Intestinal lymphoma: A pictorial review Poster No.: C-1431 Congress: ECR 2014 Type: Educational Exhibit Authors: J. Arnott, B. Khalil, S. Kar, A. Lecomte ; Southampton/UK, 1 2 2 2 1 2 Lymington/UK Keywords: Metastases, Lymphoma, Staging, Education, PET-CT, PET, CT, Oncology, Gastrointestinal tract, Abdomen DOI: 10.1594/ecr2014/C-1431 Any information contained in this pdf file is automatically generated from digital material submitted to EPOS by third parties in the form of scientific presentations. References to any names, marks, products, or services of third parties or hypertext links to thirdparty sites or information are provided solely as a convenience to you and do not in any way constitute or imply ECR's endorsement, sponsorship or recommendation of the third party, information, product or service. ECR is not responsible for the content of these pages and does not make any representations regarding the content or accuracy of material in this file. As per copyright regulations, any unauthorised use of the material or parts thereof as well as commercial reproduction or multiple distribution by any traditional or electronically based reproduction/publication method ist strictly prohibited. You agree to defend, indemnify, and hold ECR harmless from and against any and all claims, damages, costs, and expenses, including attorneys' fees, arising from or related to your use of these pages. Please note: Links to movies, ppt slideshows and any other multimedia files are not available in the pdf version of presentations. www.myesr.org Page 1 of 30
Learning objectives To review the common locations and types of lymphoma affecting the intestinal tract To review the radiological appearances with CT and PET imaging in a range of cases Background Lymphoma is a type of white blood cell cancer affecting B and T lymphocytes. Classification of lymphoma has been controversial for decades because it encompasses a wide range of different pathologies, with new subtypes being discovered regularly. Traditionally, lymphomas have been divided into Hodgkin or non-hodgkin lymphoma (NHL), depending on whether Reed-Sternberg cells are identified or not, respectively. However, this system has largely been superseded because of a need to describe the many different subtypes that have been identified. The latest classification was derived by the World Health Organisation (WHO) in 2008: [1,2] Page 2 of 30
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Fig. 1: WHO classification of lymphoma 2008 References: Evens AM, Winter JN, Gordon LI et al. Non-Hodgkin Lymphoma. 2011. Cancer Management, 13th edition. Primary lymphoma of the gastrointestinal tract is rare, whereas secondary extranodal spread to the region is relatively common.[3] The vast majority of gastrointestinal lymphoma falls into the NHL category. [4] Gastrointestinal lymphoma originates from one of four different areas within the tract: [5] Organized lymphoid tissue (Peyer's patches) Lamina propria Intraepithelial lymphocytes Mesenteric lymph nodes The stomach is the commonest site for lymphoma within the gastrointestinal tract, followed by the small intestine, pharynx, large intestine, and finally oesophagus.[3] Within the small intestine, the ileum is the commonest site for lymphoma, due to the high concentration of lymphoid tissue.[6] Within the large intestine, the caecum and rectum are the most commonly affected sites.[7] Although there may be radiological features suggestive of lymphoma, definitive diagnosis is based on analysis of histopathological samples: Page 4 of 30
Fig. 2: Diffuse large B cell lymphoma (DLBCL): large tumour cells with vesicular chromatin, prominent nucleoli and moderate to abundant amount of cytoplasm (H&E, 400 ).[7] References: Bautista-Quach MA, Ake CD, Chen M, Wang J. Gastrointestinal lymphomas: Morphology, immunophenotype and molecular features. J Gastrointest Oncol 2012;3(3):209-225. Page 5 of 30
Fig. 3: Burkitt lymphoma: characteristic 'starry sky' appearance and frequent mitotic figures (H&E, 400 ).[7] References: Bautista-Quach MA, Ake CD, Chen M, Wang J. Gastrointestinal lymphomas: Morphology, immunophenotype and molecular features. J Gastrointest Oncol 2012;3(3):209-225. Page 6 of 30
