Asynchronous tumour quadruplicity: rectosigmoid adenocarcinoma, renal cell carcinoma, prostate adenocarcinoma and neuroendocrine small-cell lung cancer - a case report.

INTRODUCTION: Multiple tumors belong to rare cancers. Almost all malignancies may occur in combination, the most common combination being gastrointestinal cancer with respiratory or urogenital tract cancer and with breast cancer in females. CASE REPORT: In 1999, a 66-year-old patient was diagnosed with a rectosigmoid tumor histologically proven as adenocarcinoma. Rectosigmoid resection was performed, followed by adjuvant radiotherapy and the Mayo Clinic FU/FA chemotherapy regimen. Radical nephrectomy was performed in January 2005 due to right kidney tumor, histologically detected as clear cell carcinoma. In February 2006, the patient underwent bilateral pelvic lymphadenectomy for biopsy-verified adenocarcinoma of the prostate with PSA 20.8 ng/ml. Radical prostatectomy was performed in April 2006. Histology demonstrated moderately differentiated adenocarcinoma in both prostatic lobes classified as Gleason score 4 (1+3), without invasion into the capsule or seminal vesicles infiltration. In June 2016, a native X-ray of the lungs revealed a subpleural small dense node in the right upper pulmonary field. PET/CT of the trunk was also performed showing liver metastasis and pulmonary deposits, including enlargement of the mediastinal nodules. In October 2016, liver biopsy was taken and the serum level of neuron-specific enolase (NSE: 93 ng/ml) was measured. Histology demonstrated neuroendocrine carcinoma of the small cell type. In November 2016, palliative chemotherapy with carboplatin and etoposide administered once a month was initiated. After 4 chemotherapy cycles, no deposits on the liver were detected by sonography. A native X-ray image of the lungs still showed a 15mm deposit, but NSE levels returned to normal. In March 2017, treatment continued with palliative radiotherapy of the right lung, mediastinal lymphatic nodes and prophylactic radiotherapy of the skull was planned as a next step. In August 2017, the patient died due to renal function failure and deterioration of the general condition. CONCLUSION: The patient worked in uranium mines and underwent radio-chemotherapy after the first malignancy - rectosigmoid tumor. Genetic examination was not performed. The patient died of therapeutic complications of the last malignancy. Our case report does not confirm findings described so far - a shortening interval between malignancies - but confirms their increasing aggressiveness. Key words: quadruplicity - rectosigmoid adenocarcinoma - renal-cell carcinoma - prostate adenocarcinoma - neuroendocrine small-cell lung cancer.

[Tubulo-squamous polyp of the vagina]. / Tubulo-skvamózní polyp vaginy.

OBJECTIVE: To decribe a case of rare type of vaginal polyp. DESIGN: Case report. SETTING: Department of Pathological Anatomy, Regional hospital Nachod. CONCLUSION: Tubulo-squamous polyp is rare type of vaginal polyp first described in 2007, with only up to 20 cases have been reported in the literature so far. It affects mostly postmenopausal women and presents as a polypoid mass occurring in the upper part of vagina. Histologically, it is composed of squamous and glandular component within fibrous stroma. The etiopathogenesis of this lesion remains unclear, but it may arise from mesonephric remnants or Skene glands (so-called female prostate). This theory is supported by the fact that some cases show expression of prostate specific acid phosphatase (PSAP) and/or prostate specific antigen (PSA).

Impairment of nasal mucociliary clearance in former smokers with stable chronic obstructive pulmonary disease relates to the presence of a chronic bronchitis phenotype.

