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Purpose: Extremity soft-tissue sarcomas are treated by wide surgical resection with normofractionated radiotherapy. Over the past 3years, several phase II trials emerged on perioperative hypofractionated radiotherapy. We aimed to review the current practice in France and Luxembourg of hypofractionated radiotherapy as curative treatment for extremity soft-tissue sarcomas., Materials and Methods: We sent an electronic adaptive survey to sarcoma radiation oncologists at 28 centres in France and the Grand Duchy of Luxembourg belonging to the NETSARC network. The questionnaire was distributed via the NETSARC mailing list in December 2021 and January 2022. It consisted of four to nine questions with closed multiple choice, or open-ended (short or long) answers. Some questions assessed agreement with proposals for pre- or postoperative hypofractionated radiotherapy for extremity soft-tissue sarcomas., Results: Of the 28 radiation oncologists surveyed, 11 (39.2 %) from nine centres responded. Of these, 55 % used hypofractionated radiotherapy, mainly for elderly and frail patients. The main reason why hypofractionated radiotherapy was not used was the lack of scientific evidence and therapeutic habits., Conclusion: Hypofractionated radiotherapy for extremity soft-tissue sarcomas remains to be investigated in high-powered studies but could be offered in the future for well-selected patients by a multidisciplinary board in a sarcoma referral centre., (Copyright © 2024 The Authors. Published by Elsevier Masson SAS.. All rights reserved.)

Phyllodes tumors, borderline (BPT) and malignant (MPT), represent a rare group of fibroepithelial breast tumors. Due to their rarity, their treatment remains poorly codified. The precise incidence of these tumors remains unknown. TPMs represent half of breast sarcomas and 1 % of breast tumors. Their treatment at the localized stage is based on surgery, that can be conservative surgery or a mastectomy. The impact of oncoplastic techniques and immediate breast reconstruction is not documented. The excision margins of the BPT and MPT must be free, a wider margin can provide a benefit in local recurrence but in also overall survival in the case of TPM. The optimal width of the excision margin is not known. In the event of positive margins, a second surgery could make up the result of an insufficient first surgery. Chemotherapy does not seem to provide any benefit on recurrence-free survival, but the available data are particularly weak. The data on adjuvant radiotherapy are more important. This allows better local control in the event of breast-conserving surgery. The benefit of post-mastectomy radiotherapy is less documented but can be considered in cases of poor prognostic factors. The management of TPM at the metastatic stage is based on the use of chemotherapy (anthracyclines, Ifosfamide) and local treatment of metastases in cases of oligometastatic disease. Due to the rarity of these tumors, it is essential that their management be discussed within a network of qualified professionals (NETSARC+)., (Copyright © 2024 Société Française du Cancer. Published by Elsevier Masson SAS. All rights reserved.)

Background: Dermatofibrosarcoma protuberans (DFSP) is a rare skin tumor. There is no standard recommendation for its surgical management. The currently used histological analysis are HES (hematoxylin eosin saffron) staining and immunohistochemistry for CD34 expression in particular cases. Fluorescent in situ hybridization (FISH) technique is only used to qualify the DFSP as translocated or non-translocated and is not used as a diagnostic method. The aim of our study was to determine by FISH (as a diagnostic method) whether cancerous cells that could not be identified through HES staining±immunohistochemistry were present at the two-centimeter margins that were found to be tumor-free., Methods: Samples from patients who underwent surgery between 2010 and 2018 were collected. Intralesional and peripheral (at 2cm margins) paraffin slides were included. An average of 7.4 slides per specimen was analyzed. Firstly, the preselected slides were reread by a senior pathologist to confirm the absence of microscopic findings of DFSP at 2cm margins. Secondly a FISH analysis was used as a quantitative diagnostic approach, in order to find the t(17;22) translocation., Results: Among the seven specimens that included 2cm margins, two samples presented one or more translocations, which were not visible in standard morphology assessments at two centimeters tumor-free margins., Conclusions: FISH analysis can have a new role in defining tumor-free margins. This would reduce the incidence of disease recurrence after resection and improve the post-operative complementary care., (Copyright © 2023 Elsevier Masson SAS. All rights reserved.)

