Syndromic presentation of a pleuropulmonary blastoma associated with congenital cystic adenomatoid malformation. A case report.
Pleuropulmonary blastoma (PPB) is a rare malignant mesenchymal pediatric tumor with a well-recognized association with congenital cystic adenomatoid malformation (CCAM). Recently, it has been described in a patient with CCAM, multiple jejunal polyps, and cystic nephroma. We describe a similar case of a unique presentation of PPB, arising in association with CCAM and with a history of intussception caused by multiple small bowel polyps.
Nur
Samina
S
Department of Pathology, New York Medical College, Westchester Medical Center, Valhalla, NY 10595, USA. sam_nur@yahoo.com
Badr
Riem
R
Sandoval
Claudio
C
Brudniki
Adele
A
Yeh
Albert
A
D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001932:Brain Neoplasms / Q000473:pathology / Q000556:secondary / Q000628:therapy; D003131:Combined Modality Therapy; D016371:Cranial Irradiation; D015615:Cystic Adenomatoid Malformation of Lung, Congenital / Q000150:complications* / Q000601:surgery; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D007077:Ileal Diseases / Q000209:etiology; D007231:Infant, Newborn; D007443:Intussusception / Q000209:etiology; D007579:Jejunal Diseases / Q000209:etiology; D008175:Lung Neoplasms / Q000150:complications / Q000473:pathology* / Q000601:surgery; D008297:Male; D008637:Mesenchymoma / Q000150:complications / Q000473:pathology* / Q000556:secondary / Q000628:therapy; D009778:Occipital Lobe / Q000473:pathology / Q000601:surgery; D010296:Parietal Lobe / Q000473:pathology / Q000601:surgery; D010580:Peutz-Jeghers Syndrome / Q000150:complications / Q000473:pathology* / Q000601:surgery; D011013:Pneumonectomy; D012120:Respiration Disorders / Q000209:etiology*; D016879:Salvage Therapy