Shunt-related abdominal metastases in an infant with medulloblastoma: long-term remission by systemic chemotherapy and surgery.

This is the first reported case of long remission of abdominal metastases spread through a ventriculo-peritoneal shunt in an infant diagnosed, four years ago, at age 1 year and 10 months, to have cerebral medulloblastoma. Two years later, while in second complete remission of his cerebral tumor, he showed abdominal metastases, successfully treated by platinum based chemotherapy and surgery. One year later, a second abdominal relapse and hepatic metastases were treated by doxorubicin administration and surgery. Since then the child remained in continuous complete remission. This unusual favorable outcome can be explained by an extreme responsiveness of the tumor, unprotected by the blood brain barrier, to systemic chemotherapy, particularly to doxorubicin administration. The need for careful surveillance of patients with ventriculo-peritoneal shunts is emphasized. Searching for new tools, such as entrapment of doxorubicin in liposomes, able to overcome the blood-brain barrier and to expose brain tumors to effective drugs, probably represents the best choice for future treatment strategies of CNS tumors.

Longitudinal assessment of cardiac status by echocardiographic evaluation of left ventricular diastolic function in thalassaemic children.

Cardiac disease is the primary cause of death in patients affected by thalassaemia major. In most cases diastolic dysfunction precedes the onset of systolic impairment at a time when appropriate therapy can prevent progression of cardiac damage. We have assessed the pattern of left ventricular filling by Doppler echocardiography in six transfusion-dependent thalassaemic patients. They were re-evaluated 5 y after the first observation, after additional blood transfusions and consequent iron load. We registered a significantly impaired relaxation pattern in all of them. A state of cardiac anoxia has been hypothesized.

Radiation late effects in children treated for orbital rhabdomyosarcoma.

BACKGROUND AND PURPOSE: The experience resulting from large cooperative studies shows that correct radiation therapy at doses adequate to the tumor bulk are crucial for local control of rhabdomyosarcoma. The aim of the present study was to document the correlation between modalities and doses of radiotherapy and radiation side effects. PATIENTS AND METHODS: Between 1980 and 1997, 19 patients affected by primary orbital rhabdomyosarcoma have been followed at the University Federico II of Naples. All but three patients, who received 45, 54 and 55 Gy respectively, have been treated by immediate radiation at the dose of 60 Gy, delivered in 2 Gy fractions, five times per week, by cobalt 60 megavoltage equipment. Combined chemotherapy using vincristine and vincristine plus dactinomycin on alternate weeks was also administered as part of induction therapy. RESULTS: An overall survival rate of 94.7% was registered. In our patients the majority of radiation late effects were paid by orbit and ocular adnexa. Side effects to lens and ocular structures were fewer and of low grade. CONCLUSIONS: Radiation therapy is still essential for local control of orbital rhabdomyosarcoma, however radiation side effects have to be carefully considered together with the therapeutic goal to be obtained.

Primary Ewing's sarcoma of the maxilla, a rare and curable localization: report of two new cases, successfully treated by radiotherapy and systemic chemotherapy.

Primary maxillary localization of Ewing's sarcoma is unusual. Involvement of facial bones is characterized by clinical and radiological features distinct from those commonly observed in other sites. Because of the above peculiarities a delay in diagnosis and thus in starting treatment is very probable in such cases. We report here two new cases of Ewing's sarcoma localized to facial bones, successfully treated by local high dosage radiotherapy and systemic chemotherapy. Our experience suggests that, especially for particular sites not suitable to radical surgery, radiation therapy can represent an effective tool to achieve local control of the tumor.

Granulocytic sarcoma in nonleukemic children: report of two new cases successfully treated by local radiation therapy and systemic chemotherapy.

Granulocytic sarcoma (GS) is a rare tumor composed of immature myeloid cells. Exceedingly rare in childhood, it has more commonly been described in association with acute myeloid leukemia. Occasional nonleukemic patients generally go on to develop overt leukemia in a mean period of 10.5 months from diagnosis of GS. We report here two new cases of GS diagnosed in nonleukemic children. They were successfully treated with local radiation therapy and conventional systemic chemotherapy. The need to suspect more often this all too frequently misdiagnosed disease is emphasized. The role of optimally delivered radiation therapy in achieving and maintaining local control of the tumor is discussed.

A novel chromosomal abnormality detected in a case of acute myelomonocytic leukemia.

A not yet recorded translocation, t(3:17)(q21;q23), detected in a case of acute myelomonocytic leukemia, is reported. In spite of an aggressive cytotoxic chemotherapy, the disease showed a rapid fatal course, confirming the dismal prognostic significance of structural, 'primary' chromosomal abnormalities in acute leukemias.

Expression of cathepsin D in malignant and in the corresponding non-malignant node-negative laryngeal samples: correlation with receptors for androgen, glucocorticoid, oestrogen and progesterone.

A standard immunoradiometric technique was used to investigate the distribution of the intracellular aspartic proteinase cathepsin D in 33 malignant and in the corresponding histologically-proven non-malignant fragments obtained from lymph node negative patients suffering from larynx cancer. In both tissues the androgen, glucocorticoid, oestrogen and progesterone receptors were also assayed. Our data indicate that cathepsin D was present in both tissues, with level significantly higher (P < 0.0001) in the cancerous fragments (with a mean of 33 +/- 3.4 pmol/mg protein) than in the corresponding non-cancerous specimen (with a mean of 20.8 +/- 2 pmol/mg protein). A significant positive association (P < 0.001) between cathepsin D and PR concentration values in the cancerous larynx was observed; accordingly, tumours expressing PR had significantly (P = 0.0005) higher cathepsin D levels than the tumours which did not contain the receptor. In contrast, such a relationship was absent in the non-malignant specimens. As regards the other steroid receptors, no significant relationship between them and cathepsin D was observed. We conclude that cathepsin D may have a role also in laryngeal carcinoma and that its association with the PR could indicate a possible role of the receptor in the biology of this disease.