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Frontiers in Oncology 2024The purpose of the study was to analyse the role of prognostic factors on the risk of recurrence and overall survival of patients with uterine adenosarcoma.
OBJECTIVE
The purpose of the study was to analyse the role of prognostic factors on the risk of recurrence and overall survival of patients with uterine adenosarcoma.
METHODS
A retrospective international multicentre study involving 46 centres collected 32 cases of uterine adenosarcoma, and these cases were included in the present subanalysis. Clinical and demographic features and tumour characteristics were gathered, as well as information on treatment and relapse. Disease-free and overall survival were analysed.
RESULTS
The 5-year disease-free survival (DFS) was 85.3% and the 5-year overall survival (OS) rate was 89.5%. The risk factors significantly associated with overall survival were age (HR 1.09, 95% CI 1.03-1.15; = 0.004) and FIGO stage II-III (HR 17.75, 95% CI 2.87-109.93; = 0.002). Patients who experienced early relapse (within 12 months) had a tumour size >30 mm and advanced stage. The majority of recurred cases were treated with radiotherapy or surgery and obtained a good response rate.
CONCLUSION
The most significant prognostic factors in uterine adenosarcoma were age and FIGO stage and, indirectly, tumour size at diagnosis. The use of secondary surgery and/or radiotherapy could help in prolonging the disease-free status of the patients.
PubMed: 38835369
DOI: 10.3389/fonc.2024.1393707 -
BMC Cancer Oct 2022Uterine sarcomas are rare and aggressive gynaecologic malignancies, characterized by a relatively high recurrence rate and poor prognosis. The aim of this study was to... (Review)
Review
BACKGROUND
Uterine sarcomas are rare and aggressive gynaecologic malignancies, characterized by a relatively high recurrence rate and poor prognosis. The aim of this study was to investigate the clinicopathological features and explore the prognostic factors of these malignancies.
METHODS
This was a single-institution, retrospective study. We reviewed the medical records of 155 patients with pathologically confirmed uterine sarcomas including uterine leiomyosarcoma (ULMS), low-grade endometrial stromal sarcoma (LG-ESS), high-grade endometrial stromal sarcoma (HG-ESS), undifferentiated uterine sarcoma (UUS) and adenosarcoma (AS) between 2006 and 2022. A total of 112 patients who underwent surgery between January 2006 and April 2019 were included in the survival analysis. The current study recorded the clinicopathological, treatment and outcome data to determine clinical characteristics and survival.
RESULTS
The most common histopathological type was ULMS (63/155, 40.64%), followed by LG-ESS (56/155, 36.13%) and HG-ESS (16/155, 10.32%). The mean age at diagnosis of all patients was 49.27±48.50 years and 32.90% (51/155) of patients were postmenopausal. Fifteen patients underwent fast-frozen sectioning, 63(54.78%) were diagnosed with malignancy, 29(25.22%) were highly suspected of malignancy that needed further clarification and 23(14.84%) were diagnosed with benign disease. A total of 124(80%) patients underwent total hysterectomy (TH) and salpingo-oophorectomy. Multivariate analyses showed that histological type and tumour size were independent prognostic factors both for overall survival (OS) (p<0.001 and P=0.017, respectively) and progression-free survival (PFS) (p<0.001 and P=0.018, respectively). Tumour stage was only significantly associated with PFS (P=0.002). Elevated preoperative NLR, PLR and postmenopausal status were significantly correlated with shorter PFS and OS in univariate analysis, but no statistically significant difference was found in multivariate analysis.
CONCLUSIONS
In patients with uterine sarcoma, in comparison to LMS and LG-ESS, UUS and HG-ESS tend to present as more aggressive tumour with poorer outcomes. Furthermore, larger tumour (>7.5 cm) were an important predictor of shorter PFS and OS.
Topics: Endometrial Neoplasms; Endometrial Stromal Tumors; Female; Humans; Leiomyosarcoma; Pelvic Neoplasms; Prognosis; Retrospective Studies; Sarcoma; Sarcoma, Endometrial Stromal; Soft Tissue Neoplasms; Uterine Neoplasms
PubMed: 36207687
DOI: 10.1186/s12885-022-10129-x -
BMC Cancer Feb 2018Extra-uterine mullerian adenosarcomas have varying biological behaviours depending on the presence of endometriosis or sarcomatous overgrowth. These behaviours manifest... (Review)
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.
