[Borderlines and malignant phyllodes tumors of the breast: From the anatomopathological challenge to the standard of care]. / Tumeurs phyllodes borderlines et malignes du sein : du défi anatomopathologique à l'élaboration d'un standard de prise en charge.

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+).

[Fatal outcome of complete resection of undifferentiated pleomorphic cardiac sarcoma : About a case]. / Évolution fatale d'une résection complète d'un sarcome cardiaque pléomorphe indifférencié : à propos d'un cas.

INTRODUCTION: Undifferentiated pleomorphic sarcoma (UPS) is a very rare malignant primary cardiac tumor with a poor prognosis. OBSERVATION: A 34-year-old woman complained of dyspnea evolving in a febrile context with altered general condition: Transthoracic echocardiography (TTE) revealed a left intra-atrial mass measuring 51/26 mm in diameter. A thoracic computed tomography (CT) revealed a mass occupying the left atrium measuring 42/28/34 mm, associated with two pulmonary metastases. The cardiac mass was resected and the postoperative suites were fatal. Histological diagnosis was UPS. CONCLUSION: We present an atypical case of invasive UPS, which was surgically resected and scheduled for radio-chemotherapy with a fatal outcome.

Case report: Hybrid reconstruction of quadriceps function after sarcoma resection using a reinnervated free flap and tendon transfer.

We describe a case of a leiomyosarcoma of the thigh, the resection of all the anterior muscular compartment, and the reanimation of knee extension, using a latissimus dorsi (LD) free flap and tendon transfer. Surgical technique and postoperative care management are described. Functional results, neuropathic pain, and range of motion (ROM) were assessed at 3 months and 12 months after discharge. A complete excision (R0) was carried out and rapid wound healing was obtained despite developing a seroma infection. The patient was able to walk without technical support nor limping at 3 months post-surgery. The patient was still in remission at 12 months follow-up, with Medical Research Council (MRC) scale assessed at 4/5 and ROM rated at 5-105°. In case of total quadriceps resection, knee extension reconstruction can be obtained with tendon transfers and reinnervated free muscular flaps. Combining these techniques could be a good strategy for rapid recovery, with optimal scarring and tissue coverage.

Ewing's sarcoma of the parotid gland: A rare entity with review of the literature.

Background: Ewing's sarcoma is a rare malignant entity. Extraosseous Ewing's sarcoma (EES) of the head and neck region is a rare occurrence, and Ewing's sarcoma of the parotid gland is even rarer. To the best of our knowledge, we reported the first case of extraskeletal ES originating from the parotid gland in the Tunisian literature. Case report: We report a rare case of EES of the parotid gland in a 35-year-old female. She presented with left parotid tumefaction. Physical examination revealed solid and fixed mass associated with facial paralysis. Magnetic resonance imaging illustrated a left intra-parotid process occupying the entire gland measuring 42 mm infiltrating the masseter and pterygoid muscles. The patient had a total left parotidectomy with ipsilateral triangular lymph node dissection. The definitive pathological examination and the immunohistochemical staining confirmed a primary peripheral neuroectodermal tumor or PNET with the presence of a specific EWING/PNET-type translocation in 60% of the tumor cells. She had an adjuvant chemotherapy (four cycles of vincristine, doxorubicin, cyclophosphamide alternating with ifosfamide and etoposide) followed by external radiotherapy. Conclusion: A clinical and radiological follow-up by cervical MRI was done every 3 months and The 10-month follow-up showed no locoregional and distant recurrence.

