t h e s u r g e o n x x x ( 2 0 1 4 ) 1 e7
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ScienceDirect The Surgeon, Journal of the Royal Colleges of Surgeons of Edinburgh and Ireland www.thesurgeon.net
Soft tissue sarcoma e A review of presentation, management and outcomes in 110 patients* Robert Bains, Ashish Magdum, Waseem Bhat*, Anu Roy, Alastair Platt, Paul Stanley Department of Plastic and Reconstructive Surgery, Castle Hill Hospital, Cottingham, Hull, UK
article info
abstract
Article history:
Soft tissue sarcomas are a rare group of mesenchymal tumours the treatment of which
Received 21 October 2013
poses oncological and reconstructive challenges. Limb-salvage surgery aims to balance
Received in revised form
adequate excision margins for disease control and preservation of important structures to
9 March 2014
retain function. Reported here is the review of the Hull Plastic surgery sarcoma service over
Accepted 7 June 2014
a twelve year period.
Available online xxx
Method: We performed a review of the Hull sarcoma database over a twelve year period between 1997 and 2009. Demographic data, tumour grade, operative details complications
Keywords:
and outcomes were recorded.
Soft tissue sarcoma
Results: The database contained a total of 435 patients with a diagnosis of sarcoma. 110
Presentation
were treated at the Plastic Surgery department over a period of 12 years between 1997 and
Management
2009. The patients treated in our department consisted of 67 males and 43 females (median
Outcomes
age 70 years). The most common histological type was leiomyosarcoma (n ¼ 23). Distribution of anatomical sites affected were head and neck (n ¼ 15), upper limb (n ¼ 17), lower limb (n ¼ 56), trunk (n ¼ 22). Large tumours (greater than 8 cm) n ¼ 30, deep tumours n ¼ 48, and high grade (Trojani 3) n ¼ 33. Patients were treated with surgical excision and postoperative radiotherapy in the high grade groups (2 and 3). A range of reconstructive procedures were required from skin grafting, functional muscle transfer and free flap reconstruction. Nine patients developed regional recurrence, six patients had grade 3 tumours. Three were not resectable. Fourteen patients developed distant metastases, seven had grade 3 tumours, six underwent chemotherapy, two were treated palliatively. There were twenty deaths in this group, of which sixteen were sarcoma related. Deaths in the high risk groups was seven (high grade), nine (deep tumours) and eight (tumour size >8 cm). There were six survivors from eleven in the group with all three of these risk factors. Conclusion: This study summarises the management of sarcoma form one unit over a twelve year period and lends further evidence to the fact that the principles of limb-salvage surgery are applicable to a wide range of tumour-types and grades, to all patient age groups and anatomical sites with good functional results and that local and free flap reconstruction provides wound cover robust enough to withstand courses of radiotherapy. Early
*
This paper was presented at the British Sarcoma Group Meeting, London. * Corresponding author. Leeds General Infirmary, Leeds LS13EX, UK. E-mail address: [email protected] (W. Bhat). http://dx.doi.org/10.1016/j.surge.2014.06.002 1479-666X/© 2014 Royal College of Surgeons of Edinburgh (Scottish charity number SC005317) and Royal College of Surgeons in Ireland. Published by Elsevier Ltd. All rights reserved.
Please cite this article in press as: Bains R, et al., Soft tissue sarcoma e A review of presentation, management and outcomes in 110 patients, The Surgeon (2014), http://dx.doi.org/10.1016/j.surge.2014.06.002
2
t h e s u r g e o n x x x ( 2 0 1 4 ) 1 e7
recurrence of high-grade disease and the development of metastasis carry a worse prognosis, especially if adjuvant therapy cannot be given. Level of evidence: 4. © 2014 Royal College of Surgeons of Edinburgh (Scottish charity number SC005317) and Royal College of Surgeons in Ireland. Published by Elsevier Ltd. All rights reserved.
Introduction Soft tissue sarcomas are a rare group of mesenchymal tumours the treatment of which poses oncological and reconstructive challenges. Limb-salvage surgery aims to balance adequate excision margins for disease control and preservation of all important structures to retain maximum function. Reported here is the review of the Hull Sarcoma service over a twelve year period.
Methods We performed a review of the Hull sarcoma database over a ten year period between 1997 and 2009. Demographic data, tumour grade, operative details complications and outcomes were recorded. Over this time four hundred and four patients had been referred to the sarcoma service and of these one hundred and ten had been managed in the Plastic surgery department. A total of one hundred and ten patients were managed in the Plastic surgery department, sixty seven male and forty three female. The age range at referral was twenty four to ninety one years with a median age of seventy and mean sixty six years. The majority of patients (n ¼ 74) had been referred directly by their general practitioner and a significant number were referred by general surgery (n ¼ 23); usually having had tissue diagnosis made. Other specialities referring patients were dermatology,6 orthopaedics,5 oncology1 and urology.1
Results Presentation The majority of patients presented with a mass (n ¼ 78). This was usually painless and did not have any significant functional impairment. Thirty one patients presented with a visible cutaneous lesion. Distribution of the tumours was similar to that described previously in that the lower limb was most commonly affected (n ¼ 56). Twenty two lesions were located in the trunk region and seventeen in the upper limb. Head and neck sarcomas numbered fifteen, all of which were superficial lesions.
dermatologist and in several cases a relatively narrow margin of excision had been performed as the diagnosis of sarcoma had not been suspected. These cases were discussed at the multi disciplinary team meeting and usually required a wider excision. Patients with a high grade lesion underwent staging computed tomography (CT). Patients presenting with deep lesions underwent imaging, often ultrasound followed by magnetic resonance scanning with gadolinium. Then, where appropriate underwent core biopsy or image guided biopsy.
