INTRODUCTION: Most patients with idiopathic pulmonary fibrosis (IPF) treated with antifibrotics (AF) have progressive disease despite treatment. A switch of AF may improve survival, but evidence from randomised controlled trials is missing. We aimed to evaluate the efficacy of an AF switch on survival and FVC decline in patients from the European MultiPartner IPF registry (EMPIRE). METHODS: The study included 612 patients who discontinued the first antifibrotic therapy. Patients were grouped and analysed from two perspectives: (1) whether they had received a second antifibrotic treatment after the discontinuation of the first therapy, and (2) a reason for discontinuation of the first AF - "lack of efficacy" (LE) and "intolerance" (INT). RESULTS: While 263 (43%) of 612 patients received no second AF ("non-switched"), 349 (57%) patients switched. Overall survival was higher in patients who received a second AF (median 50 vs. 29 months; adjusted HR 0.64, P=0.023). Similarly, the annual FVC decline was significantly reduced in switched patients: -98ml/y in switched and -172ml/y in non-switched patients (P=0.023), respectively. The switched patients had similar risk for mortality in both LE and INT groups (adjusted HR 0.95, P=0.85). The high impact of switching on survival was demonstrated in LE patients (adjusted HR 0.27, P<0.001). CONCLUSION: The patients without a second AF had significantly shorter overall survival. Our analysis suggests the importance of switching patients with an ineffective first AF therapy to a second AF therapy.
Respiračný epiteliálny adenomatoidný hamartóm (REAH) je zriedkavá benígna lézia pochádzajúca z epitelu sliznice nazálnej dutiny a prínosových dutín. V práci opisujeme prípad 51-ročného muža s anamnézou pretrvávajúceho sťaženého dýchania a pocitu upchatého nosa. Diagnostikovanú mal veľkú polypovitú masu v pravom nosovom priechodne so stopkou vyrastajúcou z čuchovej štrbiny. Makroskopicky išlo kompaktný polyp rozmerov 40 × 32 × 10 mm s hladkým povrchom. Histologické vyšetrenie potvrdilo diagnózu REAH s ložiskovo výraznou prevahou žľazových štruktúr s prechodom do seromucinózneho hamartómu. Po operácii klinické ťažkosti ustúpili a pacient je v súčasnosti bez známok recidívy. Napriek zriedkavému výskytu by mal byť REAH zahrnutý v diferenciálnej diagnostike sinonazálnych polypovitých más, najmä ak pochádzajú zo zadnej časti nazálneho septa alebo olfaktoriálnej štrbiny. Včasné rozpoznanie a správna diagnóza predchádzajú zbytočným agresívnym chirurgickým intervenciám a ďalším zaťažujúcim vyšetreniam.
Respiratory epithelial adenomatoid hamartoma (REAH) is a rare benign lesion originating from the mucosal epithelium of the nasal cavity and paranasal sinuses. We describe a 51-year-old man with a history of persistent difficulty breathing and feeling of stuffy nose. He was diagnosed to have a large polypoid mass in the right nasal cavity with a stalk arising from the olfactory cleft. Grossly, it was a compact polyp measuring 40 × 32 × 10 mm with a smooth surface. The histology confirmed the diagnosis of REAH with predominance of glandular structures with a transition to seromucinous hamartoma. After the operation, the clinical problems had disappeared and the patient was free of recurrence. Despite rare occurrence, REAH should be included in the differential diagnosis of sinonasal polypoid masses, especially of those which originate from the posterior part of the nasal septum or olfactory cleft. Early recognition and correct diagnosis prevent unnecessary aggressive surgery and other burdensome examinations.
