Academic Study XR-TEMinDREC - Combination of the Concomitant Neoadjuvant Chemoradiotherapy Followed by Local Excision Using Rectoscope and Accelerated Dispensarisation and Further Treatment of the Patients with Slightly Advanced Stages of Distant Localized Rectal Adenocarcinoma in MOÚ.

BACKGROUND: Miniinvasive approaches are a long-term trend in surgery. Maximum possible quality of life after treatment of rectal cancer is a long-term goal. Adequate radicality of surgery is a long-term necessity. It is sometimes very difficult to fulfill all the above-mentioned requirements in low-level rectal cancer. By applying a multidisciplinary approach in the treatment of mildly advanced stages of low-seated malignant rectal tumor, a treatment procedure resulting in continence preserving can be offered to a selected group of patients meeting the strict indication criteria. We document our results with respect to a small number of patients in several interesting case reports. CASES: We are following up one patient after ideal treatment course achieving downstaging after neoadjuvant treatment, with uncomplicated operation and after operation period and with a long-term complete remission. One patient achieved dehiscence of the rectum suture. After secondary healing we observed a long-term remission. In one patient a rectovaginal fits developed outside the operation site. We were forced to abdominoperineal amputation. The pathological investigation of the specimen proved radically of the local excision and lack of lymphangioinvasion; nevertheless, a positive perirectal lymph node was found. The last case report shows the limits of imaging dia-gnostics. The liver lesions described as benign were in fact liver metastases of the early rectal cancer. CONCLUSION: According to the worldwide data available, the combination of neoadjuvant chemoradiotherapy and local excision by means of an operative rectoscope is a safe alternative to a resection surgery with total mesorectal excision in T2N0 rectal cancer. However, there is a need of other studies with more patients included, optimally randomized and prospective ones, which will support these claims. Supported by MH CR - DRO (MOÚ, 00209805). The authors declare they have no potential conflicts of interest concerning drugs, products, or services used in the study. The Editorial Board declares that the manuscript met the ICMJE recommendation for biomedical papers.

Primary synovial sarcoma of the ovary and Fallopian tube - case report and review of the literature.

OBJECTIVE: Case description of advanced biphasic synovial sarcoma in the tubo-ovarian area. DESIGN: Case report. SETTING: Department of Pathology, Znojmo Hospital. METHODS: Own observation, review of the literature. CONCLUSION: The diagnosis of synovial sarcoma must be considered in all spindle cell and undifferentiated tumours in various anatomical sites including female reproductive organs.

Utilization of Next Generation Sequencing in Analysis of Circulating MicroRNAs as Predictive Biomarkers for Patients with Locally Advanced Rectal Carcinoma.

BACKGROUND: MicroRNAs (miRNA) are short non-coding RNAs involved in post-transcriptional regulation of gene expression. MiRNAs are essential regulators of both physiological processes as of pathogeneses of many diseases, and their dysregulation was observed in many malignancies including rectal cancer. Circulating miRNAs presented in blood plasma could be potential candidates for non-invasive predictive biomarkers of the response of patients with locally advanced rectal cancer to chemoradiotherapy. Presented study aims to evaluate the potential of next-generation sequencing in the analysis of circulating miRNAs. MATERIAL AND METHODS: MiRNA expression profiles were done using samples of RNA isolated from blood plasma collected during TNM restaging and paired samples collected before initiation of neoadjuvant chemoradiotherapy. Sequencing libraries were prepared using kit which implements universal molecular indices that help to sensitively filter biological bias during data analysis. Sequencing data were processed by multidimensional biostatistical approaches. CONCLUSION: We identified specific miRNA profile enabling to distinguish the patients accordingly to their response to chemoradiotherapy. This work was supported by the Czech Ministry of Health grant No. 16-31765A. The authors declare they have no potential confl icts of interest concerning drugs, products, or services used in the study. The Editorial Board declares that the manuscript met the ICMJE recommendation for biomedical papers. Submitted: 22. 2. 2019 Accepted: 27. 2. 2019.

