Hyperthermic isolation perfusion of the limbs with cytostatics after surgical excision of sarcomas

In order to prevent recurrence, isolation perfusion was applied in 15 patients after resection of sarcomas of the limbs. There were 6 liposarcomas, 4 synoviosarcomas, 2 malignant fibrohistiocytomas, 1 malignant mesenchynoma, 1 Kaposi, and 1 osteosarcoma (the only bone sarcoma). Eight sarcomas were recurrent after narrow surgery and/or radiotherapy. The treatment schedule was conducted in 2 steps: (a) removal of the tumor with safety margins at least outside the tumor capsule, followed 3–8 weeks later by (b) isolation perfusion with melphalan with or without actinomycin D under moderate hyperthermia at 39–41°C for 1 hour. Median follow-up time has been 30 months. Four of the 8 recurrent sarcomas recurred again and developed distal metastases. In contrast, none of the previously untreated patients recurred and 1 disseminated in the form of multicentric liposarcoma. Eleven are still alive with survival ranging from 8 to 103 months. It is concluded that isolation perfusion with melphalan with or without actinomycin D may be considered as an adjunct to surgery.

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Resumen Con miras a la prevención de recurrencias, se aplicó perfusión aislada en 15 pacientes después de resección de sarcomas de las extremidades. El grupo incluyó 6 liposarcomas, 4 sinoviosarcomas, 2 fibrohistiocitomas malignos, 1 mesenquimoma maligno, 1 Kaposi, y 1 osteosarcoma (el único sarcoma óseo). Ocho sarcomas representaron recurrencia después de cirugía limitada y/o radioterapia.El programa terapéutico fue conducido en 2 etapas: (a) remoción del tumor con márgenes de seguridad por lo menos por fuera de la cápsula tumoral, seguida 3–8 semanas después de (b) perfusión aislada con melfalán con o sin actinomicina D bajo hipertermia moderada a 39–41°C por 1 hora.El promedio de seguimiento ha sido de 30 meses. Cuatro de los 8 sarcomas recurrentes presentaron la recurrencia de nuevo y desarrollaron metástasis distales. En contraste, ninguno de los pacientes que no habían recibido tratamiento previo presentó recurrencia y en uno se diseminó en la forma de un liposarcoma multicéntrico. Once permanecen vivos con supervivencia entre 8 y 103 meses.Nuestra conclusión es que la perfusión aislada con melfalán con o sin actinomicina D puede ser considerada como una terapia adyuvante de la cirugía.

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Résumé Pour prévenir la récidive des sarcomes, la perfusion isolée a été employée après résection de 15 sarcomes des membres. Elle a concerné 6 liposarcomes, 4 sarcomes synoviaux, 2 fibrohistiocytomes malins, 1 sarcome de Kaposi, et 1 ostéosarcome (le seul cas de sarcome osseux).Le protocole thérapeutique a compris 2 étapes: (a) ablation de la tumeur avec une marge de sécurité suffisante passant à l' exterieur de la capsule tumorale, (b) puis après 3 à 8 semaines circulation extracorporelle pendant une heure de melphalan associé ou non à de 1 'actinomycine D sous hyperthermie modérée à 39–41°C.La médiane du temps de suivi a été de 30 mois. Quatre sur huit des récidives sarcomateuses ont récidivé à nouveau et ont vu se développer des métastases. Par contre, aucun malade qui n 'avait pas été traité préalablement n'a récidivé et un a présenté une dissémination tumorale sous la forme d'un liposarcome à foyers multiples. Onze sont encore en vie, la durée de la survie étant comprise entre 8 et 103 mois.Les auteurs concluent de ces faits que la circulation extracorporelle avec du melphalan associé ou non à de l'actinomycine D peut être considérée comme un traitement complémentaire du traitement chirurgical.

