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Gastric Lymphoma

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Primary Gastric Lymphoma (PGL)

  • Epidemiology:
    • Accounts for 15% of gastric malignancies and 2% of all lymphomas.
    • Most common symptom: Abdominal pain.
    • 50% of patients present with anemia.
    • Antrum is the most common site.
    • More common in males, typically in the 6th and 7th decades.
    • Stomach is the most common site for GI lymphomas.

Principles of Treatment

  • Surgery:
    • Primary treatment for small and large intestinal lymphomas due to the high rate of complications.
  • CRT (Chemoradiotherapy):
    • Can be used without surgery in most cases of primary gastric lymphomas.

Pathology

  1. Diffuse Large B-Cell Lymphoma (DLBCL):
    • 55% of cases.
  2. MALToma:
    • 40% of cases.
    • 90% association with H. pylori infection.
  3. Burkitt's Lymphoma:
    • 3% of cases, aggressive, affects younger patients.
  4. Mantle Cell Lymphoma:
    • 1% of cases.
  5. Follicular Lymphoma:
    • 1% of cases, common in the duodenum.

Types by Site

  • Stomach:
    • DLBCL, MALToma.
  • Duodenum:
    • Follicular lymphoma.
  • Ileum:
    • Mantle cell lymphoma, Burkitt's lymphoma.

Risk Factors

  • Immunodeficiency.
  • H. pylori infection (especially for MALToma).

Staging Systems

  • Ann Arbor, Rao, Mushoff: General lymphoma staging systems.
  • Lugano Classification and Paris System (TNM): More specific for GI lymphomas.

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Lugano Classification for GI Lymphomas

  • Constitutional Symptoms:
    • Categorized into A (absence) or B (presence) of systemic symptoms (e.g., fever, night sweats, weight loss).
  • Key Factors:
    • Depth of tumor invasion is a critical factor in staging.
    • Size of the tumor is noted, with X > 10 cm for large tumors.
  • Note:
    • No Stage III in the Lugano classification system for GI lymphomas.

Dawson's Criteria for Diagnosing Primary GI Lymphoma

  1. No palpable nodes in the body during the first clinical exam.
  2. Normal bone marrow biopsy and peripheral smear.
  3. No mediastinal nodes on chest X-ray.
  4. No liver or spleen involvement.
  5. Disease confined to the GI organ with only regional node involvement (e.g., perigastric nodes).

Treatment of Diffuse Large B-Cell Lymphoma (DLBCL)

  • Bulky DLBCL (>7.5 cm):
    • Six cycles of R-CHOP (Rituximab, Cyclophosphamide, Doxorubicin, Vincristine, and Prednisone).
    • Followed by Involved Site Radiotherapy (ISRT).
  • Non-Bulky DLBCL:
    • Two treatment options:
      1. Three cycles of R-CHOP followed by RT.
      2. Six cycles of R-CHOP without RT.

MALToma (Mucosa-Associated Lymphoid Tissue Lymphoma)

  • H. pylori:
    • 90% of MALTomas are associated with H. pylori infection.
    • t(11:18) translocation is found in 5-10% of cases.

MCQ 28: Primary Mode of Treatment for MALToma

Question:

What is the primary mode of treatment for MALToma?

  1. RT
  2. H. pylori eradication
  3. Rituximab
  4. R-CHOP

Answer:

2) H. pylori eradication

Explanation:

  • The primary treatment for MALToma associated with H. pylori is H. pylori eradication therapy. Other therapies like RT or Rituximab may be considered if this is not successful or if the tumor progresses.

MALToma – H. pylori Positive, t(11:18) Positive

  • H. pylori eradication is the first-line treatment, combined with ISRT (Involved Site Radiotherapy).
  • Rituximab can be used if radiotherapy is contraindicated.

MCQ 29: Treatment of MALToma if H. pylori Negative and t(11:18) Positive

Question:

What is the treatment of choice for MALToma if the patient is H. pylori negative and t(11:18) positive?

  1. ISRT
  2. Rituximab
  3. R-CHOP
  4. Resection

Answer:

1) ISRT

Explanation:

  • ISRT (Involved Site Radiotherapy) is the treatment of choice for MALToma when H. pylori is negative, especially in cases with the t(11:18) translocation. This translocation is associated with resistance to H. pylori eradication therapy.

Post-Chemotherapy Surveillance for MALToma

  • Endoscopy Schedule:
    • Every 3 months for the first 2 years.
    • Every 6 months during years 3-5.
    • Annually after 6 years.

MCQ 30: Lymphoma Involving Extrahepatic Biliary Tree

Question:

Which type of lymphoma involves the extrahepatic biliary tree?

a. Diffuse large cell

b. Mantle cell

c. MALToma

d. Follicular lymphoma

Answer:

d) Follicular lymphoma

Explanation:

  • Follicular lymphoma can occasionally involve the extrahepatic biliary tree, and spontaneous resolution is reported in about 23% of cases.

MCQ 31: Features of Primary GI Lymphoma – Exception

Question:

Which of the following is not a feature of primary GI lymphoma?

a. Absence of palpable nodes

b. Normal peripheral smear and BM biopsy

c. Absence of mediastinal nodes

d. Disease confined to affected segment and only regional nodes

e. Spleen or liver may be involved

Answer:

e) Spleen or liver may be involved

Explanation:

  • Primary GI lymphoma is defined by Dawson’s criteria, which include the absence of spleen or liver involvement. If the spleen or liver is involved, it is considered secondary lymphoma.

MCQ 32: Chemotherapy Regimen for Burkitt Lymphoma

Question:

The chemotherapeutic regimen used to manage Burkitt lymphoma of the small intestine after surgical excision is:

a. CHOP regimen

b. H. pylori regimen

c. CHOP + Rituximab

d. Prednisolone is replaced in CHOP by Methotrexate

Answer:

d) Prednisolone is replaced in CHOP by Methotrexate

Explanation:

  • Burkitt lymphoma is typically treated with an intensive regimen, where methotrexate replaces prednisolone in CHOP to enhance efficacy.

MCQ 33: Most Common Site for Burkitt’s Lymphoma

Question:

The most common site for Burkitt’s lymphoma is:

  1. Body and cardia of the stomach
  2. Duodenum
  3. Terminal ileum
  4. Jejunum

Answer:

3) Terminal ileum

Explanation:

  • The terminal ileum is the most common site for Burkitt’s lymphoma in the GI tract, often presenting as an aggressive, rapidly growing mass.

MCQ 34: MALToma – False Statement

Question:

Which of the following is false regarding MALToma?

  1. GI MALToma has an inferior prognosis compared to ocular MALToma
  2. 89% 5-year survival
  3. After treatment with Anti-H. pylori, follow-up endoscopy is done at 12 weeks
  4. Stage IV MALToma is treated by RT

Answer:

4) Stage IV MALToma is treated by RT

Explanation:

  • Stage IV MALToma is typically treated with R-CHOP, not radiotherapy (RT). RT is more effective in early-stage localized disease, while advanced stages require chemotherapy.