[Familiar Mediterranean fever in Czech Republic]. / Familiární stredozemní horecka v Ceské republice.

Familiar Mediterranean fever (FMF) is a well defined autosomal recessive disease occurring mostly in Mediterranean regions. Here we present the experience from one center from Czech Republic, where we follow 4 families with patients with genetically proven FMF. Three out of these 4 families cluster to one limited region in Moravia, in the heart of Europe, without any linkage to Mediterranean origin. Furthermore, majority of these patients are heterozygots presenting with well defined typical clinical symptoms. Potential pseudodominant inheritance and/or epigenetic and environmental factors might influence clinical presentation of the disease.

Radiotherapy in the treatment of epididymal adenocarcinoma - case report.

BACKGROUND: Primary epididymal carcinoma is an extremely rare disease with a bad prognosis. We describe the first report of radiotherapy for definitive treatment of such a tumour. CASE REPORT: A 35-year-old man with a tumour of the epididymis underwent orchiectomy and elective retroperitoneal lymphadenectomy. Adenocarcinoma of the epididymis and lymph node metastasis were confirmed. The patient refused the suggested adjuvant chemotherapy and returned to our centre with a local recurrence after 6 months. We indicated palliative radiotherapy to the scrotal and groin region. Complete remission was achieved and continues 3.5 years after treatment. Late effects included teleangiectasia, fibrosis and thickening of subcutaneous tissue, and depigmentation in the groin, but the patient enjoys very good quality of life including penile erection. CONCLUSION: We presented very good results of radiotherapy in the treatment of this rare malignant disease. We achieved complete remission with a good quality of life and with tolerable chronic toxicity.

Indications for venous access in oncology - recommendations of national professional societies and current state in the Czech Republic.

BACKGROUND: The aim of the paper is to present the current recommendations and indications of venous access in oncology which reflect and recognize the opinions of national and international professional societies. It focuses exclusively on the indications of intravenous catheter placement for anticancer treatment, such as medium-term and long-term venous accesses. MATERIALS AND METHODS: The survey results obtained from a national questionnaire of 24 oncology centers identified the current situation in the Czech Republic. There were evaluated relevant data on the number of and the criteria for the introduction of venous accesses provided by physicians. Comparisons were made between current oncological practice and recommendations provided by evidence-based medicine. RESULTS: At each center surveyed in the Czech Republic, an average of 130 ports and 80 permanent implanted central catheters are introduced annually. The ports are increasingly indicated, with over a half of the centers surveyed introducing ports to more than 100 patients a year, with four centers introducing a total of 1,600 ports annually. In all centers, the decision for venous access is made by an oncologist. However, most procedures are performed by a doctor of another specialization, most often by a surgeon, a radiologist or an anesthesiologist. More than a half of the indications for venous access placement result from poor peripheral venous system or complications of parenteral therapy, not from comprehensive assessment prior to the initiation of the therapy. CONCLUSION: Based on our findings, we developed general indications and recommendations for venous access to cancer patients which represent the consensus of an interdisciplinary team of specialists, predominantly from the committee of professional societies - the Society for Ports and Permanent Catheters, the Working Group of Nutritional Care in Oncology of the Czech Oncological Society and the Society of Clinical Nutrition and Intensive Metabolic Care. The number of introduced venous access catheters remains insufficient to meet the needs in the Czech Republic, which necessitates increased awareness and possibilities for safe drug administration.

Extravasation (paravasation) of chemotherapy drugs - updated recommendations (2020) for standard care in the Czech Republic from the cooperation of the Supportive Care Group of the Czech Society for Oncology, Czech Society for Hematology, Oncology Section of the Czech Nurses Association and the Society for Ports and Permanent Catheters.

BACKGROUNDS: Extravasation (paravasation) of chemotherapy drugs is a very significant complication. Preventive and therapeutic interventions reduce the risk of the complication or the extent of its consequences. A working group of authors from expert groups prepared recommendations for standard care. PURPOSE: A basic summary of recommended interventions for daily practice, defined on the basis of knowledge from long-term, proven, evidence-based practice or on the consensus opinions of the expert groups representatives. RESULTS: Preventive measures are essential and include early consideration of long-term venous access devices indications, choice of injection site, venous line control before each chemotherapy drug application, and patient education. The intervention in case of extravasation mainly involves the application of antidotes (DMSO, hyaluronidase, dexrazoxane) and the application of dry cold or heat according to the type of cytostatic drug. Subcutaneous corticosteroids, moist heat or cooling and compression are not recommended. CONCLUSION: The recommended procedures contribute to reducing the risk and consequences of extravasation. The range of recommended interventions can be expanded individually depending on individual clinical site policy and needs.

Aggressive multimodal therapy may prolong disease-free survival in recurrent primary retroperitoneal embryonal carcinoma.

INTRODUCTION: Primary retroperitoneal extragonadal tumours relapsing after initial chemotherapy have a poor prognosis. PRESENTATION OF THE CASE: We report a case of primary retroperitoneal embryonal carcinoma in a patient with negative open testes biopsy. After the first line of chemotherapy (4 cycles BEP) secondary surgery with extirpation of a retroperitoneal residual mass was performed. The residuum proved histologically to be a mature teratoma, and no adjuvant treatment was given according to current recommendations. The patient had regular follow-up. 3.5 years later, patient developed recurrence in the ipsilateral adrenal gland, which was treated with surgery and 4 cycles of salvage VeIP chemotherapy. Seven months after the second surgical intervention the patient underwent multivisceral "desperation surgery" for early metastatic disease progression followed by 2 cycles of salvage TIP chemotherapy. The patient is currently disease-free at 34 months. CONCLUSION: Initial postchemotherapy retroperitoneal lymph node dissection is crucial for local retroperitoneal disease control. Aggressively treated metastatic recurrent disease does not preclude prolonged survival. Despite a generally poor prognosis, repeated complex oncosurgical therapy for retroperitoneal extragonadal tumours may be worthwhile.