Zsolt Szentkereszty

Miller Fisher syndrome--a presenting clinical manifestation of lung cancer in a previously apparently healthy individual.

Sirs: A 54 year-old white male complained of clumsiness of the right upper extremity, diplopia and unsteadiness of gait starting about 6 days before admission. The history was negative except for smoking an average of 2 packs of cigarettes per day in the preceding 40 years. On admission he had right sided complete oculomotor nerve lesion, severe bilateral lower motoneuron facial palsy (Fig. 1A and 1B), absent Achilles reflexes, diminished other deep tendon reflexes and severe gait ataxia. The patient did not have objective sensory loss, major paresis or signs of upper motor neuron lesion. Except for an elevated erythrocyte sedimentation rate (46 mm/hour) and a borderline serum glucose level the routine blood tests were normal. The cerebrospinal fluid (CSF) had an extremely elevated protein content (3.115 g/L) associated with some pleocytosis (256 cells per microliters). The CSF cells were lymphocytes (20 %), macrophages (48 %, a few of them signet-ring cells), monocytes (12 %), and 20 % of the cells were intensely stained, atypical giant cells with large round marginal nuclei. These cells were PAS positive and were also cytokeratin positive with CK7 immunocytochemistry (Fig. 2A). Nerve conduction studies revealed mild axonal sensorimotor neuropathy. On computed tomography contrast enhancement was seen in the right Sylvian fissure (Fig. 2B). MRI detected several small (< 10 mm) contrast enhancing lesions mostly in the cerebral cortex (Fig. 2C, arrows), and in some other CSF-adjacent regions (cerebellar surface, basal ganglia adjacent to the lateral ventricle, periaqueductal gray matter) as well. Chest CT identified a small tumor in the right lung (Fig. 2D). The tumor was removed and appeared to be a carcinoma with adenomatous structure staining with PAS. With immunohistochemical examination the tumor cells were CK7 positive, CK20 negative, exhibited nuclear positivity with TTF-1, and about 5 % of the cells had nuclear positivity with Mib-1. A histological diagnosis of grade 3 bronchial adenocarcinoma was established. Miller Fisher described a syndrome of ophthalmoplegia, ataxia and areflexia as a variant of the Guillain-Barré syndrome [2]. In a series of 50 cases [4] facial palsy was present in about one third of the cases. Although a cerebrospinal fluid (CSF) cell count over 50/μl is rare in Guillain-Barré syndrome, it has been reported in several cases [6]. Axonal, predominantly, sensory neuropathy was found in 5 of 6 patients with Miller Fisher syndrome (MFS) [9]. Therefore, from the clinical signs the diagnosis of MFS could have been considered for our case. In leptomeningeal carcinomatosis the incidence of clinical signs at presentation were 11 % for oculomotor lesion, 11 % for facial nerve involvement and 15–15 % for cerebellar signs and polyradiculopathy [1]. Therefore the probability of the coincidence of the individual signs of MFS is low. There are only 4 published cases where the syndrome was associated with malignant diseases. Leptomeningeal infiltration was described by Guarino et al. [3] in 2 cases. One of them developed the syndrome 6 months after gastrectomy for cancer and the other patient 4 years after thyroidectomy for cancer and 2 years after the diagnosis of acute LETTER TO THE EDITORS

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.

Pancreatic Cancer: Multicenter Prospective Data Collection and Analysis by the Hungarian Pancreatic Study Group.

BACKGROUND AND AIMS Pancreatic cancer is a devastating disease with poor prognosis. There is very limited information available regarding the epidemiology and treatment strategies of pancreatic cancer in Central Europe. The purpose of the study was to prospectively collect and analyze data of pancreatic cancer in the Hungarian population. METHODS The Hungarian Pancreatic Study Group (HPSG) organized prospective, uniform data collection. Altogether 354 patients were enrolled from 14 Hungarian centers. RESULTS Chronic pancreatitis was present in 3.7% of the cases, while 33.7% of the patients had diabetes. Family history for pancreatic cancer was positive in 4.8%. The most frequent presenting symptoms included pain (63.8%), weight loss (63%) and jaundice (52.5%). The reported frequency of smoking and alcohol consumption was lower than expected (28.5% and 27.4%, respectively). The majority of patients (75.6%) were diagnosed with advanced disease. Most patients (83.6%) had a primary tumor located in the pancreatic head. The histological diagnosis was ductal adenocarcinoma in 90.7% of the cases, while neuroendocrine tumor was present in 5.3%. Biliary stent implantation was performed in 166 patients, 59.2% of them received metal stents. Primary tumor resection was performed in 60 (16.9%) patients. Enteral or biliary bypass was done in 35 and 49 patients, respectively. In a multivariate Cox-regression model, smoking status and presence of gemcitabine-based chemotherapy were identified as independent predictors for overall survival. CONCLUSION We report the first data from a large cohort of Hungarian pancreatic cancer patients. We identified smoking status and chemotherapy as independent predictors in this cohort.

