• Klíčová slova
• AUTOMATIC DATA PROCESSING *, CZECHOSLOVAKIA *, DERMATOLOGY *, MEDICAL RECORDS *, PUNCHED-CARD SYSTEMS *,
• MeSH
• automatizované zpracování dat * MeSH
• chorobopisy * MeSH
• dermatologie * MeSH
• děrnoštítkové systémy * MeSH
• dokumentace * MeSH
• lidé MeSH
• publikace * MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH
• Geografické názvy
• Československo MeSH

• MeSH
• antibakteriální látky terapeutické užití   MeSH
• inkubátory kojenecké MeSH
• lidé MeSH
• nemoci novorozenců prevence a kontrola   MeSH
• novorozenec MeSH
• porodní hmotnost MeSH
• těhotenství MeSH
• Check Tag
• lidé MeSH
• novorozenec MeSH
• těhotenství MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• Názvy látek
• antibakteriální látky MeSH

Goblet cell carcinoid (GCC) of the appendix is extremely rare, representing approximately 5% of all primary appendiceal neoplasms. Histologically there are three groups of GCC: group A (typical GCC), adenocarcinoma ex GCC signet ring cell type (group B), and adenocarcinoma ex GCC poorly differentiated carcinoma type (group C), which is the most aggressive. GCC metastasizes in 15-60% of cases, mainly to the ovaries, pelvis, abdominal cavity, ribs, vertebrae, and lymph nodes. Hematogenous metastasis to the liver or other parenchymal organs can occur, but this is very rare. The different organs metastases havent been described yet. The primary mode of treatment is radical surgical resection or debulking, followed by chemotherapy; however, patients with unresectable or recurrent GCC are candidates for systemic therapy. Here, we report a case of very aggressive GCC of the appendix, which had metastazed to the liver at the time of diagnosis and subsequently metastasized to the orbit.

• MeSH
• karcinoid sekundární   MeSH
• lidé MeSH
• nádory apendixu patologie   MeSH
• nádory orbity sekundární   MeSH
• pohárkové buňky patologie   MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• anglický abstrakt MeSH
• časopisecké články MeSH
• kazuistiky MeSH
• přehledy MeSH

The authors present an interesting case report of 69-year-old caucasian woman with Takotsubo cardiomyopathy. Takotsubo cardiomyopathy is a relatively recently described heart syndrome that probably develops due to the direct toxic effect of excessively released catecholamines on cardiac adrenoceptors during emotional or physical stress. The typical features include reversible left ventricular apical dyskinesis, chest pain with ST-T changes on ECG, minimal myocardial enzymatic release and the absence of coronary stenosis on coronary angiogram. Early coronary angiographic examination is highly recommended as the clinical picture of this syndrome mimics acute myocardial infarction. Betablockers are considered to be the treatment of choice.

• MeSH
• dysfunkce levé srdeční komory komplikace   diagnóza   MeSH
• echokardiografie MeSH
• elektrokardiografie MeSH
• kardiomyopatie komplikace   diagnóza   MeSH
• lidé MeSH
• senioři MeSH
• Check Tag
• lidé MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• anglický abstrakt MeSH
• časopisecké články MeSH
• kazuistiky MeSH

