<?xml version="1.0" encoding="UTF-8"?>
<!DOCTYPE root>
<article xmlns:mml="http://www.w3.org/1998/Math/MathML" xmlns:xlink="http://www.w3.org/1999/xlink" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" xmlns:ali="http://www.niso.org/schemas/ali/1.0/" article-type="other" dtd-version="1.2" xml:lang="en"><front><journal-meta><journal-id journal-id-type="publisher-id">MD-Onco</journal-id><journal-title-group><journal-title xml:lang="en">MD-Onco</journal-title><trans-title-group xml:lang="ru"><trans-title>MD-Onco</trans-title></trans-title-group></journal-title-group><issn publication-format="print">2782-3202</issn><issn publication-format="electronic">2782-6171</issn><publisher><publisher-name xml:lang="en">Publishing House ABV Press</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="publisher-id">32</article-id><article-id pub-id-type="doi">10.17650/2782-3202-2022-2-2-14-23</article-id><article-categories><subj-group subj-group-type="toc-heading" xml:lang="en"><subject>RARE NEOPLASMS AND COMPLEX CLINICAL SITUATIONS: DIFFICULTIES IN DIAGNOSIS AND SELECTION OF TREATMENT TACTICS</subject></subj-group><subj-group subj-group-type="toc-heading" xml:lang="ru"><subject>РЕДКИЕ НОВООБРАЗОВАНИЯ И СЛОЖНЫЕ КЛИНИЧЕСКИЕ СИТУАЦИИ: ТРУДНОСТИ ДИАГНОСТИКИ И ВЫБОР ТАКТИКИ ЛЕЧЕНИЯ</subject></subj-group><subj-group subj-group-type="article-type"><subject></subject></subj-group></article-categories><title-group><article-title xml:lang="en">Clinical and morphological observation of a 19-year-old patient with alveolar rhabdomyosarcoma of the head and neck region</article-title><trans-title-group xml:lang="ru"><trans-title>Клинико-морфологическое наблюдение пациента 19 лет c альвеолярной рабдомиосаркомой в области головы и шеи</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0001-8443-8816</contrib-id><name-alternatives><name xml:lang="en"><surname>Ryabukhina</surname><given-names>Yu. E.</given-names></name><name xml:lang="ru"><surname>Рябухина</surname><given-names>Ю. Е.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>Yulia E. Ryabukhina.</p><p>111 1<sup>st</sup> Uspenskoe Shosse, Lapino, Moscow region 143081.</p></bio><bio xml:lang="ru"><p>Рябухина Юлия Евгеньевна.</p><p>143081 Московская обл., д. Лапино, 1-е Успенское шоссе, 111.</p></bio><email>gemonk.yur@mail.ru</email><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0003-4927-5585</contrib-id><name-alternatives><name xml:lang="en"><surname>Fedenko</surname><given-names>A. A.</given-names></name><name xml:lang="ru"><surname>Феденко</surname><given-names>А. А.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>111 1<sup>st</sup> Uspenskoe Shosse, Lapino, Moscow region 143081.</p></bio><bio xml:lang="ru"><p>143081 Московская обл., д. Лапино, 1-е Успенское шоссе, 111.</p></bio><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0003-1564-424X</contrib-id><name-alternatives><name xml:lang="en"><surname>Zeynalova</surname><given-names>P. A.</given-names></name><name xml:lang="ru"><surname>Зейналова</surname><given-names>П. А.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>111 1<sup>st</sup> Uspenskoe Shosse, Lapino, Moscow region 143081; Bld. 2, 8 Trubetskaya St., Moscow 119991.</p></bio><bio xml:lang="ru"><p>143081 Московская обл., д. Лапино, 1-е Успенское шоссе, 111; 119991 Москва, ул. Трубецкая, 8, стр. 2.</p></bio><xref ref-type="aff" rid="aff1"/><xref ref-type="aff" rid="aff2"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-6229-7300</contrib-id><name-alternatives><name xml:lang="en"><surname>Timofeeva</surname><given-names>O. L.</given-names></name><name xml:lang="ru"><surname>Тимофеева</surname><given-names>О. Л.