Case report: Metastatic pancreatic neuroendocrine tumour associated with portal vein thrombosis; successful management with subsequent pregnancies.

Background: Splanchnic vein thrombosis due to co-existing metastatic pancreatic neuroendocrine tumour (pNET) and JAK2V617F mutation is a rare condition. Case report: Here we present a case of a young woman with complete remission of a non-functioning grade 2 pNET with unresectable liver metastases, coexisting with JAK2V617F mutation. Splenectomy and distal pancreatectomy were performed. Neither surgical removal, nor radiofrequency ablation of the liver metastases was possible. Therefore, somatostatin analogue (SSA) and enoxaparine were started. Peptide receptor radionuclide therapy (PRRT) was given in 3 cycles 6-8 weeks apart. Genetic testing revealed no multiple endocrine neoplasia type 1 (MEN-1) gene mutations. After shared decision making with the patient, she gave birth to two healthy children, currently 2 and 4 years old. On pregnancy confirmation, SSA treatment was interrupted and resumed after each delivery. Ten years after the diagnosis of pNET, no tumour is detectable by MRI or somatostatin receptor scintigraphy. PRRT followed by continuous SSA therapy, interrupted only during pregnancies, resulted in complete remission and enabled the patient to complete two successful pregnancies.

Ureteral Complications Requiring Intervention After Kidney Transplant: A Single-Center Experience.

The surgical aspect of kidney transplant can be the surgical technique itself or the use of reconstruction techniques in the case of a complication requiring reoperation. In our study, we examined particularly surgical techniques and reconstruction options for ureteral anastomoses. Data from patients who underwent kidney transplant from 2010 to 2020 (N = 433) were examined retrospectively at follow-up of at least 1 year. We sought an association between the type of ureteral anastomoses and parameters considered to be risk factors based on literature data. We did not note the complicated cases that solved spontaneously and only selected cases where the ureteral anastomosis complication (UAcomp) needed urologic, radiological, or surgical intervention. In a smaller group, we examined the correlation between BK polyomavirus and ureteral stenosis. A total of 9.2% (n = 40) of patients developed UAcomp, 67.5% (n = 27) of whom required reoperation. In complicated cases, the rate of primary ureteral anastomosis type was 60.0% (n = 24) ureteroneocystostomy (UNS) and 40.0% (n = 16) ureteroureterostomy (UU) (P = .184). After UNS, 7.7% (n = 17) of cases required reoperation, and this rate was 4.7% (n = 10) after UU (P = .164). After treatment of the UAcomp, 95.0% (n = 38) of the patients were discharged with a functioning graft, and 5.0% (n = 2) required graftectomy. Complications of ureteral anastomosis with appropriate interventions results in good graft function. The type of ureteral anastomosis is not significantly associated with UAcomp. It is important that the operating surgeon is well versed in UNS and UU techniques to be able to adapt to any situation, be it primary surgery or reoperation.

Surgical complications after kidney transplantation based on the Clavien classification, especially with regard to the types of ureteral anastomoses / Veseátültetés utáni sebészeti szövodmények elofordulása a Clavien-beosztás szerint, különös tekintettel a húgyvezeték-anastomosis típusára.

