During decades, first-line treatment of advanced cervical cancer solely consisted of platinum-based chemotherapy, associated with bevacizumab whenever possible. Since 2022, immunotherapy is part of standard therapeutic strategy with pembrolizumab on the one hand, associated with chemotherapy and bevacizumab in patients with PD-L1 positive tumors (CPS≥1), and cemiplimab on the other hand, in patients who did not receive prior immunotherapy and progress after first line regardless of PD-L1 expression. Pretherapeutic work-up includes CT of the chest, abdomen and pelvis potentially associated with 18F-FDG PET-CT and MRI in case of relapse, as well as evaluation of PD-L1 status on tumor and immune cells to define the CPS score that will determine eligibility to pembrolizumab treatment (CPS≥1). Whenever possible, molecular screening and determination of HER2 status may allow orienting patients to clinical trials. Indeed, inclusion in investigational studies must be systematically considered and early supportive care is always recommended.

The evolution of serous high grade ovarian cancer management is characterized by a more regulated patients' journey on the one hand and the development of new therapeutic options on the other hand, the selection of which is guided by tumor molecular characteristics. Surgery remains the cornerstone of treatment. It can be performed only in authorized expert sites that can demonstrate sufficient experience from highly skilled surgical teams, and quality criteria including prehabilitation and rehabilitation programs. The diagnostic step is crucial; it comprises multiple biopsies that allow reliable pathological and molecular analyses, and a comprehensive surgical staging. Determination of BRCA1/2 mutation and homologous recombination deficiency statuses by validated methods guide maintenance therapy at advanced stages and referring to oncogenetic consultation if appropriate. For these advanced diseases, the two main questions for surgical strategy are the feasibility of complete resection (without residual disease, CC-0), assessed during surgical exploration of pelvis and abdomen, and the optimal timing of this surgery (upfront or after neoadjuvant chemotherapy). In recurrent diseases, surgery remains a main piece of treatment in case of late relapse and medical treatment depends on drugs used in the first line; in early platinum resistant relapse, a new therapeutic option is available with mirvétuximab soravtansine.

Histomolecular diagnosis of endometrial cancer systematically includes the evaluation of hormonal receptors, P53 and MMR statutes (determination of PD-L1 and HRD statutes is not required). Therapeutic progress in advanced endometrial cancer is mainly related to the first-line utilization of immunotherapy associated with chemotherapy, a strategy assessed in five randomized controlled trials, although at the moment, only dostarlimab is available in France. Immunotherapy administration requires specific pretherapeutic workup and monitoring. Hormone therapy remains an option in non-aggressive, low grade endometrioid cancer, expressing hormone receptors. Treatment choice is based on clinical situation (upfront metastatic disease or relapse after adjuvant therapy, and duration of platinum-free interval in case of adjuvant therapy), disease aggressivity, molecular status (in particular, MMR status) and patients' comorbidities. PARP inhibitors are not recommended as maintenance therapy. In second line, the combination of pembrolizumab and lenvatinib is the standard treatment if chemoimmunotherapy has not been used previously. If it has been, therapeutic strategy depends on the duration of platinum-free interval. Inclusion in a clinical trial should always be considered when the patient's performance status makes it possible. The choice of the trial is guided by HER2 status in immunohistochemistry and results of new generation sequencing when available. The current trend towards the development of personalized medicine highlights the importance of pathological and molecular characterization of the tumor.

Localized or locally advanced cervical cancer is treated with a curative intent. Its management requires multidisciplinary expertise and a rigorously structured approach to optimize the probability of success. Initial workup (clinical examination, imaging, pathology) allows precise characterization of the tumour and staging according to TNM and FIGO classifications. Surgical management of early stage cancers, ranging from conization for small tumour to hysterectomy, sometimes including sentinel lymph node biopsy, is based on therapeutic algorithms that take into account stage, pathological criteria (invasion, margins, node involvement) and risk category. Postoperative treatment, when required, includes radiochemotherapy, that can be followed by brachytherapy. In locally advanced cancers, treatment consists of radiochemotherapy followed by uterovaginal brachytherapy and immunotherapy that has recently demonstrated its benefits. Since cervical cancer often develops in young women, its management raises important questions related to fertility and sometimes, to the management of cancer during pregnancy. Finally, although it is not the topic of these recommendations, it is important to highlight the major role of vaccination to avoid the vast majority of these cancers.

The prognosis for patients with high-risk neuroblastoma in the event of disease relapse/progression after first line therapy remains poor. However, over the past decade, new therapies have emerged that offer physicians, families and patients the hope of tumor control and, in some cases, a cure. Given the rapid evolution of new therapies in this field, it is strongly recommended that such cases be discussed at a multidisciplinary level and with patients/families regarding treatment options based on existing data. We summarize here the recommendations of the Neuroblastoma Committee of the Société Française de lutte contre les Cancers et les leucémies de l'Enfant et de l'adolescent (SFCE) for the treatment of patients with high-risk neuroblastoma in relapse/progression in France. These recommendations concern chemoimmunotherapy, the combination of ALK inhibitors with chemotherapy, and consolidation treatment options in the absence of tumor progression, as well as the place for early clinical trials.

