[Phaeochromocytoma and paraganglioma]. / Phéochromocytome et paragangliome.

Phaeochromocytomas and paragangliomas (PPGL) are rare neuroendocrine tumors that arise from the adrenal medulla or sympathetic and parasympathetic ganglia. These tumors produce most often catecholamines in excess, causing hypertension and sometimes severe acute cardiovascular complications. The diagnosis is based on plasma or urines metanephrines measurements and on conventional and nuclear medicine imaging. Catecholamines-producing PPGL is very unlikely if levels are normal. The diagnosis of PPGL cannot be made without visualization of a tumor. Therapeutic management consists mostly of surgical excision, after drug preparation, and should be done in referral centers. About 40% of pheochromocytomas and paragangliomas occur in the context of an autosomal inherited syndrome, making genetic testing essential. The follow-up must be prolonged because a metastatic evolution or a recurrence can be observed in about 15% of the cases.

Bowel perforation in type IV vascular Ehlers-Danlos syndrome. A systematic review.

Spontaneous gastrointestinal (GI) perforation is a well-known complication occurring in patients suffering from Type IV vascular Ehlers-Danlos syndrome (EDS IV). The aim of the present study was to review the current literature on spontaneous GI perforation in EDS IV and illustrate the surgical management and outcome when possible. A systematic review of all the published data on EDS IV patients with spontaneous GI perforation between January 2000 and December 2015 was conducted using three major databases PUBMED, EMBASE, and Cochrane Central Register of Controlled Trails. References of the selected articles were screened to avoid missing main articles. Twenty-seven published case reports and four retrospective studies, including 31 and 527 cases, respectively, matched the search criteria. A case from our institution was added. Mean age was 26 years (range 6-64 years). The most frequent site of perforation was the colon, particularly the sigmoid, followed by small bowel, upper rectum, and finally stomach. The majority of cases were initially managed with Hartmann's procedure. In recurrent perforations, total colectomy was performed. The reperforation rate was considerably higher in the "partial colectomy with anastomosis" group than in the Hartmann group. Colonic perforation is the most common spontaneous GI perforation in EDS IV patients. An unexpected fragility of the tissues should raise the possibility of a connective tissue disorder and prompt further investigation with eventual management of these high-risk patients with a multidisciplinary team approach in dedicated centres. In the emergency setting, a Hartmann procedure should be performed.

Technical difficulties of left colic artery preservation during left colectomy for colon cancer.

PURPOSE: Low-tie ligation in colorectal cancer surgery is associated with technical difficulties in left colic artery preservation. We aimed to evaluate and classify the anatomical and technical difficulties of left colic artery (LCA) preservation at its origin and along its route at the inferior border of the pancreas. METHODS: A vascular reconstruction computed tomography prospective series of 113 patients was analyzed. The inferior mesenteric artery (IMA) branching pattern according to Latarjet's classification (Type I, separate LCA origin, Type II, fan-shaped branching pattern) and the distances between the IMA and the LCA origins and between the LCA and the Inferior mesenteric vein (IMV) at the inferior border of the pancreas were measured. RESULTS: The IMA branching pattern was Type I in 80 (71 %) patients and Type II in 33 (29 %) patients. The IMA-LCA distance was 39.8 ± 12.2 mm. The LCA-IMV distance at the inferior border of the pancreas was 20.5 ± 21.7 mm. When classified based on this distance, 75 (66 %) patients were classified into the Near subgroup (<20 mm) (7.7 ± 4.1 mm) and 38 (34 %) into the Far subgroup (≥20 mm) (45.6 ± 20.4 mm, p < 0.001). A Type I subgroup F accounted for 27 % of the patients. CONCLUSIONS: Left colic artery preservation is highly feasible at its origin in more than two-thirds of cases due to the separate origin. The addition of a high IMV ligation increases the risk of damage to the LCA at the inferior border of the pancreas because the distance to the IMV is less than 20 mm in two-thirds of cases.

Professional risks when carrying out cytoreductive surgery for peritoneal malignancy with hyperthermic intraperitoneal chemotherapy (HIPEC): A French multicentric survey.

