Simultaneous unilateral laparoscopic adrenalectomy for pheochromocytoma and thyroidectomy in MEN 2A and MEN 2B syndrome.

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Leiomyosarcoma in adrenal gland.

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Laparoscopic adrenalectomy for pheochromocytoma during pregnancy.

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Difficult iatrogenic bile duct injuries following different types of upper abdominal surgery: report of three cases and review of literature.

BACKGROUND: Iatrogenic bile duct injuries (BDIs) are mostly associated with laparoscopic cholecystectomy but may also occur following gastroduodenal surgery or liver resection. Delayed diagnosis of type of injury with an ongoing biliary leak as well as the management in a non-specialized general surgical units are still the main factors affecting the outcome. CASE PRESENTATION: Herein we present three types of BDIs (Bismuth type I, IV and V) following three different types of upper abdominal surgery, ie. Billroth II gastric resection, laparoscopic cholecystectomy and left hepatectomy. All of them were complex injuries with complete bile duct transections necessitating surgical treatment. All were also very difficult to treat mainly because of a delayed diagnosis of type of injury, associated biliary leak and as a consequence severe inflammatory changes within the liver hilum. The treatment was carried out in our specialist hepatobiliary unit and first focused on infection and inflammation control with adequate biliary drainage. This was followed by a delayed surgical repair with the technique which had to be tailored to the type of injury in each case. CONCLUSION: We emphasize that staged and individualized treatment strategy is often necessary in case of a delayed diagnosis of complex BDIs presenting with a biliary leak, inflammatory intraabdominal changes and infection. Referral of such patients to expert hepatobiliary centres is crucial for the outcome.

[Pancreatic solid pseudopapillary tumor--report of three cases]. / Guz iity pseudobrodawkowaty trzustki--opis 3 przypadków.

Three cases of young females with pancreatic solid pseudopapillary tumor (SPT) were reported. They were referred to Department of Gastroenterology, because of the ultrasonographical finding of the pancreatic tale tumor. In all presented cases, proper diagnosis was made preoperatively. The patients underwent surgical treatment, and remain symptoms-free with no features of recurrence of the disease (follow-up from 6 to 36 months).

[Rare solid pancreatic tumors]. / Rzadko wystepujace lite nowotwory trzustki.

The most common tumor of the pancreas is cancer, which constitutes 85% of all pancreatic neoplasms. Cystic pancreatic tumors comprise 10% of malignancies. No more than 5% of pancreatic tumors are rare solid tumors as: neuroendocrine tumors, gastrointestinal stromal tumors, solid pseudopapillary tumors, pecomas, lymphomas, granulocytic sarcomas, schwannomas, lipomas, liposarcomas and metastases to pancreas. Nowadays, these tumors are diagnosed more commonly due to the developement and accessibility of the diagnostic imaging techniques. Moreover, the treatment and management of rare solid pancreatic tumors often differs from the management in pancreatic cancer what makes the differential diagnosis difficult and responsible challenge. The main purpose of this article is to present an actual data of epidemiology, clinical presentation, management and treatment of rare solid pancreatic tumors according to recent literature and self experience.

An intensified systemic trafficking of bone marrow-derived stem/progenitor cells in patients with pancreatic cancer.

Various experimental studies indicate potential involvement of bone marrow (BM)-derived stem cells (SCs) in malignancy development and progression. In this study, we comprehensively analysed systemic trafficking of various populations of BM-derived SCs (BMSCs), i.e., mesenchymal, haematopoietic, endothelial stem/progenitor cells (MSCs, HSCs, EPCs respectively), and of recently discovered population of very small embryonic/epiblast-like SCs (VSELs) in pancreatic cancer patients. Circulating CD133(+)/Lin(-)/CD45(-)/CD34(+) cells enriched for HSCs, CD105(+)/STRO-1(+)/CD45(-) cells enriched for MSCs, CD34(+)/KDR(+)/CD31(+)/CD45(-) cells enriched for EPCs and small CXCR4(+) CD34(+) CD133(+) subsets of Lin(-) CD45(-) cells that correspond to VSELs were enumerated and sorted from blood samples derived from 29 patients with pancreatic cancer, and 19 healthy controls. In addition, plasma levels of stromal-derived factor-1 (SDF-1), growth/inhibitory factors and sphingosine-1-phosphate (S1P; chemoattractants for SCs), as well as, of complement cascade (CC) molecules (C3a, C5a and C5b-9/membrane attack complex--MAC) were measured. Higher numbers of circulating VSELs and MSCs were detected in pancreatic cancer patients (P < 0.05 and 0.01 respectively). This trafficking of BMSCs was associated with significantly elevated C5a (P < 0.05) and C5b-9/MAC (P < 0.005) levels together with S1P concentrations detected in plasma of cancer patients, and seemed to be executed in a SDF-1 independent manner. In conclusion, we demonstrated that in patients with pancreatic cancer, intensified peripheral trafficking of selected populations of BMSCs occurs. This phenomenon seems to correlate with systemic activation of the CC, hepatocyte growth factor and S1P levels. In contrast to previous studies, we demonstrate herein that systemic SDF-1 levels do not seem to be linked with increased mobilization of stem cells in patients with pancreatic cancer.

