A randomized controlled trial on the oncologic outcomes of use of the intrauterine manipulator in the treatment of apparent uterine-confined endometrial carcinoma: the MANEC Trial.

BACKGROUND: The intrauterine manipulator used during a hysterectomy for endometrial cancer has been suggested as a reason for worsening oncologic outcomes. However, only a few non-randomized retrospective studies have investigated this association. PRIMARY OBJECTIVES: To compare 4-year recurrence-free survival in the group of patients who undergo hysterectomy using an intrauterine manipulator with that of those who undergo hysterectomy without it. STUDY HYPOTHESIS: Patients with endometrial cancer who undergo laparoscopic hysterectomy performed with an intrauterine manipulator would have a lower recurrence-free survival than patients who undergo laparoscopic hysterectomy without a manipulator. TRIAL DESIGN: Multicenter, parallel arm, open-label, randomized controlled trial. MAJOR INCLUSION/EXCLUSION CRITERIA: Adult women diagnosed with apparently uterine-confined endometrial cancer of any histology are eligible. We exclude women who had synchronous or previous (<5 years) invasive cancer, had a WHO performance score >2, and had inadequate baseline organ function. PRIMARY ENDPOINTS: 4-Year recurrence-free survival defined as any relapse or death related to endometrial cancer or treatment calculated from randomization to the date of the first recurrence-free survival event. SAMPLE SIZE: With an accrual time of 4 years, a minimum follow-up length of 4 years, and a two-sided type I error of 0.05, we need to enroll 515 women per arm to have a statistical power of 80% to reject the null hypothesis (HR for recurrence=1), assuming that patients who undergo hysterectomy with the use of the intrauterine manipulator have a 3-year recurrence rate of 12.5% and without the use of the intrauterine manipulator of 8.5% (HR for recurrence=1.50), and that 5% of patients are lost at follow-up in each arm, with a median time of 24 months. ESTIMATED DATES FOR COMPLETING ACCRUAL AND PRESENTING RESULTS: Accrual completion is expected in 2028, and result presentation in 2032. TRIAL REGISTRATION: ClinicalTrial.gov ID NCT05687084.

The thoracic surgeon: "The icing on the cake" in the treatment of epithelial ovarian cancer.

In patients with ovarian cancer, VATS should be used to exclude or confirm the presence of supradiaphragmatic disease and possibly remove it completely. The simultaneous use of laparoscopy and VATS allows for an accurate selection of patients to be sent, in a very short time, to primary surgery or neoadjuvant chemotherapy. Therefore the thoracic surgeon should be part of the multidisciplinary team in every ovarian cancer center.

Primary extra-uterine and extra-ovarian mullerian adenosarcoma: case report and literature review.

BACKGROUND: Extra-uterine mullerian adenosarcomas have varying biological behaviours depending on the presence of endometriosis or sarcomatous overgrowth. These behaviours manifest according to the tumours' histological characteristics and sites of origin. The best treatment and oncologic outcome have not been clarified because only a few cases of extra-uterine and extra-ovarian adenosarcoma have been described in the literature. Here, we report a case of primary peritoneal adenosarcoma with sarcomatous overgrowth and review all reported cases of adenosarcomas arising outside of the uterus and outside the ovaries to identify the best treatment options and clarify outcomes. CASE PRESENTATION: A 79-year-old woman was referred to our Department with an abdominal mass resembling a fibroid with a haemorrhage. Her gynaecological history was negative. A transvaginal and transabdominal ultrasound examination revealed a multicystic mass resembling an ovarian tumour arising from the pelvis and extending up to the abdomen. At laparotomy a peritoneal mass arising from Douglas peritoneum was resected. The uterus and adnexa appeared normal, and a supra-cervical hysterectomy with bilateral salpingo-oophorectomy was performed. No macroscopic residual disease was present. Final pathology diagnosed a malignant peripheral nerve sheath tumors with divergent differentiation. Four weeks later a new, multicystic mass was found. Due to the progressive poor condition, the patient died four months after diagnosis. Histological slides were reviewed by external expert pathologists and the final diagnosis was of extra-genital adenosarcoma with sarcomatous overgrowth. Furthermore, we also collected and analysed articles written in English regarding extra-uterine and extra-ovarian adenosarcomas published between January 1974 and October 2016. PubMed was used as a database for this search. Clinical and pathological characteristics, treatments and outcomes were assessed. CONCLUSIONS: Only 41 cases has been reported in literature. Previous endometriosis and sarcomatous overgrowth showed an inverse effect on prognosis. Endometriosis was confirmed to have a positive effect on disease free survival Complete surgical resection is the mainstay of treatment. A worldwide registry is urgently required to collect data to standardize treatment and to obtain reliable data on prognosis.

