Multiple Small Bowel Cavernous Hemangiomatosis: Case Report and Literature Review.

A 79 year old female individual presented to the hospital and complained of 1 month melena and anemia due to chronic gastrointestinal bleeding because of cavernous hemangiomatosis of the small bowel. After undergoing an initial video laparoscopic jejunal-ileal resection surgery 7 days after first hospitalization, given the persistence of anemia, she underwent laparotomic duodenojejunal resection surgery again 2 months later. Multiple cavernous hemangiomatosis is a rare vascular disease (7-10% of all benign small bowel tumors), and it often manifests with bleeding, which may be occult or massive; more rarely, it manifests with intestinal occlusion or perforation. Diagnoses often require the use of multiple radiological and endoscopic methods; video capsule endoscopy has significantly increased the diagnostic rate. The gold standard of treatment is surgical resection, whenever possible, balancing the need for radicality with the possible metabolic consequences of massive small intestine resections.

Case report and literature review: A young man with giant intra-abdominal Ewing sarcoma.

RATIONALE: Extraosseous Ewing sarcoma (EES) is a rare manifestation within the Ewing sarcoma tumor family (ESFT). Its clinical manifestations lack specificity, intestinal obstruction is the main symptom but can also present with abdominal pain, gastrointestinal bleeding, and other discomforts, making it prone to misdiagnosis as intestinal mesenchymal tumor. PATIENT CONCERNS: A 29-year-old male was admitted to the hospital with intestinal obstruction symptoms and abdominal CT suggesting "left abdominal occupation." DIAGNOSIS: The patient was initially misdiagnosed as intestinal mesenchymal tumor, and was later definitively diagnosed as abdominal Ewing sarcoma by postoperative pathology and genetic testing. INTERVENTIONS: Due to the patient's surgical indication, surgical resection with exploratory laparotomy was performed and then the patient underwent systemic chemotherapy. OUTCOMES: Intraoperatively, we found a 15-cm tumor originating from the proximal jejunum, with invasion into the peritoneum, duodenum, jejunum, and colon. Finally, the pathological report revealed Ewing sarcoma. LESSONS: Giant abdominal Ewing sarcoma with a diameter of 15 cm is rare. Considering postoperative pathology and genetic testing, abdominal Ewing sarcoma was suspected. The patient was successfully treated using surgery.

Non-specific presentation of metastatic small bowel adenocarcinoma with diagnostic challenges.

Diagnosing small bowel adenocarcinomas presents challenges due to non-specific symptoms, rarity and gastroscopy and colonoscopy's limited small intestine access, highlighting targeted diagnostic procedures' necessity. We present a late-diagnosed metastatic small bowel adenocarcinoma case in a man in his 80s who had asymptomatic mild iron-deficiency anaemia 1 year before diagnosis, with no active bleeding found on endoscopies. He experienced a single rectal bleeding episode 9 months prediagnosis, with subsequent severe iron-deficiency anaemia and no clear gastrointestinal source identified on gastroscopy. For 2 months, he had intermittent postprandial diarrhoea without abdominal pain, infectious or inflammatory causes. He experienced significant weight loss over 3 months prediagnosis. Subsequent gastroscopy indicated duodenal-gastric food retropulsion, suggesting a downstream blockage. Magnetic resonance enterography showed proximal jejunum thickening. Push enteroscopy confirmed jejunum adenocarcinoma. CT scans detected liver and peritoneal metastases. After one chemotherapy cycle, his condition worsened, leading to his passing 2 months post diagnosis.

Perforation of Jejunal Metastasis from Primary Lung Carcinoma with Brain Metastasis: A Rare Case.

A case describes a 49-year-old male patient who underwent emergency exploratory laparotomy for small intestinal perforation. Peritonitis was present due to perforation of the jejunal tumor. Resection of the jejunal tumor with perforation was performed followed by end-to-end anastomosis of the jejunum. The resected jejunal tumor was identified in the histopathological examination as metastatic from a clear cell variant of squamous cell/large cell carcinoma of the lung. It was associated with metastatic lesions in the brain. Metastasis from the lung carcinoma in the jejunum is a very rare condition predisposing to small intestinal perforation which is also associated with brain metastasis.

