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An Article Submitted to

World Journal of Colorectal Surgery


Manuscript 1183

Management of Primary Rectal


Lymphoma: A Single Institution
Experience
Scott R. Kelley M.D., FACS
Kevin T. Behm M.D.

Amit Merchea M.D.

Zaid M. Abdelsattar M.D.

Eric J. Dozois M.D., FACS, FASCRS

TriHealth Good Samaritan Hospital, Cincinnati Ohio, United States of America,


scott kelley@trihealth.com

Mayo Clinic, Jacksonville, Florida, United States of America

Mayo Clinic, Rochester Minnesota, United States of America

Mayo Clinic, Rochester Minnesota, United States of America

Mayo Clinic, Rochester Minnesota, United States of America


c
Copyright 2014
by the authors. All rights reserved. No part of this publication may be reproduced, stored in a retrieval system, or transmitted, in any form or by any means, electronic,
mechanical, photocopying, recording, or otherwise, without the prior written permission of the
publisher, bepress, which has been given certain exclusive rights by the author.

Management of Primary Rectal Lymphoma: A


Single Institution Experience
Scott R. Kelley M.D., FACS; Amit Merchea M.D.; Zaid M. Abdelsattar M.D.;
Kevin T. Behm M.D.; and Eric J. Dozois M.D., FACS, FASCRS

Abstract
BACKGROUND: There is a paucity of literature regarding primary rectal lymphoma. Due to
its rarity, and frequent changes in histological subtyping and classification, treatment has not been
standardized. The objective of this study was to retrospectively review our experience with the
management of primary rectal lymphoma over a 30-year period.
METHODS: All adult patients (?18 years) diagnosed with primary rectal lymphoma between
1982 and 2012 were identified. Demographics, symptoms, mode of diagnosis, type of lymphoma,
grade, stage, risk factors, tumor size, treatment(s), response, and status following treatment were
reviewed.
RESULTS: Seventeen patients were identified (10 male) with an average age of 53.6 years (range,
30-79). The most common subtypes were mucosa associated lymphoid tissue type lymphoma
(n=7, 41%) and diffuse large B cell lymphoma (n=6, 35%). Four treated with a combination
of surgical / endoscopic resection and chemotherapy achieved a complete response. Two treated
with chemotherapy alone recurred. Four endoscopic resections resulted in 2 recurrences. Out
of 5 treated with surgery alone, 2 recurred. One treated with H. pylori eradication resulted in a
complete response, and 1 refused treatment and died. Median follow-up was 52 months (range,
18-138) months. At last follow-up, 15 patients (88%) were alive with no evidence of disease.
CONCLUSIONS: Our findings suggest that multimodal therapy including both surgical / endoscopic removal and chemotherapy was associated with better outcomes, although the numbers of
different types of lymphoma are too small to draw any definitive conclusions. A multi-institutional
collaboration will be necessary to determine the best treatment approach.
KEYWORDS: Primary rectal lymphoma, management, outcomes

Kelley et al.: Management of Primary Rectal Lymphoma: A Single Institution Exper

INTRODUCTION
The gastrointestinal (GI) tract is the predominant site for extranodal lymphomas
(stomach 48%, small bowel 26%, colorectal 12%, pancreas 2%, esophagus 1%).

Well described in the literature, GI lymphomas can occur as a primary (localized)


or secondary (systemic) process. Despite their rarity, primary GI lymphomas are
important since their diagnosis, management, and prognosis is distinct from other
GI malignancies and lymphomas of other sites. While comprising less than 1%
of all GI lymphomas, primary rectal lymphoma (PRL) remains the third most
common cause of rectal cancer after adenocarcinoma (90-95%) and carcinoid
(5%). [1]

There is a paucity of literature regarding PRL. Due to its rarity, and the frequent
changes in histological subtyping and classification, treatment has not been
standardized. As a result, interpretation of outcomes in the current literature is
difficult. Using the most current staging criteria, histology, and classification
systems, we reviewed our institutions experience over a 30-year period with the
management of PRL.

