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Case 6

Case 6. F, 75 ys Erythematous nodule of the lower left leg. CD20. Bcl-6. Bcl-2. CD10. CD3. Mib-1. DIAGNOSIS. Diffuse large B cell lymphoma Work-up : peripheral blood, bone marrow, CT scan: all negative. Follow-up (after 17 months). Local recurrence of the lesion New biopsy

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Case 6

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  1. Case 6 F, 75 ys Erythematous nodule of the lower left leg

  2. CD20

  3. Bcl-6

  4. Bcl-2

  5. CD10

  6. CD3

  7. Mib-1

  8. DIAGNOSIS • Diffuse large B cell lymphoma • Work-up: peripheral blood, bone marrow, CT scan: all negative

  9. Follow-up (after 17 months) • Local recurrence of the lesion • New biopsy • Same morphology • Subcutaneous tissue involvement • But...

  10. CD5

  11. Cyclin-D1

  12. FINAL DIAGNOSIS • Primary cutaneous MCL, blastoid variant, with aberrant bcl-6/CD10 expression Cesinaro AM, Bettelli S, Maccio L, Milani M. Primary Cutaneous Mantle Cell Lymphoma of the Leg With Blastoid Morphology and Aberrant Immunophenotype: A Diagnostic Challenge. Am J Dermatopathol. 2013 Apr 22. [Epub ahead of print].

  13. Cyclin-D1+ ↑ Mib-1 CD5 - FISH breakapart (t11;14)

  14. Work-up: peripheral blood, bone marrow and CT scan negative • Therapy: local radiotherapy • 14 months later: ulcer of the tongue: MCL • Further work-up: peripheral blood, bone marrow and CT scan negative • Therapy: 3 cycles of chemotherapy • Recurrence and pulmonary complications • Death 40 months after first skin biopsy

  15. PITFALLS in MCL diagnosis • Aberrant expression of B cell markers (CD10, bcl-6) • Sometimes negativity for CD5 • Blastoid morphology: DD with diffuse large B cell lymphoma • Involvement of the skin, usually secondary • Very rare cases of bona fide primary cutaneous MCL Note:diagnostic value of IIC and FISH

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