{"paper_id":"e58bee53-30dd-4bca-83b4-05947193a81d","body_text":"Indian J Med Paediatr Oncol | Jan-Mar 2010 | Vol 31 | Issue 1 21\nAddress for correspondence:\nDr. Puliyath Geetha, \nDepartment of Obstetrics and \nGynaecology, SUT Academy \nof Medical Sciences, Vencode \nVattappara, Trivandrum, \nKerala, India. \nE-mail: anjali96@yahoo.com\nPuliyath Geetha,  \nV . Rajasekharan Nair, \nSwetha Singh\nDepartment of Obstetrics and \nGynaecology, SUT Academy of \nMedical Sciences, Vattappara, \nTrivandrum, Kerala, India\nproliferate endometrium. She was given symptomatic \ntreatment for menorrhagia. An ultrasound scan carried out \n4 months back, showed enlarged uterus (12 cm×7 cm×7 \ncm) with diffuse coarse heterogeneous myometrial echoes. \nA later scan showed further enlargement of  the uterus \n(16 cm×13 cm×10 cm) with the intramural fibroid \n10 cm×7 cm in posterior wall. She was advised myomectomy \nand was referred to our tertiary care institute. Her general \nand systemic examinations were normal. Abdominal and \npelvic examinations revealed a uniformly enlarged uterus, \n24 weeks size, firm in consistency, with restricted mobility. \nDoppler ultrasound showed a fibroid uterus 16 cm and \n12 cm, with normal vascularity. Endometrial aspiration \nwas performed due to suspected sarcomatous changes in \nview of  the rapid enlargement of  the uterus within the past \n4 months. Microscopy showed endometrial glands in the \nsecretory phase with neoplastic cells, suggestive of  low-\ngrade endometrial stromal sarcoma (LGESS) [Figure 1].\nA total abdominal hysterectomy with bilateral salpingo-\noopherectomy was performed through a vertical midline \nincision. The findings were a uniformly enlarged uterus \nwith normal-looking tubes and ovaries The tumor had \ninfiltrated the myometrium anteriorly. There were no \nmetastatic deposits elsewhere. The lymph nodes were \nnot enlarged. Grossly, the specimen had a yellowish tan. \nMicroscopically, no malignant cells could be demonstrated \nin the peritoneal washings. Characteristically uniform \noval and spindle-shaped cells, suggestive of  low-grade \nESS [Figure 2], infiltrating the entire thickness of  the \nINTRODUCTION\nCancers arising from mesodermal structures like muscles \nand connective tissue are called sarcomas. Sarcomas of  \nthe uterus are uncommon, and may arise from connective \ntissue, smooth muscle or the endometrial stroma. Uterine \nsarcoma is a rare form of  malignancy, occurring in 2–5% \nof  all patients with uterine malignancy, with an incidence of  \napproximately one to two cases per 100,000 women in the \ngeneral population. Endometrial stromal sarcomas (ESSs) \nare very rare malignant tumors that make up approximately \n10% of  all uterine sarcomas but only around 0.2% of  all \nuterine malignancies.\n[1]\nCaSE REPORT\nA 30-year-old lady presented with increased bleeding \nper vagina during periods since 1 year. She was a Para1, \nLive1, with last child birth 10 years back and no history of  \ncontraceptive use. She had attained menarchy at the age of  \n14, and her previous cycles were normal. However, for the \npast 1 year, her cycles were prolonged, lasting for 15–20 \ndays/2–3 months. She was investigated for secondary \ninfertility in a local hospital 1 year back and was treated \nwith clomiphene citrate for three to four cycles. A pelvic \nscan taken 11 months back showed normal-sized uterus \nwith a fibroid 3.7 cm×3.4 cm in the anterior myometrium. \nEndometrial thickness was 8 mm. A dilatation and curettage \nwas performed and microscopy showed a disordered \nA B S T R A C T\nEndometrial stromal sarcoma (ESS) is a rare malignant tumor of the endometrium, \noccurring in the age group of 40–50 years. t his is a case of low-grade ESS presenting \nas rapid enlargement of a fibroid uterus. Because of her secondary infertility, she \nwas planned for myomectomy. However, due to the high degree of suspicion of a \nsarcomatous change in the fibroid, in view of rapid enlargement of uterus within \nthe last 4 months, we planned for a preoperative endometrial aspiration. It showed \nlow-grade ESS, which was later confirmed by histopathology examination of total \nhysterectomy specimen. a s surgery was the main treatment for ESS, because of the \nproper preoperative diagnosis, we could plan the treatment accordingly. despite the \nrarity of the tumor, one has to consider the possibility of ESS in cases with presentation \nof rapid enlargement of a fibroid uterus.