Primary malignant lymphoma of small intestine
Introduction
Brief introduction of primary malignant lymphoma of the small intestine Primary small intestinal lymphoma (PSIL) originates from the lymphoid follicles under the small intestinal mucosa and is more common. Most intestinal lymphomas are a partial manifestation of systemic lymphoma. basic knowledge The proportion of illness: 0.0002% Susceptible people: no specific population Mode of infection: non-infectious Complications: anemia, intussusception, intestinal obstruction
Cause
Causes of primary malignant lymphoma of the small intestine
(1) Causes of the disease
It is unclear whether the incidence of PSIL is in long-term chronic celiac disease (glutenic enteropathy), immunodeficiency diseases such as AIDS patients, long-term immunosuppressive therapy and immunoproliferative intestinal disease (IPSID). Can be significantly increased, so its incidence is related to the imbalance of the body's immune system; also believe that lymphoma is associated with certain viruses (such as EBV) infection, the vast majority of this disease belongs to the source of B lymphocytes, only part of the PSIL may be complicated by chronic celiac disease Patients from T lymphocytes and Hodgkin's disease often have low cellular immunity, and it is speculated that cellular immune disorders may occur in certain viral infections, leading to the occurrence and development of this disease.
(two) pathogenesis
In recent years, primary malignant lymphoma of the small intestine can be divided into four types: 1 adult type (Western type); 2 child type; 3 immunoproliferative small-intestinal disease (IPSID); 4 enteropathy related type T Enteropathy-associated T-cell lymphoma (EATCL).
1. Good hair
Small intestine malignant lymphoma generally originates from the submucosal lymphoid follicular tissue in the small intestine, infiltrates into the intestinal wall, and can occur in any part of the small intestine. However, because the distal small intestine has abundant lymphoid tissue, malignant lymphoma is more common in the ileum. 50%), followed by the jejunum (30%) and the duodenum (10% to 15%).
2. Pathology
Most of the primary small lymphoma of the small intestine are non-Hodgkin's lymphoma. The common type is mucosa-associated tissue lymphoma (MALT lymphoma). It is seen by the naked eye: the mass is isolated or multiple, and the multiple is divided into diffuse Sexual and spatial, diffuse can be seen diffuse thickening of mucosal folds with small nodules, small polypoid protrusions, often involving a segment of the intestine; isolated tend to be ring-shaped, can cause intestinal stenosis.
Small intestinal lymphoma can be divided into four types, namely polyp type, ulcer type, aneurysm type, infiltration and narrowing type.
(1) aneurysm type: the most common, infiltrating and growing along the intestinal wall, the muscle layer of the intestinal wall and the nerve plexus in the intestinal wall are damaged, making the intestinal wall thicker and harder, losing elasticity and aneurysmal expansion, so It is called cystic dilated lymphoma. The appearance of the tumor is around the intestine. The wall is stiff and leathery. The surface is dark red or grayish white. The mucosa often has multiple nodular bulges. The lumen is dilated, due to the thickening of the intestinal wall. , can form a large lump.
(2) infiltration and narrowing type: also more common, infiltrating the intestinal wall causes thickening and stiffness, creeping disappears, the intestinal lumen narrows, and finally narrows into a small inner diameter, mainly found in cases of reticulocyte sarcoma, this type often causes intestinal obstruction.
(3) ulcer type is rare, the ulcer is located in the central part of the invasive tumor, often multiple, the lesion range is small, but it can also be a large ulcer around the intestinal lumen, often prone to bleeding and perforation.
(4) polyp type: the least seen, the main lesion in the submucosa, polypoid into the intestinal lumen, so that the mucosal folds disappear, often multiple lesions, most prone to intussusception, it is also known as polypoid Nested lymphoma.
Most of the small intestine malignant lymphomas are single-shot and have multiple lesions. However, it is difficult to confirm that multiple lesions belong to multiple primary lesions or metastases from a primary tumor. It is reported that two or two digestive tracts occur simultaneously. The above lesions accounted for 10% to 30%, the lesions were separated by normal intestinal ducts, and in malignant lymphomas, multiple lesions were common in lymphocytic sarcomas.
3. Clinical staging
According to the depth of the tumor and the extent of invasion, the pathological process of malignant lymphoma is divided into several stages, which has important guiding significance for the judgment of clinical prognosis.
(1) Contreary staging method: stage I: tumor is confined to the intestine, no metastasis; stage II: mesenteric lymph node involvement; stage III: invasion of the aorta or adjacent organs.
