The Polycomb group of proteins is required for the proper orchestration of gene expression due to its role in maintaining transcriptional silencing. It is composed of several chromatin modifying complexes, including Polycomb Repressive Complex 2 (PRC2), which deposits H3K27me2/3. Here, we report the identification of a cofactor of PRC2, EZHIP (EZH1/2 Inhibitory Protein), expressed predominantly in the gonads. EZHIP limits the enzymatic activity of PRC2 and lessens the interaction between the core complex and its accessory subunits, but does not interfere with PRC2 recruitment to chromatin. Deletion of Ezhip in mice leads to a global increase in H3K27me2/3 deposition both during spermatogenesis and at late stages of oocyte maturation. This does not affect the initial number of follicles but is associated with a reduction of follicles in aging. Our results suggest that mature oocytes Ezhip−/− might not be fully functional and indicate that fertility is strongly impaired in Ezhip−/− females. Altogether, our study uncovers EZHIP as a regulator of chromatin landscape in gametes. Polycomb Repressive Complex 2 (PRC2) plays critical roles in transcriptional silencing during development. Here the authors identify EZHIP as a cofactor of PRC2 expressed predominantly in the gonads, finding that EZHIP limits the enzymatic activity of PRC2 in germ cells in mice.

EZHIP constrains Polycomb Repressive Complex 2 activity in germ cells.

Uterine leiomyomas, the most frequent benign myomatous tumors of the uterus, often cannot be distinguished from malignant uterine leiomyosarcomas using clinical criteria. Furthermore, imaging differentiation between both entities is frequently challenging due to their potential overlapping features. Because a suspected leiomyoma is often managed conservatively or with minimally invasive treatments, the misdiagnosis of leiomyosarcoma for a benign leiomyoma could potentially result in significant treatment delays, therefore increasing morbidity and mortality. In this review, we provide an overview of the differences between leiomyoma and leiomyosarcoma, mainly focusing on imaging characteristics, but also briefly touching upon their demographic, histopathological and clinical differences. The main indications and limitations of available cross-sectional imaging techniques are discussed, including ultrasound, computed tomography, magnetic resonance imaging (MRI) and positron emission tomography/computed tomography. A particular emphasis is placed on the review of specific MRI features that may allow distinction between leiomyomas and leiomyosarcomas according to the most recent evidence in the literature. The potential contribution of texture analysis is also discussed. In order to help guide-imaging diagnosis, we provide an MRI-based diagnostic algorithm which takes into account morphological and functional features, both individually and in combination, in an attempt to optimize radiologic differentiation of leiomyomas from leiomyosarcomas.

How to differentiate uterine leiomyosarcoma from leiomyoma with imaging.

•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.

A recurrent endometrial stromal sarcoma harbors the novel fusion JAZF1-BCORL1.

Bilateral simultaneous tubal pregnancy

Fertility-sparing management of low-grade endometrial stromal sarcoma: analysis of an institutional series and review of the literature

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.

Endometrial stromal sarcoma: A review of the literature

Endometrial stromal sarcoma (ESS) is a rare malignant tumor of the endometrium, occurring in the age group of 40-50 years. This is a case of low-grade ESS presenting as rapid enlargement of a fibroid uterus. Because of her secondary infertility, she was planned for myomectomy. However, due to the high degree of suspicion of a sarcomatous change in the fibroid, in view of rapid enlargement of uterus within the last 4 months, we planned for a preoperative endometrial aspiration. It showed low-grade ESS, which was later confirmed by histopathology examination of total hysterectomy specimen. As surgery was the main treatment for ESS, because of the proper preoperative diagnosis, we could plan the treatment accordingly. Despite the rarity of the tumor, one has to consider the possibility of ESS in cases with presentation of rapid enlargement of a fibroid uterus.

Endometrial stromal sarcoma.

Bilateral ectopic pregnancies are increasing due to an increase in the incidence of pelvic inflammatory disease and increase in assisted reproductive techniques. Spontaneous conception after bilateral ectopic pregnancies is extremely rare. A 33-year-old Indian woman presented with ruptured ectopic pregnancy associated with hemoperitoneum for which she underwent a laparotomy. The diagnosis of bilateral ectopic pregnancies was made during surgery. Salpingectomy was done on one side and the other tube was conserved with salpingostomy. Six months after surgery, she conceived spontaneously and a transvaginal ultrasound examination revealed a 5-weeks live intrauterine pregnancy. A meticulous surgical technique conserving one fallopian tube resulted in subsequent viable intrauterine pregnancy. The approach to such a case along with steps to minimize tubal damage is presented.

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Geetha Puliyath

Papers

Endometrial stromal sarcoma: A review of the literature

Endometrial stromal sarcoma.

Viable intrauterine pregnancy after spontaneous bilateral tubal ectopics in a multiparous woman: a case report