Survival rates for primary malignant brain tumours in Europe

In the framework of EUROCARE, a concerted action between 45 population-based cancer registries, in 17 European countries, survival of patients with primary malignant brain tumours was investigated. Survival analysis was carried out on 16 268 patients diagnosed between 1985 and 1989 and followed-up for at least 5 years. The mean European age-standardised 5-year relative survival was 17% in men and 20% in women, with minimal intercountry variations, except for markedly lower rates in Scotland, Estonia and Poland. The age-specific analysis showed a relatively uniform survival in patients aged more than 65 years at diagnosis, but there were more marked intercountry differences in younger patients. In the 15–44 year age group (25% of the total study population) 5-year relative survival ranged between 55% (Finland and Sweden) and 27% (Poland). Generally, survival decreased with increasing age at diagnosis. The analysis of a temporal trend in survival was carried out on a subset of registries with available data from 1978–1989. Overall, there was an increase in survival over the considered study period, mostly confined to 1-year survival, suggesting that it was mostly related to improved diagnostic techniques. The most important survival increase occurred in the younger patients, both for 1- and 5-year survival, suggesting that younger patients have less biologically aggressive tumours, benefiting from the combined effect of diagnostic accuracy and effective therapies. The most marked survival increase was seen in England and Denmark, countries with low survival rates at the beginning of the study period, whereas in Finland and Germany, where survival was relatively high to begin with, no important temporal trend was seen.     

Survival of patients with skin melanoma in Europe increases further: Results of the EUROCARE-5 study

BackgroundIn Europe skin melanoma (SM) survival has increased over time. The aims were to evaluate recent trends and differences between countries and regions of Europe.MethodsRelative survival (RS) estimates and geographical comparisons were based on 241,485 patients aged 15 years and over with a diagnosis of invasive SM in Europe (2000–2007). Survival time trends during 1999–2007 were estimated using the period approach, for 213,101 patients. Age, gender, sub-sites and morphology subgroups were considered.ResultsIn European patients, estimated 5-year RS was 83% (95% confidence interval, CI 83–84%). The highest values were found for patients resident in Northern (88%; 87–88%) and Central (88%; 87–88%) Europe, followed by Ireland and United Kingdom (UK) (86%; 85–86%) and Southern Europe (83%; 82–83%). The lowest survival was in Eastern Europe (74%; 74–75%). Within regions the intercountry absolute difference in percentage points of RS varied from 4% (North) to 34% (East). RS decreased markedly with patients’ age and was higher in women than men. Differences according to SM morphology and skin sub-sites also emerged. Survival has slightly increased from 1999 to 2007, with a small improvement in Northern and the most pronounced improvement in Eastern Europe.DiscussionSM survival is high and still increasing in European patients. The gap between Northern and Southern and especially Eastern European countries, although still present, diminished over time. Differences in stage distribution at diagnosis may explain most of the geographical differences. However, part of the improvement in survival may be attributed to overdiagnosis from early diagnosis practices.     

Quality analysis of population-based information on cancer stage at diagnosis across Europe,with presentation of stage-specific cancer survival estimates: A EUROCARE-5 study

BackgroundCancer registries (CRs) are fundamental for estimating cancer burden, evaluating screening and monitoring health service performance. Stage at diagnosis—an essential information item collected by CRs—has been made available, for the first time, by CRs participating in EUROCARE-5. We analysed the quality of this information and estimated stage-specific survival across Europe for CRs with good data quality.Data and methodsSixty-two CRs sent stage (as TNM, condensed TNM or extent of disease) for 15 cancers diagnosed in 2000–2007. We assessed the quality, partly by comparing stage according to the three systems. We also developed procedures to reconstruct stage (categories: local, regional, metastatic and unknown) using information from all three systems, thus minimising the amount of missing information.ResultsModerate-to-excellent stage concordance was found for practically all 24 CRs, for which it was possible to compare at least two staging systems. However, since stage was often incorrectly assigned, and information on the presence/absence of metastases was often lacking, data on only 7/15 cancers from 34/62 CRs (15 countries) were of sufficient quality for further analysis. Cases diagnosed ≥70 years had more advanced (or lacking) stage– and worse stage-specific survival than those <70 years.ConclusionsMany European CRs collect and record reasonably accurate stage information. Others have difficulties. Both the completeness of primary data and the accuracy of stage coding need to be improved in order for CRs to fulfil their expanding roles in cancer control. We propose our stage reconstruction/checking procedures as a means of fully exploiting the stage information provided by EUROCARE CRs. More advanced (or lacking) stage at diagnosis plus poorer stage-specific survival in the elderly are worrying.     

