Bone and soft tissue sarcomas, tumors of the skinBone and soft tissue sarcomas, tumors of the skinСаркомы костей, мягких тканей и опухоли кожи2219-46142782-3687Publishing House ABV Press11510.17650/2070-9781-2021-13-2-18-25SOFT TISSUE SARCOMASСАРКОМЫ МЯГКИХ ТКАНЕЙModern approach to the treatment of sacrococcygeal chordomasСовременный подход к лечению хордом крестцово-копчиковой областиhttps://orcid.org/0000-0003-1395-5132BabkinN. S.БабкинН. С.
Russian Federation

24 Kashirskoe Shosse, Moscow 115478, Russia

Никита Сергеевич Бабкин, Россия, 115478 Москва, Каширское шоссе, д. 24

niksn0072@mail.ruhttps://orcid.org/0000-0002-1241-3019MusaevE. R.МусаевЭ. Р.
Russian Federation

27, Istra Village, Stepanovskoe, Krasnogorskiy Dst., Moscow Region 143423, Russia

Россия, 143423 Московская обл., Красногорский р-н, п/о Степановское, пос. Истра, 27

https://orcid.org/0000-0001-7592-4249BulychevaI. V.БулычеваИ. В.
Russian Federation

24 Kashirskoe Shosse, Moscow 115478, Russia

Россия, 115478 Москва, Каширское шоссе, д. 24

https://orcid.org/0000-0001-9557-3685SofronovD. I.СофроновД. И.
Russian Federation

24 Kashirskoe Shosse, Moscow 115478, Russia

Россия, 115478 Москва, Каширское шоссе, д. 24

ShchipakhinS. A.ЩипахинС. А.
Russian Federation

24 Kashirskoe Shosse, Moscow 115478, Russia

Россия, 115478 Москва, Каширское шоссе, д. 24

https://orcid.org/0000-0002-9019-8369GalustovA. M.ГалустовА. М.
Russian Federation

24 Kashirskoe Shosse, Moscow 115478, Russia

Россия, 115478 Москва, Каширское шоссе, д. 24

N.N. Blokhin National Medical Research Center of Oncology оf the Ministry of Health of RussiaФГБУ «Национальный медицинский исследовательский центр онкологии им. Н.Н. Блохина» Минздрава РоссииMoscow Cancer Hospital No 62, Moscow Healthcare DepartmentГБУЗ «Московская городская онкологическая больница № 62 здравоохранения г. Москвы»2808202113218252808202128082021Copyright ©; 2021, Babkin N.S., Musaev E.R., Bulycheva I.V., Sofronov D.I., Shchipakhin S.A., Galustov A.M.Copyright ©; 2021, Бабкин Н.С., Мусаев Э.Р., Булычева И.В., Софронов Д.И., Щипахин С.А., Галустов А.М.2021Babkin N.S., Musaev E.R., Bulycheva I.V., Sofronov D.I., Shchipakhin S.A., Galustov A.M.Бабкин Н.С., Мусаев Э.Р., Булычева И.В., Софронов Д.И., Щипахин С.А., Галустов А.М.https://creativecommons.org/licenses/by/4.0https://sarbon.abvpress.ru/jour/article/view/115

Chordomas of the sacrococcygeal region account for more than 50 % of all sacral tumors. These malignant neoplasms grow slowly and are asymptomatic for a long time. As a result, chordomas often reach large sizes and affect the neurovascular structures of the sacrum and pelvic organs. The use of en-bloc resection allows to increase survival rates and reduce the risk of progression. However, this method of chord treatment is difficult for surgeons and in most cases, after surgery, the quality of life of patients decreases. The improvement of imaging methods, the success of oncological orthopedics and radiation therapy allow performing radical organ-preserving operations. In this article, we will consider the modern concept of treatment with a sacrococcygeal chord.

Хордомы крестцово-копчиковой области составляют более 50 % всех опухолей крестца. Данные злокачественные новообразования медленно растут и долгое время протекают бессимптомно. Вследствие этого хордомы часто достигают больших размеров и поражают сосудисто-нервные структуры крестца и органы малого таза. Использование en-bloc-резекции позволяет повысить показатели выживаемости и снизить риск прогрессирования. Однако этот метод лечения хордом представляет трудность для хирургов, и в большинстве случаев после хирургического вмешательства качество жизни пациентов снижается. Совершенствование методов визуализации, успехи онкологической ортопедии и лучевой терапии позволяют выполнять радикальные органосохраняющие операции. В этой статье мы рассмотрим современную концепцию лечения хордом крестцово-копчиковой области.

