<abstract xmlns="http://www.w3.org/1999/xhtml"><p> Introduction. Congenital fibrosarcoma is a rare soft tissue neoplasm, distal extremities being more commonly involved. This tumor has a rapid growth and extensive local invasion, but metastasis is rare. It is usually observed in children younger than two years old and is present at birth in up to 30% of the cases. Purpose. Evaluation of the methods of diagnosis and treatment in a case of a newborn known from intrauterine life, with a large tumor of the right arm. Materials and methods. We present the case of a 1-day-old patient, who was transferred from maternity in our clinics with the following diagnostics: large tumor of the right thoracic member, cardiac insufficiency, prematurity, severe respiratory distress. The tumor was visible at ultrasonography from intrauterine life, and the evolution was the increase in volume with necrosis areas and hair presence on its surface. After imagistic investigation, taking into account the tumoral extension with neurovascular involvement, without the possibility of a tumoral resection, but also the imminent danger of spontaneous rupture, we decided to perform a shoulder disarticulation without a biopsy in advance. Results. Postoperative evolution and the treatment of the cardiac insufficiency were good under antibiotics. The histopathological results advocate for the diagnosis of congenital fibrosarcoma, also confirmed by immunohistochemical tests. Conclusions. Soft tissue tumors are very rare and they need a multidisciplinary evaluation for the establishment of the right treatment. Imagistic and laboratory investigations can guide the diagnosis and the therapeutical conduct. The certainty diagnosis is established only after the histopathological results.</p></abstract>

Introduction. Congenital fibrosarcoma is a rare soft tissue neoplasm, distal extremities being more commonly involved. This tumor has a rapid growth and extensive local invasion, but metastasis is rare. It is usually observed in children younger than two years old and is present at birth in up to 30% of the cases. Purpose. Evaluation of the methods of diagnosis and treatment in a case of a newborn known from intrauterine life, with a large tumor of the right arm. Materials and methods. We present the case of a 1-day-old patient, who was transferred from maternity in our clinics with the following diagnostics: large tumor of the right thoracic member, cardiac insufficiency, prematurity, severe respiratory distress. The tumor was visible at ultrasonography from intrauterine life, and the evolution was the increase in volume with necrosis areas and hair presence on its surface. After imagistic investigation, taking into account the tumoral extension with neurovascular involvement, without the possibility of a tumoral resection, but also the imminent danger of spontaneous rupture, we decided to perform a shoulder disarticulation without a biopsy in advance. Results. Postoperative evolution and the treatment of the cardiac insufficiency were good under antibiotics. The histopathological results advocate for the diagnosis of congenital fibrosarcoma, also confirmed by immunohistochemical tests. Conclusions. Soft tissue tumors are very rare and they need a multidisciplinary evaluation for the establishment of the right treatment. Imagistic and laboratory investigations can guide the diagnosis and the therapeutical conduct. The certainty diagnosis is established only after the histopathological results.

Congenital Fibrosarcoma – Case Presentation

Romanian Journal of Orthopaedic Surgery and Traumatology

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 License.

{"article-title":"Congenital Fibrosarcoma \u2013 Case Presentation"}

Introduction. Congenital fibrosarcoma is a rare soft tissue neoplasm, distal extremities being more commonly involved. This tumor has a rapid growth and...

Congenital Fibrosarcoma – Case Presentation

Data publikacji: 22 maj 2018

Zakres stron: 15 - 15

DOI: https://doi.org/10.2478/rojost-2018-0026

Słowa kluczowe
fibrosarcoma, congenital, shoulder disarticulation

© 2018 L. Dobre, published by Sciendo

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 License.

Congenital Fibrosarcoma – Case Presentation

Data publikacji: 22 maj 2018

Zakres stron: 15 - 15

DOI: https://doi.org/10.2478/rojost-2018-0026

Słowa kluczowefibrosarcoma, congenital, shoulder disarticulation

© 2018 L. Dobre, published by Sciendo

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 License.

Słowa kluczowe
fibrosarcoma, congenital, shoulder disarticulation