[Malignant tumors of the small bowel]. / Maligní nádory tenkého streva.

INTRODUCTION: Malignant tumors of the small bowel are relatively uncommon neoplasms; their incidence is around 1.5 per 100,000 inhabitants in the Czech Republic. METHOD: 104 patients underwent a resection of the small bowel because of a primary or secondary tumor over the 10-year period between 20062015 at the Masaryk Memorial Cancer Institute. Data from these patients was reviewed retrospectively. Survival rates were analyzed using the Kaplan-Meier method. RESULTS: We observed 45 primary and 59 secondary tumors. The group of primary malignancies included 12 adenocarcinomas, 14 cases of GIST and 19 cases of NET. Neuroendocrine tumors showed the best prognosis with a 62% probability of five-year survival. Patients with gastrointestinal stromal tumors had a 39% probability of five-year survival and those with adenocarcinomas had a 32% probability of five-year survival. Secondary tumors were caused mostly by metastatic colorectal carcinoma (22 times), malignant melanoma (11 times) and ovarian cancer (10 times). The probability of five-year survival was 15% in the group of secondary tumors. CONCLUSION: Malignant tumors of the small intestine are rare, but their incidence has increased in the last decades. The main prognostic factors include advancement of the tumor and radicality of surgical resection.Key words: small bowel tumors - adenocarcinoma - sarcoma - NET - GIST - lymphoma.

[Carney triad]. / Carneyho trias.

Carney triad is a synchronous or metachronous association of gastric gastrointestinal stromal tumors (GIST), pulmonary chondroma and extra-adrenal paraganglioma. The majority of patients have only one or two components of the triad, all three tumors being found in only about 2% of the patients at the time of the first diagnosis. The most common combination is gastric and pulmonary tumors. We report a case of Carney triad which was diagnosed at Masaryk Memorial Cancer Institute. A 57-year-old female patient with a history of gastric resection for leiomyosarcoma at the age of 14 and with an unclear pulmonary lesion evident on chest X-ray since as early as 2003. She was referred to our Clinic of Comprehensive Cancer Care after being diagnosed with unspecified tumors of the stomach, the left retroperitoneum and two liver metastases. Biopsy of the retroperitoneal mass was performed and histological examination showed pheochromocytoma. The patient underwent resection of the retroperitoneal tumor and wedge resection of the gastric tumor, left hemihepatectomy and left adrenalectomy (in two separate operations). The excised gastric tumor was a gastrointestinal stromal tumor (GIST) with a low risk of malignancy. Analysis of a liver specimen, however, showed two GIST metastases. No pathology was found in the left adrenal gland and the retroperitoneal tumor was positive for chromogranin A. Paraganglioma was thus diagnosed. Subsequently, mutational analysis of genes coding for succinate dehydrogenase subunits B, C and D (SDHB, SDHC, SDHD) and analysis of DNA methylation at the gene locus of SDHC was made. Carney triad was thus confirmed and the unclear pulmonary lesion could be described as benign chondroma. This report demonstrates the difficulty in distinguishing between Carney triad and Carney-Stratakis syndrome. Molecular information should improve the diagnosis of Carney triad.Key words: Carney triad - GIST pulmonary chondroma extraadrenal paragangliomaCarney-Stratakis syndrome.

[Mixed adenoneuroendocrine carcinoma (MANEC) of the gastrointestinal tract]. / Smísený adenoneuroendokrinní karcinom (MANEC) gastrointestinálního traktu.

Mixed adenoneuroendocrine carcinoma (MANEC) is a rare tumour of the gastrointestinal tract composed of adenocarcinoma and neuroendocrine carcinoma. This pathologic diagnosis was recently defined by the WHO in 2010. The tumour may appear in various levels of the digestive tract including the oesophagus, stomach, colon and appendix. Biological behaviour of MANEC seems to be quite unpredictable and the prognosis uncertain. The disease may also mimic "simple" carcinoma. Only several tens of cases have been reported so far.Our first case was diagnosed as gastric adenocarcinoma mimicking early gastric cancer in a 47 years old male. The second case was diagnosed incidentally in the appendix of a 69 years old female after operation for gynaecologic cancer. The disease was localised in both patients; the patients underwent radical surgical resections.Treatment options have not been standardised yet, but aggressive therapeutic measures are recommended. The patients may benefit from multimodal anticancer therapy.Key words: mixed adenoneuroendocrine carcinoma - MANEC - gastric cancer - appendiceal cancer.

