BACKGROUND: Making the distinction between primary mucinous and metastatic ovarian tumors is often difficult, especially in tumors with a primary source from the gastrointestinal tract, pancreas and biliary tree. The aim of the following paper is to provide an overview of the problematics, with a focus on the possibilities of the differential diagnosis at the macroscopic, microscopic and immunohistochemical level. MAIN BODY: The three main aspects of mucinous ovarian tumors are described in detail, including the comparison of the available diagnostic algorithms based on the evaluation of mostly macroscopic features, characterization of the spectrum of microscopic features, and a detailed analysis of the immunophenotype comparing 20 antibodies with the assessment of their statistical significance for differential diagnosis purposes. Specific features, including Krukenberg tumor and pseudomyxoma peritonei, are also discussed. CONCLUSION: Despite the growing knowledge of the macroscopic and microscopic features of ovarian mucinous tumors and the availability of a wide range of immunohistochemical antibodies useful in this setting, there still remains a group of tumors which cannot be precisely classified without close clinical-pathological cooperation.
- Klíčová slova
- Mucinous borderline tumor, Mucinous carcinoma, Ovarian metastases, Ovarian tumors,
- MeSH
- gastrointestinální trakt patologie MeSH
- lidé MeSH
- mucinózní adenokarcinom patologie MeSH
- nádory vaječníků patologie MeSH
- pankreas patologie MeSH
- peritoneální nádory patologie MeSH
- pseudomyxom peritonea diagnóza patologie MeSH
- Check Tag
- lidé MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- přehledy MeSH
BACKGROUND: Pseudomyxoma peritonei is a rare tumorous disease with various grades of malignancy and is characterized by production of mucinous and gelatinous masses. Development of pseudomyxoma peritonei is usually associated with rupture of appendiceal mucinous tumors and other mucinous tumors of the gastrointestinal tract or ovaries. Pseudomyxoma peritonei is usually divided into three types: low-grade, high-grade, and high-grade with signet ring cells. Staging of the disease is determined by the peritoneal cancer index. Clinical findings are highly variable depending on disease staging. The typical finding of “jelly belly” syndrome worsens with disease progression. The diagnosis is based on the pre-operative cure by imaging methods, especially computed tomography. METHODS: The Sugarbaker method involves maximal removal of tumorous masses, so-called cytoreductive surgery, and perioperative intraabdominal application of hot cytostatics (hyperthermic intraperitoneal chemotherapy) with the aim of achieving maximal liquidation of tumorous processes. RESULTS: Our results are comparable with previous published data and confirm high effectivness of this method. The results show statistically very significant extention of overall survival, disease free interval with acceptable lethality 0-12 % and morbidity 27-56%. These results promote this method as the gold standard of treatment of pseudomyxoma peritonei in selected patients.
- Klíčová slova
- HIPEC, PCI, cytoreductive surgery, hypertermic intraperitoneal chemotherapy, pseudomyxoma peritonei,
- MeSH
- cytoredukční chirurgie * MeSH
- indukovaná hypertermie * MeSH
- kombinovaná terapie MeSH
- lidé MeSH
- peritoneální nádory diagnóza patologie terapie MeSH
- pseudomyxom peritonea diagnóza patologie terapie MeSH
- Check Tag
- lidé MeSH
- Publikační typ
- časopisecké články MeSH
- přehledy MeSH
OBJECTIVE: To describe personal experience with three heterogeneous cases of pseudomyxoma peritonei. Review of the literature concentrates on the pathogenesis and biologic spectrum of the rare syndrome. DESIGN: Three case reports. SETTING: Private Biopsy Lab s.r.o. and Sikl's Department of Pathology, Charles University and Faculty Hospital, Pilsen. METHODS AND RESULTS: Included are cases from Biopsy Lab s.r.o. and from personal consulting registry of Prof. Michal, Sikl's Department of Pathology, Pilsen. Clinical presentation of three cases of pseudomyxoma peritonei documented is in details including long follow up. Broad variability of histologic appearance associated with different Clinical outcome is demonstrated. Immunohistochemistry performed by streptavidin-biotin system (LSAB+, Dako) as detection system, and diaminobenzidine tetrahydrochloride as chromogen, employed three monoclonal antibodies--MUC2 (Novocastra, 1:400), cytokeratin 7 (Dako, 1:200) and cytokeratin 20 (Dako, 1:100). CONCLUSION: Pseudomyxoma peritonei is a clinical syndrome defined as presence of massive mucinous, viscous material in the peritoneal cavity, both floating and adhering to serosal surface (jelly-belly). The histologic examination should always follow with detailed and precise description of the epithelial component. The cytological and structural quality of the epithelium constitutes two basic forms with entirely different nature biology and prognosis. First and more frequent, so-called disseminated peritoneal adenomucinosis, where primary low grade (benign) mucinous appendiceal tumor is almost constant finding, often recurs but displays favorable prognosis. Second, so-called peritoneal mucinous carcinomatosis is an extraordinary manifestation of peritoneal carcinosis following generalization of the gastrointestinal mucinous adenocarcinoma. Histopathogenesis in both types is thought to arise from MUC2 positive goblet cells in GI and ovarian involvement is secondary. A normal macroscopic finding on appendix or "uneventful" appendectomy in anamnesis is not unusual.
- MeSH
- dospělí MeSH
- lidé středního věku MeSH
- lidé MeSH
- peritoneální nádory diagnóza patologie MeSH
- pseudomyxom peritonea diagnóza patologie MeSH
- Check Tag
- dospělí MeSH
- lidé středního věku MeSH
- lidé MeSH
- mužské pohlaví MeSH
- ženské pohlaví MeSH
- Publikační typ
- anglický abstrakt MeSH
- časopisecké články MeSH
- kazuistiky MeSH
