The many faces of gastrointestinal tcell lymphoma manifest simultaneously in the same patient a 50yearold man with a history of aids presented with complaints of dyspnea on exertion,anemia,andmelena. Gastric lymphoma gastric lymphoma represents the most common site of extra nodal lymphoma, accounting for 25% of all such lymphomas, 50% of all gastrointestinal lymphomas, but comprise only 15% of all gastric malignancies. A typical case of gastric marginal zone lymphoma is illustrated in fig. Lineage differentiation of canine lymphomaleukemias and. Primary gastric lymphoma pathogenesis and treatment. Follicular lymphoma is a common type of nhl, and occurs almost exclusively in adults.
The most common histological type is mucosaassociated lymphoid tissue lymphoma, followed by diffuse large bcell lymphoma. Primary follicular lymphoma of the gastrointestinal tract annals of. We present a case of gastric tcell lymphoma with its clinical presentation, pathological, and laboratory findings, and discuss the differential diagnoses, and. Lymphomas are solid malignancies of the lymphoid system and are subdivided into hodgkins and nonhodgkins lymphomas nhls. Primary lymphoma of the gi tract, as a single site, should be limited to the tract with no. Primary involvement of gastric mucosal areas malt lymphoma, now called extranodal marginal zone bcell lymphoma of malt type in the real classification. In the united states in 20, about 9,290 persons were expected to be diagnosed with hodgkin lymphoma. Primary gastric lymphoma lymphoma that originates in the stomach itself is an uncommon condition, accounting for less than 15% of gastric malignancies and about 2% of all lymphomas. Indeed chronic antigenic stimulation causes genetic instability and clonal growth of malt. Although lymphoma can involve any part of the gastrointestinal tract, the most frequent sites in order of its occurrence are the stomach followed by small intestine and ileocecal region. Gastrointestinal lymphoma is a common form of cancer thats prevalent in breeds such as boxers and sharpies. All files from the year 1971 to the year 2000 were checked manually, and the digital data files that were present as of the year 2000 were electronically searched with the search terms.
Other mature b cell lymphomas that can arise in the gi tract are burkitt lymphoma bl, follicular lymphoma fl, mantle cell lymphoma mcl. Gastrointestinal follicular lymphoma gifl is a relatively rare disease, accounting for only 1%3. Primary gastrointestinal nonhodgkins lymphoma pginhl of small and large intestines is a group of heterogeneous, rare malignancies. Symptoms may include enlarged lymph nodes, unexplained weight loss, fatigue, night sweats and shortness of breath, cough or trouble breathing. Thus, a definitive diagnosis of anaplastic large cell lymphoma alcl of t cell origin involving multiple organs including the stomach and duodenum was made, and combined chemotherapy was entertained. Follicular nhl expression signature relative risk of death p value prognosis immune response 1 0. Alcl consists of a subgroup of nonhodgkins lymphoma. Unfortunately, this is very difficult to treat even with injectable chemotherapy. Pier francesco ferrucci1 and emanuele zucca2 1department of haematoncology, european institute of oncology ieo, milan, italy, and 2department of oncology, southern switzerland institute of oncology iosi, s.
Stomach is the most commonly involved organ followed by the small intestine, pharynx, colon, and rarely, esophagus. Lymphoma lymphoma is a cancer that develops in the white blood cells of the lymphatic system. Follicular nonhodgkin lymphoma grades 3a and 3b have a similar outcome and appear incurable with anthracyclinebased therapy. Pdf gastrointestinal lymphomas represent 520% of extranodal lymphomas and mainly occur in the stomach and small intestine. Gastric lymphoma 3 percent of gastric neoplasms 10 percent of lymphomas the most common extranodal site of lymphoma the most common site of gastrointestinal lymphoma can arise from two different sources. It was estimated that in 2008, there would be 8,220 and 66,120 new diagnoses of hodgkins and nhl, respectively, in the united states.
