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Indian Journal of Pathology &... 2023Here we intend to document a rare case of PPB type III in a 2-year male presenting with an extensive tumor occupying the right hemithorax with immunohistochemical (IHC)...
Here we intend to document a rare case of PPB type III in a 2-year male presenting with an extensive tumor occupying the right hemithorax with immunohistochemical (IHC) study. Pleuropulmonary blastoma (PPB) is a rare variably aggressive, dysodontogenetic, childhood primary intrathoracic malignancy which in up to 25% of cases can be extrapulmonary with attachment to the parietal pleura. It is found in pediatric population under 5 years of age. It was initially proposed as a distinct entity by Manivel et al. in 1988. PPB is a proliferation of primitive mesenchymal cells that initially form air-filled cysts lined by benign-appearing epithelium (type I, cystic). Later on, the mesenchymal cells outgrow the cysts with formation of focal solid areas (type II, solid and cystic) and finally, mainly solid mass (type III, solid PPB).
Topics: Humans; Male; Child; Lung Neoplasms; Pleural Neoplasms; Pulmonary Blastoma; Cysts
PubMed: 37530358
DOI: 10.4103/ijpm.ijpm_781_21 -
International Journal of Surgery Case... Jul 2023Pleuropulmonary blastoma is a rare, aggressive intrathoracic neoplasm of early childhood.
INTRODUCTION
Pleuropulmonary blastoma is a rare, aggressive intrathoracic neoplasm of early childhood.
CASE PRESENTATION
We report a case of a 4-month-old male baby who has presented with recurrent respiratory infections since birth. A surgical team was consulted due to abnormal opacification observed on a chest X-ray. An enhanced-contrast CT scan of the chest revealed a heterogenous, well-delineated mass measuring about 3,8 × 6 cm in the posterior mediastinum. A left posterolateral thoracotomy was performed. The mass was separated from the lung parenchyma, located behind the parietal pleura, and adherent to the chest wall and superior ribs. The lesion was totally removed. Histologically, the lesion was a pleuropulmonary blastoma type III. Currently, the patient is on a 6-month course of chemotherapy.
CLINICAL DISCUSSION
The aggressive, insidious behavior of PPB requires a high index of suspicion for diagnosis. The clinical manifestations and imaging modalities are atypical and nonspecific. However, PPB should be kept in mind when a huge solid or cystic mass is observed in the lung field on imaging.
CONCLUSION
Extrapulmonary pleuropulmonary blastoma is a very rare entity characterized by highly aggressive behavior and a poor prognosis. Early excision of thoracic cystic lesions in children is warranted regardless of the symptoms to avoid future mishaps.
PubMed: 37423144
DOI: 10.1016/j.ijscr.2023.108461 -
Pathologica Jun 2023Among non-small cell lung cancers (NSCLCs), sarcomatoid carcinomas account for 3%. They are rare tumours with a poor prognosis, classified into three subgroups, namely...
Among non-small cell lung cancers (NSCLCs), sarcomatoid carcinomas account for 3%. They are rare tumours with a poor prognosis, classified into three subgroups, namely pleomorphic carcinoma, pulmonary blastoma and carcinosarcoma. In the 5th edition of WHO Classification of Thoracic Tumours more space is given to SMARC4-deficient lung cancers. Although studies on SMARCA4-deficient lung tumours are limited, a small percentage of SMARCA4 loss is present within NSCLCs. This finding is clinically relevant, as the loss of the SMARCA4 gene is associated with a worse prognosis. In our study, we analysed the presence of the main catalytic subunit of the SMARCA4 gene, the BRG1 protein, in 60 sarcomatoid lung tumours. The results of our study show that 5.3% of sarcomatoid carcinomas have BRG1-loss in tumour cells, proving that a non-negligible amount of lung sarcomatoid carcinomas are SMARCA4-deficient. These data open the debate on the necessity of including the detection of SMARCA4 within a standardised immunohistochemical panel.
