imaging findings in small bowel lymphoma

1. BY DR MAIMUNA ABDULKARIM HALLIRU
DEPT OF RADIOLOGY
AMINU KANO TEACHING HOSPITAL,
KANO
15/10/2014
DISCUSS THE IMAGING FEATURES OF
SMALL BOWEL LYMPHOMA

2. OUTLINE
 INTRODUCTION
 PATHOGENESIS
 DISTRIBUTION
 RISK FACTORS
 CLINICAL FEATURES
 HISTOPATHOLOGY
 ROLE OF RADIOLOGY IN MANAGEMENT
 IMAGING MODALITIES
 IMAGING FEATURES
 RADIOLOGY OF COMPLICATIONS
 SUMMARY/CONCLUSION

3. INTRODUCTION
 The small bowel is the longest part of the GI tract
spanning a length of about 6 metres and the
mucosa of the small bowel accounts for over 90
percent of the surface of the gastrointestinal (GI)
tract.
 It is made up of three parts: proximally the
duodenum, jejunum and most distally, the ileum.

4.  The small bowel has a calibre of about 3-5 cm.
 Circular, circumferential mucosal folds- the plicae
circulares are found most numerously in the
jejunum and least in the ileum.
 The small bowel has clumps of submucosal
lymphoid tissue called the Peyer’s patches in the
jejunum and ileum. However they are most
numerous in the ileum.

5. (1)Buckley.J.A,Fishman.E.K; CT Evaluation of small bowel neoplasms-
Spectrum of disease.RSNA 1998:18;379-392
PATHOGENESIS
 The small bowel is a rare site of tumours in general
and accounts for less than 2-5 percent of all GI
neoplasms and 1 percent of GI carcinomas(1).
 Explanations:
a) the liquid contents of the small bowel may cause
less mucosal irritation than the more solid contents
of the colon;

6. b) The relatively rapid transit of intestinal contents
through the small bowel may provide shorter
exposure of its mucosa to carcinogens;
c) The much lower bacterial load in the small bowel
may result in decreased; conversion of bile acids into
potential carcinogens by anaerobic microorganisms

7. d) Benz(o)pyrene, a known carcinogen present in
various foods, is converted in less toxic metabolites
by benz(o)pyrene hydroxylase, which is present in
much higher concentrations in the small intestine
compared to the stomach and colon;
e) The increased lymphoid tissue with a high level of
(secretory) IgA expression in the small bowel may be
protective

8.  Survival from primary malignancies of the small
intestine has not improved in parallel with significant
advances in diagnostic imaging over the last four
decades.
 Curative treatment depends on early diagnosis and
surgical resection.

9. (2)Maceneaney P.M, Gasparaitis A.E;Diagnosis of Small-Bowel Tumors pp359;1-14
 Unfortunately,many cancers of the small bowel are
diagnosed at an advanced stage, after the window of
opportunity afforded by local disease has elapsed.
 Maglinte and colleagues(2) showed that there was an
average delay of almost 9 months between onset of
symptoms and diagnosis.

10.  Several factors contributed to this delay:
-delayed ordering of appropriate investigations by the
clinician.
-misinterpretation of investigations by radiologists.

11. DISTRIBUTION
 Small bowel lymphoma accounts for about 20% of
all small bowel tumours.
 The jejunum and ileum are almost equally
affected. The distal ileum is the most common
site, owing to the large amount of lymphoid tissue
that is present here.

12.  Most GI lymphomas occur in middle-aged people.
 Burkitt’s lymphoma is commoner in children.
 Enteropathy associated T-cell lymphoma is
commoner above 60 years of age.
 Males and females are equally affected.

13.  The ileocecal region is the most common location
for primary small bowel lymphoma (children and
young adults), whereas lymphoma is more often
found in the proximal jejunum in patients with
celiac disease.
 Multicentric lymphoma involving different
segments of the gastrointestinal tract occurs in
10% to 50% of cases.

14.  Non-Hodgkin's lymphoma is the commonest variety
that involves the GI tract.
 Almost 90 percent of primary NHLs of the small
bowel are of the B-cell type.
 Usually intermediate or high grade with large cell
features.

15.  Apart from cases related to celiac disease, T-cell
lymphomas are rare, as are Burkitt’s lymphoma and
Hodgkin’s disease of the small bowel.
 Burkitt’s lymphoma of the bowel is usually confined
to the ileocecal region.

