Carcinoid of the ampulla of Vater

Endocrine neoplasms only rarely occur at the ampulla of Vater, comprising mostly carcinoids and malignant carcinoids, as well as few cases of poorly differentiated endocrine carcinomas (small cell carcinomas). Only 105 cases are reported in the literature, most as single case reports. For many years, the neoplasms of the disseminated neuroendocrine cell system of the gastrointestinal tract have been subsumed as 'carcinoids'. Instead, in the latest World Health Organization (WHO) classification published in 2000, it is recommended to distinguish between (i) well-differentiated endocrine tumors (carcinoids); (ii) well-differentiated endocrine carcinomas (malignant carcinoids); and (iii) poorly differentiated endocrine carcinomas (small cell carcinomas). Patients with carcinoid tumors of the ampulla of Vater are very often free of clinical and laboratory findings that belong to the carcinoid syndrome. Approximately 26% of all patients with carcinoid tumor reported in the literature had neurofibromatosis. Besides endoscopic retrograde cholangiopancreatography, endosonography, computed tomography or magnetic resonance imaging may complete the staging approach of this tumor. The Kausch-Whipple procedure or pylorus-preserving pancreaticoduodenectomy is considered the treatment of choice for ampullary, well-differentiated carcinoids >2.0 cm and for ampullary neuroendocrine carcinomas. However, it should be considered that long-term survival of patients with ampullary carcinoids is also reported after local tumor excision (5-year survival rate of 90%). The dilemma is that the differentiation of neuroendocrine tumors cannot be assessed intraoperatively in most cases. Therefore, considering that the 5-year survival rate in patients with neuroendocrine carcinomas of the ampulla of Vater is very low without radical resection, neuroendocrine tumors of the ampulla of Vater without definite histological differentiation should undergo extended surgery.     

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 of the ampulla of Vater： Morphologic features and clinical implications

INTRODUCTION Carcinoid tumors belong to the family of neuroendocrine tumors, which usually grow slowly with distinct biologi- cal and clinical characteristics. The incidence of these tumors is approximately 2.5 in 100 000 people per year. The appendix is …     

Minimally invasive transanal surgery for localized rectal carcinoid tumors

Local excision is often fully justified for rectal carcinoid tumors. However insufficient surgical field and difficult access to proximal tumors have been drawbacks in performing pre-existing local excision procedures. A novel local excisional technique called minimally invasive transanal surgery (MITAS) has been experimented for local removal of carcinoid tumors in the rectum. A specially designed anal retractor connected to the Octopus retractor holder was used and an ENDO-stapler allowed the simultaneous excision and anastomosis to be performed. Eight patients with carcinoid tumors in the rectum (4 tumors in the upper rectum) underwent MITAS. Median distance from anal verge to proximal tumor was 6.5 cm (range, 5–12 cm). The median diameter of the tumor was 9 mm. Median operative time was 18.5 minutes and blood loss was minimal. No analgesics were needed postoperatively, and there was no morbidity or mortality. Full-thickness excision of the rectum was accomplished and the tumors confined in the submucosa were demonstrated histologically to be with free surgical margins. No recurrences have been observed with a median follow-up period of 39 months. The technique facilitates total excisional biopsy for rectal carcinoid tumors and reduces operative time, blood loss and complications. Received: 10 January 2002 / Accepted in revised form: 28 January 2002     

Rectal carcinoids: The most frequent carcinoid tumor

One hundred seventy patients with gastrointestinal carcinoid tumors were treated at Ochsner Clinic from 1958 to 1990. Ninety-four rectal carcinoid tumors were diagnosed and treated during this time. Carcinoid tumors of the rectum represented the most frequent primary site (55 percent), followed by carcinoids of the ileum (12 percent), appendix (12 percent), colon (6 percent), stomach (6 percent), jejunum (2 percent), pancreas (2 percent), and other (5 percent). One-half of rectal carcinoids were discovered during anorectal examination of asymptomatic patients. The remainder were found primarily by examination of patients for symptoms of benign anorectal conditions. The diagnosis of rectal carcinoid was made at the time of initial examination in 61 patients. This allowed definitive treatment in a single session by local excision and fulguration in 48 patients. The remainder were treated by repeat biopsy and fulguration (25 patients) or by transanal excision (12 patients). Overall, 85 carcinoid tumors of the rectum measuring <2 cm were treated by local excision and fulguration or by transanal excision, with an average five-year follow-up. There were no local recurrences. Ten patients with metastasizing rectal carcinoids averaging 4 cm were treated. All were symptomatic at presentation and fared poorly despite radical surgery. Three were alive at three years but only one survived five years. At our institution, rectal carcinoids were the most frequently detected carcinoid tumor. Small carcinoids of the rectum were adequately treated by local excision and fulguration or by transanal excision, with no local recurrence. The true incidence of rectal carcinoids is detected only with careful and complete rectal examination of the asymptomatic screening population by experienced surgeons. With more widespread screening of the well population, rectal carcinoids may become recognized as the most frequent human carcinoid tumor.Read at the XIIIth Biennial Congress of the International Society of University Colon and Rectal Surgeons, Graz, Austria, June 24 to 28, 1990.     

