Upper gastrointestinal carcinoid tumors incidentally found by endoscopic examinations

AIM: This study shares Asian clinical experiences of carcinoid tumors that originated in the upper gastrointestinal tract.METHODS: From May 1987 to June 2002, we had found only 13 cases of histologically confirmed carcinoid tumors in the upper gastrointestinal tract by endoscopic examinations. There were eight males and five females.The mean age was 53.16±20.51 years that ranged from 26 to 82 years. Each of their clinical presentations,locations, tumor morphology, and size and the treatment outcome were analyzed and discussed.RESULTS: One patient had a polypoid lesion at the lower esophagus, nine were stomach lesions and three located at the duodenum. All patients with polypoid and submucosal tumor types were of small size (＜1.7 cm) and all patients survived after simple excision or polypectomy.Four of the five patients in tumor mass forms died and the tumors were more than 2.0 cm in size.CONCLUSION: Carcinoid tumors rarely originated from the upper gastrointestinal tract and are usually found accidentally after endoscopic study. Bigger size (more than 2 cm) tumor masses may indicate a more severe disease and poor prognosis.

Carcinoid Tumors in the Middle Ear:a Case Report and Literature Review

Middle ear carcinoid tumor (MEC T) is rare. Only 46 cases of MECT have been reported in the literature since the first case of MECT was described in 1980. We present here a case of primary MECT initially diagnosed as inflammatory aural polyp. The case was a 43-year-old women complaining of right ear chronic otorrhea and hearing loss over a period of five years, with a blockage sensation in the right ear for two years. Audiometry showed conductive hearing loss in the right ear. Physical examination and CT scans showed a mass in the right external auditory canal and middle ear, surrounding the ossicular chain. Pathologic study of surgically removed specimen revealed features of carcinoid tumor with positive staining to chromogranin A and synaptophysin in tumor cells. Local radiation of 60 Gy was applied. The patient has been followed up for more than one year. Postoperative histopathological examination showed no evidence of MECT recurrence one year after surgery, but inflammatory changes in the middle ear. Relevant literatures were reviewed. Clinical, histopathological, immunohistochemical and ultrastructural features of MECT, and strategies in MECT diagnosis and management are discussed.

Endoscopic ultrasonography and submucosal resection in the diagnosis and treatment of rectal carcinoid tumors

Rectal carcinoid tumors are often diagnosed as .submucosal tumors on radiological or endoscopic examinations. Usually, its definite size and character, the infiltration of muscularis propria and vessels can not be correctly diagnosed preoperatively. In recent years, endoscopic ultrasonography （EUS） has been used in the diagnosis of submucosal tumors of the gastrointestinal tracts , Conventional endoscopic polypectomy allows local excision of local tumors, but is often associated with tumor involvement of the resection margin necessitates further interventions.

Concomitant gastric carcinoid and gastrointestinal stromal tumors:A case report

A gastric carcinoid tumor concomitant with gastrointestinal stromal tumor （GIST） is rarely encountered in clinical practice. We report a 65-year-old female who had a 0.8 cm gastric carcinoid tumor on the posterior wall of the upper gastric corpus detected during an esophagogastroduodenoscopy at a routine physical examination, and a concomitant 1.1 cm GIST on the anterior wall of the upper gastric corpus incidentally found during surgery of the gastric carcinoid tumor. Normal serum gastrin level and histological findings suggested that she had a type 111 gastric carcinoid tumor and a GIST which were categorized a very low risk of malignancy, based on their small size and lack of mitosis. Both tumors were treated successfully by surgical excision. The patient had an uneventful recovery. Neither recurrence nor metastasis was found after a 28-mo follow-up.

Clinicopathologic analysis of eight cases of pancreatic carcinoid tumors

Carcinoids of the pancreas are exceedingly rare tumors that originate from the enterochromaffin cells of the gastroenteropancreatic neuroendocrine system. According to a recent report, pancreatic carcinoids are found in only 0.58% （79/13 715 cases） of the entire carcinoid group. To date, very limited information regarding the detection and diagnosis of this entity has been reported in the available literature. Although pancreatic carcinoid tumors grow slowly and show an indolent clinical behavior, they are potentially malignant. Due to the late onset of clinical symptoms and delayed diagnosis of the tumor, there was a high incidence of distant metastases that hampered long-term survival for many patients, leading to an unfavorable overall prognosis.

