Pathway – retroperitoneal tumours

*Imaging suggestive of retroperitoneal tumour - do not biopsy *OR unexpected sarcoma pathology

Refer to Soft Tissue Sarcoma MDT at RLBUHT via Tel: 0151 706 4884 FAX 0151 706 5027 (MDT coordinator) or via [email protected]

Soft tissue sarcoma MDT

MDT will discuss with retroperitoneal tumour lead surgeon present (MDT core member)

Review imaging

Decide whether to biopsy Communicate plan to referrer Not sarcoma – Confirm the clinical nurse specialist/ key worker communicate If suspected to be complex benign case, or other malignancy, back to referrer soft tissue sarcoma MDT will remain involved if the for management retroperitoneal teams’ skills are relevant

If biopsy, specialist sarcoma pathology review

If sarcoma (or other pathology requiring similar management) Treatment decision Surgery (involving other specialty surgeons as appropriate) Radiotherapy Systemic therapy Palliative care

Page 1 of 3 Merseyside, Cheshire, Lancashire and South Cumbria suspected retroperitoneal tumour pathway. Agreed June 2013 – Review June 2014 – Review November 2015 – Next review November 2017

Aim and scope:  To ensure that patients with retroperitoneal tumours are discussed at the sarcoma soft tissue MDT (including retroperitoneal and mesenteric liposarcomas; retroperitoneal leiomyosarcomas; other rare tumours involving the abdominal cavity that are not organ-specific).  Neuroendocrine malignancies (including paragangliomas), metastatic tumours and lymphomas are not covered by this pathway  Gastrointestinal stromal tumours presenting as visceral, mesenteric or retroperitoneal masses should be considered initially via the GIST pathway.

Service information: The soft tissue sarcoma MDT is at the Royal Liverpool and Broadgreen University Hospital Trust (RLBUHT). It is a specialist MDT serving the population of Merseyside, Cheshire, Lancashire and South Cumbria. Specialists from organisations other than RLBUHT attend and are core members of the MDT.

The retroperitoneal surgeons for the soft tissue sarcoma MDT are Mr Hassan Malik from Merseyside and Cheshire (HPB surgeon at Aintree) and Mr Colin Harris from Lancashire and South Cumbria (gastro/ liver surgeon from East Lancashire Teaching Hospital). They operate at their respective trusts in these cases, so surgical follow up would be through their clinics at their sites and the local oncology clinics. They attend the soft tissue sarcoma MDT, and bring specialists in from other specialties where this would be appropriate for individual cases.

Principles:  All patients with retroperitoneal tumours should be discussed at the Liverpool Soft Tissue Sarcoma MDT prior to biopsy or treatment  All tissue suggestive of sarcoma should be reviewed by a specialised sarcoma pathologist and reporting in accordance with the guidance published by the Royal College of Pathologistsi  Clinical treatment guidelines areas per the British Sarcoma Groupii

Responsibilities: Referring clinicians:  Please send details of a patient with a suspected retroperitoneal sarcoma to the sarcoma MDT Coordinator via tel: 0151 706 4884 and fax: 0151 706 5027  The referring clinician must inform the patient that a referral has been made to the sarcoma MDT, and advise the patient of the outcome of that discussion  The referring clinician is responsible for the patient’s care, communication to the patient and the organisation of their ongoing care until care is formally transferred to another named clinician via a referral, and the patient is seen.

Retroperitoneal sarcoma surgeons:  Are core members of the soft tissue sarcoma MDT, and are committed to regular MDT attendance  Audit numbers treated surgically and outcomes of surgical treatment  Are committed to developing a specialism in this area, and working collaboratively with colleagues in other surgical specialties (including HPB, urology, gynaecology, upper GI) to draw on the skills of others where appropriate.

Liverpool Sarcoma Multidisciplinary Team  MDT Coordinator receives referrals and ensures that patients are listed for discussion at an appropriate meeting, preferably when the retroperitoneal surgical team(s) is represented. Imaging studies are requested and biopsy material is requested for pathological review (where relevant)  Lead Clinician ensures that decisions on management are correctly recorded on the Somerset Page 2 of 3 Merseyside, Cheshire, Lancashire and South Cumbria suspected retroperitoneal tumour pathway. Agreed June 2013 – Review June 2014 – Review November 2015 – Next review November 2017

Cancer Register and that the decisions are communicated to relevant clinicians.  The research lead for the MDT will ensure that all patients are considered for clinical trials, where appropriate.  The Somerset Cancer Register is the primary database for recording clinical activity for patients with retroperitoneal sarcomas. These data will be audited, as a minimum, annually.

Data capture For accurate data capture, please notify the sarcoma MDT of all sarcoma diagnoses at diagnosis, including those that are not referred to the sarcoma MDT for clinical discussions and/ or management. Please do this via email to [email protected] for the attention of Sarcoma MDT or fax to the MDT Coordinator on 0151 706 5027.

Clinical follow-up Merseyside and Cheshire:  Clinical follow up is undertaken at Aintree Hospital and Clatterbridge Cancer Centre  Scans are undertaken locally, and reviewed centrally at Aintree/ Clatterbridge Cancer Centre

Lancashire and South Cumbria:  Clinical follow up is undertaken at East Lancashire NHS trust, either Royal Blackburn or Burnley General Hospitals and scans undertaken locally and reviewed centrally. All recurrent tumours should be discussed at the soft tissue sarcoma MDT Resectable Primary Therapy  No follow up chest imaging indicated for cases of well differentiated liposarcoma unless suspicious lesions present on staging imaging.

AJCC Stage I Baseline MRI 3 months post-adjuvant therapy, then MRI at 1 year

CXR every 6 months for 1 year then annually for 5 years

No follow-up chest imaging is indicated for patients with well differentiated liposarcoma

AJCC Stage II-III Baseline MRI 3 months post-adjuvant therapy

If pulmonary metastases - baseline chest CT 3 months after operation

If no metastases: 3 monthly CXR for 2 years

6 monthly CXR for next 3 years

then annual CXR 5-10 yrs

MRI of primary site at 2 years (reserve contrast for selected cases)

Post-operative seroma – MRI +/- gadolinium; reserve US +/- biopsy for equivocal cases

PET-CT - Currently PET-CT is indicated for cases of Ewing’s Sarcoma to assess residual disease following therapy / surgery where MRI is equivocal.

C-11 Methionine PET-CT (where available) is indicated for selected cases of neurofibromatosis type-1 to facilitate detection of malignant transformation of neurofibromata where clinical/MRI criteria are equivocal.

i The Royal College of Pathologists; Standards and datasets for reporting cancers Dataset for cancer histopathology reports on soft tissue sarcomas, October 2012 ii http://www.britishsarcomagroup.org.uk/#/guidelines/4556224410 Page 3 of 3 Merseyside, Cheshire, Lancashire and South Cumbria suspected retroperitoneal tumour pathway. Agreed June 2013 – Review June 2014 – Review November 2015 – Next review November 2017