UNLABELLED: Lenalidomide has been licenced for the treatment of multiple myeloma and, in 2012, it is used as a standard treatment of relapses of the disease. Literature contains a number of publications on the effects of lenalidomide in myelodysplastic syndrome, in malignant lymphomas and chronic B lymphocytic leukaemia. The effects of the drug in rare diseases, however, have not been investigated so far. In this paper, we summarize our experience with lenalidomide in rare blood disorders. We observed an excellent effect of lenalidomide in multifocal aggressive, repeatedly relapsing Langerhans cell histiocytosis where it led to complete remission. This patient was treated with 2-chlorodeoxyadenosine and with CHOEP (cyclophosphamide, etoposide, doxorubicin, vincristine and prednisone) chemotherapy and high dose BEAM chemotherapy with autologous transplantation of haematopoietic tissue for an early disease relapse. Following another early relapse, the patient was treated with lenalidomide (25 mg). Treatment with lenalidomide induced complete remission on PET-CT. The patient was consolidated during the remission with a reduced intensity conditioning regimen and allogeneic transplantation of haematopoietic tissue. Following allogeneic transplantation, the patient has been in full remission for 10 months. We further showed an excellent effect of lenalidomide in multicentric Castleman disease with generalized involvement of lymphatic nodes, B symptoms and vasculitis. The patient was first treated R-CHOP chemotherapy (rituximab, cyclophosphamide, adriamycin, vincristine and prednisone). Due to a lack of efficacy, this was changed to the CVD combination (cyclophosphamide, thalidomide, dexamethazone). This treatment delivered complete remission but was complicated by thalidomide-associated neuropathy. Due to persistent neuropathy, thalidomide could not be used to manage further relapse and thus lenalidomide (25 mg, 11 cycles) was used. The patient has been in complete PET-CT remission for 7 months following this treatment. We observed partial efficacy in Erdheim-Chester disease. We used 2-chlorodeoxyadenosine as part of initial treatment that delivered partial regression of brain infiltrates only; fluorodeoxyglucose accumulation in the bones has not changed. Lenalidomide 25 mg was used as second line treatment. This led to complete regression of CNS infiltrates on MRI but fluorodeoxyglucose accumulation in bone lesions did not change. Regression of clinical signs and regression of fibrosis of retroperitoneum was achieved with an ongoing treatment with anakinra. A patient with multiple angiomatosis affecting the abdominal cavity, mediastinum and vertebrae and digestive tract had been stabilized with zoledronate (4 mg once every 2 months) and thalidomide (100 - 200 mg/den) for several years. However, several years of this treatment led to severe neuropathy. Consequently, we attempted to substitute thalidomide for lenalidomide. However, 10 mg of lenalidomide alone was not sufficiently effective and thus low dose of 50 mg of thalidomide was added. Combined treatment with zoledronate, lenalidomide 10 mg/day and thalidomide 50 mg/day stabilized the condition for 9 months. Due to relapsed gastrointestinal bleeding the treatment had to be changed after 9 months to thalidomide 100 mg/day and Sandostatin 0.1 mg twice daily s.c. A patient with osteosclerotic myeloma and POEMS syndrome was initially treated with CAD chemotherapy (cyclophosphamide, adriamycine and dexamethazone) that was followed by tandem high dose chemotherapy (melphalan 100 mg/m2) and autologous transplantation. Treatment with thalidomide was given due to insufficient efficacy but was not tolerated. Lenalidomide was administered as the fourth line treatment. Even though literature describes remission of POEMS syndrome following lenalidomide, four cycles did not lead to remission in our patient. CONCLUSION: We showed an effect of lenalidomide in Langerhans cell histiocytosis and in Castleman disease. The treatment led to regression of brain infiltrates in a patient with Erdheim-Chester disease. A dose of 10 mg of lenalidomide daily in combination with 50 mg of thalidomide stabilized a course of angiomatosis. Lenalidomide did not deliver the required treatment response in a patient with POEMS syndrome and multiple previous therapies.

• MeSH
• Erdheimova-Chesterova nemoc farmakoterapie   MeSH
• histiocytóza z Langerhansových buněk farmakoterapie   MeSH
• hyperplazie velkých lymfatických uzlin farmakoterapie   MeSH
• lenalidomid MeSH
• lidé středního věku MeSH
• lidé MeSH
• POEMS syndrom farmakoterapie   MeSH
• senioři MeSH
• thalidomid analogy a deriváty   terapeutické užití   MeSH
• Check Tag
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH
• přehledy MeSH
• Názvy látek
• lenalidomid MeSH
• thalidomid MeSH