Topics: Adenosarcoma; Aged; Female; Humans; Leiomyoma; Mixed Tumor, Mullerian; Ovarian Neoplasms; Uterine Neoplasms
PubMed: 29402239
DOI: 10.1186/s12885-018-4037-y -
The Oncologist Sep 2019The management of adenosarcoma is based on the limited available data. This study aimed to explore the characteristics and oncologic outcomes of patients with uterine...
BACKGROUND
The management of adenosarcoma is based on the limited available data. This study aimed to explore the characteristics and oncologic outcomes of patients with uterine and cervical adenosarcoma.
MATERIALS AND METHODS
A total of 21 and 32 cases of cervical and uterine adenosarcoma, respectively, were retrospectively reviewed in Peking Union Medical College Hospital between April 2006 and March 2019.
RESULTS
The median follow-up time was 37.5 months (range, 1-153 months). The disease progression rate (DPR) was significantly higher in patients with uterine adenosarcoma compared with those with cervical adenosarcoma (28.1% vs. 4.8%). The curve of progression-free survival significantly differed. For those with cervical adenosarcoma, the presence of a tumor stalk was a protective factor, whereas heterologous elements (HE) presented a risk factor for disease progression. For those with uterine adenosarcoma, the presence of a tumor stalk was an independent protective factor, whereas lymphovascular space invasion (LVSI) was an independent risk factor for disease progression. Moreover, HE was an independent risk factor for mortality. Fertility-sparing surgery (FSS) was performed in four and five patients with cervical and uterine adenosarcoma, respectively. Regarding FSS, combined with cases in previous studies, the DPR of patients with uterine adenosarcoma was relatively higher compared with those with cervical adenosarcoma.
CONCLUSION
We found that cervical adenosarcoma had a better prognosis than uterine adenosarcoma. The presence of a tumor stalk was a protective factor, whereas HE and LVSI were risk factors for prognosis. For those with uterine adenosarcoma, if FSS was administered, robust evaluation would be necessary. The small sample size limits the ability to make any strong conclusions about FSS.
IMPLICATIONS FOR PRACTICE
Uterine cervical adenosarcoma had a better prognosis than uterine adenosarcoma. For patients with cervical adenosarcoma, the presence of a tumor stalk was a protective factor and the presence of heterologous elements (HE) was a risk factor for disease progression. For those with uterine adenosarcoma, the presence of a tumor stalk was a protective factor and lymphovascular space invasion was a risk factor for disease progression. Moreover, HE was a risk factor for mortality. Regarding fertility-sparing surgery (FSS), the disease progression rate was higher in patients with uterine adenosarcoma compared with those with cervical adenosarcoma. For patients with uterine adenosarcoma, if FSS was administered, hysteroscopy and robust imaging evaluation would be necessary.
Topics: Adenosarcoma; Adolescent; Adult; Aged; Disease Progression; Female; Fertility Preservation; Humans; Hysteroscopy; Middle Aged; Neoplasm Recurrence, Local; Neoplasm Staging; Prognosis; Progression-Free Survival; Retrospective Studies; Risk Factors; Uterine Cervical Neoplasms; Uterine Neoplasms; Young Adult
PubMed: 31127022
DOI: 10.1634/theoncologist.2018-0791 -
Medicine Sep 2022The objective for the study was to analysis the epidemiology of adenosarcoma, and independent prognostic factors and impact of lymph node dissection (LND) of uterine...
The objective for the study was to analysis the epidemiology of adenosarcoma, and independent prognostic factors and impact of lymph node dissection (LND) of uterine adenosarcoma. Cases of patients with primary adenosarcoma were obtained from the Surveillance, Epidemiology, and End Results (SEER) database from 2000 to 2016. Overall survival was analyzed by the Kaplan-Meier method and log-rank test. The differences in baseline covariates between the 2 groups were adjusted by inverse probability of treatment weighting method. The prognostic factors were identified by univariate and multivariate Cox regression analysis and hazard ratio and 95% confidence interval (CI) of covariates were also estimated. 1129 patients with pathological primary adenosarcoma between 2000 and 2016 were identified from the surveillance, epidemiology, and end results database. The only 4 patients were male. 1027 patients with primary uterine adenosarcoma, and 53.1% underwent LND and only 3.5% patients were with positive lymph node. Age, marital status, largest tumor size, tumor grade, T stage and chemotherapy were significantly correlated with survival. Race, tumor number, LND, and radiotherapy did not affect overall survival in patients. Inverse probability of treatment weighting-adjusted K-M curve showed that LND did not improve survival and lymph node metastasis (LNM) did not affect survival. The majority of primary adenosarcoma patients are female with high incidence of uterus and rare incidence of distant metastasis. Age, marital status, tumor size, T stage, grade, and chemotherapy are independent prognostic factors of uterine adenosarcoma. LNM was not a significant prognostic risk factor, and LND did not benefit survival.