Összefoglaló. Bevezetés: Veseátültetést követoen a graft és a beteg túlélésére hatással lehetnek a posztoperatív intervenciót igénylo szövodmények. Fontos szempont a mutéttechnikai eredményesség monitorozása. Többek között az irodalomban számos lehetoség ismert a veseátültetés sarokpontjának számító ureteranastomosis elkészítésére is, de az éranastomosisok technikája szintén dönto lehet. Célkituzés és módszer: Retrospektíven vizsgáltuk a 2010 és 2020 között végzett veseátültetéseket a Debreceni Egyetem Sebészeti Klinikáján. Célul tuztük ki a sebészeti szövodmények vizsgálatát, melyeket rendszereztünk, a módosított Clavien-féle beosztás alapján. A legnagyobb figyelmet az ureteranastomosisokra fordítottuk. Minden betegnél az adott kategóriában legsúlyosabb szövodményt vettük alapul a beosztáshoz. A minimális utánkövetési ido 1 év volt. Az adatokat az SPSS statisztikai program segítségével elemeztük. Eredmények: A vizsgált periódusban 406 veseátültetés történt, melybol 24,4% (n = 99) vesetranszplantáltnál alakult ki intervenciós (sebészeti, radiológiai, urológiai) szövodmény. A betegek átlagéletkora 49,5 ± 13,7 év, 60,8% férfi volt. A kumulatív mortalitás 10,1% volt. Grade 4-es szövodmény a betegek 6,9%-ánál (n = 28), Grade 3-as a 6,7%-ánál (n = 27), Grade 2-es a 3%-ánál (n = 12), Grade 1-es a 7,9%-ánál (n = 32) jelentkezett. A veseátültetés után 20,4%-ban (n = 83) alakult ki késon induló graftfunkció. Következtetés: A legenyhébb kategóriába (Grade 1.) került a legtöbb beteg, a szövodmények jelentos része sebészi, intervenciós radiológiai és urológiai közremuködéssel megoldható volt. Az ureteranastomosisok mutéti technikája és a releváns szövodmények kialakulása között nincs szignifikáns összefüggés. Megfelelo és idoben alkalmazott korrekciós kezelés mellett a graft- és betegtúlélést nem rontja szignifikánsan az enyhe és középsúlyos (Grade 1-3.) szövodmények kialakulása. Orv Hetil. 2021; 162(26): 1038-1051. INTRODUCTION: Complications associated with postoperative intervention may affect graft and patient survival after kidney transplantation. Monitoring the effectiveness of surgery is an important aspect. Ureter anastomosis can be the pivot of kidney transplant, the same as vascular anastomosis, so efficiency of the surgical technique is important to follow up. OBJECTIVE AND METHOD: We retrospectively examined kidney transplants performed between 2010 and 2020 at the Department of Surgery of the University of Debrecen. Data were analyzed by the SPSS statistical program. We aimed to investigate surgical complications, which were systematized based on the modified Clavien classification. In one patient, the most severe complication was used as the basis for the schedule. The minimum follow-up time was 1 year. RESULTS: 406 kidney transplants were performed in the examined period, of which 24.4% (n = 99) developed renal transplant complications (surgical, radiological, urological). The mean age of the patients was 49.5 ± 13.7 years, and 60.8% were male. The cumulative mortality was 10.1%. Grade 4 complication developed in 6.9% (n = 28) of the recipients, Grade 3 in 6.7% (n = 27), Grade 2 in 3% (n = 12), and Grade 1 in 7.9% (n = 32). 20.4% of the recipients had delayed graft function. CONCLUSION: The Grade 1 group had the biggest case number, so a significant part of the complications could be solved with the help of interventional radiology and urologists. There is no significant association between the surgical technique of ureteral anastomoses and the development of related complications. With appropriate therapy, graft and patient survival are not significantly impaired by the development of Grade 1-3 complications. Orv Hetil. 2021; 162(26): 1038-1051.

Knowledge and Attitudes of Health Care Professionals and Laypeople in Relation to Brain Death Diagnosis and Organ Donation in Hungary: A Questionnaire Study.

OBJECTIVES: The aim of the present work was to assess the knowledge and attitudes of different health care workers and laypeople toward the donation and transplantation procedure. SUBJECTS AND METHODS: A survey consisting of questions regarding brain death diagnosis, legal organization or organ donation, and the transplantation procedure were sent to participants: 56 intensive care unit (ICU) doctors, 76 ICU nurses, 188 sixth-year medical students, and 320 general practitioners (GPs). Laypeople were also asked to complete the survey. RESULTS: The majority of participants reported being aware of legal regulations for organ donation in Hungary (88.5%). Roughly 25% of GPs and 60% of laypeople were unaware of the opt-out system effective in the country. Less than one-third of ICU physicians (26.9%) and nurses (34.7%) were able to list the organs that may be transplanted from a deceased donor; GPs (22.4%) and medical students (20%) performed even worse on this item. The willingness of ICU specialists (57%) and ICU nurses (45%) to support donating their own organs was moderate. CONCLUSIONS: The results of this survey indicate a need for graduate and postgraduate education and regular teaching programs regarding organ donation and transplantation. More active use of modern media is proposed to improve public awareness and acceptance of organ donation.

