Case 6Case 6

F, 75 ysF, 75 ys

Erythematous nodule of the lower left legErythematous nodule of the lower left leg

CD20

Bcl-6

Bcl-2

CD10

CD3

Mib-1

DIAGNOSISDIAGNOSIS

Diffuse large B cell lymphomaDiffuse large B cell lymphoma

Work-upWork-up: :

peripheral blood, bone marrow, CT scan:peripheral blood, bone marrow, CT scan:

all negativeall negative

Follow-up (after 17 months)Follow-up (after 17 months)

Local recurrence of the lesion Local recurrence of the lesion New biopsyNew biopsy Same morphologySame morphology Subcutaneous tissue involvementSubcutaneous tissue involvement But...But...

CD5

Cyclin-D1

FINAL DIAGNOSISFINAL DIAGNOSIS

Primary cutaneous MCL, blastoid variant, Primary cutaneous MCL, blastoid variant, with aberrant bcl-6/CD10 expressionwith aberrant bcl-6/CD10 expression

Cesinaro AM, Bettelli S, Maccio L, Milani M., Bettelli S, Maccio L, Milani M.

Primary Cutaneous Mantle Cell Lymphoma of the Leg With Blastoid Primary Cutaneous Mantle Cell Lymphoma of the Leg With Blastoid Morphology and Aberrant Immunophenotype: A Diagnostic Challenge. Morphology and Aberrant Immunophenotype: A Diagnostic Challenge. Am J Dermatopathol.Am J Dermatopathol. 2013 Apr 22. [Epub ahead of print]. 2013 Apr 22. [Epub ahead of print].

FISHbreak apart

(t11;14)

Cyclin-D1+

↑ Mib-1

CD5 -

Work-up: peripheral blood, bone marrow Work-up: peripheral blood, bone marrow and CT scan negativeand CT scan negative

Therapy: local radiotherapyTherapy: local radiotherapy 14 months later: ulcer of the tongue: MCL14 months later: ulcer of the tongue: MCL Further work-up: peripheral blood, bone Further work-up: peripheral blood, bone

marrow and CT scan negativemarrow and CT scan negative Therapy: 3 cycles of chemotherapyTherapy: 3 cycles of chemotherapy Recurrence and pulmonary complicationsRecurrence and pulmonary complications Death 40 months after first skin biopsyDeath 40 months after first skin biopsy

PITFALLS in MCL diagnosisPITFALLS in MCL diagnosis Aberrant expression of B cell markers Aberrant expression of B cell markers

(CD10, bcl-6)(CD10, bcl-6) Sometimes negativity for CD5Sometimes negativity for CD5 Blastoid morphology: DD with diffuse large Blastoid morphology: DD with diffuse large

B cell lymphoma B cell lymphoma Involvement of the skin, usually secondaryInvolvement of the skin, usually secondary Very rare cases of Very rare cases of bona fidebona fide primary primary

cutaneous MCLcutaneous MCL Note:Note: diagnostic value of IIC and FISHdiagnostic value of IIC and FISH