Fig. 4: Enteropathy associated T-cell lymphoma (EATCL): monomorphous, neoplastic lymphoid infiltrate (H&E, 500 ). The inset image (upper left, H&E, 20 ) demonstrates involvement of surface epithelium.[7] References: Bautista-Quach MA, Ake CD, Chen M, Wang J. Gastrointestinal lymphomas: Morphology, immunophenotype and molecular features. J Gastrointest Oncol 2012;3(3):209-225. Pre-disposing factors for developing intestinal lymphoma include: [9] Coeliac disease Human immunodeficiency virus (HIV) infection Inflammatory bowel disease Immunosuppression following solid organ transplantation There have been a number of developments in the staging of intestinal lymphoma over the years, but the most commonly used is still the Ann Arbor system: Page 7 of 30
Fig. 5: Ann Arbor staging system References: Boot, H. Best Practice & Research Clinical Gastroenterology. 2010. 24: 3-12 Other notable systems include the Lugano and Paris systems: Fig. 6: Lugano staging system References: Boot, H. Best Practice & Research Clinical Gastroenterology. 2010. 24: 3-12 Page 8 of 30
Fig. 7: Paris staging system References: Boot, H. Best Practice & Research Clinical Gastroenterology. 2010. 24: 3-12 Findings and procedure details CT of the chest, abdomen and pelvis is the primary staging investigation used for intestinal lymphoma in most clinical settings. Page 9 of 30
Local findings on CT imaging may include: [10] Mural thickening Luminal narrowing with mucosal destruction, and occasionally shouldering of the margins and stricture formation Broad-based ulceration Cavitation Non-specific thickening of the valvulae conniventes Aneurysmal dilatation Polypoidal intraluminal mass Extraluminal mesenteric mass A combination of different signs is often seen.[10] PET-CT is a useful adjunct for staging in most subtypes Our cases illustrate a number of these findings: Case A - Marginal cell lymphoma of the ileum (stage IV): Page 10 of 30
Fig. 8: Sagittal CT slice illustrating mural thickening in a segment of ileum. References: Radiology Department, Lymington New Forest Hospital, Lymington Page 11 of 30
Fig. 9: Oblique coronal CT slice illustrating mural thickening in a segment of ileum. References: Radiology Department, Lymington New Forest Hospital, Lymington Page 12 of 30
Fig. 10: Cine loop of coronal CT slices illustrating mural thickening in a segment of ileum. Page 13 of 30
References: Radiology Department, Lymington New Forest Hospital, Lymington Case B - Diffuse large B cell lymphoma (DLBCL) of the duodenum: Fig. 11: Coronal CT slice illustrating mural thickening with luminal narrowing and shouldering in a segment of duodenum References: Radiology Department, Lymington New Forest Hospital, Lymington Page 14 of 30
Fig. 12: Axial CT slice illustrating mural thickening in a segment of duodenum References: Radiology Department, Lymington New Forest Hospital, Lymington Page 15 of 30
Fig. 13: Axial CT slice illustrating mural thickening and luminal narrowing in a segment of duodenum References: Radiology Department, Lymington New Forest Hospital, Lymington Case C - Lymphoma in a segment of small bowel: Page 16 of 30
Fig. 14: Ultrasound image of the spleen showing splenomegaly with multiple hypoechoic regions. References: Radiology Department, Lymington New Forest Hospital, Lymington Page 17 of 30
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Fig. 15: Sagittal CT slice illustrating splenomegaly with multiple low attenuating regions, and a segment of small bowel with mural thickening. References: Radiology Department, Lymington New Forest Hospital, Lymington Fig. 16: Axial CT slice illustrating a segment of small bowel with mural thickening, and local lymph node enlargement. References: Radiology Department, Lymington New Forest Hospital, Lymington Case D - Stage II BX diffuse large B cell lymphoma (DLBCL) of the small bowel mesentery: Page 19 of 30
Fig. 17: Sagittal CT slice using maximum intensity projection (MIP) to illustrate a mesenteric mass abutting the small bowel, and encasing the superior mesenteric artery. References: Radiology Department, Lymington New Forest Hospital, Lymington Page 20 of 30
