3. Introduction
GI lymphoma is uncommon;
Extraa nodal involvement GI is more common; other site include spleen
,thymus to brain, soft tissue
Non Hodgkin’s type almost exclusively
Primary gastrointestinal involvement : the stomach
can involve any part of the gastrointestinal tract from the esophagus to the
rectum.
1.9 in 100000, a male-female ratio of 3:2.
4. Risk Factors
HIV infection
Helicobacter pylori infection,
immunosuppression after solid organ transplantation,
Celiac disease,
inflammatory bowel disease
human immunodeficiency virus infection.
5. Criteria for diagnosing primary
Extranodal lymphoma
1. No palpable superficial lymph nodes are seen.
2. Chest radiographic findings are normal (ie, no adenopathy).
3. The white blood cell count (both total and differential) is normal.
4. At laparotomy, the alimentary lesion is predominantly involved, with lymph node involvement
(if any) confined to the drainage area of the involved segment of gut.
5. There is no involvement of the liver and spleen.
6. Frequency of GI occurrence by site
(of all lymphomas)
Stomach
Small intestine
Rectum
Rest of colon
7. Role of imaging
Most common modality is CT , helps in assessing the stage of disease
a wide variety of imaging appearances and definitive diagnosis relies on histopathologic
analysis,
Pointers in Imaging
a bulky mass or diffuse infiltration
with preservation of fat planes and
no obstruction,
multiple site involvement,
associated bulky lymphadenopathy.
8. Imaging also plays an important role in the detection of complications
such as perforation, obstruction, and fistulisation.
However, advanced lymphomas arising in the gastrointestinal
tract may eventually disseminate widely and be clinically,
radiologically, and pathologically indistinguishable from
secondary gastrointestinal lymphomas
9. Staging
stage I, tumor confined togastrointestinal tract, single primary site, and multiple
noncontiguous lesions.
stage II, tumor extends into the abdominal cavity from the primary gastrointestinal site.
(II1, local nodal involvement;
II2, distant nodal involvement.
Stage III, penetration through serosa to involve adjacent organs or tissues; and
stage IV, disseminated extranodal involvement or a gastro-intestinal tract
10. Esophageal Lymphoma
Secondary to cervical and mediastinal lymph node
Contiguous spread from gastric lymphoma.
Primary lymphoma of the esophagus is a rare condition.
Primary esophageal lymphomas are predominantly B-cell type, (recent reports
diagnosing MALT lymphomas)
RADIOLOGICAL FEATURES
Submucosal infiltration or polypoid mass.
With ulceration or nodularity
Barium studies subtle mucosal and submucosal abnormalities.
CT better defines the extent of local disease and the disease stage
Perforation and fistulisation can also be sen in CT and barium studies.
11.
12. Gastric lymphoma
Comprises 3-5% of all gastric neoplasms
• Non-Hodgkin’s accounts for 80% of all gastric lymphomas
• Begins in the submucosa
• Most occur in distal body and antrum of stomach
• Almost all gastric lymphoma presents with some degree of ulceration
13. Pathology
Three distinct types of gastric lymphoma
low-grade MALT lymphoma: 60% of all primary gastric lymphomas
primary sporadic lymphoma: vast majority are B-cell non-Hodgkins lymphoma
secondary involvement of the stomach by systemic lymphoma (usually high grade)
Chronic H pylori gastritis is associated with the development of low-grade MALT Lymphoma,
having been reported to account for 50%–72% of all primary gastric Lymphomas.
MALT lymphoma is treatable and has better prognosis in early stages.
14. Nodular—single or multiple intragastric masses, easily confused with Ca
, protrude into the lumen, often with multiple ulcerations
Polypoid—barium in interstices, frequently with ulceration; sometimes resembles metastatic
disease such as melanoma
Ulcerative—shallow, saucer-like ulcer indistinguishable from Ca
Infiltrative—thickened, irregular folds, simulating the appearance of hypertrophic gastritis; about
10% present this way
15. Radiographic features
Fluoroscopy: barium meal
Appearances vary from normal, to grossly abnormal.
bull's eye appearance due to central ulceration.
filling defects
thickened gastric rugae
linitis plastica
16. CT
marked thickening of the stomach wall (2-4cm)
extensive lateral extension of the tumour (i.e. along the wall of the stomach) representing submucosal
spread .
submucosal spread encompasses the majority of the stomach, giving it a linitis plastica appearance.
uncommon for lymphoma to result in gastric outlet obstruction.
Rarely cause perigastric fat invasion.
homogeneous in attenuation, but may contain focal areas of low density representing necrosis.
Extensive retroperitoneal and local nodal enlargement.
Complications such as obstruction, perforation, or fistulisation can occur as a result of the disease
itself or of treatment and can be detected with CT and barium studies
17.
18.
19.
