[Rare case of reconstruction of the anterior thigh compartment after resection of high-grade sarcoma by hybrid technique: Latissimus dorsi free flap associated with a medial gastrocnemius pedicled flap]. / Cas rare de reconstruction de la loge antérieure de cuisse après résection d'un sarcome de haut grade par technique hybride : lambeau libre de grand dorsal associé à un lambeau gastrocnémien médial pédiculé.

INTRODUCTION: The soft tissue sarcomas of the limbs require extensive surgical excision. Reconstructive surgery plays an essential role in its management to preserve the limb, as exposed in a case of thigh sarcoma. CLINICAL CASE: A 73-year-old patient has a large sarcoma within the quadriceps. Its excision requires resection of the entire anterior thigh compartment. Preservation of the limb is permitted by reconstruction by latissimus dorsi free flap and medial gastrocnemius pedicled flap. The patient resumed walking using an orthosis at 3 months. CONCLUSION: Surgery to remove a sarcoma of a limb can threaten the integrity of the limb. Plastic surgery, with all the reconstruction techniques, contributes to the preservation of a functional limb.

Intraoperative motive for incomplete resection in primary retroperitoneal sarcoma.

INTRODUCTION: Surgical resection is the current standard of care for retroperitoneal sarcoma (RPS). Recent data suggests that up to 5% of patient have incomplete (R2) resection. The exact reason why patients scheduled for surgery with a curative intent to treat ended up with an R2 resection is largely unknown. AIM OF THE STUDY: To identify intraoperative findings responsible for incomplete (R2) resection in primary RPS. METHODS: All records of consecutive patients scheduled for a non-metastatic primary RPS surgery between 1995 and 2020 in a tertiary care sarcoma centre were retrospective analyzed. RESULTS: Among the 347 patients scheduled for surgery, 13 (3.7%) had an incomplete (R2) resection. The reasons for incomplete surgery were intraoperative finding of vascular involvement of great vessels in 5 patients, previously undetected peritoneal metastases in 5 patients, invasion of contralateral kidney/ureter in 2 patients and the need to preserve both kidneys in 1 patient because of his past medical history. Among these patients, 3 had a laparotomy without resection and 10 had a partial resection (i.e. debulking surgery). Severe postoperative complications occurred in 5 patients. The median length of stay in hospital was 19days. After a median follow-up of 12months, the median survival of patients after incomplete resection was 18months. The 1-y, 5-y and 8-y overall survival (OS) for these patients were 46%, 14%, and 7%, respectively. CONCLUSION: Incomplete (R2) resection for a primary RPS surgery is rare in specialized sarcoma center. The next steps should be to identify the preoperative criteria that lead to this accurate selection and to define the best practice in front of a peroperative discovery of an unresectable RPS. LEVEL OF EVIDENCE: III.

No 371 - Le morcellement durant la chirurgie gynécologique: Son utilisation, ses complications et les risques liés à la présence de tumeurs malignes insoupçonnées.