Diagnoses The most common diagnosis was leiomyosarcoma (n ¼ 23) and liposarcoma (n ¼ 20). Other diagnoses were as follows: dermatofibrosarcoma protruberans (n ¼ 13), fibrosarcoma (n ¼ 10), nerve sheath tumours (n ¼ 6), angiosarcoma (n ¼ 6), malignant fibrous histiocytoma (n ¼ 5), synovial sarcoma (n ¼ 5), pleomorphic sarcoma (n ¼ 4), atypical fibroxanthoma (n ¼ 4), rhabdomyosarcoma (n ¼ 3), chondrosarcoma (n ¼ 3), uncertain type (n ¼ 3), Kaposis sarcoma (n ¼ 2).
Tumour grade Tumours varied in size from one to twenty four centimetres (average size ¼ 6.95 cm, median ¼ 5.5 cm). Twenty six patients had large tumours (over 10 cm diameter on macroscopic histological examination). Size was not recorded in twenty two cases. There were thirty eight T1 tumours (under 5 cm diameter) and fifty T2 (over 5 cm). Some tumours were graded as per the Trojani system although we now use the FNCLCC diagnostic grading criteria. Grade three tumours numbered thirty five, grade two; eighteen and grade one; twenty seven. Tumour grade was not specified in thirty cases (Table 1). Tumours located superficial to deep fascia numbered forty seven, whilst those deep to deep fascia numbered forty one. Table 2 demonstrated the data of combining size and depth data (a ¼ superficial, b ¼ deep). Highest risk (deep, large and grade 3) e 10 pts Table 1 e Grade, size and depth of STS. Grade
Investigation Patients presenting with small cutaneous lesions underwent excision biopsy for diagnosis. In many patients this primary excision had been performed by the general practitioner or
Trojan 1 Trojan 2 Trojan 3 Not specified
Size 27 18 35 30
T1 (5 cm) Not known
Depth 38 50 22
T1a T1b T2a T2b Not known
35 3 12 38 22
Please cite this article in press as: Bains R, et al., Soft tissue sarcoma e A review of presentation, management and outcomes in 110 patients, The Surgeon (2014), http://dx.doi.org/10.1016/j.surge.2014.06.002
t h e s u r g e o n x x x ( 2 0 1 4 ) 1 e7
Tumour stage One patient had regional and two had distal spread of disease at presentation. One patient presented with extensive high grade, non resectable leiomyosarcoma in the gluteal region and inguinal lymphadenopathy. A second patient presented with an eleven centimetre sclerosing epithelioid fibrosarcoma, again in the gluteal region with an associated supraclavicular and pulmonary metastases. One patient with an eleven centimetre cutaneous lower limb angiosarcoma had CT detected liver and adrenal metastases at presentation.
Management e excision The primary aim in all patients was surgical excision with wide margins where possible. Three patients did not undergo excision, the first due to the fact that the primary was not resectable and regional disease was already present. The second patient was not fit for surgery and the third patient refused an operation. All three patients were elderly and frail. Unfortunately all three died following palliative radiotherapy. The intention in all resected tumours was to gain clear margins with at least a centimetre of normal tissue around the tumour. Sixty eight patients had clear margins histologically, nineteen patients had uncertain margins. Twenty three patients had margins that were confirmed to be involved on histology. Fourteen patients with histologically involved margins occurred in the large deep tumour group (T2b) and were due to the involved margin being adjacent to a vital structure such as a nerve or major vessel. Where the grade was two or three the patient was referred for post operative radiotherapy. Eleven patients in the involved margin group subsequently developed local recurrence. There were a total of three deaths in the involved margin group, two of which had developed local recurrence. Limb preserving surgery was performed wherever possible but ultimately four patients underwent amputation. Two patients underwent amputation as the primary procedure and two for control of aggressive locally recurrent disease. A primary amputation of the thumb was performed in one patient for Kaposis sarcoma (Fig. 3). A primary below knee amputation was performed for a synovial sarcoma of the foot/ankle with bone and tendon involvement. One below knee amputation was performed for multiply recurrent high grade fibrosarcoma of the foot and an above knee amputation was performed in one patient for multiple locally recurrent high grade dermatofibrosarcoma protruberans. Both of the patients who had amputations for recurrence are alive and recurrence free at least two years post amputation. The thumb
Table 2 e Data of combining size and depth data (a ¼ superficial, b ¼ deep). T1a T1b T2a T2b Not known
35 3 12 38 22
3
amputation was the only upper limb amputation performed and this was reconstructed with a pollicisation of the index finger.
