Soft Tissue Special Issue: Imaging of Bone and Soft Tissue Sarcomas in the Head and Neck

  • Ngoc-Anh TranEmail author
  • Jeffrey P. Guenette
  • Jyothi Jagannathan
Special Issue: Soft Tissue


Bone and soft tissue sarcomas of the head and neck are a heterogenous group of tumors with overlapping features. Distinguishing between the various subtypes is challenging but necessary for appropriate diagnosis and management. The purpose of this article is to discuss the role of imaging in evaluating head and neck tumors, provide a general radiographic approach in differentiating between benign versus malignant lesions and give examples of selected subtypes of bone and soft tissue sarcomas in the head and neck with classic or pathognomonic imaging findings.


Bone and soft tissue sarcomas Imaging Radiology Head and neck Sarcomas 



  1. 1.
    Burningham Z, Hashibe M, Spector L, Schiffman J. The epidemiology of sarcoma. Clin Sarcoma Res. 2012;2(1):14.PubMedPubMedCentralCrossRefGoogle Scholar
  2. 2.
    Stiller CA, Trama A, Serraino D, Rossi S, Navarro C, Chirlaque MD, Casali PG. Descriptive epidemiology of sarcomas in Europe: report from the RARECARE project. Eur J Cancer. 2013;49(3):664–95.CrossRefGoogle Scholar
  3. 3.
    Brockstein B. Management of sarcomas of the head and neck. Curr Oncol Rep. 2004;6(4):321–7.PubMedCrossRefPubMedCentralGoogle Scholar
  4. 4.
    Sturgis EM, Potter BO. Sarcomas of the head and neck region. Curr Opin Oncol. 2003;15(3):239–52.PubMedCrossRefPubMedCentralGoogle Scholar
  5. 5.
    De Schepper AM, Bloem JL. Soft tissue tumors: grading, staging, and tissue-specific diagnosis. Top Magn Reson Imaging. 2007;18(6):431–44.PubMedCrossRefPubMedCentralGoogle Scholar
  6. 6.
    van Rijswijk CS, Geirnaerdt MJ, Hogendoorn PC, et al. Soft-tissue tumors: value of static and dynamic gadopentetate dimeglumine–enhanced MR imaging in prediction of malignancy. Radiology. 2004;233(2):493–502.PubMedCrossRefPubMedCentralGoogle Scholar
  7. 7.
    Razek AA, Huang BY. Soft tissue tumors of the head and neck: imaging-based review of the WHO classification. Radiographics. 2011;31(7):1923–54.PubMedCrossRefPubMedCentralGoogle Scholar
  8. 8.
    Bastiaannet E, Groen H, Jager PL, et al. The value of FDG-PET in the detection, grading and response to therapy of soft tissue and bone sarcomas; a systematic review and meta-analysis. Cancer Treat Rev. 2004;30(1):83–101.PubMedCrossRefPubMedCentralGoogle Scholar
  9. 9.
    Tshering Vogel DW, Thoeny HC. Cross-sectional imaging in cancers of the head and neck: how we review and report. Cancer Imaging. 2016;16(1):20.PubMedPubMedCentralCrossRefGoogle Scholar
  10. 10.
    Mandell J. Core radiology: a visual approach to diagnostic imaging. Cambridge: Cambridge University Press; 2013.CrossRefGoogle Scholar
  11. 11.
    Mendenhall WM, Fernandes R, Werning JW, Vaysberg M, Malyapa RS, Mendenhall NP. Head and neck osteosarcoma. Am J Otolaryngol Head Neck Med Surg. 2010;32(6):597–600.Google Scholar
  12. 12.
    Lee YY, Van Tassel P, Nauert C, Raymond AK, Ediken J. Craniofacial osteosarcomas: plain film, CT and MR findings in 46 cases. Am J Neuroradiol. 1988;9:379–85.Google Scholar
  13. 13.
    Elkordy MA, ElBaradie TS, Elsebai HI, et al. Osteosarcoma of the jaw: challenges in the diagnosis and treatment. J Egypt Natl Cancer Inst. 2018;30(1):7–11.CrossRefGoogle Scholar
