Therapeutische Strategien bei malignen Weichteiltumoren

INTRODUCTION: This study, carried out by the Surgical Oncology Working Group (CAO) of the German Society for Surgery, was performed to analyse the strategies in the treatment of soft tissue sarcomas in adults. METHODS: In a period of 19 months the data on 292 patients suffering from soft tissue sarcomas, treated in 99 surgical departments in Germany, were analysed prospectively. A special questionnaire was developed including pretherapeutic biopsy, previous treatment, definitive surgical treatment, combined modality approach and histopathological results. RESULTS: Thirty-nine per cent of the tumours were treated in university hospitals, 36% in medical centres, 24% in regional hospitals. During the observation period two patients were treated on average (median) by each hospital. Limb-sparing treatment was performed in 96% of the extremity tumours. There was no significant difference in the frequency of R0 resections between the different hospitals. At the university hospitals local extended operations and additive measures were used more often. The indication for adjuvant radiotherapy differed: after compartmental resection, adjuvant radiotherapy was performed in 39% of cases (19/49); after wide-excision of high-grade tumours, in 45% of cases (20/44) no adjuvant radiotherapy was necessary. In spite of less radical treatment in tumours of the trunk, additional radiotherapy was not more frequently performed. CONCLUSION: To improve the quality in the treatment of soft tissue sarcomas it seems to be of great importance to avoid inadequate initial treatment (18%), to respect the rules of oncological surgery (tumour rupture in 7% of cases), to improve the histopathological examination (no R classification in 5-12%) and to develop guidelines for multimodality treatment.     

Surgical strategy in primary retroperitoneal tumours

Sixty-nine patients with primary retroperitoneal tumours (17 benign, 52 malignant including 4 malignant tumours of uncertain origin) were reviewed to determine the best form of surgical strategy. Total resection was performed in 88 per cent of benign cases and in 65 per cent of malignant cases. In 62 per cent of the total resections for malignant tumours, en bloc excision included adjacent organs or anatomical structures. Operative mortality rate (in terms of the total number of operations performed) was 5 per cent. Postoperative complications occurred in 14 per cent and recurrences in 35 per cent. The overall 5-year survival rate was 67 per cent in patients with totally resected tumours and zero in patients whose tumours were treated by partial resection or biopsy. An aggressive surgical approach aimed at total excision of the tumour is the best form of therapy currently available. In the totally resected retroperitoneal tumour, the use of adjuvant radiotherapy and/or chemotherapy depends on the grade of the malignancy and clearance as assessed histologically. Careful follow-up based on the use of computerized axial tomography and ultrasound allows early identification of recurrence at a stage when the recurrence is amenable to total resection.     

The management of diffuse-type giant cell tumour (pigmented villonodular synovitis) and giant cell tumour of tendon sheath (nodular tenosynovitis)

Giant cell tumours (GCT) of the synovium and tendon sheath can be classified into two forms: localised (giant cell tumour of the tendon sheath, or nodular tenosynovitis) and diffuse (diffuse-type giant cell tumour or pigmented villonodular synovitis). The former principally affects the small joints. It presents as a solitary slow-growing tumour with a characteristic appearance on MRI and is treated by surgical excision. There is a significant risk of multiple recurrences with aggressive diffuse disease. A multidisciplinary approach with dedicated MRI, histological assessment and planned surgery with either adjuvant radiotherapy or systemic targeted therapy is required to improve outcomes in recurrent and refractory diffuse-type GCT. Although arthroscopic synovectomy through several portals has been advocated as an alternative to arthrotomy, there is a significant risk of inadequate excision and recurrence, particularly in the posterior compartment of the knee. For local disease partial arthroscopic synovectomy may be sufficient, at the risk of recurrence. For both local and diffuse intra-articular disease open surgery is advised for recurrent disease. Marginal excision with focal disease will suffice, not dissimilar to the treatment of GCT of tendon sheath. For recurrent and extra-articular soft-tissue disease adjuvant therapy, including intra-articular radioactive colloid or moderate-dose external beam radiotherapy, should be considered.     

Functional and oncological outcomes after limb-salvage surgery for primary sarcomas of the upper limb.