- Klíčová slova
- respirační epitelový adenomatoidní hamartom,
- MeSH
- adenom chirurgie diagnostické zobrazování patologie MeSH
- diferenciální diagnóza MeSH
- endoskopie MeSH
- hamartom * chirurgie diagnostické zobrazování patologie MeSH
- lidé středního věku MeSH
- lidé MeSH
- nosní polypy chirurgie diagnostické zobrazování patologie MeSH
- paranazální dutiny * chirurgie diagnostické zobrazování patologie MeSH
- Check Tag
- lidé středního věku MeSH
- lidé MeSH
- mužské pohlaví MeSH
- Publikační typ
- kazuistiky MeSH
There are limited data on referral rates and the number of patients with idiopathic pulmonary fibrosis (IPF) who are eligible for lung transplantation. The aim of the present study was to assess adherence to the consensus of the International Society for Heart and Lung Transplantation (ISHLT) for the referral of patients with IPF among Czech interstitial lung disease (ILD) centers. Czech patients who were diagnosed with IPF between 1999 and 2021 (n = 1584) and who were less than 65 years old at the time of diagnosis were retrospectively selected from the Czech Republic of the European Multipartner Idiopathic Pulmonary Fibrosis Registry (EMPIRE). Nonsmokers and ex-smokers with a body mass index (BMI) of <32 kg/m2 (n = 404) were included for further analyses. Patients with a history of cancer <5 years from the time of IPF diagnosis, patients with alcohol abuse, and patients with an accumulation of vascular comorbidities were excluded. The trajectory of individual patients was verified at the relevant ILD center. From the database of transplant patients (1999-12/2021, n = 541), all patients who underwent transplantation for pulmonary fibrosis (n = 186) were selected, and the diagnosis of IPF was subsequently verified from the patient's medical records (n = 67). A total of 304 IPF patients were eligible for lung transplantation. Ninety-six patients were referred to the transplant center, 50% (n = 49) of whom were referred for lung transplantation. Thirty percent of potentially eligible patients not referred to the transplant center were considered to have too many comorbidities by the reporting physician, 19% of IPF patients denied lung transplantation, and 17% were not referred due to age. Among Czech patients with IPF, there may be a larger pool of potential lung transplant candidates than has been reported to the transplant center to date.
- MeSH
- dodržování směrnic statistika a číselné údaje MeSH
- dospělí MeSH
- idiopatická plicní fibróza * chirurgie MeSH
- intersticiální plicní nemoci chirurgie MeSH
- konziliární vyšetření a konzultace * statistika a číselné údaje MeSH
- lidé středního věku MeSH
- lidé MeSH
- registrace MeSH
- retrospektivní studie MeSH
- senioři MeSH
- transplantace plic * statistika a číselné údaje MeSH
- Check Tag
- dospělí MeSH
- lidé středního věku MeSH
- lidé MeSH
- mužské pohlaví MeSH
- senioři MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- Geografické názvy
- Česká republika MeSH
Úvod: Atypický intradermálny hladkosvalový tumor, nazývaný aj kožný leiomyosarkóm, predstavuje veľmi zriedkavú onkodermatologickú jednotku. Vzhľadom na indolentné klinické správanie je klasifikovaný ako nádor s neurčitým malígnym potenciálom. Kazuistika: 63-ročný muž pozoroval niekoľko rokov na extenzorovej strane hornej časti ľavej paže tumorózny kožný útvar vzhľadu prominujúcej papuly. Odstránený bol kompletne chirurgickou excíziou. Histologicky išlo o intradermálny nodul tvorený solídne rastúcou hladkosvalovou nádorovou populáciou, ktorá vykazovala ložiskové jadrové atypie, ľahko zvýšenú proliferačnú aktivitu a sporadické mitózy. Nález zodpovedal atypickému intradermálnemu hladkosvalovému tumoru. Pacient bol zaradený do onkodispenzárnej starostlivosti a klinicky ďalej sledovaný. Záver: Atypický intradermálny hladkosvalový tumor/kožný leiomyosarkóm je nádor s veľmi priaznivou prognózou, ktorého kompletné chirurgické odstránenie je kuratívne. K zvýšenému riziku lokálnej recidívy dochádza pri infiltrácii podkožia. Nutné je však jeho striktné odlíšenie od podkožného leiomyosarkómu, ktorý predstavuje etiologicky odlišné a prognosticky omnoho závažnejšie onkologické ochorenie.