BACKGROUND: Associations between nasal and bronchial impairment have been repeatedly described in chronic obstructive pulmonary disease (COPD), whereas nasal mucociliary clearance (MCC) in COPD patients is not yet fully understood. We studied nasal MCC parameters in COPD patients and compared them with healthy adults (HA) and with cystic fibrosis (CF) patients with compromised MCC. METHODOLOGY: An observational study of 98 COPD ex-smokers and subjects from control groups evaluated for nasal MCC time (NMCCt) and by digital video microscopy of nasal mucosa recording ciliary beat frequency (CBF) and ciliary beat pattern. RESULTS: The NMCCt was decreased in HA compared to those with COPD and decreased in those with COPD compared to those with CF. CBF in COPD was lower compared to HA. The index of ciliary dyskinesia in COPD patients differed from HA. We detected higher NMCCt and lower nasal CBF in patients with chronic bronchitis phenotype (CB) compared to non-CB patients. CONCLUSIONS: We confirmed the presence of impaired nasal MCC in COPD ex-smokers. These impairments were apparent predominantly in the CB phenotype.

[Resistance/sensitivity in vitro in ovarian cancer patients]. / Rezistence/senzitivita in vitro u pacientek s karcinomem ovaria.

OBJECTIVE: To assay resistance/sensitivity by MTT test in solid tumor or ascitic fluid in ovarian cancer patients. DESIGN: Prospective study. SETTING: Department of Gynecology and Obstetrics, Medical Faculty Charles University, Prague and University Hospital, Hradec Králové. METHODS: MTT - (3-(4,5-dimethylthiazol-2-yl)-2,5-diphenyltetrazolium bromide) chemosensitivity assay was performed in 32 samples of ovarian cancer tissue and 26 samples of ascitic fluid in ovarian cancer patients. We studied the in vitro drug resistance profiles of ovarian cancer specimens exposed to cisplatin, carboplatin, paclitaxel, topotecan, gemcitabin, etoposid. RESULTS: The highest frequency of resistance in vitro occured for etoposid, gemcitabin and paclitaxel and the most effective chemotherapeutical agents in vitro were cisplatinum and topotecan. Cisplatin had the lowest incidence of drug resistance in vitro than carboplatin. CONCLUSION: Resistance/sensitivity assay would improve the treatment and prognosis of ovarian cancer patients.

[Relationship between quality of life and BODE index of stable ex-smokers with chronic obstructive pulmonary disease]. / Vztah mezi kvalitou zivota a BODE indexem u bývalých kuráku ve stabilní fázi chronické obstrukcní plicní nemoci.

BACKGROUND AND AIM: This study was carried out to assess relationship between quality of life (QoL) and disease severity expressed by multifactorial prognostic index (BODE) in ex-smokers suffering from chronic obstructive pulmonary disease (COPD), minimally 8 weeks free of exacerbation. MATERIALS AND METHODS: The evaluation was performed in 98 randomly recruited COPD patients enrolled into a cross-sectional, observational CILIARY study at the Department of Pneumology, Charles University, Faculty of Medicine in Hradec Králové. In them, quality of life evaluation using the SGRQ questionnaire and the BODE index calculation was performed. We statistically compared interrelationship between BODE and COPD stages, SGRQ and COPD stages and interrelation of BODE and SGRQ. RESULTS: We found significant differences in QoL of COPD patients and QoL in group of healthy volunteers (p <0.001). Lower QoL and higher BODE score were associated with a higher stages of COPD (p < 0.001), with the exception non-significant difference in QoL (SGRQ score) and BODE index between stages I and II. Our study found positive correlation between the all SGRQ scores and multidimensional prognostic BODE index (r = 0.431-0.704). The strongest correlation (r = 0.704) was evident in activity domain of SGRQ. CONCLUSION: Our results proved close correlation ofquality of life (SGRQ) and multidimensional prognostic score (BODE) in stable COPD exsmokers' population. Both these scoring systems are useful tools for the assessment of clinical course and stratification of severity of COPD. However at present both scales are minimally used in the Czech Republic.

[Examination of function and structure of respiratory cilia of adult patients suffering from chronic obstructive pulmonary disease (COPD)--comparison of nasal and bronchial mucosa (pilot of CILIARY STUDY)]. / Porovnání struktury a funkce nazální a bronchiální sliznice u nemocných s CHOPN (pilotní projekt ciliární studie).

