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Regional variation in survival following the diagnosis of cancer.

Recent studies have documented substantial geographical variation in patterns of treatment of cancer and other diseases. Because cancer treatment is not uniform nationwide, survival following the diagnosis of cancer might also be expected to vary geographically. Survival data from the nine population-based registries in the National Cancer Institute's Surveillance, Epidemiology, and End Results (SEER) Program were analyzed for cancers of the stomach, colon, rectum, lung, breast, uterus, ovary, prostate, and bladder (n = 401,861). The patients included all non-Hispanic white patients diagnosed with cancer of one of the selected sites during 1983-1991. Regional variation in crude five-year survival rates across the nine SEER areas was most marked for cancers of the uterus and prostate. For uterine cancer, for example, five-year survival ranged from 73.2% in Connecticut to 84.0% in Hawaii. Less marked variation was observed for cancers of the colon, rectum, and breast. For cancers of the bladder, ovary, stomach, and lung, survival rates five years after diagnosis were relatively invariant across the SEER areas. Observed differences in survival rates, although statistically significant, were relatively modest from the standpoint of the practicing physician. Nonetheless, the general pattern of regional variation was unchanged after adjustment for sex, age, stage, and surgical treatment and when analysis was limited to patients younger than 70 years, who would be anticipated to have the least comorbidity.

Demography↗

Multiple primary tumors involving cancer of the brain and central nervous system as the first or subsequent cancer.

BACKGROUND: This study was undertaken to determine whether cancer of the brain and central nervous system (CNS) occurs together with other types of cancer more often or less often than would be expected due to chance. METHODS: The study was based on data collected by the Surveillance, Epidemiology, and End Results (SEER) Program for cancers diagnosed in the U.S. between 1973 and 1998. The standardized incidence ratio (SIR) for new malignancies was estimated as the number of patients diagnosed with a particular type of cancer (observed), divided by the number of diagnoses expected based on person-years of follow-up and incidence rates for SEER cancer registries. RESULTS: Among patients who were diagnosed first with cancer of the brain or CNS, statistically significant excesses were observed for cancers of bone (SIR = 14.4), soft tissue (SIR = 4.6), brain and CNS (SIR = 5.9), salivary gland (SIR = 5.1), and thyroid gland (SIR = 2.7) in addition to acute myelocytic leukemia (SIR = 4.1) and melanoma of the skin (SIR = 1.7). Excess risk of new malignancies was markedly greater after first cancers of the brain or CNS diagnosed in childhood compared with similar diagnoses in adulthood. In reverse associations, significant excesses of cancer of the brain and CNS were observed only after diagnoses of acute lymphocytic leukemia (SIR = 7.4) and cancer of the testis (SIR = 1.8) or the thyroid gland (SIR = 1.7). CONCLUSIONS: Cancer treatment appears to have been the major factor underlying most of the positive associations between brain cancer and primary cancers of other types, with a probable lesser contribution from shared genetic susceptibility. Results provide little or no evidence that brain cancer shares important etiologic factors with the common cancers of adulthood.

Antineoplastic Agents↗

Vaginal clear cell adenocarcinoma in the United States.

We elected to examine available information from several sources to approximate the annual number of cases of vaginal adenocarcinoma in the United States for recent years. Data were obtained from the Registry of Hormonal Transplacental Carcinogenesis, the Surveillance, Epidemiology and End Results (SEER) program of the National Cancer Institute, the National Cancer Databank of the American College of Surgeons Commission on Cancer, and a survey of gynecologic oncologists practicing in the United States. In 1990 a total of 33 new cases and 11 recurrences were reported, while in 1991 23 new cases and 8 recurrences were reported. Neither SEER nor the Registry appear to provide adequate surveillance for this rare disease. Phase III clinical trials are not feasible, given the small number of patients. Statistically effective phase II one-armed studies to investigate new agents in the treatment of advanced or recurrent vaginal clear cell cancer may be possible. Effective mobilization of patients and physicians will be required for such trials to be completed in a timely manner.

Adenocarcinoma, Clear Cell↗

Anticipation in familial chronic lymphocytic leukaemia.