In the united states, there are about 70,800 new cases of. Nonhodgkins lymphomas caused by malignant cancerous bcell lymphocytes represent a largesubset about 85% in the us of the known types of lymphoma the other 2 subsets being tcell lymphomas and lymphomas where the cell type is unknown. In a small proportion of cats, this disease will morph into a large cell, aggressive gi lymphoma. Recently, the incidence of intestinal follicular lymphoma has been increasing. From november 1983 to february 2001, 39 patients with a diagnosis of. Oncology fact sheet american college of veterinary. Familial hodgkin lymphoma ghsg german hodgkin study group. Between january 1974 and december 1988, a total number of 404 nhls with gi tract involvement were listed consecutively in the files of the department of. This national cancer institute nci booklet is about. Primary gastric tcell lymphoma is a rarely encountered lesion causing difficulties in endoscopic biopsy diagnosis. Protocol applies to hodgkin and nonhodgkin lymphomas of the gastrointestinal tract.
One of the characteristics of malt lymphoma is crypt infiltration and destruction, socalled lymphoepithelial lesions fig. However, biomedical research has been developing rapidly in recent years and new. Mantle cell lymphoma is an aggressive disease that manifests as multiple polyps. Fs4 mantle cell lymphoma facts i page 1 revised november 2014 introduction lymphoma is the general name for many related subtypes of cancer that arise from a type of white blood cell called a lymphocyte. Lymphoma is a general name for a group of cancers that affect the lymphatic system. Frequency of gastrointestinal involvement and its clinical. Cd10 down expression in follicular lymphoma correlates with gastrointestinal lesion involving the stomach and large intestine nobuhiko ohnishi, 1katsuyoshi takata, tomoko miyatatakata, 1yasuharu sato, akira tari,2 yuka gion, mai noujimaharada, 1kohei taniguchi, tetsuya tabata, 1keina nagakita, shizuma omote, hiroyuki takahata,3 masaya iwamuro,4 hiroyuki okada,5. It is typically a lowgrade malignancy lymphoma gifl is a type of nonhodgkin lymphoma that usually originates in the gastrointestinal gi tract, commonly in the intestine. Gastrointestinal follicular lymphoma gifl is a rare type of bcell nonhodgkins lymphoma that generally affects older adults. Lymphoma is the most commonly diagnosed neoplasm in cats, and gastrointestinal gi lymphoma is now the most common anatomic form. Bcells undergo many changes in their life cycle dependent on. Gastric lymphoma is a relatively rare malignancy in which gastric tumors arise from proliferation of monoclonal b lymphocytes 67. However, there is no consensus on the most appropriate staging system for intestinal dlbcl.
It has been known for many years that hodgkin lymphoma occurs more often in biologically related family members of affected patients than in the rest of the population. The reported frequency of gastrointestinal gi tract involvement in patients with mantle cell lymphoma mcl is 1530%. While extranodal lymphoma may arise anywhere outside lymph nodes, gastrointestinal gi tract is the most frequent site of extranodal involvement by nonhodgkins lymphoma. Primary lymphomas in the gastrointestinal tract request pdf. Although the duodenum and terminal ileum are considered to be the most common sites of origin, the development of wireless capsule endoscopy and doubleballoon enteroscopy has increased the detection. By continuing to browse this site you are agreeing to our use of cookies. However, the occurrence of enktl outside the nasal areas such as the skin, liver, soft tissue and gastrointestinal gi tract is also common. Extra nodal involvement is present in the majority of cases, with a peculiar tendency to invade the gastrointestinal tract in the form of multiple lymphomatous.
