Retroperitoneal sarcoma: a 10-year follow-up analysis using hospital-based cancer registry data in Japan.

OBJECTIVES: We sought clinical characteristics, survival outcomes, and prognostic factors for overall survival of retroperitoneal sarcoma in Japan. METHODS: A Japanese hospital-based cancer registry database with a pivotal 10-year follow-up was used to identify and enroll patients, registered from 106 institutions, diagnosed with retroperitoneal sarcoma in 2008-2009. Treating hospitals were divided by hospital care volume; high-volume hospitals and low-volume hospitals were defined as ≥ 4 and < 4 cases/year, respectively. RESULTS: A total of 91 men and 97 women were included, with a median age of 64 years. The most common histological type was liposarcoma in 101 patients, followed by leiomyosarcoma in 38 patients. The 5-year and 10-year overall survival rates were 44.1 and 28.3%. The majority of patients (n = 152, 80.9%) were treated at low-volume hospitals. High-volume hospital patients had higher 10-year overall survival rates than low-volume hospital patients (51.2% vs 23.2%, P = 0.026). Multivariate analysis revealed age over 60 years, treatment in low-volume hospitals and chemotherapy were independent predictors of unfavorable survival while treatment with surgery was an independent predictor of favorable survival. CONCLUSIONS: The possibility of surgical removal was suggested to be the most important prognostic factor for retroperitoneal sarcoma. Better survival was shown in patients treated at high-volume hospitals in our series.

Incidence of unexpected leiomyosarcoma in a Turkish province: a retrospective multi-centre study in a low income setting.

The aim of the current study was to estimate the incidence of unexpected leiomyosarcoma (LMS) in patients who underwent surgery due to leiomyomas in Konya province, and to contribute to the literature discussing comparisons with similar studies. The digital archives of eight high-volume hospitals were studied for surgeries performed due to leiomyomas between January 2012 and January 2019, and leiomyosarcoma incidence was calculated based on the data obtained. Twenty-one patients in 3703 cases were found to have unexpected leiomyosarcoma, which means we can expect one leiomyosarcoma in 176 (0.56%) surgeries. Six more malignant tumours were detected among the remaining cases. Thus, our study estimated the incidence of unexpected leiomyosarcoma as 1/176 (0.56%), which is higher than most of the studies in the literature justifying the debate started by the FDA in 2014. As the tumour biology is not yet clear, and the incidence of unexpected leiomyosarcoma tends to be so high, the key focus must be to try to detect uterine leiomyosarcomas preoperatively for robust patient care.IMPACT STATEMENTWhat is already known on this subject? The incidence of unexpected leiomyosarcoma varies widely from 1/498 to 1/8300 depending on the study method and the type of procedure, and there is still controversy, even after the FDA statement that led to a major restriction in laparoscopic surgeries due to concerns about inadvertent morcellation of leiomyosarcomas.What do the results of this study add? To the best of our knowledge, the current study found the highest incidence of unexpected leiomyosarcoma, and consequently a serious evaluation of all patients undergoing surgery due to leiomyomas preoperatively considering a leiomyosarcoma candidate is recommended.What are the implications of these findings for clinical practice and/or further research? Studies on tumour biology and novel markers must be supported for accurate preoperative diagnosis of leiomyosarcoma.

Risk of unexpected uterine leiomyosarcoma during laparoscopic procedures: Experience from a single tertiary institute in Italy.

OBJECTIVE: To evaluate the incidence of occult uterine sarcomas and investigate whether an accurate and well-established preoperative assessment for uterine fibroids could help identify uterine sarcomas more effectively. METHODS: A retrospective analysis of patients who underwent gynecological laparoscopic surgery for presumed uterine fibroids at Sant'Anna Hospital, a single tertiary institute in Turin, Italy, between January 2003 and December 2019. RESULTS: Over the 17-year period, 5826 laparoscopic surgical procedures (myomectomies or subtotal/total hysterectomies) were performed for presumed uterine fibroids. A total of 48 patients with a final diagnosis of uterine sarcoma were identified, the majority of which (n = 39; 81.3%) were recognized as suspicious uterine sarcomas during the preoperative assessment, and morcellement was avoided. The occurrence of unexpected uterine sarcomas was 0.1% (6/5826). Morcellation was conducted in one patient with uterine sarcoma. CONCLUSION: Analysis of our data showed that unexpected uterine sarcomas are uncommon. Accurate preoperative evaluation can help avoid, but does not exclude, the possibility of morcellation of unknown uterine sarcomas.

