An Unusual Case: Intravascular Journey of a Benign Uterine Tumor to the Heart

Deepak Ramchand Nenwani; N Swaminathan; S Venkatesan; C Elangovan; K Tamil Selvan

doi:10.4103/jiae.jiae_10_20

INTERESTING CASE REPORT

Year : 2022 | Volume : 6 | Issue : 1 | Page : 53-55

An Unusual Case: Intravascular Journey of a Benign Uterine Tumor to the Heart

Deepak Ramchand Nenwani, N Swaminathan, S Venkatesan, C Elangovan, K Tamil Selvan
Department of Cardiology, Institute of Cardiology, RGGGH, Chennai, Tamil Nadu, India

Date of Submission	18-Mar-2020
Date of Decision	07-Apr-2020
Date of Acceptance	21-Jun-2020
Date of Web Publication	29-Apr-2022

Correspondence Address:
Dr. S Venkatesan
Department of Cardiology, Institute of Cardiology, RGGGH, Chennai, Tamil Nadu
India

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jiae.jiae_10_20

Abstract

Intravenous leiomyomatosis (IVL) is a rare neoplasm characterized by histologically benign-looking smooth muscle cell tumor mass which grows within the intrauterine and extrauterine venous system. In this report, we present an unusual case of IVL which had originated from uterus and extended throughout the right cardiac chambers. A 36-year-old female patient came with a chief complaint of palpitation for 1-month duration. She also had a history of amenorrhea for 7 months. She had abdominal distension and doughy feeling of the abdomen. Seven months prior, ultrasound abdomen done during gynecological evaluation showed fibroid uterus. Echocardiography done showed multiple masses occupying almost all of the right atrium and moving in and out of the right ventricle. Inferior vena cava (IVC) was dilated and 90% of the IVC was filled with the mass. Cardiothoracic surgery and surgical oncology references were done. The patient was planned for simultaneous abdominal and cardiothoracic surgery. Large abdominal mass along with cardiac mass was resected along with mesentery, omentum, and abdominal lymph nodes. Histopathological and microscopic report was suggestive of leiomyoma and leiomyomatosis. Lymph node microscopy showed reactive hyperplasia.

Keywords: Benign tumor, fibroid uterus, intracardiac leiomyomatosis

How to cite this article:
Nenwani DR, Swaminathan N, Venkatesan S, Elangovan C, Selvan K T. An Unusual Case: Intravascular Journey of a Benign Uterine Tumor to the Heart. J Indian Acad Echocardiogr Cardiovasc Imaging 2022;6:53-5

How to cite this URL:
Nenwani DR, Swaminathan N, Venkatesan S, Elangovan C, Selvan K T. An Unusual Case: Intravascular Journey of a Benign Uterine Tumor to the Heart. J Indian Acad Echocardiogr Cardiovasc Imaging [serial online] 2022 [cited 2023 May 29];6:53-5. Available from: https://jiaecho.org/text.asp?2022/6/1/53/344308

Introduction

Intravenous leiomyoma lesions were first described in 1897 by Birch-Hirschfield from an autopsy case, and the first case of intracardiac extension was then reported by Durck in 1907.^[1]Since then, fewer than 300 cases have been documented in the literature till 2018.^[2] So far, only five cases of intravenous leiomyomatosis (IVL) have been reported from India, out of which one had peripheral pulmonary vessel involvement.^[3],[4] IVL with intracardiac extension needs to be distinguished from cardiac tumor such as right atrial myxoma.^{[5],[6],[7],[8]} IVL involving inferior vena cava (IVC) needs to be differentiated from Budd–Chiari syndrome, IVC thrombus, kidney and liver cancer extending to IVC, IVC angioleiomyoma, and IVC leiomyosarcomas.

Case Report

A 36-year-old female came with a history of palpitation for 1-month duration. She also complained of amenorrhea for the past 7 months and abdominal distension for the past 2 months. Seven months prior, the patient was evaluated by a gynecologist and was found to have fibroid uterus. She was anemic and had complaints of generalized weakness. No history of orthopnea/paroxysmal nocturnal dyspnea/pedal edema was present. Obstetric history was P2 L2. The last child was a girl who was now 10 years old and healthy. The patient underwent sterilization operation 10 years ago. There was no history of diabetes mellitus/sustained hypertension/tuberculosis/heart disease in the past. Abdominal distension was present more on the right side of the abdomen with doughy feeling of the abdomen on palpation. Per-speculum examination done by a gynecologist showed cervical os closed, and there was no abnormal discharge per vagina.

Investigations

Laboratory investigations showed anemia (hemoglobn 8.0 g/dL), normal renal function (blood urea 26 mg/dL, serum creatinine 0.9 mg/dL), normal random blood glucose (98 mg/dL), normal serum bilirubin (0.7 mg/dL) and normal prothrombin time and international normalized ratio (13.8 sec, 1.15, respectively).