[Multiple endocrine neoplasia type 2A in a family]. / A multiplex endokrin neoplasia-2A szindrómáról egy család kapcsán.

The authors present the case of a multiplex endocrine neoplasia type 2A (MEN2A). The 55-year-old woman underwent detailed examinations for abdominal complaints. Bilateral adrenal masses and thyroid nodular goiter were found. Based on metanephrine excretion and MIBG imaging, bilateral phaeochromocytomas were diagnosed. The thyroid nodules were confirmed by thyroidectomy as bilateral medullary thyroid carcinoma. Asymptomatic primary hyperparathyroidism was also detected. Laparoscopic adrenalectomy and parathyroid adenoma removal were performed. Based on family history and the characteristic clinical presentation, MEN2A syndrome was confirmed by genetic testing. During genetic screening of first-degree relatives, the patient's 25-year-old daughter was shown to be a gene carrier. Preventive thyroidectomy was performed and histology proved multifocal medullary thyroid cancer. In addition to the importance of genetic testing, the authors emphasize the guideline-based, but individualized approach to patients with suspected MEN2A syndrome. Orv Hetil. 2020; 161(2): 75-79.

[First outcomes, since being full member of Eurotransplant. A single center experience of cadaveric kidney transplantation]. / Eredményeink a teljes jogú Eurotransplant-tagság óta. A Debreceni Vesetranszplantációs Központ tapasztalatai.

INTRODUCTION: The first renal transplantation was completed in 1991 at the University of Debrecen. In 2013 Hungary joined Eurotransplant. AIM: The authors retrospectively compared the trends. METHOD: Comparison between Period A (from January 1, 2008 to August 31, 2013) and Period B (from September 1, 2013 to October 22, 2015). RESULTS: The proportion of living transplants rose from 3.5% to 9.1 %. During period B over 25% of utilized donors were over 60 years of age. Recipients with body mass index above 30 kg/m(2) increased from 12% to 31%. Prevalence of diabetes among recipients rose twofold. Uretero-neocystostomy was used during period A (99%) while in period B end to side uretero-ureteral anastomosis has also gained popularity (68%). In 2013 the authors introduced routine use of induction treatment. Acute rejection rate decreased from 34% to 8%. The rate of surgical complications did not change. Acute bacterial infections decreased from 41% to 33%. Cumulative renal allograft 1, 3 and 5 year survival rates were 86.6%, 85% and 82.7% in group A vs. projected rates 88%, 84% and 84% in group B, respectively. CONCLUSIONS: Despite the growing proportion of expanded criteria donors, the authors were able to maintain a low incidence of delayed graft function and a favorable graft survival. Since 2013 the authors introduced treatments for acute humoral rejection according to international standards.

[Analysis of donor scoring systems in a single Hungarian transplant centre]. / Donorszelekciós kritériumok vizsgálata a debreceni veseátültetési programban.

INTRODUCTION: To ease organ shortage many transplant centres developed different donor scoring systems, however, a general consensus among clinicians on the use of these systems does not still exist. AIM: The aim of the authors was to analyse the effect of expanded criteria donor, deceased donor score and kidney donor risk index on postoperative kidney function and graft survival. METHOD: Analysis of the characteristics of 138 kidney transplantations and 205 donors in a retrospective study of a five-year period. RESULTS: There was a trend towards rejecting donors in higher risk groups; 22.7% of standard criteria donors belonged to the high risk group of deceased donor score. Graft function was worse in high risk patients. High risk donors can be divided due to the use of deceased donor score. Patients with the highest risk had worse graft function and survival. CONCLUSIONS: With the use of these scoring systems grafts with favourable outcome can be selected more precisely.

