Ileal Tumor Research Articles

Tricuspid and pulmonary valve involvement in carcinoid heart disease

A 55-year-old woman with a history of proven carcinoid syndrome due to an enterochromaffin ileal tumour was evaluated with transthoracic echocardiography for dyspnoea on exertion. The patient had flushing and severe diarrhoea. Urinary excretion of 5-hydroxyindoleacetic acid was 250 mmol/L per 24 hours. Treatment with a somatostatin analogue had been initiated 5 months earlier. Physical examination revealed a 3/6 systolic ejection murmur at the left upper sternal border. No signs of right-sided heart failure were found. The electrocardiographic data were within normal limits. Transthoracic two-dimensional echocardiography revealed restricted motion of the septal and anterior tricuspid valve leaflets both during systole and diastole, leading to an eccentric jet and mild to moderate tricuspid regurgitation (Fig. 1 and Movie 1). Continuous wave (CW) Doppler documented an associated tricuspid stenosis with a mean diastolic gradient of 5 mmHg (Fig. 2). The pulmonary valve was also involved, defined by a limited excursion of the pulmonary valve leaflets without a complete coaptation. CW Doppler of the pulmonary valve demonstrated the presence of both pulmonary stenosis defined by a mean gradient of 40mmHg (Fig. 3) and pulmonary regurgitation (Movie 2). No patent foramen ovale was found at contrast echocardiography. Pulmonary and tricuspid valves were replaced with bioprosthetic valves. A surgical view of the tricuspid and pulmonary valve revealed immobile, thickened and retracted pulmonary and tricuspid valves leaflets (Figs. 4 and 5). Characteristic echocardiographic features of advanced carcinoid heart disease include thickening, retraction of immobile tricuspid valve leaflets with associated tricuspid regurgitation in 90% of cases and pulmonary involvement, mainly immobility of the pulmonary valve cusps, in at least 50% of cases [1]. Contrast transthoracic echocardiography should be performed systematically, searching for a right-to-left atrial shunt through a patent foramen ovate [2].

Morphologisches Erscheinungsbild neuroendokriner Tumoren des Dünndarms im MR-Enteroklysma

To determine the value of MR enteroclysis (MRE) in the localization and characterization of primary carcinoid tumors of the small bowel and to describe typical imaging features. Twenty patients with suspicion of primary small bowel carcinoid tumors (pCT) were recruited to undergo MRE following nasojejunal intubation and small bowel filling with 2.5 l of 0.5% methylcellulose solution under MR fluoroscopic guidance. MRE was performed on a 1.5 T MR scanner including T2w SSFSE, SSFP and contrast enhanced T1w GRE sequences with fat saturation. Fifteen patients, who subsequently had surgery for resection of their pCT, were retrospectively included in the study. All MRE were analyzed as for the presence, location, number, size, multiplicity and morphologic appearance of the pCT by two board certified radiologists in consensus. The conspicuity of the tumors was rated for each sequence type separately, according to a 4-point rating scale. Signal intensity measurements were performed in tumor and muscle. The presence of desmoplastic reaction, vascular involvement and lymph node metastases was also analyzed. pCT were correctly identified and localized in 14/15 patients. Due to their hyperenhancement tumors was best detected on contrast-enhanced T1w fat saturated GRE sequences. SSFSE was clearly inferior with the tumors being either hyperintense or isointense to muscle. pCT appeared as nodular intraluminal masses in 40% of the cases, as focal wall thickening in 33.3% and in 20% with both. Mean size was 25 (7-46 mm) with a tendency to smaller size for ileal tumors. MRE failed to depict superficial micronodular peritoneal spread in one patient. Desmoplastic reaction was observed in 73.3% of the cases with mesenteric masses exhibiting lower signal than the pCT due to fibrotic changes. MRE is a valuable method for the detection and localization of primary carcinoid tumors, provided that appropriate bowel distension is achieved. Various characteristic morphologic features could be identified which may contribute to characterize pCT and their loco-regional metastases.

Synchronous ileal carcinoid and primary colonic neoplasms: a case report

Primary colonic tumours with synchronous ileal carcinoid tumours are rare in occurrence and are mainly found incidentally on autopsies or pathological examination of resected surgical specimens. This article describes a case of adenomatous colonic polyps, adenocarcinoma of sigmoid colon and concurrent malignant carcinoid tumour of ileocaecal junction, detected on colonoscopic examination. The radiological staging investigations revealed no distant spread of disease. The patient was effectively treated with subtotal colectomy, resection of terminal ileum, excision of locoregional lymph nodes and the bowel continuity was restored with stapled ileo-rectal anastomosis. This article is as an example of concomitant presence of two types of malignant tumours, effectively managed surgically.

