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1.
Ann Surg Oncol ; 31(3): 1509-1518, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-38062290

RESUMO

Gastric neuroendocrine tumors (G-NET) are rare tumors arising from enterochromaffin-like cells of the gastric mucosa. They belong to a larger group called gastroenteropancreatic neuroendocrine tumors and are classified as low, intermediate, or high-grade tumors based on their proliferative indices. They are further categorized into three subtypes based on their morphologic characteristics, pathogenesis, and behavior. Types 1 and 2 tumors are characterized by elevated serum gastrin and are usually multifocal. They typically occur in the setting of atrophic gastritis or MEN1/Zollinger Ellison syndrome, respectively. Type 2 tumors are associated with the most symptoms, such as abdominal pain and diarrhea. Type 3 tumors are associated with normal serum gastrin, are usually solitary, and occur sporadically. This type has the most aggressive phenotype and metastatic potential. Treatment and prognosis for G-NET is dependent on their type, size, and stage. Type 1 has the best prognosis, and Type 3 has the worst. This review discusses the presentation, workup, and surgical management of these tumors.


Assuntos
Tumores Neuroendócrinos , Neoplasias Pancreáticas , Neoplasias Gástricas , Síndrome de Zollinger-Ellison , Humanos , Gastrinas , Tumores Neuroendócrinos/patologia , Síndrome de Zollinger-Ellison/patologia , Neoplasias Pancreáticas/cirurgia , Mucosa Gástrica/patologia , Neoplasias Gástricas/patologia
2.
Front Endocrinol (Lausanne) ; 14: 1221514, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-37867522

RESUMO

Background: Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant disorder characterized by the occurrence of multiple epithelial neuroendocrine tumors (NETs) and non-NETs in various organs. MEN1 encodes a 610-amino acid-long tumor suppressor protein, menin. The optimal treatment for multiple tumors, identification of the most critical tumors for patient prognosis, and menin immunohistochemistry findings remain controversial. Therefore, we aimed to elucidate these issues through a histological analysis of tumors and tumor-like lesions in a Japanese family, comprising a father and his two sons, who had MEN1 with Zollinger-Ellison syndrome (ZES). Patients and methods: All family members had a germline alteration in exon 10, c.1714-1715 del TC of MEN1, and exhibited multiple synchronous and metachronous tumors. The patients had pulmonary NETs, hyperparathyroidism, hypergastrinemia, pituitary adenomas, pancreaticoduodenal NETs, adrenocortical adenoma with myelolipoma, nodular goiter of the thyroid, lipomas, and angiofibroma. Most tumors were resected and histologically examined. We compared their clinical courses and tumor histology, and conducted menin immunohistochemistry (IHC). Results: Two patients died of pulmonary NET G2. One patient who underwent pancreaticoduodenectomy was cured of ZES; however, the two other patients who did not undergo pancreaticoduodenectomy suffered persistent ZES despite treatment with octreotide. Menin IHC revealed varying NET intensities, ranging from positive to negative stains. Conclusion: Pancreaticoduodenectomy is the most effective treatment for ZES. Long-term follow-up is essential for pulmonary NET G2 owing to the risk of distant metastasis and/or multiplicity. Moreover, the variability of menin IHC in MEN1-related tumors may indicate the pattern of tumor formation rather than the diagnostic utility of menin in MEN1.


Assuntos
Neoplasia Endócrina Múltipla Tipo 1 , Tumores Neuroendócrinos , Síndrome de Zollinger-Ellison , Humanos , População do Leste Asiático , Imuno-Histoquímica , Neoplasia Endócrina Múltipla Tipo 1/genética , Neoplasia Endócrina Múltipla Tipo 1/cirurgia , Neoplasia Endócrina Múltipla Tipo 1/diagnóstico , Fatores de Transcrição , Síndrome de Zollinger-Ellison/diagnóstico , Síndrome de Zollinger-Ellison/genética , Síndrome de Zollinger-Ellison/patologia
5.
Surg Today ; 53(12): 1325-1334, 2023 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-36473964

