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Right heart failure due to benign metastasizing leiomyoma: a case report of this exceedingly rare condition and review of the literature

ABSTRACT: Abstract

Background

Benign metastasizing leiomyoma (BML) is a smooth muscle tumour of genital origin occurring in women with a history of uterine or pelvic leiomyoma. Although histologically benign, it exhibits metastatic behaviour. Lungs are the most common site of metastasis. The heart is a rare site and metastasis at this location has been described in just few cases.

Case summary?

A 42-year-old woman with a resected uterine leiomyoma and a subsequent and still not-resected left periovarian solid mass began complaining of shortness of breath 2 weeks before presentation. Echocardiography showed a mass located in the right ventricular cavity, enlargement of the right ventricle, and severe tricuspid regurgitation. Cardiac magnetic resonance revealed two masses suggestive of tumours in the right ventricle causing right ventricular outflow tract obstruction. Cardiac surgery was performed and, intraoperatively, a third small mass was detected on the tricuspid valve. The masses were resected, and tricuspid valve replacement was performed. Ten days later, the patient underwent an abdominal surgery for the pelvic mass resection. Immunohistochemical analysis of the cardiac and pelvic masses corroborated the diagnosis of benign leiomyomas. The patient was discharged in good clinical condition.

Discussion?

Benign metastasizing leiomyoma to the heart is a rare condition. The pathogenesis remains controversial and includes: (i) vascular or lymphatic spread of myomatous tissue cells when leiomyoma resection or hysterectomy is performed and (ii) smooth muscle cell proliferation in multiple regions. The more usual locations of BML in the heart seem to be the tricuspid valve and the right face of the interventricular septum.

SUBMITTER: Pedrosa C

PROVIDER: S-EPMC7793117 | biostudies-literature | 2020 Dec

REPOSITORIES: biostudies-literature

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Project description:BackgroundPulmonary benign metastasizing leiomyoma (PBML) is the most common extrauterine spread of uterine leiomyoma, and its biological behavior is traditionally thought to be hormone dependent. Studies on older PBML patients have been previously reported, but limited literature has been published regarding the clinical features and treatment of PBML in young women.MethodsA total of 65 cases of PBML in women aged 45 years and younger were reviewed, including 56 cases selected from PubMed and 9 cases from our hospital. The clinical characteristics and management of these patients were analyzed.ResultsThe median age of all the patients at diagnosis was 39.0 years. PBML most commonly presented as bilateral solid lesions (60.9%), with other rare imaging manifestations. The median interval time from a pertinent gynecologic procedure to diagnosis was 6.0 years. A total of 16.7% of patients received careful observation, and all achieved stable status in a median follow-up time of 18.0 months. A total of 71.4% of patients were administered anti-estrogen therapies, including surgical castration (33.3%), gonadotropin-releasing hormone analog (23.8%) and anti-estrogen drugs (14.3%). Eight of 42 patients underwent surgical resection of metastatic lesions. Patients who underwent curative surgery for the removal of pulmonary lesions combined with adjuvant anti-estrogen therapies had favorable outcomes compared with those who only underwent surgical resection. The disease control rates of surgical castration, gonadotropin-releasing hormone analog, and anti-estrogen drugs were 85.7%, 90.0%, and 50.0%, respectively. For two patients, sirolimus (rapamycin) achieved successful relief of symptoms and control of pulmonary lesions without lowering hormone levels and causing estrogen deficiency symptoms.ConclusionsIn the absence of standard treatment guidelines for PBML, maintaining a low-estrogen environment using different kinds of antiestrogen therapies has been the mainstream strategy and has satisfying curative effects. A wait-and-see strategy might be an option, but therapeutic approaches must be contemplated when complications or symptoms progress. For PBML in young women, the negative effect on ovarian function of anti-estrogen treatment, especially surgical castration, should be considered. Sirolimus might be a new treatment option for young PBML patients, especially for those who want to preserve ovarian function.