sacral tumorsmalignant tumorschordomaопухоли крестцазлокачественные опухолихордомаAlan O., Telli T.A., Ercelep O. et al. Chordoma: a case series and review of the literature. J Med Case Rep 2018;12(1):239. DOI: 10.1186/s13256-018-1784-y.Stiller C.A., Stiller C.A., Trama A. et al. Descriptive epidemiology of sarcomas in Europe: report from the RARECARE project. Eur J Cancer 2013;49(3):684–95. DOI: 10.1016/j.ejca.2012.09.011.Walcott B.P., Nahed B.V., Mohyeldin A. et al. Chordoma: current concepts, management, and future directions. Lancet Oncol 2012;13(2):e69–e76. DOI: 10.1016/S1470-2045(11)70337-0.Stacchiotti S., Sommer J. Building a global consensus approach to chordoma: a position paper from the medical and patient community. Lancet Oncol 2015;16(2):e71–e83с. DOI: 10.1016/S1470-2045(14)71190-8.Catton C., O’Sullivan B., Bell R. et al. Chordoma: long-term follow-up after radical photon irradiation. Radiother Oncol 1996;41(1):67–72. DOI: 10.1016/s0167-8140(96)91805-8.Chambers P.W., Schwinn C P. Chordoma. A clinicopathologic study of metastasis. Am J Clin Pathol 1979;72(5):765–76. DOI: 10.1093/ajcp/72.5.765.Higinbotham N.L., Phillips R.F., Farr H.W., Hustu H.O. Chordoma.thirty-five-year study at memorial hospital. Cancer 1967;20(11):1841–50. DOI: 10.1002/1097-0142(196711)20:11<1841::aid-cncr2820201107>3.0.co;2-2.Garofalo F., di Summa P.G., Christoforidis D. et al. Multidisciplinary approach of lumbo-sacral chordoma: from oncological treatment to reconstructive surgery. J Surg Oncol 2015;112(5):544–54. DOI: 10.1002/jso.24026.Ropper A.E., Cahill K.S., Hanna J.W. et al. Primary vertebral tumors. Neurosurgery 2012;70(1):211–9. DOI: 10.1227/NEU.0b013e31822d5f17.Samson I.R., Springfield D.S., Suit H.D. et al. Operative treatment of sacrococcygeal chordoma. A review of twenty-one cases. J Bone Jt Surg 1993;75(10):1476–84. DOI: 10.2106/00004623-199310000-00008.Pandey S. Review of Sacrococcygeal chordoma. Available by: http://www.cmc.edu.np/images/gallery/Review%20Article/Y1PojeCQtQreview_article_sacrococygeal.pdf.Farsad K., Kattapuram S.V., Sacknoff R. et al. Best cases from AFIP sacral chordoma. Radiographics 2009;29(5):1525–30. DOI: 10.1148/rg.295085215.Bergh P., Kindblom L.-G., Gunterberg B. et al. Prognostic factors in chordoma of the sacrum and mobile spine: a study of 39 patients. Cancer 2000;88(9):2122–34. DOI: 10.1002/(sici)1097-0142(20000501)88:9<2122::aidcncr19>3.0.co;2-1.Ishii K., Chiba K., Watanabe M. et al. Local recurrence after S2–3 sacrectomy in sacral chordoma. J Neurosurg Spine 2009;97(1):98–101. DOI: 10.3171/spi.2002.97.1.0098.Ozaki T., Hillmann A., Winkelmann W. Surgical treatment of sacrococcygeal chordoma. J Surg Oncol 1997;64(4):274–9. DOI: 10.1002/(sici)1096-9098(199704)64:4<274::aid-jso5>3.0.co;2-6.Gray S.W., Singhabhandhu B., Smith R.A., Skandalakis J.E. Sacrococcygeal chordoma: report of a case and review of the literature. Surgery 1975;78(5):573–82.Kayani B., Hanna S.A., Sewell M.D. A review of the surgical management of sacral chordoma. Eur J Surg Oncol 2014;40(11):1412–20. DOI: 10.1016/j.ejso.2014.04.008.Fenerty K.E., Folio L.R., Patronas N.J. et al. Predicting clinical outcomes in chordoma patients receiving immunotherapy: a comparison between volumetric segmentation and RECIST. BMC Cancer 2016;16(1):672. DOI: 10.1186/s12885-016-2699-x.Gerber S., Ollivier L., Leclère J. et al. Imaging of sacral tumours. Skeletal Radiol 2008;37(4):277–89. DOI: 10.1007/s00256-007-0413-4.Schwab