Management of a giant uterine leiomyoma

Abstract

Uterine leiomyomas are the most common tumours arising from the female reproductive tract. However, giant myomas, which are greater than 11.4 kg in weight, are exceedingly rare. They may cause a pressure effect on surrounding organs, heart and lungs, which can be potentially life threatening. We present a case of a 53-year-old woman with a massive uterine mass complicated by restrictive lung disease. She underwent a total abdominal hysterectomy and bilateral salpingo-oophorectomy with frozen section and reconstruction of the abdominal wall by the gynaecologists and plastic surgeons. The excised specimen weighed 27.8 kg, which included a benign subserosal leiomyoma measuring 64 by 50.5 by 15 cm. Intraoperative blood loss was 7 L and her postoperative recovery was complicated by coagulopathy and haemorrhagic shock. This case illustrates the pivotal role of multidisciplinary care in the management of complicated surgical patients and the need for careful perioperative care.

Background

Giant uterine leiomyomas are defined as weighing 11.4 kg or more.¹ They are rare in the current healthcare landscape of increased patient awareness as most women present earlier either incidentally on routine health screening or on becoming symptomatic by causing abnormal menstruation. As they grow to massive dimensions, they may be life threatening by exerting pressure effects on surrounding organs and compromising function. In the literature, the largest uterine fibroid ever reported weighed 63.3 kg and was removed postmortem in 1888.¹ The largest uterine tumour ever removed from a patient who survived a procedure weighed 45.4 kg and was reportedly the 34th largest tumour weighing more than 18.2 kg since 1878.¹

Case presentation

Our patient is a 53-year-old postmenopausal Malay woman who is virgo-intacta. She presented with a massive abdominal mass which has been growing gradually in size for many years (figure 1). This was complicated by orthopnoea and exertional dyspnoea of 6 months’ duration and has progressively worsened. The mass has also limited her mobility, and she was largely bedridden as result. There was no abdominal pain, postmenopausal bleeding or urinary and bowel disturbances. Physical examination revealed a cachectic woman with a tense abdomen, and a large palpable mass extending up to the xiphisternum with prominent overlying abdominal wall veins. Heart sounds were normal, and auscultation of lungs revealed reduced air entry in both lung bases.

Figure 1.

Massive abdominal mass extending to xiphisternum with prominent overlying vessels.

Investigations

A CT scan showed a large multilobulated solid mass occupying most of the abdominal and pelvic cavities, measuring 47.4 cm in craniocaudal length. There were cystic components and amorphous calcification noted within this mass, which were inseparable from the uterus. Gross ascites was noted, and there were no hepatic lesions or enlarged lymph nodes. Bowel loops were not dilated, and the urinary tracts were normal, except for minimal fullness in bilateral renal pelvises (figure 2).

Figure 2.

(A) CT scan of the abdomen and pelvis showing large uterine mass distorting the anatomy of surrounding structures (sagittal view). (B) CT scan of the abdomen and pelvis showing large uterine mass distorting the anatomy of surrounding structures (coronal view).

The cardiorespiratory system was also evaluated preoperatively. Transthoracic echocardiogram showed an ejection fraction of 61% with no regional wall abnormalities. A lung function test showed restrictive lung disease with a forced expiratory volume (FEV1) of 41%, forced vital capacity (FVC) of 44% and FEV1/FVC of 91%. CT of the pulmonary arteries and lower limb Doppler studies was negative for pulmonary and distal embolism. Blood investigations showed elevated lactate dehydrogenase (LDH) of 677 U/L and thrombocytosis of 406×10⁹/L. Haemoglobin was 12 g/dL. Coagulation studies, liver and renal functions were normal.

Differential diagnosis

A pertinent differential for consideration in a patient with an enlarged uterine mass is a leiomyosarcoma. In postmenopausal women, the incidence of sarcoma is 1%–2% and they have a rapid doubling time of 4 weeks.² Uterine leiomyosarcoma behave aggressively with a high rate of recurrence and poor prognosis.³ They commonly present with abnormal uterine bleeding, abdominal distension, pain and urinary symptoms. Other differentials of a large uterine mass include adenomyosis, carcinosarcoma and endometrial stromal sarcoma.

In 2014, Nagai et al has proposed a PREoperative Sarcoma Score (PRESS) to aid in prediction of uterine sarcoma.⁴ Predictive factors in PRESS include age above 49 years old, serum LDH above 279 U/L, positive endometrial cytological findings and MRI findings showing high intratumoural signal intensity on T1 weighted images indicating intratumoural haemorrhage and coagulative necrosis, or a heterogenous mass on T2 weighted images. This scoring system was revised in 2015, and a score of four points or greater suggest a higher risk of uterine sarcoma with a proposed accuracy of 93.7%, positive predictive value of 92.3% and negative predictive value of 94.0%.⁵ Our patient scored at least six points based on age and LDH levels, signifying a high risk of leiomyosarcoma. Hence, our patient was counselled and consented for intraoperative frozen section with full staging procedure should the frozen section confirm a malignancy.

