What Is Nemaline Myopathy?

The adrenal gland is an important endocrine organ of the human body. It consists of the adrenal cortex and medulla. It secretes a variety of hormones and plays an important regulatory role in maintaining normal physiological functions. The adrenal cortex or medulla can secrete too many hormones due to hyperplasia or tumors; it can also cause hormone secretion to decline due to tumors or other diseases, resulting in corresponding adrenal hyperfunction or regression. In recent years, the development of biochemical technology and imaging technology has significantly improved the diagnosis and treatment of a variety of adrenal diseases, and also increased the number of adrenal adrenal tumors.

The adrenal gland is an important endocrine organ of the human body. It consists of the adrenal cortex and medulla. It secretes a variety of hormones and plays an important regulatory role in maintaining normal physiological functions. The adrenal cortex or medulla can secrete too many hormones due to hyperplasia or tumors; it can also cause hormone secretion to decline due to tumors or other diseases, resulting in corresponding adrenal hyperfunction or regression. In recent years, the development of biochemical technology and imaging technology has significantly improved the diagnosis and treatment of a variety of adrenal diseases, and also increased the number of adrenal adrenal tumors.

Chinese name

Adrenal disease

Foreign name

Diseaseofadrenalgland

Causes of adrenal diseases and common diseases

Adrenal hyperplasia

Adrenal hyperplasia refers to adrenal non-neoplastic hypertrophy, which has two types: primary and secondary. Cortical hyperplasia is generally bilateral, and only a few cases are unilateral. If the disease course is long, it eventually develops to bilateral. The adrenal glands of cortical hyperplasia were enlarged and thickened, and the color was darker than normal glands. They were yellow or brown. Some patients saw needle nodules to sesame-sized yellow nodules in the hyperplastic cortex, which were evenly distributed. In a few cases, the adrenal gland volume is normal, and the thickness and weight are within the normal range. There are also a few cases of adenomas coexisting with hyperplasia, especially small adenomas coexisting with nodular hyperplasia. This disease has histological similarities with small functional adenomas, but adenomas are unilateral adrenal lesions with concomitant adrenal atrophy.

Adrenal cyst

Adrenal cysts are rare, and can be divided into four types according to their pathological properties: parasitic cysts, which are mostly caused by cyst infection, and there can be ascites and grandsons in the cyst, and the outer wall of the cyst can be calcified; epithelial cysts It is caused by the abnormal development of the embryonic primordium. The inner wall is lined with columnar epithelium. Endothelial cysts are relatively common. There are two types of lymphatic and vascular in terms of tissue composition. False Cysts, mostly adrenal bleeding or tumor necrosis. A cyst formed by hemorrhage without cell lining in the cyst wall is also common.

Adrenal adenoma

Adrenocortical adenomas mostly occur on one side and are more common. Tumors are generally small, mostly 1 to 2 cm. It has a round or oval shape, has a coating, and grows slowly, but there is a possibility of malignant transformation. There may be bleeding, liquefaction, necrosis and cystic changes in the tumor. Most tumors are secretory adenomas, and the contralateral adrenal glands atrophy due to the inhibition of ACTH secretion by the pituitary gland. Among them, those who secrete excessive glucocorticoids (mainly cortisol) are called cortisol adenomas. The tumors are relatively large. Up to 3cm; those who secrete a large amount of aldosterone, called aldosterone adenoma, the tumor is relatively small, generally about 1cm; adenomas without secretion function are rare, and generally asymptomatic, most of them were found by accidental examination.

Adrenal cortex adenocarcinoma

Adrenocortical adenocarcinomas are rare clinically and can be divided into functional and non-functional categories. But more than 80% are functional cortical adenocarcinomas. Most of them occur unilaterally, the volume is often 6-8cm, the tumor is round, oval or lobular, the surface is uneven, and the tumor envelope is incomplete. The cut surface often has bleeding and necrosis. Cancer cells often infiltrate or penetrate the capsule or invade lymphatic vessels, lymph nodes or blood vessels. The tumor grows rapidly, and metastases to liver, lymph nodes, lungs, and brain occur earlier, and metastatic cancer also has the functional characteristics of the primary tumor.

Adrenal medulla lipoma

Adrenal medulla lipoma is a rare disease, and it is more common as small tumors, but there are also those as large as 10 cm or more. It is a benign tumor with no secretory function. The tumor is named after it contains mature fat cells and bone marrow cells. The disease may be caused by abnormal development of cortical reticuloendothelial metaplasia or vagal embryonic residues.

Adrenal gland pheochromocytoma

About 90% of pheochromocytomas occur in the adrenal medulla. They are the most common tumors in the adrenal medulla. Most of them are unilateral, with bilateral incidence of 10%, and are more common in children and familial patients. About 10% of pheochromocytomas outside the adrenal gland occur mainly in sympathetic ganglia and chromaffin tissues in the renal hilum, paraabdominal aorta, and bladder.

