About 5% of gastroenteropancreatic and thoracic neuroendocrine neoplasms (NENs) arise in the context of an inherited tumour syndrome. The two most frequent syndromes are: multiple endocrine neoplasia type 1 (MEN1), associated with a large spectrum of endocrine and non endocrine tumours, including duodenopancreatic, thymic and bronchial NENs, and the von Hippel-Lindau syndrome VHL, associated with pancreatic NENs. Two inherited syndromes have a low incidence of NENs: neurofibromatosis type 1 (NF1), associated with duodenal somatostatinomas, and tuberous sclerosis (TSC), associated with pancreatic NENs. Two rare syndromes have a high incidence of NENs: multiple endocrine neoplasia type 4 (MEN4), with a tumour spectrum similar to that of MEN1, and glucagon cell hyperplasia neoplasia (GCHN), involving only the pancreas. It is likely that other syndromes remain to be characterized, especially in familial small-intestinal NENs. The diagnosis is usually raised because of the suggestive clinical setting: young age at diagnosis, multiple tumours in multiple organs, familial history. Except in VHL and NF1, tumours themselves do not show specific pathological features; they usually are well differentiated and of low histological grade; their prognosis is good, except for MEN1-associated thymic NENs. The most suggestive pathological feature is their combination with various endocrine and/or non endocrine lesions in the adjacent tissue. Pathological examination is important, for a correct diagnosis and for an accurate management of the patients and their families, who must be referred to expert centers.

Light chain deposition disease is a rare anatomo-clinical disorder, which rarely leads to cystic lung destruction.

Pancreatic ductal adenocarcinoma (PDAC) is a rapidly evolving and most frequently fatal disease. Despite the enormous progress in understanding the mechanisms related to PDAC pathogenesis, the impact on patient management has not yet been possible. Pancreatic organoids can be generated from small amounts of tissue. One of the most promising applications of organoids is that they can serve as a platform for selecting the right drugs for each patient. This approach has the potential to identify individual therapeutic vulnerabilities by allowing the personalization of treatments. However, these analyzes require several weeks before obtaining enough organoids from the same individual, to carry out the tests with several drugs, and to analyze the results, which limits its use in current clinical practice for the patients with a PDAC, whose it must be remembered that half die within 6 months of diagnosis. To overcome this obstacle, we assessed the ability of transcriptomic molecular signatures to identify patients with a particular sensitivity profile to a given treatment. The approaches based on transcriptomic profiling have the enormous advantage of using very little biological material and thus significantly reducing the time to arrive at the selection of more effective drugs to each patient.

Breast cancer of young women has medico-psychological issues, particularly in the perspective of "after cancer" period. The objective of the study was to analyze the changes in management, in a decade including the publication of Cancer Plans I and II.

Breast cancer in young women requires special vigilance because of the unique condition and complex management that is involved. The indication for chemotherapy should not be based on age alone but in association with the biological characteristics of the tumour. In addition, age is not considered as demonstrated predictor of chemosensitivity. The choice of adjuvant or neoadjuvant chemotherapy should be guided by the histological type of the tumour, the stage of the tumour and the patient's comorbidities. In this subgroup of patients, the recommendations for the use of molecular signatures follow those of the general population. This manuscript summarizes the main data and recommendations on the management of breast cancer in young women in term of chemotherapy.

Early-onset of breast cancer (under the age of 40) represents only 7% of all breast cancers, but is the most common cancer in this age group in women. It is also known to be of worse prognosis, with a more aggressive tumoral behavior. The interaction of different prognostic factors contributes to the complexity of this population: tumor burden and biological features (using classical histopronostic features and genomic data) show differences from older women. Nevertheless, the prognostic impact of age varies according to the histological subtypes and seems pejorative mainly for the luminal subtype, probably with a crucial role of the hormonal environment and the treatments targeting the endocrine sensitivity of these tumors. In other subtypes, the influence of young age appears to be less significant, especially in HER2+ breast cancers.

The management of desmoid-type fibromatosis has considerably evolved these last years, toward first-line active surveillance then systemic or local symptomatic treatment in case of aggressive tumor. Magnetic resonance imaging is the modality of choice in each of these treatment settings. It needs multiparametric approach taking into account mainly the tumor size, and T2-weighted signal that is correlated with histological composition and clinical behavior. A volumetric approach should be favored for the evaluation of tumor size change. The interest of paramagnetic contrast injection and tumor enhancement requires further investigation.

To evaluate the diagnostic value of serum biomarkers in the management strategy of borderline ovarian tumours (BOT) to make management recommendations.

Contraceptive options and menopause management are frequent clinical issues among women previously treated for a borderline ovarian tumour (BOT).

