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Apart from the lethal midline carcinoma (NUT carcinoma), NUTM1 translocation has also been reported in mesenchymal tumors, but is exceedingly rare. Here, we describe a series of 8 NUTM1-rearranged sarcomas to further characterize the clinicopathologic features of this emerging entity. This cohort included 2 males and 6 females with age ranging from 24 to 64 years (mean: 51 y; median: 56 y). Tumors occurred in the colon (2), abdomen (2), jejunum (1), esophagus (1), lung (1) and infraorbital region (1). At diagnosis, 6 patients presented with metastatic disease. Tumor size ranged from 1 to 10.5 cm (mean: 6 cm; median: 5.5 cm). Histologically, 4 tumors were composed of primitive small round cells to epithelioid cells intermixed with variable spindle cells, while 3 tumors consisted exclusively of small round cells to epithelioid cells and 1 tumor consisted predominantly of highgrade spindle cells. The neoplastic cells were arranged in solid sheets, nests, or intersecting fascicles. Mitotic activity ranged from 1 to 15/10 HPF (median: 5/10 HPF). Other features included rhabdoid phenotype (4/8), pronounced nuclear convolutions (2/8), prominent stromal hyalinization (2/8), focally myxoid stroma (1/8), foci of osteoclasts (1/8), and necrosis (1/8). By immunohistochemistry, all tumors showed diffuse and strong nuclear staining of NUT protein, with variable expression of pancytokeratin (AE1/AE3) (2/8), CK18 (1/8), CD99 (3/8), NKX2.2 (2/8), cyclin D1 (2/8), desmin (2/8), BCOR (2/8), S100 (1/8), TLE1 (1/8), and synaptophysin (1/8). Seven of 8 tumors demonstrated NUTM1 rearrangement by fluorescence in situ hybridization analysis. RNA-sequencing analysis identified MXD4::NUTM1 (3/7), MXI1::NUTM1 (3/7), and MGA::NUTM1 (1/7) fusions, respectively. DNA-based methylation profiling performed in 2 cases revealed distinct methylation cluster differing from those of NUT carcinoma and undifferentiated small round cell and spindle cell sarcomas. At follow-up (range: 4 to 24 mo), 1 patient experienced recurrence at 8.5 months, 4 patients were alive with metastatic disease (5, 10, 11, and 24 mo after diagnosis), 3 patients remained well with no signs of recurrence or metastasis (4, 6, and 12 mo after diagnosis). Our study further demonstrated that NUTM1-rearranged sarcoma had a broad range of clinicopathologic spectrum. NUT immunohistochemistry should be included in the diagnostic approach of monotonous undifferentiated small round, epithelioid to high-grade spindle cell malignancies that difficult to classify by conventional means. DNA-based methylation profiling might provide a promising tool in the epigenetic classification of undifferentiated sarcomas. [ABSTRACT FROM AUTHOR]

The American Joint Committee on Cancer (AJCC) staging system for all cancer sites, including gastroenteropancreatic neuroendocrine tumors (GEP‐NETs), is meant to be dynamic, requiring periodic updates to optimize AJCC staging definitions. This entails the collaboration of experts charged with evaluating new evidence that supports changes to each staging system. GEP‐NETs are the second most prevalent neoplasm of gastrointestinal origin after colorectal cancer. Since publication of the AJCC eighth edition, the World Health Organization has updated the classification and separates grade 3 GEP‐NETs from poorly differentiated neuroendocrine carcinoma. In addition, because of major advancements in diagnostic and therapeutic technologies for GEP‐NETs, AJCC version 9 advocates against the use of serum chromogranin A for the diagnosis and monitoring of GEP‐NETs. Furthermore, AJCC version 9 recognizes the increasing role of endoscopy and endoscopic resection in the diagnosis and management of NETs, particularly in the stomach, duodenum, and colorectum. Finally, T1NXM0 has been added to stage I in these disease sites as well as in the appendix. [ABSTRACT FROM AUTHOR]

Objective: To review the evidence of uncommon but fatal adverse event of hyperammonemic encephalopathy by tyrosine kinase inhibitors (TKI) and the possible mechanisms underlying this condition and to describe the case of a patient that developed drug-induced hyperammonemic encephalopathy related to TKI. Data Sources: Literature search of different databases was performed for studies published from 1 January 1992 to 7 May 2023. The search terms utilized were hyperammonemic encephalopathy, TKI, apatinib, pazopanib, sunitinib, imatinib, sorafenib, regorafenib, trametinib, urea cycle regulation, sorafenib, carbamoyl-phosphate synthetase 1, ornithine transcarbamylase, argininosuccinate synthetase, argininosuccinate lyase, arginase 1, Mitogen activated protein kinases (MAPK) pathway and mTOR pathway, were used individually search or combined. Data Summary: Thirty-seven articles were included. The articles primarily focused in hyperammonemic encephalopathy case reports, management of hyperammonemic encephalopathy, urea cycle regulation, autophagy, mTOR and MAPK pathways, and TKI. Conclusion: Eighteen cases of hyperammonemic encephalopathy were reported in the literature from various multitargeted TKI. The mechanism of this event is not well-understood but some authors have hypothesized vascular causes since some of TKI are antiangiogenic, however our literature review shows a possible relationship between the urea cycle and the molecular inhibition exerted by TKI. More preclinical evidence is required to unveil the biochemical mechanisms responsible involved in this process and clinical studies are necessary to shed light on the prevalence, risk factors, management and prevention of this adverse event. It is important to monitor neurological symptoms and to measure ammonia levels when manifestations are detected. [ABSTRACT FROM AUTHOR]

Background: We report an unusual testicular mass with small bowel and retroperitoneal lymph node deposit proven to be myeloid sarcoma after complete histopathological and hematological workup. Myeloid sarcoma (MS) usually involves lymph nodes and head and neck regions. Uncommon sites like testis and ovary are rarely involved and pose a diagnostic challenge. Extramedullary myeloid sarcoma is most commonly associated with hematological malignancies like acute myeloid leukemia and myelodysplastic syndromes. It can precede or co-occur with AML. Considering it as a differential diagnosis in atypical presentation of testicular tumor helps in early treatment. Case presentation: We present a case of TMS with small bowel and retroperitoneal deposits presenting initially as intussusception and a vague scrotal pain. The patient underwent unilateral left radical inguinal orchidectomy. Surgical pathology revealed myeloid sarcoma of the testicle. And later jejunojejunostomy was done for small bowel deposit causing obstruction with retroperitoneal lymph node biopsy taken which revealed granulocytic sarcoma deposit. He developed peripheral blood involvement 4 weeks postoperatively, and bone marrow biopsy showed acute myeloid leukemia. Conclusions: With very short median survival period of 7.5 months, high index of suspicion is required where multifocal lesions are observed in various sites as in our case. [ABSTRACT FROM AUTHOR]