Aggressive surgical management of recurrent lymph node and pancreatic head metastases of resected fibrolamellar hepatocellular carcinoma: a case report.

CONTEXT: Fibrolamellar hepatocellular carcinoma is a rare liver tumor with the propensity to metastasize to the lymph nodes months or years after initial surgery. However, its metastatic spread to the pancreas was previously reported only in a child. CASE REPORT: We present an unusual case of a young female patient who was repeatedly treated by surgical excision of abdominal and mediastinal lymph node recurrences between 2 and 6 years after left hepatic lobectomy for fibrolamellar hepatocellular carcinoma. At 8 years following her initial surgery, the patient was diagnosed with pancreatic head metastasis and a pancreaticoduodenectomy was performed. Postoperative course was uneventful and the patient did not experience recurrence within the last 18 months. CONCLUSION: The metastasis of fibrolamellar hepatocellular carcinoma to the pancreas is highly exceptional but possible and its excision appears warranted as well.

Liver transplantation as an ultimate step in the management of iatrogenic bile duct injury complicated by secondary biliary cirrhosis.

BACKGROUND: This report summarizes a single center's experience with liver transplantation (LT) performed for secondary biliary cirrhosis resulting from iatrogenic bile duct injury (BDI) sustained during cholecystectomy. MATERIAL/METHODS: Secondary biliary cirrhosis was the indication for LT in 5 (1.7%) out of 300 LTs performed in our center between Feb 2002 and April 2011. We analyzed the medical history of the patients, perioperative course and outcome following LT. RESULTS: The BDI was classified as Strasberg A in 1 case, B in two cases, and E in 2 cases. There was no hepatic arterial or portal vein injury in any patient. All of the surgical repairs prior to the development of cirrhosis were performed in general surgical units. The median time between BDI and listing the patient for LT was 11 years. The cadaveric whole-organ LT was done in all patients using the Piggy-Back technique. All patients are alive with a median follow-up of 53 months. CONCLUSIONS: Liver transplantation in patients with secondary biliary cirrhosis appears to result from a series of inadequate multiple surgical repairs following BDI. The immediate referral of such patients to centers with bile duct surgery experience is crucial.

Pancreatoduodenectomy with a modified duct-to-mucosa pancreaticojejunostomy: an analysis of 101 consecutive patients.

BACKGROUND/AIMS: The aim of the study was to analyze in-hospital morbidity and mortality after pancreatoduodenectomy (PD) with a modified duct-to-mucosa pancreaticojejunostomy. METHODOLOGY: We retrospectively analyzed 101 consecutive patients who underwent PD at our center between January 2002 and December 2010. Two-layered duct-to-mucosa pancreaticojejunostomy was performed over an internal transanastomotic stent in all patients. RESULTS: The overall in-hospital morbidity and mortality rate was 48% and 6%, respectively. Three patients died as a consequence of local complications including mesenteric ischemia in two and acute necrotizing pancreatitis in one case. Pancreatic fistula occurred in one (1%) patient and was treated conservatively with good outcome. The wound infection was the most common surgical complication (20/101; 20%) and occurred more often in patients who had a biliary stent inserted endoscopically prior to surgery (15/38; 39%), as compared to those without the stent (5/63; 8%; p=0.0003). CONCLUSIONS: The results of the present study suggest that a two-layered duct-to-mucosa pancreaticojejunostomy with internal transanastomotic stent is a safe anastomosis, associated with a very low risk of pancreatic fistula. The presence of a biliary stent at the time of surgery represents a risk factor for the development of postoperative wound infection.

