Survival of patients managed in France for duodenal neuroendocrine tumors (D-NET): a 20-year multicenter cohort study from the GTE group: a cohort study.

INTRODUCTION: Duodenal neuroendocrine tumours (D-NETs) have a low incidence; however, their diagnosis has been increasing. Features such as tumour location, size, type, histological grade, and stage were used to adapt the treatment to either endoscopic (ER) or surgical (SR) resections. There is no consensus regarding the definitive treatment. The authors' study aimed to describe the management of non-metastatic, well-differentiated D-NETs in France and its impact on patient survival. METHODS: A registry-based multicenter study using prospectively collected data between 2000 and 2019, including all patients managed for non-metastatic G1 and G2 D-NETs, was conducted in the GTE group. RESULTS: A total of 153 patients were included. Fifty-eight benefited from an ER, and 95 had an SR. No difference in recurrence-free survival (RFS) was observed regardless of treatment type. There was no significant difference between the two groups (ER vs. SR) in terms of location, size, grade, or lymphadenopathy, regardless of the type of incomplete resection performed or regarding the pre-therapeutic assessment of lymph node invasion in imaging. The surgery allowed for significantly more complete resection (patients with R1 resection in the SR group: 9 vs. 14 in the ER group, P <0.001). Among the 51 patients with positive lymph node dissection after SR, tumour size was less than or equal to 1 cm in 25 cases. Surgical complications were more numerous ( P =0.001). In the sub-group analysis of G1-G2 D-NETs between 11 and 19 mm, there was no significant difference in grade ( P =0.977) and location ( P =0.617) between the two groups (ER vs. SR). No significant difference was found in both morphological and functional imaging, focusing on the pre-therapeutic assessment of lymph node invasion ( P =0.387). CONCLUSION: Regardless of the resection type (ER or SR) of G1-G2 non-metastatic D-NETs, as well as the type of management of incomplete resection, which was greater in the ER group, long-term survival results were similar between ER and SR. Organ preservation seems to be the best choice owing to the slow evolution of these tumours.

[Dramatic efficacy of chemotherapy with 5-fluorouracil and dacarbazine in a patient with metastatic glucagonoma and cardiac insufficiency]. / Efficacité spectaculaire d'une chimiothérapie par 5-fluoro-uracile et dacarbazine chez un malade atteint de glucagonome métastatique avec insuffisance cardiaque.

Malignant glucagonoma is an exceptional pancreatic endocrine tumour, with frequent dermatologic symptoms, diabetes and degradation of the general health status. Prognosis is unfavourable when liver metastases are present due to the usual inefficiency of chemotherapy. We report here an observation of a patient who was treated for a glucagonoma with multiple liver metastases, migratory necrolytic erythema, dilated cardiomypathy and diabetes that dramatically improved after a dacarbazin-based chemotherapy, allowing subsequent surgical resection of the primary. The patient was still alive and asymptomatic without progressive disease nearly two years after surgery.

[Complete resolution of insulin-dependent diabetes mellitus in a patient with autoimmune pancreatitis following treatment with corticosteroids]. / Disparition totale d'un diabète insulinorequérant au décours d'une pancréatite auto-immune traitée par corticothérapie.

We report here the observation of a 60-year-old man who had an autoimmune pancreatitis revealed by a jaundice, in whom an insulin-treated diabetes mellitus, which was diagnosed one month before, completely resolved after the administration of prednisolone given to treat this symptomatic pancreatitis. Neither the symptoms, nor the diabetes have relapsed after a 3-year follow-up.

Morphological imaging and CT histogram analysis to differentiate pancreatic neuroendocrine tumor grade 3 from neuroendocrine carcinoma.