BACKGROUND: Multiple primary lung cancer is a relatively rare nosological entity. This case report is a reminder and points out the pitfalls of its diagnosis and therapy. CASE REPORT: A 62-year-old patient was indicated for surgical therapy for non-small cell lung cancer of the middle lobe and right lung, which were diagnosed during a screening investigation after the patient had undergone previous mastectomy of the right breast with axillary dissection for invasive ductal adenocarcinoma. Another infiltration in the lower lobe of the same lung was removed at the same time and was classified as a primary lung carcinoma; it was a synchronous lung cancer. CONCLUSION: Lung cancer presenting with more than one primary lesion in the lung is a rare nosological entity that can be classified into two types; synchronous and metachronous. Whereas synchronous cancers arise in the lung at the same time, metachronous cancers develop after treatment of the initial lesion. The incidence of multiple lung cancer is increasing due to earlier diagnosis and because successful treatment of the initial cancer at an early stage has led to an increase in patient survival, resulting in an increase in the interval between detection of the initial cancer and detection of the second. Smoking is one of the main risk factors. Diagnosis is made difficult because metastatic disease must be excluded. Basic information is obtained from a biopsy of the tumor. The staging of more than one primary lung cancer is complex and needs to be meticulous if curative resection is being contemplated. Magnetic resonance imaging of the brain and fluorodeoxyglucose positron emission tomography should be performed to evaluate for extra-thoracic metastases. KEY WORDS: lung carcinoma - multiple cancer disease - synchronous - metachronous - diagnosis - therapyThe 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.Submitted: 22. 2. 2016Accepted: 20. 4. 2016.

• MeSH
• lidé středního věku MeSH
• lidé MeSH
• mnohočetné primární nádory diagnóza   diagnostické zobrazování   MeSH
• nádory plic diagnóza   diagnostické zobrazování   MeSH
• nemalobuněčný karcinom plic diagnóza   diagnostické zobrazování   MeSH
• PET/CT MeSH
• Check Tag
• lidé středního věku MeSH
• lidé MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH
• přehledy MeSH

The haemophagocytic syndrome (HPS) is clinically characterized by fever, pancytopenia and hepatosplenomegaly. Usually it takes an acute course with a high mortality. The pathogenetic basis is inadequate activation of the immune system--in particular Th1-lymphocytes with subsequent overproduction of cytokines and extreme activation of macrophages with haemophagocytosis. The activated cells infiltrate organs, cause tissue damage and clinical manifestations of the syndrome. From the etiological aspect two forms exist: primary (familial) with autosomal recessive inheritance and the secondary form which forms a heterogeneous sub-group, caused as a rule by infection and/or a tumour. The prognosis seems somewhat more favourable in secondary forms. In treatment which is essentially the same for both forms, chemotherapy combined with immunosuppression proves useful, in more aggressive forms chemotherapy as used in the treatment of non-Hodgkin lymphomas. The only curative method is transplantation of haematopoietic stem cells which is also the treatment of first choice in the familial form of haemophagocytosis. In the submitted paper the authors present a review of contemporary knowledge on this treacherous and relatively rare entity. The haemopgagocytic syndrome should be always taken into account in the differential diagnosis of fever with an obscure etiology. The authors assume that the haemophagocytic syndrome is rarely considered in practice and therefore is usually inadequately diagnosed and thus not treated in time. In the conclusion the authors describe the case-records of a 26-year-old female patient with haemophagocytic syndrome which developed during pregnancy.

• MeSH
• diferenciální diagnóza MeSH
• dospělí MeSH
• histiocytóza z non-Langerhansových buněk * diagnóza   etiologie   patofyziologie   terapie   MeSH
• komplikace těhotenství diagnóza   terapie   MeSH
• lidé MeSH
• těhotenství MeSH
• Check Tag
• dospělí MeSH
• lidé MeSH
• těhotenství MeSH
• ženské pohlaví MeSH
• Publikační typ
• anglický abstrakt MeSH
• časopisecké články MeSH
• kazuistiky MeSH
• přehledy MeSH

INTRODUCTION: Gastrointestinal stromal tumors (GIST) are common mesenchymal gastrointestinal tumors, however, their incidence rate is low. The tumors originate from progenitor cells of interstitial cells of Cajal-gastrointestinal pacemaker cells, and the majority of them express c-Kit, a tyrosin kinase receptor. The aim of this study was to assess the GIST treatment in a group of patients and to compare the outcomes with literature data. METHODS: The authors performed a retrospecitve analysis of all patients with histologically confirmed GISTs, who were operated in the 2nd Surgical Clinic of the Charles University Medical Faculty (LF UK) in Prague and in the Central Military Hospital Prague (UVN Praha), from 2003 to 2008. RESULTS: During the five-year period, 13 patients underwent surgery in the Central Military Hospital Prague. The commonest tumor locations were the following: stomach (46%), small intestine (duodenum 23%, jejunum 23%, ileum 8%). R0 resection was performed in 12 subjects (92%). 10 patients (77%) remain in remission, in one patient, the disease is stabilized (8%), and in one patient, the disease progression and generalization has been recorded (8%). CONCLUSION: Surgery is a standard treatment in localized tumors. Following radical resection, the patients benefit from adjacent treatment with tyrosin kinase inhibitors. Specific tyrosin kinase inhibitors have been shown effective in the treatment of metastatic and relapsing disorders. Primary surgical treatment in metastatic diseases remains a paliative option for patients with bleeding and obstruction.