Development, validation, and visualization of a web-based nomogram for predicting the incidence of leiomyosarcoma patients with distant metastasis.

BACKGROUND: Leiomyosarcoma (LMS) is one of the most common soft tissue sarcomas. LMS is prone to distant metastasis (DM), and patients with DM have a poor prognosis. AIM: In this study, we investigated the risk factors of DM in LMS patients and the prognostic factors of LMS patients with DM. METHODS AND RESULTS: LMS patients diagnosed between 2010 and 2016 were extracted from the Surveillance, Epidemiology, and End Result (SEER) database. Patients were randomly divided into the training set and validation set. Univariate and multivariate logistic regression analyses were performed, and a nomogram was established. The area under the curve (AUC), calibration curve, and decision curve analysis (DCA) were used to evaluate the nomogram. Based on the nomogram, a web-based nomogram is established. The univariate and multivariate Cox regression analyses were used to assess the prognostic risk factors of LMS patients with DM. Eventually, 2184 patients diagnosed with LMS were enrolled, randomly divided into the training set (n = 1532, 70.14%) and validation set (n = 652, 29.86%). Race, primary site, grade, T stage, and tumor size were correlated with DM incidence in LMS patients. The AUC of the nomogram is 0.715 in training and 0.713 in the validation set. The calibration curve and DCA results showed that the nomogram performed well in predicting the DM risk. A web-based nomogram was established to predict DM's risk in LMS patients (https://wenn23.shinyapps.io/riskoflmsdm/). Epithelioid LMS, in uterus, older age, giant tumor, multiple organ metastasis, without surgery, and chemotherapy had a poor prognosis. CONCLUSIONS: The established web-based nomogram (https://wenn23.shinyapps.io/riskoflmsdm/) is an accurate and personalized tool to predict the risks of LMS developing DM. Advanced age, larger tumor, multiple organ metastasis, epithelioid type, uterine LMS, no surgery, and no chemotherapy were associated with poor prognosis in LMS patients with DM.

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.

Leiomyosarcoma of the urinary bladder: A SEER database study and comparison to leiomyosarcomas of the uterus and extremities/trunk.

No well-established staging system exists for bladder leiomyosarcoma (LMS), and the current staging system does not include tumor size, a thoroughly validated prognostic parameter for sarcomas. Uterine and extremity/trunk LMS are more common than those in the bladder and have well-established staging systems incorporating tumor size. We aim to improve the understanding of LMS of the urinary bladder by assessing cancer-specific survival (CSS) and comparing LMS at this unusual anatomic site to those arising at other sites using the Surveillance, Epidemiology, and End Results (SEER) database. The SEER database (1973-2013) was queried for bladder, uterus, and trunk/extremity LMS. Multivariable Cox proportional hazard regression was performed to identify predictors of CSS for each anatomic location and used to compare outcomes at different sites. We identified 165 bladder, 4987 uterus, and 2536 extremity/trunk LMS cases. Five-year CSS was 52% for uterus, 73% for bladder, and 82% for extremity/trunk LMS. For LMS at all sites, uterine location (HR = 2.14, P < 0.001) and increasing tumor size (HR = 1.05, P < 0.001) were significant predictors of worse CSS on multivariate analysis. For bladder LMS, increasing tumor size (HR = 1.18, P = 0.003) was an independent prognostic factor and the conventional staging cut-off threshold of 5 cm for sarcomas outside the head/neck showed statistical significance in stratifying patient risk of cancer-related death. Bladder LMS appears to have clinical behavior intermediate between those of the extremities/trunk and uterus. We suggest that the conventional sarcoma staging protocols based on tumor size be applied to LMS of the urinary bladder.