Introduction: Le sarcome d'Ewing est une entité maligne rare. La localisation extra osseuse en particulier la région de la tête et du cou est caractérisée par son agressivité locorégionale. Nous rapportons le cas d'un sarcome d'Ewing de la parotide. A notre connaissance, il s'agit du premier cas rapporté dans la littérature tunisienne. Presentation du cas: Il s'agit d'une femme âgée de 35 ans qui a consulté initialement pour une tuméfaction au niveau de la glande parotide gauche. L'examen clinique a révélé une masse sous angulomandibulaire associée à une paralysie faciale périphérique gauche. Une IRM parotidienne a objectivé un processus intra-parotidien gauche occupant l'entièreté de la glande mesurant 42 mm mal limitée infiltrant modérément le muscle masséter et ptérygoïdiens. Le bilan d'extension était sans anomalie. La patiente a eu une parotidectomie gauche large avec un curage triangulaire homolatéral. L'examen anatomopathologique définitif et l'étude par hybridation in situ en fluorescence (FISH) ont confirmé la présence d'une translocation spécifique type EWING/PNET dans 60% des cellules tumorales. La patiente a été traitée par une chimiothérapie type VDC/IE (vincristine, doxorubicine, cyclophosphamide en alternance avec ifosfamide, et etoposide) suivie d'une radiothérapie externe. Un suivi clinique et radiologique trimestrielle n'a montré aucune récidive locorégionale ni à distance à ce jour soit à 10 mois de recul. Conclusion: Une surveillance clinique et radiologique trimestrielle a été faite et le contrôle à 10 mois n'a pas montré de récidive locorégionale ou à distance.

[Renal synovialosarcoma: What about pyelic cytology to make a diagnosis?] / Synovialosarcome primitif du rein : si tout était déjà dans les urines pyéliques ?

Synovialosarcoma is a malignant mesenchymal tumor of young adults that occurs in the deep soft tissues, particularly around large joints. When it occurs in more unusual sites, it could present a significant diagnostic challenge. In this case, a 19-year-old girl was treated for a pyloric mass. A pyelic urine cytology performed simultaneously with a pyloric biopsy proved to be a significant element of orientation and perfectly concordant with the histopathological aspect of the pyelic mass after nephrectomy. We report here the first case of renal synovialosarcoma documented in pyelic urine.

[Translocated sinonasal tumors]. / Tumeurs nasosinusiennes à translocation.

In recent years, several nasal cavity and sinus entities have been described with fusion genes. Salivary gland tumors with fusion genes will not be discussed in this article, but it should be kept in mind that accessory salivary glands are present in the nasal cavity and sinuses and can therefore lead to tumoral lesions. Entities with specific or more frequently described rearrangements in the nasal cavities and sinuses are DEK::AFF2 squamous cell carcinomas,;non-intestinal and non-salivary nasosinusal adenocarcinomas, some of which displaying ETV6 gene rearrangements; biphenotypic nasosinusal sarcomas, most of which displaying PAX3 gene rearrangements; and Ewing's adamantinoma-like sarcomas, which display the same rearrangements as conventional Ewing's sarcomas, mainly the EWSR1::FLI1 rearrangement. Each entity will be described morphologically, immunohistochemically, and prognostically.

Novel role of fluorescent in situ hybridization technique (FISH) in recommended surgical margins of dermatofibrosarcoma protuberans: A preliminary study.

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.

[Diagnosis of uterine sarcomas and rare uterine mesenchymal tumours with malignant potential. Guidelines of the French Sarcoma Group and Rare Gynaecological Tumours]. / Diagnostic des sarcomes utérins et tumeurs mésenchymateuses utérines rares à potentiel de malignité. Référentiels du Groupe Sarcome Français et des Tumeurs Rares Gynécologiques.

