esophageal leiomyoma
Introduction
Introduction to esophageal leiomyoma Esophageal leiomyoma (leiomyomaofesophagus) is a benign tumor originating from the esophageal smooth muscle. The site of occurrence is more common in the middle part of the esophagus, followed by the lower part, and the neck is rare. Most of the lesions occur in the wall, and the rest can be in the cavity, under the adventitia. And a small number of diffuse, so that the esophageal muscle layer is extensive tumor-like hyperplasia. Benign esophageal tumors are rare, accounting for about 0.5 to 0.8 of all esophageal tumors. Because the symptoms are mild or asymptomatic, patients often do not seek medical treatment or are ignored by clinicians. In recent years, due to advances in X-ray and other examination techniques, the number of cases discovered has gradually increased, 90% of which are esophageal leiomyoma. Esophageal leiomyoma is the most common benign esophageal tumor, accounting for the first place in benign esophageal tumors, accounting for 3/4 of benign tumors in the esophageal wall, and the ratio of esophageal cancer is 1:127 to 233. Esophageal leiomyomas are generally small and often have no clinical symptoms. The actual incidence is still unclear. Most authors believe that the incidence of esophageal leiomyoma should be higher than the literature statistics, and the autopsy discovery rate is 1100:1. According to the large-scale literature reports in recent years, esophageal leiomyomas account for 5% to 10% of the gastrointestinal leiomyoma, accounting for 50% to 80% of all esophageal benign tumors and cysts. basic knowledge The proportion of sickness: 0.2% - 0.5% Susceptible people: no specific population Mode of infection: non-infectious Complications: hiatal hernia, esophageal diverticulum, sudden death
Cause
Esophageal leiomyoma etiology
(1) Causes of the disease
The cause of esophageal leiomyomas is unclear, occurring in the esophageal muscularis, but also from the vascular muscle layer of the esophageal wall and the vagus embryonic muscle tissue. The study of esophageal leiomyomas shows about 74% of leiomyoma. Originated from the inner ring muscle, 18% originated from the submucosal muscle layer, 8% originated from the external longitudinal muscle, the tumor was expansive to the lumen, and the outer growth, 97% of the tumor was intramural growth, 2% to the mediastinum, 1 % tumors protrude into the esophageal lumen, with pedicles such as polyps. The leiomyomas originating from the inner ring muscle grow in the muscle along the long diameter of the esophagus. They protrude into the cavity due to low mucosal and submucosal resistance, originating from the submucosal muscle. The leiomyomas of the layer are more likely to protrude into the cavity, even polypoid. The leiomyomas originating from the external longitudinal muscle can grow outside the esophagus and are sometimes mistaken for mediastinal masses.
(two) pathogenesis
Esophageal leiomyosarcoma originates from the esophageal muscularis, mainly longitudinal muscles, most of which are in the esophageal wall, that is, the outer wall of the mucosa is in the shape, and the individual protruding into the lumen is polypoid, and the pedicle is connected to the esophageal wall. There are reports of vomiting from the mouth, such patients may also block the respiratory tract during vomiting and cause asphyxia. The foreign literature of esophageal leiomyomas is reported in the lower esophagus, followed by the middle segment, the upper segment is rare, and the domestic report is in the middle of the esophagus. More common, the next paragraph, the last paragraph is rare, the vast majority of tumors are single, a few are multiple, the number of multiple hair is variable, ranging from two to ten, due to the length of the disease, the size varies greatly, some people report the largest diameter 28cm, the smallest diameter of 1cm, but 85% of the tumor diameter <4cm, the minimum weight of 0.25g, the most reported in the literature reached 5000g.
Tumors generally have different shapes, mostly round or oval, accounting for 60% of all cases, irregular (ginger shape) (Fig. 1) accounting for 10%, horseshoe shape accounting for 8%, and other irregular shapes such as spiral shape. Dumbbell-shaped, cord-shaped, nodular or lobulated, or surrounding the esophagus.
