Name
Extraosseous plasmacytoma
ICD-O-3 Morphology
9734/3: Extraosseous plasmacytoma
Effective
2001 and later
Reportable
for cases diagnosed
1978 and later
Primary Site(s)
See Module 7
Primary site cannot be bone (C400-419). Most common sites of involvement: upper respiratory tract (80%), oropharynx, nasopharynx, sinuses, larynx, gastrointestinal tract, lymph nodes, bladder, CNS, breast, thyroid, testis, parotid gland, and skin
Coding Manual:
Hematopoietic Coding Manual (PDF)
Abstractor Notes
This is a localized solitary tumor in sites other than 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).
Do not code primary site to C40._ or C41._. These category codes should only be used with histology 9731/3.
FISH and immunophenotyping help to differentiate these lesions from MALT and Marginal zone lymphomas.
Do not code primary site to C40._ or C41._. These category codes should only be used with histology 9731/3.
FISH and immunophenotyping help to differentiate these lesions from MALT and Marginal zone lymphomas.
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: PH2
Alternate Names
EMP
Extraosseous (extramedullary) plasmacytoma
Plasmacytoma, extramedullary
Plasmacytoma, NOS (occurring outside of bone)
Solitary plasmacytoma (occurring outside of 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.
Extraosseous (extramedullary) plasmacytomas are localized plasma cell neoplasms that arise in tissues other than bone. They occur most commonly in the mucous membranes of the upper air passages, but they can also occur in numerous other sites, including the gastrointestinal tract, lymph nodes, bladder, breasts, thyroid, testes, parotid glands, skin and CNS.
Plasmacytomas of the upper respiratory tract spread to cervical lymph nodes in about 15% of cases.
Extraosseous (extramedullary) plasmacytomas are localized plasma cell neoplasms that arise in tissues other than bone. They occur most commonly in the mucous membranes of the upper air passages, but they can also occur in numerous other sites, including the gastrointestinal tract, lymph nodes, bladder, breasts, thyroid, testes, parotid glands, skin and CNS.
Plasmacytomas of the upper respiratory tract spread to cervical lymph nodes in about 15% of cases.
Definitive Diagnostic Methods
FISH
Genetic testing
Histologic confirmation
Immunophenotyping
Genetics Data
MYC aberrations
t(11;14)(q13;q32) (IGH.CCNDI) lacking
Immunophenotyping
C19+ (expression/positive)
CD20+ (expression/positive)
CD45+ (expression/positive)
CD56+ (expression/positive)
Cyclin DI (usually lacking)
Treatments
Chemotherapy
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)
C90.2 Extramedullary plasmacytoma (effective October 01, 2015)
Signs and Symptoms
Diagnostic Exams
Progression and Transformation
Regional recurrences occur in ~25% of patients
Occasionally metastasis to distant extraosseous sites
Transformation to plasma cell myeloma occurs in ~15% of cases
Epidemiology and Mortality
Age: 55 years median age
Incidence: 3-5% of plasma cell neoplasms
Sex: male predominance
Survival: ~70% of patients remain disease free at 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: 251-253
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (Revised 4th edition)
IARC: Lyon 2017
Section: Mature B-cell neoplasms
Pages: 251-253
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