MATERIALS / METHODS
Institutional review board approval was obtained. Surgical and pathological
databases were used to retrospectively identify all patients treated for PRL

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between 1982 and 2012 at the Mayo Clinic in Rochester, MN. We included all
adult patients (18 years) with a diagnosis of PRL based on the Dawson criteria:
no palpable superficial lymphadenopathy on initial physical examination, no
mediastinal lymphadenopathy, normal total and differential white blood cell
counts, normal bone marrow biopsy, no liver or splenic involvement, and only
regional nodes affected by disease. [2]

Patient records were reviewed for demographic features, presenting symptoms,


method of diagnosis, type of lymphoma including pathological features, history of
inflammatory bowel disease, immunocompromised status, treatments received,
tumor size, and status following treatment. Descriptive statistics are reported as a
percentage of the total and continuous variables as the mean, or median, and
range.

RESULTS
Seventeen patients were identified (10 male, 58%) with a median age of 58 years
(range, 30-79). Sixteen patients (94%) had Non-Hodgkin lymphoma. The most
common histopathological subtypes were mucosa associated lymphoid tissue
(MALT) type lymphoma (n=7, 41%) and diffuse large B cell lymphoma (DLBCL)
(n=6, 35%). A single case of Hodgkins lymphoma (HL), anaplastic large cell
lymphoma (ALCL), follicular lymphoma (FL), and mantle cell lymphoma (MCL)
were also identified (Table 1).

Kelley et al.: Management of Primary Rectal Lymphoma: A Single Institution Exper

A total of 7 patients (41%) were either taking immunosuppressants, diagnosed


with HIV, and/or had inflammatory bowel disease (IBD). A single case of ALCL
was noted in a patient taking azathioprine for chronic ulcerative colitis (CUC),
and DLBCL was found in 3 patients with CUC and 1 with indeterminate colitis
(IC). Of those with DLBCL and IBD, 2 with CUC were on immunosuppression.
HIV was noted in 1 patient with HL, and a solitary case of MALT lymphoma was
seen in a patient treated with tacrolimus and prednisone following liver
transplantation (Table 1).

Five patients were noted to be asymptomatic at presentation while the remainder


presented with a combination of rectal bleeding, weight loss, fatigue,
constipation, anorectal pain, and a change in stool caliber. In 3 patients, the
presenting symptoms were not documented (Table 1).

The method of diagnosis was not documented in 10 (58%) patients. One lesion
was found on digital rectal examination, another was diagnosed following a
proctocolectomy for CUC, and the 5 were found endoscopically (Table 1).

Stage at diagnosis was I-EA in 14 (82%), II-EA in 2 (12%), and unknown in 1


who refused further workup and treatment. Both patients with stage II-EA were
diagnosed with DLBCL. Histologic grade was only recorded in 8 (47%), with a
single case of low grade FL and high grade DLBCL. The remaining 6 cases
were all low grade MALT lymphoma (Table 2).

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Tumor size was documented in 13 (76%) patients and ranged from multiple small
rectal nodules to 15cm. Macroscopic tumor characteristics and distance from the
anal verge was not recorded (Table 2).

Treatment details and response patterns for individual patients are outlined in
Table 2, with all treatments administered electively. A single case of ALCL
associated with immunosuppression and CUC persisted following treatment with
chemotherapy (R-CHOP). A total proctocolectomy with creation of an end
ileostomy resulted in a complete response. Patients with DLBCL treated with a
combination of resection and chemotherapy resulted in a complete response in
all (n=5). One case of DLBCL in an immunocompetent patient treated with RCHOP alone resulted in recurrence. Autologous stem cell transplantation was
administered, though unsuccessful. A solitary case of FL resected
endoscopically recurred, though a complete response was obtained after
treatment with chemotherapy (Rituximab). One patient with HL associated with
HIV refused all workup and treatment. Out of 7 cases of MALT PRL all were
initially treated with resection alone, and 3 experienced recurrence. One was
treated with chemotherapy (chlorambucil), 1 with H. pylori eradication consisting
of twice daily dosing of lansoprazole 30mg, amoxicillin 500mg, and clarithromycin
500mg for 2 weeks, and 1 with re-resection, all of which had subsequent
complete pathological responses. One documented case of MCL demonstrated
compete response after undergoing a low anterior resection (Table 2).

