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Revision as of 20:22, 8 January 2018

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].^[2]

Most common cause of primary gastric lymphoma is mucosa-associated lymphoid tissue (MALT) 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 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.

Demographics

Primary gastric lymphoma commonly affects elderly patients in the fifth or sixth decade of life.^[3]

Primary gastric lymphoma is more prevalent in men than in women.

Clinical presentation

Symptoms

Common symptoms of primary gastric lymphoma include:

Epigastric discomfort
Anorexia
Weight loss
Nausea associated with or without vomiting
Occult gastrointestinal bleeding
Early satiety

Less common symptoms include :

Physical Examination

Patients with primary gastric lymphoma usually appear normal.
Physical examination of patients with primary gastric lymphoma is usually remarkable for palpable mass and peripheral lymphadenopathy.

Diagnosis

Upper gastrointestinal endoscopy with biopsy is the gold standard test for the diagnosis of gastric lymphoma.
Laparotomy and laparoscopy should be performed when the patient presents with complications such as perforation or obstruction.
Findings on upper gastrointestinal endoscopy suggestive of gastric lymphoma:
- Mucosal erythema
- A mass or polypoid lesion with or without ulceration
- Benign-appearing gastric ulcer
- Nodular lesion
- Thickened, cerebroid gastric folds

An endoscopic ultrasound (EUS) will help assess the depth of lymphoma invasion and involvement of perigastric nodes. MALT lymphoma presents as superficial spreading or diffuse infiltrating lesion whereas diffuse large B cell lymphoma has a typical mass forming lesion appearance on endoscopic ultrasound. 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

Most of the gastric lymphomas are predominantly non-Hodgkin’s lymphoma of B-cell origin. Primary gastric lymphoma can vary from being well-differentiated, low-grade lymphomas to high grade or large cell lymphomas.

Risk Factors

Risk factors for gastric lymphoma include the following:^[4]

