Type I Pleuropulmonary Blastoma versus Congenital Pulmonary Airway Malformation Type IV

Dehner, Louis P.; Messinger, Yoav; Williams, Gretchen M.; Stewart, Douglas R.; Harney, Laura A.; Schultz, Kris Ann P.; Hill, D. Ashley

doi:10.1159/000447992

Cited by 20 publications

(8 citation statements)

References 8 publications

(7 reference statements)

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“…In addition, type 1 PPBs are more likely to be multifocal than are other congenital cysts (11). Finally, data have suggested that CPAMs categorized according to the Stocker classification as histologic type 4 and PPB type 1 are in the same spectrum of pathologic entities (19,20). The differential diagnosis for types 2 and 3 PPB, particularly when they are locally aggressive and when the primary lung origin is not definitive, includes more common tumors such as neuroblastomas.…”

Section: Pleuropulmonary Blastomamentioning

confidence: 99%

Primary Lung Tumors in Children: Radiologic-Pathologic CorrelationFrom the Radiologic Pathology Archives

Lichtenberger¹,

Biko²,

Carter³

et al. 2018

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Primary lung tumors in children are rare, with a narrow range of diagnostic considerations. However, the overlapping imaging appearances of these tumors necessitate attention to key discriminating imaging and pathologic features. In the neonate and infant, the important considerations include pleuropulmonary blastoma (PPB), infantile fibrosarcoma, and fetal lung interstitial tumor. Among these tumors, imaging findings such as air-filled cysts in type 1 PPB and homogeneously low attenuation of fetal lung interstitial tumors are relatively specific. Key pathologic and genetic discriminators among this group of tumors include the DICER1 germline mutation found in PPB and the t(12,15)(p13;q25) translocation and ETV6-NTRK3 fusion gene seen in infantile fibrosarcoma. Primary lung tumors in older children include inflammatory myofibroblastic tumors (IMTs), carcinoid salivary gland-type tumors of the lung, recurrent respiratory papillomatosis, and other rare entities. IMT, a spindle-cell proliferation with inflammatory elements, is the most common lung tumor in children. Anaplastic lymphoma kinase, a receptor-type protein tyrosine kinase, is present in 50% of these tumors, and this finding may support an imaging diagnosis of IMT. Carcinoid tumors account for a substantial portion of childhood lung tumors, and their characteristic avid enhancement on images corresponds to the compressed fibrovascular stroma histologically. Furthermore, novel imaging agents used with somatostatin receptor analogs have an emerging role in the evaluation of carcinoid tumors. Although less common than mucoepidermoid carcinoma, adenoid cystic carcinoma tends to recur given the perineural spread seen histologically. Integrating radiologic and pathologic knowledge is critical to accurate diagnosis, treatment planning, and surveillance of primary lung tumors in children.Abbreviations: CPAM = congenital pulmonary airway malformation, FDG = fluorodeoxyglucose, FLIT = fetal lung interstitial tumor, GLUT1 = glucose transporter 1, IMT = inflammatory myofibroblastic tumor, NUT = nuclear protein in testis, PPB = pleuropulmonary blastoma, RRP = recurrent respiratory papillomatosis

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Section: Pleuropulmonary Blastomamentioning

confidence: 99%

Primary Lung Tumors in Children: Radiologic-Pathologic CorrelationFrom the Radiologic Pathology Archives

Lichtenberger¹,

Biko²,

Carter³

et al. 2018

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“…In a recent controversy on this subject, Dehner et al [32] argued that CPAM 4 is identical to PPB-I, and that there is a possibility that these lesions evolve from pure cystic into partially cystic-semisolid and finally into the solid type III PPB. In response, Lamas-Pinheiro et al [33] argued that YY1 and FGF10 are differently expressed in CPAM and PPB I (downregulated in PPB).…”