The role of percutaneous external drainage in the treatment of fluid collections associated with severe acute pancreatitis. What, when and how to drain?

The percutaneous drainage of the fluid collections associated with severe acute pancreatitis mainly in sterile cases is not a commonly accepted method. The aim of the present paper is to analyse the indications, the technic, the limits and results of the percutaneous drainage on the basis of the literature. The percutaneous drainage plays an important role in the treatment of the acute fluid collection, the acute pseudocyst, the pancreas abscess, and the liquified necrosis, accompanying the severe acute pancreatitis. For the septic fluid collections the percutaneous drainage is preferred as the first line treatment. In cases of sterile acute fluid collections and pseudocysts because of its relatively high iatrogenic infection rate the drainage is indicated only if it causes severe complaints. The rules of sterility have to be kept. For successful treatment of liquified necrosis the possible methods are the use of large-bore (20-30F) catheter drainage, sinus tract endoscopy, or laparoscopic assisted necrosectomy. In more than 25% of the cases drainage, along with the conservative treatment, leads to the complete recovery of the patient. In the remaining cases it is helpful in postponing the date of the operation and avoiding early surgery.

Effects of various drugs (flunixin, pentoxifylline, enoxaparin) modulating micro-rheological changes in cerulein-induced acute pancreatitis in the rat

Previously we have investigated the cerulein-induced acute pancreatitis and provided data on its micro-rheological impact in the rat. We hypothesized that non-steroid anti-inflammatory agent flunixin, the xanthine-derivate pentoxifylline and the low molecular weight heparin enoxaparin may have various beneficial effects improving microcirculatory and rheological parameters. In female rats, under general anesthesia, 10 μg/kg cerulein s.c. was administered and 2 hours afterwards microcirculation was tested by laser Doppler flowmetry on the tongue and after performing laparotomy on the small intestine, liver and pancreas prior to terminal blood sampling. From blood samples hematological parameters, blood pH, lactate concentration, erythrocyte deformability, osmoscan parameters and erythrocyte aggregation were tested. Compared to normal control in acute pancreatitis group we found severe deterioration in tissue microcirculation together with impaired erythrocyte deformability and enhanced aggregation, accompanied by acidic pH and increasing lactate concentration. Improvement was found when using flunixin (s.c.), pentoxifylline (i.p.) or enoxaparin (s.c.). These drugs could partly improve the blood flux on the surface of the investigated organs, and the flunixin had the most expressed improving effects on micro-rheological parameters. Surprisingly, the improving effect of pentoxifylline on micro-rheological parameters was not obvious (red blood cell deformability did not improved better than in the other treated groups), however, microcirculatory parameters improved.

[Analysis of clinical course of severe acute biliary and non biliary pancreatitis: a comparative study].

UNLABELLED The acute pancreatitis is a relative common disease with incidence of 5-80 per 100000 people of the population. The number of new cases has steadily increased in recent years. The two main etiological factors are alcohol and cholelithiasis. The incidence of alcoholic pancreatitis is higher in male, and the incidence of gallstone pancreatitis is higher in female. AIM To summarize the difference between the clinical course of biliary and not biliary type of severe acute pancreatitis by analyzing the data of these patients. METHODS 139 patients treated with severe acute pancreatitis were divided in two groups: biliary (A group) and non biliary (B group) of origin. The two groups were compared on the basis of sex and age, mortality, morbidity, number of surgery and hospital stay. chi 2 probe was used for the statistical analysis. RESULTS The complications in biliary group were more serious. The average mortality rate was 15.1%, 17.8% in group A and 13.8% in group B. The mortality rate of female patients in group A was significantly higher. CONCLUSION Female patients suffering from severe biliary acute pancreatitis have higher morbidity and mortality rate. Therefore an elective cholecystectomy is suggested in old female patients with serious co-morbidity and gallstones, before any complications.

