We report the case of a 67-year-old female with clear cell sarcoma (CCS) of the vulva. Grossly, the tumor was a partly exophytical vulvar mass, measuring 20 x 15 cm. At the time of presentation, the patient showed metastases to the lung, inguinal and pelvic lymph nodes. Histologically, the tumor consisted of oval or spindle cells with only mild nuclear pleomorphism and rare mitoses (up to 2/10 HPF). The cytoplasm was pale eosinophilic or clear. The tumor cells were arranged in confluent sheets. There were large areas of necrosis and surface ulceration. Immunohistochemically, the tumor cells showed expression of S-100 protein and focal melan A and HMB45 expression. Fluorescent in situ hybridization analysis revealed rearrangement of the EWSR1 gene. To the best of our knowledge, this is the first report of CCS arising in the vulva.

• Klíčová slova
• clear cell sarcoma - EWSR1 gene - melanoma of soft parts - vulva.,
• MeSH
• diferenciální diagnóza MeSH
• hybridizace in situ fluorescenční MeSH
• lidé MeSH
• nádory vulvy diagnóza   MeSH
• proteiny S100 MeSH
• sarkom z jasných buněk diagnóza   MeSH
• senioři MeSH
• vulva MeSH
• Check Tag
• lidé MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH
• práce podpořená grantem MeSH
• Názvy látek
• proteiny S100 MeSH

We report two cases (male patients 50 and 55 years old) of clear cell sarcoma ("melanoma of soft parts") arising in the lung, of which one case showed regional lymph node metastases. Histologically, both tumors displayed varying clear epithelioid and spindle neoplastic cells arranged in storiform and nested growth patterns, separated by thin fibrovascular septa. Immunohistochemical studies demonstrated positive expression of S-100 protein, HMB-45 and Melan-A in one case and S-100 protein only in the other. Fluorescence in situ hybridization showed positive EWSR1 gene rearrangement, and a presence of EWS-ATF1 fusion transcript was confirmed by RT-PCR and sequencing in one case.

• Klíčová slova
• ATF1, Clear cell sarcoma, EWS, Lung, Melanoma of soft parts,
• MeSH
• genová přestavba fyziologie   MeSH
• hybridizace in situ fluorescenční metody   MeSH
• lidé středního věku MeSH
• lidé MeSH
• nádory měkkých tkání genetika   patologie   MeSH
• protein EWS vázající RNA MeSH
• proteiny S100 metabolismus   MeSH
• proteiny vázající kalmodulin genetika   metabolismus   MeSH
• proteiny vázající RNA genetika   metabolismus   MeSH
• sarkom z jasných buněk diagnóza   genetika   MeSH
• translokace genetická genetika   MeSH
• Check Tag
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH
• Názvy látek
• EWSR1 protein, human MeSH Prohlížeč
• protein EWS vázající RNA MeSH
• proteiny S100 MeSH
• proteiny vázající kalmodulin MeSH
• proteiny vázající RNA MeSH

The disease concept of clear cell (tubulo) papillary renal cell carcinoma (CCP-RCC) as a distinct subtype of renal cell carcinoma has been recently established. First described in the setting of end stage renal disease, this tumor type is more frequently recognized and encountered in a sporadic setting. In this article, we provide an overview of the recent understanding of this tumor. Macroscopically, tumors are well circumscribed with well-developed tumor capsule. Histologically, the tumor cells are cuboidal to low columnar cell with clear cytoplasm and papillary and tubulo-papillary configuration. Immunohistochemically, tumor cells generally show diffuse expression for cytokeratin 7, CA9 (cup-shaped pattern), HIF-1, GLUT-1 and high molecular weight cytokeratin, but negative for AMACR, RCC Ma and TFE3. CD10 is negative or focally positive in most tumors. Genetically, this tumor has no characteristics of clear cell RCC or papillary RCC. Prognostically, patients with CCP-RCC behave in an indolent fashion in all previously reported cases. In conclusion, although this tumor has been integrated into recent International Society of Urologic Pathology Classification of renal neoplasia, both aspects of disease concept and clinical behavior are yet to be fully elucidated. Further publications of large cohorts of patients will truly help understand the biologic potential and the molecular underpinnings of this tumor type.

