scholarly journals Management Strategies in Advanced Uterine Leiomyosarcoma: Focus on Trabectedin

Sarcoma ◽  
2015 ◽  
Vol 2015 ◽  
pp. 1-14 ◽  
Author(s):  
Frédéric Amant ◽  
Domenica Lorusso ◽  
Alexander Mustea ◽  
Florence Duffaud ◽  
Patricia Pautier
Keyword(s):  
Therapeutic Potential ◽  
Management Strategies ◽  
Total Abdominal Hysterectomy ◽  
Abdominal Hysterectomy ◽  
Local Tumor Control ◽  
Tumor Control ◽  
Uterine Leiomyosarcoma ◽  
Chemotherapy Drugs ◽  
Risk Patients ◽  
Overall Survival Benefit

The treatment of advanced uterine leiomyosarcomas (U-LMS) represents a considerable challenge. Radiological diagnosis prior to hysterectomy is difficult, with the diagnosis frequently made postoperatively. Whilst a total abdominal hysterectomy is the cornerstone of management of early disease, the role of routine adjuvant pelvic radiotherapy and adjuvant chemotherapy is less clear, since they may improve local tumor control in high risk patients but are not associated with an overall survival benefit. For recurrent or disseminated U-LMS, cytotoxic chemotherapy remains the mainstay of treatment. There have been few active chemotherapy drugs approved for advanced disease, although newer drugs such as trabectedin with its pleiotropic mechanism of actions represent an important addition to the standard front-line systemic therapy with doxorubicin and ifosfamide. In this review, we outline the therapeutic potential and in particular the emerging evidence-based strategy of therapy with trabectedin in patients with advanced U-LMS.

Petroclival meningiomas: long-term outcomes of multimodal treatments and management strategies based on 30 years of experience at a single institution

2020 ◽  
Vol 132 (6) ◽  
pp. 1675-1682 ◽  
Author(s):  
Jin Wook Kim ◽  
Hee-Won Jung ◽  
Yong Hwy Kim ◽  
Chul-Kee Park ◽  
Hyun-Tai Chung ◽  
...  
Keyword(s):  
Adjuvant Treatment ◽  
Management Strategies ◽  
Local Tumor Control ◽  
Tumor Control ◽  
Years Of Experience ◽  
Local Tumor ◽  
Long Term Outcomes ◽  
Single Institution ◽  
Petroclival Meningiomas

OBJECTIVEA thorough investigation of the long-term outcomes and chronological changes of multimodal treatments for petroclival meningiomas is required to establish optimal management strategies. The authors retrospectively reviewed the long-term clinical outcomes of patients with petroclival meningioma according to various treatments, including various surgical approaches, and they suggest treatment strategies based on 30 years of experience at a single institution.METHODSNinety-two patients with petroclival meningiomas were treated surgically at the authors’ institution from 1986 to 2015. Patient demographics, overall survival, local tumor control rates, and functional outcomes according to multimodal treatments, as well as chronological change in management strategies, were evaluated. The mean clinical and radiological follow-up periods were 121 months (range 1–368 months) and 105 months (range 1–348 months), respectively.RESULTSA posterior transpetrosal approach was most frequently selected and was followed in 44 patients (48%); a simple retrosigmoid approach, undertaken in 30 patients, was the second most common. The initial extent of resection and following adjuvant treatment modality were classified into 3 subgroups: gross-total resection (GTR) only in 13 patients; non-GTR treatment followed by adjuvant radiosurgery or radiation therapy (non-GTR+RS/RT) in 56 patients; and non-GTR without adjuvant treatment (non-GTR only) in 23 patients. The overall progression-free survival rate was 85.8% at 5 years and 81.2% at 10 years. Progression or recurrence rates according to each subgroup were 7.7%, 12.5%, and 30.4%, respectively.CONCLUSIONSThe authors’ preferred multimodal treatment strategy, that of planned incomplete resection and subsequent adjuvant radiosurgery, is a feasible option for the management of patients with large petroclival meningiomas, considering both local tumor control and postoperative quality of life.

scholarly journals The Imaging and Pathological Features of Metastatic Leiomyosarcoma in the Gallbladder

