Name

Plasma cell myeloma

ICD-O-1 Morphology

Effective 1978 - 1991

ICD-O-2 Morphology

Effective 1992 - 2000

ICD-O-3 Morphology

Effective 2001 and later

Reportable

for cases diagnosed 1978 and later

Primary Site(s)

C421
Primary site must be bone marrow (C421)

Grade

Not Applicable

Module Rule

None

Alternate Names

Alpha PCM
Early myeloma
Evolving myeloma
Gamma PCM
Indolent myeloma
Indolent PCM
Kahler's disease
Medullary plasmacytoma
Multiple myeloma
Multiple plasmacytomas (occurring in bone or outside of bone)
Myeloma, NOS
Myelomatosis
Non-secretory myeloma
PCL
PCM
Plasma cell leukemia
Plasmacytic leukemia
Primary PCL
Smoldering 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.

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.

Definitive Diagnostic Methods

Bence-Jones protein
Bone marrow biopsy
FISH
Genetic testing
Immunophenotyping
Peripheral blood smear

Genetics Data

Five major oncogenes involved in 14q32 translocation: cyclin D1, C-MAF, FGFR3/MMSET, cyclin D3, and MAFB
High load of IGHV gene somatic hypermutation
Immunoglobulin heavy and light chain genes are clonally rearranged
Trisomies
Whole or partial chromosome deletions or translocations

Immunophenotyping

CD19-
CD38
CD56 aberrantly expressed (except PCL)
CD56- (PCL)
CD79a
CD138
VS38c

Treatments

Chemotherapy
Hematologic Transplant and/or Endocrine Procedures
Hormone therapy
Immunotherapy

Transformations to

None

Corresponding ICD-9 Codes

203.0 Multiple myeloma
203.1 Plasma cell leukemia

Corresponding ICD-10 Codes

C90.0 Multiple myeloma
C90.1 Plasma cell leukemia

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

C90.0 Multiple Myeloma (effective October 01, 2015)
C90.1 Plasma cell leukemia (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
Skeletal destruction with osteolytic lesions

Diagnostic Exams

Blood and urine immunoglobulin studies
Blood chemistry studies
Cytogenetic analysis
Skeletal survey
Twenty-four-hour urine test

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

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 Plasma Cell Neoplasms
Pages: https://www.cancer.gov/types/myeloma/hp/myeloma-treatment-pdq
Glossary