RESUMO

In patients with multiple endocrine neoplasia type 1 syndrome (MEN 1) and Zollinger-Ellison syndrome (ZES), gastrinomas arise from the duodenum, about 60% are multiple, and about 15% of patients have coexisting pancreatic gastrinomas, which can be localized by the selective arterial secretagogue injection test (SASI test). The guidelines (GLs) by the Japanese Neuroendocrine Tumor Society (JNETS) recommend surgical resection for functioning duodenopancreatic neuroendocrine tumors (NETs), including gastrinomas, in patients with MEN1 (Grade A, 100% agreement among members). Conversely, the GLs of the National Comprehensive Cancer Network (NCCN) in the USA recommend observation and treatment with proton pump inhibitors (PPIs) or exploratory surgery for occult gastrinomas. An international Consensus Statement (ICS) from the European Union (EU) also does not recommend resection of gastrinomas in patients with MEN1, despite some surgeons having reported surgery being curative for gastrinomas in MEN1 patients. In this review, we discuss the serious side effects and tumorigenic effects of the prolonged use of PPIs and the safety and curability of surgery, supported by our results of curative surgery for gastrinomas in 20 patients with MEN1 over 30 years. We conclude that surgery should be the first-line treatment for gastrinomas in MEN1 patients.


Assuntos
Gastrinoma , Neoplasia Endócrina Múltipla Tipo 1 , Tumores Neuroendócrinos , Neoplasias Pancreáticas , Síndrome de Zollinger-Ellison , Humanos , Gastrinoma/cirurgia , Gastrinoma/patologia , Neoplasia Endócrina Múltipla , Neoplasia Endócrina Múltipla Tipo 1/complicações , Neoplasia Endócrina Múltipla Tipo 1/cirurgia , Neoplasia Endócrina Múltipla Tipo 1/patologia , Tumores Neuroendócrinos/cirurgia , Tumores Neuroendócrinos/patologia , Neoplasias Pancreáticas/cirurgia , Neoplasias Pancreáticas/patologia , Inibidores da Bomba de Prótons , Síndrome de Zollinger-Ellison/cirurgia , Síndrome de Zollinger-Ellison/patologia
6.
Rev Gastroenterol Peru ; 42(2): 122-125, 2022.
Artigo em Espanhol | MEDLINE | ID: mdl-36513358

RESUMO

Gastrinomas are neuroendocrine tumors usually located in the duodenum and pancreas, in the context of a Multiple Endocrine Neoplasm and forming a Zollinger-Ellison syndrome. The location of this type of lymph node tumor is extremely unusual and its early diagnosis constitutes a real challenge to be able to establish an adequate treatment and manage the complications that these entail. We present the case of a 64-year-old male patient with a lymph node gastrinoma and whose surgical removal resulted in the immediate remission of the patient's symptoms.


Assuntos
Gastrinoma , Neoplasia Endócrina Múltipla , Neoplasias Pancreáticas , Síndrome de Zollinger-Ellison , Masculino , Humanos , Pessoa de Meia-Idade , Gastrinoma/diagnóstico , Gastrinoma/cirurgia , Gastrinoma/patologia , Neoplasias Pancreáticas/diagnóstico , Neoplasias Pancreáticas/cirurgia , Neoplasias Pancreáticas/patologia , Síndrome de Zollinger-Ellison/diagnóstico , Síndrome de Zollinger-Ellison/patologia , Síndrome de Zollinger-Ellison/cirurgia , Neoplasia Endócrina Múltipla/patologia , Linfonodos/patologia
7.
Pancreas ; 51(5): 559-562, 2022 05 01.
Artigo em Inglês | MEDLINE | ID: mdl-35973017

