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Introduction/Background In SOLO1 trial, maintenance therapy with olaparib considerably improved progression-free survival (PFS) among women with newly-diagnosed, advanced BRCA-mutated HGEOC. Based on the large real-life ESME ovarian cancer (OC) cohort, we aimed to describe the patient‘s characteristics and survival outcomes of a selected cohort of BRCA-mutated patients before the era of PARP inhibitors maintenance in first line. Methodology ESME OC is a national cohort including all patients (pts) with EOC managed in the 18 French Comprehensive Cancer Centres (NCT03275298). ESME Research program collected retrospective data from patient‘s medical records. BRCA1, BRCA2, or both mutated patients with de novo diagnosed advanced (FIGO stage III or IV) high-grade serous or endometrioid EOC were eligible. Primary objective was to describe patient‘s characteristics, clinical features and treatment patterns. Results Of the 4777 patients with OC treated in first line by platinum-based chemotherapy between 2011 and 2016, 266 were included. Median age was 57.0 (33–81). 187 pts (70.3%) harbored a BRCA1 mutation, and 75 (28.2%) a BRCA2 mutation. 66.9% of patients had FIGO stage III disease. Almost all patients (95.5%) underwent surgical resection, after neoadjuvant chemotherapy (48%), or primary debulking surgery (44.9%). 119 pts (44.7%) received maintenance therapy and majority with bevacizumab alone (78.9%). After a median follow-up of 51.7months, the median PFS (mPFS) was 28.6months [95 CI: 26.2–32.4]. Patients exhibited higher mPFS in case of FIGO stage III than stage IV (32.4 months and 25.4 months, respectively), or BRCA2 mutation than BRCA1 (33.3 months and 27.7 months, respectively). Although populations were non comparable, mPFS was close between patients treated with or without maintenance (28.3 and 29.5 months, respectively). Median PFS2 were 51.4 months and 50.4 months, respectively. Estimated 5-year OS for the whole population was 69.2% [95 CI: 65.3–73.0]. Conclusion This large ESME OC cohort described clinical features and real-life survival outcomes among pts with newly diagnosed advanced BRCA-mutated HGEOC treated in specialized centres. Disclosure This work was supported by UNICANCER. The ESME OC database is supported by an industrial consortium (Roche, Pfizer, AstraZeneca, MSD, Eisai and Daiichi Sankyo). Data collection, analyses and publications are totally managed by R&D UNICANCER independently of the industrial consortium. All the authors declare that there is no conflict of interest regarding the publication of this article. more...

International audience; BackgroundNETSARC (netsarc.org) is a network of 26 sarcoma reference centers with specialized multidisciplinary tumor boards (MDTB) aiming to improve the outcome of sarcoma patients. Since 2010, presentation to an MDTB and expert pathological review are mandatory for sarcoma patients nationwide. In the present work, the impact of surgery in a reference center on the survival of sarcoma patients investigated using this national NETSARC registry.Patients and methodsPatients’ characteristics and follow-up are prospectively collected and data monitored. Descriptive, uni- and multivariate analysis of prognostic factors were conducted in the entire series (N = 35 784) and in the subgroup of incident patient population (N = 29 497).ResultsAmong the 35 784 patients, 155 different histological subtypes were reported. 4310 (11.6%) patients were metastatic at diagnosis. Previous cancer, previous radiotherapy, neurofibromatosis type 1 (NF1), and Li–Fraumeni syndrome were reported in 12.5%, 3.6%, 0.7%, and 0.1% of patients respectively. Among the 29 497 incident patients, 25 851 (87.6%) patients had surgical removal of the sarcoma, including 9949 (33.7%) operated in a NETSARC center. Location, grade, age, size, depth, histotypes, gender, NF1, and surgery outside a NETSARC center all correlated to overall survival (OS), local relapse free survival (LRFS), and event-free survival (EFS) in the incident patient population. NF1 history was one of the strongest adverse prognostic factors for LRFS, EFS, and OS. Presentation to an MDTB was associated with an improved LRFS and EFS, but was an adverse prognostic factor for OS if surgery was not carried out in a reference center. In multivariate analysis, surgery in a NETSARC center was positively correlated with LRFS, EFS, and OS [P more...

