Pole des Maladies de lAppareil Digestif PMAD

Paris, France

Pole des Maladies de lAppareil Digestif PMAD

Paris, France
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Irtan S.,University of Paris Descartes | Galmiche-Rolland L.,University of Paris Descartes | Elie C.,Necker Enfants Malades Hospital | Orbach D.,University Pierre and Marie Curie | And 14 more authors.
Pediatric Blood and Cancer | Year: 2016

Background: Solid pseudopapillary neoplasms of the pancreas (SPPN) can relapse very late, but little is known about risk factors for recurrence and optimal treatment. We aimed to identify risk factors for recurrence and to analyze treatment modalities in all French pediatric cases of SPPN over the past 20 years. Material and methods: Data were collected from pediatric oncologists and surgeons, and also from adult pancreatic surgeons in order to identify late recurrences. Results: Fifty-one patients (41 girls) were identified. Median age at diagnosis was 13.1 years [8.7–17.9]. Abdominal pain was the commonest presenting symptom (32/49, 65%). The tumor was located in the pancreatic head in 24 patients (47%). Preoperative biopsy or cytology was performed in 14 cases (28%). All patients were operated with a median of 23 days [0–163] after diagnosis. The rate of postoperative morbidity was 29%. With a median follow-up of 65 months [0.3–221], the overall and event-free survival was 100% and 71%, respectively. Seven patients (13.7%) relapsed with a median of 43 months [33–94] after initial surgery. Six were treated surgically, either alone (n = 3) or with perioperative chemotherapy (n = 2) or hyperthermic intraperitoneal chemotherapy (n = 1). One patient in whom further treatment was not feasible was still alive at last news. Risk factors for recurrence were positive surgical margins (P = 0.03) and age less than 13.5 years at diagnosis (P = 0.03). Conclusions: SPPN recurrence in this pediatric series was a rare and late event that did not undermine overall survival. Complete surgical removal of recurrent tumors appears to be the best option. © 2016 Wiley Periodicals, Inc.


PubMed | Gustave Roussy Cancer Campus, Necker Enfants Malades Hospital, Institute Leon Berard, University Hospital and 10 more.
Type: Journal Article | Journal: Pediatric blood & cancer | Year: 2016

Solid pseudopapillary neoplasms of the pancreas (SPPN) can relapse very late, but little is known about risk factors for recurrence and optimal treatment. We aimed to identify risk factors for recurrence and to analyze treatment modalities in all French pediatric cases of SPPN over the past 20 years.Data were collected from pediatric oncologists and surgeons, and also from adult pancreatic surgeons in order to identify late recurrences.Fifty-one patients (41 girls) were identified. Median age at diagnosis was 13.1 years [8.7-17.9]. Abdominal pain was the commonest presenting symptom (32/49, 65%). The tumor was located in the pancreatic head in 24 patients (47%). Preoperative biopsy or cytology was performed in 14 cases (28%). All patients were operated with a median of 23 days [0-163] after diagnosis. The rate of postoperative morbidity was 29%. With a median follow-up of 65 months [0.3-221], the overall and event-free survival was 100% and 71%, respectively. Seven patients (13.7%) relapsed with a median of 43 months [33-94] after initial surgery. Six were treated surgically, either alone (n = 3) or with perioperative chemotherapy (n = 2) or hyperthermic intraperitoneal chemotherapy (n = 1). One patient in whom further treatment was not feasible was still alive at last news. Risk factors for recurrence were positive surgical margins (P = 0.03) and age less than 13.5 years at diagnosis (P = 0.03).SPPN recurrence in this pediatric series was a rare and late event that did not undermine overall survival. Complete surgical removal of recurrent tumors appears to be the best option.


Buc E.,Pole des Maladies de lAppareil Digestif PMAD | Couvelard A.,AP HP | Kwiatkowski F.,Center Jean Perrin | Dokmak S.,Pole des Maladies de lAppareil Digestif PMAD | And 4 more authors.
European Journal of Surgical Oncology | Year: 2014