J.H., Boland P.J., Agaram N.P. et al. Chordoma and chondrosarcoma gene profile: Implications for immunotherapy. Cancer Immunol Immunother 2009;58(3):339–49. DOI: 10.1007/s00262-008-0557-7.Murpbey M.D., Andrews C.L., Flemming D.J. et al. From the archives of the AFIP: primary tumors of the spine: radiologic-pathologic correlation. Radiographics 1996;16(5):1131–58. DOI: 10.1148/radiographics.16.5.8888395.Plathow C., Weber M.A., Debus J. et al. Bildgebende diagnostik bei chordomen des beckens: vergleich der modalitäten CT und MRT. Radiologe 2005;45(1):63–8. DOI: 10.1007/s00117-003-1002-8.Stacchiotti S., Gronchi A., Fossati P. et al. Best practices for the management of local-regional recurrent chordoma: a position paper by the Chordoma Global Consensus Group. Ann Oncol 2017;28(6):1230–42. DOI: 10.1093/annonc/mdx054.Pasalic D., Luetmer P.H., Hunt C.H. et al. Benign notochordal cell tumor of the sacrum with atypical imaging features: the value of ct guided biopsy for diagnosis. Open Neuroimag J 2013;30(7):36–40. DOI: 10.2174/1874440001307010036.Reddy P. Diagnostic histopathology of tumours. Med J Armed Forces India 1996;52(3):209. DOI: 10.1016/S0377-1237(17)30830-4.Crapanzano J.P., Ali S.Z., Ginsberg M.S., Zakowski M.F. Chordoma: a cytologic study with histologic and radiologic correlation. Cancer 2001;93(1):40–51.Pallini R., Maira G., Pierconti F. et al. Chordoma of the skull base: Predictors of tumor recurrence. J Neurosurg 2003;98(4):812–22. DOI: 10.3171/jns.2003.98.4.0812.Atalar H., Selek H., Yıldız Y. et al. Management of sacrococcygeal chordomas Int Orthop 2006;30(6):514–8. DOI: 10.1007/s00264-006-0095-x.Fuchs B., Dickey I.D., Yaszemski M.J. et al. Operative management of sacral chordoma. J Bone Joint Surg Am 2005;87(10):2211–6. DOI: 10.2106/JBJS.D.02693.Woodfield J.C., Chalmers A.G., Phillips N., Sagar P.M. Algorithms for the surgical management of retrorectal tumours. Br J Surg 2008;95(2):214–21. DOI: 10.1002/bjs.5931.Chen Y.L., Liebsch N., Kobayashi W. et al. Definitive high-dose photon/proton radiotherapy for unresected mobile spine and sacral chordomas. Spine (Phila. Pa. 1976) 2013;38(15):E930–6. DOI: 10.1097/BRS.0b013e318296e7d7.Devin C., Chong P.Y., Holt G.E. et al. Leveladjusted perioperative risk of sacral amputations. J Surg Oncol 2006;94(3):203–11. DOI: 10.1002/jso.20477.Imai R., Kamada T., Sugahara S., Tsujii H. Carbon ion radiotherapy for sacral chordoma. Br J Radiol 2011;84(1):S48–54. DOI: 10.1259/bjr/13783281.Smith J., Ludwig R.L., Marcove R.C. Sacrococcygeal chordoma: a clinicoradiological study of 60 patients. Skeletal Radiol 1987;16(1):37–44. DOI: 10.1007/BF00349926.Radaelli S., Stacchiotti S., Ruggieri P. et al. Sacral chordoma: long-term outcome of a large series of patients surgically treated at two reference centers. Spine (Phila. Pa. 1976) 2016;1(12):1049–57. DOI: 10.1097/BRS.0000000000001604.Ruggieri P., Angelini A., Ussia G. et al. Surgical margins and local control in resection of sacral chordomas. Clin Orthop Relat Res 2010;468(1):2939–47. DOI: 10.1007/s11999-010-1472-8.Xie C., Whalley N., Adasonla K. et al. Can local recurrence of a sacral chordoma be treated by further surgery? Bone Jt J 2015;97-B(5):711–5. DOI: 10.1302/0301-620X.97B5.35131.Baratti D., Gronchi A., Pennacchioli E. et al. Chordoma: natural history and results in 28 patients treated at a single institution Ann Surg Oncol 2003;10(3):291–6. DOI: 10.1245/aso.2003.06.002.Wulfften O.D.R. van, Tromp I., Ferreira