•Extra-uterine endometrial stromal sarcoma may arise in endometriosis.•Abdominal exploration for extra pelvic endometriosis is warranted.•Representative endometriotic implants should be resected and/or biopsied if clinically suspicious.

•Genomic alterations may improve diagnostic certainty and subsequent treatment of endometrial stromal sarcoma.•Novel JAZF1-BCORL1 mutation was identified.•Targeted therapeutics to down-stream targets may improve survival benefit in these patients.

The World Health Organization (WHO 2003) recognizes 3 endometrial stromal neoplasms: noninvasive endometrial stromal nodule and the 2 invasive neoplasms, endometrial stromal sarcoma (ESS), low grade and undifferentiated endometrial sarcoma (UES). It is important to note that the WHO 2003 does not define moderate atypia (an important differentiating diagnostic criterion for ESS, low grade and UE...

BACKGROUND Endometrial stromal tumours with sex-cord-like elements were first described by Clement and Scully in 1976. The recent World Health Organisation classification 2003 recognises low grade stromal sarcoma and undifferentiated endometrial sarcoma. CASE PRESENTATION A 62-year-old caucasian woman presented with recurrent postmenopausal bleeding. She underwent a diagnostic hysteroscopy wh...

A 71-year-old woman presented with a right adnexal solid mass invading the right gonadal vein and inferior vena cava up to the hepatic veins revealed by CT and confirmed by MRI. A thin-walled cyst and a solid mass were unexpectedly found in the right atrium by transesophageal echocardiography (TEE) in the operating room. Using color Doppler and air bubbles as contrast material a circumscribed c...

Endometrial stromal sarcoma is a rare condition, constituting nearly 0.2% of all reproductive tract malignancies. The condition mainly affects the peri-menopausal population; however, it can occasionally be found in younger women and adolescents. Here, this case 20-year-old primiparous female who was referred to us with main complaint vaginal bleeding (menometrorrhagia), ongoing for 6 months af...