Most colorectal lymphomas are non hodgkin lymphomas, usually of bcell origin. The gastrointestinal gi tract is a frequent site of involvement with lymphoma which usually involves the stomach, less frequently the small intestine and rarely the colon or esophagus. Most cats with gi lymphoma test negative for felv and have a median age of 9 to years. Feline small cell lymphoma feline lymphoma is one of the most common types of cancer in cats and can be divided clinically and histopathologically into lymphocytic, lowgrade, small cell lymphoma slsa or lymphoblastic, highgrade, large cell lsa llsa. The exact cause of lsa is unknown in virtually all cases. Varun introduction anywhere outside the lymph node region primary lymphoid organs. We evaluated the utility of the modified ann arbor system. Enteropathyassociated tcell lymphoma eatl, previously termed enteropathyassociated tcell lymphoma, type i and at one time termed enteropathytype tcell lymphoma ettl, is a complication of coeliac disease in which a malignant tcell lymphoma develops in areas of the small intestine afflicted by the diseases intense inflammation. Primary gastrointestinal lymphoma accounts for 3040% of all extranodal lymphomas, while it comprises only 18% of all gastrointestinal malignancies. An update of pathogenetic features ic process for the definitive neoplastic transformation of acquired malt in gastric mucosa1518 figure 2. Pdf gastrointestinal tract is the most common extranodal site involved by lymphoma with the majority being nonhodgkin type. However, this figure most likely is an underestimate because most patients with mcl involving the gi tract previously reported were examined endoscopically only if they had gi tract symptoms. Sevenyears prior to the current presentation, he presented with intussusception, underwent right hemicolectomy, and was diagnosed with a cecal.
This cancer affects the lymphatic system and weakens the immune system of the dog making it susceptible to infections that the body cant fight off. An evaluation of different staging systems the gastrointestinal tract is the most common primary extranodal site for diffuse large bcell lymphoma dlbcl. Although gastrointestinal lymphoma is usually secondary to nodal lymphoma, it can present as a primary gastrointestinal lymphoma with the majority being in the stomach 74. Primary lymphoma more often affects the cecum and rectum. Malt lymphoma mostly occurs in adults with a median age of 61 years and slight female preponderance. Diffuse large bcell lymphoma was the most common subtype 47. It accounts for approximately 7% to 24% of all canine cancers. At an early stage, tumor is confined to the gastric mucosa and its growth depends critically on h. Extranodal nkt cell lymphoma enktl is known to cause localized disease involving midline facial structures such as the nasal cavity and nasopharynx. The two major types of lymphoma are hodgkin lymphoma hl and nonhodgkin lymphoma nhl. Pdf radiological features of gastrointestinal lymphoma. However, the stomach is a very common extranodal site for lymphomas lymphomas originate elsewhere and metastasise to the stomach. Gastrointestinal lymphomas are usually not clinically specific and indistinguishable from other benign and malignant conditions.
Gastrointestinal tract is the most common extranodal site involved by lymphoma with the majority being nonhodgkin type. Chapter 17 gastric lymphoma cancer council australia. Pflgi were retrieved from the files of several french hematology and gastrointestinal. The reasons for this phenomenon have not yet been clearly determined. As with other sites, management is dependent on type, stage and age but with several additional special. The most common clinical sign of gi lymphoma is weight loss followed by decreased appetite, vomiting, and diarrhea. Canine lymphoma is a common, spontaneously occurring hematopoietic tumor in dogs and a model for nonhodgkins lymphoma in humans teske, 1994a. The absence of abnormality or the presence of just minimal gastric wall thickening or a shallow lesion at ct suggests lowgrade malt lymphoma.
Vlaams diergeneeskundig tijdschrift, 2012, 81 retrospective study 341 introduction lymphoma, also referred to as lymphosarcoma lsa or malignant lymphoma, is one of the most prevalent neoplasms in the dog. Cd10 down expression in follicular lymphoma correlates. Primary gastrointestinal nonhodgkins lymphoma of the. Mantle cell lymphoma mcl is an aggressive type of bcell nonhodgkins lymphoma that originates from small to medium sized lymphocytes located in the mantle zone of the lymph node. Typically primary gastric lymphoma occurs in adults in the 6th decade of life, without gender predilection. In the early stages, there is organization of the infiltrate, with plasma cells being found in.