• Klíčová slova
• Clear cell (tubulo) papillary renal cell carcinoma, pathology,
• MeSH
• imunohistochemie MeSH
• karcinom z renálních buněk klasifikace   patologie   MeSH
• lidé MeSH
• nádorové biomarkery metabolismus   MeSH
• nádory ledvin klasifikace   patologie   MeSH
• papilární karcinom klasifikace   patologie   MeSH
• prognóza MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH
• přehledy MeSH
• Názvy látek
• nádorové biomarkery MeSH

INTRODUCTION: To date, clear cell renal carcinoma (Grawitz tumour) remains the most frequent malignant tumour of the kidney in adults. It metastasises in more than 25% of cases, most frequently to the bones (osteolytic metastases), lungs, brain, liver, adrenal glands and the contralateral kidney. Metastases to the pancreas are rare and represent 1-4% of all malignant tumours of the pancreas. METHODS: This is a retrospective analysis of patients who were operated at the Department of Surgery in Pilsen between 2010 and 2018 for histologi-cally verified metastasis of clear cell carcinoma (Grawitz tumour) to the pancreas. RESULTS: We operated 12 patients (8 men and 4 women). The metastases appeared on average 8 years and 8 months following the primary urolo-gical surgery. The mean age of the male patients was 66.5 years and that of the female patients was 67.4 years. In our sample, the diagnostic specificity of the CT scan was 50%, the diagnostic specificity of endoscopic ultrasound (EUS) was 75% and subsequent EUS-guided fine needle aspiration biopsy performed in 100% of cases yielded a specificity of 75%. Resectability was 92%. The average length of hospitalisation was 11.5 days. Post-operative complications according to Clavien-Dindo were grade 1 in 66%, grade 2 in 1.25% and grade 5 in 8.3% of the cases. The 30-day post-operative mortality was 8.3% (one patient). Conclusion: Clear cell renal carcinoma metastases to the pancreas are very rare. However, if radically removed, the patient has a good prognosis with regards to long-term survival.

• Klíčová slova
• clear cell renal carcinoma, diagnosis, metastases to the pancreas, surgical treatment,
• MeSH
• dospělí MeSH
• karcinom z renálních buněk chirurgie   MeSH
• lidé MeSH
• nádory ledvin chirurgie   MeSH
• nádory slinivky břišní chirurgie   MeSH
• pankreas MeSH
• retrospektivní studie MeSH
• senioři MeSH
• Check Tag
• dospělí MeSH
• lidé MeSH
• mužské pohlaví MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH

An 82-year-old woman with COPD presented to the emergency department with cough, increasing sputum production, wheezing, and worsening shortness of breath for two weeks. On imaging studies, the patient was found to have a right upper lobe spiculated nodule and an endobronchial lesion with near total occlusion of the right lower lobe bronchus with sub-segmental atelectasis. Bronchoscopy with EBUS-TBNA of subcarinal and right hilar lymph nodes revealed lung cancer with clear cell phenotype. Given the predominance of clear cell morphology, the diagnosis of metastatic renal or ovarian cancer was entertained. However, there was no evidence of renal or ovarian lesions on the PET-CT scan, ruling out the possibility. Salivary gland type lung cancer (STLC), which is responsible for less than 1% of all lung cancer cases in adults, was also considered. The two distinct STLCs that may have similar morphologic appearances are hyalinizing clear cell carcinoma (HCCC) and mucoepidermoid carcinoma (MEC). The other type of tumour in the lung that demonstrates a clear cell phenotype is perivascular epithelioid cell neoplasms or PEComa, which are mesenchymal in origin. Immunohistochemical staining was strongly positive for p63, CK5/6, CK7, CK-LMW, and negative for TTF-1, Napsin A, p16, and CK20. Additional staining, including HMB-45, S-100, and mucicarmine, were also negative. Next-generation sequencing for the salivary gland fusion panel, including EWSR1-ATF1 fusion and EWSR1 gene rearrangement for HCCC and MAML2 gene rearrangements for MEC, was negative. She was diagnosed with non-small cell lung cancer favouring squamous cell carcinoma with clear cell phenotype, a rare entity.

• Klíčová slova
• Clear cell, Glycogen, Lung cancer, Phenotype, Squamous cell,
• MeSH
• adenokarcinom z jasných buněk diagnóza   patologie   genetika   MeSH
• bronchoskopie MeSH
• diferenciální diagnóza MeSH
• lidé MeSH
• nádory plic * diagnóza   patologie   genetika   MeSH
• senioři nad 80 let MeSH
• Check Tag
• lidé MeSH
• senioři nad 80 let MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH

INTRODUCTION: Our case report presents a rare occurrence of clear cell sarcoma in the genitofemoral line region that has not been described to date. CASE REPORT: A 57-year-old male patient with clear-cell sarcoma of tendons and aponeuroses is presented. The patient underwent a radical operation and adjuvant radiotherapy. CONCLUSION: The patient is still alive, in a good health condition, without local recurrence or generalised disease. Key words: clear-cell sarcoma of tendons and aponeuroses - genitofemoral line.

