Pyothorax-associated lymphoma, PAL
Definition
Primary pleural non-Hodgkin lymphoma arising in the context of a longstanding pyothorax, chronic inflammation due to artificial pneumothorax for the treatment of tuberculosis or tuberculous pleuritis. In view of its rarity, it is not included as a subtype in the current WHO classification of lymphomas. It is a subtype of DLBCL.
Epidemiology
Most cases have been reported from Japan with a strong association with EBV. With the exception of two cases2,3, it is not associated with HHV-8. It develops in patients who are immunocompetent.
Clinical features
Most patients are elderly, with a male predominance. The pyothorax has usually been present for decades. In most cases, lymphoma in restricted to the pleura.
Histopathology
Most cases are diffuse large B-cell lymphomas, usually of immunoblastic morphology and often with plasmacytoid cells. Less often the features are centroblastic or anaplastic features.
Immunohistochemistry
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CD2
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4/121
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|
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CD3
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4/121
|
|
CD4
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1/121
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|
CD5
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0/121
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|
CD7
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0/121
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|
CD8
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0/121
|
|
CD10
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0/121
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|
CD20
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9/121, 2/22, 2/23
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|
CD24
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2/23
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|
CD30
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1/1(Anaplastic morphology and B-cell immunophenotype, positive for CD45+, CD79a+, MUM-1 and EBMA-1)7
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CD45
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2/22
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CD45RA
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2/22
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CD79a
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11/121, 2/22, 2/23
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CD138
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4/12(includes 3 cases which were weak or negative for CD20, two of which showed plasmacytoid differentiation.)1
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bcl-2
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11/111, 2/22, 2/23
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|
bcl-6
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0/111
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|
MUM1
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10/101
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LMP-1
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9/111, 2/22, 2/35
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EBNA-2
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10/111, 2/22, 2/35
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|
EBER
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10/10(by RNA analysis)1,
1/2(by RNA analysis)3
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|
HHV-8
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0/111,
1/2(by RNA analysis)2 ,
1/2(by RNA analysis)3 ,
0/14(by RNA analysis)4,
0/2(by RNA analysis)5
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|
NSE
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positive6
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|
| |
Five cases showed aberrant expression of at least one T-cell marker (CD2, CD3 or CD4)1.
Differential diagnosis
Prognosis
These lymphomas have an aggressive course with a median survival of five months1.
References
1 Petitjean, B., Jardin, F., Joly, B., Martin-Garcia, N., Tilly, H., Picquenot, J.M., Briere, J., Danel, C., Mehaut, S., Abd-Al-Samad, I., Copie-Bergman, C., Delfau-Larue, M.H. and Gaulard, P. Pyothorax-associated lymphoma: a peculiar clinicopathologic entity derived from B cells at late stage of differentiation and with occasional aberrant dual B- and T-cell phenotype. Am J Surg Pathol 2002;26:724-32.
2 Ascani, S., Piccioli, M., Poggi, S., Briskomatis, A., Bolis, G.B., Liberati, F., Frongillo, R., Caramatti, C., Fraternali-Orcioni, G., Gamberi, B., Zinzani, P.L., Lazzi, S., Leoncini, L., O'Leary, J., Piccaluga, P.P. and Pileri, S.A. Pyothorax-associated lymphoma: description of the first two cases detected in Italy. Ann Oncol 1997;8:1133-8.
3 O'Donovan, M., Silva, I., Uhlmann, V., Bermingham, N., Luttich, K., Martin, C., Sheils, O., Killalea, A., Kenny, C., Pileri, S. and O'Leary, J.J. Expression profile of human herpesvirus 8 (HHV-8) in pyothorax associated lymphoma and in effusion lymphoma. Mol Pathol 2001;54:80-5.
4 Cesarman, E., Nador, R.G., Aozasa, K., Delsol, G., Said, J.W. and Knowles, D.M. Kaposi's sarcoma-associated herpesvirus in non-AIDS related lymphomas occurring in body cavities. Am J Pathol 1996;149:53-7.
5 Taniere, P., Manai, A., Charpentier, R., Terdjman, P., Boucheron, S., Cordier, J.F. and Berger, F. Pyothorax-associated lymphoma: relationship with Epstein-Barr virus, human herpes virus-8 and body cavity-based high grade lymphomas. Eur Respir J 1998;11:779-83.
6 Pileri, S.A., Dirnhofer, S., Went, P., Ascani, S., Sabattini, E., Marafioti, T., Tzankov, A., Leoncini, L., Falini, B. and Zinzani, P.L. Diffuse large B-cell lymphoma: one or more entities? Present controversies and possible tools for its subclassification. Histopathology 2002;41:482-509.
7 Verghese ET, Amer KM,Addis BJ. Pyothorax-associated lymphoma: an unusual variant of an unusual tumour. Histopathology 2007; 51:131-3
This page last revised 12.8.2007.
©SMUHT/PW Bishop