Small cell carcinoma of hypercalcemic type (SCCOHT) is a rare gynaecological neoplasm, originating mostly in the ovaries. Cervical origin of this very aggressive malignancy with unknown histogenesis is an extremely rare condition, without published management recommendations. Alterations in SMARCA4 gene are supposed to play the major role in SCCOHT oncogenesis and their identification is crucial for the diagnosis. Adequate genetic counselling of the patients and their families seems to be of great importance. Optimal management and treatment approaches are not known yet but may extremely influence the prognosis of young female patients that suffer from this very resistant disease. Nowadays, a translational research seems to be the key for the further diagnostic and treatment strategies of SCCOHT. The purpose of the case report is to provide practical information and useful recommendations on the diagnosis, management, and treatment of SMARCA4-deficient carcinoma of the uterine cervix resembling SCCOHT.

• Klíčová slova
• case report, cervical cancer, diagnostic biomarker, gynecological cancer, high-risk, personalized treatment, predictive marker,
• MeSH
• DNA-helikasy nedostatek   genetika   MeSH
• fatální výsledek MeSH
• hyperkalcemie diagnóza   genetika   metabolismus   terapie   MeSH
• jaderné proteiny nedostatek   genetika   MeSH
• lidé MeSH
• malobuněčný karcinom diagnóza   genetika   metabolismus   terapie   MeSH
• mladiství MeSH
• mutace MeSH
• nádorové biomarkery nedostatek   genetika   MeSH
• nádory děložního čípku diagnóza   genetika   metabolismus   terapie   MeSH
• transkripční faktory nedostatek   genetika   MeSH
• Check Tag
• lidé MeSH
• mladiství MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH
• Názvy látek
• DNA-helikasy MeSH
• jaderné proteiny MeSH
• nádorové biomarkery MeSH
• SMARCA4 protein, human MeSH Prohlížeč
• transkripční faktory MeSH

BACKGROUND: The objective of this study was to test the effect of chemotherapy and/or radical cystectomy (RC) and/or radiotherapy (RT) on survival of patients with non-metastatic small-cell carcinoma of the urinary bladder (SCCUB). MATERIALS AND METHODS: Within the Surveillance, Epidemiology, and End Results registry (2001-2016), we identified patients with non-metastatic (T1-4, N0, M0) SCCUB. Treatment was defined as: chemotherapy alone, chemotherapy + RC, and chemotherapy + RT. Temporal trends, cumulative incidence plots, and multivariable competing risks regression models were used. RESULTS: Of 595 patients with SCCUB, 230 (38.5%), 159 (27%), and 206 (34.5%) were treated with chemotherapy alone, chemotherapy + RC, and chemotherapy + RT, respectively. The rates of chemotherapy + RC increased (estimated annual percentage changes [EAPC], +5.9%; P = .002). Conversely, chemotherapy alone (EAPC, -1.7%; P = .1) and chemotherapy + RT rates decreased (EAPC: -2.2%; P = .08). Overall, 5-year cancer-specific mortality (CSM) rates were 44%, 29%, and 40% for patients treated with chemotherapy alone, chemotherapy + RC, and chemotherapy + RT, respectively (P = .004). Relative to chemotherapy alone, patients treated with chemotherapy + RC experienced lower CSM (hazard ratio, 0.5; P < .001). Conversely, patients treated with chemotherapy + RT did not exhibit any CSM benefit (hazard ratio, 0.8; P = .2), when compared with chemotherapy alone. CONCLUSION: In contemporary patients with SCCUB with non-metastatic disease, the rates of chemotherapy + RC are increasing. Conversely, the rates of combined chemotherapy with RT and chemotherapy alone are decreasing. These patterns of treatment are in agreement with better cancer control in patients with SCCUB. In consequence, until more robust data become available, the combination of chemotherapy and RC should represent the recommended treatment strategy.

• Klíčová slova
• Bladder cancer, Cancer specific survival, Chemotherapy, Radical cystectomy, Radiotherapy,
• MeSH
• cystektomie mortalita   MeSH
• kombinovaná terapie MeSH
• lidé středního věku MeSH
• lidé MeSH
• malobuněčný karcinom mortalita   patologie   terapie   MeSH
• míra přežití MeSH
• nádory močového měchýře mortalita   patologie   terapie   MeSH
• následné studie MeSH
• prognóza MeSH
• protokoly protinádorové kombinované chemoterapie terapeutické užití   MeSH
• retrospektivní studie 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
• časopisecké články MeSH

Small cell carcinoma of the ovary (SCCOHT) is a rare tumor typically affecting young women. It is a highly malignant tumor accompanied with poor prognosis, early relapse and low survival rates. The most significant prognostic factor is stage of the disease. Due to above mentioned factors there are no guidelines for therapy of this rare tumor. We present a case of 22-years-old patient initially treated with antibiotics under diagnosis of pelvic inflammatory disease. Due to persistent mass at left adnexa, she was indicated for diagnostic laparoscopy, converted to laparotomy and left adnexectomy with frozen section revealing unspecified malignant tumor of left ovary. A conservative operation was performed and, after diagnosis of SCCOHT was established, the patient was indicated for adjuvant chemotherapy.

