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Giant cell tumors of bone (GCTs) are rare mesenchymal tumors classified as intermediate in the WHO 2020 classification, i.e. neither completely benign nor definitely malignant, due to recurrence (frequent) and pulmonary metastases (rare). They involve the end of long bones as well as the axial bones of mature skeletons. They are made of mononuclear stromal tumor cells of (pre-) osteoblastic phenotype, mononuclear cells of the monocyte-macrophage lineage and osteoclast-like multinuclear giant cells responsible for tumor osteolysis. In 95% of cases, the stromal cells have a specific mutation in the H3F3A gene which encodes histone H3.3. The mutated H3.3 G34W protein (90% of cases) can be easily detected by immunohistochemistry, even on small samples. Many tumors or bone pseudotumors contain osteoclast-like giant cells, cells of the bone microenvironment, and should not be confused with GCT: mainly brown tumor of hyperparathyroidism, aneurysmal bone cyst, chondroblastoma, non-ossifying fibroma and central giant cell granuloma.

Cutaneous and vascular manifestations of cancer are numerous. Among paraneoplastic acral vascular syndrome, we report a case of blue toe syndrome as the first manifestation of a prostate cancer following with analysis of this syndrome according literature.A 56-year-old man, with Raynaud's phenomenon of the upper limbs for 2 to 3 years, had 4 blue toes of the left foot evolving for 18 months, without ulceration, the pulses being present. Vascular and cardiac explorations (ultrasound, angio-MRI) were normal. There was no biological or immunological abnormality except an elevated PSA level. Prostate biopsies confirmed diagnosis and abdomino-pelvic CT scan proved the bone and lymph node metastasis.The revelation of a prostate cancer with bone metastases by a blue toe syndrome is a rare situation. In a patient with a blue toe syndrome with no obvious clinical or biological abnormality, especially atheromatous, investigations should include a search for cancer, which can be revealed by blue toes.

Round cell sarcomas represent a diagnostic challenge for pathologists due to the poorly differentiated pattern of these high-grade tumors. Their diagnosis often requires large immunohistochemical panels and the use of molecular pathology. These tumors are largely dominated by Ewing sarcomas, but new families are now well characterized, including in decreasing frequency order in bone, BCOR-altered sarcomas, NFATc2-rearranged sarcomas, mesenchymal chondrosarcomas and more rarely CIC-rearranged sarcomas and myoepithelial tumors. This progress report presents microscopic, immunohistochemical and molecular features of these tumors previously named by the inappropriate term "Ewing-like" sarcomas, in order to enable any pathologist to perceive the morphological features of these sarcomas, to select the immunohistochemical panel that will lead to the diagnosis and to better guide the molecular approach needed to establish the final diagnosis.

Les tumeurs malignes osseuses de l’enfant sont des pathologies rares dont la survie s’est progressivement améliorée au cours des dernières années grâce aux progrès médicamenteux, chirurgicaux et radiothérapeutiques. Nous présentons le cas particulier d’une très jeune enfant atteinte d’un sarcome d’Ewing de la cuisse. Nous discutons de l’influence de ce jeune âge sur la prise en charge à court et à long termes. L’immaturité squelettique et le potentiel de croissance résiduelle des membres doivent être intégrés pour définir l’attitude la plus sûre sur le plan oncologique et la plus fonctionnelle. à cette fin, nous résumons les différentes options reconstructives possibles. Nous décrivons la plastie de rotation de Van Nes qui a été proposée à notre patiente et nous détaillerons les enjeux relatifs au bien-être fonctionnel et à l’image de soi. [Influence of age, growth potential and functionality in the management of bone tumours in children : example of the rotationplasty]. Malignant bone tumours in children are rare diseases whose survival rate has progressively improved in recent years thanks to advances in pharmacology, surgery and radiotherapy. We present the particular case of a very young child with Ewing's sarcoma of the thigh. We discuss the influence of this young age on short and long term care. Limb skeletal immaturity and the residual growth potential need to be integrated to define the safest oncological and functional strategy. For this purpose, we summarize the different possible reconstructive options. We describe the Van Nes rotationplasty that was proposed in our patient's case and we will detail the issues in terms of functional well-being and self-image.

