Hand and foot osteosarcoma represents ~1% of all diagnosed cases of osteosarcoma. The rarity of osteosarcoma of the hand and foot leads to frequent misdiagnosis, delayed diagnosis or incorrect treatments, which can lead to fatal consequences. Typically, salvaging the affected limb is the treatment of choice, and with the use of chemotherapy, 60-65% of patients with osteosarcoma can be treated without amputation. Due to its rarity, misdiagnosis and treatment delays are common, yet detailed reviews and analyses of such cases are limited. The present retrospective cohort study aimed to review and analyze cases of osteosarcoma located in the hand and foot. From January 2007 to January 2019, 11 patients were treated at the Masaryk Memorial Cancer Institute Sarcoma Center (Brno, Czechia), 5 cases affected the hand and 6 affected the foot. A total of 6 male patients and 5 female patients, with a mean age of 30.9±16.74 years, were diagnosed with hand or foot osteosarcoma. The mean follow-up period was 90.36±66.14 months. The mean tumor size detected during diagnosis was 4.29±1.81 cm. Osteoblastic osteosarcoma was the most common histopathological type, accounting for 4 cases (33.4%). A majority of the osteosarcomas were identified as high grade (81.8%). A total of 5 patients experienced misdiagnoses following their initial biopsy, with 2 patients initially receiving treatment outside the Masaryk Memorial Cancer Institute Sarcoma Center. The most frequently encountered misdiagnosis was giant-cell tumor of the bone. A total of 3 patients underwent limb amputation and 2 patients developed lung metastasis and succumbed to the disease. The disease-free survival period and overall survival rate were calculated using Kaplan-Meier survival analysis. The mean disease-free survival period was 82.83±60.05 months, while the overall survival rate was 72%, with a mean survival time of 90.36±56.73 months. In summary, an examination of a case series involving 11 patients diagnosed with osteosarcoma of the hand and foot was conducted. The treatment approach, clinical characteristics and patient outcomes were described. A total of four case studies of patients with osteosarcoma in the hand or foot were presented. Misdiagnosis of this disease may result in the inappropriate treatment being administered to patients, therefore, the correct and rapid diagnosis of disease is necessary for effective treatment of hand and foot osteosarcomas.

• Publikační typ
• časopisecké články MeSH

• Publikační typ
• zprávy MeSH

BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is a cutaneous sarcoma with an infiltrative growth pattern that makes it challenging to clear margins. High quality data regarding DFSP natural history, management, and outcomes are limited. METHODS: Data were retrospectively collected for adult DFSP patients who underwent resection at 10 institutions in eight countries. Demographics, tumor characteristics, treatment strategies, and outcomes were analyzed. RESULTS: Analysis included 347 patients consisting of young (median, 42 years), White (76.2%), males (54.2%) with truncal lesions (57.3%). The majority (76.8%) were symptomatic at presentation. Preoperative imaging was used in 55.9% of cases. Diagnosis was established with excisional biopsy in 50.9% versus incisional biopsy in 25.0% of cases. Despite planned margins of >1.0 cm in 67.4% of cases, only 69.0% of patients achieved R0 resection. Twenty-two percent of patients underwent at least one re-excision. R0 resection was achieved at a second procedure in 80.2% and a third procedure in 86.2%. Ultimately, R0 resection was feasible in 89.5% of all patients. Fibrosarcomatous transformation (FST) was observed in 12.6%. In total, 6.6% (N = 23) recurred (17 local, six distant). Of the six distant recurrences, 50.0% had FST. With a median follow-up of 47.0 months, disease-specific survival rate was 98.8%. In multivariable analysis, R0 margins at index resection were associated with wider circumferential margins and non-FST histology. CONCLUSIONS: In this international, multicenter collaborative, DFSP practice patterns were heterogeneous but achieved favorable recurrence rates and survival. Multiple excisions to clear margins remain commonplace and can inform future efforts to optimize margin selection.

• MeSH
• dermatofibrosarkom * patologie   chirurgie   terapie   mortalita   MeSH
• dospělí MeSH
• internacionalita * MeSH
• lidé středního věku MeSH
• lidé MeSH
• lokální recidiva nádoru patologie   MeSH
• mladiství MeSH
• mladý dospělý MeSH
• nádory kůže * patologie   chirurgie   mortalita   terapie   MeSH
• resekční okraje MeSH
• retrospektivní studie MeSH
• senioři MeSH
• výsledek terapie MeSH
• Check Tag
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• mladiství MeSH
• mladý dospělý MeSH
• mužské pohlaví MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• multicentrická studie MeSH

