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CASE
66
Burkitt’s Lymphoma (Ileum) with Intussusception
History
The patient had suffered from recurrent abdominal
pain for 1 year. The pain, central and in the right
iliac fossa, had been worse for 1 week, requiring
daily attention from the family doctor. On admission to the hospital, clinical examination revealed a
long mass lying transversely in the upper abdomen.
A contrast enema confirmed the diagnosis of intussusception (Figure 66.1). The apex reached the
splenic flexure.
Operation (1.11.96)
Laparotomy confirmed the extent of the intussusception, which was easily reduced. There were 4 discrete firm lesions palpable in the terminal ileum,
the distal one of which was the apex of the intussusception. The related small bowel mesentery
contained enlarged lymph nodes. The bowel was
obviously viable. There were several hard nodules
(2mm) over the surface of the right colon. A right
hemicolectomy, including 70cm of ileum, was
performed.
Female, 10 Years
Pathology
There were a total of nine mucosal lesions of
the ileum, 3 of which were polypoidal and firm
(15mm–30 mm). The distal lesion and 1 other were
ulcerated. There were 5 soft plaque-like abnormalities along the ileum (10mm–20 mm). Sections of the
polypoid lesions revealed a pale surface, and one of
these lesions extended through the full thickness
of the intestinal wall. Histologically, the lesions
showed atypical lymphocytic infiltration consistent
with a diagnosis of Burkitt’s Lymphoma (Figure
66.2). The nodules (nodes) on the surface of the right
colon and the mesenteric lymph nodes showed no
significant abnormalities.
Progress and Investigations
Postoperative recovery was satisfactory. Investigation to stage the patient’s disease showed no other
foci of lymphoma. The non-Hodgkin’s lymphoma
(NHL) classification was Stage III (Murphy classification: childhood NHL).
Figure 66.1: The barium enema shows the
intussusception has reached the splenic flexure.
144
Figure 66.2: The small bowel lesion shows a diffuse
infiltrate of lymphoid cells with the “starry sky”
appearance.
Diagram 66 145
Chemotherapy
The patient was treated with 6 courses of
chemotherapeutic agents, which included
cyclophosphamide, vincristine, methotrexate, doxorubicin, and cytarabine.
Follow-Up (2005)
The patient remains well without evidence of recurrence, 9 years since the resection.
Comment
In Western countries, Burkitt’s Lymphoma is sporadic and accounts for 2% of all lymphomas. They
usually present as abdominal tumors, as in this case,
and may affect the jaw and peripheral lymph nodes,
particularly in the neck. It is associated with the
Epstein-Barr virus (EBV) in 20% of cases (Western
disease). The histology shows diffuse changes with
sheets of monomorphic neoplastic lymphoid cells
evenly interspersed with histiocytes, producing the
characteristic “starry sky” pattern.
1
The prognosis
without treatment is very poor, however
chemotherapy has been effective in inducing dramatic and complete tumor regression, as in this
patient.
CASE
67
History
The patient had noted the recent onset of abdominal distention with 2 episodes of acute lower abdominal pain within 2 months. These symptoms were
accompanied by a loss of weight. The stools had
become black in color over a period of 7 days. Examination revealed a large nontender spherical mass
occupying the right lower abdomen. A barium
enema demonstrated dilatation of the terminal
ileum with distortion of the mucosal pattern. A
computerized tomography (CT) examination
revealed a mass in the lower abdomen 10 × 10 ×
8cm extending into the cecum and ascending colon.
A collection of contrast appeared within the mass,
suggesting a bowel perforation was present.
Colonoscopy revealed a large polypoid mass in the
medial aspect of the proximal ascending colon and
cecum, which obscured the ileocecal valve.
Operation (12.4.93)
A large confluent mass was confirmed in the ileocecal region. Its bulky nature suggested the possibility of a lymphoma. The mass appeared to
infiltrate the ascending colon, terminal ileum, and
mesentery. Enlarged lymph nodes were present
along the ileocolic vessels. There was no other
pathology detected within the abdomen. A right
Ileocecal Lymphoma
Male, 82 Years
hemicolectomy was performed, including removal
of 50cm of terminal ileum.
