Stereotactic radiotherapy is a very hypofractionated radiotherapy (>7.5Gy per fraction), and therefore is more likely to induce late toxicities than conventional normofractionated irradiations. The present study examines four frequent and potentially serious late toxicities: brain radionecrosis, radiation pneumonitis, radiation myelitis, and radiation-induced pelvic toxicities. The critical review focuses on the toxicity scales, the definition of the dose constrained volume, the dosimetric parameters, and the non-dosimetric risk factors. The most commonly used toxicity scales remain: RTOG/EORTC or common terminology criteria for adverse events (CTCAE). The definition of organ-at-risk volume requiring protection is often controversial, which limits the comparability of studies and the possibility of accurate dose constraints. Nevertheless, for the brain, whatever the indication (arteriovenous malformation, benign tumor, metastasis of solid tumors...), the association between the volume of brain receiving 12Gy (V12Gy) and the risk of cerebral radionecrosis is well established for both single and multi-fraction stereotactic irradiation. For the lung, the average dose received by both lungs and the V20 seem to correlate well with the risk of radiation-induced pneumonitis. For the spinal cord, the maximum dose is the most consensual parameter. Clinical trial protocols are useful for nonconsensual dose constraints. Non-dosimetric risk factors should be considered when validating the treatment plan.

Peritoneal inclusion cyst is a rare benign tumor. It usually affects women of reproductive age. Its etiology is poorly understood; a history of endometriosis, pelvic inflammatory disease and pelvic surgery are sometimes implicated in its occurrence. Its diagnosis is difficult with complex management. We report the case of a 29-year-old woman presenting a rectal mass for which the analysis of echo-endoscopic samples was not contributory. The PET-scan revealed a rectal submucosal mass and deep adenopathy. An exploratory laparoscopy was performed, and allowed to remove cystic inflammatory areas and lymph nodes. The histopathological study confirmed the diagnosis of peritoneal inclusion cyst with endometriosis and reactive adenitis. Peritoneal inclusion cyst is a rare condition that develops at the expense of the serosa. The risk of recurrence is high with a possibility of malignant transformation. Excision and monitoring are essential for good management.

Salivary gland carcinomas are rare, characterized by a diversity of histological subtypes associated with variable clinical behavior and prognosis with usually a poor response to chemotherapy. In this context, molecular alterations have been identified and represent potential therapeutic targets: overexpression of human epidermal growth factor receptor 2 (HER2) and androgen receptors in salivary duct cancer, NOTCH mutations in adenoid cystic carcinoma, NTRK gene fusion in secretory carcinoma. Screening for these molecular alterations is mandatory in all patients with recurrent or metastatic salivary gland cancer as it may allow an individualized treatment.

The thyroid is an endocrine gland playing a major role in metabolism and development by the secretion of T4 and T3 thyroid hormones. Due to its anatomical position, it is often included in the target volume for the irradiation of certain tumours and thus receives significant doses (10 to 80Gy). The treatment of breast cancer requires in most cases a breast irradiation associated or not with a lymph node irradiation. The aim of our study was to investigate prospectively the frequency of thyroid disorders in patients with breast cancer treated by radiation, with or without irradiation of the supra- and subclavicular lymph nodes.

Managing a malignant renal tumor requires, first of all, a reflection on the necessity of its treatment. It must consider the renal function, altered at the time of diagnosis in 50% of cases. The treatment method chosen depends on many factors, in particular, the predicted residual renal function, the risk of chronic kidney disease, the need for temporary or long-term dialysis, and overall long-term survival. Other factors include the size, position, and number of tumors and a hereditary tumor background. When a renal-sparing management alternative is available, total nephrectomy should no longer be performed in patients with small malignant renal masses (cT1a). This may consist of surgery (partial nephrectomy or lumpectomy), percutaneous thermo-ablation (by radiofrequency, microwave, or cryotherapy). In patients with limited life expectancy, imaging-based surveillance may be proposed to suggest treatment in case of local progression. Good coordination between urologist, radiologist, nephrologist, and sometimes radiotherapist should allow optimal management of patients with a malignant renal tumor with or without underlying renal failure.

