EOSINOPHILIC ANGIOCENTRIC FIBROSIS AS A CAUSE OF NASAL OBSTRUCTION. A CASE REPORT.

Eosinophilic angiocentric fibrosis (EAF) is a rare benign lesion of unknown aetiology, found predominantly in the upper respiratory tract. The fibrosis must be confirmed by considering other lesions in the differential diagnosis. The typical histological characteristics are the basis for establishing the diagnosis. We present a patient with long-term nasal obstruction caused by EAF. The patient had repeated resections of the lesion for its recurrences. As follows from the literature, radical resection is crucial for the treatment of EAF. This procedure may be difficult at times given the extent and localization of the disease..

Multidisciplinary team in colorectal cancer treatment - analysis of our patients in 2017.

INTRODUCTION: Multidisciplinary teams (MDTs) have become a standard part of treating oncological patients. Based on the available data, they have lead to significantly higher survival rates in the treatment of colorectal cancer (CRC). Reported negatives include potentially longer times between diagnoses and the start of appropriate treatment, and the lack of quality controls over the MTDs actions. This report aims to assess the benefits of MDTs using our own data set for 2017. METHODS: Year 2010 saw the institution of an MDT at the Central Military University Hospital in Prague, with the obligation to refer CRC patients to the MDT before the start of treatment. Having standardized the registration, we have implemented a simple procedure to track the quality of our MDTs involvement and its patient benefits: number of patients, number of referrals with proposed diagnostic and therapeutic procedure, frequency and reason of changes to original strategies, and the frequency of variations from the MDTs conclusions. RESULTS: 405 CRC patients were referred to the MDT in 2017; we have found 499 referrals in this group. The data set was formed predominantly by men (61%), with the mean age of 63 (21-91), and the median age of 64.5 years. Surgical treatment was the most commonly proposed procedure (59%), followed by systemic treatment or, as the case may be, radiotherapy. In 24% of the cases, the conclusion did not match the originally proposed procedure. The decision not to go through with the proposed surgical treatment was the most common change (66 %). We have found a difference in the quality of referral in patients examined specifically by the referring doctor, as opposed to patients whose medical records have just been sent in. We have found therapeutic variation in the MTDs conclusions in less than 5% of patients. CONCLUSION: Having analyzed our data for CRC patients referred to the MDT in 2017, we have found out that in 24% of the patients, the MDT referral leads to a change in the originally proposed diagnostic and therapeutic procedure. Consensus among the MDTs members on the CRC patients treatment guarantees an optimum procedure. What is fundamental is that the referring doctor knows the patient. Constant tracking of the MDTs outputs forms a condition for sustaining the quality of its work and a base for assessing its benefits to the patients.

Malignant tumours of the duodenum.

No comprehensive knowledge of duodenal tumours exists in the current literature; individual types of malignant tumours may be described within malignancies of the small bowel, sets of case reports, or individual cases. Ampullary carcinomas are the exception and they are detailed in the current WHO histological classification of tumours of digestive system. Neither national nor international literature sources provide a comprehensive review of their therapy. The situation is similar when searching for surgical procedures. Resection procedures on the duodenum should thus be performed in specialized centres with sufficient experience with hepato-pancreato-biliary surgery.

[Molecular cytogenetic analysis of chromosomal aberrations in cells of low grade gliomas and its contribution for tumour classification]. / Molekulárne cytogenetická analýza chromozomových aberací v bunkách nízkostupnových gliomu a její prínos pro klasifikaci nádoru.