The group of notochordal tumors consists of the benign notochordal cell tumor and the conventional, dedifferentiated and poorly differentiated chordomas in the fifth edition of the WHO classification of bone and soft tissue tumors. This update covers recent advances in the knowledge of the histogenesis and biology of these tumors and their implications in terms of diagnosis, prognosis assessment and therapeutic management.

Bone tissue can be involved by primitive or metastatic tumors and requires a specific processing both at the department of pathology and during multidisciplinary meetings. The development of fine-needle percutaneous biopsies and of molecular techniques in bone tumor pathology requires a specific management. Moreover, decalcification of samples is crucial but can be deleterious if not controlled or not appropriate. The aim of this review is to provide recommendations for management and decalcification of bone tumor samples.

Giant cell tumor is a benign epiphyseo-metaphyseal bone tumor affecting the young patient. It is characterized by an extensive osteolysis, a high potential for recurrence, a risk of malignant transformation and pulmonary metastases. Curettage and cavity filling is the most common treatment, even in the case of a pathological fracture. A wide resection with prosthetic reconstruction must sometimes be considered. Better knowledge of the role of RANK-L in the pathophysiology of these tumors has led to clinical trials involving denosumab. Treatment with denosumab is suggested for inoperable lesions, or for aggressive lesions, in particular of the spine, pelvis, and sacrum before en bloc resection.La tumeur à cellules géantes est une tumeur osseuse bénigne épiphysométaphysaire touchant le sujet jeune. Elle est caractérisée par une ostéolyse parfois extensive, un potentiel de récidive élevé, un risque de transformation maligne et de métastases pulmonaires. Le curetage-comblement est le traitement de choix, y compris en cas de fracture pathologique. Une résection plus large avec reconstruction prothétique doit parfois être envisagée. La meilleure connaissance du rôle de RANK-L dans la physiopathologie de ces tumeurs a conduit à des essais cliniques impliquant le dénosumab. Un traitement par celui-ci est proposé pour les lésions inopérables ou agressives, notamment du rachis, du pelvis et du sacrum, avant une résection en bloc.

Stereotaxic radiotherapy is performed regularly for the irradiation of non-spine bone metastases, but its place is not well understood.This article in stereotaxic radiotherapy of non-spine bones oligometastases presents the current scientific data relating to the indications, to virtual simulation, to the delineation of target volumes, to the total dose and fractionation, to the efficacy and tolerance.Oligometastatic patients are classified into 4 categories: oligorecurrences, oligometastasis, oligopersistence, oligoprogression. The prognosis will be evaluated according to the following characteristics: primary tumor, quantitative characteristics, kinetics, qualitative characteristics. The delineation of GTV includes extensions to the soft tissue and bone marrow with the aid of MRI and PET. The CTV corresponds to a margin of 2 to 5mm and the PTV to a margin of 2mm. The most widely used irradiation schemes are: 1 single fraction of 18 to 24Gy/1 fr; 24Gy/2 fr; 27 to 30Gy/3 fr; 30 to 35Gy/5 fr. Stereotaxis provides 90% local control at 1 year and good pain control. The side effects are not very marked.Stereotaxic radiotherapy is feasible, non-invasive, minimally toxic and effective with good local control and good pain relief. The main issue remains selecting the patients most likely to benefit from it.

Oligometastatic prostate cancer is among the most studied oligometastatic cancers in the literature. However few prospective studies have assessed stereotactic body radiotherapy (SBRT) for prostate cancer oligometastases. Two randomised phase II trials show a progression-free survival benefit compared with observation. Prospective registry data show very good local control and low toxicity too. Inclusion in ongoing trials should be strongly encouraged to define the role of SBRT in addition to systemic therapy. Radiation therapy to the primary tumour has been studied in randomised trials and provides an overall survival benefit in patients with low metastatic burden. The benefit is inversely correlated with the number of bone lesions using conventional imaging, up to three metastases. Radiotherapy to the primary tumour is recommended by the learned societies for patients with low metastatic burden. Its role in combination with second generation anti androgen therapy needs to be clarified.