Prognostic factors and survival outcomes of women with uterine leiomyosarcoma: A Turkish Uterine Sarcoma Group Study-003.

To assess the clinicopathological features, prognostic factors, and survival rates associated with uterine leiomyosarcoma (uLMS). Databases from 15 participating gynecological oncology centers in Turkey were searched retrospectively for women who had been treated for stage I-IV uLMS between 1996 and 2018. Of 302 consecutive women with uLMS, there were 234 patients with Federation of Gynecology and Obstetrics (FIGO) stage I disease and 68 with FIGO stage II-IV disease. All patients underwent total hysterectomy. Lymphadenectomy was performed in 161 (54.5%) cases. A total of 195 patients received adjuvant treatment. The 5-year disease-free survival (DFS) and overall survival (OS) rates were 42% and 54%, respectively. Presence of lymphovascular space invasion (LVSI), higher degree of nuclear atypia, and absence of lymphadenectomy were negatively correlated with DFS, while LVSI, mitotic count, higher degree of nuclear atypia, FIGO stage II-IV disease, and suboptimal surgery significantly decreased OS. LVSI and higher degree of nuclear atypia appear to be prognostic indicators for uLMS. Lymphadenectomy seems to have a significant effect on DFS but not on OS.

Prevalence and occult rates of uterine leiomyosarcoma.

This study aimed to assess the prevalence and occult rates of uterine leiomyosarcoma (ULMS) in women with smooth-muscle tumors undergoing gynecological surgery. A retrospective study was performed at an academic cancer center from 2008 to 2015. Patients undergoing either hysterectomy or myomectomy via laparoscopic, abdominal, vaginal, and hysteroscopic approaches were identified with the validated pathology diagnosis of either ULMS or leiomyomas. All patients initially operated at our institute were included and reviewed. The prevalence and occult rates of ULMS were calculated and compared between different age groups.Twenty-eight patients with original ULMS were identified in 9556 gynecological surgeries. The prevalence of overall and occult ULMS in our study was 0.25% (1 in 345 patients) and 0.07% (1 in 1429 patients). The proportion of occult in all ULMSs was 25%. The prevalence rates of overall ULMS were 0.21%, 0.13%, 0.52%, 2.12%, and 6.67% in the 30 to 39, 40 to 49, 50 to 59, 60 to 69, and ≥70-year age groups, respectively. There was a significantly increased risk of ULMS after 50 years of age. The prevalence rates of occult ULMS were 0.05%, 0.08%, and 0.12% for the 30 to 39, 40 to 49, and 50 to 59 year age groups, respectively. There was no statistically significant difference among age the groups. The prevalence of ULMS was 0.41% and 0.16% for solitary and multiple tumor masses, respectively. Patients with solitary uterine tumors were at a significantly increased risk of ULMS (OR = 2.601, 95% CI = 1.108-6.141).Our retrospective data in part reflects the clinical characteristics of overall and occult ULMS and forms the basis for further prevention of occult ULMS.

Preoperative clinical characteristics scoring system for differentiating uterine leiomyosarcoma from fibroid.