The surface of the tumor is smooth, with a complete fibrous envelope, tough texture, easy to separate from the esophageal mucosa. The esophageal leiomyomas can be seen with criss-crossing muscle bundles. The blood vessels are sparse, grayish white, or yellow. The cut surface sometimes shows focal hemorrhage, liquefaction or Calcification, etc., can be seen in the form of bundles, braided or swirling smooth muscle fiber bundles, the cells are long spindle-shaped, well differentiated, rich in cytoplasm, eosinophilic, clear boundaries, nucleus fusiform, no atypical mitotic figures, Some of the fibers are mucoid or hyalin degeneration, and a few of them show calcium deposits. Unlike other leiomyomas in other parts of the digestive tract, esophageal leiomyomas have few malignant changes, and the reported malignant rate is about 0.24% to 3.3%.
Prevention
Esophageal leiomyoma prevention
When diagnosing leiomyomas, the possibility of esophageal malignant tumors must be considered for the identification and exclusion. Mucosal biopsy should not be performed during esophagoscopy to avoid damage and mucosal adhesion to the tumor, which may hinder subsequent surgical removal. The effect is good, the trauma is small and the complications are less than extramucosal tumor removal. The partial esophagectomy should not be performed lightly.
Complication
Esophageal leiomyoma complications Complications of esophageal hiatus and esophageal diverticulum
The disease often occurs with some concomitant diseases, including:
1, esophageal cancer (there is no direct relationship between the two, because esophageal cancer is a frequently-occurring disease), hiatal hernia, diverticulum, esophageal hemangioma and achalasia.
2, esophageal leiomyomas complicated with postoperative esophageal fistula, pulmonary infection, anastomotic stenosis cases have also been reported, but the general situation is easy to control.
3, the tumor can cause surface mucosal ulcer necrosis, can be combined with esophageal hiatus hernia, esophageal diverticulum, and even cause esophageal perforation, there are reports of tumor causing suffocation, esophageal leiomyoma patients can also be combined with esophageal cancer, the above complications can mask smoothing The clinical symptoms of fibroids cause missed diagnosis.
Symptom
Symptoms of esophageal leiomyomas Common symptoms Hemorrhoids repeatedly vomiting blood nausea and abdominal distension between the airway and esophagus
The age of onset is more common in 20 to 60 years old, and males are 2 to 3 times more than females. It has not been reported in infants and young children, but it can be involved in any age. Because most esophageal leiomyomas are small in size, they grow slowly. More than half of the patients did not have any clinical symptoms. For other reasons, chest X-ray examination or upper gastrointestinal barium meal examination showed esophageal leiomyoma. If the patient has symptoms, the duration is longer, as early as the last century 40 In the ages, Schatzki and Hawes followed up with upper gastrointestinal barium meal examination in patients with esophageal leiomyoma, and saw that there was little or no increase in tumors. In 1977, Glanz and Grtmebaum reported 9 cases. Long-term follow-up of patients with esophageal leiomyoma, 2 of which were followed up for 15 years, showed no significant changes in tumor X-ray findings. Posthhwait and Lower reported in 1991 that they had 1 case of esophageal leiomyoma with chronic renal failure. The patient was followed up for 8 years, and the X-ray signs of the tumor did not change during the follow-up period. If the patient with esophageal leiomyoma had clinical symptoms, the main manifestation was difficulty in swallowing, which was found in 45% to 56% of the disease. In the case, unlike esophageal cancer, the progress is slow or intermittent, to a lesser extent. Generally, the esophageal obstruction caused by leiomyomas is not serious. About 1/3 of the patients have pain or fullness in the posterior or upper abdomen. Sense, most patients have a history of many years, another 1/3 of patients have acid reflux, hernia, loss of appetite and other non-specific gastrointestinal dysfunction, tumors in a few cases can increase, even compress the airway and respiratory symptoms Difficulty breathing, hoarseness, post-sternal pain, etc., less systemic symptoms, no adverse effects on nutrition, symptoms generally have a certain relationship with tumor size, location and shape, but there are also cases where the tumor has been reported to be 6cm or even 15cm long, no symptoms Conversely, 1.5 cm long tumors have difficulty swallowing. In 1972 and 1980, Ullal and Kaymakcalan reported 1 case of esophageal leiomyoma with hypertrophic osteoarthrosis. After surgical resection of the tumor, hypertrophic bone and joint The disease performance quickly subsided.
