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Soft tissue sarcoma

What's new

The European Association of Dermato-Oncology (EADO) has published a new guideline on the diagnosis and management of dermatofibrosarcoma protuberans, a rare soft tissue sarcoma. Routine imaging staging is not recommended, but preoperative MRI is suggested to assess tumor extension. Surgery based on horizontal sections (Mohs surgery) or sectioning that allows full peripheral and deep margin assessment (Peripheral and Deep En Face Margin Assessment, 3D-surgery, histological section margin control) is the first-line treatment to achieve microscopically complete resection. Imatinib is offered as neoadjuvant therapy for primary or recurrent disease when surgical resection is not feasible. .

Guidelines

Key sources

The following summarized guidelines for the evaluation and management of soft tissue sarcoma are prepared by our editorial team based on guidelines from the European Academy of Dermatology and Venereology (EADV/UEMS/EADO/EDF 2025), the American Society for Radiation Oncology (ASTRO 2021), and the European Reference Network on Rare Adult Solid Cancers (EURACAN/ESMO/ERN GENTURIS 2021).
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Classification and risk stratification

Staging: as per ERN GENTURIS/ESMO/EURACAN 2021 guidelines, do not use available staging classifications (UICC-AJCC) as they are of limited clinical value. Obtain risk assessment through the available nomograms.
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Diagnostic procedures

Pathological diagnosis: as per ERN GENTURIS/ESMO/EURACAN 2021 guidelines, confirm pathological diagnosis by a sarcoma expert pathologist according to the 2020 WHO classification.
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Medical management

General principles
As per ASTRO 2021 guidelines:
Obtain expert pathology and radiology review and multidisciplinary evaluation before initiating treatment in patients with localized STS.
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Determine the sequencing of surgery and radiotherapy in patients with primary, localized extremity and truncal STS based on multidisciplinary evaluation of patient and tumor characteristics.
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More topics in this section

  • Management of localized disease (neoadjuvant radiotherapy)

  • Management of localized disease (wide excision)

  • Management of localized disease (postoperative radiotherapy)

  • Management of localized disease (technical considerations for radiotherapy)

  • Management of localized disease (neoadjuvant chemotherapy)

  • Management of advanced disease (chemotherapy)

  • Management of advanced disease (resection of lung metastases)

Specific circumstances

Patients with dermatofibrosarcoma protuberans, histopathology: as per EADO/EADV/EDF/UEMS 2025 guidelines, confirm clinical suspicion by histopathology before extensive surgery is performed.
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More topics in this section

  • Patients with dermatofibrosarcoma protuberans (imaging)

  • Patients with dermatofibrosarcoma protuberans (surgical resection)

  • Patients with dermatofibrosarcoma protuberans (adjuvant radiotherapy)

  • Patients with dermatofibrosarcoma protuberans (targeted therapy)

  • Patients with dermatofibrosarcoma protuberans (fibrosarcomatous transformation)

  • Patients with dermatofibrosarcoma protuberans (follow-up)

  • Patients with visceral sarcomas

  • Patients with desmoid-type fibromatosis