autonomic nervous system tumor
Introduction
Introduction to autonomic nervous system tumors Tumors of the autonomic nervous system are autonomic (autonomic) tumors of the nervous system, usually divided into three special tissue types: 1 ganglioneuroma. 2 ganglion cell tumor. 3 neuroblastoma. Usually asymptomatic, it is the most common benign neurogenic tumor in children. basic knowledge The proportion of sickness: 0.01% Susceptible people: no specific population Mode of infection: non-infectious Complications: anemia
Cause
Autonomic nervous system tumor etiology
(1) Causes of the disease
1. Ganglioma is a benign tumor with good differentiation. It is composed of ganglion cells and nerve fibers. It originates from sympathetic nerves and occurs in the sympathetic ganglia, parasympathetic ganglia, posterior root ganglia and adrenal medulla. It can also be seen in the central nervous system. The mediastinal ganglioneuroma is lower than the age of schwannomas. It is the most common neurogenic tumor in childhood, which is significantly higher than neuroblastoma. There are a lot of females, which are better than sympathetic. The chain, mostly located in the posterior mediastinum, protrudes forward, is farther away from the midline, can move the pleura and lungs, but non-invasive, the tumor grows slowly, and when the tumor grows larger, the symptoms begin to appear, so the tumor is often seen in clinical 5cm or more.
2. Ganglioneuroblas-toma, also known as ganglioneuroma, consists of mature and immature ganglion cells, rarely seen, and its malignancy is between neuroblastoma and ganglioneuroma. In the meantime, Stout (1947) considered it to have two different tissue types in the natural course:
1 mixed ganglion cell tumor: mature neuroblasts predominate, there are immature neuroblasts in local areas;
2 diffuse ganglioblastoma: a diffuse mixture of well-differentiated and immature neuroblasts, mixed ganglionblastoma, with a high metastatic rate, in most reports, the rate of metastasis 65%75%, on the contrary, diffuse patients transfer less than 5%, male and female incidence rate is similar, 1/3 of patients are seen within 2 years old, half are found within 3 years old, 4/5 are seen within 10 years old, and rare after 20 years old Most of the tumors are located in the posterior mediastinum, from the sympathetic trunk, occupying the paravertebral sulcus. The tumor often develops along the neurovascular bundle, the left and right sides are similar, and a few tumors cross the midline.
3. Neuroblastoma is a highly differentiated, highly malignant tumor that originates in the sympathetic nervous system and can therefore occur in any part of the sympathetic tissue. The most common site of neuroblastoma is in the retroperitoneum. However, 10% to 20% of tumors can be primary in the mediastinum. These tumors are highly invasive and often metastasize before diagnosis. Usually, the metastatic sites include regional lymph nodes and bone, brain, liver and lung. Most of the tumors occur in children, and 75% of cases occur in children under 4 years of age. More than 50% of mediastinal neurogenic tumors in children are neuroblastoma, while 20% of children with neuroblastoma occur in the mediastinum. When such tumors occur, the tumors are gathered into small rosettes by small, round, immature cells. In the examination of ultrastructure, the particles secreted by the nerves are specific, and the neuroblastomas invade adjacent tissues. However, the opinion that the chrysanthemum group (generally considered to be a better differentiation) with more bone metastases is also suspected, because there are many well-formed chrysanthemum groups in the primary tumor, and older infants and young children. Tumors can also be completely free of turmeric-shaped clusters. In addition to bone and liver metastasis, lymph node metastasis is also common, lung metastasis is rare, spinal cord metastasis is rare, and bone metastasis is most common in bone metastases (including eyelids), resulting in increased intracranial pressure, cranial suture Open, long bone metastases are often asymptomatic, and often symmetry, extensive bone metastasis, with bone marrow destruction, causing severe anemia, almost all of the anemia in the late stages of the disease, early bone metastasis can invade the periosteum and adjacent soft tissue and bone marrow cavity, skull The periosteum of the inner plate can be displaced by the dura mater, but the subarachnoid space is the exception. The neuroblastoma occasionally differentiates into pheochromocytoma, and some functional pheochromocytoma contain neuroblastoma components. Mason et al (1952) reported that a 5-month-old infant had a thoracic neuroblastoma with excessive paroxysmal adrenaline secretion, symptoms disappeared after surgery, chromogranular cells were seen in the tumor, and the presence of an amine precursor was confirmed. In fact, in addition to neuroblastoma, ganglioneuroma and ganglioneuroma have the same situation, especially neuroblastoma, the amine in the urine of patients is Intermediate degradation of catecholamines, i.e. dopamine, 5-HIAA and VMA (the VMA), more mature neuronal differentiation, the number of cytoplasmic granules also increased catecholamine.
