Primary lung lymphoma:Lymphoma involving the lung accounts for 44% to 70%, and it involves the anterior mediastinum or paratracheal to the adjacent mediastinal lymph nodes, then to the hilum of the lung, and finally to the lung. The lung involvement can be direct spread or separate nodules, and it only involves the pleura, pericardium, or chest wall when large lesions (referring to mediastinal or paratracheal masses >30% of chest diameter) are present. The above mainly refers to Hodgkin's lymphoma. Non-Hodgkin's lymphoma involves the lung in about 50% of autopsies, the most common type being large cell type, which can also be seen in recurrence or secondary involvement after treatment or secondary to the lung.
CT shows the following manifestations:
1. Pulmonary nodule
2. Mass or mass-like fusion larger than 1 cm, with or without cavities.
3. Alveolar or interstitial exudation.
4. Pleural mass.
5. Peribronchial or perivascular thickening, with or without atelectasis.
6. Pleural effusion.
7. Hypertrophy of hilar or mediastinal lymph nodes, which is seen in more than 68% of patients with 3 or more CT abnormal signs, which can be used to distinguish other diseases.
2. Primary pulmonary lymphoma:Non-Hodgkin lymphoma: Rare primary pulmonary lymphoma can occur in any normal location of lymphoid tissue in the lung, such as bronchus-associated lymphoid tissue and mucosal-associated lymphoid tissue, lung interstitium or intrapulmonary, subpleural lymph nodes, and it is more common in the intrapulmonary and subpleural lymph nodes, especially in those older than 25 years. Lymphangiography confirmed that 18% of normal people have intrapulmonary lymph nodes in the lung substance, and staging of pulmonary non-Hodgkin lymphoma.
Primary pulmonary lymphoma mainly originates from B lymphocytes, and some people report that it can originate from centrocytes, which are derived from peripheral B lymphocytes. These B lymphocytes express κ- or λ- immunoglobulin light chains, suggesting a clone proliferation from a single B cell. It is very difficult to distinguish between benign lymphoid lesions (including lymphocytic interstitial pneumonia, pseudolymphoma) and primary pulmonary lymphocytic lymphoma.
Three indicators for distinguishing benign and malignant lymphoid lesions: ① Immature lymphocytes; ② Absence of germinal centers; ③ Involvement of hilar lymph nodes. However, some people believe that the third item cannot be used to diagnose a benign disease for sure. In summary, the main clinical problem of benign lymphoma is to distinguish between different types and make a clear diagnosis.
3. Soft tissue sarcoma:The primitive mesenchymal cells exist in some organ of the human body, and their proliferation and maturation form fatty fibrous tissue, muscle, cartilage, or bone. Tumors of mesenchymal origin originate from the stromal components of the bronchial or vascular wall or the mesenchyme of the lung substance. They penetrate the lung substance and occasionally protrude into the bronchial lumen, invade and break through the bronchial epithelium, so there are no desquamated cells, and cytological examination is not helpful.
The gross tumor is well-demarcated, encapsulated intrapulmonary mass, usually locally invasive and disseminated, possibly involving the pleura and chest wall, and rarely has cavities.
It can occur at any age, with equal incidence in males and females, and the incidence of left and right lungs is equal. Common symptoms include: cough, chest pain, shortness of breath, hemoptysis, fever, fatigue, anorexia, etc. Weight loss is often a late symptom, usually solitary, limited to one lung, with a diameter of 1-15 cm or more, generally 6-7 cm in diameter. After invasion of the chest wall, there may be pleural effusion. 15% of tumors can block the bronchus and cause changes in the distal lung, mostly hematogenous metastasis, and lymphatic metastasis is rare. Some people divide it into 3 categories:
1. Sarcoma of lung substance and bronchus (intra-)
2. Large vascular source sarcoma.
3. Small vascular source sarcoma.
4. Sarcoma of lung substance and bronchus (intra-)Primary tumors are rare, and common types include:
1. Primary pulmonary synovial sarcoma:Synovial sarcoma is a unique and rare malignant soft tissue tumor, accounting for about 12% of soft tissue sarcomas, and only 31 cases of primary pulmonary synovial sarcoma were reported in English literature before 2002.