     

Vascular homografts for vessel substitution in skeletal and soft tissue sarcomas of the limbs

Large vessel involvement by skeletal and soft tissue sarcomas of the extremities does not change the modern limb sparing surgery for those neoplasms. An arterial and, if the vein is open, a venous bypass should always be offered to any patient young or old, with high or low grade sarcoma, because preserving the limb permits quicker rehabilitation, which is particularly useful in the case of a short life expectancy. In 650 cases of skeletal sarcomas, 10 arterial (1.5%) and four venous bypasses were done, all with autologous veins but one in PTFE; we had no problems except a silent arterial occlusion. Of 1000 patients with soft tissue sarcomas, 32 (3%) had vessel involvement permitting limb sparing surgery. The arterial bypass, which is the limb-saving operation, was performed 16 times with a PTFE with one early occlusion and four cases of prosthesis infection, with two amputations despite redo operation with an autologous vein. The more recent 16 cases were, therefore, always done with biological vessel substitution--autologous vein or tissue bank vessel--with only one infection that healed without operation and one case of homograft rupture followed by amputation. Since 1999 in all 13 resected cases with an open vein, we did the arterial and the venous bypass (twice PTFE, six autologous vein, and five bank vessel) with the aim of avoiding postoperative venous hypertension, but only four of the venous bypasses remained open. Venous bypasses are a harmless, but still experimental, procedure.     

Pediatric soft tissue sarcomas

Soft tissue sarcomas in children are rare. Approximately 850 to 900 children and adolescents are diagnosed each year with rhabdomyosarcoma (RMS) or a non-RMS soft tissue sarcoma (NRSTS). RMS is more common in children 14 years old and younger and NRSTS in adolescents and young adults. Infants get NRSTS, but their tumors constitute a distinctive set of histologies. Surgery is a major therapeutic modality and radiation plays a role. RMS is treated with adjuvant chemotherapy, whereas chemotherapy is reserved for the NRSTS that are high grade or unresectable. This review discusses the etiology, biology, and treatment of pediatric soft tissue sarcomas.     

Radiosensitivity of soft tissue sarcomas

The correlation between the effectiveness of radiation therapy and the histology of soft tissue sarcomas was investigated. Of 31 cases with a soft tissue sarcoma of an extremity treated by conservative surgery and postoperative radiation of 3,000-6,000 cGy, local recurrence occurred in 12; 5 out of 7 synovial sarcomas, 4 of 9 MFH, one of 8 liposarcomas, none of 4 rhabdomyosarcomas and 2 of 3 others. As for the histological subtyping, the 31 soft tissue sarcomas were divided into spindle cell, pleomorphic cell, myxoid and round cell type, and recurrence rates were 75%, 33.3%, 16.7% and 0%, respectively. From the remarkable difference in recurrence rate, it was suggested that round cell and myxoid type of soft tissue sarcomas showed a high radiosensitivity compared to the spindle cell type with low sensitivity. Clarifying the degree of radiosensitivity is helpful in deciding on the management of limb salvage in soft tissue sarcomas of an extremity.     

Soft tissue sarcomas of the extremities and trunk in the adult

Summary Between 1970 and 1988 surgery was performed on 124 patients with soft tissue sarcomas of the extremities and trunk in the University Clinics of Hamburg. Liposarcoma, malignant fibrous histiocytoma, fibrosarcoma and malignant schwannoma were the most common histological types. High-grade sarcomas (G3) predominated, with 41 %, while 26% were graded G2 and 33% G1. Resection with wide margins all round was achieved in only 54% of the patients. The quality of the operation proved to be only therapy-related prognostic factor. In addition, tumour grade, size, regional lymph node and distant metastasis and histological type proved to be relevant to the prognosis. With multivariate analysis, distant metastasis, grade, resectability and histological type retained prognostic significance. The efficacy of adjuvant chemo- and radiotherapy was related to the quality of the preceding tumour resection. In case of gross tumour the rate of either partial or complete response was 28% for chemotherapy and 22% for radiotherapy. The mean survival time was 102 months; the 5- and 10-year survival rates were 48% and 37%, respectively.

