Intravascular leiomyoma (IVL) is usually defined as a histologically benign leiomyoma that originates in a uterine fibroid or the intrauterine vein wall and grows and expands intravenously. We report a case in which pelvic IVL was detected early and discuss the early diagnosis of and best treatment for this tumor.

BACKGROUND: In the context of female genital tract malignancy, uterine sarcoma is considered the rarest form of the disease. Despite the inert nature of low-grade endometrial sarcoma, they must be meticulously diagnosed on time, with an exact grading of the severity and staging of the disease, which further guides the treatment modality and prognosis.
METHODS: A married Asian female without any significant past medical and surgical history complained of abdominal distension and discomfort, which was progressive in nature, for which a radiological assessment was made that showed features suggestive of endometrial sarcoma. Total abdominal hysterectomy with sapingoopherectomy was done without any perioperative complications. Histology further confirmed the diagnosis. Post-operatively, the patient had an unremarkable hospital stay and was discharged home.
CONCLUSIONS: Endometrial stromal sarcoma is one of the rare malignant entities presenting usually in late adult females, but sometimes it can present at an earlier age as well. Abdominal masses in females, although usually overlooked as benign, can sometimes be associated with a malignant picture. Low-grade endometrial sarcomas have been seen to masquerade other minor benign cases, such as leiomyoma. Despite the rarity of such malignant conditions, diagnosis and management are rather straightforward, and post-operative patient prognosis has been found to be rewarding.
CONCLUSIONS: Among the uterine sarcoma cases, endometrial sarcoma comes under the malignant disease of the least occurrence. Compared to other malignant conditions, these patients present with minor symptoms like discomfort, which may go unchecked. The major factor that should be noted is the on-time diagnosis and appropriate choice of treatment modality. Overall, despite a minute prevalence and difficult diagnosis, the prognosis of the patient is rather good.

OBJECTIVE: Uterine sarcomas are a rare group of uterine malignancies. Due to the low incidence and changes in uterine sarcoma classification, risk factors are not well characterized. Our objective was to evaluate risk factors for uterine sarcoma and compare risk factors between uterine sarcoma, malignant mixed Mullerian tumors (MMMTs), and type I endometrial carcinomas.
METHODS: This nested case-control study utilized linked data from population-based medical birth and cancer registries in Denmark, Finland, Norway, and Sweden. Up to 10 controls were matched on country and birth year for each uterine cancer case. Using multivariable adjusted multinomial logistic regression, estimates of the associations between pregnancy-related factors and risk of uterine sarcoma, MMMTs, and type I endometrial carcinomas were determined.
RESULTS: Having a very-low-birth-weight infant (< 1500 vs. 2500-3999 g: OR [95% CI] 2.83 [1.61-4.96]) was associated with an increased risk of uterine sarcoma. Whereas, having a more recent pregnancy was associated with reduced risks of MMMT (< 10 vs. ≥ 30 years: 0.66 [0.20-2.23]) and type 1 endometrial carcinomas (0.35 [0.30-0.41]) but not uterine sarcomas (1.33 [0.90-1.98], p-heterogeneity < 0.01).
CONCLUSIONS: Our study provides evidence that risk factors for uterine sarcoma and MMMT, previously grouped with uterine sarcomas, vary substantially. Additionally, MMMT and type I endometrial carcinomas are more similar than uterine sarcoma in that pregnancy complications like gestational hypertension and preeclampsia were associated with reduced risks of both but not uterine sarcoma, suggesting different etiologies.

BACKGROUND: Primary uterine alveolar soft part sarcoma (ASPS) is a rare, indolent mesenchymal malignancy with less than 40 patients documented in the literature.
METHODS: We report an example of ASPS in a 61-year-old postmenopausal woman. Macroscopically, the uterus showed multiple nodular masses. Microscopic examination revealed tumor arranged in nests and alveolar pattern. The tumor cells were moderately to markedly pleomorphic, epithelioid to polygonal, with eccentrically placed nuclei, vesicular chromatin, prominent macro-nucleoli, and moderate to abundant eosinophilic cytoplasm. PAS-positive and diastase-resistant intracytoplasmic crystals were also seen in some tumor cells. On immunohistochemistry, the tumor cells showed diffuse positivity for vimentin and nuclear positivity for TFE3, a surrogate marker for ASPS. These were negative for SMA, desmin, CD10, h-caldesmon, cyclin D1, EMA, Melan A, and CD34. SMARCB1 expression was retained. Based on the histopathology and IHC, a final diagnosis of uterine ASPS was rendered.
CONCLUSIONS: Knowledge of the characteristic histopathologic and immunohistochemical features can help accurately diagnose such rare tumors. Knowledge of the characteristic histopathologic and immunohistochemical features can help accurately diagnose such rare sarcoma in an uncommon site with an unusual age.