Background: Sarcomatoid carcinoma (SCA) of the jejunum is an extremely rare malignancy of the small intestine, with only about 20 cases reported to date. This type of cancer has features of both epithelial and mesenchymal tumors. Case Presentation: This case report describes a male patient in his 70s who presented with recurrent episodes of passage of melena. Conclusion: The diagnosis of SCA was confirmed by immunohistochemistry and this case emphasizes the course and metastatic nature of the tumor including constraints surrounding management. [ABSTRACT FROM AUTHOR]

Copyright of Revista de la Facultad de Medicina Humana is the property of Instituto de Investigaciones en Ciencias Biomedicas de la Universidad Ricardo Palma and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Simple Summary: The capecitabine/temozolomide (CAPTEM) regimen has proven activity in pancreatic neuroendocrine tumors (NETs); however, data are limited in NETs of the small bowel. To observe whether this regimen has activity in this patient population, we conducted a retrospective study of all patients with small bowel NETs treated with this regimen at our institution. We found that response rates were poor among patients with low-intermediate grade tumors but higher among patients with high-grade disease. Our findings suggest that the CAPTEM regimen should be reserved for patients with higher-grade small bowel NETs. The capecitabine/temozolomide regimen has significant activity in pancreatic NETs; however, data are limited in NETs of the small bowel (midgut). A retrospective study of all patients with metastatic midgut NETs seen at Moffitt Cancer Center between January 2008 and June 2019 treated with CAPTEM was conducted. 32 patients with proven or suspected well-differentiated primary small bowel NETs (excluding duodenum) were identified. 6 patients were found to have a radiographic response (19%), 5 of whom had high-grade disease. Only one patient among 23 with low/intermediate-grade disease responded (4%), whereas the response rate for patients with high-grade disease was 56%. Among patients with low/intermediate-grade disease, 44% discontinued due to poor tolerability. The CAPTEM regimen appears to have an activity in patients with high-grade small bowel NETs and is largely inactive in patients with low/intermediate-grade tumors. [ABSTRACT FROM AUTHOR]

The objective of this study was to evaluate the effect of L-arginine supplementation on the formation of jejunal lesions and micronuclei in Wistar rats following 5-fluorouracil (5-FU) treatment. Fifty rats were separated into five groups: CG served as the control group, GArg was supplemented L-arginine, G5-FU was administered 5-FU, GArg+5-FU was supplemented L-arginine/day and administered 5-FU, and Gciclo served as a positive control group for micronuclei formation. Jejunal lesions were assessed by histopathological analysis using a scoring system with a maximum of 39 points, based on the following lesions: lymph vessel dilatation, cubic enterocytes, villous flattening, villus fusion, interstitial edema, and apical necrosis of the villi. Micronuclei were counted in polychromatic erythrocytes from the femur bone marrow. The control and GArg rats had the lowest number of jejunal lesions (6.4 ± 2.8 and 5.3 ± 2.4, respectively) and micronuclei (1.9 ± 0.6 and 1.1 ± 0.3, respectively) while the G5-FU rats had the highest number of jejunal lesions (24.2 ± 4.9) and micronuclei (36.0 ± 8.5). The GArg+5-FU rats showed significantly reduced (P < 0.05) jejunal lesions (10.2 ± 4.9) and micronuclei (15.4 ± 5.9). In conclusion L-arginine supplementation potentially reduces the jejunal lesions and DNA damage caused by 5-FU. [ABSTRACT FROM AUTHOR]

Small intestinal adenocarcinomas (SIAs) are rare. Hence, randomized controlled trials are lacking and understanding of the disease features is limited. This nationwide cohort investigates incidence, treatment and prognosis of SIA patients, to improve disease outcome. Data of 2697 SIA patients diagnosed from January 1999 through December 2019 were retrieved from the Netherlands Cancer Registry and Pathology Archive. Incidence was calculated using the revised European Standardized Rate. The influence of patient and tumor characteristics on overall survival (OS) was studied using survival analyses. The age-standardized incidence rate almost doubled from 0.58 to 1.06 per 100,000 person-years, exclusively caused by an increase in duodenal adenocarcinomas. OS did not improve over time. Independent factors for a better OS were a younger age, jejunal tumors, Lynch syndrome and systemic therapy. Only 13.8% of resected patients was treated with adjuvant chemotherapy, which improved OS compared to surgery alone in stage III disease (HR 0.47 (0.35–0.61)), but not in the limited group of deficient mismatch repair (MMR) patients (n = 53, HR 0.93 (0.25–3.47)). In the first-line setting, CAPOX was associated with improved OS compared to FOLFOX (HR 0.51 (0.36–0.72)). For oligometastatic patients, a metastasectomy significantly improved OS (HR 0.54 (0.36–0.80)). The incidence of SIAs almost doubled in the past 20 years, with no improvement in OS. This retrospective non-randomized study suggests the use of adjuvant chemotherapy for stage III disease and first-line CAPOX for metastatic patients. For selected oligometastatic patients, a metastasectomy may be considered. MMR-status testing could aid in clinical decision-making. • A large nationwide cohort study of 2697 small intestinal adenocarcinomas (SIAs). • The incidence of duodenal adenocarcinomas almost doubled with no improvement in OS. • Despite adjuvant chemotherapy prolonging OS, only 13.8% of eligible patients were treated. • In the first-line setting CAPOX prolonged OS compared to FOLFOX and capecitabine. • A metastasectomy in patients with one distant metastasis was associated with OS. [ABSTRACT FROM AUTHOR]

Background: Pancreas-sparing duodenectomy (PSD) offers definitive therapy for duodenal polyposis associated with familial adenomatous polyposis (FAP). We reviewed the long-term complications of PSD and evaluated the incidence of high-grade dysplasia (HGD) and cancer in the remaining upper gastrointestinal tract.Methods: Forty-seven FAP patients with duodenal polyposis undergoing PSD from 1992 to 2019 were reviewed. Long-term was defined as > 30 days from PSD.Results: All patients were treated with an open technique, and 43 (91.5%) had Spigelman stage III or IV duodenal polyposis. Median follow-up was 107 months (IQR, 26-147). There was no 90-day mortality. Seven patients died at a median of 10.5 years (IQR, 5.4-13.3) after PSD, with one attributed to gastric cancer. Pancreatitis occurred in 10 patients (21.3%), and two required surgical intervention. Seven patients (14.9%) developed an incisional hernia, and all underwent definitive repair. Forty-one patients (87.2%) had postoperative surveillance endoscopy over a median follow-up of 111 months (IQR, 42-138). Three patients (6.4%) developed adenocarcinoma (two gastric, one jejunal), and four (8.5%) had adenomas with HGD (two gastric, two jejunal) with a median of 15 years (IQR, 9-16) from PSD. One patient with gastric adenocarcinoma and all patients with HGD or adenocarcinoma of the jejunum required surgical intervention.Conclusion: PSD can be performed with a low but definable risk of long-term morbidity. Risk of gastric and jejunal carcinoma rarely occurs and was diagnosed decades after PSD. This demonstrates the need for lifelong endoscopic surveillance and educates us on the risk of carcinoma in the remaining gastrointestinal tract. [ABSTRACT FROM AUTHOR]

