1. Challenges of Rare Cancers…
Charlie Candish
Consultant Clinical Oncologist
Gloucestershire Hospitals NHS Foundation Trust
10th February 2016

2. Today… Referral pathways Specialist Centres
Who, where, when?
Specialist Centres
Who, where, why?
New complex treatment options
Survivorship
Information
Support
Follow Up

3. Classifying Sarcomas Bone Soft Tissue Sarcomas Others
Osteosarcoma, Ewing’s sarcoma, Other
Soft Tissue Sarcomas
Extremity
Retroperitoneal
Others
H&N, Gynaecological
GIST

4. Where and What is a Sarcoma Specialist Centre?
Sarcoma Centres x x x

5. Bone sarcomas and Age

6. Case 1 - Bone Sarcoma 17 year old (TYA!!) 2012
2WW to Bone Sarcoma Unit
= Royal Orthopaedic Hospital,
Birmingham
Staging, biopsy
Osteosarcoma
Induction chemotherapy
Young Persons Unit, QE
Access to Cheltenham
Support
Surgery
ROH
Post –operative chemotherapy
YPU then Cheltenham for mifamurtide
Follow up
Local
Thoracic surgery for lung metastasis

7. Case 2 – Soft tissue sarcoma - Extremity
35 year old male (2008)
Mass medial right thigh for “months”
2WW to Soft tissue sarcoma unit = Royal Orthopaedic Hospital
Biopsy = ? Ewings ? Extra-skeletal chondro ?Desmoplastic small round cell sarcoma
Chemotherapy
Surgery
Radiotherapy
Relapse (Lung, 2009)
Thoracic surgery
Trabectadin
Pazopanib
Trial (Marsden)

8. Case 3 – Abdominal sarcoma
59 year old, abdo pain 2011
CT (Primary care)
Referred directly to Abdominal Sarcoma Unit (QE, Birmingham)
Biopsy
GIST with exon 11 mutation
Imatinib (NICE)
Surgery at QE
Imatinib for 3 years (NICE)
Follow Up…

9. Thyroid Cancer Background
Overall good prognosis
Rare
Managed by specialists in MDT setting
Oncology – one specialist per million people
131Iodine effective for thyroid remnant ablation and metastatic disease
National Guidelines
Life long follow-up
Monoclonals becoming available

10. Stage
under 45 yrs 45 or over Stage I AnyT, AnyN, M0 pT1, N0, M0 Stage II AnyT, AnyN, M1 pT2, N0, M0 pT3, N0, M0 Stage III pT4, N0, M0 Any pT, N1, M0 Stage IV Any pT, anyN, M1

11. Prognosis…

12. Suspected Head and Neck Cancer 2 Week Wait Referral
Gloucestershire Hospitals NHS Foundation Trust: Fax
Suspected Head and Neck Cancer 2 Week Wait Referral
Thyroid 2WW Criteria
A solitary nodule increasing in size
Unexplained hoarseness or voice changes
A history of neck irradiation
Very young (pre-pubertal) patient
A family history of an endocrine tumour
Patient aged 65 years and older
ONE STOP DIAGNOSTIC CLINIC

13. Case 4 – Thyroid cancer 19 year old 32 weeks pregnant Stridor Surgery
Total thyroidectomy and LND
C- section
Calcium
Histology
Staging
TSH Suppression
Radioiodine

14. Radioactive Iodine
First dose
Second Dose

15. Dynamic Risk Stratification 9-12 months from ablation
Excellent Response
Indeterminate Response
Incomplete Response
All of the following
Suppressed and stimulated Tg < 1ug/l*
Neck US without evidence of disease
Cross sectional imaging and/or nuclear medicine imaging negative (if performed)
Any of the following
Suppressed Tg < 1ug/l and stimulated Tg ≥ 1 and < 10ug/l*
Neck US with non specific changes or stable sub centimetre nodes
Cross sectional imaging and/or nuclear medicine imaging with non-specific changes, although not completely normal
Suppressed Tg ≥ 1ug/l or stimulated Tg ≥ 10ug/l*
Rising Tg
Persistent or newly identified disease on cross-sectional and/or nuclear medicine imaging
Low risk
Intermediate risk
High risk
TSH
Indefinitely
TSH
For 5-10 yrs
TSH<0.1
Indefinitely

16. Primary Care Follow Up for Low Risk?
Yearly neck examination and referral back with any suspicion of relapse
Yearly thyroglobulin measurement and referral back if newly detectable or rising thyroglobulin or anti-thyroglobulin antibodies
Adjust levothyroxine dose to maintain TSH within the low normal range ( )

17. Survivorship Patient Information Sarcoma UK Butterfly Trust Support
CNS
Rare Cancer Support Group
Follow Up
Disease Specific
Specialist
Primary Care

18. Today… Using clinical cases demonstrated:
Referral pathways
2WW Birmingham for STS
2WW Birmingham for primary bone sarcoma
Abdominal sarcoma unit at QE
Thyroid H&N pathway
Specialist Centres
ROH
QE – Young Persons Unit
GHNHSFT for Thyroid
New complex treatment options
Survivorship