BACKGROUND: Low-grade gliomas represent a heterogeneous group of primary brain malignancies. The current diagnostics of these tumors rely strongly on histological classification. With the development of molecular cytogenetic methods several genetic markers were described, contributing to a better distinction of glial subtypes. The aim of this study was to assess the frequency of acquired chromosomal aberrations in lowgrade gliomas and to search for new genomic changes associated with higher risk of tumor progression. PATIENTS AND METHODS: We analysed biopsy specimens from 41 patients with histological dia-gnosis of low-grade glioma using interphase fluorescence in situ hybridization (I FISH) and single nucleotide polymorphism (SNP) array techniques (19 females and 22 males, medium age 42 years). RESULTS: Besides notorious and most frequent finding of combined deletion of 1p/ 19q (81.25% patients) several other recurrent aberrations were described in patients with oligodendrogliomas: deletions of p and q arms of chromosome 4 (25% patients), deletions of the short arms of chromosome 9 (18.75% patients), deletions of the long arms of chromosome 13 and monosomy of chromosome 18 (18.75% patients). In bio-psy specimens from patients with astrocytomas, we often observed deletion of 1p (24% patients), amplification of the long arms of chromosome 7 (16% patients), deletion of the long arm of chromosome 13 (20% patients), segmental uniparental disomy (UPD) of the short arms of chromosome 17 (60% patients) and deletion of the long arms of chromosome 19 (28% patients). In one patient we detected a shuttered chromosome 10 resulting from chromothripsis. CONCLUSION: Using a combination of I FISH and SNP array, we detected not only known chromosomal changes but also new or less frequent recur-rent aberrations. Their role in cancer  cell progression and their impact on low grade gliomas classification remains to be elucidated in a larger cohort of patients.

[The significance of biological intracranial meningioma behaviour for their long-term management]. / Význam biologického chování nitrolebních meningiomu pro jejich dlouhodobý management.

INTRODUCTION: From the histological point of view, most meningiomas are benign tumours. Nevertheless, their recurrence is quite frequent, and identifying the risk factors for recurrent disease is of utmost importance. MATERIAL AND METHODS: Prospective study, patients operated on in the period 2/2008-9/2009. Inclusion criteria for enrolment into the study: age 18-70 years, good health status, and previously untreated solitary intracranial meningioma. Evaluation of clinical, surgical, MRI findings and vascular endothelial growth factor serum levels (prior to procedure, 3 months after, 12 months after, 24 months after procedure and at the time of the last follow-up in 11/2011) was performed. Immunohistochemical analysis of histological samples was carried out. Further treatment (wait and see, redo surgery, treatment with radiation using the Leksell Gamma Knife, external radiotherapy) was recorded in case of a positive follow-up MRI finding (recurrent disease, progression of residual tumour). RESULTS: A total of 50 consecutive patients (34 women and 16 men) were included into the study. Average age at the time of surgery was 59.2 years (range 27-75 years). Radical resection with no residual tumour left was achieved in 35 cases. Histological analysis showed grade I meningioma in 45 cases and grade II (atypical) meningioma in 5 cases. Correlation between E-cadherin expression and recurrent meningioma was proved by immunohistochemical examinations; in the remaining investigations, however, such a correlation was not found. No correlation was detected between the serum vascular endothelial growth factor level and the expression of vascular endothelial growth factor in immunohistochemical investigation. A correlation was found between the expression of vascular endothelial growth factor in immunohistochemical investigation and peritumoural oedema. The tumour recurred in 5 cases. CONCLUSIONS: On the basis of our results as well as literature findings we can conclude that all the patients after meningioma surgery need to be carefully followed for the rest of their life by repeated MRI invetigations.

[Metastasis of melanoma malignum in distal ileum like rare causation of ileocoecal invagination--case report]. / Metastáza maligního melanomu distálního ilea jako vzácná prícina ileocekální invaginace--kazuistika.

Authors are presenting the case of a patient, whom developed acute ileocoecal invagination, as a result of the stem tumor of distal ileum. The patient underwent ileocoecal resection with the primary anastomosis end to end. Another clinical and histopatological examinations showed and established diagnosis of metastasis of melanoma malignum in distal ileum with metastasis to liver. This was the first patient's clinical manifestation of the tumor.