Topics: Adenosarcoma; Female; Humans; Lymph Node Excision; Lymph Nodes; Lymphatic Metastasis; Male; Neoplasm Staging; Risk Factors; Survival Analysis; Uterine Neoplasms
PubMed: 36197202
DOI: 10.1097/MD.0000000000030607 -
American Society of Clinical Oncology... Apr 2022Uterine sarcomas reflect the diversity of sarcoma as a whole. The most common histologies include leiomyosarcoma, high- and low-grade endometrial stromal sarcoma, and...
Uterine sarcomas reflect the diversity of sarcoma as a whole. The most common histologies include leiomyosarcoma, high- and low-grade endometrial stromal sarcoma, and adenosarcoma. These are clinically and biologically heterogeneous diseases that are challenging to treat in the advanced setting. Recent advances in our understanding of the cancer biology of uterine sarcomas has improved diagnostic evaluation and therapeutic management. Promising approaches for patients with advanced uterine leiomyosarcoma include targeting DNA damage repair pathways and depleting immunosuppressive macrophage populations. A subset of endometrial stromal sarcomas harbor potentially actionable alterations in the Wnt, cyclin D-CDK4/6-Rb, and MDM2-p53 pathways. There remains an urgent need to translate molecular findings into prospective clinical trials of novel agents for patients with these diseases; progress will depend on academic collaborations and enrollment of patients with uterine sarcoma in biomarker-driven basket studies.
Topics: Endometrial Neoplasms; Female; Humans; Leiomyosarcoma; Prospective Studies; Sarcoma; Soft Tissue Neoplasms; Uterine Neoplasms
PubMed: 35714303
DOI: 10.1200/EDBK_350541 -
Journal of Cancer 2019Our study aimed to assess factors associated with progression free survival (PFS) and overall survival (OS) in low grade endometrial stromal sarcoma (LG-ESS) and...
Our study aimed to assess factors associated with progression free survival (PFS) and overall survival (OS) in low grade endometrial stromal sarcoma (LG-ESS) and uterine adenosarcoma, and to determine the differences in clinical manifestations and outcomes between the two diseases. A total of 132 patients were enrolled in this retrospective study at Peking Union Medical College Hospital from 1998 to 2016. The associations of clinical and pathological factors with PFS and OS were evaluated. Of the 132 included patients, 104 had LG-ESS and 28 had uterine adenosarcoma. All patients were followed up for at least 1.5 years. There were significant differences between LG-ESS and uterine adenosarcoma in terms of age distribution (41.05±10.5 vs 46.11±14.96 years, P=0.042), delivery time (nulliparity=0: 18.27% vs 35.71%, P=0.046), history of the uterine leiomyoma (65.38% vs 39.29%, P=0.012), and polypoid tumor growth (14.42% vs 60.71%, P=0.007). According to the pathological findings, the proportion of uterine adenosarcoma patients with uterine leiomyoma (60.71%) was significantly higher than that for the LG-ESS patients (32.69%) (P=0.007). Uterine adenosarcoma seemed to be associated with longer PFS and OS than LG-ESS (PFS: 42.69±29.94 vs 50.50±40.50 months; OS: 58.72±37.29 vs 69.46±47.58 months), but the differences were not statistically significant. Multivariate Cox regression showed that age, menopause, history of uterine leiomyoma, stage, and hormone therapy were independent risk factors with respect to PFS, whereas age and stage were risk factors affecting OS in LG-ESS patient. Peritoneal lavage cytology and radiotherapy were risk factors affecting PFS and peritoneal lavage cytology for OS in patients with uterine adenosarcoma. The patients with advanced LG-ESS had poor prognosis. Age and history of uterine leiomyoma were associated with poor PFS, while menopause and hormone therapy were protective factors associated with improved PFS in patients with LG-ESS. Peritoneal lavage cytology and radiotherapy did not improve prognosis of uterine adenosarcoma.
