mucosa-associated lymphoid tissue lymphoma of the trachea
TRANSCRIPT
2041
□ CASE REPORT □
Mucosa-associated Lymphoid Tissue Lymphomaof the Trachea in a Patient with Breast Cancer
Hitomi Yamashita 1, Yasuto Ueda 1, Katsuyuki Tomita 1, Tsuyoshi Kitaura 1,
Tomoaki Koshobu 1, Yoshimasa Suzuki 2 and Eiji Shimizu 3
Abstract
We herein report the case of a 93-year-old woman with breast cancer on the left side. Preoperative com-
puted tomography of the chest showed irregularities and narrowing of the mid-trachea. Bronchoscopy was
performed, and the results of a biopsy supported a diagnosis of mucosa-associated lymphoid tissue (MALT)
lymphoma. The patient responded to treatment with prednisone alone, with a reduction in the size of the le-
sion. MALT lymphoma of the trachea is extremely rare, and there are only a few case reports of double can-
cer, i.e., MALT lymphoma of the trachea and breast cancer.
Key words: breast cancer, MALT lymphoma, prednisone, trachea
(Intern Med 54: 2041-2044, 2015)(DOI: 10.2169/internalmedicine.54.3925)
Introduction
Mucosa-associated lymphoid tissue (MALT) lymphoma is
most commonly found in the stomach, lungs, orbital soft tis-
sue, salivary glands and thyroid; involvement of the trachea
is extremely rare (1, 2). There are no clear guidelines for the
treatment of MALT lymphoma, and, with respect to lesions
located in the trachea, a broad range of treatments have
been shown to be effective, including surgical resection, ra-
diotherapy, bronchoscopic therapy, chemotherapy, immuno-
therapy (rituximab) and immunochemotherapy.
This report describes a rare case of MALT lymphoma of
the trachea in a 93-year-old woman with breast cancer. The
administration of prednisone alleviated her symptoms and
reduced the size of the MALT lymphoma in the trachea.
Case Report
The patient was a 93-year-old woman who presented to
the thoracic surgery department of Yonago Medical Center
complaining of a self-destructed left breast tumor. In the fol-
lowing month, the patient complained of wheezing during
movement. She had a previous medical history of hyperten-
sion and Alzheimer’s disease, which had been treated with
enalapril (5 mg daily) and donepezil (5 mg daily), respec-
tively. Her medical history did not include chronic autoim-
mune diseases, such as Sjögren syndrome and Hashimoto’s
thyroiditis, or chronic infections, such as that with Helico-bacter pylori.
On a physical examination, the patient presented with a
left breast mass and chest wheezing during inspiration, with
no other remarkable findings. Renal and liver function tests,
hemogram findings and the lactic dehydrogenase level were
normal.
A chest X-ray showed tracheal deviation to the left side
(Fig. 1). Computed tomography (CT) of the chest demon-
strated irregularities of the tracheal wall and narrowing of
the mid-trachea (Fig. 2A).
Flexible bronchoscopy was performed during intravenous
anesthesia for mastectomy, the results of which revealed a
protuberant lesion involving all the walls of the trachea, ex-
tending from the trachea to the left main bronchus (Fig. 3).
A biopsy showed aggregation of small lymphocytes, and the
morphological and immunohistochemical features confirmed
the presence of low-grade non-Hodgkin’s B-cell lymphoma
1Department of Respiratory Medicine, Yonago Medical Center, Japan, 2Department of Thoracic Surgery, Yonago Medical Center, Japan and 3Di-
vision of Medical Oncology and Molecular Respirology, Department of Multidisciplinary Internal Medicine, Faculty of Medicine, Tottori Uni-
versity, Japan
Received for publication August 18, 2014; Accepted for publication January 6, 2015
Correspondence to Dr. Katsuyuki Tomita, [email protected]
Intern Med 54: 2041-2044, 2015 DOI: 10.2169/internalmedicine.54.3925
2042
Figure 1. Chest X-ray showing a mass protruding into the tracheal lumen.
Figure 2. Chest computed tomography (CT) demonstrating irregularities of the tracheal wall and narrowing of the mid-trachea before (A) and four weeks after treatment with prednisone (B).
with plasmacytic differentiation, suggestive of MALT lym-
phoma. Hematoxylin and Eosin (H&E) staining disclosed
characteristic infiltration of centrocyte-like cells surrounding
the reactive follicles and forming lymphoepithelial lesions
(Fig. 4A). An immunohistochemical panel revealed that the
lymphoma cells were positive for CD20 and bcl2 and nega-
tive for CD5 and CD10 (Fig. 4B-E). The pathology of the
breast cancer was pT4bN0M0, stage III, solid-tubular carci-
noma, with positive estrogen- and progesterone-receptor im-
munostaining, a proliferative index as high as 5.8% and a
negative human epidermal growth factor receptor type 2
(HER2) status.
Treatment with prednisone (30 mg/day) was effective in
improving the patient’s symptoms. Chest CT performed af-
ter the four weeks of therapy showed a reduction in the size
of the lesion in the trachea (Fig. 2B), and the dose of pred-
nisone was tapered four weeks after the first dose. Although
the patient had exhibited good disease control, she died a
natural death while undergoing treatment with 10 mg/day of
prednisone in a nursing home, five months after the opera-
tion.
Discussion
MALT lymphoma was first described by Issacson and
Wright in 1983 in a small series of patients with low-grade
B cell gastrointestinal lymphoma (3). MALT lymphomas are
characterized by the presence of neoplastic marginal cells
that exhibit a variable combination of colonization of reac-
Intern Med 54: 2041-2044, 2015 DOI: 10.2169/internalmedicine.54.3925
2043
Figure 3. Bronchoscopic view showing an intratracheal pol-ypoid lesion.
