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Ann Thorac Surg 2001;72:604-605
© 2001 The Society of Thoracic Surgeons

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Case report

Pleuropulmonary blastoma: is prophylactic resection of congenital lung cysts effective?

^a Department of Thoracic Surgery, Royal Brompton Hospital, London, England, United Kingdom
^b Department of Pathology, Royal Brompton Hospital, London, England, United Kingdom
^c Department of Pediatrics, Royal Brompton Hospital, London, England, United Kingdom

Accepted for publication June 28, 2000.

Address reprint requests to Dr Goldstraw, Royal Brompton Hospital, Sydney St, SW3 6NP, London, UK
e-mail: pgoldstraw{at}rbh.nthames.nhs.uk

	Abstract

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Pleuropulmonary blastomas (PPB) are rare intrathoracic tumors that can develop in children with preexisting pulmonary cystic lesions, inferring that the prophylactic resection of such cysts might prevent the onset of these malignant tumors. We report a patient who went on to develop PPB in the right lung despite having had resections for bilateral congenital pulmonary cysts 23 months earlier. We therefore question the effectiveness of prophylactic resection of congenital lung cysts on this basis.

	Introduction

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Pleuropulmonary blastomas are rare pediatric malignancies. However, they may associate with congenital lung cysts. The current article reports a case with such an association. The effectiveness of prophylactic resection of congenital lung cysts is therefore questioned by analyzing similar cases from the literature.

A 2.5-year-old boy was referred to our Department by our pediatricians. Eighteen days after birth, he developed two episodes of left-sided pneumothorax for which he was treated with intercostal drains. He was well until 7 months of age when he represented with bilateral pneumothoraces, which were treated initially with intercostal drains. A chest radiograph and computerized chest tomogram revealed a cystic lesion in the left upper lobe and right basal consolidation suggestive of sequestration. We undertook a left thoracotomy and found a cyst measuring 10 cm in diameter adherent to the mediastinum and the anterior segment of the upper lobe and lingula. The cyst was dissected free and excised. Histology reported a benign bronchogenic cyst.

On the first postoperative day, the patient developed a further right pneumothorax that did not resolve with intercostal drainage. A further computerized chest tomogram suggested a cystic lesion in the right upper lobe. At right thoracotomy, we found a large floppy air cyst arising from the apex of the upper lobe. The cyst was excised completely. Histology reported a pleural cyst showing fibrous thickening. He was discharged 5 days later with a normal chest radiograph and continued to thrive.

He was readmitted, aged 2.5 years, with severe respiratory distress requiring intubation and ventilation. The chest radiograph and computerized chest tomogram revealed a complex solid and cystic mass occupying most of the right hemithorax, compressing the right lung against the mediastinum, appearances highly suggestive of neoplastic disease.

At thoracotomy, a small pleural effusion was found. There was a soft, lobulated mass measuring 15 x 14 x 2 cm and weighing 776 g at excision. It arose from the confluence of the fissures supplied by small branches of the pulmonary artery and draining through a large intersegmental vein to the superior pulmonary vein. By shaving the mass from the lung parenchyma, all three lobes were salvaged and the mass was excised completely. The lung appeared normal except for the area of the previous cyst resection at the apex, where there were dense adhesions and scar tissue.

Microsections showed a malignant tumor composed of spindle-shaped cells. The cytoplasm was ill defined, with numerous mitoses and necrosis. Large number of malignant giant cells were evident. The cytoplasm of the cells was indicating smooth muscle differentiation, with cross striations also suggesting skeletal muscle differentiation. Malignant cartilage formation was also present.

He had an uncomplicated postoperative recovery and was then referred for chemotherapy, which was completed successfully. At his last follow-up, 6 months after surgery and 4 months after chemotherapy, he had no evidence of recurrence.

	Comment

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Pulmonary blastoma (PB) was first described in 1945 as an embryoma of the lung [1]. It is extremely rare and accounts for less than 1% of all primary malignant lung tumors in the pediatric population [2, 3]. Manivel and associates [4] coined the term PPB to describe a specific subtype of PB seen in young patients. Denher and associates classified PPB into three groups: type 1 with purely cystic tumors, type 3 with predominantly solid tumors, and type 2 as an intermediate type [5]. Histologically, such tumors are malignant and of mesenchymal appearance.

PPB may be associated with cystic pulmonary lesions, which may be evident at the time of diagnosis or predate the appearance of the tumor, inferring that the prophylactic resection of congenital pulmonary cysts might protect patients from developing PPB.

Support of such policy comes from the reports of 7 patients who were noted to have pulmonary cystic lesions and later developed PPB in the same region of the lung (Table 1). All these patients underwent resection, 6 by lobectomy and 1 by local excision of a subpleural tumor. Unfortunately, histological details were not available in all cases but all were diagnosed as PPB. In 6 of the 7 patients, tumor developed in the area of the cysts but details are lacking as to the precise relationship of the tumor to the cyst at surgery. One patient developed a subpleural mass in the same hemithorax in which the lung cysts were observed.

Our patient had bilateral lung cysts. Histologically, the bronchogenic cyst at the left was shown to contain areas with a hint of immature cartilage formation that could represent blastoma lining the cystic wall (Fig 1). The cyst at the right appeared bland and mesothelial. However, the tumor resected 23 months later was in the right lung and anatomically distinct from the area of the previous cyst.

View larger version (141K): [in this window] [in a new window]   Fig 1. Hematoxylin and eosin sections of left lung cyst wall, showing lining of low cuboidal epithelium with mesenchyme in the wall consisting of uniform, small spindle cells with no mitoses, pleiomorphism, or necrosis.

	References

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1. Barnard W.G. Embryoma of lung. Thorac 1952;7:299-301.
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3. Domizio P., Liesner R.J., Dicks-Mireaux C., Risdon R.A. Malignant mesenchymal associated with a congenital lung cyst in a child: case report and review of the literature. Pediatr Path 1990;10:785-797.
4. Manivel J.C., Priest J.R., Watterson J., et al. Pleuropulmonary blastoma. The so-called pulmonary blastoma in childhood. Cancer 1988;62:1516-1526.[Medline]
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This Article Abstract Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Konstantinos A. Papagiannopoulos Peter Goldstraw Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Papagiannopoulos, K. A. Articles by Goldstraw, P. Search for Related Content PubMed PubMed Citation Articles by Papagiannopoulos, K. A. Articles by Goldstraw, P. Related Collections Lung - other