20. Differential diagnosis
gastric carcinoma
more likely to cause gastric outlet obstruction
more likely to be in the distal stomach
more likely to extend beyond the serosa and obliterate adjacent fat plane
more focal
lymph nodes tend to be smaller and more localized to immediate draining nodes
gastrointestinal stromal tumour (GIST)
For diffuse gastric wall thickening also consider:
gastritis
Menetrier's disease: has a rugal like pattern.
21. Small Bowel lymphoma
most common malignancy of the small bowel. ( 17-30% of all )
related to B-cell hyperactivation in HIV positive patients.
The distal ileum is classically thought to be the most common site.
A circumferential bulky mass in the intestinal wall
Predisposing conditions
AIDS
coeliac disease
organ transplant ( post transplant lymphoproliferative disorder (PTLD)
Helicobacter pylori positive patients
Can present clinically as Gi haemorrhage , perforation, obstruction.
22. TYPES
The type of lymphoma depends on the underlying predisposing condition.
H pylori: mucosa-associated lymphoid tissue lymphoma (MALToma)
PTLD: polyclonal B-cell non-Hodgkin's lymphoma (EBV associated)
HIV: B-cell non-Hodgkin's lymphoma 3 ,overall most common type.
T-cell lymphomas are seen but are uncommon 5, they have more perforation.
23. Infiltrating form:
the most common type
focal or diffuse thickening of the bowel wall with
alternating areas of dilatation or constriction of
the bowel lumen
Folds in the affected segment are thickened,
nodular, effaced and may show ulceration
24. Endoexoenteric form:
shows irregular collection of barium due to central
ulceration, associated with displacement of adjacent
bowel loops.
Associated mesentericabscess or fistula between the
tumor and adjacent bowel loops.
25. Multiple nodular pattern: It is usually seen in T-cell
lymphoma complicating celiac disease and is considered most infrequent
Polypoid form:
It causes submucosal filling defect and is often associated with intussusception
26. IMAGING
Typically involves a small segment (5-20cm).
Bowell wall thickening (1-7cm).
Aneurysmal dilatation: 30%, it occurs due to replacement of muscularis by tumor or infiltration of
myenteric nerve plexus.
30. Peritoneal lymphomatosis from primary gastrointestinal lymphoma is rare.
When involved indistinguishable from carcinomatosis.
31. Large bowel lymphoma
0.4% of all tumors of the colon,
Colorectal lymphomas constitute 6%–12% of gastrointestinal lymphomas.
Primary lymphoma more often affects the cecum and rectum.
Most colorectal lymphomas are non- Hodgkin lymphomas, usually of B-cell origin.
Mantle cell lymphoma is an aggressive disease that manifests as multiple polyps (lymphomatous
polyposis)
Polyposis may also assosciated with MALT lymphoma.
32. IMAGING
Multiple polyps ; mostly near IC valve.
a diffuse or a focal segmental lesion with extensive mucosal ulceration at double-contrast barium enema
examination
Colonic perforation (T-cell lymphoma)
.
Circumferential thickening. (with or without ulceration)
a cavitary mass excavating into the mesentery;
Intussusception may occur with cecal involvement
Focal strictures, aneurysmal dilatation, ulcerative forms with fistula formation may be seen
33.
34.
35. Primary rectal lymphoma is a rare type of gastrointestinal lymphoma and is clinically
indistinguishable from rectal carcinoma
36. MESENTRIC LYMPHOMA
The most common malignant neoplasm affecting the mesentery.
Patterns of mesenteric lymphoma at CT
multiple homogeneous masses encasing the mesenteric vessels “sandwich sign”.
large “cakelike,” mass with low-attenuation areas of necrosis displacing small bowel loops.
ill-defined infiltration of mesenteric fat, particularly after successful chemotherapy.
bulky retroperitoneal adenopathy.
ALWAYS ASSOSCIATED WITH SMALL BOWELL INVIOLVEMENT
38. Ann Arbor Staging of Extranodal
Lymphoma (Modified)
IE Lymphoma restricted to GI tract on one side of diaphragm
IE1 Infiltration limited to mucosa and submucosa
IE2 Infiltration extending beyond submucosa
IIE Lymphoma infiltrating lymph nodes on same sideof diaphragm
IIE1 Infiltration of regional lymph nodes
IIE2 Infiltration of lymph nodes beyond regional nodes
IIIE Lymphoma infiltrating GIT and/ or lymph nodeson both sides of diaphragm
IV Diffuse or disseminated involvement of liver, spleen, lung, brain
39. LUGANO CLASSIFICATION
radiological
Stage I—Tumor confined to GI tract, single primary site or multiple noncontiguous lesions.
Stage II—Tumor extends into the abdominal cavity fromthe primary GI site.
I1—Local nodal involvement
II2—Distant nodal involvement.
Stage III—Penetration through serosa to involve adjacent organs or tissues.
