Hormonal Manipulation of Benign Metastasizing Leiomyomas: Report of Two Cases and Review of the Literature

Abstract

Benign metastasizing leiomyomas (BMLs) occur predominantly in women during reproductive years. The condition is characterized by uterine leiomyomas associated with the development, typically years later, of slow-growing metastatic lesions. The most commonly affected organs are the lungs, but BMLs have been reported in lymph nodes, deep soft tissues, mesentery, bones, the central nervous system, and the heart. In many cases, these lesions have an indolent course and are discovered rather incidentally. However, occasionally they can present with debilitating symptoms or even life-threatening complications. The presence of estrogen and progester- U TERINE LEIOMYOMAS HAVE been reported in as-sociation with extrauterine benign-appearing smooth muscle tumors. This condition has been described mainly in women of reproductive age but can occur at any age. It has been referred to in the literature as benign metastasizing leiomyoma (BML). This term was introduced in 1939 by Steiner (1) in his report of a case of a 36-yr-old woman who died after massive pulmonary and hilar lymphatic metastases of a uterine leiomyoma. The interest of this condition to endocrinologists is its susceptibility to the hormonal milieu. Knowledge of this susceptibility may lead to effective clinical control of this metastasizing tumor by hormonal manipulation. We report two cases of BML in which hormonal manipulation with LHRH agonists, antiestrogens, and aromatase inhibitors (AIs) caused alleviation of symptoms and regression of the metastatic lesions. Case 1A 47-yr-old Caucasian woman consulted because of recurrent left subscapular pain that presented every few weeks with spontaneous resolution within a few days. Her complaint was initially attributed to cervical arthrosis and treated with analgesics. Three years later she developed numbness over her left hand, and a firm mass of about 5 cm was noticed in the left supraclavicular area (Fig. 1). A magnetic resonance image (MRI) revealed a large paraspinal tumor involving the brachial plexus and extending into the vertebral canal (Fig. 2). C5-T1 laminectomies, debulking of the extraspinal, and near complete removal of the intraspinal component was carried out. Pathological evaluation revealed a well-differentiated leiomyosarcoma [1-3 mitoses per 10 high-power fields (HPFs) and no necrosis] with bony involvement (Fig. 3).Radiotherapy was planned but the patient refused it. Shortly afterward she began experiencing cervical pain unrelieved by opioid analgesia, with a similar cyclical pattern to her previous pain; this prompted consultation to endocrinology. Her past medical history was remarkable for a total hysterectomy at age 36 yr for a 6-cm uterine leiomyoma. The histological blocks were retrieved and reexamined; the new report described a tumor with ill-defined infiltrative borders, areas of coagulative necrosis, mitotic rate of 4/10 HPFs, and few pathological mitotic figures but no significant cytological atypia. The conclusion was smooth muscle tumor of low malignant potential (2). The histol...

Benign metastasizing leiomyoma: clonality, telomere length and clinicopathologic analysis

“…Finally, because of their uterine origin and the presence of high levels of ERs and PRs, medical treatment with drugs such as tamoxifen or raloxifene, both selective ER modulators, may be effective in cases where surgical resection is incomplete or not clinically indicated. 18,20,25 In summary, our study supports the notion that 'benign metastasizing leiomyoma' is a result of monoclonal, hematogenous spread of a benignappearing uterine leiomyoma. This tumor possesses many morphologic, immunohistochemical, and molecular features characteristic of a benign neoplasm despite its potential for metastastic spread, which if neglected, may lead to shortened survival.…”

Oestrogen-Induced Angiogenesis and Implantation Contribute to the Development of Parasitic Myomas after Laparoscopic Morcellation

“…In our study, the non-implanted myoma fragments disclosed decreased expressions of both ERα and PR compared with implanted lesions, and this result suggested the disseminated fragments with strong positive ERα and PR might be essential in the development of PM implantations. Our study also demonstrated that depletion of oestrogen decreased the angiogenesis, proliferation and implantations, and the result was correlated with previous studies which demonstrated shrinkage of benign metastatic leiomyomatosis after bilateral salpingo-oophorectomy (BSO) surgery [53][54][55][56]. The duration of sex steroid hormone exposure after laparoscopic morcellation might also be a risk factor for the development of PMs [26].…”

Benign Metastasizing Leiomyoma

“…However, there have been described very infrequent situations in which the tumor presents a distant progression of smooth muscle cell to other organs. There are different types of growing pattern including Benign Metastasizing Leiomyoma (BML), intravascular leiomyomatosis and disseminated peritoneal leiomyomatosis [1]. The most afflicted organs are the lungs [2], but there have been documented extrapulmonary involvement in heart, spine, bone, skull base, omentum and mesentery, lymph nodes and deep soft tissue.…”