Background: Lymph node (LN) invasion in pancreatic ductal adenocarcinoma (PDAC) is the most important prognostic factor after surgical resection. The mechanisms of LN invasion include lymphatic spreading and/or direct extension from the main tumor. However, few studies have assessed the impact of these different patterns of invasion on prognosis. Patients and methods: Pathologic reports of pancreatic resections for PDAC from 1997 to 2007 were retrospectively analyzed. The mode of LN invasion was defined as follows: standard lymphatic metastases (S), contiguous from the main tumor (C) and standard with extracapsular invasion (EI). Clinical outcomes were compared according to the mode of invasion and the number of invaded LN. Results: 306 patients were reviewed. Median age at resection was 61 years (range, 34-81). Eighty seven patients were N-(28.9%) and 214 were N+ (71.1%). Of the N+ patients, 195 (91.1%) were S+, 35 (16.3%) were C+, and 24 (12.3% of the S+ patients) were EI+. Median survival in N+ patients was lower than in N-patients (29 vs. 57 months, p < 0.001). In patients without standard involvement, C+ patients (n = 19) had worse survival than C-patients (n = 47) (34 vs. 57 months, p = 0.037). In S+ patients, C status was correlated with prognosis when the number of LN S+ was <2 (p = 0.07). EI status had no influence on prognosis. On multivariate analysis, only perineural invasion (p = 0.02) and LN ratio (p = 0.042) were independent prognostic factors. Conclusion: Direct invasion of LN by the tumor is predictive of reduced survival, but has little impact compared to standard LN involvement and perineural invasion. © 2014 Elsevier Ltd. All rights reserved.


PubMed | Pole des Maladies de lAppareil Digestif PMAD, Center Jean Perrin and AP HP
Type: Historical Article | Journal: European journal of surgical oncology : the journal of the European Society of Surgical Oncology and the British Association of Surgical Oncology | Year: 2014

Lymph node (LN) invasion in pancreatic ductal adenocarcinoma (PDAC) is the most important prognostic factor after surgical resection. The mechanisms of LN invasion include lymphatic spreading and/or direct extension from the main tumor. However, few studies have assessed the impact of these different patterns of invasion on prognosis.Pathologic reports of pancreatic resections for PDAC from 1997 to 2007 were retrospectively analyzed. The mode of LN invasion was defined as follows: standard lymphatic metastases (S), contiguous from the main tumor (C) and standard with extracapsular invasion (EI). Clinical outcomes were compared according to the mode of invasion and the number of invaded LN.306 patients were reviewed. Median age at resection was 61 years (range, 34-81). Eighty seven patients were N- (28.9%) and 214 were N+ (71.1%). Of the N+ patients, 195 (91.1%) were S+, 35 (16.3%) were C+, and 24 (12.3% of the S+ patients) were EI+. Median survival in N+ patients was lower than in N- patients (29 vs. 57 months, p < 0.001). In patients without standard involvement, C+ patients (n = 19) had worse survival than C- patients (n = 47) (34 vs. 57 months, p = 0.037). In S+ patients, C status was correlated with prognosis when the number of LN S+ was <2 (p = 0.07). EI status had no influence on prognosis. On multivariate analysis, only perineural invasion (p = 0.02) and LN ratio (p = 0.042) were independent prognostic factors.Direct invasion of LN by the tumor is predictive of reduced survival, but has little impact compared to standard LN involvement and perineural invasion.


Gaujoux S.,Pole des Maladies de lAppareil Digestif PMAD | Cortes A.,Pole des Maladies de lAppareil Digestif PMAD | Couvelard A.,Pole des Maladies de lAppareil Digestif PMAD | Noullet S.,Pole des Maladies de lAppareil Digestif PMAD | And 6 more authors.
Surgery | Year: 2010

Background: Pancreatic fistula (PF) after pancreatoduodenectomy (PD) remains a challenging problem. The only commonly accepted risk factor is the soft consistency of the pancreatic remnant. Methods: In all, 100 consecutive patients underwent PD. All data, including commonly accepted risk factors for PF and PF defined according to the International Study Group of Pancreatic Fistula, were collected prospectively. On the pancreatic margin, a score of fibrosis and a score of fatty infiltration were assessed by a pathologist blinded to the postoperative course. Results: PF occurred in 31% of patients. In univariate analysis, male sex, age greater than 58 years, body mass index (BMI) ≥25 kg/m2, pre-operative high blood pressure, operation for nonintraductal papillary and mucinous neoplasm (IPMN) disease and for ampullary carcinoma, operative time, blood loss, soft consistency of the pancreatic remnant, absence of pancreatic fibrosis, and presence of fatty infiltration of the pancreas were associated with a greater risk of PF. In a multivariate analysis, only BMI ≥25kg/m2, absence of pancreatic fibrosis, and presence of fatty pancreas were significant predictors of PF. A score based on the number of risk factors present divided the patient population into 4 subgroups carrying a risk of PF that ranged from 7% (no risk factor) to 78% (3 risk factors) and from 0% to 81%, taking into account only symptomatic PF (grade B and C). Conclusion: The presence of an increased BMI, the presence of fatty pancreas, and the absence of pancreatic fibrosis as risk factors of PF allows a more precise and objective prediction of PF than the consistency of pancreatic remnant alone. A predictive score based on these 3 factors could help to tailor preventive measures. © 2010.


