Laryngeálny chondrosarkóm (LC) je raritné ochorenie, zodpovedajúce za 0,2 % všetkých laryngeálnych malignít a zhruba 3 % všetkých chondrosarkómov. Histologická diagnostika je kľúčová, kedže väčšina laryngeálnych chondrosarkómov je nízkeho stupňa (low-grade) a ich diferenciácia od benígneho chondrómu je náročná. Rast LC je pomalý s neskorým nástupom príznakov. Jedná sa o rádio-rezistentný typ nádoru, vzhľadom k čomu je liečbou prvej voľby chirurgická resekcia. Rozsah resekcie sa odvíja od histopatologického stupňa, miery rastu a rekurencie, so zohľadnením ďalšej kvality života. Prognóza pri nízko diferencovanom LC je dobrá s veľmi nízkym rizikom vzdialených metastáz. Prezentujeme prípady 62a 68-ročných pacientov s chondrosarkómom prstienkovej chrupky nízkeho stupňa s dvomi rozličnými spôsobmi rastu, ktorí podstúpili debulkizáciu (kazuistika 1) a totálnu laryngektómiu (kazuistika 2).
Laryngeal chondrosarcoma (LC) is a rare disease, accounting for 0.2% of all laryngeal malignancies and approximately 3% of all chondrosarcomas. Histological diagnosis is crucial as most laryngeal chondrosarcomas are low-grade and differentiation from benign chondroma is difficult. The growth of LC is slow with a late onset of symptoms. It is a radio-resistant type of tumor, hence why surgical resection is the first choice of treatment. The extent of resection depends on the histopathological grade, growth rate, and recurrence with consideration of future quality of life. The prognosis for low-differentiated LC is good with a very low risk of distant metastasis. We present the cases of 62and 68-year-old patients with low-grade chondrosarcoma of the annular cartilage with two different growth patterns who underwent debulking (case report 1) and total laryngectomy (case report 2).
- MeSH
- chondrosarkom * chirurgie diagnóza radioterapie MeSH
- lidé středního věku MeSH
- lidé MeSH
- nádory hrtanu * chirurgie diagnóza radioterapie MeSH
- senioři MeSH
- Check Tag
- lidé středního věku MeSH
- lidé MeSH
- mužské pohlaví MeSH
- senioři MeSH
- Publikační typ
- kazuistiky MeSH
Extraskeletal myxoid chondrosarcoma (EMC) is a rare sarcoma of uncertain lineage. Insulinoma-associated protein 1 (INSM1) has recently been described as a highly specific and sensitive immunohistochemical marker for EMC. The goal of this study was to evaluate the diagnostic significance of INSM1 immunohistochemistry in EMC. Furthermore, correlations between molecular and morphological findings were performed. Sixteen of 17 EMC cases were stained with the INSM1 antibody. Tumors with at least 5% INSM1-positive cells and any staining intensity were considered positive. Molecular testing was successfully performed in 12/17 cases. The immunohistochemical analysis detected 13 INSM1-positive (81%) and 3 INSM1-negative tumors (19%). The extent of the staining was classified as 1+ in 7 cases (44%), 2+ in 2 cases (13%), 3+ in 2 cases (13%) and 4+ in 2 cases (13%). Intensity of immunostaining was weak in 5 cases (31%), moderate in 2 cases (13%) and strong in 6 cases (38%). Molecular assays revealed 8 EWSR1::NR4A3 positive tumors (67%), 2 TAF15::NR4A3 positive tumors (17%), 1 TCF12::NR4A3 positive tumor (8%) and 1 NR4A3 positive tumor (8%) in which no other gene alteration was identified. Two of them, namely TCF12 positive and one TAF15 positive tumors, were highly cellular and partially associated with pseudopapillary architecture. Our study found that moderate/strong expression of INSM1 in more than 25% of tumor cells was present in only 31% of cases. Thus, the diagnostic utility of INSM1 is rather low. Two morphologically unique cases of non-EWSR1 rearranged EMC with an extremely rare pseudopapillary growth pattern are also reported.
