Combination of MALT Lymphoma of the
Nasopharynx and the Rectum: a Case Report
F. Elloumia, W. Sialaa, S. Yahiaouia, R.
Lahmara, T. Boudawarab, M. Elloumic, N. Tahrid, J. Daouda
1. Radiotherapy Department, Habib-Bourguiba
hospital, Sfax, Tunisie.
2. Pathology Department, Habib-Bourguiba
hospital, Sfax, Tunisie.
3. Hematology Department, Hédi-Chaker
hospital, Sfax, Tunisie.
4. Hepatogastroenterology Department,
Hédi-Chaker hospital, Sfax, Tunisie.
Corresponding author: W.
Sialaa; Email: firstname.lastname@example.org
Citation: Elloumia F, Sialaa
W, Yahiaouia S, Lahmara R, Boudawarab T, Elloumic M, Tahrid N, Daouda J. Combination
of MALT lymphoma of the nasopharynx and the rectum: a case report. J
Nasopharyng Carcinoma, 2014, 1(5): e5. doi:10.15383/jnpc.5.
Competing interests: The authors have declared that no competing
Conflict of interest: None.
Copyright:2014 By the Editorial Department of Journal of
Nasopharyngeal Carcinoma. This is an open-access article distributed under the
terms of the Creative Commons Attribution License, which permits unrestricted
use, distribution, and reproduction in any medium, provided the original
author and source are credited.
Mucosa-associated lymphoid tissue (MALT) is an
extra-nodal marginal zone B cell lymphoma, it accounts for about 7-8% of all
non hodgkinian lymphomas. It occurs most frequently in the stomach (70% of the
cases), but can also occur on non gastric tissue especially the salivary
glands, orbit, thyroid, lungs. However, colo-rectal and nasopharyngeal
involvements are extremely rare. We report the case of a combination of these
unusual locations in a 51-years-old patient in whom MALT lymphoma was diagnosed
in 2006. The patient had rectal bleeding. Colonoscopy has shown the presence of
large ulcerated folds of the rectum. Pathological and immunohistochemical study
confirmed the diagnosis of MALT lymphoma. No extra intestinal involvement was
found on the staging evaluation. The patient had a pelvic radiation at the dose
of 40 Gy with endoscopic and histological remission. Eight months after the end
of treatment, clinical examination showed an oropharyngeal mass. The computed
tomography revealed a thickening of the posterior nasopaharyngel wall extended
to the oropharynx. The diagnosis of MALT lymphoma was confirmed by the biopsy.
The patient had radiotherapy on the Wadayer ring at the dose of 44 Gy.
Currently, five years after the end of treatment, the patient is on complete remission.
Key words: Malt
lymphoma, rectum, nasopharynx
-associated lymphoid tissue (MALT) lymphoma is a low grade extra-nodal B-cell
lymphoma including marginal zone. It represents 7-8% of all non-Hodgkin
lymphomas (1). It occurs on native MALT (Lung, Small Bowl) or occurs on
acquired one resulting from a chronic inﬂammatory disorder (Stomach).
Many authors suggest that it has its origin from an antigenic stimulation of
autoimmune disorders (2).stomach is the commonest site of MALT lymphoma
followed by orbit, salivary gland, thyroid and superior respiratory tract (3).
Rectal and nasopharyngeal locations have been rarely described in the
literature. We report a case of a female patient followed for both rectal and
nasopharyngeal MALT lymphoma, and we propose, through a literature review to
discuss the etiopathogenic factors and therapeutic modalities of both
is a 51-year old woman, explored in 2006 for rectal bleeding associated with abdominal
pain. The digital rectal examination revealed a circumferential and
non-stenosing tumor. No further abnormalities were found in the clinical
examination. Blood chemistry was normal. A colonoscopy found a large ulcerated
rectal fold which was biopsied and the pathological study revealed a gastric
B-cell lymphoma of MALT type with no Helicobacter pylori. The staging including
an upper gastrointestinal endoscopy, a small bowel transit, a
thoraco-abdominal-pelvic scan, a bone marrow and a nasopharyngeal biopsy
revealed no abnormalities. The patient underwent a pelvic external beam
radiation at 40 Gy. The clinical and endoscopic post-therapeutic controls were
months after a complete remission, Mrs. H.T complained from a swallowing pain.
The oropharyngeal exam revealed a right-sided mass of the oral cavity. No
cervical nodes were found. A head and neck scan found a thickened right side
wall of the posterior nasopharyngeal mucosa with a choanal and an oropharyngeal
extension (Figure 1). The Nasopharyngeal biopsy revealed a B-small cell
lymphoma of the MALT-type with no evidence of Helicobacter pylori.
patient underwent an external beam radiation therapy of the Waldeyer Ring at 44
Gy. Currently, five years after the end of treatment, the patient is in
clinical, radiological and histological remission.