Impact of the Food and Drug Administration Safety Communication on the Use of Power Morcellator in Daily Clinical Practice: An Italian Survey.

STUDY OBJECTIVE: To assess the impact of the Food and Drug Administration (FDA) warning about power morcellation use on the daily clinical practice of Italian gynecologists. DESIGN: Electronic survey mailed to the main gynecologic centers (Canadian Task Force Classification type III). SETTING: Unit of Obstetrics and Gynecology, IRCCS - Arcispedale S. Maria Nuova di Reggio Emilia (Italy). PATIENTS: The study did not include patient data. INTERVENTION: There was no intervention. MEASUREMENTS AND MAIN RESULTS: From 490 surveys sent out, 426 replies were included in the final analysis (return rate = 86.9%). Four hundred of the 426 (93.9%) gynecologists were aware of the FDA warning. One hundred fifty of 302 (49.7%) of experienced gynecologists and 176 of 349 (50.4%) of oncology gynecologists considered laparoscopy the best approach for myomectomy. The FDA communication was considered overly restrictive by experienced and oncology gynecologists who declared that they had no intention of changing their surgical approach. Two hundred fifty of the 426 (58.7%) gynecologists declared that they would change their surgical approach only to prevent legal litigation. CONCLUSION: The FDA warning also affected Italian gynecologists. Particularly, less experienced gynecologists and those without oncologic practice seem to be more interested in avoiding legal litigation rather than a real clinical risk of upstaging an unexpected leiomyosarcoma. Fear of undiagnosed sarcoma could increase the number of laparotomies.

Polymorphisms in cyclooxygenase-2 gene in endometrial cancer patients.

The enzyme cyclooxygenase 2 is an inducible enzyme expressed at sites of inflammation and in a variety of malignant solid tumors such as endometrial cancer (EC). In EC patients, its over-expression is correlated with progressive disease and poor prognosis. The expression is encoded by a polymorphic gene, called PTGS2. The aim of the current study was to test the hypothesis that rs5275 polymorphism of PTGS2 influence the prognosis of EC patients. This paper is a retrospective cohort study. Clinical and pathological data were extrapolated and genotypes were assessed on formalin-fixed and paraffin-embedded non-tumor tissues. A total of 159 type I EC patients were included in the final analysis. Univariate analysis indicated that patients with rs5275 genotype CC have a lower risk to develop a grade (G) 2-3 endometrial cancer. rs5275 effect on EC grading was confirmed by multivariate analysis also after data adjusting for age, BMI, parity, hypertension, and diabetes. Adjusted odds ratio (OR) confirmed that patients with rs5275 genotype CC have a risk 80 % lower (OR = 0.20, P = 0.009) to develop a G2 and/or G3 EC in comparison with patients with TT or TC genotype. Differentiation of the type 1 EC is significantly and independently influenced by rs5275 polymorphism. rs5275 CC patients have a lower risk to present a G2-G3 EC.

HNF1B polymorphism influences the prognosis of endometrial cancer patients: a cohort study.

