Top

Published in:

Open Access 01-12-2021 | Magnetic Resonance Imaging | Educational Review

Imaging features of primary sites and metastatic patterns of angiosarcoma

Authors: Basrull N. Bhaludin, Khin Thway, Margaret Adejolu, Alexandra Renn, Christian Kelly-Morland, Cyril Fisher, Robin L. Jones, Christina Messiou, Eleanor Moskovic

Published in: Insights into Imaging | Issue 1/2021

Abstract

Angiosarcomas are rare, aggressive soft tissue sarcomas originating from endothelial cells of lymphatic or vascular origin and associated with a poor prognosis. The clinical and imaging features of angiosarcomas are heterogeneous with a wide spectrum of findings involving any site of the body, but these most commonly present as cutaneous disease in the head and neck of elderly men. MRI and CT are complementary imaging techniques in assessing the extent of disease, focality and involvement of adjacent anatomical structures at the primary site of disease. CT plays an important role in the evaluation of metastatic disease. Given the wide range of imaging findings, correlation with clinical findings, specific risk factors and patterns of metastatic disease can help narrow the differential diagnosis. The final diagnosis should be confirmed with histopathology and immunohistochemistry in combination with clinical and imaging findings in a multidisciplinary setting with specialist sarcoma expertise. The purpose of this review is to describe the clinical and imaging features of primary sites and metastatic patterns of angiosarcomas utilising CT and MRI.

Young RJ, Brown NJ, Reed MW, Hughes D, Woll PJ (2010) Angiosarcoma. Lancet Oncol 11:983–991. https://doi.org/10.1016/S1470-2045(10)70023-1CrossRefPubMed

Antonescu C (2014) Malignant vascular tumorsan update. Mod Pathol 27(Suppl 1):S30–S38. https://doi.org/10.1038/modpathol.2013.176CrossRefPubMed

Wang L, Lao IW, Yu L, Wang J (2017) Clinicopathological features and prognostic factors in angiosarcoma: a retrospective analysis of 200 patients from a single Chinese medical institute. Oncol Lett 14:5370–5378. https://doi.org/10.3892/ol.2017.6892CrossRefPubMedPubMedCentral

Buehler D, Rice SR, Moody JS et al (2014) Angiosarcoma outcomes and prognostic factors: a 25-year single institution experience. Am J Clin Oncol 37:473–479. https://doi.org/10.1097/COC.0b013e31827e4e7bCrossRefPubMedPubMedCentral

Abraham JA, Hornicek FJ, Kaufman AM et al (2007) Treatment and outcome of 82 patients with angiosarcoma. Ann Surg Oncol 14:1953–1967. https://doi.org/10.1245/s10434-006-9335-yCrossRefPubMed

Fayette J, Martin E, Piperno-Neumann S et al (2007) Angiosarcomas, a heterogeneous group of sarcomas with specific behavior depending on primary site: a retrospective study of 161 cases. Ann Oncol 18:2030–2036. https://doi.org/10.1093/annonc/mdm381CrossRefPubMed

Fury MG, Antonescu CR, Van Zee KJ, Brennan ME, Maki RG (2005) A 14-year retrospective review of angiosarcoma: clinical characteristics, prognostic factors, and treatment outcomes with surgery and chemotherapy. Cancer J 11:241–247. https://doi.org/10.1097/00130404-200505000-00011CrossRefPubMed

Thway K, Billings SD (2020) The WHO classification of tumours editorial board. WHO classification of tumours soft tissue and bone tumours, 5th edn. IARC Press, Lyon

Yap J, Chuba PJ, Thomas R et al (2002) Sarcoma as a second malignancy after treatment for breast cancer. Int J Radiat Oncol Biol Phys 52:1231–1237. https://doi.org/10.1016/S0360-3016(01)02799-7CrossRefPubMed

10.

Sheth GR, Cranmer LD, Smith BD, Grasso-LeBeau L, Lang JE (2019) Assessment of radiotherapy-associated angiosarcoma after breast cancer treatment in a Dutch population-based study. JAMA Oncol 5:267–269. https://doi.org/10.1001/jamaoncol.2018.6643CrossRef

11.

