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.     

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

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.     

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.     

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.     

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.     

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.     

Radical resection of a pulmonary blastoma involving the pulmonary artery.

Pulmonary blastoma is a rare malignant lesion with a poor prognosis. We described a case of a 47-year-old woman with a large biphasic pulmonary blastoma, involving the left pulmonary artery. Under cardiopulmonary bypass, it was treated with radical left intrapericardial pneumonectomy and pulmonary thromboendarterectomy. Subsequent chemotherapy and radiotherapy was used. Three years postoperatively, the patient was clinically and radiologically free of tumor.     

Prognostic significance of E-cadherin and beta-catenin in resected stage I non-small cell lung cancer.

OBJECTIVES: E-cadherin and its associated intracellular molecules, catenins, are important for cell-cell adhesion. Impaired expression of these molecules are frequently observed in several cancers. E-cadherin and beta-catenin are often expressed in non-small cell lung cancers. The aim of this study was to investigate the expressions of E-cadherin and beta-catenin and their significance as prognostic markers in pathological stage I non-small cell lung cancer. METHODS: Paraffin embedded tumor tissue blocks were obtained from 141 patients who underwent resection without preoperative radiotherapy or chemotherapy with pathological stage I non-small cell lung cancer. Tumor samples were prepared in tissue microarrays and they were stained by immunohistochemistry with antibodies against E-cadherin and beta-catenin. The expressions of E-cadherin and beta-catenin were analyzed with relation to the clinico-pathological data. The median follow-up period of the patients was 41 months (range, 2-88 months). RESULTS: Preserved expressions of E-cadherin and beta-catenin were observed in the membrane and the cytoplasm of normal epithelial cells and tumor cells. Absent or reduced expression for E-cadherin and beta-catenin were observed in 60% and 45% of all the patients, respectively. There was a significant positive correlation between E-cadherin and beta-catenin expression (P<0.01). Absent or reduced expression of E-cadherin was observed in 72.5%, 36.6%, and 60.0% of squamous cell carcinoma, adenocarcinoma, and bronchioloalveolar carcinoma, respectively. There was a significant decrease of E-cadherin expression in squamous cell carcinoma compared to adenocarcinoma (P<0.01). Patients with reduced expression of beta-catenin had poor recurrence free survival in adenocarcinoma, but not in squamous cell carcinoma. CONCLUSION: Decreased expressions of E-cadherin and beta-catenin were closely correlated in resected stage I non-small cell lung cancer. Reduced expression of E-cadherin and beta-catenin indicates tumor cell dedifferentiation and reduced expression of beta-catenin had poor recurrence free survival in adenocarcinoma of the resected stage I non-small cell lung cancer.     

Mixed malignant tumour of the lung.

A mixed malignant tumour of the lung intermediate in type between pulmonary blastoma and carcinosarcoma is described. The epithelial component consisted of squamous carcinoma, undifferentiated carcinoma, and clefts lined by bland epithelial cells. The supporting stroma was composed of pleomorphic sarcoma, fibrosarcoma, chondrosarcoma, osteosarcoma, and indeterminate mesenchymal tissue. The tumour was removed surgically, but the patient died postoperatively with rapidly developing multiple bony and soft tissue metastases. Subcutaneous metastases showed the appearnce of poorly differentiated pleomorphic sarcoma. Published reports of mixed malignant lung tumours are reviewed.     

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.     

A rare case of classic biphasic pulmonary blastoma

Biphasic pulmonary blastomas are rare tumors that together with pulmonary carcinosarcomas comprise less than 2 per cent of all lung neoplasms. They can be defined as tumors that are composed of an admixture of immature mesenchyme and epithelium and that recapitulate morphologically the embryonal structure of the lung. First described in 1945 by Barnett and Barnard, their existence is well documented although their occurrence is rare. We present a case of a 40-year-old Hispanic female that presented with a 2-month history of retrosternal and midepigastric pain. A GI etiology was initially treated unsuccessfully with antireflux medications. A chest radiograph showed a 4.6-cm mass in the right upper lobe of the lung, and computed tomography showed right apical bullous formation with no lymphadenopathy. Bronchoscopy revealed no endobronchial lesions, and biopsy was nondiagnostic. The histopathologic and immunophenotypic analysis of a right upper lobectomy specimen was diagnostic of classic biphasic pulmonary blastoma. The rarity of these tumors makes easy identification difficult. Immunohistochemical analysis must be used to arrive at the proper diagnosis. It is imperative that there be good communication between the surgeon and pathologist and that institutions have access to facilities with the ability of identifying these complex tumors.     

Pulmonary carcinosarcoma with an osteosarcomatous component

Pulmonary carcinosarcoma is a rare disease entity defined as a neoplasm, which has biphasic features consisting of both epithelial and sarcomatous components. It has been reported that the most frequent epithelial component is squamous cell carcinoma, while the most frequent sarcomatous component is rhabdomyosarcoma. Pulmonary carcinosarcomas with osteosarcoma components are even rarer. We report a case of a potentially curative resection for carcinosarcoma with an osteosarcoma component. Thoracic surgeons should be aware of this rare tumor when lung tumors with ossification are encountered.     

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.     