\nKey words: Endometrial stromal sarcoma, rapid enlargement of fibroid uterus, uterine \nsarcoma\nDOI: 10.4103/0971-5851.68848\nEndometrial stromal sarcoma\nC a S E  r E P O r twww.ijmpo.org\nArticle published online: 2021-11-19\n\n22 Indian J Med Paediatr Oncol | Jan-Mar 2010 | Vol 31 | Issue 1\nmyometrium, were noted [Figure 3]. A section from the \nright fallopian tube showed neoplastic cells in dilated \nlymphatic spaces [Figure 4]. The cervix and ovaries \nwere normal. The case was confirmed to be a low-grade \nendometrial stromal sarcoma stage 3, and she was referred \nto a regional cancer center.\nDISCUSSION\nESSs are very rare malignant tumors. Based on tumor \nmargin status and cytological features, the WHO \nhas classified endometrial stromal tumor into benign \nendometrial stromal nodule (ESN) and endometrial \nstromal sarcoma. ESN does not infiltrate the myometrium. \nThey are well circumscribed, with a pushing margin. \nESSs infiltrate the myometrium and are characterized by \nproliferation of  uniform small cells closely resembling \nthose of  endometrial stroma in the proliferative stage.\n[2] \nESS can be divided into low-grade and high-grade tumors \naccording to cell morphology and mitotic count. Low-\ngrade ESS usually occur in the young population (mean age \n39 years), contrary to the high-grade ESS (mean age  \n61 years). Low-grade ESSs have less-frequent mitosis (<3 per \n10 high-power fields) and they do not show hemorrhage and \nnecrosis. [3] Some authors have regarded high grade \nendometrial stromal sarcoma (HGESS) as an undifferentiated \nsarcoma.[4]\nMost ESSs involve the endometrium, and uterine curettage \nusually leads to diagnosis.[2] The main differential diagnosis \nof  low-grade ESS includes ESN, cellular leiomyoma and \ncellular intravenous leiomyomatosis. [5] The microscopic \nappearance of  ESS and ESN are identical. Infiltrative \nmargins and distinctive growth as worm-like cords are \nnoted in low-grade ESS, whereas the margins are well \ndemarcated in ESN. Hence, extensive sampling of  tumor \nmargins and detecting vascular invasion are extremely \nimportant in distinguishing between the two. Cellular \nleiomyomas are composed of  cells with spindle-shaped \nFigure 1: Endometrial aspiration: histopathological examination (HPE), \nendometrial glands in the secretory phase (arrow) with neoplastic cells \nsuggestive of low-grade endometrial stromal sarcoma\nFigure 3: Tumor (arrow) almost perforating right side of the myometrium\nFigure 2: HPE (high power) showing uniform oval or spindle-shaped \ncells\nFigure 4: Section from the fallopian tube showing neoplastic cells in \ndilated lymphatic spaces\nGeetha, et al.: Endometrial stromal sarcoma\n\n\nIndian J Med Paediatr Oncol | Jan-Mar 2010 | Vol 31 | Issue 1 23\nnuclei with a fascicular growth pattern, thick muscular-\nwalled vessels, cleft-like spaces and showing focal merging \nwith the adjacent myometrium.[6] In low-grade ESS, cords \nof  tumor cells infiltrate between smooth muscle and within \nlymphatic spaces. The neoplastic stromal cells resemble \nthose of  the proliferative endometrium, are monotonous in \nappearance and have a uniform size and shape. The nuclei \nare round to ovoid, with fine chromatin, and a small nucleoli \nis seen. A small amount of  cytoplasm is present and cell \nborders are indistinct. Mitotic activity is low (<10/10 high-\npower fields).\n[5] About a third to a half  of  the low-grade \nESS have extrauterine spread at the time of  diagnosis. \nWhen there is a difficulty in diagnosing between ESS and \ncellular leiomyoma, immunoreactivity with antibodies to \nCD10 and smooth muscle actin and desmin are used.