In the management of prostate cancer in recent years, innovative therapies have appeared requiring precise and reliable disease detection. In 2021, new generation imaging (PET/CT, multiparametric MRI, PET/MRI) have their place at all stages of the prostate cancer natural history to help target the lesion(s) and guide therapy and improve the results obtained. PSMA PET/CT is currently the leader in this type of imaging with a complete offer during the disease: both from diagnosis, to recurrence or in the oligo-metastatic and metastatic stage resistant to castration with a pivotal role in the PSMA theranostic approach. However, multiparametric MRI also has many detection advantages when the prostate is left in place, which suggests the potential major benefit of hybrid PSMA PET/MRI imaging.

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.

Conventional radiotherapy is a pivotal treatment in the management of bone metastasis. It is indicated primarily for palliative, analgesic, or decompressive purposes and in the prevention of severe bone events such as fractures and spinal cord compressions. It should be performed as early as possible from the onset of symptoms or within 14days following a surgical procedure of decompression or bone stabilization. Except in some cases, a pattern of 8Gy single dose is currently recommended, possibly renewable, by being vigilant on associated treatments which some, like antiangiogenics, must be imperatively suspended.

Rotationplasty can be used as an alternative to amputation when conservative treatment is not possible, for example in malignant tumours of the knee. The principle of the operation is to rotate the lower leg through 180 ° after resection of the distal femur and in this way the ankle joint replaces the knee joint. This surgical technique, although complex, allows satisfactory carcinological resection with a functional outcome superior to amputation above the knee as well as a better quality-of-life. Patients do not develop phantom pain, neuroma or stump discomfort because it is not an amputation. In addition, they can actively control their knee and having proprioception and normal contact with the ground. This results in a coordinated, harmonious and elegant gait pattern similar to the normal population. Patients are more efficient in their activities of everyday life and sports. The major disadvantage is the particular aesthetic aspect and the psychological acceptance.La plastie de rotation peut être utilisée comme alternative à l’amputation quand le traitement conservateur n’est pas possible, par exemple dans le cadre des tumeurs malignes au niveau du genou. Le principe de l’opération consiste à faire pivoter la jambe de 180° après résection du fémur distal et, ainsi, l’articulation de la cheville remplace l’articulation du genou. Cette technique chirurgicale, bien que complexe, permet une exérèse carcinologique satisfaisante, avec un résultat fonctionnel supérieur à celui de l’amputation au-dessus du genou, ainsi qu’une meilleure qualité de vie. En effet, les patients ne développent pas de douleurs fantômes, névromes ou gêne du moignon puisqu’il ne s’agit pas d’une amputation. De plus, ils peuvent contrôler activement leur genou et gardent une proprioception et une sensation d’un contact normal avec le sol. Il en résulte un schéma de démarche coordonné, harmonieux et élégant, similaire à la population normale. Les patients sont plus performants dans leurs activités quotidiennes et sportives. L’inconvénient majeur est l’acceptation psychologique de cet aspect esthétique particulier.

Oncological situations represent the majority of palliative situations. Labeling the palliative stage often comes too late in oncology. Pain comes first among discomfort symptoms with the greatest impact on quality of life. We wondered whether the evolutionary stage of the cancer was linked with the prescriptions of opioid analgesics. We observed the prescriptions of strong opioids in patients suffering from metastatic bone cancer 3 months before and after identifying the situation as palliative. This is a cross-sectional observational study performed between January 1, 2012 and December 31, 2016 using data from the French (nationwide claims database). We included 38,399 patients with cancer with at least one metastatic bone location in a palliative situation. Seventeen percent (n=6544) of patients had a prescription of opioid analgesics after palliative care labeling, 19.8 % (n=7606) had a prescription before, 31.1 % (n=11 949) had a prescription before and after and 32.0 % had no prescription of opioid analgesics. An increase in the dosage of opioid analgesics is observed between before and after labeling the stage of the disease as palliative with an average dosage ranging from 99.6 to 142.3mg per day. This study shows that labeling a situation as palliative affects prescriptions of strong opioid analgesics. An early identification of the situation as palliative is essential to provide appropriate care.

