Journal of Genital System & DisordersISSN: 2325-9728

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Case Report, J Genit Syst Disor Vol: 1 Issue: 1

Uterine Sarcoma Presenting in the Setting of Non-puerperal Uterine Inversion with Uterine Prolapse

Shireen Madani Sims*, Lauren Cooper, Sharon Byun and John Davis
Department of Obstetrics and Gynecology University of Florida, Gainesville, Florida, USA
Corresponding author : Shireen Madani Sims
Department of Obstetrics and Gynecology, University of Florida College of Medicine, PO Box 100294, Gainesville, 32610-0294 FL, USA
Tel: 352-273-7660; Fax: 352-392-3498
E-mail: [email protected]
Received: September 04, 2012 Accepted: September 25, 2012 Published: September 27, 2012
Citation: Sims SM, Cooper L, Byun S, Davis J (2012) Uterine Sarcoma Presenting in the Setting of Non-puerperal Uterine Inversion with Uterine Prolapse. J Genit Syst Disor 1:1. doi:10.4172/2325-9728.1000102

Abstract

Uterine Sarcoma Presenting in the Setting of Non-puerperal Uterine Inversion with Uterine Prolapse

Puerperal uterine inversion occurs in approximately one out of every 30,000 vaginal deliveries. Non-puerperal uterine inversion is rare with no reliable estimate of frequency in the literature; it is almost always associated with a polypoid uterine tumor. Although the most common cause of non-puerperal uterine inversion is a leiomyoma, a high index of suspicion for a coexisting malignancy must be maintained. Herein, we present a case of a non-puerperal uterine inversion in the setting of a uterine sarcoma that was accompanied by complete prolapse of the uterus into the vagina.

Keywords:

Introduction

Puerperal uterine inversion occurs in approximately one out of every 30,000 vaginal deliveries. Non-puerperal uterine inversion is rare with no reliable estimate of frequency in the literature; it is almost always associated with a polypoid uterine tumor [1]. Although the most common cause of non-puerperal uterine inversion is a leiomyoma, a high index of suspicion for a coexisting malignancy must be maintained. Herein, we present a case of a non-puerperal uterine inversion in the setting of a uterine sarcoma that was accompanied by complete prolapse of the uterus into the vagina.

Case

A 29-year-old nulliparous woman presented to our Emergency Department (ED) with a mass protruding from her vagina and heavy vaginal bleeding. Her medical history was significant for morbid obesity, with a body mass index (BMI) of 50.
The patient’s pelvic examination demonstrated a 10×15 cm necrotic mass protruding from her vagina. An MRI of the pelvis demonstrated an inverted uterus prolapsing into the vagina with retraction of the ovaries to the midline; a soft tissue mass presumed to be the uterus was seen in the vagina (Figure 1). The patient was taken to the operating room where an examination under anesthesia showed a large, necrotic, fungating mass protruding from an inverted uterus that had prolapsed into the vagina. The mass was difficult to distinguish from the uterus and the cervix was not identifiable. Excisional biopsy of the mass was performed. Pathologic evaluation demonstrated endometrial stromal sarcoma (ESS) with mild nuclear atypia and mitosis up to 2 per 10 high powered fields (HPF). The tumor was positive for estrogen and progesterone receptors, and heterologous cartilage and adjacent smooth muscle were identified. SMA was negative, and there was scattered CD68 reactivity and strong CD10 reactivity.
Figure 1: MRI of the pelvis demonstrates the classic U-shaped uterus associated with inversion. In this case, the uterus is prolapsed and located in the vagina.
One week later, the patient underwent a total abdominal hysterectomy, bilateral salpingo-oophorectomy, bilateral pelvic lymph node dissection, appendectomy, and omental biopsy by members of our Gynecologic Oncology division. A prolapsed, inverted uterus was identified at the time of surgery (Figures 2 and 3). Histopathological evaluation showed no residual tumor in the uterus, and there was no evidence of metastatic disease in any of the other specimens. The Gynecologic Oncology division recommended the patient not receive adjuvant therapy. She had an unremarkable postoperative course. At her 18-month follow-up appointment, the patient was doing well without evidence of recurrent disease.
Figure 2: Prolapsed, inverted uterus encountered at the time of surgery. The uterus has prolapsed into the vagina and is not visible in the pelvis. The ovaries are retracted to the midline.
Figure 3: Surgical specimen of the inverted uterus after removal.

Discussion

This report presented a case of a non-puerperal uterine inversion in the setting of a uterine sarcoma that was accompanied by complete prolapse of the uterus into the vagina. Uterine sarcomas account for 3-7% of uterine cancers and are typically found in women aged 45-55 years [2]. The World Health Organization (WHO) recognizes three categories of endometrial stromal tumors: endometrial stromal nodule (ESN), low grade endometrial stromal sarcoma (ESS), and undifferentiated endometrial sarcoma (UES) [3]. Endometrial stromal sarcomas account for 10% of all uterine sarcomas. They are typically low grade and have a good long term prognosis [4]. Patients with ESS may be asymptomatic, or they may present with vaginal bleeding or pelvic or abdominal pain [3].
There is no reliable estimate of the frequency of non-puerperal uterine inversion in the literature, but the condition is typically associated with a polypoid mass. Leiomyomas (71.6%), sarcomas (13.6%), and carcinomas (6.8%) have all been found present with this condition. No etiology is identified in 8% of affected patients [5]. Approximately 23 cases of non-puerperal uterine inversion due to sarcoma have been reported in the literature. To our knowledge, this is the first such case report where uterine inversion caused by a sarcoma was accompanied by complete uterine prolapse into the vagina [6]. The exact mechanism of non-puerperal uterine inversion is unknown. One theory is that contraction of the uterine musculature due to tumor prolapsing into the vagina combined with tumor-related weakening of the uterine wall results in inversion [1]. Nulliparity may also cause a reduction in thickness of the uterine wall [6]. The most common signs of non-puerperal uterine inversion are bleeding, lower abdominal or pelvic pain, and the presence of vaginal mass [1]. Anuria has also been reported when the bladder is pulled into the uterine wall [6].
Non-puerperal uterine inversion may be diagnosed by physical examination, radiologic studies, or surgical exploration. Pelvic examination may not be consistently reliable for making the diagnosis of inversion; however, the condition should be suspected when a tumor is palpable in the vagina but the fundus is not palpable and the cervix is not identifiable [6]. MRI is useful for diagnosing inversion and will often demonstrate a U-shaped uterine cavity with ovaries retracted to the midline [5]. The diagnosis of a coexisting malignancy is usually made at the time of surgery.
There is no standard treatment for uterine inversion due to uterine sarcoma but hysterectomy, bilateral salpingo-oophorectomy, and pelvic lymph node dissection have been recommended. Adjuvant radiation therapy, chemotherapy and/or hormonal therapy may be appropriate for some patients depending on the extent of disease [2].
Non-puerperal uterine inversion occurs infrequently and, when encountered, a high index of suspicion for malignancy must be maintained. Affected patients usually present with vaginal bleeding and a mass in the vagina protruding through the cervix. Our case provides one more example of how this rare condition may present and adds to the few cases reported in the literature. This is the first case where tumor-related non-puerperal uterine inversion was also associated with prolapse of the uterus into the vagina.

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