Management e reconstruction In the majority of cases primary closure of the resection site was possible (n ¼ 33). For larger superficial lesions and peripheral lesions split skin grafting was performed (n ¼ 19). Flap reconstruction was only required for larger lesions and those where excision resulted in a functional defect. Eleven cases required local random pattern flap reconstruction as follows: V-Y flap (n ¼ 2), rhomboid (n ¼ 4), transposition (n ¼ 4), tissue expander and scalp rotation (n ¼ 1). Axial pattern flaps utilised were as follows: fasciocutaneous (n ¼ 4), local muscle (n ¼ 1), pedicled groin (n ¼ 3), pedicled Latissimus Dorsi (n ¼ 3, 1 functional), posterior interosseus artery (n ¼ 1). Free flap reconstruction was performed most commonly following compartmentectomy and other large limb excisions including skin. Flaps used were as follows: groin flap (n ¼ 4) (Fig. 1), Latissimus Dorsi (n ¼ 3) (Fig. 2), Deep Inferior Epigastric Perforator flap (n ¼ 1), anterolateral thigh flap (n ¼ 1). Amputations were performed in four cases as described above.
Radiotherapy Neoadjuvant radiotherapy was not performed in any cases in this series. All patients with grade two and three lesions were considered for radiotherapy and in selected cases for palliation. In total thirty five patients underwent radiotherapy, typically at a dose of around sixty six Gy (range 50e72 Gy). All had grade two or three lesions and it was palliative in two cases. 5 patients later developed local recurrence, 3 regional recurrence, and 5 metastases.
Chemotherapy The majority of tumours in this group are considered only moderately chemosensitive and as such chemotherapy was not utilised as first line management. It was however reserved as palliation for selected patients and in total nine patients were referred for chemotherapy. Standard regimes were used with Doxarubicin as he first line choice and Ifosamide second line. Six patients were treated for distant metastases and one patient for regionally recurrent angiosarcoma. One young patient with embryonal rhabdomyosarcoma was treated with chemotherapy as part of first line management as is routine for this rare chemosensitive tumour. One patient with multiply locally recurrent dermatofibrosarcoma protuberans was treated with Imatinib with excellent resultant control of the tumour. Survival was low in this group as would be expected considering the advanced nature of the disease in this group of patients and seven of the nine subsequently died.
Please cite this article in press as: Bains R, et al., Soft tissue sarcoma e A review of presentation, management and outcomes in 110 patients, The Surgeon (2014), http://dx.doi.org/10.1016/j.surge.2014.06.002
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t h e s u r g e o n x x x ( 2 0 1 4 ) 1 e7
Fig. 1 e Patient with a 10 cm grade 3 pleomorphic rhabdomyosarcoma (T2bN0M0) in the left forearm (a). Appearance following excision and reconstruction with a free groin flap (b).
Outcomes
and one patient developed metastases. There were two sarcoma related deaths in this group.
Outcomes by stage
AJCC stage III tumours
AJCC stage I tumours (grade1, N0M0) There were a total of twenty seven patients with stage 1 disease, twelve superficial and fifteen deep tumours. Size ranged from one to twenty four centimetres maximum tumour diameter. Four patients developed locally recurrent disease in this group, all of which were treated with further resection. One patient developed regional recurrence and metastases and ultimately died of the disease.
AJCC stage II tumours (grade2/3, T1a-b/T2a, N0M0) There were twenty patients in the stage 2 group. All patients underwent excision of their tumours. Seven of these patients were treated with postoperative radiotherapy for close or involved margins of excision. Three patients developed local recurrence requiring further excision. Two patients developed regional recurrence
(grade3,T2bN0M0) Eighteen patients had stage 3 disease and all were treated with surgery. All but one patient in this group also received post operative radiotherapy. The one patient who did not undergo radiotherapy had refused treatment. Six patients subsequently developed metastatic lesions, five with lung metastases and one with a metastasis to the cheek. None of the patients with lung metastases were suitable for resection of the metastasis. The cheek metastasis was resected. All patients who developed metastases subsequently died.
Outcomes by site (all stages) (Table 3) Outcomes e regional recurrence, all groups Nine patients developed nodal disease. The histological primary diagnoses were myxoid sarcoma (n ¼ 2), atypical fibroxanthoma (n ¼ 2), liposarcoma (n ¼ 1), leiomyosarcoma (n ¼ 1), angiosarcoma (n ¼ 1), pleomorphic sarcoma (n ¼ 1) and rhabdomyosarcoma (n ¼ 1). Three of these patients had grade 3 tumours and the others were grade 2. In three patients the nodal disease was not resectable due to involvement of major
Fig. 2 e (a) Patient with a 20 cm grade 3 pleomorphic liposarcoma (T2bN0M0) which was involving the biceps, coracobrachialis, brachialis and musculocutaneous nerve. Appearance following excision and reconstruction with a pedicled functional latissimus dorsi flap (b). The patient is able to use the flap to flex the elbow (c). Patient is alive and disease free at 3 years. Please cite this article in press as: Bains R, et al., Soft tissue sarcoma e A review of presentation, management and outcomes in 110 patients, The Surgeon (2014), http://dx.doi.org/10.1016/j.surge.2014.06.002
t h e s u r g e o n x x x ( 2 0 1 4 ) 1 e7
5
Fig. 3 e This patient required amputation of the dominant right thumb for Kaposis sarcoma. Reconstruction by pollicisation of the index finger was performed. The patient recovered good hand function and is alive at 8 years.
vessels. Two of these patients had advanced disease at time of presentation and neither the primary nor the nodal spread were resectable. Four of the nine patients also developed distant metastases. There were five deaths of the nine patients who developed nodal disease.