Kelley et al.: Management of Primary Rectal Lymphoma: A Single Institution Exper

Median follow-up and disease free survival was 51 months (range, 8-138). At
last follow-up 15 patients (88%) were alive with no evidence of disease. Two
patients (12%) died from their disease at 8 and 21 months after diagnosis. In
these 2 patients, 1 with a greater than 10cm stage II-EA DLBCL experienced a
recurrence within 5 months of R-CHOP and underwent autologous stem cell
transplantation without success, and the other with HL and HIV refused all
treatment (Table 2).

DISCUSSION
This review of 17 patients treated for PRL at our institution over a 30-year period
demonstrated significant variations in treatment modalities. The most common
types were MALT and DLBCL. The best overall response rates were observed
with a multimodal approach that included resection and chemotherapy.
Although different proposals exist for diagnosing primary versus systemic rectal
lymphoma, the Dawson Criteria is the most widely used and accepted, and the
one we adhered to in our study. [2-8] The criteria include no palpable superficial
lymphadenopathy on initial physical examination, no mediastinal
lymphadenopathy, normal total and differential white blood cell counts, normal
bone marrow biopsy, no liver or splenic involvement, and only regional nodes
affected by disease. When the criteria were proposed in 1961, operative findings
dictated primary versus systemic involvement. Currently, we have the benefit of
high-resolution imaging, colonoscopic examination, and pathological confirmation

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of biopsies to assist in accurate preoperative diagnosis, classification, and


staging.

Systems for classifying lymphomas have changed dramatically through the years
making it difficult to compare results from clinical studies. Early studies
employed a completely different nomenclature than what is utilized today. In
1982, the International Working Formula Classification system was devised in an
attempt to consolidate the different classification systems (Rappaport, Lennert,
Lukes and Collins, Kiel, British National Lymphoma Investigation, Dorfman) being
utilized around the world at that time. The Working Formula Classification was a
clinical system based on histology (low, intermediate, high grade, and others),
and attempted to classify lymphomas into prognostic categories. [9] The
introduction of the Revised European American Classification (REAL) system in
1994, arranged lymphomas by cell type of origin, added immunophenotyping,
genetic, and morphologic features, and identified the thirteen most common
types of non-Hodgkins lymphoma. [10] In 2008 the World Health Organization
(WHO) instituted an updated classification system by adding information on cell
surface markers and genetics, distinguished between B, T, and natural killer (NK)
cells, and included seventy different types of known lymphomas. [11] Our study
reviews our institutions experience in the management of PRL using the most
current histological, classification, and staging criteria.

Kelley et al.: Management of Primary Rectal Lymphoma: A Single Institution Exper

Staging of lymphoma is based on the Ann Arbor system and the Cotswolds
modification. Stage I localizes to a single region, Stage II involves two separate
areas confined to one side of the diaphragm, Stage III demonstrates spread to
both sides of the diaphragm, and Stage IV encompasses diffuse or disseminated
involvement. Letter designations in reference to lymphoma include: A - absence
of constitutional symptoms, B - presence of constitutional (systemic) symptoms,
and E - involvement of a single contiguous extranodal site. Constitutional
symptoms include unexplained fevers of at least 101.5F, drenching night
sweats, and loss of more than 10% of body weight over the previous 6 months
(without dieting). PRL is restricted to stage I, and II when only regional nodes
are affected by disease. [12-13] In our series, 14 (82%) patients had Stage I-EA
disease, 2 (12%) Stage II-EA disease, and 1 (6%) who refused all workup and
treatment an unknown stage.