Incidental Finding of an Asymptomatic Jejunal Schwannoma: A Rare Case Report and Review of Literature.

BACKGROUND Schwannomas are tumors that arise from Schwann cells that surround and support nerve cells. Most common sites for presentations are head, neck, and extremities. Schwannomas of gastrointestinal tract are rare, slow-growing tumors, usually benign, arising from gastrointestinal tract's neural plexus. They are histologically distinguishable from conventional schwannomas that arise in soft tissue or the central nervous system. Preoperative diagnosis of gastrointestinal schwannoma is challenging, requiring immunohistological confirmation of the nature of the tumor. Here, we report a case of 57-year-old woman with an incidental finding of an asymptomatic submucosal jejunal schwannoma. CASE REPORT A 57-year-old woman with a medical history of hematological disorder underwent a contrast abdominal computed tomography as part of medical follow-up. The imaging revealed the presence of a jejunal mass. The patient underwent laparoscopic surgical resection of the lesion, followed by side-to-side jejuno-jejunal anastomosis with 4-cm clear surgical margins. The final pathologic study revealed the presence of jejunal schwannoma, as tested positive for S-100 protein. The patient was discharged home on the fourth postoperative day, having an uneventful recovery. CONCLUSIONS Jejunal schwannoma are usually benign and asymptomatic, and they are often discovered incidentally during diagnostic tests for other conditions; therefore, it should be included in the differential diagnosis of gastrointestinal tumors. Surgical treatment appears to be necessary to achieve a definitive diagnosis through a biopsy of the tumor tissue. Benign jejunal schwannomas have a good prognosis.

Clinicopathological features and prognosis of primary small bowel adenocarcinoma: a large multicenter analysis of the JSCCR database in Japan.

BACKGROUND: The clinicopathological features and prognosis of primary small bowel adenocarcinoma (PSBA), excluding duodenal cancer, remain undetermined due to its rarity in Japan. METHODS: We analyzed 354 patients with 358 PSBAs, between January 2008 and December 2017, at 44 institutions affiliated with the Japanese Society for Cancer of the Colon and Rectum. RESULTS: The median age was 67 years (218 males, 61.6%). The average tumor size was 49.9 (7-100) mm. PSBA sites consisted of jejunum (66.2%) and ileum (30.4%). A total of 219 patients (61.9%) underwent diagnostic small bowel endoscopy, including single-balloon endoscopy, double-balloon endoscopy, and capsule endoscopy before treatment. Nineteen patients (5.4%) had Lynch syndrome, and 272 patients (76.8%) had symptoms at the initial diagnosis. The rates for stages 0, I, II, III, and IV were 5.4%, 2.5%, 27.1%, 26.0%, and 35.6%, respectively. The 5-year overall survival rates at each stage were 92.3%, 60.0%, 75.9%, 61.4%, and 25.5%, respectively, and the 5-year disease-specific survival (DSS) rates were 100%, 75.0%, 84.1%, 59.3%, and 25.6%, respectively. Patients with the PSBA located in the jejunum, with symptoms at the initial diagnosis or advanced clinical stage had a worse prognosis. However, multivariate analysis using Cox-hazard model revealed that clinical stage was the only significant predictor of DSS for patients with PSBA. CONCLUSIONS: Of the patients with PSBA, 76.8% had symptoms at the initial diagnosis, which were often detected at an advanced stage. Detection during the early stages of PSBA is important to ensure a good prognosis.

Small intestinal angiosarcoma on clinical presentation, diagnosis, management and prognosis: A case report and review of the literature.

