Rare lung cancer
Introduction
Introduction to rare malignant tumors in the lungs Among the rare malignant tumors in the lungs, the majority are peripheral, and nearly half of the patients have no clinical symptoms, of which lymphoma accounts for 41%, carcinosarcoma accounts for 20%, mucinous epithelial cancer accounts for 15%, sarcoma accounts for 18%, and the rest is Malignant melanoma. basic knowledge Sickness ratio: 0.0001% Susceptible people: no special people Mode of infection: non-infectious Complications: lung metastases
Cause
The rare cause of malignant tumors in the lungs
Cause:
Among the rare malignant tumors in the lungs, the majority are peripheral, and nearly half of the patients have no clinical symptoms, of which lymphoma accounts for 41%, carcinosarcoma accounts for 20%, mucinous epithelial cancer accounts for 15%, sarcoma accounts for 18%, and the rest is Malignant melanoma.
Prevention
Rare malignant tumor prevention in the lungs
1. The annual physical examination is not a substitute for the screening of malignant tumors. The screening items for physical examination are different from the screening programs for malignant tumors. Clinically, people who have just participated in the physical examination of the unit organization are considered to be healthy. After 2-3 months, chest CT examination reveals lung shadow and confirms the diagnosis of lung cancer.
2. For smokers who are older than 55 years old and have a smoking index greater than 400, it is not enough to have a chest fluoroscopy or a chest radiograph at the time of physical examination. At least the chest radiograph should be examined. Early lung lesions can be detected by low-dose spiral CT examination of the chest.
3. For middle-aged and elderly people with high cancer incidence, regular and lateral chest X-ray examination or chest low-dose spiral CT examination should be added during routine health examinations; in the case of respiratory symptoms such as irritating dry cough, it should also be Go to the hospital for low-dose spiral CT examination of the chest, which is conducive to early detection of lung cancer.
Complication
Rare malignant tumor complications in the lungs Complications
Complications are the infiltration of cancerous tissues into other tissues and organs. The main pathways for metastasis of lung malignant tumors include direct spread, lymphatic metastasis, and hematogenous metastasis.
Symptom
Symptoms of malignant tumors rare in the lungs Common symptoms, night sweats, lungs, solid breath sounds, weakened nodular sclerosis, weak atelectasis
Among the rare malignant tumors in the lungs, the majority are peripheral, and nearly half of the patients have no clinical symptoms, of which lymphoma accounts for 41%, carcinosarcoma accounts for 20%, mucinous epithelial cancer accounts for 15%, sarcoma accounts for 18%, and the rest is Malignant melanoma.
Pulmonary lymphoma: divided into Hodgkin's disease and non-Hodgkin's lymphoma, which account for 0.5% or 0.33% of lung tumors, respectively. Primary lung lymphoma is rare, mostly metastases, mostly Hodge's primary mediastinum. Metastatic tumor of gold lymphoma, the prognosis of primary lung lymphoma is better than secondary.
1. Lung secondary lymphoma lymphoma involving 44% to 70% of the lungs, for the anterior mediastinum or paratracheal to adjacent mediastinal lymph nodes, then to the hilar lymph nodes, and finally to the lungs, lung involvement can be a direct diffusion or separation of the knot Section, only when a large lesion (referring to the anterior mediastinum or endotracheal mass > 30% of the chest diameter) only involved the pleura, pericardium or chest wall, the above mainly refers to Hodgkin's lymphoma, non-Hodgkin's lymphoma at autopsy About 50% of the lungs involve the lungs, the most common of which is the large cell type, which can also be seen in patients who relapse after treatment or secondary to the lungs.
CT can see the following performance
1 pulmonary nodules <1cm.
2> 1 cm mass or mass-like fusion with or without a cavity.
3 alveolar or interstitial exudation.
4 pleural mass.
5 thickening around or around the bronchi, with or without atelectasis.
6 pleural effusion.
7 hilar or mediastinal lymph node hyperplasia, more than 68% of patients can also see the above three or more abnormal signs of CT, can be used to identify other diseases.
