Prenatal presentation and outcome of children with pleuropulmonary blastoma

Miniati, Douglas N.; Chintagumpala, Murali; Langston, Claire; Dishop, Megan K.; Olutoye, Oluyinka O.; Nuchtern, Jed G.; Cass, Darrell L.

doi:10.1016/j.jpedsurg.2005.10.074

Cited by 108 publications

(63 citation statements)

References 22 publications

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“…In a recent IPPBR report, 22 of 51 IPPBR Type I cases (43%) presented with pneumothorax. 47 Type I PPB may also be discovered incidentally by in utero ultrasound 6,58 or a later chest radiograph. Type I PPB is most often a multilocular cyst located in peripheral lung adjacent to or involving visceral pleura.…”

Section: Pleuropulmonary Blastoma (Ppb)mentioning

confidence: 99%

“…5 Several of the gonadal tumors occurred in adults age 20-40 years. 6 Age at diagnosis 37 years. 7 Age at diagnosis 33 years.…”

Section: Comparative Incidence Of Developmental Lung Cysts and Pleuromentioning

confidence: 99%

“…3,4 For some physicians, cyst size and the possibility of malignancy are indications for removal of asymptomatic cysts. 2,5,6 The most common malignancy associated with early childhood cysts is pleuropulmonary blastoma (PPB), which in its earliest manifestation is purely cystic, occurring in most cases from birth through age 2 years and occasionally noted in the prenatal period. Cystic PPB is indistinguishable from non-neoplastic cysts in both clinical presentation and imaging features (Fig.…”

Section: Introductionmentioning

confidence: 99%

“…We review the current knowledge of how to assess the risk of malignancy and suggest guidelines for managing cysts. 2,5,6,9 THE MANAGEMENT CONTROVERSY With the development of more sophisticated imaging, congenital cystic lung lesions are being detected more frequently than in the past. This is especially true in the fetus because of the near ubiquity of prenatal ultrasound.…”

Section: Introductionmentioning

confidence: 99%

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Pulmonary cysts in early childhood and the risk of malignancy

Priest

Williams

Hill

et al. 2008

Pediatric Pulmonology

272

215

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Summary. Surgery for congenital and early childhood lung cysts is often dictated by symptoms such as respiratory distress, infection or pneumothorax. Asymptomatic cysts present a therapeutic dilemma: surgical intervention and ''conservative'' observation have advocates. The risk of malignancy in such cysts is considered by some an indication for surgical intervention and is reviewed in this paper. Pleuropulmonary blastoma (PPB) is the most frequent malignancy associated with childhood lung cysts. Although rare, PPB occurs predictably in certain clinical and familial situations. This unique biology of PPB can inform the cyst management decision. The earliest manifestation of PPB is a malignant lung cyst in young children, clinically and radiographically indistinguishable from benign congenital lung cysts. Histopathologic examination differentiates cystic PPB from the benign cystic variants. Surgical excision of cystic PPB (with or without chemotherapy) cures approximately 85-90% of children. If not excised, cystic PPB evolves to cystic/solid or solid high-grade sarcoma (cure rate 45-60%) by age 2-6 years. Numerous reports of ''malignancy in a congenital lung cyst'' are now understood as the characteristic progression of cystic PPB. PPB is genetically determined in many cases. Detailed family history may reveal the hallmarks of PPB in the patient or young relatives: a unique constellation of diseases including lung cysts, cystic nephroma, childhood cancers, stromal sex-chord ovarian tumors, seminomas or dysgerminomas, intestinal polyps, thyroid hyperplasias, and hamartomas. Pneumothorax and multifocal/bilateral lung cysts also characterize PPB. These diagnoses predict that a lung cyst is more likely PPB than a benign congenital cyst. Patients fitting this pattern deserve histologic diagnosis. The genetic basis for this heritable syndrome is unknown but is being actively investigated.

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Section: Pleuropulmonary Blastoma (Ppb)mentioning

confidence: 99%

“…5 Several of the gonadal tumors occurred in adults age 20-40 years. 6 Age at diagnosis 37 years. 7 Age at diagnosis 33 years.…”

Section: Comparative Incidence Of Developmental Lung Cysts and Pleuromentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Pulmonary cysts in early childhood and the risk of malignancy

Priest

Williams

Hill

et al. 2008

Pediatric Pulmonology

272

215

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“…Type 2 and type 3 PPB metastasize to the brain, liver and the bone in 30% of cases while type 1 PPB rarely metastasizes [7].…”

Section: Discussionmentioning

confidence: 99%

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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Accuracy of Chest Computed Tomography in Distinguishing Cystic Pleuropulmonary Blastoma From Benign Congenital Lung Malformations in Children

et al. 2022

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IMPORTANCEThe ability of computed tomography (CT) to distinguish between benign congenital lung malformations and malignant cystic pleuropulmonary blastomas (PPBs) is unclear.OBJECTIVE To assess whether chest CT can detect malignant tumors among postnatally detected lung lesions in children. DESIGN, SETTING, AND PARTICIPANTSThis retrospective multicenter case-control study used a consortium database of 521 pathologically confirmed primary lung lesions from January 1, 2009, through December 31, 2015, to assess diagnostic accuracy. Preoperative CT scans of children with cystic PPB (cases) were selected and age-matched with CT scans from patients with postnatally detected congenital lung malformations (controls). Statistical analysis was performed from January 18 to September 6, 2020. Preoperative CT scans were interpreted independently by 9 experienced pediatric radiologists in a blinded fashion and analyzed from January 24, 2019, to September 6, 2020.MAIN OUTCOMES AND MEASURES Accuracy, sensitivity, and specificity of CT in correctly identifying children with malignant tumors. RESULTS Among 477 CT scans identified (282 boys [59%]; median age at CT, 3.6 months [IQR, 1.2-7.2 months]; median age at resection, 6.9 months [IQR, 4.2-12.8 months]), 40 cases were extensively reviewed; 9 cases (23%) had pathologically confirmed cystic PPB. The median age at CT was 7.3 months (IQR, 2.9-22.4 months), and median age at resection was 8.7 months (IQR, 5.0-24.4 months). The sensitivity of CT for detecting PPB was 58%, and the specificity was 83%. High suspicion for malignancy correlated with PPB pathology (odds ratio, 13.5; 95% CI, 2.7-67.3; P = .002).There was poor interrater reliability (κ = 0.36 [range, 0.06-0.64]; P < .001) and no significant difference in specific imaging characteristics between PPB and benign cystic lesions. The overall accuracy rate for distinguishing benign vs malignant lesions was 81%. CONCLUSIONS AND RELEVANCEThis study suggests that chest CT, the current criterion standard imaging modality to assess the lung parenchyma, may not accurately and reliably distinguish PPB from benign congenital lung malformations in children. In any cystic lung lesion without a prenatal diagnosis, operative management to confirm pathologic diagnosis is warranted.

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Prenatal presentation and outcome of children with pleuropulmonary blastoma

Cited by 108 publications

References 22 publications

Pulmonary cysts in early childhood and the risk of malignancy

Pulmonary cysts in early childhood and the risk of malignancy

Solid (Type 3) pleuropulmonary blastoma

Accuracy of Chest Computed Tomography in Distinguishing Cystic Pleuropulmonary Blastoma From Benign Congenital Lung Malformations in Children

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