[Allograft nephrectomy - a single-center experience]. / Graftectomiák a debreceni vesetranszplantációs programban.

INTRODUCTION: Indication and timing of allograft nephrectomy is still uncertain in some cases. AIM: The aim of the authors was to summarize their experience with graftectomies. METHOD: Data from patients who underwent kidney transplantation between January 1, 2004 and December 31, 2015 were retrospectively analyzed. Frequency, indications, timing, complications as well as early and late allograft nephrectomies were reviewed. RESULTS: From 480 renal transplants, 55 graftectomies were performed (11%). Frequent indications included chronic allograft nephropathy (47%), arterial blood supply complications (13%), ureter complications (9%). 22 cases (40%) of allograft nephrectomies were urgent while 33 cases (60%) were elective. 24% of graftectomies were performed within 30 days after transplantation and 76% thereafter. CONCLUSIONS: The main indications for early graftectomies were arterial complications (31%) and chronic allograft nephropathy (62%) in cases of late graftectomies. The majority of the graftectomies were elective. Leading indication was chronic allograft nephropathy. Early and late graftectomies have different characteristics.

Horseshoe kidney transplantation.

Horseshoe kidney is a fusion anomaly found in approximately one in 400-600 people. Due to vascular and ureteral variations, transplantation with a horseshoe kidney presents a technical challenge. In our case, the isthmus connected the upper poles and contained parenchyma. It consisted of three renal arteries, five veins collected to the inferior vena cava, and two ureters and pyelons. It was implanted en bloc to the left side retroperitoneally. During the early period, cellular and humoral rejection was confirmed and treated. For a urine leak, double J catheters were implanted into both ureters. Later, the first catheter was removed. Subsequently, urinary sepsis developed, necessitating graftectomy. The uncommon anatomy of ureters and antibody-mediated rejection (AMR) may both be factors for a ureter tip necrosis led to an infected urinoma. After other Hungarian authors, we also report a horseshoe kidney transplantation that was technically successful. However, after an adequately treated but severe acute humoral rejection, the patient developed sepsis, and the kidney had to be removed. We conclude that transplantation with horseshoe kidney is technically feasible but may increase the risk for urinary complications and resultant infections. Careful consideration of risk and benefit is advised when a transplant professional is faced with this option.

Primary angiosarcoma of the pancreas mimicking severe acute pancreatitis--case report.

Primary angiosarcoma of the pancreas is an extremely rare neoplasm that often mimicks severe acute pancreatitis. A 58-year-old man was admitted with clinical and laboratory signs of severe acute pancreatitis. Contrast enhanced CT scan demonstrated haemorrhagic necrotizing inflammation of the pancreas involving the pancreatic tail, splenic hilum and small bowels with multiple peripancreatic and free abdominal fluid collection. Percutaneous drainage was performed. After 13 days, laparotomy was indicated because of persistent intra-abdominal bleeding, fever and a palpable, rapidly growing mass in the left upper quadrant of the abdomen. During the operation a necrotic, haemorrhagic mass was found in the pancreatic tail; a frozen section showed malignancy, although the tumour was unresectable. Despite all conservative and surgical therapeutic attempts, the patient died within four weeks after diagnosis. Final histology justified primary angiosarcoma of the pancreas. If a patient with signs of severe acute pancreatitis has fever without elevated PCT, the presence of a malignant tumour of the pancreas should be considered.

New insights into the neuromuscular anatomy of the ileocecal valve.