Outcomes e mortality, all groups Sixteen patients died over the period of the study where the cause of death was directly related to the sarcoma. Average follow up for these patients was seventy five months with a median follow up of twenty seven months. Twenty five patients had at least five years follow up. Age range of patients at time of death was twenty four to one hundred and one years. Four patients were under sixty years of age. The youngest patient had a grade 3 embryonal rhabdomyosarcoma. He was treated with excision and chemotherapy but died two years later (Table 4).
Discussion Soft tissue sarcomas (STS) are a rare and heterogeneous group of malignant mesenchymal neoplasms that share a common embryological and connective tissue origin. Their cellular origin may derive from various tissue sources including cartilage, fat, nerve, vessel and muscle, and may present as small cutaneous lesions, but the most commonly present in an area of large muscle mass, such as the thigh and arm. Current improvements in patient's quality of life and survival, in STS, have resulted from a combination of many advances in its management. There have been contributions from centralisation of service, multidisciplinary management1 improved surgical technique, multimodality therapy and the development of limb-sparing surgery.2
Table 3 e Outcomes by site (all stages). Upper limb N ¼ 17 AJCCII 5, AJCCIII 3 Local recurrence 5 Regional recurrence 0 Metastases 2 Deaths 2
Lower limb
Trunk
N ¼ 56 0 Local recurrence 10 Regional recurrence 4
N ¼ 22 AJCCII 5, AJCCIII 2 Local recurrence 2 Regional recurrence 3
Metastases 6 Deaths 9
Metastases 4 Deaths 5
All surgical specialities may be involved with the treatment of sarcoma due to the variety of anatomical sites which can be involved. Plastic surgeons may be involved in the excision of cutaneous or limb tumours and tumours requiring reconstruction following excision. Limb sarcoma is very common and indeed the lower limb is the most common primary site. Before 1980, bone and softtissue sarcomas of the extremities were treated almost exclusively with amputation in order to achieve adequate margins to control disease.2 Wide local excision has a greater risk of local recurrence, whereas amputation has significant morbidity because of loss of function and disfigurement yet local recurrence does not affect metastasis and survival5 providing the local recurrence is adequately treated. There has been no statistically significant survival difference found between amputation and limb salvage.3 With recent developments in adjuvant therapy such as radiotherapy more limb preserving procedures are now possible. Patients undergoing limb preserving surgical excision often require large excisions of muscle and skin and often whole compartments are excised. Following these types of excision patients often require complex flap reconstruction. These may be local, regional or free flaps. Limb-salvage surgery aims to strike a balance between achieving sufficient margins in order to control local disease, and preserving critical neurovascular and musculoskeletal structures in order to retain maximal function of the affected limb. The functional benefits of limb-salvage procedures for sarcomas have been well documented4 and more recently studies have shown a statistically significant improvement in functional outcome in limb salvage compared with ablative surgery for upper extremity sarcoma too.20 It has been our practice to perform limb preserving surgery where possible and where this approach is not deemed to compromise treatment of the primary tumour. This approach often resulted in excision of significant muscle mass in some patients and restoration of limb function was thus a consideration in the reconstructive choice. This was particularly the case in the younger patients and good cosmetic outcomes were achievable with free tissue transfer. Patients having compartmentectomy for stage III lesions routinely underwent post operative radiotherapy and again free tissue transfer was often the reconstruction of choice to allow for a well vascularised and robust soft tissue cover of the radiotherapy field. No significant complication arose
Please cite this article in press as: Bains R, et al., Soft tissue sarcoma e A review of presentation, management and outcomes in 110 patients, The Surgeon (2014), http://dx.doi.org/10.1016/j.surge.2014.06.002
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Table 4 e Summary of deaths, tumour demographics, treatment and follow-up. Histology
Angiosacroma Atypical fibroxanthoma Fibrosarcoma Fibrosarcoma Leiomyosarcoma Leiomyosarcoma Leiomyosarcoma Leiomyosarcoma Liposarcoma Liposarcoma Malignant fibrous histiocytoma (MFH) MFH Rhabdomyosarcoma Rhabdomyosarcoma NOS Synovial sarcoma
Size
Grade
Superficial/ deep
Excision margin
Chemotherapy
Radiotherapy
Age
Site
Total follow up
NR 1 cm
NR NR
Sup Sup
NR 3 cm
Yes No
No No
56 87
Trunk L limb
60 mnth 24 mnth
11 cm NR NR 6 cm 10 cm NR NR 21 cm 10 cm
NR NR Low 3 3 NR 2 2 3
Deep Sup Deep Deep Deep Sup Sup Sup Deep
LN neck Clear Clear Focally þve 5 mm NR NR
Available online at www.sciencedirect.com
ScienceDirect The Surgeon, Journal of the Royal Colleges of Surgeons of Edinburgh and Ireland www.thesurgeon.net
Soft tissue sarcoma e A review of presentation, management and outcomes in 110 patients* Robert Bains, Ashish Magdum, Waseem Bhat*, Anu Roy, Alastair Platt, Paul Stanley Department of Plastic and Reconstructive Surgery, Castle Hill Hospital, Cottingham, Hull, UK
article info
abstract
Article history:
Soft tissue sarcomas are a rare group of mesenchymal tumours the treatment of which
Received 21 October 2013
poses oncological and reconstructive challenges. Limb-salvage surgery aims to balance
Received in revised form
adequate excision margins for disease control and preservation of important structures to
9 March 2014
retain function. Reported here is the review of the Hull Plastic surgery sarcoma service over
Accepted 7 June 2014
a twelve year period.
