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Yilmaz
Bilsel, 3rd General Surgery Clinic, Haydarpasa Immune
Research and Training Center, 34668, Uskudar, Istanbul-Turkey
Emre Balik, Sumer Yamaner, Dursun Bugra, Department of
General Surgery, Istanbul University, Istanbul Medical Faculty,
34093, Capa, Istanbul-Turkey
Correspondence to: Yilmaz Bilsel, Acibadem cad. No:128/9,
Bilsel apt., Acibadem, 34178, Kadikoy, Istanbul-Turkey.
tbilsel@superonline.com
Telephone: +90-216-3396791
Fax: +90-212-5132181
Received: 2004-04-28
Accepted: 2004-06-25
Abstract
Primary rectal lymphoma is a rare presentation of
gastrointestinal lymphomas. Its clinical presentation is
indistinguishable from that of rectal carcinoma. Although surgical
resection is often technically feasible, optimal therapy for
colorectal lymphoma has not yet been identified. We report a case of
primary rectal lymphoma (non-Hodgkin's large cell lymphoma of type
B) with high-grade features that disappeared completely after
chemo-radiotherapy. This case underlines that primary treatment with
systemic chemotherapy and involved-field radiotherapy can be
successful for rectal lymphoma, with surgery reserved for
complications and chemotherapy failures.
ã
2005 The WJG Press and Elsevier Inc. All rights reserved.
Key words: Rectal lymphoma; Systemic chemotherapy;
Involved-field radiotherapy
Bilsel Y, Balik E,
Yamaner S, Bugra D. Clinical and therapeutic considerations of
rectal lymphoma: A case report and literature review. World J
Gastroenterol 2005;
11(3): 460-461
http://www.wjgnet.com/1007-9327/11/460.asp
INTRODUCTION
Lymphoma may occur as a primary lesion or as part of a
generalized malignant process involving the gastrointestinal tract.
The differentiation of these
two forms is very important, as both treatment and prognosis are
different. Primary gastrointestinal lymphomas have been described as
those in which involvement of the alimentary tract predominates or
those with symptoms of gastrointestinal involvement on presentation.
More strict guidelines include the absence of palpable peripheral
lymphadenopathy at the time of presentation; absence of mediastinal
adenopathy on a chest radiograph; a normal peripheral blood smear;
involvement at laparatomy of only the the esophagus, stomach, bowel
or regional lymph nodes (excluding retroperitoneal lymph nodes); and
absence of hepatic and splenic involvement except by direct spread
of the disease from a contagious focus[1].
Primary gastrointestinal
lymphoma accounts for 5% of all lymphomas. Colorectal involvement
accounts for 10-20% of the cases in most studies of gastrointestinal
tract lymphoma[2-5]. Primary colorectal lymphoma is an
uncommon disorder accounting for 0.05% of all colonic neoplasms and
0.1% of primary rectal tumors[6].
Gastrointestinal lymphomas remain the subject of
much debate with regard to therapeutic approaches. The role of
surgery in primary lymphoma of the rectum is controversial. Keeping
these data in mind we decided to describe the recently observed case
of a patient affected by primary lymphoma of the rectum to discuss
the treatment modalities with a brief review of the literature.
CASE REPORT
A 33 year-old male, in May 2001, was admitted to a local
hospital with complaints of pain in lower abdominal regions, rectal
bleeding, diarrhea, and weight loss. Rectoscopy verified a tumor
mass in the rectum, but histology of biopsy specimens revealed no
specific findings other than chronic inflammatory cells.
When
the patient was transferred to our clinic, he had severe abdominal
pain. There was a palpable mass on rectal examination. The mass was
smooth, firm to hard, and fixed to the rectal wall. Abdominopelvic
CT demonstrated an extensive, bulky rectal tumor (Figure 1). There
was no associated intraabdominal adenopathy. A repeat colonoscopy
showed enlargement of the lesion. Large biopsy specimens were taken
for histological diagnosis. The lesion was diagnosed as
non-Hodgkin's large cell lymphoma of B type, with high-grade
features. Results of immunohistochemical staining were CD20 (+), LCA
(+), CD5 (-), TdT (-), and cytokeratin (-). KI was more than 90%. As
additional investigations did not show any evidence of infiltration
to other organs, the disease was staged as clinical stage I rectal
lymphoma. However, the lesion was enlarged enormously during an
observation period of 10 d. This could suggest that the lesion had a
tendency to advance into a more aggressive clinical course.