Blue rubber bleb nevus syndrome (BRBNS) is a rare disorder comprised of venous malformation mostly involving the skin and gastrointestinal (GI) tract but can also involve other visceral organs. The most predominant site of GI tract involvement is the small bowel. In patients with GI lesions, treatment depends on the severity of bleeding, and extent of involvement. Conservative therapy with iron supplementation and blood transfusion is appropriate in cases with mild bleeding but in severe cases endoscopic and surgical interventions would be beneficial. Also, medical therapy with sirolimus significantly reduces bleeding. A 20-year-old woman was referred to our hospital after transfusion of six units of packed cell because of several episodes of lower GI bleeding within the past three months in the form of melena and a single episode of hematochezia. Her last hemoglobin level before admission was 10mg/dl. She underwent various unsuccessful investigations since she was eight years old to find the origin of refractory iron deficiency anemia. In upper endoscopy, five bleeding polypoid lesions were discovered in the jejunum. Lesions were excised by snare polypectomy. Over a six-month follow-up period, no signs of lower GI bleeding were noted and the patient had a normal hemoglobin level. [ABSTRACT FROM AUTHOR]

Teratomas are common tumors in children. The most common sites are gonads, however, several extragonadal sites are well known such as sacrococcygeal region, mediastinum, head and neck, stomach, and vagina. Occasionally, teratomas occur in unusual sites which pose diagnostic difficulties. Here, we describe two cases of infantile teratomas with unusual presentation. The first case was a 6-month-old girl who presented with a mass in the right side of the abdomen. Computed tomography (CT) scan showed a solid-cystic mass in the right suprarenal region. The second was a 4-month-old boy who came with a huge abdominal mass. Contrast-enhanced CT showed a tumor arising from the jejunal mesentery. On microscopic examination, they were diagnosed as mature and immature teratoma, respectively. Although neuroblastoma is the most common infantile adrenal neoplasm, the possibility of teratoma should be considered for cystic adrenal lesions. Mesenteric teratoma can be difficult to distinguish radiologically from the more common lymphangioma; however, accurate diagnosis is important in view of long-term follow-up for teratomas because of the possibility of malignant transformation. [ABSTRACT FROM AUTHOR]

The effects of bariatric surgery on the development of gastrointestinal cancers remain ill defined. We present a case of jejunal adenocarcinoma developing 19 years after biliopancreatic diversion (BPD) procedure according to Scopinaro's technique. The patient developed a marked distension of the biliopancreatic limb caused by a jejunal mass, evolving towards the so called "duodenal blowout". Emergency jejunal resection of the biliopancreatic limb, with creation of a new end-to-side jejunal-ileal anastomosis was necessary. The histological examination resulted in a moderately-differentiated ulcerated adenocarcinoma, with reactive lymph nodes and tumor-free resection margins. Tumors of the gastrointestinal system can arise following malabsorptive operations for morbid obesity, and they may be difficult to diagnose, since the symptoms are often attributed to the anatomical-functional changes resulting from this type of surgery. The case reported herein suggests that there is a need for surveillance in patients complaining of digestive symptoms after malabsorptive bariatric surgery. [ABSTRACT FROM AUTHOR]

Background: Small bowel cancer is not a single entity. Population-based studies taking into account histological diversity are scarce. The aim of this study was to report on their trends in incidence by histology in France over the past 20 years.Methods: All patients with a small bowel cancer diagnosed in 15 French administrative areas covered by a registry from the network of French cancer registries (FRANCIM) were included. Age-standardized incidence rates were estimated using the world standard population. Incidence rates were calculated by gender, age group, histology, and 5-year period.Results: The overall age-standardized incidence rates were 1.46/100,000 inhabitants in men and 0.9/100,000 inhabitants in women. Adenocarcinoma was the most common histological type (38%), followed by neuroendocrine tumors (35%), lymphoma (15%) and sarcoma (12%). Age at diagnosis and tumor location differed between adenocarcinoma and neuroendocrine tumors. The incidence of all four tumor types increased significantly over the 20-year period, with the exception of lymphoma in men. The annual percentage change for neuroendocrine tumors was 3.89% in men and 3.61% in women; for sarcoma, it was 3.38% and 4.08%, respectively. The incidence of adenocarcinoma and lymphoma also increased in women with an annual percentage change of 3.05% and 3.32%, respectively.Conclusion: Small bowel cancer incidence has increased over time. This increase occurred with different amplitudes and patterns in the four major histological types. The improvement in imaging techniques could partly explain this increase. It is necessary to determine whether predisposing conditions may contribute to this change. [ABSTRACT FROM AUTHOR]

Monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL) is a provisional entity in the 2017 World Health Organization classifications. To further elucidate the clinicopathologic features of this new disease, we carried out a retrospective, multicenter analysis of 42 patients with MEITL. The median age of the patients was 59 years (range, 20-84 years), and 27 patients (64 %) were male. Thirty-two patients (76 %) were Ann-Arbor stages I-II and 28 (67 %) were Lugano stages I-II1&2. The most frequent site of involvement was the jejunum (N = 21). Most cases expressed CD8 (79 %) and CD56 (95 %) and did not express CD30 (5 %) or EBER (0 %). The median progression-free survival was 6.9 months (95 % CI 4.3-9.6); the median OS was 14.8 months (2.4-27.2). Thirty-two patients (76 %) underwent surgery and 37 (88 %) received chemotherapy. A complete response (CR) rate was 38 %. Sixteen patients had undergone autologous stem cell transplantation (ASCT). Relapse or progression was documented in 24 cases, most frequently in the primary site (N = 23). Four cases showed central nervous system relapse. Age over 55 years, poor performance scale, advanced Lugano stage (IIE-IV), not achieving CR, and not receiving ASCT were associated with inferior OS. While the optimal management of MEITL remains undetermined, achieving CR and consolidative ASCT seem essential. As CHOP might be insufficient for achieving CR, more efficient combinations should be investigated. Additionally, considering the frequent local failure and CNS relapse, novel therapeutic approaches are required to improve survival. [ABSTRACT FROM AUTHOR]