• MeSH
• gastrointestinální stromální tumory * patologie   chirurgie   MeSH
• lidé středního věku MeSH
• lidé MeSH
• senioři nad 80 let MeSH
• senioři MeSH
• Check Tag
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• senioři nad 80 let MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• anglický abstrakt MeSH
• časopisecké články MeSH
• přehledy MeSH

Central diabetes insipidus with an onset in adulthood is very rare. Unlike in children, central diabetes insipidus in adults is more frequently caused by inflammatory processes and neoplastic infiltrations that do not originate from the neuronal tissue than primary neuronal tissue tumours. Rare histiocytic neoplasias (Langerhans cell histiocytosis, xanthogranulomatosis and Erdheim-Chester disease) have a specific affinity to hypothalamus and the pituitary stalk not only in paediatric patients but also when occurring in adults. We describe 3 cases of central diabetes insipidus with an onset in adulthood. Diabetes insipidus was the first sign of Langerhans cell histiocytosis in 2 patients, and it was the first sign of Erdheim-Chester disease in one patient. MR imaging showed pathological infiltration and dilated pituitary stalks in all 3 patients. PET-CT proved useful in differential diagnosis, showing further extracranial pathological changes either on the basis of significant glucose accumulation or on the basis of CT imaging. The Langerhans cell histiocytosis in the first patient has also manifested itself as an infiltration of the perianal area with intensive accumulation of fluorodeoxyglucose (FDG) - SUV 8.6 and gingival inflammation indistinguishable from parodontosis. Histology of the perianal infiltrate confirmed Langerhans cell histiocytosis. Infiltration of the pituitary stalk disappeared from the MR image after 4 cycles of 2-chlordeoxyadenosin (5 mg/m2 5 consecutive days). The PET-CT of the 2nd patient showed only borderline accumulation of FDG in the ENT area, while simultaneously performed CT imaging showed cystic restructuring of the pulmonary parenchyma and nodulations consistent with pulmonary Langerhans cell histiocytosis. Bronchoalveolar lavage identified higher number of CD1 and S100 positive elements, consistent, once again, with pulmonary LCH also affecting pituitary stalk and ear canal. The PET-CT of the third patient showed increased activity in the long bones and ilium near the sacroiliac joint. Biopsy of the focus in the ilium confirmed foam histiocyte infiltration immunochemically corresponding to Erdheim-Chester disease. Additional imaging assessments revealed the presence of further signs of the disease. Pituitary infiltrate biopsy in this patient did not elucidate the diagnosis but resulted in complete panhypopituarism. Central diabetes insipidus in adulthood might be the first sign of so far undiagnosed extracranial disease, in our case of histiocytic neoplasias, and PET-CT has an excellent potential to detect extracranial symptoms of these conditions. Therefore, the high-risk pituitary stalk infiltrate biopsy should always be preceded by comprehensive examination aimed at identification of extracranial manifestations of the pituitary gland diseases.

• MeSH
• centrální diabetes insipidus etiologie   MeSH
• diferenciální diagnóza MeSH
• dospělí MeSH
• Erdheimova-Chesterova nemoc komplikace   diagnóza   MeSH
• histiocytóza z Langerhansových buněk komplikace   diagnóza   MeSH
• hypofýza diagnostické zobrazování   MeSH
• lidé středního věku MeSH
• lidé MeSH
• magnetická rezonanční tomografie MeSH
• počítačová rentgenová tomografie MeSH
• pozitronová emisní tomografie MeSH
• Check Tag
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH
• přehledy MeSH