BACKGROUND: Over the last two decades, many surgical teams have developed programs to treat peritoneal carcinomatosis with extensive cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (HIPEC). Currently, there are no specific recommendations for HIPEC procedures concerning environmental contamination risk management, personal protective equipment (PPE), or occupational health supervision. METHODS: A survey of the institutional practices among all French teams currently performing HIPEC procedures was carried out via the French network for the treatment of rare peritoneal malignancies (RENAPE). RESULTS: Thirty three surgical teams responded, 14 (42.4%) which reported more than 10 years of HIPEC experience. Some practices were widespread, such as using HIPEC machine approved by the European Community (100%), individualized or centralized smoke evacuation (81.8%), "open" abdominal coverage during perfusion (75.8%), and maintaining the same surgeon throughout the procedure (69.7%). Others were more heterogeneous, including laminar flow air circulation (54.5%) and the provision of safety protocols in the event of perfusate spills (51.5%). The use of specialized personal protective equipment is ubiquitous (93.9%) but widely variable between programs. CONCLUSION: Protocols regarding cytoreductive surgery/HIPEC and the associated professional risks in France lack standardization and should be established.

Isolated hepatic perfusion: principles and results.

INTRODUCTION: Isolated hepatic perfusion allows the delivery of high dose chemotherapy while decreasing extra-hepatic toxicity, and is used mainly for patients with surgically unresectable liver tumors. PRINCIPLES: Vascular exclusion of the liver is performed after obtaining satisfactory hemodynamic tolerance, occasionally after cavocaval shunt and/or porto-systemic shunt. Perfusion entry can be arterial and/or portal while the exit is portal or caval. The perfusion circuit can be open or closed, using a circulation pump with or without oxygenation. The chemotherapy regimens used are high dose melphalan (with or without TNF-alpha), oxaliplatin, cisplatin and mitomycin, sometimes associated with moderate hyperthermia. The duration of perfusion ranges between 30 and 90 minutes according to the different protocols used. A percutaneous technique with incomplete liver vascular exclusion is also possible. RESULTS: The larger series in the literature show a response rate (partial or complete stabilization) between 60 and 80%, with approximately 5% complete morphologic responses. Morbidity and mortality are 40 and 5%, respectively, including specific morbidity related to the perfusion procedure as well as to chemotherapy. CONCLUSION: Chemotherapy delivered through isolated hepatic perfusion is a new therapeutic alternative, still under development, and can be proposed to patients with surgically unresectable primary or secondary liver tumors within clinical trials. These results seem to be promising, but are still associated with a non-negligible morbidity rate.

Real time shear waves elastography monitoring of thermal ablation: in vivo evaluation in pig livers.

BACKGROUND: Thermal ablation is a widely used minimally invasive treatment modality for different cancers. However, lack of a real-time imaging system for accurate evaluation of the procedure is one of the reasons of local recurrences. Shear waves elastography (SWE) is a new ultrasound (US) imaging modality to quantify tissue stiffness. The aim of the study was to assess the feasibility and accuracy of US elastography for quantitative monitoring of thermal ablation and to determine the elasticity threshold predictive of coagulation necrosis. METHODS: A total of 29 in vivo thermal lesions were performed in pig livers with radiofrequency system. SWE and B-mode images were acquired simultaneously. Liver elasticity was quantified by using SWE data and expressed in kilopascal. After the procedure, pathologic analysis of treated tissues was compared with US images. The sensitivity and positive predictive value of the SWE maps of tissue elasticity were calculated and compared with the boundaries of the pale coagulation necrosis areas found at pathology. RESULTS: The liver mean elasticity values before and after thermal therapy were 6.4 ± 0.3 and 38.1 ± 2.5 kPa, respectively (P < 0.0001). For a threshold of 20 kPa, sensitivity (i.e., the rate of pixels correctly detected as necrosed tissue) was 0.8, and the positive predictive value (i.e., the rate of pixels in the elastographic map >20 kPa that actually developed coagulation necrosis) was 0.83. CONCLUSIONS: Tissue areas with coagulation necrosis are significantly stiffer than the surrounding tissue. SWE permits the real-time detection of coagulation necrosis produced by radiofrequency and could potentially be used to monitor US-guided thermal ablation.