  14. 14.
    Scelsi C, Wang A, Garvin C, Bajaj M, Forseen S, Gilbert B. Head and neck sarcomas: a review of clinical and imaging findings based on the 2013 World Health Organization classification. Am J Roentgenol. 2018;212(3):1–11.Google Scholar
  15. 15.
    Juergens H, Grevener K, Dirksen U, Ranft A. Ewing sarcoma of the head and neck. J Clin Oncol. 2010;28(15):E20516.CrossRefGoogle Scholar
  16. 16.
    Murphey MD, Arcara LK, Fansburg-Smith J. Imaging of musculoskeletal liposarcoma with radiologic-pathologic correlation. Radiographics. 2005;25(5):1371–95.PubMedCrossRefPubMedCentralGoogle Scholar
  17. 17.
    Davis EC, Ballo MT, Luna MA, et al. Liposarcoma of the head and neck: the University of Texas M. D. Anderson Cancer Center experience. Head Neck. 2009;31(1):28–36.PubMedCrossRefPubMedCentralGoogle Scholar
  18. 18.
    O’Neill JP, Bilsky MH, Kraus D. Head and neck sarcomas: epidemiology, pathology, and management: epidemiology, pathology, and management. Neurosurg Clin N Am. 2013;24(1):67–78.PubMedCrossRefPubMedCentralGoogle Scholar
  19. 19.
    Lee JH, Lee MS, Lee BH, Choe DH, Do YS, Kim KH, Cho KJ. Rhabdomyosarcoma of the head and neck in adults: MR and CT findings. Am J Neuroradiol. 1996;17(10):1923–8.PubMedPubMedCentralGoogle Scholar
  20. 20.
    Fletcher CDM, Bridge JA, Hogendoorn PC, Mertens F. WHO classification of tumors of soft tissue and bone. 4th ed. Lyon: International Agency for Research on Cancer; 2013.Google Scholar
  21. 21.
    Zhu J, Zhang J, Tang G, Hu S, Zhou G, Liu Y, et al. Computed tomography and magnetic resonance imaging observations of rhabdomyosarcoma in the head and neck. Oncol Lett. 2014;8(1):155–60.PubMedPubMedCentralCrossRefGoogle Scholar
  22. 22.
    Hagiwara A, Inoue Y, Nakayama T, Yamato K, Nemoto Y, Shakudo M, et al. The “botryoid sign”: a characteristic feature of rhabdomyosarcomas in the head and neck. Neuroradiology. 2001;43(4):331–5.PubMedCrossRefPubMedCentralGoogle Scholar
  23. 23.
    Rodriguez DP, Orscheln ES, Koch BL. Masses of the nose, nasal cavity, and nasopharynx in children. Radiographics. 2017;37(6):1704–30.PubMedCrossRefPubMedCentralGoogle Scholar
  24. 24.
    Allen SD, Moskovic EC, Fisher C, Thomas JM. Adult rhabdomyosarcoma: cross-sectional imaging findings including histopathologic correlation. AJR Am J Roentgenol. 2007;189(2):371.PubMedCrossRefPubMedCentralGoogle Scholar
  25. 25.
    Freling NJ, Merks JH, Saeed P, et al. Imaging findings in craniofacial childhood rhabdomyosarcoma. Pediatr Radiol. 2010;40:1723–38.PubMedPubMedCentralCrossRefGoogle Scholar
  26. 26.
    Yousem DM, Lexa FJ, Bilaniuk LT, Zimmerman RI. Rhabdomyosarcomas in the head and neck: MR imaging evaluation. Radiology. 1990;177:683–6.PubMedCrossRefPubMedCentralGoogle Scholar
  27. 27.
    Saboo SS, Krajewski KM, Zukotynski K, Howard S, Jagannathan JP, Hornick JL, et al. Imaging features of primary and secondary adult rhabdomyosarcoma. AJR Am J Roentgenol. 2012;199(6):W694–703.PubMedCrossRefPubMedCentralGoogle Scholar
  28. 28.
    van Vliet M, Kliffen M, Krestin GP, van Dijke CF. Soft tissue sarcomas at a glance: clinical, histological, and MR imaging features of malignant extremity soft tissue tumors. Eur Radiol. 2009;19:1499–511.PubMedCrossRefPubMedCentralGoogle Scholar
  29. 29.
