A Case of Intravenous Leiomyoma of Uterus Extending to Pulmonary Artery

doi:10.21203/rs.3.rs-1725512/v1

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Case Report

A Case of Intravenous Leiomyoma of Uterus Extending to Pulmonary Artery

https://doi.org/10.21203/rs.3.rs-1725512/v1

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Background: Uterine intravenous leiomyomatosis is the leiomyoma tissue invaded into the vein outside the leiomyoma ,Reports of extending to the right pulmonary artery are relatively rare.

Case presentation: We describe a 31 year-old woman with the benign leiomyoma entended to the right ventricular lumen,, causing mechanical obstruction and corresponding clinical symptoms. The tumor located in the pulmonary artery can cause pulmonary artery obstruction, once diagnosed, surgical treatment should be performed as soon as possible.

Conclusions: In this case ,uterine leiomyoma extends to the right pulmonary is clinically more rare.

Pulmonary arteriovenous disease

hysteromyoma

cardiac surgical operation

The patient, female, 31 years old, was admitted to the hospital due to “paroxysmal syncope for 4 days”. The patient suddenly felt chest tightness and fell down due to syncope when she washed up in the morning 4 days ago. After about 2 minutes, she remitted spontaneously but felt exhausted after waking up. In the following 3 days, she had the same symptom and remitted in the same way. For making a definite diagnosis, she was admitted to our department on Nov. 27, 2021. Hysterectomy was operated for “hysteromyoma” in Sep. 2020. Physical examination: body temperature: 36.0 degree centigrade, pulse: 100 times/min, breath: 21 times/min, blood pressure: 100/85 mm Hg (1mm Hg = 0.133kPa); a 3cm traumatic scratch could be seen at the corner of the right eye; double lung breath sounds were heavy, no obvious dry and wet rales were heard, and heart boundary was not big, with low and blunt heart sound and even heart rhythm; the tumor flapping sound could be heard in the tricuspid valve auscultation area with conduction limitation. The abdomen was flat and soft, the liver, spleen and ribs were not touched, and a 10cm long transverse incision scar was seen below the umbilicus. There was no edema in both lower limbs, and the arterial pulses of the limbs were good; the vulva was normal and the vaginal stump healed well; there was a solid lump with the size as a small duck egg on the right side of pelvic cavity, with unclear boundary on the left side, inactive. At the second day after admission, the patient suddenly lost consciousness when using the toilet and didn’t respond to calls. After about 10 seconds, she remitted spontaneously and had no abnormal vital signs. At 04:35 AM, Nov. 31, 2021, the patient suffered from syncope again when defecating in in bed, accompanied by cyanosis of the lips, without urinary and fecal incontinence. Syncope lasted for about 10 minutes. After successful bedside cardiopulmonary resuscitation, the patient was transferred to CCU for further treatment. Auxiliary examination: blood routine examination: white blood cell count 11.88*10⁹/L, neutrophilic granulocyte percentage of 78.11%; urine routine, stool routine, liver and kidney function, electrolyte, thyroid function, plasma D-2 polymer and B-type natriuretic peptide were basically normal. Coagulation routine, carcinoembryonic antigen assay, alpha-fetoprotein assay, infectious disease screening test, high-sensitivity C-reactive protein assay, and erythrocyte sedimentation rate test were normal. Arterial blood gas analysis: oxygen partial pressure of 147.7 mm Hg, and oxyhemoglobin saturation of 99.2%. ECG: 1. sinus rhythm; 2. ECG with normal range. Cardiac ultrasound: The right ventricle was relatively enlarged, and the left atrium and left ventricle were not large. Multiple moderately heterogeneous echogenic nodules were observed in the right atrium and right ventricle, with loose internal structures and irregular boundaries, one of which was attached to the anterior valve body of the tricuspid valve, with the size of about 5.7cm*3.6cm, and swung between right atrium and right ventricle with cardiac cycle. A number of them were attached to the free wall of the right ventricle, and prolapsed into the right ventricular outflow tract during the systolic phase, one of which was 3.8cm*2.1cm in size, and no abnormal space was found in the proximal segment of superior and inferior vena cava. Chest far-reaching radiograph: lung texture was heavy, aortic node was slightly wide, pulmonary artery segment was flat, right cardiac margin was round, right atrium was enlarged, and heart-chest ratio was 0.51. Abdominal and pelvic ultrasound: No abnormality was seen in the ultrasonography and blood flow of liver, gallbladder, spleen, pancreas and kidney, and after the hysterectomy, no obvious mass images were found in bilateral adnexal areas. CT: In the proximal cardiac segment from the right iliac vein to the inferior vena cava, a cord-like low-density shadow was seen with irregular margins. On delayed scanning, the mass was enhanced. In the systolic phase, the mass protruded through the pulmonary valve into the main pulmonary trunk and right pulmonary artery, and in the diastolic phase, the mass retracted into the right ventricle. Pulmonary artery was not wide; the diameter of main pulmonary artery was 26cm; the filling defect shadow can be seen in right upper pulmonary artery and pulmonary artery of basal segment (Figure 1). An oval soft tissue shadow was seen in the upper right of the bladder in the pelvic cavity. The CT value was 78Hu and the size was 58.8cm * 32.9cm.

