AML-194: Mast Cell Sarcoma in a Post-Remission AML Patient: Case Report and Review of Literature

Abstract

Context Report of a case of Mast cell sarcoma. Objective Report the occurrence of MSC in AML patients. Design Observation of an AML patient developing a palatal swelling. Setting Case report of AML patient with MSC after ten years of remission. Patients or other participants Sixty-year-old male patient, known case of AML since and in remission since then, on 11-18 complained of ulcerated mass arising from gums to hard palate with gradual onset and progressive course for 8 months following tooth extraction, followed by nasal tonation and edema with right-eye proptosis. He was diagnosed with AML May-08 AMLM1 with BMA hypercellular, blast 67%, 1st cycle 3/5 BMA normocellular, blast 11%. 2nd 3/7, BMA hyper 1% blast, two more 2/5 then 4 cycles HDA last Jun-09 he didn't have an HLA-matched donor. Interventions Patient's CBC & labs were normal; BMA normocellular 2% blasts; wedge biopsy from mass confirmed having MCS after revision in two different labs; an MRI was done showing a mass of 10 × 5 × 7 cm. Patient was admitted; started steroid 60 mg daily, radiotherapy suggested systemic therapy; started Glivec 400 mg daily with reduction in the mass size and improvement of the proptosis and discharged on steroid 8 mg with Glivec 400 mg daily. After one month, mass was reduced to 20 × 9 × 19 mm. One month later, he had severe abdominal pain, he stopped his medications and was referred to surgery, where he underwent exploration for strangulated supra-umbilical hernia with perforated duodenal ulcer. During recovery, pancytopenia occurred with BMA hypocellular, blast 30%; he died before receiving therapy for relapse. Main outcome measures As regards his AML, he had a DFS of 129 months (10.75 years), while since being diagnosed with MCS, his OS was 3.76 months and 8.13 months since presentation. Results MCS is the rarest mast cell disorder with very poor prognosis and occasional MCL; out of the 23 cases reported in literature, 7 (30.4%) showed leukemic transformation and an average OS of 14.38 months (1-45). Conclusions MCS is very rare disorder with no standard of care and very poor outcome. We have no grant to acknowledge.