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 cases^2,3, 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. Less often the features are centroblastic or anaplastic features.

Immunohistochemistry

CD2	4/121
CD3	4/121
CD4	1/121
CD5	0/121
CD7	0/121
CD8	0/121
CD10	0/121
CD20	9/121, 2/22, 2/23
CD24	2/23
CD45	2/22
CD45RA	2/22
CD79a	11/121, 2/22, 2/23
CD138	4/12 (includes 3 cases which were weak or negative for CD20, two of which showed plasmacytoid differentiation.)1
bcl-2	11/111, 2/22, 2/23
bcl-6	0/111
MUM1	10/101
LMP-1	9/111, 2/22, 2/35
EBNA-2	10/111, 2/22, 2/35
EBER	10/10 (by RNA analysis)1, 1/2 (by RNA analysis)3
HHV-8	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
NSE	positive6

Five cases showed aberrant expression of at least one T-cell marker (CD2, CD3 or CD4)¹.

Differential diagnosis

• primary effusion lymphoma: presents as an effusion without a tumour mass in a setting of immunodeficiency, with a constant association with HHV-8. The neoplastic cells are CD20-/CD30+/CD38+/CD79a-/CD138+.

Prognosis

These lymphomas have an aggressive course with a median survival of five months¹.

References

¹Petitjean, 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.

²Ascani, 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.

³O'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.

⁴Cesarman, 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.

⁵Taniere, 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.

⁶Pileri, 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.

This page last revised 27.12.2002.

©SMUHT/PW Bishop