Advanced Uterine Sarcoma - an unusual case study
Completed at Crosshouse Hospital
Natalie Pierotti
University of Glasgow, Year 4
Advanced Uterine Sarcoma - an unusual case study
Introduction
Uterine sarcomas are rare malignant tumors arising from smooth muscle or connective tissue, altogether they account for between “3–7% of all malignant diseases the uterus”. 1They are sub-divided histologically; the“incidence of mixed Müllerian sarcomas is 50%, leiomyosarcoma is 30%, endometrial stromal sarcoma is 15%, and adenosarcomais 5%.”2Recent evidence has suggested however that mixedMüllerian sarcomas are in fact metaplastic carcinomas, rather than a subgroup of sarcoma.3 Patients usually present with non-specific symptoms including abnormal vaginal bleeding, postmenopausal bleeding or pelvic pain. Very few cases appear in the literature however there is a proven association with pelvic radiation and long-term tamoxifen use. Management of uterine sarcomas includes surgery, chemotherapy and radiotherapy, with prognosis dependant on the size of the sarcoma.
Case report
A 57 year old woman, parity 2+2, 8 years after menopause was referred to gynecology with:
-2 week history of left calf swelling of 4cm compared with her right
-2 week history of left sided pleuritic chest pain
-D dimmer of 5787 with a clear Doppler ultrasound of her legs
-8 month history of generalized abdominal pain
-4 and a half stone weight loss over 18 months
-Ongoing nausea and vomiting
-Supra-pubic mass noticeable for 3 months - put down to IBS by the patient
-Problems passing urine
-No history of abnormal vaginal bleeding
The patient had a past medical history of IBS and hypertension treated with lisinopril 10mg OD
A CT scan of her chest, abdomen and pelvis was arranged querying compression of the venous system. This showed a mass in the right adnexal region of mixed soft tissue and cystic components. It measured 21cm x 14cm axial length and 23cm craniocaudal length and was determined to be of ovarian origin. It also showed bilateral hydronephrosis due to compression of the ureters by the mass. There was no metastasis found.
The patient underwent a planned admission for bilateral nephrostomy insertion to manage the hydronephrosis and 3 days later was admitted for an exploratory laparotomy. Intra-operatively a mass the size of an 18-week gravid uterus was found extending from the back of the uterus filling the retroperitoneal space. The mass was very fixed and vascular with a large clot, evidence of recent hemorrhage. On attempt to assess mobility the tumor bled profusely and there was therefore no prospect of resection. Biopsies were taken of the uterine mass and the omentum to grade and stage the tumor. Due to the extensive bleeding, the abdomen was packed and left open with a vacuum assisted dressing. Blood loss was calculated at 4300mls (some of which had already hemorrhaged into the tumor), the patient was transfused 4 units and transferred to ICU.
Histopathology confirmed a diagnosis of leiomyosarcoma of the uterus, non responsive to radiotherapy and chemotherapy. Due to the size and vascular nature of the tumor, surgical resection was not an option. The management plan was therefore to stabilize the patient to allow interventional radiology and subsequent removal of the packs and closing of her abdomen.
Post operatively the patient was categorized as in Acute Kidney Injury, RIFLE criteria F with a creatinine of 246 and oliguria, secondary to the blood lost in theatre. She required ventilatory and cardiovascular support, including fluids, a further 2 units of blood and was dependant on 5ml/h of nor adrenaline to maintain blood pressure. The patient was also sedated and invasively ventilated on a BiPAP machine. Despite MABP being maintained at more than 75, the patient remained oliguric with rising potassium. A DNACPR was issued.
After stabilizations the patient successfully underwent interventional radiology and ebolisation of the right internal iliac artery and the left uterine artery. By blocking these arteries, and as a result, the blood supply to the tumor, the treatment it was hoped, would shrink, or decrease the rate of growth of the tumor. The patient unfortunately suffered side effects from this procedure. The release of necrotic tissue from the shrinking tumor resulted in increasing sepsis and moderate compartment syndrome. The patient was treated with antibiotics and had surgery to remove the packs in her abdomen and close the wound.
Despite her concurrent kidney injury,and the contrast injected during interventional radiology, the patient is not a candidate for RRT due to the poor prognosis associated with such a large sarcoma. When last followed up for this essay the patient was receiving supportive therapy with palliative care input including morphine PRN for pain, haloperidol and cyclizine for the nausea and vomiting, and lorazepam for anxiety. Her kidney function was improving. The patient was unwilling to discuss the future and her poor prognosis however after discussion with both the patient and her family, the hospital plans to discharge her home with physiotherapy and OT support.