• Klíčová slova
• SCCOHT - conservative surgery - chemotherapy.,
• MeSH
• diferenciální diagnóza MeSH
• dospělí MeSH
• hyperkalcemie * MeSH
• kombinovaná terapie MeSH
• lidé MeSH
• malobuněčný karcinom diagnóza   diagnostické zobrazování   terapie   MeSH
• nádory vaječníků diagnóza   diagnostické zobrazování   terapie   MeSH
• ovarektomie MeSH
• ultrasonografie MeSH
• Check Tag
• dospělí MeSH
• lidé MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH

The authors describe 2 tumors that, to the best of their knowledge, are hitherto undescribed. The predominant cell type was small round to fusiform dark blue cells. The dark blue cells formed distinct epithelial cords with gland-like formations with mucicarmine-positive mucus. Another distinctive component of the tumors was a mesenchymal one. The mesenchymal areas appeared benign and could be likened to a fibroma having a densely collagenous stroma, or they had spindle cells set in the myxoid background, rendering a myxoma-like appearance. Another distinctive feature was ganglion cell differentiation. Mitotic figures, including atypical forms, were found only in the small cell component. All cells were immunohistochemically negative for actin, calponin, desmin, HMB45, neurofilament protein, CD99/MIC2, Melan A, tyrosinase, serotonin, CD56, Melan A, GFAP, and S-100 protein. Cytokeratin, synaptophysin, FLI1 protein, and chromogranin antibodies reacted only in the primitive small round cells, while all the other components were cytokeratin negative. Fluorescence in situ hybridization showed that the tumors are without the EWSR1 gene translocation and gain 12p. Ultrastructurally, the cells were endowed with well-formed intercellular desmosomes membrane-bound secretory in the cytoplasm. Granules were found in the cytoplasm. We suggest the name "primitive small cell tumor with epithelial, gangliocytic, neuroendocrine, and mesenchymal differentiation" for this neoplasm.

• MeSH
• aneuploidie MeSH
• desmozomy ultrastruktura   MeSH
• epitelové buňky metabolismus   patologie   MeSH
• hybridizace in situ fluorescenční MeSH
• imunohistochemie MeSH
• kombinovaná terapie MeSH
• lidé MeSH
• lidské chromozomy, pár 12 MeSH
• malobuněčný karcinom genetika   metabolismus   patologie   terapie   MeSH
• malobuněčný sarkom genetika   metabolismus   patologie   terapie   MeSH
• nádorová transformace buněk MeSH
• nádorové biomarkery metabolismus   MeSH
• nádory měkkých tkání genetika   metabolismus   patologie   terapie   MeSH
• neuroendokrinní nádory genetika   metabolismus   patologie   terapie   MeSH
• předškolní dítě MeSH
• sekreční vezikuly ultrastruktura   MeSH
• transmisní elektronová mikroskopie MeSH
• Check Tag
• lidé MeSH
• mužské pohlaví MeSH
• předškolní dítě MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH
• Názvy látek
• nádorové biomarkery MeSH

Small cell carcinoma of the prostate (SCCP) is a rare entity. A literature review disclosed less than 150 cases. SCCP has an aggressive course and both local and distant failure is common. The optimal treatment method has not been clearly established. We review our experience with 7 patients paying attention to clinical and pathological details. Three patients had mixed tumors of both SCCP and adenocarcinoma, three had pure adenocarcinomas that occurred as small cell carcinoma and one patient had pure small cell carcinoma. All patients progressed locally and developed distant metastasis. We recommend the use of hormonal manipulation and combination of chemotherapy, as well as surgery and/or radiation therapy of the prostate for local control and we emphasize that histologic entity is important for proper treatment.

• MeSH
• adenokarcinom diagnóza   terapie   MeSH
• lidé středního věku MeSH
• lidé MeSH
• malobuněčný karcinom * diagnóza   terapie   MeSH
• nádory prostaty * diagnóza   terapie   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
• Publikační typ
• anglický abstrakt MeSH
• časopisecké články MeSH
• kazuistiky MeSH

• MeSH
• bronchogenní karcinom terapie   MeSH
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• malobuněčný karcinom terapie   MeSH
• nádory plic terapie   MeSH
• senioři MeSH
• Check Tag
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• anglický abstrakt MeSH
• časopisecké články MeSH