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[Cystic adenomatoid malformation of the lung: experience in 4 operated cases]
G Cairoli1, S Bertana, M Giuntoli
1Divisione di Chirurgia Pediatrica, Ospedale Maggiore di Bologna, Italia.
Abstract:
The congenital cystic adenomatoid malformation (MACP), represents the congenital lung malformation most frequent in pediatric age. After examining the embryology, the treatment and the evolution of the malformation we wish to report our experience of four cases of MACP in patients 2 month, 1, 2 and 3 years of age. Lobectomy has cured all patients with the exception of one; this child had a MACP of the lung associated with Rhabdomyosarcoma and died after one and half year of chemotherapy.
Insights
Congenital cystic adenomatoid malformation (MACP) is a common pediatric lung issue. Surgical resection (lobectomy) successfully treated most cases, though one patient with associated rhabdomyosarcoma did not survive.
Area of Science:
- Pediatric Surgery
- Thoracic Oncology
- Congenital Malformations
Background:
- Congenital cystic adenomatoid malformation (MACP) is the most frequent congenital lung malformation in children.
- Understanding embryology, treatment, and evolution is crucial for managing MACP.
- This study reports clinical experience with four pediatric MACP cases.
Observation:
- Four pediatric patients diagnosed with MACP presented at ages ranging from 2 months to 3 years.
- Surgical intervention, specifically lobectomy, was performed on all patients.
- One patient presented with a concurrent diagnosis of rhabdomyosarcoma.
Findings:
- Lobectomy resulted in a cure for three out of the four patients.
- The patient with MACP and rhabdomyosarcoma experienced a fatal outcome despite chemotherapy.
- The study highlights the generally favorable prognosis of MACP following surgical treatment.
Implications:
- Early diagnosis and surgical management of MACP are vital for positive patient outcomes.
- The association of MACP with other malignancies, like rhabdomyosarcoma, warrants further investigation.
- This experience underscores the importance of multidisciplinary care in complex pediatric thoracic cases.