Phase 2 study of idarubicin in pediatric brain tumors: Pediatric Oncology Group study POG 9237.

Idarubicin (IDA), the 4-demethoxy analog of daunomycin, has had significant cytotoxicity in many malignancies. In previous reports, the alcohol metabolite of IDA, 4-demethoxydaunorubicinol (idarubicinol, or IDOL), had cytotoxic activity and the ability to penetrate the blood-brain barrier. For this reason, the Pediatric Oncology Group conducted a Phase 2 trial of IDA for children with recurrent or progressive brain tumors.

Extended follow-up of long-term survivors of childhood acute lymphoblastic leukemia.

Background: Children who survive acute lymphoblastic leukemia are at risk for leukemia-related or treatment-related complications, which can adversely affect survival and socioeconomic status. We determined the long-term survival and the rates of health insurance coverage, marriage, and employment among patients who had attained at least 10 years of event-free survival.

Methods: A total of 856 eligible patients were treated between 1962 and 1992 in 13 consecutive clinical trials.

Primary perineal alveolar rhabdomyosarcoma metastatic to an extraocular muscle.

A 17-year-old boy diagnosed with an alveolar rhabdomyosarcoma involving the perineum and with extensive lymphadenopathy was treated with chemotherapy yet developed metastases to the head and neck 6 months into therapy. Ten months after initial diagnosis, while receiving salvage chemotherapy and radiotherapy, he returned with pain on movement of his left eye, proptosis, and ptosis of the left upper eyelid. Computed tomography (CT) revealed a mass within the left lateral rectus muscle that biopsy confirmed to be metastatic alveolar rhabdomyosarcoma.

Prognostic factors for children with Hodgkin's disease treated with combined-modality therapy.

Purpose: Evaluation of pretreatment factors to identify children at high risk for relapse after combined-modality therapy for Hodgkin's disease.

Patients And Methods: From 1990 to 2000, 328 pediatric patients with clinical stage I to IV Hodgkin's disease were treated with chemotherapy and low-dose involved-field radiotherapy on prospective, collaborative, risk-adapted protocols at three institutions. Pretreatment factors were analyzed by univariate and multivariate analysis for prognostic significance for 5-year disease-free survival (DFS) and overall survival (OS).

Treatment of intraocular retinoblastoma with vincristine and carboplatin.

Purpose: To evaluate the efficacy of chemoreduction using vincristine and carboplatin in preventing or delaying external-beam radiotherapy (EBRT) or enucleation in patients with intraocular retinoblastoma.

Patients And Methods: Twenty-five patients (43 eyes) with newly diagnosed intraocular retinoblastoma received primary treatment with eight courses of vincristine and carboplatin. Focal treatments were delayed until documentation of disease progression.

Survival and functional outcome of children with hypothalamic/chiasmatic tumors.

Background: The management of children with hypothalamic (H) and/or chiasmatic (C) tumors remains controversial. We evaluated the impact of clinical and neuroimaging parameters and primary therapy on overall (OS) and progression-free (PFS) survival and on neuroendocrine and neurocognitive outcome in children with H and/or C tumors.

Methods: Records were reviewed for 73 children with H and/or C tumors treated at St.

A chemosensitive pediatric extraosseous osteosarcoma: case report and review of the literature.

Osteosarcoma arising in soft tissues is exceedingly rare in children. The tumor most often affects older adults, involves the lower extremity, responds poorly to chemotherapy, and carries a grave prognosis. The authors describe a 12-year-old girl with an extraosseous osteosarcoma of the left sternocleidomastoid muscle with pulmonary metastases.

Granulocyte colony-stimulating factor and the risk of secondary myeloid malignancy after etoposide treatment.

Event-free survival for children with acute lymphoblastic leukemia (ALL) now exceeds 80% in the most effective trials. Failures are due to relapse, toxicity, and second cancers such as therapy-related myeloid leukemia or myelodysplasia (t-ML). Topoisomerase II inhibitors and alkylators can induce t-ML; additional risk factors for t-ML remain poorly defined.