OBJECTIF: La présente directive clinique conseille les gynécologues quant au recours au morcellement tissulaire pendant une chirurgie gynécologique. RéSULTATS: Le morcellement effectué au cours d'une chirurgie gynécologique peut permettre l'ablation de masses utérines volumineuses, offrant ainsi aux femmes une solution chirurgicale à effraction minimale. Les conséquences oncologiques indésirables du morcellement tissulaire devraient être atténuées par l'amélioration de la sélection des patientes, la tenue d'examens préopératoires et l'adoption de techniques novatrices réduisant au minimum la dispersion tissulaire. ÉVIDENCE: La littérature publiée a été récupérée au moyen de recherches menées dans PubMed et Medline au printemps 2014 à l'aide d'une terminologie contrôlée (« leiomyosarcoma ¼, « uterine neoplasm ¼, « uterine myomectomy ¼, « hysterectomy ¼) et de mots-clés (« leiomyoma ¼, « endometrial cancer ¼, « uterine sarcoma ¼, « leiomyosarcoma ¼, « morcellation ¼). Les résultats retenus provenaient de revues systématiques, d'essais cliniques randomisés, d'essais cliniques contrôlés et d'études observationnelles de langue anglaise ou française. Aucune restriction de date n'a été imposée. Les recherches ont été refaites régulièrement, et les résultats ont été incorporés à la directive clinique jusqu'en juillet 2017. Nous avons également tenu compte de la littérature grise (non publiée) trouvée sur les sites Web d'organismes d'évaluation des technologies de la santé et d'autres organismes liés aux technologies de la santé, dans des collections de directives cliniques et dans des registres d'essais cliniques, et obtenue auprès d'associations nationales et internationales de médecins spécialistes. VALEURS: La qualité des données probantes a été évaluée en fonction des critères décrits dans le rapport du Groupe d'étude canadien sur les soins de santé préventifs. AVANTAGES, INCONVéNIENTS ET COûTS: Les gynécologues offrent aux femmes une chirurgie à effraction minimale pouvant comprendre le recours à un morcellateur électromécanique pour faciliter le retrait des tissus. Les femmes devraient être informées que l'utilisation d'un morcellateur en présence de tumeurs utérines (sarcomes, tumeurs endométriales), cervicales ou tubo-ovariennes jusque-là insoupçonnées est associée à un risque accru de dissémination. Le morcellement tissulaire devrait être précédé d'une évaluation complète, d'une sélection appropriée des patientes et de l'obtention du consentement éclairé de ces dernières, et devrait être effectué par des chirurgiens ayant une formation adéquate en matière de pratiques de morcellement tissulaire sûres. DéCLARATIONS SOMMAIRES: RECOMMANDATIONS.

[Evaluation of the management and conformity to clinical practice guidelines of soft tissues lesions. Study performed at the competence center of Lorraine area]. / Évaluation et impact du respect du référentiel ESMO sur la prise en charge des lésions des tissus mous, étude menée au centre de référence de Lorraine.

INTRODUCTION: Despite regular recommendations issued by the European Society for Medical Oncology (ESMO), patients faced still too often inadequate care with a direct influence on prognosis. METHODS: A retrospective study was carried out at the Competence center in Lorraine Area. Patients registered in the NetSarc database between 1st, 2010 and September 1st, 2016 were included. Compliance criteria were established using the latest ESMO 2014 referential. Two groups "conforming" and "non conforming" were analyzed. A first analyze about all of the soft tissue lesion and a second only about sarcomas. RESULTS: In total, 445 patients were eligible, 344 cases were treated according to the ESMO guidelines, giving a 77.3% conformity rate (95% CI: 73.4%, 81.2%). Compliance was better for the competence center than district hospitals (P<0.001), with compliance rates of 88.7%, and 51.6%, respectively. Among the 247 sarcomas, we found a R0 resection rate better according to the ESMO guideline, 55% against 18% (P<0.001). R1 rates were 34% vs. 56% and R2 11 vs. 26% disease free survival was not related significantly to the observance of recommendations in the univariate analysis. After adjustment on potential DFS prognostic factors, in the multivariate analysis, the results were similar. CONCLUSION: Compliance with the ESMO guidelines, through appropriate management, improves the quality of surgical excision for sarcomas and avoids non-corresponding surgical gestures.

[Evaluation of the management of soft tissue sarcomas in Franche-Comté since the establishment of a multidisciplinary meeting at University Hospital. About 47 cases]. / Évaluation de la prise en charge des sarcomes des tissus mous en Franche-Comté depuis l'instauration d'une réunion de concertation pluridisciplinaire au CHRU. À propos de 47 cas.

GOALS OF STUDY: A multidisciplinary meeting (RCP) dedicated to the treatment of sarcoma was established in Franche-Comte in 2010. The goals of the study are: (a) To evaluate the treatment of sarcomas by confrontation with the existing literature; (b) To evaluate the influence of the multidisciplinary meeting on the management of sarcomas by hospitals at the regional level. MATERIALS AND METHODS: This is a retrospective single center study from 2010 to 2015 on patients with sarcoma and peripheral soft tissue drawn from a Netsarc database (National Network of sarcomas) and communicating cancer record. A database Cleanweb especially dedicated is created. RESULTS: Forty-seven patients were included: ten sarcomas at the upper member 26 to the lower limbs, 11 on the trunk. Forty patients were operated on: ten out of the university hospital, 28 at the university hospital and two in a coordinating center. Ninety percent of patients treated at the university hospital were in accordance with the recommandations. None of the patients operated out of the university hospital benefited from medical care in accordance to the recommendations. There is an increase in the number of files sent by the hospitals out of the university hospital discussed in multidisciplinary meeting, before treatment. CONCLUSION: The creation of a dedicated multidisciplinary meeting sarcoma improves the medical management of these tumors and decreases inappropriate medical managements thanks to a better education of the regional physicians.

