An association of pleuropulmonary blastoma and cystic nephroma: possible genetic association.
The association of pleuropulmonary blastoma and cystic nephroma is an uncommon entity, with only 4 cases of such an association in the same patient described in English literature. We report a 5th histologically documented case in a 32-month-old boy. The boy underwent a pulmonary biopsy that showed a pleuropulmonary blastoma and a nephrectomy that showed a cystic nephroma. The pleuropulmonary mass showed an important regression with postbiopsy chemotherapy, allowing subsequent tumorectomy. To date very little is known about this rare entity, and a genetic link between these 2 tumors is hypothesized.
Bouron-Dal Soglio
Dorotheé
D
Department of Pathology, Hôpital Sainte-Justine, 3175 Chemin de la Côte-Ste-Catherine, Montréal, Québec H3T 1C5, Canada.
Harvey
Isabelle
I
Yazbeck
Salam
S
Rypens
Françoise
F
Oligny
Luc L
LL
Fournet
Jean-Christophe
JC
D017024:Chemotherapy, Adjuvant; D002675:Child, Preschool; D020022:Genetic Predisposition to Disease; D006801:Humans; D007680:Kidney Neoplasms / Q000235:genetics* / Q000473:pathology* / Q000628:therapy; D008175:Lung Neoplasms / Q000235:genetics* / Q000473:pathology / Q000628:therapy; D008297:Male; D009378:Neoplasms, Multiple Primary; D009392:Nephrectomy; D018201:Nephroma, Mesoblastic / Q000235:genetics* / Q000473:pathology / Q000628:therapy; D010997:Pleural Neoplasms / Q000235:genetics* / Q000473:pathology / Q000628:therapy; D018202:Pulmonary Blastoma / Q000235:genetics* / Q000473:pathology / Q000628:therapy; D016896:Treatment Outcome