Goblet cell carcinoid of the appendix and mixed adenoneuroendocrine carcinoma: Report of three cases

Neuroendocrine neoplasms are the most common epithelial tumors among appendix tumors. Appendix tumors that are completely or partially composed of neuroendocrine cells are divided into two categories: Classic carcinoid tumors and goblet cell carcinoid tumors(GCCT). They are known to progress more aggressively than classic(neuro) endocrine tumors. In this study, three cases with acute appendicitis symptoms are presented, including their clinical and histopathological findings. Microscopic examination detected GCCT in two cases and mixed adenoneuroendocrine carcinoma in one case, in addition to acute appendicitis.

Endoscopic resection of carcinoid of the minor duodenalpapilla

We encountered a 65-year-old man with a carcinoid tumor of the minor duodenal papilla. Since he had liver cirrhosis and completely refused surgery, we performed an endoscopic snare papillectomy. The papillectomy was performed successfully without procedure-related complication. The specimens revealed a carcinoid tumor showing that the margin of the tumor was positive. One week later, upper GI endoscopy was performed and the biopsy specimens obtained from base of ulcer showed no neoplastic cells. We performed a duodenoscopy and CT 3, 6 and 18 mo later, and there was no macroscopic or microscopic evidence of tumor recurrence after more than 4 years.

Endoscopic submucosal dissection for small submucosal tumors of the rectum compared with endoscopic submucosal resection with a ligation device

AIMTo evaluate the efficacy and safety of endoscopic submucosal dissection (ESD) for small rectal submucosal tumors (SMTs). METHODSBetween August 2008 and March 2016, 39 patients were treated with endoscopic submucosal resection with a ligation device (ESMR-L) (n = 21) or ESD (n = 18) for small rectal SMTs in this study. Twenty-five lesions were confirmed by histological evaluation of endoscopic biopsy prior to the procedure, and 14 lesions were not evaluated by endoscopic biopsy. The results for the ESMR-L group and the ESD group were retrospectively compared, including baseline characteristics and therapeutic outcomes. RESULTSThe rate of en bloc resection was 100% in both groups. Although the rate of complete endoscopic resection was higher in the ESD group than in the ESMR-L group (100% vs 95.2%), there were no significant differences between the two groups (P = 0.462). In one patient in the ESMR-L group with a previously biopsied tumor, histological complete resection with a vertical margin involvement of carcinoid tumor could not be achieved, whereas there was no incomplete resection in the ESD group. The mean length of the procedure was significantly greater in the ESD group than in the ESMR-L group (14.7 ± 6.4 min vs 5.4 ± 1.7 min, P vs 2.8 ± 1.5 d, P CONCLUSIONBoth ESMR-L and ESD were effective for treatment of small rectal SMTs. ESMR-L was simpler to perform than ESD and took less time.

Angiogenic markers endoglin and vascular endothelial growth factor in gastroenteropancreatic neuroendocrine tumors

AIM:To investigate the expression and potential prognostic role of vascular endothelial growth factor(VEGF) and endoglin in gastroenteropancreatic neuroendocrine tumors(GEP-NETs) . METHODS:Microvessel density(MVD) in GEP-NETs was evaluated using endoglin and CD31 immunohistochemistry.In addition,tissue levels of endoglin and VEGF were determined in homogenates by ELISA. RESULTS:Endoglin was highly expressed on tumor endothelial cells.CD31 MVD in GEP-NETs was significantly higher compared to endoglin MVD(P<0.01) .Two-tofour-fold higher tissue levels of endoglin and VEGF were seen in tumors compared to associated normal tissue. This increased endoglin tissue expression in tumors was significantly related to tumor size(P<0.01) ,presence of metastases(P=0.04) ,and a more advanced tumor stage(P=0.02) ,whereas expression of VEGF was not. CONCLUSION:We suggest that endoglin is a potential marker to indicate and predict metastases,which might be useful in the post-resection therapeutic approach of patients with GEP-NETs.

