R. Dickerhoff et al., SUCCESSFUL TREATMENT OF A LOCAL RELAPSE I N AN INFANT WITH CONGENITALMESOBLASTIC NEPHROMA, Klinische Padiatrie, 207(2), 1995, pp. 48-51
A 3 1/2 month old girl was found to have a large abdominal tumor origi
nating in the upper pole of the right kidney. At laparotomy the tumor
had infiltrated the perirenal fat, the right lobe of the liver and the
diaphragm. Partial nephrectomy was performed and the tumor was comple
tely resected. However, an adequate safety margin could not be achieve
d. Histology showed a congenital mesoblastic nephroma of the cellular
subtype. Postoperatively no chemotherapy was considered necessary. 11
months after diagnosis the patient had an extensive local recurrence w
ith infiltration of the perirenal fat, mesenterium and colon. Complete
resection could not be achieved and the tumor was classified as stage
III. There was a striking morphological change from spindle cells in
the initial tumor to malignant round cells in the relapse specimen. Th
e patient was treated with Vincristine, Actinomycin-D and Adriblastin.
Radiotherapy was not given. 38 months after relapse the patient is fr
ee of disease and developing normally. Our patient obviously had an ag
gressive variant of CMN. The significance of the potentially aggressiv
e 'variant of CMN, atypical mesoblastic nephroma, is discussed and pos
sibilities are suggested for management.