16. RISK FACTORS
 Celiac disease.
 Crohn's disease.
 Systemic Lupus Erythematosus.
 Immunocompromised state.
 A history of chemotherapy.

17. CLINICAL FEATURES
 The commonest clinical features associated with
small bowel lymphoma are abdominal pain, fatigue,
malaise,gastrointestinal bleeding, nausea and
vomiting, and weight loss.
 Palpable mass in 33%
 Obstructive features and perforation occur relatively
late; in about 25% .

18. CLINICAL FEATURES
 These nonspecific clinical features, coupled with the
low incidence of small-bowel tumours, result in a low
index of suspicion on the part of the physician and
the radiologist and contribute to the slow diagnosis
of such tumours.

19.  The criteria for establishing the diagnosis of primary
gastrointestinal lymphoma are:
a) Absence of palpable lymphadenopathy;
b) Normal peripheral blood smear and bone marrow
biopsy.
c) Absence of mediastinal lymphadenopathy on chest x-
ray.

20. d) Disease grossly confined to the affected small bowel
segment, as confirmed by diagnostic imaging,
endoscopy or laparotomy.
e) Regional lymphadenopathy ;and,
f) Absence of hepatic or splenic tumour involvement
except via direct extension from primary bowel
involvement.

21.  Histopathologic types of lymphoma that
may involve the small intestine include:
 malignant lymphoma (in celiac disease).
B-cell lymphoma.
 Immunoproliferative small intestinal disease
(Mediterranean lymphoma).
HISTOPATHOLOGY & STAGING

22.  Low grade B-cell lymphoma (lymphoma of mucosa
associated lymphoid tissue).
 Follicular lymphoma.
 Burkitt's lymphoma (in children).
 Mantle cell lymphoma.
 Rarely, Hodgkin’s disease.

23. STAGING
 Classification: Ann Arbor (classic), Musshoff
(modified Ann Arbor) and Blackledge (1990).
 Recently, these classifications system have been
superseded by a Revised European-American
Lymphoma (REAL) classification, which includes
entities such as T-cell lymphoma, maltoma and
mantle-cell lymphoma.

24.  Ann Arbor Classification:
I E: Single GI tumour confined to small
intestine without lymph nodes.
II E: GI tumour focus + Regional Lymph node
involvement on one side of the diaphragm.

25. III E: GI tumour focus + non-resectable Lymph
nodes on both sides of the diaphragm.
III ES: III + splenic involvement.
IV E: GI tumour focus+ disseminated
involvement of extra-lymphatic systems (i.e
bone marrow, liver).

26. PATTERNS OF SMALL BOWEL LYMPHOMA
 Circumferential infiltration of a small bowel segment:
§ Results in a variable length of thickening and
effacement of folds.
§ Widening of the lumen rather than narrowing
from infiltration of muscularis layer with destruction
of the myenteric plexus leading to aneurysmal
dilatation, often at the antimesenteric segment. A.k.a
Intestinal aneurysm.

27.  Nodular and polypoid lesions can be variable in size
and irregularly distributed.
 These are sometimes reported to cause
intussusception. This is a relatively rare manifestation
of small-bowel lymphoma and may represent disease
originating in mantle cells or mucosa-associated
lymphoid tissue (MALT).

28.  Mucosal wall thickening with narrowing,
stricturing and occassionally shouldering may be
seen.
 Endoexoenteric lesions that can cause fistulas

29. ROLE OF RADIOLOGY IN MANAGEMENT
 In making a diagnosis.
 In staging.
 In assessing complications of the disease.

30. IMAGING MODALITIES
 Plain Radiograph
 Abdominal ultrasound scan
 Contrast Radiographic studies
 Abdominal Computed Tomography scan
 Magnetic Resonance Imaging
 Nuclear Imaging
 Angiography

31. PLAIN ABDOMINAL X-RAY
 Small bowel x-rays are currently the most widely used
studies for investigation of focal small intestinal
lesions.
 Plain abdominal radiographs may reveal obstruction,
a calcified mass or evidence of hollow viscus
perforation, but their overall yield in uncomplicated
small bowel tumours is very low.

34. ULTRASONOGRAPHY
 Endoscopic ultrasonography or endoscopic
ultrasound (EUS) has been the most recent addition
to the armamentarium of diagnostic modalities for
the examination of the small bowel.
 This new technological achievement not only
provides high resolution imaging of the
gastrointestinal wall and surrounding structures, but
also allows interventional diagnostic and therapeutic
procedures under real-time EUS guidance.

