Introduction
Uterine sarcomas are a heterogenous group of rare tumours of the uterine musculature and uterine connective tissue. Low grade endometrial stromal sarcomas (LG-ESS) are a type of uterine sarcoma which represent only around 0.2% of all uterine malignancies, but they make up approximately 7-25% of uterine sarcomas.1 The World Health Organisation (WHO) classifies the endometrial tumour as benign endometrial stromal nodules (ESN) and Endometrial stromal sarcomas (ESS). ESN are benign because they do not infiltrate the endometrium whereas the ESS are known to infiltrate into the myometrium.2 It usually affects a mean age group of 42-52yrs.3 It presents usually in perimenopausal women with abnormal uterine bleeding and enlarged uterus. A high suspicion index is required for diagnosis because they share similar clinical features with uterine leiomyomas making the diagnosis postoperatively and after histopathological evaluation.4 Low-grade endometrial stromal tumour usually appear irregular, and a yellow mass involving myometrium or endometrium with focal necrosis. It appears cystic and haemorrhagic changes are seen.5 There are chances of reoccurrence later in life.
This case report describes the endometrial stromal tumor mimicking Uterine Leiomyoma in a young, nulliparous woman who underwent Myomectomy in view of Uterine Leiomyoma.
Case Report
A 34-years young, nulliparous woman from lower middle class background presented to Outpatient department as a 34 weeks size mass abdomen which was of 6 months duration. She attained menarche at 14 years, her menstrual cycles were regular. She has been married for 6 months. Her general examination was normal. Her Cardiovascular and respiratory systemic examination was normal. Abdominal examination revealed a uniformly enlarged uterus corresponding to 34 weeks size, firm in consistency and immobile. Per speculum examination revealed a healthy cervix. On bimanual per vaginal examination mass felt was firm and globular. Cervix was high up, uterus uniformly enlarged in size, immobile, nontender, No forniceal tenderness. Baseline Blood investigations were done and were normal. Ultrasound revealed a 20 x 21.2 cm huge subserous fibroid. MRI revealed a well demarcated, pedunculated mass in the uterine cavity. She was planned for myomectomy and the intraoperative findings were, a huge mushroom shaped firm mass was seen arising from the fundus of the uterus with a pedicle which was deep-rooted into the uterus. Uterus was bulky with normal bilateral adnexa. The mass was excised and uterine myometrium was closed. Histopathological examination of mass confirmed as a Low-grade Endometrial stromal sarcoma. Subsequently there was no metastatic lesion found on CT examination.
Discussion
Endometrial stromal sarcoma is rare mesodermal tumor.6 It is one of the three common variant and the other variant is leiomyosarcoma and mixed mullerian tumors.7 Diagnosis is tricky and difficult due to its similarity with Uterine leiomyoma in clinical presentation and imaging techniques. The symptoms are nonspecific, mostly abnormal uterine bleeding and pain.8 Uterine sarcomas most often affect postmenopausal women. An early diagnosis is essential because patient’s survival is directly related to the tumor stage.9 Our patient presented at 34 years which is a rarity by itself. The definitive management of Endometrial stromal tumor is Surgical, both to establish the diagnosis as well as for the treatment.10 The definitive treatment is Total Abdominal Hysterectomy with Bilateral Salphingo Oophorectomy and excision of all grossly detectable tumor.11
Treatment
Patient was planned for Myomectomy after doing all baseline investigations, pre operative orders were carried out after obtaining informed and written consent. Myomectomy was done and the tumor was excised and sent for Histopathological examination. She was referred to Oncology for further follow up. Since the patient was young and newly married, she was counselled to plan for conception after 6 months of post-operative period.
Conclusion
ESS is a rare malignant tumor. The usual preoperative diagnosis is uterine Leiomyoma and definitive diagnosis is achieved only after histopathology of uterus. By reporting our case, we wish to stress the necessity for a high degree of suspicion to diagnose this tumor even in younger women without any menstrual irregularity. A prompt diagnosis and timely intervention are keys to improve patient’s survival.
