Abstract
Primary sarcomas of the larynx are rare and are associated with diagnostic and treatment challenges. Studies of these tumors are limited, and most examples have been reported as small series. To further increase our understanding of laryngeal sarcomas, we reviewed our experience of an adult cohort. A retrospective search for laryngeal sarcomas from our pathology archives and consultation files of one of the authors was performed. We studied 27 primary laryngeal sarcomas that included 25 males, and 2 females, with a mean age of 60 years (range 33–85). The cases included conventional chondrosarcoma (16), well-differentiated liposarcoma (2), clear cell chondrosarcoma (1), leiomyosarcoma (2), high grade myxofibrosarcoma (2), high grade myofibroblastic sarcoma (1), low-grade myofibroblastic sarcoma (1), malignant granular cell tumor (1), and Kaposi sarcoma (1). Data on treatment and follow-up was available in 17 and 16 cases, respectively. 12 patients underwent partial laryngeal resection; five had total laryngectomy, and the patient with Kaposi sarcoma received combined highly active antiretroviral therapy and chemotherapy. Three patients developed local recurrence, and two patients developed metastases. The remaining patients with follow up had a favorable outcome and were disease-free after treatment. The important differential diagnosis of spindle cell sarcoma is sarcomatoid squamous cell carcinoma, and their distinction often requires extensive sampling of the mucosal surface and immunohistochemical analysis. The mainstay of treatment for laryngeal sarcomas is surgical removal, with the extent dictated by tumor type and grade. Adjuvant therapy is reserved for high-grade sarcomas and may be given in a neoadjuvant or adjuvant setting.
References
Narozny W, Mikaszewski B, Stankiewicz C. Benign neoplasms of the larynx. Auris Nasus Larynx. 1995;22(1):38–42. https://doi.org/10.1016/s0385-8146(12)80180-5.
Saraydaroglu O, Narter S, Ozsen M, Coskun H. Non-epithelial tumors of the larynx: case series of 12 years. Eur Arch Otorhinolaryngol. 2019;276(10):2843–7. https://doi.org/10.1007/s00405-019-05527-0.
Rzepakowska A, Osuch-Wojcikiewicz E, Bruzgielewicz A, Niemczyk K. Non-epithelial neoplasms of the larynx and hypopharynx—12-years of experience. Otolaryngol Pol. 2015;69(5):9–15. https://doi.org/10.5604/00306657.1170420.
Karatayli-Ozgursoy S, Bishop JA, Hillel AT, Akst LM, Best SR. Non-epithelial tumors of the larynx: a single institution review. Am J Otolaryngol. 2016;37(3):279–85. https://doi.org/10.1016/j.amjoto.2016.01.005.
Friedman AD, Burns JA, Lutch MJ, Zeitels SM. Submucosal neoplasms of the laryngeal introitus. J Laryngol Otol. 2012;126(7):706–13. https://doi.org/10.1017/S0022215112000928.
Mantilla JG, Xu H, Ricciotti RW. Primary sarcomas of the larynx: a single institutional experience with ten cases. Head Neck Pathol. 2019. https://doi.org/10.1007/s12105-019-01106-1.
Fernandez-Acenero MJ, Larach F, Ortega-Fernandez C. Non-epithelial lesions of the larynx: review of the 10-year experience in a tertiary Spanish hospital. Acta Otolaryngol. 2009;129(1):108–12. https://doi.org/10.1080/00016480802008207.
Casiraghi O, Martinez-Madrigal F, Pineda-Daboin K, Mamelle G, Resta L, Luna MA. Chondroid tumors of the larynx: a clinicopathologic study of 19 cases, including two dedifferentiated chondrosarcomas. Ann Diagn Pathol. 2004;8(4):189–97. https://doi.org/10.1053/j.anndiagpath.2004.04.001.
Thompson LD, Gannon FH. Chondrosarcoma of the larynx: a clinicopathologic study of 111 cases with a review of the literature. Am J Surg Pathol. 2002;26(7):836–51. https://doi.org/10.1097/00000478-200207000-00002.