BACKGROUND: The National Comprehensive Cancer Network (NCCN) guidelines recommend preoperative biopsy for diagnosing dermatofibrosarcoma protuberans (DFSP) but limited data support this approach. We characterized DFSP diagnostic practices and compared clinical outcomes based on technique. METHODS: Data were collected for adult patients who underwent resection for initial DFSP presentation between 2003 and 2021 at 10 international institutions. Patients were categorized by excisional versus preoperative biopsy (incisional, punch, core needle biopsies, or fine needle aspiration), and univariate and multivariable analyses were performed. RESULTS: The cohort included 321 patients, with excisional biopsy performed in 51.4% and preoperative biopsy performed in 48.6% of patients. Biopsy type was stable throughout the study period (p = 0.08). There were no differences in sex, disease presentation, or preoperative imaging. In unadjusted analysis, biopsy varied by practitioner specialty, with general surgeons performing nearly 50% of excisional biopsies. Despite similar planned circumferential margins and anatomic location, preoperative biopsy was associated with higher index R0 rate (60.1% vs. 78.6%), fewer total excisions, and fewer complications (38.2% vs. 25.6%, all p < 0.05). However, adjuvant radiotherapy (11.7% vs. 6.0%) and final R0 rates (91.5% vs. 88.4%) were comparable regardless of technique (p > 0.05). In adjusted analysis, excisional biopsy was associated with extremity tumors (odds ratio [OR] 1.79, confidence interval [CI] 1.21-2.66, p = 0.004), treatment in non-academic settings (OR 2.28, CI 1.10-4.73, p = 0.03), and inversely with preoperative imaging (OR 0.47, CI 0.24-0.93, p = 0.03). CONCLUSION: Preoperative biopsy is associated with margin-negative resection, fewer re-excisions, and reduced complications. Clinical suspicion of DFSP is paramount, and preoperative imaging may critically inform biopsy selection prior to index resection.

• MeSH
• biopsie metody   MeSH
• dermatofibrosarkom * patologie   chirurgie   MeSH
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• mladý dospělý MeSH
• nádory kůže * patologie   chirurgie   MeSH
• následné studie MeSH
• prognóza MeSH
• resekční okraje MeSH
• retrospektivní studie MeSH
• senioři MeSH
• Check Tag
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• mladý dospělý MeSH
• mužské pohlaví MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• multicentrická studie MeSH

Myxofibrosarcoma presents an infiltrating growth pattern that results in a high tendency for local recurrence. Clear margin resection is challenging because of microscopic infiltration. The purpose of the present study was to analyze the overall and disease-free survival rates of patients with myxofibrosarcoma and the prognostic factors that determine both survival and disease recurrence. Among the 111 patients included in our study, the 5-year overall survival rate was 65.5%. An age of more than 65 years (hazard ratio [HR] 1.9 [95% confidence interval (CI) 1.4-5.6]; p < 0.001), a tumor size of more than 5 cm (HR 2.8 [95% CI 0.9-8.1]; p = 0.049) and the G3 tumor grade (HR 14.1 [95% CI 2.1-105.0]; p < 0.001) negatively affected overall survival. The 5-year recurrence-free survival rate was 49.4%. R1/R2-type resection (HR 2.4 [95% CI 1.0-5.6]; p = 0.048) had a detrimental effect on tumor recurrence. Clear margins had a positive impact on recurrence-free survival, but did not significantly affect overall patient survival, suggesting that other factors may play a more significant role in determining patient outcomes. A surgical margin of 2 mm was not sufficient to significantly influence the incidence of recurrence. Consequently, a wider surgical margin may be necessary to reduce the risk of myxofibrosarcoma recurrence.

• MeSH
• dospělí MeSH
• fibrosarkom * chirurgie   patologie   mortalita   MeSH
• lidé středního věku MeSH
• lidé MeSH
• lokální recidiva nádoru * patologie   MeSH
• míra přežití MeSH
• přežití bez známek nemoci MeSH
• prognóza MeSH
• resekční okraje * MeSH
• retrospektivní studie MeSH
• senioři nad 80 let MeSH
• senioři MeSH
• Check Tag
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• senioři nad 80 let MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH

• MeSH
• lidé MeSH
• sarkom * epidemiologie   prevence a kontrola   terapie   MeSH
• sekundární prevence * MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• přehledy MeSH

