Pediatric Hematology/Oncology and ImmunopathologyPediatric Hematology/Oncology and ImmunopathologyВопросы гематологии/онкологии и иммунопатологии в педиатрии1726-17082414-9314Fund Doctors, Innovations, Science for Children110310.24287/j.1103ORIGINAL ARTICLESОРИГИНАЛЬНЫЕ СТАТЬИResearch ArticleEvaluation of the radicality of surgical treatment of pediatric patients with osteosarcoma of the maxillofacial region in childrenОценка радикальности хирургического лечения пациентов с остеосаркомой челюстно-лицевой области у детейhttps://orcid.org/0000-0002-2607-9795ChechevE. I.ЧечевЕ. И.
Russian Federation

Pediatric Oncologist

врач-детский онколог

e.i.chechev@gmail.comhttps://orcid.org/0000-0002-3932-6257VorozhtsovI. N.ВорожцовИ. Н.
Russian Federation
e.i.chechev@gmail.comhttps://orcid.org/0000-0001-7600-6191LopatinA. V.ЛопатинА. В.
Russian Federation
e.i.chechev@gmail.comhttps://orcid.org/0000-0003-4264-1423BabaskinaN. V.БабаскинаН. В.
Russian Federation
e.i.chechev@gmail.comhttps://orcid.org/0000-0002-6848-8714KonoplevaE. I.КоноплеваЕ. И.
Russian Federation
e.i.chechev@gmail.comhttps://orcid.org/0000-0003-3650-0309ChuykoY. M.ЧуйкоЯ. М.
Russian Federation
e.i.chechev@gmail.comhttps://orcid.org/0000-0002-4451-3233GrachevNikolay SergeevichГрачевНиколай Сергеевич
Russian Federation
e.i.chechev@gmail.comThe Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology of Ministry of Healthcare of the Russian FederationФГБУ «Национальный медицинский исследовательский центр детской гематологии, онкологии и иммунологии им. Дмитрия Рогачева» Минздрава РоссииRussian Biotechnological University (ROSBIOTECH)ФГБОУ ВО «Российский биотехнологический университет (РОСБИОТЕХ)The I.M. Sechenov First Moscow State Medical University of the Ministry of Healthcare of the Russian Federation (the Sechenov University)ФГАОУ ВО «Первый Московский государственный медицинский университет им. И.М. Сеченова» Минздрава России (Сеченовский Университет)140420262511391442003202620032026Copyright ©; 2026, «D. Rogachev NMRCPHOI»Copyright ©; 2026, ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава России2026«D. Rogachev NMRCPHOI»ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава Россииhttps://creativecommons.org/licenses/by/4.0https://hemoncim.com/jour/article/view/1103

Introduction. Osteosarcoma of the maxillofacial region is an extremely rare variant of osteosarcoma, accounting for no more than 6–9% of all cases. Unlike tumors located in the bones of the extremities, osteosarcoma of the maxillofacial region is characterized by a lower tendency for distant metastasis. Due to the specific location of the primary lesion in the maxillofacial region, namely the proximity to vital structures, radical surgical intervention is associated with a number of difficulties; however, it is a key element of treatment that determines the prognosis of the disease.

Materials and methods. The study included patients under the age of 18 years with a verified osteosarcoma of the maxillofacial region, registered between 2012 and 2024 at the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology of the Ministry of Healthcare of the Russian Federation. Treatment for high-grade osteosarcoma patients was conducted according to the MAP regimen (based on the EURAMOS-1 protocol). Treatment for low-grade osteosarcoma patients was limited to surgical resection of the primary lesion.

Results. The study included 11 patients (6 boys and 5 girls) with a median age of 11 years. Seven tumors (63.6%) were located in the mandible and four tumors in the maxilla (36.4%). A histological examination revealed 9 (81.9%) cases of high-grade osteosarcoma and 2 (18.1%) cases of low-grade osteosarcoma. The stage distribution was as follows: stage IA, 18.1%; stage IIA, 63.6%; stage IIB, 9.0%; stage III, 9.0%. Local surgical control was performed in all the patients: R0 resection was achieved in 9 (81.9%) cases and R1 in 2 (18.1%) cases. The median follow-up was 7 years. The event-free survival was 91% at 1 year and 64% at 3 years and at 5 years. The overall survival was 100% at 1 year, 82% at 3 years, and 73% at 5 years.

