Abstract and keywords
Abstract (English):
The paper presents the results of a clinical observation of a 6-year-old female patient, who was admitted to the department of maxillofacial surgery of a multidisciplinary hospital under the compulsory health insurance system with a rare disease of the bone tissue of the lower jaw – desmoplastic fibroma. Desmoplastic fibroma of bone tissue (aggressive fibromatosis, desmoid fibroma) is a rare, locally aggressive, benign tumor localized in bone tissue, it is a mesenchymal fibroproliferative tumor with locally destructive growth, accounting for less than 0.03% of all human tumors. According to the literature, desmoplastic fibroma has a recurrence rate of 37% to 72%, resection of the affected bone is the preferred therapy, and, ideally, resection should be a single resection block. Wide excision is the method of choice for treating operable desmoid tumors in children. The article presents a dynamic observation within 1 year after surgical treatment. The article presents the clinical, radiological and computed tomographic picture of the disease and the results of instrumental and histological examination of a rare oncostomatological pathology. Purpose: clinical, radiological, 3D computed tomography observation of a child with desmoplastic fibroma of the lower jaw. Materials and methods: the results of a clinical examination, instrumental methods of X-ray diagnostics, stages of surgical treatment, a histological report and photographs of the patient's appearance are presented. Conclusion: Given the high regenerative capacity of bone tissue in children, it is necessary to perform surgical treatment of benign neoplasms by the type of marginal resection, that is, by the organ-preserving type, and to conduct dispensary observation of the patient during the first year.

maxillofacial surgery, desmoid fibromatosis, desmoid, neoplasm of the maxillofacial region, X-ray diagnostics

1. Dormans J.P., Spiegel D., Meyer J. et al. Fibromatoses in childhood: the desmoid fibromatosis complex // Med Pediatr Oncol. – 2001;37:126-131. doi: 10.1002/mpo.1181.

2. Horov O.G., Nikita E.I., Zagorovskaya O.V., Novikov A.G., Nechiporenko A.S. Desmoidnaya fibroma polosti nosa i okolonosovyh pazuh. Opisanie klinicheskogo sluchaya. Otorinolaringologiya. Vostochnaya Evropa. 2020;10(3):258-267. [O.G. Khorov, E.I. Nikita, O.V. Zagorovskaya, A.G. Novikov, A.S. Nechiporenko. Desmoid fibroma of the nasal cavity and paranasal sinuses. Description of the clinical case. Otorhinolaryngology. Eastern Europe. 2020;10(3):258-267. (In Russ.)].

3. Ismailzade R.S., Tarasik A.O., Shimanskiy A.T., Kochubinskiy D.V. Desmoidnye opuholi u detey. Onkopediatriya. 2015;2(2):131-138. [R.S. Ismailzade, A.O. Tarasik, A.T. Shimansky, D.V. Kochubinsky. Desmoid tumors in children. Oncopediatrics. 2015;2(2):131-138. (In Russ.)].

4. Lutkov M.A., Lazarev A.Yu. Ekstraabdominal'nyy fibromatoz (desmoid) osnovaniya cherepa: klinicheskoe nablyudenie. Ural'skiy medicinskiy zhurnal. 2015;10(133):55-58. [M.A. Lutkov, A.Yu. Lazarev. Extra-abdominal fibromatosis (desmoid) of the skull base: a clinical case. Ural medical journal. 2015;10(133):55-58. (In Russ.)].

5. Dubova E.A., Sidorenko T.V., Shchegolev A.I., Adamyan A.A. Immuhistochemical profile of primary and recurrent desmoids // Virchows Archiv-European Journal of Pathology. – 2012;461(S1):226.

6. Trigolos N.N., Makedonova Yu.A., Firsova I.V. i dr. Cementno-kostnaya displaziya chelyustey. Sovremennye problemy nauki i obrazovaniya. 2015;3. [N.N. Trigolos, Yu.A. Makedonova, I.V. Firsova et al. Cement-bone dysplasia of the jaws. Modern problems of science and education. 2015;3. (In Russ.)].