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>111 1<sup>st</sup> Uspenskoe Shosse, Lapino, Moscow region 143081.</p></bio><bio xml:lang="ru"><p>143081 Московская обл., д. Лапино, 1-е Успенское шоссе, 111.</p></bio><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-2359-0547</contrib-id><name-alternatives><name xml:lang="en"><surname>Abbasbeyli</surname><given-names>F. M.</given-names></name><name xml:lang="ru"><surname>Аббасбейли</surname><given-names>Ф. М.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>111 1<sup>st</sup> Uspenskoe Shosse, Lapino, Moscow region 143081.</p></bio><bio xml:lang="ru"><p>143081 Московская обл., д. Лапино, 1-е Успенское шоссе, 111.</p></bio><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><name-alternatives><name xml:lang="en"><surname>Chekini</surname><given-names>D. A.</given-names></name><name xml:lang="ru"><surname>Чекини</surname><given-names>Д. А.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>111 1<sup>st</sup> Uspenskoe Shosse, Lapino, Moscow region 143081.</p></bio><bio xml:lang="ru"><p>143081 Московская обл., д. Лапино, 1-е Успенское шоссе, 111.</p></bio><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-0918-3857</contrib-id><name-alternatives><name xml:lang="en"><surname>Mudunov</surname><given-names>A. M.</given-names></name><name xml:lang="ru"><surname>Мудунов</surname><given-names>А. М.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>111 1<sup>st</sup> Uspenskoe Shosse, Lapino, Moscow region 143081.</p></bio><bio xml:lang="ru"><p>143081 Московская обл., д. Лапино, 1-е Успенское шоссе, 111.</p></bio><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-1794-7247</contrib-id><name-alternatives><name xml:lang="en"><surname>Batsev</surname><given-names>A. F.</given-names></name><name xml:lang="ru"><surname>Бацев</surname><given-names>А. Ф.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>111 1<sup>st</sup> Uspenskoe Shosse, Lapino, Moscow region 143081.</p></bio><bio xml:lang="ru"><p>143081 Московская обл., д. Лапино, 1-е Успенское шоссе, 111.</p></bio><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><name-alternatives><name xml:lang="en"><surname>Sinitsyna</surname><given-names>O. V.</given-names></name><name xml:lang="ru"><surname>Синицына</surname><given-names>О. В.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>111 1<sup>st</sup> Uspenskoe Shosse, Lapino, Moscow region 143081.</p></bio><bio xml:lang="ru"><p>143081 Московская обл., д. Лапино, 1-е Успенское шоссе, 111.</p></bio><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><name-alternatives><name xml:lang="en"><surname>Zhukov</surname><given-names>A. G.</given-names></name><name xml:lang="ru"><surname>Жуков</surname><given-names>А. Г.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>111 1<sup>st</sup> Uspenskoe Shosse, Lapino, Moscow region 143081.</p></bio><bio xml:lang="ru"><p>143081 Московская обл., д. Лапино, 1-е Успенское шоссе, 111.</p></bio><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-5298-8124</contrib-id><name-alternatives><name xml:lang="en"><surname>Shestakova</surname><given-names>L. A.</given-names></name><name xml:lang="ru"><surname>Шестакова</surname><given-names>Л. А.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>111 1<sup>st</sup> Uspenskoe Shosse, Lapino, Moscow region 143081.</p></bio><bio xml:lang="ru"><p>143081 Московская обл., д. Лапино, 1-е Успенское шоссе, 111.</p></bio><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0001-5910-5892</contrib-id><name-alternatives><name xml:lang="en"><surname>Allakhverdieva</surname><given-names>G. F.