapy or procedures through interventional radiologist with poor responses. It has been shown that new treatments with inhibitors of tyrosine kinase inhibitors and rapamycin pathway for pancreatic neuroendocrine well differentiated tumors, the response in both progression-free survival and overall survival signifcantly improved. Case study: we report the case of a 44 year old male of, diagnosed with insulinoma and MEN 1, debuted with tonic clonic seizures treated with partial pancreatectomy, 7 years later after a new episode of seizures documented severe hypoglycemia and multiple liver metastases, underwent a pancreatoduodenectomy and as symptomatic treatment received high-dose corticosteroids, diaxozido, octreotide monthly and 2 lines of chemotherapy, in addition to embolization of the larger liver metastases masses, and due to disease progression tyrosine kinase inhibitors was initiated for a period of 3 months and the patient dies.


Subject(s)
Humans , Male , Adult , Pancreatectomy , Multiple Endocrine Neoplasia , Neuroendocrine Tumors , Hypoglycemia , Insulinoma , Neoplasm Metastasis , Gastrinoma , Mortality , Adrenal Cortex Hormones
16.
Journal of the ASEAN Federation of Endocrine Societies ; : 160-163, 2015.
Article in English | WPRIM | ID: wpr-998644

ABSTRACT

@#We report the case of a patient with hypoglycemic symptoms and weight gain. Biochemical investigations revealed endogenous hyperinsulinemic hypoglycemia. A CT scan and MRI of the abdomen were initially not successful in localizing a pancreatic mass. However, an endoscopic ultrasound was able to demonstrate a pancreatic head mass. Enucleation of the mass resulted in clinical and biochemical improvement. This report also demonstrates the pattern of weight loss after surgery, showing an initial phase of gradual weight loss followed by a rapid loss of weight. This pattern of weight loss after successful removal of an insulin-producing pancreatic neuroendocrine tumor is a novel addition to the existing knowledge we have about this condition.


Subject(s)
Hypoglycemia , Insulinoma , Weight Loss
17.
Endocrinology and Metabolism ; : 149-152, 2013.
Article in English | WPRIM | ID: wpr-119437

ABSTRACT

We report a rare case of severe hypoglycemia after sunitinib treatment for pancreatic neuroendocrine carcinoma. We describe the initial clinical presentation, laboratory results, pathologic findings, and managment in a patient with a nonfunctioning pancreatic neuroendocrine carcinoma with liver metastases who developed life threatening hypoglycemia after 2 months of sunitinib therapy. A 46-year-old woman presented to the emergency department with loss of consciousness from hypoglycemia. Serum C-peptide and insulin levels at fasting state revealed that the hypoglycemia resulted from endogenous hyperinsulinemia. She had been diagnosed with nonfunctioning pancreatic neuroendocrine carcinoma based on a biopsy of metastatic cervical lymph node and was being treated with sunitinib, a small molecule tyrosine kinase inhibitor. Immunohistochemical stain of the metastatic liver mass demonstrated that the initially nonfunctioning neuroendocrine carcinoma cells had changed into insulin-producing cells after sunitinib therapy. Transarterial chemoembolization of the liver masses and systemic chemotherapy with streptozotocin/adriamycin relieved the hypoglycemia. A nonfunctioning pancreatic neuroendocrine carcinoma was transformed into an insulin-producing tumor after treatment with sunitinib, causing endogenous hyperinsulinemia and severe hypoglycemia.


Subject(s)
Female , Humans , Biopsy , C-Peptide , Carcinoma, Neuroendocrine , Emergencies , Fasting , Hyperinsulinism , Hypoglycemia , Indoles , Insulin , Insulinoma , Liver , Lymph Nodes , Neoplasm Metastasis , Protein-Tyrosine Kinases , Pyrroles , Unconsciousness
18.
Gut and Liver ; : 287-294, 2012.
Article in English | WPRIM | ID: wpr-45076

ABSTRACT

Multiple endocrine neoplasia type 1 (MEN1) is an inherited autosomal dominant disease presenting with pancreatic neuroendocrine tumors (pNETs), parathyroid tumors, or pituitary tumors. Using the PubMed database, we reviewed the literature on information regarding the proper diagnosis and treatment of MEN1-associated pNET. Many cases of MEN1-associated pNET are functioning pNETs. Gastrinomas and insulinomas tend to occur frequently in the duodenum and pancreas, respectively. In addition to diagnostic imaging, the selective arterial secretagogue injection test (SASI test) is useful for localizing functioning pNET. The standard treatment is surgical resection. However, in the case of a functioning pNET, the tumor should first be accurately located using the SASI test before an appropriate surgical method is selected. In cases of a MEN1-associated non-functioning pNET that exceeds 2 cm in diameter, the incidence of distant metastasis is significantly increased, and surgery is recommended. In cases of unresectable pNET, a somatostatin analog has been shown to demonstrate antitumor effects and is considered to be a promising treatment. In addition, molecular-targeted drugs have recently been found to be effective in phase III clinical trials.


