Pseudomyxoma peritoneum
Introduction
Introduction to peritoneal pseudomyxoma Pseudomyxomaperitonei (PMP) is a low-grade mucinous mucinous tumor that occurs in the peritoneal wall, the greater omentum, and the serosal surface of the intestinal wall. The incidence is low, the incidence rate is higher than that of men, mostly middle-aged or old. It is easy to relapse after treatment, and it is a clinically difficult disease. basic knowledge The proportion of illness: 0.006% Susceptible people: no special people Mode of infection: non-infectious Complications: intestinal obstruction peritonitis intestinal fistula
Cause
The cause of peritoneal pseudomyxoma
Causes:
The etiology of peritoneal pseudomyxoma is still unclear. Because this disease is an ovarian mucinous cyst, ovarian mucinous cystadenoma or appendix mucus cyst is broken. When the mucus is discharged, it often contains epithelial cells and enters the abdominal cavity together. In terms of mucus and epithelial cells, the peritoneum stimulates the peritoneum, causing an inflammatory reaction. On the other hand, it continues to secrete mucus to accumulate a large amount of gelatinous mucus in the abdominal cavity, causing colloidal ascites, called "adhesive belly", and possibly through blood flow and lymph. According to the metastatic spread of the tube, about 45% of the tumor cells are derived from the ovary, 29% are from the appendix, 26% are indeterminate, and 1% to 2% of the ovarian tumors can develop into a peritoneal pseudomyxoma. In addition, there are still a small number of patients secondary to ovarian teratoma, ovarian fibroids, uterine cancer, intestinal mucinous adenocarcinoma, urachal cyst adenocarcinoma, small mesenteric wave cyst, common bile duct mucinous adenocarcinoma, pancreatic mucinous cystadenocarcinoma And peritoneal mesothelioma, etc., but extremely rare.
Pathogenesis:
The rupture of organ cysts, ovarian mucinous cystadenoma and other organ cysts, so that a large number of mucous tissue and mucous-rich columnar epithelium, scattered in the abdominal cavity, adhere to the wall layer, the omentum and the serosal surface of the intestinal wall, Enveloped by connective tissue of the peritoneum, forming vesicles of varying sizes. The vesicle wall is composed of very thin connective tissue. The vesicles are filled with many pale yellow, translucent jelly-like thick mucus and columnar epithelial cells, sometimes Only mucus and epithelial cells are absent, and vesicles can spread in local infiltration.
Mucus and epithelial cells can also stimulate inflammatory changes and adhesions in the peritoneum. Adhesion of the intestine can lead to adhesive intestinal obstruction, and thus the prognosis is poor. The omentum is often fused into a piece or cake, and is also known as the "retardant cake". These vesicles can be exfoliated from the peritoneum and freed in the ascites. Under the microscopic examination, the vesicle wall is composed of a thin fibrous tissue. The vesicles are filled with translucent mucinous substances, and the inner walls are covered with columnar epithelial cells secreting mucus substances, while others are not covered with epithelium, usually benign, but occasionally there are malignant.
In the interhepatic space, ileocecal or pelvic space, a mass of several centimeters to tens of centimeters is often seen. This mass can be located under the armpit or through the diaphragm to enter the chest.
The tumor tissue sections showed that the differentiated and mature epithelial cells grew in a linear infiltrating manner, scattered in the loose connective tissue, surrounded by mucus pools of different sizes, and a small number of mucus pools arranged neatly to secrete the epithelial cells of the epithelial cells. Structure, although the tumor seen is benign, it can locally infiltrate and spread, affecting important structures around it.
Prevention
Peritoneal pseudomyxoma prevention
Pay attention to rest, work and rest, life in an orderly manner, and maintaining an optimistic, positive and upward attitude towards life can be of great help in preventing diseases.
Complication
Peritoneal pseudomyxoma complications Complications, intestinal obstruction, peritonitis, intestinal fistula
Although the peritoneal pseudomyxoma rarely metastasizes, it has malignant growth and strong vitality. It adheres to the peritoneal wall layer and then grows rapidly, so that the patient's entire abdomen is a mucinous mass, which is attached to the peritoneal wall layer. Omentum, mesentery, so that the intestinal lumen, often appear intestinal obstruction, peritonitis and other complications, causing adhesive intestinal obstruction, intestinal fistula, pyloric obstruction and gallbladder obstruction, and finally cause patients can not eat, mesenteric failure.