Gastric Carcinoid Tumors: The Biology and Therapy of an Enigmatic and Controversial Lesion

Gastric carcinoid tumors were previously believed to be* rare lesions, representing less than 2% of all carcinoid tumors and less than 1 % of all stomach neoplasms. More recent studies have demonstrated that they may constitute as much as 10–30% of carcinoid tumors. Patients with conditions associated with hypergastrinemia, such as chronic atrophic gastritis. Zollinger-Ellison syndrome with multiple endocrine neoplasia type 1 (ZES-MEN-1), and pernicious anemia, display a markedly elevated incidence of gastric carcinoid tumor formation. A classification system distinguishing three types of gastric carcinoid tumor has been proposed: 1) tumors associated with chronic atrophic gastritis, 2) tumors associated with Zollinger-Ellison syndrome, and 3) sporadic lesions. Tumors that develop in association with hypergastrinemia are usually composed of entcrochroniaffinlike (ECL) cells, in contrast to sporadic lesions that contain a variety of endocrine cell types (enterochromaffin, ECL, X), In both intact animal models such as the rat and Praomys (mastomys) natalensis and in isolated purified ECL cell preparations, gastrin has been demonstrated to exert a powerful trophic effect on ECL cells, in addition to stimulating histamine secretion. It is apparent that hypergastrinemia-associated gastric carcinoids display relatively benign biological behavior. Sporadic lesions require aggressive surgical management on diagnosis. Type I and type II (hypergastrineoiia-aasoci-ated) lesions can be managed initially by endoscopic excision of accessible tumors, followed by endoscopic surveillance. If tumors recur, antrectomy and local excision may be used to remove the source gastrin, resulting in cure in the vast majority of patients.     

不同类型胃肠类癌的临床分析与比较

目的　分析不同病理类型胃肠类癌的临床特点 ,以提高对该类疾病的认识与诊治水平。方法　回顾性分析北京协和医院 1982～ 2 0 0 3年的病理分类胃肠类癌 36例 ,并根据世界卫生组织肿瘤国际组织学新分类标准 (2 0 0 0 )分为恶性类癌、类癌 2组 ;比较 2组类癌的发病年龄特点、临床表现差异、转移情况、在消化系统内分泌肿瘤和肿瘤中的发病比例以及确诊的主要手段等。结果 (1)患病比 :胃肠类癌占同期消化系统肿瘤的 0 .35 % ,占同期消化系统内分泌肿瘤的 10 .2 0 %。 (2 )年龄 :恶性类癌发病年龄为 (5 5 .8± 12 .8)岁 ,类癌发病年龄为 (45 .1± 12 .0 )岁 ,前者明显高于后者。 (3)部位 :类癌多发于直肠 (6 3% ) ,而发生于胃 (35 % )与结肠 (2 5 % )应考虑恶性类癌。(4)转移 :淋巴结与肝脏是恶性类癌常见的转移部位 (6 0 % ) ;胃与结肠部位的恶性类癌转移率明显高于直肠与其他部位。(5 )检查手段 :胃肠类癌的发现多依赖内镜检查 ,但直肠指检不应偏废。 (6 )类癌综合征的发生率低 ,恶性类癌多见。结论　胃肠类癌在消化系统肿瘤中的发病率较低 ,但并非罕见 ;直肠是类癌的好发部位 ,胃与结肠是恶性类癌的好发部位 ;恶性类癌发病年龄较大且易伴肝与淋巴结转移 ,类癌综合征多见于恶性类癌。     

Examination of endoscopical ultrasonography (EUS) on carcinoid tumors of gastrointestinal tract

