Metastasizing Uterine Leiomyoma: A Case with Cardiac and Pulmonary Metastasis

doi:10.1016/S0344-0338(96)80116-6

Pathology - Research and Practice

Volume 192, Issue 6, 1996, Pages 622-629

https://doi.org/10.1016/S0344-0338(96)80116-6 Get rights and content

SUMMARY

A case of histologically benign cardiac and pulmonary metastases from a uterine leiomyoma in a 45-year-old woman is reported. The solitary cardiac tumor consisted of five lobules with a stalk attached to the anterior papillary muscle of the right ventricle, which occupied the right ventricular cavity and almost completely- obstructed the pulmonary main trunk in the systolic cycle. Multiple small nodules were found throughout both lungs. The cardiac tumor was resected at open heart surgery and open lung biopsy of the pulmonary lesion was simultaneously performed. Both of them were histologically identical to the apparently histologically benign uterine leiomyoma which had been resected five years previously. Based on the clinical and laboratory findings, it is postulated that antegrade metastases via the venous system resulted in the cardiac metastasis from the uterine leiomyoma and the secondary pulmonary metastasis from the cardiac tumor. In contrast to the light microscopic findings, ultrastructural examination suggested the possibility of malignancy associated with the presence of immature smooth muscle cells. This is the first reported case of a so-called benign metastasizing leiomyoma occurring in the heart.

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Cited by (60)

