Congenital mesoblastic nephroma with metastasis to the brain: a case report.
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Brain Neoplasms↗
Biomedical subjects
J L Finlay
Publications and source records attributed to J L Finlay.
Hyperfractionated radiation therapy (72 Gy) for children with brain stem gliomas. A Childrens Cancer Group Phase I/II Trial.
BACKGROUND: Most children with brain stem gliomas (BSG) die within 18 months of diagnosis. Early experience suggested that hyperfractionated radiation therapy (RT) at a dose of 72 Gy, administered in 1-Gy fractions twice daily, possibly improved disease-free survival for children with BSG. METHODS: To better characterize the toxicity and possible efficacy of this dose and fractionation of RT, 53 assessable children with diffuse intrinsic or malignant BSG were treated. Survival figures also were combined with outcome in 36 patients treated in a previous pilot study. RESULTS: An objective response to treatment was observed in 28 of 53 (53%) patients; a partial response occurred in 7. No child died of treatment-related brain necrosis, although 7 of 53 did have intralesional cystic/necrotic changes within 6 weeks of completion of RT. The overall survival rate for patients in the study was 38% (+/- 6.5) at 1 year, 14% (+/- 5.4) at 2 years, and 8% (+/- 6.5) at 3 years. Leptomeningeal dissemination was observed in 4 of 48 (8%) children who had relapses. A greater than 2-month duration of symptoms before diagnosis was related to a better prognosis. There was no statistical association between any other clinical parameter, neuroradiographic finding, or pathologic finding and outcome. Combined with that in 35 patients treated in the pilot study, the survival rate in 88 children was 14% (+/- 5) at 3 years. CONCLUSIONS: The radiographic response rate is encouraging; however, it cannot be concluded that hyperfractionated RT, at this dose schedule and total dose, is superior to conventional RT.
Adolescent↗
Chemotherapy with vincristine (VCR) and etoposide (VP-16) in children with low-grade astrocytoma.
Twenty patients, aged 6 months to 20 years, with low-grade astrocytoma (LGA) participated in a chemotherapy trial of vincristine (VCR) and etoposide (VP-16). Fourteen children had recurrent progressive disease at entry on study. Prior treatment consisted of surgical resection alone (6), surgical resection and irradiation (4), surgical resection, irradiation and chemotherapy (2), surgery and chemotherapy (1), and irradiation and chemotherapy (1). Six patients were treated at initial diagnosis of LGA because they were less than 5 years old (5) or for a second primary tumor (1). Four recurrent patients and 3 newly diagnosed patients underwent surgical debulking of their tumors immediately prior to study entry. Tumors were located in the optic nerve/chiasm/hypothalamus (8), brain stem/cerebellum (4), cerebral hemispheres (3), midline structures (3), and spinal cord (2). The treatment plan administered in an out-patient setting consisted of weekly VCR 1.5 mg/m2 for 7 to 8 weeks and VP-16 100 mg/m2 for 5 days repeated every 6 weeks for a total of 18 months of therapy. Responses were evaluated by computerized tomography or magnetic resonance imaging. Of the 20 patients, 1 exhibited a partial response maintained for 12+ months, 3 exhibited minor responses maintained for a period of 10+ to 35 months, and 11 maintained stable disease for 10 to 42 months. Of the 11 patients with stable disease, 2 were withdrawn early from the study without further therapy. Five of the 20 patients developed progressive disease; for 4 of these 5, this occurred during the first course of therapy. Subsequently, these 5 died due to tumor.(ABSTRACT TRUNCATED AT 250 WORDS)
Adolescent↗
Is craniospinal irradiation required to cure children with malignant (anaplastic) intracranial ependymomas?
