Soft Tissue Sarcoma

Rhabdomyosarcomas (RMS) are the most common pediatric soft tissue sarcomas. They resemble developing skeletal muscle and are histologically divided into two main subtypes; alveolar and embryonal RMS. Characteristic genomic aberrations, including the PAX3- and PAX7-FOXO1 fusion genes in alveolar cases, have led to increased understanding of their molecular biology. Here, we determined the effect of genomic copy number on gene expression levels through array comparative genomic hybridization (CGH) analysis of 13 RMS cell lines, confirmed by multiplex ligation-dependent probe amplification copy number analyses, combined with their corresponding expression profiles. Genes altered at the transcriptional level by genomic imbalances were identified and the effect on expression was proportional to the level of genomic imbalance. Extrapolating to a public expression profiling dataset for 132 primary RMS identified features common to the cell lines and primary samples and associations with subtypes and fusion gene status. Genes identified such as CDK4 and MYCN are known to be amplified, overexpressed, and involved in RMS tumorigenesis. Of the many genes identified, those with likely functional relevance included CENPF, DTL, MYC, EYA2, and FGFR1. Copy number and expression of FGFR1 was validated in additional primary material and found amplified in 6 out of 196 cases and overexpressed relative to skeletal muscle and myoblasts, with significantly higher expression levels in the embryonal compared with alveolar subtypes. This illustrates the ability to identify genes of potential significance in tumor development through combining genomic and transcriptomic profiles from representative cell lines with publicly available expression profiling data from primary tumors. © 2009 Wiley-Liss, Inc.

The main objective was to compare parent-reported health-related quality of life (HRQL) of child and adolescent survivors of childhood cancer to that of controls who had no history of cancer. Methods We assessed HRQL of 800 child and adolescent survivors younger than 16 years and 923 randomly selected, age-and sex-matched controls from the general population in a national multicenter retrospective cohort study using the Child Health Questionnaire parent report. Participation was 69% among survivors and 57% among controls. Results Survivors had means that were consistently lower than controls on the HRQL physical summary (PH; 49.9 v 55.3; P Ͻ.005), psychosocial summary (PS; 49.4 v 52.6; P Ͻ .005), and all but one of the eight subscale scores. Clinically important survivor-control differences in means on PH were found for survivors of CNS tumors, bone tumors, lymphoma, leukemia, soft tissue sarcoma and Wilms' tumor (differences: Ϫ8.7, Ϫ7.0, Ϫ6.3, Ϫ5.4, Ϫ4.4, Ϫ3.8/100, respectively); on PS, survivors of CNS tumors were most compromised (Ϫ6.1/100). Survivor-control differences in both PH and PS were also large for survivors treated with radiation only (Ϫ5.8 and Ϫ11.9/100, respectively), or radiation combined with surgery (Ϫ6.6 and Ϫ5.9/100, respectively), or radiation combined with both surgery and chemotherapy (Ϫ7.8 and Ϫ5.1/100, respectively). Cranial radiation was associated with the most compromised HRQL. Conclusion According to parents, HRQL for survivors was somewhat poorer, overall, than for controls. Survivors of CNS tumors, lymphoma, and leukemia and those patients treated with cranial radiation were reported to have poorest HRQL. These findings support development of guidelines for levels of follow-up care for particular groups of survivors.

The objective of this article is to introduce the early Chinese clinical experience of using extracorporeal focused ultrasound (US) surgery (FUS) for the treatment of solid tumors. From December 1997 to October 2001, a total of 1038 patients with solid tumors underwent FUS ablation in 10 Chinese hospitals. The tumors included primary and metastatic liver cancer, malignant bone tumors, breast cancer, soft tissue sarcomas, kidney cancer, pancreatic cancer, abdominal and pelvic malignant tumors, uterine myoma, benign breast tumors, hepatic hemangioma and other solid tumors. In this article, pathologic changes in tumors treated with FUS, real-time diagnostic imaging for targeting, monitoring and assessment of results by follow-up images are presented. Early clinical results and complications of the technique are also reported. (E-mail: mfengwu@yahoo.com) © 2004 World Federation for Ultrasound in Medicine & Biology.