Pathology
The mass “encased both the terminal ileum and the
proximal right colon.” On section, it was pale in
color with a central cavity that communicated with
the terminal ileum in two places. The mucosa of the
ileum was otherwise normal. Within the mesentery,
seven enlarged, firm lymph nodes were present. Histological examination confirmed the diagnosis of
lymphoma (Figure 67.1). The tumor was a nonHodgkin’s large B cell type with a diffuse pattern.
It was consistent with the so-called MALT lymphoma (arising from mucosal associated lymphoid
tissue). The mesenteric lymph nodes were not
involved with lymphoma. In the cecum, a flat pale
polyp was present measuring 50 × 50 mm, which
on examination was a dysplastic tubulovillous
adenoma.
Follow-Up
Six weeks after operation, a right-sided abdominal
mass was present and a gallium scan confirmed 4
foci of lymphoma within the abdomen. Over the
next 7 months, 5 courses of chemotherapy were
administered (cyclophosphamide, mitozantrone,
vinblastine). The response to treatment was satisfactory and the patient remained in remission until
1997, when the disease recurred. At the age of 87
years, no further treatment was advised. The recurrent lymphoma was not the cause of death 4 years
9 months after operation, when he died unexpectedly during sleep.
Figure 67.1: The left of the field shows 2 lymphoid
follicles with germinal centers. The lymphoma is on the
right. Small bowel villi are seen in transverse section.
146
Comment
This patient’s clinical presentation and tumor
morphology were typical of small bowel lymphoma.
Such lesions represent 20% of small bowel tumors
and may be associated with Coeliac or Crohn’s
disease as well as immunodeficiency states. Preoperative diagnosis is unusual, but at operation a
pale bulky tumor will suggest the diagnosis is lymphoma. In this patient, the MALT B cell tumor was
low grade, which is usually associated with a more
favorable prognosis. Adjuvant chemotherapy is frequently associated with resection of the disease, particularly if regional lymph nodes are involved.
1
2
Diagram 67 147
CASE
68
Multiple Lymphoma and Ulcerative Colitis
History
The patient had noticed a few weeks of minimal
dark red bleeding from the rectum and was examined by colonoscopy. There was diffuse low grade
colitis (Figure 68.1) affecting the colon with a loss
of vascular pattern and diminution of the haustral
indentation. In the lower third of the sigmoid colon
(at 20cm), there was nodularity of the mucosa
with associated hyperemia and small submucosal
hemorrhages. Biopsy of this area revealed nonHodgkin’s lymphoma (NHL) of a diffuse type.
Immunochemistry was positive for the B cell
marker L26. Other biopsies of the mucosa showed
nonspecific chronic inflammation. Staging investigation by gallium and computerized tomography
(CT) scans revealed no other foci of lymphoma.
Surgical treatment was advised in preference to
chemotherapy.
Female, 49 Years
Figure 68.2: Diffuse large B cell lymphoma.
Operation (11.14.94)
The small bowel and colon were normal in appearance, with some thickening apparent in the lower
third of the sigmoid colon on palpation. Small firm
Figure 68.1: Endoscopy shows chronic inflammatory
changes in the mucosa of the ascending colon (1998).
lymph nodes were palpable in the mesentery of
the sigmoid and descending colon. The spleen was
enlarged 40–50% of normal size. The extent of the
resection was related to distribution of the palpable
Figure 68.3: Evidence of Colitis: surface ulceration and
distorted mucosal glands.
148
Diagram 68 149
lymph nodes. The proximal level was the upper
descending colon, and the distal level was mid
rectum. The anastomosis was performed with a
circular stapler.
Pathology
In the mucosa of the distal sigmoid, there were 5
pale, firm nodules 3–5 mm in size with surrounding
congestion and areas of mucosal hemorrhage. Histologically, the nodules were malignant lymphoma
of large cell B type (Figure 68.2). There were diffuse
as well as follicular areas of lymphoma in the bowel
wall. There was no evidence of lymphoma in any of
11 lymph nodes examined. Macroscopic evidence of
colitis was minimal, but microscopic examination
confirmed the presence of colitis (Figure 68.3).
