Search

Extremity soft-tissue sarcomas are treated by wide surgical resection with normofractionated radiotherapy. Over the past 3 years, 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. 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. 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. 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. [ABSTRACT FROM AUTHOR]

Introduction: malignant tumors including soft tissue sarcomas can occur during neurofibromatosis type 1. We report a case of sarcomatous degeneration of a plexiform neurofibroma with multivisceral metastases in a Togolese patient with neurofibromatosis type 1. Observation: A 33-year-old female patient with neurofribromatosis type 1 presented with an altered general condition, pain and changes in the appearance of the plexiform tumour of her neurofibromatosis, which had been evolving for one month. Physical examination revealed multiple coffee-white spots (greater than 10), with tumours of soft consistency and variable size, scattered over the body. There was also a larger tumour (approx. 9cmx6 cm), located on the inside of the left ankle, fixed, firm and painful to palpation. The abdomen was enlarged and tender, with no tenderness or contracture. Examination of lymph nodes was normal. Anatomopathological examination of the biopsy-exeresis of this tumour revealed a proliferation of clusters of small, rounded, undifferentiated cells with reduced cytoplasm and hyperchromatic nuclei, with clear anisokaryosis and numerous atypical mitotic figures. There were also a few vascular emboli. We therefore concluded that this plexiform neurofibroma was malignant, and the extension work-up revealed pulmonary, pancreatic and adrenal metastases. The patient died 3 weeks after diagnosis in respiratory distress. Conclusion: Malignant degeneration of plexiform neurofibromas is possible in patients with neurofibromatosis type 1. It is therefore important to monitor these patients for early detection in order to limit the risk of metastasis and death. [ABSTRACT FROM AUTHOR]

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. Samples from patients who underwent surgery between 2010 and 2018 were collected. Intralesional and peripheral (at 2 cm 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 2 cm margins. Secondly a FISH analysis was used as a quantitative diagnostic approach, in order to find the t(17;22) translocation. Among the seven specimens that included 2 cm margins, two samples presented one or more translocations, which were not visible in standard morphology assessments at two centimeters tumor-free margins. 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. [ABSTRACT FROM AUTHOR]

Les pertes de substances de la racine de la cuisse après exérèse carcinologique des sarcomes peuvent être complexes et compromettre l'arbre vasculaire du membre inférieur. Nous rapportons le cas d'une patiente présentant un sarcome récidivant de la cuisse droite. Après de multiples exérèses, le fémur et l'articulation coxo-fémorale sont exposés. Les vaisseaux fémoraux superficiels et profonds ont été pris dans la pièce d'exérèse. La couverture est réalisée par un lambeau de grand dorsal libre musculocutané, anastomosé aux vaisseaux épigastriques inférieurs profonds controlatéraux. Les vaisseaux épigastriques inférieurs profonds ont une longueur suffisante pour rejoindre l'hémi-abdomen controlatéral après dissection avec un calibre permettant des anastomoses microchirurgicales. Ils peuvent servir de vaisseaux receveurs à un lambeau en couverture de la cuisse controlatérale. 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. [ABSTRACT FROM AUTHOR]

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.)

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. [ABSTRACT FROM AUTHOR]

Les sarcomes du stroma endométrial sont des tumeurs rares de la femme jeune. Le diagnostic est posé le plus souvent en post opératoire. Nous Rapportons le cas d´une jeune fille de 22 ans admise dans un tableau de douleurs pelviennes avec métrorragies. L´imagerie a mis en évidence une masse utérine hétérogène. Le diagnostic de fibrome atypique était évoqué. Le diagnostic final est apporté par l´examen anatomopathologique de la pièce opératoire.

Abstract Background Monobloc resection of soft tissue sarcomas (STSs) has a major impact on overall survival and local recurrence. Anatomical boundaries, such as the sciatic notch, increase the risk of fragmentation of the lesion. To date there are few papers describing the optimal surgical technique to remove such STSs. The objective of this study is to describe a simultaneous anterior and posterior approach for resection of sciatic notch dumbbell tumours. Case presentation We present the surgical management of two patients diagnosed with well-differentiated liposarcomas of the sciatic notch with a retroperitoneal and gluteal extension in the two cases. Pre-operative diagnosis was made with a percutaneous biopsy including molecular analysis which demonstrated MDM2 amplification. We describe a simultaneous anterior and posterior approach, including the ligation of the posterior trunk of the internal iliac artery, to reduce intra-operative blood loss and devascularise the tumour. The anterior approach allows the evaluation of the tumour’s retroperitoneal extension, release from its pelvic attachments and control of the surrounding neurovascular structures. During the posterior approach, bleeding is reduced by the devascularisation of the gluteal musculature achieved with internal iliac artery ligation. Clear margins were achieved in both cases. No vascular, skeletal or soft tissue reconstructions were required. Conclusions Simultaneous combined anterior and posterior approaches to remove a malignant sciatic notch tumour optimises the chance of complete en bloc resection. This surgical strategy allows oncologic en bloc resection with minimal blood loss.

