Lymphoproliferative Disorders of the Gastrointestinal Tract A Review and Pragmatic Guide to Diagnosis
Department of Pathology, Alta Bates Summit Medical Center, Berkeley, California 94705, USA. Archives of pathology & laboratory medicine
(Impact Factor: 2.84).
10/2011; 135(10):1283-97. DOI: 10.5858/arpa.2011-0145-RA
The gastrointestinal tract is the most common site of extranodal lymphomas. Although all histologic categories of malignant lymphoma develop in the gastrointestinal tract, large B-cell lymphomas predominate, followed by extranodal marginal zone lymphomas of mucosa-associated lymphoid tissue (MALT) type; the latter is especially prevalent in stomach. The acceptance of extranodal marginal zone lymphoma of MALT type as a clinicopathologic entity has reduced the number of cases that formerly were interpreted as florid lymphoid hyperplasia ("pseudolymphoma"). Nonetheless, the distinction of lymphoid hyperplasia from a lymphoma of MALT type in small biopsy specimens remains problematic.
To assess the relevant morphologic, immunologic, molecular, and genetic properties of gastrointestinal lymphomas and to present a feasible tactic for diagnosis, expressly for small biopsy specimens.
Case-derived material and literature review using PubMed (National Library of Medicine).
Most gastrointestinal lymphomas are readily amenable to an unqualified diagnosis, primarily those cases consisting of monomorphic large cells whether of B- or T-cell lineage, including cases associated with enteropathy. Diagnosis for infiltrates dominated by small lymphocytes remains taxing, as the differential diagnosis embraces not only MALT lymphoma and lymphoid hyperplasia but also mantle cell lymphoma, follicular lymphoma, and chronic lymphocytic leukemia/small lymphocytic lymphoma. Adherence to strict morphologic criteria is the standard for diagnosis, but these criteria should be augmented by immunologic studies together with judicious use of molecular techniques to determine clonality. In establishing a diagnosis of gastric marginal zone lymphoma of MALT type, determination of t(11;18)(q21;q21) status may be required since this translocation has clinical ramifications.
Available from: Govind Bhagat
- " Enteropathy associated T-cell lymphoma (EATL) types I and II and extranodal NK/T-cell lymphoma, nasal type, are the most frequent types of lymphomas presenting with intestinal involvement.– Rarely, other types of PTCL such as ALK+ anaplastic large cell lymphoma and gamma-delta T-cell lymphoma can also arise in the GI tract or involve it secondarily.–. "
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ABSTRACT: Enteropathy-associated T-cell lymphomas (EATL) are rare and generally aggressive types of peripheral T-cell lymphomas. Rare cases of primary, small intestinal CD4+ T-cell lymphomas with indolent behavior have been described, but are not well characterized. We describe morphologic, phenotypic, genomic and clinical features of 3 cases of indolent primary small intestinal CD4+ T-cell lymphomas. All patients presented with diarrhea and weight loss and were diagnosed with celiac disease refractory to a gluten free diet at referring institutions. Small intestinal biopsies showed crypt hyperplasia, villous atrophy and a dense lamina propria infiltrate of small-sized CD4+ T-cells often with CD7 downregulation or loss. Gastric and colonic involvement was also detected (n = 2 each). Persistent, clonal TCRβ gene rearrangement products were detected at multiple sites. SNP array analysis showed relative genomic stability, early in disease course, and non-recurrent genetic abnormalities, but complex changes were seen at disease transformation (n = 1). Two patients are alive with persistent disease (4.6 and 2.5 years post-diagnosis), despite immunomodulatory therapy; one died due to bowel perforation related to large cell transformation 11 years post-diagnosis. Unique pathobiologic features warrant designation of indolent small intestinal CD4+ T-cell lymphoma as a distinct entity, greater awareness of which would avoid misdiagnosis as EATL or an inflammatory disorder, especially celiac disease.
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ABSTRACT: The gastrointestinal tract (GIT) is the most commonly involved site of extranodal lymphomas. The close association between chronic inflammation and specific GIT lymphomas not only provide interesting insights into the pathobiology of lymphomas but also poses unique diagnostic challenges. A clear understanding of marginal zone and mucosa associated lymphoid tissue (MALT) in health and disease is helpful to place GIT lymphomas in proper context. A wide variety of lymphomas besides MALT lymphomas occur in various parts of the GIT. The characteristic pathological, immunophenotypic, and genetic features of different GIT lymphomas categorized according to World Health Organization (WHO) classification are presented. The epidemiological, clinical, and pathological features of lymphomas occurring in each part of the GIT are summarized and the key points regarding lymphomas at each site are emphasized. A tabular summary of the important differential diagnostic considerations at each site is given and suggestions for a minimal diagnostic work up are provided.
Available from: Shaoying Li
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ABSTRACT: Extranodal NK/T-cell lymphoma, nasal type (ENKTL) is uncommon in the United States. We report 73 patients with ENKTL, including 49 men and 24 women (median age, 46 y). Sixty-three patients had nasal/upper aerodigestive tract disease; 10 had extranasal disease involving skin, small intestine, epiglottis, testis, adrenal glands, kidney, and breast. Complete staging data were available for 68 patients: 44 stage I/II and 24 stage IV. Fifteen of 69 (22%) had lymphadenopathy and 10/63 had bone marrow involvement. Histologically, 67/73 (92%) showed necrosis, and 48/70 (69%) had an angiocentric/angiodestructive growth pattern. The neoplastic cells showed a wide spectrum: medium sized (n=34), mixed small and large (n=21), large (n=13), and small (n=5). In situ hybridization for Epstein-Barr virus-encoded small RNA was positive in every case. Immunohistochemical studies showed expression of cytotoxic markers (100%), T-bet (96%), CD2 (96%), CD3 (93%), CD56 (90%), and ETS-1 (64%). Ki-67 was ≥60% in 46% cases. Therapy was known for 64 patients; 14 received only chemotherapy, 8 radiation alone, and 42 received combined radiation and chemotherapy. Median survival was 4.2 years, and 5-year overall survival was 46% (median follow-up, 3.8 y). Extranasal disease, high International Prognostic Index score, and high proliferation rate correlated with poorer prognosis. We conclude that ENKTL cases in the United States are similar to those reported in Asia and other countries. Absence of the angiocentric/angiodestructive pattern and presence of lymphadenopathy, features underemphasized in the literature, occurred in appreciable subsets of patients. The International Prognostic Index score, anatomic site of disease, and proliferation rate had prognostic value in this patient cohort.
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