Thrombotic Microangiopathies (TM) have been described since the 1960s. They are characterized by presence of mechanical haemolytic anemia associated with peripheral thrombocytopenia. TM in cancer can be related to several causes, whose cancer himself: cancer-related microangiopathic haemolytic anaemia (MAHA). Incidence of cancer related MAHA remains unknown. Cancer-related MAHA are mainly observed in mucin-producer adenocarcinomas, such as gastric (half of reported cases) and breast cancer. We conducted a review of all original published cases of TM reported in breast cancer, and we specifically investigated BC-MAHA cases. A Medline search identified 158 MAHA cases including 118 BC-MAHA, and 40 drug-related MAHA. Most of BC-MAHA occur in disseminated cancers, mainly with medullar involvement, and/or bone metastasis. Patients typically suffer from poor general state, bone pain, and/or dyspnea. Laboratory abnormalities such as myelemia or erythromyelemia in peripheral blood are frequently observed. Incidence of coagulation disorders is increased, compared to other MAHA causes. BC-MAHA prognosis is dramatically poor. Treatments classically used in other MAHA causes, such as plasmapheresis or immunoglobulins, are inefficient. Urgent anti-neoplastic therapy may be the only effective treatment, associated to symptomatic therapies (transfusions, blood pressure control).

Two forms of bone lymphomas can be distinguished: the primary bone lymphoma (PBL) and the secondary bone lymphoma (SBL). PBL is a rare disease with a good prognosis. Clinical manifestations and imaging findings are usually non-specific. Patient can present with pain, swelling of affected bone or pathologic fracture. Positron emission tomography-CT scan is a sensitive imaging modality and very useful for staging, restaging, surveillance of recurrence, and monitoring of treatment response of lymphoma. The diagnosis of PBL is often difficult and made after biopsy examination. Most patients have diffuse large B-cell lymphoma. Patients have been treated with radiotherapy, chemotherapy or combination of both. Localized disease, low IPI (International Prognostic Index) and complete remission after initial treatment were associated with a better outcome. Management of late sequelae deserves particular attention. SBL is more common than PBL; this is a disseminated lymphoma with concomitant involvement of the skeleton. We review the clinical, imaging and pathologic features of bone lymphomas; and discuss therapeutic modalities as well as prognosis of these lymphomas in the era of immunochemotherapy.

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

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

Le VIH, un mal qui ronge jusqu'à l'os. Selon une étude parue en 2018 dans la revue scientifique PNAS, la fragilité osseuse observée chez les patients séropositifs est liée à une suractivité des ostéoclastes. Ces cellules, naturellement responsables de la dégradation de l'os, ont une activité décuplée après infection par le VIH.

Aging of the population results in an increase of the incidence of cancer and bone metastases. The proximal femur is one of the most frequent locations of bone metastases. A pathological fracture has a major impact on the quality of life and potentially on survival. In case of impending fracture, prophylactic fixation is therefore strongly recommended. The management of metastases of the proximal femur depends on multiple parameters, life expectancy and fracture risk being the most important ones. If survival is estimated to be less than 6 weeks, surgery is generally not indicated. Beyond 6 weeks, surgical indication essentially depends on location of the metastases on the proximal femur and the presence of a fracture.L’augmentation de l’espérance de vie s’accompagne d’une croissance de l’incidence des cancers et des métastases osseuses. Le fémur proximal est un des sites les plus fréquents de métastases osseuses. Une fracture pathologique dans cette localisation a un impact majeur sur la qualité de vie et potentiellement sur la survie du patient. Un traitement chirurgical prophylactique est ainsi recommandé en cas de risque de fracture imminente. La prise en charge des métastases fémorales proximales se décide en fonction de multiples paramètres, dont la survie théorique et le risque de fracture sont les plus importants. Si la survie est estimée à moins de 6 semaines, une chirurgie n’est en général pas indiquée. Au-delà, l’indication chirurgicale dépend essentiellement de la localisation des lésions et de la présence d’une fracture associée.

