Intestinal follicular lymphoma is uncommon, and its endoscopic appearance has seldom
been described [1]
[2]
[3]
[4]. We report five patients with primary intestinal follicular lymphoma in whom the
whole of the gastrointestinal tract were examined using esophagogastroduodenoscopy
(EGD), colonoscopy, and double-balloon enteroscopy [5].
The clinical and endoscopic features of the five patients are summarized in Table
[1]. All the patients underwent EGD, colonoscopy, and double-balloon enteroscopy via
antegrade and retrograde approaches (Fujinon-Toshiba ES Systems Co., Tokyo, Japan).
Four patients (80 %) had lesions in multiple sites in the gastrointestinal tract.
The most frequent site was the jejunum, followed by the duodenum and the ileum. In
three patients, EGD revealed multiple small, whitish nodules in duodenum, predominantly
around the ampulla of Vater. On colonoscopy, one patient was found to have a superficially
elevated lesion with aggregates of small nodules in the rectum (Figure [1]), in addition to the ileal lesions. Interestingly, this Helicobacter pylori-negative patient showed regression of both rectal and ileal lesions on follow-up
colonoscopy 15 months after antibiotic treatment.
Figure 1 Colonoscopic view showing a superficially elevated lesion composed of aggregates of
small nodules in the rectum in patient 3.
Table 1 Clinical and endoscopic features of five patients with primary intestinal follicular
lymphoma
| Patient |
Age/Sex |
Symptoms |
Stage |
Sites involved |
EGD findings |
Colonoscopic findings |
DBE findings |
Treatment |
Response to treatment |
Clinical outcome |
| 1 |
55/M |
None |
I |
D |
Multiple small nodules (D) |
Normal |
LP-like* (I) |
Antibiotics † |
Not evaluated |
Alive with disease, 3 months |
| 2 |
58/M |
Abdominal pain, emesis |
II2
|
J, I** |
Normal |
Normal |
Stenosis, mass, fold swelling, LP (J) |
Surgery plus R-CHOP |
Complete remission |
Alive without disease, 12 months |
| 3 |
60/F |
None |
I |
J, I, R |
Normal |
Nodule aggregates (R), LP (I) |
LP (J, I) |
Antibiotics ‡ |
Partial remission |
Alive with disease, 20 months |
| 4 |
64/M |
None |
II1
|
D, J |
Multiple small nodules (D) |
Normal |
Mass, fold swelling, LP (J) |
R-CHOP |
Partial remission |
Alive with disease, 10 months |
| 5 |
71/M |
Abdominal pain, nausea |
IV |
D, J |
Multiple small nodules (D) |
Normal |
Stenosis, mass, fold swelling, LP (J) |
R-CHOP |
Partial remission |
Alive with disease,
6 months |
EGD, esophagogastroduodenoscopy; DBE, double-balloon enteroscopy; LP, lymphomatous
polyposis; R-CHOP, rituximab plus cyclophosphamide, doxorubicin, vincristine, and
prednisolone.
D, duodenum; I, ileum; J, jejunum; R, rectum.
* Numerous small polypoid lesions were observed but biopsies failed to reveal lymphoma
cells.
** Detected by intraoperative enteroscopy.
† Lansoprazole plus amoxicillin and clarithromycin for 7 days.
‡ Rabeprazole plus amoxicillin, clarithromycin, and metronidazole for 14 days |
Double-balloon enteroscopy revealed multiple polypoid lesions resembling lymphomatous
polyposis in long segments of the jejunum or ileum in four patients (80 %) (Figure
[2 ]
a, b). The remaining patient also showed lymphomatous polyposis-like lesions in the ileum,
but multiple biopsies failed to reveal lymphoma cells. Swollen Kerckring folds with
mass formation was observed in three patients, and severe jejunal stenosis in two
(Figure [2 ]
c, d). In all the patients, a histologic diagnosis of grade 1 follicular lymphoma was
made by endoscopic biopsy, with infiltration of neoplastic cells positive for CD20,
CD79a, CD10, and BCL2 but negative for CD3, CD5, and cyclin D1.
Figure 2 Double-balloon enteroscopic views of the jejunum in patients with intestinal follicular
lymphoma. Numerous polypoid lesions of varying sizes, resembling lymphomatous polyposis,
were seen, illustrated by images from patient 4 (a) and patient 5 (b). Patient 5 also showed mass formation (c), and patient 2 had a severe jejunal stenosis due to mass formation with swelling
of Kerckring folds (d).
A variety of endoscopic features of intestinal follicular lymphoma were observed.
Lymphomatous polyposis is considered to be one of the most characteristic findings,
and this can often be detected using double-balloon enteroscopy. Double-balloon enteroscopy
is a valuable tool for the detection and accurate diagnosis of the small-intestinal
lesions of this disease.
Acknowledgment
The authors are grateful for the help kindly provided by Professor Morishige Takeshita
(Fukuoka University, Japan).
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