Figure 1: An axial cut of the Head & Neck CT/Scan: a thickened right
side wall of the posterior nasopharyngeal mucosa
lymphoma is a special pathological entity; it’s an extra nodal
non-Hodgkin lymphoma. It consists on a monoclonal proliferation of
centrocytes-like B lymphocytes from the mucosa of lymphoid tissue organs. These
cells involve the epithelium and lead to lymphoepithelial lesions (1, 2). It
occurs in adults with a median age at diagnosis of 61 years. It occurs in men
as in women with a female predominance for thyroid and salivary glands
gastrointestinal tract is the first location of MALT lymphoma interesting 50%
of cases. In fact, the stomach represents by far the most common site reported
in 70% of cases, followed by the small intestine representing 20 to 30% of
cases (4). The colo-rectal location is rare, 60 to 74% of colorectal lymphoma
occurs on the caecum which is rich in lymphoid tissue (5).
MALT lymphoma is exceptional and only a dozen cases have been reported in the
literature. They are often revealed by non-specific clinical signs, including
gastrointestinal bleeding and abdominal pain. On endoscopy, lesion of rectal
MALT lymphoma appears to be indurated, budding, rarely ulcerative or
infiltrative (6, 7, 8, 9).
neck is the second most common location of MALT lymphomas (2). It occurs, in
descending order, on orbit, on salivary glands and on Waldayer Ring. MALT
lymphomas represent less than 20% of Waldeyer Ring lymphomas: it occurs on
palatine tonsils in 50-60% of cases, and nasopharyngeal mucosa in 25-30% of
cases (10, 11).
As for the
rectal MALT lymphoma, nasopharyngeal involvement is not frequent. There is no
specific endoscopic or radiological aspect to this location: an asymmetrical
thickening of the nasopharyngeal wall is most often described in endoscopy and
computed tomography (12). Despite its low-grade, MALT lymphoma tends to be
multifocal and disseminated. In fact two large studies about MALT lymphomas,
including 158 and 108 patients, reported rates of spread respectively by 34%
and 32% (13, 14). This is particular to head and neck MALT lymphoma: 50 % of
patients treated for head and neck MALT lymphoma, had at least one further
location against 20-30% for gastric MALT lymphoma (15,16). In our case, the
patient had two rare locations of MALT lymphoma.
lymphomas develop from the mucosa-associated lymphoid tissue in most cases
acquired from chronic inflammation by antigenic stimulation or by an autoimmune
disorder. The pathogenic agent has been identified for some locations, but
remains poorly understood for others. Thus, the link between gastric MALT
lymphoma and chronic gastritis Helicobacter pylori (HP) was established on
pathological, epidemiological and therapeutic arguments (17, 18).
non-gastric locations, the role of infectious agents has been identified. In
fact, cutanous lymphoma has been associated with infection by Borrelia
burghdorferi (19), and the ocular lymphoma has been associated with infection
by psitacci chlamydya (20). The etiopathogenic link could, also, be established
with some autoimmune diseases such as Hashimoto thyroiditis, in case of MALT
lymphoma of the thyroid and Sjogren and sjogren lymophomes in the salivary
glands MALT lymphoma (2).
nasopahryngeal MALT lymphoma, the role of Epstein-Barr virus has been
mentioned, but was never been clearly demonstrated (10, 11). As for colorectal
locations, the role of Helicobacter pylori remains controversial. Some authors
have shown a positive association with a regression of colorectal MALT lymphoma
after HP eradication therapy, while others have described an inefficiency of
tritherapy and suggest the role of other infectious agents present in the
intestinal flora which are remaining to be identified (21, 22, 23).
Histological study of our patient did not show the presence of HP.
treatment of MALT lymphoma is standardized for the stomach location and it
consists in the eradication of HP (17, 18). Radiotherapy is given in case of
failure of tritherapy. Outside the gastric location, there are no therapeutic
standards of MALT lymphoma. For locations in the head and neck, local control
is the mainstay treatment and it consists on surgery and / or radiotherapy
which is used as a first line treatment. In these cases, retrospective studies
evaluating radiotherapy, at doses between 25 and 40 Gy, have objectified a 98 %
local control rate and a 5 years survival rate at 70% (24, 25). The matter of
dose has been resolved in the last years and a dose of 30 Gy was adopted
consensually as sufficient for the treatment of MALT lymphoma as for the other
low-grade lymphomas (24, 26). However, the definition of target volumes to
irradiate remains debated. Some authors include all the affected organ, others
include GTV with a margin of 20 mm and include the affected organ and the first
lymph node (24,26). In our case, the patient underwent an external beam
radiation of the whole Waldayer Ring.