Background NETSARC (netsarc.org) is a network of 26 sarcoma reference centers with specialized multidisciplinary tumor boards (MDTB) aiming to improve the outcome of sarcoma patients. Since 2010, presentation to an MDTB and expert pathological review are mandatory for sarcoma patients nationwide. In the present work, the impact of surgery in a reference center on the survival of sarcoma patients investigated using this national NETSARC registry. Patients and methods Patients’ characteristics and follow-up are prospectively collected and data monitored. Descriptive, uni- and multivariate analysis of prognostic factors were conducted in the entire series (N = 35 784) and in the subgroup of incident patient population (N = 29 497). Results Among the 35 784 patients, 155 different histological subtypes were reported. 4310 (11.6%) patients were metastatic at diagnosis. Previous cancer, previous radiotherapy, neurofibromatosis type 1 (NF1), and Li–Fraumeni syndrome were reported in 12.5%, 3.6%, 0.7%, and 0.1% of patients respectively. Among the 29 497 incident patients, 25 851 (87.6%) patients had surgical removal of the sarcoma, including 9949 (33.7%) operated in a NETSARC center. Location, grade, age, size, depth, histotypes, gender, NF1, and surgery outside a NETSARC center all correlated to overall survival (OS), local relapse free survival (LRFS), and event-free survival (EFS) in the incident patient population. NF1 history was one of the strongest adverse prognostic factors for LRFS, EFS, and OS. Presentation to an MDTB was associated with an improved LRFS and EFS, but was an adverse prognostic factor for OS if surgery was not carried out in a reference center. In multivariate analysis, surgery in a NETSARC center was positively correlated with LRFS, EFS, and OS [P more...

This population-based study aimed to describe the effects of neoadjuvant chemotherapy (NC) on survival in breast cancer (BC) patients in daily practice. BC patients treated with NC followed by surgery and radiotherapy, were retrospectively selected from 1982 to 2005 using the Cote d’Or BC registry. These patients were matched for the baseline AJCC (American Joint Committee on Cancer) stage, age at diagnosis, date of diagnosis and oestrogens receptors status to those who had undergone surgery followed by adjuvant chemotherapy and radiotherapy. The prognostic effect of NC on survival in BC patients was assessed with relative survival (RS) analyses. From 1982 to 2005, 210 patients with BC diagnosed in Cote d'Or were treated with NC followed by surgery and radiotherapy. For these patients, mean age at diagnosis was 50 (SD = 11). The main tumour characteristics were clinical AJCC stage 3 (46%) and an advanced Scarff Bloom and Richardson (SBR) stage (80%). Breast conserving surgery was performed in 84 patients (40%), 151 patients (72%) were treated with anthracyclines as the NC and the 5-year RS rate was 71%. Among these patients, 92 (37%) were matched. In this population, multivariate analyses showed that the use of NC did not independently influence RS: relative excess risk = 0.93 (0.50, 1.71). more...

Background Even though neoadjuvant chemotherapy has shown no benefits on overall survival (OS), it is being widely used in the treatment of breast cancer. This is based on the assumption that it may diminish the mastectomy rate and therefore be clinically relevant for patients. Our objective was to assess the impact of neoadjuvant chemotherapy on OS and on the rate of mastectomy in patients with non-metastatic primary operable breast carcinoma in routine practice. Methods The Cote d’Or district breast cancer registry was used to analyse the OS and mastectomy rate in patients with invasive primary operable unilateral breast cancer diagnosed between 1982 and 2006. We performed Cox proportional hazard ratio (HR) analyses for OS and multivariate logistic regression for the mastectomy rate for the overall population. Different matching methods based on the propensity score were used as sensitivity analyses to ensure that corrections for selection bias were adequate. Results We analysed 1578 patients, among whom 174 had received neoadjuvant chemotherapy. Median follow-up was 11.1 years. There was no difference between the two treatment groups for OS (HR = 1.08 (95% confidence interval (CI): 0.77–1.51 for neoadjuvant chemotherapy)). The mastectomy rate was higher among patients treated with neoadjuvant chemotherapy (odds ratio 1.54 (95% CI: 1.03–2.31)). Sensitivity analyses confirmed these results: for OS, there was no difference between the two populations precisely matched using propensity scores (HR 1.08; 95% CI: 0.671–1.65). Conclusion Despite long term follow-up, neoadjuvant chemotherapy provided no benefit for either OS or the mastectomy rate in our population. more...

Background: Resistance to trastuzumab is often observed in women with human epidermal growth factor receptor 2 (HER2)-positive breast cancer and has been shown to involve multiple potential mechanisms. We examined the ability of microarray analyses to determine the potential markers of pathological complete response (pCR). Methods: We conducted an analysis of tumours from 38 patients with locally advanced HER2-positive breast cancer who had received trastuzumab combined with docetaxel. Quantitative reverse transcriptase (RT)–PCR was used to assess the expression of 30 key genes; microarray analyses were carried out on 25 tumours to identify a prognostic gene expression profile, with 13 blinded samples used to validate the identified profile. Results: No gene was found to correlate with response by RT–PCR. The microarray analysis identified a gene expression profile of 28 genes, with 12 upregulated in the pCR group and 16 upregulated in non-pCR. The leave-one-out cross-validation test exhibited 72% accuracy, 86% specificity, and 55% sensitivity. The 28-gene expression profile classified the 13 validation samples with 92% accuracy, 89% specificity, and 100% sensitivity. Conclusion: Our results suggest that genes not involved in classical cancer pathways such as apoptosis or DNA repair could be involved in responses to a trastuzumab–docetaxel-based regimen. They also describe for the first time a gene expression signature that predicts trastuzumab response. more...