RESUMO

ABSTRACT: Neurofibromatosis type (NF-1) is an autosomal dominant disorder characterized predominantly by neurocutaneous manifestations. Involvement of the gastrointestinal tract is uncommon but is associated with a significant risk of malignancy. There are a handful of case reports linking NF-1 with pancreatic neuroendocrine tumors; these include gastrin-secreting variants with the attendant Zollinger-Ellison syndrome. We present the case of a 52-year-old lady who presented with recurrent peptic ulceration and diarrhea. Serum gastrin levels were elevated and magnetic resonance imaging demonstrated the presence of a pancreatic lesion with multiple liver metastases. The lesion was moderately fludeoxyglucose avid on positron emission tomography-computed tomography. Endoscopic ultrasonography-guided sampling revealed the presence of synaptophysin positive neuroendocrine cells with positive gastrin immunostaining. A conservative approach was adopted, and the patient's symptoms improved on proton pump inhibitors. Zollinger-Ellison syndrome is an important condition, which should be kept in mind in the patient with NF-1 who presents with recurrent peptic ulceration and diarrhea. The emerging association between these 2 conditions is being examined on a cellular and immunohistochemical level.


Assuntos
Gastrinoma , Tumores Neuroendócrinos , Neurofibromatose 1 , Neoplasias Pancreáticas , Úlcera Péptica , Síndrome de Zollinger-Ellison , Diarreia , Feminino , Gastrinoma/complicações , Gastrinas , Humanos , Pessoa de Meia-Idade , Tumores Neuroendócrinos/complicações , Tumores Neuroendócrinos/genética , Neurofibromatose 1/complicações , Neurofibromatose 1/diagnóstico , Neurofibromatose 1/genética , Neoplasias Pancreáticas/diagnóstico , Síndrome de Zollinger-Ellison/complicações , Síndrome de Zollinger-Ellison/diagnóstico , Síndrome de Zollinger-Ellison/patologia
8.
Rev Esp Enferm Dig ; 114(8): 496-497, 2022 08.
Artigo em Inglês | MEDLINE | ID: mdl-35255699

RESUMO

Zollinger Ellison syndrome is an unusual entity. This termn is used to describe the clinical manifestations of a gastrin-synthesizing neoplasm. Gastrinomas occur mainly in the duodenum and pancreas. Primary gastrinomas are rarely found in other intra-abdominal sites, such as the ovary, bile ducts, spleen or kidney, or even more unusual in extra-abdominal locations. Several studies provide strong evidence that gastrinomas can also occur in the lymph nodes. However, the existence of primary lymph node gastrinomas is controversial.


Assuntos
Gastrinoma , Tumores do Estroma Gastrointestinal , Neoplasias Pancreáticas , Síndrome de Zollinger-Ellison , Feminino , Tumores do Estroma Gastrointestinal/diagnóstico por imagem , Tumores do Estroma Gastrointestinal/patologia , Tumores do Estroma Gastrointestinal/cirurgia , Humanos , Pâncreas/patologia , Neoplasias Pancreáticas/diagnóstico por imagem , Neoplasias Pancreáticas/patologia , Neoplasias Pancreáticas/cirurgia , Síndrome de Zollinger-Ellison/patologia
9.
J Clin Endocrinol Metab ; 107(5): e2110-e2123, 2022 04 19.
Artigo em Inglês | MEDLINE | ID: mdl-34922358

RESUMO

CONTEXT: Multiple endocrine neoplasia type 1 (MEN1) is a rare inherited endocrine cancer syndrome. Multiple gastro-entero-pancreatic neuroendocrine tumors (GEP-NETs) affect 30% to 80% of MEN1 patients, with the most common functioning GEP-NET being gastrinoma. Biochemical identification of hypergastrinemia may help to recognize the presence of gastrinomas before they are detectable by instrumental screening, enabling early diagnosis and start of therapy, preferably before tumor progression and metastases occurrence. OBJECTIVE: Evaluate the effectiveness of secretin stimulation test to precociously diagnose the presence of gastrin-secreting tumors. DESIGN: Results of secretin stimulation tests, performed between 1991 and February 2020, were retrospectively analyzed, as aggregate, in a cohort of MEN1 patients with GEP-NETs. SETTING: Data were extracted from the MEN1 Florentine database. PATIENTS: The study included 72 MEN1 patients with GEP-NETs who underwent a secretin stimulation test for the evaluation of gastrin secretion. OUTCOMES: A positive secretin stimulation test was assumed with a difference between basal fasting serum gastrin (FSG) and the maximum stimulated value of gastrin over 120 pg/mL. RESULTS: The secretin stimulation test showed a secretin-induced hypergastrinemia in 27.8% (20/72) of patients with GEP-NETs, and a positive test in 18 cases. The test allowed the identification of a positively stimulated hypergastrinemia in 75.0% (3/4) of patients who presented a basal FSG within the normal range. CONCLUSIONS: Diagnosis of gastrinoma is complex, difficult, and controversial. Results of this study confirm that a positive secretin stimulation test allows early diagnosis of gastrinomas, even in the presence of borderline or normal levels of nonstimulated FSG.