Cao X, Liu J, Zheng Y, Li Q, Teng Y, Li Y. Simultaneous squamous cell carcinoma with primary malignant fibrous histiocytoma of the larynx: a case report. Mol Med Rep. 2012;5(4):971–3. https://doi.org/10.3892/mmr.2012.775.
Dubal PM, Svider PF, Kanumuri VV, Patel AA, Baredes S, Eloy JA. Laryngeal chondrosarcoma: a population-based analysis. Laryngoscope. 2014;124(8):1877–81. https://doi.org/10.1002/lary.24618.
Lewis JE, Olsen KD, Inwards CY. Cartilaginous tumors of the larynx: clinicopathologic review of 47 cases. Ann Otol Rhinol Laryngol. 1997;106(2):94–100. https://doi.org/10.1177/000348949710600202.
Coca-Pelaz A, Rodrigo JP, Triantafyllou A, Hunt JL, Fernandez-Miranda JC, Strojan P, de Bree R, Rinaldo A, Takes RP, Ferlito A. Chondrosarcomas of the head and neck. Eur Arch Otorhinolaryngol. 2014;271(10):2601–9. https://doi.org/10.1007/s00405-013-2807-3.
Alexander J, Wakely PE Jr. Primary laryngeal clear cell chondrosarcoma: report of a case and literature review. Head Neck Pathol. 2014;8(3):307–10. https://doi.org/10.1007/s12105-014-0542-8.
Tallegas M, Miquelestorena-Standley E, Labit-Bouvier C, Badoual C, Francois A, Gomez-Brouchet A, Aubert S, Collin C, Tallet A, de Pinieux G. IDH mutation status in a series of 88 head and neck chondrosarcomas: different profile between tumors of the skull base and tumors involving the facial skeleton and the laryngotracheal tract. Hum Pathol. 2019;84:183–91. https://doi.org/10.1016/j.humpath.2018.09.015.
Baatenburg de Jong RJ, van Lent S, Hogendoorn PC. Chondroma and chondrosarcoma of the larynx. Curr Opin Otolaryngol Head Neck Surg. 2004;12(2):98–105. https://doi.org/10.1097/00020840-200404000-00008.
Wang J, Heffner DK, Venegas R, French SW. Atypical chondroma of the cricoid cartilage: fine-needle aspiration cytologic and histopathologic findings. Diagn Cytopathol. 1999;20(3):164–6. https://doi.org/10.1002/(sici)1097-0339(199903)20:3%3c164::aid-dc10%3e3.0.co;2-1.
Mokhtari S, Mirafsharieh A. Clear cell chondrosarcoma of the head and neck. Head Neck Oncol. 2012;4:13. https://doi.org/10.1186/1758-3284-4-13.
Hendriks T, Cardemil F, Sader C. Clear cell chondrosarcoma of the larynx. BMJ Case Rep. 2018. https://doi.org/10.1136/bcr-2018-226541.
Slootweg PJ. Clear-cell chondrosarcoma of the maxilla: report of a case. Oral Surg Oral Med Oral Pathol. 1980;50(3):233–7. https://doi.org/10.1016/0030-4220(80)90376-x.
Euteneuer S, Sudhoff H, Stein H, Delling G, Dazert S. Clear cell chondrosarcoma (CCCS) of the nasal septum. Laryngorhinootologie. 2004;83(4):232–5. https://doi.org/10.1055/s-2004-814243.
Kim TJ, Lee AH, Choi YJ, Jung ES, Kim MS, Lee KY. Clear cell chondrosarcoma in the nasal septum. Otolaryngol Head Neck Surg. 2007;137(6):972–3. https://doi.org/10.1016/j.otohns.2007.07.027.
Bjornsson J, Unni KK, Dahlin DC, Beabout JW, Sim FH. Clear cell chondrosarcoma of bone. Observations in 47 cases. Am J Surg Pathol. 1984;8(3):223–30. https://doi.org/10.1097/00000478-198403000-00009.
Han Y, Yang LH, Liu TT, Wang J, Li H, Yu G, Wang Z, Lv JJ, Zhao HY, Wang EH. Liposarcoma of the larynx: report of a case and review of literature. Int J Clin Exp Pathol. 2015;8(1):1068–72.