In order to clarify the usefulness of EUS in the diagnosis of gastrointestinal carcinoid tumor, we examined 15 patients, who had carcinoid tumors of gastrointestinal tract (stomach: 5, duodenum: 2, rectum: 8), on the diagnosis in quality and depth by comparing endoscopical ultrasonography (EUS) with resected specimen. Carcinoid tumors of gastrointestinal tract were detected as homogenous hypoechoic tumors with sharp border in all site by EUS. Especially, it was characteristic that 14 of the 17 lesions (82%) which invaded into submucosa were mainly located in the third layer, and the second layer covered the tumor at the foot, and near the top, it touched the tumor and got indistinct. We decided the depth of invasion by comparing the hypoechoic tumor with normal structure of 5 layers. The accuracy rate was 88%. In conclusion, EUS was thought to be useful in the diagnosis and choice for treatment of carcinoid tumors of gastrointestinal tract.     

Endoscopic resection with a two-channel videoendoscope for gastric carcinoid tumors

BACKGROUND/AIMS: Endoscopic resection has been used to treat hypergastrinemia-associated early carcinoid tumors of the stomach. However, indications for endoscopic treatment of these tumors have not been established. Moreover, endoscopic resection of these tumors is often difficult with conventional polypectomy, because these tumors are often located in the submucosal layer. To completely remove these tumors, we used a two-channel videoendoscope with which both grasping forceps and a polypectomy snare could be used simultaneously. METHODOLOGY: At Osaka Medical Center for Cancer and Cardiovascular Diseases, eight carcinoid tumors in six patients were removed with a two-channel videoendoscope. Reports of early carcinoid tumor in Japanese literature were reviewed to analyze the relationship between lymph node metastasis and the size and depth of involvement of these tumors. RESULTS: Six carcinoid tumors were completely removed "en bloc", but two tumors were incompletely removed. In these two patients, submucosal tumor invasion was observed on the excision line. To completely remove these tumors, the oral side, but not the top, of the tumor should be strongly grasped and pulled toward the center of the lumen as far as possible by the grasping forceps, which had been passed through the snare loop. Endoscopic follow-up studies showed no local recurrence in any patients with and without complete tumor resection during the average observation period of 30 months. A review of histological reports in Japanese literature showed that lymph node metastasis did not occur when the tumors were less than 10 mm in diameter, and could be completely removed by an endoscope. CONCLUSIONS: Endoscopic resection with a two-channel videoendoscope is a useful and safe method for resection of small carcinoid tumors of the stomach.     

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.     

Endoscopic ultrasound-assisted endoscopic resection of carcinoid tumors of the gastrointestinal tract.

INTRODUCTION: Usually found in the gastrointestinal tract, carcinoids are the most frequent neuroendocrine tumors. Most of these lesions are located in areas that are difficult to access using conventional endoscopy (small intestine and appendix); carcinoid tumors found in the gastroduodenal tract and in the large intestine can be studied endoscopically; in these cases, if localized disease is confirmed, local treatment by endoscopic resection may be the treatment of choice. Since endoscopic ultrasonography has been shown to be the technique of choice for the study of tumors exhibiting submucosal growth, the selection of patients who are candidates for a safe and effective local resection should be based on this technique. PATIENTS AND METHOD: We selected patients with gastrointestinal carcinoid tumors who were endoscopically treated between 1997 and 2002. Those patients with tumors measuring less than 10 mm, which had not penetrated the muscularis propria, and those with localized disease were considered candidates for endoscopic resection. The endpoints of this study were to assess the effectiveness (complete resection) and safety (complications) of the technique. Follow-up consisted of eschar biopsies performed one month and twelve months after the resection. RESULTS: During the aforementioned period, we resected endoscopically 24 tumors in 21 patients (mean age: 51.7 years; 71.5% males). Most lesions were incidental discoveries made during examinations indicated for other reasons. Resection was indicated in most cases as a result of the suspected presence of a carcinoid tumor after endoscopic ultrasonography. Endoscopic ultrasonography also enabled us to clearly identify the layer where the lesion had originated, as well as the size of the lesion. The carcinoid tumor was removed in 13 cases (54.2%) by using the conventional snare polypectomy technique, in 9 cases (37.5%) assisted by a submucosal injection of saline solution and/or adrenaline, and in 2 cases (8.3%) after ligating the lesion with elastic bands. In all cases the resection was complete, with no recurrence during the follow-up period, and no major complications, except for a single case in which a post-polypectomy hemorrhage occurred that was endoscopically solved. CONCLUSIONS: In properly selected patients, the endoscopic resection of carcinoid tumors is a safe and effective technique that permits a complete resection in all cases with few complications. Endoscopic ultrasonography is the technique of choice for selecting the patients who are candidates for endoscopic resection.     