Introduction: Atypical intradermal smooth muscle tumor, also called cutaneous leiomyosarcoma, represents a very rare oncodermatological entity. Due to indolent clinical behaviour it is classified as a tumor of uncertain malignant potential. Case report: A 63year old man presented with a papular skin tumor arising on the extensor size of the upper part of his left arm. The patient claimed that the lesion had been growing for several years. The tumor was completely removed by surgical excision. Histology revealed an intradermal nodule composed of a solid neoplastic smooth muscle population that exhibits focal nuclear atypia, slightly increased proliferative activity and sporadic mitoses. The diagnosis of atypical intradermal smooth muscle tumor was established. The patient was subsequently referred to oncodispensary care and followed up clinically. Conclusion: Atypical intradermal smooth muscle tumor/cutaneous leiomyosarcoma is a neoplasm with very favourable prognosis, whose complete surgical removal is curative. An increased risk of local recurrence occurs when the subcutis is involved. However, it is necessary to distinguish it from subcutaneous leiomyosarcoma, which represents an etiologically different and prognostically much more serious oncological disease.
- MeSH
- dermatochirurgické výkony metody MeSH
- leiomyosarkom * chirurgie diagnóza MeSH
- lidé středního věku MeSH
- lidé MeSH
- Check Tag
- lidé středního věku MeSH
- lidé MeSH
- mužské pohlaví MeSH
- Publikační typ
- kazuistiky MeSH
BACKGROUND AND OBJECTIVES: Nonconvulsive status epilepticus (NCSE) manifests as a change in mental status without a coma (NCSE proper) or comatose NCSE. Hypocretin-1/orexin-A (H/O) is involved in alertness and sleep maintenance. Sleep impairment and excessive daytime sleepiness (EDS) have a negative impact on cognitive functions and activities of daily living (ADL). METHODS: Patients meeting the NCSE criteria underwent cerebrospinal fluid and brain magnetic resonance imaging examinations, polysomnographies (PSG), multiple latency sleep tests (MSLT), and completed Epworth Sleepiness Scale (ESS). Montreal Cognitive Assessment was used to evaluate cognitive functions, and the Barthel Index was used to assess ADL in the acute phase (V1) and three months follow-up (V2). RESULTS: From May 2020 to May 2023, we enrolled 15 patients, eight (53.3 %) women, with a median age of 69 (14) years. The median H/O CSF concentration was 250 (63.6) pg/ml; however, only three CSF samples (20 %) decreased below the borderline concentration of 200 pg/ml. Fourteen out of 15 patients (93.3 %) completed the PSG study. The median of wakefulness after sleep onset was 167 (173.5) min, sleep efficiency (SE) was 62.9 (63) %, sleep latency (SL) was 6 (32) min, REM sleep was 2.85 (7.2) %, and REM first episode latency was 210.5 (196.5) minutes. The medians of the stages N1 NREM were 4.65 (15) %, N2 NREM 68.4 (29.9) %, and N3 NREM 21.8 (35.5) %. MSLT mean latency was 7.7 (12.6) minutes. A significant negative correlation exists between H/O CSF concentrations and the stage N1 NREM (rs = -0.612, p = 0.02), and the proportion of cumulative sleep time with oxygen saturation below 90 % in total sleep time (TST) t90 (rs = -0.57, p = 0.03). MSLT had significant negative correlation with TST (rs = -0.5369, p = 0.0478), with SE (rs = -0.5897, p = 0.0265), with apnea-hypopnea index (rs = -0.7631, p = 0.0002) and with deoxygenation index (rs = -0.8009, p = 0.0006). A positive correlation exists between MSLT and SL (rs = 0.6284, p = 0.0161) and between ESS and t90 (rs = 0.9014, p = 0.0004). The correlation between H/O CSF concentrations and EDS, cognitive performance, and ADL was not proved. CONCLUSIONS: Patients after NCSE exhibited sleep impairment and excessive daytime sleepiness. Hypocretin-1/orexin-A concentrations decreased only in 20 % of these cases.