[Large surgical resection of a shoulder sarcoma's and secure one stage reconstruction with lattissimi dorsi and serratus anterior flap. Updating about a case]. / Exérèse large d'un sarcome de l'épaule et reconstruction en un temps par double lambeau de grand dorsal et de grand dentelé pédiculé. Discussion à propos d'un cas.

INTRODUCTION: The soft tissue sarcomas (STM) are tumors developed at the expense of connective tissue. They are rare and have severe prognosis. The principles of management are recalled through an extended case of shoulder sarcoma. CLINICAL CASE: A 48-year-old patient has a sarcoma of the right pectoralis major muscle confirmed by biopsy. After multidisciplinary meeting, a wide surgical excision exposing the subclavian vessels and brachial plexus is performed with double cover flap pedicled latissimus dorsi and serratus and thin skin graft. RESULT: Healing process is acquired at 3 months. Adjuvant chemotherapy is established (adriamycin, ifosfamide) along with radiotherapy (54Gy). CONCLUSION: Sarcomas treatment has to be realized after considering multidisciplinary meeting (RCP) in dedicated structures. Surgery is the main treatment, it should ideally be R0, that is to say, integral with healthy tissue margin around the tumor (or healthy anatomical barrier). Optimal surgery performs a resection "without seen tumor". The diagnosis has to be made with a biopsy before the surgical treatment. Healing is quickly obtained due to adjuvant treatments.

[Molecular biology for sarcoma: useful or necessary?]. / Biologie moléculaire en pathologie des tissus mous : utile ou nécessaire ?

Sarcomas are a heterogeneous group of tumors. Their diagnosis is based on morphology and immunohistochemical profile, with categories of tumors according to the type of tissue that they resemble. Nevertheless, for several tumors, cellular origin is unknown. Molecular analysis performed in recent years allowed, combining histophenotype and genomics, better classifying such sarcomas, individualizing new entities and grouping some tumors. Simple and recurrent genetic alterations, such as translocation, mutation, amplification, can be identified in one of two sarcomas and appear as new diagnostic markers. Their identification in specialized laboratories in molecular pathology of sarcomas is often useful and sometimes necessary for a good diagnosis, leading to a heavy and multidisciplinary multi-step treatment.

Mesenchymal tumors and tumor-like lesions of the breast: a contemporary approach review.

The classification of the breast tumors has been revised and recently published in 2012 in the WHO blue book. Contrary to the epithelial tumors in the breast, mesenchymal tumors are rare and the classification for benign and malignant tumors is based on the same criteria in both categories, since no other specific diagnostic criteria, which would have an impact on prognosis, exist to date. The present review deals with minor changes mirroring the recent developments in the benign mesenchymal tumors (new additions are nodular fasciitis and atypical vascular lesions, while the haemangiopericytoma is removed) focusing especially on criteria to diagnose sarcomas, which represent a wide spectrum including very difficult lesions. The majority of sarcomas of the breast arise as a component of a malignant phyllodes tumor, while the pure forms are very rare. When a pure primary sarcoma of the breast is diagnosed, pathologists are encouraged to categorize the lesion according to the type of differentiation and to provide to the clinicians all the important prognostic parameters for the best treatment choice.

[Gastro-intestinal neuroectodermal tumor (GNET): A case report of a small intestine tumor with hepatic metastases]. / Tumeur neuroectodermique gastro-intestinale (GNET) : à propos d'un cas de tumeur du grêle avec métastases hépatiques.

