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Abstract
Endometrial stromal sarcomas are rare uterine malignancy of mesodermal origin. The diagnosis is usually made post operatively. The usual presentation is abnormal vaginal bleeding, abdominal lump and mild lower abdominal pain. In this case report we present a case of low grade endometrial stromal sarcoma where the preop diagnosis was fibroid uterus with cystic degenerative changes. Total abdominal hysterectomy with bilateral salpingo oophorectomy was performed. On histopathological examination it turn out a case of low grade endometrial stromal sarcoma.
Introduction
Uterine sarcomas are relatively rare tumours of mesodermal origin. They constitute 2-6% of uterine malignancies. Of these endometrial stromal sarcomas are still rarer tumour. Preoperative diagnosis is usually fibroid uterus.
We report a case of endometrial stromal sarcoma where our preoperative diagnosis was fibroid uterus with cystic degenerative changes.
Case Report(s)
A 45 years old female P2+0 was admitted to our hospital with complaint of menorrhagea and mild lower abdominal pain for the last five months. She had periods at interval of twenty five days and bleeding lasting for ten to twelve days. Flow was excessive with passage of clots. On examination she was severely anaemic. Her hemoglobin was 4.4 gm %. On per abdominal examination there was suprapubic mass corresponding to eighteen weeks size uterus. On per speculum examination cervix was healthy. On pervaginal examination uterus was uniformly enlarged to eighteen weeks size, soft in consistency and mobile. Bilateral fornices were free. Ultrasound showed an isoechoic and hypoechoic mass measuring 104mm by 90mm in the uterus suggestive of fibroid uterus. Bilateral ovaries were normal. Our clinical diagnosis was fibroid uterus with cystic degenerative changes. Total abdominal hysterectomy with bilateral salpingo-oophorectomy was performed. Prior to surgery she was transfused three units of blood. Intraoperative uterus was enlarged to eighteen weeks size with smooth surface and soft in consistency. Bilateral ovaries were normal. Cut section of the uterus showed a mass 10 x10 cm in size filling the uterine cavity with fluid filled cystic spaces. Histopathological examination of the specimen showed low grade endometrial stromal sarcoma. Post operative period was uneventful.
Discussion
Uterine Sarcomas are rare tumours of mesodermal origin. They constitute 2 to 6% of uterine malignancies. Of these, Endometrial stromal sarcomas are very rare.
They are divided into three types depending upon mitotic activity, vascular invasion and observed differences in prognosis.
1 Endometrial stromal nodule,
2 Low grade endometrial sarcoma and
3 high grade or undifferentiated endometrial stromal sarcoma. (1)
Patients most commonly undergo surgery with presumptive diagnosis of uterine fibroid or pelvic mass. The physician should have suspicion when the histopathological diagnosis of endometrial sampling yields hyperplastic stroma with few glands. (2)
Geeta Puliyath et al reported a case of endometrial stromal sarcoma in 30 yr old female where ultrasound and Doppler findings were suggestive of fibroid uterus. Because of rapid enlargement of fibroid over short period sarcomatous change was suspected. Endometrial aspiration was performed which showed secretary endometrium with neoplastic cells and this changed their decision from myomectomy to hysterectomy. (3)
Hasiakos D et al reported a case of LGSS (Low-Grade Stromal Sarcoma) of endocervix which presented as soft haemorrhagic mass on posterior cervix looking like a degenerated fibroid. (4)
Our patient had short duration of menorrhagea of four months and ultrasound findings suggestive of fibroid with cystic degenerative changes. This shows that high index of suspicion is required to make preoperative diagnosis of endometrial stromal sarcoma particularly in fibroids with any abnormal presentation such as rapid enlargement or abnormal ultrasound findings of heterogeneous mass or fibroid with degenerative changes.
Women with LGESS (Low Grade Endometrial Stromal Sarcoma) are younger than women with other uterine sarcomas, with a median age between 45 and 57 years and, generally do not have the usual risk factors for endometrial cancer. (5)
Surgery is fundamental in LGESS (Low Grade Endometrial Stromal Sarcoma) as in other sarcomas. Treatment generally consists of total abdominal hysterectomy and bilateral salpingo-oophorectomy. Due to the high recurrence risk even with localized tumors, many clinicians advocate use of adjuvant chemotherapy, radiation therapy, and/or hormone therapy to suppress tumor growth.
The surgical stage is most significant prognostic regarding recurrence and survival in LGESS (Low Grade Endometrial Stromal Sarcoma). They tend to grow slowly and commonly recur many years after initial diagnosis. (6)
Postoperative pelvic radiotherapy reduces local recurrence but has not been consistently shown to prolong the survival.
Conclusion
Endometrial stromal sarcomas are very rare tumors of mesodermal origin presenting with abnormal uterine bleeding, mostly in perimenopausal women. The usual pre operative diagnosis is fibroid and the diagnosis is made after histopathological examination. High index of suspicion of sarcoma in uterine tumors with the features not typical of fibroid can make the preoperative diagnosis of uterine sarcomas and hence better management. Our patient also had uterine tumor with not typical features of fibroid where we thought it to be fibroid with cystic degenerative changes and it came out low grade endometrial sarcoma.
References
1. Berek JS(ed). Novaks Gynaecology 14th edn. Philadelphia, Lippincott Williams and Wilkins. 2006 :1382-84.
2. Mert G, Aydin C, Cenk MG, Ali B, Seyran Y. A Case of Malignant Low Grade Endometrial Stromal Sarcoma and Review of the Literature: J Turkish german gynecol assoc.2004; 5(1):73-75.
3. Puliyath G, Nair RV, Singh S .Endometrial stromal sarcoma, A case report: Indian Journal of Medical and Paediatric Oncology.2010; 31(1):21-23.
4. Hasiakos D, Papkonstantinou K, Kondi-Paphiti A, Fotiou S. Low grade endometrial stromal sarcoma of the endocervix. Report of a case and review of literature: Eur J gynaecol Oncol.2007; 28(6):483-6.
5. Ganjoei TA, Behtash N, Shariat M, Mosavi A.Low grade endometrial stromal sarcoma of uterine corpus, a clinicopathological and survey study in 14 cases. World J Surg Oncl 2006; 4:50.
6. Mukhopadhyay P, Sharma P.P, Muraleedharan P.D, Sarkar S: A case of endometrial stromal sarcoma: The journal of obstetrics and gynecology of India. 2008;58(1):73-74.
Source(s) of Funding
Nil
Competing Interests
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