Cíl: Přehled typů děložních sarkomů doplněný ilustrativní kazuistikou raritního děložního nádoru dětského věku. Kazuistika: Naše kazuistika popisuje případ 9leté dívky s nálezem 15cm tumoru v dutině břišní, který při klinickém vyšetření břišní stěny zprolaboval vaginálně před introitus a zapříčinil život ohrožující krvácení. Toto krvácení bylo zastaveno ligací stopky tumoru a jeho resekcí. Histologické vyšetření resekátu prokázalo mülleriánský adenosarkom dělohy. Na základě stagingu byla onkogynekologickou komisí indikována laparoskopická hysterektomie s bilaterální salpingektomií. Operační výkon proběhl bez komplikací, peroperačně byla diagnostikována kompletní inverze dělohy.
Objective: An overview of the types of uterine sarcoma, supplemented by an illustrative case report of a rare uterine childhood tumor. Case report: Our case report describes the case of a 9-year-old girl with a 15-cm tumor in the abdominal cavity, which prolapsed vaginally before introitus during a clinical examination of the abdominal wall causing life-threatening bleeding. This bleeding was stopped by ligation of the tumor pedicle and its resection. Histological examination of the resected specimen described Müllerian adenosarcoma of the uterus. Based on the staging, laparoscopic hysterectomy with bilateral salpingectomy was indicated by the oncogynecological committee. The operation was performed without complications, and intraoperatively, a complete inversion of the uterus was diagnosed.
- MeSH
- Adenosarcoma pathology MeSH
- Diagnosis, Differential MeSH
- Child MeSH
- Hemostasis, Surgical methods MeSH
- Hysterectomy methods MeSH
- Uterine Inversion diagnosis MeSH
- Humans MeSH
- Uterine Neoplasms surgery pathology MeSH
- Uterine Cervical Neoplasms * pathology MeSH
- Salpingectomy MeSH
- Sarcoma * pathology MeSH
- Check Tag
- Child MeSH
- Humans MeSH
- Female MeSH
- Publication type
- Case Reports MeSH
OBJECTIVE: Uterine sarcomas are a rare and heterogeneous group of malignancies that include different histological sub-types. The aim of this study was to identify and evaluate the impact of the different prognostic factors on overall survival and disease-free survival of patients with uterine sarcoma. METHODS: This international multicenter retrospective study included 683 patients diagnosed with uterine sarcoma at 46 different institutions between January 2001 and December 2007. RESULTS: The 5-year overall survival for leiomyosarcoma, endometrial stromal sarcoma, undifferentiated sarcoma, and adenosarcoma was 65.3%, 78.3%, 52.4%, and 89.5%, respectively, and the 5-year disease-free survival was 54.3%, 68.1%, 40.3%, and 85.3%, respectively. The 10-year overall survival for leiomyosarcoma, endometrial stromal sarcoma, undifferentiated sarcoma and adenosarcoma was 52.6%, 64.8%, 52.4%, and 79.5%, respectively, and the 10-year disease-free survival was 44.7%, 53.3%, 40.3%, and 77.5%, respectively. The most significant factor associated with overall survival in all types of sarcoma except for adenosarcoma was the presence of residual disease after primary treatment. In adenosarcoma, disease stage at diagnosis was the most important factor (hazard ratio 17.7; 95% CI 2.86 to 109.93). CONCLUSION: Incomplete cytoreduction, tumor persistence, advanced stage, extra-uterine and tumor margin involvement, and the presence of necrosis were relevant prognostic factors significantly affecting overall survival in uterine sarcoma. The presence of lymph vascular space involvement and administration of adjuvant chemotherapy were significantly associated with a higher risk of relapse.
