What Is Pleuropulmonary Blastoma?

Pulmonary blastoma is a disease name, also known as lung germ tumor, which is a very rare tumor.

Pulmonary blastoma

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Pulmonary blastoma is a disease name, also known as lung germ tumor, which is a very rare tumor.

Western Medicine Name

Pulmonary blastoma

Other name

Pulmonary germ tumor

Affiliated Department

Internal Medicine-Oncology

Contagious

Non-contagious

Dr AdluriRK and colleagues at the Nottingham City Hospital in the UK reported seven patients with pulmonary blastoma of various clinical characteristics. A retrospective analysis of the hospital's database revealed that a total of 7 patients with pulmonary blastoma received surgical treatment between January 1993 and December 2004. In the same time period, a total of 889 patients with primary NSCLC underwent pneumonectomy. Get demographic and clinical details from hospital records. The Department of Pathology reviewed the histopathology report and recorded radiological images. The results showed that the age of onset of the tumor was bimodal (4 patients were younger than 49 years old and 3 patients were older than 66 years old). All patients had symptoms and tumors of varying sizes. Lesions are common in the lower lobe (lower lobe: other lung lobe 4: 3), and the pathological stage is T2N0. All patients underwent surgical resection without chemotherapy / radiation after surgery. Three of the seven patients died during the follow-up period. The cause of death was not related to the tumor, and the death time was between 24 and 29 months. The longest follow-up period was more than 9 years, and the surviving patients were in good condition. Dr. Adluri et al. Concluded that pulmonary blastoma is a rare tumor with diverse clinical features. Early detection and treatment may improve its prognosis. Further studies are needed to assess the characteristics of this tumor.

1) Well-differentiated fetal adenocarcinoma (WDFA): age of onset: minimum age of WDFA is 12 years; 8% of patients with pneumoblastoma are younger than 10 years, with an average age of 14 years; incidence: generally considered lung Cell tumors are more common than WDFA; Prognosis: Good prognosis, 5-year survival rate exceeds 90%; and 5-year survival rate of pulmonary blastoma is 10% -25%; P53: WDFA has no mutation; and pulmonary blastoma often has P53 mutation; Tissue structure: WDFA epithelial component is malignant and mesenchymal component is benign; while lung cell tumor, mesenchymal component is sarcoma. WDFA is common in Morinda spp., And pneumoblasts are occasionally or absent.

2) Thoracic blastoma: Thoracic blastoma has a biphasic structure under the microscope and often occurs in the lungs, so it is similar to pulmonary blastoma.

Identification includes:

According to the histological classification of WHO (1999), pneumoblastoma belongs to the category of malignant epithelial tumors of the lung; pneumoblastoma belongs to soft tissue tumors;

Conceptual difference: Pulmonary blastoma, a biphasic tumor, contains proepithelial components, similar to well-differentiated fetal adenocarcinoma; its original mesenchymal matrix may contain osteosarcoma, osteosarcoma or rhabdomyosarcoma. On the other hand, thoracoblastoma is a cystic and / or solid sarcoma. The cyst wall is covered with benign, metaplastic ciliated epithelium. Obviously, pneumoblastoma belongs to the specific carcinosarcoma, and pneumoblastoma belongs to the pulmonary sarcoma, whose epithelial components are metaplastic and benign cystic wall;

Age of onset: Pulmonary blastoma is more common in adults who smoke, and the average age of onset is 43 years; Pulmonary blastoma is more common in infants 1-4 years old, 90% of whom are under 10 years of age;

Occurrence site: Pulmonary blastoma, which occurs in the lungs; In addition to thoracoblastoma, which can occur in the lungs, it can also occur in extrapulmonary tissues such as the pleura and mediastinum;

Histological characteristics: Pulmonary blastomas contain primitive epithelial components and morphological characteristics of endometrial glands; their primitive mesosarcoma can include focal chondrosarcoma, osteosarcoma, and rhabdomyosarcoma. And pneumoblastoma is more similar to the structure of grape rhabdomyosarcoma, especially cystic type;

Gross manifestations: Pulmonary blastoma is a solid mass; Pulmonary blastoma can be cystic or solid. The specimens submitted for resection are more cystic than solid;

Immunohistochemical expression: The two sarcoma regions have different levels of expression of vimentin, binding protein, myoglobin, and S-100 protein. However, thoracoblastoma also positively expressed histiocytic antigen; while pneumoblastoma also positively expressed a few cells of CK. In the epithelial area, the positive expression rate of 4CgA and NSE in pulmonary blastoma is high, and a small amount of calcitonin is also positively expressed. But thoracoblastoma is only a small number of tumor cells that positively express NSE in the sarcoma area;

Prognosis: Thoracic lung tumors have a better prognosis than pulmonary tumors.