Malignant carcinoid tumor of the cystic duct:a rare cause of bile duct obstruction

BACKGROUND:Carcinoid tumors of the extrahepatic biliary tree are extremely rare malignancies,accounting for 0.2%-2%of all gastrointestinal carcinoid tumors,while carcinoids of the cystic duct are an uncommon entity and an extremely unusual cause of bile duct obstruction.METHODS:After an extensive literature review,we retrospectively analysed 61 cases of carcinoid tumor of the biliary tree as well as one additional case of a 60-yearold female with symptoms and laboratory/imaging examination findings compatible with those of a malignant biliary tract obstruction.At laparotomy,resection of the gallbladder and common bile duct was performed.Histological study revealed a well-differentiated neuroendocrine carcinoma of the cystic duct.The patient remained disease-free at 16 months.RESULTS:Our presentation is the seventh case reported in the world literature.Compared to cholangiocarcinoma,analysis of the reviewed group indicates an increased incidence of extrahepatic carcinoid tumors in younger persons along with a slight female predominance.Statistically,the most common anatomic location is the common bile duct,followed by the perihilar region and the cystic duct.Jaundice is the most common finding.Curative surgery was realized in the majority of cases and longterm disease-free survival was achieved when surgery was curative.CONCLUSIONS:Carcinoid tumors obstructing the biliary tree are extremely difficult to diagnose preoperatively,and nearly impossible to differentiate from non-neuroendocrine tumors.As surgery offers the only potential cure for both biliary carcinoids and cholangiocarcinoma,we recommend aggressive surgical therapy as the treatment of choice in every case of potentially resectable biliary tumor.

Gastric carcinoid tumor in a patient with a past history of gastrointestinal stromal tumor of the stomach

Gastrointestinal stromal tumor is the most common mesenchymal tumor in the gastrointestinal tract. It may coexist with other type of cancers,and if so,the tumors usually involve the stomach. The most common associated cancers are gastrointestinal carcinomas. We report a 65-year-old woman with a history of gastric gastrointestinal stromal tumor who had undergone subtotal segmental gastrectomy. New polypoid lesions were detected on a follow-up gastroscopy one year later. The lesions were biopsied and found to be carcinoid tumors. There was serum hypergastrinemia,and type 1 gastric carcinoid tumor was diagnosed. A total gastrectomy was performed. Pathologic examination revealed both carcinoid tumors and a recurrent gastrointestinal stromal tumor.

Treatment of solitary gastric carcinoid tumor by endoscopic polypectomy in a patient with pernicious anemia

Type I gastric cardnoid tumors result from hypergastrinemia in 1%-7% of patients with pernicious anemia. We diagnosed pernicious anemia in a 48-year-old female patient with complaint of fatigue for three months. She had no gastrointestinal symptoms. Endoscopic examination ot the upper gastrointestinal tract revealed atrophic gastritis and a polypoid lesion in the corpus of 3-4 mm in size. Endoscopic polypectomy was performed. Histopathological examination of the specimen revealed positive chromogranin A and synaptophysin stainings compatible with the diagnosis of a carcinoid tumor. Serum gastrin level was increased, urinary 5-hydroxyindoleacetic acid was within the normal range. There was no other symptom, sign, or laboratory finding of a carcinoid syndrome in the patient. No metastasis was found with indium-ill octreotide scan, computed tomographies of abdomen and thorax. Type I gastric carcinoid tumors are only rarely solitary and patients with tumors 〈 1 cm in size may benefit from endoscopic polypectomy.

Management of incidentally discovered appendiceal neuroendocrine tumors after an appendicectomy

Appendiceal neuroendocrine tumors(aNETs)are an uncommon neoplasm that is relatively indolent in most cases.They are typically diagnosed in younger patients than other neuroendocrine tumors and are often an incidental finding after an appendectomy.Although there are numerous clinical practice guidelines on management of a NETs,there is continues to be a dearth of evidence on optimal treatment.Management of these tumors is stratified according to risk of locoregional and distant metastasis.However,there is a lack of consensus regarding tumors that measure 1-2 cm.In these cases,some histopathological features such as size,tumor grade,presence of lymphovascular invasion,or mesoappendix infiltration must also be considered.Computed tomography or magnetic resonance imaging scans are recommended for evaluating the presence of additional disease,except in the case of tumors smaller than 1 cm without additional risk factors.Somatostatin receptor scintigraphy or positron emission tomography with computed tomography should be considered in cases with suspected residual or distant disease.The main point of controversy is the indication for performing a completion right hemicolectomy after an initial appendectomy,based on the risk of lymph node metastases.The main factor considered is tumor size and 2 cm is the most common threshold for indicating a colectomy.Other factors such as mesoappendix infiltration,lymphovascular invasion,or tumor grade may also be considered.On the other hand,potential complications,and decreased quality of life after a hemicolectomy as well as the lack of evidence on benefits in terms of survival must be taken into consideration.In this review,we present data regarding the current indications,outcomes,and benefits of a colectomy.

