Solitary plasmacytoma of bone

Name
Solitary plasmacytoma of bone
ICD-O-1 Morphology
9731/3
Effective 1978 - 1991
ICD-O-2 Morphology
9731/3
Effective 1992 - 2000
ICD-O-3 Morphology
9731/3
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.

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Grade
6 - B-cell
Module Rule
Module 2: PH3, PH4
Alternate Names
Osseous plasmacytoma
Plasmacytoma, NOS (occurring in bone)
Solitary myeloma
Solitary plasmacytoma
Definition
A type of cancer that begins in the plasma cells (white blood cells that produce antibodies). A plasmacytoma may turn into multiple myeloma. Clonal proliferation of plasma cells cytologically and immunophenotypically identical to those of plasma cell myeloma but manifesting as localized osseous growth.

Plasmacytoma is a localized collection of monoclonal plasma cells. Diagnosed on basis of: single bone lesion with histology consistent with a plasma cell tumor; absence of a plasma cell infiltrate in random bone marrow biopsies; no evidence of other bone lesions by radiographic examination; absence of renal failure; no hypercalcemia; no anemia.
Abstractor Notes
This is a localized tumor in the bone consisting of monoclonal plasma cells. Complete skeletal radiographs (preferably MRI) show no other lesions.

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.
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
Negative: CD19
Positive: CD38, CD56, CD79a, CD138, Cytoplasmic Ig, VS38c
Surface Ig lacking
Treatments
Radiation
Surgery
Transformations to
Transformations from
None
Same Primaries
There are no known same primaries
Corresponding ICD-9 Codes
203.8 Other immunoproliferative neoplasms
Corresponding ICD-10 Codes
C90.2 Plasmacytoma, extramedullary
Corresponding ICD-10-CM Codes (effective October 1, 2015 U.S. only)
C90.3 Solitary plasmacytoma
Signs and Symptoms
Bone pain at site of lesion
M-protein found in serum or urine
Palpable mass due to soft tissue expansion
Vertebral lesions with symptomatic cord compression
Diagnostic Exams
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
Sex: male predominance
Survival: 1/3 of patients remain disease free at 10 years, median overall survival 10 years