BACKGROUND: HNF1B (formerly known as TCF2) gene encodes for a transcription factor that regulates gene expression involved in normal mesodermal and endodermal developments. A close association between rs4430796 polymorphism of HNF1B gene and decreased endometrial cancer (EC) risk has been demonstrated. The aim of the current study was to test the hypothesis that rs4430796 polymorphism can influence the prognosis of EC patients. METHODS: Retrospective cohort study. Clinical and pathological data were extrapolated and genotypes were assessed on formalin-fixed and paraffin-embedded non-tumour tissues. The influence of patients' genotype on overall survival and progression free survival were our main outcome measures. RESULTS: A total of 191 EC patients were included in the final analysis. Overall survival differed significantly (P = 0.003) among genotypes. At multivariate analysis, a significant (P < 0.05) effect on overall survival was detected for FIGO stage, and rs4430796 polymorphism of HNF1B gene. After grouping EC patients according to adjuvant treatment, rs4430796 polymorphism resulted significantly (P < 0.001) related to overall survival only in subjects who received radiotherapy plus chemotherapy. A significant (P = 0.014) interaction between rs4430796 polymorphism and chemo-radiotherapy was also detected. Finally, only a trend (P = 0.090) towards significance was observed for rs4430796 polymorphism effect on progression free survival. CONCLUSIONS: rs4430796 polymorphism of HNF1B gene influences independently the prognosis of EC patients with a potential effect on tumor chemo-sensitivity.

Coxalgia? Call the gynecologist.

Cellular angiofibroma is a rare mesenchymal tumor that usually affects the vulvar region. It is commonly asymptomatic and treated with radical excision. We describe the first case of cellular angiofibroma located in the retroperitoneum presenting with coxalgia and laparoscopically treated. At the 6-month follow-up, the patient was coxalgia and disease free. Coxalgia is an unusual symptom for gynecologists; nevertheless, it is possible that several neoplasms may originate in the female pelvis, so any symptom requires careful evaluation. The laparoscopic approach may be effective in removing cellular angiofibroma, avoiding injuries to surrounding structures and unesthetic scarring and leading to a faster recovery.

Conversion in endometrial cancer patients scheduled for laparoscopic staging: a large multicenter analysis: conversions and endometrial cancer.

BACKGROUND: Data on patients with endometrial cancer converted to laparotomy are totally lacking. The aim of the present study was to evaluate surgical and oncological outcomes in patients with endometrial cancer scheduled for laparoscopic staging but converted to laparotomy. METHODS: Data of consecutive patients who had undergone surgery for staging endometrial cancer in seven Italian centers were reviewed. Patients' characteristics and surgical and oncological data were noted and analyzed according to surgery, i.e. laparotomy, laparoscopy, and laparoscopy converted to laparotomy. RESULTS: Seventy-one out of 512 (13.9 %) patients scheduled to laparoscopy were converted to laparotomy for reasons related to anesthesiology [38/71 (53.5 %)] or surgery [33/71 (46.5 %)]. The conversion rate varied among stages [41/460 (8.9 %), 13/27 (48.1 %), 17/25 (68.0 %) in patients with stage I, II, and endometrial cancers, respectively]. Significant (P < 0.05) differences among groups were detected in patients' age, body mass index and previous pelvic surgery, and in the distribution of stages and histotype of endometrial cancers. The Kaplan-Meier procedure showed that the cumulative probability of first recurrence (P = 0.089, 0.590 and 0.084 for stage I, II and III, respectively) and of death (P = 0.108, 0.567 and 0.372 for stage I, II and III, respectively) categorized by stages did not attain statistical significance by log-rank testing after correction for confounding factors. CONCLUSIONS: The surgical and oncological outcomes of converted patients are no different from those of patients staged successfully with laparoscopy or with laparotomy. The conversion to laparotomy should be not considered per se a complication.

Primary tuberculosis of the uterine cervix: keep it in mind.