2. Primary pulmonary lymphoma Non-Hodgkin's lymphoma: A rare primary pulmonary lymphoma can occur in any part of the lung where normal lymphoid tissue is present, such as bronchial-associated lymphoid tissue and mucosa-associated lymphoid tissue, pulmonary interstitial or lung Internal, subpleural lymph nodes, intrapulmonary and subpleural lymph nodes are more common, especially those >25 years old, lymphangiography confirmed that 18% of normal people have intrapulmonary lymph nodes in the lung parenchyma, lung non-Hodgkin's lymphoma staging.
Primary lung lymphoma is mainly derived from B lymphocytes, and it has been reported that it can be derived from central cells, which are transformed from parafollicular B lymphocytes, which express - or -immunoglobulin light chains, suggesting From the clonal proliferation of a single B cell, it is difficult to identify benign lymphoid lesions (including lymphocytic interstitial pneumonia, pseudolymphoma) and pulmonary primary lymphocytic lymphoma.
Three indicators for the identification of benign, malignant lymphoid lesions: 1 immature lymphocytes; 2 no germinal center; 3 involving hilar lymph nodes, but some people think that the third item can not be used for diagnosis must be benign disease, in short, benign lymphoma The main clinical problem is to identify each type and to make a clear diagnosis.
Lymphocytic interstitial pneumonia: Carrington and Lebowl first reported in 1966 that Liebow and Carrington further defined LIP as extensive pulmonary interstitial exudation of lymphocyte plasma cells and histiocytes in 1973, and some cases may have germinal centers, called "A wide range of lymphoid tissue hyperplasia", some people think that LIP is related to immune function defects, such as: abnormal gamma globulinemia, the vast majority of patients are adult women (50 ~ 70 years old), no specific symptoms, typical chest X-ray features In order to diffuse the bilateral lobular oozing, small 1cm, large in about 3cm nodules or patches, this tumor can be associated with immune diseases, such as: Sjoogren syndrome (1/3), connective tissue disease, itself Immune and immunodeficiency diseases (including AIDS).
Pseudolymphoma: In 1963, Saltzstein named a benign lymphocytic proliferative pseudotumor when it was differentiated from malignant lymphocytes. After that, it became a nodular lymphatic hyperplasia of the lung. Tumor, the tumor is reactive lymphoid hyperplasia, one or more nodules or local exudation in the lung, no symptoms, seen in 30 to 80 years old, an average of 51 years old, the symptoms are fever, the removal of mass can be diagnosed and treated, The recurrence rate of the operation area is low and the prognosis is good.
Small lymphocytic lymphoma: When non-Hodgkin's disease involves the lungs, 50% to 60% of patients are hyperplasia of small lymphocytes and plasmacytoid lymphocytes. The age of onset is 20 to 90 years old, the peak value is 60 years old, and men and women are equal. 1/3 asymptomatic, symptoms include: cough, hernia, chest pain, hemoptysis, etc., surgical resection or chemotherapy, radiotherapy, good prognosis, 70% to 83% can survive for 5 years, the median survival is 4 to 9.75 years.
Large cell histiocytic lymphoma: rare, can be combined with AIDS, non-AIDS patients are more common in women, non-AIDS patients in the 50 to 60 years old, hilar lymph nodes are susceptible, lesions tend to occur in the upper lung, but also lung involvement It can invade the chest wall or pleura. The cavity can be seen in mixed type (ie, large and small cell type). It should be surgically removed as much as possible. Radiotherapy should be performed when the hilar lymph nodes are positive. Chemotherapy is required for dissemination. Large cell type is more than small cell type. Invasive, the prognosis is worse, 53% of patients can relapse during the first few months to several years of treatment.
Lymphocytoma: Liebow was first reported in 1972. Its atypical lymphocyte exudation affects the blood vessels of the lungs and other organs (skin and brain). It is more common in middle-aged adults. There are more men, cough, belching, chest pain. Fever, fatigue and weight loss, chest X-ray: multiple pulmonary nodules, 0.6 ~ 0.8cm, unclear border, located in the lower lobe, cyclophosphamide and prednisone may be beneficial, 2 / 3 median survival is 14 Months, 38% of patients died within 1 year, with an average survival of 23.8 months. The disease may be peripheral T-cell lymphoma, the primary can be in the lungs, and the prognosis is poor.
Plasma cell lesions: macroglobulinemia, plasmacytoma and multiple myeloma are rare in the lungs. Noach first reported macroglobulinemia involving the lungs in 1956. Systemic symptoms may include lymphadenopathy, splenomegaly, weight loss, etc. The above should be surgically removed as much as possible, but the vast majority can only be chemotherapy, multiple myeloma can be involved in the lung as part of a single or systemic disease.