Anticipation, a phenomenon in which an inherited disease is diagnosed at an earlier age in each successive generation of a family, has been demonstrated in certain heritable neurological disorders and in multiple myeloma, non-Hodgkin's lymphoma and other haematological neoplasms. The present study was conducted to determine whether anticipation occurs in familial chronic lymphocytic leukaemia (CLL). Fourteen published reports of multigenerational familial CLL were analysed for anticipation, together with 10 previously unreported families with familial CLL, and the difference in disease-free survival between generations was determined. The difference between age at onset for each affected parent-child pair was tested against the null hypothesis that there was no difference in age at onset. The age at onset of the studied cases was also compared with that of the Surveillance Epidemiology and End Results (SEER) Program of the U.S. National Cancer Institute. The median ages at onset in the child and parent generations of all families (51.0 and 72.0 years respectively) were significantly different (P < 0.000001), and the null hypothesis was rejected (P < 0.000001). A significant difference was observed between the ages of onset of the child generation and the SEER population (P < 0.00001), but not between the parent generation and the SEER population. Anticipation characterizes familial CLL.

Age of Onset↗

Risk of perforation after colonoscopy and sigmoidoscopy: a population-based study.

BACKGROUND: Although the risk of bowel perforation is often cited as a major factor in the choice between colonoscopy and sigmoidoscopy for colorectal screening, good estimates of the absolute and relative risks of perforation are lacking. METHODS: We used a large population-based cohort that consisted of a random sample of 5% of Medicare beneficiaries living in regions of the United States covered by the Surveillance, Epidemiology, and End Results (SEER) Program registries to determine rates of perforation in people aged 65 years and older. We identified individuals who were cancer-free and had undergone colonoscopy or sigmoidoscopy between 1991 and 1998, calculated both the incidence and risk of perforation within 7 days of the procedure, and explored the impact on incidence and risk of perforation of age, race/ethnicity, sex, comorbidities, and indication for the procedure. We also estimated the risk of death after perforation. Risks were calculated with odds ratios (ORs) and 95% confidence intervals (CIs). All statistical tests were two-sided. RESULTS: There were 77 perforations after 39 286 colonoscopies (incidence = 1.96/1000 procedures) and 31 perforations after 35 298 sigmoidoscopies (incidence = 0.88/1000 procedures). After adjustment, the OR for perforation from colonoscopy relative to perforation from sigmoidoscopy was 1.8 (95% CI = 1.2 to 2.8). Risk of perforation from either procedure increased in association with increasing age (P(trend)<.001 for both procedures) and the presence of two or more comorbidities (P(trend)<.001 for colonoscopy and P(trend) =.03 for sigmoidoscopy). Compared with those who were endoscopied and did not have a perforation, the risk of death was statistically significantly increased for those who had a perforation after either colonoscopy (OR = 9.0, 95% CI = 3.0 to 27.3) or sigmoidoscopy (OR = 8.8, 95% CI = 1.6 to 48.5). The risk of perforation after colonoscopy, especially for screening procedures, declined during the 8-year study period. CONCLUSIONS: The risk of perforation after colonoscopy is approximately double that after sigmoidoscopy, but this difference appears to be decreasing. These observations should be useful to clinicians making screening and diagnostic decisions for individual patients and to policy officials setting guidelines for colorectal cancer screening programs.

Aged↗

Epidemiology of inflammatory breast cancer (IBC).

Inflammatory breast cancer (IBC) is a rare and aggressive form of breast cancer with unknown etiology and generally poor outcome. It is characterized by diffuse edema (peau d'orange) and redness (erythema), although either the disease itself or case definitions have varied over time and place, confounding temporal trends and geographic variations. In this review, we discuss case definitions for IBC and its clinical characteristics; describe its geographic variation, age and racial distribution, incidence and survival patterns, and summarize the very limited information on its epidemiologic risk factors. We also incorporate emerging data from the National Cancer Institute's (NCI) Surveillance, Epidemiology, and End Results (SEER) Program.

Adenocarcinoma↗

Male breast cancer: is the incidence increasing?