A. et al. Sacral chordoma: a clinical review of 101 cases with 30-year experience in a single institution. Spine J 2019;19(5):869–79. DOI: 10.1016/j.spinee.2018.11.002.Hanna S.A., Aston W.J.S., Briggs T.W.R. et al. Sacral chordoma: can local recurrence after sacrectomy be predicted? Clin Orthop Relat Res 2008;466(9):2217–23. DOI: 10.1007/s11999-008-0356-7.Safwat A., Nielsen O.S., Jurik A.G. et al. A retrospective clinicopathological study of 37 patients with chordoma: a Danish national series. Sarcoma 1997;1(3–4):161–5. DOI: 10.1080/13577149778254.Angelini A., Pala E., Calabrò T. et al. Prognostic factors in surgical resection of sacral chordoma J Surg Oncol 2015;112(4):344–51. DOI: 10.1002/jso.23987.Conlon K.C., Boland P.J. Laparoscopically assisted radical sacrococcygectomy: a new operative approach to large sacrococcygeal chordomas. Surg Endosc 1997;11(11):1118–22. DOI: 10.1007/s004649900544.Gennari L., Azzarelli A., Quagliuolo V. A posterior approach for the excision of sacral chordoma. J Bone Jt Surg Ser B. 1987;69(4):565–8. DOI: 10.1302/0301-620X.69B4.3611160.Park L., DeLaney T.F., Liebsch N.J. et al. Sacral chordomas: impact of high-dose proton/photon-beam radiation therapy combined with or without surgery for primary versus recurrent tumor. Int J Radiat Oncol Biol Phys 2006;65(5):1514–21. DOI: 10.1016/j.ijrobp.2006.02.059.Schulz-Ertner D., Karger C.P., Feuerhake A. et al. Effectiveness of carbon ion radiotherapy in the treatment of skull-base chordomas. Int J Radiat Oncol Biol Phys 2007;68(2):449–57. DOI: 10.1016/j.ijrobp.2006.12.059.Boriani S. Chordoma of the mobile spine: fifty years of experience Spine (Phila. Pa. 1976) 2006;31(4):493–503. DOI: 10.1097/01.brs.0000200038.30869.27.DeLaney T.F., Liebsch N.J., Pedlow F.X. et al. Phase II study of high-dose photon/proton radiotherapy in the management of spine sarcomas. Int J Radiat Oncol Biol Phys 2009;74(3):732–9. DOI: 10.1016/j.ijrobp.2008.08.058.Jian B.J., Bloch O.G., Yang I. et al. Adjuvant radiation therapy and chondroid chordoma subtype are associated with a lower tumor recurrence rate of cranial chordoma. J Neurooncol 2010;98:(1):101–8. DOI: 10.1007/s11060-009-0068-1.Moojen W.A., Vleggeert-Lankamp C.L.A., Krol A.D.G., Dijkstra S.P.D. Long-term results: adjuvant radiotherapy in en bloc resection of sacrococcygeal chordoma is advisable. Spine (Phila. Pa. 1976) 2011;36(10):E656–61. DOI: 10.1097/BRS.0b013e3181f8d1f3.Ares C., Hug E.B., Lomax A.J. et al. Effectiveness and safety of spot scanning proton radiation therapy for chordomas and chondrosarcomas of the skull base: first long-term report. Int J Radiat Oncol Biol Phys 2009;75(4):1111–8. DOI: 10.1016/j.ijrobp.2008.12.055.Tamborini E., Virdis E., Negri T. et al. Analysis of receptor tyrosine kinases (RTKs) and downstream pathways in chordomas. Neuro Oncol 2010;12(8):776–89. DOI: 10.1093/neuonc/noq003.Stacchiotti S., Longhi A., Ferraresi V. et al. Phase II study of imatinib in advanced chordoma. J Clin Oncol 2012;30(9):914–20. DOI: 10.1200/JCO.2011.35.3656.Magrini S.M., Papi M.G., Marletta F. et al. Chordoma-natural history, treatment and prognosis the Florence Radiotherapy Department Experience (1956–1990) and a critical review of the literature. Acta Oncol (Madr) 1992:31(8):847–51. DOI: 10.3109/02841869209089717.York J.E., Kaczaraj A., Abi-Said D. et al. Sacral chordoma: 40-year experience at a major cancer center. Neurosurgery 1999;44(1):74–80. DOI: 10.1097/00006123-199901000-00041.