Cherif R.,Pole des Maladies de lAppareil Digestif PMAD | Gaujoux S.,Pole des Maladies de lAppareil Digestif PMAD | Gaujoux S.,University Paris Diderot | Couvelard A.,University Paris Diderot | And 7 more authors.
Journal of Gastrointestinal Surgery | Year: 2012

Background: Parenchyma-sparing pancreatectomy (PSP), including enucleation and central pancreatectomy, has been investigated as an alternative to standard resection for pancreatic endocrine neoplasm, but the benefit/risk of these procedures remains little known. Methods: From 1998 to 2010, among 197 patients operated for well-differentiated pancreatic neuroendocrine tumors, 67 underwent PSP (45 enucleations and 22 central pancreatectomies) and 66 standard resections (35 pancreaticoduodenectomies and 31 distal pancreatectomies) for a tumor below 4 cm, without synchronous distant metastasis. Groups were compared regarding postoperative morbidity, mortality, long-term pancreatic function, and survival calculated using the Kaplan-Meier method. Results: Tumors operated by PSP had a median size of 15 mm, were mainly incidentally diagnosed (n = 46, 69 %), and nonfunctioning (n = 55, 82 %). Overall morbidity rate was higher after PSP than standard resection (SR) (76 vs 58 %, p = 0.0028), including more frequent pancreatic fistulas (69 vs 42 %, p = 0.003). Postoperative diabetes was less frequent following PSP than pancreaticoduodenectomy (5 vs 21 %; p = 0.022) but equivalent to the one observed after distal pancreatectomy (4 %, p = 1). Exocrine insufficiency was significantly less frequent after PSP than SR (3 vs 32 %; p < 0.0001). The overall and recurrence-free 5-year survival after PSP for nonfunctioning tumors was 96 and 98 %, respectively. Conclusion: In selected patients, with small and low-grade tumors, PSP are associated with excellent overall and recurrence-free survivals. These procedures are associated with an increased postoperative morbidity but an excellent postoperative pancreatic function. Therefore, they should be considered as a valid therapeutic option in selected well-differentiated pancreatic neuroendocrine tumors. © 2012 The Society for Surgery of the Alimentary Tract.


Treton X.,Pole des maladies de l'appareil digestif PMAD
Colon and Rectum | Year: 2015

During the last 15 years, numerous progresses in the field of IBD were made, and the practice has evolved significantly. Treatment of IBD has been revolutionized by the advent of anti-TNF, based on their ability to achieve mucosal healing, in contrast with previous treatments. Moreover, the learning of their use, of their side effects and of the way to develop new strategies in combination, has also changed our practices. At the same time, treatment goals have become more ambitious, with the need to obtain, when possible and reasonable, mucosal control of the disease. This was possible by the development of endoscopical techniques but also with non-invasive tools like MRI and fecal calprotectin. Finally, new treatments arrive or will soon be available, such as anti-integrin, anti-JAK kinases and cell therapy. This will need to prioritize their use, and to identify new individual factors of responses to develop a personalized medicine for IBD. © 2015, Springer-Verlag France.


Birnbaum D.J.,Pole des Maladies de lAppareil Digestif PMAD | Gaujoux S.,Pole des Maladies de lAppareil Digestif PMAD | Gaujoux S.,University Paris Diderot | Gaujoux S.,French Institute of Health and Medical Research | And 14 more authors.
Surgery (United States) | Year: 2014