BACKGROUND: Male breast cancer is rare, and little is known about state population-level patterns of incidence. The primary objective of this study was to determine the incidence of MBC in Florida in comparison with the Surveillance, Epidemiology, and End RESULTS (SEER) program data. METHODS: Study data were obtained from the Florida Cancer Data System (FCDS). All males with pathologically confirmed invasive breast carcinoma diagnosed from 1985 to 2000 were included. Age-adjusted incidence rates, regional incidence rates, and descriptive statistics were calculated. Annual percent change (APC) for the study period was calculated with a linear model. Results were compared with the SEER data. RESULTS: A total of 1396 cases of MBC were identified. Age-adjusted incidence rates increased from 0.9 cases per 100,000 in 1990 to 1.5 cases per 100,000 in 2000. In 2000, the highest rates were in the age groups of 70 to 75 years (7.9) and > or =85 years (12.5). Infiltrating ductal was the most common subtype (92%); less common subtypes included mucinous (2%) and papillary (2%). Localized disease accounted for 45% of all cases, with regional disease in 33%, distant metastases in 7%, and unstaged in 15%. Most incident cases were diagnosed in the Palm Beach-Broward region (23%). The number of cases increased from 56 in 1985 to 132 new cases in 2000. The APC for this 16-year period was 2.0% (95% confidence interval [CI], 1.05-3.01; P <.005). SEER data indicated no change in MBC incidence rates (APC, 0.5; NS). CONCLUSIONS: The incidence of MBC in Florida increased significantly between 1985 and 2000. This finding is discordant with SEER incidence data. Further epidemiologic studies are warranted to investigate regional variation.

Adult↗

Cure fraction estimation from the mixture cure models for grouped survival data.

Mixture cure models are usually used to model failure time data with long-term survivors. These models have been applied to grouped survival data. The models provide simultaneous estimates of the proportion of the patients cured from disease and the distribution of the survival times for uncured patients (latency distribution). However, a crucial issue with mixture cure models is the identifiability of the cure fraction and parameters of kernel distribution. Cure fraction estimates can be quite sensitive to the choice of latency distributions and length of follow-up time. In this paper, sensitivity of parameter estimates under semi-parametric model and several most commonly used parametric models, namely lognormal, loglogistic, Weibull and generalized Gamma distributions, is explored. The cure fraction estimates from the model with generalized Gamma distribution is found to be quite robust. A simulation study was carried out to examine the effect of follow-up time and latency distribution specification on cure fraction estimation. The cure models with generalized Gamma latency distribution are applied to the population-based survival data for several cancer sites from the Surveillance, Epidemiology and End Results (SEER) Program. Several cautions on the general use of cure model are advised.

Cohort Studies↗

Merkel cell carcinoma: changing incidence trends.

BACKGROUND: The objective of this study was to define the incidence trends of Merkel cell carcinoma (MCC), a rare and aggressive cutaneous malignancy. MATERIALS AND METHODS: All cases of MCC of the skin between 1986 and 2001 were identified using the surveillance, epidemiology, and end results (SEER) program. Overall age-adjusted, gender-specific, age-specific, stage-specific, and regional incidence rates were calculated. All rates are per 100,000 and age-adjusted to the 2000 US standard population. Estimated annual percent change (EAPC) was calculated using a linear least squares model. RESULTS: A total of 1,124 cases of MCC were identified in the SEER registries. The rate of MCC increased from 0.15 cases per 100,000 in 1986 to 0.44 cases per 100,000 in 2001. The EAPC for the time period was 8.08%. This was statistically significant (95% CI: 6.29, 9.90, P-value < 0.05). Age-specific incidence (5-year age groups) were highest in the elderly, 4.28 per 100,000 in the 85+ age group. CONCLUSIONS: MCC incidence rates have increased threefold over the 1986-2001 period. Rates are highest in the elderly population. Further etiologic studies and identification of high-risk populations are warranted.

Adult↗

Malignant mesothelioma surveillance: a comparison of ICD 10 mortality data with SEER incidence data in nine areas of the United States.

With the implementation in 1999 of ICD-10 death certificate coding in the United States, mortality data specific to malignant mesothelioma became readily available on a national basis. To evaluate the accuracy and completeness of diagnosis and coding for mesothelioma on the death certificate, mortality information was compared with incidence data. A mortality/incidence ratio was calculated for each of the nine areas covered by the SEER Program, using National Vital Statistics mortality data from 1999 and 2000, and the SEER incidence data for 1998 and 1999. The mortality/incidence ratio for the two years combined for all areas was 0.82. Only two areas (Connecticut and Atlanta) had ratios <80%. The overall correlation coefficient between mortality and incidence rates was 0.96. Thus, mortality data coded using ICD-10 can be a valid source for mesothelioma surveillance and can be instituted without major cost if a national mortality statistics program based on ICD-10 is in place, making it feasible even for developing countries.