Percutaneous tumor ablation has faced a major growth in recent years with progressive expansion of clinical indications and subsequent adoption by national and international guidelines. Such techniques have become popular due to their minimally invasive profile and precise image-guided targeting resulting into a significant decrease of the procedure-related morbidity. Traditionally, malignant, and to lesser extent benign tumors, have been treated with these new techniques in several different organs including liver, kidney, lung and bone. More recently, other anatomic districts/organs including thyroid, breast, uterus and soft tissues have also been treated. Nevertheless, in order to optimize clinical results, precise knowledge of physical principles underlying these new techniques is of paramount importance, since technical adjustments may be provided according to specific tissue features (e.g. vascularization, density, etc.) and clinical indications of the procedure. Therefore, the goal of this review article is to present all the available ablation techniques with particular regard to their underlying physical principles and most common clinical indications.

Osteoid osteoma is frequent benign tumor, descripted initially by Bergstrand in 1930 followed by Jaffe in 1935. The painful feature of the osteoid osteoma explains the specific consideration by the medical community for this entity. The debate was focused on pathologic and imaging pattern as well as the treatment modalities. Currently, the treatment options are varied and percutaneous treatment is increasingly used. The radiofrequency is widely validated as efficient method without serious adverse and with low rate of recurrence. We hope through this this work to revue the current knowledge of the treatment of osteoid osteoma.L’ostéome ostéoïde est une tumeur osseuse bénigne relativement fréquente initialement décrite par Bergstrand en 1930, puis comme une entité propre grâce aux recherches de Jaffe en 1935. Malgré sa bénignité, elle a concentré toute l’attention des radiologues comme des cliniciens car très symptomatique. Dès lors, l’ostéome ostéoïde a fait l’objet de nombreux débats dans la communauté scientifique en particulier concernant son étiologie, ses caractéristiques pathologiques, son bilan d’imagerie et son traitement. C’est sur cette dernière question que les avancées ont été les plus marquantes et c’est ainsi que les possibilités thérapeutiques bien décrites dans la littérature apparaissent variées. Toutefois, les résultats très favorables des traitements percutanés et en particulier de la radiofréquence, de même que le faible taux de récidives et de complications de ces traitements, ont amené de nombreux pays à les considérer comme le meilleur traitement en première intention. A travers cette revue de la littérature et de notre pratique clinique, nous souhaitons rapporter les connaissances actuelles thérapeutiques en perpétuelle progression grâce au progrès technique.

Due to high dose gradients, stereotactic body radiation therapy requires high precision in the location of the tumour. Uncertainties in the positioning can introduce serious damage on organs at risk and consequently can reduce tumour local control. A better tumour location can be achieved by controlling its position with an efficient inter and intrafraction imaging procedure. The various imaging techniques available on treatment systems are presented and performances are discussed. Finally, propositions are given in terms of imaging system according to the location treated by stereotactic body radiation therapy.

Skeletal-related events (SRE) are common in patients with bone metastatic lung cancer and have a negative impact on quality of life and survival. The objective of this study is to identify predictive factors for SRE occurrence among this population.We conducted a 3-year retrospective study including 100 lung cancer patients with bone metastasis.Eighty-two patients presented at least one SRE (69.5% at baseline). The median occurrence for SRE was 4.5 months and severe bone pain was the most common SRE (56%). The alkaline phosphatase serum level120IU/L (hazard ratio [sHR]=2.8; 95% confidence interval (CI) [1.5-5.4]; P=0.002) and calcemia2.6mmol/L ([sHR]=9.7; 95% CI [5.1-18.4]; P0.001) were identified as risk factors for SRE occurrence while the presence of an initial SRE was associated with a decrease of this risk ([sHR]=0.2; 95% CI [0.1-0.4]; P0.001).The elevated alkaline phosphatase serum level and hypercalcemia are risk factors for SRE occurrence in bone metastatic lung cancer patients and should be used as biomarkers to adapt current medical practice for these patients.

