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Angiosarcomas (ASs) are rare malignant vascular tumours.

They often arises as a cutaneous disease in the scalp and the face, only occasionally occur in the spine. The prognosis is generally poor due to rarity of the condition as well as lack of data of treatment options.

Systematic review

Angiosarcoma-related cerebral metastases have only been recorded in a few case reports and case series and have not been systematically reviewed to date. Our objective was therefore to perform a systematic literature review on cases of angiosarcomas metastasizing to the brain to inform current practice. All three major libraries-PubMed/MEDLINE, Embase, and Cochrane-were systematically searched, until January 2019. Articles in English reporting angiosarcoma-related cerebral metastases via hematogenous route were included. Our search yielded 45 articles (38 case reports, 5 retrospective studies, 1 case series and 1 letter to the editor), totaling 48 patients (mean age 47.9 years). The main primary site was the heart. The mean time of diagnosis of cerebral metastases following primary tumor identification was 4.9 months. In 15 cases, the brain was the only metastatic site. In cases of multiple extracerebral metastases, the most common sites were the lung and bone. Acute intracerebral supratentorial hemorrhage was the most common presenting radiological feature. Treatment strategies were almost equally divided between the surgical (with or without adjuvant treatment) and the medical arm. Mean overall survival was 7.2 months while progression-free survival was 1.5 months. To our knowledge, this is the first systematic literature review on angiosarcoma-related cerebral metastases. This pathology proves to be an extremely rare clinical entity and carries a poor prognosis, and no consensus has been reached regarding treatment 1).

Case reports

A 20-year-old male with a painful right postauricular mass following a closed head injury. Radiologic studies demonstrated a large right osteolytic and heterogeneously enhancing mass. The patient underwent right transpetrosal craniectomy for resection. Histologic studies confirmed high-grade sarcoma. Immunohistochemical stains demonstrated uniformly positive ERG endothelial marker, CD31 stain with cytoplasmic and membranous patterns of immunopositivity, positive nuclear staining for FLI-1, positive cytoplasmic and membranous staining for CD99 and STAT6, and negative SMA stains in the neoplastic cells. The Ki-67 staining showed approximately 94% positivity in the neoplastic cell nuclei. Post-surgical follow-up imaging demonstrated evidence of metastatic right cervical lymphadenopathy.

Angiosarcoma of the temporal bone is extremely uncommon. In this case report, we explore a relationship between trauma and angiosarcoma of the temporal bone. We reviewed the literature regarding the pathogenesis, diagnosis, treatment, radiologic findings, and histologic characteristics of angiosarcoma of the temporal bone 2).

An angiosarcoma patient with brain abscess, and it might be responsible for skin defect and cranial bone necrosis by surgical excision and radiation. Our patient was treated with 10 courses of triweekly paclitaxel therapy, radical radiotherapy (70 Gy), and surgical excision (2 cm margin apart from a lesion) for angiosarcoma. At two years after the operation he was diagnosed as brain abscess. Brain abscess was managed with antibiotic drugs and drainage, his clinical symptoms improved by these treatments. He achieves replace free survival without the exacerbation of angiosarcoma and brain abscess for three years 3).

A very rare case of skull angiosarcoma associated with a calcified chronic subdural hematoma is presented. An asymptomatic subdural hematoma with an atypical history and radiological features should prompt further investigation. Contrast MRI images should to be obtained early to differentiate a subdural hematoma from other pathologies 4).

A 72-year-old woman, who had visited our hospital with gait disorder and progressive consciousness disturbance approximately 3 months after a minor head injury. Initially, on reviewing the results of imaging studies, she was diagnosed with traumatic chronic SDH. Despite repeated operations thereafter, including the embolization of the middle meningeal artery, her general condition progressively worsened, and head computed tomography results repeatedly showed the recurrence of SDH. Based on histopathological and intraoperative findings, she was finally diagnosed with angiosarcoma originating from the skull. She died shortly thereafter because of aggressive recurrent intracranial SDH caused by leptomeningeal dissemination.

In addition to cancers metastatic to the skull or dura mater, angiosarcoma should be included in the differential diagnosis for patients with repeated SDH and bone defect. An effective treatment for angiosarcoma with SDH which show unfavorable prognosis has not been established; however, an early diagnosis might be useful for a novel treatment 5).

Pülhorn et al., present the case of a 75-year old man with a primary angiosarcoma of C2 and C3 who underwent occipito-cervical (to C6) fixation. A first biopsy did not result in a diagnosis and a further anterior approach with repeat biopsy had to be undertaken. The patient received adjuvant radiotherapy and at 6-month follow-up there was no radiological progression of the angiosarcoma. ASs are a rare condition and due to paucity of data relating to management cases should be reported to aid understanding and development of guidelines for diagnosis and treatment 6).


Drosos E, Kalyvas A, Komaitis S, Skandalakis GP, Kalamatianos T, Liouta E, Neromyliotis E, Alexiou GA, Stranjalis G, Koutsarnakis C. Angiosarcoma-related cerebral metastases: a systematic review of the literature. Neurosurg Rev. 2019 Jun 4. doi: 10.1007/s10143-019-01127-y. [Epub ahead of print] Review. PubMed PMID: 31165296.
Bernstock JD, Shafaat O, Hardigan A, Fox BM, Moore LS, Chagoya G, Elsayed G, Gessler F, Ilyas A, Yamashita D, McGrew B, Fisher WS 3rd, Hackney J, Sotoudeh H. Angiosarcoma of the temporal bone: case report and review of the literature. World Neurosurg. 2019 Jul 19. pii: S1878-8750(19)32013-3. doi: 10.1016/j.wneu.2019.07.107. [Epub ahead of print] PubMed PMID: 31330332.
Kuriyama H, Kajihara I, Kanemaru H, Nishimura Y, Igata T, Masuguchi S, Imaoka Y, Makino K, Ihn H. Brain abscess in an angiosarcoma patient during a disease-free interval. Drug Discov Ther. 2019;13(3):175-176. doi: 10.5582/ddt.2019.01042. PubMed PMID: 31327792.
Qin G, Xu P, Lan S, Mao Y, Huang Y, Ye J, Liang Y. Skull angiosarcoma associated with a calcified chronic subdural hematoma. Br J Neurosurg. 2019 Apr 24:1-4. doi: 10.1080/02688697.2019.1594693. [Epub ahead of print] PubMed PMID: 31014116.
Kanazawa T, Miwa T, Akiyama T, Ohara K, Kosugi K, Nishimoto M, Yoshida K. A case of aggressive recurrent intracranial subdural hematoma associated with angiosarcoma originating from the skull. World Neurosurg. 2019 Mar 9. pii: S1878-8750(19)30591-1. doi: 10.1016/j.wneu.2019.02.168. [Epub ahead of print] PubMed PMID: 30862578.
Pülhorn H, Elliot T, Clark J, Gonzalvo A. Case report: Angiosarcoma of the cervical spine. J Clin Neurosci. 2017 Aug 1. pii: S0967-5868(17)30334-X. doi: 10.1016/j.jocn.2017.07.018. [Epub ahead of print] PubMed PMID: 28778802.
angiosarcoma.txt · Last modified: 2019/07/23 20:41 by administrador