[Radical resection in patients with pancreatic head carcinomas. A retrospective survival analysis in a group of 307 subjects]. / Radikální resekce u nemocných s karcinomem hlavy pankreatu. Retrospektivní analýza prezívání u souboru 307 nemocných.

INTRODUCTION: The Czech Republic has the world's highest rates of pancreatic carcinomas. The pancreatic carcinoma is the fourth commonest cause of deaths due to malignancies, in our republic. Resection procedure is currently the only current treatment method, which has a curative potential and significantly prolongs a patient's life. AIM: To assess morbidity, mortality and survival of patients following radical and paliative procedures in the pancreatic head carcinoma patients. METHODS AND PATIENT GROUP: Only patients, who, based on the preoperative staging, were expected to require the following procedures, were indicated for surgery: I radical resection, i.e. stage I, II patients, 2 - palliative resection - i.e. stage III or IV patients, where no angioinvasion was detected preoperatively. Patients with peroperative detection of angioinvasion into the portomesenteric venous drainage area who required partial vein resection, were also included in the above subgroup. 3 - palliative bypass, where longer survival was expected. Radical resection included proximal pancreatoduodenectomy (PDE) with preservation of the pylorus according to Traverso-Longmire, with N1-2 lymphadenectomy and with reconstruction to an excluded jejunal loop. The same procedure was followed in cases of palliative resections. The collected data were statistically assessed using the Logrank test. From 05/1998 to 12/2006, a total of 307 patients with carcinomas of the pancreas and the Vater papila were treated. In 242 patients, the carcinoma was located within the pancreatic head, in 65 subjects, the pancreatic body and cauda were affected. Resection for the pancreatic head carcinoma was performed in 78 patients: 46 males, 32 females, the mean age was 63.5 y.o.a, with the median of 64 years. Bypass procedures were performed in 109 subjects and explorations in 55 subjects. RESULTS: Surgical procedures, with exception of 55 subjects who underwent exploration only, were performed in 187 subjects. Out of the total 78 PDEs, resections in stage I and II were performed in 22 subjects, in stage III in 41 subjects. In the group of 63 radical resection subjects, 2 subjects exited: the first one due to multiorgan failure, the second one for necrotizing postoperative pancreatitis. In the group of 15 palliative resections, 3 subjects exited. 10 patients died during the early postoperative period after palliative bypass procedures. A total of 15 subjects, i.e. 8%, exited during the early postoperative period. 5 subjects exited after resection procedures, i.e. 6.4%, 3% after radical resections. 3 subjects exited after palliative resections. Early complications were recorded in 44 subjects: pancreato-jejuno anastomosis insufficiency in 6 patients, insufficiency of hepaticojejunoanastomosis in 5 subjects, postoperative pancreatitis in 5 subjects, intraabdominal absces in 10 subjects, wounds infections with secondary healing in 19 subjects and cardiopulmonary complications in 33 subjects. In 19 subjects (43% of all complications), surgical revision was performed. Three-year survival rates were recorded in 60, resp. 29.5 and 39.5% of the patients in stage I, resp. II and III, while they were recorded in 15.6.% of the stage IVa subjects and only in 10.5% of the stage IVb subjects. There is a significant difference between survival rates of the stage I, II and III patients, compared to the stage IV patients (p < 0.005). There is no significant difference in the over- 3-years survival rates between the patients undergoing radical or palliative resections, however, the radical resection patients have significantly higher survival rates 3 months to 2 years postoperatively (p < 0.05). The radical resection subjects have significantly higher survival rates during the first 36 postoperative months, compared to the palliative resection and BDA subjects (p < 0.05). Comparison of sur vival rates in patients with radical or palliative resections is affected by a small number of the palliative resection subjects (n = 15), where no differences in survival rates were detected from the end of 9th postoperative month to the end of 3rd postoperative year. There is a significant difference in the survival rates between the resection and exploration subjects (p < 0.05). The survival rates differences between the subjects with palliative resections and BDAs cannot be evaluated in our study, due to nonhomogenity of the subjects. CONCLUSION: Radical PDEs for the pancreatic head carcinoma results in significantly longer survival of the subjects, compared to palliative bypasses. Stage I, II or III patients survive significantly longer, compared to those operated in stage IV.