Chordomas are rare malignant tumours, which typically occur in the axial skeleton and skull base. They arise from embryonic remnants of the notochord. They constitute less than 5 % of primary bone tumours. They are characterised by their locally aggressive potential with high frequency of recurrences and a median overall survival of 6 years. The initial therapeutic strategy must be discussed in an expert centre and may involve surgery, preoperative radiotherapy, exclusive radiotherapy or therapeutic abstention. Despite this, more than 50 % of patients will be facing recurrences with few therapeutic options available at this advanced stage. This review aims to outline current treatment options available in chordomas, as well as discussing potentiality of new therapeutic approaches through their molecular characterization and the comprehension of their immunological environment.

Stereotactic radiotherapy of oligometastases, mono- or hypofractionated, represents a fundamental change in the practice of the specialty as it was developed for a century. Despite the great heterogeneity of sites, techniques, and doses, most studies found a high local control rate, around 70 to 90% at 2 years, and reduced toxicity, around 5% of grade 3 at 2 years. Four main phase II and III trials are underway in France. Future research concerns the association of stereotactic radiotherapy with immunotherapy or different conventional chemotherapy protocols, the identification of the best clinical presentations, and optimization of fractionation and biological dose for poor prognosis localizations.

Two types of bone diseases can be observed in patients with breast or prostate cancer: fragility fractures related to osteoporosis, and skeletal related events (SRE) complicating bone metastases. Aromatase inhibitors, ovarian function suppression and tamoxifen use in pre-menopausal women, and androgen deprivation therapy, induce a decrease of bone mineral density and an increase of the incidence of fractures, which can be prevented by inhibitors of bone resorption at low doses. In addition, adjuvant bisphosphonates may be associated with benefits on disease free survival, especially regarding bone recurrences, in postmenopausal women with non-metastatic breast cancer. In the presence of bone metastases, inhibitors of bone resorption at higher doses and frequencies prevent SRE.Deux complications osseuses peuvent être observées en cas de cancer du sein ou de la prostate : les fractures ostéoporotiques et les événements squelettiques osseux compliquant les métastases osseuses. Les inhibiteurs de l’aromatase, la suppression de la fonction ovarienne et le tamoxifène en préménopause, et la déprivation androgénique induisent une diminution de la densité minérale osseuse et une augmentation de l’incidence des fractures, qui peuvent être prévenues par les inhibiteurs de la résorption osseuse (IRO) à faibles doses. Par ailleurs, les bisphosphonates pourraient diminuer le risque de récidives, en particulier osseuses, chez les patientes ménopausées avec cancer du sein non métastatique. En cas de métastases osseuses, les IRO à doses et fréquences plus importantes préviennent les événements squelettiques osseux.

The primary objective was to determine in our department the progression-free survival rate of patients with solitary bone plasmocytoma and secondarily to evaluate its diagnostic, therapeutic and evolutionary aspects.This is a retrospective review of 12 patients monitored and treated in the radiotherapy department of the Mohammed-V military medical teaching hospital in Rabat for a solitary bone plasmocytoma between January 2012 and December 2018. The average age of our patients were 53.8 years old (range: 31-72 years old). Pain was the most common telltale sign. The site of the lesions was spinal in four cases, iliac in four cases, mandibular, ribal, humeral and at the level of the astragalus in one case respectively. All patients received radiotherapy. This irradiation was delivered alone in 60% of cases or associated with surgery in 40% of cases. The average dose of radiotherapy was 47.3Gy (range: 45 to 50.4Gy) and this was delivered by a modulated volumetric arc therapy technique in ten patients and conformal tridimensional radiotherapy in two patients.Local control, defined by stability or radiological regression, was obtained in ten patients and four patients progressed to multiple myeloma, two of whom died. The average duration of follow-up was 51 months.Radiation therapy is the standard treatment for solitary bone plasmocytoma. It ensures good local control in 90% of cases. The prognosis is affected by progression to multiple myeloma, which justifies rigorous monitoring after treatment and suggests a reflection on the exact place of chemotherapy.

