Authors: B Niederle, U-F Pape, F Costa...

Topics: Consensus; Europe; Humans; Ileal Neoplasms; Jejunal Neoplasms; Neuroendocrine Tumors

PubMed: 26758972
DOI: 10.1159/000443170

Authors: Akitada Yogo, Alan Paciorek, Yosuke Kasai...

BACKGROUND

Ileal neuroendocrine tumors (i-NETs) are characterized by their multifocality and bulky mesenteric mass. Having shown that minimally invasive surgery (MIS) utilizing a hand-access port device has favorable short-term outcomes and achieves the goals of surgery for i-NETs, we sought to analyze long-term survival outcomes of MIS.

METHODS

One hundred and sixty-eight patients who underwent resection of primary i-NETs at a single institution between January 2007 and February 2023 were retrospectively studied. Patients were categorized into the MIS or open surgery cohorts on an intention-to-treat basis. Open surgery was selected mainly based on the need for hepatectomy or bulky mesenteric mass resection. Overall survival was analyzed using log-rank tests with propensity score matching (PSM) and Cox proportional hazards regression. PSM was performed to reduce standardized mean differences of the variables to <0.2.

RESULTS

Overall, 129 (77%) patients underwent MIS and 39 (23%) underwent open surgery. Twenty-seven MIS patients were converted to an open procedure. The median follow-up time was 49 months (interquartile range 23-87 months). In the PSM cohorts, overall survival did not differ significantly between the MIS and open surgery cohorts {median 99 months (95% confidence interval [CI] 91-not applicable [NA]) vs. 103 months (95% CI 86-NA), p = 0.77; hazard ratio 0.87 (95% CI 0.33-2.2), p = 0.77}.

CONCLUSIONS

MIS is an alternative to open surgery for i-NETs, achieving similar short- and long-term oncological outcomes. Bulky mesenteric mass and a plan for concurrent liver resection are potential criteria for open surgery.

Topics: Humans; Male; Female; Neuroendocrine Tumors; Middle Aged; Retrospective Studies; Survival Rate; Follow-Up Studies; Ileal Neoplasms; Minimally Invasive Surgical Procedures; Aged; Prognosis; Hepatectomy; Adult

PubMed: 38797790
DOI: 10.1245/s10434-024-15468-6

Authors: Hans Jonas Meyer, Sebastian Ullrich, Gordian Hamerla...

Topics: Breast Neoplasms; Diagnosis, Differential; Eye Neoplasms; Female; Humans; Ileal Neoplasms; Image Enhancement; Laryngeal Neoplasms; Liver Neoplasms; Magnetic Resonance Imaging; Male; Mammography; Mediastinal Neoplasms; Muscle Neoplasms; Orbital Neoplasms; Plasmacytoma; Retroperitoneal Neoplasms; Sensitivity and Specificity; Stomach Neoplasms; Tomography, X-Ray Computed; Vitreous Body

PubMed: 30296807
DOI: 10.1055/a-0604-2831

Review

Authors: Bruno Niederle, Andreas Selberherr, Martin B Niederle...

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.

Topics: Humans; Ileal Neoplasms; Jejunal Neoplasms; Liver Neoplasms; Lymphatic Metastasis; Neoplasm Staging; Neuroendocrine Tumors

PubMed: 34018081
DOI: 10.1007/s11912-021-01074-2

Authors: Sylvia L Asa, Amr Mohamed

Multiple endocrine neoplasms are a feature of multiple endocrine neoplasia (MEN) syndromes types 1, 2, 4, and 5. However, the ileum is not usually involved in these disorders. We report a series of patients with neuroendocrine tumors (NETs) involving both the pancreas and the ileum. We searched the laboratory information system and personal consultation records of the authors from 2019 to 2023 for patients who had neuroendocrine tumors (NETs) involving both the pancreas and ileum. In a series of 846 patients, we identified 4 patients with pancreatic and ileal NETs, 2 female and 2 male, ages 52 to 75. Two female patients had primary EC cell tumors of the ileum with metastasis to the pancreas that showed expression of CDX2 and serotonin similar to the ileal primary tumors. Two males had primary lesions in the 2 sites with different immunoprofiles; the ileal tumors expressed CDX2 and serotonin and were negative for ARX, whereas the pancreatic tumors expressed ARX, glucagon, and pancreatic polypeptide and were negative for CDX2 and serotonin. In both male patients, the nontumorous pancreas showed preneoplastic changes in the endocrine elements, suggesting germline predisposition to endocrine neoplasia. Testing for known genetic alterations underlying MEN syndromes has not identified a genetic alteration that can be implicated in the development of NETs in both pancreas and ileum. Our series indicates the rare occurrence of NETs in both the pancreas and ileum and emphasizes the importance of using the correct biomarkers to distinguish metastasis from primary neoplasms at the different sites. The rare occurrence of primary ileal and pancreatic NETs may represent a novel MEN syndrome with as yet unknown germline predisposition.

