Leiomyomas with adipocytic differentiation typically occur in the uterus although they may arise at several sites in the female genital tract. While these are most commonly spindled leiomyomas with a component of adipocytic tissue ("conventional lipoleiomyomas"), there is a relatively ill-defined assortment of leiomyoma variants with adipocytic differentiation. We performed a morphologic, immunohistochemical and MDM2 gene amplification analysis of a large series of gynecologic leiomyomas with adipocytic differentiation to better define the clinicopathologic spectrum. Forty four tumors from 44 patients were identified and classified as conventional lipoleiomyoma (n = 21), adipocyte-rich lipoleiomyoma (defined as tumor volume >80 % adipocytes, n = 9); cellular lipoleiomyoma (n = 9); hydropic lipoleiomyoma (n = 3); and lipoleiomyoma with bizarre nuclei (n = 2). Patient age ranged from 32 to 83 years (mean 63; median 63). Primary location included uterine corpus (35), uterine cervix (3), uterine corpus/cervix (1), broad ligament (2), parametrium (2), and round ligament (1). Tumor size was 0.6-30 cm (mean 8; median 6). None of the 34 patients with follow up developed further disease (range 1-311 months; mean 65; median 41). Immunohistochemical expression of ER, PR, HMB45, Melan A, Cathepsin K and WT-1 in lipoleiomyomas and variants was similar to patterns in non-adipocytic gynecologic leiomyomas. MDM2 amplification fluorescence in situ hybridization performed on 14 tumors was negative in all. Our findings suggest female genital tract conventional lipoleiomyomas and lipoleiomyoma variants largely parallel their non-adipocytic counterparts in morphology and immunophenotype, and may be categorized using non-adipocytic leiomyoma histologic criteria.
- MeSH
- dospělí MeSH
- hybridizace in situ fluorescenční MeSH
- leiomyom * patologie MeSH
- lidé středního věku MeSH
- lidé MeSH
- lipom * genetika patologie MeSH
- nádor z hladké svalové tkáně * MeSH
- nádory dělohy * patologie MeSH
- protoonkogenní proteiny c-mdm2 genetika MeSH
- senioři nad 80 let MeSH
- senioři MeSH
- uterus patologie 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
Cíl: Ilustrační přehled indikací relugolix kombinované terapie (RCT) při řešení symptomů spojených s děložní myomatózou. Metodika: Soubor komentovaných kazuistik z ambulantní a klinické praxe. Výsledky: Soubor zahrnuje neinvazivní metodiku definice excesivního menstruačního krvácení pomocí obrázkového záznamu (PBAC – pictorial bleeding assessment chart). Dále předkládá použití RCT jako fertilitu šetřícího postupu před plánovanou myomektomií a řešení istmického myomu jako děložního faktoru neplodnosti. Komentovány jsou případy RCT adenomyózy u primární sterility a u extragenitálních forem endometriózy. Náhlé příhody spojené s komplikacemi myomatózy v graviditě reprezentuje kazuistika o nekrotizující difuzní myomatóze v puerperiu. Diferenciálně diagnostické rozpaky adnexální patologie a myomatózy, RCT jako finální řešení neúspěšné farmakoterapie a alternativa hysterektomie v premenopauze dokreslují pestré spektrum indikací farmakologické léčby, vč. možností duální terapie RCT s aGnRH. Závěr: Relugolix kombinovaná terapie jako účinná a bezpečná kauzální léčba rozšiřuje terapeutické spektrum a možnosti specialistů na reprodukční medicínu a registrujících gynekologů. Dostupnost konzervativní léčby v kombinaci s chirurgickou léčbou vede k optimalizaci a vyšší efektivitě terapeutických postupů a zvýšení kvality života žen s myomatózou.