A
transverse loop colostomy was performed to relieve colonic
obstruction and pain. After six cycles of modified ProMACE schedule
(doxorubicin 30 mg/m2 iv on d 1, cyclophosphamide 600
mg/m2 iv on d 1 and 2, etoposide 120 mg/m2 iv
on d 1, 2 and 3, prednisone 50 mg/m2 im on d 1-6, and
methotrexate 35 mg/m2 iv on d 7), the lesion completely
disappeared. We decided not to resect the rectum. Additionally, the
patient was also given an involved-field radiation therapy (4000 cGy
in 20 fractions for over 4 wk). A complete remission was achieved
and confirmed by abdominal CT scan and colonoscopy with biopsy of
the diseased region. Afterwards, the colostomy was closed. There was
no recurrence during the 3 years of follow up using endoscopy and
biopsy.
Figure
1 Extensive, bulky rectal tumor with no associated
lymphadenopathy shown on abdominal CT scan image.
DISCUSSION
Primary lymphoma of the rectum is a rare disorder. These lesions
are predominantly non-Hodgkin lymphomas, with colorectal involvement
seen in less than 5% of autopsies for Hodgkin disease[6].
Rectal lymphoma usually presents with signs and symptoms suggestive
of primary rectal carcinoma. Patient with rectal lymphoma usually
seek treatment because of rectal bleeding or an alteration in bowel
habits. It has been reported that the majority of patients are
symptomatic complaining of abdominal pain, nausea, vomiting, fever,
and weight loss[2-6]. Abdominal CT and endoscopy with
biopsy are the most useful diagnostic tests. The growth patterns
seen in the rectum varied. The growth might be bulky and protuberant
with ulceration; it may appear as annular or plaque like thickenings
of the bowel wall or manifest as multiple lymphomatous polyposes[7].
The macroscopic appearance bears no relationship to the histologic
structure.
When CT revealed a
combination of a focally or diffusely infiltrative process of the
colon and extensive abdominal and/or pelvic adenopathy, lymphoma
should be the primary consideration in the differential diagnosis
and must be excluded by endoscopic biopsy. However, if adenopathy
was not associated with a primary colorectal lymphoma, it might be
difficult radiologically to distinguish this lesion from a primary
adenocarcinoma of the colon. This difficulty arises predominantly in
the settings of solitary mass lesions. Primary colorectal lymphomas
manifested as discrete masses tended to have a greater depth of
mural invasion than infiltrative lesions[8].
Colorectal lymphomas
commonly cause concentric wall thickening and they often destroy the
full thickness of the bowel wall without an associated desmoplastic
reaction. Some authors cited a typical wall thickness in colonic
lymphoma of 7 to 12 cm
from the lumen to the serosa[9].
Two risk factors have
been reported to be associated with the development of primary
colorectal lymphoma: inflammatory bowel disease and immunosupression
(posttransplant, AIDS, or immune disorder). The aggressive nature of
AIDS-related lymphoma could usually result in a disseminated disease
at the time of diagnosis[10,11].
Treatment
of colorectal lymphomas remains uncertain. Some studies suggested
that the primary treatment was to attempt resection when the disease
was judged to be resectable because patients with residual diseases
had a poor prognosis[12-14]. Given the morbidity
following resection of the rectum, the value of surgery for primary
lymphoma of the rectum is a subject to be debated.
In some
series, radiotherapy or chemotherapy was considered a treatment of
choice, because patients with residual diseases after surgery had a
poor prognosis, and adjuvant treatment would increase the duration
of disease free survival[15,16]. For patients with
widespread diseases, those in whom the rectum was secondarily
involved, or patients with AIDS, operation would seem inappropriate.
These patients may benefit from chemotherapy and radiotherapy.
Radiotherapy alone has
not been studied extensively in this disease, although it has been
used in a small number of patients usually in combination with
cytotoxic chemotherapy. It remains to be seen what role radiotherapy
may play in the treatment of this disease.
