Extra-Uterine Low-Grade Endometrial Stromal Sarcoma Presenting as a Urinary Bladder Mass: a Case Report with Review of t
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CASE REPORT
Extra-Uterine Low-Grade Endometrial Stromal Sarcoma Presenting as a Urinary Bladder Mass: a Case Report with Review of the Literature Subhash Yadav 1
&
Menon Santosh 1 & Ganesh Bakshi 2 & Desai Sangeeta 1
Received: 1 March 2019 / Accepted: 3 July 2019 # Indian Association of Surgical Oncology 2019
Introduction Endometrial stromal sarcoma (ESS) is a rare uterine neoplasm accounting for 0.2% of all uterine malignancies and around 15% of all uterine sarcomas. ESS is a malignant tumor which originates from invasive proliferation of cells that resemble stromal cells of the normal proliferative endometrium. Patients typically present with abdominal pain and uterine bleeding. Based on their biologic behavior and molecular aberrations, the latest World Health Organization guideline (WHO 2014) classifies the endometrial stromal tumors into endometrial stromal nodule, low-grade endometrial stromal sarcoma (LG-ESS), high-grade endometrial stromal sarcoma (HG-ESS), and undifferentiated endometrial sarcoma (UES) [1, 2]. LG-ESSs usually have low malignant potential with an indolent clinical course. However, they are well known for their late recurrences even in patients with stage I disease. More than one-third of patients with ESS develop a recurrence which necessitates a long-term follow-up. The most common sites of involvement/ recurrences include the ovary, pelvis, abdomen, and less frequently lung and vagina. Extra-uterine ESS (EESS) is a well-documented entity in the literature; however, it is a rare occurrence as a result of malignant transformation of endometriotic implants [3, 4]. ESS in the urinary bladder is extremely rare with less than 10 cases reported in literature till now to the best of our knowledge [5]. We hereby present a case of urinary
* Subhash Yadav [email protected] 1
Department of Surgical Pathology, Tata Memorial Hospital, Mumbai, Maharashtra, India
2
Department of Surgical Oncology, Tata Memorial Hospital, Mumbai, Maharashtra, India
bladder ESS with its histological, immunohistochemical, and molecular features compatible with a low-grade ESS (LG-ESS).
Case History A 45-year-old female with no comorbidities presented with history of hematuria since 2 months. Details of past history of hysterectomy done were not available. On contrastenhanced computerized tomography (CECT), a 7.5 × 5.5 cm irregularly enhancing mass was seen arising from the left lateral wall of the urinary bladder with a large extra-luminal component extending antero-laterally and abutting the soft tissue of the anterior abdominal wall. There was an associated tumor thrombus in the iliac veins. The left ovary was normal. The right ovary was not seen. Urine cytology was negative for malignancy. Subsequently, a limited transurethral resection of bladder tumor (TURBT) was done which revealed a tumor composed of round to ovoid and focal spindle cells arranged in diffuse sheets in a myxoid and hyalinized matrix with minimal pleomorphism. Mitosis was 1–2/10 high-power field (hpf). Necrosis was absent. On immuno
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