Brno, Czech Republic
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Trneny M.,I. Interni Klinika | Salkova J.,I. Interni Klinika | Dlouha J.,I. Interni Klinika | Stritesky J.,Ustav Patologie
Vnitrni Lekarstvi | Year: 2013

Non-Hodgkin's lymphoma (NHL) represent the most frequent hematological malignancy with frequent extranodal involvement. We have identified 79 pts (4.6%) out of1,712 patients with NHL, who were diagnosed in our center between 1999-2010. Five cases were primary extranodal lymphomas and we have observed one primary hepatic lymphoma (0.015%). The most frequent (61.3%) NHL subtype in our cohort was diffuse large B-cell lymphoma. B-NHL formed 92.4% of all lymphomas. We have observed high number of HBsAg positive patients (10%). The whole group have poor prognostic features with high number of patients (85%) with intermediate-high and high risk according to international prognostic index. The patients were treated with chemotherapy in 95%, B-NHL patients recieved immunochemotherapy with rituximab in 77%. The median progression free survival, resp. overall survival 4.6, resp. 8.4 years in the whole group and 1.4, resp. 8.4 years in diffuse large B-cell lymphoma were observed with median follow-up 4.5 years. The outcome of T-NHL patients was significantly worse with overall survival median 1.2 vs 8.4 years (p < 0.033). The patients with B-NHL treated by immunochemoterapy with rituximab had significant death risk reduction (HR 0.44, p = 0.03) compared to the patients treated with chemotherapy.

Objective: Presentation of three cases of primary sebaceous carcinoma of the breast particularly focusing on the clinical, biological and molecular genetic aspects regarding their possible pathogenetic relationship to the Muir-Torre and Lynch syndrome. Reviewed are basic principles of miscosatellite instability and dysregulations of mismatch repair genes by these inherited tumorous syndromes especially looking for morphologic and fenotypic parallels between sebaceous carcinomas of the breast and their cutaneous counterparts. Design: Three casuistic reports. Setting: Biopsy Lab s.r.o. and Sikl's Department of Pathology, Charles University and Faculty Hospital, Pilsen. Methods: Three casuistic reports are covered in detail including broad immunohistochemistry (LSAB+, Dako). Results: In three women aged 51 to 69 was diagnosed primary sebaceous carcinoma of the breast with maximum dimension ranged from 13 to 41 mm. Lumpectomy was performed at the smallest one and included sentinel lymph node examination turned out to be negative. The other two patients underwent modified mastectomy with axillary lymph node dissection. In tumor sized 25mm, macrometastasis 4mm in maximum dimension was identified in one axillary lymph node. Follow-up available in two women, both without regional metastasis, revealed no local or distant progression of the disease. The histology consisted of conventional G1-2 invasive duct carcinoma in all cases and sebaceous differentiation represented 10-40% of all neoplastic population. The patognomic features included cells with ample eosinophilic/clear foamy cytoplasm, partly with multiple crowded small vacuoles characteristically impressing the nuclei. All tumors were ER positive and Her2/neu 2+ lesion was not amplified. Strong diffuse nuclear expression of MLHl, PMS2, MSH2, MSH6 proteins in all cases confirmed unaltered mismatch repair genes pathway. Familial tumorous stigmas were not evident and subsequent close clinical monitoring in two of the patients tracked down no intern malignancy, including cutaneous sebaceous lesion.

To determine new data related to the expression of caspase 1, superoxiddismutase and calretinin in the placenta and basal decidua in preeclampsia. Placental and basal decidua samples from 9 preeclamptic and 9 normotensive controls were analyzed using expressions of caspase 1, superoxiddismutase and calretinin assessed by immunohistochemistry. Caspase 1 was expressed in placental syncythium in preeclampsia constantly, while in the control group the expression was weak or absent. In Langhans cells, in fetal sinusoidal capillary endothelia and in Hofbauer cells the expression was equal in both groups. Stronger expression was observed in stromal myofibroblasts in preeclampsia. In preeclampsia, expression of superoxiddismutase in syncythium, in Langhans cells and in decidual cells was weaker. Calretinin was not found in any placental structure. Sporadically, calretinin was expressed in the interstitial extravillous trophoblast cells, in decidual cells and in spiral arterioles in preeclampsia. The obtained morphological data correlating with some clinical and biochemical features contribute to understanding of the molecular background of preeclampsia etiopathogenesis.

