High incidence of gynecologic sarcomas in Israel-A comparison to European and American reports: Gynecologic Sarcoma in Israel.

OBJECTIVE: Gynecologic Sarcomas are rare, aggressive tumors. The aim of this study was to explore the incidence and outcomes of gynecologic sarcomas in a large national data registry and to compare them with reports from other countries. STUDY DESIGN: Records of gynecologic sarcomas diagnosed in Israel (1980-2014) were extracted from the National Cancer Registry and classified according to International Classification of Diseases for Oncology-3 and characterized according to anatomical site, morphology and demographics. Age-standardized incidence rates and 1, 3, 5 and 10-year relative survival rates were calculated for 3 time periods (1980-1994, 1995-2001 and 2005-2014) according to patient age, stage and years of diagnosis. RESULTS: During 1980-2014, 1271 new gynecologic sarcomas were diagnosed in Israel, with incidence slightly increasing in 1980-2004, to an age-standardized incidence rate of 13 per million women. The most common histologic diagnosis was leiomyosarcoma (48%) and the most common anatomical site was the uterus (89%). The age-standardized incidence rate for uterine sarcoma is higher in Israel (10.55 per million) than in England (7.4 per million) and Germany (5.8 per million) respectively. The 5-year overall survival was significantly poorer in patients >70-years, as compared to younger patients (p<0.001) and in those with leiomyosarcoma compared to endometrial stromal sarcoma (p<0.001). The survival rate of patients with leiomyosarcoma in Israel are comparable to survival rates reported by other studies, although substantially lower regarding endometrial stromal sarcoma. CONCLUSIONS: Uterine leiomyosarcoma was the most common gynecologic sarcoma found in the Israeli, European and American registries. Older patients and those with leiomyosarcoma have the worst prognoses. Histological and anatomical variations in Israel are comparable with global statistics, but the incidence in Israel seems higher than in Europe.

Prevalence, characteristics, and risk factors of occult uterine cancer in presumed benign hysterectomy.

BACKGROUND: Occult uterine cancer at the time of benign hysterectomy poses unique challenges in patient care. There is large variability and uncertainty in estimated risk of occult uterine cancer in the literature and prior research often did not differentiate/include all subtypes. OBJECTIVES: To thoroughly examine the prevalence of occult uterine cancer in a large population-based sample of women undergoing hysterectomy for presumed benign indications and to identify associated risk factors. STUDY DESIGN: Using the New York Statewide Planning and Research Cooperative System database, we identified 229,536 adult women who underwent an inpatient or outpatient hysterectomy for benign indications during the period October 1, 2003 to December 31, 2013 at civilian hospitals and ambulatory surgery centers throughout the state. Diagnosis of corpus uteri cancer within 28 days after the index hysterectomy was determined using linked state cancer registry data. We estimated the prevalence of occult uterine cancer (overall and by subtype) and developed and validated risk prediction models using a random split sample approach. RESULTS: Overall, 0.96% (95% confidence interval: 0.92-1.00%) of the women had occult uterine cancer, including 0.75% (95% confidence interval: 0.71-0.78%) with endometrial carcinoma and 0.22% (95% confidence interval: 0.20-0.23%) with uterine sarcoma. The prevalence of leiomyosarcoma was 0.15% (95% confidence interval: 0.13-0.17%). Seventy-one percent of the endometrial carcinomas and 58.0% of the uterine sarcomas were at localized stage. The risk for occult uterine cancer ranged from 0.10% in women aged 18-29 years to 4.40% in women aged ≥75 years; and varied from 0.14% in women undergoing hysterectomy for endometriosis to 0.62% for uterine fibroids and 8.43% for postmenopausal bleeding. The risk of occult uterine cancer was also significantly associated with race/ethnicity, obesity, comorbidity, and personal history of malignancy. Prediction models incorporating these risk factors had high negative predictive values (99.8% for endometrial carcinoma and 99.9% for uterine sarcoma) and good rule-out accuracy despite low positive predictive value. CONCLUSIONS: In women undergoing hysterectomy for presumed benign indications, 0.96% had unexpected uterine cancer. Patient characteristics such as age, surgical indication, and medical history may help guide risk stratification.