Uterine sarcomas are rare neoplasms of the uterus, some of which are associated with distinctive gene fusions. COL1A1::PDGFB fusion uterine sarcoma is a recently described entity that shares the same genetic alteration as dermatofibrosarcoma protuberans. These uterine sarcomas have a nonspecific spindle cell sarcoma appearance and are CD34 positive by immunohistochemistry. Accurate diagnosis relies on identification of the characteristic fusion by molecular genetic methods. The importance of diagnosing this entity lies in its potential response to targeted therapy with imatinib, a tyrosine kinase inhibitor successfully used in dermatofibrosarcoma protuberans, but only one prior case of COL1A1::PDGFB fusion uterine sarcoma treated with imatinib has been reported. Here, we describe a case of COL1A1::PDGFB fusion uterine sarcoma with response to imatinib after recurrence, with a brief review of this rare tumor.

Leiomyosarcomas of the uterine cervix are rare, mostly occurring in perimenopausal women. Diagnosis is based on pathology and immunohistochemistry. Surgery with a total abdominal hysterectomy and bilateral salpingo-oophorectomy remains the standard. A female patient in her 60s presented with heavy postmenopausal bleeding. Vaginal ultrasound scan and magnetic resonance imaging showed a large strongly vascularized cervical mass with features suspicious of sarcomatous degeneration. Positron Emission Tomography-Computed Tomography (PET-CT) did not reveal any evidence of metastases nor lymphadenopathy, but presence of right hydronephrosis. An abdominal hysterectomy with bilateral salpingo-oophorectomy, and end-to-end anastomosis of the right ureter, was performed. Pathology showed an International Federation of Gynecology and Obstetrics (FIGO)-stage 1B leiomyosarcoma of the uterine cervix. No adjuvant treatment was given. Adjuvant radiotherapy reduces the risk of recurrence but no survival impact. The benefit of adjuvant chemotherapy is questionable given the lack of randomized trials. Multidisciplinary research concerning molecular alterations of the disease is required to determine optimal management strategies with potential novel molecular therapies.

BACKGROUND: High-complexity and low-prevalence procedures benefit from treatment by referral centers. The volume of cases necessary to maintain high training in the treatment of gynecologic sarcoma is currently unknown. This study aimed to determine differences in survival and recurrence as a function of the volume of patients treated per center.
METHODS: The multicentric cross-sectional SARComa of the Uterus (SARCUT) study retrospectively collected cases of uterine sarcomas from 44 centers in Europe from January 2001 to December 2007. The survival of patients treated in high case-volume (HighCV) centers was compared with the survival of patients treated in low case-volume (LowCV) centers.
RESULTS: The study enrolled 966 patients: 753 in the LowCV group and 213 in the HighCV. Overall survival (OS) was 117 months, and cancer-specific survival (CSS) was 126 months. The difference was significant (respectively p = 0.0003 and 0.0004, log rank). After adjustment for other confounding factors, the remaining significant factors were age (hazard ratio [HR], 1.04; 95% confidence interval [CI], 1.03-1.05), histology (HR, 1.19; 95% CI, 1.06-1.34), extrauterine involvement (HR, 1.61; 95% CI, 1.24-2.10) and persistent disease after treatment (HR, 3.22; 95% CI, 2.49-4.18). The cytoreduction performed was significantly associated with the CSS and OS in both groups. The log rank for surgical cytoreduction was a p value lower than 0.0001 for OS, lower than 0.0001 for the LowCV centers, and 0.0032 for the HighCV centers.
CONCLUSIONS: The prognosis for patients with uterine sarcoma is directly related to complete tumor cytoreduction, histologic type, and FIGO stage, with significant differences between low and high case-volume centers. Patients with uterine sarcomas should be centralized in HighCV centers to improve their oncologic outcomes.