Introduction: Myxoid liposarcoma is a soft tissue sarcoma associated with multifocal metastases at diagnosis. These metastases are asymptomatic and occult on CT and FDG-PET and can alter the therapeutic management and prognosis. In this context, we evaluated the contribution of whole-body MRI to the initial workup of patients with myxoid liposarcoma., Method: This retrospective study was conducted between January 2015 and December 2020 at the Oscar Lambret Center. We enrolled 22 patients who were diagnosed with myxoid liposarcoma and underwent whole-body MRI at diagnosis. The number of metastases at diagnosis, their location, and the visibility of these lesions on CT were evaluated. Associations between clinical features, presence of metastasis, and their impact on management were assessed., Results: Sixteen patients (72.7%) had non-metastatic disease at the initial diagnosis, and 15 of these patients were managed using local treatment. Six patients (27.3%) had metastases at multiple locations and received chemotherapy. The main locations were the bones (n=5) and lungs (n=3). In five patients with metastases, whole-body MRI demonstrated additional lesions that were not visible on CT (bone and soft tissue lesions). Only the presence of a round cell contingent (P=0.009) was found as a criterion associated with the presence of metastases., Conclusion: The patients' young age, absence of reliable prognostic factors at diagnosis, asymptomatic nature of the lesions, and the benefits of early and targeted therapeutic management encourage the use of whole-body MRI as part of the initial work-up as it seems to provide a better initial staging compared with conventional imaging., (Copyright © 2023 Société Française du Cancer. Published by Elsevier Masson SAS. All rights reserved.)

Among the 16,000 new cases of malignant tumors of the head and neck diagnosed in France each year, 10% are not conventional squamous cell carcinomas. These so-called rare cancers are distinguished by their presentation and patterns of failure, which is important to recognize in order to offer specific adapted management and maximize the chances of tumor control. These cancers can be rare by their histology as well as their anatomical location when arising from the paranasal sinuses, salivary glands and ear. The management of these heterogeneous rare diseases of complex treatment has considerably been structured over the last 15 years, in particular via the French ENT Cancer Expertise Network (REFCOR) and international networks and registries (EURACAN, etc.). Structuration also favors research with identification of new entities and setting up of specific therapeutic trials. A first article (part 1) discusses the diagnostic and therapeutic specificities of these rare cancers, and develops the recommendations of the REFCOR concerning rare epithelial tumors, i.e., salivary tumors, sinonasal tumors, variants of conventional squamous cell carcinomas, neuroendocrine carcinomas, malignant odontogenic tumors, and ear tumors. This second article (part 2) is focused on non-epithelial tumors (sarcomas, mucosal melanomas, lymphomas, tumors of uncertain or undetermined malignancy) and describes the organization and missions of the REFCOR., (Copyright © 2023 Société Française du Cancer. Published by Elsevier Masson SAS. All rights reserved.)

Uterine leiomyosarcomas represent the most common uterine sarcomas. The prognosis is poor with metastatic recurrence in more than half of the cases. The purpose of this review is to make French recommendations for the management of uterine leiomyosarcomas within the framework of the French Sarcoma Group - Bone Tumor Study Group (GSF-GETO)/NETSARC+ and Malignant Rare Gynecological Tumors (TMRG) networks in order to optimize their therapeutic management. The initial assessment includes a MRI with diffusion perfusion sequence. The diagnosis is histological with a review in an expert center (Reference Network in Sarcoma Pathology (RRePS)). Total hysterectomy with bilateral salpingectomy, en bloc without morcellation, is performed when complete resection is possible, whatever the stage. There is no indication of systematic lymph node dissection. Bilateral oophorectomy is indicated in peri-menopausal or menopausal women. Adjuvant external radiotherapy is not a standard. Adjuvant chemotherapy is not a standard. It can be an option and consists in doxorobucin based protocols. In the event of local recurrence, the therapeutic options are based on revision surgery and/or radiotherapy. Systemic treatment with chemotherapy is most often indicated. In case of metastatic disease, surgical treatment remains indicated when resecable. In cases of oligo-metastatic disease, focal treatment of metastases should be considered. In the case of stage IV, chemotherapy is indicated, and is based on first-line doxorubicin-based protocols. In the event of excessive deterioration in general condition, management by exclusive supportive care is recommended. External palliative radiotherapy can be proposed for symptomatic purposes., (Copyright © 2023 Société Française du Cancer. Published by Elsevier Masson SAS. All rights reserved.)