Available online xxx
Method: We performed a review of the Hull sarcoma database over a twelve year period between 1997 and 2009. Demographic data, tumour grade, operative details complications
Keywords:
and outcomes were recorded.
Soft tissue sarcoma
Results: The database contained a total of 435 patients with a diagnosis of sarcoma. 110
Presentation
were treated at the Plastic Surgery department over a period of 12 years between 1997 and
Management
2009. The patients treated in our department consisted of 67 males and 43 females (median
Outcomes
age 70 years). The most common histological type was leiomyosarcoma (n ¼ 23). Distribution of anatomical sites affected were head and neck (n ¼ 15), upper limb (n ¼ 17), lower limb (n ¼ 56), trunk (n ¼ 22). Large tumours (greater than 8 cm) n ¼ 30, deep tumours n ¼ 48, and high grade (Trojani 3) n ¼ 33. Patients were treated with surgical excision and postoperative radiotherapy in the high grade groups (2 and 3). A range of reconstructive procedures were required from skin grafting, functional muscle transfer and free flap reconstruction. Nine patients developed regional recurrence, six patients had grade 3 tumours. Three were not resectable. Fourteen patients developed distant metastases, seven had grade 3 tumours, six underwent chemotherapy, two were treated palliatively. There were twenty deaths in this group, of which sixteen were sarcoma related. Deaths in the high risk groups was seven (high grade), nine (deep tumours) and eight (tumour size >8 cm). There were six survivors from eleven in the group with all three of these risk factors. Conclusion: This study summarises the management of sarcoma form one unit over a twelve year period and lends further evidence to the fact that the principles of limb-salvage surgery are applicable to a wide range of tumour-types and grades, to all patient age groups and anatomical sites with good functional results and that local and free flap reconstruction provides wound cover robust enough to withstand courses of radiotherapy. Early
*
This paper was presented at the British Sarcoma Group Meeting, London. * Corresponding author. Leeds General Infirmary, Leeds LS13EX, UK. E-mail address: [email protected] (W. Bhat). http://dx.doi.org/10.1016/j.surge.2014.06.002 1479-666X/© 2014 Royal College of Surgeons of Edinburgh (Scottish charity number SC005317) and Royal College of Surgeons in Ireland. Published by Elsevier Ltd. All rights reserved.
Please cite this article in press as: Bains R, et al., Soft tissue sarcoma e A review of presentation, management and outcomes in 110 patients, The Surgeon (2014), http://dx.doi.org/10.1016/j.surge.2014.06.002
2
t h e s u r g e o n x x x ( 2 0 1 4 ) 1 e7
recurrence of high-grade disease and the development of metastasis carry a worse prognosis, especially if adjuvant therapy cannot be given. Level of evidence: 4. © 2014 Royal College of Surgeons of Edinburgh (Scottish charity number SC005317) and Royal College of Surgeons in Ireland. Published by Elsevier Ltd. All rights reserved.
Introduction Soft tissue sarcomas are a rare group of mesenchymal tumours the treatment of which poses oncological and reconstructive challenges. Limb-salvage surgery aims to balance adequate excision margins for disease control and preservation of all important structures to retain maximum function. Reported here is the review of the Hull Sarcoma service over a twelve year period.
Methods We performed a review of the Hull sarcoma database over a ten year period between 1997 and 2009. Demographic data, tumour grade, operative details complications and outcomes were recorded. Over this time four hundred and four patients had been referred to the sarcoma service and of these one hundred and ten had been managed in the Plastic surgery department. A total of one hundred and ten patients were managed in the Plastic surgery department, sixty seven male and forty three female. The age range at referral was twenty four to ninety one years with a median age of seventy and mean sixty six years. The majority of patients (n ¼ 74) had been referred directly by their general practitioner and a significant number were referred by general surgery (n ¼ 23); usually having had tissue diagnosis made. Other specialities referring patients were dermatology,6 orthopaedics,5 oncology1 and urology.1
Results Presentation The majority of patients presented with a mass (n ¼ 78). This was usually painless and did not have any significant functional impairment. Thirty one patients presented with a visible cutaneous lesion. Distribution of the tumours was similar to that described previously in that the lower limb was most commonly affected (n ¼ 56). Twenty two lesions were located in the trunk region and seventeen in the upper limb. Head and neck sarcomas numbered fifteen, all of which were superficial lesions.
dermatologist and in several cases a relatively narrow margin of excision had been performed as the diagnosis of sarcoma had not been suspected. These cases were discussed at the multi disciplinary team meeting and usually required a wider excision. Patients with a high grade lesion underwent staging computed tomography (CT). Patients presenting with deep lesions underwent imaging, often ultrasound followed by magnetic resonance scanning with gadolinium. Then, where appropriate underwent core biopsy or image guided biopsy.