Soft tissue sarcomas (STS) account for approximately 1% of malignant tumors. Benign soft tissue tumors (STT) are about 100 times more frequent and every radiologist will be faced with a soft tissue tumor at some point. It is our intent to distribute basic knowledge to a broad audience on the subject of the general workup of soft tissue tumors, their main classifications according to the WHO 2013 (IARC 4th Ed.)  and provide easy to comprehend information on epidemiology in order to enhance alertness for these rare entities in a non-specialized, fast paced clinical environment. We chose a single sided poster format (printable to DINA0-1) to demonstrate important main features of benign versus malignant STT, large enough to quickly grasp information when required.   
There are more than 50 STS subtypes, but liposarcoma, pleomorphic sarcoma, leiomyosarcoma, synovial sarcoma, and malignant peripheral nerve sheath tumor account for 75%. We give pictorial examples for eight malignant and eight benign tumors from the main tumor categories (adipocytic tumors, fibroblastic/myofibroblastic, fibrohistiocytic, smooth muscle, pericytic, skeletal muscle, vascular, nerve sheath and tumors of uncertain differentiation+NOS) by MR-imaging.  Preferential locations for malignant (70% deep to the fascia) and benign (99% superficial) are presented in schematic drawings. Frequency of occurrence with regard to the anatomical predilection of STT is shown in a large whole body scheme. 
Approximately 60% arise in the extremities, but the trunk wall, retroperitoneum, and head/neck are also relatively common locations.
Finally, the patient management is presented in a workflow-chart differentiating between tumors >5cm in size (T2a/b) and tumors <5cm (T1). 
Initial assessment of the lesion should be done with contrast-enhanced MRI before biopsy to choose the optimal biopsy tract. Imaging, biopsy and histopathological workup and subsequent therapeutic interventions require a multidisciplinary approach in a sarcoma center. This and additional information is condensed for quick and easy guidance on STT.

OBJECTIVE: The aim of the CWS 96 Study was to achieve an optimal treatment in children and adolescents with soft tissue sarcoma (STS) implementing a further refinement of risk-adapted allocation to chemotherapy, surgery and radiotherapy. METHODS: Treatment stratification was based on tumour histology, TNM status, postsurgical stage, localisation and age. Local tumour control was ensured by surgery and risk-adapted radiotherapy. RESULTS: From 1995 to 2002, 89 patients were registered in Austria. The 3-year event-free survival (EFS) and overall survival rates (OS) were 63% ± 6% and 71% ± 6%, respectively. 59/89 patients had localised RMS-like (rhabdomayosarcoma) STS (EFS 73% ± 7%), 14 had localised Non-RMS STS (EFS, 54% ± 16%) and 15 patients had metastatic disease at diagnosis (EFS, 33% ± 12%), 1 patient had fibromatosis. The EFS rates at 3 years in patients with localised RMS-like tumours according to risk group were 92% ± 8% for low and standard risk (12 patients) and 67% ± 8% for high risk (47 patients). Favourable primary tumour sites of nonmetastatic RMS-like STS i.e. orbit, head/neck nonparameningeal or genitourinary non-bladder/prostate were diagnosed in 15 patients (1 of 15 patients died). In 44 patients with unfavourable localisation such as parameningeal, genitourinary bladder/prostate, extremity and others, 7 deceased. The 3-year EFS according to histology in patients with RMS-like STS was 61% ± 11% for RME (embryonal RMS ) (28 patients) and 71% ± 15% for RMA (alveolar RMS) (10 patients). The most common treatment failure was local relapse occurring in 21% of patients in the high-risk group. CONCLUSION: Risk-adapted individualisation of treatment led to a reduction of chemotherapy in the low- and standard-risk group without compromising survival. The outcome of RME and RMA was similar in this cohort of patients. These preliminary results after a median observation time of 2.5 years confirm the CWS 96 strategy. ZIEL: Die CWS-96-Studie für Behandlung von Weichteilsarkomen (WS) im Kindes- und Jugendalter verfolgte eine maximale Tumorkontrolle bei Minimierung von Spätfolgen durch eine verbesserte risikoadaptierte Chemo-, Operations- und Radiotherapie. METHODEN: Die Stratifikation der Patienten erfolgte nach Histologie, TNM-Status, postchirurgischem Stadium, Lokalisation und Alter. Die Lokalkontrolle wurde durch Chirurgie und risikoadaptierte Bestrahlung angestrebt. ERGEBNISSE: Im Zeitraum von 1995 bis 2002 wurden 89 Patienten registriert. Das ereignisfreie (EFS) und das absolute Überleben (OS) nach 3 Jahren lag bei 63% ± 6% bzw. 71% ± 6%. 59 von 89 Patienten hatten lokalisierte RMS-(Rhabdomysoarkom-)artige WS (EFS, 73% ± 7%), 14 lokalisierte Non-RMS-artige WS (EFS, 54% ± 16%), 15 initial disseminierte WS (EFS, 33% ± 12%) und 1 Fibromatose. Das 3-Jahres-Überleben bei lokalisierten RMS-artigen nach Risikogruppe ergab 92% ± 8% EFS für niedriges und Standard-Risiko (12 Patienten), sowie 67% ± 8% EFS für hohes Risiko (47 Patienten). Die Lokalisation der 59 nicht metastasierten RMS-artigen WS war bei 15 Patienten in als günstig definierten Regionen (Orbita, Kopf/Hals – nicht parameningeal oder urogenital – nicht Blase/Prostata: 1 von 15 Patienten verstorben), während bei 44 Patienten ungünstige Lokalisationen vorlagen (Kopf/Hals – parameningeal, urogenital – Blase/Prostata, Extremitäten und andere: 7 von 44 Patienten verstorben). Das EFS nach Histologie in der Kategorie der lokalisierten RMS-artigen lag für embryonale RMS (RME) (28 Patienten) bei 61% ± 11% und für alveoläre RMS (RMA) (10 Patienten) bei 71% ± 15%. Das Hauptproblem war das lokale Rezidiv, das bei 21% aller Patienten im Hochrisikoarm auftrat. SCHLUSSFOLGERUNG: Die risikoadaptierte Individualisierung der Therapie als oberstes Ziel führte zu Einsparungen an Chemotherapie in der Niedrig- und Standard-Risiko-Gruppe bei erhaltener guter Prognose. In dieser Patientenkohorte war das Überleben für RME und RMA ähnlich. Nach einer medianen Beobachtungszeit von 2,5 Jahren bestätigen diese ersten Ergebnisse die erfolgreiche Umsetzung des CWS-Konzeptes.