The surgical treatment of upper limb sarcoma poses an oncological and reconstructive challenge. 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 an assessment of the functional and oncological outcomes of limb salvage surgery for primary sarcoma of the upper limb and limb girdle in 72 patients referred to a specialist musculoskeletal tumour unit over 9 years. All patients underwent excision of the sarcoma with reconstruction and adjuvant treatment as needed. Functional outcome was assessed using the Toronto Extremity Salvage Score (TESS) after discharge from hospital. The upper limb sarcomas treated by limb-salvage surgery achieved planned margins of excision in 85% of cases with primary surgery. This increased to 100% with re-excision, resulting in local recurrence in 15% and survival of 75% among those at 5 years or more after surgery, while retaining good to excellent function (TESS mean of 87 out of 100). A total of 38 patients completed TESS questionnaires and, as a single group, had a mean TESS of 87. Patient age, anatomical site of tumour and adjuvant treatment made no significant difference to TESS. Liposarcomas had a significantly better TESS than leiomyosarcomas, chondrosarcomas and malignant peripheral nerve sheath tumours. Oncological outcome was assessed in terms of excision margins achieved, local recurrence, re-excision and disease-free survival. Eight patients died of disease, all with high-grade primary tumours. Fourteen had local recurrence, four low-grade disease and the remaining high-grade disease. All four low-grade recurrences were successfully re-excised, as were five of the high-grade recurrences. The remaining five died of disease, with or without further recurrences. Consistently good functional results as measured by TESS were reported by responders, but high-grade disease and early recurrence were identified as predictors of recurrence and death from disease, respectively.     

EVALUATION OF 242 CONSECUTIVE PAROTIDECTOMIES PERFORMED FOR BENIGN AND MALIGNANT DISEASE

Between 1987 and 1992, one surgeon performed 242 parotidectomies in 229 patients. Among 97 patients with benign disease, pleomorphic adenoma (46) and sialo-adenitis (28) predominated, while metastatic melanoma (22) and squamous cell carcinoma (21) were the most common diagnoses among 132 patients with malignancy. The extent of parotidectomies were: 114 ‘appropriate’ resections, 77 complete superficial parotidectomies, 35 near-total conservative and 14 radical total resections. The facial nerve was sacrificed in 19 operations and reconstructed in four. One hundred and five patients had a concomitant neck dissection and 45 patients had adjuvant radiotherapy. Postoperative facial nerve function was normal in 90% of patients with localized tumours and 55% of patients overall. Patients with sialo-adenitis or deep lobe tumours and those having a neck dissection were at greatest risk of facial weakness despite nerve preservation. At a median follow up of 34 months, 13 patients had developed tumour recurrence and attempts at salvage surgery have generally been disappointing.     

Spinal teratoma: is there a place for adjuvant treatment? Two cases and a review of the literature

Spinal teratomas are rare intradural spinal tumours, commonly of benign histological profile. The main treatment remains surgical excision which often is incomplete as the tumour is frequently intimately related to surrounding nerve roots and adjacent spinal cord. The value of adjuvant treatment in the form of radiotherapy has not been clearly identified. We report two patients with spinal teratomas in the lower thoracic and conus region, who were treated with surgical excision and did not receive radiotherapy. One of the tumours recurred at 38 months following surgical excision and required a second operation. We review in addition another 20 patients reported in the literature with such tumours. With a mean follow-up of 25 months a local recurrence rate of 10% was observed, the same in both total and subtotal excision groups. No deaths were reported. Analysis of cumulative survival from surgery to treatment failure demonstrated that recurrence occurs late, verifying a slow biological progression of this tumour type. Although no reliable information of the effect of radiotherapy can be obtained, it is difficult to see how radiotherapy could have an impact on such a slow growing tumour. Initial treatment of choice remains surgical excision. If the teratoma shows any malignant histological features or germ cell elements radiotherapy should be employed after surgical excision, even if apparently totally excised. In the presence of benign histology it would seem appropriate to avoid radiotherapy after initial surgery. In case of tumour recurrence or progression to explore the possibility of further surgery first, before considering radiotherapy which even then may have doubtful efficacy.     

Effective treatment for malignant mediastinal teratoma.