PURPOSE: To compare morphological imaging features and CT texture histogram parameters between grade 3 pancreatic neuroendocrine tumors (G3-NET) and neuroendocrine carcinomas (NEC). MATERIALS AND METHODS: Patients with pathologically proven G3-NET and NEC, according to the 2017 World Health Organization classification who had CT and MRI examinations between 2006-2017 were retrospectively included. CT and MRI examinations were reviewed by two radiologists in consensus and analyzed with respect to tumor size, enhancement patterns, hemorrhagic content, liver metastases and lymphadenopathies. Texture histogram analysis of tumors was performed on arterial and portal phase CT images. images. Morphological imaging features and CT texture histogram parameters of G3-NETs and NECs were compared. RESULTS: Thirty-seven patients (21 men, 16 women; mean age, 56±13 [SD] years [range: 28-82 years]) with 37 tumors (mean diameter, 60±46 [SD] mm) were included (CT available for all, MRI for 16/37, 43%). Twenty-three patients (23/37; 62%) had NEC and 14 patients (14/37; 38%) had G3-NET. NECs were larger than G3-NETs (mean, 70±51 [SD] mm [range: 18 - 196mm] vs. 42±24 [SD] mm [range: 8 - 94mm], respectively; P=0.039), with more tumor necrosis (75% vs. 33%, respectively; P=0.030) and lower attenuation on precontrast (30±4 [SD] HU [range: 25-39 HU] vs. 37±6 [SD] [range: 25-45 HU], respectively; P=0.002) and on portal venous phase CT images (75±18 [SD] HU [range: 43 - 108 HU] vs. 92±19 [SD] HU [range: 46 - 117 HU], respectively; P=0.014). Hemorrhagic content on MRI was only observed in NEC (P=0.007). The mean ADC value was lower in NEC ([1.1±0.1 (SD)]×10-3 mm2/s [range: (0.91 - 1.3)×10-3 mm2/s] vs. [1.4±0.2 (SD)]×10-3 mm2/s [range: (1.1 - 1.6)×10-3 mm2/s]; P=0.005). CT histogram analysis showed that NEC were more heterogeneous on portal venous phase images (Entropy-0: 4.7±0.2 [SD] [range: 4.2-5.1] vs. 4.5±0.4 [SD] [range: 3.7-4.9]; P=0.023). CONCLUSION: Pancreatic NECs are larger, more frequently hypoattenuating and more heterogeneous with hemorrhagic content than G3-NET on CT and MRI.

Time intervals to diagnosis and chemotherapy do not influence survival outcome in patients with advanced pancreatic adenocarcinoma.

BACKGROUND: The effect of treatment delay on survival in pancreatic ductal adenocarcinoma (PDAC) remains unclear. AIMS: This study aimed to assess the prognostic impact of time to diagnosis and chemotherapy in advanced PDAC and factors influencing the time intervals. METHODS: advanced PDAC patients receiving chemotherapy in five centers in the decade 2007-2016 were included. Key time points during care pathway from clinical presentation to beginning of chemotherapy were retrospectively collected. Multivariate Cox proportional hazard model was performed. RESULTS: A total of 409 patients were included (mean age 66.1 ± 10.3 years; 250 metastatic (61%); 139 received FOLFIRINOX chemotherapy (34%). The median overall survival (OS) was 7.2 months. The median times from first symptoms and from first specialist visit to the beginning of chemotherapy were respectively 100 days and 47 days. None of time intervals was significantly associated with OS. Significant prognostic factors were FOLFIRINOX chemotherapy (HR 0.6 [0.5-0.8]; P < 0.001), metastasis (HR 1.6 [1.3-2.0]; P = 0.001), WHO PS ≥ 2 (HR 1.6 [1.2-2.1]; P < 0.001) and acute pancreatitis as first symptom (HR 2.9 [1.7-4.9]; P < 0.001). Jaundice shortened time to diagnosis (P < 0.001). Acute pancreatitis (P < 0.001) and diabetes (P = 0.01) increased time to treatment. CONCLUSION: Wait times from clinical presentation to beginning of chemotherapy do not influence survival in advanced PDAC.

