Unilateral or bilateral adrenalectomy in PPNAD: six cases from a single family followed up over 40 years.

The most frequent endocrine Carney complex manifestation is a bilateral primary pigmented nodular adrenocortical disease and bilateral adrenalectomy (BA) is therefore its main treatment. In this study, a 40 years follow-up of six members of the same family with heterozygous PRKAR1A germline mutation, is reported over two generations. The first cases, two sisters with severe hyperandrogenism and Cushing syndrome (CS) diagnosed in 1972 at age 14 and 25, were successfully treated with unilateral adrenalectomy (UA). Their two brothers were then diagnosed, one with a CS-related severe osteoporosis treated with BA and the other with CS treated with UA. The second generation was diagnosed with CS signs at 7 and 21 years of age and were treated with BA and UA respectively. Out of the four patients treated with UA, the only event possibly related to CS was spontaneous episode of pulmonary embolism, 30 years after surgery. Hormonal evaluation revealed either eucortisolism in one patient or partial adrenal deficiency in two and mild hypercortisolism in one patient. For the two patients with BA, one of them accidentally died. The second one, surprisingly, recovered progressively normal cortisol secretion and circadian variation. Steroid substitution was stopped 6 years after her surgery and we demonstrated by iodocholesterol scintigraphy the presence of bilateral adrenal remnants. In conclusion, our results of long term evolution of PPNAD patients show that UA in this subset of patients could be considered to treat CS.

Time from first seen in specialist care to surgery does not influence survival outcome in patients with upfront resected pancreatic adenocarcinoma.

BACKGROUND: This study evaluated the impact of time to surgery (TTS) on overall survival (OS), disease free survival (DFS) and postoperative complication rate in patients with upfront resected pancreatic adenocarcinoma (PA). METHODS: We retrospectively included patients who underwent upfront surgery for PA between January 1, 2004 and December 31, 2014 from four French centers. TTS was defined as the number of days between the date of the first consultation in specialist care and the date of surgery. DFS for a 14-day TTS was the primary endpoint. We also analyzed survival depending on different delay cut-offs (7, 14, 28, 60 and 75 days). RESULTS: A total of 168 patients were included. 59 patients (35%) underwent an upfront surgery within 14 days. Patients in the higher delay group (> 14 days) had significantly more vein resections and endoscopic biliary drainage. Adjusted OS (p = 0.44), DFS (p = 0.99), fistulas (p = 0.41), hemorrhage (p = 0.59) and severe post-operative complications (p = 0.82) were not different according to TTS (> 14 days). Other delay cut-offs had no impact on OS or DFS. DISCUSSION: TTS seems to have no impact on OS, DFS and 90-day postoperative morbidity.

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.

What the radiologist should know about postoperative MDCT evaluation of a patient treated with cytoreductive surgery with or without hyperthermic intraperitoneal chemotherapy.

Cytoreductive surgery (CRS), often associated with hyperthermic intraperitoneal chemotherapy (HIPEC), is now a well-recognised treatment for most peritoneal malignancies in selected patients. As imaging is frequently performed postoperatively, radiologists are increasingly confronted with postoperative multidetector-row computed tomography (MDCT) examinations in these cases. In this article, after briefly describing the procedures that are currently being performed for the treatment of peritoneal metastases, the normal postoperative MDCT changes that may be encountered after these procedures are described. We then highlight complications that may arise after CRS, depending on the surgery performed, and those related to HIPEC, and illustrate their MDCT features.

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.

Abdominal tuberculosis in a low prevalence country.

OBJECTIVE: Abdominal tuberculosis is a rare disease. The clinical and radiological manifestations are non-specific and the diagnosis is difficult. Our objective was to describe the characteristics and treatment of patients presenting with abdominal tuberculosis in a low-incidence country. PATIENTS AND METHODS: We reviewed the clinical, diagnostic, treatment, and outcome features of patients presenting with abdominal tuberculosis diagnosed by bacteriological and/or histological results and managed in five French university hospitals from January 2000 to December 2009. RESULTS: We included 21 patients. The mean diagnostic delay was 13 months. Twelve patients (57%) came from a low-incidence area and only two had a known immunosuppressed condition. Eighteen patients (86%) presented with abdominal symptoms. The main organs involved were the peritoneum (n=14, 66%), the mesenteric lymph nodes (n=13, 62%), and the bowel (n=7, 33%). Sixteen patients (76%) underwent surgery, including two in an emergency setting. Seventeen patients (81%) received six months or more of anti-tuberculosis treatment. Finally, 16 patients (76%) had a positive outcome. CONCLUSION: New diagnostic procedures, and especially molecular biology, may help diagnose unusual clinical presentations of tuberculosis. Invasive procedures are frequently necessary to obtain samples but also for the treatment of digestive involvement.

Hepatic abscess: Diagnosis and management.