Primary malignant mediastinal teratoma is a rare tumour previously regarded as inevitably fatal. In a series of eight male patients with a mean age of 24 years five remain alive and well. All patients showed raised serum concentrations of human chorionic gonadotrophin or alpha fetoprotein. The patients were treated with intermittent combination chemotherapy that included cisplatin. Six patients responded to chemotherapy with a fall in human chorionic gonadotrophin or alpha fetoprotein to near normal levels and they then had radical excision of the remaining tumour. Living malignant tumour was found in four of the specimens and these patients received postoperative chemotherapy. One patient died after eight months and the remaining five patients are alive and well 13-136 months after the start of treatment. The two patients who did not undergo surgery died at one month and 15 months. Intermittent combination chemotherapy and carefully timed radical excision of these tumours would appear to have produced better results than have been reported in other series.     

Long-term results in patients with T2-3 N0 distal rectal cancer undergoing radiotherapy before transanal endoscopic microsurgery

BACKGROUND: Local excision after radiotherapy for node-negative low rectal cancer may be an alternative to radical excision. This study evaluated the results of local excision in patients with small (less than 3 cm in diameter) T2 and T3 distal rectal tumours following neoadjuvant therapy. METHODS: One hundred patients with rectal cancer (54 uT2 and 46 uT3 uN0 tumours) were enrolled. All patients underwent preoperative radiotherapy followed by local excision by means of transanal endoscopic microsurgery. RESULTS: Definitive histological examination revealed nine pT1, 54 pT2 and 19 pT3 tumours. A complete response (R0) or microscopic residual tumour (R1mic) was found in three and 15 patients respectively. Minor complications occurred in 11 patients and major complications in two. At a median follow-up of 55 (range 7-120) months, the local failure rate was 5 per cent and metastatic disease was found in two patients. The cancer-specific survival rate at 90 months' follow-up was 89 per cent, and the overall survival rate 72 per cent. Salvage abdominoperineal resection was performed in three patients, two of whom were disease free at 15 and 19 months. CONCLUSION: Treatment of small uT2 and uT3 uN0 rectal cancers with preoperative high-dose radiotherapy followed by transanal endoscopic microsurgery is an acceptable alternative to conventional radical resection.     

Intracranial hemangiopericytoma: study of 12 cases

Summary  Most hemangiopericytomas (HPCs) are located in the musculoskeletal system and the skin, while the intracranial location is rare. They represent 2 to 4% in large series of meningeal tumours, thus accounting for less than 1% of all intracranial tumours. Many authors have argued about the true origin of this tumour. The current World Health Organization classification of Central Nervous System tumours distinguishes HPC as an entity of its own, and classified it into the group of “mesenchymal, non-meningothelial tumours”. Radical surgery is the treatment of choice, but must be completed with postoperative radiotherapy, which has proved to be the therapy most strongly related to the final prognosis. HPCs have a relentless tendency for local recurrence and metastases outside the central nervous system which can appear even many years after diagnosis and adequate treatment of the primary tumour.  Twelve patients with intracranial HPC were treated at our Unit between 1978 and 1999. There were 10 women and 2 men. Ten tumours were supratentorial and most located at frontoparietal parasagittal level. The most common manner of presentation was a focal motor deficit. All tumours were hyperdense in the basal Computed Tomography scans and most enhanced homogeneously following intravenous contrast injection. In 50% of cases, tumour margins were irregular or lobulated. Seven tumours were studied with Magnetic Resonance Imaging, being six of them iso-intense with the cortical gray matter on T1-weighted and T2-weighted images. Twenty operations were performed in the 12 patients. In 10 cases radical excision could be achieved with no operative mortality. Total recurrence rate was 33.3%. Eight patients were treated with external radiotherapy at some time through the course of their disease. Eight out of the 12 patients in this series are disease-free (Glasgow Outcome Scale categories 1 and 2) after a mean follow up of 52 months.     

