Primary Leiomyoma: A Rare Space Occupying Lesion in the Right Ventricle

doi:10.1016/j.athoracsur.2013.05.097

The Annals of Thoracic Surgery

Volume 97, Issue 1, January 2014, Pages 324-326

https://doi.org/10.1016/j.athoracsur.2013.05.097 Get rights and content

A case of primary cardiac leiomyoma arising from the infundibulum of the right ventricle of a 24-year-old woman is presented. The mass protruded into the cavity of the right ventricle and caused severe right ventricular outflow tract obstruction. We resected the tumor under cardiopulmonary bypass successfully, and confirmed it histologically to be a benign leiomyoma. To the authors' knowledge, this is the first case report of a primary cardiac leiomyoma in an adult woman.

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Leiomyomas are the most common type of noncancerous neoplasm in the uterine myometrium in women of reproductive age, but occasionally occur with unusual growth patterns or in extrauterine sites that make their identification more challenging. In such a case, synchronous uterine leiomyoma or a previous hysterectomy may be indicative of the diagnosis [3]. We concluded that its cardiac involvement, which was not even listed in the extrauterine leiomyomatous classification [3], can be divided into

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Cited by (9)

Right Ventricular Outflow Tract Obstruction in Adults: A Systematic Review and Meta-analysis
2021, CJC Open
Right ventricular outflow tract obstruction (RVOTO) is a cause of hemodynamic instability that can occur in several situations, including cardiac surgery, lung transplantation, and thoracic surgery, and in critically ill patients. The timely diagnosis of RVOTO is important because it requires specific considerations, including the adverse effects of positive inotropes, and depending on the etiology, the requirement for urgent surgical intervention.
The objective of this systematic review and meta-analysis was to determine the prevalence of RVOTO in adult patients, and the distribution of all reported cases by etiology.
Of 233 available reports, there were 229 case reports or series, and 4 retrospective cohort studies, with one study also reporting a prospective cohort. Of 291 reported cases of RVOTO, 61 (21%) were congenital, 56 (19%) were iatrogenic, and 174 (60%) were neither congenital nor iatrogenic (including intracardiac tumour). The mechanism of RVOTO was an intrinsic obstruction in 169 cases (58%), and an extrinsic obstruction in 122 cases (42%). A mechanical obstruction causing RVOTO was present in 262 cases (90%), and 29 cases of dynamic RVOTO (10%) were reported. In the 5 included cohorts, with a total of 1122 patients, the overall prevalence was estimated to be 4.0% (1%-9%).
RVOTO, though rare, remains clinically important, and therefore, multicentre studies are warranted to better understand the prevalence, causes, and consequences of RVOTO.
L'obstruction de la chambre de chasse du ventricule droit (OCCVD) est une cause d'instabilité hémodynamique qui peut survenir dans plusieurs situations, y compris une chirurgie cardiaque, une transplantation pulmonaire ou une chirurgie thoracique, ou encore chez des patients en phase critique. Il est important que le diagnostic d'OCCVD soit posé rapidement, car d'une part cette affection exige la prise en compte d’éléments particuliers, y compris les effets indésirables des agents inotropes positifs et, d'autre part, en fonction de l’étiologie, une intervention chirurgicale d'urgence pourrait être nécessaire.
L'objectif de cette revue systématique associée à une méta-analyse était de déterminer la prévalence de l'OCCVD chez les patients adultes ainsi que la distribution de tous les cas rapportés en fonction de leur étiologie.
Sur les 233 rapports disponibles, on comptait 229 études ou séries de cas, et quatre études de cohortes rétrospectives, dont une qui présentait également les résultats d'une cohorte prospective. Sur 291 cas d'OCCVD rapportés, 61 (21 %) étaient d'origine congénitale, 56 (19 %) étaient d'origine iatrogène et 174 (60 %) avaient une origine qui n’était ni congénitale ni iatrogène (dont une tumeur intracardiaque). Le mécanisme de l'OCCVD était une obstruction intrinsèque dans 169 cas (58 %), et une obstruction extrinsèque dans 122 cas (42 %). Une obstruction mécanique causant l'OCCVD était présente dans 262 cas (90 %), et 29 cas d'OCCVD dynamique (10 %) ont été rapportés. Dans les 5 cohortes incluses, comptant au total 1 122 patients, la prévalence globale était estimée à 4,0 % (de 1 % à 9 %).
L'OCCVD, malgré sa rareté, n'en est pas moins importante sur le plan clinique; la réalisation d’études multicentriques serait donc justifiée pour permettre de mieux comprendre la prévalence, les causes et les conséquences de cette affection.
Primary intracardiac leiomyoma arising from cardiomyocyte progenitors at the right ventriculoseptal interface: case report with literature review
2017, Cardiovascular Pathology
Citation Excerpt :
We lost the patient to follow-up after a few visits during the initial 6 months, when he was asymptomatic and doing well. Leiomyoma is an innocuous smooth muscle tumor most commonly described in the uterine myometrium [5]. This entity may theoretically occur at any site possessing native smooth muscle cells.
Primary cardiac neoplasms are rare and are usually benign myxomas and rhabdomyomas. Cardiac leiomyomas are usually seen as a part of the spectrum of intravenous leiomyomatosis or benign metastasizing leiomyoma. De novo occurrence of primary intracardiac leiomyomas (PICL) is a rarity. Herein we describe a 14-year-old boy presenting with intermittent dyspnea for 2 years, with a large right ventricular mass suggestive of myxoma on transthoracic echocardiography, without any extracardiac lesions. Histology and immunohistochemistry of the tumor excised under cardiopulmonary bypass confirmed a PICL arising at the cardiomyocyte–smooth muscle septal interface. A review of existing literature highlights an increased incidence in young patients and an overwhelming right ventricular anatomical predilection. Abnormalities in the multipotent cardiac progenitor cells of the second heart field may provide a potential microenvironment for the histogenesis of PICL.
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
Citation Excerpt :
Leiomyomas represent less than 1% of primary cardiac tumors, but they are the most common benign uterine tumor.1,2 To our knowledge, 3 case reports of primary cardiac leiomyomas exist.10-12 Cardiac metastases may present in synchrony with the primary tumor or years later.6
Case report: Right ventricular outflow tract obstruction caused by multicomponent mesenchymal tumor
2022, Frontiers in Cardiovascular Medicine
A Rare Case of Cardiac Metastatic Uterine Intravenous Leiomyomatosis
2022, Frontiers in Cardiovascular Medicine

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Case reportPrimary Leiomyoma: A Rare Space Occupying Lesion in the Right Ventricle

Section snippets

Comment

Ann Thorac Surg

Ann Thorac Surg

Cardiac tumours: diagnosis and management

Heart

Case report
Primary Leiomyoma: A Rare Space Occupying Lesion in the Right Ventricle