Asymptomatic uterus-like mass (ULM) of the extraperitoneal space - a case report of a rare finding with unusual clinical presentation and review of the literature.

The paper presents a case of a uterus-like mass (ULM), a rare type of tumour of the female reproductive system, which did not present any clinical symptoms described in other cases of ULMs. There are 35 reported cases of this type of tumour. It is defined as a lesion composed of smooth muscle-like stromal cells with a central cavity lined with endometrial type epithelium. There are three theories on the pathogenesis of ULMs which we discuss along with clinical presentation, diagnostic features, treatment options and potential oncological implications of this type of tumour, based on our case, and the review of the literature.

Comparison of effectiveness of treatment of patients with sporadic and germline BRCA1-related ovarian cancer.

OBJECTIVES: Numerous reports suggest that the clinical course of ovarian cancer (OC) in BRCA, including BRCA1, mutation carriers (BRCA1-OC) is different than in patients with sporadic ovarian cancer (SOC). Most of the authors indicate more fa-vourable treatment results in patients with BRCA1-OC. The aim of the study was to compare the effectiveness of treatment of patients with advanced-stage (FIGO III/IV) SOC and BRCA1-OC. MATERIAL AND METHODS: Between 2004 and 2009, 957 OC patients were treated in Cracow Branch of Cancer Center, M. Sklodowska-Curie Memorial Institute, Poland. Germline BRCA1 mutation was found in 66 patients. To compare the effective-ness of treatment, the group of 47 advanced-stage BRCA1-OC patients was matched with the group of 47 advanced-stage SOC patients. Pairs of patients were matched in terms of the most important prognostic factors, i.e. stages according to FIGO, primary cytoreduction extent, tumour histologic subtype and grade, as well as year of diagnosis and treatment. RESULTS: The 5-year overall survival rate was 42.9% for BRCA1-OC patients and 34.3% for SOC patients (p = 0.354). Mean time to progression was 22.7 and 14.5 months for BRCA1-OC and SOC group, respectively (p = 0.05). Complete response to pri-mary surgery and first line chemotherapy was obtained in 42.5% and 37.9% of cases, respectively; the difference, however, did not reach the statistical significance. CONCLUSIONS: Results of combined treatment in the group of BRCA1-related OC patients seem to be better than in the group of sporadic ovarian cancer patients.

The prognostic value of the post-treatment serum CA 125 level in patients with advanced endometrial cancer.

OBJECTIVES: The goal of this analysis was to assess the prognostic value of the post-treatment serum CA 125 level in each member of a group of advanced endometrial cancer (aEC) patients in comparison to other clinical and pathological parameters. MATERIAL AND METHODS: Records of 266 patients treated at the Maria Sklodowska-Curie Memorial Cancer Centre and Institute of Oncology, Cracow Branch between the years 2006 and 2018 were included in the study. Follow-up ranged from 1 to 138 months. Progression free survival (PFS) and overall survival (OS) were set as the endpoints. The tests chi-squared, Fisher, log-rank, Mann-Whitney, Kruskal-Wallis and Cox proportional hazard ratio were used for statistical analyses. RESULTS: In the analysed group, there was a significant association between an elevated serum CA 125 level following adjuvant treatment and shorter PFS and OS. After setting a cut-off value for CA 125 there was a statistically significant correlation between the marker and PFS and OS. Multivariate analysis indicated that the post-treatment serum CA 125 level is an independent prognostic factor of the course of aEC. CONCLUSIONS: The post-treatment serum CA 125 level correlates significantly with both PFS and OS in each patient with aEC. The marker is an independent prognostic factor in this group. A low post-treatment level of the marker is a strong indicator of good 5-year survival, with 82% of patients reaching 5-year OS.

Prognostic factors in patients with primary invasive vaginal carcinoma.

AIM OF THE STUDY: Aim of the study was the assessment of prognostic factors in the group of primary invasive vaginal carcinoma (PIVC) patients subjected to radical radiation therapy MATERIAL AND METHODS: The analysis was performed for the group of 152 PIVC patients treated with intracavitary brachytherapy alone (16.5%), the combination of brachytherapy and external radiotherapy (78.9%), or external radiotherapy alone (4.6%). The relationship was investigated between treatment outcome and the following demographic, clinical and histopathological features: age, duration of pathological symptoms, number of births given, prior hysterectomy haemoglobin level, Karnofsky performance status score, primary tumour location in vagina, length of vagina involved, FIGO stage, gross appearance, histological type, and tumour grade. RESULTS: Five-year disease-free survival was observed in 46.1% of the patients (70/152). Patients below 60 years of age, with Karnofsky score of 80-90, diagnosed with PIVC in stage 10 or 110, and with tumour of grade G1 or G2 had significantly higher 5-year disease-free survival. Multifactoral analysis showed that age below 60 and FIGO stage 10 and 110 are independent favourable prognostic factors. CONCLUSIONS: The independent prognostic factors in PIVC patients treated with radical radiotherapy are patient age and FIGO stage.

[Hereditary ovarian cancer. The role of BRCA1 gene dysfunction in response to chemotherapy]. / Rodzinny rak jajnika. Rola dysfunkcji genu BRCA1 w odpowiedzi na leczenie chemiczne.