Medulloblastoma metastatic to the suprasellar region at diagnosis: a report of six cases with clinicopathologic correlation.

The presence of metastatic disease in patients newly diagnosed with medulloblastoma remains one of the most important prognostic factors that determines event-free survival. In the present study, anatomic distribution and the signal characteristics and enhancement patterns of subtle anterior third ventricular recess metastases were compared with those of the original tumor; medical records were reviewed for clinical presentation, surgical stage, treatment and long-term outcomes. All foci were clinically occult; 5 out of 6 had negative cerebrospinal fluid cytology, and in 4 out of 6, the only evidence of metastatic disease was documented suprasellar disease that resolved or significantly improved following irradiation and chemotherapy.

Preirradiation endocrinopathies in pediatric brain tumor patients determined by dynamic tests of endocrine function.

Purpose: To prospectively evaluate pediatric patients with localized primary brain tumors for evidence of endocrinopathy before radiotherapy (RT).

Methods And Materials: Seventy-five pediatric patients were evaluated with the arginine tolerance test and L-dopa test for growth hormone secretory capacity and activity; thyroid-stimulating hormone surge and thyrotropin-releasing hormone stimulation test for the hypothalamic-thyroid axis; the 1-microg adrenocorticotropin hormone (ACTH) and metyrapone test for ACTH reserve; and, depending on age, a gonadotropin-releasing hormone stimulation test to determine gonadotropin response. The study included 38 male and 37 female patients, age 1-21 years with ependymoma (n = 35), World Health Organization (WHO) Grade I-II astrocytoma (n = 18), WHO Grade III-IV astrocytoma (n = 10), craniopharyngioma (n = 7), optic pathway tumor (n = 4), and germinoma (n = 1).

Atypical teratoid/rhabdoid tumor of the central nervous system: report on workshop.

Childhood atypical teratoid/rhabdoid tumor (AT/RT) of the central nervous system (CNS) is a recently described entity. Diagnosis is based on distinctive light microscopy and immunohistochemical findings, coupled with molecular genetic analysis. Most AT/RTs demonstrate monosomy 22 or deletions of chromosome band 22q11 with alterations of the hSNF5/INI1 gene.

Treatment of unfavorable childhood Hodgkin's disease with VEPA and low-dose, involved-field radiation.

Purpose: Between January 1990 and April 1993, 56 pediatric patients with Hodgkin's disease were treated on a single-arm trial at three institutions with a regimen designed to maintain high cure rates while minimizing the potential late effects of treatment, such as infertility, second malignant neoplasms, and cardiopulmonary injury.

Patients And Methods: The regimen used combined-modality therapy with six cycles of vinblastine, etoposide, prednisone, and doxorubicin (VEPA) chemotherapy and low-dose, involved-field radiation. Unfavorable features comprised bulky presentations of localized (stage I or II) disease or advanced (stage III or IV) Hodgkin's disease.

VAMP and low-dose, involved-field radiation for children and adolescents with favorable, early-stage Hodgkin's disease: results of a prospective clinical trial.

Purpose: To evaluate outcome and assess toxicity of children and adolescents with early-stage, favorable Hodgkin's disease treated with vinblastine, doxorubicin, methotrexate, and prednisone (VAMP) and low-dose, involved-field radiation.

Patients And Methods: One hundred ten patients with clinical stages I and II, favorable (nonbulky) Hodgkin's disease were treated with four cycles of VAMP chemotherapy and 15 Gy involved-field radiation for those who achieved a complete response, or 25.5 Gy for those who achieved a partial response to two cycles of VAMP.

Craniopharyngioma: the St. Jude Children's Research Hospital experience 1984-2001.

Purpose: To review our institution's experience in the treatment of craniopharyngioma and assess the merits of initial therapy with limited surgery and irradiation.

