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Secondary Publication of Japanese Dermatological Association Guidelines: Clinical Questions of Guidelines for
Yasuhiro Fujisawa1, Yasuo Yoshioka2, Koji Yoshino3
1Department of Dermatology, Ehime University, Toon, Ehime, Japan.
Abstract:
Cutaneous angiosarcoma is a rare aggressive malignancy with poor prognosis. Due to its rarity, high-level evidence from randomized controlled trials is limited, and treatment strategies have historically been adapted from other sarcomas. These guidelines aim to provide updated recommendations based on newly available evidence to standardize clinical practice in Japan. The 2024 revision was conducted under the Japanese Dermatological Association's commission, incorporating expert reviews and public comments. Given the lack of an established staging system, recommendations were formulated through systematic literature reviews and a structured consensus process. Five clinical questions were addressed, covering first-line chemoradiotherapy (CRT), management of residual lesions post-CRT, second-line treatment options, the role of pembrolizumab in tumor mutational burden-high cases, and treatment strategies for nonhead-and-neck angiosarcomas. Key recommendations include weakly recommending CRT for large (≥ 5 cm) nonmetastatic tumors, preferring drug modification over excision for residual lesions after CRT, and equally considering docetaxel, pazopanib, or eribulin for paclitaxel-resistant cases. Pembrolizumab was weakly recommended for tumor mutational burden-high cases. For radiation-associated angiosarcoma, surgical treatment was favored over CRT, while Stewart-Treves syndrome cases were treated similarly to head-and-neck angiosarcoma. Future directions emphasize the need for multicenter registry studies and prospective trials to refine treatment strategies. As advances in genomic medicine and immunotherapy evolve, guideline updates will be essential to ensure optimal patient care.
Insights
Updated guidelines recommend chemoradiotherapy (CRT) for large cutaneous angiosarcomas and suggest specific chemotherapies for resistant cases. Pembrolizumab is recommended for high tumor mutational burden, guiding clinical practice in Japan.
Area of Science:
- Dermatology
- Oncology
- Medical Guidelines
Background:
- Cutaneous angiosarcoma is a rare, aggressive skin cancer with limited high-level evidence for treatment.
- Treatment strategies often rely on adaptations from other sarcoma types.
- The rarity of cutaneous angiosarcoma hinders randomized controlled trials.
Purpose of the Study:
- To provide updated, evidence-based recommendations for cutaneous angiosarcoma treatment in Japan.
- To standardize clinical practice for this rare malignancy.
- To address key clinical questions regarding first-line, second-line, and specific subgroup treatments.
Main Methods:
- Systematic literature reviews were conducted.
- A structured consensus process involving expert review and public comments was employed.
- Recommendations were formulated based on available evidence due to the lack of a staging system.
Main Results:
- Chemoradiotherapy (CRT) is weakly recommended for large (≥5 cm) nonmetastatic cutaneous angiosarcomas.
- For residual lesions post-CRT, drug modification is preferred over excision.
- Docetaxel, pazopanib, or eribulin are equally considered for paclitaxel-resistant cases; pembrolizumab is weakly recommended for tumor mutational burden-high cases.
Conclusions:
- The guidelines offer standardized treatment strategies for cutaneous angiosarcoma in Japan.
- Specific recommendations address chemoradiotherapy, residual lesion management, and targeted therapies like pembrolizumab.
- Future research should focus on multicenter studies and prospective trials to further refine treatment protocols.
