DICER1-mutated rhabdomyosarcoma of the ovary with teratoid features.

DICER1-mutated rhabdomyosarcoma is a rare, emerging entity with a predilection for the gynecologic and genitourinary tracts. We report here a case of DICER1-mutated rhabdomyosarcoma of the ovary in a 14 years old girl which displayed interspersed mature teratoid glands, neuroectodermal rosettes and immature blastematous-like tubes. Morphologically the sarcomatous component predominated, corresponding to a high grade spindle cell rhabdomyosarcoma with botryoid features. Islets of cartilage were present. The sarcomatous proliferation encased the teratoid glands, forming cambium layer-like arrangements. The sarcoma cells were Myogenin and MYOD1 positive, the neuroectodermal rosettes expressed SALL4 along with cytokeratins and EMA and were negative for Inhibin; immature blastematous-like tubes were negative for SALL4 and Inhibin. Whole RNA- and targeted DNA-sequencing revealed two DICER1 mutations in exon 26: c.5113G>A: p.(Glu1705Lys) and exon 12: c.1642C>T: p.(Gln548X). The sarcomatous component harbored a complex genetic profile while the teratoid component was diploid, none of the above displayed abnormality of 12p. DICER1-mutated sarcomas display pathological features similar to embryonal rhabdomyosarcomas, botryoid type. They also display heterogeneous features combining cartilage foci, teratoid mature glands, immature blastematous-like tubes and/or neuroectodermal components. Molecular testing remains necessary to confirm the diagnosis. Further studies need to clarify the nosology of DICER1-mutated sarcomas and devise specific therapeutic strategies.

Tissue cholesterol metabolism and prostate cancer aggressiveness: Ethno-geographic variations.

BACKGROUND: Prostate cancer (PCa) is more frequent and more aggressive in populations of African descent than in Caucasians. Since the fatty acid composition of peri-prostatic adipose tissue (PPAT) has been shown to differ according to the ethno-geographic origin and is involved in PCa aggressiveness, we aimed to analyze the cholesterol content of PPAT from Caucasian and African-Caribbean patients, in correlation with markers of disease aggressiveness and cholesterol metabolism in cancer tissues. METHODS: The quantification of cholesterol in PPAT was analyzed in 52 Caucasian and 52 African-Caribbean PCa patients, with in each group 26 indolent tumors (ISUP Group1 and pT2) and 26 potentially aggressive tumors (ISUP Group 3-5 and/or pT3). The expression of proteins involved in cholesterol metabolism was analyzed by immunohistochemistry on cancer tissue samples included in tissue microarrays. RESULTS: The amount of cholesterol esters was lower in PPAT from African-Caribbean patients compared with Caucasians, without any correlation with markers of disease aggressiveness. In cancer tissues from African-Caribbean patients, the expression of ABCA1 (involved in cholesterol efflux) was decreased, and that of SREBP-2 (involved in cholesterol uptake) was increased. In both groups of patients, SREBP-2 expression was strongly associated with that of Zeb1, a key player in the epithelial-to-mesenchymal transition (EMT) process. CONCLUSION: These results suggest that cholesterol metabolism differs according to the ethno-geographic origin, in both PPAT and cancer tissues. In African-Caribbeans, the orientation towards accumulation of cholesterol in cancer cells is associated with a more frequent state of EMT, which may promote PCa aggressiveness in this population.

Frequent high-risk HPV co-infections excluding types 16 or 18 in cervical neoplasia in Guadeloupe.

BACKGROUND: Cervical cancer is the fourth cancer worldwide. The Human Papilloma Virus is responsible for 99% of the cases but the distribution of its genotypes varies among populations. We aimed to identify HPV genotypes distribution in women with grade 2/3 cervical intraepithelial dysplasia or invasive cervical cancer in Guadeloupe, a French Caribbean territory with a population mainly of African descent. METHODS: We used paraffin-embedded tumors for viral DNA extraction from women diagnosed between 2014 and 2016 and identified by the population-based cancer registry. The HPV Genotyping was performed with the InnoLIPA HPV Genotyping Extra kit®. RESULTS: Overall, 213 samples out of the 321 eligible records were analyzed. The HPV status was positive for 94% of the cases. The five most common oncogenic HPV genotypes were HPV31 (47%), HPV33 (38%), HPV16 (32%), HPV44 (31%) and HPV26 (28%). HPV18 was found in only in 5% of the cases. Among the studied cases, 94% had multiple infections. More than 60% of single infections were HPV16-related, accounting for 35% of HPV16 infections. CONCLUSIONS: These results show a different distribution of oncogenic HPVs in Guadeloupe with "31 >  33 > 16" and a high frequency of multiple infections. Despite a lower coverage, the nine-valent vaccine is nevertheless adequate.

Estimating county-level tobacco use and exposure in South Carolina: a spatial model-based small area estimation approach.