Between 1970 and 1989, 17 children with histologically malignant intracranial ependymomas received treatment at the University of Pennsylvania (Philadelphia, PA). Eleven were treated with prophylactic cranial or craniospinal irradiation plus a local boost (CS-XRT), five with local (L-XRT) irradiation only, and one was treated without (NRT) irradiation. With a median survival of 2 years and a median follow-up time for long-term survivors of 6.0 years, five of 11 patients who received CS-XRT are alive compared with none treated with L-XRT and none treated with NRT. Two-year actuarial survival rates are 40% (L-XRT) and 52% (CS-XRT). When examined for other factors, age and local radiation dose remain the most significant prognostic indicators of survival. The 2-year actuarial survival for children younger than 4 years at diagnosis is 20% compared with 83% for their older counterparts. Likewise, the 2-year survival for patients treated with local radiation doses over 4500 cGy was 55% compared with 0% for patients treated with lesser doses. To date there are a total of 28 recurrences. All have occurred with local components except for six (unknown) who died before the exact site(s) could be determined. There is no significant difference in the failure rates outside the original tumor bed in the three groups. These data suggest that local relapse remains the most significant component of failure. Because intrinsic and extrinsic factors such as age and radiation dose seem to be interrelated and at least as important as the use of craniospinal irradiation, the need for prophylactic treatment for children with anaplastic ependymoma could neither be substantiated nor refuted. The use of local radiation alone, however, should be restricted to carefully designed clinical trials in which meticulous pretreatment evaluation is performed, and vigilant posttreatment evaluation of the spine and brain is mandatory.
Brain Neoplasms↗
Recurrent intracranial ependymomas in children. Survival, patterns of failure, and prognostic factors.
Thirty-six pediatric patients (ages 0.8-16.8 years) with recurrent intracranial ependymoma were treated for a total of 52 separate cases of relapse from 1970 to 1989. Therapy consisted of surgery in 33 cases and chemotherapy in 38 cases. Twelve patients received radiation at the time of first relapse, and five of these 12 who had initially been treated with surgery and chemotherapy alone were irradiated to full dose. The 2-year actuarial survival and progression-free survival (PFS) rates are 29% and 23%, respectively. Two-year survival after treatment of first relapse is 39%. Of the 52 cases, there have been 44 subsequent relapses (and one septic death), three of which have occurred in the five patients treated with definitive radiation. Twenty-seven relapses have occurred exclusively with local disease. Eight patients failed with disease outside as well as in the primary site. Survival was better for patients who had histologically benign lesions at relapse (53% versus 9%, P less than 0.02), and for patients in first versus subsequent relapse (p less than 0.005). Cisplatin and etoposide (VP-16) appeared to be the most active chemotherapeutic agents. The authors conclude that some patients with histologically benign ependymoma at first relapse may benefit from aggressive therapy, with occasional long-term, progression-free survival possible. Patients with malignant lesions, or patients who relapse a second time, are less likely to benefit from conventional therapy for a significant period of time.
Actuarial Analysis↗
Hyperfractionated radiotherapy for children with brainstem gliomas: a pilot study using 7,200 cGy.
Brainstem gliomas, constituting approximately 10% of all childhood central nervous system tumors, remain the most resistant of all brain tumors to therapy. A subgroup of high-risk patients with tumors that diffusely involve the brainstem or that microscopically demonstrate foci of anaplasia on biopsy specimens rarely survive after treatment. Conventional doses of radiotherapy result in temporary clinical improvement in the majority of these high-risk patients; however, few if any remain alive 18 months after treatment. Hyperfractionated radiotherapy, with delivery of larger numbers of smaller fractions of radiotherapy, is a possible way to increase tumor control without increasing neurological toxicity. In 1985, a multiinstitutional phase I/phase II trial, using 100 cGy of radiation therapy twice daily to a total dose of 7,200 cGy, was undertaken for patients with high-risk brainstem gliomas. At the time of writing, 24 (69%) had developed progressive disease and 11 remained in continuous progression-free remission. Actuarial progression-free survival at 20 months is approximately 30%. Twenty-three of 31 evaluable patients had an objective radiographic response to therapy. In comparison to both historical control patients and patients treated in a previous trial using 6,480 cGy of hyperfractionated radiation therapy, there was a statistically significant improvement in progression-free survival rate for patients treated with 7,200 cGy of hyperfractionated radiation therapy (p less than 0.01). To date no patient has died as a result of treatment. Six patients developed transient neurological deterioration or cystic intralesional changes, as demonstrated on magnetic resonance imaging, within 6 weeks of the completion of radiotherapy. Postmortem examination performed in 7 patients did not disclose significant radiation necrosis.(ABSTRACT TRUNCATED AT 250 WORDS)
Adolescent↗
High-dose multi-agent chemotherapy followed by bone marrow 'rescue' for malignant astrocytomas of childhood and adolescence.
