A 69-year-old man presented with abdominal and shoulder pain. Imaging showed a large pleural-based mass in his left lung. Biopsy of the mass revealed diffuse large B-cell lymphoma. He was treated with R-CHOP chemotherapy and achieved complete remission. Primary lymphoma of the pleura is extremely rare but can occur without associated conditions like HIV or prior tuberculosis. Biopsy is needed to diagnose pleural tumors due to lack of specific imaging features.

1. Primary Pleural Lymphoma Without
Associated Pyothorax
Case Report
A 69-year-old man with a prior history of bladder cancer diag-
nosed more than 20 years ago presented with a 2-month history of left
lower quadrant abdominal pain and left shoulder pain. Routine labo-
ratory studies, including lactate dehydrogenase, were normal. The
patient had a 25 pack-year smoking history and a remote history of
asbestos exposure in the 1960s when he worked as a pipe fitter. The
patient denied prior use of illicit drugs and had no history of prior
blood transfusion or tuberculosis exposure.
Work-up included a computed tomography (CT) scan of the
chest, abdomen, and pelvis, which was remarkable for a large, left,
pleural-based, enhancing mass in the costophrenic recess, extending
into the left T11-12 and T12-L1 neural foramen. A small left pleural
effusion was also present. Figure 1A (axial contrast-enhanced CT
image) shows the large pleural-based mass and the small left pleural
effusion. Positron emission tomography (PET)/CT was subsequently
performed. Figure 1B (axial PET image) demonstrates intense fluoro-
deoxyglucose (FDG) uptake of the mass, which had a maximum
standardized uptake value of 14. Figure 1C is the corresponding fused
axial image, and Figure 1D is the oblique maximum-intensity projec-
tion image.
Thepatientsubsequentlyunderwentbronchoscopy,cervicalme-
diastinoscopy, left pleuroscopy, and pleural biopsy. Histologic section
(Fig 2) of the left pleural biopsy shows portions of fibroadipose tissue
with extensive involvement by a diffuse lymphoproliferation com-
posed of large cells with round to elongated nuclei, vesicular chroma-
tin, multiple small nucleoli, and abundant cytoplasm. Frequent
mitotic figures and apoptotic bodies were present.
Immunoperoxidase studies revealed the neoplastic cells were
positive for LCA, CD20 (Fig 3), BSAP, and CD10 (Fig 4) and negative
for CD5, WT-1, and TTF-1. Scattered T cells were positive for CD3,
CD5, and BCL-2. Ki-67 (Fig 5) stain demonstrates a proliferation
index of 80%. These features were felt to be consistent with diffuse
large B-cell lymphoma of the pleura. Of note, lymph node biopsies
were negative for tumor.
The chemotherapy treatment regimen initiated was rituximab
plus cyclophosphamide, doxorubicin, vincristine, and prednisone ev-
ery3weeksforsixcycles.Eachcyclewassupportedwithpegfilgrastim.
A midtherapy PET/CT scan showed complete response with no resid-
ual FDG-avid lymphoma. There was mild residual pleural thickening
A B
C D
Fig 1.
JOURNAL OF CLINICAL ONCOLOGY D I A G N O S I S I N O N C O L O G Y
VOLUME 29 ⅐ NUMBER 14 ⅐ MAY 10 2011
© 2011 by American Society of Clinical Oncology e413Journal of Clinical Oncology, Vol 29, No 14 (May 10), 2011: pp e413-e415
140.163.254.156
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2. andparaspinalsofttissuewithuptakeequaltoorlessthanbloodpool.
Representative images are shown in Figures 6A to 6D. Figure 6A is the
axial CT image from the midtherapy PET/CT, Figure 6B is the corre-
spondingPETimageshowingnosignificantFDGuptake;Figure6Cis
the fused axial image; Figure 6D is a maximum-intensity projec-
tion image.