Fig. 18: Cine loop of coronal CT slices illustrating a mesenteric mass abutting the small bowel, and encasing the superior mesenteric artery. References: Radiology Department, Lymington New Forest Hospital, Lymington Case E - Stage IVB mantle cell lymphoma of the small bowel with high Ki-67: Page 21 of 30
Fig. 19: Coronal CT slice illustrating diffuse polypoid mural change. References: Radiology Department, Lymington New Forest Hospital, Lymington Page 22 of 30
Fig. 20: Axial CT slice illustrating diffuse polypoid mural change. References: Radiology Department, Lymington New Forest Hospital, Lymington Page 23 of 30
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Fig. 21: Sagittal CT slice illustrating diffuse polypoid mural change. References: Radiology Department, Lymington New Forest Hospital, Lymington Fig. 22: Cine loop of axial PET-CT slices illustrating avid FDG uptake in the affected small bowel segment, as well as the spleen and stomach. References: University Hospital Southampton - Southampton/UK Page 25 of 30
Fig. 23: Cine loop of axial PET-CT slices post-chemotherapy, illustrating normal uptake in the previously affected areas. References: University Hospital Southampton - Southampton/UK Case F - Mixed grade I follicular lymphoma/adenocarcinoma in the caecum: Fig. 24: Axial CT slice illustrating an intraluminal polypoid mass in the caecum. References: Radiology Department, St Mary's Hospital, Newport, Isle of Wight Page 26 of 30
Fig. 25: Virtual 3D surface reconstruction from CT colonography illustrating a polypoidal intraluminal mass. References: Radiology Department, St Mary's Hospital, Newport, Isle of Wight Page 27 of 30
Fig. 26: Cine loop of coronal CT images illustrating a polypoidal intraluminal mass in the caecum. References: Radiology Department, St Mary's Hospital, Newport, Isle of Wight Conclusion Page 28 of 30
Intestinal lymphoma is rare as a primary malignancy but relatively common as a secondary deposit.[3] Non-Hodgkin's lymphoma is more frequently seen than Hodgkin's in the intestine.[4] Small bowel is affected more commonly than large bowel.[3] Whilst radiology is useful for staging the extent of disease, a histopathology sample is essential for definitive diagnosis. CT is the primary staging investigation, but some findings may be shown with ultrasound or barium studies, and PET-CT is a useful adjunct in most subtypes. Amongst our cases, the commonest finding was mural thickening, with or without luminal narrowing. Other findings included diffuse polypoid mural change, and a mesenteric extraluminal mass. Personal information Dr Jonathan Arnott - Radiology Registrar, University Hospital Southampton Dr Bilal Khalil - Radiology Consultant, Lymington New Forest Hospital Dr Shantanu Kar - Radiology Consultant, Lymington New Forest Hospital Dr Amy Lecomte - Radiology Consultant, Lymington New Forest Hospital References 1. Swerdlow SH, Campo E, Harris NL et al. WHO Classification of Tumours 2. of Haematopoietic and Lymphoid Tissues, 4 edition. World Health Organisation. 2008. Evens AM, Winter JN, Gordon LI et al. Non-Hodgkin Lymphoma. Cancer th th Management, 13 edition. October 2011. Page 29 of 30
3. Lee WK, Lau EWF, Duddalwar VF et al. Abdominal Manifestations of Extranodal Lymphoma: Spectrum of Imaging Findings. American Journal of Roentgenology. 2008; 191:198-206 4. Lewin KJ, Ranchod M, Dorfman RF. Lymphomas of the gastrointestinal tract: a study of 117 cases presenting with gastrointestinal disease. Cancer. 1978;42(2):693. 5. Bailey HR, Billingham RP, Stamos MJ et al. Colorectal surgery. Elsevier. 2012. 6. Baert A et al. Encyclopaedia of Diagnostic Imaging. Springer. 2007. 7. Dodd GD. Lymphoma of the hollow abdominal viscera. Radiol Clin North Am 1990;28:771-783. 8. Bautista-Quach MA, Ake CD, Chen M, Wang J. Gastrointestinal lymphomas: Morphology, immunophenotype and molecular features. J Gastrointest Oncol 2012;3(3):209-225. 9. Ghai S, Pattison J, O'Malley ME et al. Primary gastrointestinal lymphoma: spectrum of imaging findings with pathologic correlation. Radiographics. 2007. Sep-Oct;27(5):1371-88. 10. Gourtsoyiannis NC, Nolan DJ. Lymphoma of the Small Intestine: Radiological Appearances. Clinical Radiology. 1988. 39: 639-645. Page 30 of 30