Search Database ICD-O-3 Code Lists

Name

Plasma cell myeloma (PCM)

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) is part of the Plasma cell neoplasms and other diseases with paraproteins lineage table in the WHO 5th edition of Hematolymphoid Tumors. (See Appendix B in the Hematopoietic Manual, Table B17)

Many times, a bone marrow biopsy report may state "Plasma cell neoplasm consistent with plasma cell myeloma." This is a definitive statement of plasma cell myeloma.

Pathology reports may also simply state "plasma cell neoplasm," without further confirmation. If the managing physician later diagnoses the patient with plasma cell myeloma based on clinical factors, this is reportable and diagnostic confirmation code would be 8; however, if the managing physician states, "based on the pathology report," then it would be a diagnostic confirmation code of 1.

Generalized or multifocal bone marrow involvement is typically present at diagnosis. Lytic bone lesions and focal tumoral masses of plasma cells also occur, most commonly in sites of active haematopoiesis.

Extramedullary involvement in the form of soft tissue masses, diffuse organ infiltration, or circulating plasma cells is usually a manifestation of advanced disease, with a higher prevalence among patients with relapsed plasma cell myeloma / multiple myeloma (PCM/MM).

The presence of a plasmacytoma and a diagnosis of plasma cell myeloma diagnosed during the initial clinical workup is evidence of advanced disease. One primary is abstracted, the plasma cell myeloma (See Rule M11)

The presence of a plasmacytoma after a diagnosis of plasma cell myeloma is evidence of advanced disease and not a new primary (See Rule M13)

The Revised International Staging System (R-ISS) for multiple myeloma, based on pretreatment serum B2M, albumin, and LDH levels, as well as on interphase FISH–based chromosomal abnormalities, provides a strong predictor of survival.
- See the SSDI Manual, Plasma Cell Myeloma schema for more information on the R-ISS staging system)

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.

Module Rule

None

Alternate Names

Early/evolving plasma cell (multiple) myeloma
Multiple myeloma
Multiple plasmacytomas (occurring in bone or outside of bone)
Myeloma, NOS
Myelomatosis
Non-secretory myeloma
Plasma cell leukemia (PCL) (primary, secondary, NOS) (see 9733/3 prior to 2010)
Plasmacytic leukemia (see 9733/3 prior to 2010)
Smoldering (asymptomatic) myeloma

Definition

Plasma cell myeloma is a bone marrow-based, multifocal neoplastic proliferation of plasma cells. Multiple myeloma is defined by the combination of plasma cell myeloma, usually associated with a serum and/or urine monoclonal immunoglobulin (Ig), and either evidence of organ damage related to the disease, or in the absence of organ damage, laboratory or imaging findings that suggest a high risk of developing end-organ damage within 2 years. (WHO 5th edition)

Definitive Diagnostic Methods

Clinical diagnosis
Cytogenetics
Genetic testing
Histologic confirmation
Immunohistochemistry
Immunophenotyping

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

CD38+ (expression/positive)
CD56- (no expression/negative)
CD138+ (expression/positive)
CD229+ (expression/positive)
CD319+ (expression/positive)

Treatments

Chemotherapy
Hematologic Transplant and/or Endocrine Procedures
Hormone therapy
Immunotherapy

Transformations to

None

Transformations from

Same Primaries

Corresponding ICD-10 Codes (Cause of Death codes only)

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
Skeletal destruction with osteolytic lesions

Diagnostic Exams

Blood and urine immunoglobulin studies
Blood chemistry studies
Bone marrow aspiration and biopsy
Cytogenetic analysis
Highly sensitive imaging (CT, PET-CT, MRI)
Serum and urine protein electrophoresis and immunofixation
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

WHO Classification of Tumours Editorial Board. Haematolymphoid tumours. Lyon (France): International Agency for Research on Cancer; 2024. (WHO classification of tumours series, 5th ed.; vol. 11). https://publications.iarc.who.int/637.
Section: Plasma cell neoplasms and other diseases with paraproteins
Pages: Part B: 625-630

International Classification of Diseases for Oncology, 3rd edition (including revisions). Geneva: World Health Organization, 2001, 2011, 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

PDQ® Adult Treatment Editorial Board. PDQ Plasma Cell Neoplasms (Including Multiple Myeloma) Treatment. Bethesda, MD: National Cancer Institute. Updated <03/28/2025>. Available at: https://www.cancer.gov/types/myeloma/hp/myeloma-treatment-pdq. Accessed <03/30/2025>. [PMID: 26389362]
Section: Plasma Cell Neoplasms (Including Multiple Myeloma) Treatment (PDQ®)–Health Professional Version
Pages: https://www.cancer.gov/types/myeloma/hp/myeloma-treatment-pdq
Glossary