Due to the
propagation and the recurrence of head and neck MALT lymphoma, systemic
therapy, either chemotherapy or targeted therapy, has been discussed. Two
retrospective studies, but with low enrollment, have shown no benefit with the
addition of anthracycline-based chemotherapy in terms of survival, but these
results remain to be validated by randomized trials (27). Similarly, the
combination of anti-CD20 monoclonal antibody (rituximab) to radiotherapy did
not improve patient outcomes; in this case also, the study had a low enrollment
and a short follow (28, 29).
their rarity, the colorectal MALT lymphomas treatment is not standardized, and
it’s very heterogeneous. Surgical resection or endoscopic mucosal
resection is considered to be the treatment of choice by some authors because
the disease remains, a long time, localized at the initial site (6, 7). In
other reported cases, patients were treated with chemotherapy, most often a
cyclophsphamide-based chemotherapy. The eradication of HP was also tested and
the reported results are controversial (21, 22, 23). The role of radiotherapy
is poorly defined. Some authors have indicated radiotherapy in combination with
chemotherapy, other in case of tritherapy failure. Only two cases of rectal
MALT lymphoma treated with radiotherapy alone were reported, and as for our
patient, complete remission was obtained. As in our case, dose levels were 40
and 45 Gy (8.30).
reported case takes its particular feature from the Association of two unusual
locations of MALT lymphomas and the choice of exclusive radiotherapy as a
treatment modality for both locations that allowed control of the disease 5
W.Sialaa, S.Yahiaouia, R.Lahmara, T.Boudawarab, M.Elloumic, N.Tahrid, J.Daouda.
Radiotherapy Department, Habib-Bourguiba hospital, Sfax, Tunisie.
Pathology Department, Habib-Bourguiba hospital, Sfax, Tunisie.
Hematology Department, Hédi-Chaker hospital, Sfax, Tunisie.
Hepatogastroenterology Department, Hédi-Chaker hospital, Sfax, Tunisie.
Non-Hodgkin’s Lymphoma Classification Project. A clinical evaluation of
the International Lymphoma Study Group classification of non-Hodgkin’s
lymphoma. Blood 1997; 89:3909-3918.
PG. Update on MALT lymphomas. Best Practice & Research Clinical Haematology
2005; 18: 57-68.
3 Zucca E,
Conconi A, Pedrinis E, Cortelazzo S, Motta T,Gospodarowicz MK. Nongastric
marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue. Blood 2003;
4 Zucca E,
Roggera E, Bertoni F, Cavalli F. Primary extranodal non-Hodgkin’s
lymphomas. Part 1: gastrointestinal, cutaneous and genitourinarylymphomas. Annals of Oncology 1997; 8:727-737.
5 Wong MT,
Eu KW. Primary colorectal lymphomas. Colorectal
Colorectal Disease 2006; 8:586-591.
6 Orita M,
Yamashita K, Okino M, Enoki T, Noshima S, Morita N. A case of MALT
(mucosa-associated lymphoid tissue lymphoma) occurring in the rectum.
Hepatogastroenterology 1999; 46: 2352-2354.
S, Ohta T, Kaji E, Kosaka T, Murakami I. EMR of mucosa associated lymphoid tissue
lymphoma of the rectum. Gastrointestinal
Endoscopy 2003; 57: 956-959.
K, Naoki T, Yuki H, Jun-ichi T, Hidekazu K, Iturou J. Successful radiotherapy
in a patient with primary rectal mucosa-associated lymphoid tissue lymphoma
without the API2-MALT1 fusion gene: a case report and review of the literature.
Leukemia Research 2008; 32:173-175.
S, Kanber Y, Charabaty-Pishvaian A, Ozdemirli M, Cohen P,Benjamin S. Primary
mucosa associated lymphoid tissue (MALT) lymphoma occurring in the rectum: a
case report and review of the literature. South Med J 2006; 99:1378-1384.
J, Lennert K. Low-grade B-cell lymphoma of mucosa-associated lymphoid tissue
type in Waldeyer’s ring. Histopathology1994; 24: 1-11.
11 Ree HJ,
Rege VB, Kinsley Re. Malignant lymphoma of Waldeyer’s ring following
gastrointestinal lymphoma. Cancer 1980;46:1528-1535.
kerevelier E, Attal P, Guermazi A, Bazelaire C. MALT Lymphomas: radiological
spectrum of a disease. Journal of radiology 2001. (On line)
Thieblemont C, Berger F, Dumontet C, Moullet I, Bouafia F, Felman P.
