Telangiectatic osteosarcoma in children and adolescents: Experience of the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology scientific and clinical group on studying sarcomas of the locomotor system

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Abstract

Introduction. Telangiectatic osteosarcoma is a rare histological subtype of osteosarcoma, accounting for 2–12 % of all cases. Although telangiectatic osteosarcoma is characterized by unique morphological and radiological features, its diagnosis might be challenging due to its similarity to aneurysmal bone cysts and giant cell tumors.

Aim. To evaluate oncologic outcomes in a large homogeneous series of children and adolescents with telangiectatic osteosarcoma.

 Materials and methods. The study included treatment-naive patients under the age of 18 with a diagnosis of telangiectatic osteosarcoma confirmed between January 1, 2012, and December 31, 2023, and prospectively registered at the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology. All patients received MAP-based chemotherapy (according to the EURAMOS-1 protocol). Treatment outcomes were assessed

using event-free survival and overall survival rates, calculated by the Kaplan–Meier method. Results. Among 368 primary osteosarcoma patients, the telangiectatic subtype was diagnosed in 40 cases (10.8 %). At initial diagnosis, 48.7 % of patients had pulmonary metastases, and pathological fractures were identified in 33.4 % of cases. Histological examination showed good response to preoperative chemotherapy in 76.3 % of patients. The median follow-up was 3.65 years. 5-year overall survival and event-free survival rates for all patients were 73.4 % and 57.3 %, respectively.

 Conclusion. Due to combination treatment including surgical tumor resection and neo- and adjuvant polychemotherapy, treatment outcomes for patients with telangiectatic osteosarcoma are similar to outcomes in patients with conventional osteosarcoma.

About the authors

E. I. Konopleva

Dmitry  Rogachev  National  Medical  Research  Center  of  Pediatric  Hematology,  Oncology  and  Immunology,  Ministry  of  Health  of  Russia

Author for correspondence.
Email: konoplevadoc@gmail.com
ORCID iD: 0000-0002-6848-8714

Elena Ivanovna Konopleva

1  Samory  Mashela  St.,  Moscow  117198

Russian Federation

A. I.  Karachunsky

Dmitry  Rogachev  National  Medical  Research  Center  of  Pediatric  Hematology,  Oncology  and  Immunology,  Ministry  of  Health  of  Russia

ORCID iD: 0000-0002-9300-198X

1  Samory Mashela St.,  Moscow  117198

Russian Federation

N. A.  Bolshakov

Dmitry  Rogachev  National  Medical  Research  Center  of  Pediatric  Hematology,  Oncology  and  Immunology,  Ministry  of  Health  of  Russia

ORCID iD: 0000-0001-6313-6712

1  Samory  Mashela  St.,  Moscow  117198

Russian Federation

D. V. Rogozhin

N.N.  Blokhin  National  Medical  Research  Center  of  Oncology,  Ministry  of  Health  of  Russia

ORCID iD: 0000-0003-0777-9152

24  Kashirskoe  Shosse,  Moscow  115522

Russian Federation

N.  G.  Uskova

Dmitry  Rogachev  National  Medical  Research  Center  of  Pediatric  Hematology,  Oncology  and  Immunology,  Ministry  of  Health  of  Russia

ORCID iD: 0000-0001-9424-1646

1  Samory  Mashela  St.,  Moscow  117198

Russian Federation

A. P.  Shcherbakov

Dmitry  Rogachev  National  Medical  Research  Center  of  Pediatric  Hematology,  Oncology  and  Immunology,  Ministry  of  Health  of  Russia

ORCID iD: 0000-0001-8129-0545

1  Samory  Mashela  St.,  Moscow  117198

Russian Federation

I.  V. Sidorov

Dmitry  Rogachev  National  Medical  Research  Center  of  Pediatric  Hematology,  Oncology  and  Immunology,  Ministry  of  Health  of  Russia

ORCID iD: 0000-0001-8578-6572

1  Samory  Mashela  St.,  Moscow  117198

Russian Federation

O.  I.  Bydanov

Dmitry  Rogachev  National  Medical  Research  Center  of  Pediatric  Hematology,  Oncology  and  Immunology,  Ministry  of  Health  of  Russia

ORCID iD: 0000-0003-3232-2322

1  Samory  Mashela  St.,  Moscow  117198

Russian Federation

G.  A.  Novichkova

Dmitry  Rogachev  National  Medical  Research  Center  of  Pediatric  Hematology,  Oncology  and  Immunology,  Ministry  of  Health  of  Russia

ORCID iD: 0000-0003-4911-0553

1  Samory  Mashela  St.,  Moscow  117198

Russian Federation

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Copyright (c) 2025 Konopleva E.I., Karachunsky A.I.,  Bolshakov N.A., Rogozhin D.V., Uskova N.G., Shcherbakov A.P., Sidorov I.V., Bydanov O.I.,  Novichkova G.A.

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