Conclusion. The cornerstone of treatment for patients diagnosed with osteosarcoma is radical surgery. In patients, for whom radical resection is not feasible, survival approaches zero.

Введение. Остеосаркома челюстно-лицевой области (ЧЛО) представляет собой исключительно редкий вариант остеосаркомы, на долю которого приходится не более 6–9% всех случаев. В отличие от опухолей, расположенных в костях конечностей, остеосаркома ЧЛО характеризуется меньшей склонностью к отдаленному метастазированию. Ввиду особенностей расположения первичного очага в ЧЛО, а именно близости жизненно важных структур, проведение радикального хирургического вмешательства сопряжено с рядом трудностей, однако является ключевым и определяющим прогноз заболевания элементом лечения.

Материалы и методы. В исследование включены пациенты с верифицированной остеосаркомой ЧЛО в возрасте до 18 лет, зарегистрированные в период с 2012 по 2024 г. в ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава России. Терапия пациентов с остеосаркомой высокой степени злокачественности (high-grade) проводилась по схеме MAP (на основе режима EURAMOS-1). Лечение пациентов с остеосаркомой низкой степени злокачественности (low-grade) ограничивалось резекцией первичного очага.

Результаты. В исследование вошли 11 пациентов (6 мальчиков, 5 девочек), медиана возраста составила 11 лет. Локализация опухолей была следующей: нижняя челюсть – 7 (63,6%), верхняя – 4 (36,4%). Гистология: high-grade – 9 (81,9%), low-grade – 2 (18,1%). Распределение по стадиям: IA – 18,1%, IIA – 63,6%, IIB – 9,0%, III – 9,0%. Всем пациентам выполнен хирургический локальный контроль: R0-резекция достигнута в 9 (81,9%) случаях, R1 – в 2 (18,1%). Медиана наблюдения составила 7 лет. Бессобытийная выживаемость: 91% (1 год), по 64% (3 и 5 лет). Общая выживаемость: 100% (1 год), 82% (3 года), 73% (5 лет).

Заключение. Ключевым элементом лечения пациентов с установленным диагнозом остеосаркомы является радикальное хирургическое вмешательство. В случае невозможности его выполнения выживаемость пациентов стремится к нулю.