Pseudomyxoma peritonei is a rare disease, usually diagnosed after the discover of "jelly belly" by laparotomy. With the progress of immunohistochemistry, most authors now acknowledge the appendix to be the principal origin of this disease. Pseudomyxoma peritonei need to considered as border line malignant disease because of its inevitable persistence and progression without adapted therapeutic approach: cytoreductive surgery combined with perioperative intraperitoneal chemotherapy (intraperitoneal chemohyperthermia and/or immediate postoperative intraperitoneal chemotherapy) into specialized centres. The principal prognostic factors are the prior surgical history, the completeness of cytoreduction and especially the histopathologic grade. more...

Resume But de l'etude. – Proposer une attitude therapeutique en fonction de la presentation clinique des cancers differencies de la thyroide chez les patients de moins de 20 ans. Patients et methodes. – Entre 1995 et 2002, 74 patients de moins de 20 ans ont ete operes d'un cancer differencie de la thyroide. Les patients ont ete divises en deux groupes : 19 patients du groupe « ADP » qui presentaient des adenopathies detectees en preoperatoire (palpation et echographie) et 55 patients du groupe « sans ADP » chez qui aucune adenopathie n'avait ete detectee avant l'intervention. Resultats. – Les 19 patients du groupe « ADP » ont beneficie d'une thyroidectomie totale avec curage ganglionnaire. Dans le groupe « sans ADP », une thyroidectomie totale a ete realisee chez 19 patients (associee a un curage dans 7 cas) et une lobo-isthmectomie chez 29 patients. Une totalisation par iode radioactif a ete administree chez 25 patients (16 « ADP » et 9 « sans ADP »). Au cours du suivi (mediane 71 mois), neuf patients « ADP » ont ete operes pour recidive ganglionnaire (45 %). Quatre patients du groupe « sans ADP » ont ete reoperes, pour une recidive dans le lobe controlateral apres lobo-isthmectomie initiale (13 % des lobo-isthmectomies). La presence d'adenopathies decelables en preoperatoire est un facteur de risque de reintervention pour recidive ganglionnaire. La survie sans reintervention a cinq et dix ans etait respectivement de 58 et 38 % dans le groupe ADP et de 96 et 91 % dans le groupe « sans ADP » (p more...

Cutaneous metastases from hepatocellular carcinomas are rare, and their diagnosis may be difficult on histological grounds. We report a case of metastatic hepatocellular carcinoma to the skin that was confirmed immunohistochemically by the expression of a hepatomitochondria-specific antigen detectable on paraffin-embedded sections (Hep Par 1). more...

Study aim. – The aim of this retrospective study was to assess our experience of the laparoscopic surgery of pheochromocytoma .We report indications and results of laparoscopic and open adrenalectomy for pheochromocytoma. Patients and methods. – Between january 1994 and may 2002, 50 patients underwent laparoscopic or open adrenalectomy for pheocromocytoma. The perioperative hemodynamic parameters were assessed for each patient. In each case, urinary metanephrine levels were measured at the second month postoperatively. The long term outcome was assessed in 44 patients. The mean follow-up was 39 months. Results. – Ten patients underwent open adrenalectomy: 8 patients for unilateral tumors (tumor size was > 8cm in 7 cases) and 2 patients for bilateral tumors (1 recurrence and 1 cystic polylobed tumor). Fourty patients underwent laparoscopic adrenalectomy: in 32 cases, including 1 patient with a bilateral tumor, no conversion was performed (tumor size was < 5cm in 29 cases). In 8 cases (20%), a conversion to an open operation was performed. The reasons to convert were bleeding and periadrenal fibrosis in 7 cases. In laparoscopic adrenalectomy group, hemodynamic troubles were not more frequent, the hospital stay was shorter and there was no recurrence. Conclusion. – Laparoscopic adrenalectomy is the Gold standard procedure for patients with pheochromocytoma. But open adrenalectomy is sometimes indicated: tumor size > 8cm, periadrenal fibrosis, and recurrence tumor. more...