Lymphatic dissemination is thought to be a rare event in breast sarcomas. The decision to perform axillary clearance is challenging. In our prospective cohort, we aimed to evaluate the frequency and factors determining lymph node (LN) involvement in breast sarcomas, with the aim of proposing a decision tree/algorithm for the realization of LN clearance in breast sarcomas., Patients and Methods: Fourty-five women were surgically treated for breast sarcomas from 1982 to 2020. Angiosarcomas and other sarcomas were compared in terms of LN involvement, recurrence, and mortality., Results: Twenty-three patients underwent axillary lymphadenectomy. Initial LN involvement was diagnosed in one case of D2-40 positive, primary angiosarcoma for which preoperative imaging detected a suspicious LN confirmed by preoperative histology. Among the 22 patients who had no initial axillary lymphadenectomy, two patients with D2-40 positive angiosarcoma had recurrent cancer in LN (internal mammary group in 1 and homolateral axilla in 1). The average follow-up in the overall population was 6.2 years (±8.3). The cohort's overall recurrence rate was 33% (15/45) and the time of recurrence after initial surgery was on average 2.4 years (±3.1). For the three patients with LN metastases, time to recurrence after surgery was 3.7 years (±4.5). There was no significant difference in the overall recurrence rate depending on whether or not lymphadenectomy was initially performed (respectively 26% vs 41% OR=1.11, P=0.29)., Discussion/conclusion: Systematic axillary clearance leads to overtreatment in breast sarcomas. A decision tree, including radiological examination of the axilla, histological type of sarcoma, and D2-40 positivity, could be a decision aid in the choice of axillary clearance., (Copyright © 2022 Société Française du Cancer. Published by Elsevier Masson SAS. All rights reserved.)

Thigh loss cover after carcinologic resection of sarcomas can be complex and compromise the vascular tree of the lower limb. We report a case of a patient with recurrent sarcoma of the right thigh. After multiple resections, the femur and hip joint are exposed. The superficial and deep femoral vessels are taken in the excisional piece. The reconstruction is performed by a free musculocutaneous latissimus dorsi flap, anastomosed to the deep inferior controlateral epigastric vessels. The deep inferior epigastric vessels are of sufficient length to join the contralateral hemiabdomen after dissection with a gauge allowing microsurgical anastomoses. They can serve as recipient vessels for a flap covering the contralateral thigh., (Copyright © 2021 Elsevier Masson SAS. All rights reserved.)

Reirradiation and irradiation of sarcoma is often difficult due to the frequent need for a high dose of radiation in order to increase tumor control. This can result in a greater risk of toxicity which can be mitigated with the use of proton therapy. The present review aims to summarize the role of proton therapy in these 2 clinical contexts., (Copyright © 2021 Société française de radiothérapie oncologique (SFRO). Published by Elsevier Masson SAS. All rights reserved.)

Introduction: Radiation therapy may cause a range of side effects of the skin within the irradiated area. Not of all the reactive effects of the skin induced by radiation therapy have to be related to some forms of radiodermatitis, and when non-standard clinical presentations overcome, it may be necessary to undertake appropriate diagnostic tools to not be in trap of wrong diagnosis., Clinical Case Description: A 76 years-old man undertook resection surgery after a neoadjuvant radiation therapy for a soft tissue sarcoma of his groin region. After surgery, he developed an acute skin reaction comparable with a severe form of radiodermatitis. Despite cares, his clinical status got worse. Only skin biopsies guided us to the right diagnosis: it was a form of a bullous pemphigoid induced by radiation therapy. The consequent appropriate treatment was finally resolute., Conclusion: These forms have to be recognized in time, to undertake skin biopsies as soon as an evocative clinical presentation appears. The appropriate treatment, which consists in local or systemic corticotherapy, is resolute in most cases., (Copyright © 2021 Elsevier Masson SAS. All rights reserved.)