In 1934, Sabrazes believed that it originated from articular synovium or differentiated towards synovium and named it synovial sarcoma. Currently, it is widely believed that synovial sarcoma is a malignant tumor originating from mesenchymal cells with synovial differentiation characteristics, classified into monophasic and biphasic types. Some cases have very poor cell differentiation, with morphology and prognosis different from the above two types, and are called poorly differentiated types. Synovial sarcoma mostly occurs in the adjacent areas of large joints in the limbs, closely related to tendons, tenosynovial structures, and synovial tissue, often located at the joint capsule, and can also occur in areas without synovial tissue, 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 masses and pain. The macroscopic examination of synovial sarcoma shows clear boundaries of the mass but no capsule, with a gray or brownish color. The cut surface is flesh-colored, friable, and may show mucinous degeneration or hemorrhagic necrosis areas. Microscopic examination shows that tumor cells have the function of differentiating into both epithelial-like cells and stromal cells. The ultrastructure of tumor cells shows a rich cytoplasm with abundant ribosomes, occasionally with expanded mitochondria, a large number of saccular rough endoplasmic reticulum, and well-developed desmosome-type cells connected together. Due to the complex tissue structure of synovial sarcoma, it is easily misdiagnosed as other soft tissue sarcomas or inflammation.
In the differential diagnosis of primary pulmonary synovial sarcoma, it is first necessary to distinguish it from metastatic synovial sarcoma to the lung. The subtypes of sarcoma must be differentiated from other primary sarcomas in the lung (fibrosarcoma, smooth muscle sarcoma, hemangiopericytoma, schwannoma), and immunohistochemical staining can help distinguish between them.
In the treatment of synovial sarcoma, it is generally recommended to perform extensive resection of the primary tumor. Depending on the clinical stage, preoperative and postoperative radiotherapy and chemotherapy can be adopted. The effective dose of radiotherapy should not be less than 40cGy, and the radiation field should include the tumor bed and 2-5cm of normal tissue around it. Chemotherapy is very important in the treatment of sarcomas, with the most commonly used regimen being VAC. In recent years, drugs such as ADM, DPP, Vp-16, IFD, DTIC, and taxanes have been used in soft tissue sarcomas and have achieved good efficacy. The factors related to the prognosis of synovial sarcoma are the patient's age, the site of the lesion, the size of the lesion, the duration of the disease, and most importantly, early diagnosis and treatment for each patient. Enzinger reported that the 5-year survival rate of synovial sarcoma is 25.2%, and a poor prognosis is associated with a tumor diameter of 10/10HPF.
2. Fibrosarcoma and smooth muscle sarcoma:Such sarcomas can occur in the trachea and lung parenchyma. Sarcomas of this type in the bronchus are more common in adolescents and may present with symptoms such as cough and hemoptysis. They are usually confined to the bronchus and do not invade the lung parenchyma. The prognosis after resection is good. Since they are prone to cause symptoms, they are often diagnosed and operated on in the early stage. The surrounding lesions are mostly solid, occasionally with cavities, and the prognosis is worse than that of bronchial fibrosarcoma. Some believe that larger tumors have a better prognosis than smaller ones due to their slower growth rate, with a 5-year survival rate of 45%. The factors affecting the prognosis include the mitotic rate of tumor cells, size, and involvement of surrounding tissues (including the chest wall, diaphragm, and mediastinum).
3, pulmonary rhabdomyosarcoma:Rare, the vast majority of rhabdomyosarcomas occupy one or more lobes of the lung, invade local tissues, especially the bronchus and pulmonary veins, and may be accompanied by congenital adenoid malformation. Pulmonary blastoma is also occasionally seen with congenital adenoid malformation, and is more common in children.
4, malignant fibrous histiocytoma:Common in the limbs and retroperitoneum of adults, rare in the lung, and less than fibrosarcoma, etc., with ages ranging from 18 to 80, with an average age of 55. Respiratory symptoms are common, with uniform distribution in all lobes. Treatment: complete resection is chosen, followed by postoperative radiotherapy and chemotherapy. Prognosis: poor, related to staging, completeness of resection, invasion of the chest wall, and metastasis.