Pheochromocytomas are mostly benign and about 2% are malignant. Tumors are more common in 20 to 40 years of age. Tumors are generally large, most of which are 3 to 5 cm in diameter, often spherical, mostly lobulated, the tumor envelope is more complete and smooth, the cut surface is brown-red, rich in blood flow, and often bleeding and cystic changes. Malignant pheochromocytoma grows invasively or metastasizes to other organs.

Neuroblastoma is derived from differentiated or undifferentiated sympathetic ganglion cells and can occur in any part of the sympathetic nerve. About half of them occur in the adrenal medulla, and the rest are located outside the adrenal glands such as retroperitoneum and mediastinum. Adrenal neuroblastomas are mostly unilateral, the tumors are nodular, hard in texture, and rich in blood supply. Early intact capsule, bleeding, necrosis, cystic changes in the tumor, often with calcification. The tumor grows rapidly and has a high degree of malignancy, which can metastasize at an early stage.

Adrenal metastases

The adrenal gland is a site where metastases are more likely to occur in the body. Lung cancer metastasis is the most common, followed by breast, thyroid, colon cancer, and melanoma. Lymphoma and kidney cancer can directly invade the adrenal gland. Metastatic cancers can develop unilaterally or bilaterally, and they can be single or multiple.

Adrenal disease

Adrenal tuberculosis is mostly bilateral and can be unilateral. Often accompanied by renal tuberculosis, peritoneal tuberculosis, and epididymal tuberculosis. When the pathological examination shows that the adrenal glands are damaged by more than 50%, various clinical manifestations appear. The disease is characterized by caseous necrosis or granulation tissue formation, often with calcification. It can affect the adrenal cortex and medulla. In severe cases, it can affect the entire adrenal gland. There are two types of gross pathological sections: Proliferative lesions: containing most tuberculous nodules and fibroblasts. Necrotic lesions: mainly cheese necrosis, with fibrous tissue in the periphery, tuberculous nodules, lymphocyte infiltration, and often calcified parts.

Adrenal bleeding

Adrenal hemorrhage is rare. Most are seen in emergencies of trauma, severe burns, pregnancy, myocardial infarction and other diseases, leading to excessive secretion of ACTH. Neonatal adrenal hemorrhage is mostly unilateral and localized. Diffuse bilateral adrenal hemorrhage is often caused by improper anticoagulation therapy, and it usually occurs within 3 weeks of treatment. If not treated in time, it can cause death.

Differential diagnosis of adrenal disease

Adrenal hyperplasia

1. Adrenal cortical nodules and cortical tumors

The latter are mostly unilateral, solitary lesions, and have clearly-defined hypoechoic nodules. They have a clear boundary with normal peripheral adrenal tissues, and functional tumors are accompanied by contralateral adrenal atrophy, which is prone to hyperplasia. the difference.

2. Adrenal hyperplasia and adrenal varices

The latter is rare. It can show that the adrenal gland is hypoechoic. Continuous multi-section observation shows a curved tubular echo. The echo of the wall is also strong. The CDFI shows colored blood flow, which can be distinguished from the former.

Adrenal cyst

Adrenal cysts should be distinguished from adrenal cysts, pancreatic tail cysts, and spleen cysts. Multi-section and multi-angle exploration, it can be found that these cysts have an attachment relationship with their source organs, and the echo of the organ capsule is discontinuous. If cysts from other organs can be ruled out, adrenal cysts should be highly suggested, liquefaction of larger solid tumors, and easily confused with adrenal cysts. At this time, attention should be paid to the characteristics of the thickness of the capsule wall and the presence of silent enhancement at the rear.

Adrenal adenoma

1. Adrenal adenocarcinoma and cortical adenoma

The former tumors are generally large, with irregular edges and lobulated leaves. The internal echoes are heterogeneous, and there may be non-echoic areas of varying sizes. If metastases occur, echoes of the metastases are displayed at the corresponding site, which can be distinguished from the latter by combining with clinical data.

2. Minimal adrenal tumors and cortical adenomas

Due to the prominent growth of the kidney surface, most tumors are located outside the outline of the kidney and are easily mixed with the latter. Continuous observation on multiple sections showed that there were local protrusions or continuous interruptions in the renal capsule, and the mass echo was not related to the adrenal gland, but could be distinguished from adenoma.

Adrenal cortex adenocarcinoma

1. Adjacent organ tumors and cortical adenocarcinoma

A full range of scans are needed to determine the exact location of the tumor, which is mostly limited to the anterior superior pole of the kidney. The boundary between tumor and peripheral organs, the range of motion, and the direction of compression can be used as the basis for identification.

2. Adrenal pheochromocytoma and cortical adenocarcinoma

The former is clear, regular and has a strong echo light band. It and the renal capsule echo form a typical "seagull sign", which is a characteristic manifestation of tumors. Combined with related clinical manifestations, it is easy to distinguish with the latter.