INTRODUCTION: Primary tuberculosis of the cervix is extremely rare, with only 20 cases reported in the English literature since 1949. Here we report the 21st case--an Italian woman with no risk factors. CASE: A 50-year-old Italian nulliparous woman was referred to our gynecology unit for vaginal discharge. Her past history was negative. Colposcopy revealed an ulcerated lesion raising the suspicion of cervical cancer. A biopsy revealed a tuberculosis granuloma, and acid-fast bacillus was identified with Ziehl-Neelsen stain. As no other site of tuberculosis was found, primary cervical tuberculosis was diagnosed. CONCLUSIONS: Although rare in industrialized countries, cervical tuberculosis may affect even women without risk factors. It should thus be taken into account in the differential diagnosis of cervical cancer.

Gastrointestinal Stromal Tumors (GISTs) Mimicking Primary Ovarian Tumors or Metastasizing to the Ovaries: A Systematic Literature Review.

Background: Gastrointestinal stromal tumors (GISTs) are a rare neoplasm, sometimes mimicking primary ovarian tumors (OTs) and/or metastasizing to the ovaries (M-OT). We performed a systematic literature review (SLR) of OTs and M-OTs, investigating differences in recurrence-free and overall survival. Methods: Our SLR was performed according to PRISMA guidelines, searching in Pubmed, Scopus, and Web of Science databases from inception until 21 April 2024. Results: Overall, 59 OTs (Group 1) and 21 M-OTs (Group 2) were retrieved. The absence of residual disease after surgery was achieved significantly in a higher percentage of patients with Group 1 GISTs (91.5%) compared with Group 2 GISTs (57.1%). Chemotherapy was more frequently administered to Group 2 patients (33% vs. 0%). Recurrence and deaths for disease were significantly more frequent in Group 2 than Group 1 cases (54.5% vs. 6.8%, and 37.5% vs. 9.8%, respectively). Conclusions: GISTs can rarely mimic primary ovarian cancers or even more rarely metastasize to the ovaries. Group 1 GISTs occurred in younger women, were not associated with elevated tumor markers, and had a better prognosis. In contrast, Group 2 GISTs occurred in older women, may exhibit elevated tumor markers, and presented a worse prognosis. However, no significant statistical difference for survival between the two studied groups was detected. Computed tomography scans can define the size of GISTs, which correlate to stage and prognostic risk classes. The gold standard treatment is complete surgical resection, which was achieved in almost all cases of Group 1 GISTs and in half of Group 2. Histopathology and immunohistochemistry are essential for the final diagnosis and guide chemotherapy treatment.

Perceived social support and quality of life in endometrial cancer patients: a longitudinal study.

Objective: This study aimed to assess the influence of medical history, perceived physician-patient communication, and perceived social support on changes in the quality of life (QoL) during the first year of follow-up in patients undergoing surgery for endometrial cancer (EC), the most prevalent gynecological cancer in Western countries, especially in Central and Eastern Europe and North America. Methods: This prospective longitudinal study included 98 EC patients. All participants completed the Short Form 36 (SF-36) and the Multidimensional Scale of Perceived Social Support (MSPSS) one month and one year after surgery. Additionally, one month after surgery, they responded to a questionnaire designed by the researchers concerning the key aspects of physician-patient communication. Results: Our findings revealed that patients reporting high social support one month after surgery demonstrated significantly improved emotional well-being (EWB) at both one month and one year after the surgery, with statistically significant higher scores in the dimension of EWB (p<0.05). The support from a significant other at one year correlates with greater PF (p<0.005), fewer limitations due to physical health (p<0.05), less pain (p<0.05), less fatigue (p<0.05), and better general and EWB (p<0.05). Conclusion: This study underscores the significance of perceived social support for patients cross endometrial cancer. The multifaceted nature of social support, encompassing emotional assistance and information sharing, emerges as a pivotal factor aiding patients in confronting the challenges inherent to EC. This form of support contributes to bolstering psychological well-being and enhancing overall QoL.

ABO/Rh Blood Group and Cervical Cancer Survival: Results from Our Own and Other Studies.