Mark RJ, Poen JC, Tran LM, Fu YS, Juillard GF (1996) Angiosarcoma: A report of 67 patients and a review of the literature. Cancer 77:2400–2406. https://doi.org/10.1002/(SICI)1097-0142(19960601)77:11%3c2400::AID-CNCR32%3e3.0.CO;2-ZCrossRefPubMed

12.

Morgan MB, Swann M, Somach S, Eng W, Smoller B (2004) Cutaneous angiosarcoma: a case series with prognostic correlation. J Am Acad Dermatol 50:867–874. https://doi.org/10.1016/j.jaad.2003.10.671CrossRefPubMed

13.

Stewart FW, Treves N (1948) Lymphangiosarcoma in postmastectomy lymphedema. a report of six cases in elephantiasis chirurgica. Cancer 1:64–81. https://doi.org/10.1002/1097-0142(194805)1:1%3c64::AID-CNCR2820010105%3e3.0.CO;2-WCrossRefPubMed

14.

White PG, Adams H, Smith PM (1993) The computed tomographic appearances of angiosarcoma of the liver. Clin Radiol 48:321–325. https://doi.org/10.1016/S0009-9260(05)81240-1CrossRefPubMed

15.

McDonald DJ, Enneking WF, Sundaram M (2002) Metal-associated angiosarcoma of bone: Report of two cases and review of the literature. Clin Orthop Relat Res. https://doi.org/10.1097/00003086-200203000-00031CrossRefPubMed

16.

Ahmed I, Hamacher KL (2002) Angiosarcoma in a chronically immunosuppressed renal transplant recipient: report of a case and review of the literature. Am J Dermatopathol 24

17.

Deyrup AT, McKenney JK, Tighiouart M, Folpe AL, Weiss SW (2008) Sporadic cutaneous angiosarcomas: a proposal for risk stratification based on 69 cases. Am J Surg Pathol 32:72–77. https://doi.org/10.1097/PAS.0b013e3180f633a3CrossRefPubMed

18.

Renn A, Adejolu M, Messiou C et al (2021) Overview of malignant soft-tissue sarcomas of the limbs. Clin Radiol. https://doi.org/10.1016/j.crad.2021.08.011CrossRefPubMed

19.

Vilanova JC, Baleato-Gonzalez S, Romero MJ, Carrascoso-Arranz J, Luna A (2016) Assessment of musculoskeletal malignancies WITH Functional MR imaging. Magn Reson Imaging Clin N Am 24:239–259. https://doi.org/10.1016/j.mric.2015.08.006CrossRefPubMed

20.

Dangoor A, Seddon B, Gerrand C, Grimer R, Whelan J, Judson I (2016) UK guidelines for the management of soft tissue sarcomas. Clin Sarcoma Res 6:1–26. https://doi.org/10.1186/s13569-016-0060-4CrossRef

21.

Scelsi CL, Wang A, Garvin CM, Bajaj M, Forseen SE, Gilbert BC (2018) Head and neck sarcomas: a review of clinical and imaging findings based on the 2013 World Health Organization Classification. AJR Am J Roentgenol 212:644–654. https://doi.org/10.2214/AJR.18.19894CrossRefPubMed

22.

Alwarqi AF, Abdurabu M, Gopalakrishnan P, Paksoy Y, Ageila MA (2021) An incidental finding of scalp angiosarcoma: a case report. Cureus 13:2–4. https://doi.org/10.7759/cureus.13610CrossRef

23.

Juan YH, Saboo SS, Tirumani SH et al (2014) Malignant skin and subcutaneous neoplasms in adults: multimodality imaging with CT, MRI, and 18F-FDG PET/CT. AJR Am J Roentgenol 202:422–438. https://doi.org/10.2214/AJR.13.11424CrossRef

24.

Razek AA, Huang BY (2011) Soft tissue tumors of the head and neck: imaging-based review of the who classification. Radiographics 31:1923–1954. https://doi.org/10.1148/rg.317115095CrossRefPubMed

25.

