British Journal of Radiology (2003) 76, 921-922
© 2003 British Institute of Radiology
doi: 10.1259/bjr/98434386
An unusual cause of respiratory symptoms in a toddler
M C Casey, FFR, RCSI
1
D Rafferty, MRCPI
2 and
M O'Neill, FFR, RCSI
1
Departments of 1 Radiology and 2 Paediatrics, Cork University Hospital, Cork, Ireland
A previously well 20-month-old girl was referred to hospital with a 1 day history of respiratory symptoms. She was tachypnoeic and on physical examination she was apyrexial with reduced air entry on the right side. Oxygen saturation was 94% in room air.
A chest radiograph was obtained on admission (Figure 1
). This merited chest drain insertion and a subsequent chest radiograph (Figure 2) and CT of the thorax (Figure 3) were performed.
What does the initial chest radiograph (Figure 1
) show? What is the cause of the symptoms? What does the second chest radiograph (Figure 2) and the CT of thorax (Figure 3) show? What is the diagnosis?
The initial chest radiograph (Figure 1) demonstrated an almost complete right-sided pneumothorax with mediastinal shift to the contralateral side with some bullae at the right base. Appearances are consistent with tension pneumothorax but inconsistent with patient's wellbeing. Repeat chest radiograph performed post chest drain insertion demonstrates re-inflation of the affected lung with right basal bullae. The CT scan (Figure 3) demonstrated right basal bullae with a solid component posteriorly. The chest drain was removed but the bullae re-expanded which merited re-insertion of chest drain. The bullae were surgically excised and a follow-up chest radiograph was performed post thoractomy (Figure 4) which demonstrates complete re-inflation of the right lung.
Histology of the bullae revealed features consistent with pleuro-pulmonary blastoma-type 2. The patient was referred to the care of a paediatric oncologist. She underwent wedge resection, a course of chemotherapy and subsequent right lower lobectomy. Follow-up imaging of thorax, liver and brain were normal.
Pleuro-pulmonary blastoma is a rare aggressive malignancy confined solely to childhood and was first described by Barrett and Barnard [1]. In 25% of cases, there is extrapulmonary involvement [2]. There are three subtypes, with type 1 being multicystic and the better prognosis, type 2 (this case) having grossly visible cysts with solid components and type 3 being exclusively solid [3]. There is a previous family history of childhood neoplasm in 25% of cases. In this case described, there is no relevant family history.
Although local recurrence is common, metastases more frequently occur to the brain, liver and bone [4]. Unfortunately recurrence and metastases usually present with more anaplastic sarcomatous tissue and a higher proportion of purely blastematous elements [4]. Prognosis in this case remains guarded as local recurrence or metastatic disease [4] occur in more than 50% of cases [5].
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Acknowledgments
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We would like to thank Professor Peter Kearney, Professor of Paediatrics and Consultant Paediatrician for his permission to use this case.
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Footnotes
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Current address for M C Casey, Department of Radiology, Montreal General Hospital, 1650 Cedar Avenue, Montreal, H3G 1A4 Quebec, Canada. 
Received for publication March 27, 2003.
Accepted for publication April 24, 2003.
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References
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- Barrett NR, Barnard WG. Some unusual thoracic tumours. Br J Surg 1944;32:447–57.
- Kelsey AM, McNally K, Birch J, Mitchell ELD. Case of extra pulmonary, pleuro-pulmonary blastoma in a child: pathological and cytogenic findings. Med Paediat Oncol 1997;29:61–4.[CrossRef]
- Dehner LP. Pleuropulmonary blastoma is THE pulmonary blastoma of childhood. Semin Diagn Path 1994;11:144–51.
- Schmaltz C, et al. Pleuro-pulmonary blastoma: a case report and review of the literature. Med Paediat Oncol 1995;25:479–84.
- Cohen MC, Kaschula ROC. Primary pulmonary tumours in childhood: a review of 31 years' experience and the literature. Pediatric Pulmonology 1992;140:222–32.
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