The Royal Marsden Solitary fibrous tumours The outcomes of 106 patients illustrating the unpredictable biological behaviour N Alexander, K Thway, JM Thomas, A Hayes, DC Strauss Mr Dirk Strauss Consultant Surgeon The Royal Marsden Introduction Solitary fibrous tumours (SFT) are rare spindle cell tumour May arise anywhere in body Characteristic hypervascular tumours The Royal Marsden Introduction Solitary fibrous tumours (SFT) are rare spindle cell tumours May arise anywhere in body Characteristic hypervascular tumours VEGF over-expressed The Royal Marsden Introduction Solitary fibrous tumours (SFT) are rare spindle cell tumour May arise anywhere in body Characteristic hypervascular tumours VEGF over-expressed Unpredictable behaviour Benign SFT: Although no malignant features are seen, the behaviour of these tumours is unpredictable. The Royal Marsden Benign vs. Malignant SFT Benign or malignant Hypercellular Nuclear polymorphism Mitotic count > 4/10hpf Presence of necrosis The Royal Marsden SFT are unpredictable Case A 42 male Vascular pelvic mass The Royal Marsden SFT are unpredictable Case A 42 male Vascular pelvic mass Case B 40 female Vascular pelvic mass The Royal Marsden SFT are unpredictable Case A EUA and core needle biopsy Solitary Fibrous Tumour (Benign) Surgical resection incomplete resection due to significant pelvic bleeding The Royal Marsden SFT are unpredictable Case A EUA and core needle biopsy Solitary Fibrous Tumour (Benign) Surgical resection incomplete resection due to significant pelvic bleeding Case B EUA and core needle biopsy x 2 Solitary Fibrous Tumour (Benign) Minimal clinical symptoms therefore opted to watch and wait The Royal Marsden SFT are unpredictable Case A 6 years following incomplete resection continuing radiological observation of residual tumour -> stable and unchanged The Royal Marsden SFT are unpredictable Case A 6 years following incomplete resection continuing radiological observation of residual tumour -> stable and unchanged Case B 13 months from diagnosis presented with right arm pain and lytic lesion in humerus + lung metastases died within 3 years of diagnosis The Royal Marsden Aim To review the clinical outcomes of patients managed with extra-pleural SFT The Royal Marsden Method Retrospective review Cases identified from histopathology database and prospective unit database Classified as benign or malignant on basis of histopathology (surgery, biopsy) Data collection included site, size, overall survival, local and systemic disease recurrence The Royal Marsden Results - Demographics 106 cases identified 51 males, 55 female median age = 60 years (range ) 58 benign vs. 48 malignant The Royal Marsden DemographicBenign (n=58)Malignant (n=48) Sex Male 30 Female 28 Male 21 Female 27 Median Age59yrs60yrs Tumour location Limb/limb girdle Abdominal Tumour Size 20cm Results - Demographics The Royal Marsden Results - Site and Size The Royal Marsden Results - Treatment 91 patients underwent surgical resection Followed up for median 45 months (range 3-144) 2 patients died in perioperative period 15 elected not to operate 9 radiologic surveillance 4 primary radiotherapy 2 primary chemotherapy The Royal Marsden Results - watch N = 9 patients: 8 abdomen/pelvis, 1 limb girdle Benign SFT on biopsy Serial cross sectional imaging Median follow up 28 months (11-60) 1 death at 37 months metastasis 5 stable disease 11 55 months 3 marginal increase over 28, 37, 60 months The Royal Marsden Results - Histopathology 58 benign vs. 48 malignant 91 patient had a biopsy + surgical resection Final pathology diagnosis of resection specimen was different to core needle biopsy in 18 patients (20%) The Royal Marsden Results - surgery BenignMalignant Local recurrence9% (4/46)31% (14/45) Time to Local recurrence 54 months (12-95) 16 months (3-84) The Royal Marsden Results - surgery BenignMalignant Local recurrence9% (4/46)31% (14/45) Time to Local recurrence 54 months (12-95) 16 months (3-84) Distant Metastasis4% (2/56) 40% (25/48) Time to Distant Metastasis 13 months 101 m0nths 30 months (0-142) The Royal Marsden BenignMalignant 5yr local recurrence free93%65% Results - surgery The Royal Marsden BenignMalignant 5yr metastasis free survival98%62% Results The Royal Marsden Results BenignMalignant 5yr overall survival96%46% 10yr overall survival96%26% The Royal Marsden Primary radiotherapy N = 4 patients 1 malignant SFT, 3 benign SFT Size 2 stable (14, 49 months) 2 regression (32, 43 months) The Royal Marsden Primary radiotherapy March 2011 > August 2013 The Royal Marsden Response Assessment in Radiotherapy T2W ADC Contrast enhanced BOLD 2 weeks following radiotherapy The Royal Marsden Conclusion Oncological behaviour of SFT is unpredictable The Royal Marsden Conclusion Oncological behaviour of SFT is unpredictable Available pathologic markers of malignancy clearly correspond to a malignant behaviour with poor prognosis The Royal Marsden Conclusion Oncological behaviour of SFT is unpredictable Available pathologic markers of malignancy clearly correspond to a malignant behaviour with poor prognosis However: many pathological markers subjective: cellularity, necrosis, pleomorphism Risk model similar to GIST (mitotic index/size/site/age/margins/??) Benign v malignant: simplistic/confusing low/intermediate/high risk SFT The Royal Marsden Conclusion Oncological behaviour of SFT is unpredictable Available pathologic markers of malignancy clearly correspond to a malignant behaviour with poor prognosis Core needle biopsy may not represent final diagnosis The Royal Marsden Conclusion Oncological behaviour of SFT is unpredictable Available pathologic markers of malignancy clearly correspond to a malignant behaviour with poor prognosis Core needle biopsy may not represent final diagnosis Late relapses can occur in SFT The Royal Marsden Conclusion A cohort of difficult location tumours may be managed with observation The Royal Marsden Conclusion A cohort of difficult location tumours may be managed with observation Radiotherapy: vascular effect +/- multikinase anti-angiogenesis inhibitors primary treatment/neoadjuvant treatment in tumours in difficult locations The Royal Marsden Thank you