BACKGROUND: Morcellation may lead to intraperitoneal spread of tumor cells, thus making prognosis of undiagnosed uterine leiomyosarcoma (ULMS) worse. However, preoperative diagnosis of ULMS remains challenging. This study aimed to design a preoperative clinical characteristics scoring system for differentiating ULMS from uterine fibroid. METHODS: This study enrolled 45 ULMS patients and 180 uterine fibroid patients in Peking Union Medical College Hospital from January 2013 to December 2018. RESULTS: The incidence of occult ULMS was 0.59% (95% CI, 0.39-0.71%). Age ≥ 40 years old (OR 2.826, 95%CI 1.326-5.461), tumor size ≥7 cm (OR 6.930, 95% CI 2.872-16.724), neutrophil-to-lymphocyte ratio (NLR) ≥ 2.8 (OR 3.032, 95%CI 1.288-7.13), number of platelet ≥298 × 109/L (OR 3.688, 95%CI 1.452-9.266) and lactate dehydrogenase (LDH) ≥ 193 U/L (OR 6.479, 95%CI 2.658-15.792) were independent predictors of ULMS. A preoperative clinical characteristics scoring system was designed based on OR values, with a total score of 7 points. Tumor size ≥7 cm, LDH ≥ 193 U/L were assigned 2 points, while age ≥ 40 years old, NLR ≥ 2.8 and number of platelet ≥298 × 109/L were assigned 1 point. Score ≥ 4 points was a useful predictor in diagnosing ULMS from fibroid (sensitivity 0.800, specificity 0.778). CONCLUSIONS: The incidence of occult ULMS was low. Age ≥ 40 years old, tumor size ≥7 cm, LDH ≥ 193 U/L, NLR ≥ 2.8 and number of platelet ≥298 × 109/L were independent predictors of ULMS. The preoperative clinical characteristics scoring system could be helpful in preoperative diagnosis of occult ULMS.

Unexpected uterine smooth muscle tumor of uncertain malignant potential and sarcoma: A single center cohort study in South Korea.

OBJECTIVE: To evaluate the risk of encountering unexpected uterine smooth muscle tumors of uncertain malignant potential (STUMPs) or sarcomas during surgical treatment of mesenchymal tumors of the uterus using morcellation. MATERIAL AND METHODS: Data were collected retrospectively from subjects who were pathologically diagnosed with uterine leiomyoma or its variants, STUMP or other premalignant mesenchymal tumors of uterus, or sarcoma during surgical treatment between July 2014 and June 2017. RESULTS: A total of 3785 women were investigated; 2824 laparoscopic procedures (74.6%) were performed, and an electronic power morcellator was used in 1636 patients (43.2%). Sixteen women (0.42%) were diagnosed with STUMP and 14 (0.37%) were diagnosed with uterine sarcoma. The incidence rate of unexpected STUMP or uterine sarcoma was 0.61% (23 of 3785 women); unexpected STUMP in 13 (0.34%), and unexpected sarcoma was in 10 (0.26%). Moreover, the unexpected leiomyosarcoma rate was 0.08% (3 in 3785). The rate of unintended morcellation of STUMPs was relatively high at 0.26% (10 in 3785), however, that for uterine sarcomas was 0.05% (2 in 3785). CONCLUSION: The risks of unintended morcellation were very low for sarcomas and STUMPs, although the risk of the latter was approximately 5-fold that of the former. To reduce the unintended dissemination of tumors, patients suspected of having malignancies should be provided adequate information regarding their treatment options as well as their associated risks. Meanwhile, improved preoperative screening methods for STUMP and sarcoma should be established.

Clinical status and prognostic factors in Japanese patients with uterine leiomyosarcoma.

OBJECTIVE: Uterine leiomyosarcoma (uLMS) is a rare gynecologic malignancy for which the currently available treatments do not consistently provide long-term disease control. This study aimed to reveal the current clinical status of uLMS to support future clinical trials. METHODS: This study enrolled patients with uLMS treated at 53 Japanese institutions from 2000 to 2012. Central pathological review (CPR) was performed. All cases were confirmed by CPR, and epidemiological features, treatment, and prognosis were analyzed statistically. RESULTS: A total of 307 patients were enrolled. A diagnosis of uLMS was confirmed in 266 patients (86.6%) of patients after CPR, of whom data for 259 were analyzed. Of these, 186 (71.8%) patients underwent complete gross resection as primary therapy. Ninety-eight patients received no additional adjuvant therapy, while docetaxel and gemcitabine was the most frequent regimen among 155 patients treated with adjuvant chemotherapy. In all cases, the median overall survival (OS) was 44.2 months. Multivariate analyses of prognostic factors in all cases identified stage III and IV disease, high serum lactate dehydrogenase level, and menopausal status as poor prognostic factors. However, in stage I cases, high serum lactate dehydrogenase level and no adjuvant treatment were identified as poor prognostic factors. The 5-year OS of patients with stage I uLMS treated with adjuvant chemotherapy was significantly better than that of those without adjuvant treatment (67.8% vs 46.7%, P = 0.0461). CONCLUSIONS: Despite complete removal of the primary lesion, the clinical course of patients with uLMS was poor due to recurrence of distant metastasis. The application of a suitable biomarker and effective adjuvant chemotherapy are required to improve the prognosis of patients with uLMS.

