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

Abstract

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

Pleuropulmonary blastoma: A report of three cases and review of literature

“…In 2015, World Health Organization classified pleuropulmonary blastoma as distinct from adult pulmonary blastoma and now falls under the category of mesenchymal neoplasms [ 8 ]. It is primarily seen in children less than 6 years age [ 1 ]. According to International Pleuropulmonary Blastoma Registry, it is mainly of 3 types – type I, II, and III (Table 1).…”

“…In recent studies, the malignancy has been recognized as part of DICER1 syndrome or PPB familial tumor and dysplasia syndrome [ 1 , 4 ]. Around 66 % patients with the malignancy have heterozygous mutation in DICER1 gene located on chromosome 14q13.2 [ 5 ].…”

“…Pleuropulmonary blastoma (PPB) is a rare and highly aggressive neoplasm. It was earlier classified with pulmonary blastoma, which constitutes 0.25%-0.50% of primary lung malignancies in all age groups [ 1 ]. PPB is the most common primary lung malignancy in children.…”

Lung ultrasound in children, WFUMB review paper (part 2)

“…Primary lung tumours in children are rare and lung carcinomas in children are extremely rare [43]. Most malignant lesions in children are metastases [44]. In the majority of cases, clinical signs such as cough and radiologic findings including pathologic mass, inflammatory densities, pleural effusion and pneumothorax are nonspecific [45][46][47].…”

Nódulo pulmonar en pediatría: más allá de la infección

“…La epidemiología de las neoplasias malignas primarias de pulmón no es bien conocida, solo se encuentran reportes y series de casos, se estima representan menos del 7% de todos los tumores en pediatría 6 . La frecuencia varía según la edad, comúnmente se han descrito: tumores carcinoides, tumores miofibroblásticos inflamatorios, blastoma pleuroupulmonar y menos frecuente carcinoma de pulmón de células pequeñas, adenocarcinoma, hemangioma pulmonar infantil y carcinoma mucoepidermoide (CME) 7 .…”