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ICD-O-1 Morphology
9730/3: Plasma cell myeloma
9830/3: Plasma cell leukemia
Effective
1978 - 1991
ICD-O-2 Morphology
9732/3: Plasma cell myeloma
Effective
1992 - 2000
ICD-O-3 Morphology
9732/3: Plasma cell myeloma
Effective
2001 and later
Reportable
for cases diagnosed
1978 and later
Primary Site(s)
C421
Primary site must be bone marrow (C421)
Coding Manual:
Hematopoietic Coding Manual (PDF)
Abstractor Notes
Plasma cell myeloma (PCM) usually has generalized bone marrow involvement. Lytic bone lesions and bone tumor masses of plasma cells also occur.
Approximately 30% of patients with solitary plasmacytoma (bone or outside of bone) defined only by radiographical skeletal survey have additional lesions identified on MRI or CT. These patients are considered to have plasma cell myeloma.
The International Staging System for Multiple Myeloma Staging for Multiple Myeloma is based on:
1. Amount of monoclonal (or myeloma) protein (M protein) in the serum and/or urine
2. Various clinical parameters such as: hemoglobin and serum calcium concentrations, number of lytic bone lesions
3. Presence or absence of renal failure.
Stage I
Stage II
Stage III
Treatment
Watchful waiting: Asymptomatic patients with no lytic lesions and normal renal function.
For patients with symptoms and advanced disease
1. Induction therapy
2. Consolidation therapy
3. Maintenance therapy
4. Supportive care
The presence of plasmacytomas after a diagnosis of plasma cell myeloma indicates an advanced stage of plasma cell myeloma. Do not abstract a new primary for the plasmacytoma(s) (9731/3 or 9734/3) after a diagnosis of plasma cell myeloma.
Approximately 30% of patients with solitary plasmacytoma (bone or outside of bone) defined only by radiographical skeletal survey have additional lesions identified on MRI or CT. These patients are considered to have plasma cell myeloma.
The International Staging System for Multiple Myeloma Staging for Multiple Myeloma is based on:
1. Amount of monoclonal (or myeloma) protein (M protein) in the serum and/or urine
2. Various clinical parameters such as: hemoglobin and serum calcium concentrations, number of lytic bone lesions
3. Presence or absence of renal failure.
Stage I
Stage II
Stage III
Treatment
Watchful waiting: Asymptomatic patients with no lytic lesions and normal renal function.
For patients with symptoms and advanced disease
1. Induction therapy
2. Consolidation therapy
3. Maintenance therapy
4. Supportive care
The presence of plasmacytomas after a diagnosis of plasma cell myeloma indicates an advanced stage of plasma cell myeloma. Do not abstract a new primary for the plasmacytoma(s) (9731/3 or 9734/3) after a diagnosis of plasma cell myeloma.
Diagnostic Confirmation
This histology can be determined by positive histology (including peripheral blood) with or without genetics and/or immunophenotyping. Review the Definitive Diagnostic Methods, Immunophenotyping and Genetics Data sections below, and the instructions in the Hematopoietic Manual for further guidance on assigning Diagnostic confirmation.
Grade
Not Applicable
Module Rule
None
Alternate Names
Alpha PCM
Alpha plasma cell myeloma
Double-hit multiple myeloma
Early multiple myeloma
Early myeloma
Evolving multiple myeloma
Evolving myeloma
Evolving plasma cell myeloma
Gamma PCM
Gamma plasma cell myeloma
High-risk multiple myeloma
Indolent myeloma
Indolent PCM
Indolent plasma cell myeloma
Kahler's disease
Medullary plasmacytoma
Multiple myeloma
Myeloma, NOS
Myelomatosis
Non-secretory myeloma
PCL
PCM
Plasmacytic leukemia
Primary PCL
Secondary plasma cell leukemia
Smoldering myeloma
Smoldering plasma cell myeloma
Triple-hit multiple myeloma
Definition
Plasma cell myeloma (PCM) is a bone marrow-based, multifocal neoplastic proliferation of plasma cells, usually associated with an M protein in serum and/or urine and evidence of organ damage related the plasma cell neoplasm.
Bone marrow is the site of origin of nearly all PCMs, and in most cases there is disseminated bone marrow involvement. Other organs may be secondarily involved. The disease spans a clinical spectrum from asymptomatic to highly aggressive. Diagnosis is based on a combination of clinical, morphological, immunological, and radiological features.
There are three clinical variants of plasma cell myeloma, all of which are coded to 9732/3.
1. Smoldering (asymptomatic) PCM: Bone marrow involvement, but no related organ or tissue impairment. Similar to MGUS in its lack of symptoms, but more likely to develop to symptomatic PCM. About 8% of patients are initially asymptomatic.
2. Non-secretory myeloma occurs when there is absence of an M protein on immunofixation electrophoresis; there is impaired (or not) secretion of immunoglobulin into the blood or urine. A bout 3% of PCM cases are non-secretory.
3. Plasma cell leukemia (PCL) occurs when the number of plasma cells in the peripheral blood is 20% of the leukocyte differential count. Other areas of involvement include spleen, liver, pleural effusions, ascites, and cerebrospinal fluid. PCL may be present at diagnosis or occur as a late feature of PCM (secondary PCL); 2-5% of myeloma cases are primary PCL. Clinical features overall are similar to PCM. Lymphadenopathy, organomegaly and renal failure are often present in PCL. PCL is an aggressive disease with short survival.
