A case of pulmonary blastoma]

A 45-year-old male with pulmonary blastoma was described. The patient suffered from headache and gait disturbance, and was diagnosed metastatic brain tumor. The histopathology of the removed tumor indicated metastasis from pulmonary blastoma. The pulmonary tumor, which had been detected on the chest x-ray film but misdiagnosed tuberculous lesion, was subsequently diagnosed pulmonary blastoma by TBLB. The patient underwent left upper lobectomy with mediastinal lymph node dissection, but died 12 months after the operation. To our knowledge, only 54 cases with pulmonary blastoma have hitherto been reported in the Japanese literature.     

Six cases of well-differentiated adenocarcinoma simulating fetal lung tubules in pseudoglandular stage. Comparison with pulmonary blastoma

Six cases of well-differentiated adenocarcinoma resembling fetal lung were studied histologically, immunohistochemically, and ultrastructurally, and compared with three cases of pulmonary blastoma. The six cases had no sarcomatous features, unlike the pulmonary blastoma. Individual tumor cells characteristically possessed clear cytoplasm with plentiful glycogen and were similar to the epithelial cells of the branching tubules in the pseudoglandular stage of the fetal lung, as well as the epithelial component of pulmonary blastoma. They also showed little differentiation toward mucous cells and ciliated cells. Although cells containing endocrine-type granules were not found ultrastructurally, a few tumor cells possessed the characteristics of endocrine cells, i.e., cytoplasm was immunohistochemically reactive with anticalcitonin and antigastrin-releasing peptide. Therefore, this type of adenocarcinoma is considered to have a histogenesis similar to that of pulmonary blastoma and may be a tumor with one-sided development of pulmonary blastoma showing only an epithelial component.     

Pulmonary blastoma

We describe a resected pulmonary blastoma in an 84-year-old male, the oldest of previously reported patients. A chest X-ray showed a 2 cm-sized abnormal shadow in the left lung field. Five months later computed tomography demonstrated a well-demarcated heterogenous mass, measuring 12 cm in diameter, in the left lower lobe of the lung. This mass was diagnosed as a carcinoma using echo-guided percutaneous biopsy. The patient underwent a left lower lobectomy. The resected specimen revealed the tumor to be a pulmonary blastoma arising from lung tissue. Preoperative diagnosis of pulmonary blastoma is extremely difficult because of the histological heterogeneity of tumor. Since it has been noted that pulmonary blastoma rapidly progresses in a short period of time, surgical treatment should be undertaken as quickly as possible when such a tumor is suspected.     

Pulmonary blastoma

We describe a resected pulmonary blastoma in an 84-year-old male, the oldest of previously reported patients. A chest X-ray showed a 2 cm-sized abnormal shadow in the left lung field. Five months later computed tomography demonstrated a well-demarcated heterogenous mass, measuring 12 cm in diameter, in the left lower lobe of the lung. This mass was diagnosed as a carcinoma using echo-guided percutaneous biopsy. The patient underwent a left lower lobectomy. The resected specimen revealed the tumor to be a pulmonary blastoma arising from lung tissue. Preoperative diagnosis of pulmonary blastoma is extremely difficult because of the histological heterogeneity of tumor. Since it has been noted that pulmonary blastoma rapidly progresses in a short period of time, surgical treatment should be undertaken as quickly as possible when such a tumor is suspected.     

Resected well-differentiated fetal pulmonary adenocarcinoma and summary of 25 cases reported in Japan

We report a case of well-differentiated fetal adenocarcinoma (WDFA), which is a variant of pulmonary blastoma. A 36-year-old man was found to have a tumor shadow in the right middle field of a chest radiograph as part of a mass screening examination, and chest computed tomography (CT) showed a 4.5-cm pulmonary mass in the right lower lobe. A diagnosis of adenocarcinoma of the lung was made based on a CT-guided needle biopsy, and right middle and lower lobectomy and lymph node dissection were performed. The postoperative pathological diagnosis was well-differentiated fetal adenocarcinoma. WDFA has a better prognosis than conventional pulmonary blastoma (biphasic pulmonary blastoma). We summarize the cases of WDFA reported in Japan and review the literature.     

Pulmonary adenocarcinoma of the fetal lung type; report of a case

An asynptomatic 58-year-old male was admitted to the hospital because of an abnormal nodule in the left lung field on screening chest X-ray. Chest computed tomography (CT) showed a tumor shadow mass in the left lower lobe. Open biopsy was performed to diagnose the mass. The cytological diagnosis was low-grade malignant adenocarcinoma, underwent left lower lobectomy. The histological diagnosis was pulmonary adenocarcinoma of the fetal lung type which was one of pulmonary blastoma (PB). Recently, the concept of pulmonary blastoma has changed. It will be useful to investigate the old PB's case reports to classify new concept.     

