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Journal ArticleDOI

A High-Grade Undifferentiated Endometrial Stromal Sarcoma Presenting as Inversion of the Uterus: A Rare Case.

TL;DR: It is important for all clinicians to keep the high degree of suspicion for ESS while working up any case of abnormal uterine bleeding, and it is important to have preoperative histopathological diagnosis of ESS.
Abstract: Endometrial stromal sarcoma (ESS) is a rare malignant tumor that constitutes about 0.2% of all uterine malignancies and 10% of uterine sarcomas. ESS is generally misdiagnosed as leiomyoma or endometrial polyp and typically discovered on histopathological examination postoperatively because of its rarity. Endometrial stromal tumors are composed of cells resembling normal endometrial stroma in its proliferative phase. The histologic diagnosis of the high grade is made if there is a high-grade sarcoma with a high mitotic index and nuclear anaplasia. The mean age of presentation of high-grade endometrial sarcoma is about 61 years with the most common presenting complaint is menorrhagia. The median overall survival for high-grade endometrial sarcoma is 53 months with optimal cytoreduction. A 49-year-old woman P2 L2 presented with nonspecific complaint of discharge and spotting per vaginum. In the present case, the provisional diagnosis by clinical findings as well as imaging was in favor of the inversion of submucous fibroid. Preoperative histopathological examination and immunohistochemistry confirmed the diagnosis of high-grade undifferentiated ESS. Haultain's operation followed by total abdominal hysterectomy and bilateral salpingo-oophorectomy was performed. The patient was referred to another center for radiotherapy. From there, she was lost to follow-up. Rarity of endometrial stromal tumor limits the clinician view to diagnose it preoperatively. We were fortunate to have preoperative histopathological diagnosis of ESS. Furthermore, as ESS is rare and undifferentiated stromal sarcoma is even rarer, literature is lacking on its optimal management. Hence, it is important for all clinicians to keep the high degree of suspicion for ESS while working up any case of abnormal uterine bleeding.
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Journal ArticleDOI
TL;DR: Even if limited by few RCTs with short-term follow-up, the data suggest that laparoscopy should be considered an effective and safe procedure for patients with early stage endometrial cancer as well as laparotomic one.

164 citations


"A High-Grade Undifferentiated Endom..." refers background in this paper

  • ...Hormone therapy with medroxyprogesterone, tamoxifen, gonadotropin‐releasing hormone analogs, and aromatase inhibitors are suggested for low‐grade ESS Stage 3‐4 and for recurrent disease.[14,15] In contrast, high‐grade undifferentiated tumors are not responsive to progestin therapy, and the poor therapeutic result suggests that radiation therapy, chemotherapy, or both should be used in combination to the surgery....

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Journal ArticleDOI
TL;DR: Tumors with ZC3H7B–BCOR fusion constitute a distinct group of endometrial stromal sarcomas with high-grade morphology that should be distinguished from other uterine mesenchymal neoplasms that may demonstrate myxoid morphology.

120 citations


"A High-Grade Undifferentiated Endom..." refers background in this paper

  • ...Moreover, in the same study, the mean age of presentation was 54 years, and they were predominantly based in endomyometrium and demonstrated tongue‐like projections.[12]...

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Journal ArticleDOI

119 citations


"A High-Grade Undifferentiated Endom..." refers background in this paper

  • ...Undifferentiated endometrial sarcoma (UES) may present as heterogeneous signal intensity voluminous polypoidal mass with more frequent myometrial involvement which can be demarcated or diffusely infiltrative on T1‐ and T2‐weighted images.[8] UES frequently has hemorrhage and necrosis and shows vascular and lymphatic invasion....

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Journal ArticleDOI
TL;DR: Lung specimens from 16 patients with metastatic ESS were reviewed, finding tumors were well circumscribed and usually solid, composed of plump spindle cells arranged in short fascicles and sex cord-like stromal differentiation was identified in three.
Abstract: Pulmonary metastases of endometrial stromal sarcoma (ESS) are uncommon and can pose diagnostic problems. We reviewed lung specimens from 16 patients with metastatic ESS. Patients were 31-77 years of age at the time of lung biopsy. Uterine ESSs were diagnosed an average of 9.8 years before lung biopsy in 11 patients. Uterine ESSs were originally called smooth muscle tumors in three additional patients. Thirteen patients were evaluated for new pulmonary nodules, seven of whom were asymptomatic. Nodules were multiple in 14 and solitary in four, ranging from 1.0 to 8.0 cm in greatest dimension. One patient died of metastatic disease; 14 were alive and seven of these were without disease (mean follow-up 4.1 years). Diagnostic considerations in 12 consultation cases included ESS, sclerosing hemangioma, carcinoid tumor, lymphangioleiomyomatosis, endometriosis, hemangiopericytoma, and lymphoma. Tumors were well circumscribed and usually solid, composed of plump spindle cells arranged in short fascicles. Two tumors were predominantly cystic. Sex cord-like stromal differentiation was identified in three. Neoplastic cells stained for vimentin (93%), estrogen and progesterone receptor (100%), smooth muscle actin (57%), desmin (50%), and keratin (46%). Metastatic ESS should be included in the differential diagnosis of nonepithelial neoplasms in women.

104 citations


"A High-Grade Undifferentiated Endom..." refers background in this paper

  • ...Distant metastasis to the lung, heart, and other sites has also been reported.[9,10] High‐grade undifferentiated tumor tends to be larger, more polypoidal as presented in the index case (Stage IB)....

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Journal ArticleDOI
TL;DR: Endometrial stromal sarcomas are rare malignant tumors of the uterus, and most of the information available in literature is based on small series or case reports.
Abstract: Endometrial stromal sarcomas are rare malignant tumors of the uterus, and most of the information available in literature is based on small series or case reports. A proper preoperative diagnosis is difficult and in most cases the diagnosis is confirmed after hysterectomy for a presumed benign disease. Endometrial sampling, ultrasound, and magnetic resonance imaging can provide diagnostic clues. Total hysterectomy with bilateral salpingo-oopherectomy is the main line of management and for early disease complete cure is a reality. Ovarian conservation may be possible in young women with early stage disease and the role of lymphadenectomy is controversial. Adjuvant hormone therapy in the form of progesterone, gonadotropin releasing hormone analogues, and aromatase inhibitors are found to be effective in preventing recurrences. Hormone therapy, radiotherapy and surgical excision of the metastasis are recommended for recurrences.

79 citations


"A High-Grade Undifferentiated Endom..." refers background or result in this paper

  • ...[2] Nearly 10%–25% affected women are premenopausal.[2] High‐grade ESS are seen in the older age group, with a median age of 61 years at presentation and are usually aggressive....

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  • ...However, many patients can be asymptomatic.[1,2,7] In spite of presenting with typical symptoms and signs, it is rarely suspected as the first diagnosis and more common causes of abnormal uterine bleeding (AUB) such as leiomyoma, endometrial polyps, or submucous fibroid are considered as the etiology....

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  • ...Furthermore, because of similarity between ESS and normal endometrial tissue curettage fragments, the diagnosis of ESS can be missed.[2] Ultrasound, especially, transvaginal can be helpful in diagnosing uterine leiomyoma, adenomyosis, or endometrial polyps but unpredictable in diagnosing ESS....

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  • ...[1] The annual incidence of ESS is 1–2/million women.[2] Past exposure to pelvic radiation, exposure to tamoxifen, unopposed estrogen, and polycystic ovary syndrome are implicated in the pathogenesis of ESS....

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  • ...High grade or undifferentiated stromal sarcoma.[2,3]...

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