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1
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Hu ML, Chiu YC, Tai WC, Huang HY, Yen HH, Chen YY, Chen CW, Su MY, Lin WP, Chiu CT, Chou JW, Feng CL, Huang SF, Shih YS, Chen SF, Ting CF, Chiang IP, Yii CY, Huang HC, Chen CT, Chang CW, Wang HY, Chang CW, Tsai CC, Lin SC, Shih SC, Tai CM, Tu CH, Lee TC, Perng DS, Shun CT, Wang HP, Lin CC, Chang WH, Chan YJ, Chen YJ, Chang YF, Tseng CH, Chen YS, Mo LR. Malignant Neoplasms of the Small Intestines. ATLAS OF SMALL INTESTINAL DISORDERS 2023:19-45. [DOI: 10.1007/978-981-99-6682-0_2] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/05/2025]
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El Bakouri A, El Wassi A, Eddaoudi Y, Bouali M, ElHattabi K, Bensardi F, Fadil A. Early Discovery Of Small Bowel Adenocarcinoma In a Patient Admitted For 4 Acute Intestinal Intussusception case report. Ann Med Surg (Lond) 2022; 82:104776. [PMID: 36268363 PMCID: PMC9577972 DOI: 10.1016/j.amsu.2022.104776] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/14/2022] [Revised: 09/18/2022] [Accepted: 09/19/2022] [Indexed: 11/29/2022] Open
Abstract
Introduction Malignant tumours of the small bowel are uncommon in clinical practice. Adenocarcinoma is the most common of these tumours, accounting for approximately 35–45% of all tumours. It may occur sporadically, in association with familial adenomatous polyposis coli or Peutz-Jeghers syndrome or hereditary non-polyposis colorectal cancer, or in association with chronic inflammatory bowel changes (such as Crohn's disease or celiac disease). Materials and methods We report a case of Early Discovery Of Small Bowel Adenocarcinoma In A Patient Admitted For 4 Acute Intestinal Intussusception in the department of Emergency visceral surgery P35 of the ibn rochd hospital in casablanca. Results Our patient was admitted to the emergency room for sub-occlusive syndrome with generalized abdominal pain of chronic appearance dating back to one month before his admission With Abdominal and pelvic ultrasound showed: intestinal parietal thickening and minimal ascites (peritoneal and/or intestinal tuberculosis? Crohn's disease) The patient underwent an abdominal-pelvic CT scan which showed: Presence of diffuse small bowel thickening, involving several small intestines and the colonic angle with intestinal invaginations (at least 3) suspecting an inflammatory or tumoral origin? To be compared with histological data and infiltration of the mesenteric fat in the sub-umbilical region with a peritoneal effusion in the Douglas. the patient was operated on in the emergency room, approached by laparotomy and found on exploration: Presence of 3 invaginations in the small intestine located at 20cm and 90cm from the Duodenojejunal Angle (DIA) as well as at 25cm from the Last part of the small intestine (DAI), with Presence of a colonic invagination at the level of the left colonic angle. the patient underwent 3 small bowel resections and one segmental colonic resection including segmental small bowel resections: the 1st one of 30 cm taking away an invagination of the small intestine at 20cm from the ADJ, the 2nd one taking away 60cm of invaginated located at 90cm from the ADJ the 3rd one taking away 20cm of invaginated located at 25cm from the DAI and a 4th resection taking away an invagination of the left colonic angle with 3 Anastomosis of the T-T small intestine and a transverse Colostomy in Bouilley Volkman. On examination by the anapathomopathologist: consistent with a small bowel tumour: well-differentiated intestinal adenocarcinoma on degenerated adenomatous polyps measuring 2.5cm and 1.7cm with an estimated 10% mucinous component with no vascular emboli and no peri-nervous sheathing. TNM stage p: pT2 with healthy resection margins in the left colon: Presence of a tubular adenoma with low grade dysplasia. Conclusion The most common symptoms of adenocarcinoma of the small bowel are obstruction, overt or covert bleeding, weight loss and jaundice. Because the small bowel has long been relatively inaccessible to routine endoscopy, the diagnosis of small bowel adenocarcinoma was often delayed for several months after the onset of symptoms. Therefore, in case of suspicion of this type of cancer, a thorough evaluation should be undertaken. Nowadays, endoscopy of the small bowel is widely available, allowing an earlier non-invasive diagnosis.
Acute Intestinal Intussusception as a cause of intestinal obstruction is often a diagnostic challenge mimicking a wide spectrum of diseases. Malignant tumours of the small bowel are uncommon in clinical practice. Adenocarcinoma is the most common of these tumours. Its diagnosis is still very difficult. The treatment of Acute Intestinal Intussusception is in most cases surgical. The diagnosis of Acute Intestinal Intussusception is histological.
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Turpin A, El Amrani M, Zaanan A. Localized Small Bowel Adenocarcinoma Management: Evidence Summary. Cancers (Basel) 2022; 14:2892. [PMID: 35740558 PMCID: PMC9220873 DOI: 10.3390/cancers14122892] [Citation(s) in RCA: 11] [Impact Index Per Article: 3.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/12/2022] [Revised: 06/06/2022] [Accepted: 06/09/2022] [Indexed: 12/04/2022] Open
Abstract
Small bowel cancers are rare diseases whose prognosis is poorer than that of colon cancers. Due to disease rarity, there is little data on small bowel adenocarcinoma (SBA) treatment, and most recommendations come from expert agreements or analogies to the management of colon cancer. Although relatively high rates of local recurrence are observed for duodenal malignancies, distant metastatic relapse remains common and requires adjuvant systemic therapy. Given the similarities between SBA and colorectal cancer, radiotherapy and chemotherapy strategies used for the latter disease are frequently pursued for the former disease, specifically for tumors located in the duodenum. However, no previous randomized study has evaluated the benefit of adjuvant chemotherapy on the overall survival of SBA patients. Most previous studies on treatment outcomes and prognostic factors in this context were based on large international databases, such as the Surveillance, Epidemiology, and End Results or the National Cancer Database. Studies are required to establish and validate prognostic and predictive markers relevant in this context to inform the use of (neo) adjuvant treatment. Among those, deficient mismatch repair tumors represent 20% of SBAs, but their impact on chemosensitivity remains unknown. Herein, we summarize the current evidence on the management of localized SBA, including future perspectives.
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Affiliation(s)
- Anthony Turpin
- UMR9020-UMR-S 1277 Canther-Cancer Heterogeneity, Plasticity and Resistance to Therapies, University of Lille, CNRS, Inserm, CHU Lille, Institut Pasteur de Lille, 59000 Lille, France
- Medical Oncology Department, CHU Lille, University of Lille, 59000 Lille, France
| | - Mehdi El Amrani
- Department of Digestive Surgery and Transplantation, Lille University Hospital, 59000 Lille, France;
| | - Aziz Zaanan
- Department of Gastroenterology and Digestive Oncology, European Georges Pompidou Hospital, AP-HP Centre, 75015 Paris, France;
- Centre de Recherche des Cordeliers, INSERM, Sorbonne Université, Université de Paris, MEPPOT, 75006 Paris, France
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Outcomes of Patients with Small Intestine Adenocarcinoma in a Canadian Province: A Retrospective Multi-Center Population-Based Cohort Study. Cancers (Basel) 2022; 14:cancers14112581. [PMID: 35681560 PMCID: PMC9252258 DOI: 10.3390/cancers14112581] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/13/2022] [Accepted: 05/19/2022] [Indexed: 11/18/2022] Open
Abstract
Background: Small intestine adenocarcinoma is a rare cancer. The current study aims to determine the outcomes of patients with small intestine adenocarcinoma in a Canadian province. Methods: This retrospective population-based cohort study assessed patients with small intestine adenocarcinoma who were diagnosed from 2008 to 2017 in Saskatchewan. A Cox proportional multivariate regression analysis was performed to determine the correlation between survival and exploratory factors. Results: 112 eligible patients with a median age of 73 years and M:F of 47:53 were identified. Overall, 75% had a comorbid illness, and 45% had a WHO performance status >1. Of the 112 patients, 51 (46%) had early-stage disease and 61 (54%) had advanced-stage disease. The median overall survival (mOS) was as follows: stage one, 59 months; stage two, 30 months; stage three, 20 months; and stage four, 3 months (p < 0.001). The median disease-free survival of patients with stage three disease who received adjuvant chemotherapy was 26 months (95% CI:23.1−28.9) vs. 4 months (0.0−9.1) with observation (p = 0.04). Patients who received chemotherapy for advanced disease had a mOS of 10 months (3.5−16.5) vs. 2 months (0.45−3.6) without chemotherapy (p < 0.001). In the multivariate analysis, stage four disease, hazard ratio (HR), 3.20 (1.84−5.40); WHO performance status >1, HR, 2.22 (1.42−3.45); lack of surgery, HR, 2.10 (1.25−3.50); and a neutrophil:lymphocyte ratio of >4.5, HR, 1.72 (1.10−2.71) were significantly correlated with inferior survival. Conclusions: Most patients with small intestine adenocarcinoma were diagnosed with advanced-stage disease. Advanced-stage disease, poor performance status, lack of surgery and a baseline neutrophil:lymphocyte ratio >4.5 were correlated with inferior survival.
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Gelsomino F, Balsano R, De Lorenzo S, Garajová I. Small Bowel Adenocarcinoma: From Molecular Insights to Clinical Management. Curr Oncol 2022; 29:1223-1236. [PMID: 35200603 PMCID: PMC8870676 DOI: 10.3390/curroncol29020104] [Citation(s) in RCA: 12] [Impact Index Per Article: 4.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/19/2022] [Revised: 02/10/2022] [Accepted: 02/14/2022] [Indexed: 12/03/2022] Open
Abstract
Small bowel adenocarcinoma (SBA) is a rare malignancy, with a rising incidence in recent decades, and accounts for roughly 40% of all cancers of the small bowel. The majority of SBAs arise in the duodenum and are associated with a dismal prognosis. Surgery remains the mainstay of treatment for localized disease, while systemic treatments parallel those used in colorectal cancer (CRC), both in the adjuvant and palliative setting. In fact, owing to the lack of prospective data supporting its optimal management, SBA has historically been treated in the same way as CRC. However, recent genetic and molecular data suggest a distinct profile from other gastrointestinal malignancies and support a more nuanced approach to its management. Herein, we briefly review the state-of-the-art in the clinical management of early-stage and advanced disease and recent discoveries of potentially actionable genetic alterations or pathways along with the most promising ongoing clinical trials, which will hopefully revolutionize the treatment landscape of this orphan disease in the foreseeable future.
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Affiliation(s)
- Fabio Gelsomino
- Department of Oncology and Hematology, Division of Oncology, University Hospital of Modena, 41124 Modena, Italy
| | - Rita Balsano
- Medical Oncology Unit, University Hospital of Parma, 43126 Parma, Italy; (R.B.); (I.G.)
| | | | - Ingrid Garajová
- Medical Oncology Unit, University Hospital of Parma, 43126 Parma, Italy; (R.B.); (I.G.)