Treatment of extremital sarcoma patients may be associated with significant functional disabilities and psychosocial distress affecting return to work (RtW) and quality of life (QoL). In this exploratory study we prospectively investigated the RtW rate, explored biomedical and psychosocial predictors of RtW, and compared generic QoL with Swiss population norms. Forty people (89 %) returned to work. Full-time employment before sarcoma diagnosis, high educational level, and low tumor grade showed an increased probability of RtW. The median age was lower in patients who returned to work, and they reported less fear of progression. Generic QoL (SF-36) was reduced in almost all dimensions when compared to a normative Swiss population. Physical functioning and fear of progression have to be addressed in the rehabilitation process. [ABSTRACT FROM AUTHOR]

Copyright of Pan African Medical Journal is the property of Pan African Medical Journal and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

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. 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. 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. [ABSTRACT FROM AUTHOR]

To evaluate the efficacy and toxicity of hypofractionated radiotherapy in non-metastatic soft tissue and bone sarcomas. 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. Median age was 37 years (range: 11–82 years). Median hypofractionated radiotherapy dose was 35 Gy (range: 20–50 Gy) 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. 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. [ABSTRACT FROM AUTHOR]

Les sarcomes cardiaques primitifs sont des tumeurs malignes rares de pronostic sombre. À travers l'observation d'un jeune patient hospitalisé pour une poussée d'insuffisance cardiaque révélant un sarcome intimal primitif de l'oreillette gauche, nous exposons les principales modalités diagnostiques et thérapeutiques de ces tumeurs ainsi que leur pronostic. 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. [ABSTRACT FROM AUTHOR]

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. 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. This case report illustrates the risk of neoplastic transformation of extramedullary hematopoiesis, and the need for a biopsy when confronted to atypical aspect. [ABSTRACT FROM AUTHOR]

Résumé Objectif de l’étude Les sarcomes mammaires sont des tumeurs hétérogènes dérivées de structures non épithéliales de la glande mammaire. Bien qu’ils représentent une entité rare, leur incidence pourrait augmenter dans les années à venir en raison de l’adoption de l’approche conservatrice dans le traitement du cancer du sein. L’objectif de ce travail était de mettre en exergue l’effet de l’irradiation postopératoire dans le traitement des sarcomes primitifs mammaires, à travers une analyse rétrospective. Matériel et méthodes Cette étude rétrospective a été menée au sein du centre Mohammed-VI de traitement du cancer entre 2004 et 2011. Le taux de survie a été calculé par la méthode Kaplan-Meier. Résultats Quinze dossiers ont été recueillis. L’âge médian était de 41,9 ans. Le traitement chirurgical a été fait chez 93 % des patientes, avec obtention de berges négatives chez 33 %. Une chimiothérapie néoadjuvante a été indiquée chez 46 % des patientes atteintes de tumeur localement évoluée et 66 % des patientes ont reçu une radiothérapie postopératoire devant une résection économique ou insuffisante. Les taux de survie globale et de survie sans rechute à 5 ans n’ont pas été significativement modifiés avec l’utilisation d’une radiothérapie adjuvante. Conclusion Du fait de la rareté de cette entité et vu le manque d’essais randomisés, une prise en charge codifiée, basée sur la preuve, fait encore défaut. Toutefois, une approche multidisciplinaire s’impose incluant une exérèse chirurgicale suivie d’une radiothérapie, selon les caractéristiques de la tumeur. 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 41.9 years. 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. [ABSTRACT FROM AUTHOR]

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.)

Le sarcome de Kaposi classique est une tumeur rare multifocale d'origine des cellules endothéliales vasculaires à caractère évolutif progressif et peu maligne. L'atteinte viscérale dans le sarcome de kaposi est parfois observée chez les patients VIH positif par contre la dissémination tumorale dans les ganglions lymphatiques viscérales dans le SK classique reste très rare. On rapporte un cas rare de sarcome de kaposi classique agressif de la main avec une évolution rapide et destructive.