The 5 largest series in the literature detailing a combined total of 70 cases of


PRL revealed a female to male ratio of 1.3:1, and an average age of 56.3 years.
Appropriately documented mean disease free survival in 4 of the studies was
58.8 months. [2-6] Other than a slight male predominance (58% / 1.4:1) this
compares well to our data.

Mucosa associated lymphoid tissue lymphoma has been the most commonly
diagnosed type of PRL since the adoption of the Internal Working Formula
Classification in 1982, and the publication by Isaacon and Wright in 1983

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describing MALT lymphoma as a distinct type of B-cell lymphoma. [14] The


second most commonly documented type is DLBCL. [15-16] Primary Hodgkins
rectal lymphoma is extremely rare with few reported cases in the literature. [1720] In our series we found 7 (41%) cases of MALT, 6 (35%) cases of DLBCL,
and 1 case each of HL, mantle cell lymphoma, FL, and anaplastic large cell
primary rectal lymphoma.

Patients typically present with non-specific symptoms including vague abdominal


pain and a change in bowel habits. Other presenting symptoms can include
weight loss, tenesmus, peritonitis, obstruction, and bleeding. In our series we
found similar symptoms. Mucosal ulceration does not typically occur and a high
index of suspicion is required to make the correct diagnosis. [4-6]

Although no direct causal link has been established, multiple risk factors are
associated with PRL. These include immunocompromised states, inflammatory
bowel disease, perineal radiation, acquired immunodeficiency (HIV/AIDS), and
male homosexuality. [7, 21-24] Our series identified 7 patients with risk factors
associated with PRL. Length of time from the initial diagnosis of IBD and the
duration and dosage of immunosuppressant medication was not recorded in our
study. For those taking immunosuppressantswe identified 4 (23%) patients taking
immunosuppressants, 1 HIV positive patient, and 5 (29%) patients with
inflammatory bowel disease. Of those with IBD, 4 had CUC and 1 was
diagnosed with IC. Among patients taking immunosuppressants in our study, 3

Kelley et al.: Management of Primary Rectal Lymphoma: A Single Institution Exper

were being treated for CUC. The length of time from the initial diagnosis and the
duration or dosage of immunosuppressant therapy was not specifically evaluated
in this study.

A variety of treatment options for PRL have been described in the literature
including observation, local or endoscopic excision, surgical resection,
chemotherapy, radiotherapy, H. pylori eradication, antibiotic therapy, intraluminal
hyperthermia, and a combination of these modalities administered in a
neoadjuvant or adjuvant setting. Most treatments are described in the context of
a single case, and few large series have been published. Prior to the Working
Formula Classification, Dawson et al. reported on 12 cases of PRL and
concluded that surgery appeared to provide a survival advantage. [2] In 1962
Culp et al., from Mayo Clinic, reviewed 20 cases over a 10 year period and
concluded the best means of treatment was a combined approach of surgery and
radiation. [3] Perry et al., from St. Marks Hospital, published their results on 22
patients and recommended surgical resection as the treatment of choice. [4]
Devine and colleagues reported an additional twelve cases in 1986 and
concluded that primary lymphoma of the rectum should be treated with surgical
excision followed by radiation therapy. If the tumor cannot be excised
completely, debulking likely does not provide palliation, and chemoradiotherapy
should be the treatment of choice. [5] The last large series to describe PRL prior
to the adoption of the Working Formula Classification was published by
Shepherd et al. in 1988, in which they concluded surgery should be the primary