Fetal lung interstitial tumor (FLIT): A proposed newly recognized lung tumor of infancy to be differentiated from cystic pleuropulmonary blastoma and other developmental pulmonary lesions

The differential diagnosis of congenital lung lesions includes a variety of pulmonary malformations, and uncommon or rare neoplasms such as the pleuropulmonary blastoma (PPB) and congenital peribronchial myofibroblastic tumor (CPMT). Although most of the congenital lesions have a predominantly cystic appearance, the exceptions of a more solid process are the type 3 congenital cystic adenomatoid or pulmonary airway malformation (CCAM-CPAM) and the CPMT. The clinical and pathologic features of a unique solid or mixed solid/cystic lung mass composed of immature interstitial mesenchyme in association with irregular airspace-like structures mimicking abnormal incompletely developed lung are presented in this report of 10 infants (7 males, 3 females) whose tumor-like lesions were detected in the prenatal period to 3 months of age (median, 1-day old). A lobectomy was done in all 10 infants and 1 infant received adjuvant chemotherapy. One of the surgical resections occurred as an ex utero, antenatal procedure because of fetal ascites. There have been no reported recurrences in those patients with greater than 12 months of follow-up ranging from 15 to 182 months (9 cases). Because of the morphologic resemblance of this mass-like lesion to fetal lung at 20 to 24 weeks gestation (as though any further pulmonary development was arrested in these localized lesions), we are proposing the designation of fetal lung interstitial tumor (FLIT) whose pathogenetic relationship, if any, to type 1 (cystic) pleuropulmonary blastoma remains uncertain to date.     

Pulmonary blastoma: report of five cases and identification of clinical features suggestive of the disease.

OBJECTIVE: Identification of clinical features suggestive of pulmonary blastoma (PB) through a retrospective comparison with cases of non-small cell lung cancer (NSCLC) operated during the same period. METHODS: Between 1977 and 1999, five patients were operated for PB at Geneva University Hospital (four women and one man, aged 32-46 years--mean 36.8) versus 1913 consecutive patients (1558 men and 355 women, mean age 61.2) for primary NSCLC. In the PB subgroup (0.3%), the pulmonary tumor was single, located in an upper lobe in all but one instance, and measured between 5 and 13 cm (mean 9.6), whereas in the total NSCLC group, 27% of patients had tumors <3 cm (T1), evenly distributed in both lungs. All but one PB patients were symptomatic, compared to 45% in the NSCLC group. RESULTS: The five patients with PB underwent curative pulmonary excisions (lobectomy in three and pneumonectomy in two) with mediastinal lymph node sampling. Pathological examination revealed extensive tumor necrosis in four, and N2 lymph node metastases in four (in the total NSCLC group, N2 disease was diagnosed in 21%). Postoperatively, three PB patients received radio- and/or chemotherapy. Four patients died between six and 30 months after the operation (mean 15), whereas 5-year survival in the NSCLC group was 32%, with a median survival of 3.7 years; the fifth patient is alive 28 months later, without any sign of recurrence. CONCLUSIONS: Compared to operated NSCLC, PB are rare, large, and symptomatic tumors; they affect younger patients and carry a worse prognosis.     

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     

Pulmonary blastoma: medium-term results from a regional center

BACKGROUND: Pulmonary blastomas are rare lung tumors that morphologically resemble fetal pulmonary structure and can exist in two forms, biphasic and monophasic. We reviewed our experience over a 12-year period with emphasis on the clinical features, management, and outcome. METHODS: Patients with a diagnosis of pulmonary blastoma from January 1988 to July 1999 were identified from the database of the Department of Histopathology, Llandough Hospital, Cardiff. Specimens had been obtained from bronchoscopy, fine-needle aspiration, trucut biopsy, and thoracotomy. RESULTS: Six patients were identified from 2,720 histologically proven lung cancers (0.2%). Median age was 35.5 years and sex ratio was equal. Overall, 4 patients underwent resection and are all alive (median, 43.5 months). Three of these had advanced tumors at presentation (stage IIIb or IV), two of which were successfully downstaged with neoadjuvant chemotherapy, and the third treated with postoperative radiotherapy. Nonresected cases succumbed at a median of 5.5 months. CONCLUSIONS: Although pulmonary blastomas are rare, those affected represent a group of patients with advanced tumors for whom a coordinated approach from both oncologists and surgeons can achieve excellent medium-term results.     

Clear cell adenocarcinoma with acomponent of well-differentiated fetal adenocareinoma; report of a case

A 69-year-old woman complaining of a cough was admitted to our hospital. Chest X-ray showed a mass in the right lower lung field. Chest computed tomography (CT) showed a tumor with notch, 3 cm in diameter, in the right lower lobe (S9-S10). The tumor was diagnosed as adenocarcinoma by the biopsy under chest CT. The patient underwent right lower lobectomy (ND2a). The tumor was whitish solid mass, 35 x 34 x 29 mm in size. Histopathologically, the tumor was diagnosed as clear cell adenocarcinoma with a component of well-differentiated fetal adenocarcinoma (WDFA), pT2N0M0, stage IB. The patient was discharged and received postoperative chemotherapy (UFT). The patient has been doing well without any tumor recurrence for 1 year postoperatively.