Biphasic Pulmonary Blastoma Associated with Cerebral Metastasis.

Pulmonary blastoma is a very rare malignant tumor of the lungs. A biphasic pulmonary blastoma was histologically diagnosed by a characteristic finding as it was mainly constituted of immature tumor tissue that had both epithelial and mesenchymal components. We present a case of a 68-year-old man with biphasic pulmonary blastoma. The patient underwent cranial metastatectomy and left lung upper lobectomy. Although the tumor was resected, there was rapid metastasis to the cranial, liver, kidney and multiple bones. Although radiotherapy and chemotherapy were administrated, the patient died about 6 months postoperatively. Close follow-up and aggressive chemotherapy should be considered for such tumours. In the light of this case, the authors review the pathologic, clinical, radiological and therapeutic features of this very rare malignant lung tumor.

Characteristics and Prognostic Analysis of 69 Patients With Pulmonary Sarcomatoid Carcinoma.

BACKGROUND: Pulmonary sarcomatoid carcinoma (PSC) is a rare malignancy. METHODS: A total of 69 patients with PSC treated at a single institution in southern China with long-term follow-up were evaluated in this study. We analyzed the clinical characteristics, immunohistochemical profiles, epidermal growth factor receptor mutation status, K-RAS mutation status, treatments, and prognosis. RESULTS: PSC mainly occurred in young male patients with a history of smoking. Most patients received multimodality treatments and the majority had early-stage disease. The median survival time was 19.1 months, and the 5-year survival rate was 17.4%. The patients without distant metastasis, with normal or higher body mass index (≥18.5), with normal hemoglobin, with smaller tumor size (≤4 cm), and those who received complete resection had significantly better overall survival (P<0.05). The patients with pleomorphic carcinoma had much worse prognosis. In a Cox regression model, M stage, pathology, and having received a complete resection were independent prognostic factors (P<0.05). CONCLUSIONS: PSC is a unique lung malignancy with poor prognosis. Patients receiving complete resection had better prognosis, likely a reflection of early-stage disease. Neither neoadjuvant nor adjuvant chemotherapy improved patient survival for those with early-stage disease. The retrospective design and small sample size limited the generalizability. Future multicenter collaborations may be necessary to determine the optimal treatment.

Classic biphasic pulmonary blastoma with brain and axillary metastases: a case report with molecular analysis and review of literature.

Pulmonary blastoma is a rare series of malignant lung tumor, which contains three categories: classic biphasic pulmonary blastoma, pluropulmonary blastoma, and well-differentiated fetal adenocarcinoma. In this study, a 19-year old female suffering with classic biphasic pulmonary blastoma and metastases in brain and axilla was presented with special interest in clinicopathological presentations, immunohistochemical features, and molecular characterizations. However, this case was misdiagnosed initially with small biopsy specimen. Comprehensive management should be used for the treatment of this malignancy.

Surgery and radiation therapy for brain metastases from classic biphasic pulmonary blastoma.

Pulmonary blastoma, a rare malignant lung tumour, can metastasise to the brain. However, there is no evidence for any effective treatment. The aim of this report is to discuss the treatment options for pulmonary blastoma and confirm the necessity for a pathological diagnosis. A 75-year-old man was admitted with progressive right-sided hemiplegia and aphasia. MRI showed multiple brain tumours. A left frontal lobe lesion was surgically resected, after which he underwent whole brain radiation (30 Gy/10 fractions). He died of an acute exacerbation of interstitial pneumonia. On performing autopsy, partial responses in the brain metastases that had been irradiated were confirmed pathologically. Thus, we present pathological confirmation that surgery and radiation therapy have therapeutic effects on brain metastases from pulmonary blastoma.

The assessment of primitive or metastatic malignant pulmonary tumors in children.