Carcinogenicity in rats of ptaquiloside isolated from bracken.

The nature of the carcinogen present in bracken fern has not yet been elucidated. Very recently, we succeeded in isolating ptaquiloside, a novel norsesquiterpene glucoside of the illudane type, from bracken. Ptaquiloside was shown to be a carcinogenic principle of bracken fern. It induces mammary cancer and multiple ileal tumors in high incidences when given orally to female Sprague-Dawley rats.

Ileal schwannoma developing into ileocolic intussusception

Intussusception is rare in adults. We describe a 47-year-old man with ileal schwannoma that led to ileocolic intussusception. Abdominal ultrasonography, abdominal CT scan and barium enema confirmed an ileal tumor. Colonoscopy revealed a peduncular submucosal tumor (SMT) 75 mm long with an ulcerated apex at the ascending colon. The provisional diagnosis was a gastrointestinal stromal tumor of the terminal ileum. Ileocecal resection was carried out and the tumor was histologically diagnosed as schwannoma. Abdominal pain resolved postoperatively. This case reminds us that ileal schwannoma should be included in the differential diagnosis of intussusception caused by an SMT in the intestine.

Intraoperative Enteroscopy in Minimally Invasive Surgery

The purpose of this study was to assess the efficacy and safety of intraoperative enteroscopy (IOE) in patients undergoing minimally invasive surgery. Twelve patients underwent minimally invasive surgery and IOE at Miyazaki University Hospital. Patients included 11 men and 1 woman. After extraction of the intestine via minilaparotomy, enterotomy was performed, and a sterilized enteroscope was inserted. Length of the skin incision was 5.7+/-0.2 cm (mean+/-standard error). Length of the small intestine observed enteroscopically was 334+/-19 cm. Distance from the ligament of Treitz to the orally observed jejunum was 11.8+/-3.6 cm. In 5 of 9 patients with Crohn disease, additional lesions were found by IOE that were not found by preoperative examination. One additional tumor was found in 1 patient with ileal tumor. Postoperative complications occurred in 2 patients. IOE is efficacious in patients undergoing minimally invasive surgery.

Massive Melena Caused by a Carcinoid of the Small Intestine: Report of a Case

We report a case of massive melena caused by a carcinoid of the small intestine. A 28-year-old woman was admitted to our department after presenting with massive melena. The source of the bleeding could not be localized by upper or lower gastrointestinal endoscopy, computed tomography (CT), or labeled red blood cell scintigraphy. Enteroscopy allowed visualization only up to the jejunum, and we could not localize the lesion. The melena subsided with conservative treatment, but the patient was readmitted 4 months later when she suffered another episode of massive melena. A contrast CT scan performed immediately showed extravascular leakage and the retention of contrast medium in the ileum. Thus, she underwent an emergency surgery, during which endoscopy confirmed a small ileal tumor accompanied by pulsating bleeding from the exposed blood vessels at its center. The small intestine was partially resected, including the swollen lymph nodes, the size of small beans. Pathological examination confirmed a carcinoid tumor 1 cm in diameter, with an arterial rupture at its center and lymph node metastasis.

Allelic alterations in well‐differentiated neuroendocrine tumors (carcinoid tumors) identified by genome‐wide single nucleotide polymorphism analysis and comparison with pancreatic endocrine tumors

Well-differentiated neuroendocrine tumors (WDNT, carcinoid tumors) are uncommon indolent neoplasms. The genetic alterations of these tumors are not well characterized. We used genome-wide high-density single nucleotide polymorphism (SNP) array analysis to detect copy number alterations in 29 WDNTs, including seven lung, seven nonileal gastrointestinal, and 15 ileal tumors, and compared with allelic imbalances in 15 pancreatic endocrine tumors (PETs). Most frequent allelic imbalances in WDNTs were losses of chromosome 18 in 10 tumors (34%), chromosome 21 or 21q in six (21%), chromosome 13 or 13q in five (17%) and chromosome 16 or 16q in four (14%) tumors, and amplification of chromosome 20 or 20p in seven (24%) tumors. We also found one tumor with loss of heterozygosity of chromosomes 10 and 15 without copy number loss. These allelic imbalances were associated with primary site of tumor: loss of chromosome 18 was present exclusively in ileal WDNTs (P = 0.001), and loss of chromosome 21 or 21q was more frequent in nonileal gastrointestinal WDNTs (P = 0.02). The tumors with loss of chromosome 21 were larger compared to tumors without loss (P = 0.03). Chromosomal aberrations were less common in WDNTs from lung and gastrointestinal tract compared to PETs (P = 0.001). Our study shows that genome-wide allelotyping using SNP array is a powerful new tool for the analysis of allelic imbalances in WDNTs, and some of these alterations are tumor site-dependent and are different than in PETs.

