Gastric lymphoma

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Mazia Fatima, MBBS [2]

Overview

Primary gastric lymphoma is cancer derived from lymphocytes (a type of white blood cell) that originates in the stomach[1]

Most common cause of primary gastric lymphoma is mucosa-associated lymphoid tissue (MALT) gastric lymphoma and diffuse large B-cell lymphoma (DLBCL) of the stomach.These account for 90% of all diagnosed cases. Lymphomas originating outside the lymph nodes are referred to as extra nodal lymphoma. Primary gastric lymphoma is the most common type of extra nodal lymphoma.

Primary gastric Lymphoma is a rare condition.[2] Gastric lymphoma accounts for less than 15% of gastric malignancies and about 2% of all lymphomas. Most of the primary gastric lymphomas are B cell non-Hodgkin lymphoma(NHL) .

Primary Gastric Lymphoma

Endoscopic image of gastric MALT lymphoma taken in body of stomach in patient who presented with upper GI hemorrhage. Appearance is similar to gastric ulcer with adherent clot.

Clinical presentation

Primary gastric lymphoma commonly affects the elderly patients with peak incidence in the sixth decade of life[3] and presenting symptoms include epigastric pain, early satiety, fatigue and weight loss.

Diagnosis

These lymphomas are often difficult to differentiate from gastric adenocarcinoma. The lesions are usually ulcers with a ragged, thickened mucosal pattern on contrast radiographs.

The diagnosis is typically made by biopsy at the time of endoscopy. Several endoscopic findings have been reported, including solitary ulcers, thickened gastric folds, mass lesions and nodules. As there may be infiltration of the submucosa, larger biopsy forceps, endoscopic ultrasound guided biopsy, endoscopic submucosal resection, or laparotomy may be required to obtain tissue.

Imaging investigations including CT scans or endoscopic ultrasound are useful to stage disease. Hematological parameters are usually checked to assist with staging and to exclude concomitant leukemia. An elevated LDH level may be suggestive of lymphoma.

Histopathology

The majority of gastric lymphomas are non-Hodgkin's lymphoma of B-cell origin. These tumors may range from well-differentiated, superficial involvements (MALT) to high-grade, large-cell lymphomas.

Other lymphomas involving the stomach include mantle cell lymphoma and T-cell lymphomas which may be associated with enteropathy; the latter usually occur in the small bowel but have been reported in the stomach.

Risk Factors

Risk factors for gastric lymphoma include the following:

Differential Diagnosis

Gastric lymphoma must be differentiated from conditions with similar presentation like:[5][6][7][8][9][10][11][12][13]

Disease Cause Symptoms Diagnosis Other findings
Pain Nausea

&

Vomiting

Heartburn Belching or

Bloating

Weight loss Loss of

Appetite

Stools Endoscopy findings
Location Aggravating Factors Alleviating Factors
Acute gastritis Food Antacids ? ? ? - ? Black stools -
Chronic gastritis Food Antacids ? ? ? ? ? - H. pylori gastritis

Lymphocytic gastritis

  • Enlarged folds
  • Aphthoid erosions
-
Atrophic gastritis Epigastric pain - - ? - ? ? - H. pylori

Autoimmune

Autoimmune gastritis diagnosis include:

Crohn's disease - - - - - ? ?
  • Mucosal nodularity with cobblestoning
  • Multiple aphthous ulcers
  • Linier or serpiginous ulcerations
  • Thickened antral folds
  • Antral narrowing
  • Hypoperistalsis
  • Duodenal strictures
GERD
  • Lower esophageal sphincter abnormalities
  • Spicy food
  • Tight fitting clothing
?

(Suspect delayed gastric emptying)

? - - - - Other symptoms:

Complications

Peptic ulcer disease
Duodenal ulcer
  • Pain aggravates with empty stomach

Gastric ulcer

  • Pain aggravates with food
  • Pain alleviates with food
? ? - - - Gastric ulcers
  • Discrete mucosal lesions with a punched-out smooth ulcer base with whitish fibrinoid base
  • Most ulcers are at the junction of fundus and antrum
  • 0.5-2.5cm

Duodenal ulcers

Other diagnostic tests
Gastrinoma - - ?