Incidence of Leiomyosarcoma at Surgery for Presumed Uterine Myomas in Different Age Groups.

STUDY OBJECTIVE: To evaluate the incidence of leiomyosarcoma (LMS) at surgery for presumed uterine myomas according to different age groups. DESIGN: A retrospective cohort study. SETTING: A tertiary referral hospital. PATIENTS: All women undergoing surgery for presumed uterine myomas between January 1, 2006, and December 31, 2016. INTERVENTIONS: Laparoscopic myomectomy, laparotomic myomectomy, total hysterectomy, or hysteroscopic myomectomy. MEASUREMENTS AND MAIN RESULTS: A total of 1398 patients underwent surgery for presumed uterine myomas. The incidence of LMS was 2.15 per 1000 surgeries (n = 3, 1/466, 0.2%). In women under 40 years old, the incidence of occult LMS was 0 (0/561). In women between 40 and 49 years old, 190 myomectomies were performed (28% of the surgeries), and the rate of LMS was 1.49 per 1000 (n = 1, 1/673, 0.15%). In women over 49 years old, a total hysterectomy was performed in 82.3% of the cases, and the incidence of LMS was 12.2 per 1000 surgeries (n = 2, 1/82, 1.2%). CONCLUSION: The incidence of occult LMS in patients under 40 years old undergoing surgery for presumed uterine myomas was 0. These findings are suggestive that the use of power morcellation in this population may be safe.

Leiomyosarcoma of the skin: review of the literature with an emphasis on prognosis and management.

Leiomyosarcoma (LMS) of the skin is rare, and no management guideline currently exists. Although LMS is historically classified as either dermal (cutaneous) or subcutaneous, definition for its classfication is inconsistent in the literature. Studies on the managenent of LMS are scarce, and there is no consensus on the appropriate surgical margin for the treatment of LMS. While a 1 cm margin may be sufficient in cutaneous LMS, wider margins may be required for subcutaneous tumors. Mohs micrographic surgery is a promising surgical modality for the treatment of cutaneous LMS. In this review, current knowledge on LMS is summarized and a practical approach to the management of this rare neoplasm is proposed.

Radiotherapy-induced malignancies in breast cancer patients with TP53 pathogenic germline variants (Li-Fraumeni syndrome).

The risk of radiotherapy-induced malignancies (RIMs) is a concern when treating Li-Fraumeni syndrome (LFS) or Li-Fraumeni Like (LFL) patients. However, the type of TP53 pathogenic germline variant may possibly influence this risk. TP53 p.R337H mutation is particularly prevalent in Brazil. We aimed to evaluate the outcomes of patients with pathogenic TP53 variants treated for localized breast cancer in a Brazilian cohort. We evaluated retrospectively a cohort of patients with germline TP53 pathogenic variants treated for localized breast cancer between December 1999 and October 2017. All patients were followed by the Hereditary Cancer Group of an academic cancer center. Our primary objective was to evaluate the occurrence of RIMs after adjuvant radiotherapy. Sixteen patients were evaluated; 10 (62.5%) had a germline TP53 p.R337H pathogenic variant. Median age was 39.8 years. Thirteen patients had invasive ductal carcinoma: 8 (61.5%) were hormone receptor-positive; 6 (46.1%), human epithelial growth factor receptor 2 (HER2)-amplified. Three patients had ductal carcinoma in situ. Most patients (N = 12/16, 75%) received adjuvant radiotherapy. After a median follow-up of 52.5 months, 2 patients (2/12, 16.6%) had RIMs. One had a fibrosarcoma and the other, a low-grade leiomyosarcoma. In the group treated with radiotherapy, one distant recurrence was diagnosed (1/12), and no loco-regional recurrence occurred. Among 4 patients who did not receive radiotherapy, 2 presented with loco-regional recurrence. In this cohort of patients with LFS enriched in TP53 p.R337H pathogenic variant, the incidence of RIMs after treatment of localized breast cancer was lower than previous literature. Nevertheless, rates of RIMs were still alarming. Early molecular diagnosis and careful evaluation of treatment risks and benefits are essential for these patients.