Adrenal medulla lipoma

This disease should be distinguished from other adrenal tumors. Other adrenal tumors are mostly round nodules, and the internal echo is mostly moderate or low echo. No morphological changes occur with respiratory movement.

Adrenal gland pheochromocytoma

1. Hepatic right posterior lobe tumor and adrenal pheochromocytoma

The former is a local bulge, showing a round-like mass echo, with a lot of stuns on the periphery, irregular capsule bulge, and a synchronized mass with liver activity. The latter has a clear boundary with the liver, and there is no local protrusion of the liver capsule, and the tumor and the liver move asynchronously during deep breathing, and the contact surfaces of the two see slippage.

2. Adrenal tumors and adrenal pheochromocytoma

The former is located in the outline of the kidney, the renal capsule is locally protruded, and the collective system is often changed by pushing and displacement. The latter is more clearly demarcated from the kidney and the kidneys are extremely stressed, but the collective system remains unchanged.

3. Pancreatic tail mass and adrenal pheochromocytoma

Multi-section and multi-directional observation of the position of the mass with the pancreas, large vessels in the abdominal cavity, and the kidney, and determine its exact anatomical location in order to make a distinction.

4. Other adrenal tumors and pheochromocytoma

See adrenal adenoma section.

5. Extra-adrenal pheochromocytoma needs to be distinguished from ganglion cell tumors, bladder tumors, etc., and can be identified based on the location, number of tumors, relationship with adjacent tissues and organs, and typical clinical manifestations.

Adrenal neuroblastoma

1. Adrenal neuroblastoma and kidney Wilm's tumor

The former may have compression and displacement of the kidney, but the outline of the kidney is still intact, and there is no loss of echo, while the latter is affected by the erosion and pressure of the kidney, which causes its morphological abnormality, and even the normal renal structure echo disappears or there is residual water in the kidney.

2. Adrenal neuroblastoma and hepatoblastoma

The latter pushes the kidneys down, but to a lesser extent. The tumor is closely connected to the liver, there is no strong echo boundary, and the activity is synchronized with the liver.

Adrenal metastases

Adrenal metastatic tumors should be distinguished from primary tumors, which are mostly unilateral and solitary. Tumors are generally intact. Functional tumors have specific clinical symptoms and abnormal laboratory indicators. Finding the primary tumor is the most powerful evidence to distinguish the two.

Adrenal tuberculosis

1. Adrenal tuberculosis and adrenal cyst

The necrotic liquefaction period of the former is easily confused with the latter, so identification is needed. The latter has a thin and smooth cyst wall, and the internal sound transmission is generally good; those with lymphocytic cysts have a clear outline and are multilocular. Cystic adenomas are large in size, the edges are smooth and neat, and there are many strong echoes of calcium deposits on the inner wall.

2. Adrenal tuberculosis and adrenal cyst

The latter showed that the cyst was closely connected to the upper kidney, and the wall of the cyst rising upward continued to the upper capsule of the kidney, and there was no speckle or flocculent echo in the capsule.

Adrenal bleeding

Liquid dark areas appear in the adrenal glands, round or oval, with borders, but not as clear as the cysts. It can be seen that there is still a part of normal adrenal gland structure. After a few days of follow-up observation, the liquid dark area gradually shrinks, and finally it can be completely absorbed.

Adrenal examination

The adrenal gland disease ultrasound examination is best performed with a fan-shaped real-time ultrasound imaging device. A convex array ultrasound imaging device can be used, and a linear array ultrasound imaging device can also obtain better results. Ultrasonic probe frequency is mostly 2.5 ~ 3.5MHz. No special preparation is required before inspection. The postures at the time of examination are supine and prone. The standard cross-sectional examination of the adrenal gland includes a transcostal coronal cross-section scan, a back longitudinal cross-section scan, and an upper abdomen cross-section scan.

Adrenal disease treatment principles

In recent years, laparoscopic technology has been widely used in the treatment of adrenal diseases in major hospitals across the country. Its advantages are unquestionable, and it has the potential to replace traditional open surgery. Regarding the indications for laparoscopic treatment of adrenal diseases, it is generally believed that as long as benign adrenal tumors with a diameter of less than 6.0 cm, such as primary aldosteronism, cortisol, smaller pheochromocytoma, non-functional adenoma, angiolipoma, Adrenal medullary cysts can be considered laparoscopic surgery. For tumors larger than 6.0cm in diameter, because the surface vessels are richer and are closely related to the inferior vena cava, they are difficult to remove freely. At the same time, when the tumor is large, the possibility of tumor malignancy is also high. Laparoscopy should be used with caution. With the continuous improvement of laparoscopic technology, the continuous improvement of surgical instruments and equipment, the improvement of surgeon's technical level and the accumulation of experience, tumor size is not an absolute contraindication for laparoscopic adrenalectomy. It should be mastered flexibly and not arbitrarily.