Uterine Sarcoma and Aromatase Inhibitors: Tom Baker Cancer Centre Experience and Review of the Literature

2012 ◽  
Vol 22 (6) ◽  
pp. 1006-1012 ◽  
Author(s):  
Alon D. Altman ◽  
Gregg S. Nelson ◽  
Pamela Chu ◽  
Jill Nation ◽  
Prafull Ghatage

ObjectivesUterine sarcomas are a rare group of mesenchymal tumors with a poor prognosis and aggressive biology. Standard treatment involves surgical staging. The role of further adjuvant treatment is unclear. The goals of this study were to determine the response rates to treatment of patients with uterine sarcomas and to review the currently available literature on the use of aromatase inhibitors (AIs).Materials and MethodsWe performed a retrospective analysis on all patients with uterine sarcoma treated with an AI between 2000 and 2010 at the Tom Baker Cancer Centre in Calgary, Alberta.ResultsFour patients with endometrial stromal sarcoma and 3 patients with leiomyosarcoma received treatment with an AI. A literature search resulted in 10 case reports and 4 retrospective studies of patients with endometrial stromal sarcoma and 1 case report and 2 retrospective studies of patients with leiomyosarcoma. On the basis of the available literature, combined with the current findings, the overall response rate of endometrial stromal sarcoma to AIs is 67% (complete response of 7% and partial response of 60%), and the partial response rate of leiomyosarcoma to AIs is 11%, with no reported complete responses.ConclusionsAromatase inhibitors are a well-tolerated class of medications that are effective in the treatment of endometrial stromal sarcomas. These medications may also have a role to help stabilize disease progression in the treatment of leiomyosarcoma. More large, prospective, multicentered trials will be needed to clarify this issue.

Author(s):  
Shailesh Puntambekar ◽  
Shetty Theertha Shankar ◽  
Arjun Goel ◽  
Shakti Panchal

Endometrial stromal sarcoma (ESS) is a case malignancy and accounts for 0.2% of all uterine malignancies and 10% of all uterine sarcomas. In the present case, an unmarried 27-year-old woman presented with complaints of on and off episodes of pain in the lower abdomen. On examination, a diffuse oval mass was felt occupying the hypogastrium, iliac and lumbar regions and extending up to the umbilicus. CT scan impression was given as? Complex retroperitoneal mass? solid cystic ovarian mass with grade I right hydro nephrosis. On laparoscopy, a huge mass measuring approximately 10×10 cm was noted arising from the uterus. Laparoscopic myomectomy was done and the specimen was retrieved in a bag and the tissue was sent for histopathological examination. The postoperative clinical course was uneventful. The patient was advised to undergo oocyte cryopreservation following which she was advised hysterectomy with bilateral salphingo-oophorectomy. Pelvic radiation was advised thereafter. ESS is a rare malignant tumor, which on histopathological examination reveals sheets of cells and endometrial stromal cell differentiation. Hysterectomy with bilateral salpingo-oophorectomy is optimum initial therapy. Radiotherapy is chosen when the tumour is inadequately excised or the pelvic disease is locally recurrent. Low grade ESS is a rare form of uterine sarcoma usually seen between 40-60 years of age with limited available data regarding the clinical course and management. Early diagnosis and management is associated with a favourable prognosis and outcome.


2006 ◽  
Vol 24 (18_suppl) ◽  
pp. 15053-15053
Author(s):  
N. Guler ◽  
D. Hizli ◽  
S. Sarici ◽  
R. Ocalan ◽  
M. Kose ◽  
...  

