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Cardiology271 papers

Angiosarcoma

Last edited: 4/14/2026

Overview

Angiosarcoma is a rare, aggressive malignancy arising from endothelial cells, commonly affecting the skin, liver, and other visceral organs. It presents with poor prognosis due to its aggressive nature and late diagnosis 13.

Diagnosis

  • Clinical Presentation: Often presents with mass lesions, pain, and systemic symptoms depending on location 15810131619.
  • Imaging: CT, MRI, and ultrasound useful for initial assessment and localization 15919.
  • Biopsy and Pathology: Essential for definitive diagnosis; immunohistochemistry aids in distinguishing from other vascular tumors 3111518.
  • Molecular Studies: Emerging evidence suggests molecular profiling can provide insights into distinct subtypes 23.

    • Management

  • Surgical Resection: Primary treatment when feasible, aiming for complete excision 137.
  • Adjuvant Therapy: Chemotherapy and radiotherapy considered for advanced or unresectable cases, though efficacy is limited 147.
  • Electrochemotherapy (ECT): Emerging as a potential adjunct for locally advanced or metastatic disease 7.
  • Targeted Therapies: Ongoing research suggests potential benefits from targeted molecular therapies based on specific molecular underpinnings 2.

    • Special Populations

  • Occupational Exposure: Workers exposed to vinyl chloride should undergo regular liver function tests due to risk of hepatic angiosarcoma 2122.
  • Elderly and Comorbidities: Management complexity increases with comorbidities; individualized treatment plans are crucial 1510.

    • Key Recommendations

  • Early Diagnosis and Surgical Resection: Prioritize early detection and radical surgical excision when possible for optimal outcomes (Evidence: Moderate 13).
  • Consider Electrochemotherapy for Advanced Cases: Electrochemotherapy can be considered as an adjunctive treatment for locally advanced or metastatic cutaneous angiosarcoma (Evidence: Weak 7).
  • Monitor Vinyl Chloride Exposed Workers: Regular medical evaluations, particularly liver function tests, are recommended for individuals with occupational exposure to vinyl chloride (Evidence: Expert opinion 2122).

    • References

    1 Fujisawa Y, Yoshioka Y, Yoshino K, Fujimura T, Naito Y, Masuzawa M et al.. Secondary Publication of Japanese Dermatological Association Guidelines: Clinical Questions of Guidelines for Cutaneous Angiosarcoma 2025. The Journal of dermatology 2025. link 2 . Initiative Suggests Angiosarcoma Therapies. Cancer discovery 2020. link 3 Ronchi A, Cozzolino I, Zito Marino F, De Chiara A, Argenziano G, Moscarella E et al.. Primary and secondary cutaneous angiosarcoma: Distinctive clinical, pathological and molecular features. Annals of diagnostic pathology 2020. link 4 Kazaz IO, Ersoz S, Colak F, Teoman AS, Kazaz SN, Karaguzel E et al.. Primary renal angiosarcoma: A case report and a short review of literature. Indian journal of pathology & microbiology 2020. link 5 Aihara S, Saito S, Oka H, Kamimura T, Yamaoka T, Kajiwara K et al.. Axillary arterial angiosarcoma in a nonfunctioning arteriovenous fistula limb of a patient undergoing hemodialysis: case report with literature review. CEN case reports 2020. link 6 Huang W, Liu T, Duan R, Yuan Y, Qu M, Zhang M et al.. Primary multiple angiosarcoma of vertebra: A case report. Medicine 2020. link 7 Campana LG, Kis E, Bottyán K, Orlando A, de Terlizzi F, Mitsala G et al.. Electrochemotherapy for advanced cutaneous angiosarcoma: A European register-based cohort study from the International Network for Sharing Practices of electrochemotherapy (InspECT). International journal of surgery (London, England) 2019. link 8 Chen CY, Wu YC, Chou TY, Yang KY. Pleural angiosarcoma mimicking pleural haematoma. Interactive cardiovascular and thoracic surgery 2013. link 9 Nakajima H, Kobayashi J, Matsuda H, Ishibashi-Ueda H. A primary angiosarcoma in the aorta. Interactive cardiovascular and thoracic surgery 2007. link 10 Schuetz B. Cutaneous angiosarcoma. Dermatology nursing 2002. link 11 Saleh HA, Tao LC. Hepatic angiosarcoma: aspiration biopsy cytology and immunocytochemical contribution. Diagnostic cytopathology 1998. link1097-0339(199803)18:3<208::aid-dc9>3.0.co;2-9) 12 Oriel JD. Moritz Kaposi (1837-1902). International journal of STD & AIDS 1997. link 13 McCleary AJ. Massive haemothorax secondary to angiosarcoma. Thorax 1994. link 14 Huerter CJ, Kunkel JR, Rouse JR. Angiosarcoma of the face and scalp. Cutis 1993. link 15 Chan KW. Angiosarcoma of the prostate. An immunohistochemical study of a case. Pathology 1990. link 16 Cheesman MG, Webster J, Rowlands D. Pericardial obliteration by angiosarcoma. British heart journal 1988. link 17 Palexas GN, Soicher ER, Sacks R, Sofianos C. Angiosarcoma of the breast. A case report. South African medical journal = Suid-Afrikaanse tydskrif vir geneeskunde 1988. link 18 Zachariades N, Economopoulou P. Maxillary angiosarcoma. International journal of oral and maxillofacial surgery 1986. link80101-6) 19 Vasile N, Lardé D, Zafrani ES, Berard H, Mathieu D. Hepatic angiosarcoma. Journal of computer assisted tomography 1983. link 20 Leoni A, Cogo R, Visonà A. Angiosarcoma of the scalp-face. Report of a case with fulminating course. Dermatologica 1983. link 21 Migliori E. Angiosarcoma of the breast. Tumori 1977. link 22 Aryanpur J. Vinyl chloride: its impact on occupational medicine practice in Iran. Journal of occupational medicine. : official publication of the Industrial Medical Association 1977. link 23 Ott MG, Langer RR, Holder BB. Vinyl chloride exposure in a controlled industrial environment. A long-term mortality experience in 594 employees. Archives of environmental health 1975. link