The landscape of uterine sarcomas is becoming more complex with the description of new entities associated with recurrent driver molecular alterations. Uterine sarcomas, in analogy with soft tissue sarcomas, are distinguished into complex genomic and simple genomic sarcomas. Leiomyosarcomas and undifferentiated uterine sarcomas belong to complex genomic sarcomas group. Low-grade and high-grade endometrial stromal sarcomas, other rare tumors associated with fusion transcripts (such as NTRK, PDGFB, ALK, RET ROS1) and SMARCA4-deficient uterine sarcoma are considered simple genomic sarcomas. The most common uterine sarcoma are first leiomyosarcoma and secondly endometrial stromal sarcomas. Three different histological subtypes of leiomyosarcoma (fusiform, myxoid, epithelioid) are identified, myxoid and epithelioid leiomyosarcoma being more aggressive than fusiform leiomyosarcoma. The distinction between low-grade and high-grade endometrial stromal sarcoma is primarily morphological and immunohistochemical and the detection of fusion transcripts can help the diagnosis. Uterine PEComa is a rare tumor, which is distinguished into borderline and malignant, according to a risk assessment algorithm. Embryonal rhabdomyosarcoma of the uterine cervix is more common in children but can also occur in adult women. Embryonal rhabdomyosarcoma of the uterine cervix is almost always DICER1 mutated, unlike that of the vagina which is wild-type DICER1, and adenosarcoma which can be DICER1 mutated but with less frequency. Among the emerging entities, sarcomas associated with fusion transcripts involving the NTRK, ALK, PDGFB genes benefit from targeted therapy. The integration of molecular data with histology and clinical data allows better identification of uterine sarcomas in order to better treat them.

[Predictive factors assessment of pathological response to neoadjuvant radiotherapy of soft tissue sarcomas]. / Évaluation de facteurs prédictifs de réponse pathologique à la radiothérapie néoadjuvante des sarcomes des tissus mous.

PURPOSE: Conserving surgery combined with radiotherapy in presence of local recurrence risk factors is standard treatment of soft tissue sarcomas, a group of rare and heterogeneous tumours. Radiotherapy is performed before or after surgery. In neoadjuvant setting, late radiation-induced toxicity is reduced and pathological response to radiotherapy could be achieved. A complete pathological response to radiotherapy has recently been shown to predict better survival. Our study aims at identifying predictive factors of pathological response to neoadjuvant radiotherapy (clinical, radiological or histological) of soft tissue sarcomas. PATIENTS AND METHODS: Clinical, imaging (MRI: perilesional oedema, necrosis, tumour heterogeneity, vasculonervous relationships) and pathological (pathological subtype, tumour grade, anticipated/obtained resection quality) data were retrospectively collected. Tumour response (imaging and pathological), patient outcome, acute and late radiation-induced toxicity, predictive factors of pathological response to neoadjuvant radiotherapy were studied. The 2-test or exact-Fisher test (qualitative variables) and by Student's t-test or Kruskal-Wallis test (quantitative variables) were used for statistical analysis. RESULTS: From April 2017 to April 2021, neoadjuvant radiotherapy (50Gy in 25 fractions) followed by surgical excision was performed to 36 consecutive patients with liposarcomas (n=17/36), or undifferentiated sarcomas (n=8/36). MRI response was complete in 1 patient, partial in 9 patients (n=9/36, 25%), stable in 21 patients (n=21/36, 58%) or in progression in 5 patients (n=5/36, 14%). Pathological response was observed in 22 patients (61%). No grade 3-4 acute radiation-induced toxicity was observed. Regarding late toxicity, 28% of patients had grade 1-2 oedema (n=10/36), 39% had a grade 1 fibrosis (n=14/36), and 30% grade 1 pain (n=11/36). No predictive factors of response to radiotherapy was statistically significant. CONCLUSIONS: Neoadjuvant radiotherapy is well-tolerated. No clinical, radiological or pathological predictive factors was identified for radiotherapy tumour response.

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.

[Contribution of whole-body MRI to the initial assessment of myxoid liposarcoma]. / Apport de l'IRM corps entier au bilan initial du liposarcome myxoïde.

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.

[Rare cancers of the head and neck on behalf of the REFCOR, part 2]. / Cancers rares de la tête et du cou sous l'égide du REFCOR, partie 2.

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.

[Uterin adenosarcoma: French Guidelines of the French Sarcoma Group and the Rare Gynecologic Tumor Group]. / Adénosarcomes mullériens de l'utérus ­ référentiels de prise en charge du GSF-GETO/NETSARC+ et du groupe TMRG.