BACKGROUND: The purpose of our study is to assess primitive and secondary malignant pulmonary tumors in children. The presence of lung tumors in newborns and infants is a point of interest to specialists in pediatric surgery, thoracic surgery and genetics due to the high death rate. The 5-years survival rate communicated by EUROCARE-study is less than 10% for primitive tumors and less than 15% in lung metastases. MATERIALS AND METHOD: We performed a retrospective study which analysed 11 children with pulmonary primary ormetastatic tumors admitted in the Pediatric Surgery Department "Prof. Dr. Al. Pesamosca" of the Emergency Clinical Hospital for Children "Maria Sklodowska Curie",Bucharest. The analysed and operated patients underwent surgery by Prof. Dr. Al. Pesamosca and the authors during the period of 1985-2011. In our series there where 4 primitive lung tumors and 7 secondary ones: 8 underwent surgery and 2 died before being operated on. The incidence of primitive pulmonary lung malignancies is higher for females, 3 to1, and secondary ones are more frequent in males, 6 to 1. RESULTS: Patients with primitive pulmonary malignancies were late diagnosed. Their age ranged between 1 to 6 years;3 were operated on, out of which 2 died, and 1 operated still survives. The 7 patients with secondary pulmonary malignancies were late diagnosed, too, probably as a consequence of a late diagnosis of the origin tumor. CONCLUSIONS: Even if all malignancies require an early diagnosis and treatment, this aim regarding malignant lung tumors is still a desideratum animating all practitioners. Primitive tumors are diagnosed presenting the main clinical manifestation abroncho pulmonary infection. Secondary lung malignancies are usually asymptomatic and are diagnosed when monitoring a patient for a malignancy with another origin. Chemotherapy,radiotherapy and surgery of malignant primitive tumors or metastatic ones in children remain unsatisfactory because of the late diagnosis and the limited methods of treatment. Nowadays genetics identified the responsible oncogenes for pulmonary blastic explosion and better results could be obtained by genetic surgery.

Pulmonary blastoma with submandibular, scrotum and adrenal metastases: case report.

A 62-year-old patient diagnosed with pulmonary blastoma with submandibular, scrotum and adrenal metastases was admitted to Sotiria General Hospital in Athens. No other such case has been published to date. The patient started receiving chemotherapy, but the scrotum metastasis grew rapidly and erupted. This led to sepsis despite surgical excision of infected and necrotic tissues and intravenous antibiotics. Treatment strategy in pulmonary blastoma should be defined by a multidisciplinary team, and surgical treatment should be considered as quickly as possible when such a tumor is suspected.

High therapeutic effectiveness of postoperative irinotecan chemotherapy in a typical case of radiographically and pathologically diagnosed pleuropulmonary blastoma.

A 2-year 9-month-old girl with a large mass in the right chest underwent middle and inferior lobectomy, after which the mass was pathologically diagnosed as a pleuropulmonary blastoma (PPB). The clinical, radiographic, and pathologic findings were typical. Three courses of postoperative chemotherapy with 2 different regimens were ineffective in preventing multiple metastases of the lung. We then instituted a protocol with irinotecan (CPT-11) and vincristine. After the third course of this protocol the metastases disappeared and there were no recurrences. PPB is a rare pediatric malignant tumor, and no adequate therapy has been defined. This is the only case reported to have been treated with vincristine/irinotecan therapy for the treatment of PPB.

Pulmonary blastoma metastatic to the ovary.

Pulmonary blastoma (PB) is a rare and aggressive pulmonary malignancy. A 38-year-old woman was diagnosed as biphasic PB (1 subgroup of sarcomatoid carcinoma) and treated by surgery, radiotherapy, and chemotherapy. Six months later, the patient presented with menstrual disorder and a mass in the abdominal-pelvic cavity. Abdominal hysterectomy and bilateral salpingo-oophorectomy showed the morphology of metastatic PB in both ovaries. The metastatic tumor was composed exclusively of epithelial components resembling well-differentiated fetal adenocarcinoma. To our knowledge, this is the first reported case of bilateral ovarian metastasis from biphasic PB.

Pulmonary blastoma with renal metastasis responds to sorafenib.

Biphasic pulmonary blastoma is a rare malignancy of the lung composed of proliferating epithelial and mesenchymal cells with a high vessel density at the tumor periphery. We report a rare case of renal metastasis of biphasic pulmonary blastoma that responded to sorafenib, an oral multikinase inhibitor. After 2 months of treatment with sorafenib, the renal tumor size decreased sufficiently to allow a safe laparoscopic radical nephrectomy. We believe that further studies are warranted to confirm the possible effects of sorafenib on pulmonary blastomas.

Cerebral metastasis and other central nervous system complications of pleuropulmonary blastoma.