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Fleites O, Pelenyi SS, Lee CK, Wisnik CA, Tariq A, Abdel-Khalek A, Tiesenga FM. Persistent Small Bowel Obstruction due to Small Bowel Adenocarcinoma: A Case Report. Cureus 2021; 13:e20233. [PMID: 35004049 PMCID: PMC8734532 DOI: 10.7759/cureus.20233] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 12/06/2021] [Indexed: 11/05/2022] Open
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Peterson C, Plunkard J, Johanson A, Izzi J, Gabrielson K. Immunohistochemical Characterization of a Duodenal Adenocarcinoma with Pulmonary, Hepatic and Parapatellar Metastases in a Common Marmoset (Callithrixjacchus). J Comp Pathol 2021; 189:1-7. [PMID: 34886977 PMCID: PMC8669625 DOI: 10.1016/j.jcpa.2021.09.002] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/27/2021] [Revised: 08/09/2021] [Accepted: 09/20/2021] [Indexed: 11/30/2022]
Abstract
An 11-year-old male common marmoset (Callithrix jacchus) presented with chronic, progressive weight loss and diarrhoea. Response to treatment with nutritional supplementation, antibiotics and immunosuppressants was modest and transient, and the animal was humanely euthanized. At necropsy, the proximal 8 cm of small intestine was diffusely pale with transmural thickening. The lungs contained coalescing tan, firm nodules measuring up to 4 mm in diameter. Histological examination revealed infiltrative mucinous adenocarcinoma of the duodenum with extensive metastases to the lungs, liver and left parapatellar adipose tissue. The mucinous matrix secreted by the primary and metastatic lesions was strongly periodic acid-Schiff positive. Warthin Starry staining for spirochaetes was negative. Pancytokeratin expression was attenuated in the primary tumour as well as in the metastases, which correlated to a poorly differentiated phenotype. To the authors' knowledge, this is the first report of a proximal duodenal adenocarcinoma with extensive metastatic disease in a common marmoset.
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Affiliation(s)
- Cornelia Peterson
- Department of Molecular and Comparative Pathobiology, Johns Hopkins University, Baltimore, Maryland, USA.
| | - Jessica Plunkard
- Department of Molecular and Comparative Pathobiology, Johns Hopkins University, Baltimore, Maryland, USA
| | - Andrew Johanson
- Department of Molecular and Comparative Pathobiology, Johns Hopkins University, Baltimore, Maryland, USA
| | - Jessica Izzi
- Department of Molecular and Comparative Pathobiology, Johns Hopkins University, Baltimore, Maryland, USA
| | - Kathleen Gabrielson
- Department of Molecular and Comparative Pathobiology, Johns Hopkins University, Baltimore, Maryland, USA
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Challenges in Crohn's Disease Management after Gastrointestinal Cancer Diagnosis. Cancers (Basel) 2021; 13:cancers13030574. [PMID: 33540674 PMCID: PMC7867285 DOI: 10.3390/cancers13030574] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/30/2020] [Revised: 01/25/2021] [Accepted: 01/29/2021] [Indexed: 12/14/2022] Open
Abstract
Simple Summary Crohn’s disease (CD) is a chronic inflammatory bowel disease affecting both young and elderly patients, involving the entire gastrointestinal tract from the mouth to anus. The chronic transmural inflammation can lead to several complications, among which gastrointestinal cancers represent one of the most life-threatening, with a higher risk of onset as compared to the general population. Moreover, diagnostic and therapeutic strategies in this subset of patients still represent a significant challenge for physicians. Thus, the aim of this review is to provide a comprehensive overview of the current evidence for an adequate diagnostic pathway and medical and surgical management of CD patients after gastrointestinal cancer onset. Abstract Crohn’s disease (CD) is a chronic inflammatory bowel disease with a progressive course, potentially affecting the entire gastrointestinal tract from mouth to anus. Several studies have shown an increased risk of both intestinal and extra-intestinal cancer in patients with CD, due to long-standing transmural inflammation and damage accumulation. The similarity of symptoms among CD, its related complications and the de novo onset of gastrointestinal cancer raises difficulties in the differential diagnosis. In addition, once a cancer diagnosis in CD patients is made, selecting the appropriate treatment can be particularly challenging. Indeed, both surgical and oncological treatments are not always the same as that of the general population, due to the inflammatory context of the gastrointestinal tract and the potential exacerbation of gastrointestinal symptoms of patients with CD; moreover, the overlap of the neoplastic disease could lead to adjustments in the pharmacological treatment of the underlying CD, especially with regard to immunosuppressive drugs. For these reasons, a case-by-case analysis in a multidisciplinary approach is often appropriate for the best diagnostic and therapeutic evaluation of patients with CD after gastrointestinal cancer onset.
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Abstract
Small bowel tumors are rare and account for approximately 3% of all gastrointestinal tumors in the United States. The incidence of small bowel neoplasms is rising-in particular, there is a rising incidence of small bowel carcinoid tumors. This may in part be due to small bowel lesion identification in an increasing number of cross-sectional imaging studies performed for other indications as well as increased use of advanced imaging techniques to assess specifically for small bowel disease. Diagnosis is a challenge owing to nonspecific clinical manifestation, rare occurrence, and low index of clinical suspicion. Yet, various small bowel neoplasms have characteristic imaging features at CT and MRI when optimal distention of the small bowel is achieved, correlating well with features seen in gross specimens. Understanding the imaging features of small bowel neoplasms is important to improve the radiologist's ability to diagnose and characterize small bowel neoplasms. Most small bowel tumors are clinically silent for long periods, and nearly half of the benign tumors are found incidentally during surgery or at cross-sectional imaging performed for other reasons. The authors review the imaging features of common benign and malignant small bowel neoplasms to aid the radiologist in diagnosis of small bowel tumors. ©RSNA, 2020.
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Affiliation(s)
- Rahul Jasti
- From the Department of Radiology, Virginia Commonwealth University Health System, 1250 E Marshall St, Richmond, VA 23298
| | - Laura R Carucci
- From the Department of Radiology, Virginia Commonwealth University Health System, 1250 E Marshall St, Richmond, VA 23298
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10
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Smith AA, Frimberger AE, Moore AS. Retrospective study of survival time and prognostic factors for dogs with small intestinal adenocarcinoma treated by tumor excision with or without adjuvant chemotherapy. J Am Vet Med Assoc 2020; 254:243-250. [PMID: 30605388 DOI: 10.2460/javma.254.2.243] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/20/2022]
Abstract
OBJECTIVE To estimate survival time for dogs with small intestinal adenocarcinoma (SIACA) following tumor excision with or without adjuvant chemotherapy and to identify factors associated with survival time. DESIGN Retrospective case series with a nested cohort study. ANIMALS 29 client-owned dogs with surgically resected, histologically diagnosed SIACA. PROCEDURES Medical records were reviewed and data collected regarding dog signalment; clinical signs; physical examination findings; PCV; serum total solids concentration; diagnostic imaging results; tumor size, location, and histologic characteristics (serosal extension, lymphatic invasion, surgical margins, and lymph node metastasis); type of adjuvant chemotherapy; NSAID administration; and survival time. Variables were assessed for associations with survival time and hazard rate via Kaplan-Meier and Cox proportional hazards analyses. RESULTS Overall median survival time for dogs with SIACA following tumor excision was 544 days (95% confidence interval, 369 to 719 days). Based on Kaplan-Meier estimates, the 1- and 2-year survival rates were 60% and 36%, respectively. On multivariate analysis, only age category was an independent predictor of survival over the follow-up period. Dogs < 8 years of age had a significantly longer median survival time (1,193 days) than dogs ≥ 8 years (488 days). Lymph node metastasis, adjuvant chemotherapy, NSAID administration, and other assessed variables were not associated with survival time. CONCLUSIONS AND CLINICAL RELEVANCE Findings suggested that SIACA in dogs carries a fair prognosis following excision, even when lymph node metastasis is present. Prospective studies are warranted to better characterize the effects of adjuvant chemotherapy or NSAID administration on survival time.
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Agrawal CR, Sharma M, Talwar V, Goel V, Singh AP. Isolated Splenic Recurrence of Treated Jejunal Cancer: A Case Report with Brief Review of Literature. Indian J Med Paediatr Oncol 2019. [DOI: 10.4103/ijmpo.ijmpo_242_17] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/04/2022] Open
Abstract
AbstractSpleen is a very infrequent site of metastasis despite the fact that it is the most vascular organ in the body. Even if systemic metastasis occurs, splenic metastasis largely remained asymptomatic due to its large functional reserve, and most often, the metastasis is detected incidentally on follow-up scan. Many cases of asymptomatic splenic metastasis detected on follow-up scans are reported in literature from different primary sites. Herein, we report a similar case of a 33-year-old male who presented with isolated splenic recurrence after a long treatment-free interval following curative treatment of adenocarcinoma jejunum. As stated, there are few previous reports of isolated splenic metastasis from carcinoma colon, but considering small intestinal adenocarcinoma, this is the first unique case reported to the best of our knowledge. Due to rarity of clinical scenario and lack of established guidelines, the treatment of such cases varies from patient to patient, and the knowledge, whatever we owe, is on the basis of case reports only.
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Affiliation(s)
| | - Manish Sharma
- Department of Medical Oncology, Rajiv Gandhi Cancer Institute and Research Centre, New Delhi, India
| | - Vineet Talwar
- Rajiv Gandhi Cancer Institute and Research Centre, New Delhi, India
| | - Varun Goel
- Rajiv Gandhi Cancer Institute and Research Centre, New Delhi, India
| | - Ajit Pratap Singh
- Department of Pathology, Rajiv Gandhi Cancer Institute and Research Centre, New Delhi, India
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Huffman BM, Jin Z, Yadav S, Patel S, Nagorney DM, Truty MJ, McWilliams RR, Halfdanarson TR, Mahipal A. Novel Prognostic Factors in Resected Small Bowel Adenocarcinoma. Clin Colorectal Cancer 2019; 18:218-225. [PMID: 31178274 DOI: 10.1016/j.clcc.2019.05.002] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/17/2019] [Accepted: 05/08/2019] [Indexed: 12/18/2022]
Abstract
BACKGROUND Small bowel adenocarcinoma (SBA) is a rare malignancy affecting approximately 3000 patients per year in the United States, and there is limited evidence prognosticating patients with resected SBA. We aimed to evaluate prognostic factors and the role of adjuvant therapy in patients with resected SBA. PATIENTS AND METHODS Two hundred forty-one patients who had resected stage I-III SBA were retrospectively identified at a single tertiary referral institution. Overall survival (OS) analysis was performed by the Kaplan-Meier method, and Wilcoxon tests were used for statistical comparisons. Cox proportional hazards were performed to identify significant variables by univariate and multivariate analysis. RESULTS Median OS for the entire group was 54.5 months (95% confidence interval [CI], 37.2-81.2 months), with 5- and 10-year OS of 48% and 35%. Median follow-up was 113.7 months (95% CI, 97.9-126.6 months). For patients with stage III disease who received adjuvant therapy, the median OS was 33.8 months (95% CI, 27.8-78.8) compared to 24.7 months (95% CI, 11.5-37.8) for patients with no adjuvant therapy (P < .01). Male sex, advanced T stage, advanced N stage, increased positive lymph node ratio, lymphocyte-to-monocyte ratio < 1.56, presence of residual disease, and earlier date of diagnosis predicted worse survival on univariate analysis. Age > 60 years, lymphocyte-to-monocyte ratio < 1.56, and advanced T stage were identified as independent negative predictors of OS for all patients by multivariate analysis. CONCLUSION Advanced age, advanced T stage, and lymphocyte-to-monocyte ratio < 1.56 independently predicted survival in resected SBA. Adjuvant therapy is associated with improved survival in patients with resected stage III SBA.