Pleuropulmonary blastoma in a 12-year-old boy presenting with pneumothorax

Pleuropulmonary blastoma is a rare pulmonary neoplasm seen in the pediatric population. We report a purely cystic-type pleuropulmonary blastoma in a 12-year-old boy who presented with spontaneous pneumothorax. He underwent partial resection of the left lower lobe under video-assisted thoracoscopy.     

Pulmonary blastoma in an adult presenting as a chronic loculated effusion: a diagnostic problem.

A 33 year old woman posed a diagnostic problem for two years with recurrent left sided chest pain and radiographic features suggestive of a loculated effusion. Diagnosis was finally made at thoracotomy which revealed an encapsulated cystic mass. Histological examination confirmed a rare monomorphic pulmonary blastoma.     

Pulmonary blastoma

Pulmonary blastoma is a rare primary malignant lung neoplasm, originating from multipotential pulmonary cells composed of immature mesenchyme and epithelium, which is morphologically similar to fetal lung tissue. It represents 0.25% to 0.5% of all primary lung tumors. There are no specific clinical features that differentiate this tumor from common lung neoplasm. We are reporting a case of pulmonary blastoma in a 70 years old male, cigarette smoker. He had initial diagnosis of hydatid cyst for which thoracotomy was done. Operative findings revealed a mass lesion in right lung involving oblique fissure. Biopsy showed malignant neoplasm with predominant sarcomatous (rhabdoid) component suggestive of pulmonary blastoma of biphasic type.     

Pulmonary blastoma with rhabdomyosarcomatous differentiation: an electron microscopic and immunohistochemical study

Pulmonary blastoma is a rare lung tumor composed of epithelial and mesenchymal elements; the latter element may show various patterns of differentiation toward mature tissue, such as cartilage, smooth muscle, and bone. Rhabdomyoblastic differentiation in pulmonary blastoma is quite rare; only five such cases have been reported. We report two cases of pulmonary blastoma with rhabdomyoblastic differentiation documented for the first time by electron microscopy and immunohistochemistry including documentation for myoglobin, actin, vimentin and desmin. The diffuse and prominent rhabdomyoblastic differentiation in one case is most unusual.     

Biphasic blastoma of the lung. Report of 4 consecutive cases and evaluation of long-term prognosis

Pulmonary blastoma is a rare malignant disease and it can occur in adults and in children. In 1952, Barnard reported the first case classified as pulmonary embryoma while in 1961 Spencer reported the first case as pulmonary blastoma. Since then 200 cases were described in literature. Four patients with adult primary pulmonary blastoma were treated in our Institute. The tumor was right sided in all cases; it belonged to upper lobe in 1 patient, to middle in 1 and to lower in 2. The patients underwent middle lobectomy in 1 case, lower lobectomy in 2 and upper lobe typical segmentectomy in one (the subject previously operated for lung adenocarcinoma). Histology detected primary pulmonary biphasic blastoma in all the cases. In only one case it was associated with hilar lymph nodal metastases. She received adjuvant chemotherapy, but after 17 months she developed distant metastases and she died 22 months after operation. About the other 3 patients: one patient died 6 months after intervention for acute cardiac disease, while two subjects are still alive and disease free 158 and 70 months after surgery. Surgical resection, when radicality could be ensured, is considered the treatment of choice, in absence of other curative therapies. The analysis of our experience confirms surgery to be a good therapeutic choice permitting to obtain long term survivals. The patient, alive ten years after the operation, is one of the longest survival case for pulmonary blastoma reported in literature.     

Pulmonary blastoma: a case report

Rao, K. M., Gupta, R. P., Das, P. B., John, S., and Walter, A. (1974).Thorax, 29, 138-141. Pulmonary blastoma. A case report of a pulmonary blastoma is presented. A tumour was suspected at surgery and on histological examination this proved to be a pulmonary blastoma. A short review of the histogenesis and methods of treatment is given.     

Biphasic pulmonary blastoma with rapid progression

We report a rare case of biphasic pulmonary blastoma (BPB) with rapid progression in a 75-year-old man. Computed tomography (CT) of the chest revealed a well-defined tumor shadow measuring 8 × 6 cm in the right upper lobe. CT-guided lung biopsy revealed malignant cells with sarcomatous elements. Right upper lobectomy and systemic lymph node dissection with chest wall resection were performed. Histopathologically, the tumor was composed of immature embryoniclike mesenchymal and epithelial components that resembled embryonic lung tissue. The final diagnosis was BPB classified as pathological stage IIB (T3N0M0). Two months later, the tumor recurred in the right supraclavicular lymph nodes. The patient died of respiratory failure due to tumor progression 7 months after surgery.     

Biphasic pulmonary blastoma: report of a case.

A 73-year-old male presented with bloody sputa for a month. Chest computed tomography (CT) showed a large mass about 7 cm in diameter in the right lower lobe. Bronchoscopic curette cytology revealed class V and a suspected adenocarcinoma, although a systemic evaluation demonstrated no metastatic lesion. The patient underwent a right lower lobectomy and mediastinal dissection. A biphasic pulmonary blastoma was histologically diagnosed by a characteristic finding that it was mainly constituted of immature tumor tissue that had both epithelial and mesenchymal components. No mediastinal lymph node metastasis was proven. Stage T2N0M0 disease was diagnosed, and the patient chose not to undergo postoperative adjuvant chemotherapy; he remains well without recurrence 36 months after the operation.     

