A high-grade undifferentiated endometrial stromal sarcoma presenting as inversion of the uterus: A rare case

Abstract

Endometrial stromal sarcoma (ESS) is a rare malignant tumor that constitutes about 0.2% of all uterine malignancies and 10% of uterine sarcomas. ESS is generally misdiagnosed as leiomyoma or endometrial polyp and typically discovered on histopathological examination postoperatively because of its rarity. Endometrial stromal tumors are composed of cells resembling normal endometrial stroma in its proliferative phase. The histologic diagnosis of the high grade is made if there is a high-grade sarcoma with a high mitotic index and nuclear anaplasia. The mean age of presentation of high-grade endometrial sarcoma is about 61 years with the most common presenting complaint is menorrhagia. The median overall survival for high-grade endometrial sarcoma is 53 months with optimal cytoreduction. A 49-year-old woman P2 L2 presented with nonspecific complaint of discharge and spotting per vaginum. In the present case, the provisional diagnosis by clinical findings as well as imaging was in favor of the inversion of submucous fibroid. Preoperative histopathological examination and immunohistochemistry confirmed the diagnosis of high-grade undifferentiated ESS. Haultain’s operation followed by total abdominal hysterectomy and bilateral salpingo-oophorectomy was performed. The patient was referred to another center for radiotherapy. From there, she was lost to follow-up. Rarity of endometrial stromal tumor limits the clinician view to diagnose it preoperatively. We were fortunate to have preoperative histopathological diagnosis of ESS. Furthermore, as ESS is rare and undifferentiated stromal sarcoma is even rarer, literature is lacking on its optimal management. Hence, it is important for all clinicians to keep the high degree of suspicion for ESS while working up any case of abnormal uterine bleeding.

Sarcoma del estroma endometrial, patología, diagnóstico, manejo y tratamiento. (Revisión Teórica)

“…El sarcoma del estroma endometrial (ESS) es un tumor maligno poco común, de mal pronóstico y alta mortalidad, que constituye alrededor del 0,2% de todas las neoplasias uterinas y el 10% de los sarcomas uterinos, se diagnostican erróneamente como leiomioma o pólipo endometrial y se descubren en el examen histopatológico postoperatorio o en histerectomía por otras causas; están compuestos por células que se asemejan al estroma endometrial normal en su fase proliferativa [21].…”

“…Según la revisión de Nalini et al [21], alrededor del 10 al 25 % de mujeres afectadas son premenopáusicas, con una edad promedio entre 42 a 55 años, mientras que el HG-ESS se observa frecuentemente en mujeres posmenopáusicas con una mediana de edad de 61 años en el momento de la presentación y suelen ser altamente agresivas [21].…”

“…Se pueden observar metástasis en los ganglios linfáticos hasta en el 10% de las pacientes, los sitios más frecuentes de diseminación son dentro de la pelvis o la vagina, seguidos de los pulmones en la mayoría de las series de casos. Las tasas de recurrencia son variables, pero en promedio, se observa en aproximadamente un tercio a la mitad de las pacientes, con una tasa más baja si se encuentra en estadio I. El tiempo hasta la recurrencia puede variar de 3 meses a 23 años, con un promedio de 65 meses, por lo tanto, se recomienda obligatoriamente un seguimiento a largo plazo [20][21][22][23][24][25][26][27][28][29][30].…”