Angiosarcoma

Jump to navigation Jump to search

WikiDoc Resources for Angiosarcoma

Articles

Most recent articles on Angiosarcoma

Most cited articles on Angiosarcoma

Review articles on Angiosarcoma

Articles on Angiosarcoma in N Eng J Med, Lancet, BMJ

Media

Powerpoint slides on Angiosarcoma

Images of Angiosarcoma

Photos of Angiosarcoma

Podcasts & MP3s on Angiosarcoma

Videos on Angiosarcoma

Evidence Based Medicine

Cochrane Collaboration on Angiosarcoma

Bandolier on Angiosarcoma

TRIP on Angiosarcoma

Clinical Trials

Ongoing Trials on Angiosarcoma at Clinical Trials.gov

Trial results on Angiosarcoma

Clinical Trials on Angiosarcoma at Google

Guidelines / Policies / Govt

US National Guidelines Clearinghouse on Angiosarcoma

NICE Guidance on Angiosarcoma

NHS PRODIGY Guidance

FDA on Angiosarcoma

CDC on Angiosarcoma

Books

Books on Angiosarcoma

News

Angiosarcoma in the news

Be alerted to news on Angiosarcoma

News trends on Angiosarcoma

Commentary

Blogs on Angiosarcoma

Definitions

Definitions of Angiosarcoma

Patient Resources / Community

Patient resources on Angiosarcoma

Discussion groups on Angiosarcoma

Patient Handouts on Angiosarcoma

Directions to Hospitals Treating Angiosarcoma

Risk calculators and risk factors for Angiosarcoma

Healthcare Provider Resources

Symptoms of Angiosarcoma

Causes & Risk Factors for Angiosarcoma

Diagnostic studies for Angiosarcoma

Treatment of Angiosarcoma

Continuing Medical Education (CME)

CME Programs on Angiosarcoma

International

Angiosarcoma en Espanol

Angiosarcoma en Francais

Business

Angiosarcoma in the Marketplace

Patents on Angiosarcoma

Experimental / Informatics

List of terms related to Angiosarcoma

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Shyam Patel [2]; Associate Editor(s)-in-Chief: Mohsen Basiri M.D.
Synonyms and keywords: Hemangiosarcoma; Pulmonary angiosarcoma; Vascular sarcoma

Overview

Angiosarcoma is a rare malignant vascular neoplasm of endothelial-type cells that line vessel walls. The peak age of incidence appears to be the 7th decade, and men are affected more commonly than women. Angiosarcoma was first described by Dr. Juan Rosai, in 1976. The pathogenesis of angiosarcoma is characterized by a rapid and extensively infiltrating overgrowth of vascular endothelial cells. Angiosarcoma may arise in any part of the body, but is more common in soft tissues than in bones. Common angiosarcoma locations include the head and neck area, kidney, liver, lung, and and the most common site of radiation-induced angiosarcoma development is the breast. The PTPRB/PLCG1 genes are associated with the development of angiosarcoma; mutation of these genes result in aberrant angiogenesis. The imaging modality of choice for diagnosing angiosarcoma will depend on the location. For pulmonary angiosarcoma, the imaging modality of choice is CT scan with intravenous contrast. For other types angiosarcoma, the imaging modality of choice is MRI. On CT scan, findings suggestive of angiosarcoma may include vascular invasion, nodular enhancement (common), and a hypoattenuating mass. The mainstay adjuvant therapy for angiosarcoma is a doxorubicin-based regimen. The response rate for chemotherapy in patients with angiosarcoma is poor.

