Sarcomas are rare cancers that affect bones or soft tissues such as muscles, nerves and other soft connective tissues, that connect or surround joints. They account for approximately 1% of Adult malignancy, but approximately 15% of Paediatric and Adolescent malignancy. It is unclear in many instances as to their aetiology – in other words, the cause of these cancers is often unclear. Due to the low incidence rate of these cancers, care must be managed by experts so that outcomes are as favourable as possible.
Care System & Principles
It is best practice for people with cancer to be overseen by a specialist who is a member of a multidisciplinary cancer care team. A multidisciplinary cancer care team is a group of health professionals that meet regularly to oversee the diagnosis and staging of cancer, as well as the development of individualised treatment plans for people with cancer.
The patient pathway is as follows:
1 – Referral→2 – Diagnosis→3 – Pathology→4 – Determination of Treatment
Patients with sarcoma may present to their general practitioners or other specialist either with unremitting bone pain (as with bone sarcomas) or a painless growing lump (often with soft tissue sarcomas).
1 – Referral
When a patient first presents to a primary care doctor or care provider, this may prompt initial simple investigations to be performed. After preliminary investigations to confirm a lump or bone abnormality, general practitioners often refer patients to an orthopaedic surgeon, a general surgeon, or a specialist bone and soft tissue sarcoma expert. The appropriate management from this point is referral to a tumour centre or bone and soft tissue sarcoma expert. From here, further investigations will be performed a needed, but will often require advanced imaging and a subsequent tissue biopsy. This will all be under the guidance of a sarcoma expert & must not be performed without consultation. From these results a rational and tailored treatment plan will be formulated.
2 – Diagnosis
Diagnosis is made after obtaining a history, performing a physical examination and completing investigations, often including a biopsy. Imaging investigations are best performed prior to biopsy to avoid biopsy artefact from confounding the results of anatomic imaging. Appropriate imaging will define the local extent of the tumour and system investigations will assess for any spread. Relevant imaging includes plain radiographs of the affected limb or body part, MRI scan of the entire body part, a CT chest and nuclear scans (often PET scanning).
3 – Pathology
A biopsy is a procedure where a small sample of cells from the tumour is taken and studied to determine exactly what cell type the cancer is from. Biopsy for histologic examination is best performed after anatomic imaging of the tumour. The safest form of biopsy is an image-guided biopsy to target the most metabolically active area of the tumour and to avoid areas of necrosis. In some circumstances an open biopsy is necessitated an d this will be performed by the Specialist Sarcoma Surgeon. All approaches for biopsy are discussed in the multidisciplinary setting and under the guidance of the Specialist Bone & Soft Tissue Sarcoma Surgeon, as this will avoid poor biopsy placement and its catastrophic complications. Pre-biopsy MRI or CT scan is very useful to plan biopsy. Biopsy will be performed in line with the ultimate operative incision to allow excision of the biopsy track and clearance of the tumour. This is best undertaken under the supervision of or after consultation with a bone and soft tissue tumour surgeon.
4 – Determination of Treatment (Achieving Multidisciplinary Care)
The results of the imaging and biopsy are discussed in a multidisciplinary setting where expert opinions related to the diagnosis and subsequent treatment can be offered. Consultation with patients and their families and support networks are integral to satisfactory outcomes & time is taken to achieve this.