Uterine leiomyosarcoma: A review of the literature and update on management options

Roberts, Monique; Aynardi, Jason T.; Chu, Christina

doi:10.1016/j.ygyno.2018.09.010

Cited by 139 publications

(211 citation statements)

References 90 publications

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“…Hyperplasia of the epidermis with hyperpigmentation of basal layer overlying the LMS has been previously described and was consistent with our patient's dermatofibroma‐like features . However, the peculiar observation of the presence of clear‐cell features to the best of our knowledge has never been described in cutaneous LMS, although a few cases of clear‐cell LMS have been reported in the uterus . Interestingly, our patient's mother died from a uterine LMS with clear‐cell changes, a malignancy in which germline RB1 mutations are recognized to be a risk factor .…”

Section: Discussionsupporting

confidence: 90%

“…The deep subcutaneous type originates from the smooth muscle vessels and has higher rates of local recurrence (37%) and metastasis (43%) . Usually, cutaneous LMS appears as a well‐circumscribed nodule in acral, post‐traumatic, and sun‐exposed regions . Our patient denied any trauma and his lesion was quite indolent, but the progressive growth and presence of a brownish pigmentation warranted prompt referral to a dermatologist.…”

Section: Discussionmentioning

confidence: 81%

“…It usually affects males between 50 and 80 years of age, occurring mostly on the scalp and the extremities . Rare variants or unusual locations of LMS have been described, but clear‐cells changes as the prominent pathological finding have never been reported in the literature for cutaneous LMS, although it has been described in uterine LMS . Here, we present the clinical‐pathological and dermoscopic features of a clear‐cell cutaneous LMS.…”

Section: Introductionmentioning

confidence: 88%

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Clear‐cell variant of superficial cutaneous leiomyosarcoma associated with RB1 mutation: Clinical, dermoscopic, and histopathological characteristics

et al. 2020

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Leiomyosarcoma is a relatively rare soft tissue tumor whose clear-cell variant has only been reported in leiomyosarcomas of the uterus. We report here for the first time a primary cutaneous clear-cell leiomyosarcoma in the trunk skin of a 49-yearold man, characterized by a very indolent clinical and dermoscopic presentation, mimicking a dermatofibroma. Genetic analysis of the otherwise healthy patient revealed a germline mutation in the retinoblastoma 1 gene (RB1); the same mutation was found in his son, who had previously developed retinoblastoma. Moreover, the mother of the patient had died of uterine leiomyosarcoma with clear-cell changes. Mutations in the RB1 gene occur commonly in human neoplasms. In this patient, we were able to link his clear-cell variant of cutaneous leiomyosarcoma with the loss of retinoblastoma protein expression, as revealed by immunohistochemical staining analysis. K E Y W O R D S clear-cell leiomyosarcoma, dermoscopy, RB1 gene mutation, superficial cutaneous leiomyosarcoma

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Section: Discussionsupporting

confidence: 90%

Section: Discussionmentioning

confidence: 81%

Section: Introductionmentioning

confidence: 88%

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Clear‐cell variant of superficial cutaneous leiomyosarcoma associated with RB1 mutation: Clinical, dermoscopic, and histopathological characteristics

et al. 2020

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“…Uterine leiomyosarcoma (uLMS) is the most common type of uterine sarcoma . Although uLMS accounts for only 1% of all uterine cancers, it has a high mortality rate owing to its aggressive nature and tendency for chemoresistance . Even though more than half the patients with uLMS are diagnosed with early stage disease, they usually have a poor prognosis.…”

Section: Introductionmentioning

confidence: 99%

“…uLMS is often misdiagnosed as a uterine fibroid or leiomyoma and is usually diagnosed incidentally after an elective hysterectomy or myomectomy . Unexpected uLMS cases are identified in 0.1–0.2% of hysterectomy specimens with suspected leiomyoma .…”

Section: Introductionmentioning

confidence: 99%

Effectiveness of adjuvant treatment for morcellated, International Federation of Gynecology and Obstetrics stage I uterine leiomyosarcoma: A Korean multicenter study