Cervical cancer is the twelfth most frequent cancer in women in France. Glassy cell carcinoma is a rare histological entity, rapidly aggressive, associated with a poor prognosis.A 30-year-old woman was admitted in an internal medicine department for polyarthralgia with high grade fever, evolving for 3 weeks. There was an inflammatory syndrome. The 18-FDG-PET-scan showed inflammatory lymph nodes as well as disseminated osteolytic lesions, and a primitive pelvic tumor. A 3cm tumor of the cervix was found during the gynaecologic examination. Histological analysis elicited a high-index mitotic carcinoma, glassy cell carcinoma type. Despite chemotherapy, the outcome was poor, with early death occurring after three months of follow-up.The glassy cell carcinoma of the cervix should be considered as an aetiology of bone metastases in young female patients.

Osteosarcoma is the most common malignant bone tumor before 25 years of age. Response to neoadjuvant chemotherapy determines continuation of treatment and is also a powerful prognostic factor. There are currently no reliable ways to evaluate it early. The aim is to develop a method to predict the chemotherapy response using radiomics from pre-treatment MRI.Clinical characteristics and MRI of patients treated for local or metastatic osteosarcoma were collected retrospectively in the Rhône-Alpes region, from 2007 to 2016. On initial MRI exams, each tumor was segmented by expert radiologist and 87 radiomic features were extracted automatically. Univariate analysis was performed to assess each feature's association with histological response following neoadjuvante chemotherapy. To distinguish good histological responder from poor, we built predictive models based on support vector machines. Their classification performance was assessed with the area under operating characteristic curve receiver (AUROC) from test data.The analysis focused on the MRIs of 69 patients, 55.1% (38/69) of whom were good histological responders. The model obtained by support vector machines from initial MRI radiomic data had an AUROC of 0.98, a sensitivity of 100% (IC 95% [100%-100%]) and specificity of 86% (IC 95% [59.7%-111%]).Radiomic based on MRI data would predict the chemotherapy response before treatment initiation, in patients treated for osteosarcoma.

Cartilaginous tumours are rare and often incidentally discovered on imaging. They can also be responsible for osteoarticular symptoms (pain, joint stiffness, deformity or fracture) and will require a clinical, radiological and a histopathological assessment. Depending on the lesion, the management may consist of observation or a complex surgical procedure that will take place after a multidisciplinary discussion including the radiologist, the pathologist and the orthopaedic surgeon. The discovery of such cartilage tumours often induces a significant dose of anxiety in the affected patients. A better knowledge of these lesions by the family practitioner and the orthopaedic surgeon therefore seems very useful to provide optimal care before referring the patient to a specialized centre.Les tumeurs cartilagineuses sont des pathologies rares, dont la découverte est souvent fortuite sur une imagerie réalisée pour une autre raison. Parfois symptomatiques (douleur, raideur articulaire, déformation, fracture), elles doivent motiver une exploration clinique, radiologique, et histopathologique. La prise en charge dépend des lésions identifiées et peut comporter un simple suivi clinique ou un geste chirurgical complexe, fruit d’une discussion menée par une équipe multidisciplinaire composée de radiologues, pathologues et chirurgiens. La découverte d’une lésion cartilagineuse induit souvent une dose d’inquiétude non négligeable chez les patients atteints. Leur connaissance, par le médecin traitant et le chirurgien orthopédiste, nous semble indispensable afin de proposer une prise en charge optimale qui devra se faire dans un centre spécialisé.

Since decades, stereotactic radiotherapy has spread out worldwide. Published results are very numerous. To clarify obviousness among all the publications, this recommendation review was written. Voluntarily, authors limited analysis of international best evidence literature on malignant tumors of lung, liver, prostate, head and neck, and metastasis of bone and brain. These data could be used to advance standardization and quality improvement of treatments performed in the nationwide radiotherapy departments and can provide useful guidance for centers worldwide.

