orbital hemangiopericytoma
Introduction
Introduction to orbital vascular epithelioma Hemangiopericyma (hemangiopericytoma) is a tumor derived from the capillaries of the capillaries, characterized by the presence of reticular fibers on the periphery of the fusiform cells, arranged around the lumen of the blood vessels lined with a single layer of endothelial cells. This tumor is relatively rare, occurring in deep tissues, such as retroperitoneal, mediastinal and lower limbs, which are rare in the sputum. Most cases are reported in China or in a few cases. basic knowledge The proportion of illness: the incidence rate is about 0.004% - 0.007% Susceptible people: no special people Mode of infection: non-infectious Complications: nausea and vomiting
Cause
Causes of orbital vascular epithelioma
(1) Causes of the disease
Not very clear yet.
(two) pathogenesis
do not know yet.
Prevention
Orbital vascular epithelioma prevention
Mainly pay more attention to living habits, early detection and early treatment.
Complication
Ocular vascular epithelioma complications Complications, nausea and vomiting, eyelid closure
May be accompanied by severe head pain, vomiting, hernia closure, corneal exposure and so on.
Symptom
Symptoms of orbital vascular epithelial tumors Common symptoms Young people's eye bags, visual impairment, eyeballs, free movement, retinal edema, conjunctival congestion, eyelid edema, hyperemia, high intraocular pressure
The symptoms and signs of orbital hemangiomas are related to the nature and location of the lesion. In general, the symptoms and signs of the anterior sacral genitalia are mild, and the symptoms and signs of the posterior or posterior iliac crest are more obvious.
The tumor in the anterior part of the anterior part occurs in the upper part of the sputum. It can be swollen and swollen. It has medium hardness, smooth surface, movable, no tenderness. It is rich in blood supply to the tumor, and there is often anastomosis between the blood vessels. It nourishes the artery and exports the vein. The surface of the tumor is distorted and dilated. When it is close to the skin, it can be seen in purple. The tumor is located in the sputum. In the posterior segment, the main symptoms and signs are eyeballs, vision loss and eye movement disorder. The eyeballs are mostly progressive, axial. Prominent, if the tumor is on one side, the eyeball is removed to the front, and the tumor is displaced to the opposite side of the tumor. Although there is no tumor around the eyeball, the pressure inside the eyeball is high, the eyeball can not be included in the ankle, and the vision is reduced. Below 0.5, this is because the tumor is located at the tip of the iliac crest, oppressing the optic nerve, causing it to edema, atrophy, or oppression of the eyeball causing refractive error, retinal edema, degeneration, complete loss of vision, and eye movement limitation often occurs. Insufficient movement in the side or in multiple directions, the optic disc edema or atrophy can be seen in the fundus, which usually occurs in the tip of the ankle causing primary optic disc atrophy, oppression of the anterior segment , Optic disc edema caused by direct topical eye pressure, retinal visible flattened or flat anterior eminence, retinal edema or chorioretinal folds.
The symptoms and signs of benign angioendothelioma are mild, while those with malignancy are heavier. Some authors reported a case of a 10-year-old malignant hemangioperic cell tumor in 1994. The course of disease was only 1 month, the visual acuity decreased from 1.5 to 0.06, and the eyelid was highly edematous. Conjunctival edema congestion, fundus examination: the disc is red, the vein is highly distorted, the movement of the eyeball is restricted in all directions, the eyeball is protruded, the affected side is 11 mm, and the vision is completely lost after 1 day.
Examine
Examination of orbital vascular epithelioma
Pathological examination: Because of the easy recurrence and metastasis of angioendothelioma, it is generally considered to be a malignant tumor in the clinic, but it can be divided into benign, marginal and malignant from the pathological point of view.
