Zentralbl Chir 2025; 150(S 01): S76
DOI: 10.1055/s-0045-1809725
Abstracts
Onkologische Thoraxchirurgie

Multimodal Therapy Approach Including Hyperthermic Intrathoracic Chemotherapy for Primary Pleural Ewing Sarcoma: A Case Report.

Authors

  • H Kaman

    1   Kliniken Der Stadt Köln Merheim, Thoraxchirurgie, Köln, Deutschland

  • T Stork

    1   Kliniken Der Stadt Köln Merheim, Thoraxchirurgie, Köln, Deutschland

  • M Ströhlein

    2   Kliniken Der Stadt Köln Merheim, Viszeralchirurgie, Köln, Deutschland

  • C S Seefeldt

    2   Kliniken Der Stadt Köln Merheim, Viszeralchirurgie, Köln, Deutschland

  • V Vieth

    3   Klinikum Ibbenbüren, Klinik für Radiologie und Neuroradiologie, Ibbenbüren, Deutschland

  • Ö Okumus

    1   Kliniken Der Stadt Köln Merheim, Thoraxchirurgie, Köln, Deutschland

  • U Dirksen

    4   Universitätsklinikum Essen, Klinik für Kinderheilkunde, Essen, Deutschland

  • M Remke

    5   Universitätsklinikum des Saarlandes, Klinik für Pädiatrische Onkologie und Hämatologie, Homburg, Deutschland

  • P Melchior

    6   Universitätsklinikum des Saarlandes, Klinik für Strahlentherapie und Radioonkologie, Homburg, Deutschland

  • S Collaud

    1   Kliniken Der Stadt Köln Merheim, Thoraxchirurgie, Köln, Deutschland
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    Background Ewing sarcoma (EWS) is a rare, highly malignant round cell sarcoma with an annual incidence of 0.1/100,000. It most commonly occurs in the second decade of life. While EWS typically arise from bone, it can also originate from soft tissues as extraskeletal EWS. Primary pleural EWS is an extremely rare entity with only a few cases reported in the literature.

    Methods & Materials We report the case of a 12-year-old female who initially presented with dyspnea. Imaging revealed a large pleural tumor occupying the left hemithorax without involvement of bony structures. An MRI-guided biopsy was performed for histological diagnosis.

    Results An EWS was diagnosed at a sarcoma reference center based on histopathology, immunohistochemistry, and molecular analysis. Staging work-up, including PET/CT, excluded metastasis. Induction chemotherapy was administered with six cycles of vincristine, ifosfamide, doxorubicin, and etoposide and two cycles of vincristine, actinomycine D and ifosfamid. Re-staging imaging showed a partial response with residual tumor limited to the pleura. The Interdisciplinary Tumor Board of the Cooperative Ewing Sarcoma Study (CESS) recommended surgical resection with hyperthermic intrathoracic chemotherapy (HITOC). Extended pleurectomy/decortication was performed via anterolateral thoracotomy, followed by HITOC with doxorubicin (15 mg/m²) and cisplatin (50 mg/m²) at 41.5°C for 60 minutes. The intraoperative course was uneventful. Five-day antibiotic therapy was administered postoperatively for a suspected pneumonia and the patient was discharged on postoperative day 11. Histopathological evaluation of the surgical specimen showed less than 10% viable tumor cells. Postoperative consolidation chemotherapy and radiation were performed as advised by the CESS tumor board. Nine months after surgery, the patient is well without evidence of recurrence.

    Conclusion Multimodal therapy including extended pleurectomy/decortication with HITOC in a pediatric patient with primary pleural EWS is feasible. Patients with ultra-rare diseases mostly benefit from interdisciplinary management within expert networks.


     

    Publication History

    Article published online:
    25 August 2025

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