Between 1987 and 2004, 74 women with stage I uterine papillary serous carcinoma (UPSC) were surgically staged at Yale University School of Medicine. This retrospective review of these patients was performed to investigate the survival of patients according to adjuvant treatment with chemotherapy and/or brachytherapy, to histologic tumor classification (mixed [10-50% serous carcinoma] vs pure UPSC [more than 50% serous carcinoma]), and to diagnostic method (dilation and curettage [D&C] or office endometrial aspiration biopsy). All patients underwent complete surgical staging, including total abdominal hysterectomy, bilateral salpingooophorectomy, peritoneal cytology, bilateral pelvic lymphadenectomy, paraaortic lymph node sampling, and omentectomy. Adjuvant treatment at the beginning of the study was either observation or treatment with abdominopelvic radiation. Since the mid-1990s, platinum-based chemotherapy regimens with or without brachytherapy (high-dose vaginal cuff radiation) have been used. Nearly all (96%) patients were postmenopausal with an average length of menopause of 18 years. Fifty-five women (74%) were diagnosed by D&C and 19 (26%) had an office endometrial aspiration biopsy. Forty-seven women (64%) had pure UPSC and 27 women (36%) had mixed serous carcinoma. Stage IA disease was diagnosed in 33 patients (45%), stage IB in 29 (39%), and stage IC in 12 (16%). Twelve of the 33 stage IA patients had no residual cancer in the hysterectomy specimen and 21 were positive for residual uterine cancer. In all, 21 women had no adjuvant therapy (16 stage IA, four stage IB, and one stage IC) and were observed during follow up. Twelve patients (five stage IA, five stage IB, and two stage IC) underwent brachytherapy alone. One woman had a preoperative cesium application, and eight received external pelvic or whole abdominal radiation. Platinum-based chemotherapy was given to 31 of 32 women who received chemotherapy, including two stage IA patients with no residual uterine disease, seven stage IA patients with residual uterine disease, 16 stage IB patients, and seven stage IC patients. In addition, 26 women who received chemotherapy also had brachytherapy. With a median follow up of 38 months, none of the 12 stage IA patients who were negative for residual UPSC developed a recurrence of disease. Ten of these women were originally diagnosed by D&C, and two had office endometrial aspiration biopsy. Of the 21 patients who had stage IA disease with residual uterine carcinoma, no recurrences were seen in the seven who received chemotherapy, but six of the 14 patients who received no chemotherapy developed a recurrence of disease. Treatment with chemotherapy resulted in an improved disease-free survival (DFS) and improved overall survival (OS) for stage IA patients with uterine residual disease compared with those who had no chemotherapy (P <0.05 for both). There were no recurrences in the follow-up period (median, 37 months) among the 15 stage IB patients who received platinum-based chemotherapy. Of 13 stage IB patients who did not have adjuvant chemotherapy, 10 patients recurred. The remaining three patients were disease free at 36, 73, and 132 months. Treatment with chemotherapy significantly improved the survival in stage IB patients (DFS and OS P <0.01 for both). Among the 12 patients with stage IC disease, one of the seven who received chemotherapy and four of the five who had no chemotherapy developed a recurrence of disease (DFS and OS, P <0.01 for both). The median follow up for the seven patients without recurrences was 57 months. Overall, the 5-year survival rate for stage I patients was 100% in those who received adjuvant chemotherapy and 46% for those with no chemotherapy. There were a total 21 recurrences among the 74 patients with UPSC. Of the 43 patients who did not receive chemotherapy, 20 (47%) recurred. Six of 21 (29%) women who did not have any adjuvant therapy recurred, including four at the vaginal cuff, two

Improved survival in surgical stage I patients with uterine papillary serous carcinoma (UPSC) treated with adjuvant platinum-based chemotherapy

Adenosarcomas are rare malignancies of the female genital tract, accounting for approximately 5 % of uterine sarcomas. Occasionally, adenosarcoma occurs in the ovaries or in extra-uterine tissue, which may be related to endometriosis. These tumors are characterized by benign epithelial elements and a malignant mesenchymal component. Pathologic diagnosis is dependent on the identification of the characteristic morphologic features. The most common immunohistochemical markers for adenosarcoma are CD10 and WT1, but these are not specific. The most frequent presenting symptom is abnormal uterine bleeding. The majority of patients present with stage I disease, with a 5-year overall survival of 60 to 80 %. Survival is influenced by the presence of myometrial invasion, sarcomatous overgrowth, lymphovascular invasion, necrosis, and the presence of heterologous elements including rhabdomyoblastic differentiation. Patients with sarcomatous overgrowth have significantly increased risk of recurrence 23 versus 77 % and decreased 5-year overall survival 50 to 60 %. Standard of care treatment is total hysterectomy with bilateral salpingo-oophorectomy without lymphadenectomy, as the incidence of lymph node metastasis is rare. Retrospective data does not support the use of adjuvant pelvic radiotherapy in uterine adenosarcomas as no survival benefit is seen. Insufficient data exists to recommend routinely neoadjuvant or adjuvant chemotherapy for uterine adenosarcomas. Limited evidence exists for the role of hormonal therapy in uterine adenosarcomas. The PIK3/AKT/PTEN pathway is mutated in ∼70 % of adenosarcomas, and this may represent a possible therapeutic target. This article reviews the current state of knowledge concerning uterine adenosarcoma and discusses the management of this rare tumor.

Uterine Adenosarcoma: a Review

Background
Uterine carcinosarcomas are rare and highly aggressive tumours. Although surgery is the cornerstone of treatment, the extent of the procedure remains controversial. We sought to evaluate the available literature data regarding the rationale of lymphadenectomy and its possible impact on survival.