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.)

Le sarcome fibromyxoïde de bas grade est une tumeur rare des tissus mous. Nous rapportons l'observation d'un patient de 54 ans consultant pour une tumeur du dos du pied droit évoluant depuis 2 ans. L'examen dermatologique notait la présence d'une tumeur bourgeonnante sous cutanée prenant la quasi-totalité du dos du pied droit, d'environ 10 cm de grand axe, douloureuse à la palpation et saignant au moindre contact. Le reste de l'examen clinique était sans particularités. L'étude histologique et immuno-histochimique d'une biopsie cutanée objectivait un processus tumoral d'allure sarcomateuse à cellules fusiformes en faveur d'un sarcome fibromyxoïde de bas grade anti-Vimentine+ anti CD34+ anti PS100- anti EMA- anti-Bcl2-. Le patient a été adressé en chirurgie et a bénéficié d'une amputation de l'avant pied de type Charcot. Le sarcome fibromyxoïde de bas grade a été décrit pour la première fois en 1987 par Evans. Moins de 150 cas sont décrits dans la littérature. Il s'agit d'une tumeur sous-diagnostiquée, souvent confondue avec d'autres entités, principalement localisée dans les tissus mous profonds des régions proximales des membres et du tronc. Histologiquement, cette variété se caractérise par l'alternance de zones myxoïdes et fibreuses constituées de cellules fusiformes. L'immunohistichimie est primordiale pour confirmer le diagnostic. Cette tumeur a un potentiel de récidive locale et de métastases même après plusieurs années, d'où l'intérêt d'un suivi à long terme. Notre observation clinique est particulière par la rareté du sarcome fibromyxoïde de bas grade ainsi que par sa localisation inhabituelle distale au niveau du pied.

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 5 cm, tumor resection during surgery. Results Median age at diagnosis was 66 years (range 46–84 years). Median follow-up was 18 months (range 6–28 months). 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 60 mm (range 30–150 mm). 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. [ABSTRACT FROM AUTHOR]

This year, several studies presented at the ESMO congress may lead to changes in clinical practises. Firstly, immunotherapy may become the first-line treatment for platinum unfit patients with locally advanced or metastatic urothelial carcinoma and the second-line treatment in hepatocellular carcinoma. Moreover, two studies have shown the superiority of centralized treatment of sarcomas and oesophageal compared with non-centralized treatment. Finally, new biomarkers have been presented in prostate cancer, including phosphatase and tension homolog loss as a predictive biomarker for the combination of abiraterone acetate and Akt inhibitor. [ABSTRACT FROM AUTHOR]

Copyright of Journal Africain D'Hépato-Gastroentérologie is the property of Lavoisier and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Sarcomas are rare, and heterogeneous cancers and their management is complex and not optimal for the majority of patients. The three key elements of the management are an accurate diagnosis, a multidisciplinary discussion in a specialized MTB, and a surgery performed in a trained team. In order to significantly improve the management of patients, three pathological and clinical national networks dedicated to sarcoma/GIST/desmoid, and gathering regional expert centers were structured in 2010 with the support of the French National Institute against Cancer (INCa) and the Directorate of Health Care Supply (DGOS) (e.g., the networks RRePS, NetSarc, and ResOs). A shared database has been implemented with the aim to collect different indicators of management quality and to follow their evolution nationwide. Five years later, the three networks cover between 73 and 91% of 4,000 expected new patients yearly. This care network organization facilitates access to pathology and clinical expertise to patients, a better initial management (imaging, biopsy), a better quality of surgery, and a better access to innovative treatments. It also structures research, optimize the use of diagnostic techniques and the instruction for expensive treatments, and standardize the management of data collection and follow-up nationwide. [ABSTRACT FROM AUTHOR]

Interventional radiology plays an increasing role in the management of soft tissue tumors. For initial diagnosis, precise and safe image-guided biopsies; soft tissue tumor biopsies must be performed in accordance with international guidelines. Then, in a therapeutic phase, interventional radiology, particularly with thermal ablation techniques (radiofrequency, cryoablation), can be proposed for specific indications like salvage treatment of recurrent tumor after surgery and radiotherapy, focal ablation for oligometastatic sarcomas, or treatment of benign locally aggressive tumors such as symptomatic hemangiomas or desmoid tumors. A multidisciplinary approach remains mandatory to achieve optimal patient selection for interventional radiology techniques. [ABSTRACT FROM AUTHOR]