Laparoscopic adrenalectomy for functioning and non-functioning adrenal tumours.

BACKGROUND: The purpose of this study was a retrospective analysis of outcomes following laparoscopic adrenalectomy (LA) performed for benign adrenal tumours responsible for various endocrinological disorders. The patients were diagnosed with non-functioning (NFT) and functioning adrenal tumours (FT) including pheochromocytoma (PH), Conn's syndrome (CO) and Cushing's (CS) syndrome. MATERIAL AND METHODS: A total of 165 LAs were carried out between August 1995 and September 2009 via either the transperitoneal (n = 38) or retroperitoneal (n = 127) approach. The analysed factors included demographic data of patients, the American Association of Anaesthesiology score (ASA), indication for surgery, tumour size and side, intraoperative and postoperative outcome of LA including duration of surgery, blood loss, time until ambulation, length of hospital stay, time until return to normal activity, the complication rate, as well as the conversion rate to open adrenalectomy. RESULTS: There were 111 patients with NFT and 54 with FT. Patients with NFT were significantly older than those with CO (p < 0.05). The mean size of the lesion differed between CO and other adrenal tumours (p < 0.05) as well as between NFT and PH (p < 0.05). All the lesions except aldosteronomas were detected predominantly in the right adrenal gland (p < 0.05). However, despite the different characteristic and clinical disorders related to laparoscopically removed adrenal tumours, the intraoperative and postoperative outcomes did not significantly differ in most cases between the analysed groups of patients. CONCLUSION: This study shows that LA is a safe, effective, and well-tolerated procedure despite the hormonal activity of the removed lesions. Minimal invasive surgery may be recommended as the 'gold standard' in the treatment of both functioning and non-functioning benign tumours of the adrenal gland.

Late diagnosis of type 2B multiple endocrine neoplasia (MEN 2B) in a 23 year-old patient.

We present a case of MEN 2B diagnosed in a 23 year-old patient on the basis of bilateral pheochromocytoma and medullary thyroid carcinoma. This young male patient also had multiple paragangliomas located along the spine, marfanoid features of body habitus and numerous mucosal neuromas of the oral cavity and intestinal ganglioneuromatosis. The patient was hospitalised several times between the ages of 11 and 14 due to heart rhythm disorders (tachycardia, multiple supraventricular beats) and pain in the precardiac area. Elevated blood pressure was not observed at that time. In 2010, the patient was admitted to hospital due to abdominal pain, nausea, vomiting and hypertension; bilateral adrenal tumours were then detected. The patient was referred to the Department of Endocrinology in Szczecin, with suspected pheochromocytoma in order to continue the diagnostic process. This resulted in the diagnosis of bilateral pheochromocytoma and medullary thyroid carcinoma. On the basis of the whole clinical picture, the diagnosis of MEN 2B was established and subsequently confirmed with genetic test results. Following the removal of adrenal tumours and thyroidectomy, the patient was referred to the Cancer Centre and Institute of Oncology in Gliwice for further treatment (X-ray therapy and further surgery due to recurrence of medullary carcinoma). This article presents a case of late MEN 2B diagnosis despite the presence of clinical symptoms suggestive of Multiple Endocrine Neoplasia observed from early childhood.

Surgical management and outcome of bile duct injuries following cholecystectomy: a single-center experience.

PURPOSE: Biliary injury is a severe complication of cholecystectomy. The Hepp-Couinaud reconstruction with the hepatic duct confluence and the left duct may offer best long-term outcome as long as the confluence remains intact (Bismuth I-III). Complex liver surgery is usually indicated in most proximal (Bismuth IV) injuries in non-cirrhotic patients. The aim of this study was to evaluate the surgical treatment and outcome of bile duct injuries managed in a referral hepatobiliary unit. METHODS: We retrospectively analyzed surgical management and outcome of biliary injuries following cholecystectomy in 35 patients (27 laparoscopic) referred to our center between June 2001 and December 2009. There was no liver cirrhosis diagnosed in any patient. High injuries (Bismuth III-IV) were found in 14 patients. Management after referral included the Hepp-Couinaud hepaticojejunostomy in 32 patients with Bismuth I-III injuries, which in four cases with biliary peritonitis was preceded by abdominal lavage and prolonged external biliary drainage. Liver transplantation was performed in two patients with Bismuth IV injuries. RESULTS: After median follow-up of 59 months (range, 6-102), 34 (97%) patients are alive and 32 (92%) remain in good general condition with normal liver function. One patient who had combined biliary and colonic injury died of sepsis before repair. Recurrent strictures following the Hepp-Couinaud repair developed in two (6%) patients with high injuries combined with right hepatic arterial injury. CONCLUSION: The Hepp-Couinaud hepaticojejunostomy offers durable results, even after previous interventions have failed. In case of diffuse biliary peritonitis, delayed biliary reconstruction following external biliary drainage may be the best option.