pediatric oncologypediatric surgerymaxillofacial surgerypediatricsдетская онкологиядетская хирургиячелюстно-лицевая хирургияпедиатрияKontio R., Hagström J., Lindholm P., Böhling T., Sampo M., Mesimäki K. et al. Craniomaxillofacial osteosarcoma – the role of surgical margins. J craniomaxillofac Surg 2019;47(6):922–5. DOI: 10.1016/j.jcms.2019.03.020Bertoni F., Dallera P., Bacchini P., Marchetti C., Campobassi A. The Istituto Rizzoli–Beretta experience with osteosarcoma of the jaw. Cancer 1991;68(7):1555–63. DOI: 10.1002/1097-0142(19911001)68:7 < 1555::aid-cncr2820680717> 3.0.co;2Guadagnolo B.A., Zagars G.K., Raymond A.K., Benjamin R.S., Sturgis E.M. Osteosarcoma of the jaw/craniofacial region: outcomes after multimodality treatment. Cancer 2009;115(14):3262–70. DOI: 10.1002/cncr.24297Smeele L.E., van der Wal J.E., van Diest P.J., van der Waal I., Snow G.B. Radical surgical treatment in craniofacial osteosarcoma gives excellent survival. A retrospective cohort study of 14 patients. Eur J Cancer B Oral Oncol 1994;30B(6):374–6. DOI: 10.1016/0964-1955(94)90014-0König M., Osnes T.A., Lobmaier I., Bjerkehagen B., Bruland Ø.S., Sundby Hall K., Meling T.R. Multimodal treatment of craniofacial osteosarcoma with high-grade histology. A single-center experience over 35 years. Neurosurg Rev 2017;40(3):449–60. DOI: 10.1007/s10143-016-0802-zFerrari D., Codecà C., Battisti N., Broggio F., Crepaldi F., Violati M. et al. Multimodality treatment of osteosarcoma of the jaw: a single institution experience. Med Oncol 2014;31(9):171. DOI: 10.1007/s12032-014-0171-9Amin M.B., Greene F.L., Edge S.B., Compton C.C., Gershenwald J.E., Brookland R.K. et al. 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 Cancer J Clin 2017;67(2):93–9. DOI: 10.3322/caac.21388Meazza C., Parisi F., Barretta F., Sironi G., Nigro O., Morosi C. et al. Pediatric craniofacial osteosarcoma: the Milano experience. Pediatr Blood Cancer 2025;72(8):e31808. DOI: 10.1002/pbc.31808Amrollahie A., Mai P.T., Portnof J., Kaltman S.I. Primary intramedullary osteosarcoma of the maxilla in a pediatric patient: a case report and review of the literature. Clin Surg 2018;3:2022.Glajzer J., Lutz R., Nobis C.-P., Olmos M., Buchbender M., Möst T. et al. Maxillofacial osteosarcoma – report of two cases with presentation of a two-staged reconstructive strategy and dental rehabilitation. Authorea 2023;09. DOI: 10.22541/au.169688247. 70133043/v1Dhima M., Arce K., Moore E.J., Rieck K.L., Salinas T.J. Novel oncologic, surgical, and prosthetic treatment of high-grade surface osteosarcoma, osteoblastic mandible type. J Oral Maxillofac Surg 2013;71(5):e224–31. DOI: 10.1016/j.joms.2012.11.003Utyuzh A.S., Yumashev A.V., Lang H.W., Zekiy A.O., Lushkov R.M. Comprehensive treatment and rehabilitation of patients with osteosarcoma of the mandible. Implant Dent 2018;27(3):332–41. DOI: 10.1097/ID.0000000000000756Ferrari D., Moneghini L., Allevi F., Bulfamante G., Biglioli F. Osteosarcoma of the jaw: classification, diagnosis and treatment. In: Osteosarcoma – biology, behavior and mechanisms. InTech, 2017. DOI: 10.5772/67564MHuvos A.G. Osteogenic sarcoma: pathologic assessment of preoperative (neoadjuvant) chemotherapy. In: Bone tumors: diagnosis, treatment and prognosis. 2nd ed. Philadelphia,1991. Рр. 122–8.Thiele O.C., Freier K., Bacon C., Egerer G., Hofele C.M. Interdisciplinary combined treatment of craniofacial osteosarcoma with neoadjuvant and adjuvant chemotherapy and excision of the tumour: a retrospective study. Br J Oral Maxillofac Surg 2008;46(7):533–6. DOI: 10.1016/j.bjoms.2008.03.010Kimura Y., Tomihara K., Tachinami H., Imaue S., Nakamori K., Fujiwara K. et al. Conventional osteosarcoma of the mandible successfully treated with radical surgery and adjuvant chemotherapy after responding poorly to neoadjuvant chemotherapy: a case report. J Med Case Rep 2017;11(1):210. DOI: 10.1186/s13256-017-1386-0Viganò L., Bettoni E., Salina F., Damilano M., Sciarra М. et al. Osteosarcoma of the jaw: radiographic evidences and differential diagnosis. Scholars Journal of Dental Sciences 