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ID naloge: 43 Letnik: 1998 Predmet: ginekologija in porodništvo
Sarkomi maternice - Klinicnopatološka študija
Avtor: Lara Besenicar
Tina Kamhi-Kiauta
Mentor: prof. Stelio Rakar
Sarkom je tumor mezenhimskega izvora, ki se pojavi v kosteh ali v mehkih tkivih. Predstavlja 2-6% vseh maternicnih tumorjev. Histološko ga delimo na leiomiosarkom, endometrijski stromalni sarkom, karcinosarkom, mešani maligni Muellerjev tumor in ostale sarkome (GOG), klinicno pa na štiri stadije (FIGO).
Predhodno obsevanje medenice je edini dokazani vzrok za nastanek sarkoma.
Klinicni znaki sarkoma so: obcutek polnosti v medenici, bolecine in obcasne krvavitve.
Poleg klinicnega pregleda je glavna diagnosticna metoda ultrazvok, ki omogoca tudi vodeno igelno biopsijo, ceprav se za to le redko odlocimo in postavimo diagnozo kar ob operaciji.
Najpogostejša oblika zdravljenja je kirurška odstranitev tumorja, ki je navadno dokoncna. Kot dodatno zdravljenje lahko uporabimo še obsevanje, kemoterapijo ali hormonsko terapijo, vendar dokazov o njihovi ucinkovitosti še ni.
Sarkom ima med vsemi tumorji maternice najslabšo prognozo, s petletnim preživetjem okrog 40%.
Zanimali so nas statisticno pomembni prognosticni dejavniki za petletno preživetje bolnic s sarkomom maternice.
Pregledali smo zdravstveno dokumentacijo 140 bolnic, ki so se med letoma 1970 in 1992 zdravile zaradi sarkoma maternice na Ginekološki kliniki in Onkološkem Inštitutu v Ljubljani.
Pogoj za uvrstitev bolnic v raziskavo so bili tudi ustrezni podatki v Registru raka za Slovenijo.
V dvomljivih primerih in ob pomanjkljivih podatkih smo vzorce še enkrat histološko pregledali in tumor ustrezno razvrstili.
Podatke smo racunalniško obdelali z deskriptivno statisticno metodo.
Najvec bolnic s sarkomom maternice je bilo v prvem stadiju (66,7%).
Histološko najpogostejši tumor je bil leiomiosarkom (46%), najredkejši pa MMMT (10%).
Ob histološkem pregledu smo v vecini primerov našli vec kot 10 mitoz na 10 VVP (58,4%).
Polipozna rašca je bila prisotna le v 12,1%, vendar je bila povezana z bistveno boljšim petletnim preživetjem (58,8%) v primerjavi s tumorji brez polipozne rašce (35%).
Pri bolnicah je bila najpogosteje narejena odstranitev maternice in adneksov obojestransko (69,3%), približno polovica jih je dobila tudi pooperativno radioterapijo ali kemoterapijo.
Recidiv se je najpogosteje pojavil v sami medenici (15,7%) oziroma kot oddaljeni zasevki v pljucih (11,4%).
Skupno petletno preživetje bolnic s sarkomom maternice je bilo 39,3%. Najboljše (42,9%) smo ugotovili pri bolnicah z leiomiosarkomom, pri karcinosarkomu in ESS je bilo preživetje 38,7%, dalec najslabše preživetje pa ima heterologni MMMT (7,1%).
S stadijem preživetje pada, od 48,3% pri prvem do 16,7% pri cetrtem stadiju.
Pooperativno zdravljenje ima statisticno nepomemben vpliv na prognozo bolezni.
Na petletno preživetje bolnic s sarkomom maternice statisticno znacilno oziroma mejno znacilno vplivajo: starost bolnice ob postavitvi diagnoze, histološki tip, stadij po FIGO, število mitoz in polipozna rašca tumorja.
Parametri, ki ne vplivajo na petletno preživetje so: število porodov, starost ob menopavzi, vrsta operacije, razširjenost tumorja na bezgavke in dodatno pooperativno zdravljenje.