Rare Tumors ◽  
2016 ◽  
Vol 8 (4) ◽  
pp. 179-181 ◽  
Author(s):  
Yi Guo ◽  
Eleanor Chen ◽  
Darin J. Davidson ◽  
Venu G. Pillarisetty ◽  
Robin L. Jones ◽  
...  
Keyword(s):  
Metastatic Disease ◽  
Uterine Fibroids ◽  
Exploratory Laparotomy ◽  
Total Abdominal Hysterectomy ◽  
Abdominal Hysterectomy ◽  
Metastatic Lesion ◽  
Uterine Leiomyosarcoma ◽  
High Grade ◽  
Initial Management ◽  
Pelvic Radiation

Uterine leiomyosarcoma is a rare and aggressive malignancy with poor overall prognosis. There have been few reports of metastatic leiomyosarcoma in the gallbladder. We report a case of a 41-year-old female who underwent total abdominal hysterectomy due to presumed uterine fibroids. The postoperative pathology revealed high-grade pleomorphic leiomyosarcoma, with involvement of the uterine serosal surface. She subsequently underwent exploratory laparotomy, followed by pelvic radiation and chemotherapy. Since initial management she has developed metastatic disease and has been under treatment and surveillance for 11 years. She has undergone multiple surgical procedures and numerous lines of systemic therapy for metastatic leiomyosarcoma, including cholecystectomy for a metastatic lesion in the gallbladder. There have been no previous reports of metastatic leiomyosarcoma in the gallbladder. Despite extensive metastatic disease this patient has had prolonged survival with multi-modality management.

scholarly journals Fumarase-deficient uterine leiomyoma: a case of a rare entity and surgical innovation

2020 ◽  
Vol 2020 (3) ◽  
Author(s):  
Hong Lee ◽  
Saman Shafiezadeh ◽  
Rajeev Singh
Keyword(s):  
Renal Cell Carcinoma ◽  
Uterine Leiomyoma ◽  
Fumarate Hydratase ◽  
Total Abdominal Hysterectomy ◽  
Abdominal Hysterectomy ◽  
Uterine Leiomyosarcoma ◽  
Rare Entity ◽  
Surgical Innovation ◽  
Final Histology ◽  
Hereditary Leiomyomatosis

Abstract We report a case of a 47-year-old female, with strong preoperative clinical and radiological suspicious of uterine leiomyosarcoma who underwent a total abdominal hysterectomy. Despite the final histology concluded as benign uterine leiomyoma, the loss of fumarate hydratase expression of the same specimen still put her at risk of having hereditary leiomyomatosis and renal cell carcinoma syndrome. Intraoperatively, an obstetric vacuum cup was used for uterine manipulation to avoid breaching of the uterine serosa.

scholarly journals Metastatic Uterine Leiomyosarcoma in the Upper Buccal Gingiva Misdiagnosed as an Epulis

2014 ◽  
Vol 2014 ◽  
pp. 1-4 ◽  
Author(s):  
Andrea Cassoni ◽  
Valentina Terenzi ◽  
Davina Bartoli ◽  
Oriana Rajabtork Zadeh ◽  
Andrea Battisti ◽  
...  
Keyword(s):  
Lung Metastases ◽  
Muscle Flap ◽  
Surgical Excision ◽  
Total Abdominal Hysterectomy ◽  
Abdominal Hysterectomy ◽  
Local Relapse ◽  
Primary Treatment ◽  
High Rate ◽  
Uterine Leiomyosarcoma ◽  
Temporalis Muscle Flap

Uterine leiomyosarcoma (LMS) is a rare tumor constituting 1% of all uterine malignancies. This sarcoma demonstrates an aggressive growth pattern with an high rate of recurrence with hematologic dissemination; the most common sites are lung, liver, and peritoneal cavity, head and neck district being rarely interested. Only other four cases of metastasis in the oral cavity have been previously described. The treatment of choice is surgery and the use of adjuvant chemotherapy and radiation has limited impact on clinical outcome. In case of metastases, surgical excision can be performed considering extent of disease, number and type of distant lesions, disease free interval from the initial diagnosis to the time of metastases, and expected life span. We illustrate a case of uterine LMS metastasis in the upper buccal gingiva that occurred during chemotherapy in a 63-year-old woman that underwent a total abdominal hysterectomy with bilateral salpingo-oophorectomy for a diagnosis of LMS staged as pT2bN0 and that developed lung metastases eight months after primary treatment. Surgical excision of the oral mass (previously misdiagnosed as epulis at a dental center) and contemporary reconstruction with pedicled temporalis muscle flap was performed in order to improve quality of life. Even if resection was achieved in free margins, “local” relapse was observed 5 months after surgery.