5, pulmonary chondrosarcoma:Age ranges from 23 to 73, with an average age of 46, with equal gender distribution. Respiratory symptoms are present, with more cases in the left lung. X-ray chest film shows calcification or ossification, grossly seen as gray sections with a capsule, clear boundaries, and under the microscope, calcification or ossification of malignant chondroma, with poor prognosis.
6, pulmonary osteosarcoma:Rare, defined as extraskeletal osteosarcoma:
(1) The tumor must be composed of a single sarcoma tissue, excluding the possibility of malignant mixed mesenchymal tumor.
(2) The bone-like or bony components must be composed of sarcoma.
(3) Primary bone tumors can be excluded. Patients are between the ages of 35 to 83, with an average age of 61, with equal gender distribution. The most common symptom is chest pain, with equal incidence on both sides. Treatment is surgical resection, with poor prognosis.
7, other sarcomas:Fat, neurogenic sarcoma, and malignant mesenchymal tumors are also rare, with poor prognosis: all the above spindle cell sarcomas, except for smooth muscle sarcoma and bronchial fibrosarcoma, have a very poor prognosis and very few survive more than one year.
Five, sarcomas originating from large blood vessels:Pulmonary artery trunk sarcoma is a tumor originating from the pulmonary artery or heart, and is mainly divided according to the main cell type of the tumor: undifferentiated, smooth muscle, and fibrosarcoma. It invades the lung and spreads to the periphery or outside the blood vessels.
It can occur between the ages of 20 to 81, with an average age of 50, slightly more common in women. Symptoms include chest pain, shortness of breath, cough, hemoptysis, and palpitations. There may be a systolic murmur of the heart, and pulmonary hypertension with proximal vascular dilation is a specific sign. In the late stage, there are signs of right heart decompensation.
X-ray chest film: shows lobulated mass adjacent to the hilum; angiography shows multiple defects in the pulmonary artery; CT and MRI are helpful for diagnosis. Treatment: surgical resection should be performed, and adjuvant therapy can be used after surgery, but the prognosis is poor.
Six, sarcomas originating from small blood vessels, including malignant or low-grade malignant pulmonary tumors of this type are: angiosarcoma, epithelioid angioendothelioma, hemangiopericytoma, and angioendothelioma. Kaposi sarcoma is not discussed here, as it has no primary lung involvement. The 'angioendothelioma' defined by Enzinger and Weiss (1983) is generally considered a vascular tumor, but its accurate histological classification as its final biological behavior cannot be determined.
Angiosarcoma: Extremely rare in the lung, most likely to be a metastatic carcinoma from the pulmonary artery trunk or the right ventricle outside the chest, which may be accompanied by hemothorax or hypertrophic pulmonary osteoarthropathy, with poor prognosis.
Epithelioid angioendothelioma: First reported by Dail and Liebow in 1975, initially named intravascular bronchopulmonary alveolar tumor, later called硬化性内皮瘤(sclerosing angioendothelioma), and finally named epithelioid angioendothelioma. It can be seen in soft tissue, lung, liver, bone, age 4-70 years, 1/3 are under 30 years old, and women are 4 times more than men. Asymptomatic or dry cough, chest X-ray and CT show: many small nodules in both lungs, about 1cm in diameter, with an average survival time of 4.6 years after diagnosis, and there are also reports of survival for 24 years after repeated surgical resection. Most die due to pulmonary insufficiency.
Angioplastic cell tumor: Originating from ubiquitous capillary pericytes, commonly located in the soft tissue of the lower extremities and retroperitoneum, equally common in men and women in the lung, with an average age of 46 years, 1/3 asymptomatic, may have chest pain, hemoptysis, shortness of breath, and cough, occasionally accompanied by pulmonary osteoarthropathy. X-ray chest film shows: lobulated-clear, uniform soft tissue shadow.
Treatment is surgical resection, prognosis varies, depending on chest symptoms, size (over 8cm), invasion of pleura and chest wall, tumor necrosis, and giant cells (>3 mitoses/10 high-power fields), with 33% metastasis in those over 5cm, 66% in those over 10cm, most recurrences within 2 years after diagnosis, and radiotherapy and chemotherapy are ineffective.