Methods And Materials: The data of 30 patients (median age 8.6 years) with a diagnosis of craniopharyngioma between April 1984 and September 1997 were reviewed.

Influence of tumor grade on time to progression after irradiation for localized ependymoma in children.

Purpose: To investigate the influence of histologic grade on progression-free survival (PFS) after irradiation (RT) for pediatric patients with localized ependymoma.

Methods And Materials: Fifty patients with localized ependymoma (median age 3.6 years, range 1-18 years at the time of RT) were treated with RT between December 1982 and June 1999.

Ocular preservation after 36 Gy external beam radiation therapy for retinoblastoma.

Purpose: The purpose of this study was to document the ocular preservation rate after 36 Gy external beam radiation therapy (EBRT) for retinoblastoma.

Patients And Methods: Forty-nine eyes of 38 patients were treated with a median dose of 36 Gy EBRT. The patient population included 7 unilateral and 31 bilateral presentations, with a median age at diagnosis of 4 months.

Survival after recurrence of Ewing tumors: the St Jude Children's Research Hospital experience, 1979-1999.

Background: Despite improved therapies, 30-40% of patients with Ewing tumors (ET) experience recurrence and have a poor prognosis. The authors analyzed factors prognostic of survival in patients with recurrent ET.

Methods: The authors assessed the relation between postrecurrence survival (PRS) and demographic, disease, and treatment factors in 71 patients who experienced recurrent ET after treatment on one of three consecutive institutional protocols.

Histopathologic grading of medulloblastomas: a Pediatric Oncology Group study.

Background: Medulloblastomas are small cell embryonal tumors of the cerebellum found predominantly in children, only slightly more than half of whom survive. Predicting favorable outcome has been difficult, and improved stratification clearly is required to avoid both undertreatment and overtreatment. Patients currently are staged clinically, but no pathologic staging system is in use.

Preliminary results from a Phase II trail of conformal radiation therapy for pediatric patients with localised low-grade astrocytoma and ependymoma.

Purpose: To estimate the local control and patterns of failure for pediatric patients with low-grade astroglial tumors (LGA) and ependymoma (EP) treated with three-dimensional conformal radiation therapy (CRT) using an anatomically defined clinical target volume (CTV).

Methods And Materials: From an ongoing, prospective Phase II trial initiated in July 1997, 102 pediatric patients with LGA (n = 38) and EP (n = 64) have been treated with CRT using an anatomically defined CTV extending 1.0 cm beyond the gross tumor volume and a 0.

Emergency re-operation for postoperative hemorrhage following partial esophagectomy for carcinoma of the esophagus and cardia of the stomach.

The clinical records of 12 cases of emergency re-operation for management of postoperative hemorrhage (POH) following partial esophagectomy and esophago-gastrostomy or colonic interposition for a group of 3690 cases of carcinoma of the esophagus (CE) and cardia of the stomach in this institute between August 1954 and April 2001 were studied. There were 10 survivors and two deaths, giving a mortality rate of 16.6% (2/12).

Effect of therapeutic ionizing radiation on the human brain.

We test a hypothesis that fractionated radiation therapy within a therapeutic dose range is associated with a dose-related change in normal brain, detectable by quantitative magnetic resonance imaging. A total of 33 patients were examined by quantitative magnetic resonance imaging to measure brain tissue spin-lattice relaxation time (T1) before treatment, and at various times during and after radiation therapy. A T1 map was generated at each time point, and radiation therapy isodose contours were superimposed on the corresponding segmented T1 map.

Second malignancy after treatment of childhood non-Hodgkin lymphoma.

Background: The objective of this report was to determine the cumulative incidence of and risk factors for second malignancy and the competing risk of death due to any other cause among patients who were treated for childhood non-Hodgkin lymphoma (NHL).

Methods: The authors retrospectively reviewed a cohort of 497 patients with NHL who were treated at St. Jude Children's Research Hospital between 1970 and 1997.