HEAD AND NECK SOFT TISSUE SARCOMAS: DIAGNOSIS AND MANAGEMENT REVIEW
Abstract
Soft tissue sarcomas (STS) of the head and neck are rare and histologically diverse malignancies that present unique diagnostic and therapeutic challenges due to their proximity to critical anatomical structures. They originate from various tissues, including fat, muscle, nerves, blood vessels, and others. Although they account for a small proportion of all STS, their management requires individualized care within experienced sarcoma centres. While surgery and radiotherapy remain the primary modalities, newer systemic and targeted strategies are increasingly being incorporated into clinical practice. In this review we summarise current state of knowledge related to the diagnosis, staging, and treatment of head and neck STS in adults. We also discuss evolving systemic and targeted therapies with potential relevance to clinical oncology practice.
References
Agaimy, A., Mueller, S. K., Harrer, T., Bauer, S., & Thompson, L. D. R. (2018). Head and Neck Kaposi Sarcoma: Clinicopathological Analysis of 11 Cases. Head Neck Pathol, 12(4), 511-516. https://doi.org/10.1007/s12105-018-0902-x
Agaram, N. P. (2022). Evolving classification of rhabdomyosarcoma. Histopathology, 80(1), 98-108. https://doi.org/10.1111/his.14449
Akagunduz, B., Akin Telli, T., Sezgin Goksu, S., Yildirim, H. C., Ozer, M., Goktas Aydin, S., Ozyurt, N., Karacin, C., Paydas, S., & Dogan, M. (2021). Assessment of Prognostic Factors and Adjuvant Treatment Modalities in Adult Head and Neck Soft Tissue Sarcoma Patients Treated With Upfront Surgery. Cureus, 13(2), e13324. https://doi.org/10.7759/cureus.13324
Amin, M. B., Greene, F. L., Edge, S. B., Compton, C. C., Gershenwald, J. E., Brookland, R. K., Meyer, L., Gress, D. M., Byrd, D. R., & Winchester, D. P. (2017). The Eighth Edition AJCC Cancer Staging Manual: Continuing to build a bridge from a population-based to a more "personalized" approach to cancer staging. CA: A Cancer Journal for Clinicians, 67(2), 93-99. https://doi.org/10.3322/caac.21388
Angiero, F., Rizzuti, T., Crippa, R., & Stefani, M. (2007). Fibrosarcoma of the jaws: two cases of primary tumors with intraosseous growth. Anticancer Res, 27(4c), 2573-2581.
Augsburger, D., Nelson, P. J., Kalinski, T., Udelnow, A., Knösel, T., Hofstetter, M., Qin, J. W., Wang, Y., Gupta, A. S., Bonifatius, S., Li, M., Bruns, C. J., & Zhao, Y. (2017). Current diagnostics and treatment of fibrosarcoma -perspectives for future therapeutic targets and strategies. Oncotarget, 8(61), 104638-104653. https://doi.org/10.18632/oncotarget.20136
Boccalatte, L. A., Gómez, N. L., Yanzon, A., Mazzaro, E. L., Cayol, F., & Figari, M. F. (2019). Head and Neck Tumors: Management of Primary Undifferentiated Pleomorphic Sarcoma. Iran J Otorhinolaryngol, 31(107), 335-342. https://doi.org/10.22038/ijorl.2019.30195.1990
Brockstein, B. (2004). Management of sarcomas of the head and neck. Current Oncology Reports, 6(4), 321-327. https://doi.org/10.1007/s11912-004-0043-0