Gastrointestinal Stromal Tumor and Papillary Renal Cell Carcinoma: A Case Report

There are only 4 reported cases of GIST found with concomitant PRCC. A 50 y o male with HTN presented after a presyncopal episode, with dizziness, and black stool for 1 month. PE remarkable only for melena. Iron deficiency anemia was seen on labs, large hiatal hernia on EGD and a normal colonoscopy. A 9 × 5 cm right pelvic mass was seen on CT scan with a meniscal sign of the oral contrast suggesting ileal neoplasm (Fig. 1), and a 5 × 4 cm right kidney mass. Laparoscopic excision of a 6 × 8 cm ileal tumor showed malignant GIST, with spindle cells, vesicular nuclei and a 5/50 hpf mitotic rate. Histochemical staining was postive for CD117. CT guided biopsy of the renal mass showed PRCC. The patient underwent a total right nephrectomy without post-op chemotherapy. GISTs are differentiated from other smooth muscle neoplasms by the identification of the CD117 antigen on immunohistochemical staining. CD117 is part of the c-kit receptor, a membrane tyrosine kinase and a product of the c-kit or KIT proto-oncogene. Other non-GIST spindle cell neoplasms of the GI tract do not carry this mutation, which is clinically relevant because the c-kit mutation is sensitive to imatinib, a selective tyrosine kinase inhibitor. Surgical resection is the treatment of choice, but only half are recurrence free for five years. Imatimib is successful as adjuvant therapy in advanced disease and an option for unresectable disease. Both PRCC and GIST have been connected to mutations in the proto-oncogenes c-MET and c-KIT however this association is only seen in recurrent familial tumors, not sporadic as seen in this patient. A case report describes a patient with metastatic GIST and PRCC, treated with imatinib. Six months later, all hepatic lesions had regressed. However, the renal masses increased in size, raising questions regarding the effect of imatinib for treatment of GIST on PRCC. In this situation, perhaps sunitinib should be considered as first-line treatment as it has been FDA-approved for treatment in imatinib refractory patients with GIST and in patients with advanced renal cell carcinoma.[figure1]Figure

Metastatic carcinoid tumor to the ovary: A clinicopathologic analysis of seventeen cases

Objectives To evaluate the clinical behavior and histologic characteristics of metastatic carcinoid tumors to the ovary. Methods Retrospective chart review and histologic specimen analysis of all patients with pathologically confirmed metastatic carcinoid tumor to the ovaries seen at the H. Lee Moffitt Cancer Center. Results Seventeen patients with metastatic carcinoid tumors to the ovary were identified. The distal ileum was the site of origin among all patients with identifiable primary tumor. Fifteen patients (88%) had bilateral ovarian metastases. Other metastatic sites (in order of frequency) included liver, peritoneum breast and bone. Fifteen patients (88%) had elevated urinary 5-hydroxyindoleacetic acid (5-HIAA), indicating active serotonin secretion, and sixteen patients (94%) had radiotracer uptake on indium-111-pentetreotide scans. Five-year survival rate was 94%. Conclusions Metastatic carcinoid tumors to the ovary typically originate from distal ileal tumors and are nearly always bilateral. Synchronous metastases involve liver and peritoneal surfaces. Favorable survival rates may be associated with octretide treatment and cytoreductive surgery.