Objective: An illustrative review of the indications for relugolix combination therapy (RCT) in the management of symptoms associated with uterine myomatosis. Methods: A set of annotated case reports from outpatient and clinical practice. Results: The file includes a non-invasive methodology for defining excessive menstrual bleeding using the pictorial bleeding assessment chart (PBAC). It also presents the use of RCT as a fertility-sparing procedure prior to elective myomectomy and the management of isthmic fibroids as an uterine factor of infertility. Cases of RCT of adenomyosis in primary sterility and in extragenital forms of endometriosis are commented. Emergent events associated with complications of myomatosis in pregnancy are represented by a case report of necrotizing diff use myomatosis in puerperium. The differential-diagnostic confusion of adnexal pathology and myomatosis, RCT as a final solution to failed pharmacotherapy, and the alternative of hysterectomy in premenopause illustrate the diverse spectrum of indications for pharmacological treatment, including the possibility of dual therapy with RCT and aGnRH. Conclusion: Relugolix combination therapy as an effective and safe causal treatment expands the therapeutic spectrum and options for reproductive medicine specialists and registering gynaecologists. The availability of conservative treatment in combination with surgical treatment leads to optimalization and greater effectiveness of therapeutic procedures and increased quality of life for women with myomatosis.
- Klíčová slova
- relugolix,
- MeSH
- adenomyóza diagnostické zobrazování farmakoterapie MeSH
- antitumorózní látky terapeutické užití MeSH
- dospělí MeSH
- hormon uvolňující gonadotropiny antagonisté a inhibitory MeSH
- kombinovaná terapie MeSH
- lidé středního věku MeSH
- lidé MeSH
- menstruační poruchy etiologie farmakoterapie MeSH
- myom * diagnostické zobrazování farmakoterapie patologie MeSH
- nádory dělohy diagnostické zobrazování farmakoterapie patologie MeSH
- silné menstruační krvácení etiologie farmakoterapie MeSH
- Check Tag
- dospělí MeSH
- lidé středního věku MeSH
- lidé MeSH
- ženské pohlaví MeSH
- Publikační typ
- kazuistiky 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
- adenosarkom * terapie patologie MeSH
- endometriální stromální sarkom * terapie patologie MeSH
- leiomyosarkom * patologie MeSH
- lidé MeSH
- lokální recidiva nádoru MeSH
- nádory dělohy * patologie MeSH
- nádory endometria * patologie MeSH
- nádory pánve * MeSH
- prognóza MeSH
- retrospektivní studie MeSH
- sarkom * diagnóza MeSH
- Check Tag
- lidé MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- multicentrická studie MeSH
OBJECTIVE: The aim of this study was to analyze the clinical and reproductive outcomes of patients treated with myomectomy who were histologically diagnosed with uterine smooth muscle tumor of uncertain malignant potential (STUMP). METHODS: Patients who were diagnosed with STUMP and underwent a myomectomy at our institution between October 2003 and October 2019 were identified. Variables of interest obtained from the institution's database included patient age, relevant medical history, pre-operative appearance of the tumor on ultrasound, parameters of the surgical procedure, histopathological analysis of the tumor, post-operative clinical course, and course of follow-up, including reinterventions and fertility outcomes. RESULTS: There were a total of 46 patients that fulfilled the criteria of STUMP. The median patient age was 36 years (range, 18-48 years) and the mean follow-up was 47.6 months (range, 7-149 months). Thirty-four patients underwent primary laparoscopic procedures. Power morcellation was used for specimen extraction in 19 cases (55.9% of laparoscopic procedures). Endobag retrieval was used in nine patients and six procedures were converted to an open approach due to the suspicious peri-operative appearance of the tumor. Five patients underwent elective laparotomy due to the size and/or number of tumors; three patients had vaginal myomectomy; two patients had the tumor removed during planned cesarean section; and two underwent hysteroscopic resection.There were 13 reinterventions (five myomectomies and eight hysterectomies) with benign histology in 11 cases and STUMP histology in two cases (4.3% of all patients). We did not observe any recurrence as leiomyosarcoma or other uterine malignancy. We did not observe any deaths related to the diagnosis. Twenty-two pregnancies were recorded among 17 women, which resulted in 18 uncomplicated deliveries (17 by cesarean section and one vaginal), two missed abortions, and two pregnancy terminations. CONCLUSIONS: Our study found that uterus-saving procedures and fertility-preservation strategies in women with STUMP are feasible, safe, and seem to be associated with a low risk of malignant recurrence, even while maintaining the mini-invasive laparoscopic approach.