Prognostic factors in malignant ovarian germ cell tumours (The Surveillance,Epidemiology and End Results experience 1978–2010)

PurposeTo evaluate the prognostic significance of age at diagnosis, extent of the disease (EOD) and socioeconomic (SES) and sociodemographic status (civil status, residency) on cause specific survival (CSS) in patients with malignant ovarian germ cell tumours (MOGCTs). To explore the cumulative incidence of a second cancer in 10-year MOGCT survivors.Patients and methods2541 patients with MOGCT, reported to the Surveillance, Epidemiology and End Results programme (1978–2010), were identified. The above mentioned prognostic factors were assessed separately for dysgerminoma and non-dysgerminoma, using Kaplan–Meier estimates and Cox Hazards Models, providing 95% confidence intervals (95% CI).ResultsFive-year CSS was 97% (95% CI, 96–98%), and 92% (95% CI, 91–93%), respectively for dysgerminoma, and non-dysgerminoma. Age >40 years at diagnosis and presence of metastases were significantly associated with cause specific mortality. Among non-dysgerminoma patients, decreasing SES (hazard ratio (HR), 1.59; 95% CI, 1.11–2.28) and treatment before 1990 (HR, 2.65; 95% CI, 1.83–3.85) increased mortality. In the adjusted analysis, patients from Michigan were almost 2.5 times more likely to die from MOGCT than patients from other states (HR, 2.48; 95% CI, 1.17–5.25). Second cancer was diagnosed in 10% of 10-year survivals who underwent radiotherapy and in 2% of survivals in non-radiotherapy group (p = .002).ConclusionsIncreased attention should be directed towards the management of elderly MOGCT patients and those with non-dysgerminoma histology with low SES. Radiotherapy should be avoided as much as possible. Survival differences related to residency may occur when new cancer treatments are introduced.     

Survival of 86,690 patients with thyroid cancer: A population-based study in 29 European countries from EUROCARE-5

BackgroundIncidence rates of thyroid cancer (TC) increased in several countries during the last 30 years, while mortality rates remained unchanged, raising important questions for treatment and follow-up of TC patients. This study updates population-based estimates of relative survival (RS) after TC diagnosis in Europe by sex, country, age, period and histology.MethodsData from 87 cancer registries in 29 countries were extracted from the EUROCARE-5 dataset. One- and 5-year RS were estimated using the cohort approach for 86,690 adult TC patients diagnosed in 2000–2007 and followed-up to 12/31/2008. RS trends in 1999–2007 and 10-year RS in 2005–2007 were estimated using the period approach.ResultsIn Europe 2000–2007, 5-year RS after TC was 88% in women and 81% in men. Survival rates varied by country and were strongly correlated (Pearson ρ = 75%) with country-specific incidence rates. Five-year RS decreased with age (in women from >95% at age 15–54 to 57% at age 75+), from 98% in women and 94% in men with papillary TC to 14% in women and 12% in men with anaplastic TC. Proportion of papillary TC varied by country and increased over time, while survival rates were similar across areas and periods. In 1999–2007, 5-year RS increased by five percentage points for all TCs but only by two for papillary and by four for follicular TC. Ten-year RS in 2005–2007 was 89% in women and 79% in men.ConclusionsThe reported increasing TC survival trend and differences by area are mainly explained by the varying histological case-mix of cases.     

Prognostic value of sarcopenia in adults with solid tumours: A meta-analysis and systematic review

BackgroundBody composition plays an important role in predicting treatment outcomes in adults with cancer. Using existing computed tomographic (CT) cross-sectional imaging and readily available software, the assessment of skeletal muscle mass to evaluate sarcopenia has become simplified. We performed a systematic review and meta-analysis to quantify the prognostic value of skeletal muscle index (SMI) obtained from cross-sectional CT imaging on clinical outcomes in non-haematologic solid tumours.MethodsWe searched PubMed and the American Society Clinical Oncology online database of meeting abstracts up to October 2015 for relevant studies. We included studies assessing the prognostic impact of pre-treatment SMI on clinical outcomes in patients with non-haematologic solid tumours. The primary outcome was overall survival (OS) and the secondary outcomes included cancer-specific survival (CSS), disease-free survival (DFS), and progression-free survival (PFS). The summary hazard ratio (HR) and 95% confidence interval (CI) were calculated.ResultsA total of 7843 patients from 38 studies were included. SMI lower than the cut-off was associated with poor OS (HR = 1.44, 95% CI = 1.32–1.56, p < 0.001). The effect of SMI on OS was observed among various tumour types and across disease stages. Worse CSS was also associated with low SMI (HR = 1.93, 95% CI = 1.38–2.70, p < 0.001) as well as DFS (HR = 1.16, 95% CI = 1.00–1.30, p = 0.014), but not PFS (HR = 1.54, 95% CI = 0.90–2.64, p = 0.117).ConclusionsThis meta-analysis demonstrates that low SMI at cancer diagnosis is associated with worse survival in patients with solid tumours. Further research into understanding and mitigating the negative effects of sarcopenia in adults with cancer is needed.     

