Right atrium and inferior vena cava leiomyoma
Introduction
Brief introduction of right atrium and inferior vena cava leiomyomas The right atrium and inferior vena cava leiomyoma (Leiomyoma) refers to the leiomyomas that originated here, very rare, and the performance is the same as other benign tumors of the inferior vena cava. basic knowledge The proportion of illness: 0.001%-0.003% Susceptible population: This disease occurs in women, and is more common in premenopausal middle-aged women, with a history of pregnancy Mode of infection: non-infectious Complications: pleural effusion
Cause
Causes of right atrium and inferior vena cava leiomyomas
Pathological features (35%):
1 The uterus is irregularly enlarged. Like the uterine leiomyoma, there are multiple worm-like shapes on the uterine muscle wall, cord-like, nodular masses, ie veins filled with tumor tissue, and the tumor in the vein is grayish white. From 1mm filament to 2 to 3cm thick strip.
2 lesions can invade the broad ligament, attachment, pelvic wall tissue, and the veins in between, such as ovarian veins.
3 In a few cases, the lesion spreads along the vein or attaches to the vein wall, into the iliac vein, renal vein, inferior vena cava, right atrium; the mass located in the blood vessel and the heart chamber is elongated, up to several tens of centimeters The thickness is not equal, and the surface is smooth; the tumor is often in the lumen of the blood vessel, swinging with the direction of blood flow, and a small pedicle attached to the blood vessel wall. In the case reported by the author, the tumor was as long as 44 cm, and the author reported that the tumor was over 55 cm. Severe obstruction of the tricuspid valve or pulmonary artery can cause death.
4 This disease can cause pulmonary embolism, but no lung metastasis, and is different from leiomyosarcoma or benign metastatic uterine leiomyoma.
There are two possible sources of tissue for leiomyomatosis:
1 uterine smooth muscle tissue.
2 uterus, smooth muscle tissue of the pelvic vein wall, Norris and Parmley reported 14 cases of this disease, of which 4 cases of pathological examination support tissue derived from uterine smooth muscle tissue, 7 cases of pathological examination support tissue from the uterine vein wall smooth muscle tissue, another 3 The case is not conclusive.
The pathological examination of the disease has the following microscopic features:
1 Leiomyomas maintain a benign morphology with few mitotic figures or mitotic figures.
2 There are abundant thick-walled blood vessels in the tumor.
3 It can be seen that the benign smooth muscle bundle invades the vein or forms a huge embolus to fill the venous cavity.
The surface of the tumor in the 4 large blood vessels is covered with an intima or endothelial cell.
5Masson trichrome staining showed different amounts of collagen fibers added to the smooth muscle fibers.
Extension mechanism (30%):
The disease originates from uterine smooth muscle tissue or uterus and pelvic internal vein wall smooth muscle tissue, gradually grows into the vein, protrudes into the thin-walled blood vessel or sinusoids, pushes the endothelium or intima, and the tumor grows in the direction of blood flow and gradually extends. Long, the surface of the endothelium or intima also grows, so the surface of the tumor that protrudes into the blood vessel is covered with a smooth inner membrane or endothelium, which is different from the mechanism of malignant tumor invasion of blood vessels or blood.
Pathophysiology (30%):
According to the different physiological barriers caused by the extent and extent of the lesion, the disease can be divided into four phases:
(1) uterine and parametrial lesions: the early stage of the disease, the lesions are limited to the uterus and the palace, in addition to local symptoms, the body has little effect.
(2) pelvic venous phase: the lesion is limited to the pelvic or obstructed one side of the iliac vein, which can cause pelvic venous congestion. Due to the slow development of the lesion, the surface of the tumor protruding into the vein is smooth, plus the expansion of the vein and the compensation of the collateral circulation, generally no Obvious manifestations of circulatory dysfunction; if combined with thrombosis, it can produce a phenomenon similar to iliac vein thrombosis, resulting in lower extremity venous return disorder.
(3) Inferior vena cava phase: the tumor extends into the inferior vena cava. When the tumor volume is small, there is no obstruction of the inferior vena cava. After the tumor volume increases, different types of inferior vena cava can occur depending on the location of the obstruction. Reflux obstruction, the patient's clinical manifestations can be similar to the inferior vena cava syndrome, Budd-Chiari syndrome, etc.; if the renal vein is blocked, the superior mesenteric vein, there will be corresponding circulatory disorders.
(4) blocked period of the heart chamber: the tumor invades the right atrium, partially obstructs the tricuspid valve, and may have the manifestation of systemic congestion like right heart dysfunction, such as liver enlargement, ascites, oliguria, edema, etc.; Syncope or sudden death; if the pulmonary artery is severely blocked, it can also die.