Cates, J. M. M. (2019). Staging soft tissue sarcoma of the head and neck: Evaluation of the AJCC 8th edition revised T classifications. Head and Neck, 41(7), 2359-2366. https://doi.org/10.1002/hed.25701
Chen, Y., Jiang, Y., Ji, Z., Jiang, P., Xu, F., Zhang, Y., Guo, F., Peng, R., Li, X., Sun, H., Lei, R., Fan, J., Li, W., & Wang, J. (2020). Efficacy and safety of CT-guided 125I seed implantation as a salvage treatment for locally recurrent head and neck soft tissue sarcoma after surgery and external beam radiotherapy: A 12-year study at a single institution. Brachytherapy, 19(1), 81-89. https://doi.org/10.1016/j.brachy.2019.09.006
Dagan, R., Indelicato, D. J., McGee, L., Morris, C. G., Kirwan, J. M., Knapik, J., Reith, J., Scarborough, M. T., Gibbs, C. P., Marcus, R. B., Jr., & Zlotecki, R. A. (2012). The significance of a marginal excision after preoperative radiation therapy for soft tissue sarcoma of the extremity. Cancer, 118(12), 3199-3207. https://doi.org/10.1002/cncr.26489
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, 20. https://doi.org/10.1186/s13569-016-0060-4
Davis, E. C., Ballo, M. T., Luna, M. A., Patel, S. R., Roberts, D. B., Nong, X., & Sturgis, E. M. (2009). Liposarcoma of the head and neck: The University of Texas M. D. Anderson Cancer Center experience. Head Neck, 31(1), 28-36. https://doi.org/10.1002/hed.20923
de Bree, R., van der Waal, I., de Bree, E., & Leemans, C. R. (2010). Management of adult soft tissue sarcomas of the head and neck. Oral Oncology, 46(11), 786-790. https://doi.org/10.1016/j.oraloncology.2010.09.001
Egger, J. F., Coindre, J. M., Benhattar, J., Coucke, P., & Guillou, L. (2002). Radiation-associated synovial sarcoma: clinicopathologic and molecular analysis of two cases. Mod Pathol, 15(9), 998-1004. https://doi.org/10.1097/01.Mp.0000026616.41545.Ff
Elsayad, K., Stockmann, D., Channaoui, M., Scobioala, S., Grajda, A., Berssenbrügge, H., Huss, S., Moustakis, C., Haverkamp, U., Kleinheinz, J., Lenz, G., Wardelmann, E., & Eich, H. T. (2019). Using Image-guided Intensity-modulated Radiotherapy on Patients With Head and Neck Soft-tissue Sarcoma. In Vivo, 33(4), 1293-1300. https://doi.org/10.21873/invivo.11602
Eriksson, M. (2010). Histology-driven chemotherapy of soft-tissue sarcoma. Annals of Oncology, 21 Suppl 7, vii270-276. https://doi.org/10.1093/annonc/mdq285
Galy-Bernadoy, C., & Garrel, R. (2016). Head and neck soft-tissue sarcoma in adults. European Annals of Otorhinolaryngology, Head and Neck Diseases, 133(1), 37-42. https://doi.org/10.1016/j.anorl.2015.09.003
Gazendam, A. M., Popovic, S., Munir, S., Parasu, N., Wilson, D., & Ghert, M. (2021). Synovial Sarcoma: A Clinical Review. Curr Oncol, 28(3), 1909-1920. https://doi.org/10.3390/curroncol28030177
George, S., Serrano, C., Hensley, M. L., & Ray-Coquard, I. (2018). Soft Tissue and Uterine Leiomyosarcoma. J Clin Oncol, 36(2), 144-150. https://doi.org/10.1200/jco.2017.75.9845
Golledge, J., Fisher, C., & Rhys-Evans, P. H. (1995). Head and neck liposarcoma. Cancer, 76(6), 1051-1058. https://doi.org/10.1002/1097-0142(19950915)76:6<1051::aid-cncr2820760620>3.0.co;2-4