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10

treatment with radiation and/or chemotherapy reserved for advanced cases. [6]
Following the Working Formula Classification only a few small case series have
been published. In 1998 Matsumoto et al. described 6 cases of mucosal
associated lymphoid tissue of the colon and rectum, of which 3 cases were
primary rectal involvement. These 3 cases were treated with a combination of
local excision, partial resection, and H. pylori eradication, and no definitive
conclusions could be drawn. [25] Niino and Yamamoto outlined 8 cases of MALT
PRL in 2010, in which all patients were treated with antibiotic therapy. Five
experienced a complete response, and 3 did not respond, which prompted
further treatment with chemotherapy. Response to chemotherapy was not noted.
[26] Chihara et al., in 2010, reported on 2 stage IIA MALT PRL patients who
received R-CHOP chemotherapy resulting in a complete response. [27] In 2012
Okamura et al. documented 3 cases of MALT PRL treated with radiotherapy
only, and concluded that radiation at a total dose of 30 Gy is both effective and
safe for the treatment of rectal MALT lymphoma. [28] In our series, a multimodal
approach including surgical / endoscopic resection and chemotherapy appeared
to provide a slight advantage.

There is a paucity of published literature regarding PRL. It is often included in


the category of lymphomas of the gastrointestinal tract, and not considered
separately. Many reported cases of PRL do not adhere to the Dawson Criteria,
describe secondary rectal involvement, illustrate previously published cases,
represent non-rectal synchronous lesions, or are poorly documented with short

Kelley et al.: Management of Primary Rectal Lymphoma: A Single Institution Exper

11

follow-up. [15, 24, 29-36]

As noted by Dawson et al. in 1961, no one centre can hope to see enough
primary intestinal (lymphoma) cases to be able to lay down any generally useful
prognostic criteria or to arrive at any definite conclusions about the best methods
of treatment. [2] Unfortunately, this statement still holds true today. Even in
large tertiary referral centers, and secondary to the rarity of the disease, the
number of cases is too small to publish generalized prognostic criteria, or to
arrive at definitive conclusions regarding the best methods of treatment. This
represents the main limitation of our study. Review of the literature would
suggest that multimodal therapy provides an advantage in regards to decreased
recurrence, although analysis of the data regarding treatment is difficult due to a
lack of uniformity, which is also a limitation in our study.

CONCLUSIONS
In our cohort, the most common type of primary rectal lymphoma was mucosa
associated lymphoid tissue and diffuse large B-cell lymphoma. Forty-one
percent of our patients had inflammatory bowel disease and/or were
immunocompromised. This study suggests that multimodal therapy that includes
both surgical / endoscopic removal and chemotherapy was associated with better
outcomes, although the numbers of different types of lymphoma are too small to
draw any definitive conclusions. A multi-institutional collaboration to collect data

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12

on this population will be necessary to determine the best treatment approach in


patients with primary rectal lymphoma.

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18

Table 1 - Demographic, Presentation, and Diagnostic Information


Case

Diagnosis

Date
Diagnosed

Age at
Diagnosis

Sex

ALCL

1/05

30

DLBCL

9/99

34

DLBCL
DLBCL
DLBCL

10/99
4/00
3/01

77
34
58

M
M
M

DLBCL

4/09

38

DLBCL

5/10

41

FL
HL
MALT
MALT
MALT
MALT
MALT

8/02
11/88
5/94
10/97
10/97
3/98
1/03

79
48
68
58
64
69
43

F
M
F
F
F
F
F

MALT

4/06

68

16

MALT

4/10

31

None
HIV
None
None
None
None
None
Immunosuppression
(Tacrolimus,
Prednisone)
None

17

MCL

2/94

71

None

3
4
5
6

8
9
10
11
12
13
14
15

Risk Factors

Symptoms

CUC and
Immunosuppression
(Azathioprine)
CUC and
Immunosuppression
(6-MP, Prednisone)
None
CUC
IC