Mucosa-associated lymphoid tissue lymphoma is a disseminated disease in one
third of 158 patients analyzed. Blood 2000;95:802-806. Thieblemont
Bastion Y, Berger F, Rieux C, Salles G, Dumontet C. Mucosa-associated lymphoid
tissue gastrointestinal and nongastrointestinal lymphoma behavior: analysis of
108 patients. J Clin Oncol 1997; 15:1624-1630.
Cheolwon S, JooryungH, Jong-Lyel R. Extranodal marginal zone B-cell lymphomaof
mucosa-associated lymphoid tissue arisingin the extracranial head and neck
region: A high rate of dissemination and disease recurrence. Oral Oncology 2008
44, 949- 955.
M, Vorbeck F, Formanek M, Osterreicher C, Valencak J,Penz M. Importance of
extensive staging in patients withmucosa-associated lymphoid tissue (MALT)-type
lymphoma. Br J Cancer 2000;83:454-457.
Wotherspoon AC, Doglioni C, Diss TC, et al. Regression of primary low-grade
B-cell gastric lymphoma of mucosa-associated lymphoid tissue type after eradication
of Helicobacter pylori. Lancet 1993; 342: 575-577.
Nobre-Leitao C, Lage P, Cravo M, Cabecadas J, Chaves P, Alberto-Santos A.
Treatment of gastric MALT lymphoma by Helicobacter pylorieradication: a study
controlled by endoscopic ultrasonography. Am J Gastroenterol 1998;93:732-736.
E, Zucca E, Mainetti C, Bertoni F, Valsangiacomo C, Pedrinis E,et al.
Eradication of Borrelia burghdorferi infection in primary marginalzone B-cell
lymphoma of the skin. Hum Pathol 2000; 31: 263-268.
AJ, Guidoboni M, Ponzoni M, De Conciliis C, Dell’Oro S, FleischhauerK, et
al. Evidence of an association between Chlamydia psittaci and ocular adnexal
lymphoma. J Natl Cancer Inst 2004; 96:571-573.
Matsumoto T, Lida M, Shimizu M. Regression mucosa-associated lymphoid tissue
lymphoma of rectum after eradication of Helicobacter pylori. Lancet 1997; 350:
Grünberger B, Wöhrer S, Streubel B, Formanek M, Petkov V, Puespoek A.
Antibiotic treatment is not effective in patients infected with Helicobacterpylori
suffering from extragastric MALT lymphoma. J Clin Oncol 2006; 24: 1370-1375.
S, Katsuhiko H, Satoh T. Regression of rectal mucosa-associated lymphoid tissue
lymphomaunrelated to Helicobacter pylori. Ann Intern Med 2000; 132: 247.
RW, Gospodarowicz MK, Pintilie M. Localized mucosa-associatedlymphoid tissue
lymphoma treated with radiation therapy has excellent clinical outcome. J Clin
Oncol 2003; 21: 4157-4164.
KR, Brown PD, Stafford SL, Ansell SM, Martenson Jr JA. Efficacy and
treatment-related toxicity of radiotherapy for early-stage primary non-Hodgkin
lymphoma of the parotid gland. Int J Radiat Oncol Biol Phys 2004; 60:
K, Kagami Y, Higuchi K. A multicenter phase II study of local radiation therapy
for stage IEA mucosa-associated lymphoid tissue lymphomas: a preliminary report
from the Japan Radiation Oncology Group (JAROG). Int J Radiat Oncol Biol Phys
2007; 69: 1181-1186.
Papaxoinis G, Fountzilas G, Rontogianni D, Dimopoulos MA, Pavlidis N, Tsatalas
C. Low-grade mucosa-associated lymphoid tissue lymphoma: a retrospective
analysis of 97 patients by the Hellenic Cooperative Oncology Group (HeCOG). Ann Oncol. 2008; 19(4): 780-786.
A, Martinelli G, Thieblemont C. Clinical activity of rituximab in extranodal
marginal zone Bcell lymphoma of MALT type. Blood 2003; 102:2741-2745.
Martinelli G, Laszlo D, Ferreri AJ, Pruneri G, Ponzoni M, Conconi A, et al.
Clinical activity of rituximab in gastricmarginal zone non-Hodgkin’s lymphoma
resistant to or not eligible for anti-Helicobacter pylori therapy. J Clin Oncol
2005; 23: 1 979-1983.
30 Foo M,
Chao MW, Gibbs P, Guiney M, Jacobs R. Successful treatment of mucosa-associated
lymphoid tissue lymphoma of the rectum with radiation therapy: report of a
case. Dis Colon Rectum 2008;51:1719-1723.