Historical Perspective

  • Angiosarcoma was first discovered by Dr. Juan Rosai, M.D. and colleagues in 1976.[1]

Classification

  • Angiosarcoma may be classified according to the clinical heterogeneity into two main groups, and every group can be subdivided into subtypes according to the anatomical location and etiology:[2][3][4]
Angiosarcoma
Primary Secondary
Cutaneous Post Radiation Angiosarcoma
Breast Lymphedema-associated Angiosarcoma
Soft tissue and Bone Angiosarcoma due to exposure to mutatgens
Visceral -

Pathophysiology

Image courtesy of Yale Rosen, contributed by Wikimedia commons

Causes

Differentiating Angiosarcoma from Other Diseases

Epidemiology and Demographics

  • The incidence of angiosarcomas can be calculated approximately 1.2 per 1,000.000 person.[5][18]
  • Angiosarcoma is more commonly observed among patients aged between 40 to 75 years old.The peak age of incidence appears is the 7th decade,[19]
  • Males are more commonly affected with angiosarcoma than females.[19]
  • The male to female ratio is 2:1.[19]
  • There is no racial predilection for angiosarcoma. however, African-Americans in the U.S are rarely affected.[20]

Risk Factors

Common risk factors in the development of angiosarcoma include chronic lymphedema, chronic exposure to polyvinyl chloride (PVC), radiation exposure, and exposure to Thorotrast.[5]

Natural History, Complications and Prognosis

Diagnosis

Diagnostic Study of Choice

Staging

Stage Grade Site Metastasis
Ia Low grade (G1) Intracompartmental (T1) No metastasis (M0)
Ib Low grade (G1) Extracompartmental (T2) No metastasis (M0)
IIa High grade (G2) Intracompartmental (T1) No metastasis (M0)
IIb High grade (G2) Extracompartmental (T2) No metastasis (M0)
IIIa Low or High grade (G1-G2) Intracompartmental (T1) Metastasis (M1)
IIIb Low or High grade (G1-G2) Extracompartmental (T2) Metastasis (M1)

History and Symptoms

  • Angiosarcomas occur at different anatomic sites and grow insidiously, then they can present with various misleading symptoms.[24]
  • The most common clinical manifestation is a gradually enlarging, painless mass.[21]
  • Some patients complain of pain or symptoms due to compression of adjacent neurovascular structures that causes pain or edema in an extremity.
  • Secondary angiosarcomas include radiation-Induced and lymphedema-associated angiosarcoma have a distinct feature, presenting as single or several ecchymotic maculopapular cutaneous lesions in the radiation field or in areas exposed to chronic lymphedema.[25]

Physical Examination

Laboratory Findings

  • There are no specific laboratory findings associated with angiosarcoma.

Electrocardiogram

Echocardiography or Ultrasound

X-ray

Imaging Findings

  • The imaging modality of choice for angiosarcoma will depend on the location.
  • For pulmonary angiosarcoma, the imaging modality of choice is CT scan with intravenous contrast.[19]
  • For other types angiosarcoma, the imaging modality of choice is MRI.

CT Scan

On CT, findings of angiosarcoma may include vascular invasion, nodular enhancement (common), and multicentric lesions.[19]

MRI

Other Imaging Findings

  • There are no other imaging findings associated with angiosarcoma.

Other Diagnostic Studies

  • There are no other diagnostic studies associated with angiosarcoma.

Treatment

Medical Therapy

Surgery

  • The mainstay of treatment for angiosarcoma is complete surgical resection with wide margins for local and locoregional disease in combination with preoperative or postoperative radiotherapy.[34][35]
  • Surgical resection in combination with radiation therapy is the treatment of choice for small angiosarcomas.[5]
  • Complete surgical resection with wide margins is preferred for local and locoregional angiosarcoma.[5]
  • Owing to the tendency for local infiltration, surgical resection should be associated with preoperative or postoperative radiotherapy.[36]
  • Surgery is not recommended on patients with large sized angiosarcomas.
  • It usually occurs after a median of six months locally or distantly and the three-year disease-free and overall survival rates both are low.[37][38]

