Lymphoma by m. Mohyi

8/6/2019 Lymphoma by m. Mohyi

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GASTROINTESTINAL

LYMPHOMA


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Lymphoma

Neoplastic (malignant) transformations of

normal lymphoid cells


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Lymphoma

Non-Hodgkin's lymphoma (NHL)

Hodgkin lymphoma (HL).


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NHL:

B cell Lymphoma.

T cell lymphoma.

NK cell lymphoma.


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Gastrointestinal Lymphoma

Localized disease involving the

gastrointestinal tract.

Presentation with gastrointestinal

symptoms or predominant lesions in the

GI tract.


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B cell lymphoma:

Indolent: IPSID.

Extranodal marginal zone B-cell lymphoma =MALT-type lymphoma.

Mantle cell lymphoma

Aggressive

Diffuse large B-cell lymphoma

Highly aggressive

Burkitt's lymphoma


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T cell lymphoma:

Aggressive:

Enteropathy-associated T-cell intestinal

lymphoma


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Gastrointestinal Lymphoma

Stomach 75 %

Small bowel (including duodenum) 9 %

Ileo-cecal region 7 % More than one GI site 6 %

Rectum 2 %

Diffuse colonic involvement 1 %


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GASTRIC LYMPHOMA Primary : Mucosa (gut)-associated lymphoid tissue tumor

Previously called

MALToma, MALT-type lymphoma, or MALT lymphoma,

Now called

Extranodal marginal zone B-cell lymphoma of MALT

type

Secondary

spread from adjacent lymph nodes


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GASTRIC LYMPHOMA

Predissposing factors:

Helicobacter pylori-associated chronicgastritis

Autoimmune diseases

Immunodeficiency syndromes (eg, AIDS) Long-standing immunosuppressive

therapy (eg, posttransplantation)


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GASTRIC LYMPHOMA

Symptoms:

Epigastric pain or discomfort

Anorexia

Weight loss

Nausea and/or vomiting Occult gastrointestinal bleeding, but

hematemesis and melena are uncommon

Early satiety


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GASTRIC LYMPHOMA

Physical examination:

Often normal Palpable mass

Peripheral lymphadenopathy


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GASTRIC LYMPHOMA

Laboratory studies

Tend to be normal at presentation. Anemia

ESR.


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GASTRIC LYMPHOMA

Upper endoscopy

A mass or polypoid lesion with or withoutulceration.

Benign-appearing gastric ulcer

Nodularity

Thickened, cerebroid gastric folds


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GASTRIC LYMPHOMA

Any suspicious appearing lesion should bebiopsied.

Conventional pinch biopsies may miss thediagnosis.

Jumbo biopsies,

Snare biopsies,

Biopsies within biopsies ("well technique"), EUS - guided fine needle aspiration biopsy

(FNAB)

Endoscopic submucosal resection.


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GASTRIC LYMPHOMA

Further evaluation:

CT chest and abdomen.

EUS for staging.

If not conclusive :

Laparoscopy or minilaparotomy.


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Intestinal lymphoma

Primary: 75 % of primary gastrointestinal

lymphomas in the Middle East and

Mediterraneans.

Depends upon its association withimmunoproliferative small intestinal

disease (IPSID).


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Intestinal lymphoma

Clinically:

Abdominal pain, Intussusception

Bowel obstruction

Small bowel lymphomas: weight loss andoccult bleeding,

Colonic and rectal lymphomas: overt oroccult bleeding and diarrhea


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Intestinal lymphoma

In IPSID lymphoma:

Alpha heavy chain paraproteinemia Lactose intolerance

Enteroenteric fistulae

Ascites Fever

Organomegaly.


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Intestinal lymphoma

Predisposing factors:

Autoimmune diseases Immunodeficiency syndromes (eg, AIDS)

Long-standing immunosuppressivetherapy (eg, posttransplantation)

Crohn's disease

Radiation therapy

Nodular lymphoid hyperplasia


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Intestinal lymphoma

CT with contrast:

IPSID: Diffuse infiltrating lesion, sometimesresembling cobblestoning.

More common in the proximal small intestine.

Western-type non-IPSID : Ulcerated, protruding, or infiltrating mass

lesions


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Intestinal lymphoma

Endoscopy

Proximal small bowel:"push" enteroscopy.

Intubation of the terminal ileum during

colonoscopy

Capsule endoscopy:

suggestive clinical presentations

suspicious radiographic findings.

Does not permit tissue sampling


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Intestinal lymphoma

Colonoscopy

Principal diagnostic modality for colorectallymphomas.

Colonoscopic findings: diffuse mucosalnodularity, colitis-like changes with

induration and ulceration, or a mass withor without ulceration.

Multiple biopsies of all lesions should beperformed using standard techniques.


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Intestinal lymphoma

Barium enema

Suggestthe diagnosis but cannot reliably

differentiate lymphoma from other conditions.