Assuntos
Gastrinoma , Neoplasia Endócrina Múltipla Tipo 1 , Neoplasias Pancreáticas , Síndrome de Zollinger-Ellison , Detecção Precoce de Câncer , Gastrinoma/diagnóstico , Gastrinoma/patologia , Gastrinas , Humanos , Neoplasia Endócrina Múltipla Tipo 1/patologia , Neoplasias Pancreáticas/diagnóstico , Neoplasias Pancreáticas/patologia , Estudos Retrospectivos , Secretina , Síndrome de Zollinger-Ellison/diagnóstico , Síndrome de Zollinger-Ellison/patologia
11.
World J Surg Oncol ; 17(1): 213, 2019 Dec 09.
Artigo em Inglês | MEDLINE | ID: mdl-31818296

RESUMO

BACKGROUND: Zollinger-Ellison syndrome (ZES) is a rare condition characterized by hypersecretion of gastrin by gastrinoma tumors leading to severe peptic ulcer disease with potential development of gastric carcinoid tumors. Herein, we report the clinical course of a 68-year-old patient with multiple endocrine neoplasia type 1 (MEN-1) who underwent several surgeries to ultimately undergo optimal tumor cytoreduction of locally advanced gastrinomas and symptomatic gastric carcinoids. The patient was subsequently maintained on octreotide long-acting release (LAR). This case report supports consideration for aggressive tumor cytoreduction and octreotide in similar patients with MEN-1-associated ZES for durable disease control and symptom management. CASE PRESENTATION: The patient is a 68-year-old male with multiple endocrine neoplasia type 1 (MEN-1), diagnosed in 1993 after presenting with recurrent renal calculi and hypercalcemia. Soon thereafter, he presented with symptoms and elevated gastrin levels suggestive of ZES prompting abdominal exploration with partial resection of the duodenum to remove gastrinoma tumor nodules. Within 4 years of the operation, he represented with intractable hypergastrinemia despite optimal medical management with peak gastrin levels exceeding 29,000 pg/mL, in 2006. In January 2007, the patient returned to the operating room for resection of regional peripancreatic and perigastric lymph nodes and enucleation of pancreatic body and tail gastrinoma tumors. Although his gastrin level decreased to 5000 pg/mL with resultant improvement of symptoms, in less than 2 years, he developed disease progression with obstructive symptomatology from enlarging gastric carcinoids and rising gastrin levels. In May of 2008, he underwent pancreaticoduodenectomy and near-total gastrectomy. Since June of 2008, the patient shows no demonstrable progression of disease and remains asymptomatic on LAR octreotide (30 mgs). Gastrin levels have been well controlled (range, 100-624 pg/mL; current 114 pg/mL). CONCLUSION: Success of this procedure in our case report highlights the potential role for optimal tumor cytoreduction and LAR octreotide to control disease progression in a patient with MEN-I and Zollinger-Ellison syndrome with locally advanced gastrinoma and secondary large gastric carcinoids.