Diagnoses The most common diagnosis was leiomyosarcoma (n ¼ 23) and liposarcoma (n ¼ 20). Other diagnoses were as follows: dermatofibrosarcoma protruberans (n ¼ 13), fibrosarcoma (n ¼ 10), nerve sheath tumours (n ¼ 6), angiosarcoma (n ¼ 6), malignant fibrous histiocytoma (n ¼ 5), synovial sarcoma (n ¼ 5), pleomorphic sarcoma (n ¼ 4), atypical fibroxanthoma (n ¼ 4), rhabdomyosarcoma (n ¼ 3), chondrosarcoma (n ¼ 3), uncertain type (n ¼ 3), Kaposis sarcoma (n ¼ 2).
Tumour grade Tumours varied in size from one to twenty four centimetres (average size ¼ 6.95 cm, median ¼ 5.5 cm). Twenty six patients had large tumours (over 10 cm diameter on macroscopic histological examination). Size was not recorded in twenty two cases. There were thirty eight T1 tumours (under 5 cm diameter) and fifty T2 (over 5 cm). Some tumours were graded as per the Trojani system although we now use the FNCLCC diagnostic grading criteria. Grade three tumours numbered thirty five, grade two; eighteen and grade one; twenty seven. Tumour grade was not specified in thirty cases (Table 1). Tumours located superficial to deep fascia numbered forty seven, whilst those deep to deep fascia numbered forty one. Table 2 demonstrated the data of combining size and depth data (a ¼ superficial, b ¼ deep). Highest risk (deep, large and grade 3) e 10 pts Table 1 e Grade, size and depth of STS. Grade
Investigation Patients presenting with small cutaneous lesions underwent excision biopsy for diagnosis. In many patients this primary excision had been performed by the general practitioner or
Trojan 1 Trojan 2 Trojan 3 Not specified
Size 27 18 35 30
T1 (5 cm) Not known
Depth 38 50 22
T1a T1b T2a T2b Not known
35 3 12 38 22
Please cite this article in press as: Bains R, et al., Soft tissue sarcoma e A review of presentation, management and outcomes in 110 patients, The Surgeon (2014), http://dx.doi.org/10.1016/j.surge.2014.06.002
t h e s u r g e o n x x x ( 2 0 1 4 ) 1 e7
Tumour stage One patient had regional and two had distal spread of disease at presentation. One patient presented with extensive high grade, non resectable leiomyosarcoma in the gluteal region and inguinal lymphadenopathy. A second patient presented with an eleven centimetre sclerosing epithelioid fibrosarcoma, again in the gluteal region with an associated supraclavicular and pulmonary metastases. One patient with an eleven centimetre cutaneous lower limb angiosarcoma had CT detected liver and adrenal metastases at presentation.
Management e excision The primary aim in all patients was surgical excision with wide margins where possible. Three patients did not undergo excision, the first due to the fact that the primary was not resectable and regional disease was already present. The second patient was not fit for surgery and the third patient refused an operation. All three patients were elderly and frail. Unfortunately all three died following palliative radiotherapy. The intention in all resected tumours was to gain clear margins with at least a centimetre of normal tissue around the tumour. Sixty eight patients had clear margins histologically, nineteen patients had uncertain margins. Twenty three patients had margins that were confirmed to be involved on histology. Fourteen patients with histologically involved margins occurred in the large deep tumour group (T2b) and were due to the involved margin being adjacent to a vital structure such as a nerve or major vessel. Where the grade was two or three the patient was referred for post operative radiotherapy. Eleven patients in the involved margin group subsequently developed local recurrence. There were a total of three deaths in the involved margin group, two of which had developed local recurrence. Limb preserving surgery was performed wherever possible but ultimately four patients underwent amputation. Two patients underwent amputation as the primary procedure and two for control of aggressive locally recurrent disease. A primary amputation of the thumb was performed in one patient for Kaposis sarcoma (Fig. 3). A primary below knee amputation was performed for a synovial sarcoma of the foot/ankle with bone and tendon involvement. One below knee amputation was performed for multiply recurrent high grade fibrosarcoma of the foot and an above knee amputation was performed in one patient for multiple locally recurrent high grade dermatofibrosarcoma protruberans. Both of the patients who had amputations for recurrence are alive and recurrence free at least two years post amputation. The thumb
Table 2 e Data of combining size and depth data (a ¼ superficial, b ¼ deep). T1a T1b T2a T2b Not known
35 3 12 38 22
3
amputation was the only upper limb amputation performed and this was reconstructed with a pollicisation of the index finger.