PET for radiation treatment planning of brain tumours

AA-PET (MET-PET and FET-PET) can be used for gross tumour volume (GTV) delineation in brain gliomas and for the differentiation between treatment-related changes (pseudo-progression, pseudo-regression) and residual/recurrent tumour. There are some data showing that MET-PET and (DOTATOC)-PET can be used for GTV delineation in meningiomas and glomus tumours. The role of PET in the visualization of the biological characteristics of the tumours: proliferation (FLT-PET), hypoxia (FMISO-PET) and angiogenesis/peptide expression (RGD-PET) should be investigated in future studies.     

Survival patterns in teenagers and young adults with cancer in the United Kingdom: Comparisons with younger and older age groups

AimsWe aimed to describe and compare survival in teenagers and young adults (TYAs) with cancer to that of younger children and older adults, to identify sub-populations at greater or lesser risk of death.MethodsWe compared survival in cancer patients diagnosed in the United Kingdom aged 13–24 years (TYAs) to those aged 0–12 (children) and 25–49 years (adults) using the National Cancer Data Repository. All cases had a first cancer diagnosis between 1st January 2001 and 31st December 2005 with censor date 31st December 2010 or death if earlier.ResultsWe found six distinct statistically significant survival patterns. In pattern 1, the younger the age-group the better the 1- and 5-year survival (acute lymphoid leukaemia, carcinoma of ovary and melanoma). In pattern 2, TYAs had a worse 5-year survival than both children and young adults (bone and soft tissues sarcomas). In pattern 3, TYAs had a worse 1-year survival but no difference at 5-years (carcinoma of cervix and female breast). In pattern 4, TYAs had better 1-year survival than adults, but no difference at 5 years (carcinoma of liver and intrahepatic bile ducts, germ cell tumours of extra-gonadal sites). In pattern 5, the younger the age-group the better the 5-year survival, but the difference developed after 1-year (acute myeloid leukaemia, carcinoma of colon and rectum). In pattern 6, there was no difference in 1- and 5-year survival between TYAs and adults (testicular germ cell tumours, ovarian germ cell tumours and carcinoma of thyroid).ConclusionTYAs with specific cancer diagnoses can be grouped according to 1- and 5-year survival patterns compared to children and young adults. To further improve survival for TYAs, age-specific biology, pharmacology, proteomics, genomics, clinician and patient behaviour studies embedded within clinical trials are required.     

Health related quality of life in the first year after diagnosis in children with brain tumours compared with matched healthy controls; a prospective longitudinal study

This paper compares parent- and self-report health-related quality of life (HRQL) in children aged 2–16 years with brain tumours, one, six and twelve months after diagnosis with matched normal controls. HRQL was assessed using the PedsQL generic core scales. 37 tumour patients and 42 controls were included in analysis of parent-report, and 27 patients and 31 controls in self-report HRQL. Parent-report scores were significantly lower in patients than controls for all PedsQL scores at all time points (max p = 0.002). Differences in self-report PedsQL between patients and controls were variable. The relationship between self- and parent-report in patients and controls was inconsistent; varied over time; and did not consistently correlate with parental depressive symptoms, suggesting parents and their children do not regard HRQL in a similar way. Prospective, longitudinal assessment of HRQL is important, but should be supplemented with other outcome measures such as health status and behaviour in this population.     

Pediatric brain tumours at a tertiary care hospital in Karachi.

