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HANDOUT 2

HANDOUT 2. B-CELL INFILTRATES. CASE 6: ADDITIONAL FINDINGS. B-cells negative with antibodies to: CD5 CD10 CD23 BCL-6 cyclin D1. DIAGNOSIS. PRIMARY CUTANEOUS MARGINAL ZONE LYMPHOMA Synonyms: extranodal marginal zone B-cell lymphoma (WHO) cutaneous immunocytoma (EORTC)

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HANDOUT 2

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  1. HANDOUT 2 B-CELL INFILTRATES

  2. CASE 6: ADDITIONAL FINDINGS • B-cells negative with antibodies to: • CD5 • CD10 • CD23 • BCL-6 • cyclin D1

  3. DIAGNOSIS • PRIMARY CUTANEOUS MARGINAL ZONE LYMPHOMA • Synonyms: • extranodal marginal zone B-cell lymphoma (WHO) • cutaneous immunocytoma (EORTC) • cutaneous follicular hyperplasia with monotypic plasma cells (Schmid et al Am J Surg Pathol 1995; 19: 12)

  4. CLINICAL Solitary or multiple tumours Good response to XRT; CR common Frequently relapse Excellent prognosis; 5-year survival >95%

  5. PCMZL and Borrelia burgdorferi • A proportion of PCMZL associated with B. burgdorferi infection. • Possibly only in some geographic locations; • Highlands of Scotland +ve • Austria (Graz/Vienna) +ve • USA -ve • Tawain -ve

  6. PATHOLOGY • Diffuse or periadnexal/perivascular infiltrate • Reactive germinal centres common • Interfollicular/diffuse neoplastic infiltrate • marginal zone cells • small lymphocytes • plasmacytoid/plasma cells • Reactive cells • histiocytes • Eosinphils

  7. Immunophenotype • CD20, bcl-2 positive • CD5, CD10, CD23, bcl-6, cyclinD1 negative • CD43 +/- • Genetics • Trisomy 3 in some • t(11;18) not found (c.f. gastric & bronchial MZL)

  8. DIFFERENTIAL DIAGNOSIS • Other small B-cell lymphomas • Cutaneous B-cell pseudolymphoma

  9. FURTHER READING Rijlaarsdam et al. Histopathology 1993; 23: 117 Bailey et al. Am J Surg Pathol 1996; 20: 1011 Cerroni et al. Am J Surg Pathol 1997; 21: 1307 Goodlad et al. Am J Surg Pathol 2000; 24: 1279 Wood et al. J Cutan Pathol 2001; 28: 502 Ye et al. Blood 2003; 102: 1012 Chunmei et al. Am J Surg Pathol 2003; 27: 1061

  10. CASE 7: ADDITIONAL FINDINGS • Stage IE on staging: bone marrow, CT chest & abdomen • t(14;18) not found

  11. DIAGNOSIS • PRIMARY CUTANEOUS FOLLICLE CENTRE CELL LYMPHOMA • (EORTC: although most cases included in this category display pure diffuse large cell morphology) • Synonyms: • Grade 3 follicular lymphoma & diffuse large B-cell lymphoma • (WHO: classifying lesion in this way may result in over-treatment)

  12. CLINICAL Solitary plaques, tumours, nodules Head & neck (scalp) Respond to local XRT: CR usual Frequent relapse Excellent prognosis: 5-year survival ~100%

  13. PATHOLOGY • As for nodal follicular lymphoma except: • Higher proportion of grade 3 lesions +/- DLBCL • Lower incidence of bcl-2 expression (0-60%) • t(14;18) rarely found

  14. PRIMARY CUTANEOUS FOLLICULAR LYMPHOMA High relapse rate but excellent survival

  15. COMPARISON OF OUTCOME WITH STAGE I NODAL FL: Disease status at end of follow-up 15/15 PCFL in complete remission at end of follow-up period compared with only 49/87 stage I nodal FL (p<0.01: c2). Goodlad et al. Am J Surg Pathol 2002

  16. DIFFERENTIAL DIAGNOSIS • Other small B-cell lymphomas • Cutaneous B-cell pseudolymphoma

  17. FURTHER READING Garcia et al. Am J Surg Pathol 1986; 10: 454 Yang et al. Am J Surg Pathol 2000; 24: 694 Cerroni et al. Blood 2000: 95; 3922 Franco et al. Am J Surg Pathol 2001; 25: 875 Aguilera et al. Mod Pathol 2001; 14: 828 Goodlad et al. Am J Surg Pathol 2002; 26: 733

  18. CASE 8: ADDITIONAL FINDINGS Confined to skin on staging CD5, CD23, cyclin D1 negative

  19. DIAGNOSIS LARGE B-CELL LYMPHOMA OF THE LEG (EORTC) Diffuse large B-cell lymphoma (WHO)

  20. DIFFUSE LARGE B-CELL LYMPHOMA ARISING PRIMARILY IN THE SKIN • Probably two subtypes • Currently best classified as per EORTC on basis of anatomic location: • Primary cutaneous follicle centre cell lymphoma • This includes cases with true follicular morphology as treatment and outcome are the same • 2. Large B-cell lymphoma of the leg