(two) pathogenesis
Autonomic nervous system tumor pathology:
Ganglion cell tumor (35%):
Those with good differentiation have capsules, most of them are larger (Stout reported the largest weight 6kg), hard or hard, spherical or elliptical, occasionally lobulated, occurring in the posterior root ganglia of the spinal nerve, mostly Dumbbell shape, uniform cut surface, grayish white or light yellow, fibrous, visible unclear boundary, translucent convex vortex nodule, different degrees of edema, although solid, but occasionally cystic degenerative zone, tumor More common calcification (about 20%), the tumor may have a dark red area, brittle, caused by many cellular components, or see focal hemorrhage, which may be a neuronal component, especially in young people.
Microscopically, there are mainly myelinated nerve fibers and collagen fibers, among which there are scattered or ganglion ganglion cells. Neuronal cells can be seen in the differentiated area. Occasionally, typical nerve cell sacs surround it, nerve cells are often degenerated, and the pericytes or hoards are seen. Sudan granules; dual core, less common multinuclear.
However, it is more common in a single nucleus, there is a nucleolus, and there is no mitotic image. The cells are mainly mature; the primordial neurite lesions are common. In the transition of cell tumors, a small number of ganglion cells are coated with sheath cells, and the retinoic and axons are undulating, or bundled; the fibers can be tight or edematous, resembling neurofibroma, but not fenced. The nerve fibers are all myelinated, and the myelin sheath disappears due to degeneration. A considerable amount of collagen matrix forms a broad band or compacts into bundles, separating nerve fibers, common glassy changes, or mucus degeneration, dispersing into a mass. Lymphocytes are easily mistaken for neuronal lesions, and more severe degeneration can form cysts, steatosis and calcification, and calcification can cause significant foreign body reactions.
Ganglioblastoma (27%):
Most of the tumor capsules are intact, a few capsules are incomplete, and a few of the naked eyes do not see the capsule (the tumor capsule is still visible under the microscope), the tumor is 2 to 17 cm long and the median is 7 cm; the tumor weight is 20 to 420 g, and the median is 28 g; More than 200g accounted for 6% to 7%, 60% of the tumor was spherical, pear-shaped or lobulated; the rest was obvious nodular; the extension to the spine was dumbbell-shaped, about 30% soft or fleshy 60% is hard or hard, mainly due to the content of nerve fibers and collagen fibers. The color of the cut surface is different, and the color is gray or brown. When the color is pale and hard, the ganglion tumor is mostly composed. The soft-skinned hemorrhagic lesions are equivalent to neuroblastoma components, occasionally calcified yellow patches, and rarely seen large pieces of necrosis.
According to Stout's indicators, it can be divided into two subtypes under the microscope: diffuse type, see a variety of differentiated cells, mainly pleomorphic cells, rich in cells, in which the neuroblasts have a tendency to nest, occasionally nuclear division Like, ganglion cells are generally immature, cytoplasmic blue-stained, containing Nissl bodies, vacuoles in the nucleus, obvious nucleoli, common singular ganglion cells and multinucleated ganglion cells, few or absent nerve sheath cells, nerves The axons are slender and not bundled. This type is obviously different from neuroblastoma. The second subtype is mixed. It is mainly composed of typical ganglionoma, and the neuroblastoma region of isolated multicellular components is seen inside. The boundary between the two is clear, and sometimes the boundary cells are infiltrated by the infiltration of the tumor cells of the neuroblastoma, and often invade adjacent thin-walled blood vessels. In the ganglion cell area, the ganglion cells and axons are mature. This type is clearly different from the diffuse type.
Neuroblastoma (18%):
Under the naked eye, this tumor can have all or part of the capsule, but the common tumor cells under the microscope pass through the capsule, and the tumor is lobulated or massive. Because it occurs in the sympathetic ganglion chain, it can invade the intervertebral foramen along the nerve. The spinal cavity often causes paraplegia due to compression of the spinal cord. The tumor is grayish white and brownish due to hemorrhage. The hemorrhage area and necrosis are common in the cut surface, and small cysts containing blood and gravel-like calcification can be seen.
Microscopically typical, it is a multi-cell mass, cells are small and regular, such as lymphocytes, or slightly larger, less cytoplasm, borders are not clear; nuclear staining is deep, cells are spherical or oval, sometimes short rods ; often formed a daisy group.
Rare giant cells and multinucleated cells, some specimens have many mitotic figures, but other specimens are rare. Tumors are separated by weak vascular connective tissue. Silver staining can be seen in the matrix with nerve fibers, extensive necrosis and hemorrhage. Hemosiderin particles and calcifications can be seen due to hemorrhage, and common tumor cells in the venules and perivascular lymphatic vessels are invaded. In some areas, the tumor cells see clear perinuclear space and slightly larger nuclei, and fine chromatin is seen in the nucleus. Small nucleoli, implied as immature nerve cells; sometimes in the slender fibrous matrix, the dense cell population is seen, the cytoplasm is clearer, and occasionally polarized, which is the early differentiation of neuroblasts.