Primary Prevention

Secondary Prevention

References

  1. Barber W, Scriven P, Turner D, Hughes D, Wyld D (2010). "Epithelioid angiosarcoma: Use of angiographic embolisation and radiotherapy to control recurrent haemorrhage". J Surg Case Rep. 2010 (5): 7. doi:10.1093/jscr/2010.5.7. PMC 3649120. PMID 24946325.
  2. Fury MG, Antonescu CR, Van Zee KJ, Brennan MF, Maki RG (2005). "A 14-year retrospective review of angiosarcoma: clinical characteristics, prognostic factors, and treatment outcomes with surgery and chemotherapy". Cancer J. 11 (3): 241–7. PMID 16053668.
  3. Schlemmer M, Reichardt P, Verweij J, Hartmann JT, Judson I, Thyss A, Hogendoorn PC, Marreaud S, Van Glabbeke M, Blay JY (November 2008). "Paclitaxel in patients with advanced angiosarcomas of soft tissue: a retrospective study of the EORTC soft tissue and bone sarcoma group". Eur. J. Cancer. 44 (16): 2433–6. doi:10.1016/j.ejca.2008.07.037. PMID 18771914.
  4. Karanian M, Coindre JM (January 2015). "[Fourth edition of WHO classification tumours of soft tissue]". Ann Pathol (in French). 35 (1): 71–85. doi:10.1016/j.annpat.2014.11.003. PMID 25532684.
  5. 5.0 5.1 5.2 5.3 5.4 5.5 5.6 5.7 5.8 5.9 Young RJ, Brown NJ, Reed MW, Hughes D, Woll PJ (October 2010). "Angiosarcoma". Lancet Oncol. 11 (10): 983–91. doi:10.1016/S1470-2045(10)70023-1. PMID 20537949.
  6. Amo Y, Masuzawa M, Hamada Y, Katsuoka K (January 2004). "Serum concentrations of vascular endothelial growth factor-D in angiosarcoma patients". Br. J. Dermatol. 150 (1): 160–1. doi:10.1111/j.1365-2133.2004.05751.x. PMID 14746640.
  7. Manner J, Radlwimmer B, Hohenberger P, Mössinger K, Küffer S, Sauer C, Belharazem D, Zettl A, Coindre JM, Hallermann C, Hartmann JT, Katenkamp D, Katenkamp K, Schöffski P, Sciot R, Wozniak A, Lichter P, Marx A, Ströbel P (January 2010). "MYC high level gene amplification is a distinctive feature of angiosarcomas after irradiation or chronic lymphedema". Am. J. Pathol. 176 (1): 34–9. doi:10.2353/ajpath.2010.090637. PMC 2797867. PMID 20008140.
  8. Guo T, Zhang L, Chang NE, Singer S, Maki RG, Antonescu CR (January 2011). "Consistent MYC and FLT4 gene amplification in radiation-induced angiosarcoma but not in other radiation-associated atypical vascular lesions". Genes Chromosomes Cancer. 50 (1): 25–33. doi:10.1002/gcc.20827. PMC 3150534. PMID 20949568.
  9. Fernandez AP, Sun Y, Tubbs RR, Goldblum JR, Billings SD (February 2012). "FISH for MYC amplification and anti-MYC immunohistochemistry: useful diagnostic tools in the assessment of secondary angiosarcoma and atypical vascular proliferations". J. Cutan. Pathol. 39 (2): 234–42. doi:10.1111/j.1600-0560.2011.01843.x. PMID 22121953.
  10. 10.0 10.1 Mittal S, Goswami C, Kanoria N, Bhattacharya A (2007). "Post-irradiation angiosarcoma of bone". J Cancer Res Ther. 3 (2): 96–9. PMID 17998731.
  11. Murphey MD, Fairbairn KJ, Parman LM, Baxter KG, Parsa MB, Smith WS (July 1995). "From the archives of the AFIP. Musculoskeletal angiomatous lesions: radiologic-pathologic correlation". Radiographics. 15 (4): 893–917. doi:10.1148/radiographics.15.4.7569134. PMID 7569134.
  12. Marušić Z, Billings SD (June 2017). "Histopathology of Spindle Cell Vascular Tumors". Surg Pathol Clin. 10 (2): 345–366. doi:10.1016/j.path.2017.01.006. PMID 28477885.