Symptom
Symptoms of peritoneal pseudomyxoma Common symptoms Mucus secretion into the liver swollen abdominal circumference enlarges ascites, bowel, dyspnea, abdominal pain, bloating
The disease has a long history, and the course of disease can be delayed for several months or years, and some can last for more than 10 years. Because of the clinically non-specific performance, the main cause is abdominal augmentation and abdominal pain. There are also recurrent episodes of lower right abdomen pain and discomfort, right lower abdomen mass or complications such as intestinal obstruction and peritonitis. The misdiagnosis rate is as high as 89.7%. The examination may have ascites and unclear nodules, so it is often misdiagnosed as Liver cirrhosis and tuberculous peritonitis, abdominal cysts, etc. have delayed treatment.
1. The main symptoms of early symptoms are non-specific, and the symptoms of ascites are obvious in the later stage.
(1) nausea, vomiting: mucinous ascites is progressive growth, early a small amount of ascites stimulate the peritoneum, only cause gastrointestinal reactions, such as nausea, vomiting, lower abdominal pain or pelvic sensation, some patients have urinary symptoms.
(2) Progressive bloating and abdominal pain: As the ascites gradually increases, the patient consciously swells gradually in the abdomen, abdominal circumference increases, abdominal pain, and breathing costs. Gradually developed into difficulty breathing, hemorrhoids, not lying down, difficult to turn over.
(3) weight loss: peritoneal pseudomyxoma grows very fast, while consuming a large amount of body nutrition, it also oppresses the abdominal organs, causing the patient's appetite to decline, the body is weak, and the body weight is progressively reduced.
(4) digestive tract obstruction: intestinal adhesion and mass compression, stenosis of the stomach and intestines, patients may have pyloric obstruction, intestinal obstruction or even obstructive jaundice, etc., clinical symptoms.
2. Signs
(1) abdominal distension: is the main sign of the disease, the following abdominal swelling is more common, such as total abdominal peritoneal involvement, may have a full abdominal high bulging, or even a full-term pregnancy.
(2) tenderness and mass: the patient's abdomen is soft, a few have tenderness, but not much. Most patients have a bump in the abdomen, especially in the right lower abdomen or lower abdomen, but the whole abdomen can be combined. The size of the mass varies from a few centimeters to tens of centimeters, and the texture is harder; the surface is uneven and the activity is small.
(3) Liver enlargement: Most patients have enlarged liver and are tough or slightly hard.
(4) Ascites sign is positive, due to different ascites viscosity, the patient may show abdominal fluctuations or behave as mobile dullness.
(5) normal bowel sounds or hyperthyroidism: abdominal auscultation of bowel sounds is basically normal, intestinal obstruction may have enhanced and gas over water.
(6) Others: Due to the early and late course of the disease and the degree of tumor invasion, the digital rectal examination may have varying degrees of fullness, rectal stenosis or touching the extra-intestinal mass. Gynecological examination often reveals uterine attachment or uterine rectal recess. Tumor.
Examine
Examination of peritoneal pseudomyxoma
1. There is no obvious abnormal change in blood routine examination, or only mild anemia. In patients with secondary infection, there may be an increase in white blood cell count.
2. There are no abnormalities in blood biochemical tests, but complicated with pyloric obstruction, intestinal obstruction, obstructive jaundice, water and electrolyte abnormalities and acid-base balance disorders, jaundice index and elevated blood bilirubin, low-grade malnutrition Proteinemia and the like.
3. When ascites is checked for abdominal abdomen, if abnormal ascites is found, it is a jelly-like or chicken-like cake. It should be suspected and the disease. Although a large amount of ascites is used, it is puncture with a thick needle of 8 to 12, and only a small amount of pale yellow is obtained. , viscous, jelly-like liquid, routine and special examination of puncture fluid can be seen fibrin and red blood cells, mucin qualitative test (Rivalta test) is generally positive, this test often has a decisive significance for the diagnosis of this disease, ascites is leakage, ascites There are no special changes to the routine.
4. Histopathological examination by rectal biopsy, sectioned as peritoneal pseudomyxoma.
5. Imaging examination
(1) X-ray abdominal plain film: It has been reported that the calcification curve of the X-ray abdominal plain film should be suspected of the disease, but this sign is not common.