    Original source

    1. [1]
      Secondary Publication of Japanese Dermatological Association Guidelines: Clinical Questions of Guidelines for Cutaneous Angiosarcoma 2025. — Fujisawa Y, Yoshioka Y, Yoshino K, Fujimura T, Naito Y, Masuzawa M et al. The Journal of dermatology (2025)
    2. [2]
      Initiative Suggests Angiosarcoma Therapies. Cancer discovery (2020)
    3. [3]
      Primary and secondary cutaneous angiosarcoma: Distinctive clinical, pathological and molecular features. — Ronchi A, Cozzolino I, Zito Marino F, De Chiara A, Argenziano G, Moscarella E et al. Annals of diagnostic pathology (2020)
    4. [4]
      Primary renal angiosarcoma: A case report and a short review of literature. — Kazaz IO, Ersoz S, Colak F, Teoman AS, Kazaz SN, Karaguzel E et al. Indian journal of pathology & microbiology (2020)
    5. [5]
      Axillary arterial angiosarcoma in a nonfunctioning arteriovenous fistula limb of a patient undergoing hemodialysis: case report with literature review. — Aihara S, Saito S, Oka H, Kamimura T, Yamaoka T, Kajiwara K et al. CEN case reports (2020)
    6. [6]
      Primary multiple angiosarcoma of vertebra: A case report. — Huang W, Liu T, Duan R, Yuan Y, Qu M, Zhang M et al. Medicine (2020)
    7. [7]
      Electrochemotherapy for advanced cutaneous angiosarcoma: A European register-based cohort study from the International Network for Sharing Practices of electrochemotherapy (InspECT). — Campana LG, Kis E, Bottyán K, Orlando A, de Terlizzi F, Mitsala G et al. International journal of surgery (London, England) (2019)
    8. [8]
      Pleural angiosarcoma mimicking pleural haematoma. — Chen CY, Wu YC, Chou TY, Yang KY Interactive cardiovascular and thoracic surgery (2013)
    9. [9]
      A primary angiosarcoma in the aorta. — Nakajima H, Kobayashi J, Matsuda H, Ishibashi-Ueda H Interactive cardiovascular and thoracic surgery (2007)
    10. [10]
      Cutaneous angiosarcoma. — Schuetz B Dermatology nursing (2002)
    11. [11]
      Hepatic angiosarcoma: aspiration biopsy cytology and immunocytochemical contribution. — Saleh HA, Tao LC Diagnostic cytopathology (1998)
    12. [12]
      Moritz Kaposi (1837-1902). — Oriel JD International journal of STD & AIDS (1997)
    13. [13]
      Massive haemothorax secondary to angiosarcoma. — McCleary AJ Thorax (1994)
    14. [14]
      Angiosarcoma of the face and scalp. — Huerter CJ, Kunkel JR, Rouse JR Cutis (1993)
    15. [15]
      Angiosarcoma of the prostate. An immunohistochemical study of a case. — Chan KW Pathology (1990)
    16. [16]
      Pericardial obliteration by angiosarcoma. — Cheesman MG, Webster J, Rowlands D British heart journal (1988)
    17. [17]
      Angiosarcoma of the breast. A case report. — Palexas GN, Soicher ER, Sacks R, Sofianos C South African medical journal = Suid-Afrikaanse tydskrif vir geneeskunde (1988)
    18. [18]
      Maxillary angiosarcoma. — Zachariades N, Economopoulou P International journal of oral and maxillofacial surgery (1986)
    19. [19]
      Hepatic angiosarcoma. — Vasile N, Lardé D, Zafrani ES, Berard H, Mathieu D Journal of computer assisted tomography (1983)
    20. [20]
      Angiosarcoma of the scalp-face. Report of a case with fulminating course. — Leoni A, Cogo R, Visonà A Dermatologica (1983)
    21. [21]
      Angiosarcoma of the breast. — Migliori E Tumori (1977)
    22. [22]
      Vinyl chloride: its impact on occupational medicine practice in Iran. — Aryanpur J Journal of occupational medicine. : official publication of the Industrial Medical Association (1977)
    23. [23]
      Vinyl chloride exposure in a controlled industrial environment. A long-term mortality experience in 594 employees. — Ott MG, Langer RR, Holder BB Archives of environmental health (1975)
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