| Session: Extramedullary manifestation of neoplastic myeloid disorder |
| Case number: 129 |
Submitter(s): Andrew L. Feldman, Curtis A. Hanson, Paul J. Kurtin.
Clinical history
A 25 year-old otherwise healthy male presented with gradual onset of epigastric pain. He was admitted to a local hospital and felt to have an acute abdomen. Laparotomy revealed only ascites. He became hemodynamically unstable intraoperatively and a large (800cc) pericardial effusion was noted and drained. On transfer to our institution, CBC was Hb 10.7, Hct 31.6, RBC 3.68, MCV 85.6, RDW 12.4, WBC 40.9, Plt 277. Bone marrow biopsy was performed. CT revealed a large mediastinal mass. The patient again became hemodynamically unstable. Emergency thoracotomy revealed a pericardial effusion with cardiac tamponade and anterior pericardiectomy was performed. The mediastinal mass was debulked. The patient received idarubicin and ARA-C, but had persistent disease and underwent bone marrow transplantation. He relapsed quickly and died 6 months after initial presentation.
Details of gross/microscopic pathology:
The mediastinal mass (submitted slides) showed diffuse infiltration by a population of medium sized cells, some showing evidence of monocytic differentiation.
The peripheral blood showed rare blasts and promonocytes; differential: 56% neutrophils, 15% lymphocytes, 14% monocytes, 2% eosinophils, 5% metamyelocytes, 6% myelocytes, 2% blasts/promonocytes, 1% nRBC.
The bone marrow showed increased blasts and promonocytes; differential: 22% neutrophils, 6% metamyelocytes, 12% myelocytes, 1% promyelocytes, 1% eosinophils, 26% blasts/promonocytes, 21% normoblasts, 3% monocytes, and 8% lymphocytes. The blasts and promonocytes were negative for peroxidase, chloroacetate esterase, and butyrate esterase.
Blasts and promonocytes also were noted in the pericardial fluid.
Immunophenotype (flow cytometry/immunohistochemistry):
Flow cytometry was performed on the mediastinal mass; 48% of the cells were blasts, positive for CD33, CD34, CD38, CD13, HLA-DR, and CD11b. They were negative for CD2, CD3, CD5, CD7, CD56, CD10, CD19, CD20, CD22, CD14, CD41, and CD61.
Immunohistochemistry on sections of the mediastinal mass showed the neoplastic cells to be positive for CD45, CD33, CD163, and lysozyme. The were negative for CD20, CD3, CD10, Pax5, CD4, CD7, CD68, CD117, CD123, CD34, and myeloperoxidase.
Cytogenetics:
Cytogenetics (bone marrow):
47-49,X,-Y,add(2)(p23),-4,+6,add(7)(p15),-9,-11,der(11)t(4;11)(q21;p15),+13,add(17)(p11),add(19)(p13.3),add(22)(q11.2),+2-6mark[cp13]/46,XY[7]
Molecular analysis:
N/A
Interesting feature(s) of submitted case:
The present case is an unusual example of acute myeloid leukemia with monocytic differentiation presenting as a mediastinal mass and pericardial effusion with cardiac tamponade in a 25 year-old male. Acute leukemias presenting with pericardial effusion and cardiac tamponade are rare. A mediastinal mass in a young adult male is more typical of lymphoblastic lymphoma than of an extramedullary myeloid tumor.
Proposed diagnosis:
Acute myeloid leukemia with monocytic differentiation, presenting as a mediastinal mass and acute cardiac tamponade.
Panel diagnosis:
agree with proposed diagnosis
Comments:
Stains performed by the panel: sections poorly preserved; MPO-, CD68PGM1-, NPM- (+in nuclei)
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