- MeSH
- chondrosarkom * diagnóza genetika MeSH
- DNA vazebné proteiny genetika MeSH
- fúzní onkogenní proteiny genetika metabolismus MeSH
- lidé MeSH
- nádory z pojivové a měkké tkáně * genetika MeSH
- receptory thyreoidních hormonů genetika MeSH
- represorové proteiny genetika MeSH
- sarkom * genetika MeSH
- steroidní receptory * genetika MeSH
- Check Tag
- lidé MeSH
- Publikační typ
- časopisecké články MeSH
- práce podpořená grantem MeSH
- MeSH
- chondrom diagnóza patologie MeSH
- chondrosarkom diagnóza patologie terapie MeSH
- Ewingův sarkom diagnóza patologie terapie MeSH
- lidé MeSH
- metastázy nádorů MeSH
- mnohočetný myelom diagnóza patologie terapie MeSH
- myositis ossificans diagnóza etiologie terapie MeSH
- nádory kostí * diagnóza klasifikace terapie MeSH
- obrovskobuněčný nádor kosti diagnóza patologie terapie MeSH
- osteom osteoidní chirurgie diagnóza patologie MeSH
- osteom chirurgie dietoterapie etiologie MeSH
- osteosarkom diagnóza patologie terapie MeSH
- Check Tag
- lidé MeSH
- Publikační typ
- přehledy MeSH
Chondrosarcoma of the thyroid cartilage is a sporadic disease with nonspecific clinical presentation. Smooth swelling of the supraglottic area should arouse suspicion of possible pathology. In addition to laryngoceles, which usually do not have a significant impact, otolaryngologists should consider chondrosarcoma of the thyroid cartilage and indicate computed tomography (CT). Late diagnosis leads to worse prognosis, particularly worse voice after more extensive surgery, need for tracheostomy, and worse survival from higher degree chondrosarcomas.
- MeSH
- chondrosarkom * diagnostické zobrazování chirurgie MeSH
- laryngektomie metody MeSH
- laryngocele * chirurgie MeSH
- lidé MeSH
- nádory hrtanu * patologie MeSH
- tyreoidální chrupavka chirurgie MeSH
- Check Tag
- lidé MeSH
- Publikační typ
- časopisecké články MeSH
Calcified chondroid mesenchymal neoplasm is a term proposed for tumors with a spectrum of morphologic features, including cartilage/chondroid matrix formation, that frequently harbor FN1 gene fusions. We report a series of 33 cases of putative calcified chondroid mesenchymal neoplasms, mostly referred for expert consultation out of concern for malignancy. Patients included 17 males and 16 females, with a mean age of 51.3 years. Anatomic locations include the hands and fingers, feet and toes, head and neck, and temporomandibular joint; 1 patient presented with multifocal disease. Radiologic review showed soft tissue masses with variable internal calcification, which occasionally scalloped bone but in all cases appeared indolent/benign. Tumors had a mean gross size of 2.1 cm and a homogenous rubbery to fibrous/gritty tan-white cut surface. Histology demonstrated multinodular architecture with a prominent chondroid matrix and increased cellularity towards the periphery of the nodules. The tumor cells were polygonal with eccentric nuclei and bland cytologic features and showed a variable amount of increased spindled / fibroblastic forms in the perinodular septa. The majority of cases had notable grungy and/or lacy calcifications. A subset of cases demonstrated at least focal areas of increased cellularity and osteoclast-like giant cells. Herein, we confirm the distinct morphologic and clinicopathologic features associated with this entity with the largest series to date, with a focus on practical diagnostic separation from similar chondroid neoplasms. Awareness of these features is critical in avoiding pitfalls, including a malignant diagnosis of chondrosarcoma.