</given-names></name><name xml:lang="ru"><surname>Аллахвердиева</surname><given-names>Г. Ф.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>111 1<sup>st</sup> Uspenskoe Shosse, Lapino, Moscow region 143081.</p></bio><bio xml:lang="ru"><p>143081 Московская обл., д. Лапино, 1-е Успенское шоссе, 111.</p></bio><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-1345-041X</contrib-id><name-alternatives><name xml:lang="en"><surname>Yurichev</surname><given-names>I. N.</given-names></name><name xml:lang="ru"><surname>Юричев</surname><given-names>И. Н.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>111 1<sup>st</sup> Uspenskoe Shosse, Lapino, Moscow region 143081.</p></bio><bio xml:lang="ru"><p>143081 Московская обл., д. Лапино, 1-е Успенское шоссе, 111.</p></bio><xref ref-type="aff" rid="aff1"/></contrib></contrib-group><aff-alternatives id="aff1"><aff><institution xml:lang="en">Clinical Hospital “Lapino” of the “Mother and Child” Group of companies</institution></aff><aff><institution xml:lang="ru">Клинический госпиталь «Лапино» группы компаний «Мать и дитя»</institution></aff></aff-alternatives><aff-alternatives id="aff2"><aff><institution xml:lang="en">I.M. Sechenov First Moscow State Medical University, Ministry of Health of Russia (Sechenov University)</institution></aff><aff><institution xml:lang="ru">Первый Московский государственный медицинский университет им. И.М. Сеченова Минздрава России (Сеченовский Университет)</institution></aff></aff-alternatives><pub-date date-type="pub" iso-8601-date="2022-06-03" publication-format="electronic"><day>03</day><month>06</month><year>2022</year></pub-date><volume>2</volume><issue>2</issue><issue-title xml:lang="en"/><issue-title xml:lang="ru"/><fpage>14</fpage><lpage>23</lpage><history><date date-type="received" iso-8601-date="2022-06-02"><day>02</day><month>06</month><year>2022</year></date><date date-type="accepted" iso-8601-date="2022-06-02"><day>02</day><month>06</month><year>2022</year></date></history><permissions><copyright-statement xml:lang="en">Copyright ©; 2022, ABV-Press</copyright-statement><copyright-statement xml:lang="ru">Copyright ©; 2022, АБВ-пресс</copyright-statement><copyright-year>2022</copyright-year><copyright-holder xml:lang="en">ABV-Press</copyright-holder><copyright-holder xml:lang="ru">АБВ-пресс</copyright-holder><ali:free_to_read xmlns:ali="http://www.niso.org/schemas/ali/1.0/"/><license><ali:license_ref xmlns:ali="http://www.niso.org/schemas/ali/1.0/">https://mdonco.abvpress.ru/jour/about/editorialPolicies</ali:license_ref></license></permissions><self-uri xlink:href="https://mdonco.abvpress.ru/jour/article/view/32">https://mdonco.abvpress.ru/jour/article/view/32</self-uri><abstract xml:lang="en"><p>Alveolar rhabdomyosarcoma (ARMS) is an aggressive primitive tumor with limited differentiation of rhabdomyoblasts. Primarily, it occurs in children and teens under 15 years of age in the head and neck region (in 40 % of cases). In patients older than 19-20 years, ARMS is significantly less common and is localized primarily on the limbs, while head and neck region is affected in about 24 % of all cases, involvement of the orbit in the tumor process is very rare. ARMS progression in adults is more aggressive than in children, and prognosis is more unfavorable due to development of regional and distant metastases at the time of diagnosis. Hence, selection of antitumor therapy is limited to drug treatment. Use of multimodal approach to therapy allows to improve treatment outcomes and increase quality of life in patients with ARMS.