BACKGROUND: Angiosarcoma is a highly malignant soft-tissue sarcoma derived from vascular endothelial cells that mainly occurs in the skin and subcutaneous tissues. Small-intestinal angiosarcomas are rare, and the prognosis is poor. CASE SUMMARY: We reported a case of primary multifocal ileal angiosarcoma and analyze previously reported cases to improve our understanding of small intestinal angiosarcoma. Small intestinal angiosarcoma is more common in elderly and male patients. Gastrointestinal bleeding, anemia, abdominal pain, weakness, and weight loss were the common symptoms. CD31, CD34, factor VIII-related antigen, ETS-related gene, friend leukemia integration 1, and von Willebrand factor are valuable immunohistochemical markers for the diagnosis of small-intestinal angiosarcoma. Small-intestinal angiosarcoma most commonly occurs in the jejunum, followed by the ileum and duodenum. Radiation and toxicant exposure are risk factors for angiosarcoma. After a definite diagnosis, the mean and median survival time was 8 mo and 3 mo, respectively. Kaplan-Meier survival analysis showed that age, infiltration depth, chemotherapy, and the number of small intestinal segments invaded by tumor lesions were prognostic factors for small intestinal angiosarcoma. Multivariate Cox regression analysis showed that chemotherapy and surgery significantly improved patient prognosis. CONCLUSION: Angiosarcoma should be considered for unexplained melena and abdominal pain, especially in older men and patients with a history of radiation exposure. Prompt treatment, including surgery and adjuvant chemotherapy, is essential to prolonging patient survival.

Small Bowel Adenocarcinoma: a Nationwide Population-Based Study.

PURPOSE: Small intestinal cancers have a non-specific clinical presentation and hence a delayed diagnosis. The prevalence of small intestinal cancers is low, and there are no cost-effective methods of screening. This study aimed to identify clinical characteristics of duodenal and jejunal adenocarcinomas that can assist in the early detection and diagnosis of disease. METHODS: Duodenal adenocarcinoma and jejunal adenocarcinoma in Explorys database (1999-2019) were compared using odds ratio (OR) with 95% confidence intervals. Data on demographic characteristics, risk factors, clinical features, and treatment were collected. RESULTS: Out of a total of 8100 patients with a diagnosis of primary adenocarcinoma of the small intestine, 5110 are primary adenocarcinoma of duodenum (63%), and 600 are primary adenocarcinoma of jejunum (7.4%). Patients with jejunal adenocarcinoma when compared with patients with duodenal adenocarcinoma are more obese (OR, 1.36) and have a significantly higher prevalence of malignant neoplasm of colon (OR, 3.07), Crohn's disease (OR, 4.42), and celiac disease (OR, 2.48). Jejunal adenocarcinoma patients presented more frequently with intestinal obstruction (OR, 1.99), whereas duodenal adenocarcinoma patients more commonly presented with iron deficiency anemia (OR, 0.16). Patients with jejunal adenocarcinoma are less likely to undergo therapy with anti-neoplastic agents when compared with duodenal adenocarcinoma (OR, 0.81). There are no differences in patients undergoing surgical intervention or a combination of surgical intervention and antineoplastic therapy. CONCLUSIONS: Jejunal adenocarcinoma is more commonly associated with colorectal cancer, celiac disease, and Crohn's disease. They also had lower odds of requiring chemotherapeutic agents.

How to Manage Small Intestine (Jejunal and Ileal) Neuroendocrine Neoplasms Presenting with Liver Metastases?

PURPOSE OF REVIEW: Small intestinal neuroendocrine neoplasms (siNENs) are slowly growing tumours with a low malignant potential. However, more than half of the patients present with distant metastases (stage IV) and nearly all with locoregional lymph node (LN) metastases at the time of surgery. The value of locoregional treatment is discussed controversially. RECENT FINDINGS: In stage I to III disease, locoregional surgery was currently shown to be curative prolonging survival. In stage IV disease, surgery may prolong survival in selected patients with the chance to cure locoregional disease besides radical/debulking liver surgery. It may improve the quality of life and may prevent severe local complications resulting in a state of chronic malnutrition and severe intestinal ischaemia or bowel obstruction. Locoregional tumour resection offers the opportunity to be curative or to focus therapeutically on liver metastasis, facilitating various other therapeutic modalities. Risks and benefits of the surgical intervention need to be balanced individually.

Massive Ascites Caused by Primary Diffuse Large B-cell Lymphoma of the Jejunum: A Case of a Rare Initial Symptom.