Radiation therapy is a standard treatment for limbs soft tissue sarcomas. Preoperative versus postoperative radiotherapy has been a controversial topic for years. With preoperative irradiation, the treatment volume is more limited, the delivered dose possibly lower and the tumor volume easier to delimit. Only one randomized trial compared these two irradiation sequences. The results in terms of local control and survival were equivalent but the risk of acute postoperative complications was higher if irradiation was administered before surgery. However, in the latest update of this trial, patients who received adjuvant irradiation exhibited more severe late toxicity than those treated preoperatively. In addition, with modern irradiation techniques such as conformal with image-guided intensity modulated radiotherapy and flap coverage techniques, the incidence of complications after preoperative irradiation were lower than historically published rates. Locally advanced proximal sarcomas and the failure of other neoadjuvant treatments are nowadays classical indications for preoperative irradiation. As with other neoadjuvant treatments, induction radiotherapy must be personalized according to the histological subtype, the tumor site and the benefit/risk ratio, which is best appreciated by a multidisciplinary surgical and oncological team in a specialized center in the management of soft-tissue sarcomas., (Copyright © 2021 Société Française du Cancer. Published by Elsevier Masson SAS. All rights reserved.)

Sarcoma consists in a group of rare malignant tumours of mesenchymal origin characterized by their vast clinical, pathological and biological heterogeneity. The pathological diagnosis of sarcoma relies classically of the differentiation features of tumour cells, with dozens of different tumour subtypes described in the last international classifications. Over the last decades, the advances in the development of new techniques of molecular biology have led to a major complexification of sarcoma classification, with the identification of multiple and specific molecular alterations that have led to significant changes for patients diagnostic, prognostic and therapeutic management. This review aims at giving an overview on the current knowledge of the molecular biology of soft tissue sarcoma, and emphasizes on their consequences for the daily management of patients., (Copyright © 2021 Société Française du Cancer. Published by Elsevier Masson SAS. All rights reserved.)

Orthopaedic surgery I has a key role on the medical pathway of adolescent and young-adult (AYA) patients with musculo-skeletal tumor from diagnosis, tumor resection, musculo-skeletal immediate or delayed reconstruction, to dealing with late complications and sequelae following reconstruction during childhood. Administrative authorizations, organization by health authorities as well as training courses are quite different for adult and pediatric surgery. However, surgery for musculo-skeletal tumors in AYA patients have neither adult nor pediatric specifities; resection-reconstruction rules and technics are the same for adult or pediatric patients, with nevertheless lower complication rate and more biologic reconstructions for the youngest, and more prosthetic reconstructions for the oldest. Late complications after bone and soft-tissue reconstructions in childhood and musculo-skeletal deformation after surgery and/or radiotherapy in childhood need the vision from "adult" orthopaedic teams for long and very long term success of the surgical project with a good knowledge of pediatric technics previously used. Thus, formal shared time and transition care are necessary between pediatric and adult surgical teams to prepare long-term follow-up of these childhood cancer survivors. Participation to the same specialized Multi-Disciplinary Board, scientific society gathering adult and pediatric surgeons, clinical trials with no age-limitation might help to erase barriers and to ease collaboration between adult and pediatric ortho-oncologic teams., (Copyright © 2020 Société Française du Cancer. Published by Elsevier Masson SAS. All rights reserved.)

Benign and malign tumors can affect the temporomandibular joint (TMJ) as any other articulation. Nevertheless, TMJ tumors are rare and mostly benign. Their clinical expression is varied including symptomatology similar to TMJ dysfunctional disorders, otologic or neurologic pathologies. In some cases, they remain totally asymptomatic. Hence, diagnosis is difficult since the symptomatology can be misleading with TMJ dysfunctional disorders or otologic disorders wrongly diagnosed. There is thus frequently a long delay between symptoms onset and diagnosis. The great variety of TMJ lesions explains the wide range of possible treatment modalities, mostly based on surgery. We provide here a review of the lesions originating from the TMJ. Tumoral or cystic mandibular lesion affecting the TMJ through local extension will not be discussed. Osteoma, osteoid osteoma, osteoblastoma, chondroma, osteochondroma, chondroblastoma, tenosynovial giant cell tumors, giant cell lesions, non-ossifying fibroma, hemangioma, lipoma or Langerhans cell histiocytosis are all possible diagnosis among the benign tumors found in the TMJ. Pseudotumors include synovial chondromatosis and aneurysmal bone cyst. Finally, malign tumors of the TMJ include mainly sarcomas (osteosarcoma, chondrosarcoma, synovial sarcoma, Ewing sarcoma, and fibrosarcoma), but also multiple myeloma and secondary metastases. We will review the clinical, radiological and histological aspects of each of these lesions. The treatment and the recurrence risk will also be discussed., (Copyright © 2020 Société Française du Cancer. Published by Elsevier Masson SAS. All rights reserved.)