Subject(s)
Diagnostic Imaging , Duodenum , Gastrinoma , Incidence , Insulinoma , Multiple Endocrine Neoplasia , Multiple Endocrine Neoplasia Type 1 , Neoplasm Metastasis , Neuroectodermal Tumors, Primitive , Neuroendocrine Tumors , Pancreas , Pituitary Neoplasms , Somatostatin
19.
Yonsei Medical Journal ; : 944-951, 2012.
Article in English | WPRIM | ID: wpr-228777

ABSTRACT

PURPOSE: Pancreatic neuroendocrine tumors (PNET) are a rare subgroup of tumors. For PNETs, the predictive factors for survival and prognosis are not well known. The purpose of our study was to evaluate the predictive factors for survival and disease progression in PNETs. MATERIALS AND METHODS: We retrospectively analyzed 37 patients who were diagnosed with PNET at Severance Hospital between November 2005 and March 2010. Prognostic factors for survival and disease progression were evaluated using the Kaplan-Meier method. RESULTS: The mean age of the patients was 50.0+/-15.0 years. Eight cases (21.6%) were described as functioning tumors and 29 cases (78.4%) as non-functioning tumors. In univariate analysis of clinical factors, patients with liver metastasis (p=0.002), without resection of primary tumors (p=0.002), or American Joint Committee on Cancer/Union for International Cancer Control (AJCC/UICC) stage III/IV (p=0.002) were more likely to demonstrate shorter overall survival (OS). Patients with bile duct or pancreatic duct invasion (p=0.031), sized-lesions larger than 20 mm (p=0.036), liver metastasis (p=0.020), distant metastasis (p=0.005), lymph node metastasis (p=0.009) or without resection of primary tumors (p=0.020) were more likely to demonstrate shorter progression-free survival (PFS). In multivariate analysis of clinical factors, bile duct or pancreatic duct invasion [p=0.010, hazard ratio (HR)=95.046] and tumor location (non-head of pancreas) (p=0.036, HR=7.381) were confirmed as independent factors for predicting shorter PFS. CONCLUSION: Patients with liver metastasis or without resection of primary tumors were more likely to demonstrate shorter OS. Patients with bile duct or pancreatic duct invasion or tumors located at body or tail of pancreas were more likely to demonstrate shorter PFS.


Subject(s)
Humans , Bile Ducts , Disease Progression , Disease-Free Survival , Joints , Liver , Lymph Nodes , Methods , Multivariate Analysis , Neoplasm Metastasis , Neuroectodermal Tumors, Primitive , Neuroendocrine Tumors , Pancreas , Pancreatic Ducts , Prognosis , Retrospective Studies , Tail
20.
Rev. chil. endocrinol. diabetes ; 4(3): 201-204, jul. 2011. tab, ilus
Article in Spanish | LILACS | ID: lil-640640

ABSTRACT

We report a 28 years old woman consulting for weight gain, progressive muscle weakness and appearance of facial plethora. Laboratory showed high cortisol and ACTH levels. Magnetic resonance of sella turcica was normal and an abdominal magnetic resonance showed a pancreatic mass, suggestive of a neuroendocrine tumor. A PET/CT with somatostatin analogues demonstrated an intense over-expression of somatostatin receptors in the tumor. The patient was subjected to a pancreatectomy and the day after surgery, ACTH levels decreased to less than 5 pg/ml. The patological study of the surgical piece showed a neuroendocrine carcinoma. The patient had a good postoperative evolution.


Subject(s)
Humans , Adult , Female , Organometallic Compounds , Pancreatic Neoplasms , Cushing Syndrome/etiology , Somatostatin/analogs & derivatives , Somatostatin , Neuroendocrine Tumors , Laparoscopy , Pancreatic Neoplasms/surgery , Pancreatectomy , Positron-Emission Tomography , Tomography, X-Ray Computed , Treatment Outcome , Neuroendocrine Tumors/surgery
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