A pilot randomized control study to evaluate endoscopic resection using a ligation device for rectal carcinoid tumors

AIM: Rectal carcinoid tumors smaller than 10 mm can be resected with local excision using endoscopy. In order to remove rectal carcinoid tumors completely, we evaluated endoscopic mucosal resection with a ligation device in this pilot control randomized study. METHODS: Fifteen patients were diagnosed with rectal carcinoid tumor (less than 10 mm) in our hospital from 1993 to 2002. There were 9 males and 6 females, with a mean age 61.5 years (range, 34-77 years). The patientshad no complaints of carcinoid syndrome symptoms. Fifteen patients were randomly divided into 2 groups: 7 carcinoid tumors were treated by conventional endoscopic resection, and 8 carcinoid tumors were treated by endoscopic resection using a ligation device. RESULTS: All rectal carcinoid tumors were located at the middle to distal rectum. The size of the tumors varied from 3 mm to 10 mm and background characteristics of the patients were not different in the two groups. The rate of complete removal of carcinoid tumors using a ligation device (100%, 8/8) was significantly higher than that of conventional endoscopic resection (57.1%, 4/7). The three patients had tumor involvement of deep margin, for which additional treatment was performed. No complications occurred during or after endoscopic resection using a ligation device. All patients in the both groups were alive during the 3-year observation period. CONCLUSION: Endoscopic resection using a ligation device is a useful and safe method for resection of small rectal carcinoid tumors.     

Carcinoid tumors are 15 times more common in patients with Crohn's disease

BACKGROUND: The coexistence of intestinal neoplasms with Crohn's disease (CD) has been reported, but the evidence of an increased risk of carcinoid tumor with Crohn's disease has been mixed. We present 4 patients with CD with associated carcinoid tumor. METHODS: The charts of 111 patients with CD who had undergone resection between June 2001 and March 2005 were reviewed. The number of incidental carcinoid tumors in patients who underwent an appendectomy was used as a control. RESULTS: Four cases of carcinoid tumor discovered in patients at resection for CD were identified. None had metastatic disease or carcinoid syndrome. These included 1 cecal (1 mm), 2 appendiceal (3 and 7 mm), and 1 transverse colon (7 mm) carcinoid tumors. None of the carcinoid tumors were identified in regions of active Crohn's disease. The incidence of carcinoid tumor in patients with Crohn's disease was 4 of 111 (3.6%). In comparison, 3 of 1199 patients (0.25%) who had appendectomies were identified as having appendiceal carcinoid tumor. Crohn's disease was associated with an increased incidence of carcinoid tumor; OR 14.9 (95% CI 2.5-102.5), P<0.0001. CONCLUSIONS: There was a significantly increased incidence of carcinoid tumor in our Crohn's patients compared to the control patients. None of the carcinoid tumors developed in areas of Crohn's disease. This suggests that the development of carcinoid tumors may be secondary to distant proinflammatory mediators, rather than a local inflammatory effect from adjacent Crohn's disease. Patients with CD may be at increased risk of developing a carcinoid tumor.     

Gastrointestinal carcinoid tumors

BACKGROUND: Carcinoid or neuroendocrine tumors of the gastrointestinal tract, although characteristically indolent, are also quite heterogeneous both with respect to histologic and endocrine features and with respect to clinical presentation and behavior. PURPOSE: This study was undertaken to review and summarize the current literature on classification controversies, site-specific carcinoid presentation and behavior, and diagnostic and management strategies for primary and advanced carcinoid tumors and the carcinoid syndrome. RESULTS: For carcinoid tumors, oncologic results depend on the location of the primary tumor, extent of locoregional and metastatic disease, functioning status of the tumor, and the feasibility of complete surgical extirpation. Whereas favorable survival rates are typically observed for appendiceal and rectal primaries, less favorable rates are often observed for colonic and ileal tumors. A search for additional tumors is generally advised because multiple carcinoids and second neoplasms are not uncommon. Because of the indolent nature of the tumor and because these therapies have been shown to improve quality and quantity of life, otherwise fit patients with advanced carcinoid disease should be treated with aggressive medical and surgical therapies. Development of a malignant carcinoid syndrome indicates the presence of a functionally active carcinoid tumor and portends a poor prognosis. CONCLUSION: Gastrointestinal carcinoids, although malignant, behave differently from other carcinomas. Results are highly variable and must be individualized according to the site of the primary tumor, extent of spread, and general condition of the patient. A prolongation of quality life can often be accomplished through aggressive medical and surgical therapies.     