Hereditary ovarian cancer is often believed to be as a distinct disease. It is diagnosed earlier than its sporadic type; serous subtypes and more advanced stages are usually observed. Mutations of genes like BRCA1, BRCA2, MMR (MLH1, MSH2, PMS1, PMS2) are strictly associated with the heredity of ovarian and also breast cancer. Systematic controls and specific procedures to lower the risk of those tumors are required for mutation carriers. Most authors emphasize better prognosis for patients with inherited type of ovarian cancer when comparing to sporadic one. It probably results from dysfunction of BRCA1 gene, inducing better response to platinum-based cytostatic drugs. This phenomenon, called "BRCAness profile", is also observed in non-hereditary ovarian cancers and it arises from somatic mutation or hypermetylation of BRCA1 promoter. Thus, the process of DNA repair is defective. Currently new groups of drugs using the BRCA1 dysfunctions are being introduced into clinical practice.

Perivascular epithelioid tumor (PEComa) of the falciform/ broad ligament.

PEComas localized in the region of falciform ligament and broad ligament are exceedingly rare. Most of them are built of spindle neoplastic cells. We report a case of epithelioid PEComa of the falciform ligament and/or broad ligament. There is only one report of such neoplasm in English-language literature. Histologically, the tumor was composed of nests of epithelioid clear cells stained positively for vimentin, HMB45, and SMA. Because of morphological features of the tumour (4 mitoses /20HPF, focal necrosis, and vascular invasion) we assess the neoplasm as potentially malignant.

[Analysis of treatment outcome in patients with endometrial cancer limited to the uterus]. / Analiza wyników skojarzonego leczenia chorych na niezaawansowanego raka endometrium.

OBJECTIVES: The aim of study was to analyse results of treatment patients with uterine-confined endometrial cancer which underwent surgery and postoperative radiotherapy in Center of Oncology in Kraków between 1985 and 1997. MATERIAL AND METHODS: The research included a group of 650 women. All patients undergo total abdominal hysterectomy with bilateral salpingo-oophorectomy and postoperative radiotherapy. 155 patients with intermediate-risk of recurrence (IA-G3, IB-G1, G2) received postoperative whole pelvic irradiation only. In the group of 495 patients with high-risk of recurrence (IB-G3, IC, II) 210 patients received brachytherapy vaginal cuff only and 285 patients whole pelvic and vaginal cuff irradiation. RESULTS: In the group of patients with intermediate-risk of recurrence five NED survival was 93.5%. In the group of patients with high-risk of recurrence five NED survival was statistically lower in patients treated with brachytherapy vaginal cuff only (83.2% vs. 71.9%). CONCLUSION: In uterine-confined endometrial cancer patients, with intermediate-risk of recurrence treated with surgery and postoperative whole pelvis irradiation, 5-year NED survival is above of 90%. In the group of patients with high-risk of recurrence the adjuvant treatment of choice is whole pelvic and vaginal cuff irradiation.

[Prognostic factors in patients with cervical--confined endometrial carcinoma treated with surgery and postoperative radiotherapy]. / Czynniki prognostyczne w grupie chorych na niezaawansowanego raka trzonu macicy leczonych chirurgicznie z uzupelniajacym napromienianiem.

OBJECTIVE: The aim of the study was to determine prognostic factors in the group of the patients with uterine--confined endometrial carcinoma treated with surgery and postoperative radiotherapy. MATERIAL AND METHODS: The authors analyzed 102 patients (mean age 58 years) with stage 1 (74 patients) and stage 11 (28 patients) endometrial carcinoma. All patients were treated with surgery (abdominal hysterectomy and bilateral adnexectomy) and postoperative radiotherapy: external beam pelvic irradiation (20 patients), vaginal cuff irradiation (33 patients) and combination (49 patients). Analysed were prognostic factors as follows: age, stage, tumor grade, depth of myometrial invasion, hormone receptors, expression of the tumor suppressor gene p53, Her 2/neu and MIB-1 (ki-67 paraffin). RESULTS: In our group of patients, multivariate analysis has identified tumor grade, progesterone receptors status and MIB-1 as independent significant prognostic factors.

[Effectiveness of surgical treatment of patients with stage IA endometrial cancer]. / Ocena skutecznosci leczenia operacyjnego chorych na raka trzonu macicy w stopniu IA zaawansowania.

OBJECTIVES: The aim of the study was to analyse results of surgical treatment patients with stage IA-G1, G2 endometrial cancer treated in Center of Oncology in Kraków between 1985 and 1997. MATERIALS AND METHODS: The research included a group of 44 women. All patients underwent total abdominal hysterectomy and bilateral salpingo-oophorectomy (TAH and BSO) without complementary treatment. RESULTS: Five NED survival was 95.5%. During the 5-year follow-up period 2 patients died, I of myocardial infarction and 1 of cerebral hemorrhage. CONCLUSION: Total abdominal hysterectomy and bilateral salpingo-oophorectomy are the treatment of choice for stage IA-G1, G2 endometrial cancer.