Figure 4. Histological examination demonstrating centro-cyte-like cells infiltrating the mucosa (A, Hematoxylin and Eo-sin staining, ×100). Many of the cells exhibited positive staining for CD20 (B, ×200) and bcl2 (C, ×200) and negative staining for CD5 (D, ×200) and CD10 (E, ×200).
tive germinal centers, plasma differentiation and destructive
epithelial infiltration forming lymphoepithelial lesions. Im-
munohistochemical examinations often show characteristic B
cell lymphoma with monoclonal expansion by monotypic
cytoplasmic immunoglobulin. Although MALT lymphomas
occur most frequently in the gastrointestinal tract, they can
also arise in a number of non-gastrointestinal sites, such as
the lungs, orbital soft tissue, salivary glands and thyroid (1).
Among nongastrointestinal MALT lymphomas, pulmonary
lymphomas are the most frequent, representing up to 19%
of MALT lymphomas (3). As primary tracheal MALT lym-
phomas are extremely rare, most reports in the literature are
case reports (4-6).
The current case involved double cancer, breast cancer
and MALT lymphoma of the trachea. A cancer prone pheno-
type may exist in MALT lymphoma patients. For example,
Zucca et al. observed a high incidence of other neoplasms
(20%) in patients with low-grade gastric MALT lym-
phoma (7). However, Au et al. reported that, in their study,
MALT lymphoma patients did not appear to have a signifi-
cantly increased rate of cancer compared with an age-
matched population followed for the same period of
time (8).
Previous reports have shown the effectiveness of a range
of treatments, including surgical resection, radiotherapy,
bronchoscopic therapy, chemotherapy, immunotherapy and
immunochemotherapy, i.e., rituximab, cyclophosphamide,
adriamycin, oncovin and prednisone (R-CHOP) (4-6). How-
ever, combination regimens have not been proven to be any
more effective than single chemotherapy regimens (9). In
the present case, despite the positive test findings for CD20,
rituximab was not selected for treatment due to the age of
the patient. In this case, treatment with prednisone alone im-
proved the patient’s symptoms and reduced the size of the
mass. Prednisone monotherapy may a less intense che-
motherapeutic regimen for older patients, and the effective-
ness of antibiotic therapy has also been reported in some
cases of pulmonary MALT lymphoma (10).
As our patient presented with symptomatic stenosis of the
airway, we selected treatment with prednisone, taking into
consideration her advanced age. Importantly, prednisone was
effective in reducing her symptoms and the size of the le-
sion.
This is the first case of MALT lymphoma of the trachea
in a patient with breast cancer treated with prednisone alone.
The details of this case suggest that prednisone monotherapy
may be an effective strategy for treating MALT lymphoma.
MALT lymphoma of the trachea is an extremely rare and
indolent disease and must be considered in the differential
diagnosis of airway lesions. Tracheal tumors may mimic the
features of asthma and chronic obstructive pulmonary dis-
ease (COPD) and should be kept in mind as a rare cause of
asthma and COPD-like symptoms. In the current case, pred-
nisone monotherapy was beneficial in managing the tracheal
lymphoma in our patient.
The authors state that they have no Conflict of Interest (COI).
Intern Med 54: 2041-2044, 2015 DOI: 10.2169/internalmedicine.54.3925
2044
References
1. Isaacson P, Wright DH. Malignant lymphoma of mucosa-
associated lymphoid tissue: a distinctive type of B-cell lymphoma.
Cancer 52: 1410-1416, 1983.
2. Zinzani PL, Poletti V, Zompatori MB, et al. Bronchus-associated
lymphoid tissue lymphoma: an update of a rare extranodal mal-
toma. Clin Lymphoma Myeloma 7: 566-572, 2007.
3. Malek SN, Hatfield AJ, Flinn IW. MALT Lymphomas. Curr Treat
Options Oncol 4: 269-279, 2003.
4. Magliari ME, Aquino RT, Gonçalves AL, et al. Mucosa-associated
lymphoid tissue lymphoma of the trachea: case report. Sao Paulo
Med J 130: 126-129, 2012.
5. Ding J, Chen Z, Shi M. Tracheal stenting for primary tracheal
mucosa-associated lymphoid tissue lymphoma. Eur J Med Res 18:
8, 2013.
6. Okubo K, Miyamoto N, Komaki C. Primary mucosa-associated
lymphoid tissue (MALT) lymphoma of the trachea: a case of sur-
gical resection and long term survival. Thorax 60: 82-83, 2005.
7. Zucca E, Pinotti G, Roggero E, et al. High incidence of other neo-
plasms in patients with low-grade gastric MALT lymphoma. Ann
Oncol 6: 726-728, 1995.
8. Au WY, Gascoyne RD, Le N, et al. Incidence of second neo-
plasms in patients with MALT lymphoma: no increase in risk
above the background population. Ann Oncol 10: 317-321, 1999.
9. Cadranwl J, Wislez M, Antoine M. Primary pulmonary lym-
phoma. Eur Respir J 20: 750-762, 2002.
10. Ishimatsu Y, Mukae H, Matsumoto K, et al. Two cases with pul-
monary mucosa-associated lymphoid tissue lymphoma success-
fully treated with clarithromycin. Chest 138: 730-733, 2010.
Ⓒ 2015 The Japanese Society of Internal Medicine
http://www.naika.or.jp/imonline/index.html