Pathology of the Pleura and Mediastinum

2018 Edition
| Editors: Timothy Craig Allen, Saul Suster

Primary Pleural Lymphoma

  • Ryan RomanoEmail author
  • Timothy Craig Allen
Reference work entry


Body cavity-based lymphoma; PAL; PEL; PPL; Primary effusion lymphoma; Pyothorax-associated lymphoma


Secondary involvement of the pleura by systemic lymphoma is more common than primary lymphomas arising in the pleura. It is generally accepted that two distinct forms of primary pleural lymphoma (PPL) exist: primary effusion lymphoma (PEL) and pyothorax-associated lymphoma (PAL).

 Primary effusion lymphoma (PEL), also termed body cavity-based lymphoma, is a large B-cell non-Hodgkin lymphoma that often presents with serous effusions and no detectable mass lesions and carries strong associations with human herpes virus 8(HHV8)/Kaposi sarcoma herpes virus (KSHV) and human immunodeficiency virus (HIV). Immunodeficiency is a hallmark association of PEL, regardless of the underlying etiology of the immunodeficiency, though a majority of cases arise specifically in association with HIV infection (Patel and Xiao 2013). However, cases have been reported in HIV-negative...

This is a preview of subscription content, log in to check access.

References and Further Reading

  1. Hirai, S., Hamanaka, Y., Mitsui, N., Morifuji, K., & Sutoh, M. (2004). Primary malignant lymphoma arising in the pleura without preceding long-standing pyothorax. Annals of Thoracic and Cardiovascular Surgery, 10, 297–300.PubMedGoogle Scholar
  2. Nakatsuka, S., Yao, M., Hoshida, Y., Yamamato, S., Iuchi, K., & Aozasa, K. (2002). Pyothorax-associated lymphoma: A review of 106 cases. Journal of Clinical Oncology, 20, 4253–4260.CrossRefGoogle Scholar
  3. Patel, S., & Xiao, P. (2013). Primary effusion lymphoma. Archives of Pathology and Laboratory Medicine, 137, 1152–1154.CrossRefPubMedGoogle Scholar
  4. Ravikumar, G., Tirumalae, R., & Das, K. (2012). Primary pleural non-Hodgkin lymphoma in a child – an exceedingly rare disease. Journal of Pediatric Surgery, 47, E29–E31.CrossRefPubMedGoogle Scholar
  5. Vega, F., Padula, A., Valbuena, J. R., Stancu, M., Jones, D., & Medeiros, L. J. (2006). Lymphomas involving the pleura: A clinicopathologic study of 34 cases diagnosed by pleural biopsy. Archives of Pathology and Laboratory Medicine, 130, 1497–1502.PubMedGoogle Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Mayo Clinic-RochesterRochesterUSA
  2. 2.Department of PathologyThe University of Mississippi Medical School, The University of Mississippi Medical CenterJacksonUSA