Malignant behavior of a well­differentiated digestive neuroendocrine tumor with peritoneal carcinomatosis: A case report.

Neuroendocrine neoplasms (NENs) are a heterogenous group of tumors, arising from enterochromaffin cells, with different biological and clinical characteristics. Well-differentiated Grade 1 (G1) small intestinal NENs are often characterized by a slow progression rate and a good prognosis. Peritoneal carcinomatosis of a G1 digestive NEN is not a very common finding, and thus there is little published evidence regarding its progression and management. The complex, multistage interplay between the peritoneum and the metastasizing neuroendocrine cells is not well understood, and a reliable predictive tool to identify these patients earlier in their disease course is lacking. The present study describes the case of a 68-year-old woman presenting with an oligosymptomatic, stage IV, small intestinal G1 NEN (pTxpN1pM1) with synchronous liver metastases, multifocal mesenteric tumor deposits and a low Ki67 labeling index (1%). Over a period of 15 months, the patient developed rapidly progressive peritoneal metastatic disease with repetitive self-limiting obstructive symptoms and eventually succumbed to her illness. The present case report discusses the potential relationship between low-grade NEN, location of the primary tumor and the metastatic site, and also speculates on the role of the underlying subcellular mechanisms, specific micro-environment, spreading modalities and therapeutic strategy.

Safety of very high-power short-duration radiofrequency ablation for pulmonary vein isolation: a two-centre report with emphasis on silent oesophageal injury.

AIMS: Very high-power short-duration (vHPSD) via temperature-controlled ablation (TCA) is a new modality to perform radiofrequency pulmonary vein isolation (PVI), conceivably at the cost of a narrower safety margin towards the oesophagus. In this two-centre trial, we aimed to determine the safety of vHPSD-based PVI with specific emphasis on silent oesophageal injury. METHODS AND RESULTS: Ninety consecutive patients with atrial fibrillation (AF) underwent vHPSD-PVI (90 W, 3-4 s, TCA) using the QDOT MICRO catheter, in conjunction with the nGEN (Bad Neustadt, n = 45) or nMARQ generator (Bruges, n = 45). All patients underwent post-ablation oesophageal endoscopy. Procedural parameters and complications were recorded. A subgroup of 21 patients from Bad Neustadt underwent cerebral magnetic resonance imaging (cMRI) to detect silent cerebral events (SCEs). Mean age was 67 ± 9 years, 59% patients were male, and 66% patients had paroxysmal AF. Pulmonary vein isolation was obtained in all cases after 96 ± 29 min. No steam pop, cardiac tamponade, stroke, or fistula was reported. None of the 90 patients demonstrated oesophageal ulceration (0%). Charring was not observed in the nMARQ cohort (0% vs. 11% in the nGEN group). In 5 out of 21 patients (24%), cMRI demonstrated SCE (exclusively nGEN cohort). CONCLUSION: Temperature-controlled vHPSD catheter ablation allows straightforward PVI without evidence of oesophageal ulcerations or symptomatic complications. Catheter tip charring and silent cerebral lesions when using the nGEN generator have led to further modification.

Endoscopic evaluation of the esophagus after catheter ablation of atrial fibrillation using contiguous and optimized radiofrequency applications.

BACKGROUND: The incidence of endoscopically detected esophageal lesions after pulmonary vein isolation (PVI) is as high as 18%. Intraesophageal temperature rise (ITR) during ablation is a predictor of esophageal injury. OBJECTIVE: The purpose of this study was to describe an ablation strategy aiming to enclose the pulmonary veins with contiguous, stable, and optimized radiofrequency applications (referred to as CLOSE-PVI). We evaluated esophageal and periesophageal injury with endoscopy in patients revealing ITR during CLOSE-PVI. METHODS: Eighty-five patients with ITR during CLOSE-PVI underwent endoscopy of the esophagus (with ultrasound in 38 patients). PVI consisted of contact force (CF)-guided encircling of the veins using 35-W applications, respecting strict criteria of intertag distance (≤6 mm) and ablation index (AI; 550 arbitrary unit [au] anterior wall, 400 au posterior wall, 300 au if ITR >38.5°C). RESULTS: Endoscopy was performed 9 ± 4 days after PVI. At the posterior wall, median power was 35 W [interquartile range (IQR) 35-35], application time 18 ± 5 seconds, CF 13 ± 6g, and AI 403 ± 38 au. A median of 5 applications [IQR 4-7] per patient over a length of 21.8 ± 6.8 mm resulted in ITR >38.5°C (median 39.9°C, IQR 39.2°C-41.2°C, range 38.6°C-50.0°C). For these applications, median power was 35 W [IQR 30-35], application time 14 ± 3 seconds, CF 12 ± 5g, and AI 351 ± 38 au. The incidence of esophageal erythema/erosion on endoscopy was 1 of 85 (1.2%) and of ulceration was 0 of 85 (0%). The incidence of mediastinal or esophageal injury on ultrasound was 0 of 38 (0%). CONCLUSION: The occurrence of esophageal or periesophageal injury after CLOSE-PVI is markedly low (1.2%). Absence of esophageal ulceration in patients with ITR suggests that this strategy of delivering contiguous, relatively high-power, and short-duration radiofrequency applications at the posterior wall is safe.

Pancreatic panniculitis as a paraneoplastic phenomenon of a pancreatic acinar cell carcinoma.

We present the case of a 59-year-old patient admitted with extreme painful erythematous subcutaneous nodules of the lower extremities in association with arthritis and peripheral eosinophilia. Upon skin biopsy, the diagnosis of pancreatic panniculitis was made. On further investigation, an underlying acinar cell type pancreas carcinoma was revealed. This clinical case does illustrate how a seemingly innocuous skin condition may herald an underlying malignant disease. The presence of pancreatic panniculitis should trigger clinicians to undertake further thorough diagnostic investigation of the pancreas.

Living related liver transplantation in an adult patient with hepatocellular adenoma and carcinoma 13 years after bone marrow transplantation for Fanconi anemia: A case report.

Fanconi anemia (FA) is an inherited bone marrow failure syndrome due to defective DNA inter-strand cross-link repair. Hematopoietic stem cell transplantation (HSCT) is curative for pancytopenia, but may not prevent the development of non-hematological malignancies. We describe a 26-year-old male patient with FA and Marfan syndrome who in 1994 underwent successful HSCT with bone marrow stem cells from his human leukocyte antigen (HLA)-identical sister. In 2006, three lesions in the liver were detected and resected. The three lesions all showed activation of the ß-catenin pathway and were histologically characterized by a highly differentiated steatotic hepatocellular carcinoma (HCC) with remnants of the underlying adenoma from which it arose, a hepatocellular adenoma with foci of well-differentiated HCC, and a cholestatic adenoma. Risk factors for the emergence of HCC included FA itself, the use of androgens for a period of 3 years preceding HSCT and toxicity of the conditioning regimen. Because of the danger of developing additional HCC, liver transplantation was proposed, taking into consideration that immunosuppression would increase the risk of other malignancies. By using part of the liver of the sister, who already acted as bone marrow donor 13 years earlier, immunosuppression could be avoided. Liver transplantation was performed in 2007 without complication. Five years after liver transplantation the patient is doing well. This case is twofold special being the first case reporting FA co-occurring with Marfan syndrome and being the first reported case of FA treated for HCC by liver transplantation from a HLA-identical sibling donor without the use of immunosuppression.