Sunday, November 27, 2022

What Is Lymphoma Of The Stomach

Research And Targeted Treatments

Lymphoma – Experience of Symptoms and Diagnosis

MALT lymphoma is uncommon so most clinical trials also include people with other types of low-grade lymphoma, including other marginal zone lymphomas.

There are several targeted treatments being tested in clinical trials for marginal zone lymphomas, including drugs already approved for other types of lymphoma. These include:

  • BTK inhibitors such as ibrutinib, acalabrutinib and zanubrutinib, which block signals that B cells send to help them stay alive and divide
  • PI3K inhibitors such as idelalisib, umbralisib and copanlisib, which block a protein involved in the growth and survival of lymphoma cells
  • immunomodulators such as lenalidomide, which change how your immune system works
  • proteasome inhibitors such as bortezomib, which disrupt the balance of proteins in lymphoma cells, interfering with chemical signals between lymphoma cells and leading to cell death
  • new antibody treatments such as obinutuzumab, ublituximab or varlilumab, which bind to proteins on the surface of lymphoma cells to help your own immune system destroy them.

Some of these might be available to you through a clinical trial. If you are interested in taking part in a clinical trial, ask your doctor if there is a trial that might be suitable for you. To find out more about clinical trials or to search for a trial that might be suitable for you, visit Lymphoma TrialsLink.

A New Malt Lymphoma Prognostic Index

In September 2017, Thieblemont et al have published the results of a new prognostic index for MALT diagnosed patients. The 3 individual features maintaining the greatest prognostic significance were age > 70 years, Ann Arbor stage III or IV, and an elevated LDH. This index identified 3 groups: low, intermediate, and high risk . The 5-year event-free survival rates in the low-, intermediate-, and high-risk groups were 70%, 56%, and 29%, respectively. The prognostic utility was retained in both gastric and nongastric lymphomas and was confirmed in the validation set, being an important tool to identify patients with MALT lymphoma at risk of poor outcomes.

How Is Malt Lymphoma Treated

Treatment is tailored to the type, stage and grade. Most slow-growing, localised MALT lymphomas respond well to treatment. Local therapies such as radiation therapy or surgery are used with early stage MALT lymphomas that occur in areas other than the stomach. More advanced MALT lymphomas are usually treated with chemotherapy regimens or with single-agent chemotherapy. Targeted therapy may also be used, either on its own or in combination with chemotherapy. People with gastric MALT lymphoma who are infected with H. pylori can achieve lengthy remission in most cases, once the infection is effectively treated with antibiotics. These work to shrink the lymphoma. Drugs that lower the production of acid in the stomach may also be given in conjunction with antibiotics. People with gastric MALT lymphoma which is not progressing may be observed without being treated initially. This is known as the watch and wait approach.

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Rare Types Of Stomach Cancer

Lymphomas are cancers of the immune system tissue that may start anywhere lymph tissues are found, including in the stomach. However, lymphomas in the stomach are rare, accounting for about 4 percent of all stomach cancers.

MALT lymphoma is a type of non-Hodgkin lymphoma that typically occurs in the stomach. The cancer starts in lymph tissue that lines the stomach.

Most people who develop MALT lymphoma of the stomach have had a bacterial infection, especially one caused by helicobacter pylori , or a viral infection. This cancer tends to be slow-growing , and is usually detected in the early stages. If its caused by an H. pylori infection, the cancer is often treated with antibiotics.

If the cancer doesnt respond to antibiotics, other treatment options include:

  • Radiation therapy
  • Targeted therapy
  • Surgery

Gastrointestinal stromal tumors, or GISTs, are a rare type of stomach cancer that forms in a special cell found in the lining of the stomach called interstitial cells of Cajal . Under a microscope, GIST cells look similar to muscle or nerve cells. These tumors may develop throughout the digestive tract, but 60 percent to 70 percent occur in the stomach. In the United States, 4,000 to 6,000 new GIST cases throughout the GI tract are diagnosed per year, according to the ACS.