Purpose This study assesses the efficacy, toxicity and pharmacokinetic profile of trabectedin with or without prophylactic dexamethasone co-treatment in patients with recurrent advanced soft tissue sarcoma (STS). Patients and methods Patients were randomized to receive trabectedin as a 3-h infusion every 3 weeks with dexamethasone or placebo in the first cycle, with the alternate in the second cycle and with the patient's choice subsequently.

Résumé Il a été démontré dans les années 1970-1980, que l'on obtenait des taux de survie équivalents chez des patients irradiés après chirurgie carcinologique « programmée » et chez des patients amputés. Malheureusement, il existe une inadéquation frappante entre les standards de prise en charge d'une tumeur des tissus mous et sa réalisation pratique courante, d'autant plus importante que le patient est au début de son histoire clinique. Les avancées thérapeutiques chirurgicales actuelles (reconstructions et perfusions de membres isolées sous circulation extracorporelle) suppléent fréquemment une prise en charge initiale inadaptée sur le plan local, afin de rester dans une optique conservatrice durable. L'isolation du membre par une circulation extracorporelle, suivie d'un « lavage » avant le rétablissement du circuit vasculaire normal, permet de réaliser des chimiothérapies locorégionales à des doses 10 à 20 fois supérieures aux possibilités par voie systémique ou intra-artérielle simple. L'adjonction du TNFα permet d'obtenir environ 30 % de réponses complètes. Les tumeurs dont l'IRM laisse prévoir une exérèse marginale ou susceptible d'entraîner des séquelles fonctionnelles importantes (perte de plusieurs muscles ou nerfs), voire de nécessiter une amputation, relèvent potentiellement de ce traitement médicochirurgical néoadjuvant. Parallèlement, la diffusion des règles chirurgicales initiales et une meilleure intégration de la chirurgie parmi les autres modalités thérapeutiques permettront également d'éviter des chirurgies itératives qui finissent par être inutilement mutilantes. © 2006 Elsevier Masson SAS. Tous droits réservés.

Buttock soft-tissue sarcomas are common entities that have been infrequently reported. Little is known about the epidemiology of soft-tissue sarcomas. The current therapeutic approach suggests that a large tumour excision with clear margins and effective adjuvant therapy are needed to improve overall survival rates. Overall survival rates vary between 25% and 40% for a period of 5 years and 48.5% for a 2-year period. A case is reported of a patient with a diagnosis of pleomorphic rhabdomyosarcoma on whom a buttockectomy was performed. A brief retrospective analysis of sarcomas in our centre is reported.

Résumé Pour faire le point sur la chirurgie des sarcomes, nous avons réalisé une revue de littérature et apporté notre expérience personnelle. Devant une tumeur des parties molles, le premier objectif est de penser au diagnostic de sarcome des tissus mous. Le diagnostic est apporté habituellement par microbiopsie au trocart, mais peut parfois demander une biopsie chirurgicale. La résection chirurgicale (R) a été mieux codifiée ces dix dernières années et distingue résection in sano (R0), maladie microscopique résiduelle (R1) et maladie macroscopique résiduelle (R2). Elle est déterminée collégialement par confrontation chirurgicopathologique selon les recommandations du Groupe sarcome français (GSF). Le risque de rechute locale est lié au type de résection et au grade tumoral. Grâce à la classification du GSF, le pronostic local des sarcomes peut être mieux défini et le traitement adapté individuellement. Ainsi, les taux de rechute locale, dans des équipes expérimentées, diminuent, passant de 20 à 10 % environ dans les meilleurs cas. Les complications après chirurgie pour sarcome sont fréquentes, avec 14 % sévères. L'évaluation fonctionnelle a été améliorée par l'apport à de nouveaux systèmes qui se complètent en fonction des critères étudiés. La perfusion de membre permet d'éviter dans certaines conditions une amputation théoriquement indiquée. En conclusion, du fait de leur rareté et de la spécificité de leur prise en charge, les sarcomes des tissus mous devraient être traités dans des centres spécialisés. © 2005 Elsevier SAS. Tous droits réservés.