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Affiliation(s)
| | - Zhaohui Jin
- Division of Medical Oncology, Mayo Clinic, Rochester, MN
| | | | - Shruti Patel
- Department of Internal Medicine, Mayo Clinic, Rochester, MN
| | - David M Nagorney
- Division of Hepatobiliary and Pancreas Surgery, Mayo Clinic, Rochester, MN
| | - Mark J Truty
- Division of Hepatobiliary and Pancreas Surgery, Mayo Clinic, Rochester, MN
| | | | | | - Amit Mahipal
- Division of Medical Oncology, Mayo Clinic, Rochester, MN.
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Wieghard N, Mongoue-Tchokote S, Young JI, Sheppard BC, Tsikitis VL. Prognosis of small bowel adenocarcinoma in Crohn's disease compares favourably with de novo small bowel adenocarcinoma. Colorectal Dis 2017; 19:446-455. [PMID: 27659145 DOI: 10.1111/codi.13531] [Citation(s) in RCA: 15] [Impact Index Per Article: 1.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/25/2016] [Accepted: 08/17/2016] [Indexed: 02/07/2023]
Abstract
AIM Limited data exist on Crohn's disease (CD)-associated small bowel adenocarcinoma (SBA). A large-scale retrospective cohort study was conducted comparing the clinical features and outcome of CD-associated SBA and de novo SBA. METHOD Data for patients with small bowel adenocarcinoma were gathered from the 1992-2010 United States Surveillance, Epidemiology and End Results cancer registry-Medicare linked database. We identified 2123 patients, of whom 179 had CD-associated and 1944 de novo SBA. The main outcome measures were overall survival (OS) and cancer-specific survival (CSS). RESULTS CD-associated SBA was most commonly located in the ileum (62% vs 31%, P < 0.0001). CD patients were diagnosed at an earlier stage (I/II), compared with de novo SBA (55% vs 32%, P < 0.0001), and were more likely to undergo surgery (81% vs 72%, P = 0.0016). Chemotherapy use was similar (25% vs 21%, P = 0.1886). Patients with CD-associated SBA had better 5-year OS (43% vs 34%, P = 0.0121) but a similar CSS (65% vs 64%, P = 0.77). There was no difference in the OS between the cohorts when stratified by stage. On multivariate analysis, CD was not significantly related to OS [hazard ratio (HR) 0.97, 95% CI: 0.79-1.20, P = 0.7889]. Surgery and the extent of lymphadenectomy improved OS for all SBA patients (HR 0.73, 95% CI: 0.60-0.88, P = 0.001), whereas chemotherapy did not (HR 1.13, 95% CI: 0.99-1.28, P = 0.0665). CONCLUSION Patients with CD-associated SBA present at an earlier stage than patients with de novo SBA, they receive more surgery but similar rates of chemotherapy, and have similar OS and CSS. The presence of CD does not worsen survival after treatment of SBA.
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Affiliation(s)
- N Wieghard
- Department of Surgery, Oregon Health and Science University, Portland, Oregon, USA
| | - S Mongoue-Tchokote
- Knight Cancer Institute, Oregon Health and Science University - Biostatistics Shared Resource, Portland, Oregon, USA
| | - J I Young
- Department of Surgery, Oregon Health and Science University, Portland, Oregon, USA
| | - B C Sheppard
- Department of Surgery, Oregon Health and Science University, Portland, Oregon, USA
| | - V L Tsikitis
- Department of Surgery, Oregon Health and Science University, Portland, Oregon, USA
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Abstract
The small intestine is a relatively privileged organ that only rarely develops malignant or even benign tumors. Given this rarity, the relative inaccessibility of the organ during routine endoscopic procedures, and the typical absence or nonspecific nature of clinical manifestations, these tumors often go undiagnosed. Treatment and prognosis are tailored to each histological subtype of tumor. This chapter will discuss the epidemiology, presentation, diagnostics, and management for the most common small bowel tumors, and will highlight the importance of recognizing patients at higher risk of small bowel neoplasia.
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Affiliation(s)
- Kamron Pourmand
- The Dr. Henry D. Janowitz Division of Gastroenterology, Icahn School of Medicine at Mount Sinai, One Gustave Levy Place, GI Division, Box 1069, New York City, NY, 10029, USA
| | - Steven H Itzkowitz
- The Dr. Henry D. Janowitz Division of Gastroenterology, Icahn School of Medicine at Mount Sinai, One Gustave Levy Place, GI Division, Box 1069, New York City, NY, 10029, USA.
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Alvi MA, McArt DG, Kelly P, Fuchs MA, Alderdice M, McCabe CM, Bingham V, McGready C, Tripathi S, Emmert-Streib F, Loughrey MB, McQuaid S, Maxwell P, Hamilton PW, Turkington R, James JA, Wilson RH, Salto-Tellez M. Comprehensive molecular pathology analysis of small bowel adenocarcinoma reveals novel targets with potential for clinical utility. Oncotarget 2016; 6:20863-74. [PMID: 26315110 PMCID: PMC4673235 DOI: 10.18632/oncotarget.4576] [Citation(s) in RCA: 37] [Impact Index Per Article: 4.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/09/2015] [Accepted: 07/20/2015] [Indexed: 01/22/2023] Open
Abstract
Small bowel accounts for only 0.5% of cancer cases in the US but incidence rates have been rising at 2.4% per year over the past decade. One-third of these are adenocarcinomas but little is known about their molecular pathology and no molecular markers are available for clinical use. Using a retrospective 28 patient matched normal-tumor cohort, next-generation sequencing, gene expression arrays and CpG methylation arrays were used for molecular profiling. Next-generation sequencing identified novel mutations in IDH1, CDH1, KIT, FGFR2, FLT3, NPM1, PTEN, MET, AKT1, RET, NOTCH1 and ERBB4. Array data revealed 17% of CpGs and 5% of RNA transcripts assayed to be differentially methylated and expressed respectively (p < 0.01). Merging gene expression and DNA methylation data revealed CHN2 as consistently hypermethylated and downregulated in this disease (Spearman −0.71, p < 0.001). Mutations in TP53 which were found in more than half of the cohort (15/28) and Kazald1 hypomethylation were both were indicative of poor survival (p = 0.03, HR = 3.2 and p = 0.01, HR = 4.9 respectively). By integrating high-throughput mutational, gene expression and DNA methylation data, this study reveals for the first time the distinct molecular profile of small bowel adenocarcinoma and highlights potential clinically exploitable markers.
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Affiliation(s)
- Muhammad A Alvi
- Northern Ireland Molecular Pathology Laboratory, Centre for Cancer Research and Cell Biology, Queen's University Belfast, Belfast, Northern Ireland, UK
| | - Darragh G McArt
- Northern Ireland Molecular Pathology Laboratory, Centre for Cancer Research and Cell Biology, Queen's University Belfast, Belfast, Northern Ireland, UK
| | - Paul Kelly
- Tissue Pathology, Belfast Health and Social Care Trust, Belfast, Northern Ireland, UK
| | - Marc-Aurel Fuchs
- Northern Ireland Molecular Pathology Laboratory, Centre for Cancer Research and Cell Biology, Queen's University Belfast, Belfast, Northern Ireland, UK
| | - Matthew Alderdice
- Northern Ireland Molecular Pathology Laboratory, Centre for Cancer Research and Cell Biology, Queen's University Belfast, Belfast, Northern Ireland, UK
| | - Clare M McCabe
- Northern Ireland Molecular Pathology Laboratory, Centre for Cancer Research and Cell Biology, Queen's University Belfast, Belfast, Northern Ireland, UK
| | - Victoria Bingham
- Northern Ireland Molecular Pathology Laboratory, Centre for Cancer Research and Cell Biology, Queen's University Belfast, Belfast, Northern Ireland, UK
| | - Claire McGready
- Northern Ireland Molecular Pathology Laboratory, Centre for Cancer Research and Cell Biology, Queen's University Belfast, Belfast, Northern Ireland, UK
| | - Shailesh Tripathi
- Computational Biology and Machine Learning Laboratory, Centre for Cancer Research and Cell Biology, Queen's University Belfast, Belfast, Northern Ireland, UK
| | - Frank Emmert-Streib
- Computational Biology and Machine Learning Laboratory, Centre for Cancer Research and Cell Biology, Queen's University Belfast, Belfast, Northern Ireland, UK
| | - Maurice B Loughrey
- Tissue Pathology, Belfast Health and Social Care Trust, Belfast, Northern Ireland, UK
| | - Stephen McQuaid
- Northern Ireland Molecular Pathology Laboratory, Centre for Cancer Research and Cell Biology, Queen's University Belfast, Belfast, Northern Ireland, UK.,Tissue Pathology, Belfast Health and Social Care Trust, Belfast, Northern Ireland, UK
| | - Perry Maxwell
- Northern Ireland Molecular Pathology Laboratory, Centre for Cancer Research and Cell Biology, Queen's University Belfast, Belfast, Northern Ireland, UK.,Tissue Pathology, Belfast Health and Social Care Trust, Belfast, Northern Ireland, UK
| | - Peter W Hamilton
- Northern Ireland Molecular Pathology Laboratory, Centre for Cancer Research and Cell Biology, Queen's University Belfast, Belfast, Northern Ireland, UK
| | - Richard Turkington
- Centre for Cancer Research and Cell Biology, Queen's University Belfast, Belfast, Northern Ireland, UK
| | - Jacqueline A James
- Northern Ireland Molecular Pathology Laboratory, Centre for Cancer Research and Cell Biology, Queen's University Belfast, Belfast, Northern Ireland, UK.,Tissue Pathology, Belfast Health and Social Care Trust, Belfast, Northern Ireland, UK
| | - Richard H Wilson
- Centre for Cancer Research and Cell Biology, Queen's University Belfast, Belfast, Northern Ireland, UK
| | - Manuel Salto-Tellez
- Northern Ireland Molecular Pathology Laboratory, Centre for Cancer Research and Cell Biology, Queen's University Belfast, Belfast, Northern Ireland, UK.,Tissue Pathology, Belfast Health and Social Care Trust, Belfast, Northern Ireland, UK
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16
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Negoi I, Paun S, Hostiuc S, Stoica B, Tanase I, Negoi RI, Beuran M. Most small bowel cancers are revealed by a complication. ACTA ACUST UNITED AC 2015; 13:500-5. [PMID: 26676271 PMCID: PMC4878621 DOI: 10.1590/s1679-45082015ao3380] [Citation(s) in RCA: 23] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/28/2015] [Accepted: 08/19/2015] [Indexed: 12/25/2022]
Abstract
Objective To characterize the pattern of primary small bowel cancers in a tertiary East-European hospital. Methods A retrospective study of patients with small bowel cancers admitted to a tertiary emergency center, over the past 15 years. Results There were 57 patients with small bowel cancer, representing 0.039% of admissions and 0.059% of laparotomies. There were 37 (64.9%) men, mean age of 58 years; and 72 years for females. Out of 57 patients, 48 (84.2%) were admitted due to an emergency situation: obstruction in 21 (38.9%), perforation in 17 (31.5%), upper gastrointestinal bleeding in 8 (14.8%), and lower gastrointestinal bleeding in 2 (3.7%). There were 10 (17.5%) duodenal tumors, 21 (36.8%) jejunal tumors and 26 (45.6%) ileal tumors. The most frequent neoplasms were gastrointestinal stromal tumor in 24 patients (42.1%), adenocarcinoma in 19 (33.3%), lymphoma in 8 (14%), and carcinoids in 2 (3.5%). The prevalence of duodenal adenocarcinoma was 14.55 times greater than that of the small bowel, and the prevalence of duodenal stromal tumors was 1.818 time greater than that of the small bowel. Obstruction was the complication in adenocarcinoma in 57.9% of cases, and perforation was the major local complication (47.8%) in stromal tumors. Conclusion Primary small bowel cancers are usually diagnosed at advanced stages, and revealed by a local complication of the tumor. Their surgical management in emergency setting is associated to significant morbidity and mortality rates.