Primary pulmonary Hodgkin's disease: the primary lung Hodgkin is not common, the average age is 42.5 years old (12-82 years old), the peak age is bimodal, the first peak is 21 to 30 years old, and the second peak is 60-80. The age of males and females is 1:4. The most common symptoms are cough, weight loss, chest pain, hernia, hemoptysis, fatigue and rash. The chest examination shows signs of lung consolidation. It can also be basically normal. Other signs include: abnormal thoracic breathing. Exercise, rash, edema and lymphadenopathy, radiology: the vast majority of lung nodules, cavities, exudation, atelectasis and pleural effusion, bronchoscopy more normal, almost all need open thoracotomy In the diagnosis, histology is most likely to be a mixed cell type of nodular sclerosis or Hodgkin.
Treatment includes surgery, chemotherapy and radiotherapy. The prognosis of patients with affected lung tissue exceeding 1 leaf is poor. Other factors with poor prognosis are: fever, night sweats, weight loss of more than 10%, pleural effusion, and cavities.
3. Soft tissue sarcoma The original interstitial cells are present in a certain organ of the human body, and their proliferation and maturation are fatty fibrous tissue, muscle, cartilage or bone. The interstitial origin of the tumor originates from the matrix component of the bronchial or vascular wall or the lung parenchyma. Interstitial, which grows into the lung parenchyma, can even protrude into the bronchial cavity, invading and breaking through the bronchial epithelium, so there is no epidermal exfoliated cells, and cytological examination is not beneficial.
The gross tumor is bordered, and there is a capsule in the lung parenchyma, usually local invasion, which may invade the pleura and chest wall, and is rare.
Can occur at any age, equal in men and women, the incidence of left and right lungs is equal, common symptoms are: cough, chest pain, hernia, hemoptysis, fever, fatigue, fear of food, etc., weight loss is often a late symptom, often single, limited to a lung It has a diameter of 1 to 15 cm or more, and a diameter of 6 to 7 cm. It may have pleural effusion after invading the chest wall, 15% of tumors obstruct the bronchus and cause distal lung changes, multiple blood metastases, and lymphatic metastasis is rare. Some people divide it into three types. :
1 lung parenchyma and bronchial (inner) sarcoma.
2 large vascular source sarcoma.
3 small vascular source sarcoma.
4. Lung parenchyma and bronchial (inner) sarcoma: primary tumors are rare, common types are:
(1) Primary pulmonary synovial sarcoma: Synovial sarcoma is a unique and rare soft tissue malignant tumor, accounting for about 12% of soft tissue sarcoma. Only 3 cases of primary pulmonary synovial sarcoma were reported in English literature before 2002.
In 1934, Sabrazes considered it to be a malignant tumor that originated from the synovial membrane or differentiated into the synovial membrane and named it synovial sarcoma. Synovial sarcoma is generally considered to be a malignant tumor characterized by synovial differentiation in mesenchymal cells. It is divided into single-phase type and two-way type. In some cases, the cell differentiation is very poor, and the morphology and prognosis are different from the above two types. It is called poorly differentiated type. Synovial sarcoma occurs mostly in the vicinity of the large joints of the extremities, and the tendon and tendon sheath. The synovial structure is closely related, often located in the joint capsule, and can also occur in the area without synovial structure, accounting for 5% to 15%.
The main clinical manifestations of synovial sarcoma occurring in the lung are chest pain, hemoptysis, shortness of breath and cough. The clinical manifestations of synovial sarcoma occurring outside the lung are mainly mass and pain, while the gross specimen of synovial sarcoma is clear. , but no capsule, gray or brown, cut surface is fish-colored, crisp, visible mucus degeneration or hemorrhagic necrosis area, microscopic examination of tumor cells with two-fold differentiation of epithelial-like cells and interstitial cells, ultrastructure showing tumor The cytoplasm of the cell is rich in ribosomes, the mitochondria are dilated, the rough endoplasmic reticulum is distributed in large numbers, and the well-developed desmosome cells are connected. Synovial sarcoma is easily misdiagnosed as other soft tissues due to its complex structure. Sarcoma or inflammation.
