Low-grade endometrial stromal sarcoma: A Turkish uterine sarcoma group study analyzing prognostic factors and disease outcomes

Ayhan, Ali; Toptaş, Tayfun; Öz, Murat; Vardar, Mehmet Ali; Kayıkçıoğlu, Fulya; Özgül, Nejat; Gökçü, Mehmet; Şimşek, Tayup; Tunç, Mehmet; Meydanlı, Mehmet Mutlu

doi:10.1016/j.ygyno.2020.12.017

Cited by 5 publications

(6 citation statements)

References 20 publications

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“…The association did not hold true for disease-specific survival, probably owing to the indolent nature of low-grade endometrial stromal sarcoma regardless of stage at presentation. These findings are consistent with prior studies 11 12. Our results also uphold the overall 2018 FIGO staging system.…”

Section: Discussionsupporting

confidence: 93%

Primary characteristics and outcomes of newly diagnosed low-grade endometrial stromal sarcoma

Smith

Jansen

Miller

et al. 2022

Int J Gynecol Cancer

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ObjectiveTo assess potential predictive variables for nodal metastasis and survival outcomes in patients with newly diagnosed, low-grade endometrial stromal sarcoma.MethodsWe performed a single-institution, retrospective analysis of consecutive patients with newly diagnosed, low-grade endometrial stromal sarcoma who presented between January 1, 1980 and December 31, 2019 and underwent hysterectomy at our institution or presented within 3 months of primary surgery elsewhere before recurrence. Patients who presented to our institution only at recurrence were excluded. Patients with <3 months of follow-up were excluded from survival analyses.ResultsWe identified 127 consecutive patients for analysis. Median age at diagnosis was 48 years (range 19–88 years); 91 (74.6%) of 127 were pre-menopausal; and 74 (58.3%) of 127 had uterine-confined, stage I tumors. Of 56 patients (44.1%) who underwent lymph node sampling, 10 (17.9%) had nodal metastasis. Of the 10 with nodal metastasis, 1 (10%) did not have lymphadenopathy or extra-uterine disease, 4 (40%) had lymphadenopathy only, 1 (10%) had extra-uterine disease only, and 4 (40%) had both. Among the 29 patients without apparent extra-uterine disease or gross lymphadenopathy, there was one occult lymph node metastasis (3.4%). Gross lymphadenopathy at time of surgery was predictive for lymph node metastasis (p<0.001). Median follow-up was 69 months (range 4–336) for the 95 patients included in the survival analyses. The 5-year progression-free survival and disease-specific survival rates were 79.8% and 90.8%, respectively. Patients with stage I tumors had longer progression-free survival than those with stage II–IV disease (p<0.001); there was no difference in disease-specific survival (p=0.63). Post-operative observation versus adjuvant therapy with hormone blockade or radiation therapy did not result in progression-free survival differences for stage I or completely resected stage II–IV disease (p=0.50 and p=0.81, respectively). Similarly, there was no disease-specific survival difference for completely resected stage II–IV disease (p=0.3).ConclusionsLymph node dissection in patients with low-grade endometrial stromal sarcoma should be reserved for those with clinically suspicious lymphadenopathy. Disease stage correlated with progression-free survival but not disease-specific survival. Post-operative therapy did not improve progression-free survival or disease-specific survival.

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Section: Discussionsupporting

confidence: 93%

Primary characteristics and outcomes of newly diagnosed low-grade endometrial stromal sarcoma

Smith

Jansen

Miller

et al. 2022

Int J Gynecol Cancer

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“…As expected, the FIGO stage ( 8 , 27 – 30 ) and tumor size ( 29 , 31 – 33 ) were found to be associated with recurrence also in our study.…”

Section: Discussionsupporting

confidence: 90%

“…Our data support that any morcellation/fragmentation of LG-ESS (not only power-morcellation) should be avoided if this tumor is suspected. As expected, the FIGO stage (8,(27)(28)(29)(30) and tumor size (29,(31)(32)(33) were found to be associated with recurrence also in our study.…”

Section: Discussionsupporting

confidence: 90%

Low-Grade Uterine Endometrial Stromal Sarcoma: Prognostic Analysis of Clinico-Pathological Characteristics, Surgical Management, and Adjuvant Treatments. Experience From Two Referral Centers

Borella

Bertero²,

Cassoni³

et al. 2022

Front. Oncol.