The gastro-intestinal neuroectodermal tumor (GNET) is a rare sarcoma of the digestive tract, which was recently recognised. The knowledge of the morphological, immunohistochemical and molecular diagnostic criteria is necessary to not mistake it for the metastasis of a melanoma or for another sarcoma of the digestive tract as the gastro-intestinal clear cells sarcoma or the malignant peripheral nervous system tumor (MPNST). We report the case of a 41-year-old patient with a GNET of the small intestine with hepatic metastasis. The histological examination showed a diffuse proliferation of epithelioid cells, which only express PS100. The presence EWSR1-ATF1 gene fusions with any melanocytic differentiation leads to the diagnosis of GNET.

Management of oligometastatic/metastatic sarcomas and place of local treatments with focus on modern radiotherapy approaches.

Soft tissue sarcomas are a rare and heterogeneous disease. For localized disease, treatment is based on surgery and radiotherapy with or without chemotherapy depending on risk factors. Upfront metastases are present in 7 to 20% of cases, and are localized to the lungs in most of cases. Disseminated disease is generally considered incurable but in selected cases, aggressive local treatment of metastases allowed long survival. Treatment of primary tumour is often debated. Our purpose is to evaluate the literature concerning the role of radiotherapy in the management of primary metastatic soft tissue sarcomas.

Conformal radiotherapy in management of soft tissue sarcoma in adults.

We present the update of the recommendations of the French society of radiation oncology on soft tissue sarcomas. Currently, the initial management of sarcomas is very important as it may impact on patients' quality of life, especially in limb soft tissue sarcomas, and on overall survival in trunk sarcomas. Radiotherapy has to be discussed within a multidisciplinary board meeting with results of biopsy, eventually reexamined by a dedicated sarcoma pathologist. The role of radiotherapy varies according to localization of soft tissue sarcoma. It is part of the standard treatment in grade 2 and 3 sarcomas of the extremities and superficial trunk>5cm. In case of R1 or R2 resection, reexcision should be discussed. In such cases, it may be delivered preoperatively (50Gy/25 fractions of 2Gy) or postoperatively. In retroperitoneal sarcomas, preoperative conformal radiotherapy with or without modulated intensity cannot be proposed systematically in daily practice. Concomitant chemoradiotherapy cannot be considered a standard treatment. Intensity-modulated radiotherapy has become widely available. Other soft tissue sarcoma sites such as trunk, head and neck and gynaecological soft tissue sarcomas will be addressed, as well as other techniques that may be used such as brachytherapy and proton therapy.

Cardiac radiation-induced sarcomas: A SEER population-based study and a literature review.

PURPOSE: The aim of this study was to better understand the incidence and the clinical characteristics of cardiac radiation-induced sarcomas (RIS). MATERIAL AND METHODS: We used the surveillance, epidemiology, and end results (SEER) program cancer registry data, the largest cancer database in the United States in order to identify all cardiac RIS between 1973 and 2015. We relied on the Memorial Sloan-Kettering Cancer Center (MSKCC)-modified 1948 Cahan criterions for RIS identification. RESULTS: Out of 8,136,951 cancer patients from the SEER database, we identified 448 patients diagnosed with cardiac sarcomas. Of these 448 cardiac sarcoma patients, two were considered to have developed a cardiac RIS: a metastatic rhabdomyosarcoma occurring after one to two years following lung carcinoma irradiation, and a soft tissue sarcoma (of unspecified type) developed six years after radiation therapy for an aggressive left-sided breast carcinoma. Based on this observation, we estimated that cardiac RIS represented about 0.4% (95% CI 0.1%-1.6%) of all cardiac sarcomas. A literature review has been conducted and yielded three additional cases of cardiac RIS. CONCLUSION: Cardiac RIS are extremely rare malignancies, associated with a very pejorative prognosis. The two reported histologies are angiosarcomas and rhabdomyosarcomas, which might be over-represented among cardiac RIS. A metastatic evolution is possible for cardiac radiation-induced rhabdomyosarcomas. Surgical excision, when feasible, is a therapeutic option and is the only specific treatment reported to this date.

[Rôle of radiotherapy for sarcomas]. / Place de la radiothérapie dans la prise en charge des sarcomes.