• MeSH
• aponeuróza * MeSH
• lidé středního věku MeSH
• lidé MeSH
• lokální recidiva nádoru MeSH
• nádory měkkých tkání * diagnóza   chirurgie   MeSH
• sarkom z jasných buněk * diagnóza   chirurgie   MeSH
• šlachy chirurgie   MeSH
• Check Tag
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH

OBJECTIVE: To describe the clinical and ultrasound characteristics of ovarian pure clear cell carcinoma. METHODS: This was a retrospective study involving data from 11 ultrasound centers. From the International Ovarian Tumor Analysis (IOTA) database, 105 patients who had undergone preoperative ultrasound examination by an experienced ultrasound examiner between 1999 and 2016 were identified with a histologically confirmed pure clear cell carcinoma of the ovary. An additional 47 patients diagnosed with pure clear cell carcinoma between 1999 and 2016 and with available complete preoperative ultrasound reports were identified retrospectively from the databases of the departments of gynecological oncology in the participating centers. The ultrasound images of all tumors were described using IOTA terminology. Clinical and ultrasound characteristics were analyzed for the whole group, and separately, for patients with and those without histologically confirmed endometriosis, and for patients with evidence of tumor developing from endometriosis. RESULTS: Median age of the 152 patients was 53.5 (range, 28-92) years and 92/152 (60.5%) tumors were FIGO Stage I. Most tumors (128/152, 84.2%) were unilateral. On ultrasound examination, all tumors contained solid components and 36/152 (23.7%) were completely solid masses. The median largest diameter of the lesion was 117 (range, 25-310) mm. Papillary projections were present in 58/152 (38.2%) masses and, in most of these (51/56, 91.1%), vascularized papillary projections were seen. Information regarding the presence, site and type of pelvic endometriosis at histology was available for 130/152 patients. Endometriosis was noted in 54 (41.5%) of these. In 24/130 (18.6%) patients, the tumor was judged to have developed from endometriosis. Patients with, compared to those without, evidence of tumor developing from endometriosis were younger (median 47.5 vs 55.0 years, respectively), and ground-glass echogenicity of cyst fluid was more common in pure clear cell cancers developing from endometriosis (10/20 vs 13/79 (50.0% vs 16.5%), respectively). CONCLUSIONS: Ovarian pure clear cell carcinoma is usually diagnosed at an early stage and typically appears as a large unilateral mass with solid components. Patients with clear cell carcinoma developing from endometriosis are younger than other patients with clear cell carcinoma, and clear cell cancers developing from endometriosis more often manifest ground-glass echogenicity of cyst fluid. Copyright © 2018 ISUOG. Published by John Wiley & Sons Ltd.

• Klíčová slova
• ovarian neoplasm, pure clear cell ovarian carcinoma, ultrasonography,
• MeSH
• adenokarcinom z jasných buněk diagnostické zobrazování   etiologie   patologie   MeSH
• dospělí MeSH
• endometrióza komplikace   diagnostické zobrazování   MeSH
• lidé středního věku MeSH
• lidé MeSH
• nádory vaječníků diagnostické zobrazování   etiologie   patologie   MeSH
• retrospektivní studie MeSH
• senioři nad 80 let MeSH
• senioři MeSH
• ultrasonografie MeSH
• věkové faktory MeSH
• Check Tag
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• senioři nad 80 let MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• multicentrická studie MeSH

AIMS: Current available data on cytokeratin 7 (CK7) immunostaining pattern in the clear cell renal cell carcinoma (RCC) spectrum is conflicting. The aim of this study was to assess CK7 immunoreactivity within the spectrum of clear cell renal neoplasms, including clear cell RCC, multicystic renal neoplasm of low malignant potential and clear cell papillary RCC-like tumours. METHODS AND RESULTS: We analysed two clones of CK7 and two tumour blocks for a total of 75 cases divided into five distinct groups: (i) low-grade clear cell RCC, (ii) high-grade clear cell RCC, (iii) multicystic renal neoplasm of low malignant potential, (iv) clear cell RCC with cystic changes and (v) clear cell papillary RCC-like tumours. We found the highest CK7 reactivity in low-grade clear cell RCC, multicystic renal neoplasm of low malignant potential and clear cell papillary RCC-like groups, ranging from 60% to 93%. CONCLUSIONS: Our findings show that CK7 immunoreactivity in clear cell RCC is variable, and the extent of staining depends on the grade and architectural growth patterns of the tumours.