Helicobacter pylori
Long-term immunosuppressant drug therapy
HIV infection

Differential Diagnosis

Gastric lymphoma must be differentiated from conditions with similar presentation like acute gastritis, chronic gastritis, atrophic gastritis, Crohn's disease, gastroesophageal reflux disease, peptic ulcer disease, gastrinoma and gastric adenocarcinoma :^[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	Nausea & Vomiting	Heartburn	Belching or Bloating	Weight loss	Loss of Appetite	Stools	Endoscopy findings
Primary gastric lymphoma	H. pylori infection	Abdominal pain Chest pain	-	-	-	-	-	?	-	-	Useful in collecting the tissue for biopsy	Other symptoms Painless swollen lymph nodes in neck and armpit Night sweats Fatigue Fever Cough or trouble breathing
Acute gastritis	H. pylori NSAIDS Corticosteroids Alcohol Spicy food Viral infections Crohn's disease Autoimmune diseases Bile reflux Cocaine use Breathing machine or ventilator Ingestion of corrosives	Epigastric pain	Food	Antacids	?	?	?	-	?	Black stools	Pangastritis or antral gastritis Erosive (Superficial, deep, hemorrhagic) Nonerosive (H. pylori)	-
Chronic gastritis	H. pylori Alcohol Medications Autoimmune diseases Chronic stress	Epigastric pain	Food	Antacids	?	?	?	?	?	-	H. pylori gastritis Atrophy Intestinal metaplasia Lymphocytic gastritis Enlarged folds Aphthoid erosions	-
Atrophic gastritis	H. pylori Autoimmune disease	Epigastric pain	-	-	?	-		?	?	-	H. pylori Mucosal atrophy Autoimmune Mucosal atrophy	Iron deficiency anemia Autoimmune gastritis diagnosis include: Antiparietal and anti-IF antibodies Achlorhydria and hypergastrinemia Low serum cobalamine
Crohn's disease	Autoimmune disease	Abdominal pain	-	-	-	-	-	?	?	Chronic diarrhea often bloody with pus or mucus Rectal bleeding	Mucosal nodularity with cobblestoning Multiple aphthous ulcers Linier or serpiginous ulcerations Thickened antral folds Antral narrowing Hypoperistalsis Duodenal strictures	Fever Fatigue Anemia (pernicious anemia)
GERD	Lower esophageal sphincter abnormalities Hiatal hernia Abnormal esophageal contractions Prolonged emptying of stomach Gastrinomas	Epigastric pain	Spicy food Tight fitting clothing	Antacids Head elevation during sleep	? (Suspect delayed gastric emptying)	?	-	-	-	-	Esophagitis Barrette esophagus Strictures	Other symptoms: Dysphagia Regurgitation Nocturnal cough Hoarseness Complications Esophagitis Strictures Barrette esophagus
Peptic ulcer disease	H. pylori Smoking Alcohol Radiation therapy Medications Zollinger-ellison syndrome	Epigastric pain sometimes extending to back Right upper quadrant pain	Duodenal ulcer Pain aggravates with empty stomach Gastric ulcer Pain aggravates with food	Antacids Duodenal ulcer Pain alleviates with food	?	?	-	-	-	Black stools	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 Well-demarcated break in the mucosa that may extend into the muscularis propria of the duodenum Found in the first part of duodenum <1cm	Other diagnostic tests Serum gastrin levels Secretin stimulation test Biopsy
Gastrinoma	Associated with MEN type 1	Abdominal pain	-	-	? (suspect gastric outlet obstruction)	?	-	-	-	Black stools	Useful in collecting the tissue for biopsy	May present with symptoms of GERD or peptic ulcer disease Associated with MEN type 1 Diagnostic tests Serum gastrin levels Somatostatin receptor scintigraphy CT and MRI
Gastric Adenocarcinoma	H. pylori infection Smoked and salted food	Abdominal pain	-	-	?	?	?	?	?	Black stools, or blood in stools	Esophagogastroduodenoscopy Multiple biopsies are taken to establish the diagnosis	Other symptoms Dysphagia Early satiety Frequent burping

Treatment

The predominant therapy for diffuse large B-cell lymphomas of the stomach is chemotherapy.
Second line therapy for MALT lymphomas is usually chemotherapy with a single agent, and complete response rates of greater than 70% have been observed.
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

↑ Dawson IMP, Cornes JS, Morrison BC. Primary malignant lymphoid tumours of the intestinal tract. Br J Surg. 1961;49:80-89.
↑ Aisenberg AC. Coherent view of non-Hodgkin's lymphoma. J Clin Oncol. 1995;13:2656-2675.
↑ Thirlby RC. Gastrointestinal lymphoma: a surgical perspective. Oncology (Huntingt). 1993;7:29-32.
↑ NEJM article
↑ 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.
↑ Sipponen P, Maaroos HI (2015). "Chronic gastritis". Scand J Gastroenterol. 50 (6): 657–67. doi:10.3109/00365521.2015.1019918. PMC 4673514. PMID 25901896.
↑ 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.
↑ Sipponen P (1989). "Atrophic gastritis as a premalignant condition". Ann Med. 21 (4): 287–90. PMID 2789799.
↑ 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.
↑ Ramakrishnan K, Salinas RC (2007). "Peptic ulcer disease". Am Fam Physician. 76 (7): 1005–12. PMID 17956071.
↑ 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.
↑ 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.
↑ 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.

Template:Gastroenterology Template:Tumors

Template:WikiDoc Sources

[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

[pmid6710074-5] 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.

[pmid25901896-6] Sipponen P, Maaroos HI (2015). "Chronic gastritis". Scand J Gastroenterol. 50 (6): 657–67. doi:10.3109/00365521.2015.1019918. PMC 4673514. PMID 25901896.

[pmid16819502-7] 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.

[pmid2789799-8] Sipponen P (1989). "Atrophic gastritis as a premalignant condition". Ann Med. 21 (4): 287–90. PMID 2789799.

