Epithelioid sarcoma in a child: difficulties of diagnosis and experience of complex treatment

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Abstract

Introduction. Epithelioid sarcoma is a rare malignant tumor of the soft tissues developing mostly in young adults. In children this pathology is diagnosed very rarely which often leads to its late verification and unplanned surgical interventions aimed at tumor resection which at early stages is mistaken for a benign neoplasm. The main difficulties are associated with non-specificity of the clinical presentation and the necessity of using immunohistochemical and molecular methods for establishment of the final diagnosis.

The article describes a clinical case of diagnosis and treatment of epithelioid soft tissue sarcoma in a child; difficulties of diagnosis associated with morphological verification and differential diagnosis of this tumor are presented, and efficacy of combination treatment consisting of radical surgical intervention and adjuvant radiotherapy is analyzed.

Clinical case. A boy, 11 years old, with epithelioid sarcoma of the soft tissues of the right shoulder. For diagnosis, medical history, clinical examination and instrumental methods – ultrasound, magnetic resonance tomography and computed tomography of the thoracic cage and abdomen, scintigraphy and positron emission tomography/computed tomography were used. Additionally, bone marrow biopsy to exclude systemic tumor advancement was performed. Treatment included radical surgical tumor resection with defect reconstruction and subsequent adjuvant radiotherapy at the tumor bed. Efficacy of the therapy was evaluated based on clinical data and results of dynamics in control examinations. No serious complications were reported, local control was achieved, as well as good quality of life of the child in short-term observation period.

Conclusion. The presented clinical case demonstrates difficulties of early diagnosis of epithelioid sarcoma in children, adds to the current clinical base, and has practical value for improvement of management of patients of pediatric age with rare tumors of the soft tissues.

About the authors

S. O. Gunyakov

State Scientific Center of the Russian Federation – A.I. Burnazyan Federal Medical Biophysical Center, Russian Federal Medical and Biological Agency

Email: wordex2006@rambler.ru
ORCID iD: 0009-0002-4796-8249
Russian Federation, 23 Marshala Novikova St., Moscow 123098

E. V. Inyushkina

State Scientific Center of the Russian Federation – A.I. Burnazyan Federal Medical Biophysical Center, Russian Federal Medical and Biological Agency; Moscow Regional Oncological Dispensary

Email: wordex2006@rambler.ru
ORCID iD: 0000-0002-3888-9656
Russian Federation, 23 Marshala Novikova St., Moscow 123098; 6 Karbysheva St., Balashikha 143900

A. V. Khizhnikov

State Scientific Center of the Russian Federation – A.I. Burnazyan Federal Medical Biophysical Center, Russian Federal Medical and Biological Agency; Moscow Regional Oncological Dispensary; Russian State Social University

Email: wordex2006@rambler.ru
ORCID iD: 0000-0001-7914-651X
Russian Federation, 23 Marshala Novikova St., Moscow 123098; 6 Karbysheva St., Balashikha 143900; Bld. 1, 4 Vil’gel’ma Peaka St., Moscow 119226

V. O. Stepanova

Moscow Regional Oncological Dispensary

Email: wordex2006@rambler.ru
ORCID iD: 0009-0005-2500-5670
Russian Federation, 6 Karbysheva St., Balashikha 143900

Maxim Yu. Rykov

Russian State Social University; Russian Research Institute of Health, Ministry of Health of Russia

Author for correspondence.
Email: wordex2006@rambler.ru
ORCID iD: 0000-0002-8398-7001
SPIN-code: 7652-0122
Russian Federation, Bld. 1, 4 Vil’gel’ma Peaka St., Moscow 119226; 11 Dobrolyubova St., Moscow 127254

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