Letters to the Editor

Sir: Many thanks for asking me to comment on the Dutch consensus document on `Diagnosis and Treatment of Soft Tissue Tumours’ . The Dutch consensus document on soft tissue sarcoma (STS) is well done, comprehensible and apart from minor remarks it could be basically accepted as a standard. Nevertheless, the document gives the impression that agreement exists among specialists in the treatment of STS, and that by simply following these guidelines, everyone is allowed to treat these lesions. In practice this is far from true. In our major Italian cancer centre, and it happens in many other European institutions, 60% of STS are seen for rescue treatment after inadequate therapies performed elsewhere. After important progress in surgical culture and procedures, the likelihood of achieving a good local control improved, but only in major institutions. Results of treatment remain very poor. Surgeons and radiotherapists insist on stressing their important role while up to 50% of patients with high-grade tumours die from metastatic disease waiting for a missing effective medical treatment. In these circumstances nothing up to now can be standardized or imposed. Surgery is also not optimal even where skill and facilities are best, but the overall survival for metastatic spread discourages us from more heavy, mutilating or dangerous procedures. The document discusses the possible dangerous impact of the unexpected ® nding of sarcoma, typically after minor operations performed in non-oncological departments. Despite expectations, these patients have, in our series, a better prognosis (data reported at the ECCO Conference, Hamburg 1997). This fact is probably due to a favourable selection of patients presenting with small super® cial lesions with a slow growing pattern. Radiotherapy (RT) is accepted as a necessary procedure complementary to surgery, but it is common experience that its effect is proportional to the quantity of tumour tissue left behind; the treatment is acceptable if only a microscopic residual is left after an adequate macroscopic complete resection. It is ineffective if macroscopic residual tumour is left, even if minimal. It should be stressed, and it is not clear in the document, that RT is not a remedy for incomplete or improvable surgery. Again the timing of RT is not yet standardized. Many centres have a satisfactory experience with pre-operative RT, and in our Institute we are performing a new protocol with pre-operative RT for retroperitoneal tumours. Chemotherapy is a most confused topic. Reading the literature, adjuvant chemotherapy is not indicated outside clinical trials. The meta-analysis stimulates ethical questions and in the near future other investigators will probably state that adjuvant chemotherapy is mandatory in selected cases. A minor remark is that the term of `compartment’ , so frequently adopted since the early 1980s, is now obsolete. In fact the only useful compartment that determines the quality of surgery is the muscular fascia. The de® nition of intramuscular or extramuscular lesion should be preferred. In conclusion sarcomas are rare, the data confused and physicians should not have the privilege to treat patients by their own feeling. It is more ethical for any eligible patient to be entered into a clinical trial, and that for any simple or complicated case of STS, or where it is suspected, the physician not familiar with this disease should contact a speci® c centre.


LETTERS TO THE ED ITOR
Sir: M any thanks for asking me to comm ent on the D utch consensus docum ent on`Diagnosis and T reatm ent of Soft Tissue Tum ours' .
The D utch consensus docum ent on soft tissue sarcom a (STS) is well done, com prehensible and apart from m inor rem arks it could be basically accepted as a standard.
N evertheless, the docum ent gives the im pression that agreement exists am ong specialists in the treatm ent of ST S, and that by sim ply follow ing these guidelines, everyone is allowed to treat these lesions. In practice this is far from true.
In our m ajor Italian cancer centre, and it hap pens in many other European institutions, 60% of ST S are seen for rescue treatm ent after inadequate therapies perform ed elsewhere. After im portant progress in surgical culture and procedures, the likelihood of achieving a good local control im proved, but only in m ajor institutions.
Results of treatment rem ain very poor. Surgeons and radiotherapists insist on stressing their im portant role while up to 50% of patients with high-grade tum ours die from m etastatic disease waiting for a m issing effective m edical treatm ent. In these circum stances nothing up to now can be standardized or im posed.
Surgery is also not optim al even where skill and facilities are best, but the overall survival for m etastatic spread discourages us from m ore heavy, m utilating or dangerous procedures. T he docum ent discusses the possible dangerous im pact of the unexpected ® nding of sarcom a, typically after m inor operations performed in non-oncological departm ents. D espite expectations, these patients have, in our series, a better prognosis (data reported at the EC CO C onference, Ham burg 1997). This fact is probably due to a favou rable selection of patients presenting w ith sm all super® cial lesions with a slow growing pattern.
Radiotherapy (RT ) is accepted as a necessary procedure com plem entary to surgery, but it is comm on experience that its effect is proportional to the quantity of tum our tissue left behind; the treatm ent is acceptable if only a m icroscopic residual is left after an adequate m acroscopic com plete resection. It is ineffective if m acroscopic residual tumour is left, even if m inim al. It should be stressed, and it is not clear in the docum ent, that RT is not a remedy for incom plete or im provab le surgery. Again the timing of RT is not yet standardized. Many centres have a satisfactory experience with pre-operative RT , and in our Institute we are perform ing a new protocol with pre-operative RT for retroperitoneal tum ours.
Chem otherapy is a m ost confused topic. Reading the literature, adjuvant chemotherapy is not indicated outside clinical trials. The m eta-analysis stimulates ethical questions and in the near future other investigators will probably state that adjuvant chem otherapy is m andatory in selected cases.
A m inor rem ark is that the term of`com partm ent' , so frequently adopted since the early 1980s, is now obsolete. In fact the only useful com partm ent that determines the quality of surgery is the m uscular fasc ia. The de® nition of intram uscular or extram uscular lesion should be preferred.
In conclusion sarcom as are rare, the data confused and physicians should not have the privilege to treat patients by their ow n feeling. It is m ore ethical for any eligible patient to be entered into a clinical trial, and that for any sim ple or com plicated case of STS, or where it is suspected, the physician not fam iliar with this disease should contact a speci® c centre.