Treatment

She underwent an ultrasound-guided drainage of the ascites for temporary relief of her respiratory symptoms. Cytology examination of the ascitic fluid revealed no malignant cells. She was also started on low molecular weight heparin and graduated compression antiembolism stockings for thromboprophylaxis preoperatively.

After careful evaluation of her baseline function by the anaesthetists and optimisation of preoperative function, she underwent total abdominal hysterectomy and bilateral salpingo-oophorectomy performed by a team consisting of general gynaecologists and gynaecology oncologists. The operation began with a midline incision extending from xiphisternum to the pubic symphysis. The rectus was deficient, and anatomy was greatly distorted due to chronic distension and mass effect on the surrounding organs. The multilobulated pelvic mass was densely adherent to the anterior abdominal wall and peritoneum with multiple feeding vessels. Difficulty was encountered on dissecting the mass from the surrounding structures, and venous bleeding was noted from the tumour surface and feeding vessels. A hand-held vessel sealing device was used to ligate the dense vascular supply and extensive adhesiolysis was done to free the mass from the bladder and pelvic brim. The fibroid was then decapsulated for ease of removal. The specimen was sent for frozen section which showed a benign leiomyoma. We then proceeded to perform a subtotal hysterectomy with bilateral salpingo-oophorectomy. This aided in the final removal of the cervix and reconstruction of the vaginal vault. Absorbable haemostatic agents were used to facilitate haemostasis. Despite careful dissection and attentive haemostasis, the intraoperative blood loss was 7 L, requiring three activations of the hospital’s massive transfusion protocol (MTP). A total of 4.1 L of packed cells, 1.1 L of fresh frozen plasma, 302 mL of cryoprecipitate and 8 L of fluids were transfused. She was also commenced of inotropic support intraoperatively.

The final excised specimen weighed 27.8 kg and measured 64 by 50.5 by 15 cm (figures 3 and 4). Histological analysis revealed interlacing fascicles of bland-appearing spindle cells with areas of stromal hyalinisation and myxoid changes. There is no evidence of nuclear atypia or malignancy. The final histological diagnosis is a benign degenerated leiomyoma.

Figure 3.

(A) Excised specimen measured 64×50.5×15 cm. (B) Cut section of excised specimen.

Figure 4.

Excised specimen weighed 27.8 kg.

As a result of chronic distension, the abdominal wall was very thin and compacted after excision of the massive fibroid. Hence, the plastic surgeons were consulted for excision of the excess skin and closure. The myofascial layer was not separated from the skin to avoid vascular compromise to the skin flap. Plastic surgeons performed a Fleur-de-Lys pattern excision and closure to address the horizontal and vertical laxity. The repair was carried out in layers to reinforce the integrity of the abdominal wall (figure 5).

Figure 5.

(A) Abdominal wall reconstructed by plastic surgeons with a Fleur-de-Lys pattern excision and closure to address the horizontal and vertical laxity. (B) Healed abdominal scar 2 months postoperatively.

Outcome and follow-up

Our patient was admitted to the intensive care unit for monitoring postoperatively. Her recovery was complicated by haemorrhagic shock requiring continued inotropic support. She also developed coagulopathy from the massive blood loss. A further 3 pints of packed cells and 2 pints of fresh frozen plasma were transfused. She was extubated and weaned off inotropes on postoperative day 3. The rest of the postoperative recovery was uneventful and was discharged on postoperative day 7.

She was reviewed in the outpatient clinic 2 months later and was noted to be in good health. Her breathlessness has resolved, and she was able to ambulate independently with minimal aid. The abdominal wound was well healed (figure 5). She defaulted further follow-up.

Discussion

Uterine leiomyoma are the most common pelvic tumour in women. Approximately 70% of woman will develop uterine leiomyoma by the age of 50.⁶ They are benign monoclonal tumours arising from the smooth muscle cells of the myometrium. They typically present with symptoms of abnormal uterine bleeding, pressure symptoms or reproductive dysfunction such as infertility or obstetric complications. They can be described based on their location in the uterus and divided into subserosal, intramural, submucosal or cervical myoma according to the International Federation of Gynaecology and Obstetrics classification.⁷

In reproductive years, fibroids are slow growing at an average growth rate of 9% over 6 months.⁸ Due to the compliance of the abdominal wall, these intra-abdominopelvic tumours may grow to a large size before causing significant pressure effect on surrounding organs, warranting surgical excision. It was recently discovered that a mutation in exon 2 of the mediator complex subunit 12 (MED12) gene is commonly found in uterine fibroids and is associated with smaller size of fibroids compared with MED12 mutation-negative tumours.^{9 10}