[Von Meyenburg complexes - multiple biliary hamartomas mimicking metastatic liver lesions]. / Von Meyenburgovy komplexy - mnohocetné biliární hamartomy imitující metastatický jaterní proces.

Von Meyenburg complexes are benign liver lesions usually consisting of dilated bile ducts surrounded by fibrous stroma. Their discovery is usually incidental and unsuspected during the early phase of the operative procedure. The sovereign diagnostic method is intraoperative frozen section examination. The complexes are not important as regards their clinical or functional significance. However, this uncommon entity should be taken into consideration in the framework of differential diagnosis of metastatic liver lesions.

[Treatment of gastrointestinal stromal tumors--a comprehensive view of the surgeon]. / Lécba gastrointestinálních stromálních tumoru--komplexní pohled chirurga.

INTRODUCTION: Gastrointestinal stromal tumors (GIST) are specific mesenchymal tumors of the gastrointestinal tract. Most of GISTs (95%) result from activating mutations in one of the receptor tyrosine kinase proteins (KIT). Tumor cells express this protein. GIST is most common in the stomach and small intestine, but may occur anywhere in the gastrointestinal tract and intra-abdominal soft tissues. The variety of its clinical presentations is related to localization of the tumor, its size and relationship to surrounding organs. Surgery is the first choice of treatment for patients with localized or potentially resectable tumors with the intention of R0 resection. Targeted therapy with imatinib (a selective inhibitor of the KIT protein) is the primary option for patients with metastatic GIST, as adjuvant treatment after surgery or neo-adjuvant therapy prior to surgery in indicated cases. This paper describes comprehensive therapy of GIST with an emphasis laid on the status of surgical treatment, and it highlights some controversial topics, e.g. the role of surgery for metastatic disease or neo-adjuvant targeted therapy. CONCLUSION: GIST is a relatively rare tumor most commonly affecting the stomach and small intestine. Surgical treatment is not replaceable in the treatment strategy of this disease. The diagnostic-therapeutic approach to patients with GIST must be comprehensive. Due to prescribing restrictions of biological therapies, these patients are inevitably directed to specialized centers where surgical treatment should also be preferably provided.

[Male breast cancer--our experience]. / Karcminom prsu u muzu--nase zkusenosti.

INTRODUCTION AND AIM: Male breast cancer (MBC) is a rare disease which represents only about 0.2% of all cancers. The ratio of female to male breast cancer is approximately 100:1. In this study, we review our experience with diagnosis, surgical treatment, and overall management of patients with MBC. METHODS: Overall, 5384 breast cancer patients underwent surgery in Masaryk Memorial Cancer Institute, Brno, Czech Republic during the period of 2001 to 2009. Among these, seventeen were men. In these patients, data on incidence, clinical symptoms, diagnostic procedures, types of surgical resection, histopathology, adjuvant systematic therapy, genetic investigation and therapeutic results were retrospectively reviewed from their medical records. RESULTS: None of the patients have distant metastases at the time of surgery. In sixteen cases an invasive cancer was histopathologically diagnosed, one patient had ductal carcinoma in situ (DCIS). Eight patients exhibited locally advanced stage of the disease (IIIB). All patients were primarily treated surgically; in no case neoadjuvant therapy was recommended. From the five patients operated primarily elsewhere, four underwent completion of modified radical mastectomy (RAME) at our institution and one patient underwent subcutaneous (nipple-sparing) mastectomy without the surgery on lymph nodes. Twelve patients were primarily diagnosed and operated at our institution. Ten of these were scheduled for RAME, one patient was recommended total mastectomy with sentinel lymph node biopsy, and one patient underwent total mastectomy without surgery on regional lymph nodes. Adjuvant therapy overall comprised radiotherapy, chemotherapy, hormonal therapy, and biological therapy. Investigation of BRCA genes was suggested in nine patients, but the results of only four of them are available to date. Two patients exhibited a mutation in BRCA genes. One mutation is explicitly pathogenic; another case represents variation with unknown clinical effect. Twelve patients of the cohort are in complete remission, two are alive with distant metastases and three died (two on cancer, one on cardiovascular disease). CONCLUSION: Our study should point out a rare form of mammary tumors--male breast cancer. There are some particular differences between male and female breast cancer though the overall clinical management of both is generally similar. There is a necessity of appropriate health education to prevent late diagnosis of breast cancer in men. Genetic testing should be recommended to every male patient with this disease.