Topics: Humans; Male; Pancreatic Neoplasms; Middle Aged; Female; Aged; Ileal Neoplasms; Neuroendocrine Tumors; Biomarkers, Tumor; Multiple Endocrine Neoplasia

PubMed: 39034742
DOI: 10.1097/PAS.0000000000002290

Review

Authors: Bogdan Stancu, Răzvan Alexandru Ciocan, Marius George Teodorescu...

Adenocarcinoma (ADK) of the ileum is an infrequent cancer that poses significant diagnostic and treatment difficulties. This article analyses the existing case literature to investigate the correlation between pyloric gland metaplasia (PGM) and the onset of ileal ADK. PGM, defined by the atypical transformation of intestinal epithelial cells into gastric-type mucosa, has been recognized as a possible precursor lesion in the spectrum of several gastrointestinal malignancies. We intend to clarify the clinical characteristics, histological results, and outcomes linked to ileal ADK originating from PGM through an exhaustive examination of recorded cases. Our findings indicate that early identification of metaplastic alterations may be essential for enhancing prognosis and informing therapeutic choices. This study emphasizes the need for additional research to elucidate the mechanisms driving this transition and to improve diagnostic precision in clinical practice.

Topics: Aged; Female; Humans; Male; Middle Aged; Adenocarcinoma; Gastric Mucosa; Ileal Neoplasms; Ileum; Metaplasia; Pylorus

PubMed: 39957020
DOI: 10.47162/RJME.65.4.05

Authors: S D Johnston, P W Johnston, D O'Rourke...

A 78 year old man presented with diarrhoea, anorexia, and progressive lower limb oedema. He was in atrial fibrillation and had a right pleural effusion and ascites. Ultrasound of the abdomen and 24 hour urinary hydroxyindoleacetic acid output indicated metastatic carcinoid syndrome. Cardiac catheterisation revealed pericardial constriction, and pericardial exploration showed a greatly thickened pericardium with no evidence of tumour invasion. The patient died within 24 hours of surgery. Necropsy findings were consistent with a diagnosis of constrictive pericarditis secondary to metastatic carcinoid syndrome.

Topics: Aged; Humans; Ileal Neoplasms; Liver Neoplasms; Male; Malignant Carcinoid Syndrome; Pericarditis, Constrictive; Pericardium

PubMed: 10525526
DOI: 10.1136/hrt.82.5.641

Review

Authors: M Anlauf, B Sipos, I Boeck...

Neuroendocrine neoplasms (NEN) of the distal jejunum and ileum derive from serotonin-producing enterochromaffin (EC) cells. Due to their low proliferation rate and their infiltrative growth, they are often discovered at an advanced disease stage when metastasis has already occurred. The biology of these tumours is different from other NEN of the digestive tract. In order to standardise and improve diagnosis and therapy, the guidelines for the diagnosis and clinical management of jejuno-ileal NEN as well as for the management of patients with liver and other distant metastases from NEN were revised by the European Neuroendocrine Tumour Society (ENETS) in 2012. This review focuses on aspects relevant for surgical pathology.

Topics: Cell Proliferation; Diagnosis, Differential; Disease Progression; Enterochromaffin Cells; Humans; Ileal Neoplasms; Ileum; Jejunal Neoplasms; Jejunum; Neuroendocrine Tumors; Practice Guidelines as Topic; Receptors, Somatostatin

PubMed: 24671468
DOI: 10.1007/s00292-013-1888-5

Review

Authors: Masahiro Tajika, Yasumasa Niwa, Vikram Bhatia...

