| Session: Extramedullary manifestation of neoplastic myeloid disorder |
| Case number: 210 |
Submitter(s): Frederick K. Racke, William L. Marsh.
Clinical history
The patient is a 55 yo female diagnosed in Nov. 2006 with chronic myelomonocytic leukemia. Her past medical history is otherwise unremarkable. In early January, 2007, the patient received a short trial of Decitabine (20mg/m2) for worsening counts. One month later, the patient reports the development of subcutaneous skin nodules, particularly evident on the chest wall. At the time of the biopsy of the skin nodule, the patient's CBC was as follows: WBC, 0.9; Hgb/Hct, 10.1/28.8%; platelets, 190 k. Her medications at this time include Evista, Valtrex, ciprofloxacin, and fluconazole. At this time one of the skin lesions on her right breast was biopsied.
Details of gross/microscopic pathology:
A 3.2 x 2.6 x 1.2 cm tissue fragment was obtained. The histologic sections show a diffuse to focally nodular infiltration of breast tissue and connective tissue by immature mononuclear cells. In some of the nodular areas, the cells are predominantly immature. In the more diffuse areas there is an admixture of immature cells along with cells with monocytic and granulocytic features. A touch preparation shows immature myelomonocytic cells along with scattered blasts.
Immunophenotype (flow cytometry/immunohistochemistry):
Immunohistochemical stains were performed on the sections. The infiltrate is positive for lysozyme, CD68, CD43, and myeloperoxidase but negative for lymphoid markers. Interestingly, immunostains for CD34 and c-kit highlight immature cells that are primarily centered around cytokeratin positive ducts with only scattered CD34 and c-kit positive cells within the more diffuse and differentiated areas of the infiltrate. An immunostain for Mib1 (Ki-67) is also increased within the nodules.
Cytogenetics:
At diagnosis, bone marrow cytogenetics reveal a deletion of chromosome 7q22.
Molecular analysis:
Interesting feature(s) of submitted case:
The case illustrates an interesting example of tumor-environment interaction. It would appear that there is something within the local environment surrounding the ducts that is influencing the phenotype of the leukemic cells, either inducing the proliferation of immature blasts or causing them to home to this microenvironment. I have personally not encountered such organization in a myeloid sarcoma and would be interested in the group's experience.
Proposed diagnosis:
Myeloid sarcoma involving the breast.
Panel diagnosis:
agree with proposed diagnosis
Comments:
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