Introduction
Uterine sarcomas constitute 1% of female genital malignancies and 3 to 7% of genital tract cancers. Endometrial sarcomas (ESS) are of two types: low-grade ESS (LG-ESS) and high-grade ESS. Usual presentation is abnormal uterine bleeding, dysmenorrhea or pelvic pain. Twenty-five percent of patients will be asymptomatic. Some patients will be diagnosed in pathology report of myomectomy specimen. Power morcellation during laparoscopic myomectomy can cause inadvertent dissemination of the tumour which will upstage the disease. Stage of tumour is an independent predictor of prognosis. Stages I and II have overall survival of 90% which falls down to 50% for stages III and IV. Not much data is available regarding prognosis and various surgical approaches. Hysterectomy with bilateral salpingo-oophorectomy remains the main stay of treatment. Some patients may need adjuvant therapy as progestational agents, aromatase inhibitor or radiation therapy.
Case 1
A 24-year-old parous lady underwent laparoscopic myomectomy with specimen retrieval by morcellation at a peripheral hospital. Two weeks later she was presented to our institution with history of profuse bleeding per vagina. Clinical examination revealed bulky uterus with a pelvic mass in the pouch of Douglas. She was further evaluated with contrast-enhanced CT scan of the abdomen and pelvis which showed a pelvic mass and multiple hypodensities in subhepatic area, paracolic gutter and omentum and along surface of bowel. The provisional diagnosis was organised hematoma or peritoneal deposits. Pathology slides were reviewed and confirmed LG-ESS.
In view of continuing recurrent bleeding per vaginum and the CT finding suspicious of intraperitoneal hematoma/multiple deposits, an emergency exploratory laparotomy was done. There was a large tumour deposit in the pouch of Douglas infiltrating uterus and sigmoid colon. There were multiple tumour deposits over omentum, small bowel serosa and bladder serosa. Complete excision of the tumour was done by hysterectomy, bilateral salpingo-oophorectomy, omentectomy, excision of all deposits anterior resection, ileal resection and anastomosis. Diversion proximal ileostomy was done to protect two-bowel resection anastomosis. Postoperative period was uneventful and discharged from hospital on 7th postoperative day. Final histopathology report confirmed disseminated LG-ESS; ER, PR-diffuse strong positive, treated with adjuvant hormonal therapy. Diversion stoma was reversed after 2 months. There was a small tumour deposit present in the mesocolon during stoma reversal. It was excised, and histologically suggestive of tumour recurrence. This shows the aggressive nature of the low-grade tumour probably due to peritoneal dissemination. The patient is continuing hormone treatment and is asymptomatic at 24 months of stoma reversal.
Case 2
A 39-year-old parous lady underwent hysterectomy with bilateral salpingo-oophorectomy for suspected fibroid uterus at peripheral hospital. Histopathological report was low-grade endometrial stromal sarcoma. Hence, she was referred to our institute. She was kept under follow-up. After a disease-free interval of seven years, the patient presented with abdominal pain. Clinical examination did not show any clinical finding suggestive of disease recurrence. An ultrasound imaging was done. It showed a 10 × 4 × 2 cm elongated mass anterior to right psoas. Computed tomography confirmed a recurrent mass abutting right ureter. Patient was planned for exploratory laparotomy. An elongated tumour mass was seen extending inside the ovarian vein reaching up to its entry into inferior vena cava. The tumour was dissected out from the retro peritoneum and excised from inferior vena cava. The final HPR confirmed endometrial stromal sarcoma recurrence. Adjuvant treatment was given with megestrol acetate. She is disease free at 4 years of second surgery.
Case 3
A 17-year-old girl with abdominal pain diagnosed as intramural fibroid of 9.5 × 8 cm at local hospital. She underwent laparotomy and biopsy from a degenerated mass. She reported to our centre in April 2010 with biopsy report of LG-ESS. Clinical examination revealed a hypogastric mass. Magnetic resonance imaging showed an irregular hyper intense mass at the fundus. Treatment option of hysterectomy with bilateral salpingo-oophorectomy was discussed with the patient and her parents. They were not willing for hysterectomy. Hence, they decided to try a course of progesterone meanwhile they would be willing for hysterectomy. She was treated with weekly hydroxyprogesterone caproate injection. She was reassessed after one month and showed decrease in size of hypogastric mass. Progesterone injection was continued for 20 months. Repeat imaging with MRI showed a hypoechoic mass of 3 × 2 cm size at fundus. Then, she proceeded with hysterectomy and bilateral salpingo-oophorectomy in February 2012. Histopathology report showed residual tumour involving full thickness myometrium and breaching serosa. Postoperative pelvic radiotherapy was given. The patient is coming for regular follow-up and is disease free at 8 years of treatment.
Case 4
A 25-year-old multiparous lady presented with menorrhagia underwent laparotomy and myomectomy. She reported to our institute with pathology report of LG-ESS. Hysterectomy with bilateral salpingo-oophorectomy was done. Histopathology report did not show residual disease. She was kept under follow-up and is disease free at 9 years.
Discussion
The incidence of uterine sarcoma is 3–7 per 100,000. Low-grade endometrial stromal sarcoma usually follows an indolent course. Late recurrences are common even with early-stage disease. The immediate postoperative extensive recurrence in the first case shows the aggressive behaviour of even low-grade tumour as a result of morcellation. In the second case, the patient had a long disease-free interval. Then, she developed a single site recurrence which was resectable. The third patient in the series was put on primary hormonal treatment as she was not willing for hysterectomy. Later hysterectomy was performed and still patient has got eight years of disease-free interval. The fourth case also has a fairly good disease-free interval of nine years following primary surgery alone without intraoperative spillage.
Electronic morcellation during tumour removal in early-stage LG-ESS leads to an intra-pelvic and intra-abdominal dissemination. It will result in upstaging and worse prognosis. Power morcellation was common during laparoscopic myomectomy before 2013. After a case report of sarcoma dissemination following morcellation, FDA issued a warning statement against the use of power morcellator during myomectomy [1]. The morcellated neoplastic cells have an enhanced seedling tendency [2]. Even before FDA warning, the Society of Gynaecologic Oncologist has given a statement of contraindication for using morcellator in suspected uterine malignancy. The incidence of occult uterine sarcoma in women undergoing myomectomy is 1 in 350 [1]. Dissemination of tumour during surgery has got a grave prognosis. Ultrasonography findings of LG-ESS resemble those of submucosal myomas, leading to the possible preoperative misdiagnosis of uterine leiomyoma. Unsuspected sarcoma in a presumed benign fibroid is very rare, but clinically significant [3–6]. Majority of patients with LG-ESS (80%) are diagnosed at an early stage confined to the uterus, and have a good prognosis. Five-year survival rate of stage I is 98% and the 10-year survival rate is 89%. In advanced disease, the overall survival rate of stage III/IV is much lower, at 66%, and the overall recurrence rate of this stage is 76%. The definitive treatment is total abdominal hysterectomy, bilateral salpingo-oophorectomy and excision of all grossly detectable tumour. LG-ESS recurs most commonly in the abdomen/pelvis (40–50%), followed by the lung (25%). Surgical resection is a reasonable option for women who relapse following a long progression-free interval > 12 to 18 months and have a solitary recurrence amenable to complete resection. Preoperative diagnosis of endometrial sarcoma may not be possible. Hence, it is better to avoid open morcellation during laparoscopic myomectomy. Many gynaecologists are using contained morcellators for tissue extraction [7, 8].
More data is needed regarding safety of the new devices. The primary aim in the surgical management of LG-ESS should be removal of the tumour without peritoneal spillage.
Footnotes
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References
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