Background: Rhabdomyosarcoma (RMS), especially primary pulmonary RMS, is an extremely rare type of soft tissue sarcoma in adults. Small bowel is an uncommon site for metastases.Case Presentation: This report described an unusual case of jejunum metastasis from primary pulmonary RMS causing intussusception in a 75-year-old man. The patient consulted for 2 weeks of continuous dyspnea. Chest computed tomography (CT) demonstrated a large mass involving the left lower lobe. Transthoracic biopsy confirmed the existence of pleomorphic RMS. Immunohistochemical studies showed positive findings about desmin and MyoD1. The results of gastroscopy, colonoscopy and abdominal CT were all negative. Positron emission tomography/CT demonstrated a fluorodeoxyglucose-reactive large lesion in the left lower lobe without metastatic lesions. The patient received synchronous chemoradiotherapy. After 9 months, the patient presented with intermittent upper abdominal pain with nausea and vomiting. CT showed small bowel dilatation secondary to intussusception. The patient subsequently received laparotomy, and the intussuscepted small bowel segment was resected. Histological examination revealed pleomorphic RMS involving the mucosa, submucosa, and muscular tissues.Conclusions: RMS is highly aggressive and metastatic. The metastatic disease can rapidly progress to cause subsequent complications. The possibility of small bowel metastasis should be considered, although it is extremely rare. [ABSTRACT FROM AUTHOR]

Intussusception is a relatively common disease in pediatric age but it is uncommon in adults. We report a case of a 49-year-old male who presented with an acute jejunojejunal intussusception revealed by abdominal pain and vomiting. He underwent an en bloc resection, and pathological findings concluded to a metastasis of a pulmonary combined small cell carcinoma and adenocarcinoma. A subsequent CT scan revealed the primitive mass of the right lung with no evidence of secondary localization. The biopsy was difficult to perform. The patient underwent a pneumonectomy with lymph node dissection confirming the same diagnosis. He made a good recovery from the surgery, and a postoperative chemotherapy was administrated, and he is in remission until this date. [ABSTRACT FROM AUTHOR]

Choriocarcinoma of the ovary is a rare neoplasm and tends to metastasize early to involve the lungs, brain, and liver. Metastasis to the gastrointestinal tract is rare. We report the case of a young lady, who presented with life-threatening gastrointestinal bleeding, secondary to jejunal involvement. The site of bleeding was localized using a computed tomography angiogram following inconclusive upper and lower gastrointestinal endoscopies. The bleeding jejunal segment was resected and the metastatic disease responded to combination chemotherapy. Metastatic choriocarcinoma of the ovary should be considered a possible diagnosis in patients presenting with gastrointestinal bleeding. [ABSTRACT FROM AUTHOR]

Gastrointestinal stromal tumors (GIST) are mesenchymal tumors of the gastrointestinal tract. Although bleeding is one of the most symptoms seen in GIST, significant acute bleeding is an unusual finding. Here in, three cases with gastrointestinal bleeding due to GIST are presented. Of these patients one had duodenal, one had jejunal and the other had ileal GIST. The duodenal GIST was detected by endoscopy while the jejunal GIST was found by capsule endoscopy and the ileal GIST was shown on magnetic resonance enterography. All patients underwent surgery and the diagnoses of GISTs were confirmed by histopathological evaluation. [ABSTRACT FROM AUTHOR]

Copyright of Oncologist is the property of Oxford University Press / USA and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Background: Neuroendocrine tumors (NETs) account for 30% of small bowel (SB) neoplasms. The objectives of this study were to evaluate the incidence of multifocality in primary small bowel neuroendocrine tumors (SBNETs) and to examine the associated outcomes.Methods: Patients with multifocal SBNET were compared to those with a solitary lesion. Only patients who underwent diagnostic workup and surgical intervention at our institution were included in this study. The primary aim of our study was surgical outcomes and mortality and recurrence. The second aim of our study was to evaluate the utility of double-balloon enteroscopy (DBE) and capsule endoscopy.Results: Of 178 patients with SBNETs during the study period, 85 met inclusion criteria. The mean age was 61.0 ± 12.6 years and 44.7% were male. The ileum was the primary tumor site for 66 patients (77.7%). Of DBE patients, 28 (62.2%) had additional lesions identified, of which 23 (82.1%) had NET confirmed on pathology. Average tumor size was 1.8 cm and most were well differentiated (89.9%), with Ki-67 of ≥ 2% (65.8%); 74.4% had nodal metastases and 51% of patients had stage IV disease. Forty-six patients (54.1%) had multifocal disease, of whom 37 (80.5%) had an ileal primary. No differences in survival or recurrence were seen for multifocal versus solitary disease.Conclusions: SBNETs have a high incidence of multifocality. DBE can be used in the preoperative assessment to detect multifocal NET. Multifocality has no impact on survival or recurrence outcomes. [ABSTRACT FROM AUTHOR]

Background: Current National Comprehensive Cancer Network guidelines for resectable small bowel neuroendocrine tumors (NETs) recommend regional lymphadenectomy. However, no consensus exists on the optimal nodal harvest.Methods: The National Cancer Database was queried for patients with resectable small bowel NETs (1998-2013). Patients with metastatic disease and missing lymph node harvest data were excluded. We performed logistic regression of factors determining nodal positivity and multivariable survival analyses.Results: Of 11,852 patients, 81.8% underwent lymphadenectomy. 79.3% were node positive (N+) and 46.9% of patients had tumors < 1 cm. Independent predictors of N+ were large tumor size, ileal location, and neuroendocrine carcinoma histology. Logistic regression found no difference between observed and expected proportions of N+ patients with lymphadenectomy greater than or equal to eight nodes. Lower metastatic node ratio predicted improved survival on multivariable analysis and is associated with high-volume institutions.Conclusion: Small bowel NETs have high rates of nodal metastasis, even in patients with small tumors, and many patients do not undergo lymphadenectomy despite the clear benefit. Lymphadenectomy of eight nodes is optimal to identify N+ patients. Additionally, minimizing metastatic node ratio with complete regional lymphadenectomy is associated with improved survival in these patients. [ABSTRACT FROM AUTHOR]

Background: Small bowel adenocarcinoma (SBA) is a rare malignancy that accounts for 1-2% of gastrointestinal tumours. We investigated the clinical characteristics, outcomes, and prognostic factors of primary SBA.Methods: We retrospectively analysed the characteristics and clinical courses of 205 SBA patients from 11 institutions in Japan between June 2002 and August 2013.Results: The primary tumour was in the duodenum and jejunum/ileum in 149 (72.7%) and 56 (27.3%) patients, respectively. Sixty-four patients (43.0%) with duodenal adenocarcinoma were asymptomatic and most cases were detected by oesophagogastroduodenoscopy (EGD), which was not specifically performed for the detection or surveillance of duodenal tumours. In contrast, 47 patients (83.9%) with jejunoileal carcinoma were symptomatic. The 3-year survival rate for stage 0/I, II, III, and IV cancers was 93.4%, 73.1%, 50.9%, and 15.1%, respectively. Multivariate analysis revealed performance status 3-4, high carcinoembryonic antigen, high lactate dehydrogenase (LDH), low albumin, symptomatic at diagnosis, and stage III/IV disease were independent factors for overall survival (OS). Ten patients (18.5%) with stage IV disease were treated with a combination of resection of primary tumour, local treatment of metastasis, and chemotherapy; this group had a median OS of 36.9 months.Conclusions: Although most SBA patients were diagnosed with symptomatic, advanced stage disease, some patients with duodenal carcinoma were detected in early stage by EGD. High LDH and symptomatic at diagnosis were identified as novel independent prognostic factors for OS. The prognosis of advanced SBA was poor, but combined modality therapy with local treatment of metastasis might prolong patient survival. [ABSTRACT FROM AUTHOR]