Common symptoms
Dysphagia
It is the most common clinical symptom, its development is slow, intermittent, and not serious. It is completely different from the progressive dysphagia caused by esophageal malignant tumor. The interval between no dysphagia and dysphagia is generally long. The severity of dysphagia is not necessarily related to the size and location of the tumor, depending on the extent to which the tumor surrounds the esophageal lumen.
2. Pain or discomfort
It manifests as a variety of pains or discomforts under the sternum, under the xiphoid or upper abdomen, including the pain and fullness of the upper abdomen. The pain can be released to the back or shoulders. It has nothing to do with diet. The patients complaint is Upper abdominal fullness, pressure or upper abdominal pain.
3. Other gastrointestinal symptoms
Including loss of appetite, nausea, belching, nausea and vomiting, these symptoms are non-specific gastrointestinal symptoms, and the second common symptom of patients with esophageal leiomyoma, some patients have heartburn, may merge with hiatal hiatus According to Gray et al. (1961), about 1/2 of patients have weight loss and weight loss. Very few esophageal leiomyomas spread to the stomach wall and under the mucosa, which can form ulcers on the surface of the local gastric mucosa and cause Bleeding, the patient's clinical manifestations are repeated hematemesis.
4. Respiratory symptoms
Some patients with esophageal leiomyoma occasionally have respiratory symptoms such as cough, difficulty breathing or asthma, which may be caused by aspiration, tumor compression of the trachea or bronchus, or huge leiomyomas that compress the lung tissue.
Due to the slow growth of esophageal leiomyoma, the above symptoms can last for several years.
Bnmeton is equivalent to the statistical symptoms of 173 cases of gastrointestinal smooth muscle tumors in 1981, as follows: 73 cases (42%) with dysphagia, 59 cases (34%) with pain, 3 cases (2%) with gastrointestinal bleeding, and 38 cases with asymptomatic (twenty two%).
5. Other rare clinical manifestations 1
In 972 and 1980, Ullal and Kaymakcalan reported that one patient with esophageal leiomyomas had a manifestation of hypertrophic osteoarthrosis (ie, Marie-Bamberger syndrome), and the performance of hypertrophic osteoarthrosis after surgical removal of the tumor. It quickly faded.
In 1980, Schabel and Rittenberg reported that a 7×7 cm esophageal leiomyomas caused mucosal ulceration and local esophageal perforation on the surface of the tumor. The patient was surgically removed and the esophageal perforation was repaired and the patient was discharged.
In 1985, Peacook et al reported that a 27-year-old female esophageal leiomyomas died of suffocation. This patient had no clinical symptoms before birth. The autopsy found that there was a 7.5 cm diameter smooth muscle on the anterior wall of the esophagus at the level of tracheal carina. The tumor compresses the tracheal membrane, causing the patient to suffocate and die. The doctor can learn from this patient. If there is a large case of esophageal leiomyoma with compressed trachea in clinical medical work, it should be recommended to perform surgery in time. Treatment to prevent accidents from happening.
Clinical symptoms are clues to the discovery of this disease, can not be diagnosed with this, the diagnosis of esophageal leiomyoma is mainly based on esophageal X-ray barium meal examination and endoscopy, clinical signs and other examinations are not helpful for diagnosis.
