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Advances in Respiratory Medicine 2021Pulmonary blastoma is a rare malignancy, accounting for less than 0.5% of primary lung tumors. It belongs to the group of pulmonary sarcomatoid carcinomas, and it is... (Review)
Review
INTRODUCTION
Pulmonary blastoma is a rare malignancy, accounting for less than 0.5% of primary lung tumors. It belongs to the group of pulmonary sarcomatoid carcinomas, and it is typically characterized by a biphasic pattern of an epithelial and a mesenchymal component. Only a few hundred cases have been reported worldwide. The aim of this study is to review and critically assess the literature regarding pulmonary blastoma.
MATERIAL AND METHODS
A narrative literature review of PubMed database from the inception of the database up to January 2021, limited to the English language, was conducted, using combinations of the following keywords: "pulmonary blastoma", "biphasic pulmonary blastoma", "sarcomatoid carcinoma".
RESULTS
Pulmonary blastoma is composed of an epithelial and a mesenchymal malignant component. Regarding pathogenesis, the origin of the biphasic cell population remains elusive. Characteristic immunohistochemical stains are supportive of diagnosis.Clinically, the symptomatology is non-specific, while 40% of the cases are asymptomatic. It is diagnosed at a younger agecompared to other types of lung cancer, and it is often non-metastatic at diagnosis allowing for surgical treatment. Data on management and survival are scarce and mainly come from isolated cases. Advances on targeted therapy may provide novel treatment options. Given the rarity of the cases, multicenter collaboration is needed in order to establish therapeutic guidelines.
Topics: Humans; Lung Neoplasms; Neoplasm Staging; Pulmonary Blastoma
PubMed: 34725809
DOI: 10.5603/ARM.a2021.0085 -
Journal of Medical Ultrasonics (2001) Oct 2022
Topics: Humans; Pulmonary Blastoma; Mitral Valve; Lung Neoplasms
PubMed: 35918507
DOI: 10.1007/s10396-022-01248-w -
European Journal of Pediatrics Dec 2017Congenital pulmonary airway malformations or CPAM are rare developmental lung malformations, leading to cystic and/or adenomatous pulmonary areas. Nowadays, CPAM are... (Review)
Review
UNLABELLED
Congenital pulmonary airway malformations or CPAM are rare developmental lung malformations, leading to cystic and/or adenomatous pulmonary areas. Nowadays, CPAM are diagnosed prenatally, improving the prenatal and immediate postnatal care and ultimately the knowledge on CPAM pathophysiology. CPAM natural evolution can lead to infections or malignancies, whose exact prevalence is still difficult to assess. The aim of this "state-of-the-art" review is to cover the recently published literature on CPAM management whether the pulmonary lesion was detected during pregnancy or after birth, the current indications of surgery or surveillance and finally its potential evolution to pleuro-pulmonary blastoma.
CONCLUSION
Surgery remains the cornerstone treatment of symptomatic lesions but the postnatal management of asymptomatic CPAM remains controversial. There are pros and cons of surgical resection, as increasing rate of infections over time renders the surgery more difficult after months or years of evolution, as well as risk of malignancy, though exact incidence is still unknown. What is known: • Congenital pulmonary airway malformations (CPAM) are rare developmental lung malformations mainly antenatally diagnosed. • While the neonatal management of symptomatic CPAM is clear and includes prompt surgery, controversies remain for asymptomatic CPAM due to risk of infections and malignancies. What is new: • Increased rate of infection over time renders the surgery more difficult after months or years of evolution and pushes for recommendation of early elective surgery. • New molecular or pathological pathways may help in the distinction of type 4 CPAM from type I pleuropulmonary blastoma.
Topics: Cystic Adenomatoid Malformation of Lung, Congenital; Disease Progression; Female; Genetic Predisposition to Disease; Humans; Lung; Lung Neoplasms; Pediatrics; Pregnancy; Prenatal Diagnosis; Pulmonary Blastoma
PubMed: 29046943
DOI: 10.1007/s00431-017-3032-7 -
BMC Pulmonary Medicine Jan 2022Pulmonary blastoma (PB) comprises a rare heterogeneous group of lung tumours typically containing immature epithelial and mesenchymal structures that imitate the... (Review)
Review
BACKGROUND
Pulmonary blastoma (PB) comprises a rare heterogeneous group of lung tumours typically containing immature epithelial and mesenchymal structures that imitate the embryonic lung tissue and extremely rarely occurs during pregnancy. Although cough and haemoptysis are the most common PB symptoms, they usually indicate other serious pregnancy-related complications.