Stage IV—Disseminated extranodal involvement or a GI tract lesion with supradiaphragmatic
nodal involvement
40. Lymphoma Variants
Burkitt’s Lymphoma
tumor of B lymphocytes seen
younger patients of less than 30 years of age. Ileocecal region is most frequently involved.
Large rapidly growing masses with mesenteric lymphadenopathy may be encountered
Mediterranean Lymphoma
Mediterranean lymphoma affects younger persons.
There is marked thickening of the mucosal folds with nodules due to massive infiltration by plasma
cells
The unaffected intestinal loops show features of malabsorption in the form of flocculation,
segmentation and dilatation
41. Multiple Lymphomatous Polyposis
a rare form of lymphoma
multiple polypoid lesions of malignant lymphoma are distributed throughout the GI
tract
43. INTRODUCTION
extranodal disease
The prevalence of extranodal involvement in non-Hodgkin lymphoma and Hodgkin disease
has increased in the past decade.
subtle or absent at conventional computed tomography.
Imaging of tumor metabolism is .the key to diagnose these sites
2-[fluorine-18]fluoro-2-deoxy-d-glucose (FDG) positron emission tomography (PET) scan.
44. Uses
To identify the involved sites.
To distinguish between lesions (primary and relapse)
Intiial staging (95% sensitivity on ombining with CT as in PET-CT)
Follow up and Treatment response assessment.
45.
46. Current revised response criteria
Indication of PET CT
PET is routinely recommended for the staging of patients with FDG-avid, potentially curable
lymphomas
PET is not routinely recommended prior to treatment for incurable, non-FDG-avid or indolent
histologic subtypes
Midtreatment PET should be performed only as a part of clinical trials.
47. Principle
3-dimensional, metabolic imaging technique that uses a radiopharmaceutical to target a specific
physiologic process.
FDG is transported into cells and phosphorylated in a similar manner to glucose
FDG-6-phosphate is not a substrate for glucose-6-phosphate isomerase and because FDG-6-
phosphate is typically not dephosphorylated in tumors
becomes trapped in the cell and reaches a near equilibrium state at approximately 60 minutes after
injection.
The positron-emitting 18F isotope to which FDG is linked decays, and the emitted positron
annihilates after “bumping” into an electron, generating 2 511-KeV photons emitted in nearly
opposite directions that are detected by the PET scanner.
48. defining positive PET findings as focal or diffuse FDG uptake above the surrounding
background in a location incompatible with normal anatomy/physiology.
the standardized uptake value (SUV), representing the ratio of the tumoral tracer concentration
to the average tracer concentration in the entire body
Radiopharmaceuticals:
49. Equipment
PET/CT combines a full-ring detector PET scanner with a multidetector helical
the PET scan is acquired immediately after the CT scan.
The images are fused to provide precise localization of abnormal lesions.
PET/CT provides more sensitive and specific imaging than either modality alone,
Radiopharmaceuticals
Radiioisotope (F18, C11, Ga 67, I123, ) linked to a Metabolic substrate or Gas .
52. Pitfalls of PET
Caution must be exercised in the interpretation of PET scans.
technical limitations,
variability of FDG avidity among the different lymphoma histologic subtypes
In the large number of etiologies of false-negative and false-positive results
False positives arise because FDG is taken up in any process associated with increased glycolysis, for
example, inflammation, infection, granulomatous disease such as sarcoidosis, and brown fat
False-negative PET scans may result from lesions below the resolution of the scanner, generally 5 to 10
mm.
53. Conclusion
Uncommon disease .
a bulky mass or diffuse infiltration
preservation of fat planes
no obstruction,
multiple site involvement,
associated bulky lymphadenopathy
CT is the most useful modality in that it provides a better overall assessment of the
disease stage.
FDG-PET is now considered as a gold standard in pre and post therapeutic
evaluation of LYMPHOMas in general.
Celiac disease has been
noted as a risk factor for small bowel adenocarcinomas,
esophageal cancer, melanoma
* can strongly suggest
the diagnosis. Imaging also plays an important role in the detection of
complications such as perforation, obstruction, and fistulization
MALT lymphoma (MALToma) is a form of lymphoma involving the mucosa-associated lymphoid tissue (MALT), frequently of the stomach, but virtually any mucosal site can be afflicted. It is a cancer originating from B cells in the marginal zone of the MALT, and is also called extranodal marginal zone B cell lymphoma.
a) Barium esophagogram shows a large polypoidal
filling defect in the midesophagus with deep ulceration in the posterior wall (arrowheads).
(b) Contrast material–enhanced computed tomographic (CT) scan obtained 3 weeks later shows fistulization with the trachea (arrow)
Primary gastric lymphoma
often originates as a low-grade MALT
lymphoma, which, it has been suggested, transforms
into intermediate or high-grade large cell
lymphoma if not diagnosed or treated in time
a mass with nodular margins and luminal narrowing in the
antrum of the stomach (arrowheads). Thickened nodular
folds (arrow) are seen more proximally in the stomach.
