Short-term and long-term outcomes of submucosal dissection for residual or recurrent colorectal tumors after endoscopic resection: Analysis of a multicenter prospective study.

OBJECTIVES: We previously demonstrated that a favorable long-term prognosis indicated that endoscopic submucosal dissection (ESD) could be the standard treatment for large colorectal epithelial neoplasms, but the usefulness of ESD for local residual or recurrent tumors with submucosal fibrosis has not been fully demonstrated. The aim of the present study was to assess the usefulness of ESD for local residual or recurrent colorectal tumors. METHODS: We conducted a nationwide multicenter prospective study to evaluate the outcomes of ESD for colorectal tumors. In this post hoc analysis, a total of 54 local residual or recurrent colorectal tumors in 54 patients were included, and we analyzed the short-term and long-term outcomes of ESD for these lesions. RESULTS: The median size of the lesions was 16.0 (interquartile range [IQR] 11-25) mm. ESD was completed in 53 cases (98.1%) with a median procedure time of 65.0 min, but it was discontinued in one case because of submucosal cancer invasion. En bloc resection was achieved in 52 cases (96.3%), whereas R0 resection was achieved in 45 cases (83.3%). Intraoperative perforation was observed in four cases (7.4%) and delayed perforation in one (1.9%), but all cases could be managed conservatively. Delayed bleeding was not observed. There were no significant differences in short-term outcomes between the rectal and colonic lesions. There was no recurrence of the tumor during the median follow-up period of 60 (IQR 50-64) months. CONCLUSION: An analysis of our multicenter prospective study suggests that ESD is an effective salvage management for local residual or recurrent colorectal lesions.

Intensive endoscopic resection for downstaging of polyp burden in patients with familial adenomatous polyposis (J-FAPP Study III): a multicenter prospective interventional study.

BACKGROUND: Total colectomy is the standard treatment for familial adenomatous polyposis (FAP). Recently, an increasing number of young patients with FAP have requested the postponement of surgery or have refused to undergo surgery. We aimed to evaluate the effectiveness of intensive endoscopic removal for downstaging of polyp burden (IDP) in FAP. METHOD: A single-arm intervention study was conducted at 22 facilities. Participants were patients with FAP, aged ≥ 16 years, who had not undergone colectomy or who had undergone colectomy but had ≥ 10 cm of large intestine remaining. For IDP, colorectal polyps of ≥ 10 mm were removed, followed by polyps of ≥ 5 mm. The primary end point was the presence/absence of colectomy during a 5-year intervention period. RESULTS: 222 patients were eligible, of whom 166 had not undergone colectomy, 46 had undergone subtotal colectomy with ileorectal anastomosis, and 10 had undergone partial resection of the large intestine. During the intervention period, five patients (2.3 %, 95 % confidence interval [CI] 0.74 %-5.18 %) underwent colectomy, and three patients died. Completion of the 5-year intervention period without colectomy was confirmed in 150 /166 patients who had not undergone colectomy (90.4 %, 95 %CI 84.8 %-94.4 %) and in 47 /56 patients who had previously undergone colectomy (83.9 %, 95 %CI 71.7 %-92.4 %). CONCLUSION: IDP in patients with mild-to-moderate FAP could have the potential to be a useful means of preventing colorectal cancer without implementing colectomy. However, if the IDP protocol was proposed during a much longer term, it may not preclude the possibility that a large proportion of colectomies may still need to be performed.

Long-term Outcomes After Endoscopic Submucosal Dissection for Large Colorectal Epithelial Neoplasms: A Prospective, Multicenter, Cohort Trial From Japan.