Google searches and medical publication trends since the 2014 US Food and Drug Administration position on power morcellation: Do these translate into patient awareness?

OBJECTIVES: Our primary objective was to assess patient awareness by observing Google Trends comparing search terms used in relation to morcellation rather than morcellation alone. Our secondary objective was to review trends and locations of publications on leiomyosarcoma and morcellation following the US Food and Drug Administration (FDA) position statement. STUDY DESIGN: To assess Internet trends, we obtained the relative search volume (RSV) for each month from 1 January 2004 to 1 March 2019 from Google Trends using the terms 'fibroid', 'morcellation', and 'fibroid cancer'. RSV ranges from 0 to 100 with 100 being peak popularity for the term, and all other monthly search activity relative to the peak. To assess academic trends, we performed a systematic review of published literature discussing fibroid morcellation within the same time period. We used a two-way independent t-test to compare median RSV, and chi-squared test to compare academic output. P < 0.05 was considered statistically significant. RESULTS: Search volume for 'morcellation' peaked during the FDA statement (RSV 0.5 to 2.9, t = 17.5, p < 0.05) but was not sustained. There is an increase in 'fibroid' activity post-FDA statement (RSV 68.8 to 76.3, t = 3.9, p < 0.05). 'Fibroid cancer' remained static throughout (t = 1.5, p = 0.1 and t=-0.5, p = 0.6). Afro-Caribbean countries had the highest RSV for 'fibroid', whereas 'morcellation' RSV was highest in predominantly Western countries. There was a significant increase in the rate of papers published on the subject following the FDA statement (6.8 vs 55.6 papers per year, 95% CI -53.96 to -43.64, p < 0.0001). No academic papers on morcellation were published from countries with the highest RSV for 'fibroid'. CONCLUSION: Our study suggests that interest in uterine fibroids has increased since the FDA statement, but the public are perhaps unaware or not concerned of the consequent potential risk of leiomyosarcoma following morcellation. Countries where fibroid interest is highest are not necessarily those that can offer power morcellation. Further studies are required to address how the Internet influences patient choice and informed consent, and how medical professionals can use it to further educate patients on the risks and benefits of laparoscopic myomectomy and power morcellation.

Rethinking the Issue of Power Morcellation of Uterine Fibroids: Is Morcellation the Real Problem or Is this Another Symptom of Disparity in Healthcare Provision?

Power morcellation remains one of the most significant developments in minimal access surgery over the past decade, allowing many more patients to benefit from the least invasive surgical route. However, its use is not without controversy, particularly with regards to the risks of an undiagnosed leiomyosarcoma. Increased media and, in particular, on-going social media coverage since events in 2014 have only served to intensify the debate, culminating in the Food and Drug Administration essentially 'banning' its use in the USA. Practice however continues to vary and this technique remains widely used in Europe and in particular the UK. The aim of this article was to review the development of power morcellation in gynaecology and the underlying risks, including that of undiagnosed leiomyosarcoma, as well as appraise the evolving literature on patient awareness and informed consent and the wider implications of morcellation restriction.

Evaluation of occult uterine leiomyosarcomas.

