Soft tissue sarcomas are a heterogeneous group of malignant tumours arising from connective tissues of mesenchymal origin. By definition sarcomas arising from bone or cartilage are excluded though they have much in common in their behaviour as well as in the approach to their management. A retrospective study of all cases of soft tissue sarcomas diagnosed and notified in the Maltese Islands over the period 1.1.87 to 31.10.89 was carried out. Information was obtained from the cancer registry, the patient’s hospital files and radiographic records. When there was reasonable doubt about the diagnosis a review of histological material submitted was carried out.peer-reviewe

Brincat, Stephen

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Maltese Medical Journal 28 Volume IT l~suc IT '90(91 MULTIMODALrry MANAGEMENf OF SOFr TISSUE SARCOMAS INTRODUCTION Soft tissue sarcomas are a heterogeneous group of malignant tumours arising from connective tissues of mesenchymal origin. By definition sarcomas arising from bone or cartilage are excluded though they have much in common in their behaviour as well as in the approach to their management In this review of our local experience mesotheliomas are also excluded. The incidence of these tumours has considerable geographic vanatIons. Hawaii has the highest incidence (6.2 per lOO,cro). The incidence in the u.K. is 0.5 to 0.8 per lOO,cro and in the U.S. 2 per lOO,cro. Over the last 34 months 24 new cases have been diagnosed and notified in the Maltese Islands. This gives an incidence of 2.27. MATERIALS AND METIIODS A retrospective study of all cases of soft tissue sarcomas diagnosed and notified in the Maltese Islands over the period 1.1.87 to 31.10.89 was carried out Information was obtained from the cancer registry, the patient's hospital ftIcs and radiographic records. When there was reasonable doubt about the diagnosis a review of histological material submitted was carried out RESULTS 24 new cases of soft tissue sarcoma were diagnosed in the period 1.1.87 to 31.10.89 Stephen Brincat (34 months). There were 14 males and 10 females with four sarcomas arising from the uterus, and two arising from breast. There were four children (less than 14 years of age) and two adolescents. Nine patients received surgery alone as their initial treatment. Of these, three patients died in the immediate post operative period, onc developed distant meta.<;tasis at ftfteen months, one developed local recurrence, onc is alive and free of disease at eighteen months and the other three are lost to follow up. Another three patients had surgery limited to biopsy only because of inoperability of the tumour. All these patient'> are now dead (survival = 1,4,6 months) from disseminated disease. Eight patients received a combination of surgery and radiotherapy as primary treatment Five are alive and free of disease, one still has not completed treatment, onc is alive and with distant metastasis and one is lost to follow up. Two patients received surgery and chemotherapy and are both alive and free of disease. Another two patients received surgery, chemotherapy and radiotherapy as their primary treatment. Of these one died of disease at four months and the other is alive and free of disease at two years. The younger patients did considerably better with all four children being presently alive and free of disease. Three of these children received part of their treatment in the UK. Onc adolescent clied with extensive local disease all treatment having been refused. The other adolescent remains alive and (Tee of disease. Of eight patients over the age of sixty years, six are dea.d, one alive with disease and one alive and free of clisease. The thirteen patients still alive have had a median follow up of seventeen months, four case,.