35. EUS image showing 5 layers of the gastric wall. 1, 3, and 5 = first,
third, and fifth layers are hyperechoic (white); 2 and 4 = second
and fourth layers are hypoechoic (black). Transducer (tr) is
surrounded by a water-filled balloon (arrows).

36. Mixed hypoechoic gastric tumour (T) disrupting the normal 5-layer
architecture at this point (long arrow). Tumour extends into the
hypoechoic muscularis propria layer (black arrows). The
hyperechoic serosal layer (short arrows) is intact.

37. ULTRASONOGRAPHY
 However, the ability of EUS in detecting and
staging small bowel lesions appears to be most
applicable only to ampullary tumours, carcinoid
tumours and vascular lesions.
 Thus the transabdominal route is still the
conventional mode employed in the general
sonographic assessment of small bowel
lymphoma.

38.  The diagnostic yield of this procedure is low. This
is due to the presence of luminal gas within the
bowel.

39. Sonogram of the left upper quadrant of abdomen showing dilated and
thickened small bowel loop (black arrow) with luminal narrowing (red arrow).

40. Large mesenteric lymph nodes in a case of small bowel lymphoma.

41. CONTRAST RADIOGRAPHIC STUDIES
 Detailed barium evaluation of the small bowel is
difficult due to the presence of multiple peristaltic
intestinal loops overlapped in a limited area.
 This results in lower sensitivity and specificity of
barium studies in the small bowel, compared to the
equivalent study of the stomach and colon.
 The reported sensitivity of the conventional (non-
enteroclysis) small-bowel study (SBS) varies
widely.

42.  An abnormality demonstrated by SBS was present
in up to 83 percent of cases of primary small-
bowel malignancies, but the tumour was directly
visualized in less than 50 percent of cases(2).
 This suboptimal diagnostic performance of the
conventional barium SBS inevitably leads to false-
negative results and diagnostic delays.
 Enteroclysis is the preferred method for detecting
small, resectable small bowel tumours.
(2) Maceneaney P.M, Gasparaitis A.E; Cancer of the Upper
Gastrointestinal Tract; Ch. 20-Diagnosis of Small-Bowel Tumors
pp359

43.  The routine small bowel follow through (SBFT) is
simple, inexpensive and non-invasive, but is
relatively insensitive.
 Much more accurate information can be obtained
using enteroclysis. In this double contrast X-ray
study, the descending duodenum is first
intubated.
 Smooth muscle relaxants such as hyoscine and
glucagon are used to dilate the small bowel.

44.  Barium and methylcellulose are then infused under
pressure into the small intestine.
 This method produces distension of the bowel and
allows the fluoroscopist to follow the movement of
the contrast material through the gut.
 As compared to the traditional small bowel series,
enteroclysis permits better visualization not only of
the intestinal lumen but also of the mucosal surface.

45. (3)D. Xynopoulos, A.A. Mihas, E. Paraskevas, D. Dimitroulopoulos, D.M. Heuman,
A.A. Mihas. Small bowel tumours. Annals of Gastroenterology 2002;15(1):18-35.
 Transient delay in passage of contrast can identify
areas of partial obstruction that are otherwise
extremely difficult to locate.
 In one series, enteroclysis had a sensitivity of 90% for
small bowel tumours, versus only 33% with SBFT(3).

46. The small-bowel enema shows an ileal segment with thickened folds. At
the antimesenteric border a short outpouching (arrows) is visible.

47. The small bowel enteroclysis image shows thickened mucosa with nodular
appearance and dilatation of the lumen.

48. Enteroclysis study demonstrating enteric contrast material filling a large cavity in a
small-bowel lymphoma.

49. Small-bowel series depict aneurysmal dilatation of a segment of
ileum

50. Short segment (left lowermost loop) with diffuse thickening of the valvulae
conniventes and a subtle background nodular pattern consistent with
“maltoma.”

51. ABDOMINAL CT SCAN
 Computed tomography has been employed in the
assessment of small-bowel pathology in two
settings:
(1) in patients with bowel obstruction and in
whom barium studies are unpleasant, difficult,
and risky.
(2) when staging tumours. Computed tomography
evaluates the level, grade, and (frequently) the
cause of the obstruction.

