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Enteritis cystica profunda (ECP), a rare and benign condition, is defined as the displacement of the glandular epithelium into the submucosa and more profound layers of the small intestinal wall leading to the formation of mucin-filled cystic spaces. ECP frequently occurs in the ileum or jejunum and is associated with diseases such as Crohn disease and Peutz-Jeghers syndrome. ECP also develops in the absence of known pathology. ECP in the duodenum is very rare and mostly occurs without associated conditions. In this report, we present a rare case of ECP without an associated disease, in the second portion of the duodenum distal to the ampulla of Vater and coexisting with lipoma within the polypoid lesion.

Gastric cystica profunda (GCP) is a rare disease characterized by multiple cystic lesions in the mucosa and/or submucosal layer. Usually, GCP occurs in stomachs that have previously been operated on. If there is no postoperative pathological results, it is challenging to diagnose GCP based on nonspecific clinical symptoms and imaging findings. This report aimed to provide a comprehensive overview of all cases of GCP reported to date. A comprehensive literature search was conducted for all reported GCP cases between 1972 and 2014. The keywords searched included "gastritis cystica profunda", "submucosal cysts of the stomach", and "heterotopic submucosal gastric glands". One retrospective case from our group was also reported and compared with those from the existing literature. A total of 52 cases were found including 37 (71.2%) men and 15 (28.8%) women (M/F ratio =2.5). The mean age of the patients was 59.9 (range, 39-91) years old. Among the cases, 58.8% (n=30) of lesions were located in the gastric body, 25.5% (n=13) of lesions were located in the fundus, 19.6% (n=9) of lesions were located in the antrum, and 3.9% (n=2) of lesions were located in the cardia, while 1 case was in the prepyloric lesion and 1 case was at the anastomotic site. Of the patients, 52% (n=26) had previously received gastric surgery. The main manifestations of GCP included abdominal pain (n=14, 36.8%) and gastrointestinal bleeding (including hematemesis and melena, n=7, 18.4%). Only 4 of the 52 cases were diagnosed before surgery, and the rest were diagnosed through postoperative histopathologic examination. GCP is difficult to correctly diagnose preoperatively due to its relative rarity and lack of typical clinical symptoms. Histopathological examination should be used for correct diagnosis. Complete surgical removal of the GCP is widely considered as the best treatment option.

Background Lipomas are the most common cause of intussusception in adults. To our knowledge, however, no cases of lipoma and ectopic gastric mucosa with gastritis cystica profunda (GCP) have been reported. We report a case of intussusception caused by a small intestinal lipoma with ectopic gastric mucosa containing GCP-component cells within the inverted Meckel’s diverticulum. Case presentation A female in her 40s underwent computed tomography for postoperative follow-up of left breast cancer. A tumor, suspected to be a lipoma, was found in the ileum. Since there were no symptoms, the patient underwent regular follow-up. However, gradual enlargement was observed, and surgery was recommended due to the risk of intussusception. After reduction via the Hutchinson technique, laparoscopically assisted partial resection of the small intestine was performed due to suspicion that the tumor was causing intussusception starting from the ileum. Histopathologic examinations revealed proliferation of mature adipose tissue in the subserosal layer, which was diagnosed as lipoma. Furthermore, adipose tissue was found in the stem area and accordingly, we diagnosed lipoma associated with the inverted Meckel’s diverticulum. Moreover, gastric mucosa-like crypt epithelium and proper glandular tissue were identified in the mucosal membrane at the area of onset, and signs of gastric pit dilatation over the submucosa and crypt epithelium hyperplasia were observed. Diagnosis was ectopic gastric mucosa containing GCP component tissue. Conclusions Intussusception in the small intestine complicated with lipoma and ectopic gastric mucosa with GCP within the Meckel’s diverticulum has not been reported, demonstrating the rarity of our case.