15053 Background: The prognosis of metastatic uterine sarcoma is poor with median survival reported between 4 to 26 months. We evaluated the efficacy and toxicity of ifosfamide (I) mesna (M) and doxorubicin (A) (IMA) chemotherapy regimen retrospectively in patients (pts) with metastatic, or recurrent uterine sarcomas. Methods: Eligible patients had measurable recurrent or metastatic disease, ECOG PS < 2, had adequate renal, hepatic and hematologic functions. The IMA regimen was ifosfamide 2500 mg/m2 days 1–3, mesna 2500 mg/m2 days 1–3, doxorubicin 60 mg/m2 day 1, repeated every 21 days. Patients were evaluated for response for each two cycles. Results: Thirty-five pts (17 leiomyosarcoma [LMS], 6 malignant mixed mesodermal tumor [MMMT], 5 endometrial stromal sarcoma, 4 carcinosarcoma, and 3 adenosarcoma) were enrolled in this study. The median age was 49 yrs (range, 18–72). Two pts were lost to follow up after the first cycle; one patient had ifosfamide encephalopathy on the third day of the first cycle. Thirty-two pts were assessable for response, toxicity, and survival. Nine pts had prior chemotherapy and 4 pts had radiotherapy. Most frequent metastatic sites were peritoneum, lung and liver. Median period of time from the diagnosis to starting IMA regimen was 14.5 months. A total of 132 cycles of chemotherapy were introduced and for each patient median number of chemotherapy cycles were 4 (range, 1–6). We observed CR in 2 pts; PR in 15 pts. Objective RR was % 48.6 (95% CI, 32% to 67%). Of 17 pts with LMS, 1 CR and 6 PRs were observed. The median progression-free survival time of the responders was 12.0 months. The median progression-free survival time of all patients was 7.0 months. NCI-CTC grade 3 or 4 leucopenia, neutropenia, thrombocytopenia, and anemia occurred in 40%, 55%, 12%, and 13%, respectively. There was no significant nausea/vomiting, nephrotoxicity. Febrile neutropenia was encountered in 6 pts. Dose modifications were required in 4 pts due to myelotoxicity. CNS toxicity was observed in one pts. Conclusions: IMA regimen has moderate anti-tumor activity (48.6%) with acceptable toxicity in pts with recurrent or metastatic uterine sarcomas. No significant financial relationships to disclose.


Rare Tumors ◽  
2020 ◽  
Vol 12 ◽  
pp. 203636132097286 ◽  
Author(s):  
Angela Duvalyan ◽  
Kirk Tran ◽  
Christopher Lee ◽  
Shefali Chopra ◽  
James Hu

Endometrial stromal sarcomas (ESS) account for 10-15% of uterine malignancies and are classified into four categories: endometrial stromal nodule (ESN), low-grade endometrial stromal sarcoma (LG-ESS), high-grade endometrial stromal sarcoma (HG-ESS) and undifferentiated uterine sarcoma (USS). Depending on stage, ESS is treated with surgery, hormone therapy, chemotherapy or targeted therapy. A rare complication of ESS with metastatic pulmonary disease is recurrent, bilateral pneumothoraces. The current literature has reported on patients with ESS who either presented with pneumothoraces at their initial diagnosis, and thus were untreated, or after treatment with surgical resection and hormone therapy. There have been no case reports of patients with ESS who presented with pneumothoraces while receiving chemotherapy. Furthermore, there have been no reported cases of patients with HG-ESS presenting with this rare complication. We would like to expand the literature by reporting on two patients with HG-ESS who presented with pneumothoraces while concurrently receiving chemotherapy.


2020 ◽  
Vol 30 (11) ◽  
pp. 1726-1732
Author(s):  
Virginia Benito ◽  
Amina Lubrano ◽  
Laureano León ◽  
Fernando Molano ◽  
Beatriz Pinar

ObjectiveTumor rupture during surgery is a risk factor for recurrence of sarcomas in other locations. However, the independent impact of rupture on prognosis is uncertain in uterine sarcomas. The aim of this study was to evaluate whether uterine rupture impacts outcomes in patients with uterine sarcoma.MethodsA retrospective analysis was carried out of all consecutive patients with uterine sarcoma managed at the Department of Gynecology and Obstetrics of the Complejo Hospitalario Universitario Insular-Materno Infantil of the Canary Islands, Spain between January 1990 and December 2016. Inclusion criteria included all patients with histologically proven uterine sarcoma. Exclusion criteria included patients with endometrial carcinoma (non-sarcomatous) and carcinosarcomas. During this period, 1981 patients were diagnosed with a uterine malignancy; 1799 were excluded because of a diagnosis of endometrial carcinoma and 85 patients were excluded for a diagnosis of carcinosarcoma. Thus, the final sample included 97 patients with uterine sarcoma (4.9%). These included leiomyosarcoma, endometrial stromal sarcoma, adenosarcoma, and liposarcoma. Surgical resection was the primary treatment, including open, laparoscopic and vaginal surgery. Survival rates were analyzed using the Kaplan–Meier method.ResultsThe median age was 52 years (range 25–90); 49.5% (48) were pre-menopausal. Distribution per histological type was: 46.4% (45) leiomyosarcoma, 23.7% (23) high-grade endometrial stromal sarcoma, 17.5% (17) low-grade endometrial stromal sarcoma, 11.3% (11) adenosarcoma, and 1% (1) liposarcoma. Uterine leiomyoma was the most frequent pre-operatively suspected diagnosis (49.5%). Iatrogenic rupture of the tumor during surgery occurred in 25.3% of cases (23). International Federation of Gynecology and Obstetrics stages I–II and III–IV were identified in 74.2% (72) and 25.8% (25) of patients, respectively. The median tumor size was 8 cm (range 2–40). The recurrence rate was 47.8% (11) for patients with intra-operative tumor rupture and 25% (17) for patients without uterine rupture (p=0.03). Disease-free survival rates at 1, 2, and 5 years for patients with uterine rupture were 72.7%, 55.4%, and 13.9%, respectively, with a median time of 39 months (95% CI 2.9 to 75). For those patients without uterine rupture, disease-free survival rates at 1, 2, and 5 years were 84.8%, 76.1%, and 71.3%, respectively, with a mean time of 208.6 months (95% CI 169 to 248.3) (p=0.01). Multivariate analysis showed that stage, histological type, and iatrogenic tumor rupture during surgery were all independent prognostic factors for overall survival (OR 7.9, 95% CI 1.6 to 38.2, p=0.01); OR 5.3, 95% CI 2.1 to 13, p<0.0001; and OR 2.6, 95% CI 1.1 to 6.5, respectively, p=0.03).ConclusionConsidering that uterine sarcomas, especially leiomyosarcomas, often occur in pre-menopausal women as bulky tumors requiring laparotomy and that they are rarely diagnosed pre-operatively, efforts should be made to avoid iatrogenic uterine rupture during surgery as it impairs patient survival.