Restorative proctocolectomy is the most common surgical option for patients with familial adenomatous polyposis (FAP). However, adenomas may develop in the ileal pouch mucosa over time, and even carcinoma in the pouch has been reported. We therefore reviewed the prevalence, nature, and treatment of adenomas and carcinoma that develop after proctocolectomy in the ileal pouch mucosa in patients with FAP. In 25 reports that were reviewed, the incidence of adenomas in the ileal pouch varied from 6.7% to 73.9%. Several potential factors that favor the development of pouch polyposis have been investigated, but many remain controversial. Nevertheless, it seems certain that the age of the pouch is important. The risk appears to be 7% to 16% after 5 years, 35% to 42% after 10 years, and 75% after 15 years. On the other hand, only 21 cases of ileal pouch carcinoma have been recorded in the literature to date. The diagnosis of pouch carcinoma was made between 3 to 20 years (median, 10 years) after pouch construction. Although the risk of malignant transformation in ileal pouches is probably low, it is not negligible, and the long-term risk cannot presently be well quantified. Regular endoscopic surveillance, especially using chromoendoscopy, is recommended.

Topics: Adenocarcinoma; Adenoma; Adenomatous Polyposis Coli; Anus Neoplasms; Cell Transformation, Neoplastic; Colonic Pouches; Humans; Ileal Neoplasms; Incidence; Intestinal Mucosa; Prevalence; Risk Factors; Time Factors; Treatment Outcome

PubMed: 24187452
DOI: 10.3748/wjg.v19.i40.6774

Review

Authors: Antongiulio Faggiano, Pasqualino Malandrino, Roberta Modica...

BACKGROUND

Everolimus, an oral mTOR (mammalian target of rapamycin) inhibitor, is currently approved for the treatment of progressive pancreatic neuroendocrine tumors (NETs). Although promising, only scattered data, often from nondedicated studies, are available for extrapancreatic NETs.

PATIENTS AND METHODS

A systematic review of the published data was performed concerning the use of everolimus in extrapancreatic NET, with the aim of summarizing the current knowledge on its efficacy and tolerability. Moreover, the usefulness of everolimus was evaluated according to the different sites of the primary.

RESULTS

The present study included 22 different publications, including 874 patients and 456 extrapancreatic NETs treated with everolimus. Nine different primary sites of extrapancreatic NETs were found. The median progression-free survival ranged from 12.0 to 29.9 months. The median time to progression was not reached in a phase II prospective study, and the interval to progression ranged from 12 to 36 months in 5 clinical cases. Objective responses were observed in 7 prospective studies, 2 retrospective studies, and 2 case reports. Stabilization of the disease was obtained in a high rate of patients, ranging from 67.4% to 100%. The toxicity of everolimus in extrapancreatic NETs is consistent with the known safety profile of the drug. Most adverse events were either grade 1 or 2 and easy manageable with a dose reduction or temporary interruption and only rarely requiring discontinuation.

CONCLUSION

Treatment with everolimus in patients with extrapancreatic NETs appears to be a promising strategy that is safe and well tolerated. The use of this emerging opportunity needs to be validated with clinical trials specifically designed on this topic.

IMPLICATIONS FOR PRACTICE

The present study reviewed all the available published data concerning the use of everolimus in 456 extrapancreatic neuroendocrine tumors (NETs) and summarized the current knowledge on the efficacy and safety of this drug, not yet approved except for pancreatic NETs. The progression-free survival rates and some objective responses seem promising and support the extension of the use of this drug. The site-by-site analysis seems to suggest that some subtypes of NETs, such as colorectal, could be more sensitive to everolimus than other primary NETs. No severe adverse events were usually reported and discontinuation was rarely required; thus, everolimus should be considered a valid therapeutic option for extrapancreatic NETs.

Topics: Adrenal Gland Neoplasms; Antineoplastic Agents; Carcinoma, Neuroendocrine; Colorectal Neoplasms; Disease-Free Survival; Everolimus; Humans; Ileal Neoplasms; Lung Neoplasms; Neuroendocrine Tumors; Pheochromocytoma; Stomach Neoplasms; Thyroid Neoplasms

PubMed: 27053503
DOI: 10.1634/theoncologist.2015-0420