Diagnosis on admission: Cardiac tumor, and myxoma of right atrium and right ventricle. Intracardiac tumor resection was performed under general anesthesia, room temperature and extracorporeal circulation on Nov. 31, 2021. Brief operation process: Disinfected with conventional active iodine, median thoracotomy was operated, and pericardium was opened; extracorporeal circulation was set up with intubation tube intubating in ascending aorta, superior vena cava and femoral vein; through the incision in the lower right atrium, the mass was cut off as close as possible to the inferior vena cava and sent it for medical examination. Visual observation: The tumor came from the inferior vena cava in a long strip, with a diameter of about 1.5~2.5cm and a resection length of about 20cm. The surface was smooth and the capsule was intact. The tumor discontinuously entered the right pulmonary artery. TEE showed during the operation: Abnormal echo disappeared in heart and pulmonary artery. There was no anesthetic accident during the operation. Postoperative examination (Figure 2) suggested intravenous leiomyomatosis. Cardiac ultrasound was performed postoperatively: After the excision of the mass in the inferior vena cava and right ventricle, the right atrium and right ventricle were smaller than those before the operation, the right ventricle was not large, there was no abnormal echo in the cardiac cavity, and the proximal segment of the inferior vena cava was not abnormal. Postoperative chest X-ray and electrocardiogram were normal. The patient was cured and left the hospital, and two-stage operation was done after the rehabilitation. On Dec. 17, 2021, the masses in the inferior vena cava, right iliac vein and abdomen were resected, and postoperative examination results indicated that the disease was of the same origin as the heart mass.

