| Session: Extramedullary manifestation of neoplastic myeloid disorder |
| Case number: 179 |
Submitter(s): Andrew L. Feldman, Rebecca F. McClure, Curtis A. Hanson.
Clinical history
A 70 year-old male presented with progressive left arm pain and weakness. He had a previous history of multiple sclerosis diagnosed in 1968. His left arm symptoms started in 2004 (C8-T1 distribution). In 2006 he developed left arm edema and was diagnosed with a DVT. He was referred to our institution for a second opinion. Medications included coumadin and Flomax. CBC was Hgb 14.8, RBC 4.93, MCV 87.4, RDW 12.9, WBC 4.9, Plt 250. MRI and PET showed a hypermetabolic mass in the left brachial plexus. Peripheral blood, bone marrow, and CSF samples were reviewed. Biopsy of the brachial plexus lesion was performed. The patient is being treated with radiation therapy and intrathecal Methotrexate with a partial response to date.
Details of gross/microscopic pathology:
Biopsy of the brachial plexus (submitted slides) showed infiltration by a blast population with extensive nerve involvement. CSF showed numerous blasts. Auer rods were absent. Butyrate esterase was negative. There was no evidence of involvement by acute leukemia or myelodysplasia in the peripheral blood and bone marrow.
Immunophenotype (flow cytometry/immunohistochemistry):
Immunohistochemistry of the brachial plexus lesion showed the tumor cells to be positive for CD33, CD34, CD43, CD117, lysozyme, and myeloperoxidase (focal). CD45RO was negative.
Flow cytometry of the CSF showed a population of blasts that expressed CD13 (variable), CD33, CD34, and CD117. They were negative for CD2, CD3, CD5, CD7, CD10, CD14, CD15, CD16, CD19, CD20, CD23, CD56, CD61, and HLA-DR.
Cytogenetics:
FISH studies performed on the brachial plexus lesion showed no anomalies of ETO, AML1, MLL, PML, RARA, ABL, or BCR.
Conventional cytogenetics on the bone marrow was 46,XY.
Molecular analysis:
N/A.
Interesting feature(s) of submitted case:
This is a rare presentation of an extramedullary myeloid tumor, presenting as isolated involvement of the brachial plexus and CSF in a 70 year-old male. To date, there has been no evidence of peripheral blood or bone marrow involvement.
Proposed diagnosis:
Extramedullary myeloid tumor involving brachial plexus and cerebrospinal fluid.
Panel diagnosis:
agree with proposed diagnosis
Comments:
Stains performed by the panel: NPM1 + in cytoplasm, CD34+
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