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Weichteilsarkome der Extremitäten und des Rumpfs bei Erwachsenen. Bericht von 124 Fällen

Zusammenfassung In der Chirurgischen Universitätsklinik Hamburg wurden von 1970–1988 124 Patienten mit Weichteilsarkomen der Extremitäten und des Rumpfs operiert. Liposarkome, maligne fibröse Histiozytome, Fibrosarkome und maligne Schwannome waren die häufigsten histologischen Typen. Niedrig differenzierte Sarkome (G3) überwogen mit einem Anteil von 41%, während 26% als G2 und 33% als G1 imponierten. Bei nur 54% der Patienten wurde eine Tumorresektion mit dreidimensional weitem Sicherheitsabstand durchgeführt. Dabei stellte sich die Qualität der Operation (RO/1/2) als einziger im Rahmen der Therapie beeinflu\barer Prognosefaktor heraus. Daneben wurde in der univariaten Analyse das Tumorgrading, die Tumorgröße, die regionalen Lymphknoten- und Fernmetastasen und der histologische Typ als prognostisch relevant ermittelt. In der multivariaten Analyse behielten die Faktoren Fernmetastasen, Grading, Resektabilität und histologischer Typ statistische Signifikanz. Der Erfolg einer adjuvanten Chemo- und Strahlentherapie war erheblich von der Qualität der vorangegangenen Tumorresektion abhängig. Bei klinisch manifestem Tumor wurde mit einer Chemotherapie bei 28% der Fälle eine Voll- oder Teilremission erreicht, mit einer Strahlentherapie bei 22% der Fälle. Die durchschnittliche Überlebenszeit betrug 102 Monate, die 5- und 10-Jahres-Überlebensrate 48% bzw. 37%.

     

Clinical management of soft tissue sarcomas

The prognosis of soft tissue sarcomas has dramatically improved over the past few decades thanks to the use of increasingly suitable multidisciplinary therapeutic approaches. An assessment of the results of our series of 21 patients, carried out in the light of the most recent literature data, has led us to revise our approach to a number of problems regarding the natural history, the nosographical classification and the therapy of these cancers. This type of tumour arises in a muscle compartment and then spreads proximally and distally within the compartment without involving adjacent structures, except in a relatively advanced phase, while as regards remote metastases the preferential diffusion route is via the bloodstream. Thorough assessment of the clinical and morphological characteristics is essential for adequate treatment: echotomography, CT, and MRI are particularly useful in preoperative staging; a microscopic examination should always include precise classification and accurate assessment of the tumour grade. Surgical management consists in extensive en bloc resection, followed by radiotherapy in the event of unclear margins and/or high grade tumour even when dealing with small sarcomas. The main indications for chemotherapy are locally advanced cases or cases with distant metastases. Thanks to these therapeutic approaches today, good results can be achieved, with 5-year survival rates of 80 and 67%, respectively, in stages I and II, and of 12 to 50% in the more advanced stages.     

Management of extremity soft tissue sarcomas

Soft-tissue sarcomas are a rare and heterogeneous group of tumors. The last few decades have seen rapid strides in surgery with function preserving alternatives for local control in these lesions becoming the norm without compromising on overall disease survival. Good functional and oncological results can be achieved with a combination of excision of the tumor, and where required, suitable adjuvant therapies. These lesions are best managed at specialty centres where the requisite multidisciplinary care can be offered to the patient to optimise results. This overview is intended as a review of current understanding and the multimodality management of these challenging tumors.     

The management of soft tissue sarcomas

BackgroundSoft tissue sarcomas are a rare and heterogeneous group of malignancies that are derived from the mesenchymal cell lines. In the last few decades, the management of these lesions has been improved by the introduction of dedicated Multi Disciplinary Teams (MDTs) where most bone and soft tissue tumours are now treated.¹Following the recent changes to management outlined by the NICE/IOGs, we believe it is pertinent to review the current thinking on soft tissue tumour management.² We also discuss the principles of diagnosis and treatment and the role of adjuvant therapy.MethodsThis is a retrospective review. In the preparation of this paper, we have referred to recent NICE guidelines in this field and have performed a Medline search of the existing literature.ResultsThe key to success is early and appropriate patient referral. Whilst the responsibility for performing surgery has shifted away from the generalist and towards the super-specialist, improvements in survivability can be achieved by promoting basic knowledge within the medical profession as a whole.ConclusionsBoth excision and biopsy of a soft tissue sarcoma by a non-specialist surgeon have been shown to increase the risk of tumour recurrence and all invasive procedures should now be performed within the MDT setting.     