Diffuse
large B cell lymphoma of the large bowel is generally treated with a
uniform therapeutic approach: aggressive surgery followed by
adjuvant chemotherapy. However, most of the studies included
patients with primary gastric or small bowel and large bowel in the
same analysis, and also patients at different stages, different
histology, and different surgical approaches. Lymphoma of the rectum
should be considered as a different clinico-pathological entity with
different behaviors, histology and clinical presentation. For this
reason, treatment should be defined based on this special clinical
condition[17].The therapeutic experience in this selected
group of patients is limited, because even in large tertiary
referral centers the number of cases is small. The analysis of those
retrospective data regarding surgery, radiation therapy, and
chemotherapy is also difficult to interpret because of lack of
uniformly accepted regimens.
This case showed that a
rectal lymphoma could be treated without resection of the rectum.
Although it is not possible to make a legitimate conclusion with a
single case, with an optimistic approach, we may conclude that the
intensity and preferred modality of optimal therapy for rectal
lymphoma should be determined by the clinical stage of the disease.
Patients with a high tumor bulk, a low-grade histology, and no
evidence of local or distant metastasis can be managed by combined
chemo-radiotherapy, reserving surgery only for complications and
chemotherapy failures.
REFERENCES
1
Dawson IM, Cornes JS, Morson BC. Primary malignant lymphoid
tumors of the intestinal tract. Br J Surg
1961; 49: 80-89
2
Kashimura A, Murakami T. Malignant lymphoma of the large
intestine-15-year experience and review of literature.
Gastroenterol Jpn 1976; 11: 141-147
3
Lewin KJ, Ranchod M, Dorfman RF. Lymphomas of the
gastrointestinal tract-a study of 117 cases presenting with
gastrointestinal disease. Cancer
1978; 42: 693-707
4
Dragosics B, Bauer P, Radaszkiewicz T. Primary
gastrointestinal non-Hodgkin's lymphomas: a retrospective
clinicopathologic study of 150 cases.
Cancer 1985; 55: 1060-1073
5
Henry CA, Berry RE. Primary lymphoma of the large intestine.
Am Surg 1988; 54: 262-266
6
Dodd GD. Lymphoma of the hollow abdominal viscera. Radiol
Clin North Am 1990; 28: 771-783
7
Ohri SK, Keane PF, Sackier JM, Hutton K, Wood CB. Primary
rectal lymphoma and malignant lymphomatous polyposis.
Two cases illustrating current
methods in diagnosis and management. Dis Colon Rectum 1989; 32:
1071-1074
8
Wyatt SH, Fishman EK, Hruban RH, Siegelman SS. CT of primary
colonic lymphoma. Clin Imaging 1994; 18: 131-141
9
Megibow AJ, Balthazar EJ, Naidich DP, Bosniak MA. Computed
tomography of gastrointestinal lymphoma. Am J
Roentgenol 1983; 141: 541-547
10
Bartolo D, Goepel JR, Parson MA. Rectal malignant lymphoma in
chronic ulcerative colitis. Gut 1982; 23: 164-168
11
Fan CW, Chen JS, Wang JF, Fan HA. Perforated rectal lymphoma
in a renal transplant recipient: Report of a case. Dis
Colon Rectum 1997; 40: 1258-1260
12
Jinnai D, Iwasa Z, Watanuki T. Malignant lymphoma of the
large intestine-operative results in Japan. Jpn J Surg
1983; 13: 331-336
13
Zighelboim J, Larson MV. Primary colonic lymphoma. Clinical
presentation, histopathologic features and outcome with
combination chemotherapy. J Clin
Gastroenterol 1994; 18: 291-297
14
Fan CW, Changchien CR, Wang JY, Chen JS, Hsu KC, Tang RT,
Chiang JM. Primary colorectal lymphoma. Dis Colon
Rectum 2000; 43: 1277-1282
15
Bellesi G, Alterini R, Messori A, Bosi A, Bernardi F, di
Lollo S, Ferrini PR. Combined surgery and chemotherapy for the
treatment of primary gastrointestinal
intermediate-and high-grade non Hodgkin's lymphoma. Br J
Cancer
1989; 60: 244-248
16
Rackner VL, Thirlby RL, Ryan JA Jr. Role of surgery in
multimodality therapy for gastrointestinal lymphoma. Am J Surg
1991; 161: 570-575
17
Aviles A, Neri N, Huerta-Guzman J. Large bowel lymphoma: An
analysis of prognostic factors and therapy in 53 patients.
J Surg Oncol 2002; 80: 111-115
Assistant
Editor Guo SY Edited by
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