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Affiliation(s)
- Ionut Negoi
- Carol Davila University of Medicine and Pharmacy Bucharest, Bucharest, Romania
| | - Sorin Paun
- Carol Davila University of Medicine and Pharmacy Bucharest, Bucharest, Romania
| | - Sorin Hostiuc
- Carol Davila University of Medicine and Pharmacy Bucharest, Bucharest, Romania
| | | | - Ioan Tanase
- Emergency Hospital of Bucharest, Bucharest, Romania
| | | | - Mircea Beuran
- Carol Davila University of Medicine and Pharmacy Bucharest, Bucharest, Romania
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17
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Li JW, Tan MTK, Ang TL, Teo EK. Chemoprevention trials of GI cancers in Asia. Best Pract Res Clin Gastroenterol 2015; 29:967-78. [PMID: 26651257 DOI: 10.1016/j.bpg.2015.09.014] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/01/2015] [Accepted: 09/02/2015] [Indexed: 01/31/2023]
Abstract
Cancers are among the leading causes of morbidity and mortality worldwide, with gastrointestinal (GI) luminal cancers accounting for a large proportion of cancer incidence. Chemoprevention of GI luminal cancers is important to reduce this cancer burden. Screening for and eradication of Helicobacter pylori (H. pylori) in areas with high gastric cancer incidence is a safe and effective strategy to decrease the incidence of gastric cancer. Eradication of H. pylori also seems to be effective in preventing metachronous cancer development after endoscopic resection of early gastric cancer. For the average risk individual, COX inhibitors may have a role in reducing the incidence of adenoma formation as well as colorectal cancer, bearing in mind its inherent risks. There is currently insufficient evidence to recommend a chemoprevention agent against esophageal and small intestine cancer.
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Affiliation(s)
- James Weiquan Li
- Department of Gastroenterology and Hepatology, Changi General Hospital, Singapore.
| | | | - Tiing Leong Ang
- Department of Gastroenterology and Hepatology, Changi General Hospital, Singapore.
| | - Eng Kiong Teo
- Department of Gastroenterology and Hepatology, Changi General Hospital, Singapore.
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18
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Chae MJ, Baek IH, Oh YM, Lim JU, Jeon JW, Shin HP, Joo KR, Lee JI. A Patient with Duodenal Mucinous Adenocarcinoma Presenting as a Laterally Spreading Tumor. Clin Endosc 2015; 48:336-9. [PMID: 26240810 PMCID: PMC4522428 DOI: 10.5946/ce.2015.48.4.336] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/19/2014] [Revised: 10/14/2014] [Accepted: 10/21/2014] [Indexed: 01/13/2023] Open
Abstract
Primary duodenal carcinoma is rare. Duodenal mucinous adenocarcinoma (DMA) is even rarer, and its associated manifestations and typical endoscopic or imaging findings are not well characterized. Herein, we report a case of primary DMA in an asymptomatic 58-year-old man who visited our hospital for a regular health screening. Upper endoscopy revealed an approximately 4-cm lesion in the second portion of the duodenum, but the mass was not visualized on computed tomography. Biopsies revealed a tubular adenoma that was subsequently resected. Frozen biopsies demonstrated DMA with a background of low-grade tubular adenoma for which we performed Roux-en-Y duodenojejunostomy and jejunojejunostomy. To our knowledge, this is the first report of a patient with DMA in Korea.
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Affiliation(s)
- Myung Joon Chae
- Department of Gastroenterology, Kyung Hee University Hospital at Gangdong, Kyung Hee University School of Medicine, Seoul, Korea
| | - Il Hyun Baek
- Department of Gastroenterology, Kyung Hee University Hospital at Gangdong, Kyung Hee University School of Medicine, Seoul, Korea
| | - Yu Mi Oh
- Department of Gastroenterology, Kyung Hee University Hospital at Gangdong, Kyung Hee University School of Medicine, Seoul, Korea
| | - Jun Uk Lim
- Department of Gastroenterology, Kyung Hee University Hospital at Gangdong, Kyung Hee University School of Medicine, Seoul, Korea
| | - Jung Won Jeon
- Department of Gastroenterology, Kyung Hee University Hospital at Gangdong, Kyung Hee University School of Medicine, Seoul, Korea
| | - Hyun Phil Shin
- Department of Gastroenterology, Kyung Hee University Hospital at Gangdong, Kyung Hee University School of Medicine, Seoul, Korea
| | - Kwang Ro Joo
- Department of Gastroenterology, Kyung Hee University Hospital at Gangdong, Kyung Hee University School of Medicine, Seoul, Korea
| | - Joung Il Lee
- Department of Gastroenterology, Kyung Hee University Hospital at Gangdong, Kyung Hee University School of Medicine, Seoul, Korea
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19
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Shenoy S. Primary small-bowel malignancy: update in tumor biology, markers, and management strategies. J Gastrointest Cancer 2014; 45:421-430. [PMID: 25339426 DOI: 10.1007/s12029-014-9658-z] [Citation(s) in RCA: 15] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
Abstract
PURPOSE Primary small-bowel malignancies (SBM) are rare tumors but their incidence is rising. An estimated 9160 new cases and 1210 deaths due to SBM may occur in the USA in 2014. We review advances made in tumor biology, immunohistochemistry, and discuss treatment strategies for these malignancies. METHODS Relevant articles from PubMed/Medline and Embase searches were collected using the phrases "small-bowel adenocarcinoma, gastrointestinal carcinoids, gastrointestinal stromal tumors, small-bowel leiomyosarcoma, and small-bowel lymphoma". RESULTS Advances in imaging techniques such as wireless capsule endoscopy, CT and MRI enterography, and endoscopy (balloon enteroscopy) along with discovery of molecular markers such as c-kit and PDGFRA for GIST tumors have improved our ability to diagnose, localize, and treat these patients. Early detection and surgical resection offers the best chance for long-term survival in all tumors except bowel lymphoma where chemotherapy plays the main role. Adjuvant therapy with imatinib has improved overall survival for GIST tumors, somatostatin analogs have improved symptoms and also inhibited tumor growth and stabilized metastatic disease in carcinoid disease, but chemotherapy has not improved survival for adenocarcinoma. CONCLUSIONS Recent advances in molecular characterization holds promise in novel targeted therapies. Currently ongoing trials are exploring efficacy of targeted therapies and role of adjuvant therapy for adenocarcinoma and results are awaited. Early detection and aggressive surgical therapy for all localized tumors and lymph node sampling particularly for adenocarcinoma remains the main treatment modality.
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Affiliation(s)
- Santosh Shenoy
- Department of Surgery, KCVA, 4801 E Linwood Blvd, Kansas City, MO, 64128, USA,
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20
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Suh CH, Tirumani SH, Shinagare AB, Kim KW, Rosenthal MH, Ramaiya NH, Baheti AD. Diagnosis and management of duodenal adenocarcinomas: a comprehensive review for the radiologist. ACTA ACUST UNITED AC 2014; 40:1110-20. [DOI: 10.1007/s00261-014-0309-4] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/13/2023]
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21
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Reynolds I, Healy P, Mcnamara DA. Malignant tumours of the small intestine. Surgeon 2014; 12:263-70. [PMID: 24637026 DOI: 10.1016/j.surge.2014.02.003] [Citation(s) in RCA: 38] [Impact Index Per Article: 3.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/06/2013] [Accepted: 02/16/2014] [Indexed: 02/06/2023]
Abstract
Adenocarcinoma, neuroendocrine tumours, sarcomas and lymphomas are the four most common malignant tumours arising in the small intestine, although over forty different histological subtypes are described. Collectively these account for only 2% of cancers of the digestive system. The incidence of small bowel cancer has increased in recent decades with a four-fold increase in carcinoid tumours. Risk factors for small bowel tumours include coeliac disease, inflammatory bowel disease and a number of genetic abnormalities. The non-specific nature of their symptoms and the difficulty in visualising these tumours with normal endoscopic techniques often results in late diagnosis. Furthermore the paucity of literature on this topic has made it difficult to standardise management. There has however been marked improvement in imaging methods resulting in earlier diagnosis in many cases. As expected, early detection of localised, well differentiated tumours followed by surgical resection with negative margins offers the best chance of long term survival. Better adjuvant treatment, notably for gastrointestinal stromal tumours, has improved 5-year survival rates significantly. Development of surveillance guidelines for at risk populations may be a valuable way of improving early diagnosis of this challenging group of conditions.
