Journal of orthopaedic science : official journal of the Japanese Orthopaedic Association · 2025
Epidemiology and prognosis of malignant peripheral nerve sheath tumor (MPNST) in Japan: A population-based analysis using the national cancer registry.
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Muramatsu Shudai, Ogura Koichi, Morizane Chigusa, Satake Tomoyuki, Iwata Shintaro, Kobayashi Eisuke, Higashi Takahiro, Toda Yu, Takemori Toshiyuki, Kondo Hiroya, Kawai Akira
Аннотация
BACKGROUND: The national epidemiology of malignant peripheral nerve sheath tumor (MPNST) remains underexplored. We analyzed Japanese domestic statistics for MPNST using the National Cancer Registry (NCR), a population-based database launched in 2016.
METHODS: We analyzed 837 MPNST cases diagnosed between 2016 and 2019, comparing them with 22,685 non-MPNST soft tissue sarcomas. We assessed demographics, treatment, and survival using Kaplan-Meier and Cox proportional hazards models.
RESULTS: The age-adjusted incidence of MPNST in Japan was 0.13 per 100,000. Unlike other sarcomas, MPNST showed a balanced sex ratio and a relatively higher proportion of adolescent and young adult (AYA) patients (18.5 % vs. 10.7 %, p < 0.001). Most MPNSTs (87.4 %) originated from the skin and soft tissues. The 3-year survival rate for MPNST patients (53.0 %) was lower than that for other sarcomas (64.3 %, p < 0.001). Surgery was associated with longer survival (hazard ratio [HR]: 2.09; p < 0.001), while no clear benefit was observed for chemotherapy or radiotherapy. Radiotherapy was more frequently used in MPNST patients (21.1 % vs. 15.6 %, p < 0.001), likely reflecting a selection bias towards non-surgical cases.
CONCLUSIONS: This is the first study to analyze the national epidemiology and prognosis of MPNST in a socio-economically homogeneous, single ethnic group. MPNST primarily affected the skin and soft tissues, with a higher incidence in the AYA group. Surgery was associated with better outcomes, whereas chemotherapy and radiotherapy implied poorer prognosis owing to selection bias for unresectable cases. The NCR provides a valuable model for research on rare diseases in homogeneous populations.
METHODS: We analyzed 837 MPNST cases diagnosed between 2016 and 2019, comparing them with 22,685 non-MPNST soft tissue sarcomas. We assessed demographics, treatment, and survival using Kaplan-Meier and Cox proportional hazards models.
RESULTS: The age-adjusted incidence of MPNST in Japan was 0.13 per 100,000. Unlike other sarcomas, MPNST showed a balanced sex ratio and a relatively higher proportion of adolescent and young adult (AYA) patients (18.5 % vs. 10.7 %, p < 0.001). Most MPNSTs (87.4 %) originated from the skin and soft tissues. The 3-year survival rate for MPNST patients (53.0 %) was lower than that for other sarcomas (64.3 %, p < 0.001). Surgery was associated with longer survival (hazard ratio [HR]: 2.09; p < 0.001), while no clear benefit was observed for chemotherapy or radiotherapy. Radiotherapy was more frequently used in MPNST patients (21.1 % vs. 15.6 %, p < 0.001), likely reflecting a selection bias towards non-surgical cases.
CONCLUSIONS: This is the first study to analyze the national epidemiology and prognosis of MPNST in a socio-economically homogeneous, single ethnic group. MPNST primarily affected the skin and soft tissues, with a higher incidence in the AYA group. Surgery was associated with better outcomes, whereas chemotherapy and radiotherapy implied poorer prognosis owing to selection bias for unresectable cases. The NCR provides a valuable model for research on rare diseases in homogeneous populations.