UNASSIGNED: Clear cell ovarian carcinoma is rare and accounts for 1%-12% of ovarian epithelial carcinomas, depending on ethnicity. The prevalence of clear cell ovarian carcinoma in Asian, White, and Black women is 11.1%%, 4.8%, and 3.1%, respectively. Magnetic resonance imaging (MRI) shows that clear cell ovarian carcinomas are typically unilocular cyst-solid (34.9%) or multilocular-solid (41.4%); only 23.7% are solid with papillary projections. MRI can detect clear cell ovarian carcinoma with a sensitivity and specificity of 90% and 87%, respectively. Notably, sometimes ovarian masses have a solid feature and should be differentiated from uterine masses. Clear cell ovarian carcinoma has a better prognosis compared to serous carcinoma when diagnosed at an early stage, but it has a poorer prognosis at an advanced stage. The absence of a residual tumor is a favorable prognostic factor in patients with advanced-stage clear cell ovarian carcinoma. Herein, we present a case in which clear cell ovarian carcinoma was misdiagnosed as uterine sarcoma because imaging showed a mass with a solid uterine-like and necrotic area. In the present case, cytoreductive surgery was performed to remove the entire tumor and its infiltration to the sigmoid colon and left ureter. Hence, the patient had a better prognosis.
UNASSIGNED: A 57-year-old Indonesian woman presented to our hospital (Dr. Soetomo General Hospital) with post-menopausal bleeding, a large solid pelvic mass, and abdominal discomfort. The patient was diagnosed with uterine sarcoma due to the solid feature observed during ultrasonography and MRI. During the surgery, the mass was observed to originate from the left ovary, and primary debulking surgery with a multidisciplinary team was performed with zero residual tumor tissue. The tumor was histopathologically confirmed as clear cell carcinoma.
UNASSIGNED: MRI of clear cell ovarian carcinoma can be misdiagnosed as uterine sarcoma due to its solid feature. Additionally, the enlarged mass distorts the anatomical landmarks. Surgery with no residual tumor improves the prognosis for advanced-stage clear cell ovarian carcinoma.

•Research surrounding treatment of leiomyosarcoma (LMS) treatment remains sparse.•Pembrolizumab/lenvatinib has been reported as a therapy for endometrial cancer, though not yet as therapy for LMS.•This report demonstrates disease regression after use of pembrolizumab and lenvatinib in a patient with recurrent LMS.

Endometrial stromal sarcoma (ESS) rarely causes infertility in young women. We report a nulligravida in her 30s who presented with primary infertility of 15 years. Hysteroscopy revealed a submucosal necrotic fibroid polyp. Ultrasonography detected multiple intramural fibroids. Open myomectomy with polypectomy was performed. Histopathology revealed low-grade ESS (LGESS) within the fibroid polyp. Subsequently, the patient underwent completion surgery. Her final diagnosis was estrogen-receptor positive LGESS stage IIIB, and she was suggested anastrozole adjuvant therapy and long-term surveillance. ESS with abnormal perimenopausal bleeding, though the most common presentation, may not always observed. Hence, a high index of suspicion of ESS should always be kept as a differential diagnosis in uterine fibroid polyp, though rare. Considering the scarcity of more extensive studies on ESS, reporting of cases will aid in formulating management protocols.
Endometriyal stromal sarkom (ESS) genç kadınlarda nadiren infertiliteye neden olmaktadır. Çalışmamızda 15 yıllık primer infertilite ile başvuran 30’lu yaşlarında bir nulligravida sunmaktayız. Histeroskopisinde submukozal nekrotik fibroid polip saptandı. Ultrasonografi birden fazla intramural fibroid olduğunu ortaya koydu. Polipektomi ile açık myomektomi yapıldı. Histopatoloji, fibroid polip içinde düşük dereceli ESS (LGESS) ortaya çıkardı. Ardından hastaya tamamlayıcı cerrahi uygulandı. Nihai tanısı östrojen reseptörü pozitif LGESS evre IIIB idi ve hastaya anastrozol adjuvan tedavisi ve uzun süreli gözetim önerildi. Anormal perimenopozal kanamalı ESS, en yaygın semptom olmasına rağmen her zaman gözlenmeyebilir. Bu nedenle, nadir de olsa uterin fibroid polipinde ESS şüphesi yüksek bir ayırıcı tanı olarak daima akılda tutulmalıdır. ESS ile ilgili daha kapsamlı çalışmaların azlığı göz önüne alındığında, olguların raporlanması yönetim protokollerinin formüle edilmesine yardımcı olacaktır.