Enteritis cystica profunda (ECP) is an uncommon benign condition arising after mucosal damage. We describe 2 cases of small intestinal adenocarcinomas associated with ECP at the distal ileum, one in a background of active Crohn ileitis (case 1), the other 22 years after pelvic radiation therapy (case 2). Both patients presented with small bowel obstruction and received ileocectomy. Macroscopic examination identified an indurated/strictured area in the distal ileum. Histologically, both cases showed a low-grade tubuloglandular adenocarcinoma arising in a background of chronic ischemic stricture and ECP lined by flat cuboidal cells with mild cytologic atypia resembling pancreatobiliary-type epithelium. There was no conventional dysplasia in the surface or adjacent mucosa. Immunohistochemically, both ECP with metaplasia and invasive carcinomas were diffusely positive for CK7 and CK19, while focally positive for CDX2 or CK20. Both cases showed normal wild-type p53 expression. Case 2 was also mismatch repair protein proficient, with membranous β-catenin staining, and retained nuclear SMAD4 expression. In summary, the 2 cases uniquely exhibits "enteritis-metaplasia-carcinoma" sequence, which has not been reported before. This process appears to bypass conventional dysplasia, be slow and indolent, independent of p53, APC/β-catenin, and SMAD4/TGFβ signaling pathways.

This publication reports enteritis cystica profunda (ECP) in the youngest patient described in the literature, being the fifth case that demonstrates an association between ECP and intestinal intussusception and the seventh case of ECP in children. A 30-day-old boy presented intense abdominal pain associated with digestive bleeding with “red currant jelly” stool. He had a history of bloody stool at 15 days old. Due to family history of atopy and normal physical examination, allergic proctocolitis was suspected and the patient temporarily improved after cow's milk and dairy products were eliminated from his mother’s diet. Abdominal ultrasound confirmed the hypothesis of intestinal intussusception. During the surgical approach, ileoileal invagination was evident together with a polypoid tumor of the intestinal wall. Resection of the lesion and ileoileal anastomosis was performed. In the postoperative period, he presented intestinal adhesions, with the need of reoperation and, subsequently, dehiscence of the aponeurosis and intestinal perforation. The patient underwent operation with good recovery. He was discharged in good health and maintained satisfactory clinical progress. The histopathological study of the resected intestine (terminal ileum) revealed a lesion formed by cystic dilations with invasion to the submucosa and musculature of the intestinal wall, lined by intestinal epithelium cells and without nuclear atypia; these findings are compatible with ECP.

Rationale: Gastritis cystica profunda (GCP) is a rare gastric lesion involving cystic dilation of the gastric glands extending into the submucosa. It is usually observed at anastomotic sites in the stomach of patients who have undergone gastric procedures. Bile reflux GCP is rare in patients who have not undergone gastric surgery. Here, we report a rare case of a patient with GCP associated with bile reflux, who had no history of gastric surgery. Patient concerns: A 50-year-old man presented with intermittent abdominal fullness for 2 years, along with nausea. He had never undergone gastric surgery. Endoscopic ultrasonography (EUS) showed a thickened gastric wall and an echo-poor submucosal layer of the gastric fundus. A 3 cm × 2 cm × 1.5 cm lesion was noted. Diagnosis: Bile reflux GCP Interventions: Endoscopic retrograde cholangiopancreatography and endoscopic submucosal dissection (ESD) were performed, and the lesion was removed. Conventional pathological examination revealed GCP with glands hyperplasia and a yellow-brown deposit, which was considered bile. The findings were consistent with a diagnosis of GCP without malignancy. Outcomes: Upper gastrointestinal barium meal revealed postoperative changes at the gastric fundus. Gastroscopy performed at 6 months after surgical resection showed superficial gastritis with bile reflux. Lessons: The findings suggest that GCP etiology varies and that GCP can be caused by bile reflux but without malignancy. Additionally, GCP is not limited to patients who have previously undergone gastric surgery. Moreover, it is difficult to identify. EUS and ESD might be good approaches for the diagnosis and treatment of GCP.

