The gastrointestinal tract (GIT) is the most common (30–40%) extranodal site involved in lymphoma. Although primary gastrointestinal lymphoma (PGIL) is a rare disease, comprising only 1–4% of gastrointestinal (GI) malignant tumors, its incidence is increasing. Different regions of the GIT are involved in different subtypes of PGIL with a various frequency that reflects the diversity of the causative agents and predisposing factors for each site and subtype of PGIL. Even though these malignant diseases are categorized under the common term of “lymphoma” they represent a heterogeneous group of malignant neoplasms which are different entities in terms of etiologic factors, predisposing conditions, pathogenesis, immunohistochemical profile, treatment strategy and prognosis. In this chapter the epidemiology of all subtypes of PGIL, factors and disorders contributing to the development of them, non-inherited and inherited conditions associated with a higher risk of them, diagnostic difficulties and pitfalls, and novel treatment strategies were comprehensively and concisely illuminated.
Follicular lymphoma (FL) is one of the most common type of indolent non- Hodgkin’s lymphoma. It originates from germinal center B cells and has characteristic translocation t(11,14) involving immunoglobulin heavy chain gene (chromosome 14q32) and Bcl2 gene (chromosome 18q21) in 90% of patients. FL presents with lymphadenopathy and/or bone marrow involvement. Diagnosis is confirmed by histological examination of lymph nodes. FL is a slow growing tumor with frequent remission and relapses. Follicular lymphoma international prognostic index (FLIPI) and progression of disease within 24 months (POD24) are most important prognostic markers. Early-stage disease is usually treated with radiotherapy. Management of advanced stage depends on disease burden. Patients with advanced stage disease may be observed in case of low burden disease and those with high disease load require treatment with chemo-immunotherapy.
Diffuse large B-cell lymphoma (DLBCL) is a heterogeneous group of diseases of the lymphatic system, which is represented by de novo and secondary tumors resulting from the transformation of indolent lymphomas. In the absence of a long history of the disease at the stage of histological transformation (HT), it is difficult to distinguish between de novo and secondary diffuse large B-cell lymphoma. According to the data of a randomized study, we obtained clinical and laboratory data that are not typical for de novo diffuse large B-cell lymphoma. These include exclusive, predominant retroperitoneal localization, compression of the ureters/kidneys with or without the development of acute renal failure (ARF), unilateral lymphostasis of the leg due to compression of the inguinal, iliac lymph nodes by the conglomerate, intratumor in the central nervous system (CNS) at the onset/relapse/progression of the disease, discordant bone marrow involvement, blood involvement, paraprotein secretion.
Cancer is the biggest health problem worldwide due to its high mortality rate. Lymphoma is defined as a group of malignant diseases that is caused by clonal proliferation of lymphocytes and is classified under two major groups: Hodgkin lymphoma and non-Hodgkin lymphoma. Genetic predisposition and some environmental factors constitute risk factors. Symptoms of the disease include unexplained fever, swelling of lymph glands, swollen abdomen, tiredness, loss of appetite, frequent infections, and weight loss. Positron emission tomography (PET) and computed tomography (CT) scans, along with MRI, are widely used for the diagnosis of lymphoma. Advanced blood and lymph node biopsy tests are used to evaluate treatment effect on blood cells and to confirm the diagnosis of lymphoma, respectively. Current treatment options include chemotherapy, radiotherapy, and bone marrow/stem cell transplantation. Development of new treatment options for cancer medications includes small molecules and monoclonal antibodies for immunotherapy. In addition, the discovery of new phytochemical agents used in complementary and alternative medicine adds perspective to the treatment of lymphoma.
Early suspicion, withholding steroids, stereotactic biopsy, and high-dose methotrexate (HD-MTX) are essential for the treatment of primary CNS lymphoma (PCNSL) making its management in lower-middle-income countries (LMIC) challenging. Novel radiological methods, clinician awareness about the disease, and utilization of drugs like thiotepa and ibrutinib which can be given on an outpatient basis may allow better management of these patients in resource-poor settings. Combined with a late presenting demographic, this results in poorer outcomes in the Indian subcontinent as compared to its western counterparts. In this review, we summarize the currently available data on PCNSL in the Indian subcontinent. We also review the current standard of care for PCNSL and present potential modifications or research areas that may potentially improve outcomes in LMIC.
