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

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Presentation on theme: "Case 6 F, 75 ys Erythematous nodule of the lower left leg."— Presentation transcript:

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

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6 CD20

7 Bcl-6

8 Bcl-2

9 CD10

10 CD3

11 Mib-1

12 DIAGNOSIS  Diffuse large B cell lymphoma  Work-up: peripheral blood, bone marrow, CT scan: peripheral blood, bone marrow, CT scan: all negative all negative

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

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15 CD5

16 Cyclin-D1

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

18 FISH break apart (t11;14) Cyclin-D1+ ↑ Mib-1 CD5 -

19  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

20 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 Note: diagnostic value of IIC and FISH


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