Istituto N azionale Tum ori M ilano, Italy
Sir: M any thanks for asking m e to com m ent on the D utch nationwide accepted consensus docum ent oǹ Diagnosis and Treatm ent of Soft T issue Tum ours' .
There is no doubt that the m echanism by which this consensus docum ent was draw n up has in itself been very therapeutic. It appears to have been democratically derived after a total of nine m eetings and clearly m uch thought and effo rt has gone into its production.
It is a very prescriptive docum ent, de® ning what should be done in virtually every circum stance draw ing on standard principles of sarcom a care. I have no doubt that the clinicians who attended the m eetings and who w ere involved in the production of these guidelines were all better inform ed and better equipped to deal with the challenges posed by soft tissue sarcom as at the com pletion of the exercise.
But there are three problem s posed by docum ents such as this: 1. The guidelines are very precise and allow for little latitude. N on-adherence to these guidelines m ay now be unacceptableÐ both clinically and m edico legally. Any aberration from the de® ned pathw ay m ay lay that clinician open to claim s of an unacceptable or im proper standard of care for that patient. This m ay be the case but equally it does not allow for latitude of innovation, particularly for the experienced clinician. F or the occasional surgeon, however, these guidelines represent a m inim um standard of care from which he or she varies at his/her peril. 2. These guidelines m ake turgid reading. H ow m any clinicians who were not actively involved in drawing up the original docum ent will have read them or even be aw are of them . How m any will go and look it up when and if they get a patient with a soft tissue sarcom a? For guidelines to be effective there needs to be a national consensus of not only how the patient should be treated but also by whom the patient should be treated. Should all patients be treated at a national centre or is there still room for thè occasional' surgeon in this ® eld of oncology. W hat are the outcom es, does it m atter?Ð I believe that these are still questions without com plete answers. 3. Any docum ent such as this m ust have an acknowledged time lim it on it such that the guidelines are reviewed and m odi® ed appropriately in light of changing ® ndings and fashion s.
How m ight guidelines such as this be used in the U nited Kingdom ?
It is an unfortunate fact that in the United Kingdom soft tissue sarcom as continue to be m anaged by a variety of surgeons using a whole host of treatments m any of w hich could not be considered optim al in light of current knowledge. T here is acceptance that treatm ent and outcomes tend to be better if patients are treated in specialist centres for all form s of cancer but the data con® rm ing this is sim ply not available for soft tissue sarcom as.
Introduction of a docum ent such as this would help to standardize treatment in the bigger centres (where treatm ent will already follow accepted guidelines) but will probably not in¯uence in the slightest the occasional surgeon who is unlikely to read let alone acknow ledge a docum ent such as this. He w ill rightly insist that there are no data show ing that treatment in a specialist centre is better for the individual. U ntil this is forthcom ing I ® rm ly believe that the m ost that can be done is to set a m inim um standard of care and to widely circulate this in a brief and succinct docum ent (see T able 1).
N ational guidelines should indeed be reached by consensus but should ideally follow the recognition of the strengths and weaknesses of a country' s current outcom es in an attempt to im prove these.

R. J. GRIMER Birm ingham Orthopaedic Oncology Birm ingham, UK.
Tab le 1. Docum ent describing m inim um standard of care in treatment of soft tissue sarcom as

Introduction
Bone and soft tissue sarcomas are a rare and heterogeneous group of tumours. M any clinicians will never see a case during their professional career. Their recognition is important however, because timely investigation and treatment can result in cure. Their management requires close collaboration between designated specialists in a m ultidisciplinary team and early referral to such a regional or supra-regional service will lead to the best clinical and cost effective care.
There guidelines have been produced in line with current accepted practice not only in this country but within Europe and the United States. They stem from consensus conferences in these countries and from published guidelines which are circulated to all interested parties in these countries. There guidelines will be submitted for agreement to the Royal College of Surgeons, The British Orthopaedic Association, The Medical Research Council.

