A 46-year-old, para 2 Caucasian woman presented to the general surgeons at our hospital with a four-day history of sudden onset severe lower abdominal pain. She had undergone laparoscopic sterilization 13 years previously and had no medical or social history of note. A laparoscopy was performed because appendicitis was suspected; this revealed a right broad ligament hematoma. This finding was attributed to an iatrogenic injury caused by insertion of the Veress needle during the laparoscopy and, in the absence of peritoneal bleeding, conservative management was initially undertaken. Two months later, she re-presented with acute severe right iliac fossa pain and a repeat ultrasound scan revealed an increase in the size of the broad ligament hematoma.
She was referred to our gynecology department and underwent a laparotomy and a total abdominal hysterectomy and bilateral salpingo-oophorectomy (TAH BSO) five months later. Intra-operative findings included a normal uterus and ovaries with a copious amount of blood in the peritoneal cavity coming from a large right broad ligament hematoma. Exploration of the hematoma released approximately 2 L of old blood and strips of yellow necrotic tissue, with no specific bleeding points identified. Within four weeks, whilst awaiting reconfirmation of the histology results, the patient presented with recurrence of severe lower abdominal pain; a repeat ultrasound scan showed a reaccumulation of hematoma in the right paravaginal space. An angiography failed to reveal a specific bleeding point but a further large volume of necrotic material with blood was removed by ultrasound-guided aspiration. A histological examination of the necrotic tissue revealed malignant polygonal tumoral cells with ample clear or finely granular eosinophilic cytoplasm, focally large pale nuclei with prominent nucleoli and scattered mitoses, with some abnormal forms. Immunohiostochemistry results were positive for HMB45, melan A, caldesmon and smooth muscle actin, leading to a final diagnosis of broad ligament PEComa.
Further management was undertaken in a tertiary oncology center (Royal Marsden Hospital, London), and two months after the TAH BSO, a full body computed tomography (CT) scan revealed a 10 × 10 × 10 cm pelvic mass to the right of the midline with a satellite lesion on the left. In addition, a right hydronephrosis secondary to the mass was diagnosed and managed with nephrostomy and ureteric stent insertion. The patient underwent palliative radiotherapy, after which the nephrostomy was removed. Her pelvic pain did not respond to the radiotherapy and has remained the main issue.
At 10 months after her initial presentation, an examination under anesthesia (EUA) and cystoscopy was performed. Operative findings included a large pelvic tumor closely related to the superior and anterior aspects of the vagina and distorting it. The bladder mucosa appeared normal except for radiation changes. At this stage, further surgery was considered to be a hazardous option and symptomatic management was continued.
Four months after the EUA and cystoscopy the pain had become unbearable for our patient and she underwent an EUA, sigmoidoscopy, cystoscopy and laparoscopy, which revealed a large retroperitoneal tumor on the right pelvic side wall extending down the right vaginal wall and distorting the vagina and bladder. No tumor breach was seen on cystoscopy or sigmoidoscopy. A fistulous connection was found between the tumor and vagina and the bowel was adherent to the tumor mass. A histological examination of the tumor showed features similar to the first sample, with scattered malignant epithelioid malignant cells with enlarged hyperchromatic pleomorphic nuclei containing abundant finely granular eosinophilic cytoplasm and distinct cell boundaries, and was consistent, as before, with malignant PEComa. There were no malignant cells in the peritoneal washings sent.
One month later, repeat CT imaging showed no tumor progression over the previous three months and at this stage her symptoms were tolerable with opioid analgesia. However, four months after this, she developed left-sided hydronephrosis and opted to have a ureteric stent rather than other surgical management options.
The patient agreed to consider the option of sirolimus one month after undergoing stenting of her left ureter, based on a report of clinical benefit in a small series of patients with PEComas treated in the USA [2
]. It was hoped that treatment with sirolimus might result in sufficient tumor shrinkage to facilitate a subsequent operation.
Over the subsequent six months, she required numerous hospital admissions with urinary tract infections. During one of these episodes, she was admitted to the coronary care unit with dilated cardiomyopathy and was diagnosed with nephrogenic diabetes insipidus requiring treatment with desmopressin.
The patient remained symptomatic with low abdominal and deep pelvic pain and multi-drug resistant recurrent urinary tract infections, along with radiation induced colitis. A CT scan performed a year after starting sirolimus showed a modest reduction in tumor size and the findings were unchanged on further imaging two months later. Three months after this, she underwent surgery in the hope of resecting the tumor. Unfortunately, extensive fibrosis was found making it impossible to identify the pelvic vessels, hence the tumor resection was abandoned. The fibrosis extended up to the kidneys but the ureters were freed from the fibrous tissue and an ileal conduit was fashioned enabling the ureteric stents to be removed. Since this time there has only been one minor episode of urinary tract infection, but pain continues to be a problem.
A recent MRI scan (see Figures and ) shows that over the entire treatment period with sirolimus, a substantial degree of tumor shrinkage has occurred, from a maximum diameter of 92 mm a year after initial presentation (prior to starting sirolimus) to 53 mm following two years of sirolimus treatment. The patient has continued with sirolimus treatment, currently at a dose of 4 mg daily, 5 mg being associated with mouth ulceration.
MRI scan of the pelvis prior to starting sirolimus, following total abdominal hysterectomy and bilateral salpingo-oophorectomy (TAH BSO), demonstrating the perivascular epithelioid cell tumor (PEComa) measuring 92 mm.
MRI scan of the pelvis after two years on sirolimus, demonstrating a reduction in size of the perivascular epithelioid cell tumor (PEComa) (measuring 53 mm) following treatment with sirolimus.