The objectives of this study were to determine the epidemiology of brain tumors during infancy and childhood and to define and segregate childhood brain tumors vis-a-vis their morphological characteristics. The present study includes pediatric brain tumors, ICD-10 category C71 encountered during 10 years (January 1989 through December 1998) at a tertiary care hospital in Karachi. Eighty one cases were included, 58 (71.6%) in males and 23 (28.4%) in females with a male to female ratio of 2.5:1. The cases were divided into 3 age groups each covering five years of life (0-4, 5-9, 10-14 years), with the greatest number in the second age group i.e. 5-9 years followed by the third age group and the 0-4 year age group. The mean age for all cases, both genders was 8.8 years (95% CI 7.9; 9.6) with a marginal variation for cases occurring in the cerebrum and cerebellum. The malignancies occurred at a younger age in the males for each subcategory by site and morphology. The morphological distribution of cases was astrocytoma (28 cases, 34.6%), primitive neuroectodermal tumor or PNET (40 cases; 49.4%), ependymoma (8 cases, 10%), mixed glioma (4 cases; 5%) and a case of oligodendroglioma. The 81 malignancies included in this study were further categorized by site into two groups, supratentorial (27 cases; 33.3%) and infratentorial (54 cases; 66.7%). The morphological categorization of supratentorial tumors was astrocytoma (17 cases; 63%), ependymoma (5 cases; 18.5%), mixed glioma (2 cases; 7.4%). PNET with rhabdoid differentiation, oligodendroglioma and pinealoblastoma comprised 1 case (3.7%) each. The 17 supratentorial astrocytoma were sub-categorized as follows - pilocytic astrocytoma (5 cases; 29.4%), grade II astrocytoma (6 cases; 35.3%); grade III astrocytoma (2 cases; 11.8%), anaplastic astrocytoma (1 case; 5.9%) and glioblastoma multiforme (3 cases; 17.7%). The morphological categorization of infratentorial tumors was astrocytoma (11 cases; 20.4%), medulloblastoma (38 cases; 70.4%), ependymoma (3 cases; 5.6%) and mixed glioma - astroependymoma (2 cases, 3.7%). The morphological sub-categorization of infratentorial astrocytoma was pilocytic astrocytoma (7 cases, 63.6%), with gemistocytic astrocytoma, grade II, grade III and anaplastic astrocytoma comprising 1 (9.1%) case each. The morphological categorization of medulloblastoma was classical medulloblastoma (15 cases; 39.5%), desmoplastic medulloblastoma (8 cases; 21.1%), medulloblastoma with astrocytic differentiation (12 cases; 31.5%), medulloblastoma with neural differentiation (2 cases; 5.3%), and neuroblastic medulloblastoma (1 case; 2.6%). The pediatric brain tumors in Karachi reflect a developing country scenario, with a strong male predisposition and a late presentation with a peak in the 5-9 year age group. There is a predominance of medulloblastoma and a paucity of astrocytomas. The current study is a single institution study and needs cautious interpretation. Population-based studies are required to determine the cancer burden due to pediatric malignancies of the brain in this population and for the morphological categorization of brain tumors in Karachi.     

Radiation‐induced brain disorders in patients with pituitary tumours

Radiation‐induced brain disorders (RIBD) are uncommon and they are grave sequelae of conventional radiotherapy. In the present report, we describe the clinical spectrum of RIBD in 11 patients who received post‐surgery conventional megavoltage irradiation for residual pituitary tumours. Of these 11 patients (nine men, two women), seven had been treated for non‐functioning pituitary tumours and four for somatotropinomas. At the time of irradiation the age of these patients ranged from 30 to 59 years (mean, 39.4 ± 8.3; median, 36) with a follow‐up period of 6?96 months (mean, 18.3 ± 26.4; median, 11). The dose of radiation ranged from 45 to 90 Gy (mean, 51.3 ± 13.4; median, 45), which was given in 15?30 fractions (mean, 18.6 ± 5.0; median, 15) with 2.8 ± 0.3 Gy (median, 3) per fraction. The biological effective dose calculated for late complications in these patients ranged from 78.7 to 180 Gy (mean, 99.1 ± 27.5; median, 90). The lag time between tumour irradiation and the onset of symptoms ranged from 6 to 168 months (mean, 46.3 ± 57.0; median, 57). The clinical spectrum of RIBD included new‐onset visual abnormalities in five, cerebral radionecrosis in the form of altered sensorium in four, generalized seizures in four, cognitive dysfunction in five, dementia in three and motor deficits in two patients. Magnetic resonance imaging (MRI)/CT of the brain was suggestive of radionecrosis in eight, cerebral oedema in three, cerebral atrophy in two and second neoplasia in one patient. Associated hormone deficiencies at presentation were hypogonadism in eight, hypoadrenalism in six, hypothyroidism in four and diabetes insipidus in one patient. Autopsy in two patients showed primitive neuroectodermal tumour (PNET) and brainstem radionecrosis in one, and a cystic lesion in the left frontal lobe following radionecrosis in the other. We conclude that RIBD have distinctive but varying clinical and radiological presentations. Diabetes insipidus and PNET as a second neoplastic disorder in adults following pituitary irradiation have not been reported previously.     