GISTs in the stomach may cause:

  • Abdominal pain
  • Poor appetite
  • Unexplained weight loss

Diagnosis may involve:

The three types of gastric carcinoid tumors are:

Treatment options include:

  • Stomach pain

Pathogenesis Of Malt Gastric Lymphoma

A case of gastric mucosa

Association with chronic H pylori

The literature has reported that approximately 75% of H pylori-positive gastric MALT lymphomas obtain complete remission after the eradication of these bacteria with antibiotic therapy, supporting the association between H pylori infection and the presence of MALT., Lymphoid cells attract to gastric MALT tissue by a chronic H pylori infection. When these cells are continuously stimulated by H pylori, they can give rise to MALT lymphomas. In addition to B cells, T cells and macrophages play an important role in MALT lymphomagenesis., Overtime, B-cell clones that still depend on antigens for growth and survival, bearing unknown mutations, will emerge. At this stage, the proliferation is monoclonal but not yet able to spread beyond the site of inflammation. With the acquisition of additional mutations, including chromosomal abnormalities, the tumor becomes antigen independent and capable of systemic spread.

In H pylori-negative gastric MALT lymphomas, the theory that infection leads to lymphomagenesis and the presence of lymphoma loses validity. Today, many feel there are various mechanisms by which pathogenesis occurs in H pylori-negative gastric MALT, including the relationship between genetic alterations ) and other activation pathways.

Genetic and molecular alterations

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Outlook For Malt Lymphoma

MALT lymphoma develops slowly and treatment is usually successful. Although the lymphoma often relapses , it can be treated again to keep it under control. Most people live with this type of lymphoma for many years. You might have periods when you feel well and dont need treatment, and other periods when your symptoms get worse and you need more treatment.

Your doctor is best placed to advise you on your outlook based on your individual circumstances.

Treatment Of Dlbcl Of The Stomach

Treatment of choice for DLBCL irrespective of anatomic site of the lesion is rituximab plus anthracycline-based combination chemotherapy: epirubicin, or adriamycin or mitoxantrone combined with cyclophosphamide, vincristine and prednisone . Although the impact of the addition of rituximab to chemotherapy regimens has not been tested in large clinical trials in patients with PG-DLBCL , treatment must include rituximab due to its proven therapeutic benefit in DLBCL . Complications of chemotherapy include gastric outlet obstruction and bleeding while gastric perforation is rare. Therefore, irrespective of the role of gastrectomy as primary treatment of patients with DLBCL of the stomach which as explained below remains controversial, the role of surgical consultant remains essential in the management of DLBCL of the stomach.

The role of consolidation radiotherapy is debated. In retrospective studies, the addition of RT was associated with a lower local relapse rate compared with chemotherapy alone . In a prospective study, the combination of six cycles of CHOP-14 followed by involved-field RT has been associated with a survival rate at 42 months of 91% . Further prospective randomized trials are required in order to answer the question about the role of RT in the treatment of PG-DLBCL.

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Symptoms From Lymphoma Affecting The Brain

Lymphomas of the brain, called primary brain lymphomas, can cause headache, trouble thinking, weakness in parts of the body, personality changes, and sometimes seizures.

Other types of lymphoma can spread to the area around the brain and spinal cord. This can cause problems such as double vision, facial numbness, and trouble speaking.

Taking Care Of Yourself

Gastric MALT Lymphoma

Lymphoma treatment can cause side effects. Talk to your medical team about ways to relieve any symptoms you have.

Also ask your doctor about changes to your diet and exercise that can help you feel better during your treatment. Ask a dietitian for help if you’re not sure what types of food to eat. Exercises like walking or swimming can relieve fatigue and help you feel better during treatments like chemotherapy and radiation. You might also try alternative therapies like relaxation, biofeedback, or guided imagery to help relieve pain.

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Side Effects Of Treatment

Treatments affect people differently. Each type of treatment or drug has a different set of possible side effects. Your medical team should give you information about the side effects associated with any treatment they recommend for you. Ask for more information if you are worried about potential side effects. Your medical team can offer advice or prescribe medicines if you experience troublesome side effects during your lymphoma treatment.

When you are in remission after your treatment, or during a period of active monitoring , you have regular follow-up appointments in the clinic.

Your follow-up appointments are to check that:

  • you are recovering well from treatment
  • you have no signs of the lymphoma coming back or getting worse
  • you are not developing any late effects .

At each appointment, your doctor examines you and asks if you have any concerns or symptoms. You might have blood tests. If you had gastric MALT lymphoma, you might have an endoscopy every 3 to 6 months for the first 2 years after your treatment. You might have other tests depending on where your lymphoma affected you.

You are unlikely to have a scan unless you have troubling symptoms.