Assuntos
Antineoplásicos Hormonais/uso terapêutico , Procedimentos Cirúrgicos de Citorredução/métodos , Neoplasia Endócrina Múltipla Tipo 1/terapia , Octreotida/uso terapêutico , Síndrome de Zollinger-Ellison/terapia , Idoso , Terapia Combinada , Humanos , Masculino , Neoplasia Endócrina Múltipla Tipo 1/complicações , Neoplasia Endócrina Múltipla Tipo 1/patologia , Prognóstico , Síndrome de Zollinger-Ellison/complicações , Síndrome de Zollinger-Ellison/patologia
13.
World J Gastroenterol ; 25(32): 4673-4681, 2019 Aug 28.
Artigo em Inglês | MEDLINE | ID: mdl-31528093

RESUMO

Zollinger-Ellison syndrome (ZES) is characterized by gastric acid hypersecretion causing severe recurrent acid-related peptic disease. Excessive secretion of gastrin can now be effectively controlled with powerful proton pump inhibitors, but surgical management to control gastrinoma itself remains controversial. Based on a thorough literature review, we design a surgical algorithm for ZES and list some significant consensus findings and recommendations: (1) For sporadic ZES, surgery should be routinely undertaken as early as possible not only for patients with a precisely localized diagnosis but also for those with negative imaging findings. The surgical approach for sporadic ZES depends on the lesion location (including the duodenum, pancreas, lymph nodes, hepatobiliary tract, stomach, and some extremely rare sites such as the ovaries, heart, omentum, and jejunum). Intraoperative liver exploration and lymphadenectomy should be routinely performed; (2) For multiple endocrine neoplasia type 1-related ZES (MEN1/ZES), surgery should not be performed routinely except for lesions > 2 cm. An attempt to perform radical resection (pancreaticoduodenectomy followed by lymphadenectomy) can be made. The ameliorating effect of parathyroid surgery should be considered, and parathyroidectomy should be performed first before any abdominal surgery for ZES; and (3) For hepatic metastatic disease, hepatic resection should be routinely performed. Currently, liver transplantation is still considered an investigational therapeutic approach for ZES. Well-designed prospective studies are desperately needed to further verify and modify the current considerations.


Assuntos
Gastroenterologia/normas , Oncologia/normas , Guias de Prática Clínica como Assunto , Síndrome de Zollinger-Ellison/cirurgia , Duodeno/citologia , Duodeno/patologia , Duodeno/cirurgia , Células Secretoras de Gastrina/patologia , Gastrinas/metabolismo , Gastroenterologia/métodos , Hepatectomia , Humanos , Fígado/citologia , Fígado/patologia , Fígado/cirurgia , Excisão de Linfonodo , Oncologia/métodos , Pâncreas/citologia , Pâncreas/patologia , Pâncreas/cirurgia , Pancreaticoduodenectomia , Paratireoidectomia , Estômago/citologia , Estômago/patologia , Estômago/cirurgia , Fatores de Tempo , Síndrome de Zollinger-Ellison/patologia
15.
Ann Surg ; 267(4): 782-788, 2018 04.
Artigo em Inglês | MEDLINE | ID: mdl-29517561

RESUMO

OBJECTIVE: To determine the role of reoperation in patients with persistent or recurrent Zollinger-Ellison Syndrome (ZES). BACKGROUND: Approximately, 0% to 60% of ZES patients are disease-free (DF) after an initial operation, but the tumor may recur. METHODS: A prospective database was queried. RESULTS: A total of 223 patients had an initial operation for possible cure of ZES and then were subsequently evaluated serially with cross sectional imaging-computed tomography, magnetic resonance imaging, ultrasound, more recently octreoscan-and functional studies for ZES activity. The mean age at first surgery was 49 years and with an 11-year mean follow-up 52 patients (23%) underwent reoperation when ZES recurred with imageable disease. Results in this group are analyzed in the current report. Reoperation occurred on a mean of 6 years after the initial surgery with a mean number of reoperations of 1 (range 1-5). After reoperation 18/52 patients were initially DF (35%); and after a mean follow-up of 8 years, 13/52 remained DF (25%). During follow-up, 9/52 reoperated patients (17%) died, of whom 7 patients died a disease-related death (13%). The overall survival from first surgery was 84% at 20 years and 68% at 30 years. Multiple endocrine neoplasia type 1 status did not affect survival, but DF interval and liver metastases did. CONCLUSIONS: These results demonstrate that a significant proportion of patients with ZES will develop resectable persistent or recurrent disease after an initial operation. These patients generally have prolonged survival after reoperation and 25% can be cured with repeat surgery, suggesting all ZES patients postresection should have systematic imaging, and if tumor recurs, advise repeat operation.