SURGICAL TREATMENT AND PROGNOSIS OF BRONCHIAL CARCINOID TUMOR

In order to understand the effect of surgery in the treatment of the bronchial carcinoid tumor and the factors affecting prognosis, 18 cases of bronchial carcinoid tumor are presented, including 5 cases with Cushing's syndrome. There were lobectomy in 10, lung wedge resection in 3, excision of intraluminal tumor of bronchus in 3, exploratory thoracotomy in 2 cases. No operation death. Pathological examination revealed 14 cases were typical carcinoid tumor and 4 cases were atypical carcinoid tumor. By 2-13 years follow-up . 3 , 5 and 10 years survival rate were 82% , 78%and 70% respectively. Bronchial carcinoid tumor is often confused with small cell carcinoma of lung, the correct diagnosis can be obtained by light microscopic, electron microscopic and immunohistochemistry studies. Those patients accompanied with ectopic-ACTH secretion always have Cushing,s syndrome , resection of tumor can produce gho result. Proper operation method depends on the location of the tumor and patient's extent of cardiac and pulmonary reserve.Atypical carcinoid tumor had high malignancy and poor prognosis. The size of tumor, lymph node involvement and adjuvant therapy seem no definite effect on the patients' survival rate.

Iridociliary Body Metastasis of Atypical Carcinoid: Case Management with Intravitreal Anti-Vascular Endothelial Growth Factor Injections

We report a rare case involving a 52-year-old female diagnosed with an atypical bronchial carcinoid tumor with metastases to the mediastinum, hilar lymph nodes, breast, and pancreas. In additional, the patient had metastases to the iris and ciliary body, resulting in progressive vision loss in her left eye. Treatment was successful by intravitreal injections of anti-vascular endothelial growth factor.

Carcinoid tumor of the duodenum and accessory papilla associated with polycythemia vera

Carcinoid tumors have been reported in a wide range of organs but most frequently involve the gastrointestinal tract; however, duodenal carcinoid tumors are rare. We report a 50-year-old male patient complaining of multiple melenas for 3 wk. The panendoscopy and endoscopic retrograde cholangiopancreaticography revealed swelling accessory papilla with an ulcer. The biopsy taken showed a carcinoid tumor. The lesion was removed by wide resection. Patient was found to have an abnormal blood cell count during the follow-up period with elevated levels of hemoglobin and hematocrit of 21.2 g/dL and 63.5%,respectively, thrombocytosis of 501 000/μL, and leukocytosis of 20 410/μL. He was diagnosed as a polycythemia vera by a hematologist after further evaluation. He received periodic phlebotomy and hydroxyurea treatment. The response was good and his hematocrit was stabilized by periodic phlebotomy in the range of 44-49% during the last 2 years. The possible origin of UGI bleeding by a duodenal carcinoid tumor, although rare, should be considered. There has been one case report of a duodenal carcinoid tumor that involved accessory papilla of the pancreas divisum and one case report of metastatic carcinoid tumor associated with polycythemia vera. It is different in our patient as compared with the latter report, which mentioned a polycythemia vera patient who was found to have a metastatic carcinoid in the 17 years follow-up period. Chemotherapy had been given before the carcinoid tumor was revealed. Our patient had no previous chemotherapy for polycythemia vera before he was found to have duodenal carcinoid tumor; this excludes the possibility of chemotherapy induced carcinoid tumor, although it had been suspected in the previous report. In our patient, the existence of both diseases may be by predisposition of each other since both diseases have an increased incidence of other neoplasm, or they may be coexistent incidentally.

Carcinoid tumor of the common bile duct: A rare complication of von Hippel-Lindau syndrome

Von HippeI-Lindau syndrome （VHL） is a rare autosomal-dominant, inherited familial cancer syndrome. Hemangioblastomas, pheochromocytomas and renal carcinoma are the frequent reported VHL tumors. Neuroendocrine tumors have also been described, mostly in the pancreas and rarely in the biliary trees. We report the second case of bile duct carcinoid in a 31-year-old VHL woman. She underwent right adrenalectomy for a pheochromocytoma in the past. She also had a positive family history of phenotypic expression of VHL syndrome. The patient presented with biliary colic. Endoscopic retrograde cholangio-pancreatography showed intraluminal bile duct mass. Surgical exploration identified a beige nodular lesion that was a carcinoid tumor on histology. This new association should be clarified by further genetic investigations.