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ObjectiveLow-grade uterine endometrial stromal sarcoma (LG-ESS) is a rare tumor characterized by an overall good survival but showing a indolent behavior and a variable risk of recurrence. There is no clear consensus on the optimal management of these tumors and no prognostic or predictive factors have been established. With this study, we evaluated the prognostic relevance of several clinical, surgical, and pathological features in patients affected by LG-ESS to identify risk factors associated with recurrence.MethodsWe retrospectively analyzed 52 LG-ESS cases, treated from January 1st, 1994, to May 31st, 2020, in two referral centers. The relationship between recurrence and clinicopathological characteristics as well as surgical treatment was investigated. Risk of recurrence and disease-free survival (DFS) were estimated by Cox regression and the Kaplan-Meier analysis, respectively.ResultsOf 52 patients with LG-ESS, 8 experienced recurrence (15%). The median follow-up was 100 months (SD ± 96, range: 15–336). By univariate analysis, fragmentation/morcellation, tumor size, FIGO stage, higher mitotic count, presence of necrosis, and lymphovascular space invasion (LSVI) resulted associated with a poorer outcome. Conversely, the surgical modality (laparotomic vs laparoscopic and hysterectomy with bilateral salpingo-oophorectomy vs local excision) and pelvic lymphadenectomy were not. Even the different modalities of adjuvant therapy (hormonal therapy, radiotherapy, and chemotherapy) showed no prognostic significance. Tumor fragmentation/morcellation and higher mitotic count resulted independent prognostic variables at multivariate analysis.ConclusionsThis data supports the avoidance of any type of morcellation if LG-ESS is suspected preoperatively. Higher mitotic count and, possibly, tumor size, advanced FIGO stage, necrosis, and LVSI could be exploited to tailor the adjuvant therapy, but these results need to be confirmed in larger prospective studies.

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“…Hence adjuvant hormone therapy is recommended for stage II to IV LGESS. [ 6 ] The treatment guidelines of LGEESS are the same as LGESS. [ 3 ]…”

Section: Discussionmentioning

confidence: 99%

“…[3] Total hysterectomy and bilateral salpingo-oophorectomy are recommended to LGESS, while additional resection for extrauterine disease depends on disease extent and resectability. [6,7] The efficacy of adjuvant antiestrogen hormone therapy to reduce the risk of recurrence has wide Acknowledgments because estrogen receptors and progesterone receptors are highly expressed in LGESS. Hence adjuvant hormone therapy is recommended for stage II to IV LGESS.…”

Section: Discussionmentioning

confidence: 99%

Primary ovarian endometrial stromal sarcoma after hysterectomy associated with multiple organ invasion: A case report

Chen

Gong

et al. 2023

Medicine

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Rationale: Endometrial stromal sarcoma (ESS) is a rare disease in patients with uterine malignancies, accounting for <1%. Low-grade endometrial stromal sarcoma (LGESS) accounts merely 0.2% of gynecologic malignant tumor. Primary low-grade extrauterine endometrioid stromal sarcomas (LGEESS) is even more uncommon, with only a few documented case reports. We report a case of primary LGEESS exhibiting widely invasion in multiple organs after hysterectomy, which is the first case reported in Jiangsu Province of China. Patient concerns: A 42-year-old nulliparous female with dysgnosia presented with a moderate amount of irregular vaginal bleeding, abdominal pain and distension, and frequent urination for 2 days. Her surgical history included a total hysterectomy and bilateral salpingectomy for uterine fibroids 6 years ago. Ultrasonography and the abdominal and pelvic computed tomography scan detected some solid polycystic masses in the pelvic and abdominal cavities. Diagnoses: The histopathology of the specimen confirmed the diagnosis of LESS in the absence of florid endometriosis. The patient was diagnosed with primary extrauterine endometrial stromal sarcoma at FIGO stage III. Interventions: Surgery and histopathology were performed. Outcome: After surgery, the patient was maintained on leuprorelin acetate microspheres with sustained release for injection at 3.75 mg once every 4 weeks while refusing further radiotherapy. Lessons: The diagnosis of primary LGEESS is challenging mainly because of their unforeseen location and nongynecologic signs and symptoms. Total hysterectomy and bilateral salpingo-oophorectomy are recommended to LGESS, while additional resection for extrauterine disease depends on disease extent and resectability.

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Low-grade endometrial stromal sarcoma: A Turkish uterine sarcoma group study analyzing prognostic factors and disease outcomes

Cited by 5 publications

References 20 publications

Primary characteristics and outcomes of newly diagnosed low-grade endometrial stromal sarcoma

Primary characteristics and outcomes of newly diagnosed low-grade endometrial stromal sarcoma

Low-Grade Uterine Endometrial Stromal Sarcoma: Prognostic Analysis of Clinico-Pathological Characteristics, Surgical Management, and Adjuvant Treatments. Experience From Two Referral Centers

Primary ovarian endometrial stromal sarcoma after hysterectomy associated with multiple organ invasion: A case report

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