Primary lung synovial sarcoma should be distinguished from lung metastatic synovial sarcoma in differential diagnosis. Secondary sarcoma should be differentiated from other primary sarcoma of the lung (fibrosarcoma, leiomyosarcoma, angioendothelioma, schwannomas). Immunohistochemical staining can help distinguish between the two.
In the treatment of synovial sarcoma, the primary tumor should be extensively resected. According to the clinical stage, the effective dose before and after surgery, chemotherapy and radiotherapy should not be less than 40cGy. The radiotherapy field should include the tumor bed and surrounding normal tissues of 2~5cm. In the treatment of sarcoma is very important, the most commonly used is the VAC program. In recent years, ADM, DPP, Vp-16, IFD, DTIC and taxane have been used in soft tissue sarcoma and have achieved good results, with synovial sarcoma. The factors related to prognosis are the age of the patient, the location of the lesion, the size of the lesion, and the length of the disease. It is crucial to diagnose and treat each patient early. Enzinger reports that the 5-year survival rate of synovial sarcoma is 25.2%. The diameter of <4cm or extensive calcification has a good prognosis, the 5-year survival rate can reach 82%, and the recurrence is more than 2 years after treatment. There are 1/3 to 1/2 cases of metastasis, and the inheritance of synovial sarcoma in recent years. Studies have found that the prognosis of synovial sarcoma is significantly correlated with the nuclear mitotic rate of the tumor. The aneuploid DNA content, especially the s phase, can be used as an objective indicator for determining the malignancy of synovial sarcoma. It is generally considered that the mitotic rate is >10/ 10HPF has a poor prognosis.
(2) fibrosarcoma and leiomyosarcoma: can occur in the trachea and lung parenchyma. Such sarcoma in the bronchi is more common in adolescents, and may have symptoms such as cough and hemoptysis, which are mostly confined in the bronchi, and do not invade the lung parenchyma. The prognosis is good. Because it is easy to cause symptoms, it is often diagnosed and operated at an early stage. The surrounding lesions are mostly solid, occasionally hollow, and the prognosis is worse than that of endobronchial sarcoma. Some people think that the smaller prognosis of the tumor is better. Its growth is slow, the 5-year survival rate is 45%, the mitotic rate of tumor cells, the size and involvement of surrounding tissues (including chest wall, hernia, mediastinum) are prognostic factors.
(3) Lung rhabdomyosarcoma: rare, most of the rhabdomyosarcoma occupy one or more lobes, invading local tissues, especially the bronchial and pulmonary veins, may be associated with congenital adenoid malformations, and pulmonary blastoma is occasionally accompanied by congenital adenoids. Malformation, more common in children.
(4) malignant fibrous histiocytoma: common in adult limbs and retroperitoneum, rare in the lungs, and less than fibrosarcoma, age seen in 18 to 80 years old, an average of 55 years old, common respiratory symptoms, uniform distribution of each leaf, treatment : Select complete resection, postoperative radiotherapy, chemotherapy, prognosis: poor, with staging, thorough resection, whether it violates the chest wall and metastasis.
(5) Pulmonary chondrosarcoma: age 23 to 73 years old, average 46 years old, equal in men and women, respiratory symptoms, more common in the left lung, calcification or ossification in the chest X-ray, generally visible in the cut surface gray, with envelope, border Clear, microscopically seen calcified or ossified malignant cartilage, the prognosis is poor.
(6) Pulmonary osteosarcoma: rare, defining extra-osseous osteosarcoma:
1 The tumor must consist of a single sarcoma tissue, except for the possibility of malignant mixed stromal tumors.
2 The bone or bone component must be composed of sarcoma.
3 The primary bone tumor can be excluded. The patients are 35 to 83 years old, with an average age of 61 years. The male and female are equal. The most common symptoms are chest pain, left and right onset, etc. The treatment is resection and the prognosis is poor.
(7) Other sarcomas: fat, neurogenic sarcoma and malignant stromal tumors are equally rare. Prognosis: All of the above spindle cell sarcomas, except for leiomyosarcoma and endobronchial sarcoma, have a poor prognosis and rarely survive for 1 year.
