N Alexander, K Thway , JM Thomas, A Hayes, DC Strauss

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


– Characteristic hypervascular tumours VEGF over-expressed

The Royal Marsden Introduction

– Solitary fibrous tumours (SFT) are rare spindle cell tumour


– 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


Case A– 42 male– Vascular pelvic mass

Case B– 40 female– Vascular pelvic mass


Case A

– EUA and core needle biopsy

– Solitary Fibrous Tumour (Benign)

– Surgical resection• incomplete

resection due to significant pelvic bleeding


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


Case A

– 6 years following incomplete resection

– continuing radiological observation of residual tumour -> stable and unchanged


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

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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

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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

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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%)

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Results - surgery

Benign Malignant

Local recurrence 9% (4/46) 31% (14/45)

Time to Local recurrence

54 months (12-95)

16 months (3-84)

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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

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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



– 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




– Core needle biopsy may not represent final diagnosis




– Core needle biopsy may not represent final diagnosis

– Late relapses can occur in SFT


– A cohort of difficult location tumours may be managed with observation


– 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

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Thank you

N Alexander, K Thway , JM Thomas, A Hayes, DC Strauss

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