Giant Uterine Leiomyoma With Surgical Difficulty.

A 45-year-old nulligravida patient came to our obstetrics and gynecology outpatient department at Prof R. D. Kandou Hospital Manado with a referral letter from a primary health clinic and diagnosed with questionable ovarian cysts. The patient had an enlarged abdomen accompanied by intermittent lower abdominal pain, and difficulty breathing, especially in the supine position that had progressed since approximately 15 years ago. The patient denied having any history of weight loss, non-menstrual vaginal bleeding, or any problems regarding micturition or defecation. The patient had a history of controlled hypertension with routine oral medications. The patient was not yet married, she experienced her first menstrual period at the age of 13 years with a regular cycle that lasts for 5 - 6 days, changed tampons 6 - 7 times a day, and denied having menstrual pain, with her last menstrual period on April 26, 2019. Vital signs and general examinations were unremarkable. Abdominal examination revealed a mobile cystic mass at the tip of the xiphoid process, another mass was also visible protruding from the umbilicus, and no lymph nodes were palpable. Gynecological examination of the hymen and lower reproductive tract was unremarkable. Transabdominal sonography revealed a large heterogenous mass that originated from the pelvic region and expanded throughout the whole abdominal cavity with difficulty differentiating the uterus from surrounding structures; no lymph node enlargement was visible. Abdominal and pelvic computed tomography (CT) scan with contrast revealed cystic mass measuring approximately 43 × 41 × 30 cm with clearly demarcated outer layer, multiple solid inner components and thick inner septations; no lymph node enlargement was visible ( Fig. 1 ). Chest X-ray results were unremarkable without any lymph node enlargement. Routine blood workout revealed low hemoglobin concentration (Hb: 8.3 g/dL) and increase in CA-125 marker was noted (CA-125: 102.6 IU/mL). The patient was initially diagnosed with questionable ovarian cyst and umbilical hernia. Exploratory laparotomy surgery with digestive surgeon and urologist supervision was planned after considering the size of the mass ( Fig. 2 ). Pelvic mass (PM) (a) and umbilical hernia (HU) (b) seen on abdomen-pelvis computed tomography (CT) scan. Pre-operative view of the patient’s abdomen. During pre-operative stages, a laparotomy was planned with a digestive surgeon and urologist’s assistance for installation of a ureteral catheter, but the installation was aborted due to intraoperative difficulty. Laparotomy began with an initial median incision that was extended as wide as needed for the mass to be easily manipulated and evacuated. After the peritoneum layer was opened the cystic mass was visibly filling the entire abdominal cavity, cyst decompression was followed with extraction of approximately 200 cc mucinous fluid out of the cysts. During exploration, a mass originating from the posterior uterus was visible with dimensions measuring approximately 57 × 51 × 21 cm and weighing 26 kg on macroscopic examination ( Figs. 3 and 4 ). Histopathological examination of the mass confirmed a uterine leiomyoma with cystic degeneration with normal adnexal structures, bilateral fallopian tubes and ovaries was identified and adhered to the posterior uterine wall. Intra-operative view of the patient’s abdomen. Giant uterine leiomyoma (myoma) after laparotomy evacuation with surrounding pelvic structures: uterus (UT), right ovary (OV. R), left ovary (OV. L), cervix (CX). After further consideration of the condition, a total hysterectomy with bilateral salpingo-oophorectomy was later performed with patient consent. Bilateral round ligaments were clamped, excised, and sutured. The vesico-uterine layer was identified and widely excised until the bottom end of the round ligaments. Bilateral ureters were observed when the posterior latum ligament was incised. The bilateral infundibulopelvic ligaments were clamped, incised, and sutured. Bilateral uterine arteries were identified while exploring the ureter to uterine artery intersections, followed with incision and suture. The vaginal vault was identified, clamped, and sutured. Gigantic mass size creates change in pelvic anatomy such as ureteral deviation, which requires caution in performing the laparotomy procedure to prevent complications in adjacent abdominal organs such as ureteral resection, gastrointestinal perforation, etc. After a total hysterectomy with bilateral salpingo-oophorectomy was performed, bleeding evaluation was performed to exclude possibilities of pelvic trauma, followed by umbilical hernia repair by a digestive surgeon. Surgical drainage was installed before wound closure. After surgery, the patient was observed for 5 days and was discharged afterwards without any complaints, and outpatient department follow-up was scheduled for the patient.