Gronchi, A., Miah, A. B., Dei Tos, A. P., Abecassis, N., Bajpai, J., Bauer, S., Biagini, R., Bielack, S., Blay, J. Y., Bolle, S., Bonvalot, S., Boukovinas, I., Bovee, J., Boye, K., Brennan, B., Brodowicz, T., Buonadonna, A., De Alava, E., Del Muro, X. G., . . . clinicalguidelines@esmo.org, G. E. a. (2021). Soft tissue and visceral sarcomas: ESMO-EURACAN-GENTURIS Clinical Practice Guidelines for diagnosis, treatment and follow-up(☆). Annals of Oncology, 32(11), 1348-1365. https://doi.org/10.1016/j.annonc.2021.07.006
Gronchi, A., Stacchiotti, S., Verderio, P., Ferrari, S., Martin Broto, J., Lopez-Pousa, A., Llombart-Bosch, A., Dei Tos, A. P., Collini, P., Jurado, J. C., De Paoli, A., Donati, D. M., Poveda, A., Quagliuolo, V., Comandone, A., Grignani, G., Morosi, C., Messina, A., De Sanctis, R., . . . Picci, P. (2016). Short, full-dose adjuvant chemotherapy (CT) in high-risk adult soft tissue sarcomas (STS): long-term follow-up of a randomized clinical trial from the Italian Sarcoma Group and the Spanish Sarcoma Group. Annals of Oncology, 27(12), 2283-2288. https://doi.org/10.1093/annonc/mdw430
Han, S. (2022). Malignant Peripheral Nerve Sheath Tumor of the Head and Neck Region. J Craniofac Surg, 33(8), 2711-2712. https://doi.org/10.1097/scs.0000000000008550
Huang, S. H., & O'Sullivan, B. (2017). Overview of the 8th Edition TNM Classification for Head and Neck Cancer. Current Treatment Options in Oncology, 18(7), 40. https://doi.org/10.1007/s11864-017-0484-y
Issels, R. D., Lindner, L. H., Verweij, J., Wessalowski, R., Reichardt, P., Wust, P., Ghadjar, P., Hohenberger, P., Angele, M., Salat, C., Vujaskovic, Z., Daugaard, S., Mella, O., Mansmann, U., Durr, H. R., Knosel, T., Abdel-Rahman, S., Schmidt, M., Hiddemann, W., . . . the European Society for Hyperthermic, O. (2018). Effect of Neoadjuvant Chemotherapy Plus Regional Hyperthermia on Long-term Outcomes Among Patients With Localized High-Risk Soft Tissue Sarcoma: The EORTC 62961-ESHO 95 Randomized Clinical Trial. JAMA Oncol, 4(4), 483-492. https://doi.org/10.1001/jamaoncol.2017.4996
Jonczak, E., Grossman, J., Alessandrino, F., Seldon Taswell, C., Velez-Torres, J. M., & Trent, J. (2024). Liposarcoma: A Journey into a Rare Tumor's Epidemiology, Diagnosis, Pathophysiology, and Limitations of Current Therapies. Cancers (Basel), 16(22). https://doi.org/10.3390/cancers16223858
Kahvecioglu, A., Bay, M., Yuce Sari, S., Yazici, G., Kuscu, O., & Cengiz, M. (2025). 1308 Conventional Fractionation vs. Stereotactic Body Radiotherapy: A Comparative Approach in Postoperative Management of Head and Neck Sarcomas. Radiotherapy and Oncology, 206, S971-S973. https://doi.org/10.1016/S0167-8140(25)00383-4
Kaur, H., Gupta, V., Mishra, D., & Yadav, V. S. (2022). Fibrosarcoma: Origin, differential diagnosis, and report of a case in the mandible. J Indian Soc Periodontol, 26(2), 169-177. https://doi.org/10.4103/jisp.jisp_188_21
Kim, W. J., & Kim, H. K. (2023). Current understanding of angiosarcoma: disease biology and evolving treatment. Arch Craniofac Surg, 24(5), 203-210. https://doi.org/10.7181/acfs.2023.00409