A Three-Decade Analysis of 3,911 Small Intestinal Neuroendocrine Tumors: The Rapid Pace of No Progress

Small intestinal neuroendocrine tumors (SI-NETs) are the most common gastrointestinal neuroendocrine tumor, but their natural history and outcome remain poorly defined, which hinders both the prediction of disease progression and appropriate therapeutic options. We examined patterns, incidence, prognosis, and outcomes of these tumors over a 30-yr period. Data were extracted from the NCI's SEER registry (1973-2002). Incidence rates, distribution, and 5-yr survival rates were analyzed and adjusted (U.S. decennial census data). Of the 18,641 NETs, 3,911 (21.0%) were SI-NETs, of which 1,953 (49.6%) were ileal. Since 1973, both SI-NET and its ileal variant have increased annually by 3.8% and 2.1%, respectively. Ileal tumors, as a percentage of SI tumors, have increased from 52% to 63.6%. The age-adjusted incidence of ileal, small intestinal, and digestive system NETs has increased 225%, 460%, and 720% over 30 yr. Ileal tumors have specifically increased in prevalence in white (274%) and black (500%) men and women (213% and 286%, respectively); an overall increase of fourfold in blacks and 2.4-fold in whites. Although 83.3% of SI-NETs were staged, 83.7% were histologically ungraded. Five-year survival rates for SI-NETs were 62.6 +/- 1% (all stages), 73.8% (localized), 72% (regional), and 43.2% (distant). These have not significantly altered since 1973 (P= 0.11). SI-NETs have increased, particularly in men and in the black population, which may be due to in vivo changes, increased clinical and pathological awareness, or increased detection of tumors. SI-NETs are malignant, diagnosed late, and survival rates have remained unchanged over 30 yr.

A case of ovarian mature cystic teratoma presenting as a pedunculated ileal tumor

We report on a 34-year-old Japanese woman who presented with a pedunculated ileal tumor and who was finally diagnosed as having a right ovarian mature cystic teratoma penetrating and protruding into the ileum. She had undergone laparoscopic left ovarian cystectomy, whose specimen was diagnosed as dermoid cyst when she was 27 years old. The colon fiberscope revealed an ileal polyp, diagnosed as mature teratoma. Because of adhesion to the necrotic nodule between the tumor and the right ovary, ileocecal resection and right ovarian cystectomy were performed. The ileal tumor contained tissues of skin, neuroglia, ganglion, choroid, retina, smooth muscle, as well as fibrous and adipose tissues, cartilage, bone, mucous epithelium, and bronchial structures with bronchial glands. The necrotic nodule showed abscess, granulation tissue, foreign body reaction, hairs, normal ileal epithelium, and the ovary with ovums.

Peritoneal carcinomatosis from a small bowel carcinoid tumour

BackgroundPeritoneal carcinomatosis from a gastrointestinal carcinoid tumour is rare and the long-term management and prognosis have not been clearly defined. The natural history is different from gastrointestinal adenocarcinoma, although its capacity to invade regional lymph nodes and generate distal metastasis can make the management more complex. Whilst the development of carcinomatosis is uncommonly reported, it may be higher than expected.Case presentationA 63 years-old woman underwent emergency surgery in 1993 for right iliac fossa pain and a mass that was found to be an ileal carcinoid tumour. Over the next ten years, further surgery was required for disseminated disease with peritoneal carcinomatosis and liver metastasis. Systemic chemotherapy had little effect, although Somatostatin was used effectively to relieve symptoms caused by the disseminated disease (flushing and diarrhoea).ConclusionPeritoneal carcinomatosis from carcinoid tumours is not well documented in the literature. Aggressive surgery must be performed in order to control the disease since chemotherapy has not been reported to be effective. With repeated surgery long-term survival can be achieved in these patients.

Chest Wall Metastasis of Ileal Carcinoid Tumor Detected With In-111 Octreotide Scan

Abstract: Carcinoid tumors are potentially malignant, generally being related to regional lymph node and liver metastases. Rarely, metastases to other sites such as the breast have been reported. Radiolabeled peptide analogs such as In-111 octreotide play a useful role in the localization and potentially in the management of carcinoid tumors and their distant metastases. The authors report a case of ileal carcinoid tumor with unusual presentation of a chest wall metastasis. Chest radiograph was normal. Thoracic CT scanning showed a soft tissue mass in the chest wall. In-111 octreotide scintigraphy revealed increased uptake in the anterior chest wall and liver. A biopsy of the lesion showed carcinoid tumor.

Synchronous Adenocarcinomas of the Ileum and Transverse Colon Detected by Capsule Endoscopy: Report of a Case

A 77-year-old woman was admitted to our hospital with anemia and anorexia. Neither gastrointestinal endoscopy nor computed tomography showed by abnormal findings; however, a capsule endoscopy, performed to detect obscure gastrointestinal bleeding, revealed a tumor in the ileum. When we tried to take biopsies of the ileal tumor by push enteroscopy via the anus, we found another tumor in the transverse colon. On exploration, tumors were identified in the ileum and the transverse colon. Thus, we resected the ileum and transverse colon with regional lymph node dissection. Histologic sections from both the ileum and colon revealed moderately differentiated adenocarcinomas with no lymph node metastasis.