Sarcomas are rare types of tumours which present wide variations in their clinical and biological characteristics. In spite of successful locoregional treatment, metastasis will occur in close to 40% of patients. As a rule, doxorubicin is the first-line therapy for patients who have a metastatic soft tissue sarcoma, with very modest results. However, targeting the molecular anomalies which are characteristic of sarcomas could lead to improved patient survival, as has been demonstrated for gastrointestinal stroma tumours and dermatofibrosarcomas. Several clinical trials to evaluate new agents such as cell cycle inhibitors or anti-angiogenic drugs are currently under way. Molecules targeting the epigenetic anomalies of sarcomas are also a promising form of treatment. Finally, the use of nanotechnologies is creating new, original therapies currently under clinical evaluation, particularly in association with radiotherapy. [ABSTRACT FROM AUTHOR]

Soft tissue and bone sarcoma are heterogeneous group of malignancies. They represent a diagnostic challenge due to their rarity, the large number of entities and the ongoing evolution of knowledge for a decade. Moreover, their accurate classification impact over treatment options. Simple and recurrent genetic alterations, such as mutation, amplification, and translocation can be identified in half the sarcomas and serve as new diagnostic markers. In this review we focus on the efficacy of molecular biology on diagnostic pathology of soft tissue and bone tumors in daily routine practice. The role of molecular genetics in the assessment of prognosis and the impact of molecular analysis on the therapeutic decision are also discussed. [ABSTRACT FROM AUTHOR]

Le dermatofibrosarcome (DFS) est une tumeur fibreuse de la peau, de croissance lente, à très haut risque de récidive locale, mais à potentiel métastatique faible. A partir d'une étude rétrospective étalée sur une période de 5 ans (décembre 2008 - décembre 2013), nous avons analysé les caractéristiques épidémiologiques et cliniques, le délai de diagnostic, le type de thérapeutique et le devenir de 32 patients présentant des tumeurs de Darier et Ferrand histologiquement prouvées. Parmi les 32 patients, 10 se sont présentés initialement au service pour une récidive tumorale. Une discrète prédominance masculine a été notée. Les DFS touchent préférentiellement l'adulte jeune. Le délai diagnostique observé est en moyenne de 4 ans. Le tronc est la localisation préférentielle (60 %), suivi par les extrémités proximales (30 %). Les 32 patients ont été traités par exérèse chirurgicale avec une marge de 5cm en surface, emportant en profondeur une barrière anatomique saine. La couverture de la perte de substance (PDS) a été réalisée après confirmation anatomopathologique du caractère carcinologique de l'exérèse, et a fait appel à différents moyens allant de la greffe cutanée aux lambeaux musculo cutanés libres. L'évolution a été marquée par la survenue d'une récidive tumorale chez 8 patients (3 cas parmi les tumeurs vues en première intention et 5 cas parmi les tumeurs vues en récidive) et les résultats ont été jugés satisfaisants sur le plan esthétique et fonctionnel. Le DFS de Darier et Ferrand est une tumeur dont le pronostic et le risque évolutif sont principalement liés au délai diagnostic et la qualité de la première exérèse. Le diagnostic tardif, rend difficile la chirurgie d'exérèse et de reconstruction Les possibilités de guérison en cas de chirurgie primaire bien conduite sont significativement supérieures à celles d'une chirurgie de rattrapage. L'amélioration du pronostic passe par une prise en charge multidisciplinaire précoce et codifiée d'où l'intérêt de la sensibilisation et de l'information du médecin généraliste pour le diagnostic précoce et l'oientation correcte de ces malades vers des centres spécialisés.

L'hémangiopericytome est une tumeur vasculaire rare classée comme sarcome des tissus mous, elle peut être bénigne ou maligne, elle prend naissance au niveau des péricytes qui sont des cellules localisées au niveau de la lame basale de l'endothélium des capillaires ce qui explique son caractère ubiquitaire. Leur traitement de référence est la chirurgie souvent complétée d'une radiothérapie. Nous rapportons un cas d'un énorme hémangiopericytome malin du pied chez une patiente de 28 ans qui nous a été adressé pour une tumeur du pied gauche nécrosée est infectée avec une biopsie qui a été réalisée revenant en faveur d'un hémangiopericytome le bilan d'extension comportant une scintigraphie osseuse et un scanner thoraco-abdomino-pelvien n'a pas objectivé de localisation secondaire Vu la taille de la tumeur et son caractère nécrosé et infectée un traitement chirurgical radical par une amputation transtibiale a été décidé après le consentement de la patiente.