BACKGROUND & AIMS: To determine the long-term outcomes after colorectal endoscopic submucosal dissection (ESD), we conducted a large, multicenter, prospective cohort trial with a 5-year observation period. METHODS: Between February 2013 and January 2015, we consecutively enrolled 1740 patients with 1814 colorectal epithelial neoplasms ≥20 mm who underwent ESD. Patients with noncurative resection (non-CR) lesions underwent additional radical surgery, as needed. After the initial treatment, intensive 5-year follow-up with planned multiple colonoscopies was conducted to identify metastatic and/or local recurrences. Primary outcomes were overall survival, disease-specific survival, and intestinal preservation rates. The rates of local recurrence and metachronous invasive cancer were evaluated as the secondary outcomes. RESULTS: The 5-year overall survival, disease-specific survival, and intestinal preservation rates were 93.6%, 99.6%, and 88.6%, respectively. Patients with CR lesions had no metastatic occurrence, and patients with non-CR lesions had 4 metastatic occurrences. Kaplan-Meier curves revealed that overall survival and disease-specific survival rates were significantly higher in patients with CR lesions than in those with non-CR lesions (P > .001 and P = .009, respectively). Local recurrence occurred in only 8 lesions (0.5%), which were successfully resected by subsequent endoscopic treatment. Multiple logistic regression analyses revealed that piecemeal resection (hazard ratio, 8.19; 95% CI, 1.47-45.7; P = .02) and margin-positive resection (hazard ratio, 8.06; 95% CI, 1.76-37.0; P = .007) were significant independent predictors of local recurrence after colorectal ESD. Fifteen metachronous invasive cancers (1.0%) were identified during surveillance colonoscopy, most of which required surgical resection. CONCLUSIONS: A favorable long-term prognosis indicates that ESD can be the standard treatment for large colorectal epithelial neoplasms. CLINICAL TRIAL REGISTRATION NUMBER: UMIN000010136.

Characteristics of colorectal neuroendocrine tumors in patients prospectively enrolled in a Japanese multicenter study: a first report from the C-NET STUDY.

BACKGROUND: This is the first report from a multicenter prospective cohort study of colorectal neuroendocrine tumor (NET), the C-NET STUDY, conducted to assess the long-term outcomes of the enrolled patients. This report aimed to elucidate the clinicopathological features of the enrolled patients and lesions. METHODS: Colorectal NET patients aged 20-74 years were consecutively enrolled and followed up at 50 institutions. The baseline characteristics and clinicopathological findings at enrollment and treatment were assessed. RESULTS: A total of 495 patients with 500 colorectal NETs were included. The median patient age was 54 years, and 85.3% were asymptomatic. The most frequent lesion location was the lower rectum (88.0%); 99.4% of the lesions were clinically diagnosed to be devoid of metastatic findings, and 95.4% were treated with endoscopic resection. Lesions < 10 mm comprised 87.0% of the total, 96.6% had not invaded the muscularis propria, and 92.6% were classified as WHO NET grade 1. Positive lymphovascular involvement was found in 29.2% of the lesions. Its prevalence was high even in small NETs with immunohistochemical/special staining for pathological assessment (26.4% and 40.9% in lesions sized < 5 mm and 5-9 mm, respectively). Among 70 patients who underwent radical surgery primarily or secondarily, 18 showed positive lymph node metastasis. CONCLUSIONS: The characteristics of real-world colorectal NET patients and lesions are elucidated. The high positivity of lymphovascular involvement in small NETs highlights the necessity of assessing the clinical significance of positive lymphovascular involvement based on long-term outcomes, which will be examined in later stages of the C-NET STUDY. TRIAL REGISTRATION NUMBER: UMIN000025215.

Optimal surveillance interval after piecemeal endoscopic mucosal resection for large colorectal neoplasia: a multicenter randomized controlled trial.

BACKGROUND AND AIMS: Colorectal neoplastic lesions (≥ 20 mm) are commonly treated via piecemeal endoscopic mucosal resection (p-EMR) but have a high rate of local recurrence. We aimed to clarify the optimal surveillance interval after p-EMR for these neoplasias. METHODS: In this multicenter (15 participating institutions) prospective, randomized trial, 180 patients recruited over a 4-year period and were classified based on tumor location, tumor diameter, histological diagnosis, institution, and number of resected specimens. The patients underwent curative p-EMR followed by scheduled surveillance colonoscopy at 3, 6, 12, and 24 months after p-EMR (group A; n = 90) or at 6, 12, and 24 months after p-EMR (group B; n = 90). The primary endpoint was cumulative local recurrence at 6 months after p-EMR. Secondary endpoints included local recurrence and the cumulative surgical resection rate of recurrent tumors during the 24-month follow-up period. RESULTS: The median tumor diameter was 25 mm (IQR 20-30). Six months after p-EMR, 12 and 6 local recurrences were noted in groups A and B, which corresponded to 13 and 8 recurrences, respectively, during the 24-month surveillance period. The primary and secondary endpoints of recurrence were not significantly different between the groups on either intention-to-treat or per-protocol analysis; no surgery case was observed in group B when a strict surveillance protocol of 6-, 12-, and 24-month follow-up post-EMR was followed. CONCLUSIONS: For patients who underwent p-EMR for neoplastic lesions, additional postprocedural 3-month surveillance did not show superior results in detecting recurrence compared with a 6-month surveillance interval. CLINICAL TRIAL REGISTRATION: UMIN000015740.

