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Introduction: Soft tissue sarcomas (STSs) are a rare and heterogenous group of tumors, with poor prognostic, judging from their frequency to relapse. Few drugs are available after the conventional first-line regimen. Since 2007, trabectedin got approval after failure of anthracyclines and ifosfamide, for advanced or metastatic STS. This led to a FDA approval in 2015, but real-world evidence is still required, complementary to the pivotal phase II and III trials. Methods: One hundred twenty-six patients with STS, treated by trabectedin between 2002 and 2019, were analyzed in this retrospective study, in two French centers. The effects of trabectedin on survival, response, and toxicity were described. All patients were tested for toxicities, and efficacy was assessed in patients exposed to at least 2 cycles of trabectedin. Results: Three median cycles were administered per patient (1–79). Among the 113 patients analyzed for efficacy, the median progression-free survival was 3.0 months (95% CI: 2.3–4.8), with an overall survival of 12.3 months (95% CI: 10.2–16.9). The rate of disease control was 46% at the end of treatment. Myxoid liposarcoma (n = 11) was the histology subtype that benefited most from this chemotherapy with median progression-free survival and overall survival of 13.3 months (95% CI: 2.3–18.7) and 27.8 months (95% CI: 3.2–64.7), respectively. Adverse events were manageable. Discussion and Conclusion: Efficacy of trabectedin is confirmed in terms of clinical benefit and low toxicity, especially for myxoid liposarcoma. Combinatory regimens are under clinical trials to optimize the place of this chemotherapy.

Hepatitis-associated aplastic anemia (HAAA) accounts for 4% of autoimmune hepatitis in children. An episode of seronegative autoimmune hepatitis is followed a few days or months later by aplastic anemia or full aplasia. This autoimmune disease could be due to a regulation defect in the immune response to a viral trigger, in a genetically predisposed individual. Other causes of hepatitis or aplastic anemia have to be ruled out. Steroids and azathioprine usually control the liver damage but do not prevent the development of aplastic anemia. Aplastic anemia is treated with either hematopoietic stem cell transplantation in patients with a sibling donor or anti-thymocyte globulins and cyclosporine. We propose guidelines to explore and treat this rare disease. We emphasize on the necessary close collaboration between hepatologists and hematologists.

Human papillomaviruses (HPV) are involved in the development of anogenital and head and neck cancers. The purpose of this study was to assess the risk of developing a second primary cancer (SPC) after a first potentially-HPV-related cancer, and to analyze the sites where SPCs most frequently occurred in these patients. All patients with a first cancer diagnosed between 1989 and 2004, as recorded by 10 French cancer registries, were followed up until December 31, 2007. Only invasive potentially-HPV-related cancers (namely, cervical, vagina, vulva, anal canal, penile, oropharynx, tongue and tonsil) were included. Standardized Incidence Ratios (SIRs) were calculated to assess the risk of SPC. A multivariate Poisson regression model was used to model SIRs separately by gender, adjusted for the characteristics of the first cancer. 10,127 patients presented a first potentially-HPV-related cancer. The overall SIR was 2.48 (95% CI, 2.34-2.63). The SIR was 3.59 (95% CI, 3.33-3.86) and 1.61 (95% CI, 1.46-1.78) in men and women respectively. The relative risk of potentially-HPV-related SPC was high among these patients (SIR=13.74; 95% CI, 8.80-20.45 and 6.78; 95% CI, 4.61-9.63 for men and women, respectively). Women diagnosed in the most recent period (2000-2004) showed a 40% increase of their relative risk of SPC as compared with women diagnosed between 1989 and 1994 (ratio of SIRs=1.40; 95% CI, 1.06-1.85). HPV cancer survivors face an increased risk of SPC, especially second cancer. Clinicians may consider this increased risk of developing HPV-related SPC during follow-up to improve subsequent cancer prevention in these patients.