Giant leiomyoma is a common benign tumor of the uterus with a measured weight greater than 11.4 kg or with a measured diameter greater than 17 cm or with measured dimensions of 33 × 28 × 22 cm [ 1 ]. Histopathologic microscopic examination of uterine leiomyoma may reveal four types of degeneration forms: hyalin, cystic, myxoid or red, and dystrophic degenerations. Hyalin degeneration is the most common form of leiomyoma degeneration; in contrast, cystic degeneration only occurs in 4% of cases [ 6 ]. Uterine leiomyoma has a potency to grow into extreme sizes when symptoms of abdominal compression occur. Increase in mass volume causes abdominal wall distention, which causes most complaints of patient discomfort. Most leiomyoma cases take decades to progress into giant leiomyoma, which is considered slow; consequently, most patients seek treatment in later stages with non-typical symptoms of mass compression. The combined pre- and post-operative mortality rate comprises 16% of all cases [ 4 , 7 ]. Pelvic physical examination is an initial method of diagnostic evaluation, nevertheless smaller myoma size increases difficulty in palpating the mass, which may result in underdiagnosed cases. Therefore, additional diagnostic imaging is required to increase diagnostic sensitivity, and to further evaluate number, size, location, and extension of the mass [ 1 ]. Ultrasonography is the most preferable initial method of imaging due to it being less invasive and its low cost. CT and magnetic resonance imaging (MRI) are advantageous in further excluding the possibility of other benign or malignant lesions. MRI has a higher specificity compared to CT scan in detecting signs of calcification or necrosis, but it is not widely available and has a higher cost [ 7 ]. Tumor markers also play an additional vital role in excluding possibilities of malignancy. Due to each having its own advantages and disadvantages, there is no single best diagnostic modality for giant uterine leiomyoma before a diagnostic laparotomy followed by histopathologic examination is performed. Possible differential diagnosis includes adenomyosis, hematometra, uterine malignancy (carcinoma, sarcoma or carcinosarcoma), retroperitoneal ovarian cysts or ovarian malignancy [ 8 ]. In this reported case, the examined tumor marker of CA-125 was 102.6 IU/mL, which is significantly high, with an RMI of 308. After considering the size of the mass and its high ovarian malignant risk, a laparotomy was planned with the assistance of a digestive surgeon and urologist. This patient’s treatment was consistent with the literature on giant uterine leiomyoma management, in which the management option depends on other factors, which include age, type of lesion, severity of symptoms, malignancy probability, and fertility conservation [ 9 , 10 ]. Indications for surgical management include abnormal uterine bleeding that does not resolve after conservative treatment, secondary iron deficiency to chronic bleeding anemia, severe abdominal pain due to mass compression that reduce the patient’s quality of life, and suspicion of malignancy. Surgical management of this patient’s tumor (diameter > 25 cm) was challenging due to massive blood loss caused by increased vascularization and possibilities of post-operative complications, which include trauma of urinary tract or gastrointestinal infection, and hematoma. Pre-operative assessment of the patient must be performed thoroughly, in order to prevent possible intra-operative and post-operative complications [ 7 , 9 ]. Proper skin excision has to be performed in order to effectively manipulate the mass and examine the upper abdominal region [ 4 , 9 ]. En-bloc evacuation reduces bleeding risk. Potential bleeding sources include collateral circulation, arterio-venous shunting, and tumor size must be properly managed, since it is prone to hypovolemic shock due to anemia and poor nutritional status. Pelvic anatomical distortion due to uterine abnormality may increase injury risk in adjacent gastrointestinal and urinary structures [ 9 ]. Strict post-operative hemodynamic and breathing evaluation is necessary to evaluate evidence of bleeding origin. If fluid and blood product resuscitation fail to re-stabilize the patient’s condition, surgical re-exploration is urgently necessary. Precise perioperative management with multidisciplinary patient care is fundamental in preventing morbidity and mortality and accelerating recovery [ 1 , 4 ]. In conclusion, giant uterine leiomyoma is a rare case in practice and management remains a challenge. Management depends on individual factors such as the patient’s desire to conserve fertility. Careful pre-operative assessment and multidisciplinary efforts are necessary to prevent further operative complications, morbidity, and mortality. The extreme size of mass complicates surgery due to pelvic anatomy distortion; therefore, proper understanding of pelvic anatomy and teamwork is necessary to ensure surgery success.

Uterine leiomyoma is considered as the most common benign gynecological tumor of the uterine myometrium layer in the female reproductive system [ 1 , 2 ]. Based on its clinical incidence, it is found in approximately 25% of women with increasing prevalence throughout reproductive age and decreasing prevalence after the menopause [ 2 ]. The pathogenesis of leiomyomas is currently dilemmatic, nonetheless hormonal stimulation of estrogen, progesterone, and other growth factors plays a significant role. Based on its location, leiomyomas are classified into: subserosa, intramural, and submucosal [ 3 ]. Smaller leiomyomas are more prevalent; nevertheless, in rare instances, it is possible for them to grow into giant myoma (weighing 11.4 kg or more) [ 2 , 4 ]. In cases of giant uterine leiomyoma, evidence-based guidelines for management are limited to conservative management. The increase in leiomyoma size often complicates surgery, which often leads to total or subtotal hysterectomy [ 5 ]. We present a case of a giant uterine leiomyoma that we managed, diagnosed and surgically managed in Prof. Dr. R.D. Kandou Hospital in Manado.