- MeSH
- Adenosarcoma * therapy pathology MeSH
- Sarcoma, Endometrial Stromal * therapy pathology MeSH
- Leiomyosarcoma * pathology MeSH
- Humans MeSH
- Neoplasm Recurrence, Local MeSH
- Uterine Neoplasms * pathology MeSH
- Endometrial Neoplasms * pathology MeSH
- Pelvic Neoplasms * MeSH
- Prognosis MeSH
- Retrospective Studies MeSH
- Sarcoma * diagnosis MeSH
- Check Tag
- Humans MeSH
- Female MeSH
- Publication type
- Journal Article MeSH
- Multicenter Study MeSH
- MeSH
- Adenosarcoma pathology therapy MeSH
- Sarcoma, Endometrial Stromal diagnosis classification pathology therapy MeSH
- Leiomyosarcoma surgery diagnosis physiopathology therapy MeSH
- Humans MeSH
- Uterine Neoplasms * surgery diagnosis classification physiopathology pathology therapy MeSH
- Prognosis MeSH
- Neoplasm Staging MeSH
- Severity of Illness Index MeSH
- Check Tag
- Humans MeSH
- Female MeSH
- Publication type
- Review MeSH
Cíl studie: Analýza klinických a histopatologických prognostických faktorů zejména u karcinosarkomů, leiomyosarkomů a endometriálních sarkomů dělohy. Typ studie: Souhrnný přehled. Název a sídlo pracoviště: Klinika porodnictví a gynekologie, LF UK v Hradci Králové; Fingerlandův ústav patologie v Hradci Králové; Klinika onkologie a radioterapie, LF UK v Hradci Králové; Katedra biologických a lékařských věd, FAF UK. Metodika: Diskuse na základě vlastních zkušeností a současného literárního přehledu. Závěr: Také mezenchymální a smíšené nádory dělohy jsou charakterizovány řadou prognostických faktorů. Kromě klinických proměnných je velmi špatná prognóza odrazem řady histopatologických a molekulárně biologických ukazatelů. Největší prognostický význam má stadium onemocnění, věk a celkový stav pacientky, ale i adekvátní léčba, a zejména pak chirurgie. U jednotlivých typů nádorů se uplatňují i faktory další. S výjimkou zastižení skutečně časného stadia onemocnění nebývá interval mezi koncem primární léčby a začátkem recidivy nádoru dlouhý. Kromě endometriálních stromálních nádorů je prognóza těchto nádorů velmi špatná.
Objective: Analysis of clinical and histopathological prognostic factors with emphasis on carcinosarcomas, leiomyosarcomas and endometrial sarcomas of uterus. Subject: Review. Setting: Department of Obstetrics and Gynaecology, Medical Faculty Hradec Králové, Charles University, Prague; The Fingerland Department of Pathology, Medical Faculty Hradec Králové, Charles University, Prague; Department of Clinical Oncology, Medical Faculty Hradec Králové, Charles University, Prague; Department of Biological and Medical Sciences, Faculty of Pharmacy, Hradec Králové; Charles University, Prague. Subject and method: Discussion about our experience and current evidence. Conclusion: Mesenchymal and mixed tumors of uterus are characterized by several prognostic factors as well. Except clinical prognostic variables, histopathological and molecular biological factors may play a role. The most important prognostic significance carry stage of disease, age and performance status of patient and sufficient therapy, especially surgery. In particular tumors some other prognostic factors are recognized. Except cases in really early stage of disease there is usually short interval between end of primary therapy and tumor recurrence. With the exception of endometrial stromal sarcoma, prognosis of these tumors remains very poor.
- Keywords
- prognostický faktor, stadium,
- MeSH
- Adenosarcoma MeSH
- Endometrial Stromal Tumors MeSH
- Financing, Organized MeSH
- Carcinosarcoma MeSH
- Leiomyosarcoma MeSH
- Humans MeSH
- Uterine Neoplasms MeSH
- Neoplasms, Complex and Mixed classification MeSH
- Neoplasms by Histologic Type MeSH
- Perivascular Epithelioid Cell Neoplasms MeSH
- Prognosis MeSH
- Rhabdomyosarcoma MeSH
- Sarcoma MeSH
- Severity of Illness Index MeSH
- World Health Organization MeSH
- Check Tag
- Humans MeSH
- Female MeSH
- Publication type
- Review MeSH
Mezenchymálne tumory tela maternice a ovaria predstavujú širšiu skupinu, v ktorej došlo v ostatných rokoch k popisu nových jednotiek, k viacerým zmenám v klasifikácii (po prehodnotení histogenézy a vzájomných vzťahov medzi jednotkami) a k upresneniu poznatkov o biologickej povahe tumorov. Tento prehľad, ktorý vzhľadom na rozsah problematiky nemôže byť úplný, sa zameriava práve na prezentáciu novších poznatkov, ako sú: spresnená diagnostika biologickej povahy u hladkosvalových tumorov, stromálne tumory maternice a ich novšie varianty, UTROSCT (uterine tumor resembling ovarian sex cord tumor), PEC-ómy a PEC-ómová diferenciácia, biologická povaha skupiny „ovariálny fibróm-celulárny fibróm-mitoticky aktívny celulárny fibróm-fibrosarkóm“, sklerotizujúci stromálny tumor a myxóm ovaria. V prípade ovaria sú teda diskutované len lézie, ktorých origom nie je špecializovaná ovariálna stróma, nakoľko tumory zo špecializovaného mezodermu ovaria predstavujú ďalšiu rozsiahlu skupinu, ktorej popis vyžaduje osobitný prehľad.