Benign metastasizing leiomyoma presenting with miliary pattern and fatal outcome: Case report with molecular analysis & review of the literature
2019, Respiratory Medicine Case Reports
Citation Excerpt :
One theory suggests that BMLs are primary pulmonary lesions that represent multifocal leiomyomatosis [14] while others contend that they represent metastases. Some have suggested hematogenous extension following uterine surgical procedures [16], but presence of BML in the absence of such invasive procedures, including in our case, makes this a less likely etiology. The possibility of BML representing a metastasis from a low grade uterine leiomyosarcoma has also been entertained, but the histology and the molecular studies in which distinct signatures are noted only for uterine leiomyomas and BMLs makes this unlikely.
Benign metastasizing leiomyoma (BML) is a rare benign smooth muscle neoplasm that originates in the uterus and metastasizes to distant sites—most commonly the lungs. BMLs are often found incidentally in patients with a history of uterine leiomyoma(s) and tend to be indolent. Occasionally they may be symptomatic and rarely follow an aggressive clinical course.
We report an unusual case of BML presenting in a 46-year-old woman as a miliary nodular pattern bilaterally in the lungs and progressive respiratory failure. Her past medical history was significant for uterine “leiomyomas” of at least 9 years' duration. Post mortem histologic evaluation of the uterine and lung lesions revealed benign smooth muscle neoplasms (leiomyomas and BMLs, respectively), and molecular analyses demonstrated identical clonal MED12 mutation, though with greater mutant allelic frequency in the uterus. We document a rare aggressive clinical course in a patient with BML, which presented as a miliary radiologic pattern mimicking an infectious etiology or interstitial lung disease.
Multiple Primary Leiomyomas in the Right Ventricle: A Rare Form of Leiomyoma
2016, Annals of Thoracic Surgery
Citation Excerpt :
There are only 3 female and 2 male cases reported in the English literature. The origins of the first 2 female cases [2–3] were identified as metastases, and the third female case [4] and 2 male cases [5–6] were identified as primary lesions. In adult women, if independent leiomyomas exist inside the pathway of the lower half of the venous system including the inferior vena cava, heart, pulmonary artery, and lungs, BMLs from uterine leiomyomas should be considered first.
A case of multiple benign leiomyomas in the right ventricle of a 47-year-old woman is presented. Multiple lesions were revealed at different locations in the right ventricle; 1 lesion extended into the right pulmonary artery. Histologic examination showed benign leiomyomas arising from the tunica media of the intramyocardial vessels. Also, the primary origin of the cardiac tumors is demonstrated by the evidence that no leiomyoma was found in the genital system 30 months after the cardiac operation. To our knowledge, this is the first case report of multiple primary cardiac leiomyomas in a premenopausal woman. The midterm prognosis after complete resection is good.
Right ventricular and epicardial tumors from benign metastasizing uterine leiomyoma
2016, Journal of Thoracic and Cardiovascular Surgery
Benign metastasizing leiomyomatosis of the lungs presenting a miliary pattern
2014, Annals of Thoracic Surgery
Citation Excerpt :
A similar pattern of presentation of BML is also reported in this case. The pathogenesis of BML is unclear, although it has been postulated that antegrade metastasis through venous channels can occur to extrauterine organs, such as the heart and the lungs, during surgical manipulation of a uterine leiomyoma [8]. Although the patient we report with miliary presentation is posthysterectomy, cases of BML, including the patient reported by Lipton and colleagues [1], have been discovered even before resection of a uterine leiomyoma [4].
Benign metastasizing leiomyomatosis is a very rare and significantly interesting pathology of the lungs. It is a challenge to clinicians when presenting a miliary pattern in preoperative radiologic imaging because it could be any other interstitial disease or infectious in etiology such as miliary tuberculosis. We report a case of innumerable tiny nodular densities spread evenly throughout both lungs in a patient with history of hysterectomy for a fibroid uterus.
Novel hormone treatment of benign metastasizing leiomyoma: An analysis of five cases and literature review
2013, Fertility and Sterility
Citation Excerpt :
In one patient, histological diagnosis may have been associated with significant morbidity, given the close proximity of the lung nodules to the heart (case 1). Another case demonstrates the reported close pathogenesis between IVL and BML, as the patient presented with an IVC/cardiac tumor consistent with IVL as well as pulmonary lesions consistent with BML (5, 42, 43) (case 4). This case series suggests that hormone therapy may be used in the treatment of BML, specifically by employing synergistic combinations with individual dosing.
To evaluate novel hormonal therapies in patients with unresectable benign metastasizing leiomyoma (BML) disease.
Case series.
National Institutes of Health (NIH).
Five subjects with the diagnosis of BML based on imaging and/or histopathologic diagnosis.
Four patients were treated with single or combination therapy of leuprolide acetate and/or an aromatase inhibitor. One patient was treated with an antiprogestin (CDB-2914).
Response to therapy was measured by tumor burden on cross-sectional imaging employing RECIST (Response Evaluation Criteria in Solid Tumors) 1.1 guidelines.
Four patients treated with single or combination therapy of leuprolide acetate and/or an aromatase inhibitor demonstrated stable disease with reduction in tumor burden. The fifth patient treated with antiprogestin (CDB-2914) had degeneration of her tumor, progression of its size, and an improvement in symptoms.
Hormone treatment with GnRH agonist and/or aromatase inhibition may be a therapeutic option to reduce tumor burden in unresectable BML disease or for those patients who wish to avoid surgical intervention. RECIST 1.1 guidelines, while traditionally used to evaluate tumor response to cancer therapeutics, may be useful in evaluating BML tumor burden response to hormone therapy.
Other Neoplastic Lesions: Benign and Malignant
2010, Medical Management of the Thoracic Surgery Patient

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^a: Yoshiki Takatsu, M.D., Department of Internal Medicine, Hyogo Prefectural Amagasaki Hospital, 1-1-1 Higashidaimotsu-cho, Amagasaki-shi, tIyogo 660, Japan, Tel.: 06-482-1521, Fax: 06-482-7430

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Metastasizing Uterine Leiomyoma: A Case with Cardiac and Pulmonary Metastasis

SUMMARY

Hum Pathol

Hum Pathol

Am J Med

Efficacy of oophorectomy in lymphangioleiomyomatosis and benign metastasizing leiomyoma

N Engl J Med

Pregnancy complicated by metastasizing leiomyoma of the uterus

Obstet Gynecol

Typical fibromyoma of the abdominal wall following hysterectomy

Ann Surg

Multiple pulmonary (hamartomatous?) leiomyomas

Light and electron microscopic study. Virchows Arch (Pathol Anat)

Leiomyomatous neoplasms of the lung: A clinical, histologic, and immunohistochemical study

Modern Pathol