Huis Veld EA, Grünhagen DJ, Verhoef C et al (2017) Isolated limb perfusion for locally advanced angiosarcoma in extremities: a multi-centre study. Eur J Cancer 85:114–121. https://doi.org/10.1016/j.ejca.2017.07.023CrossRef

26.

Lv L, Xu P, Shi Y, Hao J, Hu C, Zhao B (2016) Imaging features of soft tissue epithelioid angiosarcoma in the lower extremity: a case report. Oncol Lett 11:3457–3460. https://doi.org/10.3892/ol.2016.4407CrossRefPubMedPubMedCentral

27.

Nguyen Cuong P, Thanh Xuan N, Nhu Huy P, Nhu Tung T, Huu Son N (2020) Histopathological features of deep soft tissue epithelioid angiosarcoma in the lower extremity: a rare case report. Am J Case Rep 21:e923933. https://doi.org/10.12659/AJCR.923933CrossRefPubMed

28.

Schreiber H, Barry FM, Russell WC, Macon WL, Ponsky JL, Pories WJ (1979) Stewart–Treves syndrome. A lethal complication of postmastectomy lymphedema and regional immune deficiency. Arch Surg 114:82–85. https://doi.org/10.1001/archsurg.1979.01370250084018CrossRefPubMed

29.

Kazerooni E, Hessler C (1991) CT appearance of angiosarcoma associated with chronic lymphedema. AJR Am J Roentgenol 156:543–544. https://doi.org/10.2214/ajr.156.3.1899753CrossRefPubMed

30.

Schindera ST, Streit M, Kaelin U, Stauffer E, Steinbach L, Anderson SE (2005) Stewart–Treves syndrome: MR imaging of a postmastectomy upper-limb chronic lymphedema with angiosarcoma. Skelet Radiol 34:156–160. https://doi.org/10.1007/s00256-004-0807-5CrossRef

31.

Nakazono T, Kudo S, Matsuo Y et al (2000) Angiosarcoma associated with chronic lymphedema (Stewart–Treves syndrome) of the leg: MR imaging. Skelet Radiol 29:413–416. https://doi.org/10.1007/s002560000225CrossRef

32.

Chopra S, Ors F, Bergin D (2007) MRI of angiosarcoma associated with chronic lymphoedema: Stewart Treves syndrome. Br J Radiol 80:310–313. https://doi.org/10.1259/bjr/19441948CrossRef

33.

Glazebrook KN, Magut MJ, Reynolds C (2008) Angiosarcoma of the breast. AJR Am J Roentgenol 190:533–538. https://doi.org/10.2214/AJR.07.2909CrossRefPubMed

34.

Yang WT, Hennessy BT, Dryden MJ, Valero V, Hunt KK, Krishnamurthy S (2007) Mammary angiosarcomas: imaging findings in 24 patients. Radiology 242:725–734. https://doi.org/10.1148/radiol.2423060163CrossRefPubMed

35.

Liberman L, Dershaw DD, Kaufman RJ, Rosen PP (1992) Angiosarcoma of the breast. Radiology 183:649–654. https://doi.org/10.1148/radiology.183.3.1584913CrossRefPubMed

36.

Chesebro AL, Chikarmane SA, Gombos EC, Giardino AA (2016) Radiation-associated angiosarcoma of the breast: what the radiologist needs to know. AJR Am J Roentgenol 207:217–225. https://doi.org/10.2214/AJR.15.15888CrossRefPubMed

37.

Strobbe LJ, Peterse HL, van Tinteren H, Wijnmaalen A, Emiel JT (1998) Angiosarcoma of the breast after conservation therapy for invasive cancer, the incidence and outcome. An unforeseen sequela. Breast Cancer Res Treat 47:101–109. https://doi.org/10.1023/A:1005997017102CrossRefPubMed

38.

Cuperus E, Leguit R, Albregts M, Toonstra J (2013) Post radiation skin tumors: basal cell carcinomas, squamous cell carcinomas and angiosarcomas. A review of this late effect of radiotherapy. Eur J Dermatol 23:749–757. https://doi.org/10.1684/ejd.2013.2106CrossRefPubMed

39.