Klíčové buněčné procesy včetně proliferace, diferenciace a přežívání jsou často regulovány stejnými molekulami během ontogeneze a tumorigeneze. Je známo, že protein c-Myb se spolupodílí na řízení uvedených procesů. Aberantní aktivace tohoto proteinu narušuje rovnováhu v jejich regulaci a podporuje zvýšenou proliferaci a přežívání buněk, čímž přispívá k maligní transformaci. Náš předchozí výzkum ukázal, že c-Myb se podílí také na regulaci fyziologického vývoje kostí. Nedávno publikované výsledky, podpořené našimi preliminárními daty, potvrdily, že protein c-Myb je exprimován v osteosarkomech, nádorech kostní tkáně, které se vyznačují věkově specifickou incidencí. Cílem tohoto projektu je objasnění funkce proteinu c-Myb v různých fázích fyziologického vývoje kostí a především jeho významu v regulaci progrese a chemorezistence osteosarkomů. s využitím buněčných linií, in vivo modelů a klinické studie. Klinická část bude dále rozšířena o širší spektrum osteogenních patologií s typickým výskytem v období dospívání, což přispěje ke zhodnocení významu proteinu c-Myb v homeostáze kostí.; Ontogenesis and tumorigenesis often share regulatory molecules that modulate key cellular events including cell proliferation, differentiation and survival. The c-Myb protein has been associated with control of these processes. Aberrant activation of c-Myb disturbs the balance in favor of proliferation and survival, thus contributing to malignant transformation. Our earlier research showed that c-Myb is involved in regulation of embryonal bone development. Recent findings, including preliminary data presented by us, demonstrate that c-Myb is expressed in osteosarcomas, bone tumors with specific age-related incidence. The aim of this study is to clarify the function of c-Myb in different stages of physiological bone development, and most importantly, the relevance of c-Myb in control of osteosarcoma progression and chemoresistance using cell lines, in vivo models and a clinical study. The clinical part will be further extended to other osteogenic pathologies with high incidence in childhood to further assess the importance of c-Myb in bone homeostasis in humans.

• MeSH
• chemorezistence MeSH
• geny myb MeSH
• homeostáza MeSH
• karcinogeneze genetika   MeSH
• mladiství MeSH
• osteogeneze genetika   MeSH
• osteosarkom farmakoterapie   genetika   MeSH
• Check Tag
• mladiství MeSH

• Konspekt
• Patologie. Klinická medicína
• NLK Obory
• onkologie
• genetika, lékařská genetika
• osteologie
• NLK Publikační typ
• závěrečné zprávy o řešení grantu AZV MZ ČR

Cílem autorů této publikace je shrnout na jednom místě vše, co je v současné době považováno v diagnostice a léčbě sarkomů za důležité. Nakladatelská anotace. Kráceno; Publikace zaměřená na diagnostiku a léčbu zhoubných nádorů pojivových tkání.

• MeSH
• sarkom MeSH

• Konspekt
• Patologie. Klinická medicína
• NLK Obory
• onkologie
• NLK Publikační typ
• kolektivní monografie

• MeSH
• hodnocení léčiv MeSH
• inhibitory angiogeneze terapeutické užití   MeSH
• klinické zkoušky, fáze III jako téma MeSH
• nádory z pojivové a měkké tkáně farmakoterapie   MeSH
• pyrimidiny terapeutické užití   MeSH
• receptory vaskulárního endoteliálního růstového faktoru antagonisté a inhibitory   aplikace a dávkování   farmakokinetika   terapeutické užití   MeSH
• sarkom farmakoterapie   MeSH
• sulfonamidy terapeutické užití   MeSH

• Konspekt
• Farmacie. Farmakologie
• NLK Obory
• farmakoterapie
• onkologie
• NLK Publikační typ
• studie

Projekt je snahou o zavádění molekulárně-biologických metod do diagnostiky sarkomů měkkých tkání /SMT/ pro individualizované klinické rozhodování o optimálním léčebném postupu využitím biomarkerů s prediktivní hodnotou. U sarkomů měkkých tkání budou korelovány s klinickými parametry exprese c-myc, PDGF,FGF,Ki67,p53,p21,mdm2,Fas,Fas ligand, ICEp10,DNA profilu, apoptosis TUNEL technikou a chemoresistence in vitro MTT testem.; The project implements the methods of molecular biology to detect the biological properties of soft tissue sarcomas in clinical practice and aims optimize the therapeutic decision-making in individual cases. The biomarkers analyzed for their predictive potential in soft tissue sarkomas include c-myc,PDGF,FGF,Ki67,PCNA,DNA profile,p53,p21,mdm2,bcl-2,Fas,Fasl ligand,ICE p10,apoptosis by TUNEL technique and histoculture drug response assay.

• MeSH
• dítě MeSH
• dospělí MeSH
• imunohistochemie MeSH
• kojenec MeSH
• multivariační analýza MeSH
• nádorové biomarkery MeSH
• sarkom diagnóza   MeSH
• Check Tag
• dítě MeSH
• dospělí MeSH
• kojenec MeSH

• Konspekt
• Patologie. Klinická medicína
• NLK Obory
• onkologie
• statistika, zdravotnická statistika
• NLK Publikační typ
• závěrečné zprávy o řešení grantu IGA MZ ČR