PubMed: 31293638
DOI: 10.7150/jca.30691 -
Clinics and Practice Nov 2021Sarcomas of the uterine corpus are rare malignant neoplasms, which are further classified into mesenchymal tumors, and mixed (epithelial plus mesenchymal) tumors. The... (Review)
Review
Sarcomas of the uterine corpus are rare malignant neoplasms, which are further classified into mesenchymal tumors, and mixed (epithelial plus mesenchymal) tumors. The main issues concerning these neoplasms are the small number of clinical trials, insufficient data from evidence-based medicine, insignificant interest from the pharmaceutical industry, all of which close a vicious circle. The low frequency of these malignancies implies insufficient experience in the diagnosis, hence incomplete surgical and complex treatment. Additionally, the rarity of these sarcomas makes it very difficult to develop clinical practice guidelines. Preoperative diagnosis, neoadjuvant and adjuvant chemoradiation, target and hormone therapies still raise many controversies. Disagreements about the role and type of surgical treatment are also often observed in medical literature. There are still insufficient data about the role of pelvic lymph node dissection and fertility-sparing surgery. Pathologists' experience is of paramount importance for an accurate diagnosis. Additionally, genetics examinations become part of diagnosis in some sarcomas of the uterine corpus. Some gene mutations observed in uterine sarcomas are associated with different outcomes. Therefore, a development of molecular classification of uterine sarcomas should be considered in the future. In this review, we focus on the epidemiology, pathogenesis, pathology, diagnosis and treatment of the following sarcomas of the uterine corpus: leiomyosarcoma, low- and high-grade endometrial stromal sarcomas, undifferentiated sarcoma and adenosarcoma. Uterine carcinosarcomas are excluded as they represent an epithelial tumor rather than a true sarcoma.
PubMed: 34842646
DOI: 10.3390/clinpract11040103 -
Turk Patoloji Dergisi 2023Uterine adenosarcoma has low malignant potential, except in cases with sarcomatous overgrowth (SOG) and a high-grade morphology. We here point out the prognostic...
OBJECTIVE
Uterine adenosarcoma has low malignant potential, except in cases with sarcomatous overgrowth (SOG) and a high-grade morphology. We here point out the prognostic clinicopathological and immunohistochemical features as well as the microsatellite instability (MSI) status of high- and low-grade adenosarcomas.
MATERIAL AND METHOD
In this study, DNA mismatch repair proteins, p16, cyclin D1, ER, PR, and CD10 were examined in uterine adenosarcoma cases using immunohistochemistry. The association between these proteins and clinicopathological parameters was also evaluated.
RESULTS
ER, PR and CD10 expressions were lower and weaker in high-grade adenosarcomas with SOG compared to low-grade adenosarcomas without SOG (p < 0.05). p16 positivity was more frequent in high-grade adenosarcomas than low-grade adenosarcomas (p < 0.05). There was no statistically significant difference between cyclin D1 positivity, MSI, and other clinicopathological parameters (p ≥ 0.05). Cyclin D1 positivity and loss of CD10 expression were associated with shorter disease-free survival (DFS). Loss of ER and CD10 expression was associated with shorter overall survival (OS) (p < 0.05). MSI was not associated with DFS or OS (p ≥ 0.05).
CONCLUSION
These results suggested that p16 positivity, and loss of ER, PR, and CD10 expression were predictors of high-grade morphology. Additionally, the current study showed that cyclin D1-positive tumors had high recurrence rates; however, no significant relationships were found between MSI and DFS or OS in patients with uterine adenosarcoma. Further investigations are required to determine the importance of p16, cyclin D1, and MSI in uterine adenosarcomas.
Topics: Female; Humans; Adenosarcoma; Cyclin D1; Microsatellite Instability; Uterine Neoplasms; Sarcoma
PubMed: 35876683
DOI: 10.5146/tjpath.2022.01580 -
Therapeutic Advances in Medical Oncology 2023Uterine sarcomas are very infrequent and heterogeneous entities. Due to its rarity, pathological diagnosis, surgical management, and systemic treatment are challenging.... (Review)
Review
Uterine sarcomas are very infrequent and heterogeneous entities. Due to its rarity, pathological diagnosis, surgical management, and systemic treatment are challenging. Treatment decision process in these tumors should be taken in a multidisciplinary tumor board. Available evidence is low and, in many cases, based on case series or clinical trials in which these tumors have been included with other soft tissue sarcoma. In these guidelines, we have tried to summarize the most relevant evidence in the diagnosis, staging, pathological disparities, surgical management, systemic treatment, and follow-up of uterine sarcomas.
PubMed: 37007636
DOI: 10.1177/17588359231157645