VATS therapy of chylothorax caused by leiomyomatosis complicated with tuberous sclerosis complex.

Lymphangioleiomyomatosis with tuberous sclerosis complex is a rare disease. One of the most frequent complications of lymphangioleiomyomatosis is pleural effusion (chylothorax) wich can be treated with the use of VATS. Authors report a case of pulmonary lymphangioleiomyomatosis in a 56-year-old female patient with tuberous sclerosis complex with an 8-week history of recurrent chylothorax, dyspnea and debilitating weakness. By CT scan a flat tissue proliferation was seen in the site of the thoracic duct and it was supposed to be the reason for the pleural effusion. A VATS resection of this laesion and ligation of the thoracic duct was performed successfully. Chylothorax is often associated with pulmonary lymphangioleiomyomatosis. Lymphangioleiomyomatosis combined with tuberous sclerosis complex is extremely rare. In case of chylothorax VATS treatment is successful and may be the first choice.

Micro-rheological changes during experimental acute pancreatitis in the rat

Although microcirculatory disturbances play pivotal role in the pathomechanism of acute pancreatitis (AP), very few papers can be found which had been tested any of hemorheological parameters. The aim of our study was to analyze the hemorheological changes in cerulein-induced experimental acute pancreatitis in rat in two doses (5 and 10 μg/kg, s.c.). Male and female rats were subjected to Control group, or AP with 5 or 10 μg/kg cerulein groups. Blood samplings (lateral caudal vein) were completed before cerulein administration, and 1, 2 and 24 hours later. Hematological parameters, amylase activity, erythrocyte deformability (ektacytometry) and aggregation (light-transmission method) were tested. The presence of AP could be confirmed by amylase testing and histological examination. The earliest impairment of the red blood cell deformability could be observed 1 hour after cerulein administration in 10 μg/kg dosage. Female animals had the worst rheological results with high mortality. In conclusion, subcutaneously administrated cerulein in dosage of 5 and 10 μg/kg resulted in AP in rats, with significant changes in red blood cell deformability and alterations in erythrocyte aggregation. This model seems to be suitable for further comparative studies.

Surgical treatment of acute pancreatitis today

UNLABELLED In case of mild acute pancreatitis the treatment is basically conservative, but in severe cases surgical treatment has an important role. METHODS authors analyze the indications for operation, the timing and the technical questions of it on the basis of the literature published in the previous ten years. RESULTS AND CONCLUSIONS sterile pancreas necrosis is rarely but septic necrosis is an indication for surgery if the conservative treatment is unsuccessful. Therapy resistant multiple organ failure, abdominal compartment syndrome and other surgical complications such as bleeding, perforation need surgery treatment. In biliary pancreatitis associated with cholestasis urgent endoscopic sphincterotomy and later cholecystectomy is suggested. In case of pancreas necrosis the ideal time of operation is 21 days after the beginning of the disease because the rate of complications and mortality is high in early operations. Careful necrosectomy is important which has to be extended to the retrocolic and retroduodenal areas as well. Open abdominal surgery has more complications so it is suggested only in selected cases. Operation should be amended with postoperative bursa omental lavage.

Abdominalis compartment-syndroma súlyos acut pancreatitisben – irodalmi áttekintés

The incidence of intraabdominal hypertension or abdominal compartment syndrome, as the more severe form is called, is relatively high in patients with severe acute pancreatitis, and therefore more attention is needed to the topic. If conservative treatment fails, immediate surgical decompression is indicated. The most commonly used operation is a full thickness median laparotomy, but a transversal laparotomy may also be effective. Although subcutaneous linea alba, or bilateral anterior rectus fasciotomy is safe and effective, decompressive laparotomy is indicated in failure of these methods. The open abdomen therapy is not advised due to high morbidity. Primary closure of the abdomen is preferable.