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Connecticut Medicine Jan 2013Pulmonary blastoma is a rare malignant tumor, histologically resembling the fetal lung. Since its first description in 1945, only about 200 cases have been reported...
Pulmonary blastoma is a rare malignant tumor, histologically resembling the fetal lung. Since its first description in 1945, only about 200 cases have been reported worldwide. This tumor predominantly affects children, but has also been reported in adults with a peak incidence in the fourth decade of life. Pulmonary blastoma has a variable clinical course that cannot be determined by its histological appearance. We report a 51-year-old patient with a large biphasic pulmonary blastoma who was treated with surgical excision. The patient remains disease-free eleven months postoperatively. As relapse rates are high in patients with large biphasic (type 2) tumors, the patient is being monitored closely. Although a rare occurrence after the age of 20, pulmonary blastoma should remain in the differential diagnosis of a lung mass in an adult.
Topics: Humans; Immunohistochemistry; Keratin-7; Lung Neoplasms; Male; Middle Aged; Pulmonary Blastoma; Tomography, X-Ray Computed; Transcription Factors; beta Catenin
PubMed: 23427368
DOI: No ID Found -
Revue Medicale Suisse Feb 2007Pneumoblastoma (PB) is a rare, malignant, primary, pulmonary tumour, of young adults. Its discovery is fortuitous in asymptomatic patients. It is a well-delimited,... (Review)
Review
Pneumoblastoma (PB) is a rare, malignant, primary, pulmonary tumour, of young adults. Its discovery is fortuitous in asymptomatic patients. It is a well-delimited, homogeneous lesion in the lung periphery. Histologically, its structure looks like a normal fetal lung. The surgical resection is the treatment of choice. The radiotherapy is an empirical palliative treatment to relieve dyspnea when other treatments failed. The prognosis is bad: 16% survive 5 years and 8% beyond 10 years, all treatments included. Metastases could appear in the liver, the brain and the bone.
Topics: Adult; Chemotherapy, Adjuvant; Humans; Lung Neoplasms; Male; Prognosis; Pulmonary Blastoma; Radiotherapy, Adjuvant; Survival Analysis; Switzerland
PubMed: 17424798
DOI: No ID Found -
Annals of Thoracic and Cardiovascular... Feb 1998Pulmonary blastoma is a rare tumor containing both mesenchymal and/or epithelial elements that mimics the embryonal tissues of the developing lungs. We are presenting...
Pulmonary blastoma is a rare tumor containing both mesenchymal and/or epithelial elements that mimics the embryonal tissues of the developing lungs. We are presenting two cases of pulmonary blastoma for its rarity and reviewed the literature for clinical features, investigative findings, classification, management and prognosis.
Topics: Antineoplastic Combined Chemotherapy Protocols; Child, Preschool; Female; Humans; Lung Neoplasms; Male; Pulmonary Blastoma; Radiography
PubMed: 9495909
DOI: No ID Found -
Indian Journal of Pediatrics Aug 2001A three-and-half-year-old boy presented with recurrent chest infections, fever and weight loss of three month duration not responding to antibiotics. The chest X-ray and...
A three-and-half-year-old boy presented with recurrent chest infections, fever and weight loss of three month duration not responding to antibiotics. The chest X-ray and CT scan revealed a large well-circumscribed mass in right upper thorax with collapse of right upper lobe. A preoperative diagnosis could not be made even after fine needle aspiration cytology. Thoracotomy and right upper lobectomy was done and the biopsy report was an inflammatory pseudotumor. The child remained well for three months after which his symptoms and the mass recurred. The histopathology slides were reviewed and revealed a biphasic malignant tumor suggestive of Pulmonary Blastoma (PB). Patient received four cycles of chemotherapy followed by re-exploration. The recurrent tumour could only be excised partly and the child succumbed to persistent shock postoperatively. The final histopathological diagnosis was confirmed as PB. Primary pulmonary neoplasms in children are rare and of these PB which is even rarer, constitutes less than 15%. The report highlights that the lack of familiarity with this entity still causes error in the diagnosis of PB.