In 2009, Ian et al reported a case of a 47-year-old premenopausal woman who was referred to the gynaecological oncology clinic for increasing abdominal girth and abdominal mass causing shortness of breath and abdominal discomfort.¹¹ The surgeons suspected leiomyosarcoma and performed a total abdominal hysterectomy, bilateral salpingo-oophorectomy, appendectomy and partial omentectomy. The final specimen measured 74 cm and weighed 26.94 kg. It was confirmed to be a benign leiomyoma. Blood loss was 1.8 L. The patient subsequently became hypotensive and tachycardic and was reoperated on for haemorrhagic shock arising from a haemoperitoneum during which 2 L of blood was evacuated. The patient was discharged on postoperative day 3 from re-exploration.

In 2011, a similar case was reported by Ryan et al on a 33-year-old woman with a 31-centimetre uterine fibroid who underwent a total abdominal hysterectomy. The excised specimen weighed 11.6 kg. Her postoperative course was complicated by intra-abdominal haemorrhage requiring repeat laparotomy as well. The patient also developed disseminated intravascular coagulation with a total blood loss of 5 L. A total of 16 units of packed cells, 6 units of fresh frozen plasma, 3 packs of platelets and 5.8 L of fluids were transfused.¹²

These cases illustrate the high morbidity and technical difficulties in surgical excision of giant uterine leiomyoma. Hence, careful perioperative care is essential in ensuring the best surgical outcome. Preoperative optimisation of the patient is critical, and careful evaluation of the cardiorespiratory and renal function is recommended. The patient should be evaluated for deep vein thrombosis and be treated accordingly. In view of anticipated massive blood loss, any preoperative anaemia should be corrected. In the absence of any contraindication, preoperative internal iliac artery balloon placement with uterine artery embolisation should also be considered to reduce intraoperative blood loss. Mechanical bowel preparation prior to surgery may reduce the risk of bowel injury. Preoperative insertion of a central venous line and intra-arterial catheter would assist in monitoring of the patient’s haemodynamic status during prolonged surgery.

Intraoperatively, the patient should be positioned to allow adequate ventilation with an effort to reduce compression of the inferior vena cava. The surgery must be performed by an experienced surgeon, preferably with support from the gynaecology oncologists, who are accustomed to retroperitoneal dissection that facilitates surgery and are familiar with identification of important structures such as ureters in a patient with distorted anatomy. There should be careful dissection and attentive haemostasis. Additional assistance from the general surgeons, urologists or plastic surgeons may also be required. The haemodynamic status of the patient must also be closely monitored both intraoperatively and postoperatively with prompt replacement of fluid losses.

Our patient had an intraoperative blood loss of 7 L, requiring activation of the MTP. This is a centralised systematic clinical workflow implemented by the National Blood Services Group, and blood components were transfused in a ratio of 1:1:1 (packed red blood cells: whole blood-derived platelets: fresh frozen plasma).¹³ The MTP was initially created for military trauma settings, but has been extended to non-trauma general medical, surgical and obstetrics patients. The MTP had allowed our patient to receive accelerated delivery of blood products from the blood bank for timely resuscitation, which improved her survival outcome.

Postoperatively, we recommend prompt implementation of thromboprophylactic measures. These include calf compressors and consideration of low molecular weight heparin on resolution of coagulopathy. Physical and occupational therapists are instrumental in preventing excessive physical deconditioning postsurgery and facilitating early recovery of strength and ambulation.

In conclusion, surgical excision of large fibroids is technically challenging and carries a high risk of morbidity and mortality, given the risks of massive haemorrhage and postoperative complications. Our case illustrates the importance of multidisciplinary care in the management of complex surgical patients and the need for comprehensive preoperative assessment, optimal intraoperative management and careful postoperative care.

Learning points.

• Uterine leiomyoma are the most common pelvic tumour in women. They are generally slow growing and may reach massive sizes before causing pressure symptoms on adjacent organs. A rapid growing tumour should raise a suspicion of malignant transformation.

• The revised PREoperative Sarcoma Score may help to predict the risk of malignancy in a suspicious uterine mass and aid in decision for surgery.

• Preoperative optimisation of patient function and workup of comorbidities allow for better preparation and anticipation of potential surgical difficulties and complications.

• Close monitoring of intraoperative haemodynamic status with prompt replacement of fluid losses is critical.

• Postoperative development of haemorrhagic shock and coagulopathy should be anticipated and managed accordingly.

• Multidisciplinary care in the management of complex surgical patients is essential in optimisation of surgical outcomes.