Prognostic factors and survival in 1396 patients with uterine leiomyosarcomas: emphasis on impact of lymphadenectomy and oophorectomy.

BACKGROUND: The objectives of the current study were to determine the prognostic factors associated with disease-specific survival (DSS) and to analyze the role of lymphadenectomy (LND) and oophorectomy in the management of uterine leiomyosarcomas (LMS). METHODS: Data were abstracted from the Surveillance, Epidemiology, and End Results database (1988-2003). Kaplan-Meier and Cox proportional hazards regression models were used for analyses. RESULTS: The median age of the 1396 patients was 52 years. There were 951 patients (68.1%) with International Federation of Gynecology and Obstetrics (FIGO) stage I disease, 43 patients (3.1%) with stage II disease, 99 patients (7.1%) with stage III disease, and 303 patients (21.7%) with stage IV disease. Distribution by tumor grade included 87 patients with grade 1 tumors, 208 with grade 2, and 509 patients with grade 3 tumors. The 5-year DSS rates for patients with stage I, II, III, and IV disease were 75.8%, 60.1%, 44.9%, and 28.7%, respectively. Lymph node metastases were identified in 23 of 348 patients (6.6%) who underwent LND. The 5-year DSS rate was 26% in patients who had positive lymph nodes compared with 64.2% in patients who had negative lymph nodes (P < .001). Of 341 patients aged <50 years with stage I or II disease, 240 (70.4%) underwent oophorectomy. There was no difference in 5-year DSS based on oophorectomy. On multivariate analysis, older age at diagnosis, more recent year of diagnosis, African-American race, higher tumor grade, higher stage of disease, and lack of primary surgical treatment all were associated significantly with worse survival. CONCLUSIONS: Independent predictors of DSS in patients with uterine LMS included age, race, stage, grade, and primary surgery. Oophorectomy was not found to have an independent impact on survival.

Primary intratesticular leiomyosarcoma in a mixed race man: a case report.

Primary leiomyosarcoma of the testis is an extremely rare condition with only a few reported cases in the literature. None has been reported in men of Afro-Caribbean descent. This report describes the first case of a primary leiomyosarcoma in a mixed race man. Several different immunostains were used to confirm the morphological suggestion of a primary intratesticular smooth muscle tumour. Scrotal ultrasound and abdominal computed tomography imaging were used to exclude paratesticular and retroperitoneal pathology. Where reported, primary intratesticular leiomyosarcoma is described as an indolent tumour with the potential for distant metastases. Its occurrence in younger men is usually associated with anabolic steroid abuse. Although the number of reported cases is very few, radical orchidectomy followed by surveillance appears to be the treatment of choice.

Uterine sarcomas in South African black women: a clinicopathologic study with ethnic considerations.

BACKGROUND: There is considerable evidence for a higher incidence of uterine sarcomas in blacks when compared to whites. However, whether this higher incidence is related to differences in clinicopathologic presentation is not known. PATIENTS AND METHODS: We reviewed slides and clinical charts of 81 patients with a primary diagnosis of uterine sarcoma referred between 1991 and 1999 to Kalafong Academic and Pretoria Academic Hospital. After review, 49 cases remained for study. RESULTS: Uterine sarcomas were distributed between leiomyosarcoma (LMS) (39%), carcinosarcoma (CS) (49%) and endometrial stromal sarcoma (ESS) (12%). LMS and ESS tend to present at an earlier age when compared to CS (respectively p < 0.008 and 0.02). Of women with LMS more women are premenopausal when compared to CS (p < 0.009). Lower abdominal pain is more common in LMS (p < 0.009), whereas bleeding is more common in women suffering from CS (p < 0.01). Lymphovascular space involvement and cervical involvement are more common in CS when compared to LMS. In CS, the carcinoma component has most of the metastatic potential. CONCLUSION: Among black South African women different clinicopathologic features for uterine LMS, CS and ESS are observed. We also present genetic and/or hormonal factors possibly contributing to the pathophysiology of uterine sarcomas in blacks.