Type 1 pleuropulmonary blastoma in a 3-year-old male with a cystic lung mass

Al-Backer, Nouf; Puligandla, Pramod S.; Su, Wendy; Anselmo, Mark; Laberge, Jean-Martin

doi:10.1016/j.jpedsurg.2006.07.015

Cited by 10 publications

(10 citation statements)

References 9 publications

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“…According to Priest et al a five-year survival rate for type 1 PPB is 83% while for type 2 and type 3 is 42% [4].…”

Section: Discussionmentioning

confidence: 97%

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Solid (Type 3) pleuropulmonary blastoma

Gaxa¹,

Hlatshwayo²,

Motene³

2017

Case Rep Int

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Introduction:Pleuropulmonary blastoma is a rare solid and/or cystic progressive and aggressive malignant intra-thoracic tumor of childhood which belongs to a group of dysontogenetic neoplasms such as Wilms tumor, neuroblastoma and hepatoblastoma. The incidence of pleuropulmonary blastoma ranges between 0.25 and 0.5% of primary lung tumors making this entity a rare primary malignant lung tumor. Case Report: A two-month-old male neonate was referred from a regional hospital with a three-day history of cough, fever and shortness of breath. No previous medical history reported. The patient was delivered at term through a normal vaginal delivery which was uneventful. Birth weight 3 kg, length 48 cm, Apgar score 10/10 and the head circumference was 35 cm. The patient's retro-viral disease status was negative. On percussion there was dullness and bilateral crepitations. There was bilateral decreased air-entry. No signs of cardiac failure. The rest of the systems were within

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“…According to Priest et al a five-year survival rate for type 1 PPB is 83% while for type 2 and type 3 is 42% [4].…”

Section: Discussionmentioning

confidence: 97%

“…Progression of pleuropulmonary blastoma from type 1 to type 3 is speculated as a natural history of PPB and this tumor can occur in the lungs, pleura, mediastinum and the diaphragm [4,5].…”

Section: Discussionmentioning

confidence: 99%

Solid (Type 3) pleuropulmonary blastoma

Gaxa¹,

Hlatshwayo²,

Motene³

2017

Case Rep Int

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“…The histologic components of type III PPB generally include blastematous islands, cartilaginous nodules, rhabdomyoblasts, and anaplastic cells (Priest et al, 2006;Taube et al, 2006). As with cystic PPB, the presence of primitive blastematous cells within the solid tumor diagnoses PPB (Al-Backer, Puligandla, Su, Anselmo, & Laberge, 2006).…”

Section: Pathophysiology and Prognosismentioning

confidence: 99%

“…Unlike type I PPB, both type II and type III PPB may be associated with metastasis, with the brain being the most common metastatic site. PPB may also spread to bones, lymph nodes, liver, pancreas, kidneys, and adrenal glands (Al-Backer et al, 2006;Priest et al, 2007).…”

Section: Pathophysiology and Prognosismentioning

confidence: 99%

Pleuropulmonary Blastoma

Fosdal

2008

J Pediatr Oncol Nurs

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Pleuropulmonary blastoma (PPB) is a dysontogenetic neoplasm of childhood that involves lung and/or pleura. There is an increased incidence of neoplasias and dysplasias among young relatives of children with PPB. Pathophysiologically, PPB evolves from a cystic to solid state over time. It is subclassified as type I (purely cystic), type II (both cystic and solid elements), and type III (completely solid). Type II and type III may be associated with metastasis, with the brain being the most common metastatic site. The absence of epithelial malignancy in PPB is a feature that distinguishes it from the adult-type pulmonary blastoma. The clinical presentation includes signs and symptoms associated with various respiratory disorders. To make a definitive diagnosis of PPB, an examination of the cystic fluid or solid tumor is required. Treatment for PPB consists primarily of surgery and chemotherapy. Nursing care is directed toward maintaining normal respiratory and neurological function, maintaining normal fluid and electrolyte balance, minimizing side effects associated with treatment, and providing education for the family.

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“…Pleuropulmonary blastoma (PPB) is a rare primary pulmonary neoplasm of early childhood arising in the lung or visceral pleura [1]. PPB occurs most commonly in children less than five years of age and differs from adult pulmonary blastoma in its presentation and gross and microscopic features.…”

mentioning

confidence: 99%