Prevention
Prevention of right atrium and inferior vena cava leiomyomas
Right atrial inferior vena cava leiomyomatosis is rare, but its clinical and pathological aspects are specific; this disease occurs in women, derived from uterine and pelvic lesions, is a chronic progressive neoplastic disease, can cause The vena cava and right atrium obstruction cause severe hemodynamic disorder. Because the lesion is benign, the surgical treatment is effective, and should not be mistaken for the vena cava and intracardiac metastases of the uterus or pelvic malignant tumor. Surgical treatment.
1. In the heart, heart surgeons and obstetrics and gynecology should improve the understanding and vigilance of this disease, and conduct comprehensive and in-depth examination of suspicious patients, which is the key to the correct diagnosis of this disease.
2. Once the diagnosis is confirmed, early diagnosis and treatment should be performed to prevent complications.
Complication
Complications of right atrium and inferior vena cava leiomyomas Complications pleural effusion
There may be complications such as right heart dysfunction and inferior vena cava obstruction syndrome.
Symptom
Symptoms of right atrium and inferior vena cava leiomyomas Common symptoms Painful abdominal pain Edema Short pleural effusion Ascites Heart murmur syncope Right heart dysfunction Sudden death
The disease occurs in women, and is more common in premenopausal middle-aged women, with a history of pregnancy. When the tumor is confined to the uterus, para-uterine or pelvic, the clinical manifestations are not much different from the general uterine fibroids, such as uterus enlargement, Increased menstruation, pelvic mass, abdominal pain and other symptoms, if the lesion spread to the inferior vena cava and right atrium caused by circulatory disorder, flustered, shortness of breath, liver, oliguria, ascites, lower extremity edema, pleural effusion, heart murmur and other clinical manifestations It seems that right heart dysfunction is also a type of Budd-Chiari syndrome. Some patients may have no symptoms. Only when they have physical examination for other reasons, they will find heart murmur. Further ultrasound examination will reveal a right atrial tumor. The diagnosis is right heart. Tumors neglect the tumors of the inferior vena cava and pelvic and uterine lesions. Individual patients have syncope or sudden death, mostly due to changes in tumor position, incarcerated obstruction in the tricuspid valve or pulmonary artery. Many patients have a history of hysterectomy. I think that the general uterine leiomyoma is neglected by intravenous tumors.
Examine
Examination of right atrium and inferior vena cava leiomyomas
1. Echocardiography: It can be confirmed whether there is a mass in the right heart chamber, the location of the tumor, the relationship with the tricuspid valve, the pulmonary artery, the displacement, size and movement state of the tumor during the cardiac cycle.
2. Transesophageal ultrasound: It can clearly show that the tumor grows into the right atrium from the inferior vena cava, which can help identify other tumors, myxomas, etc.
3. CT examination and magnetic resonance examination: It can reveal the location, size, extent of obstruction, and the nature of the mass in the inferior vena cava.
4. Transfemoral venous venography: The size, length, and degree of circulatory obstruction can be determined, and a complete and continuous image can be provided.
Diagnosis
Diagnosis and differentiation of right atrium and inferior vena cava leiomyomas
diagnosis
To improve the understanding and vigilance of this disease, comprehensive and in-depth examination of suspicious patients is the key to the correct diagnosis of this disease. For patients with multiple uterine fibroids, lesions involving parametrial tissue or accessories, further examination should be conducted to exclude the If the uterus is surgically removed, gross pathological examination and microscopic examination should be performed carefully to identify whether the disease is present. For patients with right heart cavity tumors, especially female patients, the history of uterine fibroids and the history of uterine surgery should be asked. And gynecological examination; at the same time comprehensive examination, pay attention to the presence or absence of tumors connected to the heart tumor in the inferior vena cava.
Echocardiography, abdominal B-mode ultrasonography, CT examination, magnetic resonance examination, inferior vena cava and right atrial angiography are used to determine the diagnosis of the disease, establish the extent of tumor expansion, estimate the size of the tumor and the degree of circulatory disorders, and surgery. The choice of approach is decisive.
In summary, the diagnostic criteria for inferior vena cava and right atrial leiomyomatosis are as follows:
1. There are uterine fibroids: or female patients with a history of uterine fibroids or uterine surgery.
2. There is a heart murmur: similar to the performance of right heart dysfunction, or inferior vena cava obstruction syndrome, or Budd Chiari syndrome.
3. Ultrasound: CT and magnetic resonance examination, or angiographic examination revealed a mass or space-occupying lesion in the right heart chamber and inferior vena cava.
Differential diagnosis
1. Vascular and cardiac cavity metastasis of uterine leiomyosarcoma: benign neoplastic metastasis of uterine leiomyoma, the metastatic lesions of both diseases are intermittent, unlike the disease, which is a long strip of continuous mass, and There are lung metastases, and detailed examination is not difficult to distinguish.
2. Right heart cavity tumors: such as myxoma, fibroids, rhabdomyomas, etc., are located only in the heart, no tumor in the vena cava.
3. Endometrial stromal fibroids in the lymphatic vessels, uterine lesions can be similar to this disease, but do not extend to the blood vessels, local lesions can be identified by pathological section.
