AJR 2002; 179:1443-1445
© American Roentgen Ray Society
Recurrent Non-Hodgkin's Lymphoma of the Appendix
Douglas S. Katz1,
Leonard B. Stein2 and
Joseph P. Mazzie1
1 Department of Radiology, Winthrop-University Hospital, 259 First St., Mineola,
NY 11501.
2 Department of Gastroenterology, Winthrop-University Hospital, Mineola, NY
11501.
Received February 22, 2002;
accepted after revision May 15, 2002.
Address correspondence to D. S. Katz.
Introduction
The gastrointestinal tract is the most common site of extranodal lymphoma.
However, the appendix is only rarely the primary site of lymphoma at initial
presentation, and recurrent lymphoma of the appendix is even rarer. To our
knowledge, few reports describe the CT appearance of lymphoma involving the
appendix. We report the findings of a patient with a history of non-Hodgkin's
lymphoma treated 9 years earlier who presented with lower gastrointestinal
bleeding due to non-Hodgkin's lymphoma that had recurred in the appendix.
Case Report
A 66-year-old man presented to our institution with a 1-week history of
intermittent rectal bleeding. No associated diarrhea, abdominal pain, nausea,
vomiting, or light-headedness was present. An urgent outpatient flexible
sigmoidos-copy revealed diverticulosis and luminal blood, and the patient was
admitted to the hospital.
The patient had been diagnosed 9 years earlier with non-Hodgkin's lymphoma,
large B-cell type, which was treated and went into complete remission. The
patient underwent CT of the abdomen and pelvis at our institution at the time
of his initial diagnosis, which showed that the cecum and the appendix (Figs.
1A and
1B) were normal.
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Fig. 1A. —66-year-old man with history of lymphoma diagnosed and
treated 9 years earlier and 1-week history of intermittent rectal bleeding.
Axial CT scans through upper pelvis from examination obtained 9 years earlier
show normal cecal base (arrow, A) and appendix
(arrow, B) filled with barium.
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Fig. 1B. —66-year-old man with history of lymphoma diagnosed and
treated 9 years earlier and 1-week history of intermittent rectal bleeding.
Axial CT scans through upper pelvis from examination obtained 9 years earlier
show normal cecal base (arrow, A) and appendix
(arrow, B) filled with barium.
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|
At physical examination, the patient's vital signs were stable, and he
appeared to be in no acute distress. The findings from the examination of the
heart, lungs, and abdomen were normal. Digital rectal examination revealed no
masses, but a small amount of bright red blood was detected. Laboratory
studies obtained at admission were significant for a hemoglobin of 13 g/dL and
a hematocrit level of 37%.
A colonoscopy was performed. A bleeding, ulcerated mass noted at the cecal
base was biopsied. Immediately after colonoscopy, abdominal CT was performed.
This examination showed marked enlargement of the entire appendix (Figs.
1C and
1D), which was distended to 3
cm in diameter, with the lumen measuring approximately 50 H in density. The
base of the appendix protruded slightly into the cecal lumen. No other masses
or enlarged lymph nodes were present in the abdomen or pelvis.
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Fig. 1C. —66-year-old man with history of lymphoma diagnosed and
treated 9 years earlier and 1-week history of intermittent rectal bleeding.
Axial CT scans obtained at time of current admission reveal marked distention
of appendix (arrows), which measures 3 cm in diameter and coils in
and out of transverse axial plane. Appendiceal density (measured at level
shown in D) was approximately 50 H.
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Fig. 1D. —66-year-old man with history of lymphoma diagnosed and
treated 9 years earlier and 1-week history of intermittent rectal bleeding.
Axial CT scans obtained at time of current admission reveal marked distention
of appendix (arrows), which measures 3 cm in diameter and coils in
and out of transverse axial plane. Appendiceal density (measured at level
shown in D) was approximately 50 H.
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The biopsy results were interpreted as an adenoma, and the patient
underwent resection of the appendix and the cecum. At laparotomy, no
additional masses or enlarged lymph nodes were discovered in the abdomen or
pelvis.
The final pathologic diagnosis was recurrent large cell lymphoma, B-cell
type, of the appendix. The tumor involved the appendiceal orifice and also
completely filled the appendiceal lumen. No evidence of lymphoma was present
in any of the resected lymph nodes in the surgical specimen.
Discussion
The incidence of primary appendiceal lymphoma has been estimated at 0.015%
of all appendectomy specimens
[1]. In one series of 8699
appendectomy specimens, 101 were neoplasms, and only three were lymphomas
[2]. Appendiceal lymphomas are
exclusively non-Hodgkin's B-cell lymphomas, especially Burkitt's lymphoma
[2]. In contrast to other
non-Hodgkin's lymphomas, appendiceal non-Hodgkin's lymphomas usually present
when patients are in the second to third decade of life
[2].