</p></abstract><trans-abstract xml:lang="ru"><p>Альвеолярная рабдомиосаркома (АРМС) - агрессивная примитивная опухоль с ограниченной Дифференцировкой рабдомиобластов. В основном встречается у детей и подростков в возрасте до 15 лет, развиваясь наиболее часто в области головы и шеи (в 40 % случаев). У пациентов старше 19-20 лет АРМС наблюдается значительно реже, локализуется, как правило, в области конечностей, а на участки головы и шеи приходится около 24 % всех случаев, при этом вовлечение в опухолевый процесс орбиты встречается крайне редко. Течение АРМС у взрослых более агрессивное, чем у детей, а прогноз более неблагоприятный из-за развития регионарных или отдаленных метастазов на момент установления диагноза. В связи с этим выбор программы противоопухолевой терапии ограничен проведением лекарственного лечения. Благодаря применению мультимодального подхода к терапии возможно улучшить результаты лечения и повысить качество жизни больных АРМС.</p></trans-abstract><kwd-group xml:lang="en"><kwd>alveolar rhabdomyosarcoma</kwd><kwd>orbit</kwd><kwd>nasopharynx</kwd><kwd>regional lymph node metastases</kwd><kwd>of multimodal approach to therapy</kwd></kwd-group><kwd-group xml:lang="ru"><kwd>альвеолярная рабдомиосаркома</kwd><kwd>орбита</kwd><kwd>носоглотка</kwd><kwd>метастаз в регионарный лимфатический узел</kwd><kwd>мультимодальный подход к терапии</kwd></kwd-group><funding-group/></article-meta></front><body></body><back><ref-list><ref id="B1"><label>1.</label><mixed-citation>Moon H.S., Kwon S.W., Lee J.H. A case of alveolar rhabdomyosarcoma of the ethmoid sinus invading the orbit in an adult. Korean J Ophthalmol 2006;20(1):70-5. DOI: 10.3341/kjo.2006.20.1.70.</mixed-citation></ref><ref id="B2"><label>2.</label><mixed-citation>Shields J.A., Shields C.L. Rhabdomyosarcoma: review for the ophthalmologist. Surv Ophthalmol 2003;48(1):39-57. DOI: 10.1016/s0039-6257(02)00415-0.</mixed-citation></ref><ref id="B3"><label>3.</label><citation-alternatives><mixed-citation xml:lang="en">Paltsev M.A., Anichkov N.M. Atlas of pathology of human tumors. Moscow: Meditsina, 2005. Pp. 68-71. (In Russ.).</mixed-citation><mixed-citation xml:lang="ru">Пальцев М.А., Аничков Н.М. Атлас патологии опухолей человека. М.: Медицина, 2005. С. 68-71.</mixed-citation></citation-alternatives></ref><ref id="B4"><label>4.</label><mixed-citation>Karcioglu Z.A., Hadjistilianou D., Rozans M., DeFrancesco S. Orbital rhabdomyosarcoma. Cancer Control 2004;11(5):328-33. DOI: 10.1177/107327480401100507.</mixed-citation></ref><ref id="B5"><label>5.</label><mixed-citation>Lanzkowsky P. Manual of Pediatric Hematology and Oncology. 3rd edn. V. 1. N.Y.: Academic Press, 2000.</mixed-citation></ref><ref id="B6"><label>6.</label><mixed-citation>Pathology and genetics of tumours of soft tissue and bone. Ed. by C.D.M. Fletcher, K.K. Unni, F. Mertens. Lyon: IARC Press, 2002. Р. 141-154.</mixed-citation></ref><ref id="B7"><label>7.</label><mixed-citation>Esnaola N.F., Rubin B.P., Baldini E.H. et al. Response to chemotherapy and predictors of survival in adult rhabdomyosarcoma. Ann Surg 2001;234(2):215-23. DOI: 10.1097/00000658-200108000-00012.</mixed-citation></ref><ref id="B8"><label>8.</label><mixed-citation>Wu T.H., Huang J.S., Wang H.M. et al. Long-term survivors of adult rhabdomyosarcoma of maxillary sinus following multimodal therapy: case reports and literature reviews. Chang Cung Med J 2010;33(4):466-71.</mixed-citation></ref><ref id="B9"><label>9.</label><mixed-citation>Hawkins W.G., Hoos A., Antonescu C.R. et al. Clinicopathologic analysis of patients with adult rhabdomyosarcoma. Cancer 2001;91(4):794-803.</mixed-citation></ref><ref id="B10"><label>10.