Rectal Pain, Weight


Loss

CUC and
Immunosuppression
(Infliximab, Prednisone)
None

ABD Abdominal
ALCL Anaplastic Large Cell Lymphoma
BRBPR Bright Red Bleeding Per Rectum
CUC Chronic Ulcerative Colitis
DLBCL Diffuse Large B-Cell Lymphoma
DRE Digital Rectal Examination
FL Follicular Lymphoma
HIV Human Immunodeficiency Virus
HL Hodgkins Lymphoma
IC Indeterminate Colitis
MCL Mantle Cell Lymphoma
MALT Mucosa Associated Lymphoid Tissue
NR Not Recorded
6MP 6 mercaptopurine

Medical Refractory CUC


(BRBPR, diarrhea, ABD
discomfort)
BRBPR and Anal Pain
BRBPR
Asymptomatic
Medical Refractory CUC
(BRBPR, diarrhea, ABD
discomfort)
Rectal Pain, Weight
Loss, Stool Caliber
Change
Asymptomatic
NR
NR
Asymptomatic
BRBPR
Asymptomatic
Asymptomatic, Anemia
Fatigue

Method of
Diagnosis
NR

Post Surgery
NR
NR
Palpable lesion
on DRE
NR

NR
Colonoscopy
NR
NR
Colonoscopy
Colonoscopy
NR
Colonoscopy
Colonoscopy

Constipation, Bloating,
Intermittent Nausea
NR

NR
NR

Kelley et al.: Management of Primary Rectal Lymphoma: A Single Institution Exper

19

Table 2 - Tumor Information


Case

Diagnosis

Stage

Grade

Tumor Size

Treatment

Response

ALCL

I-EA

NR

NR

R-CHOP

2
3
4

DLBCL
DLBCL
DLBCL

I-EA
I-EA
II-EA

NR
NR
NR

TPC/EI+CHOP
APR + CHOP

5
6
7

DLBCL
DLBCL
DLBCL

I-EA
I-EA
II-EA

High
NR
NR

10cm
4.5cm
Multiple small
rectal nodules
8mm
NR
>10cm

Persistence after chemo


CR after TPC / EI
CR
CR
CR

FL

I-EA

Low

5mm

ER

9
10

HL
MALT

NR
I-EA

NR
NR

NR
4cm

Refused
TAE

11

MALT

I-EA

Low

15cm

ER

12
13
14
15

MALT
MALT
MALT
MALT

I-EA
I-EA
I-EA
I-EA

Low
Low
Low
Low

3.5cm
1.2cm
1cm
8mm

TAE
ER
ER
HPE

16

MALT

I-EA

Low

1cm

TAE

17

MCL

I-EA

NR

NR

LAR

ER + CHOP
ER + R-CHOP
TPC/IPAA
R-CHOP

CR
CR
Recurred - 2010
Autologous stem cell
transplant
Recurred - Year NR
CR after Rituxan
N/A
Recurred - 1997
CR after Chlorambucil
Recurred - 2002
CR after repeat ER
CR
CR
CR
CR
Recurred - 2010
CR after HPE
CR

Follow-up
(months)
48

Status at Last
Follow-up
NED

71
52
138

NED
NED

133
24
21

NED
NED
NED
Deceased

120

NED

8
98

Deceased
NED

107

NED

53
29
78
49

NED
NED
NED
NED

18

NED

50

NED

ALCL Anaplastic Large Cell Lymphoma


APR Abdominoperineal Resection
CHOP Cyclophosphamide / Hydroxydaunorubicin / Oncovin / Prednisone
CR Complete Response
DLBCL Diffuse Large B-Cell Lymphoma
EI End Ileostomy
ER Endoscopic Removal
FL Follicular Lymphoma
HPE H. pylori Eradication
HL Hodgkins Lymphoma
IPAA Ileal Pouch Anal Anastomosis
LAR Low Anterior Resection
MCL Mantle Cell Lymphoma
MALT Mucosa Associated Lymphoid Tissue
NED No Evidence of Disease
NR Not Recorded
RCHOP Rituxumab + CHOP
TAE Transanal Excison
TPC Total Proctocolecotmy

Produced by The Berkeley Electronic Press, 2014