Biomedical subjects

A S Pappo

Publications and source records attributed to A S Pappo.

77 records · Page 5Linked to original sources

PubMedDate not supplied

Brain metastases in pediatric Ewing sarcoma and rhabdomyosarcoma: the St. Jude Children's Research Hospital experience.

PURPOSE: Although brain metastases rarely occur in children with solid tumors, pediatric Ewing sarcoma (ES) and rhabdomyosarcoma (RMS) are among those most likely to metastasize to the brain. The authors review their institution's experience of brain metastases of ES and RMS. PATIENTS AND METHODS: The clinical characteristics, therapy, and outcome of all patients treated at St. Jude Children's Research Hospital over a 36-year period who had ES or RMS with brain metastases were reviewed. RESULTS: Of 419 patients with RMS, 10 (2.4%) had brain metastases. Of 335 patients with ES, 11 (3.3%) had brain metastases. The median age of the 21 patients was 10.4 years (range, 0.4-18.0 years) at the time of primary diagnosis. All had clinical signs of central nervous system (CNS) involvement. Outcome was dismal: The median duration of survival after diagnosis of brain metastasis was 2.7 months. The estimated survival 1 year after detection of brain involvement was 23.8%+/-8.5% (mean +/- standard error). One patient, who underwent chemotherapy, surgical resection, and radiotherapy, at the time of this writing is a long-term survivor. CONCLUSIONS: Brain metastases are rare in children with ES and RMS, but carry a grave prognosis. Because most brain metastases are accompanied by signs of neurologic involvement, routine imaging studies of asymptomatic children are not necessary. Combined-modality treatment offers the best chance of long-term survival.

Adolescent↗

PubMedDate not supplied

Hematologic abnormalities and acute myeloid leukemia in children and adolescents administered intensified chemotherapy for the Ewing sarcoma family of tumors.

PURPOSE: Current treatment of the Ewing sarcoma family of tumors (ESFT) includes intensive multiagent chemotherapy with topoisomerase II inhibitors, alkylating agents, and granulocyte colony-stimulating factor (G-CSF). This treatment approach has been associated with myelodysplasia and acute myeloid leukemia. Because macrocytosis and thrombocytopenia are distinctive features of myelodysplasia, the authors evaluated a cohort of patients treated for ESFT to determine the degree and duration of macrocytosis and thrombocytopenia and their relation with the development of therapy-related hematologic malignancies. PATIENTS AND METHODS: The study group consisted of 73 patients with ESFT treated on two consecutive protocols (EW92 and EW87). Both chemotherapy regimens incorporated the same agents but differed in cumulative drug dose, dose per course, and the use of G-CSF. Platelet counts and the mean corpuscular volume (MCV) of erythrocytes were determined at diagnosis and during follow-up visits after completion of treatment. RESULTS: Patients in the EW92 group had significantly greater MCVs after treatment than did the less intensively treated EW87 group. These changes persisted throughout the 40-month observation period. Patients in the EW92 group also had lesser mean platelet counts after treatment than those in the EW87 group. MCV differences (from baseline) were inversely related to platelet counts. The cumulative incidence of treatment-related acute myeloid leukemia was 7.8%+/-4.7% at 4 years in the EW92 group and zero in the EW87 group. CONCLUSION: Patients treated for ESFT with intensive chemotherapy that includes large doses of alkylators, topoisomerase II inhibitors, and G-CSF characteristically have persistently elevated MCVs and decreased platelet counts after completion of therapy. These hematologic abnormalities may represent stem cell damage, predisposing patients to myelodysplasia and acute myeloid leukemia, but further study is needed to establish this relation.

Adolescent↗

PubMedDate not supplied

Dermatofibrosarcoma protuberans: the pediatric experience at St. Jude Children's Research Hospital.

The clinical characteristics and outcome of children with dermatofibrosarcoma protuberans (DFSP) treated at a single institution over a 34-year period were reviewed. A retrospective chart review identified five children with dermatofibrosarcoma protuberans. The diagnosis was confirmed in all cases by one of the authors. Age; sex; clinical group; tumor invasiveness and size, nodal status, and metastases (TNM stage); therapy; and outcome were retrieved from the medical records of these patients. The median age at diagnosis was 12.5 years. There were four boys, and all tumors arose in the extremities or trunk. All children underwent at least two surgical procedures to achieve negative or surgically microscopic positive margins. In addition, one patient received adjuvant radiotherapy for microscopically positive margins. All patients survive free of disease 1 month to 16.5 years after diagnosis. Dermatofibrosarcoma protuberans is rare among children and adolescents. Recognition of this pathologic entity is important for its proper treatment. Surgical resection (with 3-cm margins) is currently the standard therapy for DFSP.

Adolescent↗

PubMedDate not supplied

Brain metastases in paediatric solid tumours.

Brain metastases in children with cancer are rare and their incidence is significantly lower (5-10%) than that reported in adults. The development of metastatic brain tumours in children is usually a manifestation of advanced disease and commonly occur after, or at the time of progression at other sites. This review summarises the salient clinical features of the most common paediatric solid tumours that metastasize to the brain including neuroblastoma, musculoskeletal sarcomas, germ cell tumours and melanoma.

Brain Neoplasms↗

PubMedDate not supplied

Pediatric primary diffuse large cell lymphoma of bone with t(3;22)(q27;q11).

PURPOSE: A child with a primary lymphoma of bone (PLB) with a t(3;22)(q27;q11) is described. METHODS: An 11-year-old boy had a 5-week history of back pain and a destructive lesion of S1 that contained an epidural component. Histologic evaluation of a biopsy confirmed the diagnosis of diffuse large B-cell non-Hodgkin lymphoma. Karyotypic analysis disclosed a t(3;22)(q27;q11), but the amount of tumor tissue was insufficient for molecular studies of the BCL-6 gene. RESULTS: The patient remains free of disease 4 years after completion of intensive systemic chemotherapy and intrathecal chemotherapy. CONCLUSIONS: The lymphoma in the patient described in this report is highly unusual because of the coexistence of pediatric PLB and a t(3;22)(q27q11).

Bone Neoplasms↗