Outcomes of endoscopic submucosal dissection for colorectal neoplasms: Prospective, multicenter, cohort trial.

OBJECTIVES: Endoscopic mucosal resection (EMR) is the gold standard for the treatment of noninvasive large colorectal lesions, despite challenges associated with nonlifting lesions and a high rate of local recurrence. Endoscopic submucosal dissection (ESD) offers the possibility of overcoming these EMR limitations. However, a higher risk of complications and longer procedure time prevented its dissemination. As ESD now provides more stable results because of standardized techniques compared with those used earlier, this study aimed to quantify the rates of en bloc and curative resections, as well as ESD complications, in the present situation. METHODS: A multicenter, large-scale, prospective cohort trial of ESD was conducted at 20 institutions in Japan. Consecutive patients scheduled for ESD were enrolled from February 2013 to January 2015. RESULTS: ESD was performed for 1883 patients (1965 lesions). The mean procedure time was 80.6 min; en bloc and curative resections were achieved in 1759 (97.0%) and 1640 (90.4%) lesions, respectively, in epithelial lesions ≥20 mm. Intra- and postprocedural perforations occurred in 51 (2.6%) and 12 (0.6%) lesions, respectively, and emergency surgery for adverse events was performed in nine patients (0.5%). CONCLUSIONS: This trial conducted after the standardization of the ESD technique throughout Japan revealed a higher curability, shorter procedure time, and lower risk of complications than those reported previously. Considering that the target lesions of ESD are more advanced than those of EMR, ESD can be a first-line treatment for large colorectal lesions with acceptable risk and procedure time. (Clinical Trial Registration: UMIN000010136).

Paget's disease derived in situ from reserve cell hyperplasia, squamous metaplasia, and squamous cell carcinoma of the esophagogastric junction: a case report.

BACKGROUND: Extramammary Paget's disease (EMPD) of the esophagus is a rare tumor, with most cases originating from invasive adenocarcinoma of the esophagus. Pure esophageal Paget's disease, in which no underlying invasive carcinoma component is present, is extremely rare. In this report, we describe a case of EMPD of the esophagogastric junction with no evidence of invasive carcinoma. CASE PRESENTATION: An 81-year-old Japanese woman with a 2-week history of abdominal distension presented to our hospital for assessment. Endoscopic examination revealed a mild elevated granular lesion, with a slightly depressed irregular mucosa, in the distal esophagus, with EMPD confirmed by biopsy. Thoracoscopic esophagectomy with lymph node dissection was performed, with Paget cells observed on microscopic examination in the lower part of the esophageal epithelium. Only a few Paget cells stained positively for PAS/Alcian blue. Immunohistochemically, negative staining for CK5 and p63 were identified in the Paget cells, with positive staining for CK7. Furthermore, an intraepithelial squamous cell carcinoma, with squamous metaplasia and reserve cell hyperplasia, was observed in the gastric mucosa of the esophagogastric junction, adjacent to the Paget cells. CONCLUSIONS: EMPD of the esophagus is a rare disease. We report a case of EMPD that was probably derived from a gastric squamous cell carcinoma, with squamous cell metaplasia and reserve cell hyperplasia, in the esophagogastric junction, which, to our knowledge, is the first report of this type of EMPD in the clinical literature.

Detectability of colorectal neoplastic lesions using a novel endoscopic system with blue laser imaging: a multicenter randomized controlled trial.

