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Radical resection of pleuropulmonary blastoma
Mohammad Akbar Bhat1, Shabir Hussain Shah1
1Department of Cardiovascular and Thoracic Surgery, Sheri-Kashmir Institute of Medical Sciences, Srinagar, Jammu and Kashmir, India.
Insights
Pleuropulmonary blastoma (PPB), a rare childhood lung cancer, requires surgical removal and chemotherapy. This case highlights successful treatment of type II PPB in a 5-year-old boy.
Area of Science:
- Pediatric Oncology
- Thoracic Surgery
- Rare Diseases
Background:
- Pleuropulmonary blastoma (PPB) is a rare intrathoracic neoplasm in early childhood.
- It typically affects the lung or visceral pleura.
Observation:
- A 5-year-old boy presented with cough, chest pain, and dyspnea.
- Imaging revealed a large mass in the left hemithorax.
- Previous surgery for a congenital lung cyst was noted.
Findings:
- CT-guided fine needle aspiration cytology was inconclusive.
- En bloc resection of the mass, left lower lobe, ribs, and diaphragm was performed.
- Histopathology confirmed type II Pleuropulmonary blastoma.
Implications:
- Radical tumor resection and adjuvant chemotherapy are crucial for salvaging patients with type II or III PPB.
- This case demonstrates a successful multimodal treatment approach.
- Long-term follow-up is essential for pediatric patients with PPB.
Abstract:
Pleuropulmonary blastoma (PPB) is a rare intrathoracic neoplasm of early childhood that occurs in the lung or visceral pleura. We present a case of a 5-year-old boy with a cough, left-sided chest pain and progressive dyspnea on exertion. The patient had had an operation for a congenital lung cyst of the left lower lobe, six months prior to the present admission. A CT showed a heterogeneously enhancing mass lesion filling the left hemithorax. CT-guided fine needle aspiration cytology of the mass was inconclusive. En bloc resection of the mass along with left lower lobe, the involved ribs and left diaphragm was performed. The diaphragmatic defect was repaired using prolene mesh. Histopathology of the resected specimen showed solid and cystic areas consistent with type II PPB. The patient received adjuvent chemotherapy and was doing well 23 months after surgery. Radical resection of the tumor followed by adjuvent chemotherapy provides the only hope of salvaging cases of type II or type III PPB.