[pmid25133039-9] 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.

[pmid17956071-10] Ramakrishnan K, Salinas RC (2007). "Peptic ulcer disease". Am Fam Physician. 76 (7): 1005–12. PMID 17956071.

[pmid17985090-11] 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.

[pmid15621988-12] 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.

[pmid21390139-13] 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.

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@@ Line 14: / Line 14: @@
 [[Image:Gastric MALT lymphoma 2.jpg|thumb|left|300px|Endoscopic image of gastric [[MALT lymphoma]] taken in body of [[stomach]] in patient who presented with [[upper gastrointestinal bleed|upper GI hemorrhage]].  Appearance is similar to [[gastric ulcer]] with adherent clot.]]
 ===Demographics===
-Primary gastric lymphoma commonly affects elderly patients in the fifth or sixth decade of life.<ref>Thirlby RC. Gastrointestinal lymphoma: a surgical perspective. Oncology (Huntingt). 1993;7:29-32.</ref>
+* Primary gastric lymphoma commonly affects [[elderly]] [[patients]] in the fifth or sixth decade of [[life]].<ref>Thirlby RC. Gastrointestinal lymphoma: a surgical perspective. Oncology (Huntingt). 1993;7:29-32.</ref>
-Primary gastric lymphoma is more prevalent in men than in women.
+* Primary gastric lymphoma is more [[Prevalence|prevalent]] in [[men]] than in [[women]].
 ===Clinical presentation===
 ====Symptoms====
-Common symptoms of primary gastric lymphoma include:
+Common [[symptoms]] of primary gastric lymphoma include:
-*Epigastric discomfort
+*[[Epigastric]] [[discomfort]]
-*Anorexia
+*[[Anorexia]]
-*Weight loss
+*[[Weight loss]]
-*Nausea associated with or without vomiting
+*[[Nausea]] associated with or without [[vomiting]]
-*Occult gastrointestinal bleeding
+*[[Occult]] [[gastrointestinal bleeding]]
-*Early satiety
+*[[Early satiety]]
-Less common symptoms include :
+Less common [[symptoms]] include :
-*Fever
+*[[Fever]]
-*Night sweats
+*[[Night sweats]]
 ====Physical Examination====
-*Patients with primary gastric lymphoma usually appear normal.
+*[[Patients]] with primary gastric lymphoma usually appear normal.
-*Physical examination of patients with primary gastric lymphoma is usually remarkable for palpable mass and peripheral lymphadenopathy.
+*[[Physical examination]] of [[patients]] with primary gastric lymphoma is usually remarkable for [[palpable]] [[mass]] and [[Lymphadenopathy|peripheral lymphadenopathy]].
 ===Diagnosis===
-*Upper gastrointestinal endoscopy with biopsy is the gold standard test for the diagnosis of gastric lymphoma.
+*[[Endoscopy|Upper gastrointestinal endoscopy]] with [[biopsy]] is the gold standard test for the [[diagnosis]] of [[gastric]] [[lymphoma]].
-*Laparotomy and laparoscopy should be performed when the patient presents with complications such as perforation or obstruction.
+*[[Laparotomy]] and [[laparoscopy]] should be performed when the [[patient]] presents with [[complications]] such as [[perforation]] or [[obstruction]].
-*Findings on upper gastrointestinal endoscopy suggestive of gastric lymphoma:
+*Findings on [[Endoscopy|upper gastrointestinal endoscopy]] suggestive of [[gastric]] [[lymphoma]]:
-**Mucosal erythema
+**[[Mucosal]] [[erythema]]
-**A mass or polypoid lesion with or without ulceration
+**A [[mass]] or [[Polypoidy|polypoid]] [[Lesions|lesion]] with or without [[ulceration]]
-**Benign-appearing gastric ulcer
+**[[Benign]]-appearing [[gastric]] [[ulcer]]
-**Nodular lesion
+**[[Nodular]] [[Lesions|lesion]]
-**Thickened, cerebroid gastric folds
