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Pulmonary Blastoma

Classic Pulmonary Blastoma


Presentation

  • Pleural effusion may be present but is not the predominant abnormality. Our cases are unusual as recurrent pleural effusions in the absence of a significant lung mass were the presenting findings.[ncbi.nlm.nih.gov]
Recurrent Pleural Effusion
  • Our cases are unusual as recurrent pleural effusions in the absence of a significant lung mass were the presenting findings.[ncbi.nlm.nih.gov]
Soft Tissue Mass
  • Notes: Computed tomography scans of the chest were obtained at baseline and after 1 month of treatment with crizotinib. ( A ) Chest computed tomography revealed a soft tissue mass in the left upper lobe abutting the anterior–lateral pleura. ( B ) Computed[dovepress.com]
Exertional Dyspnea
  • Available from: Sir, A 17-year-old male presented to our hospital with complaints of exertional dyspnea of two months and non-productive cough of one month duration.[indianjcancer.com]
Malignant Pleural Effusion
  • There now is mounting evidence that it may be effective; Kummet and Doll initially described its use as a second‐line agent in the treatment of a patient whose metastatic disease presented as malignant pleural effusions 18 months after initial resection[onlinelibrary.wiley.com]
Loss of Initiative
  • The patient reported no symptoms of shortness of breath, hemoptysis, chest pain, fever, chills, or weight loss. Amoxicillin initially was prescribed without symptomatic response.[onlinelibrary.wiley.com]
Hunger
  • Each episode happens as a result of hunger, rather than eating food. Hypoglycemia in this patient was episodic and a result of hunger as well [ 7 ].[ncbi.nlm.nih.gov]
Psychiatric Symptoms
  • In all patients with epithelial or mesenchymal tumors that exhibit neuro psychiatric symptoms, NICTH should be considered [ 7 ]. Our patient reported weakness, tiredness, and excessive sweating.[ncbi.nlm.nih.gov]

Workup

  • He underwent workup for distant metastasis, the findings of which were unremarkable. Therefore, left lower lobectomy was performed with lymph node dissection.[kjim.org]
Mediastinal Shift
  • Pulmonary blastoma usually presents radiologically as a well-defined mass lesion on chest radiography, which may be large enough to completely opacify the hemithorax and cause mediastinal shift.[ncbi.nlm.nih.gov]
  • Unilateral lung whiteout on plain film with mediastinal shift to the opposite side. Usually, there are no adjacent rib erosions or calcifications. Ultrasound A poor imaging modality for this diagnosis.[radiopaedia.org]
  • Chest radiography revealed complete opacification of the right hemithorax, contralateral mediastinal shifting consistent with right sided large pleural effusion ( Fig. 1 ).[kjco.org]
  • Radiographic findings of primary PPB benign-appearing cystic lung disease with or without pneumothorax partial opacification of a portion of the lung suggesting pneumonia complete opacification of the hemithorax mediastinal shift and tension pneumothorax[telemedicina.med.muni.cz]
Left Pleural Effusion
  • However, she finally developed progressive disease with enlarged left lung lesion and increased left pleural effusion, achieving a progression-free survival of 3 months.[dovepress.com]
Anterior Mediastinal Mass
  • Chest radiograph and computed tomography of thorax revealed an anterior mediastinal mass that was subjected to ultrasound-guided fine-needle aspiration cytology.[jcytol.org]
Normocytic Anemia
  • The laboratory examination revealed only a mild normocytic anemia. The serum carcinoembryonic antigen (CEA) was 2.3 ng/mL. Bronchoscopy was performed and was normal, without evidence of an intrabronchial lesion.[onlinelibrary.wiley.com]

Treatment

  • The world literature was reviewed with regard to the use of adjuvant chemotherapy in the treatment of pulmonary blastoma.[ncbi.nlm.nih.gov]

Prognosis

  • 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.[ncbi.nlm.nih.gov]

Etiology

  • 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.[ncbi.nlm.nih.gov]
  • Etiology Little is known about the pathogenesis and histogenesis of PB. Molecular studies indicate that mesenchymal and epithelial components are derived from a single precursor cell.[orpha.net]
  • In spite of diligent investigations by many competent investigators, we still do not know the ecological niche inhabited by the etiologic agent. We have many tantalizing clues but no definite answers.[books.google.ro]
  • It is a biphasic tumor containing fetal adenocarcinoma (typically of low grade) and primitive mesenchymal stroma. [8], [9] The etiology is still uncertain, but there is strong evidence in the literature showing the correlation between cigarette smoking[ijournalhs.org]
  • […] described by Manivel et al. in 1988. [7] The familial type of the tumor is seen in familial tumor predisposition syndrome, also known as DICER1-PPB or DICER1 tumor predisposition syndrome. [1] Germline mutation in the DICER1 gene has been considered a major etiologic[ccij-online.org]

Epidemiology

  • Given the small number of cases and recent re-classification, interpreting the published epidemiology and clinical features of this disease is difficult.[ncbi.nlm.nih.gov]
  • Pulmonary embryoma Definitions PB monophasic type (well-differentiated fetal adenocarcinoma) Glandular component recapitulates embryonic development of lung PB biphasic type Composed of epithelial glandular component and sarcomatous elements CLINICAL ISSUES Epidemiology[basicmedicalkey.com]
  • Summary Epidemiology PB is a rare tumor, accounting for less than 0.25% of all primary malignant lung tumors, with only around 350 cases having been reported in the literature so far.[orpha.net]
  • Types type 1 pleuropulmonary blastoma (purely cystic PPB) type 2 Pleuropulmonary blastoma (solid and cystic PPB) Epidemiology sporadic pleuropulmonary blastoma familial pleuropulmonary blastoma ( 8636815 ) Macroscopy cystic or solid ( 10870072 ) unilateral[humpath.com]
Sex distribution
Age distribution

Pathophysiology

  • The possible pathophysiological causes of hypoglycemia include increases in secretion of IGF, invasion of the liver by a tumor, disruption of counter regulatory mechanisms as a result of adrenal blockage, increase on consumption of glucose by the tumor[ncbi.nlm.nih.gov]
  • Macklin published further insights into the pathophysiology of spontaneous Macklin's Syndrome occurring from a severe asthmatic attack.[findzebra.com]

Prevention

  • 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[ncbi.nlm.nih.gov]
  • Every attempt should be made to remove completely all cystic remnants to prevent reccurence of PPB. In case of reccurence, Type I recurrences frequently progress to more serious Type II or III disease.[telemedicina.med.muni.cz]
  • How can Pulmonary Blastoma be Prevented? Currently, there is no known prevention method for Pulmonary Blastoma.[dovemed.com]
  • SPN Prevention Avoiding the possible causes may help prevent SPN from forming.[webmd.com]

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