A High-Grade Undifferentiated Endometrial Stromal Sarcoma Presenting as Inversion of the Uterus: A Rare Case.
TLDR
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.read more
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Journal ArticleDOI
Low-grade endometrial stromal sarcoma recurring with multiple bone and lung metastases: report of a case
TL;DR: This is the first detailed reported case of multiple bone recurrence in a patient with low-grade ESS, and the long-term follow-up after treatment is recommended.
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Localized high grade endometrial stromal sarcoma and localized undifferentiated uterine sarcoma: a retrospective series of the French Sarcoma Group
Marie Meurer,Anne Floquet,Isabelle Ray-Coquard,François Bertucci,M. Auriche,A Cordoba,Sophie Piperno-Neumann,Sébastien Salas,M. Delannes,T Chevalier,Antoine Italiano,J.-Y. Blay,Julien Mancini,Patricia Pautier,Florence Duffaud +14 more
TL;DR: Adjuvant radiotherapy and adjuvant chemotherapy appear to improve overall survival and total hysterectomy and bilateral oophorectomy is the standard treatment for high grade endometrial stromal sarcoma and undifferentiated uterine sarcomas.
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Low-grade endometrial stromal sarcoma of the endocervix. Report of a case and review of the literature.
TL;DR: This case represents an extrauterine low-grade ESS arising in the endocervix, where a problem in the differential diagnosis was encountered and which was finally treated only with surgery.
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Hormonal treatment of endometrial stromal sarcomas (ESS): A possible indication for aromatase inhibitors
T. Lindner,Daniel Pink,Albrecht Kretzschmar,A. Mrozek,Peter C. Thuss-Patience,Peter Reichardt +5 more
TL;DR: A rare disease with probably less than 250 new cases in the US or EU per year often incidentally discovered after hysterectomy (Hy), ESS usually express steroidal receptors.