- MeSH
- kohortové studie MeSH
- lidé středního věku MeSH
- lidé MeSH
- magnetická rezonanční tomografie MeSH
- orexiny * mozkomíšní mok MeSH
- polysomnografie * MeSH
- poruchy nadměrné spavosti * mozkomíšní mok MeSH
- průřezové studie MeSH
- senioři MeSH
- spánek fyziologie MeSH
- status epilepticus * mozkomíšní mok MeSH
- Check Tag
- lidé středního věku MeSH
- lidé MeSH
- mužské pohlaví MeSH
- senioři MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
Úvod: Atypický fibroxantóm (AFX) je zriedkavý mezenchýmový nádor kože s nejasným histogenetickým pôvodom a neurčitým biologickým správaním. Charakteristický je výraznou štrukturálnou variabilitou, ktorá spôsobuje pri bioptickom vyšetrení značné diagnostické rozpaky. Stanovenie diagnózy sa nezaobíde bez použitia širokého spektra imunohistochemických markerov. Kazuistika: 79-ročný muž s anamnézou viacpočetných keratinocytových karcinómov kože pozoroval vznik ďalšej tumoróznej lézie na ľavom líci, ktorá imponovala ako bazocelulárny karcinóm. Odstránená bola totálnou chirurgickou excíziou. Tumor pozostával z pomerne ohraničeného intradermálne rastúceho nodulu tvoreného populáciou pleomorfných nádorových buniek so svetlou cytoplazmou. Imunohistochemicky bol pozitívny na vimentín, CD163, CD68, CD10 a CD99. Histomorfologický obraz a imunofenotyp zodpovedali svetlobunkovému variantu AFX. Po stanovení diagnózy bol pacient ďalej sledovaný onkodermatológom bez ďalšej terapeutickej intervencie. Záver: Svetlobunkový AFX je veľmi zriedkavý nádor postihujúci preferenčne oblasť hlavy u starších mužov. Zvyčajne je ťažko diagnostikovateľný, pretože imituje široké spektrum primárnych aj sekundárnych nádorov kože. Vždy musí byť správne odlíšený od iných kožných malignít so svetlobunkovou diferenciáciou, ktoré majú omnoho agresívnejšie biologické správanie, horšiu prognózu a vyžadujú úplne inú liečebnú stratégiu.
Introduction: Atypical fibroxanthoma (AFX) is a rare mesenchymal skin tumor of unknown histogenesis and unclear biological behaviour. It is characterized by marked structural variability, which causes great diagnostic embarrassment during biopsy examination. Establishing a correct diagnosis can not be done without using a wide spectrum of immunohistochemical markers. Case report: A 79-year-old man with a history of multiple keratinocytic skin carcinomas noticed another lesion arising in the left cheek. It looked like basal cell carcinoma and was completely removed by surgical excision. The tumor consisted of a relatively circumscribed intradermal nodule composed of pleomorphic neoplastic cell population with clear cytoplasm. It immunohistochemically expressed vimentin, CD163, CD68, CD10 and CD99. A pathologic diagnosis of clear cell AFX was done. After establishing the diagnosis, the patient was followed-up by an oncodermatologist without further therapeutic intervention. Conclusion: Clear cell AFX is a unique tumor preferentially affecting the head region in older men. It is usually difficult to diagnose because it mimics a wide range of primary and secondary skin neoplasms. It must always be correctly distinguished from other cutaneous malignancies with clear cell differentiation, which have a much more aggressive biological behaviour, worse prognosis and require a completely different therapeutic strategy.