Microbial contamination of the liver parenchyma leading to hepatic abscess (HA) can occur via the bile ducts or vessels (arterial or portal) or directly, by contiguity. Infection is usually bacterial, sometimes parasitic, or very rarely fungal. In the Western world, bacterial (pyogenic) HA is most prevalent; the mortality is high approaching 15%, due mostly to patient debilitation and persistence of the underlying cause. In South-East Asia and Africa, amebic infection is the most frequent cause. The etiologies of HA are multiple including lithiasic biliary disease (cholecystitis, cholangitis), intra-abdominal collections (appendicitis, sigmoid diverticulitis, Crohn's disease), and bile duct ischemia secondary to pancreatoduodenectomy, liver transplantation, interventional techniques (radio-frequency ablation, intra-arterial chemo-embolization), and/or liver trauma. More rarely, HA occurs in the wake of septicemia either on healthy or preexisting liver diseases (biliary cysts, hydatid cyst, cystic or necrotic metastases). The incidence of HA secondary to Klebsiella pneumoniae is increasing and can give rise to other distant septic metastases. The diagnosis of HA depends mainly on imaging (sonography and/or CT scan), with confirmation by needle aspiration for bacteriology studies. The therapeutic strategy consists of bactericidal antibiotics, adapted to the germs, sometimes in combination with percutaneous or surgical drainage, and control of the primary source. The presence of bile in the aspirate or drainage fluid attests to communication with the biliary tree and calls for biliary MRI looking for obstruction. When faced with HA, the attending physician should seek advice from a multi-specialty team including an interventional radiologist, a hepatobiliary surgeon and an infectious disease specialist. This should help to determine the origin and mechanisms responsible for the abscess, and to then propose the best appropriate treatment. The presence of chronic enteric biliary contamination (i.e., sphincterotomy, bilio-enterostomy) should be determined before performing radio-frequency ablation and/or chemo-embolization; substantial stenosis of the celiac trunk should be detected before performing pancreatoduodenectomy to help avoid iatrogenic HA.

Genome-wide paternal uniparental disomy as a cause of Beckwith-Wiedemann syndrome associated with recurrent virilizing adrenocortical tumors.

Beckwith-Wiedemann syndrome (BWS) is an overgrowth syndrome characterized by fetal macrosomia, macroglossia, and abdominal wall defects. BWS patients are at risk to develop Wilms tumor, neuroblastoma, hepatoblastoma, and adrenal tumors. A young woman with BWS features, but with inconclusive genetic evidence for the disease, came to clinical observation for signs of virilization at the age of 16 years. An adrenocortical tumor was diagnosed and surgically resected. The tumor underwent 2 local relapses that were also surgically treated. The patient was also operated to remove a breast fibroadenoma. SNP arrays were used to analyze chromosome abnormalities in normal and tumor samples from the patient and her parents. The patient presented genome-wide mosaic paternal uniparental disomy (patUPD) both in the adrenocortical and the breast tumors, with different degrees of loss of heterozygosity (LOH). The more recent relapses of the adrenocortical tumor showed a loss of part of chromosome 17p that was absent in the first tumor. Analysis of a skin biopsy sample also showed mosaic patUPD with partial LOH, while no LOH was detected in leukocyte DNA. This case shows that virilizing adrenocortical tumors may be a clinical feature of patients with BWS. The SNP array technology is useful to diagnose genome-wide patUPD mosaicism in BWS patients with an inconclusive molecular diagnosis and underlines the tumorigenic potential of the absence of the maternal genome combined with an excess of the paternal genome.

Practice patterns in complex ventral hernia repair and place of biological grafts: a national survey among French digestive academic surgeons.

BACKGROUND: Despite the prevalence of complex ventral hernias, there is little agreement on the most appropriate technique or prosthetic to repair these defects, especially in contaminated fields. Our objective was to determine French surgical practice patterns among academic surgeons in complex ventral hernia repair (CVHR) with regard to indications, most appropriate techniques, choice of prosthesis, and experience with complications. METHODS: A survey consisting of 21 questions and 6 case-scenarios was e-mailed to French practicing academic surgeons performing CVHR, representing all French University Hospitals. RESULTS: Forty over 54 surgeons (74%) responded to the survey, representing 29 French University Hospitals. Regarding the techniques used for CVHR, primary closure without reinforcement was provided in 31.6% of cases, primary closure using the component separation technique without mesh use in 43.7% of cases, mesh positioned as a bridge in 16.5% of cases, size reduction of the defect by using aponeurotomy incisions without mesh use in 8.2% of cases. Among the 40 respondents, 36 had experience with biologic mesh. There was a strong consensus among surveyed surgeons for not using synthetic mesh in contaminated or dirty fields (100%), but for using it in clean settings (100%). There was also a strong consensus between respondents for using biologic mesh in contaminated (82.5%) or infected (77.5%) fields and for not using it in clean setting (95%). In clean-contaminated surgery, there was no consensus for defining the optimal therapeutic strategy in CVHR. Infection was the most common complication reported after biologic mesh used (58%). The most commonly reported influences for the use of biologic grafts included literature, conferences and discussion with colleagues (85.0%), personal experience (45.0%) and cost (40.0%). CONCLUSIONS: Despite a lack of level I evidence, biologic meshes are being used by 90% of surveyed surgeons for CVHR. Importantly, there was a strong consensus for using them in contaminated or infected fields and for not using them in clean setting. To better guide surgeons, prospective, randomized trials should be undertaken to evaluate the short- and long-term outcomes associated with these materials in various surgical wound classifications.

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.