An unusual case of a jejunal mesenteric pseudocyst treated by laparoscopic resection is reported. A 44-year-old woman was admitted to our hospital with intermittent upper abdominal pain and diarrhea. Physical examination revealed slight periumbilical tenderness, and no masses were palpable. Contrast-enhanced computed tomography showed a 4-cmsized nonenhancing high-density mass with a heterogeneous pattern on a proximal small bowel loop. Based on these findings, a gastrointestinal stromal tumor accompanied by hemorrhagic and cystic change, a mesenteric hematoma, or a desmoid tumor was diagnosed. Laparoscopy was performed to obtain an accurate diagnosis. Exploration of the abdominal cavity identified a 4-cm mass originating from the mesentery of the jejunum. Segmental resection of the jejunum and its mesentery, including the mass, was performed. Macroscopically, the mass appeared to be a cystic mass of the jejunal mesentery. The mass within the cyst lumen consisted of white clayish material with no specific pathology. The final pathological diagnosis was a mesenteric pseudocyst. The patient had an uneventful postoperative course. [ABSTRACT FROM AUTHOR]

Background: The clinicopathological characteristics of small intestinal neuroendocrine neoplasms (SI-NENs) and the prognostic validity of WHO grading classification for SI-NENs are still unknown in Asian patients.Methods: 277 patients and 8315 patients with SI-NENs were retrieved respectively from eleven Chinese hospitals and Surveillance, Epidemiology, and End Results (SEER) cancer registry. Overall survival was used as the major study outcome. Survival analysis using Kaplan-Meier analysis with log-rank test and cox regression analysis were applied.Results: Clinicopathological characteristics of SI-NENs were quite different among different races. Duodenum was the predominant tumor site in Chinese patients and Asian/Pacific Islander patients but not in white patients from SEER database. Patients with duodenal NENs tended to have more localized disease than patients with jejunal/ileal NENs which were confirmed by patients from SEER database. Grade 3 or poorly differentiated/undifferentiated tumor were more common and tumor size was significantly larger in ampullary NENs compared with that in non-ampullary duodenal NENs. As for the prognostic validity of WHO grading classification, survival between patients with grade 1 and grade 2 disease was not significantly different. Ki-67 index of 5% might be a better threshold between grade 1 and grade 2 than Ki-67 index of 2% in SI-NENs.Conclusions: Our study revealed that the clinicopathological characteristics of SI-NENs among different races were quite different. This might because duodenal NENs was much more common in Chinese patients and Asian/Pacific Islander patients. Duodenal NENs and jejunal/ileal NENs, ampullary and non-ampullary duodenal NENs shared different characteristics. Ki-67 index of 5% might be a better threshold between grade 1 and grade 2 in SI-NENs. [ABSTRACT FROM AUTHOR]

Background: Although ectopic pancreatic tissue is common in the upper gastrointestinal tract, the incidence of ectopic pancreatic tissue in the jejunum is low, and malignant transformation in ectopic pancreatic tissue is rare. Furthermore, pancreatic-type acinar cell carcinoma (ACC) developing in the jejunum and ACC accompanied by tumor thrombus are extremely rare.Case Presentation: A 78-year-old-woman presented with melena. Abdominal computed tomography images and endoscopic examination revealed a submucosal jejunal mass with tumor thrombus extending into a jejunal vein. The patient underwent a curative resection combined with a partial jejunectomy and partial pancreatectomy. Histopathological examination of the resected tissue showed tumor cells with a homogeneous acinar architecture identical to pancreatic-type ACC and tumor thrombus. Postoperatively, she was followed for 10 months and had no recurrence.Conclusion: We present an extremely rare case of pancreatic-type ACC in the jejunum with extensive tumor thrombus invading into the mesenteric venous system. This type of cancer has not been reported previously but should be considered in the differential diagnosis of a jejunal mass. [ABSTRACT FROM AUTHOR]

We hereby present the case of a 58-year-old male who underwent a total gastrectomy for gastric neoplasm. During the surgery, a tumor mass in the jejunum was identified, considered as metastasis, and resected. The histopathological examination of the jejunal lesion showed ectopic pancreas. In this area, two pathological distinct lesions were identified, one histologically compatible with pancreatic intraepithelial neoplasia (PanIN) type 2 lesion and the other with morphologic criteria for endocrine microadenoma. To our knowledge, this is the first case that evidences the presence of a concomitant premalignant exocrine lesion and benign endocrine lesion in a heterotopic pancreas (HP). [ABSTRACT FROM AUTHOR]

A 12-year-old, 3.6-kg, spayed female domestic shorthaired cat had a 2-month history of anorexia and weight loss. Abdominal ultrasonography and computed tomography revealed an exophytic mass originating from the jejunum with very poor central and poor peripheral contrast enhancement. On day 14, surgical resection of the jejunum and mass with 5-cm margins and an end-to-end anastomosis were performed. Histopathological examination revealed the mass was a transmural, invasive cancer showing exophytic growth and originating from the small intestinal muscle layer. Immunohistochemical analysis of tumor cells revealed diffuse positivity for KIT protein and negativity for desmin and S-100. The mass was diagnosed as a gastrointestinal stromal tumor (GIST). Ultrasonographic findings indicated the tumor probably metastasized to the liver and omentum, as seen in humans and dogs. The owner rejected further treatment at the last visit on day 192. To our knowledge, this is the first report of intestinal tumor and metastasis in feline GIST and its imaging features. [ABSTRACT FROM AUTHOR]

The article discusses updates to the consensus guidelines of medical personnel society European Neuroendocrine Tumor Society (ENETS) for the diagnosis and treatment of neuroendocrine neoplasms of the jejunum and ileum. Topics of the consensus guideline include prognosis of non-metastatic neuroendocrine neoplasms (NENs) from small bowel, its disease prognosis and tumor-specific hormone-hypersection-related symptoms. It also highlights the diagnostic modality for transthoracic echocardiography.