Kungwengwe, G., Clancy, R., Vass, J., Slade, R., Sandhar, S., Dobbs, T. D., & Bragg, T. W. H. (2021). Preoperative versus Post-operative Radiotherapy for Extremity Soft tissue Sarcoma: a Systematic Review and Meta-analysis of Long-term Survival. Journal of Plastic, Reconstructive & Aesthetic Surgery, 74(10), 2443-2457. https://doi.org/10.1016/j.bjps.2021.05.043
Lin, X., Davion, S., Bertsch, E. C., Omar, I., Nayar, R., & Laskin, W. B. (2016). Federation Nationale des Centers de Lutte Contre le Cancer grading of soft tissue sarcomas on needle core biopsies using surrogate markers. Human Pathology, 56, 147-154. https://doi.org/10.1016/j.humpath.2016.06.008
Luppi, M., Barozzi, P., Rasini, V., & Torelli, G. (2002). HHV-8 infection in the transplantation setting: a concern only for solid organ transplant patients? Leuk Lymphoma, 43(3), 517-522. https://doi.org/10.1080/10428190290011994
Mariggiò, G., Koch, S., & Schulz, T. F. (2017). Kaposi sarcoma herpesvirus pathogenesis. Philos Trans R Soc Lond B Biol Sci, 372(1732). https://doi.org/10.1098/rstb.2016.0275
Mendenhall, W. M., Mendenhall, C. M., Werning, J. W., Riggs, C. E., & Mendenhall, N. P. (2005). Adult head and neck soft tissue sarcomas. Head and Neck, 27(10), 916-922. https://doi.org/10.1002/hed.20249
Munuswamy, N., Sundar, M., Krishnan, K., Chandran, M., & R, K. K. (2024). Undifferentiated Pleomorphic Sarcoma: A Case Report. Cureus, 16(11), e73422. https://doi.org/10.7759/cureus.73422
O'Steen, L., Saldivar, B., Kharod, S., Bassett, B., Morris, C. G., & Mendenhall, W. M. (2020). Radiotherapy for Adult Soft Tissue Sarcomas of the Head and Neck. American Journal of Clinical Oncology, 43(9), 667-669. https://doi.org/10.1097/COC.0000000000000729
O'Sullivan, B., Davis, A. M., Turcotte, R., Bell, R., Catton, C., Chabot, P., Wunder, J., Kandel, R., Goddard, K., Sadura, A., Pater, J., & Zee, B. (2002). Preoperative versus postoperative radiotherapy in soft-tissue sarcoma of the limbs: a randomised trial. Lancet, 359(9325), 2235-2241. https://doi.org/10.1016/S0140-6736(02)09292-9
O'Sullivan, B., Gullane, P., Irish, J., Neligan, P., Gentili, F., Mahoney, J., Sellmann, S., Catton, C., Waldron, J., Brown, D., Witterick, I., Freeman, J., & Bell, R. (2003). Preoperative radiotherapy for adult head and neck soft tissue sarcoma: assessment of wound complication rates and cancer outcome in a prospective series. World Journal of Surgery, 27(7), 875-883. https://doi.org/10.1007/s00268-003-7115-4
Palassini, E., Ferrari, S., Verderio, P., De Paoli, A., Martin Broto, J., Quagliuolo, V., Comandone, A., Sangalli, C., Palmerini, E., Lopez-Pousa, A., De Sanctis, R., Bottelli, S., Libertini, M., Picci, P., Casali, P. G., & Gronchi, A. (2015). Feasibility of Preoperative Chemotherapy With or Without Radiation Therapy in Localized Soft Tissue Sarcomas of Limbs and Superficial Trunk in the Italian Sarcoma Group/Grupo Espanol de Investigacion en Sarcomas Randomized Clinical Trial: Three Versus Five Cycles of Full-Dose Epirubicin Plus Ifosfamide. Journal of Clinical Oncology, 33(31), 3628-3634. https://doi.org/10.1200/JCO.2015.62.9394