The incidence (rate/100,000) of BOT gradually increases with age from 15-19 years of age and peaks at nearly 4.5 cases/100,000 for the 55-59 year age group (NP3). In the presence of a benign ovarian mass, the standardized risk ratio of serous and mucinous BOT is 1.69, (95% CI 1.39-2.03) and 1.75, (95% CI 1.45-2.10), respectively (NP2). At diagnosis, a median age of diagnosis of OFA is 46 years, unilateral forms (79.7% of cases) are predominant compared to cancers (45.3%) (<0.001) and FIGO I stages represent nearly 63.7% of cases (NP3). The 5-year survival rates for FIGO I, II, III, IV stages are: 99.7% (95% CI: 96.2-100%), 99.6% (95% CI: 92.6-100%), 95.3% (95% CI: 91.8-97.4%), 77.1% (95% CI: 58.0-88.3%), respectively (NP3). Survivors at 5 years for serous and mucinous tumours are 99.7% (95% CI: 99.2-99.9%), 98.5% (95% CI: 96.9-99.3%), respectively (NP3). An epidemiological association exists between personal BOT risk and: (1) a familial history of BOT/certain cancers (pancreas, lung, bone, leukemia) (NP3), (2) a personal history of benign ovarian cyst (NP2), (3) a personal history of pelvic inflammatory disease (IGH), (4) the use of intrauterine device levonorgestrel (NP3), (5) the use of oral contraceptive pills (NP3), (6) multiparity (NP3), (7) hormone replacement therapy (NP3), (8) high consumption of coumestrol (NP4), (9) medical treatment of infertility with progesterone (NP3), (10) non-steroidal anti-inflammatory drug (NSAID). An epidemiological association exists between previous/actual tabacco consumption and the risk of mucinous ovarian BOT (NP2). Relative risk (RR) varies between 2.2 and 2.7, however the relationship is not necessarily a causal one. An epidemiological association exists between overweight/obesity and the risk of serous BOT (NP2). RR varies between 1.2 to 1.8. The high Vitamin D was inversely associated to the risk of serous BOT (NP4). The risk of mucinous BOT was lowered with paracetamol use (OR=0.77; 95% CI: 0.60-0.98) (NP3). However, the relationship between these factors and BOT is not necessarily a causal one and no screening modality can be proposed in the general population (gradeC).

To provide guidelines for clinical practice from the French College of Obstetrics and Gynecology (CNGOF), based on the best evidence available, concerning early stage borderline ovarian tumors (BOT).

To evaluate the surgical management of borderline ovarian tumors (BOT) in the framework of recommendations for clinical practice made by the National College of Obstetricians and Gynecologists (CNGOF) METHODS: This is a comprehensive review of the literature on the advanced stages of BOT. Bibliographic selection was conducted in PubMed from 2007 to 2019 inclusive, selecting publications in English and French. Articles were selected on the basis of the title, then the abstract and finally the full article. The levels of evidence of the studies were defined according to the scale proposed by the High Authority of Health (HAS).

To provide clinical practice guidelines from the French college of obstetrics and gynecology (CNGOF) based on the best evidence available, concerning epidemiology of recurrence, the risk or relapse and the follow-up in case of borderline ovarian tumor after primary management, and evaluation of completion surgery after fertility sparing surgery.

To determine the place of imaging, tumour markers, type of treatment and surgical route, follow-up, delivery mode, and re-staging in case of BOT during pregnancy, in order to provide guidelines.

To provide recommendations for the diagnosis and management of the recurrence of Borderline Ovarian Tumour (BOT).

This work was carried out under the aegis of the CNGOF (Collège national des gynécologues et obstétriciens français) and proposes guidelines based on the evidence available in the literature. The objective was to define the diagnostic and surgical management strategy, the fertility preservation and surveillance strategy in Borderline Ovarian Tumor (BOT). No screening modality can be proposed in the general population. An expert pathological review is recommended in case of doubt concerning the borderline nature, the histological subtype, the invasive nature of the implant, for all micropapillary/cribriform serous BOT or in the presence of peritoneal implants, and for all mucinous or clear cell tumors (grade C). Macroscopic MRI analysis should be performed to differentiate the different subtypes of BOT: serous, seromucinous and mucinous (intestinal type) (grade C). If preoperative biomarkers are normal, follow up of biomarkers is not recommended (grade C). In cases of bilateral early serous BOT with a desire to preserve fertility and/or endocrine function, it is recommended to perform a bilateral cystectomy if possible (grade B). In case of early mucinous BOT, with a desire to preserve fertility and/or endocrine function, it is recommended to perform a unilateral adnexectomy (grade C). Secondary surgical staging is recommended in case of serous BOT with micropapillary appearance and uncomplete inspection of the abdominal cavity during initial surgery (grade C). For early-stage serous or mucinous BOT, it is not recommended to perform a systematic hysterectomy (grade C). Follow up after BOT must be pursued for more than 5 years (grade B). Conservative treatment involving at least the conservation of the uterus and a fragment of the ovary in a patient wishing to conceive may be proposed in advanced stages of BOT (grade C). A new surgical treatment that preserves fertility after a first non-invasive recurrence may be proposed in women of childbearing age (grade C). It is recommended to offer a specialized consultation for Reproductive Medicine when diagnosing BOT in a woman of childbearing age. Hormonal contraceptive use after serous or mucinous BOT is not contraindicated (grade C).

To determine the place of imaging and the performance of different imaging techniques (transvaginal ultrasound with or without Doppler, scoring, CT, MRI) to differentiate benign tumour, borderline ovarian tumour (BOT) and malignant ovarian tumor. Differentiate the histological subtypes of BOT (serous, sero-mucinous, mucinous) and prediction in imaging of the possibility of conservative treatment.