A case of tracheal chondrosarcoma

Background. Chondrosarcoma of the trachea is an extremely rare malignant tumor, accounting for only 0.1 % of all tracheal tumors. It is believed that chondrosarcoma arises from malignant degeneration in mesenchymal pluripotent cells of cartilage tissue, ossified cartilage, and enchondromas. Chondrosarcoma of the trachea is the least-studied tumor, with only 35 cases described in the literature from 1959 to 2020. Surgery with R0 resection remains the gold standard treatment of tracheal chondrosarcoma. The median age of patients at the time of diagnosis was 68 years, and 91 % were male. The most common symptoms at the initial visit were dyspnea (up to 80 % of cases). Tumors located in the proximal third of the trachea were observed in 54 % of cases, while tumors in the distal third of the trachea were found in 29 % of cases. Low-grade tumors (g1) were the most common (54 %). Computed tomography of the chest is considered the gold standard for the detection of tracheal tumors. Purpose: To demonstrate a successful case of treatment of tracheal chondrosarcoma. Case presentation. A 67-year-old male patient was diagnosed with a tracheal tumor. On 04.07.2022, surgical treatment was performed: circular resection of the 2nd, 3rd, and 4th tracheal rings with lymph node dissection of the regional paratracheal lymph nodes R0. The postoperative period was uneventful. Adjuvant therapy was not given. At the 2-year follow-up, no anastomotic complications were reported. Conclusion. To date, circular resection of the trachea (R0) with end-to-end tracheal anastomosis is the gold standard for treating tracheal chondrosarcoma. this approach was performed in 77 % of cases with the median follow-up time of more than 30 months and relapse rate of only 6 %. It should be noted that adjuvant chemotherapy was not used in these cases due to the extremely low sensitivity of the tumor to chemotherapy drugs.

1. Mohajeri G., Hekmatnia A., Ahrar H., Hekmatnia F., Nia R.B., Afsharmoghadam N., Eftekhari M., Jafarpishe S. A rare case of laryngotracheal chondrosarcoma in a patient with past history of radioiodine therapy for thyroid cancer. Adv Biomed Res. 2013; 2: 74. doi: 10.4103/2277-9175.115802.

2. Maia D., Elharrar X., Laroumagne S., Maldonado F., Astoul P., Dutau H. Malignant transformation of a tracheal chondroma: The second reported case and review of the literature. Rev Port Pneumol (2006). 2016; 22(5): 283–86. doi: 10.1016/j.rppnen.2016.04.003.

3. Herget G.W., Strohm P., Rottenburger C., Kontny U., Krauß T., Bohm J., Sudkamp N., Uhl M. Insights into Enchondroma, Enchondromatosis and the risk of secondary Chondrosarcoma. Review of the literature with an emphasis on the clinical behaviour, radiology, malignant transformation and the follow up. Neoplasma. 2014; 61(4): 365–78. doi: 10.4149/neo_2014_046.

4. Nazeri E., Gouran Savadkoohi M., Majidzadeh-A K., Esmaeili R. Chondrosarcoma:An overview of clinical behavior, molecular mechanisms mediated drug resistance and potential therapeutic targets. Crit Rev Oncol Hematol. 2018; 131: 102–9. doi: 10.1016/j.critrevonc.2018.09.001.

5. Amyere M., Dompmartin A., Wouters V., Enjolras O., Kaitila I., Docquier P.L., Godfraind C., Mulliken J.B., Boon L.M., Vikkula M. Common somatic alterations identified in maffucci syndrome by molecular karyotyping. Mol Syndromol. 2014; 5(6): 259–67. doi: 10.1159/000365898.

6. Wagnetz U., Patsios D., Darling G., Las Heras F., Hwang D. Tracheal chondrosarcoma-a rare complication in Maffucci syndrome. Br J Radiol. 2009; 82(981): 178–81. doi: 10.1259/bjr/17386896.

7. de Almeida J.R., Pagedar N.A., Keshavjee S., Gilbert R. Chondrosarcoma of the trachea in a patient with Maffucci syndrome. J Otolaryngol Head Neck Surg. 2010; 39(2).

8. Daniels A.C., Conner G.H., Straus F.H. Primary chondrosarcoma of the tracheobronchial tree. Report of a unique case and brief review. Arch Pathol. 1967; 84(6): 615–24.

9. Fallahnejad M., Harrell D., Tucker J., Forest J., Blakemore W.S. Chondrosarcoma of the trachea. Report of a case and five-year follow-up. J Thorac Cardiovasc Surg. 1973; 65(2): 210–13.

10. Weber A.L., Shortsleeve M., Goodman M., Montgomery W., Grillo H.C. Cartilaginous tumors of the larynx and trachea. Radiol Clin North Am. 1978; 16(2): 261–67.

11. Slasky B.S., Hardesty R.L., Wilson S. Tracheal chondrosarcoma with an overview of other tumors of the trachea. J Comput Tomogr. 1985; 9(3): 225–31. doi: 10.1016/0149-936x(85)90066-9.

12. Matsuo T., Kinoshita S., Iwasaki K., Shibata M., Ushio T., Kawata S., Gotanda T. Chondrosarcoma of the trachea. A case report and literature review. Acta Cytol. 1988; 32(6): 908–12.