CASE PRESENTATION
The article presents the unusual case of a 22-year-old pregnant woman diagnosed with PB during pregnancy.
CONCLUSIONS
PB is characterized by poor prognosis and patients' outcome relies on a rapid diagnosis. Surgery remains the most common and effective treatment. Due to the extreme rarity, the literature contains only single mentions of PB in pregnancy, thus its impact on the course of pregnancy and the developing fetus remains unknown.
Topics: Cesarean Section; Chemotherapy, Adjuvant; Female; Humans; Infant, Newborn; Lung Neoplasms; Male; Pregnancy; Pulmonary Blastoma; Treatment Outcome; Young Adult
PubMed: 34983474
DOI: 10.1186/s12890-021-01804-z -
International Journal of Surgical... Dec 2017Pulmonary blastoma is a rare malignant lung tumor with aggressive behavior and dismal prognosis. It is extremely rare in children aged <18 years, and little is known... (Review)
Review
Pulmonary blastoma is a rare malignant lung tumor with aggressive behavior and dismal prognosis. It is extremely rare in children aged <18 years, and little is known about its genetic alteration and pathogenesis. Although surgical resection and adjuvant chemotherapy or radiotherapy have been applied in several cases, no standard treatment guidelines with sufficient evidence have been established. In this article, we report a case of a large pulmonary blastoma in a 7-year-old girl whose initial presentation was progressive dyspnea and productive cough. We subsequently present a review on cases involving young patients as well as genetic analysis data available in the literature.
Topics: Child; Female; Humans; Lung Neoplasms; Pulmonary Blastoma
PubMed: 28675947
DOI: 10.1177/1066896917718622 -
Seminars in Diagnostic Pathology May 1994Pulmonary blastoma was defined by Spencer and others as a neoplasm with histopathological features thought to be reminiscent of Wilms' tumor. Unlike the other... (Review)
Review
Pulmonary blastoma was defined by Spencer and others as a neoplasm with histopathological features thought to be reminiscent of Wilms' tumor. Unlike the other embryonal-fetal neoplasms that typically occur in early childhood, the majority of pulmonary blastomas have been reported in adults. One explanation offered by Spencer for the delayed clinical presentation of pulmonary blastoma is the continued development of lung parenchyma well past the postnatal period. It has been proposed that the pulmonary blastoma is a variant of carcinosarcoma, which is seen almost exclusively in adults. The classic pulmonary blastoma is described as a neoplasm with a mixture of primitive tubular profiles, immature blastema, and a spindle cell stroma. More recently, some have included the well-differentiated adenocarcinoma of fetal type as a pure epithelial expression of pulmonary blastoma. By contrast, most cases of pulmonary blastoma in children have been described as having an exclusive mesenchymal composition, either embryonal rhabdomyosarcoma or a complex, mixed blastematous and sarcomatous neoplasm. Some pulmonary blastomas in children, particularly those with only embryonal rhabdomyosarcoma, have presented as a peripheral multicystic lesion that has been interpreted as a developmental cyst, either cystic adenomatoid malformation or bronchogenic cyst. Other tumors have been described as intrathoracic and anatomically separate from the lung, like some extralobar sequestrations. We have proposed the designation pleuropulmonary blastoma for these pulmonary or extrapulmonary neoplasms of childhood. We propose that this neoplasm is the rightful pulmonary blastoma rather than the pulmonary blastoma of Spencer, which has been the long-time pretender to the title.
Topics: Adolescent; Adult; Child; Child, Preschool; Female; Humans; Lung Neoplasms; Male; Pleural Neoplasms; Pulmonary Blastoma
PubMed: 7809508
DOI: No ID Found -
Pediatric and Developmental Pathology :... 2023Congenital cystic pulmonary lesions (CCPLs) are represented by the following entities: congenital pulmonary airway malformation (CPAM), formerly congenital cystic... (Review)
Review
Congenital cystic pulmonary lesions (CCPLs) are represented by the following entities: congenital pulmonary airway malformation (CPAM), formerly congenital cystic adenomatoid malformation, extra- and intralobar sequestration (EIS), congenital lobar emphysema (overexpansion), and bronchogenic cyst. The developmental model of CPAM histogenesis by Stocker proposed perturbations designated as CPAM type 0 to type 4 without known or specific pathogenetic mechanisms along the airway from the bronchus to the alveolus. This review highlights mutational events either at the somatic level in KRAS (CPAM types 1 and possibly 3) or germline variants in congenital acinar dysplasia, formerly CPAM type 0, and pleuropulmonary blastoma (PPB), type I, formerly CPAM type 4. The potential for overt malignant progression exists in the case of PPB type I and CPAM type 1 in some cases to well-differentiated mucinous adenocarcinoma. On the other hand, CPAM type 2 is an acquired lesion resulting from interruption in lung development secondary to bronchial atresia. The latter is also regarded as the etiology of EIS whose pathologic features are similar, if not identical, to CPAM type 2. These observations have provided important insights into the pathogenetic mechanisms in the development of the CPAMs since the Stocker classification.