Management e reconstruction In the majority of cases primary closure of the resection site was possible (n ¼ 33). For larger superficial lesions and peripheral lesions split skin grafting was performed (n ¼ 19). Flap reconstruction was only required for larger lesions and those where excision resulted in a functional defect. Eleven cases required local random pattern flap reconstruction as follows: V-Y flap (n ¼ 2), rhomboid (n ¼ 4), transposition (n ¼ 4), tissue expander and scalp rotation (n ¼ 1). Axial pattern flaps utilised were as follows: fasciocutaneous (n ¼ 4), local muscle (n ¼ 1), pedicled groin (n ¼ 3), pedicled Latissimus Dorsi (n ¼ 3, 1 functional), posterior interosseus artery (n ¼ 1). Free flap reconstruction was performed most commonly following compartmentectomy and other large limb excisions including skin. Flaps used were as follows: groin flap (n ¼ 4) (Fig. 1), Latissimus Dorsi (n ¼ 3) (Fig. 2), Deep Inferior Epigastric Perforator flap (n ¼ 1), anterolateral thigh flap (n ¼ 1). Amputations were performed in four cases as described above.
Radiotherapy Neoadjuvant radiotherapy was not performed in any cases in this series. All patients with grade two and three lesions were considered for radiotherapy and in selected cases for palliation. In total thirty five patients underwent radiotherapy, typically at a dose of around sixty six Gy (range 50e72 Gy). All had grade two or three lesions and it was palliative in two cases. 5 patients later developed local recurrence, 3 regional recurrence, and 5 metastases.
Chemotherapy The majority of tumours in this group are considered only moderately chemosensitive and as such chemotherapy was not utilised as first line management. It was however reserved as palliation for selected patients and in total nine patients were referred for chemotherapy. Standard regimes were used with Doxarubicin as he first line choice and Ifosamide second line. Six patients were treated for distant metastases and one patient for regionally recurrent angiosarcoma. One young patient with embryonal rhabdomyosarcoma was treated with chemotherapy as part of first line management as is routine for this rare chemosensitive tumour. One patient with multiply locally recurrent dermatofibrosarcoma protuberans was treated with Imatinib with excellent resultant control of the tumour. Survival was low in this group as would be expected considering the advanced nature of the disease in this group of patients and seven of the nine subsequently died.
Please cite this article in press as: Bains R, et al., Soft tissue sarcoma e A review of presentation, management and outcomes in 110 patients, The Surgeon (2014), http://dx.doi.org/10.1016/j.surge.2014.06.002
4
t h e s u r g e o n x x x ( 2 0 1 4 ) 1 e7
Fig. 1 e Patient with a 10 cm grade 3 pleomorphic rhabdomyosarcoma (T2bN0M0) in the left forearm (a). Appearance following excision and reconstruction with a free groin flap (b).
Outcomes
and one patient developed metastases. There were two sarcoma related deaths in this group.
Outcomes by stage
AJCC stage III tumours
AJCC stage I tumours (grade1, N0M0) There were a total of twenty seven patients with stage 1 disease, twelve superficial and fifteen deep tumours. Size ranged from one to twenty four centimetres maximum tumour diameter. Four patients developed locally recurrent disease in this group, all of which were treated with further resection. One patient developed regional recurrence and metastases and ultimately died of the disease.
AJCC stage II tumours (grade2/3, T1a-b/T2a, N0M0) There were twenty patients in the stage 2 group. All patients underwent excision of their tumours. Seven of these patients were treated with postoperative radiotherapy for close or involved margins of excision. Three patients developed local recurrence requiring further excision. Two patients developed regional recurrence
(grade3,T2bN0M0) Eighteen patients had stage 3 disease and all were treated with surgery. All but one patient in this group also received post operative radiotherapy. The one patient who did not undergo radiotherapy had refused treatment. Six patients subsequently developed metastatic lesions, five with lung metastases and one with a metastasis to the cheek. None of the patients with lung metastases were suitable for resection of the metastasis. The cheek metastasis was resected. All patients who developed metastases subsequently died.
Outcomes by site (all stages) (Table 3) Outcomes e regional recurrence, all groups Nine patients developed nodal disease. The histological primary diagnoses were myxoid sarcoma (n ¼ 2), atypical fibroxanthoma (n ¼ 2), liposarcoma (n ¼ 1), leiomyosarcoma (n ¼ 1), angiosarcoma (n ¼ 1), pleomorphic sarcoma (n ¼ 1) and rhabdomyosarcoma (n ¼ 1). Three of these patients had grade 3 tumours and the others were grade 2. In three patients the nodal disease was not resectable due to involvement of major
Fig. 2 e (a) Patient with a 20 cm grade 3 pleomorphic liposarcoma (T2bN0M0) which was involving the biceps, coracobrachialis, brachialis and musculocutaneous nerve. Appearance following excision and reconstruction with a pedicled functional latissimus dorsi flap (b). The patient is able to use the flap to flex the elbow (c). Patient is alive and disease free at 3 years. Please cite this article in press as: Bains R, et al., Soft tissue sarcoma e A review of presentation, management and outcomes in 110 patients, The Surgeon (2014), http://dx.doi.org/10.1016/j.surge.2014.06.002
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Fig. 3 e This patient required amputation of the dominant right thumb for Kaposis sarcoma. Reconstruction by pollicisation of the index finger was performed. The patient recovered good hand function and is alive at 8 years.
vessels. Two of these patients had advanced disease at time of presentation and neither the primary nor the nodal spread were resectable. Four of the nine patients also developed distant metastases. There were five deaths of the nine patients who developed nodal disease.