Death Certificates↗

Explaining geographic variation in breast and cervical cancer incidence rates in US Hispanic women.

OBJECTIVE: This study examined geographic variation in incidence rates for two cancers common in US Hispanic women and considered some potential explanations, by using data from several high-quality cancer registries. METHODS: Age-standardized incidence rates (ASIRs) per 100,000 Hispanic women per year were analyzed for breast and cervical cancer in the population-based cancer registries of the Surveillance, Epidemiology and End Results (SEER) Program. The percentage potentially misclassified (PPM) as Hispanic, because of the frequent absence of maiden name, was estimated. Sociodemographic characteristics of the cancers and of the Hispanic population in eight areas were compared. RESULTS: The ASIRs varied from 80.9 to 113.3 for breast and 8.2 to 19.9 for cervix cancers. The PPM was higher for breast (16%) than for cervix (7%). Differences in PPM across SEER areas did not appear consistent with geographic variation in ASIRs, while some variation in ASIRs was consistent with differences in sociodemographic characteristics. Registry data on subgroups (defined by ancestry and birthplace) were too incomplete for analyses of CONCLUSIONS: Some of the geographic variation appeared to be explained by sociodemographic factors, but improvements in cancer registries are needed to analyze ASIRs for Hispanic subgroups.

Adolescent↗

Is there a 'cancer corridor' in Louisiana?

Cancer mortality rates in South Louisiana are higher than the national averages, leading to the area's designation as a "cancer corridor". This study was conducted to assess whether incidence data substantiate the reputation derived from mortality statistics. Age-adjusted cancer incidence rates for 1983-1987 were calculated for South Louisiana as a whole, for five regional divisions of it, and for the combined nine areas of the Surveillance, Epidemiology, and End Results (SEER) program. Significantly lower (p < 0.0001) incidence rates were found in South Louisiana among white females, black males, and black females for cancers of all sites combined; among women of both races for cancer of the breast; among men of both races for cancers of the colon and prostate; and among whites of both sexes for melanoma and rectal cancer. South Louisiana incidence rates were significantly higher than the SEER rates only for lung and larynx cancers in white males. The excess of lung cancer was statistically significant in four out of five regions while the laryngeal cancer excess was significant only in the New Orleans area. The excessive mortality rates reported for South Louisiana are not the result of excessive incidence. These results indicate poorer cancer prognosis in this region, a phenomenon that deserves more scrutiny by the health profession.

Female↗

Glottic cancer in Ontario, Canada and the SEER areas of the United States. Do different management philosophies produce different outcome profiles?

We compared the management and outcome of glottic cancer in Ontario, Canada to that in the Surveillance, Epidemiology and End Results (SEER) Program areas in the United States to determine whether the greater use of primary radiotherapy with surgery reserved for salvage in Ontario was associated with similar survival and better larynx retention rates than the U.S. approach where primary surgery is used more often. Electronic, clinical and hospital data were linked to cancer registry data and supplemented by chart review where necessary. Initial treatment and survival in patients diagnosed in the SEER areas from 1988 through 1994 were compared to patients from Ontario diagnosed from 1982 through 1995. Actuarial laryngectomy rates were compared for patients over 65 at diagnosis in the two regions. Analyses were conducted over all cases and stratified by disease stage. In localized disease (T1 or T2), conservative treatment was the most common initial treatment in both regions, although total laryngectomy was used more often in SEER than Ontario (6.2% vs. 0.2%, respectively, P <.001). In advanced disease (T3 or T4), total laryngectomy was more commonly used as initial treatment in SEER (62.9% vs. 21.0% in Ontario, P < or =.001). Over all cases, the relative survival rate was 80% in Ontario at 5 years compared to 78% in SEER (P =.33). In localized disease, the relative survival rates were 4 to 5% higher in Ontario from the second year on, while in advanced disease 2 to 3% higher rates in SEER did not approach statistical significance. Actuarial laryngectomy rates at 3 years differed between the two regions, with a 4% higher rate in SEER (P =.01). In localized disease, 12.6% of Ontario patients had a laryngectomy by 3 years postdiagnosis compared to 17.9% in SEER (P =.05). In advanced disease, the rates were 63.3% and 79.2%, respectively (P =.07). There are large differences in the management of glottic cancer between the SEER areas of the U.S. and Ontario and no evidence that a policy emphasizing radiotherapy with surgery reserved for salvage is associated with worse survival. Ultimate laryngectomy rates are lower in Ontario for localized disease and may be lower for advanced disease. Conservation treatment should be used for localized disease while the treatment decision in advanced disease may be especially sensitive to patient values for voice retention versus initial cure.