Introduction: Extramedullary hematopoiesis is a complication of myeloproliferative neoplasms or of chronic hemolysis. The more frequent localizations are splenic, ganglionic or paraspinal. Rarely, extramedullary hematopoiesis is associated with solid cancer., Case Report: We report an original case of sarcoma located in an extramedullary hematopoiesis mass in a 72-year-old woman suffering from hereditary spherocytosis. An asymptomatic right paravertebral mass was found in 2004; the biopsy confirmed extramedullary hematopoiesis. In 2016, the patient was hospitalized due to paravertebral pain. Computed tomography showed the extension of the right paraspinal mass to pleura and mediastinum as well as vertebral bone lysis. Positron emission tomography showed an intense hypermetabolism. The biopsy showed undifferentiated sarcoma., Conclusion: This case report illustrates the risk of neoplastic transformation of extramedullary hematopoiesis, and the need for a biopsy when confronted to atypical aspect., (Copyright © 2020. Published by Elsevier Masson SAS.)

Gastrointestinal stromal tumors (GIST) are the most common non-epithelial tumors of the gastrointestinal tract. Wild-type GISTs (WT-GIST) consist of a rare heterogeneous group characterized by the lack of activating mutations in the tyrosine kinase receptor (Kit) and/or platelet derived growth factor receptor A (PDGFRA). However, WT-GIST is characterized by other genomic alterations, including dehydrogenase succinate (SDH) deficiency or mutations in the Ras pathway. Recent studies have reported many mutations in others genes that may be incriminated in the development of WT-GISTs. Moreover, WT-GIST is frequently associated with hereditary cancer syndromes such as the Carney Triad and Type 1 Neurofibromatosis (NF1). WT-GIST affects usually young and pediatric patients. Most WT-GIST subtypes are insensitive to imatinib; therefore, their therapeutic management is somewhat different from usual GISTs. This review resumes the molecular and therapeutic features of this rare entity., (Copyright © 2020 Société Française du Cancer. Published by Elsevier Masson SAS. All rights reserved.)

Purpose: To evaluate the efficacy and toxicity of hypofractionated radiotherapy in non-metastatic soft tissue and bone sarcomas., Patients and Methods: Thirty patients underwent hypofractionated radiotherapy between 2007 and 2015. Overall, 17 patients underwent primary hypofractionated radiotherapy, nine underwent hypofractionated radiotherapy for reirradiation, and four received a boost dose via hypofractionated radiotherapy after external beam radiotherapy. Most common disease sites were head and neck and retroperitoneum. Hypofractionated radiotherapy was administered with a definitive, adjuvant, or neoadjuvant intent., Results: Median age was 37 years (range: 11-82 years). Median hypofractionated radiotherapy dose was 35Gy (range: 20-50Gy) in three to five fractions. Median follow-up was 21 months (range: 1-108 months). One- and 2-year overall survival rate was 75% and 52%, respectively. One- and 2-year local recurrence-free survival rate was 59% and 48%, with local recurrence rates of 16% and 33% in 1 and 2 years, respectively. Univariate analysis revealed tumour size (P=0.04), hypofractionated radiotherapy intent (P=0.016) and reirradiation (P=0.001) as prognostic factors for local recurrence-free survival. Severe late toxicity was observed in one patient as grade 3 trismus., Conclusion: Hypofractionated radiotherapy as the primary treatment or for reirradiation has been shown to be safe in the treatment of bone and soft tissue sarcomas. It can provide relatively good local control and survival rates., (Copyright © 2019 Société française de radiothérapie oncologique (SFRO). Published by Elsevier Masson SAS. All rights reserved.)