Twenty-one lesions from 19 patients with chronic irradiation enteritis (CIE) were examined to elucidate correlations with the histological findings and either the elapsed time interval or the macroscopic features. The lesions were divided into the early CIE group (E group; the lesions resected within 2 years after irradiation) of 10 lesions and the late CIE group (L group; the lesions resected more than 8 years after irradiation) of 11 lesions. Based on the macroscopic features, the lesions of CIE were divided into three types: ulcerative stricture type (U type; 11 lesions), serosal adhesion type (A type; 6 lesions) and wall sclerosing type (S type; 4 lesions). Only A type lesions were observed in the E group, and U type lesions were significantly more frequently encountered in the L group (9 of 11; 82%) than in the E group (2 of 10; 20%). Moderately to markedly degenerated changes of the vessel wall (8 of 11; 73%), enteritis cystica profunda (8 of 11; 73%), atypical epithelia (7 of 11; 64%), and the occurrence of fistula (2 of 11; 18%) were all significantly more frequently present in the L group than in the E group. No radiation-induced colorectal carcinomas were observed. The authors thus conclude that CIE is a slowly progressive disease. The late CIE showed macroscopically ulcerative stricture type properties with tissue degradation, such as fistulas, perforation, and dysplastic epithelia compared with early CIE; thus, long-standing CIE should be followed for the early identification of further complications. The classification of CIE based on macroscopic features is, therefore, considered to be useful to understand the clinical course of this disease better.

A gastric inverted hyperplastic polyp is a rare type of gastric polyp and is characterized by downward growth of a variety of mucosal components into the submucosa. The polyp consists of columnar cells resembling foveolar epithelium and pyloric gland epithelium and can coexist with gastritis cystica profunda. Frequently, adenocarcinoma can coexist, but the relation is not clear. A 77-year-old male underwent endoscopic submucosal dissection due to early gastric cancer. A gastric inverted hyperplastic polyp was found in the removed specimen and gastric cystica profunda was also found. We report a case of gastric inverted hyperplastic polyp coexisting with gastric cystica profunda and gastric adenocarcinoma.

Enteritis cystica profunda is a very rare disease in which a mucin-filled cystic space is surrounded partially with nonneoplastic columnar epithelium in the submucosa of the small intestine. Most of the cases are accompanied by intestinal diseases, and the disease usually occurs in the jejunum and the ileum and there has been no report of a case that occurred in the ampulla of Vater. A 58-year-old healthy female patient without any particular symptom visited the hospital to get additional examination for a mass found on the ampulla of Vater by accident. In esophagogastroduodenoscopy, a cystic mass showing a positive pillow sign was found on the ampulla of Vater. Endoscopic retrogradecholangiopancreatography was conducted as choledochocele was suspected, but no abnormality was found in the biliary system. In endoscopic ultrasonography, multiseptated cystic structures were detected in the submucosal layer of the duodenum. The lesion was resected completely through endoscopic snare polypectomy and the case was histologically diagnosed as enteritis cystica profunda.

Enteritis cystica profunda and diffuse colitis cystica profunda are exceedingly rare conditions. We report here the first case in which these two entities are associated in one patient. This 48-year-old woman presented initially with a solitary ulcer of the rectum and developed later deep ileal and colonic ulcerations with intervening normal mucosa, polypoid masses, and a dense fibrosis of the bowel wall. Colon and ileum specimens demonstrated submucosal mucous cysts and occasionally herniation of mucosal epithelium into the submucosa, which led to the diagnosis of enteritis and colitis cystica profunda. The exact nature of the underlying ileocolonic disease, which necessitated colectomy and extensive ileal resection, remained undetermined although an aggravating role of iterative surgical procedures is possible. Enteritis cystica profunda and diffuse colitis cystica profunda therefore seem to share a common pathogeny and have to be considered as lesions complicating an underlying disease rather than specific and autonomic diseases.