The quality of the initial management of sarcomas is fundamental because it conditions the patient's quality of life and his overall survival. Radiotherapy should be discussed in a multidisciplinary consultation meeting within the framework of the Netsarc+network. The place of radiotherapy in patients with soft tissue or bone sarcoma depends on the histology and tumour location, knowing that it is most often associated with surgery which remains the main treatment. It is part of the standard treatment for grade II and III deep limb sarcomas of 5cm or greater in size and Ewing's sarcomas. In these indications, conformal radiotherapy with modulation of intensity is used routinely, in combination with IGRT. In other locations, such as retroperitoneal sarcomas or uterine sarcomas, radiotherapy is not a standard of care and must be discussed according to the prognostic criteria related to the patient, the tumour, and the previously received treatments. New techniques, such as proton therapy, hadron therapy (carbon ions) are techniques particularly suited to bone sarcomas considered to be radioresistant. However, large prospective trials are lacking in these rare indications, explaining the lack of recommendations of a high level of evidence.

[Diagnostic strategy in pediatrics soft tissue sarcomas]. / Stratégie diagnostique des sarcomes des tissus mous de l'enfant et adolescent.

Soft tissue sarcomas in children are rare tumor, representing around 6 to 7% of children cancer. They spread mostly sporadically (90%) and therefore are rarely associated to an underlying constitutional genetic disease (10%). About half of those sarcomas are rhabdomyosarcomas and the others are a very heterogenous histologic group with various bio-pathologies and prognosis. Clinical presentation is mainly a soft tissue lump often difficult to distinguish from more frequent benign causes (malformative, infectious, benign, or pseudotumor). Inappropriate initial diagnosis work-up has a strong impact on soft tissue sarcomas' prognosis. Adapted complementary investigations (first ultrasound and MRI) are important to compile arguments for a malign origin and to indicate a biopsy. However, predictive value of imaging exams still remains imperfect, and histological analysis by percutaneous image-guided biopsy and sometimes by surgical biopsy is often necessary. Authors realize an update on optimal diagnostic pathway including molecular tests in presence of a soft tissue mass in a child.

Pre- or postoperative radiotherapy for soft tissue sarcomas.

Sarcomas are rare tumours arising from mesenchymal tissue. A multimodal management in an expert centre combining surgery and radiotherapy is the current standard of care for localized soft-tissue sarcomas of the extremities, to enable limb-sparing strategies. The delivery of pre- radiotherapy or postoperative radiotherapy offers similar local control and survival rates but the toxicity profile is quite different: preoperative radiotherapy increases the risk of wound complications and postoperative radiotherapy affects long-term functional outcomes. While postoperative radiotherapy has long been the rule, especially in Europe, technical improvements with image-guided- and intensity-modulated radiotherapy associated with a better management of postoperative wounds has tended to change practices with more frequent preoperative radiotherapy. More recently the possibilities of a hypofractionated regimen or potentiation by nanoparticles to increase the therapeutic index plead in favour of a preoperative delivery of radiotherapy. The aim of this paper is to report pros and cons of pre- and post-operative radiotherapy for soft-tissue sarcomas.

[Desmoplastic small round cell tumor in children, adolescents and young adults]. / Les tumeurs desmoplastiques à petites cellules rondes de l'enfant, de l'adolescent et du jeune adulte.

Desmoplastic small round cell tumor (DSRCT) is a rare sarcoma that typically affects pediatric and young adult patients with a median age in the general and in the pediatric population of 24.6 years (range 4-58 years) and 15.0 years (range 0-21 years) respectively, with a strong male predominance. This tumor is characterized by a specific t(11;22)(p13;q12) that results in fusion of EWS and WT1 genes which can be demonstrated by RT-PCR or by FISH. DSRCT most frequently presents as an intra-abdominal primary mass associated with peritoneal seeding and a highly aggressive pattern of spread. Generally, all tumors showed the typical histologic findings of variably sized clusters of poly-phenotypic small, round, or spindled cells lying in a desmoplastic stroma. Treatment of this malignancy remains a challenge. The combination of polychemotherapy regimens and aggressive surgery followed by whole abdomen radiation therapy represents nowadays a classical protocol for DSRCT. The survival rate of DSRCT patients is still disappointing around 20 %. However, the survival of patients who had complete resection of the tumor appears better. Hopes are turning to targeted therapeutics against this simple genomic sarcoma. Authors summarize medical knowledge of this rare tumor.