Laparoscopic omega-loop gastric bypass for the conversion of failed sleeve gastrectomy: early experience.

OBJECTIVES: Despite the initial effectiveness of sleeve gastrectomy (SG), some patients who undergo this purely restrictive technique have inadequate weight loss or renewed weight gain and persistent obesity-related co-morbidities with their potentially lethal complications. In such patients, the conversion of SG by the addition of a malabsorptive technique may then be necessary. PATIENTS AND METHODS: Conversion of SG to a mini gastric bypass (MGBP) was evaluated for failure of weight loss. An ante-colic end-to-side stapled gastro-jejunal anastomosis was performed laparoscopically, connecting the long narrow gastric tube to the jejunum at a point 200cm downstream from the ligament of Treitz. RESULTS: Between October 2006 and February 2012, 651 laparoscopic MGBP were performed for morbid obesity. Twenty-three of these patients (3.5%) had previously undergone SG. The conversion from SG to MGPB was performed laparoscopically in 19 of the 23 patients (81%) at a mean interval of 26.3months (8.2-63.7). The 30-day postoperative mortality rate was zero and the morbidity rate was 9.5%. The mean BMI before MGBP was 44±7.7kg (35.8-55.4). Conversion of SG to MGBP resulted in additional weight loss, achieving a mean BMI of 39.9 with a 26.8% loss of excess BMI (EBL) at 3months, mean BMI of 36.5 with 37.2% EBL at 12months, mean BMI of 36.2 with 48.6% EBL at 18months, and mean BMI of 35.7 with EBL of 51.6% at 24months. The overall mean EBL was 57.3±19.5% (range: 25-82%) at 42.3months (range 16.7-60.8months). CONCLUSION: Conversion of SG to MGBP is feasible, safe and effective, and results in significant additional weight loss. Definitive results at 2 and 5years are awaited for the long-term procedure validation.

Platelet dysfunction associated with the novel Trp29Cys thromboxane A2 receptor variant.

BACKGROUND: Genetic variations that affect the structure of the thromboxane A2 receptor (TP receptor) provide insights into the function of this key platelet and vascular receptor, but are very rare in unselected populations. OBJECTIVES: To determine the functional consequences of the TP receptor Trp29Cys (W29C) substitution. PATIENTS/METHODS: We performed a detailed phenotypic analysis of an index case (P1) with reduced platelet aggregation and secretion responses to TP receptor pathway activators, and a heterozygous TP receptor W29C substitution. An analysis of the variant W29C TP receptor expressed in heterologous cells was performed. RESULTS: Total TP receptor expression in platelets from P1 was similar to that of controls, but there was reduced maximum binding and reduced affinity of binding to the TP receptor antagonist [(3) H]SQ29548. HEK293 cells transfected with W29C TP receptor cDNA showed similar total TP receptor expression to wild-type (WT) controls. However, the TP receptor agonist U46619 was less potent at inducing rises in cytosolic free Ca(2+) in HEK293 cells expressing the W29C TP receptor than in WT controls, indicating reduced receptor function. Immunofluorescence microscopy and cell surface ELISA showed intracellular retention and reduced cell surface expression of the W29C TP receptor in HEK293 cells. Consistent with the platelet phenotype, both maximum binding and the affinity of binding of [(3) H]SQ29548 to the W29C TP receptor were reduced compared to WT controls. CONCLUSION: These findings extend the phenotypic description of the very rare disorder TP receptor deficiency, and show that the W29C substitution reduces TP receptor function by reducing surface receptor expression and by disrupting ligand binding.

Hyperthermic isolated liver perfusion with melphalan and bevacizumab.