7. Yadrina A.V., Karpenko V.Yu., Novikova O.V., Bychkova N.M., Derzhavin V.A., Buharov A.V. Prognosticheskie faktory recidivov pri hirurgicheskom lechenii desmoidnyh fibrom tulovischa i konechnostey. Issledovaniya i praktika v medicine. 2019;6;1:21-32. [A.V. Yadrina, V.Yu. Karpenko, O.V. Novikova, N.M. Bychkova, V.A. Derzhavin, A.V. Bukharov. Prognostic factors of recurrence in the surgical treatment of desmoid fibroids of the trunk and extremities. Research and practice in medicine. 2019;6;1:21-32. (In Russ.)].

8. Yadrina A.V., Bolotina L.V., Novikova O.V., Karpenko V.Yu., Bychkova N.M., Kostrygin A.K., Sychenkova I.Yu. Opyt primeneniya doksorubicina i dakarbazina v lechenii nerezektabel'nyh desmodnyh fibrom ekstraabdominal'noy lokalizacii. Onkologiya. Zhurnal im. P.A. Gercena. 2019;8;3:180-186. [A.V. Yadrina, L.V. Bolotina, O.V. Novikova, V.Yu. Karpenko, N.M. Bychkova, A.K. Kostrygin, I.Yu. Sychenkova. Experience of using doxorubicin and dacarbazine in the treatment of unresectable desmodal fibroids of extra-abdominal localization. Oncology. Journal them. P.A. Herzen. 2019;8;3:180-186. (In Russ.)]. DOI 10.17116/onkolog2019803115180

9. Yadrina A.V., Karpenko V.Yu., Novikova O.V., Bychkova N.M., Derzhavin V.A., Buharov A.V. Rekonstruktivno-plasticheskie operacii v lechenii desmoidnyh fibrom ekstraabdominal'noy lokalizacii. Vestnik travmatologii i ortopedii im. N.N. Priorova. 2019;1:42-48. [A.V. Yadrina, V.Yu. Karpenko, O.V. Novikova, N.M. Bychkova, V.A. Derzhavin, A.V. Bukharov. Reconstructive plastic surgery in the treatment of extra-abdominal desmoid fibroids. Bulletin of Traumatology and Orthopedics. N.N. Priorov. 2019;1:42-48. (In Russ.)].

10. Muller J. Uber den feineren Bau und die Formen der krankhaften Geschwullste. Erste Lieferung Berlin : G. Reimer. 1838.

11. Jaffe H.L. Tumors and thrombus conditions of the bones and joints. Philadelfia : Les & Febiger. 1958.

12. Crim J.R., Gold R.H. Desmoplastic fibroma of bone. Radiographic analysis // Radiology. – 1989;172:827-832. DOI: 10.1148/radiology.172.3.2772196

13. Gebhardt M.C., Campbell C.J., Schiller A.L., Mankin H.J. Desmoplastic fibroma of bone. A report of eight cases and review of the literature // J. Bone Joint. Surg. Am. – 1985;67:732-747.

14. Griffith J., Irby W.B. Desmoplastic fibroma // Oral. Surg. Oral. Med. Oral. Pathol. – 1965;20:269-275.

15. Harsha V.T., Ravi K.A. Desmoplastic fibroma of the mandible // J. Oral. Maxillofac. Pathol. – 2014;26(2):222-224.

16. Nag H.L., Kumar R., Bhan S. Radiotherapy for desmoplastic fibroma of bone: a case report // J. Orthop. Surg. (Hong Kong). – 2003;11(1):90-93. DOI: 10.1177/230949900301100117

17. Nithya S., Sundaravel S. Desmoplastic Fibroma // J. Oral. Maxillofac. Pathol. – 2015;19(2):270.

18. Stefanidis K., Benakis S., Tsatalou E. Computed tomography and magnetic resonance imaging of desmoplastic fibroma with simultaneous manifestation in two unusual locations // Iran. J. Radiol. – 2015;12;3:218. DOI: 10.1186/1752-1947-5-28

19. Vanhoenacker F.M., Hauben E. Desmoplastic fibroma of bone: MRI features // Skeletal. Radiol. – 2000;29;3:171-175. DOI: 10.1007/s002560050589

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