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    Pyothorax Associated Lymphoma

    Definition

    • Pleural based lymphoma associated with chronic pleural inflammation

    Diagnostic Criteria

    • Large cell lymphoma involving pleura
      • Arises in setting of chronic pyothorax
      • Tumor mass typically present
        • May involve lung, chest wall or pericardium
      • Abundant basophilic cytoplasm
        • Most cases immunoblastic
    • EBV positive at least 70% of cases
    • Most reported cases B lineage
      • Infrequent T lineage or biophenotypic cases reported
      • HHV8 negative in all but one reported case

    Yasodha Natkunam MD PhD
    Robert V Rouse MD
    Department of Pathology
    Stanford University School of Medicine
    Stanford CA 94305-5342

    Original posting:: May 1, 2006

    Supplemental studies

    Immunohistology

    • B lineage in almost all reported cases
      • CD138 about 50%
      • Infrequent T and biphenotypic cases reported.
    • Aberrant T markers CD2,3,4 reported in 5/12 cases tested
    • EBV LMP positive
    • HHV8 negative in all but one reported case

    Genetic analysis

    • EBV present by in situ hybridization in 70 to 100% of cases
    • HHV8 negative in all but one reported case

    Differential Diagnosis

    Both are EBV positive. One HHV8+ pyothorax associated lymphoma has been reported by O'Donovan et al.
    Pyothorax Associated Lymphoma Primary Effusion Lymphoma
    Associated with chronic inflammation No association with inflammation
    No association with immunosuppression Majority associated with immunosuppression
    HHV8 negative HHV8 positive
    Pleural mass No mass lesion

    Clinical

    • Involves pleural cavity
      • May involve lung, chest wall or pericardium
    • Most cases follow pyothorax induced for treatment of tuberculosis
      • Mean interval 37 years
      • Less commonly involves tuberculous pleuritis
    • Mean age 64, range 46-82
    • M:F 12:1
    • 2 year survival depends on stage
      • 70% if low stage
      • 16% if high stage

    Grading / Staging / Report

    Grading is not applicable

    Ann Arbor Staging System

    • Stage I
      • I if involvement of a single lymph node region
      • IE if involvement of a single extralymphatic organ or site
    • Stage II
      • II if two or more lymph node regions on same side of diaphragm
      • IIE if localized involvement of an extralymphatic organ or site and one or more lymph node regions on the same side of the diaphragm
    • Stage III
      • III if Involvement of lymph node regions on both sides of the diphragm
      • IIIS if spleen involved
      • IIIE if extralymphatic site involved
    • Stage IV
      • Diffuse or disseminated involvement of one or more extralymphatic organs or tissues, with or without associated lymph node involvement
    • Systemic Symptoms in 6 months preceding admission
      • Fever, night sweats, 10% weight loss
      • A = absent
      • B = present
    • Extranodal sites are also designated
      • M+ = marrow
      • L+ = lung
      • H+ = liver
      • P+ = pleura
      • O+ = bone
      • D+ = skin and subcutaneous tissue
    • Although originally designed for Hodgkin lymphoma, the Ann Arbor System is also used for non-Hodgkin lymphomas.

    The pathology report should contain the following information:

    • Diagnosis in the World Health Organization (WHO) classification
      • Equivalent diagnosis in other classifications used by relevant clinicians
    • Results of supplementary studies if performed
    • Relationship to other specimens from the same patient
    • Information relevant to staging if available

    Lists

    Types and variants of large B cell lymphoma

    Bibliography

    • Warnke RA, Weiss LM, Chan JKC, Cleary ML, Dorfman RF . Tumors of the Lymph Nodes and Spleen, Atlas of Tumor Pathology, AFIP Third Series, Fascicle 14, 1995
    • Jaffe ES, Harris NL Stein H, Vardiman JW . Pathology and Genetics of Tumours of Haematopoietic and Lymphoid Tissues, World Health Organization Classification of Tumours 2001
    • Aozasa K, Takakuwa T, Nakatsuka S. Pyothorax-associated lymphoma: a lymphoma developing in chronic inflammation. Adv Anat Pathol. 2005 Nov;12(6):324-31.
    • O'Donovan M, Silva I, Uhlmann V, Bermingham N, Expression profile of human herpesvirus 8 (HHV-8) in pyothorax associated lymphoma and in effusion lymphoma. Mol Pathol 2001 Apr;54(2):80-5
    • Taniere P, Manai A, Charpentier R, Terdjman P, Boucheron S, Cordier JF, 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 Mar;11(3):779-83
    • Iuchi K, Aozasa K, Yamamoto S, Mori T, Tajima K, Minato K, Mukai K, Komatsu H, Tagaki T, Kobashi Y, et al. Non-Hodgkin's lymphoma of the pleural cavity developing from long-standing pyothorax. Summary of clinical and pathological findings in thirty-seven cases. Jpn J Clin Oncol 1989 Sep;19(3):249-57 36/37
    • Ohsawa M, Tomita Y, Kanno H, Iuchi K, Kawabata Y, Nakajima Y, Komatsu H, Mukai K, Shimoyama M, Aozasa K. Role of Epstein-Barr virus in pleural lymphomagenesis. Mod Pathol 1995 Oct;8(8):848-53
    • Nakatsuka S, Yao M, Hoshida Y, Yamamoto S, Iuchi K, Aozasa K. Pyothorax-associated lymphoma: a review of 106 cases. J Clin Oncol. 2002 Oct 15;20(20):4255-60.
    • Petitjean B, Jardin F, Joly B, Martin-Garcia N, Tilly H, Picquenot JM, Briere J, Danel C, Mehaut S, Abd-Al-Samad I, Copie-Bergman C, Delfau-Larue MH, 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 Jun;26(6):724-32.
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