Cutaneous angiosarcoma is a type of rare and locally aggressive malignancy requiring individualised treatment owing to paucity of randomised trials. We present the case of a middle-aged cancer survivor with locally advanced angiosarcoma of scalp managed with surgery, radiotherapy, chemotherapy and targeted therapy over a course of 6 years for two recurrences. The first recurrence was preceded by opsoclonus myoclonus syndrome, a type of paraneoplastic neurological syndrome (PNS), rarely reported in sarcomas. The second recurrence had a rapid clinical course, which led to a therapeutic dilemma of best supportive care versus active management. A trial of weekly paclitaxel was started that was continued for a total of 12 cycles with good objective clinical response. Presently, he is tolerating maintenance pazopanib well and is symptom free for 6 months. In cutaneous angiosarcoma patients, PNS may be a harbinger of recurrence and aggressive, multimodality treatment helps prolong survival.
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Contributors KP and ND: Conception, design of work and drafting of manuscript. DK and RK: Revision and final approval of manuscript.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Provenance and peer review Not commissioned; externally peer reviewed.
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