Onkologische Chirurgie der Wirbelsäule

Spine surgery has been significantly influenced during the past 20 years by improvements in anaesthesia and radiology. This progress has also been promoted by technical developments in spinal instrumentation, mainly the introduction of pedicle screws and anterior support with cages. Both techniques allow correction and stabilisation methods that have had a major effect on tumour surgery. These advancements have allowed less experienced spine surgeons to perform tumour surgery, which may have a negative effect on the outcome. From our point of view, it should be required that tumour surgery be performed only in hospitals managing a certain number of tumours annually. For optimal results, en bloc resection and intralesional marginal resection in particular are highly demanding of the surgeon’s technical skills and experience. Second and third operations complicate the intervention unnecessarily. Normally, R0 resection can not be achieved by a second or third revision. For this reason tumour surgery requires a standardised overall concept which must be suited to individual problems. This can be best decided in a tumour board meeting for choosing the options for adjuvant therapy. Only by such a coordinated effort may good mid- and long-term results be achieved. It must be pointed out that en bloc resection is the only surgical therapy that makes a curative approach possible. On the other hand it can also be demonstrated that by making extended, intralesional marginal resections as radical as possible, good mid-term results can be achieved. Here the adjuvant chemo- and radiotherapy play an important role.     

Biopsy of musculoskeletal tumours--beware

BACKGROUND: Biopsy of musculoskeletal tumours is hazardous and, when poorly performed, can compromise limb salvage surgery and patient survival. The aim of the present paper is to examine the early management of such patients referred to the Department of Orthopaedic Surgery, Royal Prince Alfred Hospital, Sydney, Australia with particular reference to biopsy. METHODS: We conducted a prospective audit of all patients referred to our musculoskeletal tumour service during 2002. Inclusion criteria were: all patients with primary tumours of the musculoskeletal system. We compared the outcome of patients biopsied prior to referral with that of patients biopsied in a recognized treatment centre. RESULTS: One hundred and forty-two patients were included. The referring surgeon performed biopsies in 29 cases, of which 20 were malignant lesions. The senior author biopsied the remaining 113 cases, of which 57 were malignant. Definitive treatment was hindered by a badly performed biopsy in 38% of patients biopsied by the referring surgeon. In 25% the definitive treatment had to be changed either to a more radical procedure than would have originally been necessary or to palliative rather than curative intent. Patients biopsied elsewhere were more likely to have an incomplete excision requiring re-excision, more likely to require amputation, and more likely to require adjuvant radiotherapy. CONCLUSIONS: There is a high complication rate when patients with musculoskeletal tumours are biopsied by surgeons inexperienced in their management. These patients are better served by early referral to a specialist centre where staging investigations including biopsy can be performed with minimal morbidity.     

Audit of local recurrence rates following 'ultra'-conservative surgery for invasive breast cancer--a boost to the breast?

BACKGROUND: Conservative breast surgery with postoperative radiotherapy and appropriate systemic therapy is associated with similar outcomes when compared with mastectomy. The reported 5 year local recurrence rate varies between 3% and 15%. We prefer a more conservative 'complete' local excision rather than 'wide' local excision combined with post-operative radical radiotherapy and tumour bed boost with the aim of achieving optimal cosmesis. AIMS: Our review was undertaken to assess whether or not this 'ultra' conservative approach was compromising long-term local control. METHODS: Case notes and pathology reports of patients who underwent conservative surgery for breast cancer from January 1983 to February 2001 were accessed for this audit. Patient demographic data and tumour characteristics were noted. The primary outcome data were the number of local recurrences following invasive breast cancer at 5 and 10 years and the distance from the tumour to the closest margin of excision. RESULTS: At 5 and 10 years there were 16/451 and 5/124 local recurrences, with a local recurrence rate of 3.5% (95% CI, 1.7-4.7%) and 4.1% (95% CI, 0.47-6.5%), respectively. Complete data with regards to the closest histological margin of excision were available in 423 patients. One hundred and sixty-five patients (39%) had their tumours excised with a distance of less than 1 mm to the closest margin. Nearly, all tumours (97.8%) were excised with the distance to the closest margin less than 1 cm and 81% with 5 mm or less. CONCLUSION: It is possible to achieve low local recurrence rates after very conservative surgery for breast cancer when this is combined with radical radiotherapy and an additional tumour bed boost.     