OBJECTIVES: To determine the frequency of occult uterine leiomyosarcomas following hysterectomies and myomectomiesperformed for benign reasons at our clinic and to draw comparisons with similar studies in the literature. MATERIAL AND METHODS: All hysterectomies and myomectomies that have been performed for benign reasons at our clinicbetween 2010 and 2017 were retrospectively examined via the hospital's information system and the patients that werefound to have leiomyosarcomas were analysed. The incidence of occult uterine leiomyosarcoma per 1000 surgeries at ourclinic was calculated using the Wilson score interval. RESULTS: A total of 6,173 hysterectomies were performed, and occult uterine leiomyosarcoma was identified in 5 patients. Theincidence of occult uterine leiomyosarcoma was calculated to be 0.08% (95% CI 0.03-0.018%). Only 1 of the 771 patientswho underwent myomectomy was identified with occult uterine leiomyosarcoma, making its incidence in myomectomy0.12% (95% CI 0.02-0.073%). When all the patients are considered, occult uterine leiomyosarcoma was identified in 6 of the6,944 patients, and the general incidence of occult uterine leiomyosarcoma was calculated as 0.08% (95% CI 0.03-0.018%). CONCLUSIONS: In our study, the incidence of occult uterine sarcoma following myomectomy and hysterectomy was foundto be lower than that reported in the literature. The reason for this lower incidence includes not only genetic causes andracial differences but also preoperative imaging, endometrial and cervical sampling that is performed on every patient.

A Systematic Review of the Treatment of Superficial Leiomyosarcoma With Mohs Micrographic Surgery.

BACKGROUND: Wide local excision (WLE) with 2 to 5 cm margins has been conventionally used for the treatment of superficial leiomyosarcoma (LMS). Because margin control is the strongest predictor of clinical recurrence, many dermatologic surgeons have recently recommended Mohs micrographic surgery (MMS) over wide local excision (WLE) as the primary treatment modality. OBJECTIVE: To determine the aggregate rate of local recurrence after treatment of superficial LMS with MMS among the few reports in the literature. METHODS: A systematic literature search using the PubMed/MEDLINE database and the Cochrane Library was performed from inception to June 2017. One case report from our institution was included. RESULTS: A meta-analysis of 14 reports of 48 cases of superficial LMS treated with MMS showed a mean recurrence rate of 2.08% to 6.25% with a mean follow-up period of 1570.9 days, compared to reported recurrence rates of 30% to 50% for WLE. Among these cases there were no reports of distant metastases. CONCLUSION: Treatment of superficial leiomyosarcoma with MMS shows markedly lower rates of recurrence compared to reported rates of recurrence after WLE. Further prospective trials with larger sample sizes are needed to compare both modalities.

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.

Incidence and groups at risk for unexpected uterine leiomyosarcoma: a Dutch nationwide cohort study.

OBJECTIVE: To estimate the risk of uterine leiomyosarcoma in patients undergoing gynecological surgery and also to identify groups at risk for unrecognized uterine leiomyosarcoma. METHODS: A national cohort study was performed evaluating all uterine leiomyosarcoma (ULMS) diagnosed in The Netherlands between January 2000 and September 2015. Cases were identified and supplied by the nationwide network and registry of histo- and cytopathology in The Netherlands (PALGA). Unexpected and expected ULMS were compared. Approval for this study was granted by the Medical Ethics Committee of all participating hospitals and by the review board of PALGA. RESULTS: 262 original cases were included. The overall incidence of ULMS in our study was 0.25% or 1:400 patients. The incidence of unexpected ULMS was 0.12% or 1:865 patients. Preoperatively, a malignancy was unexpected in 46% of the cases and expected in 54%. Abnormal uterine bleeding constituted most of the symptoms. 90% of women underwent abdominal hysterectomy and/or bilateral salpingo-oophorectomy. CONCLUSIONS: Leiomyosarcoma are rare. Women aged 40-50 years with abnormal uterine bleeding are most at risk for unexpected ULMS. In contrast, this risk is low in postmenopausal women. ULMS were highly uncommon in women aged under 40 years.