CPT-11, a semi-synthetic derivative of camptothecin, was investigated for its activity in panels of 15 human ovariancancer lines and 10 human soft-tissue sarcoma lines grown s.c. in nude mice. Various factors were analyzed that may be of influence on the extent of tumor-growth inhibition induced by CPT-11. At equitoxic doses, CPT-11 was more effective in the daily ϫ5 schedule than the weekly ϫ2 schedule, although a 2-fold higher dose was administered in the weekly ϫ2 schedule. Since i.p. and i.v. injections were similarly effective, the selected treatment schedule was 20 mg/kg i.p. daily ϫ5, starting when tumors measured approximately 150 mm 3 . Growth inhibition of H75% was obtained in 8/15 human ovarian-cancer lines and in 6/10 human soft-tissue sarcoma lines. A weak correlation was found between topoisomerase-I mRNA in xenograft tissues and sensitivity to CPT-11. Relative topoisomerase-I expression was highest in MRI-H-207 and WLS-160 xenografts, in which CPT-11 was able to induce cures of all tumors. The high efficacy in the 2 panels of human tumor lines suggests over-prediction of its potential clinical activity in these tumor types. The difference in efficacy of CPT-11 between species may be related to the metabolism of the drug, since CPT-11 is converted more efficiently into SN-38 in mice. In addition, mice may be less sensitive to SN-38-induced side-effects. On the basis of the preclinical data, frequent administration of lower doses of CPT-11 should be considered in order to increase response rates in the clinic. Int.

Soft tissue sarcomas (STS) are rare tumors arising from the connective tissues. STS can arise at any anatomic site, can demonstrate varied behavior and prognosis, and therefore present a formidable challenge in management. The local treatment of STS demands technical complexity in the application of diagnostic tools, including pathology and imaging, as well as treatment approaches, including surgical ablation and reconstruction, radiotherapy, and, in defined cases, chemotherapy. The understanding of the management of these lesions is profoundly dependent on the multidisciplinary setting, where experience has been gained and skills are available to increase the likelihood of a successful result. Several proven options are available for optimal local management, and the choice of approach depends on the prevailing practice and resource profile of the treating center. With modern approaches, the local control rate can be expected to be at least 90% for extremity lesions, which constitute the most common STS. The experience in other anatomic sites is less favorable as a result of a combination of late diagnosis, technically difficult access sites, and possibly less familiarity with these less common presentations. The disappointing results make it all the more important for patients to be referred to a multidisciplinary setting with experience in sarcoma management to maximize the chance of successful local outcome.

BACKGROUND: In a series of 575 patients 21 years of age with soft tissue sarcomas (STSs), the authors investigated the association patterns between symptom interval (ie, the period between the onset of the first symptoms or signs of the disease and its definitive diagnosis) and patient/tumor characteristics or disease outcome (in terms of survival). METHODS: The analysis was based on multivariate models (linear for associations with patient/tumor characteristics and Cox's for survival). RESULTS: The symptom interval ranged between 1 week and 60 months (median, 2 months) and tended to be longer the older the patient (ie, the interval was longer in adolescents than in children) and the larger the tumor's size, and for tumors located at the extremities and for nonrhabdomyosarcoma STSs (as opposed to rhabdomyosarcomas). A longer symptom interval unfavorably influenced survival (P ¼ .002), which was also significantly affected by the patient's age and the size and surgical stage of the tumor. A different pattern of association between symptom interval and survival emerged for different types of STS histology. CONCLUSIONS: Our study points to an independent prognostic effect of symptom interval that cannot be explained by its associations with other factors, such as patient's age or the site, size, stage, and histology of the tumor. Future studies should focus more on the possible causes of symptom interval in pediatric STS populations to enable corrective measures to be implemented to reduce the diagnostic delay.

The pathological features of 155 adult patients with soft-tissue sarcomas were studied retrospectively, in an attempt to set up a grading system for these tumors. As the first step, seven histological criteria (tumor differenti-ation, cellularity, importance of nuclear atypia, ...

Ninety-four soft-tissue sarcomas of head and neck occurring among 10,700 malignancies, seen during the period 1953–1980 in the head-and-neck surgical department, were studied with reference to age, sex, ethnic origin, site, histological type and survival. The objective was to review our own experience with sarcomas in these sites seen in the Department of Pathology, A.C. Camargo Hospital. The predominant site was the head. Fibrosarcoma was the most frequent type in adults and rhabdomyosarcoma, located exclusively in the head, was the most frequent in children.

High-dose-rate intraoperative radiation therapy (HDR-IORT) has historically provided effective local control (LC) for patients with unresectable and recurrent tumors. However, IORT is limited to only a few specialized institutions and it can be difficult to initiate an HDR-IORT program. Herein, we provide a brief overview on how to initiate and implement an HDR-IORT program for a selected group of patients with gastrointestinal and pelvic solid tumors using a multidisciplinary approach. Proper administration of HDR-IORT requires institutional support and a joint effort among physics staff, oncologists, surgeons, anesthesiologists, and nurses. In order to determine the true efficacy of IORT for various malignancies, collaboration among institutions with established IORT programs is needed.