Malignant epithelial parotid tumours

Formal parotidectomy was performed in 120 patients by one surgeon over 7 years. Eighteen patients were referred with malignant salivary tumours (eight recurrent). Low-grade tumours (LGT) were treated by surgery alone; untreated high-grade tumours (HGT) were treated by pre- or post-operative radiotherapy according to clinical findings. The aim of surgery was to obtain tumour clearance, to preserve the facial nerve where possible, and to perform radical neck dissection for HGT when peroperative jugulodigastric lymph node biopsy confirmed metastasis. Five patients (all HGT) with complete facial palsy due to tumour underwent radical parotidectomy; of the remainder, only one suffered further deterioration of facial nerve function after surgery. After 5 years median follow-up from presentation, four patients with HGT have died from metastases; one has developed local recurrence. No patient with LGT has died or developed local recurrence. The survival difference between patients with HGT and LGT is statistically significant (P less than 0.05). A logical management policy for malignant parotid tumours requires knowledge of tumour grade.     

Management of carcinoma of the extrahepatic bile ducts.

The records of 80 cases of carcinoma of the extrahepatic bile ducts were studied. The median duration of survival, in months, of patients who were treated by curative surgery, palliative surgery and radiation, palliative surgery alone and biopsy alone was 21.7, 9.3, 5.5 and 1, respectively. The best results were obtained by excision of the tumour, but only 10% of tumours were considered resectable. A more aggressive surgical approach to tumours still confined to the bile ducts might be expected to increase the resectability rate and improve survival. The use of a U tube is recommended because it ensures greater comfort even though survival is no longer than when a T tube is used. The roles of adjuvant radiotherapy and chemotherapy need further study. Because of some encouraging responses with the use of radiotherapy and the infusion of 5-fluorouracil the authors are currently evaluating these two methods of treatment.     

Gastric leiomyoblastoma: three interesting cases

The leiomyoblastoma of the stomach has emerged as a separate entity different from the well known benign and malignant gastric tumours of smooth muscle origin. The histologic picture is very typical but has puzzled many pathologists in the past. It is important to consider the possibility of this tumour at operation because it will influence the type of operation to be carried out. Even with seeminly hopeless spread a curative resection is often possible. Per-operative frozen section diagnosis is imperative. Enucleation alone is inadequate. Wide local excision without removal of the regional lymph nodes is often sufficient. More radical operations have no apparent effect on survival. A total gastrectomy is only indicated in cases of multiple localisations or recurrences. Primary and secondary excision of metastases should be considered. Prognosis is relatively good and is best evaluated by a combination of clinical and histologic criteria.     

Multi-modality approach in curative local treatment of early rectal carcinomas

Objective Despite recent advances, surgery remains the mainstay for the management of rectal carcinoma. The conventional surgical treatment for low rectal carcinoma is total mesorectal excision. This results in either abdomino‐perineal excision of the rectum (APER) with permanent colostomy or low anterior resection (LAR) usually with a covering stoma. Local resection is an alternative treatment option and this could be offered either using manual trans‐anal resection (TAR) or transanal endoscopic microsurgery (TEM) if the tumour is situated higher. Patients Patient selection is an important factor if local resection is used. No further treatment is necessary for T1 tumours with clear surgical resection margins. Conventional radical surgery should be offered for T1 tumours with close resection margins (<1 mm) or T2 tumours with higher risk of lymph node metastases. Patients were treated by postoperative chemo‐radiotherapy or radiotherapy, if further radical surgery was not considered appropriate or if the patient refused further surgery. Using this approach, we describe our experience of 100 patients treated from January 1992 to June 2002. Results Only 13 patients had surgery alone and 87 patients had radiotherapy either pre‐operative (33 patients), postoperative (25 patients) or radical radiotherapy alone (29 patients). Local recurrence occurred in 10% of patients and salvage surgery was offered in over half (6 patients) of these patients. At median follow up of 33 months (range 3–120 months), the overall survival was 77% reflecting the fact that the majority of these patients were elderly with coexisting medical problems. However, cancer specific survival was 96%. More importantly, only 9 patients had colostomies and colostomy‐free survival in our cohort of patients from Liverpool was 91%. Conclusion We concluded that in selected patients, who were not medically fit (ASA 111 or above) or those who were unable to accept a permanent colostomy, local treatment could be offered with curative intent using a multimodality approach. In our experience, relapses can be salvaged effectively and we recommend a long‐term close follow up policy.     

