Soft-tissue sarcoma in Japan: National Cancer Registry-based analysis from 2016 to 2019.

BACKGROUND: No previous reports have characterized national profiles of soft-tissue sarcoma overall. We examined the nationwide statistics for soft-tissue sarcoma in Japan using data from the population-based National Cancer Registry. METHODS: We identified 23 522 soft-tissue-sarcoma patients who were entered in the National Cancer Registry during 2016-19 using International Classification of Diseases-Oncology, Third Edition codes for cancer topography and morphology. We extracted data on patient demographics, tumor details (reason for diagnosis, tumor location, histology, extent of disease), hospital volume/type, treatment, and prognosis for each patient. RESULTS: Soft-tissue sarcoma showed a slight male preponderance. Approximately 5500-6000 new cases were diagnosed as soft-tissue sarcoma per year, with the age-adjusted incidence of soft-tissue sarcoma being 3.22/100000/year. The age distribution showed a single peak in the 70-79 age range, and sex-stratified data showed it was higher in men. The most common histologic subtype was liposarcoma. The most frequent tumor locations were the soft tissue and skin, followed by the retroperitoneum. Extent of disease was categorized as: "localized" (31.3%), "regional" (38.9%), or "distant" (10.5%). We found significant associations between overall survival and sex, age, tumor location, facility type, hospital volume, reason for diagnosis, extent of disease, and surgical treatment. CONCLUSIONS: This is the first study to outline the epidemiology, clinical features, treatment, prognosis, and significant factors affecting prognosis of soft-tissue sarcoma in Japan using the National Cancer Registry. Documenting our data regarding elderly patients' outcomes is essential so other countries showing similar population-aging trends can learn from our experiences. LEVEL OF EVIDENCE: Prognostic studies, Level III.

Rare cancers in Japan: definition, clinical features and future perspectives.

This review introduces the definition, epidemiology and therapeutic challenges of rare cancers and describes the establishment of the Rare Cancer Center at the National Cancer Center, Japan. Rare cancers are defined as malignant tumors with an incidence rate of less than 6 cases per 100 000 individuals. Due to their low incidence rate, medical treatment for rare cancers is more challenging than for more common cancer types. Specifically, 190 types of cancers, including bone and soft tissue sarcomas, gastrointestinal stromal tumors (GISTs), neuroendocrine tumors and gliomas, are classified as rare cancers. Individually, each of the rare cancers accounts for less than 1% of all cancers, but collectively they account for 15% of all cancers. On the basis of their medical management, rare cancers can be subclassified into two types: Type I (rare cancers within cancer-rare organs) and Type II (rare cancers within cancer-common organs). Most importantly, the outcomes for rare cancers are poorer compared to those of common cancers. In 2014, the Rare Cancer Center was established at the National Cancer Center to address the various challenges related to rare cancers. The Rare Cancer Center has adopted a multifaceted approach for overcoming these challenges, including active sharing of information through a dedicated website and an online seminar series 'Rare Cancer Meet the Expert', providing medical support through telephone consultations via a 'Rare Cancer Hotline', supporting basic research and establishing the 'MASTER KEY Project' aimed at developing new treatments.

[Sarcoma].

Challenges in the treatment of sarcoma as a rare cancer include(1)inexperience of sarcoma physicians due to the small number of patients,(2)insufficient information due to the lack of evidence,(3)lack of opportunities to provide information to non-specialists and to educate young physicians,(4)low adherence to standard treatments presented in clinical practice guidelines,(5)insufficient number of researchers engaged in basic research on rare cancers and their research funds, and(6) low number of clinical trials for the development of orphan drugs. This paper describes the current status and future prospects for the centralization and networking of sarcoma treatment, problems in pathological diagnosis, clinical evidence creation, information provision and human resource development, and information dissemination and consultation support to sarcoma patients.

Serum beta2-microglobulin level is a significant predictor of mortality in maintenance haemodialysis patients.

BACKGROUND: Beta(2)-microglobulin (beta(2)-M) is recognized as a surrogate marker of middle-molecule uraemic toxins and is a key component in the genesis of dialysis-associated amyloidosis. Few studies have evaluated the association of beta(2)-M levels with clinical outcome in dialyzed patients. METHODS: The prognostic implication of serum beta(2)-M levels for the survival of haemodialysis patients was examined in 490 prevalent haemodialysis patients (60.1 +/- 11.8 years, haemodialysis duration of 87.4 +/- 75.7 months, 288 males and 202 females; 24% diabetics). The patients were divided into two groups according to their serum beta(2)-M levels: lower beta(2)-M group (n = 245) with serum beta(2)-M <32.2 mg/L (the median serum beta(2)-M) and higher beta(2)-M group (n = 245) with that >or=32.2 mg/L. RESULTS: During the follow-up period of 40 +/- 15 months, there were 91 all-cause deaths, and out of them, 36 were from cardiovascular diseases. Kaplan-Meier analysis revealed that all-cause mortality in the higher beta(2)-M group was significantly higher compared to that in the lower beta(2)-M group (P < 0.001). Multivariate Cox proportional hazards analyses showed that serum beta(2)-M level was a significant predictor for all-cause mortality (hazard ratio, 1.05; 95% CI, 1.01-1.08; P = 0.005), and for non-cardiovascular mortality (hazard ratio, 1.06; 95% CI, 1.02-1.10; P = 0.006), after adjustment for age, gender, haemodialysis duration, the presence of diabetes, serum albumin and serum C-reactive protein. CONCLUSION: These results demonstrate that the serum beta(2)-M level is a significant predictor of mortality in haemodialysis patients, independent of haemodialysis duration, diabetes, malnutrition and chronic inflammation, suggesting the clinical importance of lowering serum beta(2)-M in these patients.