Lymphoma Of The Stomach

Epidemiology

Gastric lymphoma represents about 5% of all malignant gastric tumors and is increasing in incidence. Most gastric lymphomas are non-Hodgkin’s lymphomas , and the stomach is the most common extranodal site for non-Hodgkin’s lymphomas. Patients with gastric lymphoma are generally younger than those with gastric adenocarcinoma, but the male predominance remains.

Clinical Manifestations

Patients commonly present with symptoms and signs similar to those of gastric adenocarcinoma. Lymphoma in the stomach can be a primary tumor, or it can be secondary to disseminated lymphoma.

B-cell lymphomas of the stomach are most commonly large cell with a high-grade type. Low-grade variants are noted in the setting of chronic gastritis and are termed mucosa-associated lymphoid tissue lymphomas. MALT lesions are strongly associated with H. pylori infection.

Diagnosis

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Pathogenesis Of Gastric Dlbcl

Gastric DLBCL is sometimes called high-grade gastric lymphoma. Compared to low-grade MALT lymphoma, high-grade gastric lymphoma is reported to be associated with a lower complete remission rate and shorter survival. It has remained unclear whether DLBCL arises de novo in the stomach or whether it transforms from low-grade MALT lymphomas.,

Oncogene Bcl-6 is frequently present in the majority of extranodal high-grade lymphomas. An overexpression of this gene could explain the development of both gastric DLBCL and DLBCL developing in other sites. Bcl-6 promoter region could be altered due translocations, somatic hypermutations, or deregulating mutations. These genetic rearrangements cause an overexpression of the gene, which seems to predict a better prognosis.

High levels of Bcl-6 expression were detected in germinal center B-cell like cases, independent of Bcl-6 genetic aberrations. In the rest of non-GCB lymphomas, mutations that produce Bcl-6 deregulating were correlated importantly with a high Bcl-6 expression level. However, no correlation was found between survival rates and the Bcl-6 expression level in the non-GCB cases.

Pathogenesis Of Dlbc Gastric Lymphomas

Lymphoma Of The Stomach Photograph by Medimage/science ...

In a high percentage of extra-nodal high-grade lymphomas, the oncogene BCL6 is altered due to translocations, somatic hyper-mutations or deregulating mutations involving the promoter region. These re-arrangements induce an over-expression of the gene, which seems to predict a better prognosis . This information also appears to be true for diffuse large B-cell gastric lymphomas which, analogous to a DLBCL developing in any other site, can be immuno-histochemically subclassified into at least two subgroups: GCB and non-GCB DLBCL. In gastric large-cell lymphomas, high BCL-6 expression was detected in all GCB cases, irrespective of BCL6 genetic alterations. In the non-GCB subgroup, BCL6 deregulating mutations correlated significantly with a high BCL-6 expression level. No noteworthy correlation was found between the BCL-6 expression level and survival in the non-GCB cases, which had significantly poorer outcomes than the GCB subgroup .

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Initial Symptoms Of Lymphoma

Painless swelling in the upper body lymph nodes, i.e., the neck, collarbone region, armpits or groin. This checklist lists the most common symptoms of lymphoma:

  • Chills/temperature swings
  • Persistent itch all over the body without an apparent cause or rash
  • General fatigue

More advanced lymphoma may present with the following symptoms:

  • In certain instances, people feel pain in the lymph nodes after drinking alcohol.
  • If the lymphoma involves lymphatic tissue within the abdomen, bowel or stomach, fluid may build up causing swelling near the intestines, potentially leading to sensations of abdominal pressure, pain, diarrhea and/or indigestion.
  • The enlarged lymph node sometimes causes other symptoms by pressing against a vein , or against a nerve .
  • Some people experience lower back pain that is unexplained. It is thought that this may be caused by expanding lymph nodes pressing on nerves.
  • As lymphomas progress and cancerous lymphocytes spread beyond the lymphatic system, the body loses its ability to fight infection. The generalized symptoms that develop may be confused with signs of influenza, tuberculosis, other infections such as infectious mononucleosis or other cancers.

General Histological And Immunophenotypic Characteristics

Non-Hodgkin lymphomas histological classification has been one of the most controversial problems in the field of Haematoncology. Primary extra-nodal forms were specifically taken into consideration for the first time in 1994, with the REAL classification â the first to identify different clinicalâpathological realities according to all the information available at that time: morphological, immunophenotypic, genetic and clinical . The World Health Organization classification originates directly from the REAL classification and distinguishes over 30 different forms, and, as a result, constitutes a very heterogeneous group of lympho-proliferative tumours having a wide variety of biological, clinical and response-to-treatment behaviour patterns .