The tumors are mostly pink to purple-red, similar to cavernous hemangioma. They are soft and elastic. Benign people are often covered with intact or incomplete capsules. Malignant hemangiomas are invasive and are found around the surgery. The structural boundaries are unclear, the blood vessels in the tumor are rich, and many blood vessel branches are "antler"-like. About 1/3 of the area is vascular sinus space, half of the area is solid and mixed with sinus space, only a small part is solid, and the blood vessels are lined with endothelium. The cells are attached to the basement membrane of reticular fibers and collagen fibers. The basement membrane is dense tumor cells, round or elliptical, with unclear borders, medium cytoplasm, clear nuclear membrane, small nucleoli, visible nuclear division. Croxatto and Font said that reticular fiber staining is helpful for the diagnosis of this disease. The tumor is rich in reticular fibers and radiates from the blood vessel wall to form a network. The tumor cells are located in the mesh, and the reticular fibers of each specimen are different. Occasionally, mucin-like degeneration, the number of mitotic figures can be used as the main basis for distinguishing the nature of tumors. Four or fewer mitotic cells are found in 40 high power fields, which are benign vascular epithelial tumors. 50% of the specimens belong to this category, 15% of the specimens are mildly metamorphosed, and mitosis is increased. Fourteen mitotic cells are found in every 40 high-power fields, which are good, malignant borderline lesions, 1/3 are malignant, and cell-shaped, 40 High-power field of view can find 35 mitotic tumor-like cells on average. The lesions have small vascular cavities, abundant cells, often necrotic and hemorrhagic areas. Although they can be divided into good, borderline and malignant under the microscope, this does not indicate the prognosis. Benign angioendothelioma may also have local recurrence and metastatic death.
Electron microscopy showed that the vascular epithelial cells were surrounded by the vascular lumen lined with endothelial cells. The cytoplasm was light, the nucleus was round or elliptical, the cytoplasmic organelles were sparse, and there were some prolonged organelle processes. The vascular epithelioma was a soft tissue tumor. Mesenchymal cells, immunohistochemical staining can be distinguished from other vascular neoplasms, which are positively stained for actin and vimentin.
1. X-ray examination of X-ray films showed increased density of sputum, longer duration of disease, long-term increase in intra-orbital pressure, non-specific sacral enlargement, angiography, especially DSA, is meaningful for diagnosis because the tumor is rich in blood supply and there is a short circuit between blood vessels. In addition to the discovery of ophthalmic artery displacement, there are more contrast agents in the tumor, which can show the contour of the lesion and the small vascular network in the arterial phase. This contrast image remains in the late stage of the vein, such as less intravascular vessels, with entities For the main structure, angiography is of little value.
2. Ultrasound exploration B-mode ultrasound can show the contour and acoustic characteristics of the tumor. Generally, benign vascular epithelioma shows a round or elliptical shape with clear boundaries. The malignant vascular epithelial cell tumor is irregular in shape and presents an invasive edge, that is, the boundary is unclear. It is not smooth, the echo in the tumor is much, the intensity and distribution depend on the tissue structure of the lesion. If there are more vascular sinus cavities in the lesion, the internal echo is more and stronger, similar to cavernous hemangioma, but the echo spot is unevenly distributed. It can be deformed after being compressed, which also means that there are more blood vessels and less blood vessels. The tumors in the tumor-like tumors are less, and there is even a lack of echo in the region. For tumors with inconsistent structure, it is necessary to comprehensively check to form correctly. Concept, color Doppler ultrasound showed rich color blood flow, pulse Doppler found that the tumor blood vessels are mostly arterial spectrum, and the flow is faster, cavernous hemangioma, venous hemangioma due to slow blood flow, lack of color blood flow, The spectrogram is also the venous waveform, ie the blood flow wave is parallel to the baseline, which can be distinguished from the vascular epithelial cell tumor.
3. CT scan CT shows that the tumor is a high-density block, located inside or outside the muscle cone, with a round or irregular shape, clear boundary, uniform internal density, CT value +30 ~ +67HU, average +50HU, injection of contrast agent After the obvious enhancement, CT value can reach 138HU, such high enhancement value is rare in other tumors. This aspect indicates that the blood vessels in the tumor are rich, and there are more contrast agents in the blood flow; on the other hand, due to the destruction of blood-tissue barrier, angiography The agent oozes more from the blood vessels, and the malignant vascular epithelial cell tumor can erode the sacral wall in addition to the irregular shape and high degree of enhancement.
4. MRI MRI, like CT, clearly shows the location, shape and boundary of the lesion, which is superior to CT scan in distinguishing the relationship between the tumor and the optic nerve and extraocular muscle.
Diagnosis
Diagnosis and diagnosis of orbital hemangiomas
The clinical manifestations of hemangiopericytoma are similar to those of benign or malignant tumors in the sputum, and lack specificity. Although the anterior tibial tumor can be sputum and purple-red elastic mass, it is difficult to distinguish from cavernous hemangioma, and the imaging diagnosis is certain. Help, the final diagnosis of hemangiopericytoma depends on biopsy. When HE staining is difficult to diagnose, reticular fiber staining is helpful for diagnosis.
And cavernous hemangioma can be identified by imaging findings, and the final identification depends on the pathological findings.