Resume But de l’etude : Les buts de notre etude retrospective etaient d’evaluer les facteurs pronostiques de recidive locoregionale chez les patients operes d’un corticosurrenalome malin et d’evaluer l’interet des reinterventions. Materiel et methode : De 1985 a 2001, 22 patients ont ete operes d’un corticosurrenalome malin. Nous avons etudie l’influence sur la survie sans recidive locoregionale du stade tumoral, du nombre de criteres de Weiss, de l’index mitotique, de l’exerese elargie aux organes de voisinage et du traitement par Op’DDD. Puis nous avons evalue l’interet des reinterventions. Resultats : Sept patients ont eu une recidive locoregionale apres un recul median de 25 mois et le taux de survie actuarielle sans recidive locoregionale a cinq ans etait de 50 %. Les patients dont la tumeur etait de stade I ou II, dont le nombre de criteres de Weiss etait ⩽ 6 ou dont l’index mitotique etaient ⩽ 20 mitoses/50 HPF avaient statistiquement une survie sans recidive locoregionale prolongee. L’exerese elargie aux organes de voisinage semblait diminuer le risque de recidive locoregionale. L’Op’DDD n’avait pas d’influence. Quatre patients ont eu une exerese iterative : deux sont vivants sans recidive 54 et 8 mois apres la reintervention et deux sont decedes, 19 et 25 mois apres la reintervention. Trois patients n’ont pas eu d’exerese iterative. Ils sont decedes dans les huit mois. Conclusion : Outre la moindre agressivite tumorale intrinseque (tumeurs de stade I et II, index mitotique faible), une meilleure prevention des recidives locoregionales peut reposer sur une exerese etendue en monobloc aux organes de voisinage. Les reinterventions peuvent prolonger la survie en cas d’exerese complete de la recidive. L’Op’DDD n’a pas fait la preuve de son efficacite. more...

Aim of the study: Primary hyperparathyroidism usually affects elderly patients. Juvenile primary hyperparathyroidism is rare, and raises diagnostic and pronostic problems. The aim of this retrospective study on 24 patients is to establish clinical, histological, and therapeutic features of juvenile primary hyperparathyroidism. Patients and methods: From 1986 to 2001, 673 patients were treated for primary hyperparathyroidism in our department. Twenty four patients were younger than 30 years old (3,5%). There were 14 women and 10 men. Mean age was 23 year (14–30). Clinical manifestations, pathologics findings and postoperative results were studied. Results: Sixteen patients presented a sporadic form of primary hyperparathyroidism with a single adenoma. Clinical manifestations were renal symptoms in 11 cases and acute hypercalcemia syndrome in 2 cases. Seven patients had a NEM I syndrome: parathyroid lesions were 6 hyperplasia and one adenoma. A 27 years old woman presented a recurrent familial isolated hyperparathyroidism. She was operated on 10 years before and at reoperation parathyroid carcinoma was found. Nineteen patients were cured after a post operative follow up ranging from 3 to 168 months. One patient had an asymptomatic hypercalcemia recurrence. Two patients presented permanent hypoparathyroidism treated whith calcitriol and calcium. Conclusion: Sporadic forms represent majority of cases of juvenile hyperparathyroidism. Renal manifestations are usual. Nevertheless, multiple endocrine neoplasia type 1 has to be evocated. more...

This population-based study aimed to describe the effects of neoadjuvant chemotherapy (NC) on survival in breast cancer (BC) patients in daily practice. BC patients treated with NC followed by surgery and radiotherapy, were retrospectively selected from 1982 to 2005 using the Côte d'Or BC registry. These patients were matched for the baseline AJCC (American Joint Committee on Cancer) stage, age at diagnosis, date of diagnosis and oestrogens receptors status to those who had undergone surgery followed by adjuvant chemotherapy and radiotherapy. The prognostic effect of NC on survival in BC patients was assessed with relative survival (RS) analyses. From 1982 to 2005, 210 patients with BC diagnosed in Côte d'Or were treated with NC followed by surgery and radiotherapy. For these patients, mean age at diagnosis was 50 (SD = 11). The main tumour characteristics were clinical AJCC stage 3 (46%) and an advanced Scarff Bloom and Richardson (SBR) stage (80%). Breast conserving surgery was performed in 84 patients (40%), 151 patients (72%) were treated with anthracyclines as the NC and the 5-year RS rate was 71%. Among these patients, 92 (37%) were matched. In this population, multivariate analyses showed that the use of NC did not independently influence RS: relative excess risk = 0.93 (0.50, 1.71). more...

We report a 9-year-old girl with a vulvar autoimmune bullous dermatosis. A diagnosis of localized bullous pemphigoid or cicatricial pemphigoid was made on the basis of immunohistologic data. Since the lesions were unresponsive to topical corticosteroids but healed completely on dapsone at a dosage of 1.5 mg/kg/day, we favor the diagnosis of vulvar cicatricial pemphigoid. Only two such cases have been reported thus far. The diagnostic criteria and therapeutic modalities are discussed. more...