Pasquali, S., Pizzamiglio, S., Touati, N., Litiere, S., Marreaud, S., Kasper, B., Gelderblom, H., Stacchiotti, S., Judson, I., Dei Tos, A. P., Verderio, P., Casali, P. G., Woll, P. J., Gronchi, A., Tissue, E. S., & Bone Sarcoma, G. (2019). The impact of chemotherapy on survival of patients with extremity and trunk wall soft tissue sarcoma: revisiting the results of the EORTC-STBSG 62931 randomised trial. European Journal of Cancer, 109, 51-60. https://doi.org/10.1016/j.ejca.2018.12.009
Pavlidis, E. T., & Pavlidis, T. E. (2023). New trends in the surgical management of soft tissue sarcoma: The role of preoperative biopsy. World J Clin Oncol, 14(2), 89-98. https://doi.org/10.5306/wjco.v14.i2.89
Prudner, B. C., Ball, T., Rathore, R., & Hirbe, A. C. (2020). Diagnosis and management of malignant peripheral nerve sheath tumors: Current practice and future perspectives. Neurooncol Adv, 2(Suppl 1), i40-i49. https://doi.org/10.1093/noajnl/vdz047
Ramakrishnan, N., Mokhtari, R., Charville, G. W., Bui, N., & Ganjoo, K. (2022). Cutaneous Angiosarcoma of the Head and Neck-A Retrospective Analysis of 47 Patients. Cancers, 14(15). https://doi.org/10.3390/cancers14153841
Ramírez-Amador, V., Anaya-Saavedra, G., & Martínez-Mata, G. (2010). Kaposi's sarcoma of the head and neck: a review. Oral Oncol, 46(3), 135-145. https://doi.org/10.1016/j.oraloncology.2009.12.006
Saluja, T. S., Iyer, J., & Singh, S. K. (2019). Leiomyosarcoma: Prognostic outline of a rare head and neck malignancy. Oral Oncol, 95, 100-105. https://doi.org/10.1016/j.oraloncology.2019.06.010
Scott, L. J. (2018). Apatinib: A Review in Advanced Gastric Cancer and Other Advanced Cancers. Drugs, 78(7), 747-758. https://doi.org/10.1007/s40265-018-0903-9
Sharifi, N., Zeydi, A. E., Habibi, M. R., Ghafari, R., Baradari, A. G., & Nouraei, M. (2013). Decreasing blood loss and the need for transfusion after CABG surgery: a double-blind randomized clinical trial of topical tranexamic acid. Turkish Journal of Medical Sciences. https://doi.org/10.3906/sag-1206-37
Sharma, N., George, N. A., Singh, R., Iype, E. M., Varghese, B. T., & Thomas, S. (2018). Surgical Management of Head and Neck Soft Tissue Sarcoma: 11-Year Experience at a Tertiary Care Centre in South India. Indian Journal of Surgical Oncology, 9(2), 187-191. https://doi.org/10.1007/s13193-018-0755-5
Skapek, S. X., Ferrari, A., Gupta, A. A., Lupo, P. J., Butler, E., Shipley, J., Barr, F. G., & Hawkins, D. S. (2019). Rhabdomyosarcoma. Nat Rev Dis Primers, 5(1), 1. https://doi.org/10.1038/s41572-018-0051-2
Somatilaka, B. N., Sadek, A., McKay, R. M., & Le, L. Q. (2022). Malignant peripheral nerve sheath tumor: models, biology, and translation. Oncogene, 41(17), 2405-2421. https://doi.org/10.1038/s41388-022-02290-1
Stanbouly, D., Litman, E., Lee, K. C., & Philipone, E. (2021). Synovial sarcoma of the head & neck: A review of reported cases in the literature. J Stomatol Oral Maxillofac Surg, 122(5), 505-510. https://doi.org/10.1016/j.jormas.2020.12.001
Stavrakas, M., Nixon, I., Andi, K., Oakley, R., Jeannon, J. P., Lyons, A., McGurk, M., Urbano, T. G., Thavaraj, S., & Simo, R. (2016). Head and neck sarcomas: clinical and histopathological presentation, treatment modalities, and outcomes. Journal of Laryngology and Otology, 130(9), 850-859. https://doi.org/10.1017/S0022215116008604