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Affiliation(s)
- Ian Reynolds
- Department of Colorectal Surgery, Beaumont Hospital, Dublin, Ireland
| | - Paul Healy
- Department of Colorectal Surgery, Beaumont Hospital, Dublin, Ireland
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22
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Egan L, D'Inca R, Jess T, Pellino G, Carbonnel F, Bokemeyer B, Harbord M, Nunes P, Van der Woude J, Selvaggi F, Triantafillidis J. Non-colorectal intestinal tract carcinomas in inflammatory bowel disease: results of the 3rd ECCO Pathogenesis Scientific Workshop (II). J Crohns Colitis 2014; 8:19-30. [PMID: 23664498 DOI: 10.1016/j.crohns.2013.04.009] [Citation(s) in RCA: 54] [Impact Index Per Article: 4.9] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/02/2013] [Accepted: 04/05/2013] [Indexed: 02/08/2023]
Abstract
Patients with inflammatory bowel diseases (IBD) have an excess risk of certain gastrointestinal cancers. Much work has focused on colon cancer in IBD patients, but comparatively less is known about other more rare cancers. The European Crohn's and Colitis Organization established a pathogenesis workshop to review what is known about these cancers and formulate proposals for future studies to address the most important knowledge gaps. This article reviews the current state of knowledge about small bowel adenocarcinoma, ileo-anal pouch and rectal cuff cancer, and anal/perianal fistula cancers in IBD patients.
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Affiliation(s)
- Laurence Egan
- Discipline of Pharmacology & Therapeutics, NUI, Galway, Ireland.
| | - Renata D'Inca
- Department of Surgical, Oncological and Gastroenterological Sciences, University of Padua, Italy
| | - Tine Jess
- Department of Epidemiology Research, Statens Serum Institut, National Health Surveillance & Research, Denmark
| | | | - Franck Carbonnel
- Service de Gastroentérologie, CHU de Bicêtre, Assistance Publique-Hôpitaux de Paris, Université Paris Sud, France
| | - Bernd Bokemeyer
- Department of General Internal Medicine I, Christian-Albrechts-University, University Hospital Schleswig-Holstein, Kiel, Germany
| | - Marcus Harbord
- Chelsea & Westminster Hospital, 369 Fulham Road, London, UK
| | - Paula Nunes
- Lisbon University & Higher School of Health Technology of Lisbon, Lisbon, Portugal; Hospital Cuf Descobertas, Lisbon, Portugal
| | | | | | - John Triantafillidis
- Department of Gastroenterology and Center for IBD "Saint Panteleimon" Hospital, Nicea, Greece
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23
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Basu N, Skinner HG, Litzelman K, Vanderboom R, Baichoo E, Boardman LA. Telomeres and telomere dynamics: relevance to cancers of the GI tract. Expert Rev Gastroenterol Hepatol 2013; 7:733-48. [PMID: 24161135 PMCID: PMC3892561 DOI: 10.1586/17474124.2013.848790] [Citation(s) in RCA: 23] [Impact Index Per Article: 1.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/17/2022]
Abstract
Aberrations in telomere length and telomere maintenance contribute to cancer development. In this article, we review the basic principles of telomere length in normal and tumor tissue and the presence of the two main telomere maintenance pathways as they pertain to gastrointestinal tract cancer. Peripheral blood telomeres are shorter in patients with many types of gastrointestinal tract cancers. Telomere length in tumor DNA also appears to shorten early in cancer development. Tumor telomere shortening is often accompanied by telomerase activation to protect genetically damaged DNA from normal cell senescence or apoptosis, allowing immortalized but damaged DNA to persist. Alternative lengthening of telomeres is another mechanism used by cancer to maintain telomere length in cancer cells. Telomerase and alternative lengthening of telomeres activators and inhibitors may become important chemopreventive or chemotherapeutic agents as our understanding of telomere biology, specific telomere-related phenotypes and its relationship to carcinogenesis increases.
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Affiliation(s)
- Nivedita Basu
- Division of Gastroenterology and Hepatology Department of Medicine Mayo Clinic 200 First Street SW Rochester, MN 55905 Tel: 507-266-4338; Fax: 507-266-0350
| | - Halcyon G. Skinner
- Department of Population Health Sciences School of Medicine and Public Health University of Wisconsin Madison, WI 53726 Tel: 608-265-4654
| | - Kristin Litzelman
- Department of Population Health Sciences School of Medicine and Public Health University of Wisconsin Madison, WI 53726 Tel: 608-265-4654
| | - Russell Vanderboom
- Division of Gastroenterology and Hepatology Department of Medicine Mayo Clinic 200 First Street SW Rochester, MN 55905 Tel: 507-266-4338; Fax: 507-266-0350
| | - Esha Baichoo
- Division of Gastroenterology and Hepatology Department of Medicine Mayo Clinic 200 First Street SW Rochester, MN 55905 Tel: 507-266-4338; Fax: 507-266-0350
| | - Lisa A. Boardman
- Division of Gastroenterology and Hepatology Department of Medicine Mayo Clinic 200 First Street SW Rochester, MN 55905 Tel: 507-266-4338; Fax: 507-266-0350
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24
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Abstract
Small bowel cancers account for 3% of all gastrointestinal malignancies and small bowel adenocarcinomas represent a third of all small bowel cancers. Rarity of small bowel adenocarcinomas restricts molecular understanding and presents unique diagnostic and therapeutic challenges. Better cross-sectional imaging techniques and development of enteroscopy and capsule endoscopy have facilitated earlier and more-accurate diagnosis. Surgical resection remains the mainstay of therapy for locoregional disease. In the metastatic setting, fluoropyrimidine and oxaliplatin-based chemotherapy has shown clinical benefit in prospective non-randomized trials. Although frequently grouped under the same therapeutic umbrella as large bowel adenocarcinomas, small bowel adenocarcinomas are distinct clinical and molecular entities. Recent progress in molecular characterization has aided our understanding of the pathogenesis of these tumours and holds potential for prospective development of novel targeted therapies. Multi-institutional collaborative efforts directed towards cogent understanding of tumour biology and designing sensible clinical trials are essential for developing improved therapeutic strategies. In this Review, we endeavour to outline an evidence-based approach to present-day management of small bowel adenocarcinoma, describe contemporary challenges and uncover evolving paradigms in the management of these rare 'orphan' neoplasias.
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Affiliation(s)
- Kanwal Raghav
- Department of Gastrointestinal Medical Oncology, The University of Texas MD Anderson Cancer Centre, Unit #426, 1515 Holcombe Boulevard, Houston, TX 77030, USA
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25
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Fu T, Guzzetta AA, Jeschke J, Vatapalli R, Dave P, Hooker CM, Morgan R, Iacobuzio-Donahue CA, Liu B, Ahuja N. KRAS G>A mutation favors poor tumor differentiation but may not be associated with prognosis in patients with curatively resected duodenal adenocarcinoma. Int J Cancer 2013; 132:2502-2509. [PMID: 23065691 PMCID: PMC3579006 DOI: 10.1002/ijc.27910] [Citation(s) in RCA: 13] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/09/2012] [Accepted: 09/10/2012] [Indexed: 12/24/2022]
Abstract
KRAS mutations have been found in duodenal adenocarcinomas and may have prognostic significance. The purpose of this study was to classify clinicopathological characteristics, microsatellite instability and KRAS mutations and identify possible prognostic role of KRAS mutations in duodenal adenocarcinomas. Demographics, tumor characteristics and survival were recorded for 78 patients with duodenal adenocarcinomas (Stages I-III). KRAS mutations were detected in 27 (34.6%) cases, of which the majority (74.1%) were G>A transitions. Multivariate logistic regression analysis showed that KRAS G>A mutation was significantly associated with late stage (p = 0.025) and poor tumor differentiation (p = 0.035), when compared with wild-type and other than G>A mutations. KRAS G>A mutation carriers were at increased risk for distant relapse (p = 0.022) and had significantly shorter overall survival (OS; log-rank p = 0.045) and a trend toward shorter relapse-free survival (RFS; log-rank p = 0.062) when compared with those who did not carry the KRAS G>A mutation. In multivariate analyses, there was a significant correlation between ≥ 3 positive lymph nodes and poor OS (p < 0.001) and RFS (p = 0.001) and KRAS G>A mutation carriers demonstrated no effect on clinical outcome. In conclusion, KRAS G>A mutation correlates significantly with late stage and poor tumor differentiation in duodenal adenocarcinoma. Among patients who undergo a curative resection of duodenal adenocarcinoma, KRAS G>A mutation carriers will more likely experience distant relapse but may not exhibit a poor prognosis. The number of positive lymph nodes should be incorporated in future staging systems.
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Affiliation(s)
- Tao Fu
- Department of Gastrointestinal Surgery, Daping Hospital, Third Military Medical University, Chongqing 400042, China
- Department of Surgery and Oncology, The Sydney Kimmel Comprehensive Cancer Center, The Johns Hopkins University School of Medicine, Baltimore, MD 21231, USA
| | - Angela A. Guzzetta
- Department of Surgery and Oncology, The Sydney Kimmel Comprehensive Cancer Center, The Johns Hopkins University School of Medicine, Baltimore, MD 21231, USA
| | - Jana Jeschke
- Department of Surgery and Oncology, The Sydney Kimmel Comprehensive Cancer Center, The Johns Hopkins University School of Medicine, Baltimore, MD 21231, USA
| | - Rajita Vatapalli
- Department of Surgery and Oncology, The Sydney Kimmel Comprehensive Cancer Center, The Johns Hopkins University School of Medicine, Baltimore, MD 21231, USA
| | - Pujan Dave
- Department of Surgery and Oncology, The Sydney Kimmel Comprehensive Cancer Center, The Johns Hopkins University School of Medicine, Baltimore, MD 21231, USA
| | - Craig M. Hooker
- Department of Surgery and Oncology, The Sydney Kimmel Comprehensive Cancer Center, The Johns Hopkins University School of Medicine, Baltimore, MD 21231, USA
| | - Richard Morgan
- Department of Pathology, The Sydney Kimmel Comprehensive Cancer Center, The Johns Hopkins University School of Medicine, Baltimore, MD 21231, USA
| | - Christine A. Iacobuzio-Donahue
- Department of Pathology, The Sydney Kimmel Comprehensive Cancer Center, The Johns Hopkins University School of Medicine, Baltimore, MD 21231, USA
| | - Baohua Liu
- Department of Gastrointestinal Surgery, Daping Hospital, Third Military Medical University, Chongqing 400042, China
| | - Nita Ahuja
- Department of Surgery and Oncology, The Sydney Kimmel Comprehensive Cancer Center, The Johns Hopkins University School of Medicine, Baltimore, MD 21231, USA
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26
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Sendt W, Wurst C, Settmacher U, Altendorf-Hofmann A. [Adenocarcinoma of small bowel. An underdiagnosed disease]. Chirurg 2012; 83:374-80. [PMID: 21901468 DOI: 10.1007/s00104-011-2159-8] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/08/2023]
Abstract
BACKGROUND Small bowel adenocarcinoma is a rare disease. The diagnosis is often disguised by nonspecific and varied presenting symptoms. Adenocarcinoma of the small bowel is typically detected at a late stage and with a poor prognosis. PATIENTS AND METHODS The records of 42 patients with small bowel adenocarcinomas diagnosed in 2 surgical clinics between 1995 and 2009 were reviewed for patient and tumor characteristics, treatment effects and survival. RESULTS The tumor locations were the duodenum (50%), jejunum (38%) and ileum (12%). In comparison to patients seen before 2004 the observed frequency of emergency operations or palliative procedures as well as stage distribution did not change. The median overall survival was 19 months with a 5-year overall survival of 20%. For patients with resections, reduced performance status, higher stage and residual disease after resection predicted decreased overall survival in univariate analysis. Residual disease and pT category were predictive of survival in multivariate analysis. CONCLUSION Complete resection provides the only means of a cure. In cases where curative resection cannot be performed the prognosis remains poor. Further study on the methods for early detection and effective adjuvant chemotherapy should be investigated, however, the available data are limited.
