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Pathologic
and clinical features of primary pulmonary extranodal marginal zone
B-cell lymphoma of MALT type. Am J Surg Pathol 2001
Aug;25(8):997-1008.
We
reviewed pathologic, phenotypic, and clinical features of extranodal
marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT)
type primarily involving lung to address unresolved questions
regarding behavior and pathologic features of unambiguously diagnosed
pulmonary MALT lymphoma. Lung specimens from 50 patients were
reviewed. Forty-one had low-grade MALT lymphoma. Nine had low-grade
MALT lymphoma and diffuse large B-cell lymphoma. The patients included
32 women and 18 men with a median age of 68 years (range 34-88 years).
Half of the patients were asymptomatic at the time lymphoma was
diagnosed. Radiographic abnormalities were more commonly unilateral
(37 patients) than bilateral (12 patients). Localized masses or
nodules occurred in 39 patients. Associated autoimmune disorders (29%)
and monoclonal gammopathies (43%) were common. Low-grade lymphomas
formed intraparenchymal masses composed of centrocyte-like cells,
plasmacytoid lymphocytes, and plasma cells that formed
lymphoepithelial lesions and exhibited a lymphangitic growth pattern.
Mediastinal lymph nodes were involved histologically in 44% of cases.
Lymphoma-specific survival was 71.7% at 10 years, and overall survival
was significantly worse than age-and gender-matched control patients.
None of the following features predicted those patients who had an
adverse outcome: systemic symptoms, presence of autoimmune disorders
or paraproteinemia, anatomic distribution and number of pulmonary
lesions, lymph node involvement, or presence of anthracycline-treated
large B-cell lymphoma. |
Although
the lung is frequently involved by disseminated lymphoma, isolated
pulmonary lymphoma is rare, accounting for less than 1% of all extranodal
localized disease.
Three broad categories of lymphoma of the lung require recognition: in
rare instances, large B cell type lymphoma can present primarily in the
lung ; a second variant is by T cell lymphoma presenting as an
angiocentric process.
However, the most common histologic subtype is represented by low-grade
mucosa-associated lymphoid tissue (MALT) lymphoma, often in the past
considered as a pseudotumor because of its long indolent natural history.
Visit:
Pulmonary Lymphoproliferative Disease
;
Lymphocytic
Interstitial Pneumonia / Follicular
Bronchiolitis
In
1963, Salzstein distinguished pulmonary lymphoma from “pseudolymphoma”.
Pseudolymphoma was regarded as a reactive condition similar to pseudolymphomas
seen at other sites.
Most cases are now thought to be neoplastic from onset and arise from pulmonary MALT.
Age and sex:
Patients present in their fifth to seventh decades. It is slightly
more common in male.
Clinical
presentation:
In
low-grade disease, the commonest presentation is an asymptomic mass on
routine chest x-ray.
High-grade lymphomas are however, nearly always
symptomatic.
Constitutional symptoms include
weight loss, fever and night sweats.
Previous or synchronous MALT lymphoma at other extranodal
sites may be present.
Etiology:
Unlike gastric lymphomas
where there is a clinical association with previous Helicobacter
pylori infection, there is no consistent clinical association with
pulmonary counterparts. Some cases of pulmonary lymphomas have been described
in patients with autoimmune disorders, some with fibrosing alveolitis.
Diagnosis:
Surgical lung biopsies and resection have been the
commonest diagnostic procedures.
Identification of gene rearrangements using the polymerase chain reaction
has successfully proven monoclonality in bronchoalveolar lavage fluid
and
both bronchoscopic and transbronchial biopsies, in situations where
previous diagnoses had been classified as equivocal.
Microscopic
features:
Image1
[The
bronchiolar epithelium is infiltrated but is not destroyed]
;
Image2
[The
infiltrate expands and destroys the interstitium, consists of neoplastic
cells (black arrow) which are CD20 positive and reactive CD3
positive T-cell population in the backround (brown arrow).]
Low-grade tumours are composed of centrocyte-like, lymphocyte-like or monocytoid cells.
These are all
thought to be variations of the same neoplastic cell.
Features of plasmacytoid differentiation are often seen in the smalll
lymphocytes, and Russell or Dutcher bodies may be present. A
distinguishing feature of the lymphoid cell population is the presence of
a rim of clear cytoplasm surrounding the nucleus of the cells, resembling
that of centrocytes or monocytoid B-cells.
In resection
specimens, and to a lesser extent in open lung biopsies, a characteristic
pattern of spread along bronchovascular bundles and interlobular septa is
seen.