Smardova J.,Ustav Patologie | Koptikova J.,Institute Biostatistiky A Analyz
Klinicka Onkologie | Year: 2014

The p53 tumor suppressor is an evergreen of molecular oncology. Since its discovery in 1979, it has been subjected to intensive investigation. The p53 protein is composed of "only" 393 amino acid residues, and function of almost each of them has been addressed in detail. Somatic mutations are extremely frequent, they can be found almost in each of the p53 codons and in all types of tumors. Inherited p53 mutations are rare but very penetrant, and they are typically associated with development of a broad spectrum of tumors. However, in 2001, the p53 research provided an unexpected discovery: the R337H allele was found in southern Brazil. This allele was atypically associated with only one type of tumor - childhood adrenocortical carcinoma and it exhibited low penetrance. Therefore, new data on functioning and impact of the R337H mutation were highly desired. The results obtained during a few following years helped to elucidate not only this specific p53 variant but also provided insight into general principles of mutant p53 variants function. It also turned out that all R337H alleles that are very frequent in southern Brazil originate from one common ancestor.

Large cell neuroendocrine carcinoma of the urinary bladder is rare. In the last five years, we have had the opportunity to see this type of cancer in an 88-year-old and in a 66-year-old males. In both cases, transurethral resection of carcinoma of the bladder was carried out. In the first case, urothelial carcinoma was detected and deeper in the bladder wall, large cell neuroendocrine carcinoma structures were found. In the second case, the bladder was only infiltrated with large cell neuroendocrine carcinoma. Both tumors expressed NSE, CD56 and synaptophysin. Other markers, such as those against calcitonin, chromogranin, PP, VIP, serotonin, gastrin, glucagon and somatostatin did not react with the tumor. In the first case, no tumor dissemination was found; in the second case, clinical methods confirmed dissemination into the liver, left adrenal gland, spleen and paracaval lymph nodes. Given his age, the first patient only received symptomatic therapy. The other patient underwent chemotherapy and his condition is stable. Paraneoplastic manifestations of the tumors were not clinically found. Histogenetic origin of neuroendocrine tumors is not fully clarified. In some cases, tumor development is thought to be associated with Brunns nests, cystitis cystica and urothelial carcinoma stem cells.

An unusual case of fetal polycystic kidney disease is reported. Oligohydramnios and enlarged hyperechogenic kidneys were found at 21 weeks. The pregnancy was terminated and fetal autopsy performed. The histopathological pattern of fetal kidneys was consistent with glomerulocystic disease and this raised suspicion of autosomal dominant polycystic kidney disease (ADPKD). Initially, the family history seemed to be negative for ADPKD. The mother's diagnosis was established only after the abortion of the affected fetus. She had no symptoms of renal disease. Multigenerational involvement was revealed on the mother@s side. Mechanisms leading to prenatal ADPKD and prognosis of the pediatric patients are discussed.

Smardova J.,Ustav Patologie | Koptfkova J.,Institute Biostatistiky a Analyz
Klinicka Onkologie | Year: 2016

Background: The somatic mutation theory explaining the process of carcinogenesis is generally accepted. The theory postulates that carcinogenesis begins in a first renegade cell that undergoes gradual transformation from a healthy to a fully malignant state through the accumulation of genetic and epigenetic "hits". This theory focuses specifically on mutations and genetic aberrations, and their impact on cells. It considers tumors as populations of sick cells that lose control of their own proliferation. The theory was put forward by Robert Weinberg and Douglas Hanahan, and is the predominant view in current cancer biology. By contrast, the tissue organization field theory proposed by Carlos Sonnenschein and Ana Soto considers loss of physiological structure and function by a tissue as key events in tumor development According to this theory, tumors arise at a tissue rather than at a cellular level. It is based on a presumption that proliferation status, rather than quiescence, is the default position of cells in multicellular organisms. Aim: Thearticle aims to provide answers to following questions: Are the views of proponents of the somatic mutation theory (the reductionists) and proponents of the tissue organization field theory (the organicists) incompatible and incommensurable, even when the mainstream of tumor biology has shifted its attention from tumor cells toward the tumor microenvironment? Where to find a third interconnecting systemic approach? Is it useful to be aware of the controversy between reductionists and organicists? What this awareness contributes to? How do these alternative views influence practical oncology and tumor biology in general? Conclusion: Whether the true position is held by reductionists or organicists is unimportant. What is important is to be aware of the existence of these two concepts because this knowledge makes the way we think about tumor origin and development, and how we set up and interpret our experiments, more precise.

