Pleomorphic high grade endometrial stromal sarcoma with YWHAE gene amplification may be a novel variant with poor prognosis.

Endometrial stromal neoplasms are classified by the World Health Organization (WHO) into endometrial stromal nodule (ESN), low grade (LGESS), high grade (HGESS), and undifferentiated uterine sarcoma (UUS). HGESS is subclassified based on molecular findings, YWHAE or BCOR. The HGESS with YWHAE::NUTM2A/B (alias YWHAE::FAM22A/B) fusion usually have relatively monomorphic (as with most fusion-associated malignancies) rounded to epithelioid cells with eosinophilic cytoplasm, vesicular nuclei, nucleoli, and mitotic figures >10/10 HPF. We present a 66-year-old woman with post-menopausal bleeding found to have a heterogeneous solid-cystic uterine mass on CT who underwent total hysterectomy, bilateral salpingo-oophorectomy, omentectomy, and pelvic lymph node dissection. A 15.0×9.0 cm variegated uterine mass with hemorrhage and necrosis was identified. Histologically, the tumor was hypercellular with haphazard fascicles, microcysts, and tongue-like destructive myometrial invasion. Tumor cells exhibited marked pleomorphism and high mitotic activity with atypical mitotic figures. There was extensive cyclin-D1 and subset CD10 immunopositivity. FISH showed YWHAE amplification but without rearrangement. Interestingly, we found only two other reported cases of pleomorphic HGESS with YWHAE gene amplification upon review of 259 cases from cBioPortal database, one of which was reported as carcinosarcoma with heterologous elements. Of note, all three YWHAE amplified cases were diagnosed at high-stage and succumbed to disease within six months. Our case appears to be the third case of YWHAE-amplified pleomorphic HGESS, possibly a new variant of uterine sarcoma with aggressive biologic behavior that needs further evaluation.

Use of pembrolizumab in MSI-high uterine leiomyosarcoma; a case report and review of the literature.

•Overall prognosis of uterine leiomyosarcoma (ULMS) is poor with a low 5-year survival rate.•Microsatellite instability (MSI)-high ULMS is not well documented in current literature.•Immune checkpoint inhibitors such as pembrolizumab have been shown to have good efficacy in treating MSI-high solid tumors.•Targeting MSI-high ULMS with pembrolizumab can potentially maintain a patient's quality of life and extend overall survival.

Hepatic Hemangioma on SPECT Myocardial Perfusion Imaging.

Extracardiac uptake on SPECT MPI, seen with physiologic radiotracer distribution, benign or malignant neoplasm, infection, inflammation, or granulomatous disease, may confound the accuracy of MPI or lead to unsuspected pathology. A 38-year-old woman with end-stage renal disease on hemodialysis had SPECT MPI for renal transplant preoperative risk stratification, showing left anterior descending artery ischemia and an intense extracardiac soft tissue focus adjacent to the diaphragmatic right ventricle concerning for focal infection related to prior coronary or gastric surgeries or tumor. CT revealed focal herniation of liver containing a flash-fill hemangioma into the left hemithorax accounting for this focal uptake.