Functional paraganglioma, an uncommon cause of seminoma metastasis, diagnostic and therapeutic management.

We report a 62-year-old male, with history of uncontrolled hypertension (3 drugs) in who, it was found an incidentally left retroperitoneal hypervascular tumor (5.7 x 3.5 cm) in the CT. It was demonstrated contact between tumor and left renal artery and ureter, without regional lymphadenopathy. The patient was asymptomatic, but had increased of norepinephrine (1141.1 pg/mL), epinephrine (93.3 pg/mL) and serotonin blood levels (264.1 g/L). The MRI confirm the tumor, with serotonin affinity in the 68Galium-Edotreotide PET/CT (DOTATATE) but not in the MIBG/Scan. Therefore, a paraganglioma was the suspected diagnosis. Tumor board recommended excision, due to the risk of being a functional paraganglioma. The tumor was resected through laparoscopic approach after alpha-beta-adrenergic blockade. Postoperatively, hypertension and norepinephrine blood levels were normalized. The histological report surprisingly showed a Seminoma (positivity for PLAP, OCT ¾, c-kit, MDM2) and the surgical margins were free.

Capsule endoscopy diagnosis of gastrointestinal melanoma.

Melanoma is a cancer that frequently metastasises to the small bowel, but most cases are asymptomatic and are diagnosed postmortem. Therefore, CT and PET CT cannot detect all lesions and conventional endoscopic study only detects 10-20% of lesions. In this study, we present the case of a 68-year-old patient with a history of cutaneous melanoma and a diagnosis of intestinal melanoma. Thanks to capsule endoscopy, two lesions compatible with cutaneous melanoma metastasis to the small bowel were detected, allowing a much more effective surgical planning. Capsule endoscopy is an innovative technique that improves preoperative diagnosis, as it is able to detect bowel segments that cannot be inspected by conventional endoscopy. It also has a better resolution than conventional CT, improving sensitivity in the detection of lesions.

Gastric pneumatosis, an uncommon complication of a lung transplant, prognosis, diagnostic and treatment management.

We present a case of a 40-year-old woman with double-lung transplant with extracorporeal circulation (EC) due to pulmonary hypertension (pulmonary veno-occlusive disease form (PVOD)) secondary to mixed connective tissue disease (MCTD). On day 6 postoperatively, abdominal pain and distension was noticed, since abdominal CT scan was performed, showing emphysematous gastritis with gastric wall ischemia and peritonitis. Therefore, emergent surgery was proposed. By open surgery approach, a total gastrectomy with Roux-en-Y and esophagojejunal anastomosis was performed. On day 6 after gastrectomy, intraluminal bleeding of the esophagojejunal anastomosis was detected in control CT, which was not need any aggressive treatment. Postoperative evolution was favourable, being discharged from the intensive care unit on the day 34 and from the hospital two months later.

Exogenous aralar/slc25a12 can replace citrin/slc25a13 as malate aspartate shuttle component in liver.

The deficiency of CITRIN, the liver mitochondrial aspartate-glutamate carrier (AGC), is the cause of four human clinical phenotypes, neonatal intrahepatic cholestasis caused by CITRIN deficiency (NICCD), silent period, failure to thrive and dyslipidemia caused by CITRIN deficiency (FTTDCD), and citrullinemia type II (CTLN2). Clinical symptoms can be traced back to disruption of the malate-aspartate shuttle due to the lack of citrin. A potential therapy for this condition is the expression of aralar, the AGC present in brain, to replace citrin. To explore this possibility we have first verified that the NADH/NAD+ ratio increases in hepatocytes from citrin(-/-) mice, and then found that exogenous aralar expression reversed the increase in NADH/NAD+ observed in these cells. Liver mitochondria from citrin (-/-) mice expressing liver specific transgenic aralar had a small (~ 4-6 nmoles x mg prot-1 x min-1) but consistent increase in malate aspartate shuttle (MAS) activity over that of citrin(-/-) mice. These results support the functional replacement between AGCs in the liver. To explore the significance of AGC replacement in human therapy we studied the relative levels of citrin and aralar in mouse and human liver through absolute quantification proteomics. We report that mouse liver has relatively high aralar levels (citrin/aralar molar ratio of 7.8), whereas human liver is virtually devoid of aralar (CITRIN/ARALAR ratio of 397). This large difference in endogenous aralar levels partly explains the high residual MAS activity in liver of citrin(-/-) mice and why they fail to recapitulate the human disease, but supports the benefit of increasing aralar expression to improve the redox balance capacity of human liver, as an effective therapy for CITRIN deficiency.

Bile duct schwannoma, an uncommon cause of a bile duct tumor, diagnosis and therapeutic management.