[Primary breast sarcomas: About 30 cases treated at Salah-Azaiez institute in Tunisia]. / Sarcomes mammaires primitifs : à propos de 30 cas traités à l'institut Salah-Azaiez de Tunis.

PURPOSE: To identify retrospectively prognostic factors of primary breast sarcoma and review its treatment modalities. MATERIALS AND METHODS: This is a descriptive study on 30 cases of primary breast sarcoma. We carried out a univariate and multivariate analysis correlating clinical, pathological and therapeutic parameters with disease-free survival and overall survival. RESULTS: The mean age was 46.8 years. The mean tumour size was 10cm. The 30 cases were 18 phyllodes sarcomas, eight angiosarcomas, three liposarcomas and a case of granulocytic sarcoma. Sixteen patients had adjuvant radiotherapy and only seven patients received adjuvant chemotherapy. The median follow-up was 64 months. Overall survival rates at 3 and 5 years were 49.1% and 33.7%. Disease-free survival rates at 3 and 5 years were 22.8% and 15.2% respectively. The analytical study of the following parameters: tumour size and presence or absence of node or distant metastases, showed no correlation with overall survival nor with disease-free survival. Furthermore, adjuvant radiotherapy did not improve overall survival (P=0.298; hazard ratio [HR]=1 [0.982-1.04]) nor disease-free survival (P=0.61; HR=0.942 [0.862-1.029]). By univariate analyses, we identified a correlation between overall survival, surgical margins (>1cm) (P=0005; HR=3.4 [1.217-9.919]) and tumour necrosis (P=0.028; HR=0.099 [0.014-0.682]). We did not find any independent prognostic factor by multivariate analysis. CONCLUSION: The prognosis of primary breast sarcoma seems to depend essentially on optimal surgical excision (margin over 1cm). The only potential histological parameter correlated with the prognosis is the presence of tumour necrosis. The histological subtype should not be considered as a prognostic marker for overall or disease-free survival in patients with primary breast sarcoma.

[Brachytherapy for sarcomas]. / Curiethérapie des sarcomes.

The standard of care for local treatment for extremities soft tissue sarcomas relies on conservative surgery combined with external beam radiotherapy. Brachytherapy can be realized instead of external beam radiotherapy in selected cases, or more often used as a boost dose on a limited volume on the area at major risk of relapse, especially if a microscopic positive resection is expected. Close interaction and communication between radiation oncologists and surgeons are mandatory at the time of implantation to limit the risk of side effects. Long-term results are available for low-dose rate brachytherapy. Nowadays, pulsed dose rate or high-dose-rate brachytherapy are more often used. Brachytherapy for paediatric sarcomas is rare, and has to be managed in reference centres.

[Radiotherapy of adult soft tissue sarcoma]. / Radiothérapie des sarcomes des tissus mous de l'adulte.

Incidence of soft tissue sarcoma is low and requires multidisciplinary treatment in specialized centers. The objective of this paper is to report the state of the art regarding indications and treatment techniques of main soft tissue sarcoma localisations.

[Radiotherapy for soft tissue sarcomas: Technical evolution and impact on clinical benefit]. / Radiothérapie des sarcomes des tissus mous des membres : évolution technique et impact sur le bénéfice clinique.

The standard treatment for extremity soft tissue sarcomas is based on the association of surgery and radiotherapy. This strategy allows local control improvement with the risk of increased toxicity. There is therefore a growing interest to identify those patients who will benefit from radiotherapy and those who will have the same local control with surgery alone. Furthermore, the development of toxicity has been correlated with the extension of the irradiated volume and the volume receiving high doses. Technological development as intensity modulated radiotherapy and image-guided radiotherapy allows limited irradiated volume improving the protection of the organs at risk leading to clinical benefit improvement. Moreover, efforts are being done to improve local control for the patients at high risk of local relapse. In this paper, we discuss all these mentioned aspects.