[Does Bolande's malignant tumor exist?].
Congenital mesoblastic nephroma (CMN) is a rare renal neoplasm which Bolande differentiated from Wilms tumour given its benign nature. We describe 7 cases of CMN which have been treated over the last ten years, in order to highlight to what extent a tumour which is generally considered to be benign can have an aggressive behaviour. 5 patients were diagnosed during their neonatal period (3 of them prenatally) and 2 after the third month of life. Clinical presentation, simple abdominal radiology, echography and abdominal CT scan, which showed characteristic images, led us to a final diagnosis. Tumoral resection was carried out in the 6 typical CMN. The atypical or aggressive CMN was urgently operated under critical circumstances due to spontaneous tumoral rupture. The pathological study showed a CMN of cellular variety with a sarcomatous component. Despite adjuvant chemotherapy, there was a tumoral recurrence which required a surgical second-look and subsequent chemotherapeutical treatment. Bolande's tumour is generally benign, but there is an aggressive cellular variant that fits into the intermediate zone of a pathological spectrum. This aggressive variant have different clinical, radiological and anatomo-pathological features and therefore it involves radical surgical treatment and additional chemotherapy with a rigorous follow-up in the short and the long term.
Martínez Ibáñez
V
V
Servicio de Oncología Pediátrica, Hospital Universitario Materno-Infantil Vall d'Hebrón Barcelona.
Peiró Ibáñez
J L
JL
Terradas
M
M
Lorenzo
M
M
Enríquez
G
G
Sánchez de Toledo
J
J
Marques
A
A
Boix-Ochoa
J
J
D000005:Abdomen / Q000473:pathology*; D000008:Abdominal Neoplasms / Q000151:congenital / Q000473:pathology* / Q000601:surgery; D003131:Combined Modality Therapy; D004358:Drug Therapy; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007813:Laparotomy; D008297:Male; D009364:Neoplasm Recurrence, Local; D018201:Nephroma, Mesoblastic / Q000151:congenital / Q000473:pathology* / Q000601:surgery
Existe el tumor de Bolande maligno?