2020;7:147–55. DOI: 10.36347/sjds.2020.v07i10.002Maldonado A.R., Spratt J.S. Osteogenic sarcoma of the mandible and maxilla. South Med J 1986;79(11):1453–5. DOI: 10.1097/00007611-198611000-00034Kaemmerer P., Shabazfar N., Makoie N., Moergel M., Al-Nawas B. Clinical, therapeutic and prognostic features of osteosarcoma of the jaws – experience of 36 cases. J Craniomaxillofac Surg 2011;40:541–8. DOI: 10.1016/j.jcms.2011.10.001Astl J., Belsan T., Michnova L., Kubeš J., Filipovsky T., Blecha J. et al. Highly aggressive osteosarcoma of the ethmoids and maxillary sinus – a case of successful surgery and proton beam radiotherapy in a 65-year-old man. Medicina (Kaunas) 2022;58(9):1141. DOI: 10.3390/medicina58091141Hadley C., Gressot L., Patel A., Wang L., Flores R., Whitehead W. et al. Osteosarcoma of the cranial vault and skull base in pediatric patients: report of 3 cases. J Neurosurg Pediatr 2014;13(4):380–7. DOI: 10.3171/2013.12.PEDS13359Ramirez A.L., Townsend A.N., Weber L., Piccinini P.S., Wolfe E.M., Taylor M.W. et al. An oncoplastic approach to primary pediatric pterygomaxillary osteosarcoma. Cleft Palate Craniofac J 2024;61(6):1054–60. DOI: 10.1177/10556656231154814Noguchi T., Sugiura Y., Okada N., Tsuchiya Y., Hyasaka J.I., Sasaguri K.I. et al. A modified preauricular and transmandibular approach for surgical management of osteosarcoma of the mandibular condyle within the masticator space and infratemporal fossa: a case report. J Med Case Rep 2019;13(1):58. DOI: 10.1186/s13256-019-1975-1Cassoni A., Brauner E., Pucci R., Terenzi V., Mangini N., Battisti A. et al. Head and neck osteosarcoma – the ongoing challenge about reconstruction and dental rehabilitation. Cancers (Basel) 2020;12(7):1948. DOI: 10.3390/cancers12071948Грачев Н.С., Лопатин А.В., Бабаскина Н.В., Марков Н.М., Ворожцов И.Н., Старокорова К.Д. и др. Комбинированная реконструкция нижней челюсти у детей с использованием индивидуального эндопротеза с искусственным височно-нижнечелюстным суставом и пластикой свободным малоберцовым лоскутом. Вопросы гематологии/онкологии и иммунопатологии в педиатрии 2025;24(1):138–43. DOI: 10.24287/1726-1708-943-2151 [Grachev N.S., Lopatin A.V., Babaskina N.V., Markov N.M., Vorozhtsov I.N., Starokorova K.D. et al. Combined reconstruction of the mandible in children using a customized endoprosthesis with a temporomandibular joint implant and fibula free ftap reconstruction. Pediatric Hematology/Oncology and Immunopathology 2025;24(1):138–43. (In Russ.)].Clark J.L., Unni K.K., Dahlin D.C., Devine K.D. Osteosarcoma of the jaw. Cancer 1983;51(12):2311–6. DOI: 10.1002/1097-0142(19830615)51:12< 2311::aid- cncr2820511224> 3.0.co;2-zHerrmann A., Zöller J. Zur klinik und therapie des osteogenen sarkoms in Kieferbereich. Clinical features and treatment of osteogenic sarcoma of the jaws. Dtsch Z Mund Kiefer Gesichtschir. 1990;14(3):180–6. [In German].Russ J., Jesse R. Management of osteosarcoma of the maxilla and mandible. Am J Surg 1980;140(4):572–6. DOI: 10.1016/0002-9610(80)90215-9Tyndall R.G., Popescu O.E., Derek P., Steinbok P. Osteogenic sarcoma of the skull: long-term outcome of a rare tumor. Childs Nerv Syst 2018;34(11):2149–53. DOI: 10.1007/s00381-018-3937-0Lewis M., Perl A., Som P.M., Urken M.L., Brandwein M.S. Osteogenic sarcoma of the jaw. A clinicopathologic review of 12 patients. Arch Otolaryngol Head Neck Surg 1997;123(2):169–74. DOI: 10.1001/archotol.1997.01900020051007Ketabchi A., Kalavrezos N., Newman L. Sarcomas of the head and neck: a 10-year retrospective of 25 patients to evaluate treatment modalities, function and survival. Br J Oral Maxillofac Surg 2011;49(2):116–20. DOI: 10.1016/j.bjoms.2010.02.012Румянцев А.Г., Варфоломеева С.Р., Грачев Н.С., Карачунский А.И., Новичкова Г.А. Принципы и инструменты доказательной медицины в детской гематологии/онкологии. Доктор.Ру 2015;10(111):6–13. [Rumyantsev A.G., Varfolo-meeva S.R., Karachunskiy A.I., Grachev N.S., Novichkova G.A. The principles and tools of evidence-based medicine in pediatric hematology/oncology. Doctor.Ru 2015;10(111):6–13. (In Russ.)].