Challenges of Rare Cancers… Charlie Candish Consultant Clinical Oncologist Gloucestershire Hospitals NHS Foundation Trust 10th February 2016 charles.candish@glos.nhs.uk

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

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

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

Bone sarcomas and Age

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

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)

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…

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

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

Prognosis…

Suspected Head and Neck Cancer 2 Week Wait Referral Gloucestershire Hospitals NHS Foundation Trust: Fax 08454 222564 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

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

Radioactive Iodine First dose Second Dose

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 0.3-2.0 Indefinitely TSH 0.1-0.5 For 5-10 yrs TSH<0.1 Indefinitely

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 (0.3-2.0)

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

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