- Klíčová slova
- atypický fibroxantom,
- MeSH
- imunohistochemie metody MeSH
- lidé MeSH
- nádory kůže * chirurgie diagnóza MeSH
- senioři MeSH
- Check Tag
- lidé MeSH
- mužské pohlaví MeSH
- senioři MeSH
- Publikační typ
- kazuistiky MeSH
Východiska: Alveolárny sarkóm mäkkých tkanív (alveolar soft part sarcoma – ASPS) je veľmi zriedkavá mezenchýmová malignita nejasného pôvodu. Väčšinou postihuje mladých ľudí, pričom približne štvrtina prípadov sa diagnostikuje v detskom veku. Prípad: Jedenásťročné dievča pozorovalo nebolestivú podkožnú „hrčku“ v oblasti ľavého lakťa. Zobrazovacie vyšetrenia potvrdili solídnu mäkkotkanivovú intramuskulárnu rezistenciu suspektného vzhľadu. Vykonaná bola jej chirurgická excízia. Resekát pozostával z laločnatého nádoru rozmerov 35 × 20 × 12 mm. Histologicky bol tvorený organoidne rastúcou epiteloidnobunkovou populáciou s vyznačenou pseudoalveolárnou mikroarchitektonikou. Imunohistochemicky exprimoval TFE3 a geneticko-molekulová analýza preukázala fúziu génu ASPSCR1:: TFE3. Spektrum nálezov potvrdzovalo diagnózu ASPS. Následne bola vykonaná široká reexcízia jazvy bez mikroskopického dôkazu nádorových reziduí. Pacientka je v súčasnosti bez známok lokálnej recidívy či metastáz. Záver: ASPS sa považuje za agresívny a prognosticky nepriaznivý chemorezistentný nádor. U detí má lepšiu prognózu v porovnaní s dospelými. Z terapeutického hľadiska je kľúčové včasné rozpoznanie tumoru v lokalizovanom štádiu a jeho kompletné chirurgické odstránenie. Vzhľadom na tendenciu vzniku neskorých metastáz je nutný dlhodobý dôkladný follow-up pacienta.
Background: Alveolar soft part sarcoma (ASPS) is a very rare mesenchymal malignancy of uncertain origin. It mostly affects young people, with about a quarter of cases being diagnosed in children. Case: An 11-year-old girl had a painless subcutaneous “lump” in the left elbow area. Imaging exams revealed a solid soft-tissue intramuscular mass of suspicious appearance. A surgical excision of lesion was performed. The biopsy consisted of a lobular tumor measuring 35 × 20 × 12 mm. Histology revealed an epithelioid-cell population arranged in organoid pseudoalveolar pattern. It immunohistochemically expressed TFE3 and harbored the ASPSCR1:: TFE3 gene fusion. A diagnosis of ASPS was established. Subsequently, a wide re-excision of the scar was performed without microscopic residual tumor. The patient is currently without evidence of local recurrence or metastasis. Conclusion: ASPS is considered an aggressive and prognostically unfavorable chemoresistant neoplasm. Children have a better prognosis compared to adults. Early detection of tumor in a localized stage with complete surgical removal remains a mainstay therapeutic option. Due to its tendency to late metastases, a long-term thorough follow-up of the patient is necessary.
- MeSH
- alveolární sarkom měkkých tkání * chirurgie diagnóza MeSH
- diagnostické zobrazování metody MeSH
- dítě MeSH
- imunohistochemie metody MeSH
- lidé MeSH
- výsledek terapie MeSH
- Check Tag
- dítě MeSH
- lidé MeSH
- ženské pohlaví MeSH
- Publikační typ
- kazuistiky MeSH
Úvod: Hidradenokarcinóm je zriedkavý malígny adnexálny nádor histogeneticky odvodený z intradermálnych duktov potných žliaz. Klinicky sa spočiatku často považuje za inú benígnu (nenádorovú) kožnú chorobnú jednotku, čo prispieva k jeho oneskorenej diagnostike. Kazuistika: 51-ročný muž pozoroval v poslednom roku „zapálenú hrčku“ pod pravou pazuchou. Lézia imponoval ako hidradenitis suppurativa. Po jej chirurgickej incízii došlo v danej lokalite k progresii kožnej tumoróznej rezistencie. Lézie bola kompletne exstirpovaná. Mikroskopické vyšetrenie potvrdilo solídne rastúci „high-grade“ karcinóm, ktorého histomorfológia a imunofenotyp favorizovali diagnózu hidradenokarcinómu. Pacient mal následne vykonanú širokú reexcíziu jazvy bez prítomných nádorových reziduí. Ďalšie klinické vyšetrenia nepotvrdili známky recidívy či metastatického postihnutia. Záver: Hidradenokarcinóm kože je považovaný za nádor s agresívnym biologickým správaním. Vzhľadom na svoju raritnosť predstavuje v onkodermatologickej praxi diagnostickú a terapeutickú výzvu. Včasné rozpoznanie a kompletné chirurgické odstránenie tumoru so širokými resekčnými okrajmi sú kritické pre ďalšiu prognózu. Pacienti musia byť dlhodobo klinicky sledovaní.
