Introuction

The incidence of leiomyosarcoma in patients with leiomyomas is reported to be 0.2 percent ₁. Leiomyomas can cause infertility either by mechanical obstruction or interference with implantation. But leiomyosarcoma presenting with infertility is unique. Management of such a rare case is discussed here.

Case Report

A 35 year old female was admitted in the Department of Obstetrics and Gynecology for investigation and management of infertility. She was married for four years. Her menstrual cycles were regular with average flow and there was no history of dysmenorrhoea. Her general and systemic examination were clinically within normal limits. On per abdomen examination there was a suprapubic pelvic mass of 14 weeks pregnant uterus size. Per speculum showed healthy vagina and cervix. Bimanual examination also revealed irregularly enlarged uterus corresponding to the size of 14 weeks pregnant uterus.

On investigation the haematological study, liver and kidney function tests were within normal limits. Endometrial histopathology showed secretary pattern. On ultrasonography the uterus showed multiple fibroids, the largest being seven × six cm size on right cornual part and ovaries were normal with normal follicles. Considering her infertility hysterosalphingography was done after the exclusion of tuberculosis following endometrial acid fast bacilli culture study. The hysterosalphingography and laparoscopic chromopertubation showed left fimbrial and right cornual block of fallopian tubes. A course of Danazol theray was administered for three months with the view to reduce the vascularity and size of myoma prior to surgery. Exploratory laparatomy with myomectomy and tuboplasty was planned.

On laparotomy a total of five myomas could be seen. Two myomas, one of the size four × three cm and the other of three × three cm were present on the anterolateral wall of uterus at the cornu and two subserous sessile fibroid of size two × three cm on lateral wall of uterus were seen. There was difficulty in enucleation of the right cornual fibroid which was largest of all and had poorly circumscribed capsule. The myomectomy was completed carefully with minimum blood loss. Left side fimbriolysis was done and patency of the tube was restored .The right side tuboplasty was not attempted. On gross examination the largest myoma looked hyalanized and had no whorled appearance. On histopathology (Fig.1) of the larger fibroid borderline leiomyosarcoma and the other fibroids as benign leiomyoma were diagnosed.

Figure 1

Figure 1: Microphotograph shows pleomorphic spindle cells with mitotic activity. H&E X 400.

On consultation with oncologist and pathologist, the patient was given the choice for hysterectomy or to delay the treatment for one year which would give her a chance to conceive. As the couple did not want to take any risk of waiting she underwent hysterectomy. She is now on follow up and has had a disease free interval till date.

Discussion

Melilli et al (1998) in their study of seven years reported 17 cases of uterine sarcomas where the age group ranged from 42-84 years and almost all of them were postmenopausal and pleuriparae. Sarcomatous changes is reported to occur in 0.3-0.8% of benign uterine leiomyomas and usually occur between 43-53 years of age₂ In this case there was no suspicion of sarcoma as she was young and the condition is rare. The unique features of this case being the age of the patient. This is the first case of leiomyoscarcoma presenting at such a young age. Also the rarity of this entity makes it an unique case in itself. It is reported that leiomyoma, as the sole cause of infertility accounts for less than three percent and the chance of conception following myomectomy is believed to be 40 percent₁. Medical therapy like danazol, GnRH agonist temporarily reduces the size of myoma but it is very expensive and complicated. Thus surgical treatment is preferred for symptomatic uterine myomas ₃. In this case with the aim to reduce the uterine blood flow course of danazol therapy for three months was given but she did not respond to it. Intraoperatively the tumor had ill defined capsule and there was difficulty in enucleating it during the myomectomy. The above case stresses the fact that one should suspect malignancy if medical therapy fails to reduce the size and /or if the myoma has ill defined capsule causing difficulty in enucleating it out during myomectomy. A careful clinical and histopathological diagnosis is also requird for proper management of the patient and the clinician should not be too hasty in categorizing and diagnosing the patient.

Correspondence to

Dr. Shilpaja Karpate, Flat 24,Huntersway, Enfeild, Middlesex, EN28NL,London, UK Ph. 00447515857197 Email: sjk_12@yahoo.co.in