Molecular cytogenetic stratification of recurrent oligodendrogliomas: utility of interphase fluorescence in situ hybridization (I-FISH).

In oligodendroglial brain tumours, losses of chromosomal material of the short arm of chromosome 1 and long arm of chromosome 19 have been shown to predict responsiveness to chemotherapy and prolonged patients' survival. Therefore, the correct diagnosis of these genetic alterations in tumours of oligodendroglial origin is particularly important. To detect deletions of 1p36 and/or 19q13.3 in oligodendroglial cells we used dual-colour I-FISH with locus-specific DNA probes. I-FISH was performed on isolated whole cell nuclei, prepared from fresh non-fixed tumour tissue samples resuspended in media and processed using a standard cytogenetic procedure, thus bypassing the problem of nuclear truncation. We examined 16 patients with histologically proved oligodendrogliomas (5x oligodendroglioma, 9x anaplastic oligodendroglioma, 2x anaplastic oligoastrocytoma). The results of molecular cytogenetic analyses were correlated with morphological and clinical findings. Molecular cytogenetic analyses were successful in 15 patients and, due to a non-adequate tissue specimen, were uninformative in one patient only. Combined deletions 1p36/19q13 were proved in 13 patients. However, in six of them additional genetic alterations typical for high-grade astrocytoma were found, which could have negative influence on the prognosis. One patient had isolated deletion of 1p36 and another had a normal genetic pattern without any chromosomal alterations. In summary, I-FISH on isolated cell nuclei is a powerful tool for detecting chromosomal aberrations in tumour cells. A systematic molecular cytogenetic analysis may advance diagnosis, prognostic stratification, and targeted treatment of patients with brain tumours.

Elevated and deregulated expression of HDAC3 in human astrocytic glial tumours.

Abnormal expression of histone deacetylases may contribute to the establishment of a cancer specific transcription profile. We examined expression of HDAC3 in human non-malignant gliosis and glial astrocytic tumours. Samples from four non-malignant gliosis and 17 astrocytic gliomas (six of grade II, one of grade III and ten of grade IV) removed for therapeutic purposes were assayed for HDAC3 expression at mRNA and protein levels. HDAC3 mRNA was detected in non-tumorous gliosis as well as in all examined glial tumours. Seven out of eleven examined high-grade tumours showed an elevated number of copies of HDAC3 mRNA. Western blot analysis detected high levels of expression of HDAC3 in the majority of the examined tumours. Immunohistochemistry and immunofluorescence made on a collection of 35 astrocytic tumours detected nuclear as well as cytoplasmic HDAC3 expression in all of those tumours. While the distribution of HDAC3 was both nuclear as well as cytoplasmic and moderate in intensity in non-malignant tissues and low-grade gliomas, high-grade tumours expressed HDAC3 in a focally deregulated pattern that included strongly pronounced cytoplasmic localization. Confocal microscopy and additional co-localization analysis detected nuclear HDAC3 in all tumours examined. We conclude that HDAC3 expression is elevated in human astrocytic tumours and its expression pattern is deregulated at the cellular level in high-grade gliomas.

The effect of partial hepatectomy on the genotoxicity of aflatoxin B1. II.

The influence of partial hepatectomy (PH) on the genotoxic effect of aflatoxin B1 (AFB1) mycotoxin in male Chinese hamsters (Cricetulus griseus) in vivo was studied after a repeated i.p. application of small doses of AFB1 during 8 weeks. The frequency of aberrant cells did not increase after repeated application and persisted throughout the whole period on a relatively stable level. No cumulative genotoxic effect of repeated doses of AFB1 was observed. PH decreased the genotoxic effect of AFB1 only in week 4, while in weeks 6 and 8 no significant differences between hepatectomized and nonhepatectomized animals were recorded.