Aged↗

Evidence for an association between cutaneous melanoma and non-Hodgkin lymphoma.

BACKGROUND: Over the past 2 decades both cutaneous melanoma (CM) and non-Hodgkin lymphoma (NHL) incidence rates have increased substantially. One approach to better understanding the etiologic basis for these increases is to examine the risk of NHL in CM survivors and the risk of CM in NHL survivors. METHODS: To explore the possible association between CM and NHL, the authors followed cohorts of CM and NHL patients registered through the National Cancer Institute's Surveillance, Epidemiology, and End Results (SEER) program between 1973 and 1996 and identified patients who developed CM after NHL and NHL after CM. The number of observed cases then were compared with the number of expected cases to see if CM survivors were at an increased risk of NHL or if NHL survivors were at an increased risk of CM. RESULTS: Between 1973 and 1996, 54,803 CM patients and 62,597 NHL patients who met the authors' inclusion criteria were identified through SEER. The authors found statistically significant elevated risks of NHL among CM survivors (standardized incidence ratio [SIR], 1.42; 95% confidence interval [CI], 1.23-1.63) and CM among NHL survivors (SIR, 1.75; 95% CI, 1.48-2.07). CONCLUSIONS: These results support an association between CM and NHL. Although detection bias and posttherapy effects may explain part of this association, shared genetic or etiologic factors, such as sunlight exposure, also may play a role.

Female↗

Ductal carcinoma in situ: trends in geographic, temporal, and demographic patterns of care and survival.

While long-term prognosis is excellent, treatment of ductal carcinoma in situ (DCIS) remains controversial and inconsistent. The purpose of this study was to track geographic and temporal patterns of care for women diagnosed with DCIS, and analyze subsequent cancer-specific risk of mortality. Subjects for this study were 41,245 women diagnosed with primary DCIS in the National Surveillance, Epidemiology, and End Results (SEER) program from 1973 through 2000. Variables analyzed included patient age, year of diagnosis, SEER site of residence at the time of diagnosis, treatment (surgery, radiation), race, age, and hormone (estrogen and progesterone) receptor status. The percentage of women diagnosed with DCIS out of the total number of women diagnosed with breast cancer increased steadily between 1973 and 2000, with the largest increase occurring around 1985. Breast conserving surgery (BCS) utilization increased over time for each SEER site to the current proportions, ranging from 49.5% in Utah to 76.9% in Connecticut. Younger women were significantly more likely to receive mastectomy and had significantly lower risk of death. Women receiving BCS who also had radiation therapy (RT) had a significantly lower risk of death, although those receiving mastectomy had the lowest risk of death. Black women and Asian/Pacific island women were significantly more likely to receive BCS, although black women were less likely to receive follow-up RT and had a significantly increased risk of death. Racial and age differences in the treatment of DCIS resulted in significantly disparate rates of survival, which should be considered in public health programming. Mastectomy utilization resulted in improved survival, although additional studies may elucidate the interaction of treatment with patient age.

Adult↗

Myeloid leukemia in Prader-Willi syndrome.

OBJECTIVE: To evaluate the frequency of cancers recorded by the Surveillance, Epidemiology, and End Results (SEER) Program in persons with Prader-Willi syndrome (PWS) METHODS: A survey was mailed in 1994 to 1852 registrants of the PWS Association (USA) inquiring about a diagnosis of any type of benign tumor or cancer (malignant tumor or leukemia). The risk of developing cancer was then estimated by comparing the observed number of cancers in the PWS population during 1975 to 1994 to the expected number in the general US population using data from the 1971-1994 SEER Cancer Statistics Review. RESULTS: Of the 1852 persons, 1160 (63%) responded, or 75% (1160/1552) of those who received the survey. The total number of observed cancer cases in the PWS study population was 8 versus 4.80 expected in the general US population (P =.1610). Three cases of myeloid leukemia were observed versus 0.075 leukemias expected (P =.0001). CONCLUSIONS: There appears to be an increased risk of myeloid leukemias, but not other cancers, among persons with PWS.