Section: Discussionmentioning

confidence: 99%

Pleuropulmonary blastoma type I might arise in congenital pulmonary airway malformation type 4 by acquiring a Dicer 1 mutation

et al. 2020

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Congenital pulmonary airway malformation (CPAM) occurs most commonly in infants. It is divided into 5 types. The most common types 1 and 2 are cystic, type 0 presents as bronchial buds without alveolar tissue, most likely corresponding to alveolar dysgenesis, while type 3 is composed of branching bronchioles and appears as a solid lesion. A defect in the epithelialmesenchymal crosstalk might be the underlying mechanism for all. Type 4 is a peripheral cystic lesion with a thin cyst wall covered by pneumocytes. CPAM 4 has been mixed up with pleuropulmonary blastoma (PPB) type I and some authors question its existence. We investigated five cases of CPAM type 4 for the presence or absence of rhabdomyoblasts, and for markers associated with CPAM development. In addition, all cases were evaluated for mutations within the Dicer gene and for mutations of the RAS family of oncogenes. All five cases showed smooth muscle actin and desmin-positive cells; however, only one case showed a few cells positive for MyoD. The same case showed a mutation of Dicer 1. All cases were negative for mutations of the RAS family of genes. Fibroblast growth factor 10 was similarly expressed in all cases, and thus cannot be used to differentiate CPAM4 from PPB-I. Low expression of the proliferation marker Ki67 was seen in our CPAM 4 cases and the probable PPB-I case. YingYang-1 protein seems to play an active role in the development of PPB-I. CPAM 4 can be separated from PPB-I based on the presence of rhabdomyoblasts and mutations in Dicer 1 gene. These cells might not be numerous; therefore, all available tissue has to be evaluated. As CPAM 4 morphologically looks very similar to PPB-I, it might be speculated, that there exists a potential for progression from CPAM 4 to PPB-I, by acquiring somatic mutations in Dicer 1.

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“…PPB is the most common primary malignancy of the lungs in childhood . More than 65% of these patients have germline DICER1 mutations.…”

Section: Discussionmentioning

confidence: 99%

“…PPB is the most common primary malignancy of the lungs in childhood. [1][2][3][4] More than 65% of these patients have germline DICER1 mutations. Many PPBs may initially manifest as pulmonary multilocular cysts with subsequent progression.…”

Section: Discussionmentioning

confidence: 99%

Clinical analysis of early death in children with pleuropulmonary blastoma in a single center in China

Chai

Zhang

et al. 2019

Pediatric Blood & Cancer

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Objective Pleuropulmonary blastoma (PPB) is a rare malignant tumor in childhood that is highly invasive and has poor prognosis. We retrospectively analyzed patients with PPB who died within 30 days in hopes of providing a basis for improving diagnosis and treatment. Methods We retrospectively reviewed six children with PPB who died within 30 days of admission at our hospital from January 2004 to March 2018, including their clinical features, pathological diagnosis, course of treatment, and major causes of death. Results Six patients (two female, four male; median age, 38 months) were included. All patients presented with respiratory symptoms. Chest imaging showed that all tumors had diameters greater than 10 cm, with varying degrees of serous effusion. Four patients underwent ultrasound‐guided fine‐needle aspiration (FNA), one patient underwent exploratory thoracotomy, and one underwent partial tumor resection. Five cases were type III, and another was type II. Four patients developed adverse events while waiting for pathological results after FNA, and four patients were treated with chemotherapy but their tumors failed to decrease in size one to two weeks later. The median hospitalization to death time was 17 days (range, 5–24 days). Conclusions PPB often presents with respiratory symptoms that rapidly develop into respiratory distress. The rapid tumor enlargement contributes to the disease's progression. Chemoreduction in such tumors is not obviously effective, and the mortality rate is high. The main causes of death were respiratory failure and sepsis. Further clinical studies will be required to evaluate the role of initial biopsy compared with upfront total excision.

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Type I Pleuropulmonary Blastoma versus Congenital Pulmonary Airway Malformation Type IV

Cited by 20 publications

References 8 publications

Primary Lung Tumors in Children: Radiologic-Pathologic CorrelationFrom the Radiologic Pathology Archives

Primary Lung Tumors in Children: Radiologic-Pathologic CorrelationFrom the Radiologic Pathology Archives

Pleuropulmonary blastoma type I might arise in congenital pulmonary airway malformation type 4 by acquiring a Dicer 1 mutation

Clinical analysis of early death in children with pleuropulmonary blastoma in a single center in China

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