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Affiliation(s)
- W Sendt
- Klinik für Allgemein- und Viszeralchirurgie, Krankenhaus St. Joseph-Stift, Schwachhauser Heerstr. 54, 28209, Bremen, Deutschland.
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27
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Jejunal adenocarcinoma: an elusive diagnosis. Indian J Surg Oncol 2012; 2:197-201. [PMID: 22942611 DOI: 10.1007/s13193-011-0101-7] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/14/2011] [Accepted: 10/28/2011] [Indexed: 01/13/2023] Open
Abstract
Tubercular stricture of small bowel is a common cause of subacute intestinal obstruction in India. In a small subset of patients the cause of the intestinal obstruction could be adenocarcinoma of jejunum. It is difficult to diagnose preoperatively. The point of concern is compared to tubercular stricture, the treatment of jejunal adenocarcinoma is always surgical in the form of cancer directed surgery. The operating surgeon needs to have a very high suspicion for jejunal adenocarcinoma because the timely treatment offers the best survival in otherwise an aggressive disease with bleak prognosis.
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28
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Umman P, Adiyodi V, Narayan C. Small bowel adenocarcinoma - report of two cases and review of literature. Indian J Surg 2012; 75:123-7. [PMID: 24426406 DOI: 10.1007/s12262-012-0598-1] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/21/2012] [Accepted: 06/05/2012] [Indexed: 12/29/2022] Open
Abstract
Although accounting for 90 % of the intestinal surface area, small bowel adenocarcinomas are not common. The majority of these lesions are incidentally detected during laparotomy for intestinal obstruction or perforation. The symptoms associated with these lesions are not very specific and preoperative diagnosis is rare. We report two cases of jejunal adenocarcinomas detected in patients undergoing laparotomy for acute abdomen and review the literature for small bowel adenocarcinomas.
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Affiliation(s)
- Philip Umman
- Department of General Surgery, Govt. T D Medical College, Alappuzha, Kerala India
| | - Vineeth Adiyodi
- Department of General Surgery, Govt. T D Medical College, Alappuzha, Kerala India
| | - Chanchal Narayan
- Department of General Surgery, Govt. T D Medical College, Alappuzha, Kerala India
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29
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Inoue Y, Hayashi M, Satou N, Miyamoto Y, Hirokawa F, Asakuma M, Shimizu T, Kayano H, Yamamoto M, Yamana H, Okuda J, Egashira Y, Tanigawa N. Prognostic Clinicopathological Factors After Curative Resection of Small Bowel Adenocarcinoma. J Gastrointest Cancer 2011; 43:272-8. [PMID: 21607548 DOI: 10.1007/s12029-011-9290-0] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/13/2023]
Affiliation(s)
- Yoshihiro Inoue
- Department of General and Gastroenterological Surgery, Osaka Medical College Hospital, 2-7 Daigaku-machi, Takatsuki City, Osaka, 569-8686, Japan.
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30
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Overman MJ, Hu CY, Wolff RA, Chang GJ. Prognostic value of lymph node evaluation in small bowel adenocarcinoma: analysis of the surveillance, epidemiology, and end results database. Cancer 2010; 116:5374-82. [PMID: 20715162 DOI: 10.1002/cncr.25324] [Citation(s) in RCA: 85] [Impact Index Per Article: 5.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/10/2009] [Revised: 11/20/2009] [Accepted: 12/08/2009] [Indexed: 12/17/2022]
Abstract
BACKGROUND The presence of distant metastases and the completeness of resection are important prognostic factors in patients with small bowel adenocarcinoma (SBA); however, the influence of lymph node metastasis on patient outcome has not been well characterized. The objective of the current study was to evaluate the impact of the number of positive and negative lymph nodes on survival after curative resection. METHODS Patients who had SBA diagnosed between 1988 and 2005 were identified from the Surveillance, Epidemiology, and End Results (SEER) registry. Cox proportional hazards regression analyses were performed after adjusting for age, sex, race, tumor stage, tumor grade, and primary site. Five-year disease-specific survival (DSS) was determined, all patients were categorized according to the total lymph nodes (TLNs) assessed, and patients with stage III disease also were categorized according to the number of positive lymph nodes (PLNs) and the PLN-to-TLN ratio (the lymph node ratio [LNR]). RESULTS In total, 1991 patients (n=1216 with stage I/II SBA and n=775 with stage III SBA) were analyzed. Survival depended on the TLNs assessed. The 5-year DSS rate for patients with stage II disease was associated with the TLNs assessed (44%, 69%, and 83% for 0 TLNs, 1-7 TLNs, and>7 TLNs, respectively). The 5-year DSS for patients with stage III disease was associated with the number of PLNs (58% and 37% for <3 PLNs and ≥3 PLNs, respectively). Among patients with stage III disease, the LNR was even more predictive of survival than stratification by the number of PLNs. CONCLUSIONS Survival after surgical resection for stage I, II, and III SBA was associated with the TLNs assessed. Stratifying patients with stage III disease into those with <3 PLNs and ≥3 PLNs significantly improved prognostication.
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Affiliation(s)
- Michael J Overman
- Department of Gastrointestinal Medical Oncology, The University of Texas M. D. Anderson Cancer Center, Houston, Texas 77030, USA.
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Halfdanarson TR, McWilliams RR, Donohue JH, Quevedo JF. A single-institution experience with 491 cases of small bowel adenocarcinoma. Am J Surg 2010; 199:797-803. [PMID: 20609724 DOI: 10.1016/j.amjsurg.2009.05.037] [Citation(s) in RCA: 146] [Impact Index Per Article: 9.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/04/2009] [Revised: 04/27/2009] [Accepted: 05/04/2009] [Indexed: 02/08/2023]
Abstract
BACKGROUND The optimal treatment of small bowel adenocarcinoma is unknown. METHODS The records of 491 patients with small bowel adenocarcinoma diagnosis between 1970 and 2005 were reviewed for patient and tumor characteristics, treatment effects, and survival. RESULTS The median age at diagnosis was 62 years. The most common tumor locations were the duodenum (57%), jejunum (29%), and ileum (10%). The median overall survival was 20.1 months, with a 5-year overall survival of 26%. Greater age, male sex, higher stage and grade, residual disease after resection, and a lymph node ratio of 50% or greater predicted decreased overall survival in univariate analysis. Age and stage were predictive of survival in multivariate analysis. The overall survival with metastatic disease was poor. Adjuvant therapy was not associated with longer overall survival (P = .44). CONCLUSIONS The prognosis of patients with small bowel adenocarcinoma is poor. Complete resection provides the only means of cure, and the role for adjuvant therapy remains uncertain.
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Nicholl MB, Ahuja V, Conway WC, Vu VD, Sim MS, Singh G. Small bowel adenocarcinoma: understaged and undertreated? Ann Surg Oncol 2010; 17:2728-32. [PMID: 20458546 DOI: 10.1245/s10434-010-1109-x] [Citation(s) in RCA: 42] [Impact Index Per Article: 2.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/06/2009] [Indexed: 12/17/2022]
Abstract
BACKGROUND Primary small bowel adenocarcinoma (SBA) is a rare, chemoresistant tumor with an aggressive clinical nature. Surgery is the mainstay of therapy, but the extent of lymph node (LN) recovery necessary for optimal care of jejunoileal SBA is unknown. MATERIALS AND METHODS The SEER database was queried to identify patients whose primary jejunoileal SBA was diagnosed between 1995 and 2005. Patients were grouped by AJCC stage and number of LNs recovered from the surgical specimen. RESULTS Of 1444 patients with primary SBA, 93 (6.4%), 529 (36.6%), 356 (24.7%), and 466 (32.3%) were initially diagnosed with stage I, II, III, and IV disease, respectively. Five-year overall survival (OS) rate was 59.8%, 39.5%, 27.0%, and 3.2% for patients with stage I, II, III, and IV SBA, respectively. When ≥10 nodes were recovered, OS rate increased nonsignificantly in stage I (73.2% vs. 55.6%) and significantly in stage II (61.8% vs. 32.9%, P < .001) but was unchanged in stage III (27.4% vs. 27.3%, P = .13). Recovery of ≥10 nodes occurred in 26.9%, 23.6%, and 42.1% of patients with stage I, II, and III SBA, respectively. Multivariate analysis identified age, AJCC stage, site of primary tumor, recovery of ≥10 LNs, and number of positive nodes as significant for OS. CONCLUSIONS We have found SBA staging is largely inadequate. Our results suggest recovery of ≥10 LNs ensures accurate staging. Improvement in stage II SBA OS after adequate LN may reflect a high degree of understaging in this dataset rather than a therapeutic effect of LAD.
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Affiliation(s)
- Michael B Nicholl
- John Wayne Cancer Institute at Saint John's Health Center, Santa Monica, CA, USA
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Overman MJ, Kopetz S, Lin E, Abbruzzese JL, Wolff RA. Is there a role for adjuvant therapy in resected adenocarcinoma of the small intestine. Acta Oncol 2010; 49:474-9. [PMID: 20397775 DOI: 10.3109/02841860903490051] [Citation(s) in RCA: 63] [Impact Index Per Article: 4.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/06/2023]
Abstract
BACKGROUND The benefit of adjuvant therapy for resected small bowel adenocarcinoma has not been proven. We undertook a retrospective analysis to evaluate the benefit of adjuvant therapy in a clearly defined patient population with curatively resected small bowel adenocarcinoma. MATERIAL AND METHODS We identified 54 patients with small bowel adenocarcinoma who underwent margin-negative surgical resection and were evaluated after surgery at the University of Texas, M. D. Anderson Cancer Center between 1990 and 2008. Disease-free survival (DFS) and overall survival (OS) were estimated. RESULTS Median age was 55 years and primary tumor site was duodenum in 67%, jejunum in 20%, and ileum in 13%. Thirty patients (56%) received adjuvant therapy consisting of systemic chemotherapy with or without radiation in 28 and radiation alone in two. Patients who received adjuvant therapy had significantly higher tumor stage and rate of lymph node involvement. Five-year DFS and OS did not differ between treatment groups. In multivariate analysis, the use of adjuvant therapy was associated with improved DFS (HR 0.27; 95% CI 0.07-0.98, P = 0.05) but not OS (HR 0.47; 95% CI 0.13-1.62, P = 0.23). In patients with a high risk of relapse (defined as a lymph node ratio >or=10%), adjuvant therapy appeared to improve OS, P = 0.04, but not DFS, P = 0.15. DISCUSSION The use of adjuvant therapy for curatively resected small bowel adenocarcinoma was associated with an improvement in DFS. This finding strongly supports further investigation of adjuvant chemotherapy in this tumor type.
