Endometrial stromal sarcomas (ESS) are rare tumors of endometrial stromal origin that account for approximately 1% of all uterine malignant neoplasms and are responsible for a significant proportion of mortality due to uterine malignancies.The basic characteristic of these tumors is that they remain in disguise unless proven by histopathology after hysterectomy and hence often leads to preoperative misdiagnosis. Even dilatation and curettage or endometrial biopsy fails to diagnose it as the curetting is taken from the endometrium and ESS tends to grow toward the myometrium.
A 55‑year‑old female,presented with postmenopausal spotting on and off for the past 6 months, cough with expectoration, and significant weight loss for past 2 months.She was postmenopausal for 3 years.
On examination, her general condition was poor. She was cachexic with weight 27 kg and body mass index of 15 kg/m2 .Abdominal examination revealed a lump corresponding to 14 weeks size gravid uterus, hard in consistency, and restricted mobility in the suprapubic region.On bimanual examination, a hard mass was felt, which was nonmobile and nontender. Her hemoglobin was 7.1 g%. Ultrasonography showed evidence of a well‑defined heterogeneous hypoechoic mass lesion 20 mm × 17 mm in part of the myometrium of the fundal region likely intramural fibroid and bulky uterus.
However, the magnetic resonance imaging (MRI) report was suggestive of endometrial carcinoma involving more than half of the myometrium. CT report suggestive of traction bronchiectasis and discrete random nodules with feeding vessel signs in the right lower lobe of lung, likely metastases.
Surprisingly, there was an enlargement of uterine mass to approximately 20 weeks in 2 weeks duration with increase in pain abdomen and fall in hemoglobin to 5.2 g %. A repeat ultrasonography was done which showed a large lobulated mass lesion with heterogeneous echotexture, size 13.7 cm × 7.3 cm.The patient complained of severe pain in the left leg with unilateral edema.Seeing the rapid growth of mass and falling hemoglobin, a working diagnosis of leiomyosarcoma was made.
intraoperatively, the mass was up to the umbilicus, adherent to the omentum and sigmoid colon. It had actually ruptured inside the abdomen with tumor tissue and necrosed material adherent to the bowel, abdominal wall, and lateral pelvic wall. Adhesiolysis was done. Total abdominal hysterectomy with bilateral salpingo‑oophorectomy and infracolic omentectomy was done. The specimen was sent for histopathology. The resected specimen measured 13 cm × 13 cm × 4 cm. The outer surface was congested with multiple surface deposits. Cut section showed dilated endometrial and endocervical cavity. Endometrial cavity was filled with friable gray–brown growth. Multiple small growths were also seen in the myometrial cavity
Conclusively, High‑grade endometrial sarcomas are rare and aggressive tumors having a very poor prognosis. Early diagnosis in the initial stages is of paramount importance to increase the overall survival.
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