(2) Mapvi staging method: Mapvi report clinical staging has important guiding significance for judging prognosis. He divided it into four phases: stage I: tumor is confined to intestinal tube, lesion is monofocal, no lymph node metastasis; stage II: tumor involvement Adjacent tissue; stage III: tumor involvement in regional lymph nodes; stage IV: extensive invasive or distant metastasis of the tumor, and the prognosis of stage I and II surgery is better.
4. Transfer route
Small intestine malignant lymphoma can be spread by direct spread, lymphatic or bloodway. The tumor can infiltrate along the intestinal wall, or penetrate the serosa directly into the mesentery, the omentum, the abdominal wall or adjacent organs, and occasionally penetrate the intestinal tube to form Guilty.
Regional lymph node metastasis is the main mode of metastasis of small intestine malignant lymphoma. It is generally more common than adenocarcinoma. It usually reaches the lymph nodes around the intestine, along the mesenteric lymphatic vessels to the regional lymph nodes, and then to the mesenteric lymph nodes. Later, it can be transferred to the axillary lymph nodes. In the para-aortic lymph nodes and even further lymph nodes, lymph node metastasis can be single-shot, but it is common for multiple fusions into a mass.
Trans-hepatic metastasis is less, and more often in the advanced stage, to the liver, pancreas, kidney, lung, brain and other organs, liver metastasis is the most common, can be a single nodule or multiple nodules.
Prevention
Prevention of primary malignant lymphoma of the small intestine
There is no effective preventive measure for this disease. Early detection and early diagnosis are the key to the prevention and treatment of this disease.
Complication
Complications of primary small malignant lymphoma of the small intestine Complications anemia intussusception
Anemia, small intestine perforation, intussusception, and intestinal obstruction are common complications of this disease, and are also common causes of patient visits.
1. Diarrhea and bloating: 1/3 of cases have abdominal distension, diarrhea or steatorrhea, which may be related to the extensive infiltration of the tumor and obstruction of the mesenteric lymphatic vessels and excessive bacterial growth in the intestine.
2. Some cases have fever, anemia, hypoproteinemia and weight loss.
Symptom
Symptoms of primary malignant lymphoma of the small intestine Common symptoms Lymphatic outflow bloating abdominal pain Abdominal mass Appetite loss Lymph node swelling Diarrhea Fatigue High heat nausea
The disease course is short, more than half a year, no specific clinical symptoms, clinical manifestations vary, mainly manifested as abdominal pain, abdominal mass, abdominal distension and other three symptoms, the disease Liu Jun reported that the three were 79.3%, 57.7 % and 37.9%; followed by diarrhea, fever, intestinal bleeding, nausea and vomiting.
Rapid weight loss, fatigue, gastrointestinal bleeding, most cases of bleeding less, with anemia, the abdomen can touch the mass, the size of the tumor is different, hard, nodular, tenderness, poor mobility, more cases due to perforation, obstruction Intussusception and emergency admission, more than half of the primary malignant lymphoma of the child's intestine was admitted to the hospital due to acute abdomen, which was manifested as intussusception or similar symptoms of acute appendicitis.
Abdominal pain
Occasionally, most patients often have intermittent pain, and more often occur after eating. As the disease progresses, abdominal pain is aggravated, and nausea and vomiting may be accompanied by episodes; the upper part of the duodenum and ileum may have an ulcer-like episode; Diffuse intestinal wall infiltration and progressive intestinal obstruction can cause chronic spasm pain; intussusception and perforation can cause acute abdominal pain, and abdominal pain is related to tumor location, most of which are located in the mid-abdomen, umbilical and lower abdomen.
2. Abdominal block
Nearly half of the patients can have abdomen and abdomen, mostly in the umbilical cord or right lower abdomen. The diameter of the tumor is usually about 5cm at the time of treatment. The texture is different, most of them can push the displacement, a few have tenderness pain, and the tumor is mostly due to the tumor itself or regional Mesenteric lymph nodes are caused by enlargement, and individual small intestine perforations are formed by wrapping the surrounding mesentery.
3. Diarrhea and bloating
One third of the cases have abdominal distension, diarrhea or steatorrhea, but this kind of diarrhea has no urgency or severe pus and bloody stools. It is easy to have bloating constipation or bowel habits after the end of the ileum is involved. It is rare to have a large amount of blood in the stool. Diarrhea may be associated with extensive tumor infiltration and obstruction of the mesenteric lymphatic vessels and bacterial overgrowth in the intestine.
