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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 F, 75 ys Erythematous nodule of the lower left leg
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 • Same morphology • Subcutaneous tissue involvement • But...
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].
Cyclin-D1+ ↑ Mib-1 CD5 - FISH breakapart (t11;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
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