Prevention
Autonomic nervous system tumor prevention
Diet is the most important thing, we must manage the patient's diet.
Complication
Autonomic nervous system tumor complications Complications anemia
Usually accompanied by anemia.
Symptom
Symptoms of autonomic nervous system tumors Common symptoms Iridic heterochromia, trigeminal nerves, one or two branches... Spontaneous sweating, abdominal pain, strabismus
1. Gangliocystoma: It is benign, has a family history, usually asymptomatic, it is the most common benign neurogenic tumor in children. This tumor is also seen in older children, adolescents and young people, and slightly more women. Reed et al (1979) reported that 47% of patients were >20 years old. If the tumor gradually increased, symptoms associated with sphincter-derived tumors may occur above 5.0 cm. Horner syndrome may occur in violation of the cervical sympathetic ganglia. , iris heterochromia, etc., but this tumor rarely expands into the intervertebral canal.
2. Ganglioblastoma: The incidence rate of males and females is similar, occurring in younger age groups, about 30% within 2 years old; 50% within 3 years old; 70% to 80% found within 10 years old; those over 20 years old It is rare to have paraplegia, chronic diarrhea and pain in certain areas in addition to symptoms associated with neurogenic tumors.
3. Neuroblastoma: The incidence rate of males is slightly higher than that of females, and occurs in young children and infants. Most of them occur in children under 3 years old. More than 50% of neurogenic tumors in children's mediastinum are neuroblastomas. In addition to symptoms similar to neurogenic tumors, paraesthetic and other spinal cord compression-related neurogenic symptoms are present in 1/3 of children with mediastinal neuroblastoma, and in some cases, paracancerous syndrome may occur. Such as a large number of watery diarrhea, abdominal distension and vasoactive intestinal peptide (VIP)-related abdominal pain, may be associated with autoimmune strabismus multi-muscle palsy syndrome is a difficult to explain symptom group; cerebellum, brainstem ataxia With rapid eye movement (dance-like eyes); due to tumor-derived neural crest cells containing APUD cells, there may be chromophoric cell syndrome caused by catecholamine secretion, such as sweating, skin flushing, elevated vanilla almonds in the urine Acid is (VMA), it is the product of the degradation of these substances, because this tumor is good to invade adjacent tissues, often early blood transfer, bone, liver is more common, long bones can often appear asymptomatic, bone wide Pan-transfer with bone marrow destruction can cause severe anemia. Liver metastasis can cause jaundice and metatarsal metastasis. It is often a special phenomenon of neuroblastoma. In the late stage of the disease, almost all anemia occurs, because lymphatic metastasis often occurs. There are accompanying symptoms and signs.
After understanding the medical history, based on age and clinical manifestations, combined with laboratory examinations and auxiliary examinations should consider autonomous nerve cell tumors, and the diagnosis still requires pathological examination.
Examine
Examination of autonomic nervous system tumors
Determination of urinary catecholamine degradation products: dopamine, dopa, 5-hydroxyindole acetic acid and vanillyl mandelic acid (VMA), especially vanilla mandelic acid (measured 24h urine VMA total), this value increased, there are To help diagnose neuroblastoma, the other two types of autonomic neuroblastomas are relatively few. The level of these substances is reduced to normal after successful tumor resection, and if it rises, it suggests recurrence.
1. X-ray examination: The X-ray findings of ganglion cell origin vary depending on the differentiation of the tumor. Benign ganglionoma is a solid block of the paraspinal sulcus, and the boundary is clear. 50% of the cases can be seen. Calcification, the bone near the tumor has changed due to tumor compression, and it is not uncommon for the tumor to grow into a spinal canal. The patients with ganglionoma and neuroblastoma show that the tumor in the paravertebral sulcus is relatively Clear, while the latter X-ray shadow is often referred to as "ghosting". Most tumors can see punctate calcification. In cases of neuroblastoma, bone erosion and invasion of the spinal canal are often seen, while ganglion cells Cases of tumors are rare, but large autonomic neuroblastomas in the chest have also been reported, and their X-ray findings show signs similar to those of giant mediastinal sheath-derived tumors.
2. CT: In addition to the common features of neurogenic tumors, ganglioneuroma, ganglioblastoma, and neuroblastoma often have calcification and are spotted high-density shadows. Arc calcification, neuroblastoma mostly irregular irregular mass, tumor often lobulated and protrude into the lung field, the tumor can be divided into two parts by arc separation, the mass can oppress the adjacent structure and move the blood vessel Move forward and the trachea moves to the opposite side.
3. MRI: In the enhanced T2 image, ganglionblastoma is a high-density heterogeneous spiral, and the low-density curved and nodular bands form a spiral.
Diagnosis
Diagnosis of autonomic nervous system tumors
Differential diagnosis
It should be distinguished from renal embryonal tumors. The malignant degree of renal embryonal tumors can be high and develop rapidly, while neuroblastomas have a higher degree of malignancy and metastasis occurs very early.