A 60-year-old male was admitted to the hospital because of abdominal pain and vomiting. Ultrasonography, abdominal CT and barium enema indicated an intussusception at the ileocecal region. On laparotomy ileo-ileo-colic intussusception extending to the ascending colon through the cecum was noticed. After reducing the intussus-ception manually, the ileum including the tumor was excised with end-to-end anastomosis. The resected tumor was the submucosal tumor located about 18cm distant from the ileum end. Histologically it was a hamartomatous polyp. Since dislocated mucosa was found in the submucosal layer and muscle layer, the tumor was diagnosed as enteritis cystica profounda. Our recent experience with intussusception due to enteritis cystica profounda of the small intestine is reported together with a reveiw of the literature.

Diaphragm disease (DD) is an uncommon gastrointestinal abnormality typical of nonsteroidal anti-inflammatory drug (NSAID)-induced injury. DD of the small intestine is well defined in its gross appearance (multiple circumferential stenosing lesions); however, its histologic features have been studied in few cases. To better define and update the histologic features of DD, we describe 10 cases in which patients underwent resection of portions of small intestine for DD. Selected gross features and 12 microscopic features were assessed. The typical gross appearance of DD was associated with focal chronic injury of the mucosa with mild to moderate inflammatory infiltrate. A wide array of additional abnormalities was found: eosinophilic enteritis (3 cases), inflammatory fibroid polyp (1 case), enteritis cystica profunda (1 case), villous atrophy (1 case), and neuromuscular and vascular hamartoma-like changes (9 cases). DD and, therefore, NSAID-related injury, should be considered in the differential diagnosis of several conditions affecting the small intestine.

Therapeutic pelvic irradiation is notorious for the production of clinically significant sequela after a long latency. One of the rarest of these complications is proctitis cystica profunda (PCP). To study the histologic changes of chronic radiation proctitis, we evaluated 35 female Wistar rats that had received a single exposure of 22.5 Gy of radiation to the rectum and were then followed for one year. We identified PCP and its precursor lesions in 18 rats. The fully developed lesion consisted of a focal expansion of the submucosa by dilated cystic spaces lined by a single layer of benign epithelial cells. Usually, PCP evolved as glands herniated between small defects in the muscularis mucosae. Mitotic figures were not recognized in the cells lining the herniating glands. In two rats, the radiation had apparently caused large ulcers, which had subsequently reepithelialized, resulting in prominent submucosal glandular tissue. Although the number of goblet cells in the displaced epithelium was reduced, the cells had rather mature appearances ultrastructurally. Glands displaced into the submucosa were encased by an intact basal lamina but lacked in muscularis.

Human intestinal capillariasis is a rare parasitosis that was first recognized in the Philippines in the 1960 s. Parasitosis is a life threatening disease and has been reported from Thailand, Japan, South of Taiwan (Kaoh-Siung), Korea, Iran, Egypt, Italy and Spain. Its clinical symptoms are characterized by chronic diarrhea, abdominal pain, borborygmus, marked weight loss, protein and electrolyte loss and cachexia. Capillariasis may be fatal if early treatment is not given. We reported 14 cases living in rural areas of Taiwan. Three cases had histories of travelling to Thailand. They might have been infected in Thailand while stayed there. Two cases had the diet of raw freshwater fish before. Three cases received emergency laparotomy due to peritonitis and two cases were found of enteritis cystica profunda. According to the route of transmission, freshwater and brackish-water fish may act as the intermediate host of the parasite. The most simple and convenient method of diagnosing capillariasis is stool examination. Two cases were diagnosed by histology. Mebendazole or albendezole 200 mg orally twice a day for 20-30 d is the treatment of choice. All the patients were cured, and relapses were not observed within 12 mo.

Enteritis cystica profunda (ECP) is a very rare hyperplastic benign lesion of the small intestine. Histologically, it is characterized by mucin-filled cystic spaces frequently lined by benign epithelium within the submucosa and muscularis propria. ECP occurs much less frequently than gastritis cystica profunda (GCP) or colitis cystica profunda (CCP). It is most often seen in adults with Crohn`s disease, Peutz-Jeghers syndrome, hamartomatous polyp, or congenital anomalies. We encountered a case of ECP (90 × 15 mm) accompanied by an elongated lipoma in the duodenal bulb, in the absence of any other disease. An endoscopic polypectomy proved sufficient for diagnosing and treating the ECP. To our knowledge, this is the first reported case of ECP accompanied by a lipoma in the duodenal bulb in the absence of any other intestinal disease.