  13. 13.0 13.1 Kiyohara T, Kumakiri M, Kobayashl H, Itoh K, Lao LM, Ohkawara A, Nakmura H (February 2002). "Spindle cell angiosarcoma following irradiation therapy for cervical carcinoma". J. Cutan. Pathol. 29 (2): 96–100. doi:10.1034/j.1600-0560.2002.290206.x. PMID 12150140.
  14. Sinclair SA, Sviland L, Natarajan S (April 1998). "Angiosarcoma arising in a chronically lymphoedematous leg". Br. J. Dermatol. 138 (4): 692–4. doi:10.1046/j.1365-2133.1998.02188.x. PMID 9640382.
  15. de Keizer RJ, de Wolff-Rouendaal D, Nooy MA (2008). "Angiosarcoma of the eyelid and periorbital region. Experience in Leiden with iridium192 brachytherapy and low-dose doxorubicin chemotherapy". Orbit. 27 (1): 5–12. doi:10.1080/01676830601168926. PMID 18307140.
  16. Vora R, Anjaneyan G, Gupta R (November 2014). "Cutaneous angiosarcoma of head and neck". Indian J Dermatol. 59 (6): 632. doi:10.4103/0019-5154.143575. PMC 4248527. PMID 25484419.
  17. Fletcher CD, Beham A, Bekir S, Clarke AM, Marley NJ (October 1991). "Epithelioid angiosarcoma of deep soft tissue: a distinctive tumor readily mistaken for an epithelial neoplasm". Am. J. Surg. Pathol. 15 (10): 915–24. doi:10.1097/00000478-199110000-00001. PMID 1718176.
  18. Ferrari A, Sultan I, Huang TT, Rodriguez-Galindo C, Shehadeh A, Meazza C, Ness KK, Casanova M, Spunt SL (December 2011). "Soft tissue sarcoma across the age spectrum: a population-based study from the Surveillance Epidemiology and End Results database". Pediatr Blood Cancer. 57 (6): 943–9. doi:10.1002/pbc.23252. PMC 4261144. PMID 21793180.
  19. 19.0 19.1 19.2 19.3 19.4 19.5 19.6 Sturgis EM, Potter BO. Sarcomas of the head and neck region. Curr Opin Oncol. 2003 May. 15(3):239-52
  20. Sturgis EM, Potter BO (May 2003). "Sarcomas of the head and neck region". Curr Opin Oncol. 15 (3): 239–52. doi:10.1097/00001622-200305000-00011. PMID 12778019.
  21. 21.0 21.1 21.2 21.3 Gaballah AH, Jensen CT, Palmquist S, Pickhardt PJ, Duran A, Broering G, Elsayes KM (July 2017). "Angiosarcoma: clinical and imaging features from head to toe". Br J Radiol. 90 (1075): 20170039. doi:10.1259/bjr.20170039. PMC 5594986. PMID 28471264.
  22. Lydiatt WM, Patel SG, O'Sullivan B, Brandwein MS, Ridge JA, Migliacci JC, Loomis AM, Shah JP (March 2017). "Head and Neck cancers-major changes in the American Joint Committee on cancer eighth edition cancer staging manual". CA Cancer J Clin. 67 (2): 122–137. doi:10.3322/caac.21389. PMID 28128848.
  23. Jawad MU, Scully SP (July 2010). "In brief: classifications in brief: enneking classification: benign and malignant tumors of the musculoskeletal system". Clin. Orthop. Relat. Res. 468 (7): 2000–2. doi:10.1007/s11999-010-1315-7. PMC 2882012. PMID 20333492.
  24. Young, Robin J; Brown, Nicola J; Reed, Malcolm W; Hughes, David; Woll, Penella J (2010). "Angiosarcoma". The Lancet Oncology. 11 (10): 983–991. doi:10.1016/S1470-2045(10)70023-1. ISSN 1470-2045.
  25. Cui L, Zhang J, Zhang X, Chang H, Qu C, Zhang J, Zhong D (2015). "Angiosarcoma (Stewart-Treves syndrome) in postmastectomy patients: report of 10 cases and review of literature". Int J Clin Exp Pathol. 8 (9): 11108–15. PMC 4637645. PMID 26617830.
