Extraoseus Ewing‘s Sarcoma, Primary Affection of Uterine Cervix – Case Report

Česká verze

Authors: O. Bílek ¹; M. Holánek ¹; M. Zvaríková ¹; P. Fabian ²; B. Robešová ³; M. Procházková ⁴; D. Adámková Krákorová ¹
Authors place of work: Klinika komplexní onkologické péče, Masarykův onkologický ústav, Brno ¹; Oddělení klinické a experimentální patologie, Masarykův onkologický ústav, Brno ²; Centrum molekulární bio logie a genové terapie, Interní hematologická a onkologická klinika LF MU a FN Brno ³; Oddělení radiologie, Masarykův onkologický ústav, Brno ⁴
Published in the journal: Klin Onkol 2015; 28(4): 284-287
Category: Kazuistika
doi: https://doi.org/10.14735/amko2015284

Summary

Background:
Ewing‘s sarcoma is usually diagnosed in adolescents and young adults, peak of incidence is around 15 years of age. Primary localization is mostly in the skeleton of long bones and chest wall. Primary extraosseous involvement rarely occurs, incidence increases with age.

Case:
We present a case report of a 57‑year‑ old patient with locally advanced tumors of the cervix, clinical stage IIB. Due to histological and molecular genetic examination revealing EWS‑ ERG fusion gene, Ewing‘s sarcoma was diagnosed. CT revealed pathological pelvic lymphadenopathy and multiple pulmonary bilateral methastases, scintigraphy did not prove any affection of skeleton. The patient underwent a two‑stage intensive chemotherapy regimens VIDE (vincristine, ifosfamide, doxorubicin, etoposide) and VAI (vincristine, actinomycin D, ifosfamide). During the second phase, concomitant radiotherapy of pelvis was aplied. According to PET/ CT, complete remission was achieved. Whole‑ lung irradiation was applied in consolidation of the result.

Conclusion:
Primary Ewing‘s sarcoma of the cervix is an extremely rare disease. To our knowledge, only 12 cases was presented until this time. The average age at time of diagnosis was 35 years. Unlike the previous reports, we initially diagnosed distant metastases. The treatment was led according to the protocol Ewing 2008 designed for primary skeletal Ewing‘s sarcoma. Currently, 18 months after the therapy, the patient is without signs of disease. However, long‑term follow-up is necessary.

Key words:
Ewing‘s sarcoma – uterine cervix – cytogenetics – EWS-ERG – chemotherapy – radiotherapy

The authors declare they have no potential conflicts of interest concerning drugs, products, or services used in the study.

The Editorial Board declares that the manuscript met the ICMJE “uniform requirements” for biomedical papers.

Submitted:
3. 6. 2015

Accepted:
25. 7. 2015

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