  21. 1.0 Upper body (n=17) 0.8 0.6 Cumulative DSS Lower body (n=13) 0.4 0.2 [p=0.0047] 0.0 0 50 100 150 200 250 300 Months Primary cutaneous DLBCL on upper body has significantly better prognosis than primary cutaneous B-cell lymphoma on the leg Goodlad et al. Am J Surg Pathol; In press

  22. COMPARED TO PCFCCL/LBCL ON UPPER BODY, LARGE B-CELL LYMPHOMA OF THE LEG: • More often female • Older age • More often multiple lesions • Significantly poorer prognosis (5YS <60% c.f. >95% • Significantly higher incidence of bcl-2 expression (~100%) • Less frequent CD10/bcl-6 expression • More often large round cells (centroblasts/immunoblasts) than large cleaved cells • t(14;18) rare at either site

  23. N.B. standard treatment for nodal DLBCL is aggressive CTX (anthracycline based); this would be overtreatment for majority of primary cutaneous DLBCL irrespective of location

  24. DIFFERENTIAL DIAGNOSIS • CTCL, large cell types, non-epidermotropic • T/NK cell lymphomas

  25. REFERENCES Vermeer et al. Arch Dermatol 1996 Geelen et al. J Clin Oncol 1998; 16: 2080 Fernandez-Vazquez et al. Am J Surg Pathol 2001; 25: 307 Grange et al. J Clin Oncol 2001; 19: 3602 Fink-Puches et al. Blood 2002; 99: 800 Goodlad et al. Am J Surg Pathol; In press

  26. CASE 9: ADDITIONAL FINDINGS Polyclonal kappa/lambda Polyclonal IgH re-arrangement

  27. DIAGNOSIS CUTANEOUS B-CELL PSEUDOLYMPHOMA Synonyms: lymphocytoma (benigna) cutis Spiegler-Fendt sarcoid B-cutaneous lymphoid hyperplasia

  28. CUTANEOUS B-CELL PSEUDOLYMPHOMA • Cutaneous infiltrate histologically simulating CBCL • Cliincally may also mimic lymphoma • solitary red nodule/plaque (85-90%) • generalised/multifocal lesions (10-15%)

  29. AETIOLOGY • Idiopathic • Borrelia burgdorferi • Tattoo (red) • Injection sites • Acupuncture • Trauma • Vaccination • Gold piercing earrings • COMMON THEME IS REACTION TO ANTIGEN

  30. PATHOLOGY • Diffuse or nodular infiltrate (Grenz zone) • Reactive polytypic B-cells • Often in nodules +/- germinal centres • T-cell rich areas in between • Prominent vasculature • Macrophages, plasma cells, eosinophils • PRESERVED IMMUNOARCHITECTURE

  31. B-CLH: IMMUNOARCHITECTURE • T-cell areas • CD3 + • few B-cells • B-cell nodules • CD20, CD23

  32. DIFFERENTIAL DIAGNOSIS: CUTANEOUS INFILTRATES RICH IN SMALL B-CELLS • B-cell pseudolymphoma • Marginal zone lymhpoma • Follicular lymphoma • (Secondary involvement by: • B-CLL • Mantle cell lymphoma)

  33. NATURE OF LYMPHOID FOLLICLES? • REACTIVE FOLLICLES • Found in all three but rare in FL • Appearance as at other sites • Zonation • Tingible body macrophages • Mitotic figures • Well formed mantles • Uniform CD10/bcl-6 expression by GCCs • Bcl-2 negative

  34. 2. COLONISED FOLLICLES • Typical of MZL • Distinct compartments • Reactive GCC: CD10/bcl-6 +ve, bcl-2 -ve • Neoplastic MZ cells: CD10/bcl-6 -ve, bcl-2 +ve • 3. NEOPLASTIC FOLLICLES • Only seen in FL • Same as in nodal FL • No zonation • Monotonous appearance • Few TBMs, MFs (NB grade 3 FL) • Absent/poorly formed mantles • Uniform CD10/bcl-6 staining • Bcl-2 usually +ve (but significant % -ve cases)

  35. NATURE OF INTERFOLLICULAR INFILTRATE? • B-CELL PSEUDOLYMPHOMA • T-cells >> B-cells • NO confluent sheets of B-cells • Polytypic light chain immunohistochemistry • Epidermal changes • e.g. parakeratosis, atrophy, acanthosis, spongiosis • 2. MARGINAL ZONE LYMPHOMA • Clusters/sheets of marginal zone cells • >75% B-cells • Light chain restriction • Aberrant CD43 expression

  36. 3. FOLLICULAR LYMPHOMA • Clusters of CD10/bcl-6+ve B-cells • Useful when bcl-2 –ve • CD10 may be down-regulated

  37. POLYMERASE CHAIN REACTION • Can be helpful but use limited by: • Most FL are t(14;18) negative • False negatives relatively common • False positive results when very few B-cells • Some CBCPL are monoclonal • Some CBCPL progress to overt lymphoma

  38. THE MOST IMPORTANT DECISION: SHOULD THE PATIENT BE STAGED?

  39. FURTHER READING Ritter et al J Cutan Pathol 1994; 21: 481 Baldassano et al. Am J Surg Pathol 1999; 23: 88 de Leval et al. Am J Surg Pathol 2001; 25: 732 Nihal et al. Hum Pathol 2003; 34: 617

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