The effect of partial hepatectomy on the genotoxicity of aflatoxin B1.

The influence of partial hepatectomy on the genotoxic effect of aflatoxin B1 (AFB1) mycotoxin in male Chinese hamsters (Cricetulus griseus) was studied after application of a single i.p. dose of 1.0 mg AFB1/kg. Changes in the fractions of proliferating bone marrow cells, values of the mitotic index of liver cells and morphologic changes in liver tissue were also monitored. Partial hepatectomy reduced significantly the mutagenic activity of AFB1 measured by the frequency of chromosome aberrations in bone marrow cells during 5 days. In hepatectomized animals AFB1 cytotoxicity was significantly reduced as evaluated by changes in the values of proliferating bone marrow cell fractions. There were no important morphologic changes in the liver. In hepatectomized AFB1 treated animals mitotic activity in liver tissue was substantially lower than in hepatectomized but AFB1 untreated animals.

Severe liver injuries due to sulfamethoxazole-trimethoprim and sulfamethoxydiazine.

Two cases of severe sulfonamide liver injury are described. In one patient, fulminant liver failure developed after two days of taking sulfamethoxazole-trimethoprim. The patient was treated with resin hemoperfusion and recovered completely. Another patient became jaundiced after using sulfamethoxydiazine for ten days. Very severe intrahepatic cholestasis developed and cleared up only after high-dose prednison treatment. Marked hyperbilirubinemia persisted for four months and aminotransferase activity had not normalized ten months after the onset of the disease.

[Transpedicular biopsy of thoracolumbar spine]. / Transpedikulární biopsie torakolumbální pátere.

INTRODUCTION: Despite advanced diagnostic methods, biopsy of vertebral body remains to be a very important step in treatment of spinal pathologies. Aim of the study was to present experience with mini-open transpedicular biopsy of the vertebral bodies of the thoracolumbar spine in the circumstances of present trend of CT guided needle biopsy preference. MATERIAL AND METHODS: Standard surgical technique was used in obtaining samples in a group of 38 patients during 1996-2001. Indication for biopsy was to identify the etiology of pathological findings on plain films, CT scans or MRI. 21 patients were men and 17 women average age was 59 years (26-76). In 16 cases the biopsy was performed during posterior or combined approach, when spine was stabilized and decompressed, in 22 cases biopsy was done alone from minimal 3-5cm long midline incision, these patients were followed up for 3-6 years. All biopsies were performed in general anesthesia with average duration of 29 minutes. 5 patients were re-biopsied after CT guided needle biopsy performed in another institution, which resulted in inadequate sample collection. Biopsies were performed by 155 mm long trocar with outer diameter of 4.5mm, inner diameter of 3,5mm, providing adequate sample of tissue. RESULTS: The pathology was identified in all cases, second examination of the sample had to be done in one case (97.4%). Average duration of the procedure (29 min) and low radiation exposure (0.1-0.3 min) was reached. DISCUSSION: Although the technique is more invasive than percutaneous, it was not associated with any substantial morbidity, we did not experience any neurological or other complications. As other authors, who deal with spinal neoplasms, we consider the possibility of radical biopsy tract excision during following posterior surgery as very important. CONCLUSION: Based on our results we consider the mini-open fluoroskopic guided transpedicular biopsy with a 3.5-mm inner diameter trocar as safe and effective method of vertebral biopsy. Minimally invasive but still an open approach is advocated to obtain a representative tissue sample.

[Spindle cell and cuboidal renal cell carcinoma (loopoma). 10 case reports]. / Vretenobunecný a kuboidální renální karcinom (loopom). Popis 13 prípadu.