In the large group of uterine and ovarian tumors, the knowledge was updated in recent years substantially. New entities were defined and changes in classification of the lesions were performed. This review is limited to updates, such as evaluation of uterine smooth muscle tumors, new variants of uterine stromal tumors, uterine tumors resembling ovarian sex-cord tumor (UTROSCT), perivascular epithelioid cell tumors (PEC-omas), ovarian fibroma and fibrosarcoma, sclerosing stromal tumor and myxoma. Group of tumors of specialized gonadal stroma is not discussed as it represents particular area and thus requires a separated review article.
- MeSH
- Adenosarcoma diagnosis classification pathology MeSH
- Endometrial Stromal Tumors diagnosis classification pathology MeSH
- Leiomyoma diagnosis classification pathology MeSH
- Leiomyosarcoma diagnosis classification pathology MeSH
- Humans MeSH
- Uterine Neoplasms classification pathology MeSH
- Neoplasms by Histologic Type MeSH
- Neoplasms by Site MeSH
- Ovarian Neoplasms classification pathology MeSH
- Neoplasms, Fibrous Tissue diagnosis classification pathology MeSH
- Check Tag
- Humans MeSH
Popísaný je neobvyklý prípad gastrointestinálneho stromálneho tumoru (GIST) s glandulárnou zložkou. Šlo o tumor gastrického fundu u 93-ročnej ženy. Histológia lézie bola tvorená diferen- covanou vretenobunkovou štruktúrou typického GIST-u, v ktorej boli difúzne prítomné z časti cystické žliazky. Kolumnárny epitel žliazok bol podobný pylorickému a foveolárnemu typu, s lo- žiskami intestinálnej metaplázie a miestami s dyspláziami. Stromálna zložka tumoru bola imuno- histochemicky pozitívna na CD117 (c-kit) a CD34, negatívne boli myoidné a neuroidné markery. Elektronmikroskopicky šlo o nediferencované vretenovité bunky. Sliznica a submukóza v blízkos- ti tumoru vykazovali fokálne známky gastritis cystica profunda so žliazkami podobnými ako vnú- tri tumoru. V histogenéze tumoru je predpokladaná kolízia GIST-u a gastritis cystica profunda.
A case of gastrointestinal stromal tumor (GIST) with an unusual glandular component is reported. The tumor was found in the gastric fundus of a 93-year-old woman. Histologically, the lesion showed a biphasic adenosarcoma-like structure. Typical low-grade spindle cell patterns of GIST were intermingled with numerous and partly cystic glands. The glandular epithelium had pyloric/foveolar-like appearance, with foci of intestinal metaplasia and low-grade dysplasia. The stromal component was immunoreactive for CD117 (c-kit) and CD34, and negative for myoid and neuroid markers. The ultrastructural examination found nondescript and undifferentiated spindle cells. The gastric mucosa and submucosa near the tumor contained a small area with features of gastritis cystica profunda, with glands similar to those present inside the tumor. Therefore, a collision of GIST and gastritis cystica profunda is suggested in the histogenesis of the lesion.
- MeSH
- Adenocarcinoma diagnosis pathology ultrastructure MeSH
- Adenosarcoma diagnosis pathology MeSH
- Biopsy MeSH
- Gastritis diagnosis pathology MeSH
- Immunohistochemistry MeSH
- Humans MeSH
- Stomach Neoplasms diagnosis pathology MeSH
- Check Tag
- Humans MeSH
- Female MeSH
- Publication type
- Case Reports MeSH
- MeSH
- Adenosarcoma pathology therapy MeSH
- Adult MeSH
- Histological Techniques MeSH
- Hysterectomy MeSH
- Hysteroscopy MeSH
- Humans MeSH
- Uterine Neoplasms diagnosis classification therapy MeSH
- Prognosis MeSH
- Check Tag
- Adult MeSH
- Humans MeSH
- Female MeSH
- Publication type
- Case Reports MeSH