Brenn T, Fletcher CDM (2006) Postradiation vascular proliferations: an increasing problem. Histopathology 48:106–114. https://doi.org/10.1111/j.1365-2559.2005.02293.xCrossRefPubMed

40.

Chikarmane SA, Gombos EC, Jagadeesan J, Raut C, Jagannathan JP (2015) MRI findings of radiation-associated angiosarcoma of the breast (RAS). J Magn Reson Imaging 42:763–770. https://doi.org/10.1002/jmri.24822CrossRefPubMed

41.

Best AK, Dobson RL, Ahmad AR (2003) Best cases from the AFIP: cardiac angiosarcoma. Radiographics 23:141–145. https://doi.org/10.1148/rg.23si035140CrossRef

42.

Burke AP, Cowan D, Virmani R (1992) Primary sarcomas of the heart. Cancer 69:387–395. https://doi.org/10.1002/1097-0142(19920115)69:2%3c387::aid-cncr2820690219%3e3.0.co;2-nCrossRefPubMed

43.

Janigan DT, Husain A, Robinson NA (1986) Cardiac angiosarcomas: a review and a case report. Cancer 57:852–859. https://doi.org/10.1002/1097-0142(19860215)57:4%3c852::AID-CNCR2820570428%3e3.0.CO;2-SCrossRefPubMed

44.

Bruna J, Lockwood M (1998) Primary heart angiosarcoma detected by computed tomography and magnetic resonance imaging. Eur Radiol 8:66–68. https://doi.org/10.1007/s003300050341CrossRefPubMed

45.

Yahata S, Endo T, Honma H et al (1994) Sunray appearance on enhanced magnetic resonance image of cardiac angiosarcoma with pericardial obliteration. Am Heart J 127:468–471. https://doi.org/10.1016/0002-8703(94)90149-XCrossRefPubMed

46.

Gaballah AH, Jensen CT, Palmquist S et al (2017) Angiosarcoma: clinical and imaging features from head to toe. Brit J Radiol. https://doi.org/10.1259/bjr.20170039CrossRefPubMedPubMedCentral

47.

Ho CKL, Wang E, Au WK, Cheng LC (2009) Primary Cardiac angiosarcoma of left atrium. J Cardiac Surg 24:524–525. https://doi.org/10.1111/j.1540-8191.2009.00872.xCrossRef

48.

Bruegel M, Muenzel D, Waldt S, Specht K, Rummeny EJ (2013) Hepatic angiosarcoma: cross-sectional imaging findings in seven patients with emphasis on dynamic contrast-enhanced and diffusion-weighted MRI. Abdom Imaging 38:745–754. https://doi.org/10.1007/s00261-012-9967-2CrossRefPubMed

49.

Pickhardt PJ, Kitchin D, Lubner MG, Ganeshan DM, Bhalla S, Covey AM (2015) Primary hepatic angiosarcoma: multi-institutional comprehensive cancer centre review of multiphasic CT and MR imaging in 35 patients. Eur Radiol 25:315–322. https://doi.org/10.1007/s00330-014-3442-0CrossRefPubMed

50.

Yi LL, Zhang JX, Zhou SG et al (2019) CT and MRI studies of hepatic angiosarcoma. Clin Radiol 74:406.e1-406.e8. https://doi.org/10.1016/j.crad.2018.12.013CrossRef

51.

Koyama T, Fletcher JG, Johnson CD, Kuo MS, Notohara K, Burgart LJ (2002) Primary hepatic angiosarcoma: findings at CT and MR imaging. Radiology 222:667–673. https://doi.org/10.1148/radiol.2223010877CrossRefPubMed

52.

Yu RS, Chen Y, Jiang B, Wang LH, Xu XF (2008) Primary hepatic sarcomas: CT findings. Eur Radiol 18:2196–2205. https://doi.org/10.1007/s00330-008-0997-7CrossRefPubMed

53.

Semelka RC, Nimojan N, Chandana S et al (2018) MRI features of primary rare malignancies of the liver: a report from four university centres. Eur Radiol 28:1529–1539. https://doi.org/10.1007/s00330-017-5102-7CrossRefPubMed

54.

