Mesoblastic nephroma--a report from the Gesellschaft fur Pädiatrische Onkologie und Hämatologie (GPOH).
BACKGROUND
Surgery alone is the appropriate first-line treatment for patients with mesoblastic nephroma (MN). Nevertheless, there are reports of local recurrences and metastasis, especially in the cellular subtype. The authors evaluated the outcome of patients with MN who were enrolled in either the International Society of Pediatric Oncology (SIOP) 93-01/GPOH or the SIOP 2001/GPOH Nephroblastoma Study and Trial.


METHODS
In total, 50 patients with MN were analyzed. Eleven patients were suspected antenatally of having a renal tumor. The median age at diagnosis was 18.5 days. Central pathologic review was performed for all specimens. The median observation time was 4.2 years.


RESULTS
Forty-five patients underwent initial surgery. Five patients older than 6 months received preoperative chemotherapy. Twenty-nine tumors were classic MN, and 21 tumors were cellular MN. Nine patients had a Stage III MN, 5 of those patients had tumor ruptures, and 8 had positive surgical margins. After they underwent nephrectomy, 40 patients received no further treatment. For the entire group, event-free survival (EFS) (94%) and overall survival (OS) (95%) were excellent. Patients with a cellular MN, patients with age 3 months or older, and patients with Stage III MN had lower EFS. Three patients developed recurrent disease, and 2 of those patients died. Metastases to the brain, lung, and liver were observed in 1 patient.


CONCLUSIONS
Radical nephrectomy with accurate surgical-pathologic staging is the standard of care for children with MN. Nonetheless, a subgroup of patients with MN (Stage III cellular MN in patients age 3 months or older) tends to develop recurrences more often. Further prospective studies will be needed to verify this finding and should help determine whether these patients may benefit from adjuvant therapy.
Furtwaengler
Rhoikos
R
Department of Pediatric Hematology and Oncology, Saarland University Hospital, Homburg/Saar, Germany. kirfur@uniklinik-saarland.de
Reinhard
Harald
H
Leuschner
Ivo
I
Schenk
Jens P
JP
Goebel
Ulrich
U
Claviez
Alexander
A
Kulozik
Andreas
A
Zoubek
Andreas
A
von Schweinitz
Dietrich
D
Graf
Norbert
N
D000367:Age Factors; D001707:Biopsy, Needle; D017024:Chemotherapy, Adjuvant; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D005260:Female; D005858:Germany; D006801:Humans; D007150:Immunohistochemistry; D007223:Infant; D007231:Infant, Newborn; D007680:Kidney Neoplasms / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy*; D016015:Logistic Models; D008297:Male; D009367:Neoplasm Staging; D009392:Nephrectomy / Q000379:methods; D018201:Nephroma, Mesoblastic / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy*; D010372:Pediatrics; D011379:Prognosis; D016016:Proportional Hazards Models; D012189:Retrospective Studies; D018570:Risk Assessment; D012955:Societies, Medical; D018709:Statistics, Nonparametric; D016019:Survival Analysis; D016896:Treatment Outcome