Whereas surgical resection is the only curative treatment for liver tumors, effective treatment for isolated unresectable lesions when there is tumor progression in spite of several lines of chemotherapy remains to be found. We report herein two cases of patients treated by a 1-hour Hyperthermic Isolated Liver Perfusion (HILP) with a combination of melphalan and bevacizumab leading to complete response. The first patient had liver metastases secondary to previously resected malignant glucagonoma and the second, recurrent hepatocellular carcinoma. We used bevacizumab in association with melphalan for HILP because of the additional effect of an anti-VEGF antibody in these highly vascularized tumors and its locally restricted delivery to the isolated hepatic vascular compartment despite of its classic contraindication in association with surgery. The protocol was approved by the Ethics Committee. Enhanced CT scans during follow-up showed complete tumor necrosis as early as the second postoperative day. Patients had 27 and 7 months disease-free survival and 48 and 41 months overall survival after HILP, for neuroendocrine liver metastases and HILP plus liver transplantation for HCC respectively. Under very specific conditions, bevacizumab in HILP can provide excellent tumor response in hopeless clinical cases of liver tumors.

Outcomes of adrenalectomy in patients with unilateral primary aldosteronism: a review.

Aldosterone hypersecretion in primary aldosteronism is unilateral (aldosterone producing adenoma and primary unilateral hyperplasia) or bilateral (idiopathic adrenal hyperplasia). Laparoscopic adrenalectomy is nowadays the preferred approach to treat patients with unilateral primary aldosteronism. We review the outcomes of this intervention in recently published series. Laparoscopic adrenalectomy has a morbidity of 5-14%, mortality below 1%, and a mean hospital stay around 3 days. It generally results in the normalization of aldosterone secretion and in a large decrease of blood pressure and antihypertensive medication, but normotension without treatment is only achieved in 42% of all cases. Normotension following adrenalectomy is more likely in young and lean women with recent low grade hypertension than in obese men with long-standing high grade hypertension or a family history of hypertension. However, individual prediction of the blood pressure outcome is not accurate and predictors of hypertension cure should not be used to select patients for surgery. Age, associated health conditions and preferences of the patient are more relevant to this end.

[Not Available]. / Implications immunologiques potentielles du curage ganglionnaire : Exemple du cancer colorectal.

F. Pagès, A. Berger, F. Zinzindohoué, A. Kirilovsky, J. Galon, W.-H. Fridman Lymph node dissection is an integral part of the surgical resection of colon cancers; it completes the wide regional resection of tumor and it allows prognostic evaluation through accurate staging. Studies have demonstrated an immune reaction to the tumoral site which attests to an ongoing dialog between the tumor and systemic defenses. The regional lymph nodes constitute an important first line of immune defense where initial host response is initiated or, inversely, they may participate in a local state of immunosuppression. This article reviews current knowledge on intra-tumoral and nodal immune status in colorectal cancers and attempts to evaluate the potential immunologic implications of lymph node dissection.

[Not Available]. / Implications immunologiques potentielles du curage ganglionnaire : Exemple du cancer colorectal.

F. Pagès, A. Berger, F. Zinzindohoué, A. Kirilovsky, J. Galon, W.-H. Fridman Lymph node dissection is an integral part of the surgical resection of colon cancers; it completes the wide regional resection of tumor and it allows prognostic evaluation through accurate staging. Studies have demonstrated an immune reaction to the tumoral site which attests to an ongoing dialog between the tumor and systemic defenses. The regional lymph nodes constitute an important first line of immune defense where initial host response is initiated or, inversely, they may participate in a local state of immunosuppression. This article reviews current knowledge on intra-tumoral and nodal immune status in colorectal cancers and attempts to evaluate the potential immunologic implications of lymph node dissection.

[Potential immunologic consequences of lymph node dissection in colorectal cancer]. / Implications immunologiques potentielles du curage ganglionnaire: Exemple du cancer colorectal.

Lymph node dissection is an integral part of the surgical resection of colon cancers; it completes the wide regional resection of tumor and it allows prognostic evaluation through accurate staging. Studies have demonstrated an immune reaction to the tumoral site which attests to an ongoing dialog between the tumor and systemic defenses. The regional lymph nodes constitute an important first line of immune defense where initial host response is initiated or, inversely, they may participate in a local state of immunosuppression. This article reviews current knowledge on intra-tumoral and nodal immune status in colorectal cancers and attempts to evaluate the potential immunologic implications of lymph node dissection.