scholarly journals Combination of Sorafenib and Transarterial Chemoembolization in Selected Patients with Advanced-Stage Hepatocellular Carcinoma: A Retrospective Cohort Study at Three German Liver Centers

Cancers ◽  
2021 ◽  
Vol 13 (9) ◽  
pp. 2121
Author(s):  
Christine Koch ◽  
Markus Göller ◽  
Eckart Schott ◽  
Oliver Waidmann ◽  
Mark op den Winkel ◽  
...  
Keyword(s):  
Hepatocellular Carcinoma ◽  
Overall Survival ◽  
Standard Of Care ◽  
Local Tumor Control ◽  
Tumor Control ◽  
Significant Difference ◽  
Tace Group ◽  
Group A ◽  
Overall Survival Benefit ◽  
Group B

Background and Aims. Systemic treatment with sorafenib has been the standard of care (SOC) in patients with advanced Barcelona Clinic Liver Cancer (BCLC) stage C hepatocellular carcinoma (HCC) for more than a decade. TACE has been reported to allow better local tumor control in selected patients with BCLC stage C HCC. Methods. A retrospective analysis of patients with BCLC stage C HCC that were treated with sorafenib and TACE was conducted; they were compared to BCLC stage C patients treated either with TACE or sorafenib in the same period of time outside a clinical trial. Results. A total of 201 patients with BCLC stage C were identified, who were treated with either sorafenib and TACE (group A; n = 54), sorafenib (group B; n = 82) or TACE (group C; n = 65). No significant difference in baseline characteristics was observed. Time to progression was 7.0 months (95% CI: 4.3–9.7), 4.1 months (95% CI: 3.6–4.7) and 5.0 months (95% CI: 2.9–7.1) in groups A, B and C, respectively, and overall survival was 16.5 months (95% CI: 15.0–18.1), 8.4 months (95% CI: 6.0–10.8) and 10.5 months (95% CI: 7.5–13.6), respectively (group A vs. group B: p < 0.001; group A vs. group C: p = 0.0023). Adverse events of grade 3/4 occurred in 34% of patients in group A. Conclusions. Although sorafenib is a SOC in patients with BCLC stage C HCC, TACE is frequently used as an additional locoregional treatment in selected patients. This combined approach resulted in a significant overall survival benefit in selected patients, although randomized trials have not yet proven this benefit.

scholarly journals Metastatic Uterine Leiomyosarcoma in a Nullipara with Primary Infertility: A Case Report

2019 ◽  
Vol 4 (11) ◽  
Author(s):  
Okechukwu B. Anozie ◽  
Johnbosco I. Nwafor ◽  
Chidi U. Esike ◽  
Chukwuemeka I. Ukaegbe ◽  
Richard L. Ewah ◽  
...  
Keyword(s):  
Total Abdominal Hysterectomy ◽  
Abdominal Hysterectomy ◽  
Uterine Leiomyosarcoma ◽  
Presenting Symptoms ◽  
Primary Infertility ◽  
History Of ◽  
Uterine Cancers ◽  
Operative Findings ◽  
High Index Of Suspicion ◽  
Lost To Follow Up

Uterine leiomyosarcoma accounts for 1-2% of uterine cancers. It is an extremely aggressive malignancy associated with a poor prognosis. Women affected may vary in age, but are most common between 4th and 7th decades of life. Presenting symptoms mimic uterine leiomyoma. Preoperative diagnosis of uterine leiomyosarcoma is difficult and often made at time of surgical resection. We report a case of Mrs A.E, a 40 year old nullipara with history of primary infertility of 20 years duration, who presented with abdominal swelling of 3 years and vaginal bleeding of 7 weeks duration. Abdominopelvic ultra-sonography done at presentation was suggestive of leiomyoma. She was scheduled for myomectomy and subsequently had total abdominal hysterectomy and omentectomy following intra-operative findings of features suggestive of leiomyosarcoma with evidence of metastasis to omentum. These findings were confirmed on histology of the specimen. She received one cycle of combination chemotherapy but was lost to follow up. Uterine LMS is an aggressive tumour, therefore, a high index of suspicion is needed especially for huge uterine nodules and such patients must be closely monitored for adequate management.

