Management

Management
You have a patient with a mass sitting in your office. Now what? Overall principles are important. You need to start with a detailed history and a careful examination. This will help guide whether you think this might be benign or malignant. Then you can decide on which investigation you might need to undertake or decide whether to refer the patient.

If the patient has a sarcoma they are best managed in a subspecialist, multidisciplinary clinic by experienced physicians. This has been shown to improve outcomes. They should be referred prior to biopsy as seeding of the biopsy tract can interfere with surgical management.

SummaryManagementF

Primary Care Management

The rarity of sarcomas makes them a huge challenge for primary physicians and care-givers. The vast majority of soft tissue lumps will be benign lipomas, but sometimes there might be something more serious.

Soft Tissue Tumours

Detection

Soft tissue sarcomas can be any size, anywhere, at any age. However, the following characteristics should make you suspicious of a soft tissue sarcoma.

  • Deep lesions
    • Lumps that lie deep to the deep fascia are suspicious
    • Test by having the patient tense the underlying muscle; if the lump remains mobile, it is probably superficial
  • Large lesions
    • Lumps >5 cm, and certainly >8 cm are suspicious
  • Fast growing lesions
    • Lesions that are growing quickly are of particular concern and warrant referral

Investigation

  • Imaging
    • MRI scanning is the investigation of choice, but expensive
    • Not all lesions need MRI
    • Ultrasound scanning is useful for location and monitoring of all lesions
  • Biopsy
    • In general, biopsies are best done by the treating surgeon as these tumours are very transplantable
    • Requires experienced pathology expertise
    • Core needle biopsy (Fine Needle Aspirate is inadequate)
    • A bad biopsy is better than a bad excision

Excision

  • Small, superficial lesions can be safely excised by family physicians or non-specialist surgeons
  • These represent the vast majority of soft tissue masses
  • Longitudinal incisions should be made where possible
  • In the event a lesion is sarcoma, radiation therapy and re-excision of the wound bed gives excellent results

Referral

  • Most multidisciplinary groups are happy to assess any patient with a suspected sarcoma

Bone Tumours

Detection

  • History
    • Unexplained bone or joint pain that persists
    • Night Pain
    • Noticed a lump
    • Under 25 or over 50 years
    • Previous radiation to the area
    • B-type symptoms (weight loss, etc) are uncommon and late signs
  • Examination
    • Detectable mass
    • Tenderness over a bony prominence

Investigation

  • Unexplained bone or joint pain >6 weeks deserves an X-ray
  • Plain X-ray remains the Gold Standard Investigation
    • Detects almost all bone tumours
    • Often Diagnostic
    • Interpretation does require specialist expertise

Referral

  • Multidisciplinary groups are happy to review any suspicious lesion on an X-ray
  • Electronic imaging makes review easy and can save patients a lot of travel
  • If the X-ray shows a sarcoma, there is no need to wait for cross-sectional imaging before referral

Specialist Care Management

Multidisciplinary Teams

Patients have better outcomes

  • Better survival rates
  • Lower amputation rates
  • Better functional outcomes

Team members have subspecialist interests

  • Have appropriate subspecialist training
  • Dedicate a significant part, if not all, of their practice to the treatment of sarcoma patients
  • Work together in a productive manner
  • Include a dedicated Nurse Practitioner to help patients navigate their complex treatment

Teams contain a wide range of experts

  • Pathologists
  • Radiologists
  • Radiation Oncologists
  • Medical Oncologists
  • Subspecialist Surgeons, including Orthopaedic Oncologists, General Surgical Oncologists, Thoracic, ENT Surgeons, etc
  • Reconstructive Surgeons, including Plastic and Vascular Surgeons
  • Nursing Staff
  • Counselling Staff
  • Administrative Staff

Teams function in a structured manner

  • Meeting on a regular basis to review all cases
  • Co-ordinating assessment and biopsy of suspicious lesions
  • Using radiology, pathology and clinical expertise to make an accurate diagnosis
  • Creating an individualized treatment plan that co-ordinates all aspects of the treatment pathway prior to any surgery or other interventions
  • Reviewing patients as they progress

Broad Treatment Principles

Each patient is different and will receive an individualized plan, but the following principles are generally applicable.

Bone Tumours: Benign

  • Most benign incidental tumours can simply be watched
  • Some are more aggressive and require surgical treatment
  • Surgery may involve curettage or wide resection

Bone Tumours: Malignant

  • Surgery with wide resection usually required
    • Often leads to large skeletal defects with complex reconstructions
  • Chemotherapy for Osteosarcomas and Ewing Family Tumours
  • Radiation Therapy for Ewing Family Tumours

Soft Tissue Tumours: Benign

  • Can often be removed by non-specialists
  • Some, such as fibromatosis, are often best managed non-operatively
  • Radiation Therapy used for some lesions

Soft Tissue Tumours: Malignant

  • Wide resection surgery is often required
    • Require specialist expertise in a high-volume centre to get the best results
    • Plastic Surgery is a key part of many reconstructions
  • Radiation Therapy
    • Given to large tumours or where surgical margins will be close
    • Preoperative treatment gives better long-term functional results, but increases short-term wound complications
  • Chemotherapy
    • Role is uncertain, decisions are made on a case-by-case basis