Trifiletti, D., Amdur, R. J., Dagan, R., Indelicato, D. J., Mendenhall, W. M., Kirwan, J. M., Yeung, A. R., Werning, J. W., & Morris, C. G. (2012). Radiotherapy following gross total resection of adult soft tissue sarcoma of the head and neck. Practical Radiation Oncology, 2(4), e121-e128. https://doi.org/10.1016/j.prro.2012.01.003
Vassiliou, L.-V., Lalabekyan, B., Jay, A., Liew, C., Whelan, J., Newman, L., & Kalavrezos, N. (2017). Head and neck sarcomas: A single institute series. Oral Oncology, 65, 16-22. https://doi.org/10.1016/j.oraloncology.2016.12.005
Vitzthum, L. K., Brown, L. C., Rooney, J. W., & Foote, R. L. (2016). Head and Neck Soft Tissue Sarcomas Treated with Radiation Therapy. Rare Tumors, 8(2), 6165. https://doi.org/10.4081/rt.2016.6165
Workman, A. D., Farquhar, D. R., Brody, R. M., Parasher, A. K., Carey, R. M., Purkey, M. T., Nagda, D. A., Brooks, J. S., Hartner, L. P., Brant, J. A., & Newman, J. G. (2018). Leiomyosarcoma of the head and neck: A 17-year single institution experience and review of the National Cancer Data Base. Head and Neck, 40(4), 756-762. https://doi.org/10.1002/hed.25054
Wu, Q., Wang, J., Li, S., Liu, J., Cheng, Y., Jin, J., & Zhong, Y. (2023). Comparison of Definitive Radiotherapy-Based Treatment and Surgical-Based Treatment for Locally Advanced Head and Neck Soft Tissue Sarcoma. J Clin Med, 12(9). https://doi.org/10.3390/jcm12093099
Yadav, J., Bakshi, J., Chouhan, M., & Modi, R. (2013). Head and neck leiomyosarcoma. Indian J Otolaryngol Head Neck Surg, 65(Suppl 1), 1-5. https://doi.org/10.1007/s12070-011-0305-8
Yang, J., Gao, J., Qiu, X., Hu, J., Hu, W., Wu, X., Zhang, C., Ji, T., Kong, L., & Lu, J. J. (2019). Intensity-Modulated Proton and Carbon-Ion Radiation Therapy in the Management of Head and Neck Sarcomas. Cancer Med, 8(10), 4574-4586. https://doi.org/10.1002/cam4.2319
Ye, L. L., Li, R., Dou, S., Shao, Z., Ji, T., & Zhu, G. (2020). A Phase II Trial of Radiotherapy Concurrent with Apatinib in Locally Advanced Bone and Soft Tissue Sarcoma of the Head and Neck: Preliminary Results. International Journal of Radiation Oncology*Biology*Physics, 108(3), S131-S132. https://doi.org/10.1016/j.ijrobp.2020.07.862
Young, R. J., Brown, N. J., Reed, M. W., Hughes, D., & Woll, P. J. (2010). Angiosarcoma. Lancet Oncol, 11(10), 983-991. https://doi.org/10.1016/s1470-2045(10)70023-1
Views:
221
Downloads:
191
Copyright (c) 2025 Michał Bereza, Mateusz Dembiński, Julia Prabucka-Marciniak, Edyta Szymańska

This work is licensed under a Creative Commons Attribution 4.0 International License.
All articles are published in open-access and licensed under a Creative Commons Attribution 4.0 International License (CC BY 4.0). Hence, authors retain copyright to the content of the articles.
CC BY 4.0 License allows content to be copied, adapted, displayed, distributed, re-published or otherwise re-used for any purpose including for adaptation and commercial use provided the content is attributed.