Background. Sporadic nonfunctioning pancreatic neuroendocrine tumors (NF-PNETs) are increasingly diagnosed as incidentalomas, and their resection is usually recommended. The prognostic significance of this diagnosis feature is poorly studied, and management of these tumors remains controversial. Clinical, pathologic characteristics and outcome of resected incidentally diagnosed NF-PNET (Inc) were compared with resected symptomatic NF-PNET (Symp) to better assess their biologic behavior and tailor their management. Methods. From 1994 to 2010, 108 patients underwent resection for sporadic nonmetastatic NF-PNET. Diagnosis was considered as incidental in patients with no abdominal symptoms or symptoms unlikely to be related to tumor mass. Patients with Inc were compared with patients with Symp, regarding demographics, postoperative course, pathology, and disease-free survival (DFS). Results. Of the 108 patients, 65 (61%) had incidentally diagnosed tumors. Pancreas-sparing pancreatectomies (enucleation/central pancreatectomy) were performed more frequently in Inc (62% vs 30%, P = .001). Inc tumors were more frequently <20 mm (65% vs 42%, P = .019), staged T1 (62% vs 33%, P = .0001), node negative (85% vs 60%; P = .005), and grade 1 (66% vs 33%, P = .0001). One postoperative death occurred in the Inc group, and postoperative morbidity was similar between the two groups (60% vs 65%, P = .59). DFS was substantially better in the Inc group (5-year DFS = 92% vs 82%, P = .0016). Conclusion. Incidentally diagnosed NF-PNETs are associated with less aggressive features compared with symptomatic lesions but cannot always be considered to be benign. Operative resection remains recommended for most. Incidentally diagnosed NF-PNET may be good candidates for pancreas-sparing pancreatectomies. © 2014 Mosby, Inc. All rights reserved.


PubMed | Pole des Maladies de lAppareil Digestif PMAD
Type: Journal Article | Journal: Journal of gastrointestinal surgery : official journal of the Society for Surgery of the Alimentary Tract | Year: 2012

Parenchyma-sparing pancreatectomy (PSP), including enucleation and central pancreatectomy, has been investigated as an alternative to standard resection for pancreatic endocrine neoplasm, but the benefit/risk of these procedures remains little known.From 1998 to 2010, among 197 patients operated for well-differentiated pancreatic neuroendocrine tumors, 67 underwent PSP (45 enucleations and 22 central pancreatectomies) and 66 standard resections (35 pancreaticoduodenectomies and 31 distal pancreatectomies) for a tumor below 4 cm, without synchronous distant metastasis. Groups were compared regarding postoperative morbidity, mortality, long-term pancreatic function, and survival calculated using the Kaplan-Meier method.Tumors operated by PSP had a median size of 15 mm, were mainly incidentally diagnosed (n=46, 69 %), and nonfunctioning (n=55, 82 %). Overall morbidity rate was higher after PSP than standard resection (SR) (76 vs 58 %, p=0.0028), including more frequent pancreatic fistulas (69 vs 42 %, p=0.003). Postoperative diabetes was less frequent following PSP than pancreaticoduodenectomy (5 vs 21 %; p=0.022) but equivalent to the one observed after distal pancreatectomy (4 %, p=1). Exocrine insufficiency was significantly less frequent after PSP than SR (3 vs 32 %; p<0.0001). The overall and recurrence-free 5-year survival after PSP for nonfunctioning tumors was 96 and 98 %, respectively.In selected patients, with small and low-grade tumors, PSP are associated with excellent overall and recurrence-free survivals. These procedures are associated with an increased postoperative morbidity but an excellent postoperative pancreatic function. Therefore, they should be considered as a valid therapeutic option in selected well-differentiated pancreatic neuroendocrine tumors.


PubMed | Pole des Maladies de lAppareil Digestif PMAD
Type: Journal Article | Journal: Surgery | Year: 2010

Pancreatic fistula (PF) after pancreatoduodenectomy (PD) remains a challenging problem. The only commonly accepted risk factor is the soft consistency of the pancreatic remnant.In all, 100 consecutive patients underwent PD. All data, including commonly accepted risk factors for PF and PF defined according to the International Study Group of Pancreatic Fistula, were collected prospectively. On the pancreatic margin, a score of fibrosis and a score of fatty infiltration were assessed by a pathologist blinded to the postoperative course.PF occurred in 31% of patients. In univariate analysis, male sex, age greater than 58 years, body mass index (BMI) > or =25 kg/m(2), pre-operative high blood pressure, operation for nonintraductal papillary and mucinous neoplasm (IPMN) disease and for ampullary carcinoma, operative time, blood loss, soft consistency of the pancreatic remnant, absence of pancreatic fibrosis, and presence of fatty infiltration of the pancreas were associated with a greater risk of PF. In a multivariate analysis, only BMI > or =25 kg/m(2), absence of pancreatic fibrosis, and presence of fatty pancreas were significant predictors of PF. A score based on the number of risk factors present divided the patient population into 4 subgroups carrying a risk of PF that ranged from 7% (no risk factor) to 78% (3 risk factors) and from 0% to 81%, taking into account only symptomatic PF (grade B and C).The presence of an increased BMI, the presence of fatty pancreas, and the absence of pancreatic fibrosis as risk factors of PF allows a more precise and objective prediction of PF than the consistency of pancreatic remnant alone. A predictive score based on these 3 factors could help to tailor preventive measures.

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