From open to laparoscopic adrenalectomy: thirty years' experience of one medical centre.

INTRODUCTION: Laparoscopic adrenalectomy (LA) has become the standardized treatment of benign adrenal lesions over the last two decades, making the indications to open adrenalectomy (OA) limited. The purpose of this study was to show the thirty years of experience in open (OA) and laparoscopic adrenalectomy (LA) gained in one medical centre as well as to compare the results of OA and LA performed for benign adrenal lesions. MATERIAL AND METHODS: Three hundred patients underwent 127 open and 173 laparoscopic adrenalectomies between 1979 and 2009 at M. Curie Hospital in Szczecin, Poland. Analyzed factors included patients demographic data, ASA score, indication for surgery, tumour size and side, characteristics of the removed tumours, intraoperative and postoperative outcome of LA and OA, postoperative pain sensation, intraoperative and postoperative complications, and conversion rate from LA to OA. Tumours with diameter exceeding 8 cm were excluded. RESULTS: There were no significant differences regarding the analyzed preoperative data in both groups of patients. The mean operative time was longer in the LA group (137 v. 82 min., p < 0.0001) and the blood loss was lower in LA group (110 v. 254 mL, p < 0.0001). The mean time until resumption of normal diet was shorter after LA (22 v. 44 h), as was the mean time until ambulation (17 v. 36 h), mean length of the hospital stay (4.6 v. 6.8 days), and mean time until return to normal activities (14 v. 23 days, p < 0.0001 for each difference). The analgesic requirement on the first and the second day postoperatively was lower in the LA group (p < 0.0001). The incidence of intraoperative and postoperative complications did not differ significantly between both analyzed groups. The rate of the conversion from LA to OA was 16%. The histopathological diagnosis was adenoma of the adrenal gland in the majority of cases. CONCLUSIONS: This study shows that LA is a safe, effective, and well-tolerated procedure. It may be recommended as a "gold standard" surgery in a case of benign functioning or non-functioning adrenal tumours with diameter less than 8 cm. (Pol J Endocrinol 2010; 61 (1): 94-101).

Partington-rochelle pancreaticojejunostomy for chronic pancreatitis: analysis of outcome including quality of life.

BACKGROUND/AIMS: Obstruction of the main pancreatic duct in chronic pancreatitis (CP) leads to an increased intraductal and intraparenchymal pressure causing pain. In this study we evaluated the outcome of surgical treatment of CP including the quality of life following Partington-Rochellepancreaticojejunostomy (PRP) performed for intractable pain. METHODOLOGY: Between July 2002 and May 2008, PRP was performed in 17 patients in whom the diameter of the main pancreatic duct exceeded 7mm and there was no inflammatory tumor in the pancreatic head. Perioperative morbidity and mortality were analyzed in all patients. The long term outcome including the quality of life (Karnofsky index) was evaluated in 9 patients who were followed with a mean 28 (range 13-60) months since surgery. RESULTS: Complications in the postoperative period were found in 3 (18%) patients including 1 death due to a myocardial infarction shortly after surgery. All patients submitted to the long-term evaluation reported a significant (p < 0.0001) pain reduction by an average of 6.2 (5-8) points in a 10-points visual analogue scale. The Karnofsky index increased significantly from a mean 52% (40-70%) before surgery up to 82% (70-90%) following surgery and long-term. CONCLUSIONS: PRP leads to a substantial quality of life improvement in patients with CP.

[Recurrent variceal bleeding in a patient with portal and splenic vein thrombosis secondary to complex thrombophilia]. / Nawracajace krwawienia z zylaków przelyku u chorej z zakrzepica zyly wrotnej i sledzionowej w przebiegu zlozonej trombofilii.

