Endometrial stromal sarcoma - An aggressive uterine malignancy

Endometrial stromal sarcoma - An aggressive uterine malignancy

Clinical history: A 42yr nulliparous female with Chronic Kidney Disease presented with acute urinary retention.She also had constipation since 1 month and irregular per-vaginal bleeding since 3 months. Sonography done 7 months back had shown thickened endometrium of ~20mm for which she was not willing for further evaluation. Imaging findings: Sonography was performed as preliminary assessment and showed a large well-defined heterogeneous mass lesion distending the endometrial cavity. Transabdominal Sonography showed a large mass lesion (11x9x10cms) in endometrial cavity showing heterogenous echogenicity extending through cervical canal into the vaginal cavity. Good internal vascularity appreciated on Colour Doppler. MRI revealed large lobulated polypoidal mass in the endometrial cavity from fundo-posterior wall prolapsing into the vagina. Posteriorly mass was infiltrating the cervix / vaginal vault with extra-uterine extension. Multiple linear T2 hypointense bands and diffusion restriction noted in the mass. There was compression on the urinary bladder and distal ureters causing bilateral hydroureteronephrosis. Rectosigmoid was indented with maintained fat planes. There was no evidence of pelvic or abdominal lymphadenopathy. Diagnosis of large endometrial malignant polyp was made. Contrast enhanced MRI was not performed due to CKD status. Histopathology proved it as undifferentiated endometrial stromal sarcoma Discussion: Endometrial Stromal Sarcoma(ESS) is a very rare neoplasm with literature showing incidence of about 0.2% of all uterine malignancies and 10-15% of all uterine mesenchymal malignancies. As far as the case is concerned, such a case has not been published so far in JRCR. Low grade ESS are common in middle aged females with mean age of 39yr and the high grade ESS are seen in older age group with mean age of 61yr. In our case High-grade ESS (USS) presented in a relatively younger age which was very aggressive and had rapidly progressed in few months. High grade ESS are currently known as undifferentiated stromal sarcomas. Sonography alone cannot diagnose uterine sarcomas. Low grade ones can mimic leiomyomas. MRI of ESS commonly shows polypoidal endometrial mass, heterogeneously isointense on T1 and hyperintense on T2 sequences.Low grade ESS shows variable presentation, from a polypoidal mass to an intramyometrial lesion mimicking leiomyoma which can be differentiated by lesser enhancement, extensive necrosis and peripheral hypointense rim on T2wt sequences of ESS. High grade tumors (USS) show more frequent myometrial involvement which can be well demarcated or diffusely infiltrative. Infiltrative margins may cause worm-like nodular extensions described as bag of worms appearance. Large malignant uterine tumors have varied presentation and have overlapping features due to endo-myometrial involvement. MRI is helpful in characterization of the lesions. High grade ESS (UES) is a very aggressive neoplasm with few specific f

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  • Dr.CHAITRA SANTHOSH 2015-06-23 13:08:54

    yes Dr.Prakash it has been confirmed by histopathology. I do have the slides.


  • prakash khashaba 2015-06-21 19:11:47

    whether it has been confirmed by histopathology..