FAILURE OF TREATMENT IN CHLDHOOD EXTRACRANIAL AND EXTRATESTICULAR MALIGNANT GERM CELL TUMORS

Proceedings of the fifth germ cell tumor Conferenc

Granular acute lymphoblastic leukemia in children. "Aieop Cooperative Group for Cytology of Acute Leukemias".

Granular acute lymphoblastic leukemia (ALL) is a rare morphological variant of ALL, characterized by cytoplasmic azurophil granules or inclusions, positive for aspecific esterase and acid phosphatase, with heterogeneous features at the ultrastructural level.In an attempt to determine whether the presence of granules or inclusions marks a biologically distinct variety of ALL with peculiar clinical features, a prospective morphological review was undertaken of children entering AIEOP protocols for ALL in the period from 1985 to 1989.Of 531 cases examined, 16 (3\%) were found to have greater than 1\% granular bone marrow blasts, with 7 cases (1.3\%) having greater than 10\%. The presence of granules or inclusions was associated with the immunophenotype of "common" ALL. There was no clear association with FAB type L1 or L2 nor with particular clinical or hematological findings at presentation. Complete remission was achieved in all cases; one patient died of infection in remission at 3 months and 2 patients relapsed after 12 and 32 months, respectively, while the others are still in remission after a minimum follow-up of 24 months.In conclusion, granular morphology seems to have no prognostic importance in children ALL

Mortality from second tumour among long-term survivors of retinoblastoma: a retrospective analysis of the Italian retinoblastoma registry.

22Survivors of retinoblastoma (Rb) are at high riskof dying from second malignant tumour. The occurrence of second malignant neoplasm (SMN) and related mortality in a cohort of 1111 cases from the Italian Retinoblastoma Registry was analysed, considering the possible role of both genetic and iatrogenic causes. Rb patients had a greater than 10-fold excess in overall mortality compared with the general population (standardized mortality ratio (SMR) 10.73, 95% CI 9.00–12.80). Their excess risk attributable to cancers other than Rb was 14.93 95% CI 10.38–21.49). Survivors of hereditary Rb had an SMR for all causes of 16.25 (95% CI 13.20–20.00), whereas their SMR for all cancers was 25.72 (95% CI 17.38–38.07). Survivors of unilateral sporadic Rb had an SMR of 4.12 from all cancers (95% CI 1.55–10.98) and a much higher excess for overall mortality (SMR 13.34, 95% CI 10.74–16.56). As expected, survivors of hereditary Rb had higher mortality from cancers of the bone (SMR 391.90, 95%CI 203.90–753.20) and soft tissue (SMR 453.00, 95% CI 203.50–1008.40), small intestine (SMR 1375.50, 95% CI 344.00–5499.70), nasal cavity (SMR 13.71, 95% CI 1.93–97.35) and cancers of the brain and central nervous system (SMR 41.14, 95% CI 13.2–127.55)reservedmixedACQUAVIVA A; CICCOLALLO L; RONDELLI R; BALISTRERI A; ANCAROLA R; COZZA R; HADJISTILIANOU D; DE FRANCESCO S; P. TOTI; PASTORE G; HAUPT R; CARLI M; SANTORO N; DI CATALDO A; FIORILLO A; INDOLFI P; NUCCI P; SANDRI A; PORTA F; PORCARO AB; TAMARO P; MORGESE GAcquaviva, A; Ciccolallo, L; Rondelli, R; Balistreri, A; Ancarola, R; Cozza, R; Hadjistilianou, D; DE FRANCESCO, S; Toti, P.; Pastore, G; Haupt, R; Carli, M; Santoro, N; DI CATALDO, A; Fiorillo, A; Indolfi, P; Nucci, P; Sandri, A; Porta, F; Porcaro, Ab; Tamaro, P; Morgese,