Uterine adenosarcoma is a very rare malignancy defined as a biphasic tumor composed of both benign epithelial component and a malignant sarcoma component. The stage of the disease is determined by the presence of myometrial invasion and the extent of extra-uterine disease. The most important histopronostic factors are the existence of a sarcomatous overgrowth defined by a sarcomatous contingent occupying more than 25 % of the volume of the tumor (directly correlated to the grade of the disease), the presence of a heterologous and/or a high-grade component. Stage I adenosarcomas without sarcomatous overgrowth have a good prognosis, with an overall 5-year survival of up to 80 %. In localized disease, complete surgical removal is recommended. The role of hormone therapy, chemotherapy and adjuvant radiotherapy is not established. If possible, relapses should be re-treated surgically, with the aim of achieving complete resection. In the advanced inoperable or metastatic setting, hormone therapy is an option for low-grade adenosarcomas with estrogen receptor (ER) and progesterone receptor (PR) overexpression. For high-grade tumors, the standard chemotherapies are doxorubicin-based combinations, although an integrated approach of surgery and medical treatment should also be considered in this setting.

[Uterin sarcoma, high-grade stroma, indifferenciated, referential]. / Sarcomes utérins du stroma de haut grade et sarcomes indifférenciés ­ Référentiels de prise en charge du Groupe Sarcome Français et du Groupe des Tumeurs Rares Gynécologiques.

High-grade endometrial stromal sarcoma (HGESS) and uterine undifferentiated sarcoma (UUS) are rare uterine malignancies arising from mesenchymal endometrial cells. They are characterized by aggressive behavior and poor prognosis. Median age of diagnostic is 55years. The most common symptoms are vaginal bleeding, abdominal pain, and pelvic mass. Approximately 65 % are diagnosed witch advance disease stage III or IV according to the International Federation of Gynecology and Obstetrics classification. Median overall survival is around 20months. The management of the disease must be discussed in multidisciplinary staff meetings. The standard management of HGESS and UUS is total hysterectomy with bilateral oophorectomy. Systematic lymphadenectomy is not recommended. Adjuvant therapies, such as chemotherapy and radiotherapy must be discussed. In case of oligo-metastasic disease, surgery of the primary tumor and metastasis must be discussed and if not operable the standard management is doxorubine-based chemotherapy.

[Uterine leiomyosarcoma - French guidelines from the GSF/NETSARC and TMRG groups]. / Léiomyosarcomes utérins ­ Référentiel de prise en charge du GSF-GETO/NETSARC+ et du groupe TMRG.

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.

[Rare tumors: Angiosarcoma]. / Tumeurs rares : angiosarcome.

Angiosarcomas are a rare subtype representing 1-2% of soft tissue sarcomas. Risk factors are rarely elucidated but radiotherapy and lymphedema are the most common ones, usually following local treatment for local breast cancer. Despite the improvement of our knowledge, the prognosis remains poor with 35-40% of 5 year-overall survival. Local treatment when feasible should include a R0 surgery completed with adjuvant radiation. When metastatic, front lines chemotherapies include doxorubicine or weekly paclitaxel. If possible, in oligometastatic patients, metastasectomy should always be considered allowing the best responses. The knowledge of angiosarcoma's biology is rapidly increasing and new biomarkers are emerging. The use of immunotherapy in particular subtypes including head and neck angiosarcomas shows promising results. The model of the angiosarcoma project, a patient-participating study, seems to be an excellent way to study rare tumors. We should focus our efforts on understanding the underlying molecular biology to propose the best precision medicine for those patients.

[Endometrial stromal sarcoma: French Guidelines from the French Sarcoma Group and the Rare Malignant Gynecologic Tumors Group]. / Sarcomes du stroma endométrial de bas grade : référentiels de prise en charge du GSF-GETO/NETSARC+ et du groupe TMRG.

