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Neuroendocrinology 2016
Topics: Consensus; Europe; Humans; Ileal Neoplasms; Jejunal Neoplasms; Neuroendocrine Tumors
PubMed: 26758972
DOI: 10.1159/000443170 -
Annals of Surgery Feb 2011Surgical management of incidental Meckel's diverticulum(MD) is a highly debated controversial issue that has never been discussed from the oncological standpoint.
BACKGROUND
Surgical management of incidental Meckel's diverticulum(MD) is a highly debated controversial issue that has never been discussed from the oncological standpoint.
OBJECTIVE
To describe the epidemiology and risk of Meckel's diverticulum cancer (MDC) and compare it with other ileal malignancies.
METHODS
Data were obtained from 163 cases of MDC and 6214 cases of non-Meckelian ileal cancer, between 1973 and 2006, from the Surveillance, Epidemiology, and End Results database.
RESULTS
Mean annual incidence was 1.44 (± 1.12) per 10 million population,with a 5-fold increase in the last few decades. Incidence increases with age,with a mean age at diagnosis of 60.6 (±15.1) years. Adjusted risk of cancer in the MD was at least 70 times higher than any other ileal site. Disease was localized in 67% at presentation and malignant carcinoids constituted the major histologic type (77%). One-third of patients have had lifetime occurrence of other malignancies and in 13% of these patients, MDC was the first malignancy. Median tumor size was 7 mm. Median overall survival was 173 months (95% confidence interval [CI], 124-221 months), with 1- and 5-year relative survival rates of 85.8% (95% CI, 76.9%-91.4%) and 75.8% (95%CI, 64.9%-83.8%), respectively. Cox proportional hazards model revealed that age, histologic type, and metastatic disease were independent factors affecting survival.
CONCLUSIONS
MD is a "hot-spot" or high-risk area for cancer in the ileum.With risk that increases with age and high possibility of curative resection with negligible operative mortality, incidental MD is best treated with resection.
Topics: Adenocarcinoma; Age Distribution; Aged; Carcinoid Tumor; Female; Humans; Ileal Neoplasms; Incidence; Male; Meckel Diverticulum; Middle Aged; Prevalence; SEER Program; Sex Distribution; United States
PubMed: 21135700
DOI: 10.1097/SLA.0b013e3181ef488d -
RoFo : Fortschritte Auf Dem Gebiete Der... Nov 2018
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 -
Current Oncology Reports May 2021Small intestinal neuroendocrine neoplasms (siNENs) are slowly growing tumours with a low malignant potential. However, more than half of the patients present with... (Review)
Review
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 -
BMC Urology Jan 2022Uretero-arterial fistulas (UAFs) are uncommon and pose a diagnostic dilemma, making them life threatening if not recognized and treated expediently. UAFs to small... (Review)
Review
BACKGROUND
Uretero-arterial fistulas (UAFs) are uncommon and pose a diagnostic dilemma, making them life threatening if not recognized and treated expediently. UAFs to small arteries such as a branch of the inferior mesenteric artery (IMA) are very uncommon and present a further diagnostic and treatment challenge. There should be a high index of suspicion for UAFs when intervening on patients with history of treated pelvic cancers and long-standing ureteric stents experiencing hematuria not attributable to another cause.
CASE PRESENTATION
We present a case of a fistula formed between a distal branch of the IMA-superior rectal artery-and an ileal-conduit in a patient with a long-standing reverse nephroureterostomy (Hobbs) catheter presenting with abdominal pain and hematuria through the conduit. During a tube exchange, contrast injection demonstrated a fistula with the superior rectal artery, multiple ileal intraluminal blood clots, and active extravasation. The patient became tachycardic and hypotensive, actively bleeding through the ileal-conduit, prompting a massive transfusion protocol. Successful endovascular coiling of the superior rectal artery was performed with resolution of active extravasation and stabilization of the patient. The patient recovered and was discharged in stable condition 10 days later.
CONCLUSIONS
Although UAFs are uncommon, our case demonstrated key predisposing risk factors to fistula development; pelvic cancer surgery, pelvic radiation, and a prolonged ureteric stent through the ileal-conduit. Typically, UAFs arise from communication with the iliac arterial system, however in this instance we have demonstrated that fistulization to other arterial vessels is also possible. Endovascular management has become the preferred method of therapy, typically involving the placement of covered stents when involving the iliac arterial system. In this instance stent grafting was not possible due to the small caliber vessel and therefore had to be embolized.
Topics: Aged; Cystectomy; Embolization, Therapeutic; Humans; Male; Mesenteric Artery, Inferior; Neoplasm Recurrence, Local; Postoperative Complications; Prostatectomy; Radiotherapy; Risk Factors; Stents; Urinary Bladder Neoplasms; Urinary Diversion; Urinary Fistula; Vascular Fistula
PubMed: 35101008
DOI: 10.1186/s12894-022-00961-5 -
Neuroendocrinology 2021Small-intestinal neuroendocrine tumors (SI-NET) are situated preferentially within the ileum. The aim was to describe a potential difference in location between unifocal...