- MeSH
- císařský řez MeSH
- fertilita MeSH
- kojenec MeSH
- laparoskopie * metody MeSH
- lidé MeSH
- myomektomie * škodlivé účinky MeSH
- nádor z hladké svalové tkáně * patologie MeSH
- nádory dělohy * patologie MeSH
- předškolní dítě MeSH
- retrospektivní studie MeSH
- těhotenství MeSH
- uterus patologie MeSH
- Check Tag
- kojenec MeSH
- lidé MeSH
- předškolní dítě MeSH
- těhotenství MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- práce podpořená grantem MeSH
- MeSH
- diferenciální diagnóza MeSH
- dítě MeSH
- lidé MeSH
- mladiství MeSH
- nádory dělohy diagnostické zobrazování epidemiologie patologie MeSH
- nádory vaginy diagnostické zobrazování epidemiologie patologie MeSH
- nádory vaječníků diagnostické zobrazování epidemiologie patologie MeSH
- nádory vulvy * diagnostické zobrazování epidemiologie patologie MeSH
- Check Tag
- dítě MeSH
- lidé MeSH
- mladiství MeSH
- ženské pohlaví MeSH
Cellular leiomyoma (CL) represents an uncommon variant of uterine leiomyoma with limited data concerning its immunohistochemical and molecular profile. We performed a comprehensive analysis of 52 CL cases all of which were analyzed immunohistochemically. Molecular analysis was possible in 32 cases with sufficient DNA, and 38 cases with sufficient RNA. The immunohistochemical results showed a high expression of smooth muscle markers (calponin (100%), desmin (100%), smooth muscle actin (98.1%), caldesmon (96.1%), transgelin (96.1%), smooth muscle myosin heavy chain (86.5%), and smoothelin (61.5%)). Concerning markers of endometrial stromal differentiation, the expression of CD10 was observed in 65.4% cases (42.2% with H-score > 50), and IFITM1 in 36.5% cases (1.9% with H-score > 50). 36.5% showed HMGA2 overexpression at the IHC level, associated with increased mRNA expression in 14/14 cases. The rearrangement of the HMGA2 gene was detected in 13.2%. Chromosome 1p deletion was found in 19.3%, while 9.4% of tumors showed a pathogenic mutation in the MED12 gene. In conclusion, CL is immunohistochemically characterized by a high expression of "smooth muscle" markers commonly associated with a co-expression of "endometrial stromal" markers, where IFITM1 shows superior performance compared to CD10 regarding its specificity for differentiation from endometrial stromal tumors. The sensitivity of smoothelin in CL seems rather low, but no data is available to assess its specificity. On a molecular level, the most common mutually exclusive aberration in CL affects HMGA2, followed by chromosome 1p deletions and MED12 mutations.
- MeSH
- chromozomy chemie metabolismus MeSH
- leiomyom * patologie MeSH
- lidé MeSH
- mediátorový komplex genetika metabolismus MeSH
- mutace MeSH
- nádory dělohy * patologie MeSH
- nádory endometria * genetika MeSH
- neprilysin analýza MeSH
- protein HMGA2 MeSH
- Check Tag
- lidé MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
Cíl: Podat aktuální pohled na problematiku laparoskopické elektrické morcelace. Metodika: Rešerše dostupné literatury na vybrané téma. Výsledky: Laparoskopická morcelace byla zavedena do gynekologické operativy v 90. letech. V roce 2014 bylo americkým Úřadem pro kontrolu potravin a léčiv vydáno negativní stanovisko stran jejího užívání pro riziko možného rozsevu maligních buněk. Od té doby se použití morcelace snížilo, a to především ve Spojených státech. Některé zdravotnické organizace a instituce započaly nové výzkumy stran bezpečnosti této chirurgické metody. Po dalších letech mnohé z nich přistupují k morcelaci opět smířlivě za dodržení určitých podmínek. Závěr: Morcelace má prostor v laparoskopické operativě i v roce 2022 za předpokladu správné selekce pacientek a případně využití možnosti chráněné „in-bag“ morcelace.
