Name
Solitary plasmacytoma of bone
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.
Coding Manual:
Hematopoietic Coding Manual (PDF)
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.
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
M-protein found in serum or urine
Palpable mass due to soft tissue expansion
Diagnostic Exams
Blood and urine immunoglobulin studies
Blood chemistry studies
Bone marrow aspiration and biopsy
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
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
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