Sporadic pancreatic neuroendocrine tumor: Surgery of the primary tumor.

The management of patients with sporadic pancreatic neuroendocrine tumors (PNET) is multi-disciplinary and often, multimodal. Surgery has a large part in treatment because it is the only potentially curative therapeutic modality if resection can be complete. The update reviews the operative indications and the different surgical techniques available (including parenchymal-sparing surgery) to treat the primary lesion according to patient status, preoperative work-up and whether the tumor is functioning or not. The place of observation for "small" non-functional sporadic PNET is also discussed.

Preoperative imaging and pathologic classification for pancreatic neuroendocrine tumors.

The management of patients with pancreatic neuroendocrine tumor (PNET), whether hormonally secretory or not, is multidisciplinary and often multimodal. Surgical treatment plays a central role because complete resection is the only potentially curative treatment. The choice of the therapeutic plan for a PNET requires precise localization of the primary tumor (which may sometimes be multiple in case of genetic predisposition), confirmation of the diagnosis of PNET, a search for metastases (mainly hepatic), and identification of the main histoprognostic factors. This update focuses on the WHO 2017 histological classification and recent innovations in the preoperative assessment of PNET using conventional and isotopic imaging. The aim is to not only allow the mapping of primary and metastatic lesions but also to predict tumor aggressiveness.

Open retromuscular large mesh reconstruction of lumbar incisional hernias including the atrophic muscular area.

PURPOSE: Lumbar incisional hernias (LIH) are a rare wall defect, whose surgical management is challenging because no recommendation exists. Moreover, LIH are frequently associated with flank bulging which should be taken into account during LIH surgical repair. We aimed to describe a cohort of patients operated on for LIH using a homogeneous surgical technique and to report surgical outcomes. METHODS: The records of all consecutive patients operated on in a specialized surgical center between January 2009 and January 2015 were retrospectively reviewed. The same open technique was performed, i.e., using a mesh into the retroperitoneal space posteriorly, placed with the largest overlap inferiorly and posteriorly, and fixed through the controlateral abdominal wall muscles under strong tension to correct the flank bulging. RESULTS: The cohort included 31 patients, of median age 62, who presented two or more comorbidities in 68% of cases. LIH was recurrent in 45% of patients, and was related to nephrectomy in 61% of patients. The mesh was totally extraperitoneal in 65% of patients. The postoperative mortality rate was null. The rate of specific surgical complications was 32.3%, and the rate of overall postoperative morbidity (Clavien-Dindo classification) was 38.7%. After a median follow-up of 27.5 months, the recurrence rate was 6.5% and 9.7% reported chronic pain. CONCLUSION: The open approach for LIH repair was safe and enabled treating flank bulging simultaneously in all patients. Due to the paucity of adequate scientific studies, this reproducible open method could help moving toward a standardization of LIH surgical management.

[Digestive neuroendocrine tumors]. / Tumeurs neuroendocrines digestives.

Digestive neuroendocrine tumors (NETs) are a group of rare tumors with increasing incidence. Pathological analysis is critical to establish the diagnosis and evaluate tumor grade that relies on differentiation and proliferation index. NETs are mostly diagnosed at an advanced stage because of late occurrence of nonspecific symptoms, and can be associated with hormone hypersecretion. Chromogranin A is the main biochemical marker of NETs. Extension workup relies on conventional imaging (CT-scan, MRI) and isotopic imaging including somatostatin-receptor scintigraphy, which should be soon replaced by positron-emitting scintigraphy. The main prognostic factors include tumor stage, metastatic volume, histological differentiation and grade. Hormonal syndromes and poorly differentiated tumors are the two therapeutic emergencies. The treatment of localized well-differentiated tumors relies on endoscopic or surgical resection depending on the location and aggressiveness. Surgical removal is the only potentially curative treatment of metastatic NETs but is rarely feasible and is associated with almost constant relapse. Other antitumor therapies include somatostatin analogs, systemic chemotherapy, liver trans-arterial chemo-embolization, targeted therapies and peptide-receptor radionuclide therapy. Management strategy relies on primary tumor location, tumor aggressiveness, metastatic volume and the presence of extra-hepatic metastases. It must take into account the risk of cumulated toxicity in patients whose survival is often prolonged.