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Affiliation(s)
- Michael J Overman
- Department of Gastrointestinal Medical Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX 77030, USA.
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Merkel S, Weber K, Perrakis A, Göhl J, Hohenberger W. Tumoren des unteren Gastrointestinaltrakts. Chirurg 2010; 81:117-22; 124-26. [DOI: 10.1007/s00104-009-1814-9] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/17/2022]
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Retained capsule endoscope leading to the identification of small bowel adenocarcinoma in a patient with undiagnosed Crohn disease. Ann Diagn Pathol 2009; 13:390-3. [DOI: 10.1016/j.anndiagpath.2009.06.011] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/09/2009] [Revised: 05/30/2009] [Accepted: 06/29/2009] [Indexed: 01/13/2023]
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Half E, Bercovich D, Rozen P. Familial adenomatous polyposis. Orphanet J Rare Dis 2009; 4:22. [PMID: 19822006 PMCID: PMC2772987 DOI: 10.1186/1750-1172-4-22] [Citation(s) in RCA: 368] [Impact Index Per Article: 23.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/18/2009] [Accepted: 10/12/2009] [Indexed: 02/06/2023] Open
Abstract
Familial adenomatous polyposis (FAP) is characterized by the development of many tens to thousands of adenomas in the rectum and colon during the second decade of life. FAP has an incidence at birth of about 1/8,300, it manifests equally in both sexes, and accounts for less than 1% of colorectal cancer (CRC) cases. In the European Union, prevalence has been estimated at 1/11,300-37,600. Most patients are asymptomatic for years until the adenomas are large and numerous, and cause rectal bleeding or even anemia, or cancer develops. Generally, cancers start to develop a decade after the appearance of the polyps. Nonspecific symptoms may include constipation or diarrhea, abdominal pain, palpable abdominal masses and weight loss. FAP may present with some extraintestinal manifestations such as osteomas, dental abnormalities (unerupted teeth, congenital absence of one or more teeth, supernumerary teeth, dentigerous cysts and odontomas), congenital hypertrophy of the retinal pigment epithelium (CHRPE), desmoid tumors, and extracolonic cancers (thyroid, liver, bile ducts and central nervous system). A less aggressive variant of FAP, attenuated FAP (AFAP), is characterized by fewer colorectal adenomatous polyps (usually 10 to 100), later age of adenoma appearance and a lower cancer risk. Some lesions (skull and mandible osteomas, dental abnormalities, and fibromas on the scalp, shoulders, arms and back) are indicative of the Gardner variant of FAP. Classic FAP is inherited in an autosomal dominant manner and results from a germline mutation in the adenomatous polyposis (APC) gene. Most patients (~70%) have a family history of colorectal polyps and cancer. In a subset of individuals, a MUTYH mutation causes a recessively inherited polyposis condition, MUTYH-associated polyposis (MAP), which is characterized by a slightly increased risk of developing CRC and polyps/adenomas in both the upper and lower gastrointestinal tract. Diagnosis is based on a suggestive family history, clinical findings, and large bowel endoscopy or full colonoscopy. Whenever possible, the clinical diagnosis should be confirmed by genetic testing. When the APC mutation in the family has been identified, genetic testing of all first-degree relatives should be performed. Presymptomatic and prenatal (amniocentesis and chorionic villous sampling), and even preimplantation genetic testing is possible. Referral to a geneticist or genetic counselor is mandatory. Differential diagnoses include other disorders causing multiple polyps (such as Peutz-Jeghers syndrome, familial juvenile polyps or hyperplastic polyposis, hereditary mixed polyposis syndromes, and Lynch syndrome). Cancer prevention and maintaining a good quality of life are the main goals of management and regular and systematic follow-up and supportive care should be offered to all patients. By the late teens or early twenties, colorectal cancer prophylactic surgery is advocated. The recommended alternatives are total proctocolectomy and ileoanal pouch or ileorectal anastomosis for AFAP. Duodenal cancer and desmoids are the two main causes of mortality after total colectomy, they need to be identified early and treated. Upper endoscopy is necessary for surveillance to reduce the risk of ampullary and duodenal cancer. Patients with progressive tumors and unresectable disease may respond or stabilize with a combination of cytotoxic chemotherapy and surgery (when possible to perform). Adjunctive therapy with celecoxib has been approved by the US Food and Drug Administration and the European Medicines Agency in patients with FAP. Individuals with FAP carry a 100% risk of CRC; however, this risk is reduced significantly when patients enter a screening-treatment program.
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Affiliation(s)
- Elizabeth Half
- Familial Cancer Clinic, Gastroenterology Dept, Meir Hospital, Kfar Saba, Israel.
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Tan KY, Moriya Y, Konishi F. Multimedia articles. Small-bowel tumors with extensive mesenteric involvement can be resected with careful dissection of the mesenteric vessels with good outcomes. Dis Colon Rectum 2009; 52:1184-1185. [PMID: 19581866 DOI: 10.1007/dcr.0b013e3181a537fa] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/08/2023]
Abstract
We show the video of the dissection and resection of a locally advanced jejunal adenocarcinoma, with the involvement of multiple loops, to the base of the mesentery. Complete resection was made possible by careful dissection of the jejunal vessels and superior mesenteric artery at the root of the small-bowel mesentery (see Video, Supplemental Digital Content 1, http://links.lww.com/A1308). The long-term outcome for this patient is good.
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Affiliation(s)
- Kok-Yang Tan
- Department of Surgery, Alexandra Hospital, Singapore.
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Sameshima S, Tomozawa S, Koketsu S, Okada T, Miyato H, Iijima M, Kojima M, Kaji T. Intramucosal adenocarcinoma of the ileum originated 40 years after ileosigmoidostomy. World J Surg Oncol 2009; 7:41. [PMID: 19379525 PMCID: PMC2676285 DOI: 10.1186/1477-7819-7-41] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/19/2009] [Accepted: 04/21/2009] [Indexed: 01/13/2023] Open
Abstract
Background Small bowel adenocarcinomas (SBAs) are rare carcinomas. They are asymptomatic and usually neither endoscopy nor contrast studies are performed for screening Case presentation A 72-year-old Japanese male had a positive fecal occult blood test at a regular check-up in 2006. He suffered appendicitis and received an ileosigmoidostomy in 1966. A colonoscopy revealed an irregular mucosal lesion with an unclear margin at the ileum side of the anastomosis. A mucosal biopsy specimen showed adenocarcinoma histopathologically. Excision of the anastomosis was performed for this patient. The resected specimen showed a flat mucosal lesion with a slight depression at the ileum adjacent to the anastomosis. Histological examination revealed a well differentiated intramucosal adenocarcinoma (adenocarcinoma in situ). Immunohistological staining demonstrated the overexpression of p53 protein in the adenocarcinoma. Conclusion Adenocarcinoma of the ileum at such an early stage is a very rare event. In this case, there is a possibility that the ileosigmoidostomy resulted in a back flow of colonic stool to the ileum that caused the carcinogenesis of the small intestine.
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Affiliation(s)
- Shinichi Sameshima
- Department of Surgery, Hitachi Yokohama Hospital, 550 Totsuka-cho, Totsuka-ku, Yokohama, Kanagawa 244-0003, Japan.
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Kala Z, Kysela P, Meluzinova H. Small-bowel tumors in the elderly 65+ years: 10 years of experience. Z Gerontol Geriatr 2009; 41:403-7. [PMID: 18327695 DOI: 10.1007/s00391-007-0478-7] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/22/2022]
Abstract
UNLABELLED Primary, secondary, benign or malignant tumorous diseases of the small intestine are rare. They are very often diagnosed by accident or as a cause of acute abdomen. Less serious symptoms can be overseen mainly in older patients. This work represents a prospective evaluation of a diagnostic and therapeutic algorithm in the management of the small-intestine tumor below the ligamentum Treitzi in patients over 65 years of age. RESULTS A population of 96 patients who underwent surgery for a small-intestinal tumor in our hospital from 1996 to 2006 is presented. Thirty-four of these patients were older than 65 years. Ultrasound of the small intestine was sufficient to make the diagnosis in 21 of them. Elective surgery (laparoscopy was highly preferred) could be offered to 31 of these older patients. Perioperative mortality was zero. CONCLUSION The small-bowel ultrasound can be recommended as the first choice method. All complaints regarding the gastrointestinal tract should be verified. Older patients can be safely offered an elective laparoscopic surgery with acceptable morbidity and mortality.
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Affiliation(s)
- Z Kala
- Department of Surgery, Faculty Hospital Brno, Brno, Czech Republic
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Overman MJ, Kopetz S, Wen S, Hoff PM, Fogelman D, Morris J, Abbruzzese JL, Ajani JA, Wolff RA. Chemotherapy with 5-fluorouracil and a platinum compound improves outcomes in metastatic small bowel adenocarcinoma. Cancer 2008; 113:2038-45. [PMID: 18759326 DOI: 10.1002/cncr.23822] [Citation(s) in RCA: 100] [Impact Index Per Article: 5.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/03/2023]
Abstract
BACKGROUND Metastatic small bowel adenocarcinoma (SBA) has a poor prognosis. Because of the rarity of SBA, only a few studies have evaluated the role of chemotherapy in the treatment of metastatic SBA; thus, the benefit, if any, of adding a platinum compound to fluorouracil (5-FU) is unknown. The objective of this retrospective study was to determine whether the addition of a platinum compound to 5-FU provided any benefit in the treatment of patients with metastatic SBA. METHODS The authors identified 80 patients with metastatic SBA who were treated with chemotherapy at the University of Texas M. D. Anderson Cancer Center between 1978 and 2005. Response rates, progression-free survival (PFS), and overall survival (OS) were compared between patients who received 5-FU and a platinum compound and patients who received other chemotherapy combinations. RESULTS The median patient age was 53 years. The primary tumor site was the jejunum in 35 patients (43%), duodenum in 30 patients (38%), ileum in 6 patients (8%), and nonspecified small bowel in 9 patients (11%). Of all 80 patients, 29 patients (36%) received 5-FU and a platinum compound, 41 patients (51%) received 5-FU without a platinum compound, and 10 patients (13%) received non-5-FU-based treatment. Compared with other chemotherapy regimens, treatment with 5-FU and a platinum agent resulted in a higher response rate (46% vs 16% with other regimens; P = .01) and longer median PFS (8.7 months vs 3.9 months; P < or = .01) but not better OS (14.8 months vs 12 months; P = .1). In multivariate analysis, treatment with 5-FU and a platinum compound was a significant predictor of response (odds ratio, 4.5; 95% confidence interval [CI], 1.3-15.8; P = .02) and PFS (hazard ratio. 0.49; 95% CI, 0.29-0.84; P = .01) but only reached borderline significance for OS (hazard ratio, 0.63; 95% CI, 0.37-1.07; P = .08). CONCLUSIONS To the authors' knowledge, the current analysis represents the largest number of patients with metastatic SBA treated with chemotherapy in the literature, and the results suggested that the combination of 5-FU and a platinum compound leads to a higher response rate and PFS compared with other chemotherapy regimes. The authors concluded that prospective investigation of platinum analogues in the treatment of SBA is warranted.