4. Other
Some cases also have fever, anemia, hypoproteinemia and weight loss and fatigue, fever is irregular low fever, a few are periodic attacks, body temperature from 39 to 41 ° C, morning low afternoon high, spontaneous sweating without chills, broadcast High fever can occur in patients with loose lymphoma or small intestinal lymphoma with perforation or peritonitis. Liu Jun et al reported that 34.5% of patients have different degrees of anemia and hypoproteinemia, which is related to intestinal bleeding caused by tumor rupture and long-term malnutrition; Loss may be caused by lymphatic obstruction, small bowel dysfunction or partial intestinal obstruction caused by bacterial retention, pancreatic function and bile salt metabolism disorder, a small number of patients may have intestinal perforation, intestinal obstruction and intussusception and other clinical manifestations, this May be associated with the proliferation and development of small intestinal lymphoma, thickening of the intestinal wall, narrowing of the lumen of the intestine, and tumor growth of the tumor, combined with the invasion of the tumor to destroy the entire intestinal wall.
5. Signs
Most patients may have abdominal masses and lumps of different sizes. The general texture is hard, the surface is nodular, with mild tenderness, sometimes multiple nodules, and signs of incomplete intestinal obstruction are more common. Abdominal bulging, intestinal type, gas over water and bowel sounds hyperthyroidism, accompanied by acute perforation showed signs of diffuse peritonitis.
Examine
Examination of primary malignant lymphoma of the small intestine
1. Blood routine examination:
About 60% of cases have small cell hypochromic anemia.
2. Other blood tests:
ESR can increase and serum albumin can be reduced.
3. Fecal occult blood test:
Fecal occult blood test can be positive in 40% to 50% of cases.
4. Histopathological examination.
Auxiliary inspection
1. X-ray barium meal inspection
In particular, double contrast examination of small intestines is the most important auxiliary examination. The preoperative diagnosis rate is 30% to 70%, which can be generally divided into the following performances:
(1) Diffuse lesions: a wide range of lesions, all small intestines can be abnormal, most or all of the normal mucosal folds of the small intestine disappear, and numerous small polypoid filling defects can be seen in the intestinal lumen, ranging from mung beans to large peas. 0.5 to 1 cm in diameter, the lumen of the intestine is different, and a serrated notch can be seen along the intestinal wall, and polyps or other lesions can be seen in the stomach.
(2) multiple nodular filling defects, the edge of the lesion is clear, the mucosal pattern is disordered, destroyed or disappeared.
(3) stenotic lesions: central stenosis, marginal stiffness, mucosal folds as thin as lines, narrow range of stenosis is generally long, eccentricity is narrow, and one side of the stenosis has a large filling defect that protrudes into the intestine to make it thin. The lesions were limited, the external pressure was narrow, the intestinal lumen became thin and there was external pressure. The mucosal folds in the stenosis were still normal, the lesion range was longer, the boundary with the normal small intestine was unclear, and the proximal intestinal lumen was dilated.
(4) Dilated lesions: manifested as irregular expansion of the intestinal lumen, far beyond the scope of the tumor, the dilated segment is often the same as the stenosis segment, mucosal destruction, peristalsis disappears, the intestinal wall is stiff, aneurysm-like changes, and the intestinal motility is weakened. After a few hours, the dilated bowel can still be seen with expectorant retention.
(5) Intussusception: presents a typical intussusception X-ray manifestation, mostly caused by polypoid lesions, the intussusception is mostly located in the distal part of the small intestine, the most common is the end of the ileum, the above signs can be staggered, combined with clinical often diagnosis.
2. Endoscopy
Malignant lymphoma occurs at the end of the ileum and can be examined by colonoscopy and biopsy. Endoscopy is also helpful in the diagnosis of malignant lymphoma in the upper jejunum.
3.B ultrasound and CT
Abdominal masses, there are nodular masses between the intestines. B-ultrasound and CT help to understand its location, size, relationship with surrounding organs and lymph node metastasis, liver metastasis, ascites, etc., CT Examination of contrast in the intestinal lumen contrast, visible irregular expansion or stenosis of the intestinal lumen, lesions are generally more extensive, but early lesions, difficult to determine.
4. Surgical exploration
If the above diagnosis has not been clearly diagnosed, early laparotomy should be performed to achieve the purpose of diagnosis and treatment.