Background: This prospective multicenter study assessed and compared the impact of different surgical procedures on quality of life (QoL) in breast cancer patients. Patients and methods: The EORTC QLQ-C30 and the EORTC QLQ-BR-23 questionnaires were used to assess global health status (GHS), arm (BRAS) and breast (BRBS) symptom scales, before surgery, just after surgery and 6 and 12 months later. The Kruskal–Wallis test with the Bonferroni correction was used to compare scores. A mixed model analysis of variance for repeated measurements was then applied to assess the longitudinal effect of surgical modalities on QoL. Results: Before surgery, GHS (P = 0.7807) and BRAS (P = 0.7688) QoL scores were similar whatever the surgical procedure: sentinel node biopsy (SLNB), axillary node dissection (ALND) or SLNB + ALND. As compared with other surgical groups, GHS 75.91 [standard deviation (SD) = 17.44, P = 0.041] and BRAS 11.39 (SD = 15.36, P < 0.0001) were better in the SLNB group 12 months after surgery. Whatever the type of surgery, GHS decreased after surgery (P < 0.0001), but increased 6 months later (P = 0.0016). BRAS symptoms increased just after surgery (P = 0.0329) and until 6 months (P < 0.0001) before decreasing (P < 0.0001). Conclusions: SLNB improved GHS and BRAS QoL in breast cancer patients. However, surgeons must be cautious, SLNB with ALND results in a poorer QoL. more...

Background Trastuzumab plus chemotherapy has become the standard of care for women with human epidermal growth factor receptor 2 (HER2)-positive metastatic breast cancer. Trastuzumab-based pre-operative systemic (neo-adjuvant) therapy (PST) also appears promising, warranting further investigation. Patients and methods Patients with HER2-positive, stage II/III non-inflammatory, operable breast cancer requiring a mastectomy (but who wished to conserve the breast) received weekly trastuzumab and 3-weekly docetaxel for six cycles before surgery. The primary end point was pathological complete response (pCR) rate, determined from surgical specimens. Results Thirty-three patients were enrolled. The majority (79%) had T2 tumors, with 42% being N1/2. Twenty-nine patients completed six cycles of therapy and one patient withdrew prematurely due to progressive disease. A complete or partial objective clinical response was seen in 96% (73% and 23%, respectively) of patients. Surgery was performed in 30 patients, breast conserving in 23 (77%). In an intention-to-treat analysis, tumor and nodal pCR was seen in 14 (47%) patients. Treatment was generally well tolerated. Grade 3/4 neutropenia occurred in 85% of patients while febrile neutropenia was encountered in 18%. Only three patients withdrew prematurely due to toxicity. No symptomatic cardiac dysfunction was reported. Conclusions PST with trastuzumab plus docetaxel achieved promising efficacy, with a high pCR rate and good tolerability, in women with stage II or III HER2-positive breast cancer. more...

Pseudomyxoma peritonei is a rare disease, usually diagnosed after the discover of "jelly belly" by laparotomy. With the progress of immunohistochemistry, most authors now acknowledge the appendix to be the principal origin of this disease. Pseudomyxoma peritonei need to considered as border line malignant disease because of its inevitable persistence and progression without adapted therapeutic approach: cytoreductive surgery combined with perioperative intraperitoneal chemotherapy (intraperitoneal chemohyperthermia and/or immediate postoperative intraperitoneal chemotherapy) into specialized centres. The principal prognostic factors are the prior surgical history, the completeness of cytoreduction and especially the histopathologic grade. more...

Cutaneous metastases from hepatocellular carcinomas are rare, and their diagnosis may be difficult on histological grounds. We report a case of metastatic hepatocellular carcinoma to the skin that was confirmed immunohistochemically by the expression of a hepatomitochondria-specific antigen detectable on paraffin-embedded sections (Hep Par 1). more...

Symptomatic zinc deficiency can occur in exclusively breast-fed infants. We report a case in a preterm infant.A 3-months-old exclusively breast-fed premature infant presented with peri-orificial and acral eczematoid lesions. Laboratory investigations revealed lowered zinc levels in the infant's serum and in her mother's milk. A rapid healing occurred after oral zinc supplementation.Zinc deficiency in breast-fed infants is a rare disease caused by a low level of zinc in mother's milk. The clinical features resemble those of acrodermatitis enteropathica. Oral zinc supplementation is required until weaning. more...

