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Monosomy 22 in a malignant peripheral nerve sheath tumour of the kidney in childhood: a genetic link with other malignant paediatric renal neoplasms?

A recurrent malignant spindle cell neoplasm of the kidney, occurring in a 11-year-old girl and corresponding to a malignant peripheral nerve sheath tumour is reported. The neurogenic differentiation was substantiated by the presence of PGP 9.5 neurofilament and S-100 protein positivity and by the ultrastructural features. Cytogenetic analysis revealed monosomy of chromosome 22 in the tumour while the constitutional karyotype was normal. Primary malignant peripheral nerve sheath tumour of the kidney is extremely rare and should, on morphology, be differentiated from stromal predominant Wilms' tumour and clear cell sarcoma of kidney. Involvement of chromosome 22 has been described in a number of malignant renal tumours of childhood, such as Wilms' tumour (deletion), clear cell sarcoma (translocation), and rhabdoid tumour (deletion and translocation), thus suggesting common molecular mechanisms in pediatric malignant renal tumours.

Child↗

Risk of disease recurrence and second neoplasms in survivors of childhood cancer treated with growth hormone: a report from the Childhood Cancer Survivor Study.

GH deficiency is common in survivors of childhood cancer, especially in those treated with radiation to the brain. The impact of GH therapy on disease recurrence has been studied in survivors of pediatric brain tumors, but few data are available on the risk of disease recurrence in survivors of other tumor types who are treated with GH. Likewise, the risk of second neoplasms (SN) associated with GH use has not been systematically evaluated. We studied 361 GH-treated cancer survivors (including 172 brain tumor survivors) from among 13,539 survivors enrolled in the Childhood Cancer Survivor Study, a cohort of 5-yr survivors of childhood cancer. Using a time-dependent Cox model, we compared risk of recurrence, risk of SN, and risk of death between survivors who did and did not receive treatment with GH. The relative risk of disease recurrence was 0.83 (95% confidence interval, 0.37-1.86; P = 0.65) for GH-treated survivors. The relative risk of recurrence was not increased for any of the major cancer diagnoses. GH-treated subjects were diagnosed with 15 SN, all solid tumors and no secondary leukemias, for an overall relative risk of 3.21 (95% confidence interval, 1.88-5.46; P < 0.0001). This was mainly due to a small excess number of SN observed in GH-treated survivors of acute leukemia. The risk of death was not associated with GH use (P = 0.43). We conclude that GH therapy does not appear to increase the risk of disease recurrence or death in survivors of childhood cancer. The increased number of SN, particularly in survivors of acute leukemia, is of concern, but the data need to be interpreted with caution given the small number of events.

Adolescent↗