INTRODUCTION: Borderline between upper and lower respiratory tract pathology is probably artificial (bronchial asthma). Also inflammation of bronchial mucosa during chronic obstructive pulmonary disease (COPD) is likely combined with inflammatory involvement of nasal mucosa. Ciliary edge of respiratory epithelium is very important part of mucosa layer. AIM: To investigate and compare nasal and bronchial ciliary beat frequency (CBF), degree of nasal and bronchial ciliary dyskinesia, presence of ciliary akinesia and incidence of spinocellular metaplasia in the both mucosa localities among of stable COPD patients (pts). METHOD: Nasal and bronchial mucosa were obtained in the course of bronchoscopy examination of COPD pts in general intravenous anesthesia. Native samples of mucosa tissue were assessed by digital high-speed video microscopy (1,000x magnification). Paired t-test was used to evaluate differences in average frequencies. Significance level was alpha = 0.05. Mode was used to describe "index of dyskinesia", as a measure of association was used K coefficient. MATERIAL: Seventeen COPD pts (6 weeks free of exacerbation) at the age 47-80 (average 64.2 years +/- 9.7) were examined (13 male), average FEV1 61% predic. value (21-81, +/- 15). All patients were active smokers (average 42 pack years +/- 22.8) and all suffered from bronchitic (daily sputum production) phenotype of COPD. RESULTS: We did not find any difference in average ciliary beat frequencies between nose (6.0 Hz +/- 1.3) and bronchus (5.9 +/- 1.3) locality (p = 0.427). We find weak association between nose and bronchus in "ciliary akinesia" (kappa = 0.282) but medium association in "metaplasia" (kappa = 0.485), in index of dyskinesia (kappa = 0.733). CONCLUSION: We did not find in our data any difference in nasal and bronchial ciliary beat frequencies and we found medium association between nasal and bronchial spinocellular metaplasia and index of ciliary dyskinesia. Possible generalization of these results would require further investigation and analysis.

Repeat chemosensitivity of epithelial ovarian carcinoma in a BRCA1 mutation carrier to paclitaxel/platinum combination chemotherapy.

We present here a case of a BRCA1 mutation carrier with repeat responsiveness of recurrent EOC to paclitaxel/platinum. The patient had complete response to the combination of paclitaxel/platinum in the first line. Subsequent four recurrences also showed a complete response to this combination. The chronic toxicity, including hypersensitivity and nephrotoxicity could be controlled by modifying the regimen. In conclusion, recurrent EOC in BRCA1 mutation carriers may retain sensitivity to paclitaxel/platinum combination chemotherapy, and this combination could be therapy of first choice in this patient population.

Meningeal carcinomatosis as a late complication of brain metastases of epithelial ovarian carcinoma.

The brain represents a rare site of metastasis in patients with epithelial ovarian carcinoma (EOC). In recent decades there has been an apparent increase in the number of EOC patients diagnosed with brain metastases, probably as a result of improved prognosis of patients with advanced tumors, but cases of meningeal carcinomatosis complicating EOC remain rare. A patient with Stage III EOC had brain metastases diagnosed 31 months after primary surgery. The isolated brain metastases were controlled with radiosurgery, surgery and chemotherapy. Forty-five months after the diagnosis of brain metastases, meningeal carcinomatosis was diagnosed which led, despite intrathecal therapy, to a fatal outcome. At autopsy, the disease was limited to the central nervous system. Meningeal carcinomatosis may represent a late fatal complication of brain metastases of EOC.

[Appendiceal mucocele in differential diagnosis of tumors in pelvic region]. / Mukokéla apendixu v diferenciální diagnóze tumoru malé pánve.