Naša študija zajema v primerjavi s sorodnimi raziskavami v svetovni literaturi veliko število bolnic, kar omogoca natancnejše vrednotenje rezultatov in uporabo izsledkov pri vsakdanjem delu.
Vse dosedanje raziskave sarkomov maternice so bile retrospektivne, saj je bolnic s to boleznijo razmeroma malo. Pri prospektivni raziskavi, narejeni v smiselnem casovnem obdobju, bi zato moralo sodelovati vec raziskovalnih skupin na mednarodni ravni, ki bi v študijo uvrstile bolnice iz vec držav.
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[Abstract / English version]
Uterine sarcomas - A clinical pathological study
Author: Lara Besenicar
Tina Kamhi-Kiauta
Mentor: prof. Stelio Rakar
Uterine sarcomas are tumours of mesenchymal origin which can arise either in bones or in soft tissues. They represent 2-6% of all uterine tumours. Histologicaly they are divided into five groups according to GOG: leiomyosarcomas, endometrial stromal sarcomas, carcinosarcomas, mixed malignant Muellerian tumours and other sarcomas; whereas clinically they can be staged into four groups (FIGO).
So far only previous pelvic irradiation has been proven to cause the occurrence of uterine sarcomas.
Clinical symptoms of uterine sarcoma can be: presence of an abdominal mass, pelvic pain or occasional abnormal vaginal bleeding.
Diagnosis can be confirmed by ultrasound, which can also be helpful with needle biopsy. Usually diagnosis is made at the operation itself and ultrasound is thus rarely used.
The most frequent method of treatment is surgery alone. Radiotherapy, chemotherapy or hormonal therapy may be used as adjuvant therapy, although there is so far no evidence of beneficial effect of adjuvant treatment.
Of all uterine tumours sarcomas have the worst prognosis, the five-year survival rate being around 40%.
In our study we tried to determine prognostic factors that influence the five-year survival rate of patients with uterine sarcomas.
We surveyed medical documentation of 140 patients who have been treated for uterine sarcoma during the period 1970 - 1992 at The Department of Gynecology and The Institute of Oncology in Ljubljana.
In order to be included into our study group the patients also had to be registered in The Cancer Registry of Slovenia.
In doubtful cases or cases of missing histological information we reviewed the histological samples and re-classified the tumour.
The results were obtained by using a descriptive statistical method.
The majority of patients presented with stage I of sarcoma (66,7%).
The most frequent histological type in our study was leiomyosarcoma (46%) and the least frequent type was MMMT (10%).
In most tumours the mitotic count was more than 10 per 10 HPF (58,4%).
Polypous growth was present in only 12,1% of cases but these patients had a better five-year survival rate (58,8%) than those with tumours with no polypous growth (35%).
The most frequent type of operation performed in patients was hysterectomy with bilateral salpingo-oophorectomy (69,3%). Approximately 50% of surgically treated patients were in addition treated with adjuvant radiotherapy or chemotherapy.
Recurrence of the disease was mostly pelvic (15,7%) or presented itself as distant metastases in lungs (11,4%).
Overall five-year survival rate of patients with uterine sarcoma was 39,3%. It was the highest in patients with leiomyosarcoma (42,9%), in patients with carcinosarcoma or ESS it was 38,7% and the worst five-year survival rate was in patients with heterologous MMMT (7,1%).
With ascending clinical stage the five-year survival rate decreased from 48,3% in stage I to 16,7% in stage IV of uterine sarcoma.
Postoperative treatment had no statistically significant influence on prognosis of uterine sarcoma.
Factors of statistical or borderline statistical significance favouring the five-year survival rate were: age of patient, histological type, FIGO stage, mitotic index and polypous growth.
Factors that did not influence the five-year survival rate were: number of deliveries, age of onset of menopause, type of operation, presence of metastatic lymph nodes and adjuvant postoperative treatment.
Our study included, relative to similar studies in the literature, a larger number of patients which makes a more precise evaluation of results and an application of findings to routine clinical work possible.
All investigations of uterine sarcomas up to now have been retrospective due to the rarity of the disease. A prospective study performed within a sensible time period should result from collaboration of a number of research groups contributing patients from a number of countries.
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