The neuroanatomy of the ileocecal valve (ICV) is poorly understood. A better understanding of this important functional component of the gastrointestinal tract would enable surgeons to reconstruct an effective valve following surgical resection of the ICV. ICVs were examined in young pigs (N = 5) using frontal and transverse paraffin embedded and frozen sections. Hematoxylin+Eosin (H+E) staining, acetylcholinesterase (AchE), and NADPH-diaphorase (NADPH-d) histochemistry and protein gene product 9.5 (PGP 9.5) and C-kit immunohistochemistry were performed. The H+E staining revealed that the ICV consists of three muscle layers: an external circular muscle layer continuous with that of the ileal circular muscle layer, an inner circular muscle layer continuous with that of the cecal circular muscle layer, and a single longitudinal muscle layer, which appears to be secondary to a fusion of the ileal and cecal longitudinal muscle layers. The AchE, NADPH-d, and PGP 9.5 staining revealed two distinct coaxial myenteric plexuses, together with superficial and deep submucosal plexuses. The C-kit immunostaining showed a continuous myenteric ICC network within the ICV. The structure of the neuromuscular components within the ICV suggests that the valve is a result of a simple intussusception of the terminal ileum into the cecum. This knowledge may help surgeons in their future attempts at reconstructing more anatomically and functionally suitable ICVs following surgical resection of native ICVs.

Colorectal carcinoma rearranges cell surface protein topology and density in CD4+ T cells.

Previously, we described conserved protein clusters including MHC I and II glycoproteins, ICAM-1 adhesion molecules, and interleukin-2 and -15 receptors in lipid rafts of several human cell types. Differential protein-protein interactions can modulate function, thus influence cell fate. Therefore, we analyzed supramolecular clusters of CD4(+) T cells from draining lymph nodes and peripheral blood of colorectal carcinoma patients, and compared these to healthy controls. Superclusters of MHC I and II with IL-2/15 receptors were identified by confocal microscopy on all cell types. Flow-cytometric FRET revealed molecular associations of these proteins with each other and with ICAM-1 as well. In draining lymph nodes expression levels of all these proteins were lower, and interactions, particularly between IL-2/15 receptors and MHC molecules weakened or disappeared as compared to the control. Stimuli/local conditions can rearrange cell surface protein patterns on the same cell type in the same patient, having important implications on further function and cell fate.

[Increasing dominance of laparoscopic techniques in the surgery of the spleen in hematologic syndromes]. / A laparoszkópia elterjedése a lépsebészetben haematológiai kórképek esetén.

Parallel with the evolution of minimally invasive techniques more and more organs became the subject of different laparoscopic operations. The spleen was not an exception to this trend, the first laparoscopic splenectomy was performed in 1991. In the present publication the authors give an overview of their own initial experience with the technique. Between the time period of January 1996 and April 2005, 204 splenectomies were carried out at the 1st Dept. of Surgery, University of Debrecen. The indication was haematological in 113 cases, the choice of operation was laparoscopic splenectomy in 18 cases. The male-female ratio was 7 to 11, the mean age was 45.6 years (21-71). The average operation time lasted 106 minutes (60-200 min.), the mean hospital stay was 11.9 days (5-50 days). Laparoscopy had to be converted to open procedure in three cases, because of bleeding and adhesions. In one case, laparoscopic reoperation was necessary with the indication of subphrenic haematoma on the fifth postoperative day. One death occurred in this series from bilateral pneumonia. The authors conclude that laparoscopic splenectomy can be carried out safely, blood loss is limited, and the widely recognized advantages of laparoscopic techniques can be secured for the patients.

[Fenotypical diversity of hereditary non-polyposis colorectal carcinoma. Pedigree and genetical analysis of two mutation carrier patients]. / A herediter nonpolipózis kolorektális karcinóma fenotípusának sokszínuisége. Két, igazolt mutáció hordozó beteg családfa analízise.