Topics: Antineoplastic Combined Chemotherapy Protocols; Child, Preschool; Fatal Outcome; Humans; Lung Neoplasms; Male; Pulmonary Blastoma; Tomography, X-Ray Computed
PubMed: 11563261
DOI: 10.1007/BF02752427 -
The Journal of Spinal Cord Medicine Jul 1995Pulmonary blastoma (PB) is an uncommon primary lung malignancy. This neoplasm was first described by Barrett and Barnard in 1945. The tumor is composed of immature... (Review)
Review
Pulmonary blastoma (PB) is an uncommon primary lung malignancy. This neoplasm was first described by Barrett and Barnard in 1945. The tumor is composed of immature epithelial and mesenchymal tissues which may recapitulate early embryological lung development. Under the microscope, the globular component resembles immature bronchus and connective tissue as seen in embryonic lung. More than one hundred cases have been reported in the literature. PB is more frequent in older people and in males and tends to affect blacks at younger ages. Symptomatology varies from asymptomatic to symptoms of a non-specific pulmonary disease. Cough, hemoptysis, dyspnea, chest pain, respiratory distress, fever, anorexia and weight loss are the most common presenting features. The most common roentgenologic pattern is a well-demarcated peripheral lesion, encapsulated by compression or atelectatic lung tissue, although in some cases there is a tendency to lobulation and cavitation. The size of the mass varies from a small peripheral nodule to a mass occupying the entire lobe or hemithorax. The treatment of choice has been surgical excision, radiation and, in selected cases, a combination of chemotherapy with radiation. The prognosis of this malignancy is poor; overall five-year survival is approximately 16 percent. No correlation has been established between histopathologic criteria and survival. The factors that indicate poor prognosis are tumor recurrence, metastasis at initial presentation, tumor size over 5 cm and lymph node metastasis. Liver, central nervous system and bones are the most frequent location of distant metastases. A rare case is presented of a pulmonary blastoma with an upper lip metastasis occurring in a paraplegic male. Diagnosis was confirmed by autopsy findings.(ABSTRACT TRUNCATED AT 250 WORDS)
Topics: Humans; Lip; Lip Neoplasms; Lung; Lung Neoplasms; Male; Middle Aged; Neoplastic Cells, Circulating; Pulmonary Blastoma
PubMed: 7552426
DOI: 10.1080/10790268.1995.11719394 -
General Thoracic and Cardiovascular... Jun 2007Biphagic 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,... (Review)
Review
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 alpha-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 alpha-fetoprotein antibody. Adjuvant chemotherapy consisted of cisplatin and etoposide. After surgery, the serum level of alpha-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.
Topics: Combined Modality Therapy; Endodermal Sinus Tumor; Humans; Lung Neoplasms; Lymph Node Excision; Male; Middle Aged; Neoplasm Recurrence, Local; Pulmonary Blastoma; Tomography, X-Ray Computed; alpha-Fetoproteins
PubMed: 17642278
DOI: 10.1007/s11748-007-0112-z -
Medical and Pediatric Oncology Dec 1995Pleuro-pulmonary blastoma is a rare malignant lung tumor in children and distinct from ordinary pulmonary blastoma of adulthood. Combination chemotherapy plays an... (Review)
Review
Pleuro-pulmonary blastoma is a rare malignant lung tumor in children and distinct from ordinary pulmonary blastoma of adulthood. Combination chemotherapy plays an important role in the treatment of this tumor, but so far no attempt of high dose chemotherapy with subsequent bone marrow or blood stem cell transplantation has been published. The case is reported of a 2-year-old boy with pleuro-pulmonary blastoma who achieved partial remission with conventional chemotherapy and surgery. Subsequent administration of high dose melphalan, etoposide, and carboplatin, followed by autologous blood stem cell transplantation resulted in a 3-month disease-free interval but did not prevent eventual local recurrence, leading to death within a few weeks. Clinical presentation, pathology, management, and prognosis are discussed and the literature reviewed.