BACKGROUND AND AIMS: Most studies have not reported an improvement in the detection of adenomas with the use of image-enhanced colonoscopy methods, possibly because of the darkness of the images. To overcome this limitation, a new-generation endoscopic system has been developed. This system has 2 blue-laser imaging (BLI) observation modes. The BLI observation was set to BLI-bright mode to detect lesions. We aimed to evaluate the efficacy of BLI in detecting lesions. METHODS: This study was designed as a randomized controlled trial with participants from 8 institutions. We enrolled patients aged ≥40 years. The participants were randomly assigned to 2 groups: observation by using white-light imaging (WLI) with a conventional xenon light source (WLI group) or observation by using BLI-bright mode with a laser light source (BLI group). All of the detected lesions were resected or had a biopsy taken for histopathologic analysis. The primary outcome was the mean number of adenomas per patient (MAP) that were detected per procedure. RESULTS: The WLI and BLI groups consisted of 474 and 489 patients, respectively. The MAP was significantly higher in the BLI group than in the WLI group (mean ± standard deviation [SD] WLI 1.01 ± 1.36, BLI 1.27 ± 1.73; P = .008). Adenoma detection rate in the BLI group was not significantly higher than in the WLI group. Observation times differed significantly, with BLI (9.48 minutes) being longer than WLI (8.42; P < .001). The mean (± SD) number of polyps per patient was significantly higher in the BLI group compared with the WLI group (WLI 1.43 ± 1.64, BLI 1.84 ± 2.09; P = .001). CONCLUSIONS: A newly developed system that uses BLI improves the detection of adenomatous lesions compared with WLI. (Clinical trial registration number: UMIN 000014555.).

Poorly cohesive adenocarcinoma of the ampulla of Vater: a case report.

A 47-year-old Japanese male was submitted to pancreaticoduodenectomy for an ampullary cancer. Pathologically, the ampullary cancer was poorly cohesive adenocarcinoma without tubular structure. Moreover, locoregional lymph nodes were swollen with hypervascularity, plasmacytes infiltration, and hemorrhage. Our case seems to be different from usual poorly differentiated adenocarcinoma.

Evaluation of abdominal circumference and salivary amylase activities after unsedated colonoscopy using carbon dioxide and air insufflations.

OBJECTIVE: To assess and compare abdominal distention and stress in unsedated colonoscopy using carbon dioxide (CO2 ) and air insufflations. METHODS: Two hundred and five patients underwent colonoscopic examinations without sedation using either CO2 or air insufflation. Abdominal circumference and salivary amylase (sAMY) activities before and 0 and 15 min after colonoscopy were measured by a nurse who was blinded to the grouping of the patients. RESULTS: In all, 102 and 103 patients were randomly recruited in the CO2 and air insufflation groups, respectively. sAMY activities before and 0 and 15 min after colonoscopy were not significantly different between the two groups. Abdominal circumference measured immediately and 15 min after colonoscopy was significantly smaller in CO2 insufflation group than in the air insufflation group (81.2 cm vs 84.0 cm, and 79.7 cm vs 83.6 cm, respectively; P <0.05). The increasing ratio of abdominal circumference immediately after colonoscopy was not significantly different between the two groups; however, the ratio at 15 min after colonoscopy using CO2 insufflation was significantly lower than that in the air insufflation group (1.007 vs 1.028, P <0.001). CONCLUSION: sAMY activities after unsedated colonoscopy using CO2 insufflation were not improved; however, CO2 insufflation decreases abdominal circumference after colonoscopy compared with air insufflation.

A case of mixed adenoneuroendocrine carcinoma of the stomach with focal intestinal metaplasia and hypergastrinemia.

Among neuroendocrine neoplasms, mixed exocrine and endocrine characteristics with at least 30% of each component are classified into mixed adenoneuroendocrine carcinoma (MANEC), according to the 2010 World Health Organization classification. We experienced a rare case of MANEC of the stomach with focal intestinal metaplasia and hypergastrinemia. A 76-year-old Japanese male was diagnosed as having gastric adenocarcinoma and underwent total gastrectomy. The pathologic diagnosis was MANEC of the stomach accompanied by unusual mucosal atrophy without Helicobacter pylori infection, the characteristics of which were different from both type A and type B atrophic gastritis. The patient has a history of long-term use of a proton pump inhibitor. Additional serum chemistry examination using preoperatively obtained plasma from the patient revealed hypergastrinemia. The mechanism of gastric MANEC carcinogenesis is still unclear, but that might be correlated with unusual intestinal metaplasia and hypergastrinemia in this case.

Intrahepatic biliary dilatation caused by a small simple hepatic cyst: report of a case.

Biliary obstruction caused by small simple cysts is very rare. We present a case of biliary dilatation caused by a simple cyst with a 4-cm diameter. The patient was a 75-year-old woman referred to our hospital for evaluation of a cystic tumor associated with peripheral biliary duct dilatation in the left segment of the liver. Computed tomography and magnetic resonance imaging showed that the cyst probably communicated with the intrahepatic bile duct. Malignant tumors, including intrahepatic papillary neoplasms of the bile duct, could not be ruled out; therefore, we performed surgery with the patient's consent. Histopathologic examination of the resected liver showed that the cystic lesion was a simple cyst. The finding that even small simple cysts can obstruct the biliary tract is important for the management of cystic lesions of the liver.