Biphagic pulmonary blastoma with a lesion of yolk sac tumor

Biphagic pulmonary blastoma (BPB) is one of the rare pulmonary malignant neoplasms. Here, we describe a case of BPB with a histological feature of yolk sac tumor, accompanied with an elevated serum α-fetoprotein level (531.1 ng/ml). The tumor was detected in the right upper lobe as a 10-cm mass. The patient underwent right upper lobectomy and mediastinal lymph node dissection. Histological examination revealed that the tumor was composed of immature mesenchymal and epithelial elements, and it was accompanied by foci of yolk sac tumor-like features that showed positive staining for α-fetoprotein antibody. Adjuvant chemotherapy consisted of cisplatin and etoposide. After surgery, the serum level of α-fetoprotein decreased below normal. Local recurrence was disclosed 27 months after surgery, and radiation therapy was performed. The patient is currently alive 70 months after surgery.     

Pulmonary blastoma.

Pulmonary blastoma is now accepted as a distinctive neoplasm. It remains rare, and only 28 cases have been reliably recorded. A further two cases are now reported, and the previous literature is reviewed. There are no specific clinical or radiological features of pulmonary blastoma. The presentation can be that of any other pulmonary tumour although a peripheral situation is usual and a large size is often attained before detection. Pulmonary blastoma is a mixed tumour with malignant epithelial and connective tissue components with a distinctive resemblance to fetal lung. The treatment of choice is surgical excision but the overall prognosis is poor. It is doubtful whether the tumour has a true blastomatous origin.     

A left pulmonary artery aneurysm secondary to pulmonary hypertension

We report a case of pulmonary trunk aneurysm extending into the left pulmonary artery, due to pulmonary hypertension secondary to mitral valve disease. The mitral valve was replaced with a bileaflet mechanical prosthesis. A Dacron graft interposed between main trunk and left pulmonary artery branch, and right pulmonary branch attached to the graft in an end-to-side fashion. Early postoperative angiogram revealed a very successful treatment.     

Mucinous adenocarcinoma of the lung in association with congenital pulmonary airway malformation

Congenital pulmonary airway malformation (CPAM) is a rare developmental abnormality of the lung that has been associated with the presence of rhabdomyosarcoma, pleuropulmonary blastoma, and most commonly bronchioalveolar carcinoma (BAC) of the lung. Here, we report the case of an 8-year-old patient who developed KRAS mutation positive stage IV mucinous adenocarcinoma of the lung in association with CPAM. This case reflects the previously recognized progression of CPAM to malignancy and suggests that BAC arising in CPAM may take a more aggressive course than previously recognized.     

Pulmonary Blastoma: Report of Two Cases

Pulmonary blastomas are a group of rare malignant neoplasms subdivided into three categories: classic biphasic pulmonary blastoma (CBPB), well-differentiated fetal adenocarcinoma (WDFA), and pleuropulmonary blastoma (PPB). We report herein the cases of two men with CBPB. Both were heavy smokers and presented with a history of hemoptysis. Physical examination revealed slightly significant findings, chest radiographs showed a large pulmonary mass, confirmed by computed tomography, and bronchoscopic biopsies were not diagnostic. A left and right inferior lobectomy was performed and a diagnosis of CPBP was confirmed by histological examination. In the first patient, local recurrence with multiple bilateral lung metastases was found 6 months later and despite chemotherapy, he died of respiratory failure 1 year after his operation. In the second patient, a subcutaneous metastasis was found in the right subscapular region 2 months later, and a cerebral metastasis in the right posterior parietal lobe 4 months later. Partial remission was achieved by cerebral irradiation, but 6 months later the patient died of cardiac failure while in a coma. We conclude that more aggressive and multidisciplinary treatment should be adopted for CBPB, and because of its low incidence, it is important to unify individual experiences in a central registry to gather as much information as possible regarding the biological and clinical features of this unusual disease.     

Pulmonary blastoma in a child: report of a case

We describe herein the case of a 2-year-old girl found to have a pulmonary blastoma (PB). The child was admitted to our hospital with the chief complaints of coughing and left-sided chest pain. On admission, a chest X-ray revealed a large mass in the left lung, which measured 10 cm in diameter. Computed tomography and magnetic resonance imaging showed a marginally and heterogeneously enhanced tumor filling the left hemithorax. Pathologic findings of the fine-needle aspiration were suggestive of neuroblastoma. Subsequently, a left pneumonectomy with lymph node dissection was performed and histopathological examination confirmed that the tumor was a PB (type III). After the definitive diagnosis was made the patient received combination chemotherapy, and no evidence of recurrence has been seen in the 5 months since surgery. Received: September 4, 2000 / Accepted: March 6, 2001