Vaginal embryonal rhabdomyosarcoma

2021 ◽  
Vol 20 (3) ◽  
pp. 174-177
Author(s):  
N.I. Minashkina ◽  
◽  
V.A. Strykov ◽  
T.G. Dyadik ◽  
I.V. Karachentsova ◽  
...  
Keyword(s):  
Tumor Response ◽  
Management Strategies ◽  
Young Patients ◽  
Reproductive Function ◽  
High Dose Rate ◽  
Local Tumor Control ◽  
Embryonal Rhabdomyosarcoma ◽  
Tumor Control ◽  
High Dose ◽  
Local Tumor

Vaginal embryonal rhabdomyosarcoma is a relatively rare tumor, which is more commonly diagnosed in children under three years of age and is rarely associated with organ-sparing treatment. Neoadjuvant chemotherapy followed by radiotherapy or surgery can be currently used as local tumor control. A clinical case of vaginal embryonal rhabdomyosarcoma in a 5-year-old girl is presented. Considering the tumor response to polychemotherapy, high dose rate intracavitary radiation therapy (brachytherapy) was chosen as the method of local control. Brachytherapy reduces the need for surgery and radiation of large regions in children and enables young patients to realize their reproductive function in the future. Key words: brachytherapy, management strategies, embryonal rhabdomyosarcoma

scholarly journals Uterine myxoid leiomyosarcoma: a diagnostic challenge

2017 ◽  
Vol 6 (7) ◽  
pp. 3170
Author(s):  
Jayalakshmi Durairaj ◽  
Swaramya Chandrasekaran
Keyword(s):  
Urinary Retention ◽  
Vaginal Bleeding ◽  
Total Abdominal Hysterectomy ◽  
Abdominal Hysterectomy ◽  
Uterine Leiomyosarcoma ◽  
Diagnostic Challenge ◽  
Uterine Sarcomas ◽  
Myxomatous Degeneration ◽  
Abnormal Vaginal Bleeding

Uterine sarcomas are histologically diverse tumours which comprise about 3-7% of uterine malignancies. Myxoid leiomyosarcoma (MLMS) is an extremely uncommon variant of uterine leiomyosarcoma. This report is of a 35 year old woman who presented with urinary retention and abnormal vaginal bleeding, who underwent a total abdominal hysterectomy for cervical fibroid. A diagnosis of leiomyoma with myxomatous degeneration was made. The woman subsequently developed a recurrence within 5 months of the surgery and was diagnosed with MLMS following biopsy. This case reiterates the uncertainties associated with the diagnosis of MLMS and the need for heightened vigilance in women with myxomatous degeneration of leiomyoma.

scholarly journals Leiomyosarcoma in pregnancy: Incidental finding during routine caesarean section

2021 ◽  
Vol 4 (3) ◽  
pp. 092-095
Author(s):  
Tang Toon Wen ◽  
Jessie Phoon Wai Leng
Keyword(s):  
Caesarean Section ◽  
Reproductive Tract ◽  
Incidental Finding ◽  
Elective Caesarean Section ◽  
Breech Presentation ◽  
Total Abdominal Hysterectomy ◽  
Abdominal Hysterectomy ◽  
Female Reproductive Tract ◽  
Uterine Leiomyosarcoma ◽  
In Pregnancy

Uterine leiomyosarcoma (LMS) is uncommon tumour arising from the female reproductive tract. Incidence of LMS in pregnancy is extremely rare, with only 10 cases reported thus far in medical literature. We present a case of myomectomy performed during elective caesarean section for breech presentation, due to its easy accessibility and well contracted uterus. Subsequent histology revealed LMS on final specimen. Patient subsequently underwent total abdominal hysterectomy, bilateral salpingo-oophorectomy. No chemotherapy was given as she opted for close clinical- radiological monitoring instead. This case report highlights the importance of discussion with patients regarding the risk of occult malignancy in a fibroid uterus. Appropriate management of uterine leiomyosarcoma in pregnancy remains unclear. Consideration of removing an enlarging leiomyoma during caesarean section might be ideal in view of its malignant potential, just like in this case; however, location of the tumour and risk of bleeding needs to be weighed. Ultimately, management of such cases needs proper discussion between obstetrician and the patient.

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