12-lead electrocardiography showed sinus tachycardia without any significant abnormality [Figure 1].

Figure 1: Pictorial diagram showing journey of uterine mass to heart. IVC: Inferior vena cava

Click here to view

Echocardiogram showed dilated right atrium (RA) and right ventricle (RV) with multiple spindle-shaped, homogenous masses of varying sizes with well-defined border, occupying 90% of RA and moving in and out of the RV with cardiac contraction [Video 1].

Video 1: Apical four-chamber view showing a mass in the right atrium and right ventricle.

[Additional file 1]

Subcostal view showed that IVC was dilated and filled with a homogenous mass with well-defined border which was occupying 90% of the lumen. The mass was extending from the proximal part of IVC to RA [Video 2].

Video 2: Subcostal view showing a mass invaginating from the inferior vena cava to the right atrium.

[Additional file 2]

Ultrasound abdomen showed bulky uterus with multiple fibroids and mild hydronephrosis.

Computed tomography of kidney, bladder done showed no significant abnormality. Contrast-enhanced computed tomography chest also showed no significant abnormality in the trachea, bronchus, or lung.

Magnetic resonance imaging abdomen showed bulky uterus of size 20 cm × 16 cm with multiple large degenerative fibroids. Posterior wall of the uterus was compressing both the ureters. Right adnexal adhesion was present.

Cardiothoracic and surgical oncology opinion was taken, and a combined single-stage procedure was planned.

Surgery was done with median sternotomy and laparotomy on the beating heart. Initially, hysterectomy was done; then, the mass was removed after opening IVC and later RA was opened and the mass was mobilized proximally toward IVC. Total organs removed were bulky uterus measuring 25 cm × 20 cm × 17 cm, one ovary measuring 3 cm × 2 cm × 1 cm, and one fallopian tube of 2-cm length. Three linear masses measuring 13 cm, 9 cm and 7 cm in length were removed from the IVC. Mesentery measuring 30 cm in length along with 5 intra-abdominal lymph nodes was also removed [Figure 2]a and [Figure 2]b.

Figure 2: (a) Intraoperative image; (b) tissues removed during surgery

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Histopathological and microscopic report showed features of leiomyoma and leiomyomatosis involving IVC, RA, and RV. Lymph node biopsy showed reactive hyperplasia. Ovary and fallopian tube biopsy specimen showed normal histology and microscopy.

At the last follow-up at 4 months, the patient showed no evidence of recurrence. She also had no abdominal and cardiac complaints.

Discussion

IVL typically affects premenopausal women, particularly those who have undergone hysterectomy or myomectomy for uterine leiomyoma. Previous reports indicate that IVL develops in only 0.1% of uterine leiomyomas and that 10%–40% of IVL cases exhibit intracardiac involvement.^[2] Mechanical obstruction will occur in 10%–30% of the patients due to the tumor involving the IVC and right cardiac cavity as well as lung. Approximately 0.1% of the patients suffer sudden death due to intracardiac obstruction.^[7] Tumor mass removal is the treatment of choice. One-stage or two-stage surgical approach is usually considered. Many cases reported earlier were diagnosed intraoperatively during surgery for RA/RV mass. However, in our case, single-stage approach was considered as intravenous extension of mass in the abdomen was well diagnosed at presentation. Following surgery, IVL recurrence rate of 30% from 7-month to 17-year follow-up has been reported. Hence, all the patients need long-term follow-up and have to undergo another surgery if necessary.^[9]

Knauer proposed that the neoplasm arose from estrogen-induced smooth muscle cell proliferation in the wall of the uterine veins,^[10] while Sitzenfry declared that the neoplasm originated from uterine leiomyoma that invaded the venous system. IVL has been linked to specific chromosome involvement. It has been proposed that IVL arises from a uterine leiomyoma with a t(12; 14)(q15; q24). The presence of an extra copy of 12q15-qter and/or loss of 14q24-qter may be a critical genetic event leading to intravascular intrusion and proliferation.^[11] One study has shown that HOXA13 RNA sequence detected by reverse transcription – polymerase chain reaction method helps in differentiating uterine myxoma from IVL.^[12] Despite their histological benignity, these lesions have a tendency to metastasize and are closely related to the conditions called “benign metastasizing leiomyoma” and “intracaval mass and cardiac extension.” The primary treatment of IVL is hysterectomy and excision of any extrauterine tumor, when technically feasible. Anti-estrogenic therapy has been suggested as potentially useful in controlling unresectable tumors because of their location, such as when there is peripheral pulmonary artery involvement.

Implication

Cardiac tumors are rare clinical problems. This case report highlights a remote possibility of a common extracardiac tumor such as fibromyoma invading intravenous circulation and reaching the heart and presenting as a cardiac problem.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initial s will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Acknowledgement

We are thankful to Dr. Prof. Joseph Raj, Dr.Prof.Rajkumar and Dr. Prof.Sritharan for successfully performing the surgery in this patient.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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