Appendiceal lymphoma may present clinically as acute appendicitis
[3,
4]. A review of 46 patients
with appendiceal lymphoma reported that 31 presented with acute or subacute
right lower quadrant pain, and five lymphomas were incidentally discovered at
appendectomy [5]. Patients with
appendiceal lymphoma may also complain of anorexia, weight loss, and nausea
and vomiting, and a right lower quadrant mass may be present at physical
examination [1]. Massive
enlargement of the appendix may be present. Rarely, intussusception or lower
gastrointestinal bleeding may also occur
[1,
2].
Most patients with appendiceal lymphoma reported in the medical literature
did not undergo preoperative cross-sectional imaging, and, to our knowledge,
only scant descriptions exist of appendiceal lymphoma revealed on CT. One such
report of a 65-year-old man who presented acutely with bright red blood in his
rectum described a large ovoid soft-tissue mass in the right lower quadrant on
CT that protruded into the cecal lumen. Primary lymphoma of the appendix was
discovered at surgery [2].
Recently, the Armed Forces Institute of Pathology
[4] reported five cases of
non-Hodgkin's lymphoma of the appendix retrospectively discovered on CT. Three
patients had the initial diagnosis of acute appendicitis, and all three had
periappendiceal inflammatory changes on CT. One patient presented with lower
gastrointestinal bleeding, and another patient had a relapse of a lymphoma
that was initially diagnosed 6 years earlier. CT showed markedly homogeneous
enlargement of the appendix and preservation of the appendiceal morphology in
all five patients. The mean appendiceal diameter was 3.2 cm. In some patients,
the appendiceal wall, which measured more than 1 cm, could be distinguished
from the lumen, which also showed aneurysmal dilatation. Only three of the
five patients had associated retroperitoneal or mesenteric adenopathy. Of the
four patients in whom preoperative CT reports were available, in none was an
appendiceal neoplasm prospectively considered
[4].
Although quite uncommon, mucoceles of the appendix are less rare than
appendiceal lymphoma and should be considered in the differential diagnosis of
appendiceal masses. Mucoceles represent the obstructive dilatation of the
appendiceal lumen by accumulated mucoid material
[6]. Most mucoceles are now
attributed to mucin-secreting tumors, which may be benign or malignant. The
mucocele appears as a low-density oval or round mass that markedly expands the
appendix or that is situated in the expected location of the appendix
[6]. The density of the luminal
contents of the mucocele depends on the exact content and composition of the
mucin but is typically near water density
[7], whereas the density of the
appendiceal mass revealed on CT in our patient was consistent with that of a
soft-tissue lesion.
It is difficult to establish guidelines for therapy of appendiceal
lymphoma. However, because of its rarity, primary appendiceal lymphoma can be
successfully treated with an appendectomy, with or without a limited right
hemicolectomy [1,
3]. Adjunctive therapy depends
on the stage of the disease and the histopathology
[1,
5]. In our patient, it is
difficult to know whether additional therapy was indicated because no other
evidence of recurrent disease was discovered in the abdomen or pelvis.
In summary, radiologists should be aware that lymphoma—both at
initial presentation and when recurrent—can rarely present as an
appendiceal mass on CT and that the absence of associated adenopathy in the
abdomen and pelvis does not exclude the diagnosis of appendiceal lymphoma.
References
- Rao SK, Aydinalp N. Appendiceal lymphoma: a case report.
J Clin Gastroenterol
1991;13:588
-590[Medline]
- Carpenter BW. Lymphoma of the appendix. Gastrointest
Radiol 1991;16:256
-258[Medline]
- Hanna GB, Frizelle FA, Santoro GA. Lymphoma of the appendix: a case
report. G Chir
1997;18:219
-221[Medline]
- Pickhardt PJ, Levy AD, Rohrmann CA Jr, Abbondanzo SL, Kende AI.
Non-Hodgkin's lymphoma of the appendix: clinical and CT findings with
pathologic correlation. AJR
2002;178:1123
-1127[Abstract/Free Full Text]
- Pasquale MD, Shabahang M, Bitterman P, Lack EE, Evans SR. Primary
lymphoma of the appendix: case report and review of the literature.
Surg Oncol
1994;3:243
-248[Medline]
- Zissin R, Gayer G, Kots E, Apter S, Peri M, Shapiro-Feinberg M.
Imaging of mucocele of the appendix with emphasis on the CT findings: a report
of 10 cases. Clin Radiol
1999;54:826
-832[Medline]
- Madwed D, Mindelzun R, Jeffrey RB Jr. Mucocele of the appendix:
imaging findings. AJR
1992;159:69
-72[Abstract/Free Full Text]
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Introduction
Case Report
