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

BMJ Case Rep. 2021 Jun 25;14(6):e241824. doi: 10.1136/bcr-2021-241824.


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.

Keywords: chemotherapy; dermatology; neurology; oncology; radiotherapy.

Publication types

  • Case Reports

MeSH terms

  • Hemangiosarcoma*
  • Humans
  • Male
  • Middle Aged
  • Neoplasm Recurrence, Local
  • Opsoclonus-Myoclonus Syndrome*
  • Salvage Therapy
  • Scalp