Objective: Rare disease. Background: Angioleiomyoma in the small intestine is a rare cause of gastrointestinal bleeding. Only 7 cases of angioleiomyoma in the small intestine were reported in the English literature, with 4 of them causing gastrointestinal bleeding. The diagnosis of angioleiomyomas in the small intestine before surgery is difficult. Case Report: We report the case of a 42-year-old man with recurrent melena who underwent repeated esophagogastroduodenoscopy and colonoscopy, without positive finding. During a double-balloon enteroscopy, an elevated lesion with a diameter of 6 mm was found in the jejunum. The lesion was resected laparoscopically assisted with double-balloon enteroscpy. A microscopic examination showed fibric membrane of the mass and numerous vascular channels surrounded by proliferated smooth muscle. There were exudative fibrin and many thrombi formed by red blood cells. Immunohistochemistry was positive for SMA and CD34. A pathological diagnosis of jejunal angioleiomyoma with thrombus was established. During a 5-year follow-up, there was no further gastrointestinal bleeding. Conclusions: The gastroenterologists should consider angioleiomyoma in the small intestine when assessing obscure gastrointestinal bleeding. [ABSTRACT FROM AUTHOR]

Gastrointestinal stromal tumours (GISTs) are rare tumour. It comprises 0.2% of gastrointestinal tumours and only 0.04% of small intestinal tumours. Usually this tumour is found in people in their 60s.These tumours can be found anywhere in the gastrointestinal tract but most commonly found in the stomach (40–60%). Patients with GISTs usually have vague and nonspecific symptoms. We present a 17year-old with complain of abdominal pain, distension, and few episodes of vomiting. All laboratory investigations were normal. The ultrasonography (USG) and computed tomography (CT) were suggestive of jejuno-jejunal intussusception. The patient had emergency exploratory laparotomy with resection of the segment with five cm healthy margin, and end to end anastomosis. Patient was discharged after 4 days and remains well. This case report highlights the rarity of GISTs in the concern of age (17 year old), site (Jejunum) as well as presentation (intussusception). [ABSTRACT FROM AUTHOR]

Background: Primary jejunal gastrinomas are exceedingly rare, and data for long-term follow-up is limited. Until now, only six cases of gastrinomas arising from the jejunum have been reported in the English literature. Case Presentation: Presented is a case of a primary gastrinoma located in the proximal jejunum. After surgical resection of the tumor, eugastrinemia was quickly achieved and after a 10-year follow-up period, the patient was still disease-free. Conclusions: This case report demonstrates that surgical resection of a primary jejunal gastrinoma without evidence of metastasis can be curative, with a good long-term prognosis. [ABSTRACT FROM AUTHOR]

Gastrointestinal stromal tumors (GISTs) represent the majority of primary nonepithelial neoplasms of the digestive tract, most frequently expressing the KIT protein detected by immunohistochemical staining for the CD117 antigen. Jejunal GISTs account for approximately 10 % of GISTs. Patients usually present with abdominal discomfort. Jejunal GISTs may cause symptoms secondary to obstruction or hemorrhage. Pressure necrosis and ulceration of the overlying mucosa may cause gastrointestinal bleeding, and patients who experience significant blood loss may suffer from malaise and fatigue. Literature has classified small-bowel GISTs on the basis of size, and various established guidelines have advised conservative management of small jejunal GISTs (<2 cm). We here report the clinical, macroscopic, and immunohistological features of a small jejunal GIST presenting with acute lower gastrointestinal hemorrhage in a 50-year-old postmenopausal woman necessitating an emergency laparotomy to control the bleed. The management of very small (<2 cm) small-bowel GISTs is controversial. While guidelines are primarily based on the risk of malignancy in GISTs, no guideline predicting the risk of complications in small-bowel GISTs exists. Hence, these tumors should be removed even if incidentally detected. [ABSTRACT FROM AUTHOR]

The ovaries are common site of metastasis in a variety of primary neoplasms. Multiple tumors such as breast, lung, and pancreas have been reported to metastasize to the ovary, however; the colon and stomach are the most common primary cancer sites that of ovarian metastasis. An ovarian mass mostly originates from its self-tissue, but sometimes it can be a metastasis of a gastrointestinal system tumor. Such cases are often misdiagnosed as primary ovarian cancers. A 42-year-old woman was admitted to our hospital with pelvic pain. She had a history of her complaints for two months. Bilateral large ovarian mass was detected in transvaginal ultrasound. Laparotomy was performed, the pathologist suggested inspection of the stomach after the frozen section analysis; therefore, an irregular mass on the stomach was detected. The general surgeon was attended to the operation, and an inoperative stomach tumor was reported by the general surgeon. After that due to the partial obstruction of jejunum, a gastrojejunostomy was performed. It is in fact difficult to distinguish between metastatic mucinous carcinomas and primary mucinous carcinomas of the ovary, due to the similar appearance of as cystic tumors on gross examination. The clinicians should be aware of the likely concomitant gastrointestinal system tumor when a large and bilaterally mass was detected on physical examination. This case also reminds that a systemic examination is necessary even if the large ovarian tumors suspicious of primary malignancy were noticed. [ABSTRACT FROM AUTHOR]

Neoplasms of the small intestine are rare. Leiomyomas are the one of the benign neoplasms that can be found in the small intestine. These lesions usually remain asymptomatic and are often found incidentally; however, they can become symptomatic if obstruction occurs or the tumor outgrows its blood supply resulting mucosa. Their clinical manifestations can include abdominal pain, diarrhea, melena, a palpable abdominal mass and weight loss. Due to their vague presentation, leiomyomas of the small intestine are typically diagnosed after failed conservative therapy for other pathologies such as duodenal ulcers and diverticulosis. This case report describes a 34-year-old male who developed an acute GI bleed due to a symptomatic leiomyoma of the jejunum. He was initially treated with non-operative conservative management; however, his symptoms remained unresolved and he went on to require surgical intervention. [ABSTRACT FROM AUTHOR]

Gastrointestinal stromal tumors (GIST) are relatively rare and often present with vague symptoms. Their first clinical manifestation as acute abdomen due to perforation is extremely rare. We report a rare case of spontaneous perforation of exophytic gastrointestinal stromal tumor (GIST) causing perforation of jejunum. A 61 year, male patient presented with symptoms of acute abdomen. After evaluation, a laparotomy was performed, where perforation of a tumor in the jejunum was found. The perforated part along with the tumor was resected and the histopathological examination showed that the tumor was GIST. In emergency setting the patients with perforation peritonitis, a diagnosis of spontaneous perforation of GIST though rare should be kept in mind specially in elderly patients. [ABSTRACT FROM AUTHOR]