- MeSH
- chondrosarkom * patologie MeSH
- chrupavka patologie MeSH
- lidé středního věku MeSH
- lidé MeSH
- nádory kostí * diagnostické zobrazování genetika patologie MeSH
- nádory z pojivové a měkké tkáně * diagnostické zobrazování genetika MeSH
- prsty nohy patologie MeSH
- Check Tag
- lidé středního věku MeSH
- lidé MeSH
- mužské pohlaví MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
Chondrosarcoma of the hand is a rare disease, but is one of the more common malignancies of the hand. Biopsies and imaging are a fundamental step in determining correct diagnosis, grading and selection for best treatment. We describe the case of a 77-year-old male complaining of a painless swelling in the proximal phalanx of the third ray of left hand. A biopsy was performed and the histology revealed a G2 chondrosarcoma. The patient underwent III ray amputation with metacarpal bone disarticulation and sacrifice of the radial digit nerve of the fourth ray. Definitive histology revealed grade 3 CS. Eighteen months after surgery, the patient is apparently disease-free with a good functional and aesthetic outcome although with persistent paresthesia of the fourth ray. Although there is no agreement in the literature for the treatment of low-grade chondrosarcomas, wide resection or amputation can be considered the mainstay treatment for high-grade tumors. Key words: chondrosarcoma, proximal phalanx, ray amputation, surgical treatment, tumor hand.
- MeSH
- amputace MeSH
- chondrosarkom * diagnóza patologie chirurgie MeSH
- kosti a kostní tkáň patologie MeSH
- lidé MeSH
- nádory kostí * chirurgie MeSH
- ruka MeSH
- senioři MeSH
- Check Tag
- lidé MeSH
- mužské pohlaví MeSH
- senioři MeSH
- Publikační typ
- časopisecké články MeSH
- kazuistiky MeSH
Předmětem příspěvku je analýza 18 biopticky diagnostikovaných tumorů v dutině nosní u psů. Tyto tumory tvořily 0,02 % z biopsií dodaných k vyšetření. Všechny tumory se vyskytly u jedinců čistokrevných plemen, ale plemenná dispozice k vývoji tumoru nebyla patrná. Ve 12 případech se jednalo o samce, samic bylo 6. Věkové rozmezí pacientů bylo 5–15,5 roku, průměrný věk byl 10,4 roku. Adenokarcinom byl diagnostikován v 7 případech, přechodní karcinom ve 4 případech a spinocelulární karcinom u jednoho psa. Olfaktorický neuroblastom, karcinoid a chondrosarkom se vyskytly každý ve dvou případech.
The subject of the contribution is the analysis of 18 biopsy-diagnosed tumors in the nasal cavity of dogs. These tumors accounted for 0.02 % of the biopsies submitted for examination. All tumors occurred in individuals of purebred breeds, but breed predisposition to tumor development was not evident. In 12 cases it was a male, 6 were females. The age range of the patients was 5–15.5 years, the average age was 10.4 years. Adenocarcinoma was diagnosed in 7 cases, transitional carcinoma in 4 cases, and squamous cell carcinoma was diagnosed in one dog. Olfactory neuroblastoma, carcinoid, and chondrosarcoma occurred in two cases each.