The tumor is round, elliptical, and has irregular shapes, such as lobulated, spiral, ginger-shaped, horseshoe-shaped around the esophagus. Esophageal leiomyomatosis has multiple tumors that can thicken the entire esophageal wall. There are certain difficulties in diagnosis. The tumor is tough, with a complete envelope, smooth surface, mainly growing outside the cavity, slow growth, white or yellow cut surface, and tissue sections are well-differentiated smooth muscle cells, long fusiform, border Clearly, the tumor cells are arranged in a bundle or a spiral, in which a certain amount of fibrous tissue is mixed, and nerve tissue is occasionally seen. Esophageal leiomyoma becomes sarcoma, and some literature reports 10.8%, but some scholars It is believed that sarcoma is another independent disease and there is no direct evidence that it is caused by malignant transformation of smooth osteoma.
Examine
Examination of esophageal leiomyoma
X-ray diagnosis
(1) Chest X-ray film: 8% to 18% reported tumor shadows, calcification plaques were seen in fibroids in about 1.8% of cases, and tumors were misdiagnosed as posterior mediastinal tumors, and 60% of soft tissue shadows were reported in China. The leiomyomas with larger esophageal growth are ejected from the mediastinal pleura to the lung field. Soft tissue shadows can be seen from the flat slices of the cells. The visible rate is 8% to 18%, which should be considered in the differential diagnosis of mediastinal tumors. In this disease, calcifications can be seen on the flat slices of individual leiomyomas, and some reports reach 1.8%.
(2) esophageal barium meal examination: esophageal barium meal examination is one of the main methods to diagnose this disease, its X-ray performance depends on tumor size, morphology and growth mode, intraluminal filling defect is a common X-ray manifestation, the defect is mostly half-moon shape The boundary is clear, and the angle between the upper and lower ends of the filling defect and the normal esophagus is slightly acute or slightly obtuse with the tumor protruding into the esophageal cavity, and the surface mucosa is intact, and the mucosal folds of the lesion disappear as a "smear sign" or only a few rough lines. The esophageal lumen can be highly expanded, but the expectorant passes smoothly, the esophagus adjacent to the lesion is soft, and the peristalsis is normal.
When the tincture reaches the upper edge of the tumor, the slow and small amount of the remaining tincture outlines the upper and lower poles of the tumor, which is a cup-shaped sign with a semicircle perpendicular to the long axis of the esophagus.
Because the tumor supports a flat surface-covered mucosa, a thin layer of tincture is attached to it, which is a waterfall or smear.
The sacral column is shunted for the filling defect of the tumor, but the proximal end of the esophagus does not expand, and the passage of the esophagus is also unobstructed. Larger leiomyomas, especially those near the sacral sac, can make the esophagus flat and narrow, but in another From one angle, the esophagus can be widened, and the leiomyomas in the esophagus and stomach joint can deform and form the lumen.
As the tumor grows, the following signs can appear on the X-ray:
1 external pressure sign: the tumor is expansive and uniform growth, complete envelope, semi-circular compression on the esophagus, local convex to the cavity, the esophagus shows a semi-circular or curved filling defect, that is, external pressure notch Mass.
2 acute angle sign, ring sign and smear sign: due to the angle between the tumor protrusion and the esophageal wall junction, the tincture first fills the upper part of the tumor and forms an acute angle with the esophageal wall. The larger the tumor, The more clearly this angle is formed, the sputum is shunted down along both sides of the tumor, which constitutes the upper and lower edges of the tumor and the contrast of the sputum on both sides, and the tumor bulge is light, that is, the smear.
3 The tumor showed an expansive growth. The X-ray showed a local spindle-like change in the esophageal wall. The esophageal wall was flattened and the esophageal lumen was relatively narrow, while the expectorant was passed smoothly and the obstruction was not obvious.
4 Because the tumor generally does not invade the mucosa and does not form an ulcer, the mucosa is smooth and complete, and tumors within 1 cm below the wall are not easily found.