Background: Cervical cancer is the most common genital cancer worldwide and is mainly caused by a persistent human papillomavirus infection. Well-known prognostic factors are age, histology, stage, stromal invasion, tumor size, and tumor grade. The relationship between the ABO and Rh system with cervical cancer has been studied since the 1950s, though without obtaining clear results. Here we investigated the association between the ABO blood group and Rh system and consecutively treated cervical cancer patients in our department. Methods: Clinical charts of cervical cancer patients treated and followed from 2010 to 2021 were checked for inclusion and exclusion criteria. Clinical and pathological data were recorded in a separate, anonymous, password-protected electronic database. All relevant data were extrapolated and used for final analysis. Results: A population of 143 cervical cancer patients was analyzed in this study. 47.6% (68/143) were blood group O, 36.4% (52/143) were blood group A, 8.4% (12/143) were blood group AB, and 7.7% (11/143) were blood group B. 14.9% (21/141) were RhD negative, while 85.1% (120/141) were RhD positive. No significant association was found between the ABO group and survival. However, patients with blood types B and AB had a higher BMI than the other blood types. RhD-negative patients exhibited a lower age at diagnosis (P=0.035) and had a higher overall survival compared to RhD-positive patients. Conclusions: The RhD factor appears to influence cervical cancer OS, but the data are too weakly significant to draw a definitive conclusion. Further studies with larger samples are needed to confirm this finding and to investigate the true impact of blood groups in female cancers.

Trends in cervical cancer: A decade­long analysis of incidence, survival and demographic disparities in a Northern Italian province.

While cervical cancer is relatively uncommon in Western countries, it continues to pose significant concern due to its high mortality rates. Intraepithelial forms [cervical intraepithelial neoplasia (CIN) 2 and CIN 3] are more prevalent in regions with comprehensive screening coverage, particularly in areas that use the Papanicolaou test and HPV detection. The present study aims to characterize the trends of precancerous lesions and infiltrating tumors over a decade, assessing survival rates by age, stage and country of origin. A total of 1,752 CIN 2/3 lesions and 152 infiltrating cancers were recorded in the Reggio Emilia Cancer Registry between January 2008 and December 2018. A clear increase in CIN 2/3 lesions was observed over the years while infiltrating tumors remained rare and the numbers of cancer cases declined. The 5-year survival rate was found to be 67%, with survival closely correlated with stage (88, 82, 74 and 12% for stages I, II, III and IV, respectively) and age (84, 80 and 37% for ages <40, 40-65 and 65+, respectively). Multivariable analysis confirmed an excess risk for advanced stages III (HR 3.1; 95% CI, 1.3-7.3) and IV (HR 19.6; 95% CI, 8.2-46.7), as well as in women 65+ (HR 2.8; 95% CI, 1.0-8.1). Analysis of national origins revealed comparable stage distribution for Italians and non-Italians at stage I (41 vs. 47%), while a notable disparity emerged for stage IV (28 vs. 6%). This difference could be attributed to the average age of Italians compared to non-Italians (59 vs. 49 years). Overall, the present study underscored the importance of continued surveillance and intervention strategies targeting advanced-stage disease, particularly in older populations and among non-Italian patients. Efforts to enhance screening coverage and access to timely interventions remain critical in reducing the burden of cervical cancer and improving survival outcomes.

Primary neuroendocrine neoplasms of the vulva: A review of the MITO rare cancer group.

Gynecological neuroendocrine neoplasms are rare entities and can be divided into two groups: carcinoids and neuroendocrine carcinomas. Due to their rarity their management is not standardized. The aim of this work is to summarize and discuss the current literature evidence on this pathology. A scoping literature review was performed in multiple databases. Thirty-one studies were included: 30 case reports and one case series. Patients' age ranged between 28 and 92 years. Surgery was the most used treatment and the surgical approach included local excision (N = 16/31; 51.6%) with (N = 5/16; 31.25%) or without (N = 11/16; 68.75%) inguinal lymphadenectomy. Adjuvant radiotherapy was delivered in 12 (38.7%) cases; instead, platinum-based therapies were frequently used when chemotherapy was chosen for adjuvant treatment. The overall survival ranged between 20 days to 4 years. However, further research is needed; currently, multimodal approach including surgery, chemotherapy and radiotherapy appeared safe and feasible for the treatment of these rare and aggressive diseases.