Two histological types are particularly frequent in primary extra-nodal presentations. In fact, as opposed to their nodal counterpart, they are characterised by a preponderance of diffuse large B-cell lymphomas and marginal zone B-cell lymphomas of the MALT-type . Follicular, mantle cell and peripheral T-cell lymphomas are hardly represented at all .

Histological type

The t translocation involves genes in the heavy chains of immunoglobulins and the MALT1 gene. It is present in approximately 20% of MALT lymphomas, and its incidence appears to vary according to the disease site, being common in lymphomas localised in the salivary glands and ocular adnexa but extremely rare in the gastro-intestinal tract .

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Enhancing Healthcare Team Outcomes

Research is limited, given the low incidence of gastric lymphoma. More clinical trials are needed to develop a uniform staging system and define the usefulness of various imaging techniques when staging gastric lymphoma. Education and incorporation of many specialties, including gastroenterology, radiology, hematology, oncology, and surgical oncology, are paramount in progressing the diagnosis and treatment of gastric MALT lymphoma. Personalized and targeted treatments appear to be the future in the management of gastric lymphomas.

Article Details

How Gastric Malt Lymphoma Is Treated

H. pylori and gastric MALT lymphoma (HIGAN Lecture 1)

Gastric lymphoma is a form of non-Hodgkin lymphoma that arises from tissue that forms the lining of the stomach, the mucosa-associated lymphoid tissue . Because gastric MALT lymphoma usually is caused by Helicobacter pylori bacteria, it often can be treated successfully with antibiotics.

For the minority of cases that are not associated with H. pylori infection, or those that do not respond to antibiotics, other treatment options include radiation, chemotherapy, and surgery. What’s more, because gastric MALT lymphoma is such a slow-growing cancer, many people with the disease are able to forgo treatment altogether and can instead be monitored closely by their doctor.

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What Is Diffuse Large B

  • Diffuse Large B-Cell Lymphoma of Stomach is a rare, B-cell non-Hodgkins lymphoma that affects older adults. It is a subtype of lymphoma of stomach that is more aggressive and rapid-growing than other subtypes
  • In majority of cases, the lymphoma is a type of primary non-Hodgkin lymphoma. This means that it first involves the stomach and later can involve other parts of the body including the lymph nodes and bone marrow
  • DLBCL of Stomach may be associated with autoimmune disorders. However, the cause of the condition is unknown. It is reported that certain genetic factors may be involved in its formation and development
  • The lymphoma can cause abdominal pain, diarrhea, gastrointestinal tract bleeding, swollen stomach, and other general signs and symptoms, such as anemia, fatigue, and appetite loss
  • Systemic therapy for Diffuse Large B-Cell Lymphoma of Stomach may be employed, if there is the involvement of other organs. The treatment modality may include chemotherapy, radiation therapy, and targeted therapy as necessary
  • The prognosis depends on many factors including the progression of the condition, response to treatment, and overall health of the individual. In general, the prognosis of Diffuse Large B-Cell Lymphoma of Stomach is guarded and is mainly based on the stage of the tumor

General information on lymphoma and lymphocytes:

  • There are 3 different kinds of lymphocytes:
  • What Are The Possible Complications Of Lymphoma Of Stomach

    The complications due to Lymphoma of Stomach or Gastric Lymphoma may include:

    • Involvement of local and distant organs: It can lead to systemic or disseminated disease in some cases
    • Loss of function of the organ/area to which cancer has spread due to systemic involvement
    • If the abdomen is affected, it can cause intestinal obstruction that results in urine outflow obstruction and kidney damage
    • Weakened immune system can be a complication, which can become more severe during treatment. Due to this, individuals are more vulnerable to infections there is an increased risk of developing serious complications from such infections
    • Occasionally, the tumor can transform into a more aggressive form or subtype of lymphoma

    There may be complications related to chemotherapy used in treating the condition, which may include:

    • Side effects such as dizziness, vomiting, appetite loss, mouth ulcers, and hair loss
    • The treatment can also cause infertility in men and women. Hence, measures to protect the individualâs fertility must be considered, before starting chemotherapy

    The treatment measures can also give rise to secondary cancers, such as skin cancer.

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