Discussion
Although pleural involvement by systemic lymphoma is rela-
tively common, primary pleural lymphoma is extremely rare.1
Ap-
proximately 16% of patients with non-Hodgkin’s lymphoma (NHL)
present with or subsequently develop pleural involvement during the
course of the disease.1
Two types of primary pleural lymphomas have
been described in the literature—the body cavity-based lymphoma in
patients with HIV and pyothorax-associated pleural lymphoma in
patients with a history of tuberculosis.2
It is thought that long-
standing pleural inflammation is an important factor in the develop-
ment of lymphoma in the latter scenario.2
It has been postulated that
there is chronic stimulation of B cells in the pleural cavity because the
most common malignant lymphoma arising in the pleura has been
reported to be diffuse large B-cell NHL.3
Other possible etiologic
factors reported include history of autoimmune disease, such as
Sjo¨gren’s syndrome, rheumatoid arthritis, chronic lymphocytic thy-
roiditis, and Epstein-Barr virus (EBV) infection.2,3
In body cavity–
based lymphoma, also referred to as primary effusion lymphoma,
human herpes virus-8/Kaposi’s sarcoma herpes virus infection is al-
ways present.4
HIV infection is present in most patients, and EBV
infectionispresentinapproximately50%ofpatients.4
Themajorityof
pyothorax-associated lymphomas have occurred in the Japanese pop-
ulation decades after development of tuberculosis-related pyothorax
or treatment of pulmonary tuberculosis by artificial pneumothorax.4
EBV infection of tumor cells is present in 100% of patients.4
Both
primaryeffusionlymphomaandpyothorax-associatedlymphomaare
clinically aggressive.4
Primary pleural NHL in an immunocompetent patient is rare.2
Malignantlymphomaarisinginthepleuracomprises2.4%ofprimary
Fig 2.
Fig 3.
Fig 4.
Fig 5.
Giardino et al
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140.163.254.156
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3. chest wall tumors.3
Differential diagnosis of pleural tumors includes
mesothelioma, localized fibrous tumor of the pleura, metastatic dis-
ease, and lymphoma. Metastatic disease represents the most common
neoplasm.5
Approximately 40% of pleural metastases arise from lung
carcinoma, 20% from breast carcinoma, 10% from lymphoma, and
the remaining 30% from other primary sites.5
Metastatic disease and
mesotheliomaweredifferentialconsiderationsinthispatientgiventhe
history of bladder cancer and asbestos exposure.
Radiologic features alone cannot reliably establish a diagnosis.
Definitive diagnosis almost invariably requires biopsy.5
In pleural
lymphoma, the lymphomatous deposits arise from lymphatic chan-
nels and lymphoid aggregates in the subpleural connective tissue be-
low the visceral pleura.5
Although any type of lymphoma can involve
the pleura, diffuse large B-cell lymphoma has been reported as most
frequent, followed by follicular lymphoma, with rates of approxi-
mately 60% and 20%, respectively.1
In summary, we report an extremely rare case of primary pleural
lymphomaoccurringinanimmunocompetentpatientwithcomplete
response to rituximab plus cyclophosphamide, doxorubicin, vincris-
tine, and prednisone chemotherapy at midcycle PET/CT. Although
rare, pleural tumors do not have specific imaging features, and diag-
nosis typically requires biopsy.
Angela Giardino, Kevin N. O’Regan, Jonathan Hargreaves,
Jyothipriya Jagannathan, David Park, Nikhil Ramaiya,
and David Fisher
Dana-Farber Cancer Institute, Boston, MA
AUTHORS’ DISCLOSURES OF POTENTIAL CONFLICTS OF INTEREST
The author(s) indicated no potential conflicts of interest.
REFERENCES
1. Vega F, Padula A, Valbuena JR, et al: Lymphomas involving the pleura. Arch
Pathol Lab Med 130:1497-1502, 2006
2. Ahmad H, Pawade J, Falk S, et al: Primary pleural lymphomas. Thorax
58:908-909, 2003
3. Hirai S, Hamanaka Y, Mitsui N, et al: Primary malignant lymphoma arising in
the pleura without preceding long-standing pyothorax. Ann Thorac Cardiovasc
Surg 10:297-300, 2004
4. Mitchell A, Meunier C, Ouellette D, et al: Extranodal marginal zone
lymphoma of mucosa-associated lymphoid tissue with initial presentation in the
pleura. Chest 129:791-794, 2006
5. Dynes MC, White EM, Fry WA, et al: Imaging manifestations of pleural
tumors. Radiographics 12:1191-1201, 1992
DOI: 10.1200/JCO.2010.33.5323; published online ahead of print at
www.jco.org on March 7, 2011
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A B
C D
Fig 6.
Diagnosis in Oncology
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Copyright © 2011 American Society of Clinical Oncology. All rights reserved.