Outcomes e mortality, all groups Sixteen patients died over the period of the study where the cause of death was directly related to the sarcoma. Average follow up for these patients was seventy five months with a median follow up of twenty seven months. Twenty five patients had at least five years follow up. Age range of patients at time of death was twenty four to one hundred and one years. Four patients were under sixty years of age. The youngest patient had a grade 3 embryonal rhabdomyosarcoma. He was treated with excision and chemotherapy but died two years later (Table 4).
Discussion Soft tissue sarcomas (STS) are a rare and heterogeneous group of malignant mesenchymal neoplasms that share a common embryological and connective tissue origin. Their cellular origin may derive from various tissue sources including cartilage, fat, nerve, vessel and muscle, and may present as small cutaneous lesions, but the most commonly present in an area of large muscle mass, such as the thigh and arm. Current improvements in patient's quality of life and survival, in STS, have resulted from a combination of many advances in its management. There have been contributions from centralisation of service, multidisciplinary management1 improved surgical technique, multimodality therapy and the development of limb-sparing surgery.2
Table 3 e Outcomes by site (all stages). Upper limb N ¼ 17 AJCCII 5, AJCCIII 3 Local recurrence 5 Regional recurrence 0 Metastases 2 Deaths 2
Lower limb
Trunk
N ¼ 56 0 Local recurrence 10 Regional recurrence 4
N ¼ 22 AJCCII 5, AJCCIII 2 Local recurrence 2 Regional recurrence 3
Metastases 6 Deaths 9
Metastases 4 Deaths 5
All surgical specialities may be involved with the treatment of sarcoma due to the variety of anatomical sites which can be involved. Plastic surgeons may be involved in the excision of cutaneous or limb tumours and tumours requiring reconstruction following excision. Limb sarcoma is very common and indeed the lower limb is the most common primary site. Before 1980, bone and softtissue sarcomas of the extremities were treated almost exclusively with amputation in order to achieve adequate margins to control disease.2 Wide local excision has a greater risk of local recurrence, whereas amputation has significant morbidity because of loss of function and disfigurement yet local recurrence does not affect metastasis and survival5 providing the local recurrence is adequately treated. There has been no statistically significant survival difference found between amputation and limb salvage.3 With recent developments in adjuvant therapy such as radiotherapy more limb preserving procedures are now possible. Patients undergoing limb preserving surgical excision often require large excisions of muscle and skin and often whole compartments are excised. Following these types of excision patients often require complex flap reconstruction. These may be local, regional or free flaps. Limb-salvage surgery aims to strike a balance between achieving sufficient margins in order to control local disease, and preserving critical neurovascular and musculoskeletal structures in order to retain maximal function of the affected limb. The functional benefits of limb-salvage procedures for sarcomas have been well documented4 and more recently studies have shown a statistically significant improvement in functional outcome in limb salvage compared with ablative surgery for upper extremity sarcoma too.20 It has been our practice to perform limb preserving surgery where possible and where this approach is not deemed to compromise treatment of the primary tumour. This approach often resulted in excision of significant muscle mass in some patients and restoration of limb function was thus a consideration in the reconstructive choice. This was particularly the case in the younger patients and good cosmetic outcomes were achievable with free tissue transfer. Patients having compartmentectomy for stage III lesions routinely underwent post operative radiotherapy and again free tissue transfer was often the reconstruction of choice to allow for a well vascularised and robust soft tissue cover of the radiotherapy field. No significant complication arose
Please cite this article in press as: Bains R, et al., Soft tissue sarcoma e A review of presentation, management and outcomes in 110 patients, The Surgeon (2014), http://dx.doi.org/10.1016/j.surge.2014.06.002
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Table 4 e Summary of deaths, tumour demographics, treatment and follow-up. Histology
Angiosacroma Atypical fibroxanthoma Fibrosarcoma Fibrosarcoma Leiomyosarcoma Leiomyosarcoma Leiomyosarcoma Leiomyosarcoma Liposarcoma Liposarcoma Malignant fibrous histiocytoma (MFH) MFH Rhabdomyosarcoma Rhabdomyosarcoma NOS Synovial sarcoma
Size
Grade
Superficial/ deep
Excision margin
Chemotherapy
Radiotherapy
Age
Site
Total follow up
NR 1 cm
NR NR
Sup Sup
NR 3 cm
Yes No
No No
56 87
Trunk L limb
60 mnth 24 mnth
11 cm NR NR 6 cm 10 cm NR NR 21 cm 10 cm
NR NR Low 3 3 NR 2 2 3
Deep Sup Deep Deep Deep Sup Sup Sup Deep
LN neck Clear Clear Focally þve 5 mm NR NR
