Question: 20170047


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#1:   MP/H Rules
#2:   Multiple primaries
#3:   Heme & Lymphoid Neoplasms


Source 1:   Heme & Lymph Manual & DB
Notes:   January 2015
Source 2:   Subject matter expert


MP/H Rules, Multiple Primaries--Heme and Lymphoid Neoplasms:  Does Rule M3 at (single primary) or Rule M13 (multiple primaries) apply in a case with chronic myelomonocytic leukemia seen in the blood and biopsy and granulocytic sarcomas identified in skin biopsies?  See Discussion.


Physical Exam text: Pt presented with left foot pain.  He also was noted to develop a leukocytosis, predominantly with neutrophilia, significant neck pain, and multiple 1 cm skin nodules. FH: Grandmother: colon cancer.

9/29/15 Left upper arm mass, biopsy

Specimen-1: Microscopic Description = The H\T\E stained sections reveal soft tissue with a diffuse large mononuclear cell infiltrate; the cells show nuclear folding and irregularities and abundant cytoplasm; apoptosis and focally admixed eosinophils are noted.  Increased mitotic rate is present as well.

Specimen-1: Diagnosis = Left Upper arm mass, biopsy.  Myeloid sarcoma, monoblastic subtype (analogous to acute myeloid leukemia, M5).

Immunohistochemical stains:  Immunohistochemical stains are performed for further characterization.  The overall antigenic profile is as follows:  CD45(leukocyte common antigen [LCA, +]), lysozyme(+), CD68(+), KI-67(+, 80-85%), myeloperoxidase(MPO, -), CD20(-), CD79(-), PAX-5(-), CD2(-), CD3(-), CD5( -),CD30(-), CD7(-), CD34(-), CD117(-), S-100(-), CD1a(-), TdT(-), cytokeratin marker (AE1/AE3, -)


Peripheral Blood:  Left leukocytosis including hypogranulated neutrophils, thrombocytopenia, and over 5,000 Monocytes including small, atypical forms.

Bone Marrow Biopsy and Aspirate:  Chronic Myeloproliferative Neoplasm (SEE NOTE).

NOTE:  Given the monocytic abnormalities seen in the peripheral blood and the biopsy, the best subclassification for this myeloproliferative neoplasm is chronic myelomonocytic leukemia. These findings, however, should be correlated with recent skin biopsies that according to Dr. ____ were diagnosed as granulocytic sarcomas.


ISCN KARYOTYPE: nuc ish(ABL1,BCR)x2[200]

Summary:  Normal result with probes ABL1 and BCR for translocation 9;22 in 200 interphase cells.

Clinical Interpretation:  Fusion of the BCR and ABL1 genes, indicating translocation 9;22, was not detected in 200 interphase cells from this sample. Statistically significant (95% Cl) normal cutoff value for fusion of BCR/ABL1 probes is >1.5%.


Facility reported only one primary. Treatment was reported-

10/3/2015- Hydroxyurea. 10/19/2015-After confirmation of diagnosis, underwent induction with cytarabine and idarubicin.   2/23/2016-Conditioning regimen - Fludarabine Busulfan, Methotrexate

2/29/2016- matched related donor (sister) stem cell transplant


PET/CT scan showed Diffusely hypermetabolic bone marrow consistent with infiltrating tumor with maximal SUV 5.2, Multiple hypermetabolic skin and subcutaneous nodules consistent with active tumor with maximal SUV ranging from 2.6-9.1, Bilateral ground glass infiltrates and small pleural effusions again seen.   Clinically, the pulmonary infiltrates were thought most likely to represent edema.



Abstract a single primary using Hemaptopoietic and Lymphoid Neoplasms Multiple Primaries Rule M3.  According to our expert pathologist consultant, code as an acute monoblastic/monocytic leukemia, (M5 type) (9891/3) based on the information provided.


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