To establish clinical practice guidelines for the management of women with cervical cancer.

The incidence of kidney cancer is rising. It is the 7th most common cancer in men and the 10th most common in women. Diagnosis is based on imaging (thoraco-abdominopelvic computed tomography scan +/- abdominal magnetic resonance) and histopathology. Clear cell carcinoma is the most frequently observed histological subtype. Management of localized kidney cancer involves surgery or ablative treatments. Active surveillance is indicated in the indolent oligometastatic setting with local treatment in case of localized progression. Apart from this specific situation, two first-line therapeutic strategies are recommended in the metastatic setting : a dual immunotherapy regimen or the combination of immunotherapy with an antiangiogenic tyrosine kinase inhibitor. Both combinations have demonstrated superior survival outcomes compared to sunitinib, the previous standard of care until 2019. Treatment selection should be individualized, considering the characteristics of the disease (histology, tumour burden, location of metastases and if they are threatening, speed of progression), potential side effects of the treatments, the patient's general health, comorbidities and preferences.

Giant cell tumours (GCTs) are benign primary bone tumours that frequently present with local recurrence and occasionally malignant transformation to high-grade sarcoma. Surgery is the mainstay of treatment and generally consists of intralesional curettage. Denosumab was approved by the European Medicines Agency (EMA) in 2014 for the treatment of skeletally mature adults and adolescents with unresectable GCTs or where resection is likely to result in severe morbidity.

Osteosarcoma (OS) and Ewing Sarcoma (ES) are the two most frequent malignant bone tumors in children, adolescents and young adults. In case of disease recurrence, both are characterized by an aggressive behaviour and a relatively poor overall survival rate, with approximately a third of patients having a long-term disease-free survival. In case of recurrent or refractory (R/R) disease, the therapeutic strategy should be discussed in multidisciplinary staff meetings with expertise in bone sarcoma management. The standard management of R/R OS depends on the disease-free interval and the number and sites of metastases and is primarily surgical in patients with isolated lung metastases or local relapse. On the other hand, conventional chemotherapy remains the standard for R/R ES and include high-dose ifosfamide, cyclophosphamide with topotecan and irinotecan with temozolomide.

Ewing sarcomas are the 2nd cause of malignant bone tumors in children and young adults. After induction chemotherapy, local treatment is essential and most often includes surgery of the primary tumor. Radiotherapy may be necessary as an exclusive local procedure when surgery is not possible, or in an adjuvant situation when the surgical procedure must be completed (incomplete resection or poor histological response in particular). Radiotherapy can also concern metastatic sites, particularly in cases of pulmonary metastases and/or in an oligo-metastatic situation. This article provides a review of current indications for radiotherapy, technical modalities of irradiation (delineation of volumes, recommended doses), and the results of recent studies.

Patients who develop Ewing sarcoma with extra-pulmonary metastasis have a poor prognosis. A recent French protocol, CombinaiR3, was set up to evaluate the efficacy of induction chemotherapy followed by high-dose chemotherapy and metronomic maintenance treatment. It is now closed for inclusions and while waiting for the results, we propose a French consensus guideline for the management of patients diagnosed with Ewing sarcoma with extra-pulmonary dissemination. Main recommendations include induction chemotherapy with nine cycles of vincristine/doxorubicin/cyclophosphamide alternating with ifosfamide/etoposide. In case of insufficient response, other chemotherapy combination or inclusion in a clinical trial should be considered. Induction chemotherapy should be followed by local treatment, consisting of surgery and/or radiotherapy. Optimal local treatment is a milestone in the management of patients with Ewing sarcoma and should be discussed with experts and surgeons/radiotherapists working in the sarcoma network. High-dose chemotherapy (HDC) containing busulfan and melphalan followed by autologous stem-cell transplantation is still unclear, with contradictory results. HDC will then be discussed in national tumor board and administered to patients when compatible with local treatment. Given the high relapse rate observed among these metastatic patients, maintenance chemotherapy (so called metronomic regimen) will then be given for two years.

Osteosarcomas of the mandible represent 3-8% of osteosarcomas. The rarity of this anatomic site and its specific treatment explain that only retrospective and a few prospective studies are available in literature. However, there is a consistent evidence on the natural history and treatment of these tumors, which clearly differentiates them from osteosarcomas of the long bones. The aim of this study was to draw up recommendations based on these data and on a retrospective study by the French Sarcoma Group (GSF-GETO). Osteosarcomas of the mandible should be centrally reviewed by an expert pathologist. MDM2, GNAS and RASAL1 status should be checked, and a fragment should be frozen. Complete surgical resection with wide margins is the cornerstone of treatment. Mandibular reconstruction techniques can reduce the sequelae. Contrary to the validated treatment for osteosarcomas of limbs, the role of chemotherapy to prevent metastasis or local recurrence has yet to be clarified for mandibular osteosarcomas. The role of postoperative radiotherapy, in adults, should be discussed for these tumors, whose wide soft-tissue resection may be difficult to confirm. In children, adjuvant chemotherapy is preferable in cases of uncertain/possibly incomplete resection. Relapse of mandibular osteosarcomas is primarily local. Pulmonary metastases are delayed and less frequent than in long-bone osteosarcomas. The overall survival rate at five years is about 70%.