Topics: Humans; Diagnosis, Differential; Neoplasms, Glandular and Epithelial; Carcinoma, Non-Small-Cell Lung; Lung Neoplasms; Carcinoma; Lung; DNA Helicases; Nuclear Proteins; Transcription Factors
PubMed: 37387441
DOI: 10.32074/1591-951X-847 -
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 -
Children (Basel, Switzerland) May 2023Fetal lung interstitial tumor (FLIT) is an extremely rare pediatric lung tumor that shares radiological features with congenital pulmonary malformations (cPAM) and other... (Review)
Review
Fetal lung interstitial tumor (FLIT) is an extremely rare pediatric lung tumor that shares radiological features with congenital pulmonary malformations (cPAM) and other lung neoplasms. A review of the literature, together with the first European case, are herein reported. A systematic and manual search of the literature using the keyword "fetal lung interstitial tumor" was conducted on PUBMED, Scopus, and SCIE (Web of Science). Following the PRISMA guidelines, 12 articles were retrieved which describe a total of 21 cases of FLIT, and a new European case is presented. A prenatal diagnosis was reported in only 3 out of 22 (13%) cases. The mean age at surgery was 31 days of life (1-150); a lobectomy was performed in most of the cases. No complications or recurrence of disease were reported at a mean follow-up of 49 months. FLIT is rarely diagnosed during pregnancy, may present at birth with different levels of respiratory distress, and requires prompt surgical resection. Histology and immunohistochemistry allow for the differentiation of FLIT from cPAM and other lung tumors with poor prognosis, such as pleuropulmonary blastoma, congenital peri-bronchial myofibroblastic tumor, inflammatory myofibroblastic tumor, and congenital or infantile fibrosarcoma.
PubMed: 37238376
DOI: 10.3390/children10050828 -
Annals of Medicine and Surgery (2012) May 2023Pleuropulmonary blastoma (PPB) is a very rare, aggressive, embryonal pulmonary malignancy that mostly affects children under the age of 5 years. According to the...
Pleuropulmonary blastoma (PPB) is a very rare, aggressive, embryonal pulmonary malignancy that mostly affects children under the age of 5 years. According to the histological features, three subtypes of PPB have been recognized: type I (purely cystic), type II (grossly visible cystic and solid elements), and type III (purely solid). The authors report a case of a 10-month-old male infant with type I PPB, who was clinically misdiagnosed with pneumothorax, that he presented complaining of shortening of breath, fever, and cough. Radiographs of the patient showed right pneumothorax, so he had managed accordingly in another centre without improvement. Then Computed Tomography showed a huge right upper lobe sepated pneumocyst, which was treated surgically and the diagnosis was confirmed by combining the imaging and the histopathological examination as PPB type I. PPB is a relatively rare tumour, and it is important to put PPB with their subtypes within the differential diagnoses of any pulmonary lesion in children below the age of 5 or 6 years, as the early diagnosis will help to give early management. Hence, the patient may have a better outcome.
PubMed: 37229013
DOI: 10.1097/MS9.0000000000000514 -
Frontiers in Oncology 2023Pulmonary blastoma (PB) is a rare and invasive malignancy of the lungs with a poor prognosis. Although the mainstay treatment of PB is surgery, and radiotherapy and...
Pulmonary blastoma (PB) is a rare and invasive malignancy of the lungs with a poor prognosis. Although the mainstay treatment of PB is surgery, and radiotherapy and chemotherapy have been reported, no standard therapy exists for patients inoperable in advanced stages. Moreover, little is known about driver mutation status and immunotherapy efficacy. This paper presents a male patient diagnosed with classic biphasic PB using CT-guided lung biopsy pathology and immunohistochemistry. The patient's symptoms included cough, chest pain, shortness of breath, hemoptysis, and hypodynamia. The primary focus of this paper is to discuss the impact of anti-PD-1 immunotherapy on PB. The patient experienced progression-free survival (PFS) of over 27 months following sintilimab second-line anti-PD-1 therapy. The patient has currently survived for nearly 40 months with a satisfactory quality of life.
PubMed: 37124510
DOI: 10.3389/fonc.2023.1146204 -
African Health Sciences Dec 2022There are limited data on histological classification of primary lung cancer from sub-Saharan Africa. Furthermore, the time trends of age-truncated incidence rates of...
BACKGROUND
There are limited data on histological classification of primary lung cancer from sub-Saharan Africa. Furthermore, the time trends of age-truncated incidence rates of lung cancer by histological phenotype in Zambia are also unknown.