A multicentric trial (Olympia-MITO 13) on the accuracy of laparoscopy to assess peritoneal spread in ovarian cancer.

OBJECTIVE: The objective of the study was to prospectively evaluate the accuracy of laparoscopy performed in satellite centers (SCs) to describe intraabdominal diffusion of advanced ovarian cancer (AOC). STUDY DESIGN: Patients with a clinical/radiological suspicion of AOC were included in the protocol. SCs were selected among those surgeons, spending a short intensive training period at the coordinator center (CC) to learn the application of staging laparoscopy (S-LPS) in AOC. All women underwent S-LPS at the SCs, and the surgical procedure was recorded and blindly reviewed at the CC. Calculating specificity, positive and negative predictive values, and the accuracy for each parameter with respect to the CC assessed the diagnostic performance of S-LPS. The Cohen's kappa was used to test the interobserver agreement of each parameter. RESULTS: One hundred sixty-eight cases were considered eligible for the study. A per-protocol analysis was performed on 120 cases. The worst laparoscopic assessable feature was mesenteric retraction, whereas the remaining variables ranged from 99.2% (peritoneal carcinomatosis) to 90% (bowel infiltration). All but 1 SC (SC number 4) reached an accuracy rate of 80% or greater for both single parameters and overall score. The Cohen's kappa and the P value for overall predicitive index value were 0.685 and .01, respectively, but improved to 0.773 and .388 after removing the SC number 4 from the analysis. CONCLUSION: S-LPS allows an accurate and reliable assessment of intraperitoneal diffusion of disease in AOC patients in trained gynecological oncology centers.

Gonadotropin releasing hormone agonist and levonorgestrel-intrauterine device followed by in vitro fertilization program as management strategy for an infertile endometrial cancer patient: a case report.

BACKGROUND: A progressive delay in the age of first conception results in an increased frequency of endometrial cancer patients in reproductive age and desiring childbearing. CASE: A 38-year-old infertile woman with stage I endometrioid adenocarcinoma was treated with gonadotropin releasing hormone agonist (GnRHa) and levonorgestrel-releasing intrauterine device (LNG-IUD). After disease remission, she underwent a controlled ovarian stimulation for standard in vitro fertilization (IVF) program and had a pregnancy delivering a healthy male baby. Total laparoscopic hysterectomy with bilateral salpingo-oophorectomy was performed four months after delivery. The patient is free of disease after 3-year follow-up. CONCLUSION: GnRHa plus LNG-IUD followed by IVF program is a safe and effective fertility-sparing strategy to manage infertile patients with stage I endometrial cancer.

The role of minimally invasive surgery in epithelial ovarian cancer treatment: a narrative review.

Objectives: The aim of this narrative review is to summarize the available evidence on the use of minimal invasive surgery (MIS) in the management of epithelial ovarian cancer (EOC). Background: MIS is currently performed to stage and treat EOC at different stage of presentation. We will evaluate risks and benefits of minimally invasive surgery for early stage EOC treatment, then potential advantages provided by staging laparoscopy in identifying patients suitable for primary cytoreductive surgery (PDS) will be discussed. Finally we will investigate the growing role of MIS in the treatment of advanced EOC after neoadjuvant chemotherapy (NACT) and in the treatment of EOC recurrence. Methods: An electronic database search was performed on PubMed, Medline, and Google Scholar for relevant studies up to December 2022. Conclusion: LPS represents a feasible surgical procedure for the staging and treatment in early, advanced and EOC relapse in selected patients treated in high-volume oncological centers by surgeons with adequate experience in advanced surgical procedures. Despite the increasing use of MIS over the last few years, randomized clinical trials are still needed to prove its effectiveness.

An Innovative Drug Repurposing Approach to Restrain Endometrial Cancer Metastatization.