7, Carcinosarcoma:Such tumors are mainly composed of epithelial and stromal components, with the epithelial component often being squamous cell carcinoma, and the stromal component often being fibrosarcoma. Compared with a single sarcoma and squamous cell carcinoma, the differentiation degree and prognosis of carcinosarcoma are significantly worse.
It is more common in patients over 50 years old, with a male-to-female ratio of about 5 times, most common in the distal bronchus, the tumor often grows slowly, more intraluminal growth, and less commonly invades the tracheal wall. It can also be seen to invade the surrounding lung tissue, local lymph node metastasis, and distant metastasis, especially brain metastasis is most common. Symptoms include cough, hemoptysis, chest pain and discomfort, and can also have pulmonary osteoarthropathy. Peripheries may be asymptomatic. The characteristics of lung carcinosarcoma are as follows:
1, More common in elderly males.
2, Polypoid mass in the large bronchial lumen.
3, Histology: The malignant epithelial cell clusters can be seen to be composed of a stroma consisting of atypical spindle-shaped or polymorphic sarcoma cells.
4, Immunohistochemistry: cytokeratin antibody shows epithelial component, while the stroma reacts to vimentin (a type of protein).
Should be as surgically removed as possible, with less than 20% one-year survival rate, a few patients can survive for more than 5 years or longer, and there are also those who can live for 10-20 years.
8, Pulmonary blastoma:It is composed of two malignant stromal and epithelial component tumors, whose composition is similar to that of a carcinosarcoma, resembling the lung in the 3-month embryo state, therefore named pulmonary blastoma. In 1952, Bamard first reported the disease under the name of embryonal tumor; in 1961, Spencer named it blastoma.
It is divided according to the histological characteristics:
1, Well-differentiated embryonal adenocarcinoma, resembling the malignant epithelial component of the lung of an embryo, but without malignant stroma.
2, Biphasic mesothelioma, with an average age of 35 years (1-72 years), slightly more women than men, 41% asymptomatic, can cough, hemoptysis, shortness of breath, physical examination is unremarkable, and individual respiratory sounds are weakened.
X-ray chest film: Unilateral lung mass, can be peripheral or central, no special findings in laboratory tests, bronchoscopy and puncture are beneficial for diagnosis, 54% are well-differentiated embryonal adenocarcinoma, 46% are biphasic embryonal tumors, the former in size of 1-10cm (average 4.5cm), the latter 2-27cm (average 10.2cm), histologically malignant glands and adult sarcomatoid or embryonic stroma components.
Treatment is surgical resection, which can be supplemented with chemotherapy, the tumor mass
28. Malignant teratoma:Rare intrapulmonary teratoma, the vast majority of malignant cases occur in the left upper lobe, the cause is unknown, half are malignant, easily confused with embryonal tumors, with poor prognosis.
26. Malignant ependymoma:The cause of the lesion is unknown, it is speculated to be a byproduct of the treatment process of small cell carcinoma, Crotty reported the first case of small cell carcinoma after treatment in 1992, a solitary peripheral ependymoma with ultrastructure and immunological characteristics significantly different from small cell tumors.
24. Bronchogenic malignant melanoma:29 to 80 years old can be affected, with no difference between men and women, most located in the hilum and bronchi, tracheal disease is rare, symptoms, physical examination and radiology are the same as primary bronchogenic carcinoma, diagnosis of primary bronchogenic malignant melanoma, it is necessary to exclude other primary sites first, the reference criteria are as follows:
22. Previous history of skin lesions (especially melanoma) surgery was absent;
21. There was no history of skin lesions (especially melanoma) surgery;
20. There was no history of surgery for eye tumors;
19. The morphology is characteristic of the primary tumor;
18. There was no melanoma in other organs at the time of resection;
17. Autopsy showed no primary melanoma in other organs, and all cases must be differentiated from melanotic carcinoid, with immunology and ultrastructure as the differences between the two.
Once the primary tumor is diagnosed, it should be surgically removed as much as possible, with a moderate prognosis, and it is reported that the longest survival time can be up to 11 years.