Prognostic impact of number of examined lymph nodes on survival of patients with appendiceal neuroendocrine tumors

BACKGROUND The prognosis of patients with appendiceal neuroendocrine tumors(ANETs) is related to lymph node(LN) metastasis and other factors.However,it is unclear how the number of examined LNs(ELNs) impact on survival.AIM To determine the factors affecting the cancer-specific survival(CSS) of patients with ANET and to evaluate the impact of the number of ELNs on survival.METHODS A total of 4583 ANET patients were analyzed in the Surveillance,Epidemiology,and End Results database.Univariate survival analysis was used to identify factors related to survival and the optimal number of ELNs and lymph node ratio(LNR) were determined by the Kaplan–Meier method.The survival difference was determined by CSS.RESULTS Except for sex,the other factors,such as age,year,race,grade,histological type,stage,tumor size,ELNs,LNR,and surgery type,were associated with prognosis.The 3-,5-,and 10-year CSS rates of ANET patients were 91.2%,87.5,and 81.7%,respectively(median follow-up period of 31 mo and range of 0-499 mo).There was no survival difference between the two surgery types,namely,local resection and colectomy or greater,in both stratifications of tumor size ≥ 2 cm(P = 0.523)and < 2 cm(P = 0.068).In contrast to patients with a tumor size < 2 cm,those with a tumor size ≥ 2 cm were more likely to have LN metastasis(χ~2 = 378.16,P < 0.001).The optimal number of ELNs was more than 11,7,and 18 for all patients,nodenegative patients,and node-positive patients,respectively.CSS rates of patients with a larger number of ELNs were significantly improved(≤ 10 vs ≥ 11,χ~2 =20.303,P < 0.001;≤ 6 vs ≥ 7,χ~2 = 11.569,P < 0.001;≤ 17 vs ≥ 18,χ~2 = 21.990,P < 0.001;respectively).ANET patients with an LNR value ≤ 0.16 were more likely to have better survival than those with values of 0.17-0.48(χ~2 = 48.243,P < 0.001) and 0.49-1(χ~2 = 168.485,P < 0.001).CONCLUSION ANET ≥ 2 cm are more likely to develop LN metastasis.At least 11 ELNs are required to better evaluate the prognosis.For patients with positive LN metastasis,18 or more LNs need to be detected and lower LNR values(LNR ≤ 0.16) indicate a better survival prognosis.

Meckel Diverticulum Carcinoid Tumor Complicated by Ileal Invagination. About One Case at Vichy Hospital

Meckel’s diverticulum is a common pathology in children and rare in adults. We present a case of a 79-year-old patient in which a significant gastrointestinal bleeding, whose paraclinical explorations confirmed a fatty tumor of the terminal ileum. The exploratory coelioscopy revealed a tumor of the Meckel diverticulum complicated of intestinal invagination, resected at the same time with resection and extracorporeal anastomosis by mini coelio-guided laparotomy. Histology confirmed the presence of heterotopical tissue of the duodenal mucosa with Brunner cells and a carcinoid tumor. In conclusion, this clinical case shows that coelioscopy can be considered a very important diagnostic and therapeutic tool in this pathology especially in the elderly.

Primary hepatic neuroendocrine carcinoma:clinical analysis of 11 cases

BACKGROUND: Primary hepatic neurocridocrine carcinoma (PHNEC) is extremely rare, and fewer than 300 cases have been reported in the English/Chinese-language literature, therefore it is difficult to make a proper diagnosis and determine a therapeutic approach. METHODS: Eleven PHNEC patients were admitted to our hospital between January 1996 and May 2008. Laboratory examination, digestive endoscopy, B-ultrasonography, CT, MRI, or PET-CT were performed on the patients for preoperative diagnosis. All patients received liver resection. Some patients received transcatheter arterial chemoembolization (TACE), percutaneous ethanol injection treatment (PEIT), or octreotide injection when a recurrence was found. The patients' clinical data were recorded and all patients were followed up. RESULTS: The patients were confirmed pathologically as having PHNEC. Their median follow-up time was 33 months (12-107 months). All patients survived, and the longest postoperative survival time was 107 months, the longest disease-free survival time was 98 months, the 1-year survival rate was 100%, and the 1-year recurrence rate was 45.5% (5/11). CONCLUSIONS: Since PHNEC is easy to confuse with hepatocellular carcinoma, careful screening of symptoms is needed to avoid misdiagnosis. Resection is the first choice of treatment for PHNEC and provides the most favorable outcomes including long-term survival. Other treatment such as TACE and PEIT can be considered as well, especially when a tumor recurs.