To update the recommendations issued by the National Cancer Institute (INCa) on the management of women with abnormal cervical cytology.

The incidence of follicular-derived thyroid cancers has increased worldwide in recent decades, mainly papillary thyroid cancers at low recurrence risk. A process of de-escalation in the initial management and follow-up of these patients has therefore been implemented in parallel. This article provides the best practice recommendations made by the French learned societies (Société française d'endocrinologie, Société française de médecine nucléaire, Association française de chirurgie endocrine, Société française d'oto-rhino-laryngologie et de chirurgie de la face et du cou), european and international learned societies (European Society for Medical Oncology and the American Thyroid Association), in the management of follicular-derived thyroid cancer without distant metastases. The extent of thyroid surgery and lymph node dissection, strategies of radioiodine ablation, follow-up protocols and the management of excellent prognosis papillary cancers ≤ 10 mm will be addressed.

In this article, we propose a consensus delineation of postoperative clinical target volumes for the primary tumour in maxillary sinus and nasal cavity cancers. These guidelines are developed based on radioanatomy and the natural history of those cancers. They require the fusion of the planning CT with preoperative imaging for accurate positioning of the initial GTV and the combined use of the geometric and anatomical concepts for the delineation of clinical target volume for the primary tumour. This article does not discuss the indications of external radiotherapy (nor concurrent systemic treatment) but focuses on target volumes when there is an indication for radiotherapy.

Pheochromocytomas and paragangliomas are rare neuroendocrine tumors, developed respectively in the adrenal medulla and in extra-adrenal locations. Their malignancy is defined by the presence of distant metastases. Forty percent of them are inherited and can be part of different hereditary syndromes. Their management is ensured in France by the multidisciplinary expert centers of the ENDOCAN-COMETE national network "Cancers of the Adrenal gland", certified by the National Cancer Institute and discussed within multidisciplinary team meetings. The diagnostic and therapeutic work-up must be standardized, based on an expert analysis of clinical symptoms, hormonal biological secretions, genetics, morphological and specific metabolic imaging. In the context of a heterogeneous survival sometimes beyond seven to ten years, therapeutic intervention must be justified. This is multidisciplinary and relies on surgery, interventional radiology, external or internal radiotherapy and medical treatments such as sunitinib or dacarbazine and temodal chemotherapy. The personalized approach based on functional imaging fixation status and genetics is progressing despite the extreme rarity of this disease.

French recommendations for clinical practice, Nice/Saint-Paul-de-Vence 2022-2023: Management of advanced cervical cancer The prognosis of cervical cancer remained pejorative until recently, first-line treatment consisting of platinum-based chemotherapy, associated with bevacizumab whenever possible, without any other therapeutic innovation for several years. However in 2022, immunotherapy appeared in the therapeutic landscape. Pembrolizumab can now be prescribed, thanks to the early access status granted by the HAS in September 2022, in patients with PD-L1 positive tumors. In parallel, bevacizumab generic is now reimbursed, allowing its association with chemotherapy on top of pembrolizumab, if indicated. For patient relapsing after platinium salts, and who never received immunotherapy, cemiplimab could be delivered and reimboursed since spring 2023, whatever could be PD-L1 status. Pretherapeutic work-up includes imaging combining MRI and PET/CT or CT of the chest, abdomen and pelvis, as well as evaluation of PD-L1 status on tumor and immune cells to define the CPS score that will determine eligibility to pembrolizumab treatment (CPS > 1). Possibilities of locoregional treatment depend on individual situations and are discussed on a case-by-case basis in multidisciplinary meetings. Early supportive care is always recommended and inclusion in clinical trials must be systematically considered.

French recommendations for clinical practice Nice-Saint-Paul de Vence 2022-2023: histomolecular diagnosis of endometrial carcinomas The characterisation of endometrial carcinomas has been recently modified and enriched by molecular classification, the integration of which now impacts therapeutic decisions on whether adjuvant therapy should be administered or not in localized tumors, and influences treatment selection in advanced disease. Mandatory information includes histological type according to WHO 2020 classification, histological grade, hormone receptors status and molecular classification, the main new elements to provide being analysis of MMR proteins, p53 status and POLE status in selected cases. Sampling and preparation of material must be performed adequately to allow complete analysis. Numerous markers can be used to better define histological type, distinguish between primary lesion or metastases, or provide prognostic information. Determination of MMR/MSI profile is complex but well defined by guidelines that precisely describe techniques to be used and interpretation rules. Knowledge of POLE status is useful to guide therapeutic strategy, especially to consider de-escalation in stages I and II, in particular in case of high grade and/or p53 mutated tumors. This is why indications of POLE determination must be well defined. Finally, oncogenetics consultation is recommended in dMMR tumors (except in case or MLH1 promoter methylation) and in patients with evocative familial history.

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.

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.