EUS may have limited impact on the endoscopic management of gastric carcinoids

Summary Background. Carcinoids are occasionally found during gastroscopy. Endoscopic ultrasonography (EUS) can determine the depth of invasion and vascularity of submucosal tumors, including carcinoid tumors. Thus, EUS can lead to an informed decision as to whether to attempt endoscopic or surgical excision of a carcinoid. The three cases described here were found by EUS to be amenable to endoscopic resection of submucosal carcinoid tumors. In each case, the margin of the specimen obtained led to uncertainty regarding the completeness of gastroscopic excision of the tumor. Guidelines for follow-up of gastric carcinoid are few. The incompleteness of endoscopic resection of submucosal tumors, with tumor found at the specimen margin, indicates that careful follow-up and/or consideration of other means of excision are indicated.     

Endoscopic resection of an ampullary carcinoid presenting with upper gastrointestinal bleeding： A case report and review of the literature

Ampullary carcinoid is a rare tumor that can present with gastrointestinal bleeding, obstructive jaundice or pancreatitis. Some of these tumors are associated with Von Recklinghausen disease. The usual surgical options are a biliary-enteric anastomosis, Whipple procedure or rarely a local resection. The mean survival does not appear to be much different after a pancreaticoduodenectomy versus local surgical excision. We report a very rare case of a non-metastatic ampullary carcinoid causing upper gastrointestinal bleeding, which was managed by endoscopic ampullectomy.     

Factors associated with complete local excision of small rectal carcinoid tumor

Purpose

Although small rectal carcinoid tumors can be treated using local excision, complete resection can be difficult because tumors are located in the submucosal layer. We evaluate the factors associated with pathologically complete local resection of rectal carcinoid tumors.

Methods

Data were analyzed of 161 patients with 166 rectal carcinoid tumors who underwent local excision with curative intent from January 2001 to December 2010. A pathologically complete resection (P-CR) was defined as an en bloc resection with tumor-free lateral and deep margins. The study classified treatments into three categories for analysis: conventional polypectomy (including strip biopsy, snare polypectomy, and hot biopsy), advanced endoscopic techniques (including endoscopic mucosal resection with cap and endoscopic submucosal dissection), and surgical local excision (including transanal excision and transanal endoscopic microsurgery). We evaluated the P-CR rate according to treatment method, tumor size, initial endoscopic impression and the use of endoscopic ultrasound (EUS) or transrectal ultrasound (TRUS).

Results

The mean tumor size was 5.51?±?2.43 mm (range 2–18 mm) and all lesions were confined to the submucosal layer. The P-CR rates were 30.9, 72.0, and 81.8 % for conventional polypectomy, advanced endoscopic techniques, and surgical local excision, respectively. Univariate analysis showed that P-CR was associated with treatment method, use of EUS or TRUS, and initial endoscopic impression. Multivariate analysis showed that only treatment method was associated with P-CR.

Conclusion

Pathologically complete resection of small rectal carcinoid tumors was more likely to be achieved when using advanced endoscopic techniques or surgical local excision rather than conventional polypectomy.     

Carcinoid tumors

Carcinoid tumors most commonly occur in the gastrointestinal tract and are known best for the bizarre manifestations of the carcinoid syndrome caused by the release of tumor products. However, many carcinoid tumors do not present with or cause the carcinoid syndrome, and this has led to the development of new diagnostic markers for these tumors. New treatments have emerged recently and are being developed, because although these tumors are relatively indolent, they can metastasize. The biology of carcinoid tumors of the gastrointestinal tract can be classified in large part by the embryologic regions of the gut in which they occur. More carcinoid tumors will be discovered as the indications and use of gastrointestinal endoscopies increase, so the diagnosis and initial management of carcinoid tumors will fall largely on the gastroenterologist.     

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

Type I gastric carcinoid 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-111 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.