Recurrent angiosarcoma of scalp with opsoclonus myoclonus syndrome: role of salvage treatment

Abstract

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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“…175 Some evidence exists that pazopanib may reveal increased effectiveness in cutaneous AS cases, but usually in multimodal therapy. 177 Similar results arise from the SPIRE study, where pazopanib was administered to patients with advanced STSs. In ASs subtype, a partial response was seen in 38% of cases, and 13% of all patients discontinued the drug due to serious AEs.…”

“…Pazopanib demonstrated its activity in randomized, placebo‐controlled, phase III trial, showing a significant improvement in progression‐free survival and a favourable trend in OST in patients with STS who previously received anthracycline or ifosfamide based chemotherapy 175 . Some evidence exists that pazopanib may reveal increased effectiveness in cutaneous AS cases, but usually in multimodal therapy 177 . Similar results arise from the SPIRE study, where pazopanib was administered to patients with advanced STSs.…”