The management of soft tissue sarcomas

The management of soft tissue sarcomas presents one of the more exciting areas of the application of multimodal therapy for malignancy. Rapid developments have taken place in the understanding of the histogenesis, histopathology, prognostic indicators and treatment approaches. With the combination of local irradiation and adjuvant chemotherapy, limb sparing operations can now be widely advocated with amputation being reserved for proximal difficult lesions. Retroperitoneal sarcomas, however, remain a major challenge with little progress in results of therapy, other than improved operative morbidity in the last 10-15 years. Current prospective, randomized studies of the management policies designed to maximize survival while minimizing morbidity should provide further insights in the near future. Our current and past experience with more than 150 new cases each year allows a brief summary of the understanding and management of these difficult problems.     

Prognostic factors of soft tissue sarcomas

Background The management and outcome of soft tissue sarcomas is strongly influenced by the rarity of these tumors. Different determinations of tumor-, patient and treatment related facts with predominant influence on outcome and prognosis make comparison of results difficult. Methods To select the prognostic variables, a critical review of all points in the management of soft tissue sarcomas is necessary. Results A variety of clinical, pathological, surgical and adjuvant treatment variables results in relative and absolute prognostic value. The duration of symptoms, the physician delay, the kind of diagnosis, staging and multidisciplinary management are important variables. The anatomical site, sex, age and histologic category seems to play a secondary role. The prognostic value of the size of tumors is related to their localization and resectability, time of diagnosis, quality of resection and adjuvant treatment. Tumor localization and tumor size strongly influences resectability. Tumor grade and quality of surgical resection and therefore local tumor control are the most important prognostic variables. The risk of dissemination and metastatic disease is also highly correlated with tumorgrade, surgical treatment and adjuvant therapy. Conclusions The clinical evaluation and treatment of patients with soft tissue sarcomas are challenging problems. Revised definitions and concepts in prospective multicenter treatment modalities are necessary to improve the prognosis and select factors of prognosis.     

Management of extremity soft tissue sarcomas

This article provides an understanding of the evaluation, staging, and management of patients with extremity soft tissue sarcoma. Although there are straightforward guidelines to the management of patients with extremity soft tissue sarcoma, each patient presents with a unique tumor, and considerations for tumor control, functional outcome, and the toxicity of therapy must be considered. As is true for patients diagnosed with sarcoma at other anatomic sites, a multidisciplinary team approach streamlines care with attention to the complexities and intricacies of choosing and delivering optimal therapy.     

Adult head and neck soft tissue sarcomas

BACKGROUND: The purpose was to determine the optimal treatment for adult patients with head and neck soft tissue sarcomas. METHODS: We conducted a review of the pertinent literature. RESULTS: Local control after surgery alone or combined with radiotherapy was obtained in approximately 60% to 70% of the patients. The probability of local control is influenced by histologic grade, tumor size, and surgical margins. Patients with high-grade tumors and/or positive margins have improved local control if adjuvant radiotherapy is used. Distant metastases occurred in 10% to 30% of patients. The 5-year overall and cause-specific survival rates varied from approximately 60% to 70% and are affected by age, histologic grade, previous treatment of tumor, invasion of deep structures, and adequacy of surgery. CONCLUSIONS: The optimal treatment for adult head and neck soft tissue sarcomas is surgery. Adjuvant radiotherapy improves outcomes for those with high-grade tumors and/or positive margins. Radiotherapy alone will cure a small subset of patients with unresectable tumors.     

Treatment of soft tissue sarcomas of the leg

An analysis of data of 548 patients with different diseases of soft tissues of lower extremities after using a complex of diagnostic measures (clinical, radionuclide data, thermography, puncture, biopsy) has shown that the number of erroneous conclusions may be minimized to 2%. Modern advances of plastic surgery can widen indications for preserving operations in malignant tumors of soft tissues of lower extremities without prejudice to the main principle of oncology concerning its fascial character. Combined treatment can also improve the results.