The aim of this retrospective study was to assess our experience of the laparoscopic surgery of pheochromocytoma. We report indications and results of laparoscopic and open adrenalectomy for pheochromocytoma.Between january 1994 and may 2002, 50 patients underwent laparoscopic or open adrenalectomy for pheocromocytoma. The perioperative hemodynamic parameters were assessed for each patient. In each case, urinary metanephrine levels were measured at the second month postoperatively. The long term outcome was assessed in 44 patients. The mean follow-up was 39 months.Ten patients underwent open adrenalectomy: 8 patients for unilateral tumors (tumor size was8 cm in 7 cases) and 2 patients for bilateral tumors (1 recurrence and 1 cystic polylobed tumor). Fourty patients underwent laparoscopic adrenalectomy: in 32 cases, including 1 patient with a bilateral tumor, no conversion was performed (tumor size was5 cm in 29 cases). In 8 cases (20%), a conversion to an open operation was performed. The reasons to convert were bleeding and periadrenal fibrosis in 7 cases. In laparoscopic adrenalectomy group, hemodynamic troubles were not more frequent, the hospital stay was shorter and there was no recurrence.Laparoscopic adrenalectomy is the Gold standard procedure for patients with pheochromocytoma. But open adrenalectomy is sometimes indicated: tumor size8 cm, periadrenal fibrosis, and recurrence tumor. more...

The aim of this retrospective study was to propose a therapeutic strategy according to clinic and pathologic presentations, in differentiated thyroid carcinoma in patients less than 20 years of age.From 1995 to 2002, 74 patients less than 20 years of age were operated on for a differentiated thyroid carcinoma. Patients were divided in two groups according to the presence of lymph node (LN) detected before operation (19 "with LN" and 55 "without LN").Surgery consisted of total thyroidectomy and lymph node dissection in the group "with LN". In the second group "without LN", total thyroidectomy was performed in 19 patients (associated with lymph node dissection in seven) and loboisthmectomy in 29 patients. Post operative radio iodine ablation was performed in 25 patients (16 "with LN" and nine "without LN"). During the follow up (median 71 months), 9/19 patients "with LN" underwent surgery for lymph node recurrence. Four patients of the "without LN" group were reoperated for recurrence in the controlateral thyroid lobe after initial loboisthmectomy. Initial presence of lymph nodes was a risk factor for reoperation. Survival without reoperation at 5 and 10 years was 58% and 38% for the "with LN" group and 96% and 91% for the "without LN" group respectively (P0.001). At the time of analysis, all patients were alive. Ninety percent of patients were in remission (68% of "with LN" and 98% of "without LN" patients).The risk of reoperation for lymph node recurrence during first post operative year is important in the "with LN" group. Therapeutic strategy consists of total thyroidectomy, complete lymph node dissection, radio iodine administration and l-thyroxine treatment. In "without LN" patients risk of lymph node recurrence is low. Treatment consist of total thyroidectomy, lymph node dissection and radio iodine administration are not systematic. more...

Aims Health-related quality of life (HRQoL) is a dynamic process which depends on the adaptation of the patient and reflected by a Response Shift (RS) effect. RS results in a recalibration, a reprioritization and a reconceptualization of key HRQoL domains. Longitudinal analyses of HRQoL have to take into account the possible occurrence of RS. However, there is no standard of statistical analysis to characterize RS. Two complementary methods are investigated to characterize RS. Methods This work builds on data of a prospective multicenter study including all primitive breast cancer patients or suspicion. HRQoL was evaluated using the EORTC QLQ-C30 and QLQ-BR23 at baseline, after surgery, at 3 months and 6 months, according to the « then-test/post-test » design: the retrospective assessments done after surgery and at 3 months refer to baseline HRQoL; the retrospective measurement done at 6 months refers to HRQoL at 3 months. The order then-test and post-test of HRQoL questionnaires was randomized. Recalibration was explored by Multiple Correspondence Analyses (MCA) and the Linear Logistic Model with Relaxed Assumptions (LLRA) of Item Response Theory (IRT). LLRA gives trend of item easiness parameters. Reprioritization and reconceptualization were explored by Principal Component Analyses (PCA). Results Between February 2006 and February 2008, 381 patients were included, 90% had a confirmed breast cancer. PCA show a secondary reprioritization of the QLQ-C30's dimensions. Fatigue and pain remain priority symptoms. Secondary symptoms are insomnia at baseline, diarrhea after surgery, nausea and vomiting at 3 months and 6 months. A stronger and stronger link between functional scales reflects a reconceptualization. Main recalibration's profiles reflected by MCA are from one modality to an adjacent one. A lower or upward recalibration of each dimension is reflected by the IRT model. Based on retrospective assessment at six months of HRQoL at three months, arm and breast symptoms were overestimated with trend parameters equal to -0.59 and -1.05 (p Conclusions IRT models have mainly been used to validate HRQoL questionnaires. This work shows their interest to characterize occurrence of RS. Further analyses should be lead to validate their abilities to characterize all RS components. Disclosure All authors have declared no conflicts of interest. more...