Topics: Child, Preschool; Humans; Lung Neoplasms; Male; Pleural Neoplasms; Pulmonary Blastoma
PubMed: 7565313
DOI: 10.1002/mpo.2950250612 -
Bulletin Du Cancer Oct 2012Pulmonary sarcomatoid carcinomas are a rare group of tumors accounting for about 1 % of non-small cell lung carcinoma (NSCLC). In 2004, World Health Organization... (Review)
Review
Pulmonary sarcomatoid carcinomas are a rare group of tumors accounting for about 1 % of non-small cell lung carcinoma (NSCLC). In 2004, World Health Organization classification united under this name all the carcinomas with sarcomatous or sarcomatous-like component with spindle cell or giant cell appearance. There are five subtypes: spindle cell carcinoma, giant cell carcinoma, pleomorphic carcinoma, carcino-sarcoma and pulmonary blastoma. Clinical characteristics are not specific from the others subtypes of NSCLC. Epithelial to mesenchymal transition pathway may play a key role. Patients are frequently symptomatic. Tumors are voluminous more often peripherical than central, with strong fixation on FDG TEP CT. Distant metastasis are frequent with atypical locations such as peritoneal or retroperitoneal sites. These tumors have poorer prognosis than the other NSCLC subtypes because of great aggressivity, and frequent chemoresistance. Here, we present a review of litterature in order to better describe these tumors.
Topics: Carcinoma; Carcinoma, Giant Cell; Carcinoma, Non-Small-Cell Lung; Carcinosarcoma; Epithelial-Mesenchymal Transition; Humans; Lung Neoplasms; Prognosis; Pulmonary Blastoma; Rare Diseases; Tumor Burden
PubMed: 22889810
DOI: 10.1684/bdc.2012.1619 -
Indian Pediatrics Feb 1997
Topics: Age of Onset; Child; Diagnosis, Differential; Humans; Lung Neoplasms; Male; Pulmonary Blastoma; Tuberculosis
PubMed: 9255019
DOI: No ID Found -
European Journal of Cardio-thoracic... Feb 2006Pulmonary blastoma is a rare primary lung tumor with poor prognosis that commonly presents at a younger age than the non-small cell lung carcinoma (NSCLC). Classically...
BACKGROUND
Pulmonary blastoma is a rare primary lung tumor with poor prognosis that commonly presents at a younger age than the non-small cell lung carcinoma (NSCLC). Classically they are large, symptomatic tumors with lymph nodal metastasis and carry poor prognosis. We report our experience of 7 patients with pulmonary blastoma who presented with varying clinical features.
METHODS
Retrospective analysis of our database revealed seven patients with pulmonary blastoma that were operated between January 1993 and December 2004. During the same time, 889 lung resections were performed for primary NSCLC. Demographic and clinical details were obtained from hospital notes. The histopathology reports were reviewed with the department of pathology and the radiological images were reported.
RESULTS
The tumors showed a bimodal pattern in age at incidence being (four patients were less than 49 years and three more than 66 years). All patients were symptomatic and demonstrated variable sized tumors. Lesions were common in lower lobes (lower:other lobes -4:3) and were staged T2N0 pathologically. All of the patients underwent surgical resections and had no chemotherapy/radiotherapy following surgery. Three of the seven patients died during follow-up due to unrelated causes between 24 and 29 months. The longest follow-up was more than 9 years and the survivor continues to do well.
CONCLUSIONS
Pulmonary blastomas are rare tumors but can present with differing clinical features. Early detection and treatment may improve prognosis. Further larger series are needed to evaluate the characteristics of the tumor.
Topics: Adult; Age Distribution; Age of Onset; Aged; Aged, 80 and over; Female; Humans; Lung Neoplasms; Male; Middle Aged; Pulmonary Blastoma; Sex Distribution; Survival Rate; Tomography, X-Ray Computed; Treatment Outcome
PubMed: 16387506
DOI: 10.1016/j.ejcts.2005.11.035