Prognoses and improvement for head and neck cancers diagnosed in Europe in early 2000s: The EUROCARE-5 population-based study

BackgroundHead and neck (H&N) cancers are a heterogeneous group of malignancies, affecting various sites, with different prognoses. The aims of this study are to analyse survival for patients with H&N cancers in relation to tumour location, to assess the change in survival between European countries, and to investigate whether survival improved over time.MethodsWe analysed about 250,000 H&N cancer cases from 86 cancer registries (CRs). Relative survival (RS) was estimated by sex, age, country and stage. We described survival time trends over 1999–2007, using the period approach. Model based survival estimates of relative excess risks (RERs) of death were also provided by country, after adjusting for sex, age and sub-site.ResultsFive-year RS was the poorest for hypopharynx (25%) and the highest for larynx (59%). Outcome was significantly better in female than in male patients. In Europe, age-standardised 5-year survival remained stable from 1999–2001 to 2005–2007 for laryngeal cancer, while it increased for all the other H&N cancers. Five-year age-standardised RS was low in Eastern countries, 47% for larynx and 28% for all the other H&N cancers combined, and high in Ireland and the United Kingdom (UK), and Northern Europe (62% and 46%). Adjustment for sub-site narrowed the difference between countries. Fifty-four percent of patients was diagnosed at advanced stage (regional or metastatic). Five-year RS for localised cases ranged between 42% (hypopharynx) and 74% (larynx).ConclusionsThis study shows survival progresses during the study period. However, slightly more than half of patients were diagnosed with regional or metastatic disease at diagnosis. Early diagnosis and timely start of treatment are crucial to reduce the European gap to further improve H&N cancers outcome.     

Treatment of metachronous colorectal cancer metastases in the Netherlands: A population-based study

BackgroundThis study aimed to describe the treatment of metachronous colorectal cancer metastases in a recent population-based cohort.MethodPatients with stage I-III colorectal cancer (CRC), diagnosed between January 1st and June 30th^, 2015 who were surgically treated with curative intent were selected from the Netherlands Cancer Registry. Follow-up was at least 3 years after diagnosis of the primary tumour. Treatment of metachronous metastases was categorized into local treatment, systemic treatment, and best supportive care. Overall survival was estimated using Kaplan-Meier method.ResultsOut of 5412 patients, 782 (14%) developed metachronous metastases, of whom 393 (50%) underwent local treatment (LT) with or without systemic therapy, 30% of patients underwent only systemic therapy (ST) and 19% only best supportive care (BSC). The most common metastatic site was the liver (51%) followed by lungs (33%) and peritoneum (22%). LT rates were 69%, 66%, and 44% for liver-only, lung-only and, peritoneal-only metastases respectively. Patients receiving LT and ST were significantly younger than patients receiving LT alone, while patients receiving BSC were significantly older than the other groups (p < 0.001). Patients with liver-only or lung-only metastases had a 3-year OS of 50.2% (43.3–56.7 95% CI) and 61.5% (50.7–70.6 95% CI) respectively. Patients with peritoneal-only disease had a lower 3-year OS, 18.1% (10.1–28.0 95% CI).ConclusionPatients with metastases confined to the liver and lung have the highest rates of local treatment for metachronous metastatic colorectal cancer. The number of patients who underwent local treatment is higher than reported in previous Dutch and international studies.     

Survival of children with malignant germ cell, trophoblastic and other gonadal tumours in Europe

Collaborators of the EUROCARE study had provided records on 1263 cases of germ cell, trophoblastic and other gonadal neoplasms, registered in 34 cancer registries in 16 European countries over the period 1978--1992 and followed-up until the end of 1994. Observed 5-year actuarial survival for 490 cases diagnosed in 1985-1989 was 80% (95% confidence interval (CI)=(76, 83)). The corresponding figures were calculated for the intracranial and intraspinal germ cell tumours (68%, 95% CI=(57, 76)), other non-gonadal germ cell tumours (76%, 95% CI=(68, 82)), gonadal germ-cell tumours (89%, 95% CI=(85, 93)) and gonadal carcinomas (50%, CI=(24, 76)). Relatively large differences in survival were observed between age-sex subgroups, which also differed with histology, with extremely poor survival of young children with intracranial and intraspinal germ cell tumours. Lower survival was observed in the countries with formerly socialist economies. Time trends in survival were examined for the entire study period, including only the cases registered in the large contributing registries. For all germ cell tumours, the risk ratios calculated in the Cox regression analysis were markedly lowered for the years after the reference period of 1978--1981. The improved outcome is attributed to treatment advances.     

