Lymphoma & related disorders
Mature B cell neoplasms
Large B cell lymphomas-special subtypes
Pyothorax associated
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PubMed Search: Pyothorax associated lymphoma[TI]
Table of Contents
Definition / general | Essential features | Terminology | ICD coding | Epidemiology | Sites | Etiology | Clinical features | Diagnosis | Laboratory | Prognostic factors | Case reports | Treatment | Microscopic (histologic) description | Positive stains | Negative stains | Molecular / cytogenetics description | Sample pathology report | Differential diagnosis | Additional references | Board review style question #1 | Board review style answer #1Cite this page: Owczarczyk AB, Perry AM. Pyothorax associated. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/lymphomadiffusepyo.html. Accessed April 25th, 2024.
Definition / general
- Large B cell lymphoma that arises in chronically inflamed tissue and is associated with EBV infection
Essential features
- Majority of cases associated with 20+ year history of pyothorax in patients treated with artificial pneumothorax for pulmonary tuberculosis or tuberculous pleuritis
- Primary site of involvement is the pleural / pleural space, with or without involvement of nearby structures
- Tumor cells are positive for CD20, CD79a and EBER but negative for CD10, BCL6 and HHV8
- Aggressive disease with poor prognosis
Terminology
- Pyothorax associated lymphoma
- Pyothorax associated diffuse large B cell lymphoma
ICD coding
- ICD-O: 9680/3 - malignant lymphoma, large B cell, diffuse, NOS
Epidemiology
- Majority of cases associated with 20+ year history of pyothorax in patients treated with artificial pneumothorax for pulmonary tuberculosis or tuberculous pleuritis
- Mean age: 70 years old
- Marked male predominance
- References: Ann Oncol 2007;18:122, J Clin Oncol 2002;20:4255
Sites
- Pleural
- Contiguous invasion of adjacent structures: lung, diaphragm, pericardium, mediastinum, liver
- Very rarely involves in the bone marrow
- References: Ann Oncol 2007;18:122, J Clin Oncol 2002;20:4255, Adv Anat Pathol 2005;12:324
Etiology
- Important factors in the pathogenesis of this lymphoma include history of an artificial pneumothorax, EBV infection and cytokines (IL10, IL6) and reactive oxygen species produced in longstanding pyothorax
Clinical features
- Common presenting symptoms:
- Chest / back pain (50 - 60%)
- Fever (~40%)
- Chest wall swelling (40%)
- Productive cough with or without hemoptysis and dyspnea (24%)
- References: Ann Oncol 2007;18:122, J Clin Oncol 2002;20:4255, Adv Anat Pathol 2005;12:324
Diagnosis
- Biopsy
Laboratory
- Leukocytosis without leukemic cells
- Elevated lactate dehydrogenase (LDH)
Prognostic factors
- Aggressive, with 5 year overall survival rate of 20 - 35% (J Clin Oncol 2002;20:4255)
- Patient achieving complete remission with chemotherapy or radiotherapy 5 year overall survival of 50%
- Unfavorable prognostic factors:
- Poor performance status
- High serum LDH, alanine transaminase or urea
- High clinical stage
Case reports
- 70 and 75 year old men with the first 2 cases of pyothorax associated lymphoma detected in Italy (Ann Oncol 1997;8:1133)
- 75 year old woman with chronic posttraumatic empyema (Acta Haematol 2015;134:155)
- 76 year old man from Germany with pyothorax (Thorac Surg Sci 2004;1:Doc03)
- 79 year old man with chest wall mass (Acta Radiol 1997;38:833)
Treatment
- Chemotherapy with or without radiotherapy
Microscopic (histologic) description
- Diffuse infiltrate of large lymphoma cells
- Immunoblastic, centroblastic or sometimes plasmacytoid morphology with round nuclei, 1 - 2 large nucleoli
- Prominent necrosis and angiocentric growth often present
- References: Ann Oncol 2007;18:122, J Clin Oncol 2002;20:4255, Adv Anat Pathol 2005;12:324
Positive stains
- CD45
- CD20
- CD79a
- EBV latency III pattern
- Latency III cells express:
- All 9 latency associated viral proteins:
- 6 EBNAs (EB nuclear antigen; 1, 2, 3A, 3B, 3C and LP)
- 3 LMPs (latent membrane protein; 1, 2A and 2B)
- EBER noncoding RNAs
- All 9 latency associated viral proteins:
- This latency pattern is also seen in acute EBV infection and a subset of HIV related and post-transplant related lymphoproliferative diseases
- EBV latency III pattern is generally detected clinically by the presence of positive cells for EBER ncRNA by ISH and EBV proteins LMP1 and EBNA2 by IHC (Oncogene 2007;26:1297, Human Herpesviruses: Biology, Therapy, and Immunoprophylaxis - EBV gene expression and regulation, 1st edition, 2007, Histopathology 2021;79:451)
- Latency III cells express:
- Subset can lose CD20 / CD79a and express CD138 / MUM1
- References: Ann Oncol 2007;18:122, J Clin Oncol 2002;20:4255, Adv Anat Pathol 2005;12:324
Negative stains
- CD10
- BCL6
- HHV8
- CD30
- T cell markers (occasionally lymphoma can express 1 or more T cell markers)
- References: Ann Oncol 2007;18:122, J Clin Oncol 2002;20:4255, Adv Anat Pathol 2005;12:324
Molecular / cytogenetics description
- Immunoglobulin genes clonally rearranged and hypermutated
- Complex karyotypes
- TP53 mutation (70% of cases) (Cancer Res 1998;58:1105)
- Distinct gene expression profile from nodal diffuse large B cell lymphomas (Cancer Sci 2004;95:828)
- Overexpression of IFI27: gene expressed by B cells by stimulation of interferon alpha
Sample pathology report
- Pleura, mass, resection:
- Diffuse large B cell lymphoma associated with chronic inflammation
Differential diagnosis
- EBV+ diffuse large B cell lymphoma, NOS:
- Patients present with extranodal masses without involvement of body cavities
- Primary effusion lymphoma:
- Occurs in setting of immunodeficiency (e.g., HIV infection)
- Cytology can include immunoblastic, plasmablastic or anaplastic features
- All cases associated with HHV8 infection
- Pan B cell markers negative
- Systemic lymphoma involving body cavity lining:
- Generally, the patient will have a tissue based mass and stage IV disease
Additional references
Board review style question #1
A 70 year old man presents with chest pain and a remote history significant for tuberculous pleuritis and artificial pneumothorax. Imaging reveals a nodular thickening of the right pleural. Biopsy was performed, showing sheets of large cells with central nucleoli, frequent mitotic activity and patchy necrosis. Molecular studies demonstrate immunoglobulin gene rearrangement and cytogenetics reveals a complex karyotype. Which of the following is true about this disease entity?
- Cells are positive for CD10 and BCL6
- Patients are usually female and immunocompromised
- This is a subset of primary effusion lymphoma
- Tumor cells are HHV8 positive
- Tumor cells demonstrate a type 3 EBV latency pattern
Board review style answer #1
E. Tumor cells demonstrate a type 3 EBV latency pattern
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Reference: Pyothorax associated diffuse large B cell lymphoma
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Reference: Pyothorax associated diffuse large B cell lymphoma