Primitive intimal sarcoma is a rare malignant cardiac tumor. Through the observation of a 41-year-old man who presented with an acute heart failure revealing a left atrial intimal sarcoma, we discuss different diagnostic and therapeutic modalities., (Published by Elsevier Masson SAS.)

We describe herein the inaugural manifestations, the radiological and histological diagnosis criteria for and the outcome of epithelioid hemangioendothelioma (EHE). Most of EHE (90%) display a specific reciprocal chromosomic translocation t(1;3)(p36;q23-25), which is associated with the synthesis of fusion protein WWTR1/CAMTA1. EHE are low-grade vascular sarcomas. EHE could be initially localized or multifocal. At localized stage, EHE are best treated with focal treatments. At multifocal stage, the upfront strategy is watchful follow-up. Some multifocal EHE display very indolent course with spontaneous stable disease for years or decades. In case of progressive multifocal EHE, there is no consensual treatment. Diagnostic and clinical management of EHE requires interdisciplinary expertise from labeled centers., (Copyright © 2018 Société Française du Cancer. Published by Elsevier Masson SAS. All rights reserved.)

Sarcomas are a common type of tumor within the pediatric population. The utilization of proton therapy as a primary attribute the ability to spare adjacent healthy tissue, therefore, proton therapy has become a preferential indication in pediatrics compared to other photon irradiation modalities. Proton therapy is also a proven and historically validated irradiation technique in the treatment of chondrosarcomas and chordomas of the skull base and spine. Additionally, proton therapy can potentially limit irradiated healthy tissue volumes in adults and limit the risk of acute and late toxicities. The evaluation of the effectiveness of proton therapy in sarcomas is underway in many clinical situations in prospective trials, some of which are randomized., (Copyright © 2018 Société Française du Cancer. Published by Elsevier Masson SAS. All rights reserved.)

Purpose: Primary breast sarcomas are heterogeneous tumours derived from non-epithelial mammary gland structures. Although they represent a rare entity, their incidence may increase in the coming years owing to conservative approach considered in the treatment of breast cancer. The aim of this work was to highlight the effect of postoperative irradiation in the treatment of these tumours., Materials and Methods: This is a retrospective study conducted at the Mohammed-VI centre for cancer treatment between 2004 and 2011. Survival rates were calculated by the Kaplan-Meier method., Results: Fifteen cases were collected. The median age was 41.9years. Phyllode sarcoma accounted for 66% of this series. Surgical treatment was performed in 93% of the patients with negative margins in 33.33% of the cases. Neoadjuvant chemotherapy was indicated in 46% of the patients with locally advanced tumours and 66% of the patients received postoperative radiotherapy for positive or close margins. Five years overall survival and relapse free survival was not significantly different with the use of adjuvant radiotherapy., Conclusion: Due to the rarity of this entity and the absence of randomized trials, evidence based management is still lacking. However, a multidisciplinary approach is to be required including surgical excision followed by radiotherapy, depending on the tumour characteristics., (Copyright © 2018 Société française de radiothérapie oncologique (SFRO). Published by Elsevier Masson SAS. All rights reserved.)