Adolescent↗

Age and survival from squamous cell carcinoma of the oral tongue.

BACKGROUND: A worse outcome for young patients with head and neck squamous cell carcinoma has been previously suggested in the literature. This issue has been investigated with respect to squamous cell carcinoma of the oral tongue. METHODS: The Surveillance, Epidemiology and End Results (SEER) program tumor registries were used. Cases of squamous cell carcinoma of the oral tongue (ICD-9 codes 141.1-141.5) diagnosed from 1988-1993 in which this cancer was the one and only cancer were included (n = 749). Disease-specific survival was evaluated with respect to age, type of surgical treatment, and radiotherapy while stratifying for stage using Cox proportional hazards analysis. A secondary analysis included the additional variables, tumor size and nodal status. (These fields were recorded in SEER for only about half of the cases in the primary analysis.) RESULTS: Analysis revealed that increasing age predicted worse disease-specific survival. A 10-year increase in age was associated with an 18% increase in risk of death. Surgical therapy with excision of the primary tumor alone or excision plus neck dissection predicted improved survival, whereas the use of radiotherapy was associated with worse survival. (The latter may reflect bias associated with positive surgical margins or premorbid conditions.) In the secondary analysis, age, tumor size, and positive lymph node status were associated with significantly worse disease-specific survival, whereas surgical excision plus neck dissection was associated with improved survival. CONCLUSION: The SEER database shows increased disease-specific mortality with increasing age in patients with cancer of the oral tongue. Surgical therapy is associated with improved survival, whereas the use of radiotherapy, increasing tumor size, and positive lymph node status are associated with worse outcome.

Adult↗

Mammography use, breast cancer stage at diagnosis, and survival among older women.

BACKGROUND: Women age 65 years and older account for most newly diagnosed breast cancers and deaths from breast cancer. Yet, older women are least likely to undergo mammography, perhaps because mammography's value is less well demonstrated in older women. OBJECTIVE: To investigate the relationship between prior mammography use, cancer stage at diagnosis, and breast cancer mortality among older women with breast cancer. DESIGN: Retrospective cohort study using the Linked Medicare-Tumor Registry Database. SETTING: Population-based data from three geographic areas included in the National Cancer Institute's Surveillance, Epidemiology, and End Results (SEER) program. PARTICIPANTS: Women aged 67 and older diagnosed with a first primary breast cancer, from 1987 to 1993, residing in Connecticut, metropolitan Atlanta, Georgia, or Seattle-Puget Sound, Washington. MEASUREMENTS: Medicare claims were reviewed and women were classified according to their mammography use during the 2 years before diagnosis: nonusers (no prior mammograms), regular users (at least two mammograms at least 10 months apart), or peri-diagnosis users (only mammogram(s) within 3 months before diagnosis). Mammography utilization was linked with SEER data to determine stage at diagnosis and cause of death. Our main outcome variables were (1) stage at diagnosis, classified as early (in situ/Stage I) or late (Stage II or greater), and (2) breast cancer mortality, measured from diagnosis until death from breast cancer or end of the follow-up period (December 31, 1994). RESULTS: Older women who were nonusers of mammography were diagnosed with breast cancer at Stage II or greater more often than regular users (adjusted odds ratio (OR), 3.12; 95% confidence interval (CI), 2.74-3.58). This association was present within each age group studied. Nonusers of mammography were at significantly greater risk of dying from their breast cancer than regular users for all women (adjusted hazard ratio (HR), 3.38; 95% CI, 2.65-4.32) and for women within each age group. Even assuming a lead time of 1.25 years, nonusers of mammography continued to be at increased risk of dying from breast cancer. Our findings remained significant for all women and for the two youngest age groups (67-74 years, 75-85 years), although the benefit was no longer statistically significant for the oldest women (85 years and older). CONCLUSIONS: Older women who undergo regular mammography are diagnosed with an earlier stage of disease and are less likely to die from their disease. These data support the use of regular mammography in older women and suggest that mammography can reduce breast cancer mortality in older women, even for women age 85 and older.

Aged↗