Radio-recurrent spermatic cord leiomyosarcoma requiring radical surgery and reconstruction using a myocutaneous (tensor fascia lata) flap: a case report and review of the literature

Leiomyosarcomas (LMS) of the spermatic cord are exceedingly rare tumours. Radical inguinal orchiectomy and high ligation of the cord is the standard primary procedure. The extent of soft tissue excision required, including margins, and the precise role of adjuvant radiotherapy (RT), however, remains unclear. A 58-year-old male underwent excision of a large inguinoscrotal mass and orchiectomy. Histological examination revealed the mass to be a LMS of the spermatic cord with the tumour extending to one of the resection margins. Further surgery was nevertheless withheld for fear of creating a significant anatomical defect and hence the patient was referred for radiation therapy. He developed locoregional recurrence 2.5 years later which necessitated radical excision of soft tissues in the lower–anterior abdominal wall and inguinal region, and reconstruction using a tensor fascia lata flap. This report emphasizes the need for primary radical surgery until negative histological margins are achieved even if it involves sacrificing some adjacent normal anatomy. If margins are positive after primary surgery, re-excision should be the rule rather than the exception. Adjuvant RT, though useful, should not be considered as a substitute for complete surgical clearance. To the authors knowledge, this is the first report in medical literature of a case of spermatic cord LMS recurring after surgery and RT, requiring further radical surgery. The pathophysiology and the management of this complex and rare tumour are also reviewed.     

The indication and surgical results of local excision following radiotherapy for low rectal cancer

Aim Radical surgery of rectal cancer is associated with significant morbidity, and some patients with low‐lying lesions must accept a permanent colostomy. The objective of this study was to evaluate the outcome of local excision followed by adjuvant radiotherapy for rectal cancer for curative purposes. Method One hundred and seven patients with rectal carcinoma performed with local excision were analysed retrospectively. Results The procedures of local excision were trans‐anal resection in 83 patients, trans‐sacral resection in 16, trans‐sphincteric local resection in five, and trans‐vaginal resection in three. The overall disease‐free survival rate was 80.4% (86/107), including 90.0% (54/60) for T1 and 72.3% (34/47) for T2 tumours, respectively. Eighty‐two of 107 patients underwent adjuvant postoperative radiotherapy after local excision, and 25 did not, and the DFS rates between radiation and nonradiation group were significantly different for T2 [81.6% (31/38) vs 33.3% (3/9), P < 0.05], but not for T1 tumours (90.9%vs 87.5%, P > 0.05). The rates of local recurrence and distant metastasis were 13.1% (14/107) and 4.7% (5/106), respectively, and the median time to relapse was 15 months (range: 10–53) for local recurrence and 30 months (21–65) for distant recurrence. The risk factors for local recurrence were large tumour (≥3 cm), poorly differentiated adenocarcinoma and T2 tumour. Conclusions Local excision followed adjuvant radiotherapy is an alternative and feasible technique for small T1 rectal cancer in selected cases.     

Surgical clearance of invasive cardiac leiomyosarcoma with concomitant pneumonectomy.

The management of cardiac leiomyosarcoma is still controversial. Due to the rare occurrence and late presentation of such tumours the treatment is essentially palliative. We report on a case in a young female who presented with catastrophic haemodynamic instability and pulmonary venous obstruction by a large mass in her left atrium. Right pneumonectomy was performed as part of emergency surgery in this patient with a left atrial tumour of unknown histology. We feel that an attempt at complete clearance of these tumours is justified, as surgery remains the only chance of long-term survival. Postoperatively, adjuvant chemotherapy and/or radiotherapy may have a role.     

Pre-operative assessment of soft tissue sarcomas by computed tomography

The current trend towards limb-sparing surgery in patients with soft tissue sarcomas has increased the need for accurate pre-operative assessment of the primary tumour. Tumour site, extent and relationship to bone and neurovascular structures determine whether adequate radical surgery is feasible or whether more limited excision as a part of multimodality treatment is appropriate. Pre-operative computed tomography (CT) was compared with operative findings in 38 patients with soft tissue sarcomas. CT prediction of muscle group involvement by tumour was correct in 95 per cent and prediction of major artery or vein invasion was correct in 92 per cent and 97 per cent respectively. Assessment of tumour relationship to major peripheral nerve was accurate for thigh sarcomas but was not possible in upper limb tumours. While CT confirmed frank bony involvement in two patients, it failed to distinguish between tumours closely adjacent to periosteum and those with periosteal invasion. Detection of residual or recurrent tumour nodules is less reliable; CT is most accurate before any surgical intervention.