Fibromatosis is the most common primary tumor of mesentery. Mesenteric fibromatosis is a very rare lesion which does not metastasise but can be locally aggressive. Fibromatosis may be classified as extra-abdominal, abdominal wall, or intra-abdominal. The etiology of fibromatosis has not been determined. Most of the patients with mesenteric fibromatosis are asymptomatic. Herein, we presented a case who was 45 years old, complained of left lower quadrant pain. Preoperative evaluation included abdominal ultrasound, computed tomography (CT). According to the CT report, the patient was diagnosed with gastrointestinal stromal tumor. A mobile mass was seen on the jejunal mesentery during laparoscopic exploration. The tumor was removed by wide excision with safe margins. Patients was discharged on the fifth day of the operation. The histological diagnosis was of jejunal agressive fibromatosis. Even though there are different types of treatment protocols, surgery with negative surgical margin is preferred to prevent local recurrence. In these type of cases safe margins can be achieved with wide laparoscopic excision. [ABSTRACT FROM AUTHOR]

The gastrointestinal (GI) tract is the most common extranodal site of involvement in non-Hodgkin lymphoma (NHL). Primary GI NHL is frequently discussed in survival analyses. Primary intestinal NHL is significantly different from primary gastric NHL with regard to its clinical features, pathological subtype, treatment and prognosis. The small intestine is involved in lymphoma less often than the large intestine. The present study aimed to analyze the clinical and pathological characteristics of primary NHL of the small intestine and its prognostic factors. A retrospective analysis was performed on clinical data from 313 cases of NHL that occurred between 1995 and 2008 in the Tri-Service General Hospital (National Defense Medical Center, Taipei, Taiwan). Among these cases, 11 cases of primary NHL of the small intestine were identified. A Cox model was used to perform the multivariate analysis. The Kaplan-Meier method was used for the survival analysis. From the 11 patients with primary NHL of the small intestine, seven patients were male (63.6%) and four patients were female (36.3%). Furthermore, nine patients (81.8%) were diagnosed with B-cell lymphoma, of which five (45.5%) were also diagnosed with diffuse large B-cell lymphoma (DLBL). Abdominal pain and/or distention were present in six (54.5%) of the patients and jejunum involvement was also observed in six (54.5%) of the 11 patients. The mean overall survival (OS) time of the 11 patients was 27.2 months and the four-year survival rate was 36.3%. The mean OS time in the patients with jejunum involvement was shorter than in those without jejunum involvement (16.9 vs. 39.6 months), although this difference was not significant (P=0.657). Surgical treatment was performed on four of the six patients with jejunum involvement due to an acute abdomen or perforation-related peritonitis. The results of the present study indicate that DLBL is the most common subtype of primary lymphoma of the small intestine, and that the site involved in NHL may affect the potential for surgery in patients with intestinal lymphoma. Furthermore, patients with primary lymphoma of the small intestine have been found to have a poor outcome compared with those with lymphoma in other regions of the GI tract. In the present study, a similar trend was observed, however, the sizes of the subgroups of primary lymphoma of the small intestine were too small for individual analysis. [ABSTRACT FROM AUTHOR]

Other than that in the duodenum, adenocarcinoma in the small bowel is rare. The present study describes a case of adenocarcinoma with adenoma in the jejunum. A 70-year-old male was admitted to hospital due to dehydration induced by abdominal discomfort and difficulty with oral intake. Computed tomography revealed a tumor in the upper side of the jejunum, which was subsequently resected. The tumor contained adenocarcinoma and adenoma. The protein expression of p53 and Ki-67 was analyzed in the normal mucosa, adenoma and adenocarcinoma. The number of epithelial cells expressing p53 and Ki-67 was found to increase in the adenoma tissue compared with that in the normal mucosa. In the adenocarcinoma tissue, the number of cells expressing p53 and Ki-67 further increased, suggesting that an adenoma-adenocarcinoma sequence may occur in the small bowel, similar to that observed in the large bowel. [ABSTRACT FROM AUTHOR]

Primary Small bowel tumours are rare accounting for only 3-6% of GI neoplasm; 1-2% of these are malignant. Their presentation is usually with nonspecific symptoms that causes delay in diagnosis and consequently a worse outcome for the patient. We report a case of Jejunal adenocarcinoma where early diagnosis and treatment lead to good outcome [ABSTRACT FROM AUTHOR]

Aims.The objectives of this studywere (a) to report our experience regarding the association between neurofibromatosis type 1 (NF1) and gastrointestinal stromal tumors (GISTs); (b) to provide a systematic review of the literature in this field; and (c) to compare the features of NF1-associated GISTs with those reported in sporadic GISTs. Methods.We reported two cases of NF1-associated GISTs. Moreover we reviewed 23 case reports/series including 252 GISTs detected in 126 NF1 patients; the data obtained from different studieswere analyzed and compared to those of the sporadic GISTs undergone surgical treatment at our centre. Results. NF1patients presenting with GISTs had a homogeneous M/F ratio with a mean age of 52.8 years. NF1-associated GISTs were often reported as multiple tumors, mainly incidental, localized at the jejunum, with a mean diameter of 3.8 cm, a mean mitotic count of 3.0/50 HPF, and KIT/PDGFRα wild type.We reported a statistical difference comparing the age and the symptoms at presentation, the tumors' diameters and localizations, and the risk criteria of the NF1-associated GISTs comparing to those documented in sporadic GISTs. Conclusions. NF1-associated GISTs seem to have a distinct phenotype, specifically younger age, distal localization, small diameter, and absence of KIT/PDGRFα mutations. [ABSTRACT FROM AUTHOR]

Whether laparoscopy-assisted resections for gastrointestinal stromal tumors (GISTs) of small bowel can achieve more benefits on the aspect of postoperative outcomes than open surgery remains a question. The aim of this study is to evaluate the benefits of laparoscopy-assisted surgery for GISTs of jejunum and ileum on the perioperative outcomes and long-term relapse-free survival. From January 2004 to December 2010, 81 patients who underwent either laparoscopy-assisted (LAP group, n = 43) or open surgery (OPEN group, n = 38) were included in final analysis after the eligibility criteria. Clinicopathological characteristics of the selected patients were similar between the two groups. Oral intake was significant earlier (P < 0.001) and postoperative hospital stay duration was significantly shorter (P < 0.001) in the LAP group than in the OPEN group. The complication rate of patients in the LAP group and OPEN group was 9.3 and 26.3 per cent, respectively (P = 0.04). No significant difference was observed in terms of 5-year relapse-free survival between LAP and OPEN groups (91.1 vs 93.8%, P = 0.4). Laparoscopy-assisted surgery for GISTs of jejunum and ileum could get preferable short-term postoperative outcomes and similar long-term relapse-free survival compared with open surgery, so it should be recommended for GISTs of the small intestine. [ABSTRACT FROM AUTHOR]

