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Blastoma

Embryonal Tumor


Presentation

  • The baby in this case like most reported cases presented with a picture of pulmonary infection and respiratory difficulty.[ncbi.nlm.nih.gov]
  • In a child with blastoma, the condition is usually present at birth. Certain syndromes and inherited conditions can make specific types of blastoma more likely.[medicalnewstoday.com]
  • Various degrees of anaplasia were present in 68% of patients ( Table 3 ).[ncbi.nlm.nih.gov]
  • The median age for the diagnosis of neuroblastoma is 22 months, being rare in adolescence and adulthood but showing poor prognosis when it does present in those age groups.[en.wikipedia.org]
  • (CWS). ( 28103635 ) Sparber-Sauer M...CWS Study Group 2017 42 It's Rare So Be Aware: Pleuropulmonary Blastoma Mimicking Congenital Pulmonary Airway Malformation. ( 28405541 ) Haider F...Al-Hashimi H 2017 43 A Case Report of Pleuropulmonary Blastoma Presenting[malacards.org]
Fatigue
  • […] high fatigue shall take a Head Chopper for sure.[steamcommunity.com]
  • The most common signs used for diagnosis are abdominal distention and discomfort, generalized fatigue, loss of appetite and secondary anemia.[en.wikipedia.org]
Anemia
  • The most common signs used for diagnosis are abdominal distention and discomfort, generalized fatigue, loss of appetite and secondary anemia.[en.wikipedia.org]
Pleural Effusion
  • Pleural effusion was found in 77% of the cases (85/111) with available data; pneumothorax at diagnosis was more common in cystic/solid type II patients (40/62 or 65%) versus type II/III patients and type III patients (6/30 or 20%).[ncbi.nlm.nih.gov]
Aspiration
  • Malformation Type IV. ( 27562209 ) Dehner LP...Hill DA 2017 38 Reply to the Letter to the Editor 'Type I Pleuropulmonary Blastoma versus Congenital Pulmonary Airway Malformation Type IV'. ( 27577236 ) Lamas-Pinheiro R...Henriques-Coelho T 2017 39 Fine needle aspiration[malacards.org]
Strabismus
  • The incidence is of one case per every 15,000 to 20,000 live births, and some of the most common symptoms of this disease are leukocoria and strabismus, iris rubeosis, hypopyon, hyphema, buphtalmia, orbital cellullites and exophthalmia.[en.wikipedia.org]
Withdrawn
  • Messinger and Children’s Hospitals of Minnesota had a patent application in his name for methods and kits to detect the DICER mutation; it was withdrawn on July 3, 2014.[ncbi.nlm.nih.gov]

Workup

  • The diagnostic workup requires computed tomography of the chest, and interrogation for associated diseases such as CN is recommended.29 Because metastatic disease has never been found with type I PPB, there is no need for metastatic workup.[ncbi.nlm.nih.gov]
Mediastinal Shift
  • Chest X-ray showed an opacity filling the right lower lobe and leading to mediastinal shift to the opposite side. Computerized Tomography (CT) of thorax revealed a large mass in the right hemithorax (7x5x3cm) containing solid and cystic components.[ncbi.nlm.nih.gov]
  • shift, most respond to early surgery and/or chemotherapy with dramatic improvement ( Supporting Fig. 4 [see online supporting information ]).[ncbi.nlm.nih.gov]
Pleural Effusion
  • Pleural effusion was found in 77% of the cases (85/111) with available data; pneumothorax at diagnosis was more common in cystic/solid type II patients (40/62 or 65%) versus type II/III patients and type III patients (6/30 or 20%).[ncbi.nlm.nih.gov]

Treatment

  • Treatment Treatment for blastoma is often effective and there is a high survival rate for many types. Blastomas respond well to treatment, and doctors consider them curable.[medicalnewstoday.com]
  • "Pediatric hepatoblastoma: diagnosis and treatment". Translational Pediatrics. 3 (4): 293–9. doi : 10.3978/j.issn.2224-4336.2014.09.01. PMC 4728840. PMID 26835349. Gilbertson RJ (April 2004). "Medulloblastoma: signalling a change in treatment".[en.wikipedia.org]
  • Though radiotherapy is an alternative treatment modality, especially in cases with high risk of recurrence, it causes severe morbidity, especially in younger children.[ncbi.nlm.nih.gov]
  • High-dose chemotherapy and stem cell rescue were used as part of the primary treatment in only 8 cases (3%).[ncbi.nlm.nih.gov]
  • 1 Novel intronic DICER1 variation associated with pleuropulmonary blastoma in two siblings. ( 30665929 ) 2019 2 Successful treatment of metastatic cerebral recurrence of pleuropulmonary blastoma. ( 30677214 ) Nakano Y...Hara J 2019 3 Clinical analysis[malacards.org]

Prognosis

  • However, the mutation which allows proliferation of incompletely differentiated cells can vary from patient to patient and a mutation can alter the prognosis.[en.wikipedia.org]
  • Patients with pleural, mediastinal or extra pulmonary involvement at the time of diagnosis have worse prognosis than those without such involvement [ 4, 13 ].[ncbi.nlm.nih.gov]
  • PPB can progress from the purely cystic, curable Type I to a high-grade Type III having a dismal prognosis. We sought to determine the frequency of DICER1 mutation in the largest cohort of PPB patients to date.[ascopubs.org]
  • Conclusion Cystic type I/Ir PPB has a better prognosis than type II, and type II has a better outcome than type III.[ncbi.nlm.nih.gov]

Epidemiology

  • ., c Division of Cancer Epidemiology and Genetics, National Cancer Institute, Bethesda, Md., d Westat, Rockville, Md., and e Department of Pathology, Children's National Medical Center, Washington, D.C., USA Abstract Get article FullText PDF Login / Register[karger.com]
  • Acknowledgments FUNDING SUPPORT This work was supported by the Division of Cancer Epidemiology and Genetics of the National Cancer Institute’s Intramural Research Program.[ncbi.nlm.nih.gov]
Sex distribution
Age distribution

Prevention

  • Prevention It is not possible to prevent blastomas. While having certain inherited syndromes may increase the risk of certain blastomas, these links are not well understood, and it is not currently possible to prevent these syndromes occurring.[medicalnewstoday.com]
  • As complete tumor ablation is essential to prevent local recurrence and allow any chance of survival, the main goal of therapy should be radical surgery, followed by chemotherapy.[ncbi.nlm.nih.gov]
  • Whether the use of chemotherapy can prevent progression remains unresolved.[ncbi.nlm.nih.gov]

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