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Epithelioid sarcoma in children

E Gross1, B N Rao, A Pappo

  • 1Department of Surgery, Hematology-Oncology, Diagnostic Imaging, St Jude Children's Research Hospital, Memphis, TN 38105, USA.

Journal of Pediatric Surgery
|December 1, 1996
PubMed
Summary
This summary is machine-generated.

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Epithelioid sarcoma in children is rare, with most presenting localized disease. While two patients died, six achieved remission with surgery, chemotherapy, and radiation, indicating a hopeful prognosis with multimodal treatment.

Area of Science:

  • Pediatric Oncology
  • Surgical Pathology
  • Rare Cancers

Background:

  • Epithelioid sarcoma is an uncommon soft tissue sarcoma with limited data on its natural history and prognosis in pediatric patients.
  • Understanding the characteristics and outcomes of pediatric epithelioid sarcoma is crucial for developing effective treatment strategies.

Purpose of the Study:

  • To describe the clinical characteristics, treatment, and outcomes of pediatric patients with epithelioid sarcoma.
  • To evaluate the prognosis and survival rates for children diagnosed with epithelioid sarcoma.

Main Methods:

  • Retrospective review of eight pediatric patients diagnosed with epithelioid sarcoma treated at St Jude Children's Research Hospital over 11 years.
  • Analysis of patient demographics, tumor location, disease stage (localized vs. metastatic), treatment modalities (surgery, chemotherapy, radiation), and follow-up data.

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  • Evaluation of treatment response and survival outcomes.

    • Main Results:

    • Seven of eight pediatric patients presented with localized epithelioid sarcoma; one had metastatic disease at diagnosis.
    • Common primary tumor sites included the upper extremity (four patients). Mean symptom duration before diagnosis was 8.3 months.
    • Six patients treated with multimodal therapy (surgery, chemotherapy, radiation) achieved no evidence of disease, with follow-up ranging from 12 months to over 10 years; two patients died from progressive disease within 9 months.

    Conclusions:

    • Epithelioid sarcoma in children often presents as localized disease, with multimodal treatment offering a favorable prognosis for the majority of patients.
    • Aggressive surgical resection, chemotherapy, and radiation are vital for achieving long-term remission in pediatric epithelioid sarcoma.
    • Further research is needed to optimize treatment protocols for this rare pediatric malignancy.