Topics: Humans; Cystic Adenomatoid Malformation of Lung, Congenital; Lung; Pulmonary Blastoma; Lung Neoplasms; Respiratory System Abnormalities; Bronchopulmonary Sequestration
PubMed: 37334833
DOI: 10.1177/10935266221146823 -
European Journal of Cardio-thoracic... May 1998
Topics: Adult; Humans; Lung Neoplasms; Male; Pulmonary Blastoma
PubMed: 9663550
DOI: 10.1016/s1010-7940(98)00061-x -
BMC Research Notes May 2014Pulmonary blastomas are a rare aggressive neoplasm comprising 0.25-0.5% of all primary lung tumors and portend a poor prognosis. They display a biphasic histology with... (Review)
Review
BACKGROUND
Pulmonary blastomas are a rare aggressive neoplasm comprising 0.25-0.5% of all primary lung tumors and portend a poor prognosis. They display a biphasic histology with mesenchymal and epithelial components. Historically, the term pulmonary blastoma had included both pure fetal adenocarcinomas, pleuropulmonary blastomas as well as the classic biphasic blastomas. However recent World Health Organisation re-classifications separated well-differentiated fetal adenocarcinomas and pleuropulmonary blastomas from the biphasic tumours.
CASE PRESENTATION
We present a case of a systemically well 67-year-old Caucasian male who presented with haemoptysis. Investigations confirmed the presence of a large right-sided lung mass and biopsy identified non-small cell carcinoma. The resected tumour was markedly necrotic revealing a biphasic pattern. It was composed of malignant glandular tissue with sub-nuclear vacuoles, associated with a pleomorphic stromal malignant blastematous component, characteristic of classic biphasic pulmonary blastoma.
CONCLUSION
We present a case of a classic biphasic pulmonary blastoma, a rare lung cancer occurring at an earlier age and portending to poorer prognosis than other more common lung cancers. Given the small number of cases and recent re-classification, interpreting the published epidemiology and clinical features of this disease is difficult. Many earlier reports may have included fetal adenocarcinomas (in particular high grade variant), which need to be considered when discussing treatment and prognosis with newly-diagnosed patients. Much could be gained from a central registry of individual experiences to improve our understanding of this rare lung cancer.
Topics: Aged; Humans; Male; Positron-Emission Tomography; Pulmonary Blastoma; Tomography, X-Ray Computed
PubMed: 24885892
DOI: 10.1186/1756-0500-7-294 -
Lung Cancer (Amsterdam, Netherlands) Mar 2024Pulmonary blastomas (PB) are an extremely rare type of lung cancer. Currently, no standard treatment exists for PB. Immunotherapy with checkpoint inhibitors and... (Review)
Review
Pulmonary blastomas (PB) are an extremely rare type of lung cancer. Currently, no standard treatment exists for PB. Immunotherapy with checkpoint inhibitors and anti-angiogenesis treatments has been an effective method for lung cancer; however, studies on PB treatment are lacking. Herein, we present a case report of successful conversion therapy with immunotherapy and targeted therapy for PB. After receiving treatment with a PD-1 inhibitor (penpulimab) and a multi-target tyrosine kinase inhibitor (anlotinib) treatment, the patient showed an impressive response and underwent a successful operation. We also summarized and reviewed literature reports on PubMed from January 1, 2000, to December 31, 2022, using the keyword "pulmonary blastoma", discussing the efficacy and specifics of chemotherapy and radiotherapy. Immunotherapy, in combination with targeted therapy, should be considered a potential therapeutic strategy for PB.
Topics: Humans; Pulmonary Blastoma; Lung Neoplasms; Immunotherapy; Immune Checkpoint Inhibitors; Protein Kinase Inhibitors
PubMed: 38280290
DOI: 10.1016/j.lungcan.2024.107476