4. High-risk multiple myeloma: Having at least one of the mutations related with poor prognosis including; t(4;14) t(14;16), t(14;20), del 17p, p53 mutation, gain 1q and del 1p.
5. Double-hit multiple myeloma: Having at least two of the mutations related with poor prognosis including; t(4;14) t(14;16), t(14;20), del 17p, p53 mutation, gain 1q and del 1p.
6. Triple-hit multiple myeloma: Having at least three of the mutations related with poor prognosis including; t(4;14) t(14;16), t(14;20), del 17p, p53 mutation, gain 1q and del 1p.
Bone marrow is the site of origin of nearly all PCMs, and in most cases there is disseminated bone marrow involvement. Other organs may be secondarily involved. The disease spans a clinical spectrum from asymptomatic to highly aggressive. Diagnosis is based on a combination of clinical, morphological, immunological, and radiological features.
There are three clinical variants of plasma cell myeloma, all of which are coded to 9732/3.
1. Smoldering (asymptomatic) PCM: Bone marrow involvement, but no related organ or tissue impairment. Similar to MGUS in its lack of symptoms, but more likely to develop to symptomatic PCM. About 8% of patients are initially asymptomatic.
2. Non-secretory myeloma occurs when there is absence of an M protein on immunofixation electrophoresis; there is impaired (or not) secretion of immunoglobulin into the blood or urine. A bout 3% of PCM cases are non-secretory.
3. Plasma cell leukemia (PCL) occurs when the number of plasma cells in the peripheral blood is 20% of the leukocyte differential count. Other areas of involvement include spleen, liver, pleural effusions, ascites, and cerebrospinal fluid. PCL may be present at diagnosis or occur as a late feature of PCM (secondary PCL); 2-5% of myeloma cases are primary PCL. Clinical features overall are similar to PCM. Lymphadenopathy, organomegaly and renal failure are often present in PCL. PCL is an aggressive disease with short survival.
4. High-risk multiple myeloma: Having at least one of the mutations related with poor prognosis including; t(4;14) t(14;16), t(14;20), del 17p, p53 mutation, gain 1q and del 1p.
5. Double-hit multiple myeloma: Having at least two of the mutations related with poor prognosis including; t(4;14) t(14;16), t(14;20), del 17p, p53 mutation, gain 1q and del 1p.
6. Triple-hit multiple myeloma: Having at least three of the mutations related with poor prognosis including; t(4;14) t(14;16), t(14;20), del 17p, p53 mutation, gain 1q and del 1p.
Definitive Diagnostic Methods
Bence-Jones protein
Bone marrow biopsy
FISH
Genetic testing
Immunophenotyping
Peripheral blood smear
Serum Protein Electrophoresis (SPEP)
Genetics Data
Five major oncogenes involved in 14q32 translocation: cyclin D1, C-MAF, FGFR3/MMSET, cyclin D3, and MAFB
Immunoglobulin heavy and light chain genes are clonally rearranged
Trisomies
Whole or partial chromosome deletions or translocations
Immunophenotyping
CD19- (no expression/negative)
CD38
CD56 + (aberrant expression/positive) (except PCL)
CD56- (no expression/negative) (PCL)
CD79a
CD138
VS38c
Treatments
Chemotherapy
Hematologic Transplant and/or Endocrine Procedures
Hormone therapy
Immunotherapy
Transformations to
None
Transformations from
Same Primaries
Corresponding ICD-9 Codes
203.0 Multiple myeloma
Corresponding ICD-10 Codes
C90.0 Multiple myeloma
Corresponding ICD-10-CM Codes (U.S. only)
C90.0 Multiple Myeloma (effective October 01, 2015)
Signs and Symptoms
Anemia
Bence-Jones protein accumulation in the renal tubules causing renal damage
Bone pain
End-organ damage
Hypercalcemia
Pathological fractures
Serum monoclonal protein
Skeletal destruction with osteolytic lesions
Diagnostic Exams
Progression and Transformation
Extramedullary involvement usually indicates advanced disease
Epidemiology and Mortality
Age: 70 years median age (rare in children and adults less than 30)
Incidence: 10-15% of hematopoietic malignancies
Mortality: 20% of deaths from hematopoietic malignancies
Race: Occurs in african americans twice as much as caucasians
Sex: male predominance
Survival: Stage I: 62 months median survival; Stage II: 44 months median survival; Stage III: 29 months median survival
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: Mature B-cell neoplasms
Pages: 243-250
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (Revised 4th edition)
IARC: Lyon 2017
Section: Mature B-cell neoplasms
Pages: 243-250
International Classification of Diseases for Oncology, Third Edition, Second Revision. Geneva: World Health Organization, 2020.
Section: ICD-O-3.2 (2020) Morphological Codes
Pages: http://www.iacr.com.fr/index.php?option=com_content&view=category&layout=blog&id=100&Itemid=577
Section: ICD-O-3.2 (2020) Morphological Codes
Pages: http://www.iacr.com.fr/index.php?option=com_content&view=category&layout=blog&id=100&Itemid=577
National Cancer Institute
Section: General Information About Plasma Cell Neoplasms
Pages: https://www.cancer.gov/types/myeloma/hp/myeloma-treatment-pdq
Section: General Information About Plasma Cell Neoplasms
Pages: https://www.cancer.gov/types/myeloma/hp/myeloma-treatment-pdq