Radiation therapy in the management of primary malignant lymphoma of the brain

This study reviewed 21 patients with primary malignant lymphoma of the brain. A rapid onset of symptoms was observed in most patients with a median interval of 10 weeks between first symptom and diagnosis. All received cranial irradiation following either gross total removal of tumor (9 patients), biopsy alone (1l patients) or no surgery (1 patient). Two patients received short courses of chemotherapy. Early clinical improvement was commonly observed following commencement of radiation but this was not sustained. Overall survival from diagnosis was 47 % at one year and 16 % at two years. Analysis of treatment parameters failed to indicate any well-defined factors which may have resulted in an improved survival. There was a suggestion that males fared better than females sad that irradiation of the entire brain to a minimum dose of 5000 rad was associated with longer survival.     

A Population-Based Study of Incidence and Survival of 1588 Thymic Malignancies: Results From the California Cancer Registry

BackgroundThymic malignancies are rare and there are limited contemporary population-based epidemiological studies for this uncommon cancer.Patients and MethodsAdults aged 20 years and older diagnosed with thymic malignancies between 1988 and 2015 were identified from the California Cancer Registry (n = 1588). Trends in age-adjusted incidence rates were examined overall and according to race/ethnicity, and the proportion diagnosed according to stage was evaluated over time. Cox proportional hazards regression was used to estimate hazard ratios (HRs) for overall survival (OS), and Fine and Gray competing risks regression for cause-specific survival (CSS).ResultsAge-adjusted incidence increased on average 2.08% per year over the study period (95% confidence interval [CI], 1.30%-2.86%; P < .0001), with an incidence of 0.277 cases per 100,000 in 2015. Incidence was highest among Asian/Pacific Islander and non-Hispanic black individuals. The proportion of unknown stage at diagnosis declined as localized diagnoses increased over time. Compared with patients with thymoma, those with thymic carcinoma had significantly worse OS (HR, 1.63; 95% CI, 1.33-2.01; P < .0001) and CSS (subdistribution HR, 2.99; 95% CI, 2.29-3.91; P < .0001). Advanced stage at diagnosis was also associated with worse survival. Surgical intervention was associated with better prognosis for patients with localized (HR, 0.08; 95% CI, 0.02-0.30; P = .0002) or regional disease (HR, 0.14; 95% CI, 0.06-0.34; P < .0001).ConclusionThymic malignancy incidence is increasing in California. There was incidence variation across race/ethnicity, which warrants future study. These findings provide contemporary insight into the incidence and prognostic factors of thymic malignancies.     

Outcome and predictive factors of local recurrence and distant metastases following primary surgical treatment of high-grade malignant phyllodes tumours of the breast

AIM: High-grade malignant phyllodes tumour (MPT) is a rare but aggressive breast malignancy and forms approximately 25% of all phyllodes tumours. The aim of the study was to determine parameters that influence outcome in high-grade MPT. METHODS: This study consisted of 48 women diagnosed with high-grade MPT. All patients were treated primarily with surgery by local excision (LE, margins <1 cm), wide local excision (WLE, margins > or =1 cm) or mastectomy. Cox's regression was used for multivariate analysis of the data. RESULTS: The mean patient age was 47 (range 21-85) years and the average tumour size was 7.8 (range 1.5-20) cm. Ten patients were treated with LE, 14 with WLE and 24 with mastectomy. The median follow-up was 9 years (range 5 months-28 years). Local recurrence (LR) occurred in 19 patients (40%) at mean time of 28 (range 5-84) months after primary treatment. Distant metastasis (DM) occurred in 13 (27%) patients at average time of 25.6 (range 6-120) months. LR, subsequent metastatic spread and survival following treatment of MPT were related to tumour size and excision margins, but not to other clinical or histopathological characteristics. CONCLUSION: Tumour size and surgical margins were found to be the principal determinants of LR and DM. Complete surgical excision, by mastectomy if necessary, is important in the primary surgical treatment of high-grade MPT.