Introduction
Peritoneal pseudomyxoma(pseudomyxoma peritonei, 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.
Cause
(1) Causes of the disease
The etiology of peritoneal pseudomyxoma is still unclear. Because the disease is formed by ovarian mucinous cyst, ovarian mucinous cystadenoma or appendix mucus cyst, when the mucus is discharged, it often contains epithelial cells and enters the abdominal cavity together. On the other hand, mucus and epithelial cells stimulate the peritoneum, causing an inflammatory reaction. On the other hand, the mucus is continuously secreted to accumulate a large amount of gelatinous mucus in the abdominal cavity, resulting in colloidal ascites, called "gumab". It may also be caused by blood flow and lymphatic metastatic spread. According to statistics, about 45% of tumor cells are derived from the ovary, 29% are from the appendix, 26% are indeterminate, and 1% to 2% of ovarian tumors can develop into peritoneal pseudomyxoma.
In addition, a small number of patients are 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.
(two) pathogenesis
The rupture of organ cysts such as appendix mucinous cysts and ovarian mucinous cystadenomas causes a large amount of mucous tissue and mucous-rich columnar epithelium to spread in the abdominal cavity and adhere to the serosal surface of the parietal, omentum and intestinal wall. It is wrapped by the connective tissue of the peritoneum and forms vesicles of varying sizes. The vesicle wall is composed of very thin connective tissue, which is filled with many pale yellow, translucent jelly-like thick mucus and columnar epithelial cells, sometimes only mucus and epithelial cells are absent. 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, so the prognosis is poor; the omentum often fuses into a piece or cake, and is also known as the "retardant cake". These vesicles can be exfoliated from the peritoneum and freed from the ascites. Under microscopic examination, the vesicle wall is composed of a thin fibrous tissue filled with a translucent mucoid material. The inner wall is covered with columnar epithelial cells secreting mucus, and some are not covered with epithelium. Usually benign, but occasionally there are vicious people.
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 there were a few cord-like epithelium in the mucus pool that were neatly arranged and secreted into the epithelial cells. structure. Although the tumor seen is benign, it can locally infiltrate and spread, affecting important structures around it.
symptom
The disease generally 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, it is mainly due to abdominal enlargement and abdominal pain. There are also repeated episodes of lower right abdomen pain, discomfort, right lower quadrant mass or complications such as intestinal obstruction and peritonitis. The rate of misdiagnosis was as high as 89.7%. The examination may have ascites and unclear nodules, which are often misdiagnosed as cirrhosis and tuberculous peritonitis, abdominal cysts, etc., and delay 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 abdominal distension and abdominal pain: As the ascites gradually increases, the patient consciously swells in the abdomen gradually, the abdominal circumference increases, the abdominal pain and pain, and the breathing is laborious; gradually develop into difficulty breathing, hemorrhoids, supine, difficulty in turning over .
(3) weight loss: peritoneal pseudomyxoma grows very fast, while consuming a large amount of body nutrition, it also oppresses the abdominal organs, causing loss of appetite, general malaise, and weight loss.
(4) digestive tract obstruction: adhesion of intestinal fistula and compression of the mass, 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. If the total abdominal peritoneal involvement, there may be high abdominal bulging, even as a full-term pregnancy (Figure 2).
(2) tenderness and mass: the patient's abdomen is soft, a few are tender, but not significant; most patients may have a bump in the abdomen, especially in the right lower abdomen or lower abdomen, but the whole abdomen can be licked; The size varies from a few centimeters to tens of centimeters in size, 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 exposure to extraintestinal masses. Gynecological examinations often reveal tumors in the uterus attachment or in the uterine rectum.
diagnosis
In recent years, the disease can be diagnosed by abdominal wear, B-ultrasound, CT, laparoscopic, and mass biopsy.
1. Some patients have a history of appendix and ovarian surgery
2. Clinical features Self-reported bloating, abdominal pain, weight loss, abdominal augmentation; physical examination of the general condition of the general condition, abdominal distension, and can touch the uneven rugged, this is a prominent feature of the disease.
3. Laboratory and auxiliary examinations Diagnostic abdominal puncture only draws a small amount of viscous liquid; B-ultrasound and CT show multiple masses and ascites signs in the abdomen
Identification
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 cysts and abdominal metastatic cancer. There are the following points to rule out "cirrhosis ascites":
1. The patient has no history of liver disease and generally has a good health condition.
2. Long-term service for liver and diuretic drugs is ineffective, and 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. Due to the thick nature of ascites, the thickness of the dark area changes little and slowly during body movement, which is easily misdiagnosed as abdominal cyst. Due to thickening of the omentum, it is a blocky strong echo, and some areas are seen to adhere to the intestine, so it is easily misdiagnosed as tuberculous peritonitis. From the clinical point of view and B ultrasound image can be distinguished from often misdiagnosed cirrhosis, tuberculous peritonitis, peritoneal mesothelioma.
complication
Although the peritoneal pseudomyxoma rarely metastasizes, it has malignant growth and strong vitality. It adheres to the peritoneal wall layer and then grows rapidly. It can be seen that the patient's entire abdomen is a mucinous mass, which is attached to the peritoneal wall layer and large. Omentum, mesentery, so that the intestinal lumen, often appear intestinal obstruction, peritonitis and other complications, causing adhesions of intestinal obstruction, intestinal fistula, pyloric obstruction and gallbladder obstruction, and finally cause patients can not eat, mesenteric failure.
treatment
Western medicine treatment
If the disease can be detected early, early surgical resection and chemotherapy, many patients can be clinically cured. Peritoneal pseudomyxoma is characterized by extensive invasion of the abdomen, surgery is not easy to complete, easy to relapse after surgery. In order to avoid recurrence, intraoperative injection of chemotherapy drugs and a large number of hormones in the anastomosis of the tumor, the prognosis is better. Radiotherapy and chemotherapy have not been effective in treating this disease. Some people have not achieved therapeutic effects with drugs such as trypsin, hyaluronidase and acetylcysteine. Therefore, some scholars advocate a combination of surgery and chemotherapy.
Combination therapy
(1) Excision of primary tumors such as ovary and appendix, as well as peritoneal nodules and retinal masses to alleviate tumor compression. Even if the ovaries and appendix look normal, they should be removed to avoid recurrence.
(2) suction mucus gelatinous ascites, if the suction tube is blocked, you can use gauze to clean the sticky ascites, wash the abdominal cavity with warm water, and press the yarn pad to stop bleeding.
(3) The abdominal cavity is then lavaged with an alkylating agent.
(4) When the tumor tissue is difficult to be completely removed, a plastic tube can be placed in the abdominal cavity, and an anticancer drug such as fluorouracil is injected from the catheter after surgery, and 32P is injected before extubation.
2. Intraperitoneal irradiation after intraperitoneal irradiation can improve the survival rate.
The above content is for reference only, please consult the relevant physician or relevant medical institution if necessary.
prevention
Prognosis
After the diagnosis is clear, it should be actively treated with surgery, plus chemotherapy and radiotherapy, and the prognosis is good. Peritoneal pseudomyxoma is a low-grade malignant tumor with minimal blood and lymphatic metastasis and a long survival period. It has been reported to have a 5-year survival rate of 45% and a 10-year survival rate of 40%, but it is prone to recurrence and requires reoperation or surgery. Anticancer drug treatment.
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