- MeSH
- biopsie * metody veterinární MeSH
- chondrosarkom diagnóza veterinární MeSH
- histologické techniky metody MeSH
- imunohistochemie metody MeSH
- karcinoid diagnóza veterinární MeSH
- karcinom diagnóza veterinární MeSH
- nosní dutina patologie MeSH
- olfaktorický estezioneuroblastom diagnóza veterinární MeSH
- psi MeSH
- zvířata MeSH
- Check Tag
- psi MeSH
- zvířata MeSH
Given the low incidence of musculoskeletal tumors during pregnancy, publications on the subject are scarce and treatment guidelines nonexistent. We present five cases of musculoskeletal tumors in pregnant women, three with metastasizing malignant neoplasms and two with aggressive giant cell tumors. The three patients diagnosed during their gestational period were operated before the end of pregnancy, adapting surgical techniques to minimize risk to mother and fetus. Adjuvant therapies were postponed until the end of gestation. All newborns were delivered at term vaginally, except for one where a cesarean section was required. After a mean follow-up of 69.96 months (±56.38), all patients were free of disease, except for the one diagnosed with an extraskeletal myxoid chondrosarcoma who died at 4 years from diagnosis. Surgery plays a key role in the treatment of musculoskeletal tumors diagnosed during pregnancy. These patients must be treated by multidisciplinary teams at sarcoma reference hospitals, involving the obstetrics team in the decision-making process, and adapting each step of the diagnosis and treatment to the gestational period. Key words: pregnancy, musculoskeletal tumors, sarcoma, cancer, oncological surgery.
- MeSH
- chondrosarkom * MeSH
- císařský řez MeSH
- končetiny MeSH
- lidé MeSH
- nádory z pojivové a měkké tkáně * MeSH
- novorozenec MeSH
- sarkom * MeSH
- těhotenství MeSH
- Check Tag
- lidé MeSH
- novorozenec MeSH
- těhotenství MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- přehledy MeSH
This is the first histological and molecular analysis of two chondrosarcomas with target-like chondrocytes that were compared with a group of conventional chondrosarcomas and enchondromas. The unique histological feature of target-like chondrocytes is the presence of unusual hypertrophic eosinophilic APAS-positive perichondrocytic rings (baskets). In the sections stained with Safranin O/Fast green, the outer part of the ring was blue and the material in the lacunar space stained orange, similarly to intercellular regions. Immunohistochemical examination showed strong positivity for vimentin, factor XIIIa, cyclin D1, osteonectin, B-cell lymphoma 2 apoptosis regulator (Bcl-2), p53 and p16. The S-100 protein was positive in 25 % of neoplastic cells. Antibodies against GFAP, D2-40 (podoplanin), CD99, CKAE1.3 and CD10 exhibited weak focal positivity. Pericellular rings/baskets contained type VI collagen in their peripheral part, in contrast to the type II collagen in intercellular interterritorial spaces. Ultrastructural examination revealed that pericellular rings contained an intralacunar component composed of microfibrils with abundant admixture of aggregates of dense amorphous non-fibrillar material. The outer extralacunar zone was made up of a layer of condensed thin collagen fibrils with admixture of non-fibrillar dense material. NGS sequencing identified a fusion transcript involving fibronectin 1 (FN1) and fibroblast growth factor receptor 2 (FGFR2) at the RNA level. At the DNA level, no significant variant was revealed except for the presumably germline variant in the SPTA1 gene.
- MeSH
- chondrocyty chemie patologie ultrastruktura MeSH
- chondrosarkom * chemie diagnóza patologie MeSH
- extracelulární matrix chemie metabolismus ultrastruktura MeSH
- imunohistochemie MeSH
- lidé MeSH
- nádory kostí * diagnóza metabolismus MeSH
- proteiny S100 metabolismus MeSH
- Check Tag
- lidé MeSH
- Publikační typ
- časopisecké články MeSH
The authors present a successful complex treatment of a Czech girl with Ollier ́s disease during the growth period, the aim of which was to correct the biomechanical axis and length of the lower limbs.
- MeSH
- chondrosarkom MeSH
- dítě MeSH
- dolní končetina patologie MeSH
- enchondromatóza * diagnóza genetika terapie MeSH
- externí fixátory MeSH
- lidé MeSH
- ortopedické výkony metody MeSH
- osteogeneze pomocí distrakčního aparátu MeSH
- radiografie MeSH
- vrozené deformity dolní končetiny MeSH
- Check Tag
- dítě MeSH
- lidé MeSH
- ženské pohlaví MeSH
- Publikační typ
- kazuistiky MeSH