</label><mixed-citation>Inarejos Clemente E.J., Navallas M., Barber Marti'nez de la Torre I. et al. MRI of rhabdomyosarcoma and other soft-tissue sarcomas in children. Radiographics 2020;40(3):791-814. DOI: 10.1148/rg.2020190119.</mixed-citation></ref><ref id="B11"><label>11.</label><mixed-citation>Lee H.L., Lee M.S., Lee B.H. et al. Rhabdomyosarcoma of the head and neck in adults: MR and CT findings. AJNR Am J Neuroradiol 1996;17(10):1923-8.</mixed-citation></ref><ref id="B12"><label>12.</label><mixed-citation>Rodeberg D.A., Garcia-Henriquez N., Lyden E.R. et al. Prognostic significance and tumor biology of regional lymph node disease in patients with rhabdomyosarcoma: a report from the Children's Oncology Group. J Clin Oncol 2011;29(10):1304-11. DOI: 10.1200/JCO.2010.29.4611.</mixed-citation></ref><ref id="B13"><label>13.</label><mixed-citation>Michael C. Mesenchymal and fibro-osseous tumors. In: Principle and practice of ophthalmic plastic and reconstructive surgery. Ed. by S. Bosniak. V. 2. Philadelphia: W.B. Saunders, 1996.</mixed-citation></ref><ref id="B14"><label>14.</label><mixed-citation>Soft Tissue Sarcoma. NCCN Clinical Practice Guidelines in Oncology. Version 1.2022.</mixed-citation></ref><ref id="B15"><label>15.</label><citation-alternatives><mixed-citation xml:lang="en">Fedenko A.A., Bokhyan A.Yu., Gorbunova V.A., et al. Practical recommendations for the drug treatment of soft tissue sarcomas. Zlokachestvenniye opukholy: Prakti-cheskie rekomendacii RUSSCO = Malignant Tumors: Practical Guideline by RUSSCO 2021;11(3s2-1);277-86. (In Russ.).</mixed-citation><mixed-citation xml:lang="ru">Феденко А.А., Бохян А.Ю., Горбунова В.А. и др. Практические рекомендации по лекарственному лечению сарком мягких тканей. Злокачественные опухоли: Практические рекомендации RUSSCO #3s2 2021;11(3s2-1);277-86.</mixed-citation></citation-alternatives></ref><ref id="B16"><label>16.</label><mixed-citation>Shields C.L., Shields J.A., Honavar S.G., Demirci H. Primary ophthalmic rhabdomyosarcoma in 33 patients. Trans Am Ophthalmol Soc 2001;99:133-42; discussion 142-3.</mixed-citation></ref><ref id="B17"><label>17.</label><mixed-citation>Arndt C.A., Hawkins D.S., Meyer W.H. et al. Comparison of results of a pilot study of alternating vincristine/doxorubicin/ cyclophosphamide and etoposide/ ifosfamide with IRS-IV in intermediate risk rhabdomyosarcoma: а report from the Children's Oncology Group. Pediatr Blood Cancer 2008;50(1):33-6. DOI: 10.1002/pbc.21093.</mixed-citation></ref><ref id="B18"><label>18.</label><mixed-citation>Rajwanshi A., Srinivas R., Upasana G. Malignant small round cell tumors. J Cytol 2009;26(1):1-10. DOI: 10.4103/0970-9371.54861.</mixed-citation></ref><ref id="B19"><label>19.</label><mixed-citation>Bahrami A., Gown A.M., Baird G.S. Aberrant expression of epithelial and neuroendocrine markers in alveolar rhabdomyosarcoma: a potentially serious diagnostic pitfall. Mod Pathol 2008;21(7):795-806. DOI: 10.1038/modpathol.2008.86.</mixed-citation></ref><ref id="B20"><label>20.</label><mixed-citation>Newton W.A. Jr., Gehan E.A., Webber B.L. et al. Classification of rhabdomyosarcomas and related sarcomas. Pathologic aspects and proposal for a new classification - an intergroup rhabdomyosarcoma study. Cancer 1995;76(6):1073-85. DOI: 10.1002/1097-0142(19950915)76:6&lt;1073::aid-cncr2820760624&gt;3.0.co;2-l.</mixed-citation></ref><ref id="B21"><label>21.</label><mixed-citation>Rudzinski E.R., Anderson J.R., Chi Y.Y. et al. Histology, fusion status, and outcome in metastatic rhabdomyosarcoma: a report from the Children's Oncology Group. Pediatr Blood Cancer 2017;64(12):e26645. DOI: 10.1002/pbc.26645.</mixed-citation></ref></ref-list></back></article>