PURPOSE: Local health statistics are increasingly requested for policy-making and programmatic purposes; however, population-based surveys are often inadequate to support direct estimation for small areas. Model-based estimation techniques can be used to create local estimates for public health outcomes. Using the 2014-2015 South Carolina (SC) Adult Tobacco Survey, we examined tobacco-related outcomes at the county level using a spatial multilevel, poststratification approach. METHODS: To create county-level tobacco estimates, we used a two-level model with a spatially intrinsic conditional autoregressive random intercept. Stratum-specific (race, age, and sex) estimates for each county were then created and averaged based on population data obtained from the U.S. Census. RESULTS: The estimated prevalence of current smoking in SC counties among adults ranged from 7.4% to 35.1%, and the percentage reporting ever trying an e-cigarette ranged from 4.2% to 30.2%. Model validation showed considerable agreement between direct and indirect estimates (Pearson and Spearman correlations all >0.75) that varied by the sample size of the outcome, as hypothesized. CONCLUSIONS: Data from the SC Adult Tobacco Survey were used to develop county-level estimates of multiple tobacco-related outcomes using a spatial multilevel, poststratification approach. The results showed heterogeneity in smoking behaviors across the state along with marked spatial correlation.

[Mammary analog secretory carcinoma of the parotid gland]. / Carcinome salivaire sécrétoire, analogue des carcinomes sécrétoires mammaires.

Mammary analog secretory carcinoma (MASC) of the parotid gland is a rare and recently described lesion. We report the case of a 46-year-old man with a tumor of the parotid gland which was carried to the diagnosis of MASC. Diagnostic was confirmed by highlighting the ETV6-NTRK3 gene translocation. However, some morphologic and immunohistochemical features are suggestive of this entity. This carcinoma should be distinguished from its main differential diagnoses: acinic cell carcinoma and low grade cribriform cystadenocarcinoma.

Evaluating correlates of adolescent physical activity duration towards National Health Objectives: analysis of the Colorado Youth Risk Behavioral Survey, 2005.

BACKGROUND: While numerous studies have examined the relationships among correlates of physical activity (PA), less attention has been given to identifying the correlates of low PA duration. The main objective of the current study was to examine correlates of low PA duration, team sports participation and smoking behaviors among adolescents. METHODS: Data from the 2005 Colorado Youth Behavioral Risk Survey were analyzed using Cox proportional hazard models. We evaluated associations between two measures of low PA duration, assessed as per Healthy People 2010 (HP2010) objectives and 2008 Physical Activity Guidelines (PAG) for Americans, and smoking behaviors, participation in the physical education (PE) and team sports, controlling for age, gender and other behavioral characteristics. RESULTS: Forty percent and 70%, respectively, of adolescents did not meet the 2008 PAG and HP2010 objectives. After adjustment, smoking remained associated with failure to meet the 2008 PAG. However, no significant relationship was found with low PA duration as per the HP2010 objectives. The risk of low PA was higher among girls for both outcome measures. Likewise, adolescents who reported no participation in team sports presented a 7-fold higher risk of low PA as per the 2008 PAG and 51% higher risk of low PA as per the HP2010 objectives compared with the group with team sports participation. CONCLUSIONS: Regular participation in school PE and team sports may represent an important avenue for increasing PA duration and reducing smoking behaviors among adolescents.

[Primary neuroendocrine tumor of the fallopian tube: a case report]. / Carcinome neuro-endocrine primitif de la trompe. A propos d'un cas.

Primary neuroendocrine carcinoma arises mainly in the gastrointestinal tract, lungs and pancreas, localization in the fallopian tube is exceptional with only one case being reported in literature. This second case concerns a 54-year-old patient, diagnosed at the Pierre-Zobda-Quitman hospital. Tumor histology revealed a poorly differentiated carcinoma with medium to large cells, marked atypia and high mitotic activity. Immunostaining was positive for chromogranine A, synaptophysine and CD56. Primary fallopian tube neuroendocrine carcinoma requires cross referencing of the radiological, pathological and immunohistochemical findings and discarding the differential diagnoses.

[Undifferentiated embryonal sarcoma of the liver in an adult: a case report]. / Sarcome indifférencié (embryonnaire) hépatique développé chez un adulte: à propos d'un cas.

Undifferentiated embryonal sarcoma of the liver is rare. It is usually observed in children and adolescents. We report one case of embryonal sarcoma of the liver arising in patient without any antecedent. The only symptom was right scapular pain. The liver scan showed a multicystic lesion suspicious for infectious origin or a tumor. Serologies for ecchinococcus, schistosomiasis and brucellosis were negative. The treatment was a right hepatectomy. On gross examination, the tumor was unencapsulated, multicystic and contained large areas of necrosis admixed with gelatinous areas. Microscopically, there were epithelioid and spindle tumor cells in a myxoïd stroma. Lipoblastic-like or rhabdomyoblastic-like, giant cells and PAS positive hyaline globules in the cell cytoplasm were present. The tumor cells expressed vimentin, cytokeratin (KL1), alpha-1-antitrypsin and smooth muscle actin. This observation shows that embryonal sarcoma of the liver may develop in adult patients and should be taken into consideration in any differential diagnosis of cystic hepatic tumor.