Primary tumors of jejunum and ileum as a cause of intestinal obstruction: A case control study

Background Small-bowel tumors are rare and account for 1–2% of all gastrointestinal neoplasms. Most of these tumors are found at surgery indicated for other diagnosis or intestinal obstruction. The rarity, unclear presentation and diagnostic difficulty of these tumors stimulated our interest to review our experience with emergency surgery for intestinal obstruction secondary to jejunoileal tumors. Methods We reviewed 17 patients operated on for intestinal obstruction secondary to benign and malignant primary tumors of jejunum and ileum at our institution the last 10 years. Results The series comprised 8 male and 9 female patients, most of them younger than 49 years of age. The most frequent tumors found were GIST (36%) followed by lymphomas (24%) and adenocarcinomas (18%). Most tumors (65%) were located in the ileum. Mean survival for patients with malignant tumors was 19.5 ± 13 months, and for patients with benign tumors 72 ± 20 months ( p < 0.05). Conclusion Jejunoileal tumors present frequently in patients younger than 49 years of age. Ileal tumors are more likely to develop intestinal obstruction than jejunal tumors. Emergency surgery for these patients precludes a complete and negative margin resection and constitutes a risk factor for residual disease and short-term survival.

Ileal carcinoid tumor with liver metastases and cardiac involvement treated with intraarterial liposomal doxorubicin and valve replacement

Unless carcinoid are in general slow-growing tumors, some cases could be frankly malignant. The commonest cause of death in patients suffering a carcinoid tumor is liver failure due to tumor progression. When tumors have a fast evolution a multidisciplinary approach must be perform. This case report is an example of this specific situation.

Galectin-4 Expression in Carcinoid Tumors

Galectins (Gal) are an evolutionarily conserved family of 15 carbohydrate-binding proteins (lectins) that are widely distributed in normal and neoplastic cells in a wide range of organisms. They have roles in inflammation, cell adhesion, tumor progression, and metastasis. The function and distribution of Gal-3 and Gal-1 are well characterized, but less information is available about Gal-4. Recent studies have localized Gal-4 in the enterochromaffin cells of the porcine and murine small intestine. We examined the expression of Gal-4 in primary and metastatic human ileal carcinoid tumors as well as in carcinoid tumors of the stomach, lung, and rectum. A total of 44 primary and 42 ileal metastatic carcinoid tumors were examined by immunohistochemistry using tissue microarrays (TMA) with monoclonal antibodies to Gal-4, Gal-3, and Gal-1. Pulmonary (n = 7), rectal (n = 6), and gastric (n = 6) carcinoids were examined with larger tissue sections. A total of 18 pancreatic neuroendocrine tumors were also examined with larger tissue sections. Western blots of three ileal carcinoids were also done. Gal-4 was most highly expressed in the ileal carcinoids and the levels of expression tended to be higher in primary ileal carcinoids compared to the metastatic tumors (p = 0.069). All 18 pancreatic neuroendocrine tumors were negative for Gal-1, Gal-3, and Gal-4. Western blot showed a 32 kDa band for Gal-4 in the ileal carcinoids. Gal-3 and Gal-1 were not detected in the metastatic ileal carcinoids by Western blotting. Gastric carcinoids also expressed Gal-4, but very few pulmonary or rectal carcinoids were positive for Gal-4 (p = 0.002). Lower levels of Gal-1 and Gal-3 expression were present in ileal carcinoids compared to primary pulmonary and rectal tumors. These results show a differential distribution of Gal-4 in carcinoid tumors in different locations of the gastrointestinal tract and the lungs.

Endométriose iléale isolée

Endometriosis is a common condition, but its exclusive localization on the ileum is very rare. Unless there is catamenial exacerbation of symptoms, and considering the lack of specificity of results, diagnosis can be difficult. We report the case of a 50 year-old woman presenting with chronic pain in the right lower quadrant. Initial explorations revealed an ileal tumor which was not characterized before the occurrence of acute small bowel obstruction. Ileo-caecal resection by laparotomy relieved the symptoms and alllesions were removed. Diagnosis of ileal endometriosis was made by pathological examination of the resected specimen.

Metástasis tardía en mama de tumor carcinoide intestinal

We present the case of a woman who underwent surgery for an ileal carcinoid tumor and who subsequently presented breast metastases. The rarity of this case is discussed and a literature review is presented.