Context: Ifosfamide remains one of the major cytotoxic drugs for sarcoma management. Ifosfamid-related encephalopathy (IRE) is a rare but severe adverse event, without clearly identified risk factors., Method: We have carried out a single-center, retrospective study to assess the occurrence and the risk factors for IRE after the two first cycles of chemotherapy. We have collected the data-describing patients, biological data, tumors characteristics (histology, leptomeningeal metastasis) and ifosfamide administration modalities., Results: From September 2008 to November 2013, we have identified 8 IRE out of 187 patients (4.2% [CI95%: 1.8-8.2]). The median age was 27 (0-78). Histologies were adult soft tissue sarcomas (78 patients), osteosarcoma (48), ewing sarcoma (41) and rhabdomyosarcoma (26). Most of factors were not associated with IRE. Only 8 patients have received aprepitant, none of them experienced IRE. Under univariate analysis, the risk factors for IRE were: PS≥2 (OR=9.52 [CI95%: 2.38-38.80]), albumin≤36g/L (OR=9.79 [CI95%: 1.19-80.26]), leptomeningeal metastasis (OR=13.20 [CI95%: 2.76-63.19]), 4 or 5 successive days of ifosfamide administration (OR=6.00 [CI95%: 1.40-25.60]). Under multivariate analysis, the risk factors for IE were: PS≥2 (OR=16.00 [IC95%: 2.80-67.00]), leptomeningial metastasis (OR=23.56 [IC95%: 2.01-456.80]) and 4 or 5 days of ifosfamide administration (OR=57.45 [IC95%: 1.66-35.00])., Conclusion: Ifosfamide administration must be given with caution in patients with poor performans status. A 4 to 5 days fractioned ifosfamide and leptomeningeal metastasis seems associated with increased risk for IRE, whatever the total administered dose., (Copyright © 2016 Société Française du Cancer. Published by Elsevier Masson SAS. All rights reserved.)

Purpose: Spermatic cord sarcoma is a rare disease, which management remains controversial due to the lack of guidelines. The standard therapeutic approach is surgical: wide soft-tissue resection with radical inguinal orchidectomy, The diagnosis is made during the analysis of the specimen. The high rate of local recurrence indicates adjuvant radiotherapy of the tumor bed. The aim of this series is to determine the efficacy and safety of postoperative intensity-modulated radiotherapy for spermatic cord sarcomas., Patients and Methods: Our series included five consecutive cases of spermatic cord sarcoma treated between 2011 and 2014. The indications for radiotherapy were: R1 status after initial surgery, R1 status after wide en bloc resection and orchiectomy, high French federation of cancer centers (FNCLCC) grade, tumor size over 5cm, tumor resection during surgery., Results: Median age at diagnosis was 66years (range 46-84years). Median follow-up was 18months (range 6-28months). Four patients had repeat surgery after incomplete removal. All surgeries were orchidectomy with primary ligation of testicular vessels. One patient did not have an in sano margin after the second surgical procedure. The median tumor size was 60mm (range 30-150mm). No recurrence was observed during the follow-up., Conclusion: No grade 4 toxicities were reported and the most frequent acute toxicity was dermatitis. No recurrence was reported after adjuvant intensity-modulated radiotherapy. The treatment is feasible and well tolerated and seems to provide encouraging results regarding locoregional control of the disease. Dynamic or rotational intensity-modulated radiotherapy is now recommended to decrease acute toxicities while improving the efficacy of this approach., (Copyright © 2016 Société française de radiothérapie oncologique (SFRO). Published by Elsevier SAS. All rights reserved.)

We report the case of a 55-year-old woman suffering from a type I neurofibromatosis (also known as Von Recklinghausen neurofibromatosis) who was diagnosed with a high-grade schwannosarcoma of the median nerve, between the upper third and the medium third of the arm, upon contact with the humerus, invading the humeral vessels. The oncologic treatment of this tumour consisted in the amputation of the arm through the surgical neck of the humerus. In order to create a laterothoracic claw, to bring a partial function of the upper limb back, we decided to realize a free fillet forearm flap. This composite flap was composed of the radius and the ulna, all the forearm muscles and the skin of the anterior side of the forearm. A humeroradial plate osteosynthesis was done and the flap was then harvested with the radial pedicle, and anastomosed to the axillar artery. This procedure gave our patient a functional stump, giving back the arm functionality, especially the claw movement., (Copyright © 2014 Elsevier Masson SAS. All rights reserved.)

