Neonatal Pleuropulmonary Blastoma, Type 1

TE Herman and MJ Siegel Department of Radiology, Mallinckrodt Institute of Radiology, St Louis Children’s Hospital, Washington University School of Medicine, St Louis, MO, USA

Journal of Perinatology (2008) 28, 82–84; doi:10.1038/sj.jp.7211863 nature of this association is not understood. But it is widely believed that the lesion arises from congenital lung cysts. Case Unfortunately, the most common cystic lesions of the lung, A 3660 g full-term infant was born after an uncomplicated congenital cystic adenomatoid malformation of the lung and pregnancy. A fetal sonogram at 20 weeks had been normal. bronchogenic cyst, can only histologically be differentiated from The child had Apgars of 7 and 8 at 1 and 5 min respectively. this are cystic lesion with a neoplastic potential. This latter lesion However at approximately 10 min after delivery he became dusky has been given the name cystic mesenchymal hamartoma or type 1 and tachycardic. A chest radiograph was obtained (Figure 1), and PPB. Three types of PPB are now recognized. Type 1 is an entirely subsequently a CT scan (Figures 2 and 3) was obtained. A cystic lesion; type 2 is mixed cystic and solid; and type 3 is entirely multiloculated cystic mass was found to be present in the solid.7,8 The cystic lesion, as occurred in this case, is usually right lower lobe. The patient was taken to the operating room located in the periphery of the lobe in which it arises or attached to where a thoracotomy was performed. the pleura, and in 70% of the cases it occurs in the right lung, particularly the right lower lobe.7,9 It is believed that these three

Denouement and discussion The patient was taken to the operating room where the cystic mass was found to arise from the peripheral basilar portion of the right lower lobe. This was resected without a lobectomy, leaving most of the right lower lobe in place. Histological examination demonstrated hypercellular septae in this cystic mass. There were primitive mesenchymal elements with rare nodules of mature cartilage, consistent with pleuropulmonary blastoma (PPB). Because of this diagnosis, a repeat thoracotomy for complete lower lobectomy was performed. PPB is a rare intrathoracic dysembryonic tumor occurring primarily in children less than 5 years of age, although it has been reported in an adult of 36 years.1,2 The tumor can arise from the lung, pleural or both.3 Histologically, the lesion is different from the adult-type pulmonary blastoma, which contains epithelial elements. PPB does not have the epithelial elements occurring in the adult-type pulmonary blastoma.4 PPB rather has a primitive blastema along with a malignant mesenchymal sarcomatous component, most often embryonal rhabdomyosarcoma or chondrosarcoma.5 Interestingly, however, PPB shares features with pulmonary blastoma and with pulmonary rhabdomyosarcoma an intriguing association with congenital lung cysts.6 The exact

Correspondence: Dr TE Herman, Mallinckrodt Institute of Radiology, Washington University School of Medicine, St Louis Children’s Hospital Department of Radiology, 510 South Kingshighway Blvd, St Louis, MO 63110, USA. E-mail: [email protected] Figure 1 Anteroposterior chest radiograph. Large air-ﬁlled cysts occupy most of Received 28 June 2007; revised 4 September 2007; accepted 5 October 2007 the right lung with shift of the mediastinum toward the left. Neonatal pleuropulmonary blastoma, type 1 TE Herman and MJ Siegel 83

neoplasms, Hodgkin’s disease, leukemia, ovarian teratoma and small bowel polyps.7,9,11 Cystic nephroma is a rare benign renal tumor, which is usually an isolated mass, but in patients with PPB it is often bilateral and multifocal.11 The most common site of metastases from PPB is the central nervous system, especially the brain, but also the orbit and iris.10 Other common sites of metastases include bones, lymph nodes, liver, pancreas, kidney and adrenals. Clinical presentations include respiratory distress especially for large type 3 lesions, fever, chest pain, cough, anorexia, weight loss and spontaneous pneumothorax. Cytogenetically polysomy, especially trisomy 8, is often present. It is interesting that trisomy 8 is also known to be associated with cystic renal masses.5 Some patients have a loss of heterozygosity at 11p15 (WT2 locus).10 Interestingly, type 3 PPB often resembles Wilm’s tumor histologically. The appearance of PPB and its differential diagnosis from imaging studies depends upon the type. Type 1 lesions must be Figure 2 Coronal reformatted chest CT without intravenous contrast showing that the cysts are thin walled. histologically differentiated from cystic adenomatoid malformation and bronchogenic cyst. The management of congenital lung cysts has therefore changed. Lesions thought to be CCAM must be followed to involution or at least yearly reimaged if not removed.12 Type 2 lesions may be characteristic and allow a correct diagnosis since both the cystic and solid components can be visualized. Type 3 is usually a large lesion often opacifying the hemithorax and associated with a pleural effusion. The solid type 3 PPB must be differentiated from other pulmonary tumors of childhood, such as inﬂammatory myoﬁbroblastic tumor, Askin thoracoabdominal primitive neuroectodermal tumor, pulmonary metastases and pulmonary lymphoma.1 The prognosis of PBB is in general poor, with a 5-year survival of only 45%.3 The prognosis is best for type 1, which has a survival of 83%, but only 42% for type 2 and type 3.9 Common sarcoma therapy such as vincristine, adriamycin and cytoxan has not been shown to be particularly effective in improving the prognosis.

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