A 74-year-old man presented with abdominal swelling. Computed tomography revealed massive ascites and localized thickening of the small intestinal wall. Enteroscopy showed ulcerative lesions along the circumference of the jejunum. Histological examination showed dense proliferation of large lymphoid atypical cells, and immunohistochemistry showed CD20 and CD10 positivity, CD3 negativity, and Ki67 labeling index >80%. Cytology of the ascitic fluid revealed large lymphoid cells. These findings suggest that small intestine primary diffuse large B-cell lymphoma (DLBCL) caused the ascites. Massive ascites as an initial symptom of primary DLBCL of the jejunum is rare. Herein, we describe this unusual presentation.

A Plasma Protein Biomarker Strategy for Detection of Small Intestinal Neuroendocrine Tumors.

BACKGROUND: Small intestinal neuroendocrine tumors (SI-NETs) are difficult to diagnose in the early stage of disease. Current blood biomarkers such as chromogranin A (CgA) and 5-hydroxyindolacetic acid have low sensitivity (SEN) and specificity (SPE). This is a first preplanned interim analysis (Nordic non-interventional, prospective, exploratory, EXPLAIN study [NCT02630654]). Its objective is to investigate if a plasma protein multi-biomarker strategy can improve diagnostic accuracy (ACC) in SI-NETs. METHODS: At the time of diagnosis, before any disease-specific treatment was initiated, blood was collected from patients with advanced SI-NETs and 92 putative cancer-related plasma proteins from 135 patients were analyzed and compared with the results of age- and sex-matched controls (n = 143), using multiplex proximity extension assay and machine learning techniques. RESULTS: Using a random forest model including 12 top ranked plasma proteins in patients with SI-NETs, the multi-biomarker strategy showed SEN and SPE of 89 and 91%, respectively, with negative predictive value (NPV) and positive predictive value (PPV) of 90 and 91%, respectively, to identify patients with regional or metastatic disease with an area under the receiver operator characteristic curve (AUROC) of 99%. In 30 patients with normal CgA concentrations, the model provided a diagnostic SPE of 98%, SEN of 56%, and NPV 90%, PPV of 90%, and AUROC 97%, regardless of proton pump inhibitor intake. CONCLUSION: This interim analysis demonstrates that a multi-biomarker/machine learning strategy improves diagnostic ACC of patients with SI-NET at the time of diagnosis, especially in patients with normal CgA levels. The results indicate that this multi-biomarker strategy can be useful for early detection of SI-NETs at presentation and conceivably detect recurrence after radical primary resection.

A Rare Case of Well-Differentiated Neuroendocrine Tumor Arising From a Jejunal Pancreatic Heterotopia.

Pancreatic heterotopia is a well-described entity occurring at multiple abdominal sites, most commonly the stomach and small intestine. They can develop similar disease processes as the pancreas ranging from acute pancreatitis, cyst formation, or neoplasms, most commonly ductal adenocarcinoma. Neuroendocrine tumors (NETs) arising in pancreatic heterotopias are exceedingly rare with only 3 prior published cases. In this article, we describe the first reported case of a NET arising in a jejunal pancreatic heterotopia in a 59-year-old woman presenting with abdominal pain and diarrhea. The submucosal tumor was composed of a well-differentiated NET (World Health Organization grade 1) directly intermixed with a heterotopic pancreas consisting of acinar cells, islet cells, and ducts. This case illustrates that NETs can occur in association with pancreatic heterotopias at any site. Also, the importance for pathologists to recognize that pancreatic heterotopias can give rise to a variety of neoplasms and is not limited to ductal adenocarcinoma.

Unusual presentation of a giant jejunal gastrointestinal stromal tumor.

AIM: Gastrointestinal stromal tumours (GISTs) are rare tumours. Tumour rupture is an additional adverse prognostic factor and should be recorded, regardless of whether it took place before or during surgery. CASE REPORT: A case report of hemoperitoneum from spontaneous rupture of a gastrointestinal stromal tumor of the jejunum is presented. The patient underwent an urgent laparotomy. An "en bloc" resection was performed. CONCLUSION: The information in the literature is examined. Spontaneous rupture of the tumor with concomitant hemoperitoneum is an important prognostic factor in these patients. The hemoperitoneum contributes to a worse prognosis because of its ability to produce peritoneal seeding KEY WORDS: Gist.