Diagnosis
Diagnosis and differential diagnosis of primary malignant lymphoma of the small intestine
diagnosis
The diagnosis of primary malignant lymphoma of the small intestine is difficult, the clinical manifestations are varied, and there is no regularity. The laboratory tests are lack of characteristics, and there is no specific and reliable diagnostic method. Therefore, the preoperative diagnosis rate of this disease is very low, when the patient appears Irregular abdominal pain, diarrhea, weight loss, loss of appetite, fever and other gastrointestinal symptoms, examination of the abdominal mass of the mass, especially in the case of incomplete intestinal obstruction, should consider the possibility of small intestinal malignant lymphoma, through the full Gastrointestinal barium meal examination helps to locate the diagnosis. CT and B-ultrasound examination is helpful to judge whether the tumor is metastasized. Endoscopic direct vision and clamped biopsy for histopathological examination can confirm the diagnosis.
Domestically reported 101 cases of intestinal lymphoma, only 8 cases were diagnosed before surgery; Liu Jun et al reported that 29 cases of primary malignant lymphoma of the small intestine only 2 cases were diagnosed as malignant lymphoma before surgery, the remaining 27 cases were in operation After diagnosis, the rate of misdiagnosis is as high as 93.1%. The prognosis of this disease depends on early diagnosis and thorough surgical treatment. Therefore, in addition to actively exploring effective diagnostic methods, it is necessary to first improve the vigilance of the disease and familiarize with its clinical manifestations, Bawson proposed 5 Diagnostic criteria:
1. The superficial lymph nodes of the whole body are not swollen.
2. There is no lymph node enlargement in the mediastinum.
3. The tumor is located in the intestine or is involved in the lymph node of the drainage area.
4. Liver spleen and bone marrow without lymphoma lesions.
5. The total number and classification of peripheral blood leukocytes were normal.
At this time, a comprehensive and meticulous examination should be carried out, and laboratory and other auxiliary examinations should be further carried out for suspicious cases.
If the above diagnosis has not been clearly diagnosed, early laparotomy should be performed to achieve the purpose of diagnosis and treatment.
Differential diagnosis
It is mainly distinguished from Crohn's disease, intestinal tuberculosis and small bowel cancer in intestinal inflammatory diseases.
Crohn's disease
There may be segmental stenosis, signs of pebbles or pseudopolyps, sometimes difficult to distinguish from malignant lymphoma, but Crohn's disease generally has a long history, often has a history of recurrence and perianal abscess, may have abdominal masses, often due to Local inflammation perforation formed internal hemorrhoids, sputum examination showed internal iliac lesions, segmental stenosis was smooth, proximal segment expansion was more obvious, linear ulcer was on the mesenteric side, and mucosa was concentrated, intestinal fistulas could be gathered, and wheel-like changes, small intestine Malignant lymphoma generally has no internal hemorrhoid formation, and the clinical manifestations are severe. The narrow segment of the X-ray is not segmental, the edges are not smooth, the nodules vary in size, and the ulcers and cavities are large and irregular.
2. Intestinal or peritoneal tuberculosis
Abdominal mass can also occur, sometimes difficult to identify with malignant lymphoma, but the former generally have a history of tuberculosis, low fever, night sweats and erythrocyte sedimentation rate, abdominal examination has a facial sensation, the general condition does not appear progressive deterioration, small intestinal tuberculosis X-ray showed proliferative type of single or multiple localized intestinal stenosis, the edge was smoother than malignant lymphoma, and the proximal expansion was more obvious; the ulcer type was generally perpendicular to the longitudinal axis of the intestine, ulcer of malignant lymphoma The location is uncertain, and the shadow is large and irregular.
3. Small bowel cancer
The lesions are often limited and rarely reach the mass. Even if there are smaller, limited masses, the X-ray barium meal examination is only a localized intestinal stenosis and mucosal destruction.
4. Immunoproliferative small bowel disease (IPSID)
It is a unique small intestinal lymphoma originally reported in Eastern Jews and Arabs, also known as Mediterranean lymphoma or alpha-heavy chain disease. Typical symptoms include chronic diarrhea, steatorrhea, accompanied by vomiting and abdominal cramps. Also known as clubbing, a rare feature of many IPSID patients is that there is an abnormal IgA in the blood and intestinal secretions, the -heavy chain is shortened, and no light chain is involved, and IPSID occurs mostly in the intestines. In areas where bacteria and parasites are infected, it is believed that the cause may be caused by long-term repeated stimulation of B lymphocytes in the small intestine by intestinal microbial antigens, causing cell mutation and malignant transformation. Abnormal -chains are plasma cells from the small intestine. As a result, IPSID patients often die from progressive malnutrition and failure, or die from invasive lymphoma.