Is primary tumor resection associated with a longer survival in colon cancer and unresectable synchronous metastases? A 4-year multicentre experience.

AIM: To explore the survival impact of primary tumor resection (PTR) in patients with metastatic colon cancer (mCC) and unresectable metastases. METHODS: We retrospectively studied a multicenter cohort of consecutive mCC patients with unresectable metastases receiving first-line chemotherapy. A weighted Cox proportional regression model was used to balance for clinical variables associated with the probability of undergoing PTR, using inverse probability of treatment weighting (IPTW) based on a propensity score. RESULTS: Ninety-six patients were included. PTR was performed in 69 (72%). The rates of secondary resection of metastases (p = 0.02) and bevacizumab administration (p = 0.02) were higher in the PTR group. Raw median overall survival (OS) was 23.1 months (95%CI[14.6-27.8]) in the PTR group and 22.1 months (95%CI[12.3-23.7]) in the non-PTR group (p = 0.11). After adjustment on IPTW, OS was 23.1 months (95%CI[17.0-28.7]) in the PTR group and 17.2 months (95%CI[13.5-22.2]) in the non-PTR group (HR 0.68; 95%CI[0.50-0.93]; p = 0.016). This result remained significant on multivariate analysis (HR 0.71; 95%CI[0.50-1.00]; p = 0.05). CONCLUSION: In mCC patients with unresectable metastases receiving chemotherapy, up-front PTR was independently associated with prolonged OS. Patients eligible for secondary metastases resection and/or bevacizumab may benefit the most from PTR. Randomized controlled trials are mandatory.

Laparoscopic thermal ablation of splenic metastases initial experience and present aspects.

UNLABELLED: Splenic metastases develop in less than 1% of all metastatic cancers, and typically occur in a setting of disseminated disease. When isolated splenic metastasis occurs, the patient may be a candidate for aggressive treatment consisting mainly of splenectomy as described in the literature. However, the increased incidence of post-operative morbidity and severe infection after splenectomy are well known. We report a case of splenic metastasis that developed from colorectal cancer and was treated by laparoscopic-guided radiofrequency ablation. We reviewed the few reported cases of splenic metastasis (from colorectal and other primary cancers) treated by thermal ablation using radiofrequency (RF) or microwave (MW) energy sources. DISCUSSION: Many studies have proved that thermal ablation for benign splenic pathology is both feasible and safe with no sacrifice in efficacy. However only a few cases of MW or RF treatment of splenic secondary tumor have been described; no complications have been reported with this treatment in contrast to the 15 to 27% morbidity rate for splenectomy. CONCLUSION: When treatment of splenic metastasis is proposed with curative intent, thermal ablation by RF or by MW seems to be a feasible and safe technique resulting in spleen conservation with a low morbidity rate. Because of these features, thermal ablation seems an ideal treatment modality to obliterate splenic metastasis and may be an indispensable tool in the armamentarium of modern splenic surgery.

Gastric juvenile polyposis with high-grade dysplasia in pachydermoperiostosis.

Pachydermoperiostosis (PDP) is the primary form of hypertrophic osteoarthropathy. It is a very rare disease consisting of pachydermia, digital clubbing and radiologic periostosis. Various digestive symptoms in PDP are seen in 11-49% of patients and juvenile polyps may be found at gastric endoscopy. We report here the history of a patient with PDP who was referred for assessment of severe anemia. Endoscopy of the upper digestive tract showed multiple polyps of the stomach with two huge lesions exhibiting foci of high-grade dysplasia. This observation suggests that PDP can be considered as a precancerous condition of the stomach and systematic screening using endoscopy should be considered in these patients.