+**Thickened, cerebroid [[gastric]] folds
-An endoscopic ultrasound (EUS) will help assess the depth of lymphoma invasion and involvement of perigastric nodes. MALT lymphoma presents as superficial spreading or diffuse infiltrating lesion whereas diffuse large B cell lymphoma has a typical mass forming lesion appearance on endoscopic ultrasound.
+An [[Endoscopic ultrasound|endoscopic ultrasound (EUS)]] will help assess the [[Depth of field|depth]] of [[lymphoma]] [[invasion]] and involvement of [[Lymph nodes|perigastric nodes]]. [[MALT lymphoma]] presents as [[superficial]] spreading or [[diffuse]] infiltrating [[lesion]] whereas [[diffuse]] [[Diffuse large B cell lymphoma|large B cell lymphoma]] has a [[Mass|typical mass]] forming [[lesion]] appearance on [[Endoscopy|endoscopic]] [[ultrasound]]. [[Imaging]] [[Investigational product|investigations]] including [[CT]] [[Scan|scans]] or [[endoscopic ultrasound]] are useful to stage [[disease]].  [[Hematological]] parameters are usually checked to assist with [[Staging (pathology)|staging]] and to exclude concomitant [[leukemia]].  An elevated [[lactate dehydrogenase|LDH]] level may be suggestive of [[lymphoma]].
-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 [[lactate dehydrogenase|LDH]] level may be suggestive of lymphoma.
 ===Histopathology===
-Most of the gastric lymphomas are predominantly non-Hodgkin’s lymphoma of B-cell origin. Primary gastric lymphoma can vary from being well-differentiated, low-grade lymphomas to high grade or large cell lymphomas.
+Most of the gastric lymphomas are predominantly [[Non-Hodgkin lymphoma|non-Hodgkin’s lymphoma]] of [[B-cell|B-cell origin]]. Primary gastric lymphoma can vary from being well-differentiated, low-grade [[lymphomas]] to high grade or large cell [[lymphomas]].
 ===Risk Factors===
-Risk factors for gastric lymphoma include the following:<ref>[http://content.nejm.org/cgi/content/full/330/18/1267 NEJM article]</ref>
+[[Risk factors]] for gastric [[lymphoma]] include the following:<ref>[http://content.nejm.org/cgi/content/full/330/18/1267 NEJM article]</ref>
 *''[[Helicobacter pylori]]''
-*Long-term immunosuppressant drug therapy
+*Long-term [[immunosuppressant]] [[drug]] [[therapy]]
-*[[HIV]] infection
+*[[HIV]] [[infection]]
 ==Differential Diagnosis==
@@ Line 342: / Line 342: @@
 ==Treatment==
-*The predominant therapy for diffuse large B-cell lymphomas of the stomach is chemotherapy.
+*The predominant [[therapy]] for [[Diffuse large B-cell lymphoma|diffuse large B-cell lymphomas]] of the [[stomach]] is [[chemotherapy]].
-*Second line therapy for MALT lymphomas is usually chemotherapy with a single agent, and complete response rates of greater than 70% have been observed.
+*Second line [[therapy]] for [[MALT lymphoma|MALT lymphomas]] is usually [[chemotherapy]] with a single agent, and complete response rates of greater than 70% have been observed.
-*Chemotherapy includes treatment with CHOP with or without rituximab.
+*[[Chemotherapy]] includes [[Treatments|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.
+*The mainstay of [[therapy]] for [[MALT lymphoma|MALT lymphomas]] is [[antibiotic]] treatment to eradicate [[H.pylori peptic ulcer disease pathophysiology|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.
+*Single drug [[chemotherapy]] is recommended as a second line therapy for [[MALT lymphoma|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.
+*In case of complications like [[gastric outlet obstruction]], the recommended treatment is [[Gastrectomy|subtotal gastrectomy]] followed by post-operative.
 ==References==