Findings on barium enema include :

Mass lesions with mucosal destruction

Mucosal nodularity

Infiltration of the submucosa with associatedstricture

Bulky extracolonic masses


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Intestinal lymphoma

Exploratory laparotomy

When the lesion is not accessible viaendoscopy or when endoscopic biopsies

are unavailable or non-diagnostic.


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Colorectal Lymphoma

Half of colorectal lymphomas arediscovered at an advanced stage and

have spread to other organs or to the bonemarrow.

Tumors that have metastasized are harder

to treat than ones that haven't.


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Mantle cell lymphoma (MCL)

Can involve the stomach, small intestine,

or colon.

The involvement of multiple sites,particularly the small intestine and colon,

is referred to as "lymphomatous

polyposis."

The mean age at presentation is 55 years,

with a male predominance.


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Clinicaly:

Abdominal pain,

diarrhea,

hematochezia,

weight loss,

fatigue.

Liver involvement in 26 %of the patients,

Malignant cells seen in the peripheralblood


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Endoscopywith biopsy is procedure of

choice.

upper gastrointestinal endoscopy. Push enteroscopy

Capsule endoscopy for abnormalities seen

in the more distal small bowel or forsymptomatic patients with negative

colonoscopy and standard upper

endoscopy.


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Typical small nodular or polypoid tumors

(2 mm to more than 2 cm in size), with or

without normal intervening mucosa, can

be seen by colonoscopy or enteroscopy.


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Large cerebroid folds may be seen in the

stomach.


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Barium radiographs of the small bowel

may show numerous round filling defects

in the lumen or a tumor mass in the

terminal ileum.


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Abdominal CT with oral contrast may

reveal ileocecal thickening, an obvious

mass which may cause obstruction,

retroperitoneal lymphadenopathy, and

hepatic or splenic enlargement.

CT-guided needle biopsy can be used for

an accessible mass; adequate tissue mustbe obtained in order to allow an accurate

diagnosis. section on Lymph node and

tissue biopsy).


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Burkitt's lymphoma

EndemicBurkitt's lymphoma

A disease of childhood with a peak incidence at abouteight years of age [

Gastrointestinal manifestations are infrequent but mayinclude obstruction or intussusception

"Burkitt-like" ("sporadic"Burkitt's) lymphoma

A wider age distribution, with only 50 % of cases

affecting children. It often presents with gastrointestinal

manifestations, particularly abdominal pain andobstructive symptoms caused by ileocecal

involvement


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Enteropathy-associated T-cell

lymphoma (EATL) Sequela of gluten-sensitive enteropathy (celiac sprue)

The mean age of patients with EATL is 60 years.

Most patients have a several month to several year history of

abdominal pain and weight loss, but only a small proportion have a

history of celiac disease dating back to childhood

Patients often present with acute bleeding, obstruction, or

perforation.

Clinical deterioration of celiac disease, despite compliance with a

gluten-free diet, should raise suspicion of the possible presence of

lymphoma.


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Endoscopy with biopsy

Large circumferential ulcers without overt

tumor masses are found in the jejunum.

Biopsies of the involvedmucosa

demonstrate lymphoma,

Biopsies of the normalappearing mucosa

usually show villous atrophy

Laparotomy may be necessary to confirm

the diagnosis


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EXTRANODAL MARGINAL

ZONEB

-CELL The association of MALT lymphomas with H. pylori has beendramatically demonstrated by regression of gastric MALT

lymphomas following treatment aimed at eradicating H. pylori .

Five-year survival for these patients is as high as 80 to 90 %following single agent chemotherapy or radiation therapy ,

The standard of care for localized gastric extranodal MZL which

fails antibiotic therapy or is H. pylori negative is radiation therapy,

with >90 percent long-term disease-free survival.

Multiagent chemotherapy


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Diffuse large B-cell lymphoma

(DLB

CL) Systemic chemotherapy. For MALT lymphomas, and perhaps all gastric

lymphomas, surgery may be a treatment which

is no longer necessary


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IPSID Lymphoma

Surgery is rarely indicated because intestinal

involvement is generally diffuse.

For patients with established disease, radiation therapyand/or combination chemotherapy combined with

nutritional support are the mainstays of treatment.

Five-year survival rates as high as 70 percent in patients

treated with combination chemotherapy plus tetracycline

to control diarrhea and malabsorption.


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Mantle cell lymphoma

Systemic chemotherapy is the treatment of choice,

Limited experience suggests that aggressive

chemotherapy followed by autologous stem celltransplantation may benefit younger patients.

Surgery has a relatively small role in the management ofthis disease, but may be of value in patients presenting

with bowel obstruction


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BURKITT'S AND BURKITT-

LIKE LYMPHOMAS Chemotherapy is the mainstay oftreatment

Resection is often required to alleviate

symptoms or avoid perforation during

chemotherapy .


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Enteropathy-associated T-cell

intestinal lymphoma The prognosis is poorand five-year survival isapproximately 10 %

Multidrug chemotherapy therapy in patients whohave minimal gastrointestinal symptoms prior to

the diagnosis of lymphoma, and can tolerate

therapy.

Patients should also be maintained on a gluten-

free diet.


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Lymphoma by m. Mohyi