  26. Donnell RM, Rosen PP, Lieberman PH, Kaufman RJ, Kay S, Braun DW, Kinne DW (October 1981). "Angiosarcoma and other vascular tumors of the breast". Am. J. Surg. Pathol. 5 (7): 629–42. doi:10.1097/00000478-198110000-00005. PMID 7199829.
  27. Bendel, Emily C.; Maleszewski, Joseph J.; Araoz, Philip A. (2011). "Imaging Sarcomas of the Great Vessels and Heart". Seminars in Ultrasound, CT and MRI. 32 (5): 377–404. doi:10.1053/j.sult.2011.06.001. ISSN 0887-2171.
  28. Wenger DE, Wold LE (November 2000). "Malignant vascular lesions of bone: radiologic and pathologic features". Skeletal Radiol. 29 (11): 619–31. doi:10.1007/s002560000261. PMID 11201031.
  29. Lahat G, Dhuka AR, Hallevi H, Xiao L, Zou C, Smith KD, Phung TL, Pollock RE, Benjamin R, Hunt KK, Lazar AJ, Lev D (June 2010). "Angiosarcoma: clinical and molecular insights". Ann. Surg. 251 (6): 1098–106. doi:10.1097/SLA.0b013e3181dbb75a. PMID 20485141.
  30. Penel N, Italiano A, Ray-Coquard I, Chaigneau L, Delcambre C, Robin YM, Bui B, Bertucci F, Isambert N, Cupissol D, Bompas E, Bay JO, Duffaud F, Guillemet C, Blay JY (February 2012). "Metastatic angiosarcomas: doxorubicin-based regimens, weekly paclitaxel and metastasectomy significantly improve the outcome". Ann. Oncol. 23 (2): 517–23. doi:10.1093/annonc/mdr138. PMID 21566149.
  31. Skubitz KM, Haddad PA (July 2005). "Paclitaxel and pegylated-liposomal doxorubicin are both active in angiosarcoma". Cancer. 104 (2): 361–6. doi:10.1002/cncr.21140. PMID 15948172.
  32. In GK, Hu JS, Tseng WW (August 2017). "Treatment of advanced, metastatic soft tissue sarcoma: latest evidence and clinical considerations". Ther Adv Med Oncol. 9 (8): 533–550. doi:10.1177/1758834017712963. PMC 5524246. PMID 28794805.
  33. Vo KT, Matthay KK, DuBois SG (2016). "Targeted antiangiogenic agents in combination with cytotoxic chemotherapy in preclinical and clinical studies in sarcoma". Clin Sarcoma Res. 6: 9. doi:10.1186/s13569-016-0049-z. PMC 4896001. PMID 27274393.
  34. Lydiatt WM, Shaha AR, Shah JP (November 1994). "Angiosarcoma of the head and neck". Am. J. Surg. 168 (5): 451–4. doi:10.1016/s0002-9610(05)80097-2. PMID 7977971.
  35. Arifi S, Belbaraka R, Rahhali R, Ismaili N (2015). "Treatment of Adult Soft Tissue Sarcomas: An Overview". Rare Cancers Ther. 3: 69–87. doi:10.1007/s40487-015-0011-x. PMC 4837937. PMID 27182479.
  36. Guadagnolo BA, Zagars GK, Araujo D, Ravi V, Shellenberger TD, Sturgis EM (May 2011). "Outcomes after definitive treatment for cutaneous angiosarcoma of the face and scalp". Head Neck. 33 (5): 661–7. doi:10.1002/hed.21513. PMC 4090937. PMID 20960566.
  37. Buehler D, Rice SR, Moody JS, Rush P, Hafez GR, Attia S, Longley BJ, Kozak KR (October 2014). "Angiosarcoma outcomes and prognostic factors: a 25-year single institution experience". Am. J. Clin. Oncol. 37 (5): 473–9. doi:10.1097/COC.0b013e31827e4e7b. PMC 3664266. PMID 23428947.
  38. Seinen JM, Styring E, Verstappen V, Vult von Steyern F, Rydholm A, Suurmeijer AJ, Hoekstra HJ (August 2012). "Radiation-associated angiosarcoma after breast cancer: high recurrence rate and poor survival despite surgical treatment with R0 resection". Ann. Surg. Oncol. 19 (8): 2700–6. doi:10.1245/s10434-012-2310-x. PMC 3404270. PMID 22466664.