Low-grade endometrial stromal sarcoma (LG-ESS) accounts for approximately 15% of all uterine sarcomas. Median age of patients is around 50 years and half of the patients are premenopausal. In all, 60% of cases present with FIGO stage I disease. Preoperatively radiologic findings of ESS are not specific. Pathological diagnosis remains essential. This review aimed to present the French guidelines for low grade ESS treatment within the Groupe sarcome français - Groupe d'étude des tumeurs osseuse (GSF-GETO)/NETSARC+ and tumeur maligne rare gynécologique (TMRG) networks. Treatments should be validated in multidisciplinary team involved in sarcomas or rare gynecologic tumors. Hysterectomy is the cornerstone of treatment for localized ESS, and morcellation should be avoided. Systematic lymphadenectomy in ESS does not improve the outcome and is not recommended. Leaving the ovaries in situ in stage I tumors could be discussed for young women. Adjuvant hormonal treatment could be considered, for two years for stage I with morcellation or stage II and livelong for stages III or IV. Nevertheless, several questions remain, such as optimal doses, regimens (progestins or aromatase inhibitors) and duration of therapy. Tamoxifen is contraindicated. Secondary cytoreductive surgery if feasible for recurrent disease, appears to be an acceptable approach. Systemic treatment for recurrent or metastatic disease is mainly hormonal, with or without surgery.

[Rectal leiomyosarcoma, a rare malignant tumor diagnosed in ulcerative colitis]. / Le léiomyosarcome rectal, une tumeur maligne rare diagnostiquée au cours de la rectocolite ulcéro-hémorragique.

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.

Preoperative ultra-hypofractionation radiotherapy in extremity/trunk wall soft tissue sarcoma - A meta-analysis of prospective studies.

PURPOSE: The neoadjuvant radiotherapy is now standard treatment in soft tissue sarcoma. Using ultra-hypofractionation radiotherapy shorten the treatment time. In the era of COVID pandemic, using less fraction to treat patient is an urgent need. Thus, we aim to use meta-analysis to investigate the clinical efficacy of preoperative stereotactic body radiotherapy. MATERIAL AND METHODS: PRISMA guideline was used in this study. PubMed, Cochrane and Embase were used. We include only prospective study. The main endpoint was set as wound complication rate. Other endpoints include R0 resection rate, overall survival, local control, and distant metastasis free survival. RESULTS: Seven studies were included. The pooled wound complication rate is 0.30 (95% CI=0.26-0.35). The pooled R0 resection rate is 0.87(95%CI: 0.74-0.94). The pooled 2-year overall survival is 0.86 (95%CI: 0.72-0.94). The pooled 2-year local control rate is 0.96(95%CI: 0.89-0.99). The pooled 2-year distant metastasis free survival is 0.60 (95%CI=0.50-0.70). CONCLUSION: Neoadjuvant ultra-hypofractionation radiotherapy in soft tissue sarcoma is a feasible and well tolerable treatment.

An institutional review of treatment outcomes in extraosseous Ewing's sarcoma- the largest Asian experience.

PURPOSE: To analyze the treatment strategies, outcomes and factors impacting these outcomes in extraosseous ewings sarcoma (EOES). MATERIAL AND METHODS: A search of the hospital database yielded a total of 109 EOES patients registered in last 10 years out of which 25 patients were excluded from analysis due to incomplete medical records. Demographic and clinical characteristics were reported using descriptive statistics. Overall survival (OS) was taken from the time of diagnosis to death. Patients who were alive or lost to follow up were censored from the survival analysis. A total of 12 clinical and treatment related variables were taken into univariate analysis and those showing significance or a trend towards significance were taken up for multivariate analysis. Further a cluster analysis was done in a quest to find a subgroup which would have a better survival outcome as compared to other clusters. SPSS version 23 was used for statistical analysis. RESULTS: Chest wall (n=26), lower extremity (n=22) and paraspinal area (n=14) were the common sites involved. Localised swelling (n=43) was the most common presenting symptom and the median time to presentation was 2 months. Overall survival of the entire cohort at 5 years was 52 percent. Stage at presentation had a significant impact (P value<0.001) on estimated median OS (localised 70 months versus 36 months in metastatic stage). Cluster analysis showed that, patients with localised stage at presentation, good response to chemotherapy, negative resection margin and no adjuvant RT had a median survival of 69 months. CONCLUSION: Judicious use of trimodality treatment in EOES yields optimal results and it also adds significantly onto the scarce literature on this subtype of sarcoma.