Objective: To report epidemiologic, anatomoclinical treatment and results about a Tunisian retrospective serie of 14 patients with uterine sarcoma (US)., Patients and Methods: A retrospective study of 14 cases of uterine sarcoma treated in the Radiotherapy Unit of Farhat Hached Hospital of Sousse between 1995 and 2008. Epidemiologic and anatomoclinical features were assessed. A complete work-up including abdominal ultrasonography and abdominopelvic CT scan were perfomed in 7 and 10 cases, respectively., Results: The median age was 48.5 years (15 to 78) without previous medical history of irradiation or prolonged drug exposition. There were 3 cases of leimyosarcoma, 7 cases of mixed Mullerian tumor and 4 cases of endometrial stromal sarcoma. The diagnosis was made postoperatively in 6 patients and after curettage in 8 cases. According to the classification of the International Federation of Gynecology and Obstetrics (FIGO), 8 patients were in FIGO stage I, 3 in stage II and 3 in stage III. Hysterectomy was associated with annexectomy in 12 cases. Bilateral pelvic lymphadenectomy was performed in one patient. Eight of our 14 patients underwent postoperative pelvic radiotherapy, associated with adjuvant chemotherapy in 3 cases. The evolution was marked by the occurrence of local recurrence in 4 patients who did not have adjuvant therapy with lung metastases in one case and peritoneal carcinomatosis in another case. With a medium follow-up from 54 months, 7 patients are free from disease, 5 died of their disease (after a mean of 24 months) and 2 patients were lost to follow-up., Discussion and Conclusion: Uterine sarcomas are rare tumors with poor prognosis. Adjuvant therapy (radiotherapy and/or chemotherapy should be discussed due to the high risk of recurrence or metastases., (Copyright © 2014 Elsevier Masson SAS. All rights reserved.)

Purpose: The present observational study reports the results of a multi-institutional dummy-run designed to estimate the consistency of interobserver variability in clinical target volume delineation in two different cases of soft-tissue sarcomas in which postoperative and preoperative radiotherapy were prescribed, respectively. The purpose of this work was to quantify interobserver variability in routine clinical practice., Patients and Methods: Two different cases of soft-tissues sarcomas were chosen: a case of postoperative and a case of preoperative radiation therapy. Participating centres were requested to delineate clinical target volumes according to their experience in both cases. Descriptive statistic was calculated for each variable (volume, diameters) separately for two cases. Box-whiskers plots were used for presentation of clinical target volume. A Shapiro-Wilk's test was performed to evaluate the departures from normality distribution for each variable. The comparison between relative variations of diameters was evaluated using the Student's t test., Results: Several variations affecting both volumes and diameters were observed. Main variations were observed in the craniocaudal and laterolateral diameters. Each case showed similar dispersion, indicating a lack of reproducibility in volumes definition., Conclusions: This observational study highlighted that, in the absence of specific instructions or guidelines, the interobserver variability can be significant both in postoperative and preoperative radiotherapy of soft-tissue sarcomas., (Copyright © 2013 Société française de radiothérapie oncologique (SFRO). Published by Elsevier SAS. All rights reserved.)

Purpose: Primary cardiac sarcomas represent less than 10 yearly cases in France. Their median survival is approximately 18 months. The treatment consists of surgery when possible. The role of chemotherapy and radiation therapy is controversial, especially with respect to limiting cardiac radiation dose that is theoretically incompatible with the requirement of a tumoricidal dose for sarcoma. A recent series of 124 cases of the French Sarcoma Group suggested a benefit of radiation therapy on progression-free survival., Patients and Methods: The dosimetric data of 12 patients were analyzed., Results: There was variety in radiotherapy modalities and definition of target volumes, doses and techniques are evolving more conformal plans. Irradiation appeared feasible with conventional fractionation with respect to toxicities (although probably underestimated due to short follow-up and dismal prognosis) and previously demonstrated benefit of radiotherapy for primitive cardiac sarcomas., Conclusion: A scheme of 45Gy in 1.8Gy per fraction to a preoperative volume with an additional dose of 14Gy in 7 fractions on areas at risk or residual disease and margins 1cm, may be proposed based on the preliminary data of this study. Intensity modulated radiotherapy with daily cone-beam CT-scanner should be evaluated., (Copyright © 2014 Société française de radiothérapie oncologique (SFRO). Published by Elsevier SAS. All rights reserved.)

We report the case of a man presenting with a Pierre Marie-Bamberger syndrome. This paraneoplastic syndrome revealed an undifferentiated intracardiac sarcoma. This case emphasizes the need for multimodality imaging to characterize intracardiac tumor., (Copyright © 2013 Elsevier Masson SAS. All rights reserved.)