5. Sarcoma of large blood vessel origin Pulmonary artery stem sarcoma is a tumor of the pulmonary artery originating from the pulmonary artery or heart. The main cell types of the tumor are divided into: undifferentiated, smooth muscle and fibrosarcoma, distal to the blood vessel or outside the blood vessel. Invade the lungs to spread.
Can be seen in 20 to 81 years old, an average of 50 years old, women are slightly more common, the symptoms are chest pain, hernia, cough, hemoptysis and palpitations, may have systolic murmur, pulmonary hypertension with proximal vasodilation is a specific indication, late Right heart decompensation performance.
X-ray chest X-ray: see lobulated para-glandular mass; angiography see multiple defects in the pulmonary artery; CT and MRI can help diagnosis, treatment: should be performed, after surgery can assist other treatment, but the prognosis is poor.
6. Small blood vessels originating from sarcoma malignant or low-grade malignant lungs are: angiosarcoma, epithelioid hemangioendothelioma, angioendothelioma and hemangioendothelioma, Kaposi sarcoma is not discussed here because it has no primary lung The "vascular aneurysm" defined by Enzinger and Weiss (1983) is generally considered to be a vascular tumor, but its accurate histological classification cannot be determined as its ultimate biological behavior.
Angiosarcoma: Very rare in the lungs, most likely metastatic carcinoma of the right ventricle of the pulmonary trunk or extrathoracic, may be associated with hemothorax or hypertrophic pulmonary osteoarthrosis, with poor prognosis.
Epithelioid hemangioendothelioma: Dail and Liebow were first reported in 1975, initially called intravascular bronchoalveolar tumor, later called sclerosing endothelial tumor, and finally named epithelioid hemangioendothelioma, visible in soft tissue, lung, liver, bone, age 4 to 70 years old, 1/3 is under 30 years old, women are 4 times as many as men, asymptomatic or have dry cough, X-ray and CT: many small nodules of the lungs, about 1cm in diameter, average survival after diagnosis 4.6 In the year, there were also reports of people who survived 24 years after repeated surgical resection, mostly due to pulmonary insufficiency.
Angioendothelioma: originated from ubiquitous capillary epithelial cells, often located in the lower extremity soft tissue and retroperitoneum, occurring in the lungs of men and women equal, average 46 years old, 1/3 asymptomatic, may have chest pain, hemoptysis, hernia and cough Even with pulmonary osteoarthrosis, X-ray chest showed: lobulated-boundary clear, soft tissue with uniform density.
Treatment for surgical resection, prognosis is different, according to chest symptoms, size (more than 8cm), pleural and chest wall invasion, tumor necrosis and giant cells (> 3 mitosis / high power field), > 5cm, 33% transfer, 66% of patients with >10cm had metastasis, and more recurrence within 2 years after diagnosis.
7. Carcinosarcoma These tumors are mainly composed of epithelial components and interstitial components. The epithelial components are often squamous cell carcinoma. The interstitial components are often fibrosarcoma. Compared with single sarcoma and squamous cell carcinoma, the differentiation degree and prognosis of carcinosarcoma are significantly worse. .
More common in patients over 50 years old, males are about 5 times more common in women, the most common in the distal bronchus, the tumor often grows slowly, more is intraluminal growth, invasive tracheal wall growth is less common, but also visible invasion Peripheral lung tissue, local lymph node metastasis and distant metastasis, especially brain metastasis are the most common symptoms. Cough, hemoptysis, chest pain and discomfort are also visible. There may be pulmonary osteoarthrosis. The surrounding can be asymptomatic. The lung cancer sarcoma has the following characteristics:
More common in older men.
2 large bronchial cavity polypoid mass.
3 Histology: Malignant epithelial cell clusters can be seen as a matrix composed of atypical spindle or pleomorphic sarcoma cells.
4 Immunohistochemistry: Cytokeratin antibodies display epithelial components, while matrix responds to vimentin.
Surgery should be performed as much as possible. The 1-year survival rate is less than 20%. A few patients can survive for more than 5 years or longer, and those who live for 10 to 20 years.
8. Pulmonary blastoma is composed of two malignant interstitial and epithelial tumors. Its composition is similar to carcinosarcoma, which is called embryonic state of the lung in 3 months. It is called lung blastoma. In 1952, Bamard was an embryonic tumor. The noun first reported the disease; in 1961, Spencer was named blastoma.
