Uterine angiolipoleiomyoma. A case report and systematic literature review

Abstract

Uterine angiolipoleiomyomas are rare, benign mixed mesenchymal lesions. A manifestation in the gynecological region is quite uncommon, with few cases described in the literature so far. We present an interesting case of a 59-year-old woman diagnosed with uterine angiolipoleiomyoma, and the results of the conducted systematic review of the literature. The patient presented with a pelvic mass masquerading as a leiomyoma on the ultrasound and postmenopausal vaginal bleeding. At laparotomy, a large uterus was noticed and the histopathology set the diagnosis of angiolipoleiomyoma. Immunohistochemistry revealed negativity for Melan-A and HMB-45 melanoma-specific antibodies and positivity for Van Gieson and orcein histochemical stains. We systematically reviewed the literature. The eligible articles were those written in English, excluding animal studies and studies reporting angiolipoleiomyomas in other regions beside the uterus. The present case is one of the 10 cases of uterine angiolipoleiomyoma reported in the literature. In 8 out of 11 (72.7%) cases, uterine angiolipoleiomyomas arose from the corpus of the uterus, while in 2 (18.1%) cases they were located at the cervix, and in one case (9%) angiolipoleiomyoma was located in the broad ligament. Concerning symptoms, four of the patients (36.4%) presented with abdominal and pelvic pain, two (18.1%) with postmenopausal vaginal bleeding, one with menometrorrhagia (9%), and one with uterine prolapse and cystocele (9%). Immunohistochemical staining of uterine angiolipoleiomyomas was positive for SMA in 4 patients (36.4%), positive for desmin in 3 cases (27.3%), positive for anti-S-100 protein antibody in 2 patients (18.1%), while in one case (9%) immunopositivity was observed for CD31. Only our case (9%) was also tested for CD34, Van Gieson and orcein, the first of these being negative and the other two positive (at the blood vessels in a specialized pattern). Three of the patients (27.3%) were also tested for HMB-45 and all three were immunonegative. In order to establish the diagnosis of uterine angiolipoleiomyomas, ultrasonography and additional MRI may help the preoperative prediction of a benign mass. Immunohistochemistry will show strong positivity of alpha-smooth muscle actin and desmin. Complete abdominal hysterectomy is the preferable treatment.