Subsequent expansion and destruction of the alveolar walls and
filling of the alveolar spaces give rise to nodules that coalesce
centrally, often with hyaline sclerosis towards their centers.
Reactive follicles with
well-formed germinal centers may be seen in 70% of cases
Other features of MALT lymphomas, such as lymphoepothelial lesions, are
often seen.
Diagram
This pattern of spread is not seen in high-grade tumours,
in which the infiltrate is more diffuse and destructive.
Giant lamellar
bodies may be rarely noted.
Necrosis is almost always
limited to the high-grade tumors.
The neoplastic cells are of B-cell
phenotype with a variable reactive T-cell population in the background.
Light chain restriction is observed in 30-70% of cases.
Amplification of
the immunoglobulin heavy chain gene with the polymerase chain reaction has
shows monoclonality in 60% of low-grade lymphomas, but only occasionally
in high-grade disease.
| Primary
Lung Small B-Cell Lymphoma versus Lymphoid Hyperplasia: Evaluation of
Diagnostic Criteria in 26 Cases.Am J Surg Pathol.2002
Jan;26(1):76-81
Primary lung non-Hodgkin's
lymphoma is a rare neoplasm mostly represented by low-grade B-cell
lymphomas of mucosa-associated lymphoid tissue. Their diagnostic
criteria are now well defined on surgical specimens, but pathologists
may experience difficulties in distinguishing them on exiguous
biopsies from benign lymphoid hyperplasia and other lymphomas.
Therefore, we examined a series of 26 lung lymphoid lesions to further
define the pathologic features of either lymphoma or lymphoid
hyperplasia on small specimens. We observed 16 primary lung
non-Hodgkin's lymphomas with a large predominance of low-grade
mucosa-associated lymphoid tissue-type lymphomas (87.5%, n = 14).
There were no autoimmune disorders, but three patients had a
concomitant infectious disease (hepatitis C virus and Helicobacter
pylori gastritis). One patient presented with a synchronous pulmonary
adenocarcinoma. As well as the classical mucosa-associated lymphoid
tissue cellular infiltrate, immunohistochemical characterization of
the 14 mucosa-associated lymphoid tissue-type lymphomas revealed the
CD20+/CD43+ centrocyte-like cell phenotype in 10 cases (71.5%).
Although the lymphoepithelial lesions observed in all lymphomatous
cases have been reported in lung lymphoid hyperplasia, the
determination of B-cell CD20+/CD43+ phenotype of the intraepithelial
lymphocytes highly increased the specificity of lymphoepithelial
lesions. A monoclonal immunoglobulin heavy chain gene rearrangement
was present in 71.4% of the mucosa-associated lymphoid tissue-type
lymphoma specimens. Investigation of H. pylori by polymerase chain
reaction detection was negative, even for the two cases associated
with H. pylori gastritis. |
|
Primary
pulmonary non-Hodgkin's lymphoma.
Jpn J Clin Oncol. 2004 Sep;34(9):510-4
BACKGROUND: Primary pulmonary non-Hodgkin's lymphoma is a very rare
neoplasm. It is represented most commonly by marginal zone B-cell
lymphoma of mucosa-associated lymphoid tissue (MALT) type. Although
there have been a few reviews of this lymphoma, clinical features,
diagnostic procedure, optimal management and prognostic factors have
not been well defined. METHODS: We reviewed the medical records of 24
patients who were pathologically and clinically diagnosed as primary
pulmonary lymphoma between September 1995 and June 2003. RESULTS:
There were 13 patients with MALT lymphoma and two with MALT lymphoma
accompanied by large B-cell lymphoma, seven with diffuse large B-cell
lymphoma and two with anaplastic large cell lymphoma. Half the
patients were asymptomatic at presentation; 46% had respiratory
symptoms and 16.7% had B-symptoms. Initial radiological findings were
variable including nodules, masses, infiltrates or consolidation. The
majority of patients (66.7%) needed surgical approaches (open
thoracotomy or video-assisted thoracoscopy) for definite diagnosis.
Bronchoscopy was performed in 83%, but only 30% showed a diagnostic
yield. The 13 patients with MALT lymphoma were treated with a variety
of modalities such as observation, surgery and single or combination
chemotherapy, and combination chemotherapy was administered to 11
patients with non-MALT lymphoma regardless of surgery. The overall
survival rate at 3 years for all 24 patients was 86% with a median
follow-up of 32 months. CONCLUSION: Although this entity of lymphoma
appears to have a good prognosis, further clinical experience and
long-term follow-up are needed to identify prognostic factors. |
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