BACKGROUND: Current classification systems of neoplasms arising from renal parenchyma distinguish 5 categories of renal cell carcinoma (RCC), i.e. conventional RCC, papillary RCC, chromophobe RCC, collecting duct/medullary RCC and unclassified RCC. We present 13 cases of unusual and unclassified spindle and cuboidal renal cell carcinomas. METHODS AND RESULTS: The studied group consisted of 13 patients (7 men and 6 women). They ranged in age from 22 to 65 years (mean 57.3). Generally, the tumours were well circumscribed and confined to the kidney, whitish to grey on section with a diameter 4.5-13 cm (mean 8.6 cm). One patient was investigated for loin pain and nocturia. Three patients had staghorn nephrolithiasis and vague sonographic findings in renal parenchyma. In one patient the renal tumour was found when examined on follow-up examination for prostatic adenocarcinoma. None of our patients was known to have elevated levels of parathyroid hormone due to hyperplasia, adenoma or carcinoma of the parathyroid gland. Clinical follow-up of the patients ranged from 9 months to 8 years (mean 2.3 years). Microscopically, the tumours were composed of two main populations of cells: flattened, spindle cells with sparse cytoplasm and small cuboidal cells with clear to light eosinophilic cytoplasm. Eight patients are currently well without signs of recurrence or metastasis, one had metastasis in the regional lymph node at the time of nephrectomy, one died of unrelated cause, and three were lost to follow-up. CONCLUSIONS: We present 13 cases of unclassified RCC. Our cases were histologically, immunohistochemically and ultrastructurally similar to the hitherto reported case reports of this variant of RCC. It is obvious, that that variant of RCC should be recognised as a new subtype of RCC.

[Metaplastic spindle-cell (fibromatosis-like) carcinoma of the breast--report of 4 cases]. / Metaplastický vretenobunecný (fibromatosis-like) karcinom prsu--popis ctyr prípadu.

The authors report about clinico-morphological features in four cases of spindle-cell metaplastic carcinoma of the breast, closely mimicking benign soft tissue fibromatosis or nodular fasciitis. All patients were females aged 54-72 years with a palpable nodule within the breast, 20-35 mm in size. Histologically, the tumor infiltrated surrounding tissue; it consisted of spindle cells with relatively bland morphology, arranged in a fascicular, storiform or random fashion. Mitotic activity was low, focally the tumors revealed regressive changes (hyalinization, myxoid degeneration). Cytokeratin expression was proven in all four cases. In two patients the tumor metastasized and the patients died of it; this fact confirms recently published data that despite its deceptively benign appearance, fibromatosis--like carcinoma of the breast is undoubtedly a malignant process with potentially aggressive behaviour. Differential diagnosis of this unusual variant of metaplastic breast carcinoma is discussed together with related therapeutic issues.

[Carcinoid of the duodenum in neurofibromatosis]. / Karcinoid duodena pri neurofibromatóze.

The authors described a very rare case of duodenal carcinoid in neurofibrimatosis in a 53-year-old patient who was treated by local excision of the tumour. In the discussion they draw attention to the possible development of carcinoid in neurofibromatosis as a manifestation of systemic affection of neuroectodermal tissues.

Haemorrhage from a radiosurgically treated arteriovenous malformation after its angiographically proven obliteration: a case report.

Small lower-grade Spetzler-Martin arteriovenous malformations (AVMs) are mainly treated by microsurgical resection or stereotactic radiosurgery. The choice of treatment largely depends on the referring centre's preference and the patient's decision. We present here a patient with an AVM repeatedly treated at our Leksell Gamma Knife unit with radiographically confirmed obliteration of the AVM which subsequently began bleeding. This case demonstrates the possibility of late complications in radiosurgically treated AVMs even after their demonstrable obliteration. Meticulous histological examination was performed, proving patency of the AVM nidus. The risk of haemorrhagic complications of radiosurgically removed AVMs despite angiographic proof of their obliteration is, in our view, a cogent argument for preferring surgical resection if the AVM is accessible and for prolonged follow-up after radiosurgical treatment of an AVM.