2020 ◽  
Author(s):  
Chao Ding ◽  
Jianqing Zhu

Abstract Background:Tumor morcellation happened to facilitate iatrogenic metastasis for patients with occult uterine sarcoma. The optimal remedial procedure for these patients needed to be established. Methods:Data were retrospectively collected and analyzed from patients with occult uterine sarcoma undergoing morcellation. Results:23 consecutive patients with uterine sarcomas undergoing morcellation were accessed between Jan 2008 and Dec 2018, including 15 patients with uterine leiomyosarcoma and 8 with endometrial stromal sarcoma. Cytoreduction as a remedial procedure was significantly associated with better disease-free survival (P=0.031), and none of 7 patients undergoing cytoreduction suffered recurrence, while total hysterectomy (P=0.194) and adjuvant therapy (P=0.159) had no such benefit. There was no significant difference in disease-free survival between laparoscopic and open surgery (p=0.321). Conclusion:Cytoreduction was considered as the optimal remedial procedure for patients with occult uterine sarcoma undergoing morcellation.


2006 ◽  
Vol 53 (3) ◽  
pp. 67-72 ◽  
Author(s):  
S. Vrzic-Petronijevic ◽  
I. Likic-Ladjevic ◽  
M. Petronijevic ◽  
R. Argirovic ◽  
N.N. Ladjevic

Introduction: Uterine sarcomas are rare gynecological neoplasms and their classification is complicated. Uterine sarcoma is usually diagnosed in postmenopausal women and the diagnosis is often accidental and postoperative. Aim of this study was to present clinical and pathological characteristics of uterine sarcomas, diagnostic procedures, treatment and two-, three- and five-years cumulative survival rates. Materials and methods: The retrospective study of 61 cases of uterine sarcomas was conducted. Cases were distributed into groups based on definitive diagnosis of uterine sarcoma: group of leiomyosarcomas (LMS), carcinosarcoma (CS), endometrial stromal sarcomas (ESS), adenosarcomas (AS) and other rare uterine sarcomas. We investigated patients with clinical and pathological characteristics of uterine sarcomas, diagnostic procedures and treatment. Survival rate was calculated by Kaplan-Meier method. Results: From 61 patients 43 patients (70.49%) were postmenopausal. Mean period from menopause until appearance of symptoms was 14,63 years. One or more risk factors were present in 46 (75.4%) patients. Diagnosis of uterine sarcoma were established averagely 7.38 months after appearance of symptoms. 50 patients (82.0%) underwent one or more diagnostic procedures. Preoperative diagnosis of uterine sarcoma was established in 42.5% of patients. 53 (86.9%) of patients were treated operatively. The most used operative procedure (60,7%) was total hysterectomy with bilateral salpingooophorectomy. Postoperative pathohistologic analysis showed that low grade (LG) leiomyosarcoma were present in 19 (35.9%) cases, high grade (HG) leiomyosarcoma in 1 (1.9%) case, carcinosarcoma in 14 (26.4%) cases, low grade (LG) endometrial stromal sarcoma in 5 (9.4%) cases, high grade (HG) endometrial stromal sarcoma in 9 (17.0%) cases, adenosarcoma in 2 (3.8%) cases, and 2 cases of rare uterine sarcomas: 1 (1.9%) MALT HG lymphoma and 1(1.9%) malignant hemangiopericytoma. In one case of ESS (1.9%) only adenomyosis was found postoperatively suggesting that the whole tumour was removed during diagnostic procedure. Eight patients were not treated operatively. Two-years cumulative survival rate was 74.3%, three-years cumulative survival rate was 71.1%, and five years survival rate was 64.3%. Discussion: Average age, percent of postmenopausal patients and the mean age at the time of menopause in our studied correlate with current data. Clinical presentation of uterine sarcoma is associated with obesity and hypertension in more than 30% of cases, which is approved in our study. For early diagnostics it is important to notice that risk factors are similar to those connected with far more frequent endometrial carcinoma. Postmenopausal abnormal bleeding was the main reason for medical examination, explaining relatively short period for establishing the diagnosis in this group of patients. The variety of clinical findings in our studied group showed that the diagnosis must be based on preoperative pathohistology. Conclusion: Adequate diagnosis and treatment of uterine sarcoma is possible with regular yearly or more frequent follow-up, especially in postmenopausal women with known risk factors present. We need special attention for unclear symptoms and postmenopausal bleeding and we need to use all diagnostic procedures soon as possible including preoperative histology because early metastases are characteristic for uterine sarcomas. Factor of the most important predictive value is histologic grade. .