Recurrent, spontaneous esophageal ruptures associated with antiphospholipid antibody syndrome: report of a case.

A 52-year-old man was admitted to our hospital with a spontaneous esophageal rupture (Boerhaave syndrome) and was successfully treated. Eight years after the first incident, he was readmitted with a recurrent rupture. Recurrence of Boerhaave syndrome is extremely rare, with only 7 cases reported in the English literature. During treatment, the patient was also diagnosed with antiphospholipid syndrome (APS). Although APS is known to cause a variety of symptoms due to vascular thrombosis, recurrence of Boerhaave syndrome, coincident with APS, has never been reported. The pathogenesis of Boerhaave syndrome has not been clearly determined. This report serves to increase awareness of the risk of APS, which results in an increased risk of spontaneous rupture of the esophagus.

Propofol sedation during endoscopic treatment for early gastric cancer compared to midazolam.

Endoscopic submucosal dissection (ESD) has been proposed as the gold standard in the treatment of early gastric cancer because it facilitates a more accurate histological assessment and reduces the risk of tumor recurrence. However, the time course of ESD for large gastric tumors is frequently prolonged because of the tumor size and technical difficulties and typically requires higher doses of sedative and pain-controlling drugs. Sedative or anesthetic drugs such as midazolam or propofol are used during the procedure. Therapeutic endoscopy of early gastric cancers can often be performed with only moderate sedation. Compared with midazolam, propofol has a very fast onset of action, short plasma half-life and time to achieve sedation, faster time to recovery and discharge, and results in higher patient satisfaction. For overall success, maintaining safety and stability not only during the procedure but also subsequently in the recovery room and ward is necessary. In obese patients, it is recommended that the injected dose be based on a calculated standard weight. Cooperation between gastroenterologists, surgeons, and anesthesiologists is imperative for a successful ESD procedure.

Mucosal esophageal squamous cell carcinoma with intramural gastric metastasis invading liver and pancreas: a case report.

A 59-year-old Japanese man was admitted to our hospital because of a 1-month history of dysphagia. Endoscopic examination revealed a superficial esophageal squamous cell carcinoma and a giant gastric tumor. Computed tomography showed that the gastric tumor was directly invading the liver and pancreas. Because of the risk of the gastric tumor causing obstruction and bleeding, we performed a subtotal esophagectomy, proximal gastrectomy, left lateral segmentectomy of liver, and pancreatosplenectomy with gastric tube reconstruction. Final pathological findings were superficial esophageal carcinoma penetrating the muscularis mucosae with an intramural gastric metastasis directly invading the liver and pancreas. The patient received postoperative adjuvant chemotherapy, yet died 8 months postoperatively of complications of local recurrence. Early-stage esophageal carcinoma with intramural gastric metastasis is very rare. To our knowledge, this is the first case of mucosal esophageal carcinoma with intramural gastric metastasis directly invading other organs.

Evaluation of Pharyngeal Function between No Bolus and Bolus Propofol Induced Sedation for Advanced Upper Endoscopy.

This study aimed to assess pharyngeal function between no bolus and bolus propofol induced sedation during gastric endoscopic submucosal dissection. A retrospective study was conducted involving consecutive gastric cancer patients. Patients in the no bolus group received a 3 mg/kg/h maintenance dose of propofol after the initiation of sedation without bolus injection. All patients in the bolus group received the same maintenance dose of propofol with bolus 0.5 mg/kg propofol injection. Pharyngeal functions were evaluated endoscopically for the first 5 min following the initial administration of propofol. Fourteen patients received no bolus propofol induction and 13 received bolus propofol induction. Motionless vocal cords were observed in 2 patients (14%) in the no bolus group and 3 (23%) in the bolus group. Trachea cartilage was not observed in the no bolus group but was apparent in 6 patients (46%) in the bolus group (P < 0.01). Scope stimulated pharyngeal reflex was observed in 11 patients (79%) in the no bolus group and in 3 (23%) in the bolus group (P < 0.01). Propofol induced sedation without bolus administration preserves pharyngeal function and may constitute a safer sedation method than with bolus.