Background Time to quality of life (QoL) score deterioration (TD) is a method of longitudinal QoL data analysis that has been proposed for breast cancer (BC) patients (Hamidou et al Oncologist 2011). As for RECIST criteria, the optimal definitions dealing with reference should be explored. This study aims to study the impact of changes in internal standards (CIS) of response-shift (RS) and the influence of baseline QoL expectancies on TD. Methods A prospective multicenter study including all women hospitalized for a primary BC was conducted. The EORTC-QLQ-C30 and BR-23 questionnaires were used to assess the QoL at baseline, at the end of 1st hospitalization, and 3 and 6 months after. CIS was investigated by the then-test method. QoL expectancy was assessed at baseline using Likert scale. Deterioration was defined as a decrease in QoL scores reaching at least the mean difference identified as minimal clinically important difference (MCID) using Jaeschke's transition question. Sensitivity analyses were done using the then-test score as reference score, and considering 5 and 10 points as MCID. TD was estimated using Kaplan-Meier method. Cox regression analyses were used to identify factors influencing TD. Results From February 2006 to February 2008, 381 women were included. For role functioning dimension, the median TD increased from 3.2 months [95% CI: 3.1-3.36] to 4.76 months [3.3-6.2] when adjusting on CIS. For body image when adjusting on CIS, sentinel lymph node biopsy became significantly associated with longer TD (HR: 0.64[0.43-0.94]) as compared to axillary lymph node dissection, radiotherapy to a shorter TD (HR: 0.63[0.42-0.95] and the type of surgery had no effect on TD. For global health, cognitive and social functioning dimensions, patients expecting deterioration in their QoL had a significantly shorter TD. For fatigue and breast symptom scales, patients expecting no change had a significantly shorter TD, as compared to patients expecting an improvement. Sensitivity analyses using a MDCS of 5 or 10 confirmed these results. Conclusions Our results suggest that it would be more accurate to take into account CIS component of RS as well as QoL expectancies to estimate TD of QoL scores in patient with BC. Disclosure All authors have declared no conflicts of interest. more...

The aim of this retrospective study was to identify prognostic factors on local recurrence in patients with adrenocortical carcinoma and to assess the effect of reoperations.From 1985 to 2001, 22 patients were operated for adrenocortical carcinoma. We evaluated the correlation between actuarial survival without local recurrence and tumor staging, Weiss criteria, mitotic index, extensive resection and Op'DDD therapy by univariate analysis. Then we evaluated the effect of reoperations on survival.Local recurrence was observed in 7 patients and the 5-years actuarial survival without local recurrence was 50%. Tumor stage (I et II versus III et IV), Weiss criteria (or = 6 criteria versus6) and mitotic index (or = 20 mitoses/50 HPF versus20) affected survival without local recurrence. Extensive resection could reduce local recurrence rate. Op'DDD therapy was ineffective in prolonging survival without local recurrence. Four patients underwent repeat resections: 2 patients were still alive with disease free, 54 and 8 months after reoperations and 2 patients died, 19 and 25 months after reoperations. Three patients did not have repeat resection. They rapidly died within 8 months.Pathologic features (tumor staging and mitotic index) affected local recurrence prognostic. But extensive resection to adjacent organs could facilitate complete resection tumor and reduce the local recurrence rate. Complete repeat resection of local recurrence can improve survival. The disabling effects of Op'DDD were important and its efficacy was not clear. more...

Primary hyperparathyroidism usually affects elderly patients. Juvenile primary hyperparathyroidism is rare, and raises diagnostic and prognostic problems. The aim of this retrospective study on 24 patients is to establish clinical, histological, and therapeutic features of juvenile primary hyperparathyroidism.From 1986 to 2001, 673 patients were treated for primary hyperparathyroidism in our department. Twenty four patients were younger than 30 years old (3.5%). There were 14 women and 10 men. Mean age was 23 year (14-30). Clinical manifestations, pathologics findings and postoperative results were studied.Sixteen patients presented a sporadic form of primary hyperparathyroidism with a single adenoma. Clinical manifestations were renal symptoms in 11 cases and acute hypercalcemia syndrome in 2 cases. Seven patients had a NEM I syndrome: parathyroid lesions were 6 hyperplasia and one adenoma. A 27 years old woman presented a recurrent familial isolated hyperparathyroidism. She was operated on 10 years before and at reoperation parathyroid carcinoma was found. Nineteen patients were cured after a post operative follow up ranging from 3 to 168 months. One patient had an asymptomatic hypercalcemia recurrence. Two patients presented permanent hypoparathyroidism treated with calcitriol and calcium.Sporadic forms represent majority of cases of juvenile hyperparathyroidism. Renal manifestations are usual. Nevertheless, multiple endocrine neoplasia type 1 has to be evocated. more...