Golledge J, Fisher C, Rhys-Evans PH. Head and neck liposarcoma. Cancer. 1995;76(6):1051–8. https://doi.org/10.1002/1097-0142(19950915)76:6%3c1051::aid-cncr2820760620%3e3.0.co;2-4.
Wenig BM, Weiss SW, Gnepp DR. Laryngeal and hypopharyngeal liposarcoma. A clinicopathologic study of 10 cases with a comparison to soft-tissue counterparts. Am J Surg Pathol. 1990;14(2):134–41. https://doi.org/10.1097/00000478-199002000-00005.
Wenig BM, Heffner DK. Liposarcomas of the larynx and hypopharynx: a clinicopathologic study of eight new cases and a review of the literature. Laryngoscope. 1995;105(7 Pt 1):747–56. https://doi.org/10.1288/00005537-199507000-00013.
Powitzky R, Powitzky ES, Garcia R. Liposarcoma of the larynx. Ann Otol Rhinol Laryngol. 2007;116(6):418–24. https://doi.org/10.1177/000348940711600605.
Peng KA, Grogan T, Wang MB. Head and neck sarcomas: analysis of the SEER database. Otolaryngol Head Neck Surg. 2014;151(4):627–33. https://doi.org/10.1177/0194599814545747.
Stavrakas M, Nixon I, Andi K, Oakley R, Jeannon JP, Lyons A, McGurk M, Urbano TG, Thavaraj S, Simo R. Head and neck sarcomas: clinical and histopathological presentation, treatment modalities, and outcomes. J Laryngol Otol. 2016;130(9):850–9. https://doi.org/10.1017/S0022215116008604.
Thariat J, Kirova Y, Sio T, Choussy O, Vees H, Schick U, Poissonnet G, Saada E, Thyss A, Miller RC. Mucosal Kaposi sarcoma, a Rare Cancer Network study. Rare Tumors. 2012;4(4):e49. https://doi.org/10.4081/rt.2012.e49.
Mochloulis G, Irving RM, Grant HR, Miller RF. Laryngeal Kaposi’s sarcoma in patients with AIDS. J Laryngol Otol. 1996;110(11):1034–7. https://doi.org/10.1017/s0022215100135698.
Dupont C, Vasseur E, Beauchet A, Aegerter P, Berthe H, de Truchis P, Zucman D, Rouveix E, Saiag P. Long-term efficacy on Kaposi’s sarcoma of highly active antiretroviral therapy in a cohort of HIV-positive patients. CISIH 92. Centre d’information et de soins de l’immunodeficience humaine. AIDS. 2000;14(8):987–93. https://doi.org/10.1097/00002030-200005260-00010.
Bower M, Dalla Pria A, Coyle C, Andrews E, Tittle V, Dhoot S, Nelson M. Prospective stage-stratified approach to AIDS-related Kaposi’s sarcoma. J Clin Oncol. 2014;32(5):409–14. https://doi.org/10.1200/JCO.2013.51.6757.
Khadivi E, Taziky MH, Jafarian AH, Nasseri Sadr M. Laryngeal leiomyosarcoma, a case report and review of articles. Iran J Otorhinolaryngol. 2013;25(73):253–8.
Skoulakis CE, Stavroulaki P, Moschotzopoulos P, Paxinos M, Fericean A, Valagiannis DE. Laryngeal leiomyosarcoma: a case report and review of the literature. Eur Arch Otorhinolaryngol. 2006;263(10):929–34. https://doi.org/10.1007/s00405-006-0092-0.
Marioni G, Bertino G, Mariuzzi L, Bergamin-Bracale AM, Lombardo M, Beltrami CA. Laryngeal leiomyosarcoma. J Laryngol Otol. 2000;114(5):398–401. https://doi.org/10.1258/0022215001905698.
Cambruzzi E, Cruz RP, Gava VG, Pegas KL. Undifferentiated high-grade pleomorphic sarcoma of the larynx treated with partial laringectomy. Braz J Otorhinolaryngol. 2016. https://doi.org/10.1016/j.bjorl.2016.11.005.