Laboratoire d’accueil : Registre des tumeurs du doubs et du territoire de Belfort, EA 3181 « Carcinogenese epitheliale, facteurs predictifs et pronostiques », 2, place Saint-Jacques, 25000 Besancon. Responsable : Dr Anne-Sophie Woronoff ( asworonoff@chu-besancon.fr ) Etat de la question Les papillomavirus humain (HPVs) sont impliques dans le developpement de nombreux cancers ano-genitaux et de l’oropharynx. Cette etude evaluait le risque de developper un second cancer primitif (SCP) apres un premier cancer potentiellement lie a HPV et etudiait les localisations de survenues de ces SCP. Materiel et methodes Les patients ayant developpe un premier cancer entre 1989 et 2004 residant dans 8 departements francais etaient enregistres dans les bases de donnees de registres de cancer et suivis jusqu’au 31 decembre 2007. Seuls les patients ayant developpe un premier cancer potentiellement lie a HPV ont ete analyses (col de l’uterus, vagin, vulve, canal anal, penis, oropharynx, langue, amygdale). Des ratios standardises d’incidence (SIR) ont ete calcules pour comparer le risque de developper un SCP dans cette population par rapport a la population generale. Un modele multivarie de Poisson a ensuite ete realise pour analyser les caracteristiques du premier cancer. Resultats Au total, 10 127 patients ont presente un premier cancer potentiellement lie a HPV. Parmi eux, 1117 (11,00 %) ont declare un SCP. Le SIR global etait de 2,48 (IC 95 % : 2,34–2,63). Les SIRs etaient de 3,59 (IC 95 % : 3,33–3,86) et 1,61 (IC 95 % : 1,46–1,78) chez les hommes et les femmes, respectivement. Le risque de developper un SCP potentiellement lie a HPV etait augmente (SIR = 13,74, IC 95 % : 8,80–20,45 et 6,78, IC 95 % : 4,61–9,63 chez les hommes et les femmes, respectivement). Les femmes ayant presente un premier cancer potentiellement lie a HPV entre 2000 et 2004 avaient plus de risque de developper un SCP par rapport aux femmes ayant presente un premier cancer entre 1989 et 1994 (SIR ratio = 1,40, IC 95 % : 1,06–1,85) Conclusion Le fait de presenter un premier cancer potentiellement associe a HPV est a l’origine d’une augmentation du risque de developper un SCP, particulierement dans les sites lies a HPV. Ces resultats pourraient aider les praticiens dans le suivi de ces patients a haut-risque en termes de prevention et de detection precoce de ces SCP.

Contexte La drepanocytose SC represente 30 % des syndromes drepanocytaires majeurs. Peu de cohortes neonatales s’interessent au devenir clinique de ces patients. Nous decrivons dans une cohorte neonatale l’incidence des complications aigues et chroniques des drepanocytaires SC. Methode Etaient inclus tous les patients SC depistes neonataux suivis au centre hospitalier de Creteil, en excluant ceux dont le suivi avait ete effectue dans un autre centre avant l’âge de 6 mois. Le suivi prospectif recueillait l’apparition des complications aigues necessitant une hospitalisation et les evenements apparus au cours du suivi lors des bilans systematiques. Les donnees ont ete censurees a la date de la derniere visite. Resultats Quatre-vingt patients ont ete suivis pendant une duree mediane de 7,99 ans soit 708 patients annees entre 1986 et 2013. Soixante patients (75 %) ont presente au moins une complication aigue. Le risque cumule de syndrome thoracique aigu et de sequestration splenique aigue a 10 ans est de 51 % et 15 % respectivement soit une incidence de 7,2 et 1,4 pour 100 patients annees (PA) respectivement. Le taux de crises vaso-occlusives est de 30,01 pour 100 PA. Il n’y a pas eu de complication infectieuse ni d’accident vasculaire cerebral ni de deces. La retinopathie est la complication chronique la plus frequente avec une incidence de 1,34 pour 100 PA, IC 95 % (0,69–2,67) soit un risque cumule de 23,8 % a 15 ans. Discussion Le suivi de cette cohorte met en avant le poids de la maladie avec un nombre important de complications. Le depistage neonatal permet de prevenir les complications infectieuses graves grâce notamment a l’education parentale. Le suivi chronique de ces patients est indispensable pour depister precocement les complications. Aujourd’hui aucune therapeutique n’est validee dans la drepanocytose SC, l’utilisation d’un programme de saignees comme chez l’adulte pourrait etre une alternative pour diminuer l’incidence de ces complications.