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Diagram 65
Postoperative Course
The patient’s general condition remained poor with
difficulty in establishing adequate oral intake.
Upper gastrointestinal (GIT) bleeding and systemic
candidiasis complicated the patient’s weakened
state. She succumbed 4 weeks after operation. No
autopsy was performed.
Comment
The diagnosis of TB was not made until after the
patient’s death when the slides were examined for
a further opinion in an academic department of
pathology. No acid fast bacilli (AFB) were seen or
obtained by culture from the tissue removed at both
141
resections. Confirmation of intestinal tuberculosis
is known to be difficult. Findlay et al report the positivity of tests as follows: Mantoux 31%, chest x-ray
29%, histology 56%, and tissue culture 27%.1
Where doubt exists, a therapeutic trial of antitubercular treatment has been recommended,2 and such
treatment would have benefited this patient. The
original description of regional ileitis (later known
as Crohn’s disease) was based on the review of
pathology specimens at Mount Sinai Hospital, New
York. The authors referred to ileocecal tuberculosis
in the differential diagnosis and stated, “In all of
our first cases of regional ileitis, the diagnosis
of ileocecal tuberculosis was the unvarying best
possibility.”3
12.22.86
5.27.93
P A R T
VII
Lymphoma
C A S E
66
Burkitt’s Lymphoma (Ileum)
with Intussusception
Female, 10 Years
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
(2 mm) over the surface of the right colon. A right
hemicolectomy, including 70 cm of ileum, was
performed.
Figure 66.1: The barium enema shows the
intussusception has reached the splenic flexure.
144
Pathology
There were a total of nine mucosal lesions of
the ileum, 3 of which were polypoidal and firm
(15 mm–30 mm). The distal lesion and 1 other were
ulcerated. There were 5 soft plaque-like abnormalities along the ileum (10 mm–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 66.2: The small bowel lesion shows a diffuse
infiltrate of lymphoid cells with the “starry sky”
appearance.
Diagram 66
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
145
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.