Name

Solitary plasmacytoma of bone

ICD-O-1 Morphology

9731/3: Solitary plasmactyoma of bone
Effective 1978 - 1991

ICD-O-2 Morphology

9731/3: Solitary plasmactyoma of bone
Effective 1992 - 2000

ICD-O-3 Morphology

9731/3: Solitary plasmactyoma of bone
Effective 2001 and later

Reportable

for cases diagnosed 1978 and later

Primary Site(s)

C400-C419
Primary site must be bone (C400-C419). The most common bones involved are the vertebrae. The thoracic vertebrae are more commonly involved then the cervical or lumbar. Other common bones include the ribs, skull, pelvis, femur, clavical, and scapula.

Abstractor Notes

This is a localized solitary tumor in the bone consisting of monoclonal plasma cells. Complete skeletal radiographs (preferably MRI) show no other lesions. If additional lesions are found on MRI or CT (or other radiological surveys), this is diagnostic of plasma cell myeloma (see 9732/3).

There is no evidence of bone marrow plasmacytosis other than the solitary lesion. Vertebral lesions may be associated with symptomatic cord compression.

Patient presents with bone pain at the site of the lesion or with a pathological fracture. Soft tissue extension may produce a palpable mass.
An M-protein is found in the serum or urine in 24-72% of patients.

Local control is achieved by radiotherapy. Plasma cells are usually easily recognizable in tissue specimens. Even when the diagnosis is apparent, determination of light chain type is suggested.

Plasma cell neoplasm was previously included as an alternate name for plasmacytoma. The phrase "plasma cell neoplasm" is an umbrella term used for many diseases, some of which are not reportable. If the only diagnosis is "plasma cell neoplasm," then do not report this case. If it is "plasma cell neoplasm" consistent with a plasmacytoma or multiple myeloma, then the case would be reportable.

If the diagnosis is "plasmacytoma" and there is no additional information to indicate if it's a plasmacytoma of the bone or an extramedullary plasmacytoma, default to plasmacytoma of the bone and assign histology, 9731/3.

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

Module 2: PH3, PH4

Alternate Names

Osseous (medullary) plasmacytoma
Plasma cell tumor
Plasmacytoma, NOS (occurring in bone)
Plasmacytoma of bone
Solitary myeloma
Solitary osseous (medullary) plasmacytoma
Solitary plasmacytoma (occurring in bone)

Definition

Solitary plasmacytomas are single localized tumors consisting of monoclonal plasma cells with no clinical features of plasma cell myeloma (PCM)(see 9732/3) and no physical or radiological evidence of additional plasma cell tumors.

Definitive Diagnostic Methods

Genetic testing
Histologic confirmation
Immunophenotyping

Genetics Data

Ig clonal rearrangements, complex karyotypes with multiple chromosomal gains/losses; also translocations, deletions, mutations

Immunophenotyping

CD19- (no expression/negative)
CD38+ (expression/positive)
CD56+ (expression/positive)
CD79a+ (expression/positive)
Cytoplasmic Ig+ (expression/positive)
Surface Ig lacking
VS38c+ (expression/positive)

Treatments

Radiation therapy
Surgery

Transformations to

Transformations from

None

Same Primaries

Corresponding ICD-9 Codes

203.8 Other immunoproliferative neoplasms
238.6 Plasma cells

Corresponding ICD-10 Codes

C90.2 Plasmacytoma, extramedullary

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

C90.3 Solitary plasmacytoma (effective October 01, 2015)

Signs and Symptoms

Bone pain at site of lesion
M-protein found in serum or urine
Palpable mass due to soft tissue expansion
Pathologic fractures at site of lesion
Vertebral lesions with symptomatic cord compression

Diagnostic Exams

Blood and urine immunoglobulin studies
Blood chemistry studies
Cytogenetic analysis
Tissue biopsy
Twenty-four-hour urine test

Progression and Transformation

2/3 of patients eventually evolve to generalized myeloma or additional solitary or multiple plasmacytomas

Epidemiology and Mortality

Age: 55 years median age
Incidence: 3-5% of plasma cell neoplasms
Sex: male predominance
Survival: 1/3 of patients remain disease free at 10 years, median overall survival 10 years

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: 250-251

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

National Cancer Institute
Section: General Information About Plasma Cell Neoplasms
Pages: https://www.cancer.gov/types/myeloma/hp/myeloma-treatment-pdq
Glossary