Lung sarcoma: Both lung blastoma and lung sarcoma are biphasic lung malignancies, but they are also different. Age of onset: The peak age of lung cancer sarcoma is 6080 years old, 15% are under 50 years old; the average age of lung blastoma is 43 years old, and 20% are under 20 years old; Gender: lung cancer sarcoma, more common in males, male to female ratio is 5: 1-11 = 1; Pneumoblastoma is slightly more common in women, and the ratio of male to female is 1: 1-1: 2; Histological characteristics: Non-small cell carcinoma and heterologous sarcoma are seen under the microscope of lung cancer sarcoma. Non-small cell cancers are poorly differentiated cancers, and most of them are mixed; sarcomas are mostly degenerative rhabdomyosarcomas and osteosarcomas. In the case of pulmonary blastocytes, the epithelial part is highly differentiated fetal adenocarcinoma, showing unity; sarcoma can be differentiated or anaplastic rhabdomyosarcoma, osteosarcoma, or fibrosarcoma; prognosis:

Lung cancer sarcoma has a poor prognosis. The average survival time for lung cancer sarcoma was 9 months, the 2-year survival rate was 30%, and the 5-year survival rate was 16%. In addition, pulmonary blastoma should also be distinguished from endometrial cancer with malignant teratoma and metastatic nephroblast of the mediastinum.

Case 1 female, 50 years old, was admitted to the hospital on October 2, 1996 due to "a physical examination revealed a shadow of the upper right lung for 20 days". There was no cough, chest pain or fever before admission. CT examination showed that the density of the right upper lung was about 4 cm in diameter and uniformly increased, with lobes and clear edges. A thoracotomy was performed for the right upper lung tumor. During the operation, a 4cm × 3.5cm × 3cm mass in the anterior segment of the right upper lung lobe was seen, with a umbilical concave sign on the surface, and a lymph node in the right hilar, about 2 cm in diameter. Right upper lobe resection + hilar lymph node dissection. Postoperative pathology: The tumor section was gray-white. Microscopically, the tumor was composed of fibrous histiocyte-like cells (immunohistochemical Vinentin (++)), interstitial and naive alveolar epithelium, and adenoid-like structures (both EMA and CK (++) ), The tumor is clearly demarcated from the surrounding lung tissue, there is a large amount of cholesterol crystals in the center and atheromatous, hilar lymph nodes have no cancer metastasis. Pathological diagnosis: Pulmonary blastoma, postoperative chemotherapy with cyclophosphamide, vincinine, doxorubicin, and dysomycin regimen for 2 cycles. There was no recurrence during 2 years of follow-up after discharge. Case 2 male, 34 years old, was admitted to the hospital on January 6, 1998 because of "recurrent cough with left chest pain for more than 2 months". The patient had mild shortness of breath, no sputum and fever. Examination: The general condition is acceptable, the superficial lymph nodes are not enlarged, the left upper lung is cloudy, and the breathing sounds are weak. The chest radiograph showed an increase in the density of the upper left lung, a lumpy shadow, lobes, and incomplete edges. Fiberoptic microscopy was normal. The left upper lung cancer was explored on January 16th. During the operation, a tumor of about 10cm × 9cm × 8cm was found in the left upper lung lobe. A lymph node is about 4cm × 3cm. Left upper lobectomy and hilar lymph node removal were performed. Pathological examination: Microscopic tumors contain spindle cells, interstitial and naive alveolar epithelium, and chondrosarcoma can be seen in some areas. Immunohistochemistry CK, EMA can identify various components. Pathological diagnosis: pulmonary blastoma, hilar lymph node metastasis. Three cycles of local radiotherapy and CAP chemotherapy were performed. It has been 9 months after operation, and no recurrence was seen during follow-up. Discuss pneumoblastoma is rare. Its clinical manifestations are also not characteristic. Preoperative diagnosis is difficult, and the main diagnosis depends on surgery + pathological examination. Surgical resection is the main treatment, and the effect of radiotherapy and chemotherapy is difficult to be confirmed because of insufficient data. In terms of prognosis, Shu Yusheng et al. (1) collected 115 patients reported in the literature at home and abroad, of which 108 cases were surgically resected and 26 died within 1 year. The 2 and 5 year survival rates were 58% and 39%, respectively. After surgery, radiotherapy and chemotherapy in 2 cases admitted in our hospital, no recurrence has been observed so far, suggesting that as long as the pulmonary blastoma can be diagnosed in time, the treatment effect is still relatively good.