INTRODUCTION
Small-intestinal neuroendocrine tumors (SI-NET) are situated preferentially within the ileum. The aim was to describe a potential difference in location between unifocal and multiple ileal-NET.
PATIENTS AND METHODS
Between December 2010 and December 2019, all consecutive patients who underwent resection in our European Neuroendocrine Tumor Society Center of Excellence, of at least 1 non-duodenal SI-NET, were retrospectively included. The main objective was to prove that multiple ileal-NET were mostly located on the left side of the superior mesenteric artery (SMA) axis (defined as 40 cm from the ileocecal valve), and unifocal ones on the right side.
RESULTS
Ninety-four patients were included, 6 with unifocal jejunal-NET located 35 cm (range, 10-60) from the duodenojejunal angle (DJA), 44 (47%) with unifocal ileal-NET and 44 (47%) with multiple ileal-NET. The median number of tumors in multiple ileal-NET was 7 (range, 2-95), within a median small bowel segment of 105 cm (10-240). The median length between the proximal tumor and the DJA was 428 cm (300-635) and 540 cm (350-725) for the distal one; 40 (91%) of them were located on the left side of the SMA axis. In contrast, unifocal ileal-NET were located at a median distance of 577 cm (305-820) from the DJA (p < 0.001, compared to multiple ileal-NET); 30 (68%) of them were on the right side of the SMA axis (p < 0.001).
CONCLUSION
Multiple ileal-NET are mostly located on the left side of the SMA axis. Further studies are warranted to explore the embryological origin of unifocal versus multiple ileal-NET.
Topics: Adult; Aged; Aged, 80 and over; Female; Humans; Ileal Neoplasms; Male; Middle Aged; Neoplasms, Germ Cell and Embryonal; Neuroendocrine Tumors; Retrospective Studies
PubMed: 32998140
DOI: 10.1159/000511849 -
World Journal of Gastroenterology Oct 2013Restorative proctocolectomy is the most common surgical option for patients with familial adenomatous polyposis (FAP). However, adenomas may develop in the ileal pouch... (Review)
Review
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 -
The Oncologist Jul 2016Everolimus, an oral mTOR (mammalian target of rapamycin) inhibitor, is currently approved for the treatment of progressive pancreatic neuroendocrine tumors (NETs).... (Review)
Review
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 -
Der Pathologe May 2014Neuroendocrine neoplasms (NEN) of the distal jejunum and ileum derive from serotonin-producing enterochromaffin (EC) cells. Due to their low proliferation rate and their... (Review)
Review
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 -
PloS One 2021The current understanding of clinicopathological features and genomic variants of small-bowel cancer is limited, in part due to the rarity of the disease. However,...
The current understanding of clinicopathological features and genomic variants of small-bowel cancer is limited, in part due to the rarity of the disease. However, understanding of these factors is necessary for the development of novel therapeutic agents for small-bowel cancer. Thus, we aimed to identify the clinicopathological features and genomic variants associated with its prognosis and recurrence. We retrospectively examined 24 consecutive patients with primary small-bowel cancer surgically treated between May 2005 and August 2018 and collected 29 tumor specimens. The 29 lesions were subjected to mismatch repair status evaluation, using immunohistochemistry (IHC), and targeted genomic sequencing, after which they were analyzed using a panel of 90 cancer-related genes. IHC revealed that 45% (13/29) of the lesions exhibited deficient mismatch repair. The most common genomic variants in small-bowel cancers were in TP53 (48%, 13/27), followed by KRAS (44%, 12/27), ARID1A (33%, 9/27), PIK3CA (26%, 7/27), APC (26%, 7/27), and SMAD4, NOTCH3, CREBBP, PTCH1, and EP300 (22%, 6/27 each). Overall survival and disease-specific survival of patients with tumor mutational burden (TMB) ≥10 mutations/Mb (n = 17) were significantly better than those of patients with TMB <10 mutations/Mb (n = 6). Additionally, patients with a mutant SMAD4 had poorer recurrence-free survival than those with wild-type SMAD4. Our results suggested that TMB and SMAD4 mutations were associated with the prognosis of small-bowel cancer patients. Thus, cancer genomic analysis could be useful in the search for biomarkers of prognosis prediction in small-bowel cancers.
Topics: Adult; Aged; Biomarkers, Tumor; CREB-Binding Protein; DNA-Binding Proteins; Female; Humans; Ileal Neoplasms; Jejunal Neoplasms; Male; Middle Aged; Mutation; Receptors, Notch; Smad Proteins; Survival Analysis; Transcription Factors; Tumor Suppressor Protein p53; ras Proteins
PubMed: 34014970
DOI: 10.1371/journal.pone.0241454