Familial hyperchylomicronemia is a rare autosomal recessive disease caused by lipoprotein lipase deficiency.A nine month-old girl presented with eruptive xanthomas revealing a familial hyperchylomicronemia. No lipoprotein lipase activity was found. DNA analysis revealed a novel homozygous non-sense mutation of the lipoprotein lipase gene at the codon 288. The parents were heterozygous carriers.Familial hyperchylomicronemia usually presents with eruptiva xanthomas, abdominal pain, pancreatic manifestation and lipemia retinalis. Papulo-nodular xanthomas occur more frequently in children as in our case. Eighty lipoprotein lipase gene mutations have been recorded to date. The gene locates on chromosome 8. Only 9 non-sense mutations have been described which lead to a truncated protein. In our case, no enzymatic activity was detected probably due to an absence of secretion of the enzyme, even though catalytic activity persisted. The homozygous carrier status leads to hyperchylomicronemia whereas the heterozygote status may lead to mixed hyperlipidemia with an increased risk of atherosclerosis. The screening of lipoprotein lipase gene mutations should be carried out in all families with hyperchylomicronemia, regardless of the presence or absence of xanthomas. more...

We report the case of a 69-year-old woman who presented a papular eruption on the eyelids. Histological features revealed a tuberculoid granuloma with a central caseating necrosis. Laboratory and radiological investigations revealed no tuberculosis and no systemic granulomatosis. Absence of vascular symptoms, inefficiency of cyclines and histopathological findings excluded granulomatous-type rosacea. Lupus miliaris disseminatus faciei (LMDF) was our final diagnosis. On the basis of our findings and a literature review, we believe that LMDF is an entity distinct from either skin tuberculosis or granulomatous-type rosacea. However, its name is confusing, and we propose to change it to 'facial idiopathic granulomas with regressive evolution (FIGURE)'. more...

The frequency of duodenal adenomas in patients with, familial adenomatous polyposis is high. Duodenal adenoma has malignant potential, and duodenal adenocarcinoma is one of the main causes of death in patients who have had previous proctocolectomy. A conservative approach to the treatment of duodenal adenomas (nonsteroidal anti-inflammatory drugs, endoscopy, polypectomy through duodenotomy) is inefficient and unsafe. When invasive cancer occurs in duodenal adenomas, the result of surgery is poor. We have performed prophylactic pancreaticoduodenal resection (PDR) for nonmalignant severe duodenal polyposis in five patients since 1991. No operative mortality was observed. One patient developed a pancreatic fistula which was successfully managed by medical treatment. The mean follow-up was 35 months. All five patients are still alive and have a good functional outcome. Prophylactic PDR may be indicated in familial adenomatous polyposis when duodenal polyposis is severe. Stages III and IV of Spigelman's classification, periampullary adenoma, age above 40, and family history of duodenal cancer are factors that may lead to the decision to perform prophylactic PDR. more...

16019 Background: IP epinephrine (E) has been shown to enhance the accumulation and antitumor activity of IP cisplatin in rats with peritoneal carcinomatosis (Duvillard et al. Int J Cancer, 1999). E induces a vasoconstriction of peritoneal vascular bed which limits platin peritoneal leakage and enhances drug penetration into tumor nodules. Results of a phase I study with the association of IP E and IP cisplatin in patient with refractory peritoneal carcinomatosis has been reported (Molucon-Chabrot et al, Anticancer Drugs, 2006). We conducted a phase I study to evaluate intraoperative IP chemotherapy with the association of cisplatin and escalated dose of E. Methods: 18 patients (pts) with recurrent ovarian cancer were enrolled. The median age was 56 years old (range 42–66). After an optimal cytoreductive surgery, IP chemotherapy was performed during surgery by filling twice the peritoneal cavity with 3 liters of an isotonic saline pre heated at 37°C and 90 mg of cisplatin associated with increasing concentrations of E during 1 hour. Results: E was escalated according to the following dosage: 0, 1, 2, 3 mg/L (3 pts by step). No toxicity was observed during and after intraoperative IP chemotherapy in the first 10 patients. The 2nd patient included in the 3 mg/L level of E experienced a tachycardia ≥ 120/min, ventricular extrasystoles (VES) ≥ 2/min, increase of troponine plasmatic concentration without significant electric sign of cardiac ischemia. The 3rd patient at this level experienced a tachycardia ≥ 120/min with ventricular VES ≥ 2/min and no increase of troponine concentration or electric sign of cardiac ischemia. According to the protocol design, 6 additional patients were included in the lower dose of 2 mg/L of E and no toxicity occurred. One can consider that the recommended dose of E was established. Conclusion: The combination of IP E with IP cisplatin in intraoperative chemotherapy after optimal cytoreductive surgery is feasible. The limiting toxicity was the cardiovascular effect which occurred with 3 mg/L dose of E. A phase II-III study is planned to investigate if IP chemotherapy adds to a second look surgery for the initial treatment of stage III ovarian carcinoma. No significant financial relationships to disclose. more...