Objective: To summarize recent data and knowledge of laparoscopic power morcellation. Methods: Review of articles. Results: Laparoscopic morcellation has been introduced to gynecologic surgery in 90's. In 2014, Food and Drug Administration announced negative statement about the morcellation use due to the risk of potential spreading of malignant tumor cells. This statement reduced utilization of morcellation, especially in the United States. Since that, many health institutions and organizations started new researches focused on the safety of this surgical technique. After a couple of years, the morcellation is considered as a useful tool if certain rules are followed. Conclusion: Morcellation has a place in laparoscopic operative procedures even in 2022, in condition of correct selection of patients and possible utilization of contained in-bag morcellation.
- MeSH
- hysterektomie metody MeSH
- laparoskopie metody MeSH
- leiomyom chirurgie MeSH
- lidé MeSH
- morcelace * metody přístrojové vybavení škodlivé účinky MeSH
- myomektomie * metody MeSH
- nádory dělohy chirurgie patologie MeSH
- Check Tag
- lidé MeSH
- ženské pohlaví MeSH
- Publikační typ
- přehledy MeSH
- Klíčová slova
- submukózní myom,
- MeSH
- diagnostické zobrazování metody MeSH
- hysteroskopie metody MeSH
- leiomyom diagnóza etiologie patologie terapie MeSH
- lidé MeSH
- myom * chirurgie diagnóza etiologie klasifikace terapie MeSH
- myometrium diagnostické zobrazování patologie MeSH
- nádory dělohy diagnóza etiologie klasifikace patologie MeSH
- Check Tag
- lidé MeSH
- ženské pohlaví MeSH
- Publikační typ
- přehledy MeSH
- MeSH
- gynekologická onemocnění * diagnóza etiologie patologie terapie MeSH
- lidé MeSH
- myom MeSH
- nádory dělohy diagnóza patologie terapie MeSH
- nádory ženských pohlavních orgánů diagnóza patologie terapie MeSH
- nemoci cervix uteri diagnóza patologie terapie MeSH
- nemoci dělohy diagnóza patologie terapie MeSH
- nemoci ovaria diagnóza etiologie patologie MeSH
- nemoci vejcovodů diagnóza etiologie MeSH
- nemoci vulvy diagnóza etiologie terapie MeSH
- ultrasonografie metody MeSH
- Check Tag
- lidé MeSH
- ženské pohlaví MeSH
- Publikační typ
- přehledy MeSH
Leiomyoma with bizarre nuclei (LBN) is an uncommon variant of uterine smooth muscle neoplasm. Involvement of fumarate hydratase (FH) has been suggested in the pathogenesis of a subset of LBN. The goal of our study is to assess the clinicopathological, morphological, immunohistochemical and molecular findings focusing on FH in LBNs (n = 108) and compare it with the findings in usual leiomyomas (UL; n = 50) and leiomyosarcomas (LMS; n = 42). Immunohistochemically, loss of FH expression was found in 67/108 of LBN, 1/50 of UL and in no LMS. Class 4/5 FH mutations were detected in 15/53 LBN with sufficient DNA quality for molecular analysis. Pathogenic variants of the FH gene were detected in neither UL nor LMS. Local recurrence after surgery was present in 18/92 of LBN patients, 7 of which were histologically verified and 2 of which were found to be LBN. Our results confirmed that LBN behave in a benign fashion, although they may relapse. FH gene mutations were a common finding only in LBN, but not in UL and LMS. Immunohistochemistry with an antibody against FH seems to have a good sensitivity (87%) and moderate specificity (58%) with regard to predicting FH gene mutations and could be used as a screening method in tumors with features suggestive of FH alterations to identify patients who are at risk for the FH aberrations.
- MeSH
- buněčné jádro patologie MeSH
- dospělí MeSH
- fumarasa genetika MeSH
- leiomyom genetika patologie MeSH
- lidé středního věku MeSH
- lidé MeSH
- mutace MeSH
- nádorové biomarkery genetika MeSH
- nádory dělohy genetika patologie MeSH
- senioři MeSH
- Check Tag
- dospělí MeSH
- lidé středního věku MeSH
- lidé MeSH
- senioři MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH