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<p>Angiosarcomas are extremely rare, highly malignant neoplasms of vascular origin, commonly seen in the dermis, soft tissue, or vessel-rich organs. Head and neck angiosarcomas are only 2.6% with only 8 reported cases of primary temporal bone angiosarcoma. Reporting here a case of a 22-year-old female patient diagnosed with left temporal bone angiosarcoma. Patient presented with a mass in the left ear canal and swelling in the post-auricular region for 15 days, CE- HRCT scan temporal bone showed a large heterogenous expansile peripherally enhancing aggressive soft tissue density lesion centered in the left mastoid region; intracranial, extradural extension with bony erosion up to posterior part of petrous bone. Biopsy confirmed the diagnosis of primary angiosarcoma of left temporal bone. Patient initially improved on chemotherapy (6 cycles of gemcitabine and docetaxel) but had a recurrence on 3-month follow-up. Hence, was given 15 cycles of high-dose palliation therapy (45&#xa0;Gy), followed by radiation therapy (60&#xa0;Gy), improved symptomatically with cessation of progression, currently still on monthly follow-up. Considering the rarity of the diagnosis and its symptom overlap with the more commonly seen chronic otitis media, this report will assist clinicians in reaching an accurate diagnosis and formulating an effective and pragmatic management plan.</p>

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A Rare Case Report of Primary Angiosarcoma of Temporal Bone

  • 期刊论文

  • 发表日期：

    2025年10月24日

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  • Zohda Tayyaba,
  • Varsha Rao,
  • Sana Ansari,
  • Sabeeh Beig,
  • Md Aftab

摘要

Angiosarcomas are extremely rare, highly malignant neoplasms of vascular origin, commonly seen in the dermis, soft tissue, or vessel-rich organs. Head and neck angiosarcomas are only 2.6% with only 8 reported cases of primary temporal bone angiosarcoma. Reporting here a case of a 22-year-old female patient diagnosed with left temporal bone angiosarcoma. Patient presented with a mass in the left ear canal and swelling in the post-auricular region for 15 days, CE- HRCT scan temporal bone showed a large heterogenous expansile peripherally enhancing aggressive soft tissue density lesion centered in the left mastoid region; intracranial, extradural extension with bony erosion up to posterior part of petrous bone. Biopsy confirmed the diagnosis of primary angiosarcoma of left temporal bone. Patient initially improved on chemotherapy (6 cycles of gemcitabine and docetaxel) but had a recurrence on 3-month follow-up. Hence, was given 15 cycles of high-dose palliation therapy (45 Gy), followed by radiation therapy (60 Gy), improved symptomatically with cessation of progression, currently still on monthly follow-up. Considering the rarity of the diagnosis and its symptom overlap with the more commonly seen chronic otitis media, this report will assist clinicians in reaching an accurate diagnosis and formulating an effective and pragmatic management plan.