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Affiliation(s)
- Michael J Overman
- Department of Gastrointestinal Medical Oncology, The University of Texas M. D. Anderson Cancer Center, Houston, Texas 77030, USA.
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Reddy VB, Aslanian H, Suh N, Longo WE. Asymptomatic ileal adenocarcinoma in the setting of undiagnosed Crohn’s disease. World J Gastroenterol 2008; 14:4690-3. [PMID: 18698685 PMCID: PMC2738795 DOI: 10.3748/wjg.14.4690] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/06/2023] Open
Abstract
A 53-year old previously healthy male underwent a screening colonoscopy for detection of a potential colorectal neoplasm. The terminal ileum was intubated and a mass was noted. Examination of the colon was normal. The biopsy of the ileal mass was consistent with an adenocarcinoma arising from the terminal ileum. His father who had never been previously ill from gastrointestinal disease died of natural causes, but was found to have Crohn’s disease postmortem. The patient underwent exploratory laparotomy and a right hemicolectomy with a 30 cm section of terminal ileum in continuity. Findings were consistent with ileal adenocarcinoma in the setting of Crohn’s disease. The patient made an uneventful recovery. The pathology was stage 1 adenocarcinoma. This is a unique case in that on a screening colonoscopy, a favorable ileal adenocarcinoma was discovered in the setting of asymptomatic, undiagnosed ileal Crohn’s disease in a patient whose father had Crohn’s disease diagnosed postmortem.
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Abstract
Small bowel neoplastic disease is a rare but dreaded occurrence in Crohn's disease (CD) and the diagnosis is often disguised by nonspecific and varied presenting symptoms mimicking active or obstructive CD. As such, the diagnosis is all too often delayed, typically detected at a late stage, and with a poor prognosis. CD has become a well-recognized risk factor for the development of small bowel adenocarcinoma. The data, however, are limited and based on case reports, retrospective studies, and review of the literature.
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Affiliation(s)
- Richard C Feldstein
- Division of Gastroenterology, Hepatology and Nutrition, Department of Medicine, North Shore University Hospital-Long Island Jewish Medical Center, New York University School of Medicine, Manhasset, New York, USA
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Abstract
A 65-year-old woman with a medical history of diabetes mellitus type 2, hypertension, an old cerebrovascular accident, and seizure disorder presented to the emergency room with lower abdominal pain of 4 weeks duration. Upon physical examination, her abdomen was soft and bowel sounds were present, but there was diffuse tenderness in her lower abdomen with some guarding. A computed tomography scan of her abdomen with oral and intravenous contrast showed significantly thickened small bowel loops with subjacent lymphadenopathy. Biopsies obtained during esophagogastroduodenoscopy and colonoscopy showed acute and chronic inflammation. A double balloon enteroscopy (DBE) was then performed, which showed stricture in the jejunum from which the biopsy was obtained. The biopsy showed marginal cell lymphoma. The patient is presently undergoing chemotherapy. Double balloon enteroscopy is a new elegant endoscopical technique that seems promising, as the endoscopist can reach undiscovered small bowel segments. It seems to be well tolerated and safe. For the first time, it provides the means to endoscopically investigate and treat disorders of the small intestine that have previously been inaccessible to conventional endoscopy.
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Anastassiades CP, Anavekar NS, McDonald FS. 78-year-old man with emesis and jaundice. Mayo Clin Proc 2008; 83:221-4. [PMID: 18241633 DOI: 10.4065/83.2.221] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/23/2022]
Affiliation(s)
- Constantinos P Anastassiades
- Mayo School of Graduate Medical Education, Division of General Internal Medicine, Mayo Clinic, 200 First St SW, Rochester, MN 55905, USA
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Tougeron D, Lefebure B, Savoye G, Tuech JJ, di Fiore F, Michel P. Small-bowel adenocarcinoma in patient with Crohn's disease: report of a series of three cases. Scand J Gastroenterol 2008; 43:1397-1400. [PMID: 18609183 DOI: 10.1080/00365520801918042] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/04/2023]
Abstract
Patients affected with Crohn's disease (CD) have a recognized, but low relative risk of developing small-bowel adenocarcinoma (SBA). In fact, SBA develops in 2.2% of patients who have long-standing CD and it is seldom diagnosed preoperatively because of its rarity. A retrospective analysis of all cases of SBA in CD patients since 1980 was carried out in Rouen University Hospital. Three patients with known or unknown CD who presented with SBA with long-term follow-up were analysed. In our first case, the occlusive syndrome revealed SBA and CD simultaneously. Most ileal carcinomas in CD are located in strictures and are often incidentally diagnosed postoperatively, as in our three cases. Digestive surgeons and gastroenterologists must be aware that the diagnosis of SBA in CD is often made fortuitously on histological examination after surgical resection for an occlusive syndrome. Failure to detect SBA in patients with CD results in late diagnosis, with poor survival.
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Affiliation(s)
- David Tougeron
- Department of Hepatogastroenterology, Rouen University Hospital, Northwest Canceropole, France.
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46
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Adenocarcinoma and Other Small Intestinal Malignancies. Oncology 2007. [DOI: 10.1007/0-387-31056-8_43] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/22/2022]
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Abstract
For advanced adenocarcinomas, which are the most frequent tumours of the lower GI tract, the concept of radical lymphnode dissection is well accepted. The quality of lymphadenectomy for these malignancies has a strong effect on cancer-related survival. Based upon a strict quality control program with outcome evaluated according to internal results, the technique and extent of lymph node dissection have been continuously developed over the last three decades. These are described in detail, including instructive pictures to clarify the surgical steps needed. Apart from multivisceral resection in far advanced cases, which still have a chance of cure if adequate guidelines are followed, two additional steps in the so-called radical surgical treatment of these tumours are prerequisites for cure. The first is complete mobilisation of the intestine involving complete mesocolic excision with complete retention of the visceral fascia and covering potential lymph node metastases and extranodal spread on the intestinal side. The second step is the central tying of the tumor's supplying vessels. Following these rules and with no adjuvant systemic treatment, 5-year survival figures of 80% can be reached, even for UICC stage III disease.
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Affiliation(s)
- W Hohenberger
- Chirurgische Klinik, Universität Erlangen-Nürnberg, Krankenhausstrasse 12, 91054 Erlangen.
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Kelsey CR, Nelson JW, Willett CG, Chino JP, Clough RW, Bendell JC, Tyler DS, Hurwitz HI, Morse MA, Clary BM, Pappas TN, Czito BG. Duodenal adenocarcinoma: patterns of failure after resection and the role of chemoradiotherapy. Int J Radiat Oncol Biol Phys 2007; 69:1436-41. [PMID: 17689032 DOI: 10.1016/j.ijrobp.2007.05.006] [Citation(s) in RCA: 45] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/08/2007] [Revised: 03/07/2007] [Accepted: 05/05/2007] [Indexed: 02/06/2023]
Abstract
PURPOSE To report patterns of disease recurrence after resection of adenocarcinoma of the duodenum and compare outcomes between patients undergoing surgery only vs. surgery with concurrent chemotherapy and radiation therapy (CT-RT). METHODS AND MATERIALS This was a retrospective analysis of all patients undergoing potentially curative therapy for adenocarcinoma of the duodenum at Duke University Medical Center and affiliated hospitals between 1975 and 2005. Overall survival (OS), disease-free survival (DFS), and local control (LC) were estimated using the Kaplan-Meier method. Univariate regression analysis evaluated the effect of CT-RT on clinical endpoints. RESULTS Thirty-two patients were identified (23 M, 9 F). Median age was 60 years (range, 32-77 years). Surgery alone was performed in 16 patients. An additional 16 patients received either preoperative (n = 11) or postoperative (n = 5) CT-RT. Median RT dose was 50.4 Gy (range, 12.6-54 Gy). All patients treated with RT also received concurrent 5-fluorouracil-based CT. Two patients treated preoperatively had a pathologic complete response (18%), and none had involved lymph nodes at resection. Five-year OS, DFS, and LC for the entire group were 48%, 47%, and 55%, respectively. Five-year survival did not differ between patients receiving CT-RT vs. surgery alone (57% vs. 44%, p = 0.42). However, in patients undergoing R0 resection, CT-RT appeared to improve OS (5-year 83% vs. 53%, p = 0.07). CONCLUSIONS Local failure after surgery alone is high. Given the patterns of relapse with surgery alone and favorable outcomes in patients undergoing complete resection with CT-RT, the use of CT-RT in selected patients should be considered.
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Affiliation(s)
- Chris R Kelsey
- Department of Radiation Oncology, Division of Medical Oncology and Transplantation, Duke University Medical Center, Durham, NC 27710, USA.
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Safatle-Ribeiro AV, Franzini TAP, Kuga R, Ishida RK, Baba ER, Mendes DC, Ishioka S, Sakai P. Double-balloon enteroscopy in the diagnosis of an adenocarcinoma of the fourth portion of the duodenum: report of a case. Clinics (Sao Paulo) 2007; 62:353-6. [PMID: 17589678 DOI: 10.1590/s1807-59322007000300022] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/21/2022] Open
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Abstract
There has been a multitude of case reports, case series, hospital-based, and population-based studies that link CD to various types of cancers. When each of these studies is scrutinized, however, there is only enough evidence to support a link between colorectal adenocarcinoma, SBA, and squamous and adenocarcinomas that are associated with perianal fistulizing disease. All of the studies of large bowel adenocarcinoma or SBA follow patients in an era during which there were far fewer effective medicines to treat CD and surgery was more commonplace. The only surveillance study of patients who had extensive, long-duration Crohn's colitis showed a 22% risk for developing neoplasia (low-grade, high-grade, or cancer) after four surveillance examinations. Overall results from this study and the multitude of the other studies show that the risk for cancer in Crohn's colitis is equal to that in UC given equal extent and duration of disease. Patients who have Crohn's colitis that affects at least one third of the colon and with at least 8 years of disease should undergo screening and surveillance, just as in UC. Although the absolute risk for SBA in CD is low (2.2% at 25 years in one study), we should not rule out screening and surveying for this complication that is associated with significant morbidity and mortality in patients who have long-standing, extensive, small bowel disease. The risk for lymphoma and leukemia in CD is low, but immunomodulators and biologics may increase this risk. The evidence that links carcinoid tumors to CD is weak, and population-based studies need to be done. The study of cancers that are associated with CD is an evolving field that surely will change given that immunomodulators and biologics are being used with greater frequency.
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Affiliation(s)
- Sonia Friedman
- Department of Medicine, Harvard Medical School, Boston, MA 02115, USA.
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