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Cutaneous lesions as the first manifestation of systemic follicular lymphoma

Cutaneous lesions as the first manifestation of systemic follicular lymphoma. 高雄榮民總醫院 廖嘉賓 洪千惠 吳介山. Clinical history. 70 Y/O, male Multiple dark red firm plaques over trunk and thighs for two years Elevated in recent six months Past history: hypertension without regular treatment

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Cutaneous lesions as the first manifestation of systemic follicular lymphoma

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  1. Cutaneous lesions as the first manifestation of systemic follicular lymphoma 高雄榮民總醫院 廖嘉賓 洪千惠 吳介山

  2. Clinical history • 70 Y/O, male • Multiple dark red firm plaques over trunk and thighs for two years • Elevated in recent six months • Past history: hypertension without regular treatment • No body weight loss • LAP: negative

  3. Differential diagnoses • Scleroderma • Kaposi sarcoma • Cutaneous lymphoma • Metastatic tumor

  4. CD20 BCL-6 CD10 BCL-2

  5. Pathological diagnosis-Follicle center lymphoma

  6. Lab data Tumor markers (CEA, AFP, CA199, CA125, PSA): WNL

  7. CT • Lymphadenopathy (1cm) in bilateral axillae and mediastinum

  8. PET

  9. Positive: CD20 Bcl-2 Bcl-6 Negative: CD3 CD10 Immunostains

  10. Diagnosis Follicular lymphoma with secondary cutaneous involvement

  11. Discussion

  12. Follicular lymphoma - epidemiology • Frequency: 1/20,000 people per year • 20% of all lymphomas with the highest incidence in the USA and western Europe • Lower in Asia and developing countries • Adult, median age: 6th decade • M:F=1:1.7 CA Cancer J Clin 2008;58:305-317

  13. Clinical features • Widespread multiple lymphadenopathy • Bone marrow involvement: 60% • In 25-35% patient, transformation or progression to high grade lymphoma, DLBCL (at a rate of 3% per year) • May primary in skin, GI, particular duodenum, ocular adnexa, breast, testis WHO classification of tumours of haematopoietic and lymphoid tissues. Lyon, France, IARC, 2008.

  14. Histopathology • Small and large cleaved follicle center cells - centrocyte • Large noncleaved follicle center cells – centroblast • Follicular, diffuse, or mixed follicular and diffuse patterns

  15. Grade - Berard criteria • Centroblast/per high power field • Grade 1: 0~5 • Grade 2: 6~15 • Grade 3: >15 • 3a: centrocytes are still present • 3b: centroblast without centrocyte • Histological grade (3 vs 1&2) correlate with prognosis in FL WHO classification of tumours of haematopoietic and lymphoid tissues. Lyon, France, IARC, 2008.

  16. Immunohistochemistry & Genetics • Positive: Bcl2, Bcl6, CD10, CD20 • Negative: CD5, CD23, CD43, cyclin D1 • Grade3 lack of CD10 expression • t(14;18) -> overexpression of bcl2 WHO classification of tumours of haematopoietic and lymphoid tissues. Lyon, France, IARC, 2008.

  17. Prognosis • Prognosis related to the extent of the disease at diagnosis • Usually survive ten years or longer • Impact on prognosis of cutaneous involvement: unknown • Follicular Lymphoma International Prognostic Index (FLIPI) CA Cancer J Clin 2008;58:305-317

  18. Follicular Lymphoma International Prognostic Index (FLIPI) CA Cancer J Clin 2008;58:305-317

  19. Treatment • CVP + Rituxan (R-CVP) • CVP: cyclophosphamide, vincristine, prednisone • CHOP + Rituxan (R-CHOP) • CHOP: CVP + doxorubicin • Radioimmunotherapy such as Bexxar or Zevalin • Fludarabine + Rituxan (RF) CA Cancer J Clin 2008;58:305-317

  20. Blood. 2005;105:1417-1423 • Addition of rituximab (anti-CD20 chimeric monoclonal antibody) to first-line chemotherapy improves long-term outcomes

  21. Primary vs secondary cutaneous FL

  22. Conclusion • Follicular lymphoma is an indolent lymphoma with occasional transformation to high grade lymphoma in elderly • Centrocyte and centroblast • Tx with CHOP or CVP + Rituximab • Primary cutaneous follicle center lymphoma has better prognosis than systemic disease

  23. Thanks for your attention

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