(2) digestive tract tincture examination: generally no abnormal changes, but pyloric obstruction occurs, intestinal obstruction, external pressure gastric wall defects, intestinal stricture.
(3) B-ultrasound: no trauma, low cost, reliable, should be the first choice, if B-ultrasound found that the liquid area of the peritoneal cavity is slightly grayish white, diffuse distribution of large spots, spots, halos slowly, with deep breathing, Changes in position, pressure or shock exploration see "flowers" fluttering, should be highly suspected as peritoneal pseudomyxoma.
It is characterized by an irregular small cystic anechoic area on the inner wall of the abdominal cavity or the surface of the intestine (as shown in Figure 4); and a large honeycomb-like anechoic area in the abdominal cavity. The boundary is unclear, and a small dot-like echo is visible inside. As the body position changes, it can be seen that the small dot echo flutters in the anechoic zone. The small cyst generally has no smooth and complete cyst wall. More small cysts are clustered together to form a honeycomb structure, and the small cystic structure can also be attached. The surface of the liver, bladder and uterus, according to the characteristics of the sonogram, combined with medical history, the diagnosis of this disease is not difficult.
(4) CT examination: liver and spleen were compressed, scallop-like defects appeared on the edge of liver and spleen, thickening of peritoneum, low-density shadow of a large number of water samples in abdominal cavity, CT value 20Hu, significantly higher than ascites, showing diffuse cyst of abdominal cavity and pelvic cavity Sexual mass, cyst size varies, mostly below 1cm, omental peritoneal infiltration thickening; lesion CT value is lower, mostly around 3Hu; liver margin is multiple fan sag, no liver metastasis; a large amount of ascites, often Separation phenomenon, the ascites is jelly-like, the density is low, pushing the intestinal tube to the center.
6. Exploratory laparoscopic or laparoscopic surgery Because the disease is rare, it is misdiagnosed due to lack of understanding, and even the puncture and its test results have not been thought of. The laparoscopic or laparoscopic surgery may be necessary to confirm the diagnosis. It is filled with white transparent, semi-solid viscous liquid. There are many homogeneous tumors or multiple cystic masses. Some of them are firmly attached to the peritoneum, and the tumor can be taken for pathological examination.
Diagnosis
Diagnosis and diagnosis of peritoneal pseudomyxoma
Diagnostic criteria
In recent years, the disease can be diagnosed by abdominal wear, B-ultrasound, CT, laparoscopy, and mass biopsy.
1. Some patients have a history of appendix, ovarian surgery
2. Clinical features Self-reported bloating, abdominal pain, weight loss, abdominal uplift; physical examination of the general condition is good, abdominal swelling, and can touch the uneven lumps, this is a prominent feature of the disease.
3. Laboratory and auxiliary examination Diagnostic abdominal puncture only draws a small amount of viscous liquid; B-ultrasound and CT show multiple abdomen and ascites signs in the abdomen.
Differential diagnosis
The disease lacks specific clinical manifestations in the early stage, so it needs to be differentiated from cirrhosis ascites, tuberculous peritonitis or advanced cancer. The disease is often misdiagnosed as cirrhosis, tuberculous peritonitis, abdominal cyst and abdominal metastases. When you can exclude "cirrhosis ascites":
1. The patient has no history of liver disease and generally has a good health condition.
2. Long-term service to protect the liver, diuretic drugs are invalid, abdominal circumference is increased.
3. The physical examination of the abdomen is not like the "frog belly", the voiced area is not on the abdomen, no moving dullness.
4. Abdominal water can not be extracted from the abdomen, and the thick needle can suck out the jelly-like mucus.
5. B-ultrasound examination of a large number of echo-free dark areas in the abdominal cavity is separated.
6. Liver function is normal and platelets are not low.
Because peritoneal pseudomyxoma is quite rare, although it has its characteristic B ultrasound image, it is often not recognized by medical workers. Because the nature of ascites is sticky, the thickness of the dark zone changes little and slowly during body movement, which is easily misdiagnosed as Abdominal cysts, due to thick omental thickening, blocky strong echo, some areas see adhesion to the intestinal tract, it is easy to be misdiagnosed as tuberculous peritonitis, from the clinical perspective and B ultrasound image can be often misdiagnosed cirrhosis, tuberculous peritonitis , differential identification of peritoneal mesothelioma.