[Use of local hemostyptic drugs in liver resections]. / Vyuzití lokálních hemostyptik pri jaterních resekcích.

INTRODUCTION: Bleeding during and following liver resection continues to be an object of surgeon's attention and a potential source of significant morbidity. Topical hemostatic agents are used during surgical intervention when conventional methods are not sufficient because of the site of surgery or degree of bleeding. A variety of agents with different effects is now available. AIM: To verify the efficacy and safety of cellulose based local hemostat during liver resections. METHODS: In 67 cases of liver resection the surface was covered by Traumacel TAF net which was left in place. RESULTS AND CONCLUSION: There were no postoperative bleeding in this group of patients. No other serious complication has been observed. Traumacel is a safe and effective local hemostat during liver resections.

Hemostatic efficacy of fibrin sealant (human) on expanded poly-tetrafluoroethylene carotid patch angioplasty: a randomized clinical trial.

PURPOSE: The efficacy of solvent-detergent-treated fibrin sealant (human [FSH]) for controlling anastomotic bleeding from expanded polytetrafluoroethylene (ePTFE) patch angioplasty during carotid endarterectomy was evaluated, and FSH was compared with thrombin-soaked gelatin sponge (Gelfoam; TSG). METHODS: The study was of a randomized, open-label, single-site, single-treatment, parallel design that took place in a referral center with hospitalized patients. Forty-seven adult patients (33 men, 14 women) underwent elective carotid endarterectomy. Patients were randomized to receive either FSH (N = 24) or TSG (N = 23). FSH was obtained as an investigational new drug. FSH was applied as a liquid by means of a dual-syringe technique. Heparin anticoagulation, patch thickness, and suture type were standardized. Two different needle sizes were used (CV-6, PT-13: N = 21 [FSH: N = 10, TSG: N = 11]; CV-6, PT-9: N = 26 [FSH: N = 14, TSG: N = 13]). The FSH or TSG was applied to the ePTFE patch, and then blood flow was restored through the carotid artery. Degree of anticoagulation was assessed by anti-factor Xa activity. The time from restoration of carotid blood flow until achieving hemostasis was recorded. The blood loss from patch suture hole bleeding was measured. Completion intraoperative duplex ultrasound scanning was performed in all cases. Heparin was reversed with protamine sulfate. The primary end point was successful hemostasis within 15 minutes of restoration of carotid blood flow. The secondary end points were the amount of blood loss caused by suture line bleeding and the time to achieve hemostasis. RESULTS: There was no difference in the number of patients with complete hemostasis at 15 minutes (TSG, 13 of 23; FSH, 12 of 24; P =.77). The measured blood loss was 99.0 +/- 119.9 (SD) mL for TSG, and 105.0 +/- 107.9 mL for FSH (P =.86). The time to hemostasis was the same for both groups (TSG, 16.5 +/- 16.5 minutes; FSH, 16.6 +/- 14.2 minutes; P =.97). Within both treatment groups, the use of larger needles (PT-13) was associated with greater blood loss (FSH, 169.7 +/- 124.2 mL; TSG, 172.7 +/- 151.5 mL) than was the use of smaller needles (PT-9; FSH, 58.8 +/- 66.3 mL; TSG, 34.1 +/- 25.6 mL; P =.036, P =.001, respectively). There were no postoperative strokes or bleeding complications in either group. No abnormalities were shown in either group by means of completion carotid duplex ultrasound scanning. CONCLUSION: FSH was equivalent, but not superior to, TSG in achieving hemostasis during carotid endarterectomy performed with ePTFE patch angioplasty. Adhesion properties of FSH to ePTFE are possibly different than those to native tissue and warrant additional investigation.