Assuntos
Reoperação , Síndrome de Zollinger-Ellison/cirurgia , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Seguimentos , Humanos , Neoplasias Hepáticas/secundário , Masculino , Pessoa de Meia-Idade , Imagem Multimodal , Estudos Prospectivos , Recidiva , Análise de Sobrevida , Resultado do Tratamento , Síndrome de Zollinger-Ellison/diagnóstico por imagem , Síndrome de Zollinger-Ellison/patologia
16.
Endocrine ; 60(1): 15-27, 2018 04.
Artigo em Inglês | MEDLINE | ID: mdl-29019150

RESUMO

PURPOSE: Analyze the role of somatostatin analogues (SSAs) in the treatment of sporadic and MEN1-related gastrinomas, trying to define whether recent trials have changed the landscape of gastrinoma therapy. METHODS: We evaluate the rationale of SSA use in the treatment of gastrinomas, summarize the current literature concerning the effect of SSAs on the control of Zollinger-Ellison syndrome (ZES) and gastrinomas tumor progression and discuss their role in the most recent guidelines. RESULTS: The medical treatment of gastrinoma and related ZES is aimed at controlling acid hypersecretion and tumor progression, in inoperable patients. The use of proton pump inhibitors (PPIs) to control the syndrome is a cornerstone in the ZES therapy. SSAs are not usually indicated for antisecretory purpose, because PPIs are considered the treatment of choice, due to their long lasting high efficacy and oral availability. The antiproliferative effect of SSAs has been established by two placebo-controlled trials that have clearly demonstrated a significant increase in progression free survival in patients affected by non-functioning well-differentiated advanced neuroendocrine tumors (NETs). The recent ENETS guidelines recommend the use of SSAs in advanced well differentiated NETs as antiproliferative agents. CONCLUSIONS: The high sstr-expression in gastrinomas make them highly responsive to SSAs and support the use of such drugs to counteract the tumour growth in patients not amenable to surgical cure. Unfortunately, limited data, mainly case reports or small series, support the use of SSAs in advanced gastrinomas, therefore, it is difficult to quantify their ability to control tumour growth and disease progression.


Assuntos
Antineoplásicos Hormonais/uso terapêutico , Gastrinoma/tratamento farmacológico , Neoplasias Pancreáticas/tratamento farmacológico , Somatostatina/análogos & derivados , Síndrome de Zollinger-Ellison/tratamento farmacológico , Gastrinoma/patologia , Humanos , Octreotida/uso terapêutico , Neoplasias Pancreáticas/patologia , Inibidores da Bomba de Prótons/uso terapêutico , Resultado do Tratamento , Síndrome de Zollinger-Ellison/patologia
18.
Chirurg ; 87(4): 271-9, 2016 Apr.
Artigo em Alemão | MEDLINE | ID: mdl-26960437

RESUMO

Neuroendocrine neoplasms (NEN) represent a group of potentially malignant tumors, which can be located in every section of the gastrointestinal tract, the pancreas and the bronchopulmonary system. Gastroduodenal NENs have a relatively good prognosis in comparison to other subentities, e.g. pancreatic or ileojejunal NENs. In the stomach there are four different types of NENs, while in the duodenum there are five types and all vary in the malignant potential and the therapeutic approach. Due to the simple access endoscopic methods not only have diagnostic but also important therapeutic relevance in this subgroup. Lesions smaller than 1 cm can easily be resected with forceps or snare resection and for larger lesions up to 2 cm more invasive strategies, such as endoscopic mucosal resection (EMR) or endoscopic submucosal dissection (ESD) are available. Important criteria in gastric NEN for the risk evaluation of endoscopic treatment alone are the size of the lesion, depth of invasion and the tumor biology, e.g. neuroendocrine tumor (NET) G1/G2 versus neuroendocrine carcinoma (NEC) G3. In duodenal NEN the aforementioned risk factors also apply and in addition only lesions outside the ampulla of Vater should be endoscopically resected whereas periampullary lesions need to be addressed surgically. As an individualized therapeutic approach the possibility of a combined endoscopic and laparoscopic resection technique exists. Follow-up endoscopic investigations are necessary, especially in gastric type 1 NENs, which have a tendency to relapse.