Choi

Kim

Hong

et al. 2019

J of Obstet and Gynaecol

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Aim: To evaluate the effectiveness of adjuvant treatment for morcellated, uterus-confined leiomyosarcoma in a multicenter setting. Methods: We identified patients with International Federation of Gynecology and Obstetrics stage I uterine leiomyosarcoma primarily treated with surgery between 2003 and 2016. Among them, patients who underwent one of the following morcellation methods were included: (i) power morcellation; (ii) intracorporeal morcellation using scalpels or electrocautery; and (iii) vaginal morcellation. Patients' survival outcomes were compared according to the implementation of adjuvant treatment. Results: From 13 institutions, 55 patients were included; 31 for adjuvant treatment group and 24 for surgery only group. The clinicopathological characteristics including the mass size, morcellation methods, extent of surgery, and mitotic count were similar between the groups. In the adjuvant treatment group, 67.7%, 19.4% and 12.9% of patients received chemotherapy, chemoradiation and radiation, respectively. After a median follow-up of 50.5 months, the adjuvant treatment and surgery only groups showed similar overall survival (5-year rate, 92.0% vs 90.4%; P = 0.959). No significant difference in progression-free survival was observed between the two groups (3-year rate, 46.1% vs 78.2%; P = 0.069). On multivariate analyses, adjuvant treatment did not affect progression-free survival (adjusted HR, 2.138; 95% CI, 0.550-8.305; P = 0.273). The adjuvant treatment group showed a trend towards more common distant metastasis, compared to the surgery only group (25.8% vs 4.2%; P = 0.062). The incidences of pelvic, retroperitoneal, and abdominal recurrences were not different between the groups. Conclusion: Despite its frequent use in clinical practice, adjuvant treatment did not improve the survival outcomes of patients with morcellated, International Federation of Gynecology and Obstetrics stage I uterine leiomyosarcoma.

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Impact of perioperative characteristics on the recurrence risk and survival of patients with uterine leiomyosarcoma

Gracia,

Yildirim,

Macuks

et al. 2023

Intl J Gynecology & Obste

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ObjectivesTo analyze the impact of perioperative characteristics on the risk of recurrence in patients with uterine leiomyosarcomas.MethodsA sub‐analysis of the SARComa of the UTerus (SARCUT) study, which is a multicentric cross‐sectional pan‐European study that included 390 patients diagnosed with leiomyosarcoma, between 2001 and 2007. Perioperative factors related to risk of recurrence and survival were analyzed.ResultsThe 5‐year and 10‐year disease‐free survivals (DFS) were 46% and 55%, respectively. Overall survival at 5 and 10 years was 34% and 47%, respectively. The most important factors related to global recurrence were the incomplete cytoreduction (hazard ratio [HR] 2.87; 95% confidence interval [CI] 1.91–4.31); performing bilateral adnexectomy (HR 2.71; 95% CI 1.23–5.93); tumor persistence after any treatment (HR 2.38; 95% CI 1.39–4.06); and adjuvant chemotherapy administration (HR 2.55; 95% CI 1.82–3.58) or adjuvant radiotherapy (HR 2.26; 95% CI 1.53–3.32). The major factors significantly associated with pelvic relapse were tumor persistence after any treatment (HR 3.63; 95% CI 1.83–7.20) and adjuvant radiotherapy (HR 2.74; 95% CI 1.44–5.20). Incomplete cytoreduction was the most important factor associated with distant relapse (HR 1.91; 95% CI 1.22–2.97). The most important factors related to overall survival were tumor persistence after any treatment (HR 4.59; 95% CI 2.51–8.40), incomplete cytoreduction (HR 3.68; 95% CI 2.44–5.56), tumor margin involvement (HR 2.41; 95% CI 1.64–3.55) and adjuvant chemotherapy (HR 1.91; 95% CI 1.31–2.78).ConclusionsComplete cytoreduction is the main prognosis factor impacting the DFS and overall survival of patients with uterine leiomyosarcoma. Adjuvant chemotherapy administration was associated with decreased rates of DFS and overall survival. The adjuvant radiotherapy was associated with a higher risk of global recurrence.

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Uterine leiomyosarcoma: A review of the literature and update on management options

Cited by 139 publications

References 90 publications

Clear‐cell variant of superficial cutaneous leiomyosarcoma associated with RB1 mutation: Clinical, dermoscopic, and histopathological characteristics

Clear‐cell variant of superficial cutaneous leiomyosarcoma associated with RB1 mutation: Clinical, dermoscopic, and histopathological characteristics

Effectiveness of adjuvant treatment for morcellated, International Federation of Gynecology and Obstetrics stage I uterine leiomyosarcoma: A Korean multicenter study

Impact of perioperative characteristics on the recurrence risk and survival of patients with uterine leiomyosarcoma

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