Plasma Cell Myeloma - 20-Year Comparative Survival and Mortality of Three Plasma Cell Myeloma ICD-O-3 Oncologic Phenotypes by Age, Sex, Race, Stage, Cohort Entry Time-Period and Disease Duration: A Systematic Review of 111,041 Cases for Diagnosis Years 1973-2014: (SEER*Stat 8.3.4).

BACKGROUND: -The values of SEER site recode variables are based on the primary site and histology data fields submitted to SEER by the registries. The site recode variables define the major cancer site/histology groups that are commonly used in the reporting of cancer incidence data and are added to the SEER databases as a convenience for researchers. These codes and definitions are periodically updated and changed by the National Cancer Institute as newer and more applicable information becomes available. Because this myeloma analysis includes cases diagnosed 2010+, the ICD-O-3 recode-updates with adjustment for WHO 2008 hematopoietic histologies that account for changes in the obsolete classification of hematopoietic histology codes, and the assignment of new names (ie, multiple myeloma-MM - to - plasma cell myeloma-PCM) is adhered to and used here. Plasma cell myeloma (PCM) is a bone-marrow based multifocal plasma cell malignancy (primary site C421). PCM is characterized by a single clone of plasma cells, believed to be derived from lymphoid B cells, and spans a clinical spectrum from asymptomatic to aggressive forms, plus disorders caused by the deposition of abnormal immunoglobulin chains in tissue. The current myeloma group ICD-O-3 histologic morphology types consists of: ICD-O-3 9731: Plasmacytoma, NOS, occurring in bone (osseous plasmacytoma malignancy data reportable to SEER only beginning since 1986); ICD-O-3 9732: Plasma cell myeloma - composed of three clinical variants: a) asymptomatic, b) Non-secretory myeloma, and c) Plasma cell leukemia (all coded to 9732); ICD-O-3 9734: Extramedullary plasmacytoma; anatomic sites other than bone.

OBJECTIVE: -Using the statistical database of SEER*Stat 8.3.4 (produced 4/14/2017 for diagnosis years 1973-2014), to assess, determine, compare, and summarize the occurrence, long-term survival and mortality indices of the three morphologic types of myeloma by age, sex, race and stage in two-cohort entry time-periods (1973-1994 and 1995-2014). All analyses are accomplished within the context of current SEER Site Recode ICD-O-3 (1/27/2003) definitions, terminologies and descriptions, and also in accordance with the rules of the consolidated Hematopoietic and Lymphoid Neoplasm Coding Manual data base (effective 1/1/2010 - release date January 2015).

METHODS: -Population data including 111,041 cases collected by the National Cancer Institute's Surveillance, Epidemiology, and End Results (SEER) Frequency Database (18 SEER Registries Research Data + Hurricane Katrina Impacted Louisiana Cases, November 2016 Submission, 1973-2014 varying) for diagnosis years 1973-2014: Relative Survival Statistics were analyzed in two cohorts: 1973-1994 and 1995-2014. Survival statistics were derived from: SEER*Stat Database: Incidence - SEER 9 Regs Research Data, November 2016 Submission (1973-2014) <Katrina/Rita Population Adjustment> Released April 2017.

RESULTS: -Tables 1-3 provide basic SEER comparative survival and mortality data of the three myeloma oncotypes by age, sex, stage and disease duration of patients in the 1973-2014 time-period. Epidemiologic, demographic, and case statistics data extracted from the most current NCI Cancer Statistics Review (CSR 2010-2014) are included.

CONCLUSIONS: -Recent SEER age-adjusted incidence trends, 2011-2014, for all races has been downward, with an annual percentage change (APC) of -2.5% per year. Mean age in plasma cell myeloma (PCM) patients was about 1-year less in males (67.8 yrs) than in females (69.2 yrs). PCM is accompanied by a very high excess mortality and much reduced 5-year relative survival ratio especially in older age groups. Generally, first year excess death rates (EDRs) decreased with duration but increased with advancing entry age, and there was no sex difference. First year EDRs in blacks, all ages combined, was quite high but lower than EDRs in whites. Median survival, actual survival and 5-year relative survival ratios diminished precipitously to extremely low levels with increasing entry age attesting to the lethal character of this disease especially in older patients.