Introduction: Hidradenocarcinoma is a rare malignant adnexal tumor which arises from the intradermal ducts of sweat glands. It often clinically mimics a benign (non-neoplastic) skin disease, which contributes to delayed diagnosis. Case report: A 51-year old man had noticed an "inflamed lump" in his right armpit for the last year. The lesion clinically appeared as a suppurative hidradenitis. After its surgical incision, it was seen a progression of cutaneous tumorous resistance at a given location. The mass was completely excised. Histopathology revealed a solid high-grade carcinoma with a histomorphology and immunophenotype favoring the diagnosis of hidradenocarcinoma. The patient subsequently underwent a wide local reexcision with no tumor residues in the tissue. Further clinical examinations did not confirm tumor recurrence or metastases. Conclusion: Cutaneous hidradenocarcinoma is considered to be a neoplasm which exhibits an aggressive biological behaviour. Due to its rarity, it represents the diagnostic and therapeutic challenges in oncodermatological practice. Early detection and complete surgical resection of the tumor with wide surgical margins are critical for further prognosis. The patients must be closely followed up.
- MeSH
- časná diagnóza MeSH
- lidé středního věku MeSH
- lidé MeSH
- nádory potních žláz * diagnóza patologie terapie MeSH
- paže chirurgie patologie MeSH
- progrese nemoci MeSH
- vzácné nemoci * diagnóza patologie terapie MeSH
- Check Tag
- lidé středního věku MeSH
- lidé MeSH
- mužské pohlaví MeSH
- Publikační typ
- kazuistiky MeSH
INTRODUCTION: The antifibrotic drug nintedanib is used for the treatment of idiopathic pulmonary fibrosis (IPF). We analysed the effect of nintedanib on antifibrotic treatment outcome in real-world cohorts of Czech EMPIRE registry. PATIENTS/METHODS: Data of 611 Czech IPF subjects, 430 (70%) treated with nintedanib (NIN group), 181 (30%) with no-antifibrotic treatment (NAF group) were analysed. The influence of nintedanib on overall survival (OS), pulmonary function parameters as forced vital capacity (FVC) and diffusing lung capacity for carbon monoxide (DLCO), as well as GAP score (gender, age, physiology) and and CPI (composite physiological index) were investigated. RESULTS: During 2 year follow-up we observed that nintedanib treated patients had longer OS, compared to those treated with no-antifibrotic drugs (p < 0.00001). Nintedanib reduces risk of mortality over no-antifibrotic treatment by 55% (p < 0.001). We have observed no significant difference in the rate of FVC and DLCO decline between the NIN and NAF group. Changes within 24 months from baseline in CPI were not significant between the groups (NAF and NIN). CONCLUSION: Our real-practice study showed the benefit of nintedanib treatment on survival. There were no significant differences between NIN and NAF groups in changes from baseline in FVC %, DLCO % predicted and CPI.
- MeSH
- idiopatická plicní fibróza * farmakoterapie MeSH
- lidé MeSH
- plíce MeSH
- registrace MeSH
- vitální kapacita MeSH
- výsledek terapie MeSH
- Check Tag
- lidé MeSH
- Publikační typ
- časopisecké články MeSH
- Geografické názvy
- Česká republika MeSH