    Gründahl JE, Hallermann C, Schulze HJ, Klein M, Wermker K. Cutaneous angiosarcoma of head and neck: a new predictive score for locoregional metastasis. Transl Oncol. 2015;8:169–75.PubMedPubMedCentralCrossRefGoogle Scholar
  30. 30.
    Patel SH, Hayden RE, Hinni ML, et al. Angiosarcoma of the scalp and face: the Mayo Clinic experience. JAMA Otolaryngol Head Neck Surg. 2015;141:335–40.PubMedCrossRefPubMedCentralGoogle Scholar
  31. 31.
    Mullins B, Hackman T. Angiosarcoma of the head and neck. Int Arch Otorhinolaryngol. 2015;19(3):191–5.PubMedPubMedCentralCrossRefGoogle Scholar
  32. 32.
    Chikarmane SA, Gombos EC, Jagadeesan J, Raut C, Jagannathan JP. MRI findings of radiation-associated angiosarcoma of the breast (RAS). J Magn Reson Imaging (JMRI). 2015;42(3):763–70.CrossRefGoogle Scholar
  33. 33.
    Kransdorf MJ, Murphey MD. Imaging of soft tissue tumors. Philadelphia, PA: Saunders; 1997.Google Scholar
  34. 34.
    Torreggiani WC, Khalid AI, Munk PL, Nicolau S, O’Connell JX, Knowling MA. Dermatofibrosarcoma protuberans MR imaging features. AJR. 2002;178:989–93.PubMedCrossRefPubMedCentralGoogle Scholar
  35. 35.
    Millare GG, Guha-Thakurta N, Sturgis EM, El- Naggar AK, DebnAm JM. Imaging findings of head and neck dermatofibrosarcoma protuberans. AJNR. 2014;35:373–8.PubMedCrossRefPubMedCentralGoogle Scholar
  36. 36.
    Hoesly PM, Lowe GC, Lohse CM, Brewer JD, Lehman JS. Prognostic impact of fibrosarcomatous transformation in dermatofibrosarcoma protuberans: a cohort study. J Am Acad Dermatol. 2015;72(3):419–25.PubMedCrossRefPubMedCentralGoogle Scholar
  37. 37.
    McCarville MB, Muzzafar S, Kao SC, et al. Imaging features of alveolar soft-part sarcoma: a report from Children’s Oncology Group study ARST0332. AJR. 2014;203:1345–52.PubMedCrossRefPubMedCentralGoogle Scholar
  38. 38.
    Kim HS, Lee HK, Weon YC, Kim HJ. Alveolar soft-part sarcoma of the head and neck: clinical and imaging features in five cases. AJNR. 2005;26:1331–5.PubMedPubMedCentralGoogle Scholar
  39. 39.
    Suh JS, Cho J, Lee SH, Shin KH, Yang WI, Lee JH, et al. Alveolar soft part sarcoma: MR and angiographic findings. Skeletal Radiol. 2000;29(12):680–9.PubMedCrossRefPubMedCentralGoogle Scholar
  40. 40.
    Tian L, Cui CY, Lu SY, Cai PQ, Xi SY, Fan W. Clinical presentation and CT/MRI findings of alveolar soft part sarcoma: a retrospective single-center analysis of 14 cases. Acta Radiol. 2016;57(4):475–80.PubMedCrossRefPubMedCentralGoogle Scholar
  41. 41.
    Viry F, Orbach D, Klijanienko J, Freneaux P, Pierron G, Michon J, et al. Alveolar soft part sarcoma-radiologic patterns in children and adolescents. Pediatr Radiol. 2013;43(9):1174–8.PubMedCrossRefPubMedCentralGoogle Scholar
  42. 42.
    Sood S, Baheti AD, Shinagare AB, Jagannathan JP, Hornick JL, Ramaiya NH, et al. Imaging features of primary and metastatic alveolar soft part sarcoma: single institute experience in 25 patients. Br J Radiol. 2014;87(1036):20130719.PubMedPubMedCentralCrossRefGoogle Scholar

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© Springer Science+Business Media, LLC, part of Springer Nature 2020

Authors and Affiliations

  1. 1.Department of RadiologyBrigham & Women’s Hospital, Harvard Medical SchoolBostonUSA
  2. 2.Department of ImagingDana-Farber Cancer Institute, Harvard Medical SchoolBostonUSA

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