Unresectable or metastatic disease occurs in 40% to 60% of soft-tissue sarcoma (STS) patients and portends a poor prognosis. For decades, doxorubicin has formed the backbone of systemic treatment, with response rates of approximately 26%. Patients progressing following first-line therapy were left with few proven options. No other cytotoxic chemotherapy agent or combination has demonstrated superiority to doxorubicin. Advances in

Soft tissue tumors (STTs) are rare mesenchymal neoplasms accounting for less than 1% of adult cancers. More than 50 different subtypes of STTs have been identified, with this number expected to grow as our understanding of the complex genetic landscape of these diseases improves. As the classification of soft tissue neoplasms continues to diversify, so does the approach to therapy. Accurate histopathologic diagnosis, utilizing the appropriate ancillary immunohistochemical and molecular diagnostic platforms, underpins the oncologic management of soft tissue sarcomas. As increasing numbers of reproducible genetic abnormalities in soft tissue neoplasms are defined, molecular genetic and molecular cytogenetic investigations have become a standard part of the ancillary diagnostic repertoire. However, other soft tissue neoplasms lack reproducible genetic abnormalities, and for these, traditional histology and immunohistochemistry remain the cornerstones for diagnosis. Here, we give an overview of histology-driven therapy in STTs, highlighting the critical role of accurate surgical pathology in guiding the systemic treatment of patients with these neoplasms, and the importance of close collaboration between the surgical pathologist and the oncologist. We also summarize what is considered standard practice in nonhistology-and histology-driven therapy.

BACKGROUND. Single-agent gemcitabine and vinorelbine have activity in treatment of patients with soft-tissue sarcomas. The combination of gemcitabine plus vinorelbine has activity against several forms of metastatic carcinoma with acceptable toxicity. This study evaluated the efficacy and tolerability of gemcitabine plus vinorelbine in advanced soft-tissue sarcoma.

Background. The goal of this study was to determine the oncologic outcomes in localized resectable soft-tissue sarcoma after pre-versus postoperative radiation. Methods. Literature searches through MEDLINE, EM-BASE, CancerLit, and the Cochrane Database were performed with the following search terms: sarcoma, radiation, preoperative, and postoperative. Two reviewers independently assessed all eligible publications with the Detsky Quality Scale for Randomized Trials and the Newcastle-Ottawa Quality Assessment Scale for casecontrol studies. The primary outcome measure was the pooled odds ratio and 95% confidence intervals (95% CI) for the risk of local recurrence calculated through the fixedand random-effects methods. Time-dependent survival data were calculated as an average across all studies. Results. Five eligible studies were identified including a total of 1,098 patients. The P value for heterogeneity was 0.259, and the variability (I 2 ) in results across studies due to true differences in treatment effect was 25%. The risk for local recurrence was lower in the preoperative group with an odds ratio of 0.61 (95% CI 0.42-0.89) by means of the fixedeffects method, and an odds ratio of 0.67 (95% CI 0.39-1.15) by means of the random-effects method. Average survival was 76% (range 62-88%) in the preoperative group and 67% (range 41-83%) in the post-operative group.

Background: Extrapulmonary tuberculosis is one of the great mimickers of medicine, and often masquerades as malignancy. As a result, patients may be referred to oncologists and surgeons for further evaluation and management, delaying the institution of appropriate anti-tuberculous drug therapy.

Purpose: A retrospective analysis was undertaken to determine the efficacy of postoperative radiotherapy in patients withprimary sarcoma of the breast. Methods and Materials: Ten patients with high-grade nonmetastatic primary sarcoma of the breast were treated at the National Cancer Institute, NIH, between 1979 and 1989 with mastectomy and adjuvant radiotherapy. Chemotherapy was given to three patients as part of a randomized trial.

Synovial sarcomas are high-grade malignant mesenchymal tumors that account for 10% of all softtissue sarcomas. Almost 95% of these tumors are characterized by a nonrandom chromosomal abnormality, t(X;18)(p11.2;q11.2), that is observed in both biphasic and monophasic variants. In this article, we present the case of a 57-year-old woman diagnosed with high-grade biphasic synovial sarcoma in which conventional cytogenetic analysis revealed the constant presence of a unique t(18;22)(q12;q13), in addition to trisomy 8. The rearrangement was confirmed by fluorescence in situ hybridization. The use of the whole chromosome painting probes WCPX did not detect any rearrangements involving chromosome X, although reverse-transcriptase polymerase chain reaction (PCR) analysis demonstrated the conspicuous presence of a SYT/SXX1 fusion gene. Spectral karyotyping (SKY) was also performed and revealed an insertion of material from chromosome 18 into one of the X chromosomes at position Xp11.2. Thus, the karyotype was subsequently interpreted as 47,X,der(X)ins(X;18) (p11.2;q11.2q11.2),der(18)del(18)(q11.2q11.2)t(18;22)(q12;q13),der(22)t(18;22). Real-time PCR analysis of BCL2 expression in the tumor sample showed a 433-fold increase. This rare finding exemplifies that thorough molecularecytogenetic analyses are required to elucidate complex and/or cryptic tumor-specific translocations. Ó