BACKGROUND: Pleuropulmonary blastoma (PPB) is a rare tumor of pleura and lung in young children. Central nervous system (CNS) complications, particularly cerebral parenchymal metastases, occur in aggressive forms of PPB: Types II and III PPB. This article evaluates cerebral and meningeal metastases, cerebrovascular events (CVA) caused by tumor emboli, spinal cord complications, and intracranial second malignancies in PPB. PROCEDURE: International PPB Registry and literature cases were evaluated for CNS events. Cerebral metastasis patients were evaluated for gender, side of origin of PPB, PPB Type, interval from diagnosis to metastasis, status of chest disease, treatment, and outcome. Standard statistical methods were used to calculate the cumulative probability of cerebral metastasis and survival following metastasis. RESULTS: Thirty-nine cases of cerebral metastasis were identified in 5/53 Registry Type II cases, 15/44 Registry Type III cases, and 19/143 literature Type II/III cases. Metastases occurred 1-60, median 11.5 months after diagnosis. Chest disease was controlled in 50% of children at time of metastasis. The cumulative probability of cerebral metastasis by 5 years from diagnosis was 11% for Type II patients (95%CI (confidence interval): 2-20%) and 54% for Type III patients (95%CI: 31-76%). Seven children survive cerebral metastasis. Other CNS complications were post-operative CVA (five cases), spinal cord invasion or compression (six), leptomeningeal disease (three), and second intracranial malignancies (two). CONCLUSIONS: Cerebral metastasis is more frequent in PPB than in other childhood sarcomas. Clinicians should screen for this complication. Diverse other CNS complications are less common and require careful diagnosis.

Combined small cell carcinoma with pulmonary blastoma and adenocarcinoma: case report and clonality analysis.

We describe a rare tumor occurring in the left pulmonary lobe of a 71-year-old Japanese man. The tumor, which was resected by left lower lobectomy, measured 65 x 50 x 50 mm. Histologic examination revealed papillary adenocarcinoma in small cell carcinoma, and chondrosarcoma. Also, the blastemal cells were located between the small cell carcinoma and the chondrosarcoma, and intermingled with both components. In blastemal cells, some glands resembled a well-differentiated fetal adenocarcinoma. The tumor was diagnosed as combined small cell carcinoma with pulmonary blastoma and papillary adenocarcinoma according to the 2004 WHO classification. Immunohistochemically, the small cell carcinoma expressed TTF-1, pancytokeratin, CD56, synaptophysin, and S100 protein, while blastemal cells expressed vimentin, desmin, smooth muscle actin, CD56, and S100 protein. To investigate whether the tumor was clonal or not, p53 gene mutation of each tumor component was analyzed by laser-captured microdissection, polymerase chain reaction-single-strand conformation polymorphism and direct sequencing. Despite the histologic complexity, all components showed the same mutation at exon5 of the p53 gene. These results indicate that the tumor was clonal and arose from a relatively primitive cell, and that p53 mutation occurred before histologic metamorphosis or differentiation.

Solitary brain metastasis from classic biphasic pulmonary blastoma: a case report and review of the literature.

BACKGROUND: Classic biphasic pulmonary blastoma (CBPB) is a rare and aggressive primary malignancy, brain metastases of this type of tumor are even rarer. CASE REPORT: A 51-year-old male patient with a solitary cerebral metastasis, diagnosed ten months after left pneumonectomy for a CBPB, was treated by surgery and accelerated hypofractionated radiotherapy. RESULTS: The patient died 15 months after partial removal of the brain metastasis. Literature review revealed only 4 cases of solitary brain metastases from this type of malignancy. The present case is the second one reported with a combined treatment of surgery and radiotherapy resulting in the longest survival. CONCLUSION: The best treatment for CBPB is difficult to determine because of the small number of cases, however, the combination of surgery with radiotherapy seems to be effective. The effectiveness of chemotherapy has not been ascertained.

Imaging findings in pleuropulmonary blastoma.

BACKGROUND: Pleuropulmonary blastoma (PPB) is a rare tumor of the chest seen in young children and recently recognized as distinct from the pulmonary blastoma typically encountered in adults. OBJECTIVE: The purpose of this study is to review and describe the findings of PPB on radiography and CT in four patients. METHODS: Radiographs and CT findings were reviewed in four patients with pathologically proven PPB. RESULTS: All four cases demonstrated large masses in the right hemithorax with heterogeneous low attenuation, pleural effusion, contralateral mediastinal shift, and lack of chest wall invasion. CONCLUSION: When a large pleural-based mass is identified in a young child, PPB should be considered. Suggestive findings include absence of chest wall invasion, presence of pleural fluid, right-sided location, and heterogeneous low attenuation.

Tumor recurrence at thoracostomy tube insertion sites: a report of two pediatric cases.

Recurrence of thoracic malignant tumors at port sites used for thoracoscopic procedures in adults have been described. However, there are no reports of tumor recurrence at thoracostomy tube or thoracoscopic trocar insertion sites after operation for thoracic malignancies in children. The authors report 2 cases of tumor recurrence at thoracostomy tube insertion sites after intraoperative gross spillage of pleuropulmonary blastoma and malignant epithelial thymoma and discuss approaches that may potentially prevent this devastating complication.