52.  In patients with intestinal obstruction secondary
to a small bowel tumour, the tumour is relatively
large and may therefore be identified by CT.
 However, diagnosis of early non-obstructive
tumours of the small bowel by standard CT is
usually serendipitous.

53.  Neoplastic disease should be suspected on CT if the
thickness of the wall of the small bowel is > 1.5 cm or
if there are discreet mesenteric masses larger than
1.5 cm.
 Radiologically, diagnosis and staging are performed
with a combination of CT and barium studies.
 Computed tomography may also be used to guide
percutaneous biopsy of the bowel or a nodal mass.

54.  Computed tomography (CT) can identify masses but
is insensitive for diagnosis of small tumours and has a
limited ability to differentiate between tumour types.
 Large adenocarcinomas and lymphomas are often
mistaken for each other.
 Its greatest utility seems to be in the preoperative
staging and evaluation of metastases.

55. (3)D. Xynopoulos, A.A. Mihas, E. Paraskevas, D. Dimitroulopoulos, D.M. Heuman, A.A. Mihas.
Small bowel tumours. Annals of Gastroenterology 2002;15(1):18-35.
 Computerized tomographic (CT) enteroclysis, a
combination of CT scan with barium infusion through
a nasogastric (NG) tube, has been the subject of
recent favourable reports from Europe but remains
an investigational tool at present (3).
 The images acquired overcome the problem of
overlapping bowel loops encountered in conventional
barium studies, and there is the potential of
performing multiplanar reformatting.

56.  Computed tomography tends to be very sensitive
but not specific for the detection of mesenteric
infiltration, regional lymphadenopathy, and
distant metastases(2).
(2) Maceneaney P.M, Gasparaitis A.E; Cancer of the Upper
Gastrointestinal Tract; Ch. 20-Diagnosis of Small-Bowel Tumors pp359

57. CT scan demonstrates a polypoid mass (arrow)

58. CT scan demonstrates a bulky mass encasing the small bowel just beyond the
ligament of Treitz (arrow). In addition to the wall thickening, the marked
luminal dilatation (aneurysmal dilatation) helped differentiate this
lymphoma from adenocarcinoma.

59. CT scan demonstrates infiltration of the ileum in the left lower quadrant
(arrow). The mass is exophytic and partially necrotic.

60. CT scan of the abdomen shows a loop of small bowel in the mid-abdomen with
a markedly thickened wall.

61. Dilated lumen at the site of the mass and prestenotic dilatation
of the duodenum (red arrow).

62. Lymphoma in the terminal ileum.

63. A huge endoexoenteric mass involving a loop of ileum extending into
adjacent tissues with mural gas bubbles extending into adjacent tissues
indicating an enteric fistula.

64. Huge multilobullated abdominal mass in a patient with small bowel
lymphoma due to lymphadenopathy.

65. MAGNETIC RESONANCE IMAGING
 Magnetic resonance imaging (MRI) is used for
staging cancers and not for the primary
detection of small bowel tumours.
 Development of contrast agents, coils, and
rapid pulse sequences have improved MRI
of the GI tract.

66.  Fast pulse sequences allow for imaging during a
breath-hold and minimize the motion artefact
associated with peristalsis and respiration.
 Magnetic resonance (MR) has been used to
evaluate small-bowel obstruction, and MR
enteroclysis has been reported.

67.  Bowel contrast is important in MR to avoid
mistaking bowel loops for adenopathy or
mesenteric masses.
 Positive-contrast agents such as gadolinium,
manganese, or ferric ammonium citrate reduce T1
relaxation times.
 These agents can accentuate “ghosting” artefacts
from peristalsis. Rapid imaging techniques and
anti-peristaltic agents minimize these effects.

68.  Negative contrast agents may be classed as
diamagnetic preparations (e.g., barium) and
superparamagnetic iron oxides.
 These negative agents represent the major
options for radiologists.
 As more contrast preparations are developed and
approved, it is likely that oral contrast
administration will become routine and that the
role of MRI in small-bowel pathology will evolve.

69.  Small bowel lymphoma typically shows as
asymmetrical but circumferential wall thickening
with associated luminal dilatation (intestinal
aneurysm).
 Significant enlargement of mesenteric lymph
nodes.
 Low signal intensity on T1, slightly increased on
T2, hypovascular and thus very little contrast
enhancement.