13. Ryabov A., Pikin O., Sokolov V., Volchenko N. Primary chondrosarcoma of the trachea with extensive extraluminal growth. Eur J Cardiothorac Surg. 2017; 52(3): 601–3. doi: 10.1093/ejcts/ezx118.

14. Farrell M.L., Gluckman J.L., Biddinger P. Tracheal chondrosarcoma: a case report. Head Neck. 1998; 20(6): 568–72. doi: 10.1002/(sici)1097-0347(199809)20:6<568::aid-hed13>3.0.co;2-d.

15. Kiriyama M., Masaoka A., Yamakawa Y., Niwa H., Fukai I., Yamada T. Chondrosarcoma originating from the trachea. Ann Thorac Surg. 1997; 63(6): 1772–73. doi: 10.1016/s0003-4975(97)00331-7.

16. Heuermann M., Bekker S., Czeczok T., Gregory S., Sharma A. Tracheal chondrosarcoma: A case report, systematic review, and pooled analysis. Cancer Rep (Hoboken). 2022; 5(7). doi: 10.1002/cnr2.1537.

17. Maish M., Vaporciyan A.A. Chondrosarcoma arising in the trachea: a case report and review of the literature. J Thorac Cardiovasc Surg. 2003; 126(6): 2077–80. doi: 10.1016/s0022-5223(03)00949-8.

18. Mirza F., Paul S., Stiles B.M., Piotti K.C., Kawaguchi K.R., Altorki N.K. Adult onset asthma. Ann Thorac Surg. 2010; 90(2). doi: 10.1016/j.athoracsur.2010.05.040.

19. Kutzner E.A., Park J.S., Zaheer S., Inman J.C. Tracheal Chondrosarcoma: Systematic Review of Tumor Characteristics, Diagnosis, and Treatment Outcomes with Case Report. Case Rep Oncol Med. 2017. doi: 10.1155/2017/4524910.

20. Grillo H.C. Surgery of the Trachea and Bronchi. Hamilton, London: BC Decker Inc, 2004. 872 p.

21. Andolfi M., Vaccarili M., Crisci R., Puma F. Management of tracheal chondrosarcoma almost completely obstructing the airway: a case report. J Cardiothorac Surg. 2016; 11(1): 101. doi: 10.1186/s13019-016-0498-8.

22. Carretta A., Ciriaco P., Bandiera A., Muriana P., Arrigoni G., Negri G.Therapeutic strategy for tracheal chondrosarcoma: report of two cases. Monaldi Arch Chest Dis. 2020; 90(1). doi: 10.4081/monaldi.2020.1223.

23. Daddi G., Puma F., Avenia N., Santoprete S., Casadei S., Urbani M. Resection with curative intent after endoscopic treatment of airway obstruction. Ann Thorac Surg. 1998; 65(1): 203–7. doi: 10.1016/s0003-4975(97)01124-7.

24. Mathisen D.J. Tracheal Resection and Reconstruction: How I Teach It. Ann Thorac Surg. 2017; 103(4): 1043–48. doi: 10.1016/j.athoracsur.2016.12.057.

25. Barisione E., Rossi G.A., Salio M. Gradually worsening dyspnea and dry cough in an elderly patient. Monaldi Arch Chest Dis. 2017; 87(2): 834. doi: 10.4081/monaldi.2017.834.

26. Catanzano A.A., Kerr D.L., Lazarides A.L., Dial B.L., Lane W.O., Blazer D.G., Larrier N.A., Kirsch D.G., Brigman B.E., Eward W.C. Revisiting the Role of Radiation Therapy in Chondrosarcoma: A National Cancer Database Study. Sarcoma. 2019. doi: 10.1155/2019/4878512.

27. Radzhabova Z.A., Kotov M.A., Mitrophanov A.S., Ponomareva O.I., Radzhabova M.A., Levchenko E.V. Chondrosarcoma of trachea: case report of successful surgical treatment of local recurrence. Head and Neck Tumors (HNT). 2019; 9(2): 95–98. (in Russian). doi: 10.17650/2222-1468-2019-9-2-95-98. EDN: JZNCGQ.

28. Mendonça V., Jorge M., Monteiro-Grillo I., Palhano M.J., Feijó S. Tracheal chondrosarcoma. Clin Transl Oncol. 2010; 12(8): 576–80. doi: 10.1007/s12094-010-0557-x.

29. Italiano A., Mir O., Cioffi A., Palmerini E., Piperno-Neumann S., Perrin C., Chaigneau L., Penel N., Duffaud F., Kurtz J.E., Collard O., Bertucci F., Bompas E., Le Cesne A., Maki R.G., Ray Coquard I., Blay J.Y. Advanced chondrosarcomas: role of chemotherapy and survival. Ann Oncol. 2013; 24(11): 2916–22. doi: 10.1093/annonc/mdt374.

30. Salminen U.S., Halttunen P., Taskinen E., Mattila S. Recurrence and malignant transformation of endotracheal chondroma. Ann Thorac Surg. 1990; 49(5): 830–32. doi: 10.1016/0003-4975(90)90039-9.