Although well described in the colon, enteritis cystica profunda is an extremely rare lesion of the small intestine with only a few cases documented in English medical literature. It is a benign condition most often associated with Crohn's disease or Peutz-Jeghers syndrome in the adult. It has been described presenting as ileoileal intussusception in the pediatric population. We report a case of enteritis cystica profunda found incidentally on exploratory laparotomy with evidence of interval development over a 5-month period confirming trauma as the etiology.

Enteritis cystica profunda (ECP) is an exceedingly rare lesion of the small intestine. It has identical histopathologic features as colitis cystica profunda, which is mainly a disease of adults and a rare benign lesion of the distal colon and recturn of uncertain cause. Only 12 cases of ECP with only one pediatric case, have been reported in English-language medical literature. Here the authors present the second pediatric case of ECP with the literature review and discussion of the etiology.

Although there have been only 15 published case reports of enteritis cystica profunda, the condition is frequently seen by pathologists. Its importance lies in the resemblance to bowel adenocarcinoma.

L'enterite kystique profonde, une affection tout a fait inhabituelle, est caracterisee par l'apparition de cavites kystiques non neoplasiques dans la paroi de l'intestin grele. Le troisieme cas d'enterite kystique profonde chez l'enfant est presente avec une mention particuliere pour le diagnostic et le traitement. Cette rare entite peut simuler un abdomen aigu et doit rester a l'esprit dans le diagnostic differentiel. Le traitement chirurgical par excision du segment interesse est souhaitable dans ces cas.

In addition to hamartomatous polyps of the small intestine, a patient with Peutz-Jeghers disease had gross and microscopic lesions of the ileum that were analogous to colitis cystica profunda in the large intestine and rectum. To the authors' knowledge, this is the first such case in an adult to be reported. They suggest the designation, "enteritis cystica profunda." The histology of the lesions and their association with a disease known to produce hamartomatous lesions lend support to the proposition that some cases of colitis cystica are also hamartamatous. It is also believed that some reported cases of small-intestinal adenocarcinoma in patients with Peutz-Jeghers syndrome are in reality examples of the benign process of enteritis cystica profunda.

A patient with a history of abdominal discomfort and rectal bleeding was discovered to have two hamartomatous polyps, one of the jejunum and one of the colon. The former was associated with mucinous cysts at its base. The findings were similar to those described for colitis cystica profunda. This case is a rare example of cystica profunda disease of the small bowel. Uniquely, it was associated with a hamartomatous polyp.

The hereditary condition known as Peutz-Jeghers syndrome is characterized by mucosal pigmentation and gastrointestinal polyps. The polyps, usually pedunculated hamartomas, are significant only for the symptoms they cause. Intramural lesions also have been described with gross microscopic features that are often interpreted as malignant. Careful evaluation may show most of these lesions to be enteritis cystica profunda. These rarely diagnosed but benign tumors have very different implications for treatment and prognosis.

Enteritis cystica profunda (ECP), an uncommon condition, is characterized by nonneoplastic cystic spaces within the wall of the small bowel. The cases of two patients who had ECP in association with Crohn's disease are reported, and the role of mucosal damage in the pathogenesis of ECP is discussed. Pathologists must recognize this entity and distinguish it from adenocarcinoma, which may arise with increased frequency in the small bowel in patients with Crohn's disease.

Three cases of gastritis glandularis et cystica profunda are presented, two associated with severe chronic gastritis and the other with invasive gastric carcinoma and chronic gastritis. This poorly known entity consists of benign downgrowths of deep gastric glands through the muscularis mucosae into, the submucosa. The pattern is primarily adenomatous with secondary cyst formation, which varies in extent and severity. The lesion is related to the chronic gastritis but why it should occur so rarely in such a common condition as chronic gastritis remains obscure. More documented cases are needed before a meaningful analysis can be attempted.