70. Diffuse mural thickening in a loop of small bowel due
to lymphoma.

71. Thickening in a loop of small bowel due to lymphoma.

72. Lymphoma in the proximal small bowel seen as
circumferential wall thickening (arrow).

73. Ileal wall thickening due to B-cell lymphoma

74. Ileal lymphoma with mesenteric lymphadenopathy.

75. Mesenteric lymphadenopathy (red arrows).

76. NUCLEAR IMAGING
 Small bowel lymphoma typically are depicted as
hot spots due to increased uptake of radionuclide.
 PET-CT the usual modality of investigation.

77. Diffuse large B-cell lymphoma

78. Lymphoma in proximal jejunum.

79. EATL lymphoma in a patient with celiac disease.

80. B-cell Lymphoma in proximal small bowel.

81. ANGIOGRAPHY (DSA, MRA, CTA)
 Angiography may be used to evaluate and
embolize bleeding from ulcerated and vascular,
more aggressive subtypes.
 Angiography is occasionally used as an aid
to surgical planning.

82. ANGIOGRAPHY (DSA, MRA, CTA)
 Lymphoma is a relatively hypovascular tumour.
Therefore findings on angiography are usually:
 i- Mild tumour blush.
 ii- Splaying/Displacement of the mesenteric
vessels.
 iii-Rarely, extravasation of contrast medium in
aggressive, bleeding lesions.

83. Superior mesenteric angiogram showing extravasation of contrast from
the sixth jejunal branch (arrow).

84. Enteropathy associated T-cell lymphoma.
Pre- embolization. Post- embolization.

85. RADIOLOGY OF COMPLICATIONS

86. INTUSSUSCEPTION
 This complication is often encountered with the
polypoid or nodular form of lymphoma where the
tumour acts as a lead point.

92. Characteristic reniform appearance of ileocolic intussusception in
the right iliac fossa. Proximal small bowel dilatation.

94. Ileal-ileal intussusception in a patient with multifocal small
bowel lymphoma .

95. MESENTERIC LYMPHOMA
 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.

96. Small bowel and mesenteric lymphoma

97. Sandwich Sign of Mesenteric Lymphoma. A mesenteric artery (arrow) is
sandwiched between two homogeneous isodense masses of lymphoma.

98. Sandwich Sign of Mesenteric Lymphoma. A mesenteric artery (arrow) is
sandwiched between two homogeneous hypoechoic masses of lymphoma ; l.

99. ENTERIC FISTULAE/PERFORATION
 Seen with the endoexoenteric /exophytic forms
which are associated with necrosis, extension into
adjacent tissues eventually leading to fistulae and
bowel perforation.

101. DIFFERENTIAL DIAGNOSIS
 Tuberculosis
 Inflammatory small bowel disease
 Carcinoma.

102. TUBERCULOSIS
Contrast enhanced CT shows gross circumferential wall thickening of the
jejunum in a patient with gastrointestinal dissmeninated mycobacterium
avium intracellulare.

103. INFLAMMATORY BOWEL DISEASE
Crohn’s disease of the small bowel with mucosal wall
thickening (black arrows) and fat stranding (white arrows).

104. INFLAMMATORY BOWEL DISEASE
Thick walled mass in terminal ileum, representing small
bowel.

105. ADENOCARCINOMA
An irregular mass in
the proximal
jejunum (upper
image).
There is a large
conglomerate of
hypodense lymph
nodes in the
adjacent mesentery,
consistent with
necrotic lymph node
metastases (lower
image).

106. ADENOCARCINOMA
Extensive wall thickening of the proximal jejunum with
aneurysmatic dilatation (arrow).

108. TREATMENT AND PROGNOSIS
 Malignant lymphoma of the small bowel is treated
with surgical resection, usually followed by
chemotherapy to prevent perforation.
 Radiation therapy may also be used.

109.  Poor prognosis is associated with:
§ Stage greater than IIE (nodal disease above and
below the diaphragm).
§ Tumour size greater than 10 cm
§ T cell type
§ Presentation as an acute abdomen

110. SUMMARY/CONCLUSION
 Small bowel lymphoma is rare and due to low
index of suspicion on the part of both clinicians
and radiologists, there is usually delay in diagnosis
and treatment with resultant poor prognosis.
 Be conversant with clinical presentation and
imaging findings to redirect physicians where
necessary and to increase diagnostic accuracy.

111. THANK YOU FOR YOUR TIME AND
ATTENTION