Induction chemotherapy can produce dramatic necrosis in sarcomas-raising the question of whether or not radiation is necessary. This study reviews the clinical outcome of a subset of patients with high-grade extremity soft tissue sarcomas (STS) who were treated with induction chemotherapy and surgical resection but without radiation. Nonmetastatic, large, high-grade STS of the pelvis and extremities were treated with intra-arterial cisplatin, adriamycin, and, after 1995, ifosfamide. After induction, oncologic resection and histologic evaluation were performed. Good responders with good surgical margins were not treated with radiation. Thirty-three patients, with a median follow-up of 5 years, were included. Limb salvage rate was 94%. Median tumor necrosis was 95%. Four patients developed metastatic disease with three subsequent deaths. Two local recurrences occurred; both patients were salvaged with reresection and adjuvant external beam radiotherapy, although one died of metastatic disease 10 years later. Relapse-free and overall survival is 80% and 88% at 5 and 10 years by Kaplan-Meier analysis. Intensive induction chemotherapy can be extremely effective for high-grade STS, permitting limb-sparing surgery in lieu of amputation. Radiation may not be necessary if a good response to induction chemotherapy and negative wide margins are achieved. All patients with large, deep, high-grade STS of the extremities should be considered candidates for induction chemotherapy.

A multidisciplinary approach that includes active participation by a radiologist is beneficial for optimal diagnostic evaluation and treatment of soft-tissue sarcomas. Recent advances in diagnostic imaging include a better understanding of the practical roles of ultrasonography, computed tomography, and magnetic resonance imaging in the evaluation of primary and recurrent soft-tissue sarcomas. The potential role of positron emisson tomography scanning and dynamic magnetic resonance imaging in the evaluation of soft-tissue sarcomas has been preliminarily evaluated. The clinically practical utility of radiologic imaging in the evaluation of soft-tissue sarcomas is discussed, and ongoing research is briefly reviewed.

Sarcomas are uncommon malignant tumors arising from mesenchymal tissue, accounting for approximately 10% of soft-tissue sarcomas and less than 1% of all malignant neoplasms. The most frequent subtypes of retroperitoneal sarcomas are liposarcoma. Total surgical extirpation appears the only chance for patient cure since medical therapies have shown little efficacy. We have analyzed our series of 32 retroperitoneal liposarcoma that undergone surgical resection and with at least 5 years of follow-up. We have included 21 patients who underwent a complete resection (R0) and 6 patients who underwent an incomplete resection (R1). However, the operation's goal was a complete tumor resection with tumor-free resection margins. The 5-year survival was 85.7% when we performed a R0 resection, while it was 33.3% when we performed a R1 resection with a significant statistical difference (p = 0.02). Surgery is the gold standard for treatment of liposarcoma. Despite advances in diagnostic modalities, surgical techniques and the adoption of more aggressive procedure, such disease still has a propensity for local recurrence, even after an apparent complete resection. Therefore, locoregional recurrence remains the main cause of death in patients with a retroperitoneal liposarcoma. Moreover when the tumor recurs locally, the treatment of choice is to remove the recurrence. Our experience shows that a complete resection of primary lesions and further resection of recurrences early detected with CT examination could improve the overall survival of patients with retroperitoneal liposarcoma. Moreover in our series, the patients who undergo R1 resection had a worse prognosis.

Purpose: To evaluate adjuvant chemotherapy and interpolated accelerated radiotherapy (RT) for adult patients with high-risk soft tissue sarcoma in the extremities or trunk wall. Methods and Materials: High-risk soft tissue sarcoma was defined as high-grade malignancy and at least two of the following criteria: size $8 cm, vascular invasion, or necrosis. Six cycles of doxorubicin and ifosfamide were prescribed for all patients. RT to a total dose of 36 Gy (1.8 Gy twice daily) was inserted between two chemotherapy cycles after marginal margin resection regardless of tumor depth or after wide-margin resection for deep-seated tumors. RT was boosted to 45 Gy in a split-course design in the case of intralesional margin resection. Results: A total of 119 patients were eligible, with a median follow-up of 5 years. The 5-year estimate of the local recurrence, metastasis-free survival, and overall survival rate was 12%, 59%, and 68%, respectively. The group receiving RT to 36 Gy had a local recurrence rate of 10%. In contrast, the local recurrence rate was 29% in the group treated with RT to 45 Gy. The presence of vascular invasion and low chemotherapy dose intensity had a negative effect on metastasis-free and overall survival. Toxicity was moderate after both the chemotherapy and the RT. Conclusions: Accelerated RT interposed between chemotherapy cycles in a selected population of patients with high-risk soft tissue sarcoma resulted in good local and distant disease control, with acceptable treatmentrelated morbidity. The greater radiation dose administered after intralesional surgery was not sufficient to compensate for the poorer surgical margin. Vascular invasion was the most important prognostic factor for metastasis-free and overall survival. Ó 2011 Elsevier Inc.