Nous illustrons à travers ce cas l'agressivité des sarcomes utérins et la difficulté de leur prise charge aux stades avancés. Nous rapportons le cas d'une patiente de 58 ans, qui a consulté pour une masse abdomino-pelvienne apparue depuis trois mois et augmentant rapidement de volume. L'examen a trouvé une patiente cachectique, une énorme masse abdomino-pelvienne dure, fixe, mal limitée, arrivant à 7 cm au dessus de l'ombilic. Une échographie et un scanner abdomino-pelvien ont objectivé une volumineuse masse développée au dépens de l'utérus, faisant 29*16 cm (a, b) avec dilatation pyélo-calicielle. Vu l'âge, le fibrome était peu probable et un sarcome utérin a été suspecté. Après réanimation, la patiente a eu une laparotomie médiane xypho-pubienne révélant une énorme tumeur lisse polylobée de couleur chamois, richement vascularisée (c, d), fixée. Une tentative de libération s'est soldée d'une hémorragie cataclysmique nécessitant de la mise sous noradrénaline et des transfusions sanguines. Une exérèse rapide de la tumeur a été réalisée avec une hystérectomie sub-totale compliquées d'une plaie du sigmoïde. On a complété par une intervention de Hartman. Il persistait du résidu tumoral accolé aux anses grêles et au rétropéritoine avec un saignement diffus. Un paking a été réalisé, enlevé le lendemain avec bonne hémostase. La patiente n'a jamais pu être extubée et est malheureusement décédée en réanimation après 15 jours dans un tableau de défaillance multi-viscérale. L'examen histologique a conclu à un léiomyosarcome utérin de 7 kg infiltrant le tissu adipeux et le colon sigmoïde.

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.)

Copyright of Pan African Medical Journal is the property of Pan African Medical Journal and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Résumé Nous rapportons le cas d’une patiente de 55 ans atteinte d’une neurofibromatose de type I ou maladie de Recklinghausen. Elle présentait un schwannosarcome du nerf médian, de haut grade de malignité, à l’union des tiers supérieur et moyen du bras, au contact de l’humérus. Le traitement carcinologique de cette tumeur imposait une amputation du bras au niveau du col chirurgical de l’humérus, et donc une désarticulation d’épaule. Dans le but d’améliorer la fonction résiduelle du membre supérieur, et afin de créer une pince latéro-thoracique, nous avons décidé de réaliser un lambeau libre total d’avant-bras banque. Ce lambeau composite a été composé du radius, de la totalité des muscles de l’avant-bras, ainsi que de la peau de la face antérieure de l’avant-bras, prélevée avec le pédicule radial proximal. Il a été anastomosé en proximal à l’artère axillaire après ostéosynthèse huméro-radiale par plaque. Le lambeau a permis à cette patiente active d’obtenir un moignon fonctionnel, rétablissant la fonction du bras et permettant une action de pince. Summary 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. [ABSTRACT FROM AUTHOR]

Patients with chronic inflammatory diseases (IBD) of the digestive tract are known to have an increased risk of colorectal cancer. These are usually adenocarcinomas, and the occurrence of malignant mesenchymal tumours, particularly leiomyosarcomas, is exceptional. We report one case in a 40-year-old woman, followed for 9 years for ulcerative colitis. The tumour measured 2cm in length and infiltrated the entire rectal wall as far as the subserosa. It was composed of fusiform cells, with 5 mitoses for 10 fields at ×400 magnification, and expressing actin, desmin and caldesmone under immunohistochemical study. We review the 2 cases of leiomyosarcomas associated with Crohn's disease and 3 cases developed during ulcerative colitis published in the literature., (Copyright © 2022 Elsevier Masson SAS. All rights reserved.)