Biliary Schwannoma is an uncommon tumor in the digestive system and extremely rare in the bile duct. We report 48 year-old male with an incidentally found in the CT, a tumor in the hepatic hilum (3.2 cm), abutting the first portion of the duodenum, without common bile duct obstruction or lymphadenopathy. The patient was asymptomatic and had normal tumor markers. The endoscopic ultrasound (EUS) demonstrated contact between the tumor, common bile duct and right hepatic artery and the biopsy suggested of mesenchymal tumor. Tumor board recommended excision of the tumor, due to the risk of malignancy and biliary obstruction. The tumor was resected through laparoscopic approach, with a Roux-en-Y hepatico-jejunostomy. The histological report showed a biliary Schwannoma (positivity for S100 and focal GFAP), which infiltrated the external wall of the bile duct, without affecting the cystic duct, surgical margins were free of neoplasia.

Mechanical triangular esophagogastrostomy: Technical aspects and initial results.

INTRODUCTION: Esophageal cancer represents the eighth neoplasm worldwide. The therapeutic approach is interdisciplinary, with surgery being the most effective option. Several techniques have been proposed to perform esophagogastrostomy after esophagectomy, among them mechanical triangular esophagogastrostomy (MT), with a little experience published in the Western literature on the latter. The objective of this study is to describe the technical aspects and initial results of MT anastomosis. METHODS: A retrospective review of the patients who underwent esophagectomy according to the McKeown technique was performed, those in which MT anastomosis was implemented, between October 2017 and March 2020 in our hospital. RESULTS: 14 patients were included, with a mean age of 63 years. The mean operative time was 436 min (360-581), being diagnosed of anastomotic leak (AL) 3 of the 14 patients (21.4%), as well as 3 patients presented anastomotic stenosis (AS). The median stay was 20 days, without any death in the series. DISCUSSION: Multiple publications suggest the superiority in terms of AL and AS of the mechanical triangular anastomosis, which was also observed in our series, in which despite the small sample, a rapid improvement was observed in the indicators after the first cases. Therefore, this type of anastomosis may be a safe option for performing esophagogastric anastomosis after esophagectomy, being necessary more definitive conclusive studies.

Minimally Invasive Elective Surgery as a Treatment of Bowel Invagination in a Peutz-Jeghers Syndrome Case.

We present a case of a 24-year-old woman with Peutz-Jeghers syndrome, recurrent colic abdominal pain, and lower gastrointestinal bleed for the last 5 years. Colonoscopy showed hamartomas without any dysplasia. In the enteral magnetic resonance imaging, a distal jejunum and ileum invagination, secondary to hamartomas was detected. The patient was referred to the Surgery Department and despite few symptoms, elective surgery was proposed. By laparoscopic surgery approach, the entire bowel was carefully revised, 3 intussusceptions and bowel volvulus were found, 2 in jejunum and 1 in ileum, causing incomplete obstruction and intestinal dilatation, with a diameter of 6 cm. These intussusception areas were marked with a silk filament, after achieving devolvulation and disinvagination. A 5-cm laparotomy was done, to externalize the entire bowel, to explore it manually, to verify the absence of other lesions, and locate silk points. By longitudinal enterotomies on the antimesenteric intestinal border where silk filaments were located, the polyps were removed through their stalk, and the enterotomies were transversely closed. Postoperative evolution was favorable, starting oral tolerance on the fourth day and being discharged from the hospital on the seventh day. Eight months later, the patient was asymptomatic with a better quality of life.

Mechanical triangular esophagogastrostomy: Technical aspects and initial results. / Esofagogastrostomía triangular mecánica: aspectos técnicos y resultados iniciales.

INTRODUCTION: Esophageal cancer represents the eighth neoplasm worldwide. The therapeutic approach is interdisciplinary, with surgery being the most effective option. Several techniques have been proposed to perform esophagogastrostomy after esophagectomy, among them mechanical triangular esophagogastrostomy, with a little experience published in the Western literature on the latter. The objective of this study is to describe the technical aspects and initial results of triangular esophagogastrostomy anastomosis. METHODS: A retrospective review of the patients who underwent esophagectomy according to the McKeown technique was performed, those in which triangular esophagogastrostomy anastomosis was implemented, between October 2017 and March 2020 in our hospital. RESULTS: A total of 14 patients were included, with a mean age of 63 years. The mean operative time was 436minutes (360-581), being diagnosed of anastomotic leak 3 of the 14 patients (21.4%), as well as 3 patients presented anastomotic stenosis. The median stay was 20 days, without any death in the series. CONCLUSIONS: Multiple publications suggest the superiority in terms of anastomotic leak and anastomotic stenosis of the mechanical triangular anastomosis, which was also observed in our series, in which despite the small sample, a rapid improvement was observed in the indicators after the first cases. Therefore, this type of anastomosis may be a safe option for performing esophagogastric anastomosis after esophagectomy, being necessary more definitive conclusive studies.