Discussion
The patient was confirmed on biopsy to have a uterine leiomyosarcoma, a type of rare uterine sarcoma accounting for approximately 2% of malignant tumors of the uterus. This is an aggressive tumor arising from smooth muscle cells, very few cases of which are reported in the literature. They are associated with a poor prognosis, which may be due to their “location in the vascular myometrium of the uterus, which allows for early invasion and widespread metastases.”4
Leiomyosracomas are often diagnosed late due to the non-specific nature of their presentation, as was the case with this patient. The median age at presentation is between “47 and 56 years of age, although the range varies widely.”5Abnormal vaginal bleeding and pelvic or abdominal pain are the most frequent symptoms however many are not diagnosed until the late stages. Similarly imaging is often not conclusive and a diagnosis is only made after surgical biopsy. A history of pelvic radiation therapy or tamoxifen use,6 have been demonstrated to be risk factors for leiomyosarcomas, as have obesity and nuliparity.7 These factors however are not sufficient to warrant screening, especially if considering its low incidence.
Leiomyosarcomas are staged according to the modified 1988 International Federation of Gynecology and Obstetrics (FIGO) criteria, with points assigned to size/extent of the tumor, the number of lymph nodes involved and whether there is evidence of metastasis. “Overall, tumor stage has been confirmed as the strongest prognostic variable. The reported 5-year overall survival ranged from 62–65% in studies that included predominantly stage I disease, in contrast with studies with a higher proportion of advanced disease where the 5-year overall survival rate was as low as 29%.”8Wu et alalso demonstrated that age >50 years, stage III or IV disease and tumor size >11 cm significantly decreased overall survival.9
Surgery is the mainstay of treatment for uterine leiomyosarcomas, a total abdominal hysterectomy and bilateralsalpingo- oophorectomy is recommended. There is conflicting evidence as to the benefit of radiotherapy and chemotherapy. Nonrandomized studies have reported improved survival rates from adjuvant chemotherapy however more evidence and randomized controlled studies are needed.10 If surgical resection is not an option, as was the case with this patient, then interventional radiology and embolisation have a role to play. Usually a treatment for symptomatic fibroids, this procedure involves insertion of a catheter and the injection of an embolic agent to block the artery. This procedure aims to shrink, or alternatively halt the growth of the leiomyosarcoma, providing symptomatic relief and preventing hemorrhage. As was the case with the patient, the side effect is the release of necrotic tissue into the pelvic cavity and sepsis.
Conclusion
The patient was diagnosed with advanced leiomyosarcoma of the uterus and as a result has a very poor prognosis, when considered with her kidney injury. The question remains, is there anything that could have been done differently? Due to the rarity of her tumor and the non-specific nature of her presentation it is unlikely that much could have been done differently, especially with the lack of abnormal vaginal bleeding pointing to a gynecological cause. It is also possible that the diagnosis of IBS clouded the diagnosis and delayed her presentation.
1. Harry, V et al.Review Uterine leiomyosarcomas:a review of the diagnostic andtherapeutic pitfalls.The Obstetrician & Gynaecologist.2007;9:88–94 10.1576/toag.9.2.088.27309
2. Vellanki et al. A rare case of uterine leiomyosarcoma: a case report. J Med Case Reports.2010; 4: 222.10.1186/1752-1947-4-222
3.Harry, V et al.Review Uterine leiomyosarcomas:a review of the diagnostic andtherapeutic pitfalls.The Obstetrician & Gynaecologist.2007;9:88–94 10.1576/toag.9.2.088.27309
4.Harry, V et al.Review Uterine leiomyosarcomas:a review of the diagnostic andtherapeutic pitfalls.The Obstetrician & Gynaecologist.2007;9:88–94 10.1576/toag.9.2.088.27309
5. Harry, V et al.Review Uterine leiomyosarcomas:a review of the diagnostic andtherapeutic pitfalls.The Obstetrician & Gynaecologist.2007;9:88–94 10.1576/toag.9.2.088.27309
6.Yildirim Y, Inal MM, Sanci M, Yildirim YK, Mit T, Polat M, et al. Development of uterine sarcoma after tamoxifen treatment for breast cancer: report of four cases. Int J Gynecol Cancer 2005;15:1239–42.
7. Harry, V et al.Review Uterine leiomyosarcomas:a review of the diagnostic andtherapeutic pitfalls.The Obstetrician & Gynaecologist.2007;9:88–94 10.1576/toag.9.2.088.27309
8.Harry, V et al.Review Uterine leiomyosarcomas:a review of the diagnostic andtherapeutic pitfalls.The Obstetrician & Gynaecologist.2007;9:88–94 10.1576/toag.9.2.088.27309
9. Wu TI, Chang TC, Hsueh S, Hsu KH, Chou HH, Huang HJ, et al. Prognostic factors and impact of adjuvant chemotherapy for uterine leiomyosarcoma. GynecolOncol 2006;100:166–72. doi:10.1016/j.ygyno.2005.08.010
10. Vellanki et al. A rare case of uterine leiomyosarcoma: a case report. J Med Case Reports.2010; 4: 222.10.1186/1752-1947-4-222