Assuntos
Neoplasias Duodenais/patologia , Neoplasias Duodenais/cirurgia , Duodenoscopia/métodos , Gastroscopia/métodos , Neoplasias Gástricas/patologia , Neoplasias Gástricas/cirurgia , Humanos , Gradação de Tumores , Invasividade Neoplásica , Estadiamento de Neoplasias , Prognóstico , Fatores de Risco , Síndrome de Zollinger-Ellison/patologia , Síndrome de Zollinger-Ellison/cirurgia
19.
World J Gastroenterol ; 19(45): 8312-20, 2013 Dec 07.
Artigo em Inglês | MEDLINE | ID: mdl-24363522

RESUMO

AIM: To describe our patients affected with ectopic biliary tree gastrinoma and review the literature on this topic. METHODS: Between January 1992 and June 2012, 28 patients affected by duodenopancreatic endocrine tumors in multiple endocrine neoplasia type 1 (MEN1) syndrome underwent surgery at our institution. This retrospective review article analyzes our experience regarding seventeen of these patients subjected to duodenopancreatic surgery for Zollinger-Ellison syndrome (ZES). Surgical treatment consisted of duodenopancreatectomy (DP) or total pancreatectomy (TP). Regional lymphadenectomy was always performed. Any hepatic tumoral lesions found were removed during surgery. In MEN1 patients, removal of duodenal lesions can sometimes lead to persistence or recurrence of hypergastrinemia. One possible explanation for this unfavorable outcome could be unrecognized ectopic localization of gastrin-secreting tumors. This study described three cases among the seventeen patients who were found to have an ectopic gastrinoma located in the biliary tree. RESULTS: Seventeen MEN1 patients affected with ZES were analyzed. The mean age was 40 years. Fifteen patients underwent DP and two TP. On histopathological examination, duodeno pancreatic endocrine tumors were found in all 17 patients. Eighty-one gastrinomas were detected in the first three portions of the duodenum. Only one gastrinoma was found in the pancreas. The mean number of gastrinomas per patient was 5 (range 1-16). Malignancy was established in 12 patients (70.5%) after lymph node, liver and omental metastases were found. Three patients exhibited biliary tree gastrinomas as well as duodenal gastrinoma(s). In two cases, the ectopic gastrinoma was removed at the same time as pancreatic surgery, while in the third case, the biliary tree gastrinoma was resected one year after DP because of recurrence of ZES. CONCLUSION: These findings suggest the importance of checking for the presence of ectopic gastrinomas in the biliary tree in MEN1 patients undergoing ZES surgery.


Assuntos
Neoplasias dos Ductos Biliares/patologia , Neoplasias Duodenais/patologia , Gastrinoma/patologia , Neoplasia Endócrina Múltipla Tipo 1/patologia , Neoplasias Pancreáticas/patologia , Síndrome de Zollinger-Ellison/patologia , Adulto , Neoplasias dos Ductos Biliares/cirurgia , Biópsia , Neoplasias Duodenais/cirurgia , Feminino , Gastrinoma/cirurgia , Humanos , Excisão de Linfonodo , Masculino , Pessoa de Meia-Idade , Neoplasia Endócrina Múltipla Tipo 1/cirurgia , Pancreatectomia , Neoplasias Pancreáticas/cirurgia , Pancreaticoduodenectomia , Estudos Retrospectivos , Fatores de Tempo , Tomografia Computadorizada por Raios X , Resultado do Tratamento , Adulto Jovem , Síndrome de Zollinger-Ellison/cirurgia
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