Eighty-six patients with locally advanced, high-grade soft tissue sarcomas of the extremities were studied prospectively in order to determine the efficacy of hyperthermic perfusion (HP) or hyperthermic antiblastic perfusion (HAP) as the first step of a combined multimodality therapy. The immediate response was evaluated in terms of tumor regression, and results confirmed the in vivo sensitivity of human sarcomas to the selective antineoplastic action of heat alone or combined with drugs (melphalan, actinomycin D, and cis-platinum). HAP has been shown to be simpler and safer than HP, and it is now currently routinely employed. As far as the long-term cure is concerned, all the patients have been evaluated for functional results, locoregional control, and survival, according to the different treatment schedules. The first clinical trials employed HP or HAP followed by delayed surgery alone. In 11 of 17 evaluable patients treated with HP, and in 17 of 29 treated with HAP, conservative surgery could be performed. A high incidence of locoregional relapse (24%) occurred, with low overall survival rates: 50.1% and 31.7% at 5 and 10 years after HP plus surgery, and 47.9% after HAP plus surgery at both 5 and 10 years. The protocol was, therefore, modified to include continuous intraarterial infusion of Adriamycin® (ADR) (17 patients) or radiotherapy (9 patients) before surgery. The results obtained thus far may be summarized as follows: (a) conservative surgery with functional limb-salvage was possible in all patients; (b) the percentage of locoregional failure decreased to approximately 12% after HAP + ADR infusion + excision, the 5- and 10-year overall survival rates both being 77.6 %, and the 5- and 10-year disease-free rates both being 57.8%; (c) no local recurrences occurred in the group treated with HAP + radiotherapy + excision with a 5-year overall survival rate of 71.5% and a 5-year disease-free rate of 50.4%. In conclusion, the combined multimodality approaches employed appear to have improved both functional results and long-term cure, even though these must be further confirmed on a larger series of patients. Une étude prospective concernant 86 malades qui présentaient un sarcome des parties molles des membres de stade évolutif avancé a été entreprise pour déterminer l'efficacité de la perfusion hyperthermique ou de la perfusion hyperthermique antiblastique en tant que première étape d'un traitement à modalités multiples. La réponse immédiate a été appréciée en fonction de la régression tumorale. Les résultats ont confirmé la sensibilité in vivo des sarcomes humains à l'action antinéoplasique sélective de la chaleur employée isolemment ou combinée avec des drogues (melphalan, actinomycine D, et cis-platinum). La perfusion hyperthermique antiblastique s'est montrée plus simple et plus sûre que la perfusion hyperthermique, et de ce fait est devenue une méthode thérapeutique normalement employée. Pour apprécier l'action thérapeutique à long terme tous les malades ont été étudiés en tenant compte des résultats fonctionnels, du contrôle loco-régional, et de la survie obtenus selon les différentes thérapeutiques appliquées. Les premiers essais ont eu recours à l'hyperthermie thermique ou à l'hyperthermie thermique antiblastique suivie d'une intervention chirurgicale. Chez 11 des 17 malades traités par l'hyperthermie thermique, et chez 17 des 29 malades soumis à l'hyperthermie antiblastique le traitement chirurgical conservateur a pu être réalisé. Les résultats furent les suivants: fréquence importante des récidives loco-régionales (24%); taux global de survie bas: 50.1% et 31.7% à 5 ans et 10 ans après perfusion hyperthermique suivie de chirurgie, ce taux étant de 47.9% après perfusion hyperthermique antiblastique suivie de chirurgie à 5 ans et 10 ans. En fonction de ces résultats le protocole thérapeutique fut modifié en y ajoutant une transfusion intra-artérielle continue d'Adriamycine® (17 malades) ou de la radiothérapie (9 malades) avant l'intervention. Les résultats obtenus à ce jour peuvent se résumer ainsi: (a) la chirurgie conservatrice permettant de sauver un membre fonctionnel est toujours possible; (b) la poucentage d'échec régional décroit environ jusqu' à 12% après perfusion hyperthermique antiblastique associée à la perfusion d'Adriamycine® et l'excision, le taux global de survie à 5 ans et 10 ans étant de 77.6%, le taux d'absence de la maladie à 5 ans et 10 ans étant de 57.8%; (c) aucune récidive locale n'est survenue dans le groupe traité par perfusion hyperthermique antiblastique associé à la radiothérapie et à l'exérèse, le taux global de survie à 5 ans étant de 71.5% et le taux d'absence de la maladie à 5 ans étant de 50.4%. En conclusion le traitement qui a été employé associant plusieurs modalités thérapeutiques a entrainé une amélioration des résultats fonctionnels et de la cure à long terme encore que ce fait demande a été confirmé par une étude étendue à un plus grand mombre de malades. Ochenta y seis pacientes con sarcomas de los tejidos blandos de las extremidades, de alto grado histológico, e invasión local avanzada fueron estudiados en forma prospectiva con el objeto de determinar la eficacia de la perfusión hipertérmica (PH) o la perfusión hipertérmica antiblástica (PHA) como primer paso dentro de una terapia combinada multimodal. La respuesta inmediata fue valorada en términos de la regresión tumoral, y los resultados confirmaron la sensibilidad in vivo de los sarcomas humanos a la acción antineoplásica selectiva del calor sólo o combinado con drogas (melfalán, actinomicina D, y cis-platino). La PHA ha demostrado ser más sencilla y más segura que la PH y actualmente es utilizada en forma rutinaria. En lo referente a curación a largo plazo, todos los pacientes han sido evaluados en cuanto a resultados funcionales, control locorregional, y supervivencia, de acuerdo a los diferentes programas terapéuticos. En los primeros ensayos clínicos se utilizó PH o PHA seguida de cirugía solamente. En 11 de 17 pacientes valorables tratados con PH y 17 con PHA, fue posible realizar cirugía conservadora. Se presentó una incidencia alta de relapso locorregional (24%), con tasas bajas de supervivencia global: 50.1% y 31.7% a 5 y 10 años con PH y cirugía, y 47.9% con PHA y cirugía tanto a 5 como a 10 años. El protocolo fue consecuentemente modificado para incluir una infusión intraarterial continua de Adriamicina® (ADR) (17 pacientes) o radioterapia (9 pacientes) antes de la cirugía. Los resultados logrados hasta el momento pueden ser resumidos así: (a) la cirugía conservadora con salvamento del miembro fue posible en la totalidad de los pacientes; (b) el porcentaje de falla locorregional disminuyó aproximadamente 12% después de PHA + infusión de ADR + resección, con supervivencias globales a 5 y 10 años de 77.6%, y tasas de estado libre de enfermedad a 5 y 10 años de 57.8%; (c) no se presentaron recurrencias locales en el grupo tratado con PHA + radioterapia + resección, con una tasa de supervivencia global a 5 años de 71.5% y una tasa de estado libre de enfermedad a 5 años de 50.4%. En conclusión, los aproches con terapia combinada multimodal empleados parecen haber mejorado tanto los resultados funcionales como las tasas de curación a largo plazo, aunque estos resultados aún deben ser reconfirmados en una serie mayor de pacientes.