Haemangiomas are vascular malformations, frequently cutaneous, hepatic and splenic. Respiratory involvement and multiple localisations are uncommon.We present a rare case of multiple cavernous haemangiomas in a 35 year old woman presenting with repeated haemoptysis. Thoracic CT scanning showed a mass in the left lower lobe associated with lymph node enlargement above and below the diaphragm, heterogeneous splenomegaly and a single spinal lesion without hypermetabolism on PET scanning. Enbronchial ultrasound-guided trans-bronchial needle aspiration was not contributory. Histopathological diagnostic was made firstly by splenectomy with lumbar-aortic curettage and then by lobectomy for haemostasis. A final diagnosis of multiple cavernous haemangiomas involving lung, lymph nodes, spleen and bone was made.Bronchopulmonary cavernous haemangiomas associated with extra-thoracic lesions are exceptionally rare and their presentation, suggesting, a malignant cause, often leads to surgical resection for diagnostic and, eventually, therapeutic management. We report an original case of cavernous haemangiomas involving lung, lymph nodes, spleen and bone.

Nearly 75% of patients with metastatic melanoma develop brain metastases. We here report the case of an 83 year-old woman hospitalized for secondarily generalized clonic seizures of the left leg with partial convulsive seizures in the Resuscitation Department. Melanoma resection of the left ankle had been performed 6 months before her admission. Neurological examination showed left ataxic crural monoparesis. Electro-encephalogram showed central and right frontal focus with left-sided dissemination. Gadolinium-enhanced magnetic resonance imaging (MRI) of the brain showed multiple supratentorial and subtentorial encephalic lesions with varying size and shape, with T1 hypersignal (A and A'), haemorrhage on T2*-weighted sequences (B and B'), gadolinium-enhancing T1 with perilesional edema on Flair sequences. Positron emission tomography (PET) showed multiple lymph node and bone metastases. Lymph node biopsy was negative for VE1 antibody with no BRAFV600E mutation by immunohistochemistry. An increase in the number of metastatic lesions was observed during control brain CT scan despite 10 brain radiotherapy sessions motivating palliative care. Epileptic seizures were controlled with levetiracetam. In patient with multiple hemorrhagic and spontaneous brain lesions, it is essential to obtain informations on patient's history of melanoma and to perform a thorough dermatologic examination in order to investigate its cause and to establish adequate therapeutic treatment.

To assess pain prevalence, its features and its management in a radiotherapy department of a French public general hospital. To highlight strategies to improve pain screening and treatment.Designed in conjunction with pain management specialists, a cross-sectional study on pain was carried out. All patients treated in the department being interviewed with a standardised questionnaire during 2 days.Among 91 patients, 63.7% reported pain in daily life. They respectively represented 100%, 85.7% and 83.3% all of the patients treated for brain tumours, for bone metastasis and for head and neck cancers. Only 7.7% of patients reported pain during radiotherapy sessions. Among patients reporting pain, 70.7% received pain relief treatment and 60.8% of them thought this was adequate. While 51.6% of patients knew there was a specialist pain unit in the hospital, only 5.5% were offered a consultation with it. This unit provides non-pharmacological pain management techniques.This study confirms the importance, the underestimation and undertreatment, of pain management in radiotherapy departments. We recommend using a standardised questionnaire to identify patients at highest risk of pain, and the use of specialised pain relief teams when needed. A radiation therapist could act as a referrer to the pain relief team. Pain management remains teamwork, with links to specialised units.

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

Les méningiomes intra osseux primitifs sont des méningiomes ectopiques rares, qui constituent 1 à 2% de tous les méningiomes. Le but de ce travail est d'illustrer les différents aspects à l'imagerie contribuant à une approche diagnostique et de discuter leurs différents diagnostics différentiels. Nous rapportons, à ce propos, deux cas de méningiomes intra osseux à localisation ptérionale, documentés par TDM et IRM.