Mathew RP, Sam M, Raubenheimer M, Patel V, Low G (2020) Hepatic hemangiomas: the various imaging avatars and its mimickers. Radiol Med 125:801–815. https://doi.org/10.1007/s11547-020-01185-zCrossRefPubMed

55.

Kim B, Byun JH, Lee JH et al (2018) Imaging findings of primary hepatic angiosarcoma on gadoxetate disodium-enhanced liver MRI: comparison with hepatic haemangiomas of similar size. Clin Radiol 73:244–253. https://doi.org/10.1016/j.crad.2017.09.015CrossRefPubMed

56.

Falk S, Krishnan J, Meis JM (1993) Primary angiosarcoma of the spleen: a clinicopathologic study of 40 cases. Am J Surg Pathol 17:959–970. https://doi.org/10.1097/00000478-199310000-00001CrossRefPubMed

57.

Neuhauser TS, Derringer GA, Thompson LD et al (2000) Splenic angiosarcoma: a clinicopathologic and immunophenotypic study of 28 cases. Mod Pathol 13:978–987. https://doi.org/10.1038/modpathol.3880178CrossRefPubMed

58.

Vrachliotis TG, Bennett WF, Vaswani KK, Niemann TH, Bova JG (2000) Primary angiosarcoma of the spleen - CT, MR, and sonographic characteristics: report of two cases. Abdom Imaging 25:283–285. https://doi.org/10.1007/s002610000034CrossRefPubMed

59.

Thompson WM, Levy AD, Aguilera NS, Gorospe L, Abbott RM (2005) Angiosarcoma of the spleen: imaging characteristics in 12 patients. Radiology 235:106–115. https://doi.org/10.1148/radiol.2351040308CrossRefPubMed

60.

Karakas HM, Demir M, Ozyilmaz F, Cakir B (2001) Primary angiosarcoma of the spleen: in vivo and in vitro MRI findings. Clin Imaging 25:192–196. https://doi.org/10.1016/S0899-7071(01)00286-8CrossRefPubMed

61.

Yamashita H, Endo K, Teshima R (2012) Angiosarcoma of the proximal humerus: a case report and review of the literature. J Med Case Rep 6:1–6. https://doi.org/10.1186/1752-1947-6-347CrossRef

62.

Murphey MD, Andrews CL, Flemming DJ, Temple HT, Smith WS, Smirniotopoulos JG (1995) From the archives of the AFIP. Musculoskeletal angiomatous lesions: radiologic-pathologic correlation. Radiographics 15:893–917. https://doi.org/10.1148/radiographics.15.4.7569134CrossRefPubMed

63.

Wenger DE, Wold LE (2000) Malignant vascular lesions of bone: radiologic and pathologic features. Skelet Radiol 29:619–631. https://doi.org/10.1007/s002560000261CrossRef

64.

Vermaat M, Vanel D, Kroon HM et al (2011) Vascular tumors of bone: Imaging findings. Eur J Radiol 77:13–18. https://doi.org/10.1016/j.ejrad.2010.06.052CrossRefPubMed

65.

Detorakis EE, Chryssou E, Raissaki M et al (2013) Primary renal angiosarcoma: radiologicpathologic correlation and literature review. Tumori. https://doi.org/10.1177/030089161309900328CrossRefPubMed

66.

Leggio L, Addolorato G, Abenavoli L et al (2006) Primary renal angiosarcoma: a rare malignancy. A case report and review of the literature. Urol Oncol 24:307–312. https://doi.org/10.1016/j.urolonc.2005.10.002CrossRefPubMed

67.

Tateishi U, Hasegawa T, Kusumoto M et al (2003) Metastatic angiosarcoma of the lung: spectrum of CT findings. AJR Am J Roentgenol 180:1671–1674. https://doi.org/10.2214/ajr.180.6.1801671CrossRefPubMed

68.

Yogi A, Miyara T, Ogawa K et al (2016) Pulmonary metastases from angiosarcoma: a spectrum of CT findings. Acta Radiol 57:41–46. https://doi.org/10.1177/0284185115571789CrossRefPubMed

69.

