| Session: Extramedullary manifestation of neoplastic myeloid disorder |
| Case number: 035 |
Submitter(s): Mohamed Y. Elkhalifa.
Clinical history
The patient is a 48 years old man who presented with a three months history of multiple, rapidly enlarging, subcutaneous nodules. A biopsy of a subcutaneous nodule, performed at an outside institution, was interpreted as T Cell Lymphoma. At that time the patient had normal CBC indices and bone marrow examination showed no involvement by lymphoma. The patient received CHOP chemotherapy without significant response. His medical condition continued to gradually deteriorate, and the patient expired shortly after the 6th cycle of chemotherapy. The subcutaneous nodule biopsy material was referred to King Faisal Specialist Hospital and Research Center for a second opinion.
Details of gross/microscopic pathology:
Histopathology of the skin nodule:
Sections show a well-circumscribed subcutaneous nodule, composed predominantly of large sized mononuclear cells with indented to folded nuclei, fine nuclear chromatin and small nucleoli. The majority of cells have a moderate to large amount of fine to coarsely granular eosinophilic cytoplasm. Mitotic figures and apoptotic bodies are seen.
There is complete sparing of the epidermis and dermis.
Immunophenotype (flow cytometry/immunohistochemistry):
The neoplastic cells were strongly positive for CD4 and weakly positive for CD45 and CD43. Focal localized cytoplasmic positivity was noted with anti CD56, CD7 and CD15.
The neoplastic cells were negative for CD1a, CD2, CD3, CD5, CD8, CD20, CD30, CD45RO, CD68, CD117, CD138, Fascin, Alk-1, TdT, EMA and HHV-8.
Approximately 50% of the neoplastic cells showed overexpression of P53.
Cytogenetics:
Not Done.
Molecular analysis:
Not done.
Interesting feature(s) of submitted case:
Plasmacytoid dendritic cell malignancies are rare, recently recognized, hematologic neoplasms characterized by distinct clinical, morphologic and immunologic features. The are thought to arise from the plasmacytoid dendritic cell type 2, a novel hematopoietic cell with unique morphologic and immunophenotypic profile. Clinically, plasmacytoid dendritic cell malignancies have a rappidly progressive course with extremely poor outcome.
Proposed diagnosis:
Plasmacytoid Dendritic Cell Tumor/Leukemia (also refered to as CD4+CD56+ hematodermic neoplasm).
Panel diagnosis:
agree with proposed diagnosis
Comments:
Panel comment: CD15 positivity is unusual. Stains performed by the panel: CD56 strongly +, CD123+, NPM- (+in nuclei)
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