Recurrent and metastatic mesoblastic nephroma in infancy.

Abstract

Local recurrence or metastasis of mesoblastic nephroma is extremely uncommon. The first report of a child with mesoblastic nephroma to have both of these problems is presented. The usual course of mesoblastic nephroma is benign, with nephrectomy being curative. Adjuvant treatment is not necessary and may be lethal. However, a review of the five patients who developed locally recurrent and/or metastatic tumor indicates that chemotherapy and radiotherapy are of benefit and can produce long-term disease-free survival.

Mesoblastic nephroma—A report from the Gesellschaft fur Pädiatrische Onkologie und Hämatologie (GPOH)

“…2,3,4,18 -21,29 We observed no difference in the gender ratio of the subtypes. In addition, contrary to a previous suggestion, 21 we found no association between gender and the risk of tumor recurrence.…”

“…Nevertheless, we believe that a group of patients with a high risk of recurrence can be identified by combining these risk factors. The dismal prognosis for the 5 patients at risk in our series and the proven therapeutic effect of chemotherapy, both in literature 6,10,11,21,[33][34][35] and in our patients, suggest that this group should receive adjuvant treatment. Consequently, approximately 10% of patients (5 of 50 in our cohort) would receive additional treatment.…”

“…Furthermore, tumor rupture itself is a risk factor for recurrence, because 2 of our patients who had tumor ruptures developed recurrences (Table 4), in contrast to other reports. 6,21,28 Surgeons should be aware of this increased risk of rupture, especially in older patients with MN, because patients with cellular MN are older and often have larger, cystic tumors and therefore have a greater risk of recurrence.…”

“…However it has been shown repeatedly that chemotherapy, with or without irradiation, is effective in patients with metastatic disease or recurrence. 6,10,11,21,34,35 McCahon et al 33 achieved sustained remission after initial chemotherapy only in patients who had inoperable, ETV6-NTRK3-positive CFS and cellular MN. In our studies, 3 patients with Stage III, cellular MN received chemotherapy, and all 3 are in their first continuous, complete remission (Table 4), whereas the 2 patients who received no further treatment developed recurrent disease.…”

“…Since then, risk factors and risk-adapted therapy have been discussed controversially. 15,[17][18][19][20][21] It has been reported that positive surgical margins, 17,20 patient age, 16,21 and cellular MN 15 may be correlated with a greater risk of recurrence. Recent studies revealed that cellular MN differs genetically from classic MN.…”