SUMMARY MALIGNANT FIBROUS HISTIOCYTOMA OF THE LUNG Malignant fibrous histiocytoma (MFH) is a soft tissue sarcoma rarely associated with lung. The most common form is sto- riform pleomorphic type. The treatment is complete surgical resection. The survival is directly related to the size of tu- mor. In this report, a fifty two-year-old male patient presented with MFH located paravertebraly in

Purpose Angiogenesis plays a crucial role in metastatic progression of soft tissue sarcomas (STS). Endothelial cells are the primary target of metronomic chemotherapy. We report the safety and the efficacy of metronomic chemotherapy in metastatic STS patients. Methods The medical charts of 26 metastatic STS patients treated at Institut Bergonie (Bordeaux, France) with metronomic etoposide (100 mg/day orally for 21 consecutive days, repeated every 4 weeks) were reviewed by two independent investigators. Results Median age was 49. All but three patients received prior treatment with doxorubicin and/or ifosfamide. One patient (4%) had partial response and 11 patients (42%) had stable disease for more than 24 weeks.

Survival of patients aged 15-24 years, diagnosed with cancer during the period of 1990-1994, is described within Europe. Data on 15 101 patients, extracted from the files of the 56 adult cancer registries included in the EUROCARE-3 database, representing 20 European countries, were analysed and compared. Five-year survival for 'all cancers combined' was 75% in males (ranging from 59% in Estonia to 89% in Iceland), and 78% in females (ranging from 59% in Estonia to 89% in Norway). The Northern European countries (except Denmark) and Austria had the highest survival figures, while survival in the Eastern European countries was lower than the European average. Denmark, UK, and the pool of the central European countries, had intermediate survival figures. Haemopoietic tumours were the most common malignancies: 5-year survival was high for Hodgkin's disease (89%), intermediate for non-Hodgkin's lymphoma (68%) and lower for acute lymphoblastic leukaemia (ALL) (47%) and acute myeloblastic leukaemia (AML) (39%). Five-year survival for gonadal germ cell cancers, the second most common malignancy in young adults, was 90%. Five-year survival for the other cancers under consideration was as follows: 89% for skin melanoma, 66% for all Central Nervous System (CNS) tumours, 57% for bone tumours, 58% for osteosarcoma, 42% for Ewing's sarcoma, 57% for soft-tissue sarcomas, 99% for thyroid carcinoma, 82% for uterine cervical carcinoma, and 83% for ovarian carcinoma. For more 'adult-specific tumours', 5-year survival was good for colon (77%) and lung (60%) cancers, and less favourable, compared with adults, for breast cancer (68%). Adolescents (15-19 years) had significantly worse survival than young adults (20-24 years) for all malignancies combined. Survival for Hodgkin's lymphoma, CNS tumours, melanoma and colon cancer showed marked regional variability. Since many of the tumours occurring in young adults are curable, these results should encourage, without delay, efforts to identify obstacles to improving outcome and reducing geographical inequalities in survival for this group of patients. #