Endometrial stromal sarcoma: An aggressive uterine malignancy

Abstract

Endometrial stromal sarcoma (ESS) is an aggressive uterine sarcoma. We report a case of a large endometrial stromal sarcoma in a 42 year nulliparous woman with chronic kidney disease presenting with acute urinary retention and irregular per vaginal bleeding. Ultrasound and Doppler imaging revealed a heterogeneous mass in the endometrial cavity with internal vascularity. Magnetic resonance imaging (MRI) revealed a large lobulated mass in the endometrial cavity extending into the vagina, causing local mass effect. Multiple linear hypointense bands on magnetic resonance T2 weighted (T2wt) images were characteristic of ESS. MRI is a very useful imaging modality in characterizing the lesion and also for the staging. It is necessary to distinguish these tumors from benign as well as other uterine malignancies for better management. We also review relevant literature discussing imaging findings of ESS.

Keywords: Endometrial stromal sarcomas; MRI; Uterus; high grade sarcomas; myometrial bands; undifferentiated endometrial sarcomas; uterine mesenchymal neoplasms.

Figure 1

42 year old female with endometrial stromal sarcoma Findings: Heterogeneous echogenic mass within the endometrial cavity is shown (red asterisk) which is causing distension and extending into the cervix (white arrow) and vagina (red arrow). Technique: B-mode Transabdominal sonogram of the pelvis. Longitudinal section of uterus. Ultrasonography was performed using 3–5MHz curvilinear transducer.

Figure 2

42 year old female with endometrial stromal sarcoma Findings: Transabdominal sonogram of transverse section of the uterus showing enlarged uterus with a heterogeneous mass in the endometrial cavity showing internal vascularity (red arrow). Technique: Power Doppler performed using 3–5MHz curvilinear transducer.

Figure 3

42 year old female with endometrial stromal sarcoma Findings: Transabdominal sonogram of longitudinal section of both kidneys shows dilatation of pelvicalyceal system and ureters indicating hydroureteronephrosis (red arrows). Technique: Transabdominal sonogram of the abdomen at lumbar regions performed using 3–5MHz curvilinear transducer.

Figure 4

42 year old female with endometrial stromal sarcoma Findings: Coronal T1Wt image showing enlarged uterus with hypointense mass extending up to vagina distending the cervical canal (white arrows). Technique: Philips 1.5 Tesla MRI, Coronal T1Wt image of pelvis, TR/TE - 612ms/10ms, 4.0mm slice thickness, noncontrast. FOV - 240. Matrix - 320 × 320.

Figure 5

42 year old female with endometrial stromal sarcoma Findings: Coronal T2Wt image showing a large heterogeneous hyperintense mass in the endometrial cavity extending from the fundal region up to the vagina, causing distension of the cervical canal (white arrows). Uterus appears superiorly displaced by the mass. Technique: Philips 1.5 Tesla MRI, Coronal T2Wt image of pelvis, TR/TE - 2800ms/90ms, 4.7mm slice thickness, noncontrast. FOV - 240. Matrix - 320 × 320.

Figure 6

42 year old female with endometrial stromal sarcoma. Findings: Axial Fat suppressed T2Wt image of the pelvis at the level of the uterine fundus showing enlarged uterine endometrial cavity with a hyperintense lesion arising from the posterior aspect of the endometrium (red asterisk). The endo-myometrial junction is not clearly delineated in the posterior aspect (red arrow). Technique: Philips 1.5 Tesla MRI, Axial Fat suppressed T2Wt image of the pelvis, TR/TE - 3700ms/70ms, 5mm slice thickness, noncontrast. Fat suppression technique - SPAIR. FOV - 240. Matrix - 320 × 320.

Figure 7

42 year old female with endometrial stromal sarcoma. Findings: Sagittal Fat suppressed T2Wt image of the pelvis showing a large, heterogeneously hyperintense large mass arising from the fundal endometrial cavity (yellow asterisk) extending into the cervix (white arrow) and vagina (red arrow). Few hypointense linear bands are noted which are characteristic for ESS. Note the posterior wall infiltration beyond the cervix (yellow arrow). Technique: Philips 1.5 Tesla MRI, sagittal Fat suppressed T2Wt image of the pelvis, TR/TE-3700ms/70ms, 5mm slice thickness, noncontrast. Fat suppression technique - SPAIR. FOV - 240. Matrix - 320 × 320.

Figure 8

42 year old female with endometrial stromal sarcoma Findings: Axial Diffusion weighted image showing high signal intensity mass within the endometrial cavity (red asterisk). Technique: Philips 1.5 Tesla MRI, Axial DWI of pelvis, b-factor =800s/mm2, TR - 2000ms, TE - 60ms, 5.0mm slice thickness. FOV - 240. Matrix- 192 × 192

Figure 9

42 year old female with endometrial stromal sarcoma. H & E 40× showing diffuse sheets of cells showing fine chromatin with occasional prominent nucleoli with mitotic figures (blue arrows).

Figure 10

42 year old female with endometrial stromal sarcoma. H & E 10× showing uniform oval to spindle shaped cells with scant to moderate cytoplasm.

Figure 11

42 year old female with endometrial stromal sarcoma, postoperative. Findings: CT abdomen showing soft tissue density nodules along the inner aspect of parietal peritoneal layer suggesting peritoneal spread. Technique: Multidetector 128 slice Computed tomography of the abdomen and pelvis, 120kVp, Auto mAs, 1mm slice thickness, noncontrast.