There are a wide range of benign bone tumours and conditions that are tumour-like in nature.  Many benign tumours of bone can be locally destructive and aggressive and can present with quite worrying features that can lead to misdiagnosis as cancers.  The term ‘benign’ indicates that the tumour tends to be a localised issue and will not have the ability to spread itself to other bones and organs.  This is the difference when compared to cancers of the bones (which are malignant tumours and can metastasise to other areas of the body).

 

Just because a tumour is benign, however, does not mean that it can be ignored.  Many benign bone tumours can be very locally aggressive and cause significant problems if they are not correctly attended to.  Many benign bone tumours do have the capacity to destroy the bone and cause fractures and also joint pathologies and problems.

 

Again, due to the rarity and complexity in the diagnosis and management of these conditions, their care must be managed by experts so that outcomes are as favourable as possible.

 

CARE SYSTEM & PRINCIPLES

It is best practice to have the care of (benign) bone tumours & tumour-like lesions to be overseen by a specialist who is a member of a multidisciplinary care team with experience in the diagnosis and management of these conditions.  A multidisciplinary care team is a group of health professionals that meet regularly to oversee the diagnosis and staging of these conditions, as well as the development of individualised treatment plans for people with these tumours.

 

The South Australian Sarcoma & Bone Tumour Unit was established in 2015 & is an integrated Multidisciplinary System in SA for dealing with tumours (both benign and malignant) of connective tissue & musculoskeletal origin.  It provides a collaborative, multidisciplinary approach providing coordinated and comprehensive care for all tumours of the bone & also tumour-like lesions.  Specialist care is provided to all patients who present with bone and soft tissue tumours, and paediatric care is undertaken in collaboration with specialists from the Women’s & Children’s Hospital.  The service receives referrals from South Australia & Northern Territory & collaborates with The Victorian Bone and Soft Tissue (Sarcoma) Tumour Service, The Sydney Sarcoma Services at the Royal Prince Alfred Hospital & The Bone Tumour Unit of The Royal National Orthopaedic Hospital in Stanmore, London.

 

The principles of care are:

• Care will be delivered based on best practice.
• The service delivery model is based on coordinated multidisciplinary team care.
• Services will be arranged to provide ease of access and equity of care for patients, regardless of their point of entry.
• Care will be planned and delivered in partnership with patients, families and carers, as appropriate.
• Physical, psychosocial, spiritual and cultural needs will be addressed throughout the patient journey from screening, diagnosis and treatment to follow-up, survivorship and end-of-life phases of care.
• Research will be integral to clinical practice (“research-led clinical practice”), and care will be evidence-based as well as evidence-generating.
• Education and training will be integral to clinical practice.

 

BENIGN BONE TUMOUR PATIENT PATHWAY

Patients with benign bone tumours may be unaware of their presence until the bone is under strain or breaks.  Sometimes lesions are found incidentally when people are being investigated for other problems – as is the case commonly for cartilage tumours and simple bone cysts.  Occasionally lesions produce pain that can be functional or when the bone thins considerably, unremitting and deep.  It is sometimes very difficult to differentiate between benign and malignant lesions, hence the need for expert care and review.

 

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 tumour or bone abnormality, general practitioners often refer patients to an orthopaedic 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 tumour expert.  From here, further investigations will be performed a needed, but will often require advanced imaging and sometimes a subsequent tissue biopsy.  This will all be under the guidance of a tumour 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 lesion is from.  Biopsy for histologic examination is best performed after anatomic imaging of the tumour or lesion.  The safest form of biopsy is an image-guided biopsy to target the most representative area of the lesion.  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 complications.  Pre-biopsy MRI or CT scan is very useful to plan biopsy.  Biopsy will be performed in line with any ultimate operative incision.  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.

 

TREATMENTS FOR BENIGN TUMOURS & TUMOUR-LIKE LESIONS

The SA Sarcoma & Bone Tumour Unit provides a range of treatment options for patients.

 

ADJUVANT MEDICAL TREATMENTS

Some benign but locally aggressive tumours may necessitate medical management prior to any surgical interventions.  Others still may rely entirely on medical management, with surgery only being contemplated if all other avenues of treatment fail.  All treatment strategies are discussed in the multidisciplinary setting and often a Medical Oncologist will lead the medical treatment of these tumour classes, despite their benign nature.  On other occasions a Metabolic Bone Physician may be consulted.  In all cases the appropriate specialist will lead the care that is required of the pathology diagnosis of the lesion.

 

SURGERY

A comprehensive range of surgery is provided by the SA Sarcoma & Bone Tumour Unit.  Surgery is performed with curative or palliative intent, depending on the specific clinical case of each patients’ problems.  Occasionally, a multidisciplinary decision is made to withhold surgery.

The principles of surgery include:

• Surgery will always be planned and performed by an expert in the field of Bone Tumour Surgery.
• Adequate and appropriate imaging and pathological investigation of the tumour.
• Multidisciplinary input for all cases prior to operative intervention
• Thorough pre-surgical planning and reconstructive planning to allow best care based on the pathology
• Aiming for limb-salvage surgery where possible.
• Early (pre-surgical) involvement of experienced plastic surgical team for soft tissue reconstructions where needed.
• The timing of surgery will often follow specific guidelines, dictated by the nature of any neo-adjuvant treatment.

In unusual cases, collaboration with the The Sydney Sarcoma Services at the Royal Prince Alfred Hospital is necessitated to affect the surgical goals & this may require patients to travel interstate.

 

Surgical care supervised by the Bone Tumour Unit is provided at Flinders Medical Centre, Flinders Private Hospital, Stirling Private Hospital & the Women’s and Children’s Hospital.