OBJECTIVE: To report two cases of appendiceal mucocele in patients with suspected gynecological pathology. DESIGN: Case report. SETTING: Department of Obstetrics and Gynecology, Medical Faculty of Charles University and Faculty Hospital Hradec Králové. SUBJECT AND METHOD: We report two cases of appendiceal mucocele diagnosed and treated at our department. CONCLUSION: Appendiceal mucocele is a rare pathology, characterized by abnormal accumulation of mucous in appendix lumen. It is four times more common in females with a mean age of about 55 years. The pathogenesis could be neoplastic or non-neoplastic. Appendiceal mucocele with its anatomic location must be considered in terms of differential diagnosis of masses in pelvic region. Preoperative diagnosis is important, alerting the surgeon of an unintended rupture during surgery and avoiding the development of pseudomyxoma peritonei. US and CT were reported to be valuable.

[Chemoresistance/Chemosensitivity of ovarian cancer--a case report]. / Chemorezistence/chemosenzitivita ovariálního karcinomu--kazuistika.

OBJECTIVE: To describe chemoresistance and chemosensitivity of two ovarian cancer patients and influence on their medical outcome. SETTINGS: Department of Obstetrics and Gynecology, Medical Faculty Charles University Prague and University Hospital, Hradec Králové. SUBJECT AND METHOD: A case report of two ovarian cancer patients with defined chemoresistance/chemosensitivity of their tumors. We used WST-MTT-1 test for the assessment of chemoresistance/chemosensitivity. CONCLUSION: The chemoresistance/chemosensitivity assay would improve the treatment and prognosis of ovarian cancer patients.

[Sclerosing stromal tumor--a rare benign ovarian neoplasm]. / Sklerozující stromální tumor--vzácný gonadostromální nádor ovaria.

Sclerosing stromal tumor (SST) is a rare benign ovarian neoplasm of the sex cord-stromal category occurring predominantly in young women (usually younger than 30 years of age). Histologically, the tumor is characterized by cellular heterogenity, prominent vascularisation, and a pseudolobular appearance composed of cellular and hypocellular areas. We report a case of the ovarian SST in a 17-year-old girl. Clinical, microscopic, immunohistochemical, ultrastructural findings and differential diagnosis are disscussed.

[News in the examination of the ciliary activity in the respiratory tract mucosa]. / Novinky ve vysetování aktivity rasinek sliznice respiracního traktu.

Ciliary activity assessment attracts a great interest and has undergone dynamic progress since the last decades. The ciliary function is typically impaired in primary ciliary dyskinesia (PCD). The cilia are mostly examined in vitro, the sample is taken away from the nasal mucosa. Digital videomicroscopy is accessible for the cilia observation in vitro and represents a gold standard. Manual reading using this method is lengthy in comparison with a photomultiplier. Using the photomultiplier, light captured in an optical fibre is analysed automatically by a computer. With technological progress other methods gain ground such as automatical analysis of variance of grey degrees or photomultiplier connected with a scanning confocal microscope. A medium 199 is mostly used for preservation of the functioning cilia. To retain ciliary beat frequency (CBF) it is necessary to use a heated microscope stage. Average physiological CBF value is 11.5 Hz in adult population and 12.8 Hz in children.

[Rare tumours of myometrium--intravenous leiomyomatosis and benign metastatic leiomyoma]. / Vzácné nádory myometria--intravenózní leiomyomatóza a benigní metastazující leiomyom.

Leiomyomas are the most common benign uterine neoplasms of the myometrium. Rarely, can be observed unusual manifestations of these tumours. We present a case of intravenous leiomyomatosis in a 52-year-old woman and a case of benign metastasizing leiomyoma in a 45-year-old woman. Intravenous leiomyomatosis is an uncommon condition in which morphologically benign smooth muscle is present within the lumina of the veins. Benign metastasizing leiomyoma is a very rare phenomenon where histologically benign smooth muscle tumours are present at distant sites, particularly in the lungs, in women having histologically benign uterine leiomyoma. The diagnostic features, etiology, histology, therapeutic management and prognosis of these extremely rare conditions are discussed.

Mucoepidermoid carcinoma of the breast.