BACKGROUND: Endometrial cancer (EC) is the most common gynecologic tumor and the world's fourth most common cancer in women. Most patients respond to first-line treatments and have a low risk of recurrence, but refractory patients, and those with metastatic cancer at diagnosis, remain with no treatment options. Drug repurposing aims to discover new clinical indications for existing drugs with known safety profiles. It provides ready-to-use new therapeutic options for highly aggressive tumors for which standard protocols are ineffective, such as high-risk EC. METHODS: Here, we aimed at defining new therapeutic opportunities for high-risk EC using an innovative and integrated computational drug repurposing approach. RESULTS: We compared gene-expression profiles, from publicly available databases, of metastatic and non-metastatic EC patients being metastatization the most severe feature of EC aggressiveness. A comprehensive analysis of transcriptomic data through a two-arm approach was applied to obtain a robust prediction of drug candidates. CONCLUSIONS: Some of the identified therapeutic agents are already successfully used in clinical practice to treat other types of tumors. This highlights the potential to repurpose them for EC and, therefore, the reliability of the proposed approach.

Primary Ovarian Leiomyosarcoma Is a Very Rare Entity: A Narrative Review of the Literature.

BACKGROUND: Primary ovarian leiomyosarcoma is a very rare malignancy characterized by unclear management and poor survival. We reviewed all the cases of primary ovarian leiomyosarcoma to identify prognostic factors and the best treatment. METHODS: We collected and analyzed the articles published in the English literature regarding primary ovarian leiomyosarcoma from January 1951 to September 2022, using PubMed research. Clinical and pathological characteristics, different treatments and outcomes were analyzed. RESULTS: 113 cases of primary ovarian leiomyosarcoma were included. Most patients received surgical resection, associated with lymphadenectomy in 12.5% of cases. About 40% of patients received chemotherapy. Follow-up information was available for 100/113 (88.5%) patients. Stage and mitotic count were confirmed to affect survival, and lymphadenectomy and chemotherapy were associated with a better survival rate. A total of 43.4% of patients relapsed, and their mean disease-free survival was 12.5 months. CONCLUSIONS: Primary ovarian leiomyosarcomas are more common in women in their 50s (mean age 53 years). Most of them are at an early stage at presentation. Advanced stage and mitotic count showed a detrimental effect on survival. Surgical excision associated with lymphadenectomy and chemotherapy are associated with increased survival. An international registry could help collect clear and reliable data to standardize the diagnosis and treatment.

Clinical Review of Mucosal Melanoma: The 11-Year Experience of a Referral Center.

INTRODUCTION: Mucosal melanoma is a rare neoplasm. Late diagnosis is caused by occult anatomic sites and scarcity of symptoms. Novel biological therapies have now become available. Demographic, therapeutical and survival records on mucosal melanoma are scarce. OBJECTIVES: To provide an 11-year retrospective clinical review of real-world data on mucosal melanomas managed in a tertiary referral center in Italy. METHODS: We included patients with histopathological mucosal melanoma diagnoses from January 2011 to December 2021. Data were collected until the last known follow-up or death. Survival analysis was performed. RESULTS: Among 33 patients, we found 9 sinonasal, 13 anorectal and 11 urogenital mucosal melanomas (median age 82, females 66.7%). Eighteen cases (54.5%) presented with metastasis (p<0.05). In the urogenital subgroup, only 4 patients (36.4%) had metastasis at diagnosis, all in regional lymph nodes. Sinonasal melanomas were surgically managed with a debulking procedure (44.4%); every case of anorectal and urogenital melanomas underwent radical surgery (30.8% and 45.5%). Fifteen patients were treated with biological therapy (p<0.05). Radiation therapy was used in all melanomas of the sinonasal region (p<0.05). Overall survival was longer for urogenital melanomas (26 months). Univariate analysis showed an increased hazard ratio for death in patients with metastasis. A negative prognostic value of metastatic status was reported by the multivariate model, while administration of first-line immunotherapy demonstrated a protective role. CONCLUSIONS: At diagnosis, the absence of metastatic disease is the most relevant factor that influences the survival of mucosal melanomas. Moreover, the use of immunotherapy might prolong the survival of metastatic mucosal melanoma patients.