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, which determines their local invasiveness, and their hematogenous/nodal spread. Their diagnosis can be difficult and often requires comprehensive immunohistochemistry and genomic techniques. Expert pathology review is recommended in the cases of undifferentiated tumors, sarcomas and at the slightest diagnostic doubt. These rare cancers can also be rare by their anatomical location when arising from the paranasal sinuses, salivary glands and ear. Their location requires knowledge of their specific extension routes, and may call for a specific surgical technique (skull base endoscopic sinus surgery, extended total parotidectomy, etc.) and adapted radiotherapy to spare healthy organs surrounding the tumor. This article (part 1) discusses the diagnostic and therapeutic specificities of these rare cancers, and develops the recommendations of the French ENT Cancer Expertise Network (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. A 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.

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.

The aim of our study was to evaluate the morbidity and mortality, as well as the oncogical results of patients who had undergone surgical procedure for a kidney cancer with thrombus extension into the inferior vena cava.

Squamous cell carcinoma of the male perineum is exceptional. We report the case of a 42-year-old patient with no previous medical history, presenting with pelvic discomfort lasting for 4 months. The patient was treated for perineal abscess in a health centre in Bamako. The anatomo-pathological examination confirmed the diagnosis. Treatment depends on the stage of the lesion and its location but it is associated with a poor prognosis. Given the results achieved in patients with epidermoid cancers of the esophagus and anus, treatment was based on therapeutic protocols combining chemotherapy and radiotherapy. The purpose of this work was to report the first case in our hospital unit.

Nodular Lymphocyte predominant Hodgkin lymphoma (NLPHL) are rare lymphomas in pediatric patients comprising less than 10 % of all Hodgkin lymphoma (HL). They are for the most part diagnosed at stage I or II and indolent with lymphadenopathy often preceding the diagnosis by many months/years. Survival is excellent. Historically, patients were treated according to classical HL protocols. Due to high toxicity and excellent prognosis, management of NLPHL shifted to de-escalation protocol with good results. No treatment beyond surgical resection was proposed for localized unique nodal disease completely resected. The closed European protocol (EuroNet PHL LP1) evaluated the efficacy of low intensity chemotherapy protocol based on CVP courses (cyclophosphamide vinblastine prednisone) for stage IA/IIA not fully resected. Final results are not yet available. Advanced stage NLPHL are rare and there is no clinical trial and no consensus treatment in children. The SFCE lymphoma committee recently established recommendations for staging and treatment of limited and advanced NLPHL in children based on current practices and published results. The goal was to allow homogeneous practice on a national scale. If incomplete resection for patients with stage I/IIA combination of low intensity chemotherapy (CVP) and rituximab is recommended. For intermediary and advanced stage intensification with AVD (adriamycine vinblastine dacarbazine) or CHOP courses (cyclophosphamide doxorubicine vincristine prednisone) combined with rituximab are advocated. In children, there is no indication for first-line local treatment with radiotherapy.

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.

Even if each rare ovarian tumor (ROT) has a low incidence, the sum of all these entities represents almost the half of all ovarian neoplasms. Thus, development of dedicated clinical trial emerged as a prerequisite to improve their managements. Owing to the spreading of dedicated institutional networks and (supra)national collaborations, the number of clinical trials has increased the past few years, with different types of trials; while some focused on specific molecular features, others assessed innovative molecules. Furthermore, relevant randomized clinical trials were designed as a mean to position new treatment options. Currently, innovative molecular-driven trials, based on master protocol trials are emerging and may shed light towards the improvement of personalized medicine regarding ROT.

Darrier-Ferrand dermatofibrosarcoma (DFSC) is the most common cutaneous sarcoma. It generally affects subjects with an average age of 40 years, without gender or race predominance. It is a tumour characterised by a slow evolution and local aggressiveness. The reasons for consultation are pain, pruritus or rapidly progressive evolution. There are no specific imaging studies for this tumour. The diagnosis of certainty is based on immunohistochemistry with positive CD34 labelling. Treatment is surgical based on wide excision.