Résumé Objectif Rapporter les aspects épidémiologiques, anatomo-cliniques, pronostiques et thérapeutiques des sarcomes utérins (SU) à partir d’une série rétrospective de 14 cas tunisiens. Patientes et méthodes Notre étude rétrospective a porté sur 14 cas de SU, colligés de 1995 à 2008 dans le service de cancérologie-radiothérapie du CHU Farhat Hached de Sousse. Nous avons analysé les données épidémiologiques, anatomo-cliniques et les résultats thérapeutiques. Le bilan initial avait comporté : anamnèse, examen clinique, données de l’imagerie (radiographie du thorax, échographie abdominopelvienne dans 10 cas et TDM abdominopelvienne dans 7 cas), classification FIGO modifié. Ces patients ont eu un traitement par hystérectomie, radio- et ou chimiothérapie. Résultats Les 14 patientes avaient un âge moyen de 48,5 ans (15 à 78 ans) et n’avaient pas d’antécédent pathologique particulier, notamment irradiation antérieure ou exposition médicamenteuse dans l’enfance. Il s’agissait de leiomyosarcomes (LMS) dans 3 cas, tumeur mûllérienne mixte (TMM) dans 7 cas (50 %) et d’un sarcome du stroma endométrial (SSE) dans 4 cas. Le diagnostic a été posé en postopératoire dans 6 cas et en préopératoire sur biopsie de curetage biopsique dans 8 cas. Les SU étaient classés stade I dans 8 cas, II dans 3 cas et III dans 3 cas. L’hystérectomie a été associée à une annexectomie dans 12 cas et à un curage iliaque bilatéral dans un cas. Huit de nos patientes ont eu en adjuvant une radiothérapie pelvienne et 3 une chimiothérapie. Avec un recul moyen de 54 mois, nous avons observé 4 rechutes locales, des métastases pulmonaires dans un cas et une carcinose péritonéale dans un autre cas. Sept patientes sont vivantes en rémission, 5 sont décédées de progression tumorale et 2 sont perdues de vue. Discussion et conclusion Les SU restent des tumeurs rares, tardivement diagnostiquées et de mauvais pronostic. Un traitement adjuvant par radio et/ou chimiothérapie doit être discuté pour les formes à haut risque et localement avancées. 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. [ABSTRACT FROM AUTHOR]

Copyright of Pan African Medical Journal is the property of Pan African Medical Journal and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Rare pulmonary tumors are defined as lung tumors with unusual histology. Overall, these tumors account for less than 1% of all lung primary tumors, while they correspond to more than 100 different histological, clinical, radiological, and prognostic entities. Rare pulmonary tumors can develop from orthotopic, heterotopic, or hematopoietic tissues. Some rare histological subtypes are specific to the lung, whereas others, rarely occurring within the lung, correspond to tumors more frequent in other organs. Some rare pulmonary tumors have been individualized recently in histopathological classifications. The most frequent rare pulmonary tumors are, in decreasing order of frequency: carcinoids, sclerosing hemangioma, inflammatory myofibroblastic tumors, mucosa-associated lymphoid tissue (MALT) lymphoma, and sarcomatoid carcinoma. These rare tumors are usually characterized, in addition to a low incidence, by the absence of clinical and radiological actualized characterization, by the low number of specialists for each tumor subtype, and by the absence of specific therapeutic data. For patients, rare pulmonary tumors may then hamper equal access to the care. Recognition of rare pulmonary tumors is based on specific clinical and radiological signs suggesting the diagnosis, on a specific diagnostic and imaging strategy, and on the determination of their primary or secondary nature. The establishment of specific standards of care for rare pulmonary tumors requires collaboration to better characterize their clinical and therapeutic features. [ABSTRACT FROM AUTHOR]

Résumé: Objectif de l’étude: Les sarcomes cardiaques primitifs représentent moins de dix cas par an en France. La durée médiane de survie des patients survie est de l’ordre de 18mois. Le traitement repose sur la chirurgie lorsqu’elle est possible. La place de la chimiothérapie et de la radiothérapie est controversée. La stratégie thérapeutique est d’autant plus complexe en ce qui concerne la radiothérapie que la dose limitante au cœur est théoriquement peu compatible avec la prescription d’une dose tumoricide pour un sarcome. L’analyse d’une série récente de 124 cas du groupe sarcome français a suggéré un bénéfice de la radiothérapie sur la survie sans progression. Patients et méthodes: Une analyse de 12 dosimétries a été réalisée. Résultats: Il existe une importante disparité dans la définition des volumes cibles, des doses et les techniques évoluent vers des irradiations plus conformationnelles. Une irradiation apparaissait faisable au vu de la toxicité rapportée (néanmoins probablement sous-estimée en raison un recul court et du pronostic défavorable) et du bénéfice démontré de la radiothérapie pour des sarcomes cardiaques primitifs. Conclusion: Un schéma de 45Gy en 25 fractions dans un volume établi sur l’imagerie préopératoire avec un complément de dose de 14Gy en sept fractions dans une zone à risque ou un reliquat macroscopique, avec des marges de 1cm, pourrait être proposé. Les résultats d’une irradiation conformationnelle avec modulation d’intensité et tomographie conique quotidienne devront être évalués. [ABSTRACT FROM AUTHOR]

Abstract: 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 &y& Elsevier]