Presentation
Primary bone tumours can occur at any age. Whereas oteosarcoma and Ewing' s sarcoma have their peak incidence in adolescence and early adulthood, chondrosarcoma and osteosarcomas secondary to radiation damage and Paget' s disease increase in frequency with age. Pain is the commonest presenting symptoms in patients with primary bone tumours. Unfortunately, this is a common complaint after m inor trauma and sporting injuries, and a high index of suspicion is required. Features suggestive of bony malignancy are: · night pain · non-mechanical pain (ie. continuous pain not aggravated by exercise) · swelling that is not associated with a joint The changes seen on plain radiographs can be subtle, such as periosteal reaction, bone destruction, new bone formation (calci® cation) and soft tissue swelling. These ® ndings are not speci® c of a bone tumour but are suggestive and always warrant further investigation.
Soft tissue sarcomas increase in frequency with age. Some (particularly in younger patients) may be associated with familial syndromes such as neuro® brom atosis and Li-Fraumeni syndrome. They usually present as a painless m ass. Features of a soft tissue lump suggestive of malignancy are: · size . 5 cm diameter · enlarging · painful · deep to fascia · solid mass (not cystic) · recurrence at site of previous excision (whatever the previous histology) Any such lesions should immediately be referred to a specialist sarcoma service.

Investigations
The biopsy should ideally be undertaken by the specialist surgeon who will be responsible for the de® nitive operation because the biopsy tract will need to be resected at that procedure. An excision biopsy or`shell-out' is never appropriate because sarcomas typically form a pseudocapsule that is not a barrier to spread. The increasing use of cytogenetic analysis in diagnosis and m onitoring of treatment requires fresh tissue from the initial biopsy.
When a sarcoma is suspected or demonstrated, staging investigations should be perform ed by the specialist team before biopsy, because haematoma, oedem a and scar tissue reduce the yield of diagnostic inform ation and delineation of the tumour extent, particularly in relation to neurovascular structures. Films of suspected sarcomas should be reviewed by Radiologists trained and experienced in this work. The following are required before biopsy to de® ne the primary tumour: · high de® nition plain radiographs in two planes · C.T. or preferably M .R.I scan of the tumour The following are required before de® nitive surgery to exclude metastases: · CXR and C.T scan of thorax · bone scan (for prim ary bone tumours) · FBC, U 1 E, LFT, LDH · in selected cases, bone marrow biopsy Close co-operation is required between the radiologists, surgeon, pathologist and oncologists. Sarcomas are not only a heterogeneous group of tumours but a wide range of appearances can occur within individual tumours. Special training and m em bership of national bone or soft tissue sarcoma pathology panels is required to gain experience in the interpretation of these rare tumours. In order to be able to comment on grade, necrosis, mitotic rate and adequacy of the margins, the pathologist m ust be able to orientate the specimen accurately before tissue retraction occurs. Trojani grading should be used for soft tissue sarcomas. Pathology review by a sarcoma panel is obligatory for entry into clinical trials and leads to revision of the diagnosis in 10± 30% of cases.

D e® nitive S urgery
Surgery for primary bone tumours and most soft tissue sarcomas should only be undertaken by appropriately trained surgical oncologists. This will usually imply an orthopaedic oncologist for bone tumours or a surgeon working in a recognised sarcoma unit with appropriate training and experience. It is essential they work within a multidisciplinary team with appropriate support facilities. There is no place for the ª occasional operatorº for these tumours.

Surgery is de® ned as:-
Incom pleteÐ when any m acroscopic tumour is left in situ. This requires re-excision.
M arginalÐ when the pseudocapsule is visible or any clearance margin is , 1 cm without fascia W ide -when there is a cuff of normal tissue at least 1 cm laterally (or a fascial plane) and 3 cm longitudinally R adicalÐ when the entire muscular compartment C ontaminatedÐ when rupture of the pseudocapsule results in tumour spillage

Adjuvant Treatm ent
For primary bone tumours, pre and post operative chemotherapy should be given in accordance with the current MRC/EOI protocols. Referral to a regional oncology unit is essential for this.
For soft tissue sarcomas, the role of adjuvant chemotherapy is uncertain. Patients with high grade tumours should be considered for enrolment into the current EORTC soft tissue sarcoma study. This requires an early decision regarding chem otherapy and all cases should be discussed at a multidisciplinary meeting following de® nitive histology of the resected tumour. Adjuvant radiotherapy is required for most soft tissue sarcomas and close links between the operating surgeon, surgical unit and the local radiotherapist are essential.

Fo llow u p and Recurrence
Patients can present with local recurrence of any sarcoma m any years after prim ary surgery. They should be promptly referred back to the specialist sarcoma team for consideration of de® nitive surgery. Some patients will have metastases at presentation and many others will develop them at a later date. Some of these, particularly pulmonary m etastases, can be treated by surgery. For this reason, sarcoma patients should be followed up by the specialist sarcoma team, with regular C XRs. W idespread metastases are incurable, but can be palliated by chemotherapy in a proportion of case. Treatment should be given by a medical oncologist in the specialist team using current protocols of the M RC and EORTC groups.