Granuloblastic sarcoma: onset with cutaneous polymorphic lesions.

A 54 year-old-woman developed papulo-erythematous lesions on her face, arms, and trunk .These lesions showed polymorphic features. (Figure 1) Biopsy revealed an extramedullary (cutaneous and subcutaneous) infiltration of poorly differentiated myeloid cells CD34+, CD68+, LYSOZIME+, MPO+, CAE-, CD3-.(Figure 2-3) The bone marrow aspiration was compatible with chronic myelomonocytic leukemia with normal cytogenetics (46 XX), and normal peripheral blood cell count. She was given standard induction chemotherapy for acute myeloid leukemia with Ara-c 100 mg/mq for 7 days (continuous infusion) and Idarubicine 10 mg/mq for 3 days. She obtained complete regression of cutaneous lesions, but bone marrow aspiration showed an increase of blastic cells (10-15%). After discharge she developed acute cholecystitis and bronchopneumonia. The following cycle of chemotherapy was postponed because she underwent cholecystectomy. Complete remission of cutaneous lesions lasted 62 days. Afterwards she developed acute myeloid leukemia with complex cytogenetics ( 48 XX, +8, +8, der(17), t(1;17)q21p13 [9]/ 49 idem, +del(4)q21 [8]/ 49 idem, +4 [14]/46 XX [1]) without cutaneous relapse. The patient died during salvage chemotherapy in aplasia because of multiorgan failure.