A case of very well-differentiated adenocarcinoma with carcinoid tumor in the ascending colon.

Malignant tumors with mixed glandular and neuroendocrine characteristics with at least 30% of each component are classified as mixed adenoneuroendocrine carcinoma (MANEC) by the World Health Organization 2010 classification. We report here a case of very well-differentiated adenocarcinoma accompanied by carcinoid tumor, categorized as MANEC. A 41-year-old Japanese man was clinically diagnosed with ascending colon cancer and underwent right hemicolectomy. Using an immunohistologic technique, the pathologic diagnosis was very well-differentiated adenocarcinoma accompanied by carcinoid tumor and marked eosinophil infiltration, which was categorized as MANEC. By immunohistochemical analysis, tumor cells of the carcinoid component exhibited very low proliferation activity. Our case was thought to be MANEC without high malignant potential. MANEC as per the World Health Organization 2010 classification seems to include tumors with diverse grades of malignancy, and it might need to have subclassifications according to the malignancy potential of the tumor cells.

Retroperitoneal liposarcoma with leiomyosarcomatous differentiation.

We herein describe a 60-year-old Japanese man with a giant retroperitoneal liposarcoma undergoing leiomyosarcomatous differentiation. He was admitted to our hospital because of a 5-month history of dysphagia and abdominal distention. Abdominal computed tomography showed a giant tumor that occupied the entire retroperitoneal space. The majority of the mass was lipomatous and low density; both a heterogenous and solid mass were also present. A giant retroperitoneal liposarcoma was diagnosed, and tumor resection was performed. At surgery, the tumor was mostly isolated from the retroperitoneum and other organs. Histopathologically, the tumor comprised well-differentiated and dedifferentiated liposarcoma with heterologous differentiation of the leiomyosarcomatous components, which is a rare phenomenon in liposarcoma. The patient was alive 3 years after the first treatment, although he has had 3 local recurrences (approximately one recurrence yearly) and has been treated by repeated resection and radiotherapy.

Comparison of narrowband imaging with autofluorescence imaging for endoscopic visualization of superficial squamous cell carcinoma lesions of the esophagus.

Aim. To compare narrowband imaging (NBI) and autofluorescence imaging (AFI) endoscopic visualization for identifying superficial esophageal squamous cell carcinoma (SCC). Methods. Twenty-four patients with superficial esophageal carcinomas diagnosed at previous hospitals were enrolled in this study. Lesions were initially detected using white-light endoscopy and then observed with both NBI and AFI. Endoscopic images documented each method, and three endoscopists experienced in esophageal imaging retrospectively reviewed respective images of histologically confirmed esophageal SCCs. Images were assessed for quality in identifying superficial SCCs and rated as excellent, fair, or poor by the three reviewers with interobserver agreement calculated using kappa (κ) statistics. Results. Thirty-one lesions histologically confirmed as superficial esophageal SCCs were detected in 24 patients. NBI images of 27 lesions (87%) were rated as excellent, three as fair, and one as poor compared to AFI images of 19 lesions (61%) rated as excellent, 10 as fair and two as poor (P < 0.05). Moderate interobserver agreement (κ = 0.42, 95% CI 0.24-0.60) resulted in NBI while fair agreement (κ = 0.35, 95% CI 0.18-0.51) was achieved using AFI. Conclusion. NBI may be more effective than AFI for visualization of esophageal SCC.

A case of diffuse neuronal hypertrophy in acute appendicitis.

We report a case of neuronal hypertrophy associated with acute appendicitis in which significant neuronal fibers and the number of ganglion cells increased in the absence of inflammatory cells. Differential diagnosis from diffuse ganglioneuromatosis by the pathologic findings of resected specimen was difficult. A 33-year-old Japanese female visited our hospital complaining of acute abdominal pain. The patient underwent appendectomy upon the diagnosis of acute appendicitis on the day of admission. Postoperative examinations found no neoplastic lesions in other organs or inherited disorders such as multiple endocrine neoplasia (MEN) type 2b and von Recklinghausen's disease (VRD). The pathologic diagnosis was neuronal hypertrophy of the appendix. Because the clinical outcomes of diffuse ganglioneuromatosis and neuronal hypertrophy of the gastrointestinal tract are quite different, clinical and pathologic examination should be carefully carried out for lesions in which significant proliferation of neuronal components is seen.