CT helped confirm antral thickening
Endoscopic ultrasound can be used to assess as well as stage the MALT lymphoma
A. a mass with luminal narrowing in the gastric antrum
and deep ulceration in the inferior wall (arrow). Other nodules of various sizes (arrowhead) are seen adjacent to the mass.
b. Contrast-enhanced CT scan shows diffuse, homogeneous gastric antral wall thickening with a lobulated inner surface and a smooth well-defined outer wall
Thickened gastric walls mainly at ht antrum
Infiltrative—thickened, irregular folds, simulating the appearance of hypertrophic gastritis; about 10% present this way
thickening of
the gastric wall involving the fundus and proximal body
(arrowheads). Note that the perigastric fat planes are
well maintained even though the tumor is very bulky.
Adenopathy is seen with both adenocarcinoma
and lymphoma, but if it extends below the
renal hila or the lymph nodes are bulky, lymphoma
is more likely
the greater amount of lymphoid
tissue in this portion of the bowel
shows mucosal distortion and nodularity in the second part of
duodenum with extravasation of barium from the lateral aspect
(C) CECT shows extensive mural thickening
of the duodenum with presence of air in the lateral wall
suggestive of ulceration
Contrast-enhanced CT scan shows a
markedly thickened terminal ileum
Barium FT shows intrinsic duodenal mass with mucosal destruction
and polypoidal filling defects.
Contrast-enhanced CT scan shows circumferential
thickening of the duodenum with stranding in the adjacent mesentery and loss of fat plane with likely invasion
into the head of the pancreas (P
irregular
thickening of the terminal ileum and the cecum
with stranding in the adjacent mesentery
Fig 2 concentric wall thickening
of the terminal ileum (arrow) with stranding
in the adjacent mesentery
Ileocaecal thickening with thickened appendix.
Air fluid level seen here suggests obstruction.
Contrast-enhanced CT scan obtained at the level of the right iliac fossa
shows an ileocecal mass (arrowheads) without proximal obstruction.
diffuse omental and peritoneal lymphomatosis (arrowheads) with left paraaortic lymphadenopathy (A).
Immunohistochemical markers alone can distinguish
(a) Image from a doublecontrast
barium enema study shows multiple aphthous ulcers (thin arrows) and segmental luminal narrowing (thick
arrows) in the transverse colon. (b) Close-up radiograph of the splenic flexure shows multiple irregular ulcers (arrows)
soft-tissue mass involving the ileocecal junction (arrow in
a) and the cecum (M), with coning of the tip of the mas
a bulky nodular upper rectal mass (M) that arises from the anterior wall and extends into the anterior perirectal fat
T1-weighted MR image shows a hypointense mass (M) that arises from the anterior wall of the upper rectum and extends anteriorly to the mesorectal fascia (arrowheads).
T2 image .
Small bowel enema showing nodular fold
thickening of jejunal loops in a case of Mediterranean
Lymphoma
Barium study showing thickened folds in body of stomach
extending into duodenum and a polypoid lesion in the fundus, (B) Terminal ileum is enlarged with multiple nodular filling defects distorting the mucosa.
refers to lymphomatous infiltration of anatomic
sites other than the lymph nodes. Almost any organ can be affected
by lymphoma, with the most common extranodal sites of involvement
being the stomach, spleen, Waldeyer ring, central nervous system,
lung, bone, and skin
Certain PET/CT patterns are suggestive of extranodal
disease and can help differentiate tumor from normal physiologic FDG
activity, particularly in the mucosal tissues, bone marrow, and organs of
the gastrointestinal tract
(eg, diffuse large B-cell lymphoma and Hodgkin disease- to assses the disease extent.
(eg, glucose metabolism, amino acid metabolism, DNA synthesis)
The most widely used pharmaceutical is the radiolabeled glucose analog fluorine-18-deoxyglucose (FDG)
is often used as a semiquantitative measure of the degree of FDG uptake and aids in the interpretation of PET scans.
faster than the combination of emission and transmission PET scans required to obtain attenuation-corrected PET images.
PET/CT is essentially replacing stand-alone PET scanners
MALT of the stomach. Axial
fused PET/CT image shows a circumferential
focus of FDG accumulation in the stomach
(arrowheads) and diffuse bone marrow activity
(arrow). Diffuse FDG activity in the
stomach has a broad differential diagnosis,
including physiologic uptake, gastritis, and
primary gastric carcinoma. Biopsy is necessary
to confirm the diagnosis.
Recent attempts to standardize PET in clinical trials and incorporation of this technology into uniformly adopted response criteria improved the interpretation of PET scan. Results