Name

Myeloid sarcoma

ICD-O-1 Morphology

9930/3: Myeloid sarcoma
Effective 1978 - 1991

ICD-O-2 Morphology

9930/3: Myeloid sarcoma
Effective 1992 - 2000

ICD-O-3 Morphology

9930/3: Myeloid sarcoma
Effective 2001 and later

Reportable

for cases diagnosed 1978 and later

Primary Site(s)

See Module 7
Primary site cannot be bone marrow (C421). Most common sites of involvement: skin, lymph nodes, gastrointestinal tract, bone, soft tissue and testis.

Grade

Not Applicable

Module Rule

None

Alternate Names

Chloroma
Extramedullary myeloid tumor
Granulocytic sarcoma
GS

Definition

Myeloid sarcoma is a tumor mass consisting of myeloid blats, with or without maturation, occurring at an anatomical site other than the bone marrow.

Abstractor Notes

Myeloid sarcoma may occur de novo; it may precede or coincide with AML, or represent acute blastic transformation of myelodysplastic syndromes. Myeloid sarcoma may also be the initial manifestation of relapse in a patient with previously diagnosed AML

If Myeloid Sarcoma occurs simultaneously or after an acute myeloid leukemia, see M3 and Module 5:PH10.

Definitive Diagnostic Methods

Genetic testing
Histologic confirmation
Immunophenotyping

Genetics Data

CBFB amplication
Inv(16)
KMT2A-MLLT3 fusion rearrangement
Loss of 5q
Loss of 16q
Loss of 20q
Monosomy 7
Monosomy 16
NPM1
t(8;21)(q22;q22)
Trisomy 4
Trisomy 8
Trisomy 11

Immunophenotyping

CD33 expression
CD34 expression or negative
CD56 expression
CD56 negative
CD68 (KP1 but not PGM1) expression
CD123 positive
CD303 positive
KIT (CD117) expression
NPM1

Treatments

Chemotherapy
Radiation therapy

Transformations to

There are no known transformations

Transformations from

There are no known transformations

Corresponding ICD-9 Codes

205.3 Myeloid sarcoma

Corresponding ICD-10 Codes

C92.3 Myeloid sarcoma

Corresponding ICD-10-CM Codes (U.S. only)

C92.3 Myeloid sarcoma (effective October 01, 2015)

Signs and Symptoms

Easy bruising or bleeding
Fatigue
Fever
Petechiae
Shortness of breath
Weakness
Weight loss or loss of appetite

Diagnostic Exams

CT (CAT) scan
Cytogenetic analysis
Immunophenotyping
Lumbar puncture
Peripheral blood smear
Physical exam and history
Reverse transcription-polymerase chain reaction test (RT-PCR)

Progression and Transformation

None

Epidemiology and Mortality

Age: 56 years median (1 month to 89 years range)
Sex: male predominance
Survival: higher probability or prolonged survival for patients who have allogeneic or autologous BM transplantation

Sources

Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J (Eds):
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (Revised 4th edition)
IARC: Lyon 2017
Section: Acute myeloid leukemia and related precursor neoplasms
Pages: 167-168

International Classification of Diseases for Oncology, Third Edition, First Revision. Geneva: World Health Organization, 2013.
Section: ICD-O-3.1 (2011) Morphological Codes
Pages: http://codes.iarc.fr/codegroup/2

National Cancer Institute
Section: General Information About Acute Myeloid Leukemia
Pages: https://www.cancer.gov/types/leukemia/hp/adult-aml-treatment-pdq
Glossary