Lee YR, Choi YW, Lee KJ, Jeon SC, Park CK, Heo JN (2005) CT halo sign: the spectrum of pulmonary diseases. Br J Radiol 78:862–865. https://doi.org/10.1259/bjr/77712845CrossRefPubMed

70.

Tateishi U, Hasegawa T, Muramatsu Y, Moriyama N (2003) Hepatic metastases of soft tissue angiosarcoma: CT and MR imaging findings. Abdom Imaging 28:660–664. https://doi.org/10.1007/s00261-003-0008-zCrossRefPubMed

71.

Wu JS, Hochman MG (2009) Soft-tissue tumors and tumorlike lesions: a systematic imaging approach. Radiology 253:297–316. https://doi.org/10.1148/radiol.2532081199CrossRefPubMed

72.

Strauss DC, Qureshi YA, Hayes AJ, Thway K, Fisher C, Thomas JM (2010) The role of core needle biopsy in the diagnosis of suspected soft tissue tumours. J Surg Oncol 102:523–529. https://doi.org/10.1002/jso.21600CrossRefPubMed

73.

Kang TW, Lee MW, Choi D et al (2016) Safety of percutaneous biopsy for hepatic angiosarcoma: results of a multicenter Korean survey. J Vasc Interv Radiol 27:846–851. https://doi.org/10.1016/j.jvir.2016.01.148CrossRefPubMed

74.

Lucey BC, Boland GW, Maher MM, Hahn PF, Gervais DA, Mueller PR (2002) Percutaneous nonvascular splenic intervention: a 10-year review. AJR Am J Roentgenol 179:1591–1596. https://doi.org/10.2214/ajr.179.6.1791591CrossRefPubMed

75.

Painter CA, Jain E, Tomson BN et al (2020) The Angiosarcoma Project: enabling genomic and clinical discoveries in a rare cancer through patient-partnered research. Nat Med 26:181–187. https://doi.org/10.1038/s41591-019-0749-zCrossRefPubMed

76.

Florou V, Rosenberg AE, Wieder E et al (2019) Angiosarcoma patients treated with immune checkpoint inhibitors: a case series of seven patients from a single institution. J Immunother Cancer 7:213. https://doi.org/10.1186/s40425-019-0689-7CrossRefPubMedPubMedCentral

77.

Jones RL, Ravi V, Brohl AS et al (2019) 1667O - Results of the TAPPAS trial: an adaptive enrichment phase III trial of TRC105 and pazopanib (P) versus pazopanib alone in patients with advanced angiosarcoma (AS). Ann Oncol 30:v683. https://doi.org/10.1093/annonc/mdz283CrossRef

78.

Ravi V, Wagner M, Chen TW-W et al (2020) A phase II study of oraxol in the treatment of unresectable cutaneous angiosarcoma. J Clin Oncol 38:11517. https://doi.org/10.1200/JCO.2020.38.15_suppl.11517CrossRef

Title: Imaging features of primary sites and metastatic patterns of angiosarcoma
Authors: Basrull N. Bhaludin
Khin Thway
Margaret Adejolu
Alexandra Renn
Christian Kelly-Morland
Cyril Fisher
Robin L. Jones
Christina Messiou
Eleanor Moskovic
Publication date: 01-12-2021
Publisher: Springer International Publishing
Keywords: Magnetic Resonance Imaging
Magnetic Resonance Imaging
Computed Tomography
Metastasis
Computed Tomography
Published in: Insights into Imaging / Issue 1/2021
Electronic ISSN: 1869-4101
DOI: https://doi.org/10.1186/s13244-021-01129-9

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Imaging features of primary sites and metastatic patterns of angiosarcoma

Abstract

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Please log in to get access to this content

Other articles of this Issue 1/2021

A primer on deep learning and convolutional neural networks for clinicians

Bringing AI to the clinic: blueprint for a vendor-neutral AI deployment infrastructure

Identifying the deceiver: the non-neoplastic mimickers of genital system neoplasms

Understanding PI-QUAL for prostate MRI quality: a practical primer for radiologists

Letter to the editor: “Not all biases are bad: equitable and inequitable biases in machine learning and radiology”

Osteoid osteoma: the great mimicker