Uterine intravenous leiomyomatosis refers to that with a clear clinical history of uterine fibroids, the leiomyoma seen by the naked eye extends into the vascular space and can grow in small, medium and large veins; the benign leiomyoma tissue invaded into the vein outside the leiomyoma and rarely extended to the right ventricular lumen, causing mechanical obstruction and corresponding clinical symptoms.^[1] Compared with 2 previous cases of leiomyoma invading right atrium and right ventricle, uterine leiomyoma extends to the right pulmonary artery in this case, which is clinically more rare. 70% ~ 80% of this disease occurs in women aged from 30 to 50 years old, and there may be two occurrence mechanisms: Most cases are related to uterine leiomyoma, which is caused by one or more uterine leiomyomas with extensive vascular invasion; a few tumors originate directly from the smooth muscle of the venous wall. Right heart tumor presents a variety of clinical manifestations^[2], lacking specificity, and varies with tumor location, tumor size and tumor nature, generally including systemic manifestations and cardiac manifestations, such as fever, body mass reduction, anemia, shortness of breath, palpitations, syncope and embolism. For example, if the tumor is close to the superior vena cava inlet, it mainly causes superior vena cava obstruction syndrome. If the tumor is located at the inferior vena cava inlet, it may cause inferior vena cava obstruction. If the tumor is located near the tricuspid valve, it often causes tricuspid valve destruction, leading to incomplete closure of the tricuspid valve. In the case of tricuspid valve occlusion, syncope or sudden death may result. The tumor located in the pulmonary artery can cause pulmonary artery obstruction, increase of right ventricular load, and the occurrence of right heart failure, which is easy to be misdiagnosed as pulmonary artery embolism. Part of metastatic cardiac tumor is the first clinical manifestation of cardiovascular system symptoms, and few abnormalities of primary lesions are easy to cause misdiagnosis. The possibility of metastatic neoplasm should be considered if it is diagnosed as a right ventricular tumor involving the superior or inferior lumen, even if there is no evidence of primary lesion. Compared with other imaging equipment, methods and techniques, 256-slice spiral CT can clearly show the cardiac structure and tumor shape, size, part and activity, which can provide a better basis for diagnosis. For patients with tumor-induced obstruction, valve failure or risk of peripheral embolism, once diagnosed, surgical treatment should be performed as soon as possible. There are mainly two surgical methods: one-stage radical cure, staging operation.^[3] The one-stage radical cure is suitable for patients with good general conditions and mild circulatory disorders. Right heart tumor is resected under extracorporeal circulation, uterus, appendage and other pathological tissues are removed via abdomen after stable circulation, and pelvic dissection is performed. For the staging operation, Gynecological surgery or venous surgery involving the abdomen is performed 2 to 4 weeks after open heart surgery. The former has long operation time, big trauma and many postoperative complications, but the staging operation avoids the abovementioned disadvantages. The previous two similar cases in our hospital performed the staging operation treatment.

According to some reports, estrogen receptor was found in the tumor invading the vena cava and right atrium. It was believed that the occurrence and evolution of this disease were related to estrogen^[4], and the effect of estrogen in the body could promote tumor growth and recurrence, thus affecting its prognosis. Therefore, it is effective to prevent the recurrence and spread of the disease if anti-estrogen drugs are used as adjuvant therapy after the operation.

Ethics approval and consent to participate: Informed written consent was obtained from the patient for publication of the case details.

Consent for publication: Written informed consent for publication of their clinical details and clinical images was obtained from the patient .A copy of the consent form is available for review by the Editor of this journal

Availability of data and materials: Data sharing not applicable to this article as no datasets were generated or analyzed during the current study

Competing interests: We declare no competing interest.

Funding: The authors have no funding

Author’s contributions: Yuanqin Huang wrote a first draft of this manuscript. Dan Rao edited one additional drafts of this manuscript. Quan Gan revised the manuscript. Zhenlu Zhang creats both figures.. All authors edited the manuscript and approved the final draft.

Acknowledgements: Special thanks to the Clinical Lab for their relentless support in collecting and storing the pathological specimen.. Moreover ,many thanks to Zhenlu Zhang for his great amount of assistance in collecting and storing the patient’s samples.

Holly R Harris,Jessica L Petrick,Lynn Rosenberg, et al. The epidemiology of uterine fibroids: Where do we go from here? [J].Fertil Steril,2022,117(4):841-842
Geum Seon Sohn,SiHyun Cho,Yong Man Kim, et al. Current medical treatment of uterine fibroids. [J].Obstet Gynecol Sci,2018,61(2):192-201
Liqiang Huang,Gaofeng Shi,Qi Wang, et al. Pulmonary and mediastinum metastasis of uterine leiomyoma: A case report. [J].Medicine (Baltimore),2019,98(49):e18276
J Julie Kim,Takeshi Kurita,Serdar E Bulun, et al. Progesterone action in endometrial cancer, endometriosis, uterine fibroids, and breast cancer. [J].Endocr Rev,2013,34(1):130-62

No competing interests reported.

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A Case of Intravenous Leiomyoma of Uterus Extending to Pulmonary Artery

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