INTRODUCTION: The phenotype of HNPCC shows great diversity. Investigation of the disease needs the application of both the Amsterdam and Bethesda Guidelines. The clinical diagnosis of HNPCC can be established by means of thorough family history containing more generations. The immunohistochemistry and MSI investigation of the tumorous tissue as well as the detection of mutations based on DNA sequencing could reinforce the existence of the possible hereditary tendency. PATIENTS AND METHODS: Two pedigrees were selected based on the above-mentioned protocol at the Surgical Institute of the University of Debrecen, Medical and Health Science Center. Amongst first-degree relatives of the 31-year old male patient suffering from colorectal carcinoma (1st patient), three other colorectal, one gastric, one breast and one lung tumors have been found. Two genetic alterations of hMSH2 gene were detected in this family, which were also detectable in other family members. The mutation of exon 7 was not at that time available in international databases, so it was detected by us for the first time. We were able to find alterations of both hMLH1 and hMSH2 genes in the case of the 25-year old patient with synchronous colorectal carcinomas (2nd patient). These alterations could be detected in other family members as well. The whole pedigree contains only one other case of colorectal carcinoma besides the index person. CONCLUSION: Several HNPCC families would be missed in case of considering the Amsterdam Criteria alone. The application of the Bethesda Guidelines is absolutely necessary for the detection of families with poor history at the first screening. The association of a polymorphism and a pathogen mutation in one person could lead to early onset of colorectal carcinoma.

[The role of positron emission tomography (PET) in the detection of local recurrence and metastases of colorectal cancer]. / A pozitronemissziós tomográfia (PET) szerepe a colorectalis carcinomák lokális recidívájának és metasztázisainak felismerésében.

We presented here the results of PET imaging of 12 patients, previously operated on for colorectal cancer and followed at the 1st Department of Surgery, University of Debrecen. The tests were carried out using 0.15 mCi/kg FDG injections. Whole body imaging was performed in eleven patients. The indication for PET was elevated tumor marker levels in three patients, although CT scan was negative. The PET scan showed lymph node, hepatic and disseminated lymph node metastases with liver involvement in these patients. Suspicious lesions were found on CT scan in the pelvis of four patients. Local recurrence was identified in three of them, PET was negative in the fourth case. Bone scan suggested rib metastasis in one patient, which was not supported at PET investigation. In one patient, the malignant nature of large retroperitoneal lymph nodes could not be determined by CT. PET imaging proved that they were malignant and detected a previously unknown pulmonary metastasis at the same time. In one patient both pulmonary and liver metastases were seen on CT, whereas PET confirmed only the latter. Similarly, CT failed to identify liver metastasis detected at ultrasound, while PET proved it. Finally, a pulmonary metastasis detected on X-ray, could be confirmed by PET only. Based on our experience, we recommend PET-scanning with FDG when conventional imaging is equivocal and/or elevated tumor marker levels are present during follow-up. FDG-PET is important in the detection of local recurrence and metastases as well. It is advisable to use PET more often in the evaluation of patients with recurrent colorectal cancer in order to diagnose recurrences in earlier stages, which helps to identify patients who will benefit from surgery.

[Clinical significance of HNPCC, surgical aspects of early recognition]. / A HNPCC klinikai jelentosége, korai felismerésének sebészi vonatkozásai.

A hereditary background may be demonstrated in approximately 15-20% of colorectal carcinomas. Familial adenomatous polyposis syndrome (FAP) constitutes about 1% of this patient population whereas hereditary non-polyposis colorectal carcinoma (HNPCC) makes up a further 3-6% of colorectal malignancies. The clinical features of HNPCC are dominant right colon localization, early age of onset, high prevalence of synchronous and metachronous tumors. Germline mutations of the so-called mismatch repair genes can be demonstrated in the genetic background of HNPCC. Screening and careful follow-up of these families are essential since the lifetime occurrence of colorectal carcinomas and HNPCC associated tumors has an 80-85% prevalence. The recognition of the affected families may be accomplished by taking a thorough family history, spanning several generations based on the Amsterdam and Bethesda Criteria, immunohistological investigations of the removed specimens and finally the exact identification of the pathologic MMR gene mutations. Radical surgical intervention is advised in cases of proven mutation carriers who are suffering from CRC. The index persons and their family members must be under regular control for their lifetime, with one-to-two year intervals to prevent fatal disease. The initiation of a national HNPCC register would further decrease the mortality and morbidity of the disease.