Most esophageal leiomyomas can be diagnosed by X-ray examination. Another purpose of barium meal examination is to find other complications, such as esophageal diverticulum and esophageal hiatal hernia. It is reported that the incidence of esophageal hiatal hernia is higher.
However, the following conditions are easily misdiagnosed:
1 the tumor is small, especially less than 1cm;
2 esophagography showed filling defect of esophageal mucosa;
3 esophageal cancer without destruction of esophageal mucosa;
4 combined with esophageal or cardia disease masks the imaging features of leiomyomas, clinical X-ray imaging patients with atypical esophageal leiomyoma should be further examined to confirm the diagnosis.
2. Endoscopic diagnosis
Esophagoscopy is also the main means of diagnosing the disease. It can identify the location, size, shape and number of the tumor. The fiber optic endoscope can be seen as a circular, elliptical or non-plastic mass protruding into the cavity. The surface mucosa is smooth and complete, and the color is good. Normally, when swallowing, the tumor has a slight up and down movement. The esophagus around the mass is soft and the movement is normal. When the tumor is large, the esophageal cavity is eccentrically narrow, but the wall is not stiff, and the endoscope can still pass without resistance. Because the texture of leiomyomas is hard and suspected to be a leiomyomas, biopsy should not be taken to avoid local submucosal tissue inflammation or infection. Tumor and tumor tissue adhesion may occur, causing mucosal damage when the tumor is removed by peeling the mucosa. The opportunity to increase complications, unless accompanied by local mucosal erosion, inflammation and difficult to diagnose, generally does not advocate biopsy on the normal mucosal surface of the tumor.
3. CT or MRI examination
The disease can be clearly diagnosed by X-ray esophagography and esophagoscopy, but there are a few cases, especially the middle thoracic esophageal leiomyoma is easily confused with aortic aneurysm, vascular impression or deformity. CT examination is helpful for differential diagnosis and diagnosis. To determine the nature of the lesion, CT examination can confirm the tissue type by measuring the decay coefficient, show the relationship between the tumor and the aorta, and determine the size and extent of the tumor, which is helpful to identify leiomyoma and esophageal cancer with submucosal growth. Can also show the relationship between tumor and aorta, avoid unnecessary angiography, CT scan, magnetic resonance imaging, CT and magnetic resonance (MRI) examination of esophageal barium meal and fiber esophagoscopy after most of the diagnosis can be clear, a small number of cases Especially in the middle of leiomyomas, sometimes mixed with aortic aneurysm, vascular compression or deformity, CT and MRI can help differential diagnosis, CT can also understand the expansion of the tumor to the outside of the tube and the exact location, help In the design of the surgical plan and incision, |B ultrasound can also find certain tumors.
4. Esophageal ultrasonography
Ultrasound endoscopy features a well-defined hypoechoic or weak echo with a well-defined boundary, with occasional echogenic lesions. A few patients have heterogeneous echoes and irregular edges, and the surface is a mucosa that is normally performed by ultrasound scanning, which usually occurs in the middle layer. Occasionally, it can also be seen on the second layer. A small number of patients have thickening of the muscularis, and the leiomyomas can be oppressed, but do not invade the surrounding tissues, accompanied by heterogeneous echoes, unclear or irregular submucosal tumors. Considering leiomyomas or leiomyosarcoma, images of inter-wall abnormalities and nearby lymph nodes can be found to help identify esophageal malignancies, esophageal varices, esophageal submucosal tumors, and posterior mediastinal tumors.
The esophageal cytology examination has no direct significance for the diagnosis of this disease, and is mainly used to distinguish from esophageal cancer.
Diagnosis
Diagnosis and differentiation of esophageal leiomyoma
diagnosis
It can be diagnosed based on medical history, clinical symptoms and laboratory tests.