Gao C, Guo W-J, Wang A-Y. Significance of decreased apoptosis, role of Bid expression and Bcl-Xl/Bid ratio in human jejunal stromal tumors. APMIS 2012; 120: 171-81. Given that the studies on human small intestinal stromal tumors are very limited, our study was designed to determine the significance of apoptosis, the role of some apoptosis-related proteins, including Bax, Bad, Bid, Bcl-2, Bcl-Xl, and Bcl-W, in jejunal stromal tumors (JST). For this purpose, 52 samples were examined by terminal deoxynucleotidyl transferase-mediated dUTP nick end labeling and immunohistochemistry, including 40 cases from normal jejunum and 12 cases from the JST. The results showed that a significantly decreased apoptosis was observed in JST compared with normal jejunum (median of apoptotic index, 0.13% vs 15.17%, p < 0.001). In univariate analysis, statistically significant differences were shown in percentage of positive-expression of Bid (0.0% vs 77.5%, p < 0.001), Bcl-2 (91.7% vs 42.5%, p = 0.003), and Bcl-Xl (75.0% vs 35.0%, p = 0.014), but not in Bax, Bad, and Bcl-W. Moreover, Bcl-Xl was an independent parameter associated with JST by multivariate Logistic analysis. For the role of ratios of anti-apoptotic proteins to pro-apoptotic proteins, Bcl-Xl/Bid ratio was the most preferable index. In conclusion, decreased apoptosis and expression of Bid, increased level of Bcl-Xl may play some important roles in human JST, and Bcl-Xl/Bid ratio may be as a new potentially associated index, although they should be validated in more studies. [ABSTRACT FROM AUTHOR]

GIST is the commonest mesenchymal tumour of the gastrointestinal tract. Jejunal GIST is rare and spontaneous perforation of asymptomatic jejunal GIST is unique. A review of the English literature reveals only fifteen cases of perforated GIST till date. Pre-operative diagnosis is difficult. Diagnosis is confirmed on histopathology and immunohistochemistry. Complete removal with postoperative imatinib therapy entails optimal treatment. Perforated GIST is associated with high recurrence. [ABSTRACT FROM AUTHOR]

Multimodal treatment options for advanced gastroenteropancreatic neuroendocrine tumours (NET) of jejunum/ileum and of pancreatic origin are reviewed. Current topics being discussed are: European Neuroendocrine Tumour Society 2006/7, American Joint Cancer Committee/Union Internationale Contre le Cancer 2009 and WHO 2010 recommendations for grading and staging of NET; surgery of the primary tumour in distant metastasised disease; surgery of metastatic liver disease and impact on survival; somatostatin analogues for symptom control and for tumour control; selective internal radiation therapy with 90Y-microspheres as novel local ablative therapy in liver metastases; peptide receptor radionuclide therapy; novel chemotherapy regimens (eg, temozolomide) and novel targeted therapies (eg, sunitinib and everolimus). [ABSTRACT FROM AUTHOR]

Background: An association between small bowel adenocarcinoma and Crohn's disease (CD) is well-established. We present our recent experience with this entity in order to further elucidate its clinicopathological features and update our series from 1991.Methods: A retrospective review was undertaken of all surgical patients with small bowel adenocarcinoma and CD seen at our institution between 1993 and 2009. Follow-up was assessed until time of death or by interview with survivors. Survival was calculated based on TNM (tumor extent, lymph node status, metastases staging) staging and comparing between our current and previous series.Results: Twenty-nine patients (ten females and 19 males) were identified and followed for a median of 2 years. The median age at onset of CD symptoms was 25, and the median age at cancer diagnosis was 55.4, for a mean interval of 25.3 years. Twenty-two cancers were ileal and five were jejunal. There were no cancers in excluded intestinal loops. Significant differences in 2-year survival were determined for: node-negative (79.3%, 95%CI 58.3-100%) versus node-positive cancers (49% %, 95%CI 20.0-78.0%), and for localized (92.3%, 95%CI 77.8-100%) versus metastatic disease (33.3%, 95%CI 6.6-60%). Overall 36-month survival was 69.3% (95%CI 51.5-87.1%) compared to 40% among those without excluded loops in our series from 1991. Sixteen patients had long periods of quiescent disease before diagnosis (7-45 years), and 16 required surgery for bowel obstruction that was refractory to medical management. Adequate information was not retrievable for three patients.Conclusions: A comparison to our previous series reveals similar clinical characteristics and a high rate of node-positive cancer at diagnosis. Our findings also confirm two important clinical indicators of malignancy: recrudescent symptoms after long periods of relative quiescence and small bowel obstruction that is refractory to medical therapy. [ABSTRACT FROM AUTHOR]

An 18-year-old girl presented with abdominal pain and a tumor was subsequently detected in the jejunum. We therefore carried out a wedge resection of the jejunum. The diagnosis of GIST was confirmed histologically, and a mutation in exon 9 of the c-kit gene was observed. GISTs are rare in pediatric populations and pediatric GISTs occur predominantly in females and are characterized by a multifocal gastric location and a wild-type phenotype for the c-kit genes. The features of pediatric GISTs of the small intestine have not yet been categorized, and to date, only 11 cases in patients younger than 18 years have been reported. These cases did not occur primarily in females and tended to present as single tumors with mutations in the c-kit gene. This suggests that these cases do not have the same features as pediatric gastric GISTs, but instead are similar to adult GISTs. In pediatric populations, GISTs of the small intestine were expected to show a better response to imatinib treatment than gastric GISTs because of the alterations in the c-kit gene. [ABSTRACT FROM AUTHOR]

Background: Despite having a dramatically larger surface area than the large intestine, the small intestine is an infrequent site for the development of adenocarcinoma. To better understand the molecular abnormalities in small bowel adenocarcinoma (SBA), we characterised a number of candidate oncogenic pathways and the immunophenotype of this rare cancer.Methods: Tissue microarrays were constructed from tumour samples from 54 patients with all stages of the disease. Immunohistochemistry and microsatellite instability (MSI) testing were conducted.Results: The profile of cytokeratin 20 and 7 coexpression was variable, but expression of caudal type homeobox transcription factor 2 (CDX2) was present in 70% of cases. In this young population (median age 54 years), loss of mismatch repair (MMR) proteins occurred in 35% of patients, with confirmed MSI in 100% of tested cases. Expression of vascular endothelial growth factor-A (VEGF-A) and epidermal growth factor receptor (EGFR) was common, occurring in 96 and 71% of patients, respectively. Only one case showed HER2 expression and none showed loss of phosphatase and tensin homologue mutated on chromosome 10 (PTEN).Conclusions: These results suggest that alterations in DNA MMR pathways are common in SBAs, similar to what is observed in large bowel adenocarcinomas. Furthermore, the high percentage of tumours expressing both EGFR and VEGF suggests that patients with this rare cancer may benefit from therapeutic strategies targeting EGFR and VEGF receptor (VEGFR). [ABSTRACT FROM AUTHOR]