Dvorak K.,IV. Interni Klinika | Smid V.,IV. Interni Klinika | Jaksa R.,Ustav patologie
Gastroenterologie a Hepatologie | Year: 2016

Intravesical BCG application is an established and relatively simple treatment for superficial carcinomas of the urinary bladder. It is usually well tolerated and has only mild side effects. We report a case of a 76-year-old immunocompetent man in whom this treatment led to a rare complication characterized by granulomatous hepatitis and pneumonitis. Elastography of the liver was used during diagnosis.

Objective: The aim of the present work was to evaluate the results of quantitative determination of liver iron and copper content using atomic absorption spectrometry on liver tissue specimens obtained by percutaneous liver biopsy. Patients: A cohort of 83 patients was divided into 4 groups according to histological findings: group I (normal histological picture; n = 27), group II (chronic hepatitis; n = 33), group III (cirrhosis; n = 10), group IV (hemochromatosis; n = 10), and 3 cases of Wilson disease. Results: As expected, in group IV (hemochromatosis) we detected a significantly increased iron content in the liver tissue (3.73 ± 1.93 mg/g) and concentration of ferritin in the serum (966 ± 560 μg/L); (p < 0.001), along with an increased value of transferrin saturation (0.66 ± 0.20); (p < 0.05) versus group I. A statistically significant Pearson linear correlation was seen between the liver iron and ferritin concentration (r = 0.6573; p < 0.001) and between liver iron and transferrin saturation (r = 0.6878; p < 0.001). The liver copper content in groups I, II and IV did not differ significantly. The group of patients with liver cirrhosis (group III) showed significantly increased values of liver copper (247 ± 161 μg/g) as well as serum copper (24.8 ± 7.2 μmol/L) versus values in group I (52.5 ± 29.4 μg/g and 16.1 ± 5.0 μmol/L) respectively, (p < 0.001). In connection with the finding of increased copper content in group III (cirrhosis), we also observed significantly elevated alkaline phosphatase activity (p < 0.05). Conclusions: Out of the iron metabolism serum parameters which show a correlation with liver iron, the highest suitability is exhibited by ferritin and transferrin saturation. The indication for liver tissue copper content determination is an unequivocal part of the diagnostics of Wilson disease. This procedure cannot be replaced by the histochemical proof of copper in a bioptic specimen of liver tissue. However, copper values of around 250 μg/g of dry liver tissue have to be evaluated carefully, and other possible causes have to be taken into consideration.

Doubkova M.,Klinika Nemoci Plicnich a Tuberkulozy | Moulis M.,Ustav Patologie | Skrickova J.,Klinika Nemoci Plicnich a Tuberkulozy
Vnitrni Lekarstvi | Year: 2015

Common variable immunodeficiency disorder belongs to the most common primary human immunodeficiencies and it is characterized by primary defective immunoglobulin production. Hypogammaglobulinemia manifests in every age, usually in adult people. There is no gender predisposition. The prevalence is 1:25 000-1:50 000. The ethiopathogenesis of the majority of CVIDs is unknown. The main clinical respiratory symptoms include recurrent respiratory infects, especially bacterial etiology, sinusitis, bronchitis, pneumonia, leading to bronchiectasis and lung fibrosis. Interstitial lung fibrosis and granulomatosis often manifest at diagnosis of CVID and they are negative prognostic factors of the disease.

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