\n[7]\nSurgery is the final resort for diagnosis and primary \ntreatment of  ESS. Preoperative diagnosis is mostly a uterine \nfibroid. Ultrasound and magnetic resonance imaging are \ninconclusive. If  the diagnosis is known, the extent of  \nsurgery is planned according to the stage of  the tumor. \nThe FIGO staging for carcinoma of  the corpus uteri has \nbeen applied to ESS.\nThe treatment is total abdominal hysterectomy, bilateral \nsalpingo-oophorectomy and pelvic and periaortic selective \nlymphadenectomy. Cytologic washings are obtained from \nthe pelvis and abdomen. Hormone therapy with medroxy \nprogesterone, tamoxifen, gonadotropin releasing hormone \n(GnRH)\n  analogues and aromatase inhibitors are suggested \nfor low-grade ESS stage 3–4 and for recurrent disease.[8,9]\nCONCLUSION\nBecause of  the rarity of  the tumor, ESS may not be \nfamiliar to the gynaecologists. In young patients, it \ncould be mistaken for a fibroid and more conservative \nmanagement may have been planned accordingly. High \ndegree of  suspicion in a rapidly enlarged uterus led to an \nendometrial aspiration in this case, which showed low-grade \nESS. Therefore, the treatment plan was shifted from a \nconservative myomectomy to a laparotomy, total abdominal \nhysterectomy and bilateral salpingo-opherectomy. The case \nhighlights the necessity for high degree of  suspicion and \nproper preoperative diagnosis in this rare type of  tumor, \nespecially in young patients.\nREFERENCES\n1. ashraf-Ganjoei t, Behtash N, Shariat M, Mosavi a. low \ngrade endometrial stromal sarcoma of uterine corpus, a \nclinico-pathological and survey study in 14 cases. World J \nSurg Oncol 2006;4:50.\n2. Policarpio-Nicolas Ml, Cathro HP, Kerr SE, Stelow EB. \nCytomorphologic features of low-grade endometrial stromal \nsarcoma. am J Clin Pathol 2007;128:265-71.\n3. Koyama t, togashi K, Konishi I, Kobayashi H, Ueda H, \nKataoka Ml, et al . Mr imaging of endometrial stromal \nsarcoma: Correlation with pathologic findings. AJR Am J \nroentgenol 1999;173:767-72.\n4. Amant F, Vergote I, Moerman P. The classification of uterine \nsarcoma as ‘high grade endometrial stromal sarcoma’ should \nbe abandoned. Gynecol Oncol 2004;95:412-3.\n5. Oliva E, Clement PB, Young rH. Endometrial stromal tumours: \nan update on a group of tumours with a protean phenotype. \nadv anat Pathol 2000;7:257-81.\n6. Sumathi VP, McCluggage WG. Cd10 is useful in demonstrating \nendometrial stroma at ectopic sites and in confirming a \ndiagnosis of endometriosis. J Clin Pathol 2002;55:391-2.\n7. Baker P, Oliva E. Endometrial stromal tumours of the uterus: \na practical approach using conventional morphology and \nancillary techniques. J Clin Pathol 2007;60:235-43.\n8. NCCN Clinical practice guidelines in oncology. Uterine \nneoplasms. Vol 2. 2009.\n9. linder t, Pink d, Kretzschmar a, Mrozek a, Patience PC, \nreichardt P. Hormone treatment of endometrial stromal \nsarcomas: a possible indication for aromatase inhibitors. J \nClin Oncol 2005;23:16S : 9057.\nSource of Support: Nil, Conflict of Interest: None declared.\nGeetha, et al.: Endometrial stromal sarcoma\nStaying in touch with the journal\n1) Table of Contents (TOC) email alert \n Receive an email alert containing the TOC when a new complete issue of the journal is made available online. To register for TOC alerts go to \nwww.ijmpo.org/signup.asp.\n2) RSS feeds \n Really Simple Syndication (RSS) helps you to get alerts on new publication right on your desktop without going to the journal’s website. \nYou need a software (e.g. RSSReader, Feed Demon, FeedReader, My Yahoo!, NewsGator and NewzCrawler) to get advantage of this tool. \nRSS feeds can also be read through FireFox or Microsoft Outlook 2007. Once any of these small (and mostly free) software is installed, add  \nwww.ijmpo.org/rssfeed.asp as one of the feeds.","source_license":"CC0","license_restricted":false}