Primary stenting of atherosclerotic renal artery ostial stenosis.

Percutaneous transluminal angioplasty for atherosclerotic ostial lesions of the renal arteries has resulted in high restenosis rates. Recent reports of angioplasty with intravascular stenting show improved results over angioplasty alone. The purpose of this study is to review the results of primary stenting of ostial renal artery stenosis at our institution. Twenty one patients (11 men, 10 women, age 63 +/- 11 years), with atherosclerotic renal artery ostial stenosis in association with hypertension or renal insufficiency underwent renal angioplasty with primary stenting during a 2-year period. Medical records were reviewed for indications, technical success, complications, restenosis, response of hypertension and response of renal insufficiency. A technical success was defined as a normal postprocedure arteriogram with no residual stenosis and no residual gradient. Restenosis was defined as > or =60% diameter reduction identified by arteriography, or duplex scan demonstrating a renal artery to aortic ratio of > or =3.5. Thirty-three stents were placed in 25 arteries with four patients having bilateral procedures. All patients were hypertensive. Nine patients (43%) had chronic renal insufficiency (creatinine > or =1.5 mg/dl). One patient was on hemodialysis. The immediate technical success rate was 95%. Six complications occurred in four patients (two pseudoaneurysms, two dissections requiring additional stents, renal failure, and atheroembolization). Mean arterial blood pressure improved from 117 +/- 13.4 to 103 +/- 12.8 mmHg (p = 0.002) after stenting. Serum creatinine levels decreased from 1.48 +/- 0.57 to 1.31 +/- 0.41 (p = 0.07). Eight patients developed restenosis. The mean follow up was 13 +/- 7 months. Life table analysis showed a cumulative restenosis rate of 65 +/- 18% at 24 months. We advise caution in the application of renal stenting for the treatment of ostial lesions, particularly in patients for whom standard surgical revascularization options are available.

Presentation and management of venous aneurysms.

PURPOSE: Venous aneurysms have been reported to occur in most major veins. These aneurysms may be misdiagnosed as soft tissue masses or as inguinal or femoral hernias. Venous aneurysms of the deep system have been associated with deep venous thrombosis (DVT) and pulmonary embolism (PE). To more precisely characterize these lesions, we reviewed our experience with the disease. METHODS: A retrospective analysis of our experience over 22 years was performed. The presentation and management of these lesions were reviewed and compared with the literature. RESULTS: Thirty-nine venous aneurysms were reported in 30 patients. There were 14 men and 16 women. The patients' ages ranged from 3 to 75 years. Thirty aneurysms were located in the lower extremities, four in the upper extremity, and five in the internal jugular vein. Fifty-seven percent of lower extremity aneurysms occurred in the deep system. Patients' symptoms were a mass (75%) associated with pain (67%) and swelling (42%). Thromboembolism occurred in six patients, DVT in three, and PE in three. Eight of nine patients (89%) who had aneurysms of the superficial venous system had their condition misdiagnosed. Diagnosis was made by phlebography (60%), color flow duplex scanning (27%), continuous-wave Doppler scanning (10%), or magnetic resonance imaging (10%). The aneurysm size ranged from 1.7 to 6.0 cm. Management consisted of tangential excision in five (17%), total excision in 23 (77%), and observation in seven (6%). CONCLUSIONS: Venous aneurysms are unusual vascular malformations that occur equally between the sexes and are seen at any age. Most patients have a painful mass of the extremity, and diagnosis is achieved by radiologic examination. Superficial venous aneurysms of the inguinal region are often misdiagnosed. Thromboembolism is more common in aneurysms involving the deep venous system. Because of their potential morbidity, management should be surgical in the majority of cases.