Margherita Lo Curto

Italian registry of patients off therapy after childhood acute lymphoblastic leukemia. Results after first phase of data collection

The Italian Registry of Off‐Therapy patients after childhood tumors now includes 760 subjects with acute lymphoblastic leukemia. These patients were all removed from treatment by December 31, 1981, and were followed in 35 different institutions. All the children have received multiple‐drug treatment, combined, in 79.7% of the cases, with cranial irradiation. Thirty‐nine (5%) experienced a relapse before treatment suspension. Total duration of antileukemic therapy ranges between 18 and 131 months (median, 38). At the last updating (December 31, 1981), 699 subjects were alive, 6 were lost to follow‐up, and 55 had died. Life‐table analysis shows that 90.8% were alive and 77% were alive in continuous complete remission at 36 months, whereas at 66 months, the cumulative proportions were 88% and 75.5%, respectively. One hundred thirty‐six of 760 relases after therapy suspension were reported: 83 in male patients and 53 in female patients (P < 0.01). The longest interval between relapse and treatment suspension was 64 months. Six of 55 died in continuous complete remission 3 to 44 months after treatment suspension. Five births of apparently normal babies to female patients have been reported. A general outline of the project and the future program are given.

Teratoma with a malignant somatic component in pediatric patients: The Associazione Italiana Ematologia Oncologia Pediatrica (AIEOP) experience

Teratoma with a malignant somatic component (TMSC) is rare but described in adults, whereas information on pediatric presentation is sparse.

Perlman syndrome: clinical report and nine-year follow-up.

We present the clinical and follow‐up data of a female infant with Perlman syndrome from birth to the age of 9 years. Main features of Perlman syndrome include polyhydramnios, fetal overgrowth, neonatal macrosomia, macrocephaly, dysmorphic facial features, visceromegaly, nephroblastomatosis, and a predisposition for Wilms tumor. In our patient, the nephromegaly with nephroblastomatosis was not present at birth or during the neonatal period; it became evident in the first months of postnatal life. A Wilms tumor was diagnosed when she was about 1 year old. Long term follow‐up documents the natural history of Perlman syndrome and allows us to establish the long‐term prognosis of the affected individuals.