Haberal I, Samim E, Astarci M, Ozeri C. Radiation-induced malignant fibrous histiocytoma of the neck in a patient with laryngeal carcinoma. Am J Otolaryngol. 2001;22(2):146–9. https://doi.org/10.1053/ajot.2001.22579.
Karkos PD, Dova S, Sotiriou S, Markou K, Kostopoulos I. Double primary malignant fibrous histiocytoma and squamous cell carcinoma of the larynx treated with laser laryngeal conservation surgery. Ecancermedicalscience. 2016;10:636. https://doi.org/10.3332/ecancer.2016.636.
Sabesan T, Xuexi W, Yongfa Q, Pingzhang T, Ilankovan V. Malignant fibrous histiocytoma: outcome of tumours in the head and neck compared with those in the trunk and extremities. Br J Oral Maxillofac Surg. 2006;44(3):209–12. https://doi.org/10.1016/j.bjoms.2005.06.006.
Soh KB, Westmore GA, Moir AA, Colloby PS. Malignant fibrous histiocytomas of the larynx–report of two cases. Ann Acad Med Singapore. 1996;25(6):878–81.
Kordac P, Nikolov DH, Smatanova K, Kalfert D. Low-grade myofibroblastic sarcoma of the larynx: case report and review of literature. Acta Medica (Hradec Kralove). 2014;57(4):162–4. https://doi.org/10.14712/18059694.2015.82.
Covello R, Licci S, Pichi B, Spriano G, Vidiri A, Morelli L, Rosenberg AE. Low-grade myofibroblastic sarcoma of the larynx. Int J Surg Pathol. 2011;19(6):822–6. https://doi.org/10.1177/1066896910393958.
Qiu X, Montgomery E, Sun B. Inflammatory myofibroblastic tumor and low-grade myofibroblastic sarcoma: a comparative study of clinicopathologic features and further observations on the immunohistochemical profile of myofibroblasts. Hum Pathol. 2008;39(6):846–56. https://doi.org/10.1016/j.humpath.2007.10.010.
Chiang MJ, Fang TJ, Li HY, Chen IH, Lee KF. Malignant granular cell tumor in larynx mimicking laryngeal carcinoma. Am J Otolaryngol. 2004;25(4):270–3. https://doi.org/10.1016/j.amjoto.2004.01.005.
Bradford Bell EJ, Thomas GR, Leibowitz J, Velez Torres JM. Benign and malignant granular cell tumor of the hypopharynx: two faces of a rare entity. Head Neck Pathol. 2020. https://doi.org/10.1007/s12105-020-01157-9.
Aksoy S, Abali H, Kilickap S, Harputluoglu H, Erman M. Metastatic granular cell tumor: a case report and review of the literature. Acta Oncol. 2006;45(1):91–4. https://doi.org/10.1080/02841860500341132.
Fanburg-Smith JC, Meis-Kindblom JM, Fante R, Kindblom LG. Malignant granular cell tumor of soft tissue: diagnostic criteria and clinicopathologic correlation. Am J Surg Pathol. 1998;22(7):779–94. https://doi.org/10.1097/00000478-199807000-00001.
AbdullGaffar B, Keloth T. Laryngeal sarcomas: a case series of 5 cases. Ann Diagn Pathol. 2018;37:35–41. https://doi.org/10.1016/j.anndiagpath.2018.09.007.
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JVT, AER, JADP, and EMD contributed to the study conception and design. Material preparation, data collection, and analysis were performed by JVT, EMD, JL, DW, GT, FC, DA, CGF, and JADP. JVT and AER wrote and edited the manuscript. JVT and AER provided radiographs and micrographs. All authors read and approved the final manuscript.
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Velez Torres, J.M., Martinez Duarte, E., Diaz-Perez, J.A. et al. Primary Sarcomas of the Larynx: A Clinicopathologic Study of 27 Cases. Head and Neck Pathol 15, 905–916 (2021). https://doi.org/10.1007/s12105-021-01314-8
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DOI: https://doi.org/10.1007/s12105-021-01314-8