<; have been lost to follow up and seven patients are dead. Table 1 lists all our patients giving histology, site or primary, treatment received and result of follow up. DISCUSSION In comparing incidenccs of soft tissue sarcomas from different regions confusion arises because of uncertainty over which cases to include and which to exclude. For example soft tissue sarcomas arising from viscera are often excluded so that leiomyosarcomas of the uterus or bowel would not be included. Rhabdomyosarcoma arising from bladder, uterus or vagina however is usually included. Likewise some would include mesothelioma but most would not Using DR. STEPIIEN HRINCAT (MD MRCP FRCR) ONCOUJGY DEPT. BOFFA HOSPITAL FWRIANA Maltese Medical J ournal 29 Volume IT Issue IT '90/91 TABLE I HISTOLOGY Leiomyosa Leiomyosa Leiomyosa Rhabdomyosa Liposa Alveolar Sa Angiosa M.F.H. M.FH C.C .Sa. Neurofib Sa Myxosa Cystosa Pleom.Sa Fibro.Sa Rhabdomyosa Rhabdomyosa M.FH Stromal Sa D.F.P. Fibro.Sa Leiomyosa Leiomyosa Rhabdomyosa M.FH C.C.Sa. D.F.P. AM.T. Hyst S Ch RT SITE AGE SEX Ileum 72 M Uterus 80 F Liver 62 M Abdo 56 M Thigh 70 M Thigh 17 F Pelvis 65 F Head 3 M Elbow 51 M Kidney 6 mth M Colon 47 M Abdo 79 M Breast 43 F Pelvis 17 F Thigh 56 M Chest 4 M Leg 30 M Chest 50 M Uterus 76 F Shoulder 44 M Breast 44 F Uterus 32 F Uterus 62 F Bladder 7 F Malignant Fibrous Histiocytoma Clear Cell Sarooma Dermatofibrosarcoma Protuberans Autologous Marrow Transplant Hysterectomy Surgery Chemotherapy Radiotherapy these criteria that is excluding viscerdl leiomyosarcomas our incidence would fall to 1.7 per lOO,(Xn Becausc of the rarity of these tumours few centres have gained much experience in their management. Most important studies on the subject have been the result of mul ti centre trials. The prognosis of the various soft tissue sarcomas varies somewhat from one histological variety to another, as does the natural history. By and large however the grade and size of the primary tumour is more important in determining prognosis and treatment than the 1 ST TREATMENT Surgery Pall.RT Surgery Chemo Surg + RT Surg + RT S+ Ch + RT Surgery Surgery Surg + Ch Surgery Pt. Excision Mastectomy Surgery Surgery S+Ch+AMT Surg + RT Surgery Hyst + RT Surg + RT Mastectomy Hyst + RT Hyst + RT Ch + S + RT FOLLOW UP Died 4 wks post op Died 4 rnths post biopsy Died 1 wk post op Died 6 mths post biopsy FOD 6 mths FOD 9 mths Died 4 mths post op FOD 18 mths Immediate post op FOD 14 mths post op Liver mets at 15 mths Died 1 wk post op FOD 11 mths Died 1 mth Lost to follow up FOD 2 yrs FOD 18 mths Recur x 2 after Surg Lost to Follow up FOD 2 yrs Lost to Follow up FOD 2 yrs Alive wtth lung mets FOD 2 yrs histological variety of sarcoma. Embryonal rhabdomyosarcoma is the exception and the behaviour and treatment of this tumour is closer to that of other paediatric solid tumours than to that of the other soft tissue sarcomas. The brief discussion on management that follows refers to non- visceral soft tissue sarcoma and is confined to general principles. Soft tissue sarcomas usually spread by local extension along tissue planes often far from palpable tumour. The development of local recurrence following primary treatment doubles the probability that the patient will die of the sarcoma. Following simple excIsIon recurrence rates are of the order of 70 to 90% (fable 2). Radical surgery is associated with much lower local recurrence rates, but often necessitates amputation of a limb or other mutilating surgery. For surgery to be radical the tumour which is not visualized at operation must be removed en bloc with a 2/3 centimetre margin in all directions. The results of Simon et al (1) provide an excellent review of what can be attained by radical surgery. Their local recurrence rate for all patients was 20%. Tumours in mcessible sites such as pelvis, buttock, groin or shoulder girdle recur more than those in extremities. Maltese Medical Journal 30 Volume IT Issue IT '90(91 Increasing size is also associated with increasing risk of recurrence. The work of Suit and Russell (2) (fable 3) has clearly shown that equally good local control of the primary tumour can be achieved by a combination of simple excision and radiotherapy. The two groups, surgery alone vs. surgery plus radiotherapy in our series are not -comparable. The numbers for comparable cases are too small for meaningful analysis. Radiotherapy can be given pre or post­operatively with advantages