X-ray esophageal barium meal examination is the main diagnostic method of this disease. Combined with clinical manifestations, it can often be diagnosed by angiography. The appearance of sputum meal depends on the size and growth pattern of the tumor. The filling defect in the cavity is the main performance, and the defect is round. Or elliptical, the edge is smooth and sharp, and the boundary with the normal esophagus is clear. The boundary between the upper and lower ends of the filling defect and the normal esophagus is acute or slightly obtuse with the tumor protruding into the lumen. The contour of the tumor perpendicular to the long axis of the esophagus in the orthotopic position is due to barium meal. The contrast shows a semi-circular shadow, and there is a ring sign. The mucosa of the tumor is ejected and the fold disappears. The tincture is less than the surrounding area, forming a thin layer, forming a waterfall sign or smear sign. The soft tissue shadow can be seen in the filling defect, and the sputum passing condition is observed under fluoroscopy. It stops slightly above the tumor, and then passes through the band between the tumor and the contralateral esophageal wall, like a small ditch, and the food near the tumor. The wall is soft, the contraction is good, the proximal esophagus does not expand, and multiple leiomyomas or horseshoe-shaped masses surround the esophagus, making the lumen uneven, and the mucosa does not show Clear, should pay attention to the identification of esophageal cancer, the latter wall stiffness, irregular filling defects, mucosal destruction and sputum, etc., esophageal leiomyomas and mediastinal tumor pressure changes are different: the latter wall filling defect Lighter, the tangential tumor and the wall of the tube become obtuse angles, the esophagus double side wall is simultaneously offset to one side, esophageal barium meal examination can also find other associated diseases, such as esophageal diverticulum, hiatal hernia.
Differential diagnosis
1. The esophageal leiomyomas with larger mediastinal tumors can cause soft tissue shadows in the mediastinum when they grow out of the wall. They are easily mistaken for mediastinal tumors. Therefore, the tumors with close relationship between the posterior mediastinum and the esophagus should not be satisfied with the diagnosis of mediastinal tumors. Should be alert to the presence of esophageal leiomyoma.
2, esophageal cancer multiple leiomyomas or irregular shaped mass surrounded by esophagus, resulting in uneven lumen, mucosal display is unclear and difficult to distinguish with esophageal cancer, esophageal cancer can be seen tube wall stiffness, filling defects irregular, mucosal damage and The characteristics of mucosal tumors such as sputum, some endoluminal esophageal cancer or carcinosarcoma can be similar to leiomyomas, but careful observation of mucosal irregularities, and no soft tissue shadow outside the cavity, larger esophageal esophageal involvement of the esophagus Longer, the mucosa of the lesion area is thin, and may be accompanied by hyperemia and other manifestations, so it is easy to mistake the mucosa during esophageal angiography and diagnosed as esophageal cancer.
3, mediastinal lymphadenopathy or inflammatory mass due to esophageal leiomyomas symptoms of dysphagia, barium meal examination showed a filling defect in the middle of the esophagus, esophagoscopy showed a smooth spherical lesion in the middle of the esophagus, which is in the mediastinal lymph node enlargement or Similar manifestations were found in cases of inflammatory masses. In this case, if a lateral radiograph or a CT scan is taken at the same time as esophageal barium angiography, it may be clearly diagnosed as external pressure esophageal obstruction.
4. Some physiological variations such as the external pressure of the right vagus subclavian artery or saccular aneurysm, the left main bronchus, and the smooth indentation area produced by the aortic arch, also need to be differentiated from the less common vertebral attachment compression, although the esophagus Barium meal examination is the first choice for the diagnosis of esophageal leiomyoma, but if it is difficult to identify with external pressure lesions, CT is an excellent further examination, especially in the lesions at the level of aortic arch and tracheal carina, CT examination appears to be more important.
5, other diseases Sometimes tuberculous lymphadenitis can invade part of the esophageal wall to produce similar changes with leiomyomas, tumors located in the lower esophagus must be distinguished from left atrial impression.