OBJECTIVES
The objective of this study was to determine histological types of lung tumours at the University Teaching Hospital (UTH) in Lusaka, Zambia.
METHODS
This was a retrospective pilot study of lung tumour biopsies collected from the histopathology laboratory at the UTH over a period of one year. Tissue sections were stained and when seen, lung cancer was classified using standard histological methods. Data were analysed using IBM SSPS version 23.
RESULTS
A total of 23 lung cancer tissues were retrieved. Histological types included eleven (47.8%) squamous cell carcinoma (SCC), six (26.1%) adenocarcinoma, two (8.7%) small cell carcinoma, two (8.7%) large cell carcinoma, 1 (4.3%) inflammatory myofibroblastic tumours and 1 (4.3%) pleural pulmonary blastoma. The results showed that the most affected age group was 60-69 years with most of the histological subtype in this age group being SCC. There was no statistically significant difference of histological subtypes across age groups, p=0.12.
CONCLUSION
This study has shown that the most commonly diagnosed type of primary lung cancer is squamous cell carcinoma. More data are needed to further corroborate this observation.
Topics: Humans; Pilot Projects; Zambia; Retrospective Studies; Universities; Lung Neoplasms; Hospitals, Teaching; Carcinoma, Squamous Cell
PubMed: 37092044
DOI: 10.4314/ahs.v22i4.5 -
Frontiers in Oncology 2023The incidence of primary lung cancer (LC) in children and adolescence was rare. We analyzed data from a SEER database to better define the incidence, clinical...
BACKGROUND
The incidence of primary lung cancer (LC) in children and adolescence was rare. We analyzed data from a SEER database to better define the incidence, clinical characters, pathology, treatment, and outcomes of rare primary malignant pulmonary tumors in childhood and adolescence.
METHODS
Patients were chosen from the SEER database (SEER*Stat 8.4.0 software) from 2000 to 2019 and all patients were pathologically diagnosed with primary malignant tumors of the lung and bronchus. Demographic characteristics of patients (age, gender, race, primary site, laterality, location, differentiation grade, operation methods, histology, and history of radiotherapy and chemotherapy), as well as TNM stage and survival time, were collected.
RESULTS
A total of 301 cases of children ≤19 years of age with a primary malignant pulmonary tumor were reported to the SEER database from 2000 to 2019. There were 143 men (47.5%) and 158 women (52.5%). Whites represented majority of patients (79.7%), followed by Black (13.6%) and others (6.7%). As for the primary site, the main site was the lower lobe (33.2%), followed by the upper lobe (26.9%). Most of the patients (80.4%) underwent surgery. Lobectomy (39.9%) is the main operation method. Only 28 (9.3%) patients received radiotherapy and 112 (37.2) patients received chemotherapy. Carcinoid tumor was the most common histology (29.6%), followed by pulmonary blastoma (PB) (22.3%), mucoepidermoid carcinoma (MEC) (12.3%), adenocarcinoma (10.3%), neuroendocrine tumor (NET) (5.7%), squamous cell carcinoma (SCC) (5.3%), atypical carcinoma (2.3%). The mean follow-up time was 100 months. For the entire group of children and adolescents, the 1-year OS was 89.1%, and the 3-year overall survival (OS) was 79.7%. the 5-year OS was 77.9%, the 10-year OS was 75.7%, and the 15-year OS was 73.9%. And 1-year lung cancer specificity survival (LCSS) was 89.8%, and the 3-year LCSS was 80.4%. the 5-year LCSS was 79.4%, the 10-year LCSS was 77.7%, and the 15-year LCSS was 75.9%. The OS of atypical carcinoma, carcinoid tumor, and MEC were in the top three.
CONCLUSIONS
Primary LC in children and adolescent were rare and histopathological diverse. Fortunately, children and adolescents with LC had an overall favorable outcome after treatment. Histology, differentiation grade, surgery, TNM stage, and therapeutic modalities have important influence on OS. The further treatment experience of each pathological type would make better evidence-based practice possible.
PubMed: 37091171
DOI: 10.3389/fonc.2023.1053248