and disadvantages for both techniques. If radiation is given pre-Dperatively (3) the volume to be radiated is smaller, inoperable tumours can become operable and the tumour is more clearly outlined at operation. The risk of dissemination at operation is also reduced and treatment starts immediately removing the worrying risk of tumour regrowth post-Dperatively especially if wound healing is delayed while waiting to start radiotherapy. The main disadvantage is that there may be a delay in wound healing. When a small lesion is excised and on histological examination turns out to be a sarcoma radiotherapy can only be given post- operatively. Treatment volumes post-Dperatively must include all areas of possible contamination and are necessary larger. In our series all patients receiving radiotherapy did so post- operatively. Chemotherapy is used to reduce the size of large tumours thereby facilitating subsequent surgery. It is also used palliatively in the treatment of metastatic disease or of fungating inoperable primaries. Its role in adjuvant treatment is still uncertain but ongoing trials are being carried out in an attempt to identify a drug regimen that may improve results. To date one of the more effective ways of delivering chemotherapy in the treatment of sarcomas arising in an extremity has been by intra-arterial perfusion. Because of the complex interaction between the different modalities of treatment available in the management of soft tissue sarcomas a planned combined approach between surgeons and radiotherapists/oncologists is essential. It is also very useful for the clinicians to fully discuss these complex cases with their colleagues in pathology and diagnostic radiology. Both surgery and radiotherapy have to be meticulously planned and executed to obtain optimal results. We now have the facilities to treat the large majority of cases of soft tissue sarcoma adequately in Malta. We will continue to refer most of our children to the U.K. when intensive chemotherapy or highly complex surgery is needed. The chemotherapy programme in these cases which often goes on for several months can usually be continued in Malta. Likewise any radiotherapy needed could TABLE n Results of Surgery Alone. also be delivered in Malta with few exceptions. REFERENCES I) SIMON MA. ENNEKfNG WF: 77IE MANAr,FMFNf OF SOfT 77SSUE SARCOMAS OF 771E EXTREM{f7ES 1976. 1. BONE JOfNT SURG. 58·A; 317·327. 2) surr HD, RUSSEU WO; RADIATION 77ft."RAPY OF SOfT 77SSUE SARCOMAS. CANCER 1975 36; 759 . 764. 1975 . 3) surr IlD. PROPPE KII, MANKIN w. roT AL; PREOPERATIVE RADIATION 'I1IERAPY FOR SARCOMA OF SOfT 'f1SSUE, CANCER 1981 47; 2267 . 2274. 1981. Sirnon et al. definition of local reaJrrence rates: 0/0 100 following 80-100 following 50 following 10-20 following 5 following - - _. -- ­ ----~ incisional biopsy excisional biopsy wide excision radical resection amputation TABLE ill Results of post-operative radiation 1} M.D. Anderson 80% local control wtth 80% good functional result. 2} Massachusetts General Hospttal 92% local control at 2 years 84% local control at 5 years 3} Distal lesions 1 00% control (46/46) Proximal sttes (upper arm, thigh) 36% control. The copyright of this article belongs to the Editorial Board of the Malta Medical Journal. The Malta Medical Journal’s rights in respect of this work are as defined by the Copyright Act (Chapter 415) of the Laws of Malta or as modified by any successive legislation. Users may access this full-text article and can make use of the information contained in accordance with the Copyright Act provided that the author must be properly acknowledged.  Further distribution or reproduction in any format is prohibited without the prior permission of the copyright holder. This article has been reproduced with the authorization of the editor of the Malta Medical Journal (Ref. No 000001)  

Sirnon et al. definition of local reaJrrence rates:

Multimodality management of soft tissue sarcomas

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Multimodality management of soft tissue sarcomas

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