Auxiliary liver transplantation for acute liver failure.

BACKGROUND: In patients with acute liver failure (ALF) who fulfil criteria, liver transplantation is the only effective treatment which can substitute metabolic and excretory function of the liver. Auxiliary liver transplantation was developed because a significant minority of patients with ALF who fulfil transplant criteria can have a complete morphological and functional recovery of their liver. The favourable outcome reported in European series using auxiliary partial orthotopic liver transplantation (APOLT), the greater experience as well as the lessons from split liver and from living related donors have revived interest in this approach. In selected patients aged <40 years without haemodynamic instability, the use of ABO-compatible, non-steatotic grafts harvested from young donors with normal liver function can restore liver function and prevent the occurrence of irreversible brain damage. In the majority of cases the auxiliary graft is a right graft which is placed orthotopically after a right hepatectomy in the recipient. After standard immunosuppression, the recovery of the native liver is assessed by biopsies, hepatobiliary scintigraphy and computed tomography. When, on the basis of histological, scintigraphical and morphological data, there is evidence of sufficient regeneration of the native liver, immunosuppression can be discontinued progressively. Complete regeneration of the native liver can be observed in >50% of patients, who can be withdrawn from immunosuppression. Therefore the advantages of auxiliary transplantation seem to balance favourably with the potential inconvenience of this technique in selected patients.

Prolonged improvement of total pancreatic allograft function by previous intrathymic bone marrow cell injection in rats.

Donor-specific induction of tolerance was previously achieved in the diabetic rat by intrathymic injection of pancreatic islets. It allowed a secondary islet graft in any site without immunosuppression. Since total pancreatic graft in man is metabolically more proficient than islet graft, we attempted tolerance induction for total vascularized pancreas transplantation in diabetic BN recipient rats by an intrathymic bone marrow cell (BMC) injection from Lewis donor rats, associated to an antilymphocyte antibody (ALS) administration. Control groups consisted of isogenic grafts, allogenic grafts without tolerance induction and allogenic grafts with ALS alone. In all grafted groups, mean blood glucose and plasma insulin were normalised within 24 h. Graft rejection (clinically suggested by diabetes recurrence and later confirmed by histology) appeared at 18 +/- 2 postoperative days in the absence of intrathymic BMC injection and at 36 +/- 8 days in the group with BMC injection (p < 0.05). Intrathymic bone marrow graft was successful in delaying rejection in our study.

Adjustable gastric banding in a public university hospital: prospective analysis of 400 patients.

BACKGROUND: Laparoscopic application of an adjustable gastric band (LAGB) is considered the least invasive surgical option for morbid obesity. It has the advantage of being potentially reversible and can improve quality of life. METHOD: Between April 1997 and January 2001, 400 patients underwent LAGB. There were 352 women and 48 men with mean age 40.2 years (16-66). Preoperative mean body weight was 119 kg (85-195) and mean body mass index (BMI) was 43.8 kg/m2 (35.1-65.8). RESULTS: Mean operative time was 116 minutes (30-380), and mean hospital stay was 4.55 days (3-42). There was no death. There were 12 conversions (3%). 40 complications required an abdominal reoperation (10%), for perforation (n = 2), gastric necrosis (n = 1), slippage (n = 31), incisional hernia (n = 2) and reconnection of the tube (n = 4). We noticed 7 pulmonary complications (2 ARDS, 5 atelectasis) and 30 minor problems related to the access port. At 2 years, mean BMI had fallen from 43.8 to 32.7 kg/m2 and mean excess weight loss (EWL) was 52.7% (12-94). CONCLUSION: LAGB is a very beneficial operation with an acceptable complication rate. EWL is 50% at 2 years if multidisciplinary follow-up remains assiduous. Surveillance for late anterior stomach slippage within the band is essential.