According to histological characteristics, it is divided into:
1 Differentiated embryonic adenocarcinoma, which is a malignant epithelium-like embryonic lung, but has no malignant matrix.
2 biphasic blastoma, the average age of 35 years (1 to 72 years old), female slightly more than male, 41% asymptomatic, cough, hemoptysis, hernia, no abnormal physical examination, individual breath sounds weakened.
X-ray chest radiograph: unilateral lung mass, can be peripheral or central type, no special test, fiberoptic bronchoscopy and puncture are useful for diagnosis, 54% are well differentiated embryo adenocarcinoma, 46% are biphasic blastoma The size of the former is 1 to 10 cm (average 4.5 cm), and the latter is 2 to 27 cm (average 10.2 cm). The histology is malignant gland and adult sarcoma-like or embryonic interstitial components.
Treatment with surgical resection can be supplemented with chemotherapy. The prognosis of tumors <5cm is better. The prognosis of differentiated embryonic adenocarcinoma is better than that of bipolar blastoma. Metastasis and recurrence are the reasons for poor prognosis.
9. Malignant teratoma Intrapulmonary teratoma is rare, most of the malignant occurs in the left upper lobe, the cause is unknown, half is malignant, easy to be confused with blastoma, poor prognosis.
10. The cause of malignant ependymoma is unknown. It is speculated that it is a chemical production in the treatment of small cell carcinoma. Crotty first reported in 1992 that a patient with small cell carcinoma after treatment, a single peripheral peripheral ependymoma And immunological characteristics are significantly different from small cell tumors.
11. Bronchial malignant melanoma can occur in 29 to 80 years old. There is no difference between men and women. Most of them are located in carina and bronchus. The incidence of trachea is rare. Symptoms and radiology are the same as those of primary bronchial carcinoma. Diagnosis of primary bronchial malignant melanin Tumors, first need to exclude other primary sites, the reference criteria are as follows: 1 previous history of no skin lesions (especially melanoma).
2 history of surgery without eye tumors.
3 single lung tumors.
4 morphology is the primary tumor feature.
5 no melanoma of other organs at the time of resection; 6 no autoimmune melanoma of other organs at the time of autopsy, all cases must be differentiated from black carcinoid, immune and ultrastructural difference between the two.
Once the primary tumor is diagnosed, it should be surgically removed and the prognosis is moderate. It is reported that the longest survival can be 11 years.
Examine
Examination of rare malignant tumors in the lungs
1. The sputum cytology examination is simple and easy, but the positive detection rate is only 50% to 80%, and there is a false positive of 1% to 2%. This method is suitable for censuses in high-risk groups, as well as isolated images in the lungs or diagnosed with unexplained hemoptysis.
2. Percutaneous lung puncture cytology is suitable for peripheral lesions and is not suitable for thoracotomy for various reasons. Other methods have failed to establish histological diagnosis. At present, it is preferred to use a fine needle in combination with CT, which is safer to operate and has fewer complications. The positive rate was 74% to 96% in malignant tumors and 50% to 74% in benign tumors. Complications include pneumothorax 20% to 35% (about 1/4 of which need to be treated), a small amount of hemoptysis 3%, fever 1.3%, air embolism 0.5%, and needle implant 0.02%. Thoracic surgery has fewer applications because of thoracoscopic examination and thoracotomy.
3. Thoracic puncture cytology in patients with suspected or diagnosed lung cancer, there may be pleural effusion or pleural dissemination, cell analysis of pleural effusion by thoracentesis can be clearly staging, for some cases, can also provide a basis for diagnosis . For lung cancer with pleural effusion, bronchoalic adenocarcinoma has the highest detection rate, and its positive rate of cytological diagnosis is 40% to 75%. If the cytological analysis of the pleural effusion obtained by puncture cannot be diagnosed, consider further examinations such as thoracoscopic surgery.
Diagnosis
Diagnosis and diagnosis of malignant tumors rare in the lung
Most of the tumors are located in the peripheral part of the lungs, and the volume is small. Most of them are single-shot, round, elliptical, lobulated or nodular, with uniform density, sharp edges and very few burrs. X-ray chest X-ray, tomography, bronchography, CT scan and other examinations have a high diagnostic value for the display and analysis of the characteristics of benign lung tumors, and the final diagnosis depends on histopathological examination.