(suspect gastric outlet obstruction)

? - - - Useful in collecting the tissue for biopsy

Diagnostic tests

Gastric Adenocarcinoma - - ? ? ? ? ? Esophagogastroduodenoscopy
  • Multiple biopsies are taken to establish the diagnosis
Other symptoms
Primary gastric lymphoma - - - - - ? - - Useful in collecting the tissue for biopsy Other symptoms

Treatment

The predominant therapy for diffuse large B-cell lymphomas of the stomach is chemotherapy.</ref>. Second line therapy for MALT lymphomas is usually chemotherapy with a single agent, and complete response rates of greater than 70% have gain been reported [14].

Chemotherapy includes treatment with CHOP with or without rituximab. The mainstay of therapy for MALT lymphomas is antibiotic treatment to eradicate H.pylori. Regression is seen in about 60% of cases with eradication therapy alone. Single drug chemotherapy is recommended as a second line therapy for MALT lymphomas and is associated with a complete resolution of symptoms in greater than 70% cases. In case of complications like gastric outlet obstruction, the recommended treatment is subtotal gastrectomy followed by post-operative.

References

  • Fauci, et al. Harrison's Principles of Internal Medicine,16th Ed.

Notes

  1. Dawson IMP, Cornes JS, Morrison BC. Primary malignant lymphoid tumours of the intestinal tract. Br J Surg. 1961;49:80-89.
  2. Aisenberg AC. Coherent view of non-Hodgkin's lymphoma. J Clin Oncol. 1995;13:2656-2675.
  3. Thirlby RC. Gastrointestinal lymphoma: a surgical perspective. Oncology (Huntingt). 1993;7:29-32.
  4. NEJM article
  5. Sugimachi K, Inokuchi K, Kuwano H, Ooiwa T (1984). "Acute gastritis clinically classified in accordance with data from both upper GI series and endoscopy". Scand J Gastroenterol. 19 (1): 31–7. PMID 6710074.
  6. Sipponen P, Maaroos HI (2015). "Chronic gastritis". Scand J Gastroenterol. 50 (6): 657–67. doi:10.3109/00365521.2015.1019918. PMC 4673514. PMID 25901896.
  7. Sartor RB (2006). "Mechanisms of disease: pathogenesis of Crohn's disease and ulcerative colitis". Nat Clin Pract Gastroenterol Hepatol. 3 (7): 390–407. doi:10.1038/ncpgasthep0528. PMID 16819502.
  8. Sipponen P (1989). "Atrophic gastritis as a premalignant condition". Ann Med. 21 (4): 287–90. PMID 2789799.
  9. Badillo R, Francis D (2014). "Diagnosis and treatment of gastroesophageal reflux disease". World J Gastrointest Pharmacol Ther. 5 (3): 105–12. doi:10.4292/wjgpt.v5.i3.105. PMC 4133436. PMID 25133039.
  10. Ramakrishnan K, Salinas RC (2007). "Peptic ulcer disease". Am Fam Physician. 76 (7): 1005–12. PMID 17956071.
  11. Banasch M, Schmitz F (2007). "Diagnosis and treatment of gastrinoma in the era of proton pump inhibitors". Wien Klin Wochenschr. 119 (19–20): 573–8. doi:10.1007/s00508-007-0884-2. PMID 17985090.
  12. Dicken BJ, Bigam DL, Cass C, Mackey JR, Joy AA, Hamilton SM (2005). "Gastric adenocarcinoma: review and considerations for future directions". Ann Surg. 241 (1): 27–39. PMC 1356843. PMID 15621988.
  13. Ghimire P, Wu GY, Zhu L (2011). "Primary gastrointestinal lymphoma". World J Gastroenterol. 17 (6): 697–707. doi:10.3748/wjg.v17.i6.697. PMC 3042647. PMID 21390139.
  14. Hammel P et al. Efficacy of single-agent chemotherapy in low-grade B-cell mucosa-associated lymphoid tissue lymphoma with prominent gastric expression. J Clin Oncol 1995 Oct;13(10):2524-9.


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