• Klíčová slova
• clear cell renal cell carcinoma, cytokeratin 7, immunohistochemistry, kidney,
• MeSH
• dospělí MeSH
• karcinom z renálních buněk patologie   MeSH
• keratin-7 biosyntéza   MeSH
• lidé středního věku MeSH
• lidé MeSH
• nádorové biomarkery analýza   MeSH
• nádory ledvin patologie   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
• Názvy látek
• keratin-7 MeSH
• nádorové biomarkery MeSH

Previous reports of monomorphic clear cell carcinoma of the salivary glands have shown inconsistent results with immunohistochemistry, especially for S-100 protein, and this has led to uncertainty about the nature of these tumours. We believe that much can be explained by considering this group as comprising not one but two separate neoplasms, one epithelial and the other myoepithelial. The former has been described as hyalinizing clear cell carcinoma--it generally occurs in the minor salivary glands, and strongly expresses cytokeratins but not S-100 protein or alpha smooth muscle actin. In contrast, this study presents five primary malignant tumours of the major salivary glands also composed largely of a single population of clear cells, but displaying histological and immunohistochemical features of myoepithelial differentiation, such as the formation of collagenous spherules and expression of S-100 protein and actin. A small number of similar tumours have been reported previously. We, therefore, believe that these neoplasms represent a clear cell variant of malignant myoepithelioma (myoepithelial carcinoma).

• MeSH
• adenokarcinom z jasných buněk patologie   MeSH
• aktiny analýza   MeSH
• dospělí MeSH
• imunohistochemie MeSH
• lidé středního věku MeSH
• lidé MeSH
• myoepiteliální nádor patologie   MeSH
• nádory slinných žláz patologie   MeSH
• proteiny S100 analýza   MeSH
• senioři MeSH
• Check Tag
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• Názvy látek
• aktiny MeSH
• proteiny S100 MeSH

BACKGROUND/AIM: The treatment of advanced clear cell renal cell carcinoma (ccRCC) is based on stratification of patients according to prognosis (favorable, intermediate, and poor). The aim of the study was to improve prognostication by biomarkers involved in angiogenesis. PATIENTS AND METHODS: The study group consisted of 20 patients who underwent surgery for ccRCC. Gene expression analysis was peformed on a set of matched (primary tumor, metastasis, n=20+20) FFPE tissue samples. An additional analysis was done on expression data of 606 patients obtained from the TCGA Kidney Clear Cell Carcinoma (KIRC) database. Quantitative estimation of mRNA of selected genes (TaqMan human Angiogenesis Array, 97 genes) was performed by a real-time RT-PCR method with TaqMan® arrays. RESULTS: Using the Cox regression model, 4 genes (PDGFB, FGF4, EPHB2 and BAI1) were identified whose expression was related to progression-free interval (PFI). Further analysis using the Kaplan Meier method conclusively revealed the relationship of BAI1 expression to prognosis (both datasets). Patients with higher BAI1 expression had significantly shorter PFI and overall survival. CONCLUSION: We showed that tumor tissue BAI1 expression level is a prognostic marker in ccRCC. Therefore, this gene might be involved in a prognostic panel to improve scoring systems on which the management of metastatic ccRCC patients is based.

• Klíčová slova
• BAI1, Clear cell renal cell carcinoma, FFPE, biomarkers, prognosis, treatment,
• MeSH
• analýza přežití MeSH
• angiogenní proteiny genetika   MeSH
• karcinom z renálních buněk genetika   mortalita   chirurgie   MeSH
• lidé MeSH
• nádorové biomarkery genetika   MeSH
• nádory ledvin genetika   mortalita   chirurgie   MeSH
• prognóza MeSH
• receptory spřažené s G-proteiny genetika   MeSH
• regresní analýza MeSH
• regulace genové exprese u nádorů MeSH
• sekvenční analýza hybridizací s uspořádaným souborem oligonukleotidů MeSH
• stanovení celkové genové exprese metody   MeSH
• upregulace * MeSH
• Check Tag
• lidé MeSH
• mužské pohlaví MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• Názvy látek
• ADGRB1 protein, human MeSH Prohlížeč
• angiogenní proteiny MeSH
• nádorové biomarkery MeSH
• receptory spřažené s G-proteiny MeSH