Interdigitating dendritic cell sarcoma is a rare neoplasm forming part of the group of malignancies derived from histocytic cell line. This nosological unit can be detected only by special immunohistochemical exams. A young man aged 25 found a tumorous swelling in the proximal part of his left crus. The pathological process affected proximal tibial epiphysis and adjacent soft tissues. The first FDG-PET examination performed in the process of determining the clinical stage of the disease showed a high activity in the site of primary tumour (SUV 7.71) and in the site of regional inguinal node (SUV 4.25). Histological examination of a diagnostic excision specimen of the tumour in the tibia and the extirpated enlarged regional nodes in the left groin led to the diagnosis of interdigitating dendritic cell sarcoma. The diagnosis was confirmed pathologically by another two centres in the Czech Republic and, due to the unusual nature of the diagnosis, also in Regensburg, Germany. Treatment started with chemotherapy, applied to patients with aggressive lymphomas in the framework of clinical studies, i.e. a combination of MegaCHOP. After 4 cycles, however, there was no visible response on the site of primary tumour. MegaCHOP therapy was therefore discontinued after the 4 cycles. Subsequently, we referred the patient for a high-dose chemotherapy with autologous bone marrow transplantation, similarly to aggressive lymphomas. The collection of blood producing stem cells from peripheral blood was successfully performed after ESHAP chemotherapy. A verificatoin FDG-PET examination was performed before high-dose chemotherapy. Increased activity was detected only in left proximal crus, with an SUV of 4.6. One month after ESHAP chemotherapy, BEAM high-dose chemotherapy with autologous transplantation of blood forming tissue was performed. High-dose chemotherapy was followed up by radiotherapy targeted on the primary tumour in the crus (70 Gy). The third verification FDG-PET examination was performed 3 months after radiotherapy. The examination showed a continuing higher activity in the region of the primary tumour (SUV 2.69) and a new centre of activity was detected in the left inguinal nodes region (SUV4.09). The activity corresponded to the presence of viable tumour tissue in the primary nidus and new metastases in inguinal nodes, without proofs of further proliferation at the time. Nodes of the left groin were removed. Histological examination showed affection of the node by the same type of tumour, i.e. a continuing activity of the disease despite chemotherapy. Due to suspected continuation of viable tumour in the crus judging by the intensity of accumulation of FDG-PET and the proof of a new affection of regional nodes, surgical treatment was preferred after the failure of chemotherapy. After the removal of inguinal nodes, left knee joint exarticulation was performed. This was followed by regional inguinal node region radiotherapy (56 Gy). The last fourth PET-CT examination carried out 4 months after the radiation therapy of the inguinal region showed massive dissemination into the region ofileac and paraaortic nodes (lymphadenopathy up to 6 cm in diameter) with an activity of 5.9 to 6.73 SUV units. Currently, we test the sensitiveness of the disease to 2-chlordeoxyadenosin and look for additional therapeutic options. To our knowledge, the above description is the first documented case of interdigitating dendritic cell sarcoma located in the tibia and crus soft tissue. We have not found any description of high-dose therapy supported by autologous transplantation of blood-forming tissue for this type of tumour in relevant literature. In this case, we record chemoresistance to high-dose chemotherapy and certain radiosensitivty of the tumour at the same time.

• MeSH
• bérec * MeSH
• chemorezistence * MeSH
• cytarabin aplikace a dávkování   MeSH
• dospělí MeSH
• etoposid aplikace a dávkování   MeSH
• karmustin aplikace a dávkování   MeSH
• lidé MeSH
• melfalan aplikace a dávkování   MeSH
• nádory kostí diagnóza   farmakoterapie   terapie   MeSH
• nádory měkkých tkání diagnóza   farmakoterapie   terapie   MeSH
• počítačová rentgenová tomografie MeSH
• pozitronová emisní tomografie MeSH
• protokoly protinádorové kombinované chemoterapie aplikace a dávkování   terapeutické užití   MeSH
• sarkom z interdigitujících dendritických buněk diagnóza   farmakoterapie   terapie   MeSH
• tibie * MeSH
• transplantace periferních kmenových buněk * MeSH
• Check Tag
• dospělí MeSH
• lidé MeSH
• mužské pohlaví MeSH
• Publikační typ
• anglický abstrakt MeSH
• časopisecké články MeSH
• kazuistiky MeSH
• přehledy MeSH
• Názvy látek
• cytarabin MeSH
• etoposid MeSH
• karmustin MeSH
• melfalan MeSH