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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. Introduction. Solitary fibrous tumours (SFT) are rare spindle cell tumour - PowerPoint PPT Presentation
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The Royal Marsden
Solitary fibrous tumours
The outcomes of 106 patients illustrating the unpredictable biological behaviourN Alexander, K Thway, JM Thomas, A Hayes, DC
Strauss
Mr Dirk StraussConsultant 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 MarsdenBenign vs. Malignant SFTBenign or malignant
– Hypercellular
– Nuclear polymorphism
– Mitotic count > 4/10hpf
– Presence of necrosis
The Royal MarsdenSFT are unpredictable
Case A– 42 male– Vascular pelvic mass
The Royal MarsdenSFT are unpredictable
Case A– 42 male– Vascular pelvic mass
Case B– 40 female– Vascular pelvic mass
The Royal MarsdenSFT are unpredictable
Case A
– EUA and core needle biopsy
– Solitary Fibrous Tumour (Benign)
– Surgical resection• incomplete
resection due to significant pelvic bleeding
The Royal MarsdenSFT 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 MarsdenSFT are unpredictable
Case A
– 6 years following incomplete resection
– continuing radiological observation of residual tumour -> stable and unchanged
The Royal MarsdenSFT 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
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Method– Retrospective review 2000-2012
– 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
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Results - Demographics
• 106 cases identified
• 51 males, 55 female
• median age = 60 years (range 18 - 88)
• 58 benign vs. 48 malignant
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Demographic Benign (n=58) Malignant (n=48)
Sex Male 30Female 28
Male 21Female 27
Median Age 59yrs 60yrsTumour location• Limb/limb girdle• Abdominal
3325
2523
Tumour Size• <5cm• 5-10cm• 10-15cm• 15-20cm• >20cm
6221558
2141485
Results - Demographics
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Results - Site and Size
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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
The Royal Marsden
The Royal Marsden
Results - surgery
Benign Malignant
Local recurrence 9% (4/46) 31% (14/45)
Time to Local recurrence
54 months (12-95)
16 months (3-84)
The Royal Marsden
Results - surgery
Benign Malignant
Local recurrence 9% (4/46) 31% (14/45)
Time to Local recurrence
54 months (12-95)
16 months (3-84)
Distant Metastasis 4% (2/56) 40% (25/48)
Time to Distant Metastasis
13 months101 m0nths
30 months(0-142)
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Benign Malignant5yr local recurrence free
93% 65%
Results - surgery
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Benign Malignant5yr metastasis free survival
98% 62%
Results
The Royal Marsden
Results
Benign Malignant5yr overall survival 96% 46%
10yr overall survival 96% 26%
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Primary radiotherapy
N = 4 patients– 1 malignant SFT, 3 benign SFT
Size– 2 stable (14, 49 months)– 2 regression (32, 43 months)
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Primary radiotherapy
March 2011 –> August 2013
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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