Follow-Up (2005)
No chemotherapy was administered, since the localized disease appeared to be adequately removed by
operation. Periodic examinations by gallium and CT
scans have shown no evidence of recurrent lymphoma. The patient remains well more than 10
years since operation except for occasional episodes
of diarrhea, which last for a few days. Surveillance
colonoscopies with random biopsies have continued
and reveal no change in the endoscopic or macroscopic appearances, which is consistent with a
diffuse chronic nonspecific colitis of moderate
degree (Figure 68.2).
colitis.2It is not yet established whether the association of lymphoma and chronic ulcerative colitis
(CUC) is a definite risk factor or coincidence, but
some of the literature does support the former.
Multifocal involvement is not uncommon in gastrointestinal lymphoma, and the small multiple
nodules were of interest in this patient. Wagonfeld
et al have described a patient with CUC and 22 lymphomas in the colon.4The severity and duration of
the colitis is variable in patients with coexistent
lymphoma, and the patient described here has been
asymptomatic most of the time. Surgery remains
the mainstay for colorectal lymphoma. If the lymph
nodes are not involved, chemotherapy is usually
omitted.
3
Comment
Hopefully this patient’s prognosis is favorable due to
minimal localized lymphoma with no evidence of
recurrence in over 10 years. It is unusual for gastrointestinal lymphoma to be a small lesion at the
time of diagnosis. The obvious and numerous lymph
nodes in the sigmoid and left colon mesentery determined the proximal level of the resection but proved
negative for lymphoma. Chronic colitis coexisting
with malignant lymphoma is rare and was first
1
reported by Bargen in 1928.
Baker reported an inci-
dence of 5 lymphomas in 2500 patients with chronic
For a full-page image of this figure see the
appendix.
CASE
69
History
The patient presented with a history of bright blood
on the stool for only a few days. He otherwise felt
well. On rectal examination, a large firm polypoid
lesion was palpable on the left anterior aspect of the
lower third of the rectum 5cm from the anal verge.
It was moveable on palpation. Colonoscopy revealed
no other pathology in the colon.
Operation (10.28.99)
The lesion was attached to the wall of the rectum
by a broad pedicle, which could be readily constricted by closure of the diathermy snare. The
polyp was removed with coagulation current (Figure
69.1) as a total specimen. The underlying rectal
muscle remained intact.
Pathology
The polyp measured 25 × 22 × 12mm. It was pale
pink in color, with a lobulated surface. Histologically, the lesion was covered by colonic mucosa
with some inflammatory reaction. Beneath the
mucosa, there was a nodular mass infiltrating the
submucosa composed of a “monotonous population” of intermediate to large sized lymphoid cells.
Numerous mitotic figures and apoptotic cells were
Lymphoma of the Rectum
Male 63, Years
scattered throughout. Immunoperoxidase stains
identified the lesion as a malignant non-Hodgkin’s
lymphoma of diffuse large B cell type (Figure 69.2).
Further Treatment/Follow-Up (2004)
Investigations revealed no other foci of lymphoma.
The patient was treated with 4 cycles of chemotherapy (cyclophosphamide, doxorubacin, vincristine,
and prednisolone). The site of the lymphoma
healed readily, and regular clinical, endoscopic, and
computerized tomography (CT) examinations have
shown no evidence of recurrent disease 4 years 6
months after removal of the rectal lesion.
Comment
Gastrointestinal lymphoma accounts for 2%–5% of
gastrointestinal tract (GIT) malignancy1and is the
most common extranodal site.2Au et al report the
incidence in the GIT as: stomach, 57%: small bowel,
22%; large bowel, 7%; gallbladder, 2%; and multiple sites, 11%.1The prognosis for control of this
patient’s disease is estimated to be in the vicinity of
70%. The technique of diathermy snare with strong
coagulation current was used in this patient only
because the lesion was in the extraperitoneal
rectum.
Figure 69.1: Endoscopic view of the defect after
diathermy snare removal. Blood clot in the proximal part
of the defect.
150
Figure 69.2: The lymphoma is apparent beneath the
mucosa.
Diagram 69 151
PART
VIII
Anorectal Disease