The role of lymphadenectomy in uterine carcinosarcomas (malignant mixed mullerian tumours): a critical literature review

Uterine carcinosarcoma (UCS) is an aggressive malignancy. With an incidence of 2/100,000 females and a 5 years Survival at stage IV of 0%, it is an uncommon type of cancer with a very poor prognosis. Histologically, UCS is a biphasic neoplasm consisting of a mixture of malignant epithelial and mesenchymal components but there is now enough clinical-pathological evidence to consider UCS as metaplastic carcinoma in which the mesenchymal part retains epithelial features. The principal treatment in early/locally-advanced UCS is surgery; because of its aggressiveness, it generally presents distant metastases at diagnosis. Adjuvant radiotherapy and chemotherapy have uncertain effect. Chemotherapy alone or associated with radiotherapy seems to improve disease free survival (DFS) and overall survival (OS) in stage III and IV UCS. No advantages in OS and DFS have been shown with radiotherapy alone. The present review summarizes and analyzes the most important news about this type of gynaecological cancer.

Rare uterine cancer: carcinosarcomas. Review from histology to treatment.

Uterine sarcomas comprise leiomyosarcoma, endometrial stromal sarcoma, adenosarcoma, undifferentiated endometrial sarcoma, and their variants. Carcinosarcoma is historically classified as sarcoma, but it is now regarded as a metaplastic carcinoma. Uterine sarcomas are rare, and are traditionally staged in the same way as endometrial carcinoma. Because of their different clinical and biological behaviours, the International Federation of Gynecology and Obstetrics introduced a new staging system in 2009 for leiomyosarcoma, endometrial stromal sarcoma and adenosarcoma, and carcinosarcoma, respectively. Following an extensive literature review no good evidence was found to support the modification of the staging system. This is mainly because of the rarity of the sarcomas and the heterogeneity of the reports, the different diagnostic criteria and treatments changing over the decades the retrospective nature and small sample size in most studies, and the lack of uniform pathological review even in large studies. Currently, evidence is still lacking about the use of preoperative imaging for staging purpose, and uterine sarcomas remain to be surgically staged. Total hysterectomy is the cornerstone for both staging and treatment. Newer evidence shows that routine lymphadenectomy and bilateral salpingo-oophorectomy may not be necessary, unless in the presence of extra-uterine spread, suspicious ovaries or lymph nodes, and certain poor histological types, such as undifferentiated endometrial sarcoma and adenosarcoma with sarcomatous overgrowth. More research and data collection are definitely needed in order to verify and further revise the current staging systems.

Staging of uterine sarcomas.

Clinicopathologic analysis of uterine sarcomas from a single institution in the Canary Islands.

Objective: The aim of this study was to evaluate the clinicopathological data and prognosis factors corresponding to patients with papillary serous carcinoma of the endometrium treated at a single institution. Methods: Medical and anatomopathological records were reviewed in the Department of Gynecological Oncology of the Canarian University Hospital between 1989 and 2006. Only pure cases of papillary serous carcinoma of the endometrium were included. Survival rates were analyzed using the Kaplan-Meier technique. Results: The study included 61 patients. Stages I, II, III, and IV were identified in 32.8%, 19.7%, 31.1%, and 8.2% of patients, respectively. Event-free survival rates after 5 and 10 years were 59% and 40%, respectively, with a median time of 83 months (95% confidence interval, 63-110). Relapse occurred in 40.5% of the patients. Overall survival rates after 5 and 10 years were 37.7% and 29%, respectively, with a median time of 55 months (95% confidence interval, 40-70). A univariate analysis showed that prognosis factors were tumor markers, stage, myometrial infiltration, lymphovascular invasion, and ganglia involvement. A multivariate analysis showed that tumor markers, stage, and myometrial infiltration had an independent influence on overall survival. Conclusions: Papillary serous carcinoma of the endometrium is a very aggressive type of endometrial carcinoma that behaves more similar to ovarian carcinoma than to endometrial carcinoma. Tumor markers at diagnosis, stage, and myometrial infiltration mainly determine prognosis at our institution.

https://ijgc.bmj.com/content/ijgc/19/8/1364-1369.full.pdf

Pure papillary serous tumors of the endometrium: a clinicopathological analysis of 61 cases from a single institution.

article i nfo Objective: Toevaluatetheclinicopathologicdataandprognosticfactorsforpatientswithuterinesarcomastreated at a single institution, with special emphasis on malignant mixed mullerian tumors (MMMT). Methods: Medical and anatomic pathology records were reviewed. Survival rates were analyzed using the Kaplan-Meier method. Results: The study included 89 patients: 48.4% with MMMT; 22.4% with leiomyosarcomas; 20.2% with endometrial stromal sarcomas; and 9% with adenosarcomas. FIGO stages I, II, III, and IV were identified in 57.3%, 9.0%, 22.5%, and 7.8% of patients respectively. Event-free survival rates after 2, 5, and 10 years were 70%, 61%, and 55% respectively, with a median time of 90 months (95% CI, 41-140 months). Overall survival rates after 2, 5, and 10 years were 50%, 45%, and 39% respectively, with a median time of 43 months (95% CI, 3-83 months).

Clinicopathologic analysis of uterine sarcomas from a single institution in the

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Clinicopathologic analysis of uterine sarcomas from a single institution in the Canary Islands.

Pure papillary serous tumors of the endometrium: a clinicopathological analysis of 61 cases from a single institution.

Clinicopathologic analysis of uterine sarcomas from a single institution in the