2021 ◽  
Author(s):  
Chao Ding ◽  
Jianqing Zhu

Abstract Background:Tumor morcellation happened to facilitate iatrogenic metastasis for patients with occult uterine sarcoma. The optimal remedial procedure for these patients needed to be established. Methods:Data were retrospectively collected and analyzed from patients with occult uterine sarcoma undergoing morcellation. Results:23 consecutive patients with uterine sarcomas undergoing morcellation were accessed between Jan 2008 and Dec 2018, including 15 patients with uterine leiomyosarcoma and 8 with endometrial stromal sarcoma. Hysterectomy + omentectomy as a remedial procedure was significantly associated with better disease-free survival (P=0.031), and all 7 patients undergoing the previous procedure remain disease free. However, nether simple hysterectomy (P=0.194) nor adjuvant therapy (P=0.159) had such benefits. There was no significant difference in disease-free survival between laparoscopic and open surgery (p=0.321). Conclusion:Hysterectomy + omentectomy was considered as the optimal remedial procedure for patients with occult uterine sarcoma undergoing morcellation.


1981 ◽  
Vol 67 (4) ◽  
pp. 341-348 ◽  
Author(s):  
Rado Kenda ◽  
Giuseppe De Palo ◽  
Salvatore Andreola ◽  
Gaetano Bandieramonte ◽  
Giovanni Lupi ◽  
...  

The clinical and pathologic features of 34 uterine sarcomas were studied to determine the natural history of the disease. Sixteen patients had leiomyosarcoma, five mixed mesodermal sarcoma, ten endometrial stromal sarcoma, two carcinosarcoma and one endolymphatic stromal myosis. The patients were treated without an unique protocol. At 3 years the actuarial relapse-free survival was 53.6 %: 68.4 % in stage I-II patients and 22.2 % in stage III-IV patients. As regards the histologic subtype mixed mesodermal sarcomas had the best prognosis; endometrial stromal sarcomas the worst. The necessity of a uniform clinical and histologic classification as well as the importance of controlled clinical trials are pointed out.


2009 ◽  
Vol 19 (2) ◽  
pp. 245-248 ◽  
Author(s):  
Frédéric Amant ◽  
An Coosemans ◽  
Vincent Renard ◽  
Els Everaert ◽  
Ignace Vergote

AbstractThe clinical impact of ET-743 (trabectedin; Yondelis) in women with leiomyosarcoma and undifferentiated uterine sarcoma in patients previously treated with chemotherapy is investigated. Current data show a clinical benefit in 2/5 patients, of which 1 had a partial remission during 9 months. Pooling the treatment outcomes with literature data, a response in 5 (38%) of 13 patients and a clinical benefit in 7 (54%) of 13 patients for all high-grade uterine sarcomas is calculated. When only uterine leiomyosarcomas are concerned, response rate is 5 (45%) of 11 patients with a clinical benefit in 7 (64%) of 11 patients. These results on the use of ET-743 in uterine sarcoma patients support the conductance of larger trials.


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