Thrombophilia in adults is one of main causes of portal vein thrombosis. Esophageal and gastric varices, ascites and hypersplenism are well known complications of portal hypertension. There are controversial issues on the management, especially anticoagulant therapy and surgical treatment of these patients. We present a 42-years old woman with a history of three acute coronary episodes suffering from recurrent variceal bleeding due to portal and splenic vein thrombosis in the course of myeloproliferative disorder and protein C deficiency. It was 10 months delay of diagnosis. She was successfully treated with medical and surgical treatment (esophageal stapler transection, cardial devascularization, and splenectomy). In the paper we discuss complexity of diagnosis and surgical treatment.

[Liver albumin dialysis (MARS)--treatment of choice in Amanita phalloides poisoning?]. / Dializa albuminowa watroby (MARS)--leczenie z wyboru w ciezkim zatruciu muchomorem sromotnikowym?

UNLABELLED: Amanita phalloides is a direct life-threatening poisoning because of acute multiorgan failure. Urgent liver transplantation (LTx) is the last chance to save patient's life in severe cases. In many cases of mushroom poisoning the patient dies because of unavailability of a liver graft. Liver albumin dialysis (MARS) is a promising treatment to bridge the patient to LTx or stabilize his or her condition until spontaneous liver regeneration occurs. CASE REPORT: Four family members (father, mother and two sons) were eating self-collected mushrooms (Russula vesca). Typically for the Amanita phalloides poisoning, the first symptoms appeared in all persons more than 12 hours after mushroom ingestion. Because they did not improve, the whole family was admitted to the Regional Hospital in Ketrzyn (24 hours after mushroom ingestion). Mycological examination of gastric washings was positive only in the mother, in whom the Amanita phalloides spores were found. During the first 48 hours of poisoning the biochemical indexes of liver injury were observed in all persons. The whole family members were sent to centers where liver albumin dialysis could be performed: the mother was admitted to the Department of Nephrology and Dialysis Therapy in Olsztyn, the father and the first son were admitted to the Clinical Toxicology Department in Krak6w, and the second son was admitted to the Department of Internal Medicine and Acute Poisonings in Gdansk. Three albumin dialysis procedures were performed in the case of mother with complete liver recovery. After the first liver albumin dialysis, the father of the family was disqualified from the following procedures because of severe coagulation disturbances (GI bleeding), and died the fourth day after mushroom ingestion. The first son fulfilled the King's College criteria and was accepted for high urgency liver transplantation. After two albumin dialysis procedures had been able and the patient was urgently sent to the Department of General and Transplantation Surgery in Szczecin, where liver transplantation was successfully performed. The second son was treated conservatively with improvement of general condition and biochemical indexes and no albumin dialysis procedure was necessary. CONCLUSION: Liver albumin dialysis may be effective in severe Amanita phalloides poisoning to stabilize the condition of a patient until spontaneous liver regeneration occurs or as a bridge to LTx. In cases of a family poisoning, proper coordination and cooperation among toxicology departments and transplant centers is required.

Early results of a new liver transplant program in Szczecin, Poland.

New liver transplant programs have usually been associated with a significant learning curve. This learning curve, however, can be minimized or avoided if certain conditions are met. In this paper we describe the establishment of a new liver transplant program in Szczecin, Poland and present its early results. Four members of the team underwent training in clinical liver transplantation in major centers in Europe. Transplant protocols were then adopted, all the necessary facilities set up, and multidisciplinary team created. Between February 2002 and August 2003, 22 adult orthotopic liver transplantations (OLT) were performed in 21 patients (13 male; mean age 46.3 years; range 33-62), including I retransplant for early hepatic artery thrombosis. Eighteen (86%) patients are alive between 4 and 22 (median 11) months after OLT. Seventeen patients have normal liver function and 14 of them have resumed full life activity. Three patients died: one of bacterial peritonitis (day 6), one of chronic rejection with allograft failure (month 4) and one following massive stroke (month 10). Surgical complications occurred in 7 patients (33%). We believe that proper training of vital team members at established transplant centers with good results, availability of adequate equipment and all the facilities required with strict adherence to transplant protocols are all paramount for a successful start of a liver transplant program.