Mucoepidermoid carcinoma of the breast is a very rare type of neoplasm with a very distinct histology, immunohistochemistry as well as prognostic characteristics. Two cases of this type of breast carcinoma are presented. Both tumors were microscopically composed of intermediate, epidermoid and glandular cells. The first case was a high grade tumor with focal necroses, where epidermoid and mucinous cells were found only as isolated elements. The second case was of low grade, the squamous cells showed keratinization and glandular cells formed distinct small lumina. The prognostic characteristics, differential diagnosis, grading system and immunohistochemical profile of these rare neoplasms are described.

[Angiomyxoma of the vulva--a case report]. / Angiomyxoma vulvae--kazuistika.

OBJECTIVE: The authors demonstrate the case of a rare vulvar angiomyxoma in a 57-year-old female. DESIGN: Case report. SETTINGS: Department of Obstetrics and Gynecology, Medical Faculty Charles Uni Prague and Faculty Hospital, Hradec Králové. SUBJECT AND METHOD: A rare case of pedunculated angiomyxoma involving the vulva of a 57-year-old female is reported. Complete excision of the tumor was performed. CONCLUSION: Angiomyxoma is a rare locally infiltrative neoplasm of the vulvovaginal region, perineum and pelvis. Notorious recurring after surgical resection is due to its infiltrative border that makes it difficult to attain a cleared margin at the time of resection.

[Lipomatous hypertrophy of the cardiac interatrial septum]. / Lipomatózní hypertrofie mezisínového srdecního septa.

Lipomatous hypertrophy of the interatrial septum (LHIS) is a rare lesion of unknown origin usually diagnosed as an incidental finding during autopsy. It can be associated with supraventricular arrhythmias, venous return obstruction and sudden cardiac death. Five necropsy cases (4 females, 1 male) of LHIS were encountered during the last 31 years; only one case from this series was diagnosed ante mortem. The patients' mean age was 68 years; their mean BMI was 28.4. The mean size of the lesion was 31 mm. In three patients the LHIS was asymptomatic, two patients experienced relapsing multifocal atrial tachycardia and sick sinus syndrome, respectively. Histologically, all cases consisted of a mixture of mature and brown adipose tissue with foci of cardiomyocytes. For a pathologist the knowledge of LHIS is important because of an increasing possibility of its ante mortem diagnosis by imaging methods with a following endomyocardial biopsy, and also because it may appear as a cause of sudden cardiac death.

[Small bowel carcinoid]. / Karcinoid tenkého streva.

Tumors of the small intestine can be fatal from the oncologic point of view. Its quite sporadic incidence and absence of early clinical symptoms lead to its very difficult diagnosis. Carcinoid stays as the second most often tumor of the small intestine and it is qualified by some authors as semimalignant. The case report presented is aimed at that issue.

[Prognostic markers in ovarian carcinoma--retrospective study]. / Prognostické markery u ovariálního karcinomu--retrospektivní studie.

Ovarian carcinoma is one of the most common cancers in women. The high mortality is due mostly to the fact that the tumour is frequently diagnosed in advanced stages. The aim of our study was to find immunohistochemically detectable significant prognostic markers for invasive ovarian carcinoma. There were two areas of research: the expression of hormonal receptors by tumour cells, and the examination of proliferation activity of the tumour cell by means of antibody Ki-67. Tumour samples from 96 patients with carcinoma of ovary were evaluated (age 27-82 years, mean 55.2 years). Size of residual tumour (p = 0.00002), FIGO stage (p = 0.001), age (p = 0.018), expression of progesterone receptors (p = 0.004), coexpression of steroid receptors (p = 0.039), proliferation activity of the tumour cell (p = 0.04), and chemotherapy (p = 0.018) were significant predictors of survival in univariate analysis. Borderline significance was found in other evaluated parameters: grade (p = 0.063) and histology of carcinoma (p = 0.085). Expression of estrogen receptors and radiotherapy were not correlated to survival in univariate analysis. Multivariate analysis revealed that only clinical parameters were significant prognostic factors: size of residual tumor (p < 0.0000), chemotherapy (p = 0.0009), radiotherapy (p = 0.0097), and age (p = 0.0048).