Between April 1986 and March 1989, ten patients under 21 years of age with histologically confirmed malignant astrocytoma, received marrow-ablative chemotherapy with either thiotepa and Etoposide (five patients) or thiotepa, Etoposide and BCNU (five patients), followed by bone marrow 'rescue'. Nine patients had glioblastoma multiforme (GBM), and one patient had an intrinsic brain stem anaplastic astrocytoma (AA). Seven patients were treated for recurrent tumor. Two patients who developed GBM as second malignancies were treated directly following surgical resection. One patient had received irradiation only for recently diagnosed cervical spinal cord GBM. Thiotepa was administered at a total dose of 600-900 mg/M2 over three days, Etoposide was administered at a total dose of 1500 mg/M2 over three days, and BCNU was administered at a total dose of 600 mg/M2 over four days. Non-hematopoietic toxicities have been mainly transient, predictable and acceptable, consisting of oropharyngeal mucositis, cutaneous hyperpigmentation, erythema and desquamation. Four patients achieved complete responses (CR), as determined by radiographic evaluation (CT and/or MRI) on day 28 post-marrow infusion. The mean remission duration of those with CR is 290+ days; two patients presently remain in remission. Two patients achieved partial responses (PR, greater than 50% tumor shrinkage) by day 28 post-marrow infusion; both developed disease progression, at day 61 and 94 post-marrow infusion, respectively. One patient, with a brain stem AA, had stable disease maintained for 13 months post-marrow infusion. With a total (CR + PR) response rate of 60%, these regimens merit evaluation in broader categories of recurrent brain tumor patients, as well as in patients with newly-diagnosed GBM.
Adolescent↗
Poor-risk non-lymphoblastic lymphoma of childhood: results of an intensive pilot study.
Children with "poor-risk" nonlymphoblastic lymphoma, especially those with marrow or nervous system (CNS) involvement at presentation, have fared poorly even on aggressive chemotherapy regimens. We report here the results of a pilot study of 30 children treated with a highly intensive chemotherapy regimen. This regimen includes an intensive Induction Phase consisting of three cycles of CHOP therapy (cyclophosphamide, doxorubicin, vincristine, and corticosteroids) as well as intensive intrathecal therapy with each cycle. This is followed by a CNS Consolidation Phase consisting of a single cycle of CHOP therapy with five intrathecal doses of "triple" chemotherapy (methotrexate, cytosine arabinoside, and hydrocortisone). Thereafter, a Maintenance Phase consists of alternating cycles of 1) cytosine arabinoside and 6-thioguanine, 2) oral methotrexate and VP-16, and 3) CHOP, for a duration that varied from 36 to 72 wk. Neither debulking surgery nor radiation therapy were recommended. There were 20 patients with Stage III disease (St. Jude's Staging System) and an additional ten patients with bone marrow and/or CNS involvement. The latter group included six patients with B-cell leukemia, three of whom also had CNS disease at presentation. Two additional patients had CNS disease without marrow involvement. Twenty-nine of 30 patients achieved a complete response. Six patients died with recurrent or progressive disease. Twenty-three patients are alive without any adverse events between 21 and 65 mo after diagnosis, with the median time of survival not yet reached (at least 32 mo). All seven adverse events occurred within 7 mo of diagnosis. Event-free survival for all patients is 77%, for Stage III patients is 80%, and for patients with marrow and/or CNS involvement is 70%. This pilot study offers encouragement for improvement in the prognosis of children with "poor-risk" nonlymphoblastic lymphoma and merits evaluation in a Phase III randomized trial in the multicenter cooperative group setting.
Adolescent↗
Patterns of antigen expression in hepatoblastoma and hepatocellular carcinoma in childhood.
Two hepatocellular carcinomas and six hepatoblastomas were examined for the presence of 13 antigens using immunoperoxidase, avidin-biotin, staining techniques. Primary antibodies were directed against alpha-fetoprotein (AFP), alpha-1-antitrypsin (AAT), lysozyme (LYS), carcinoembryonic antigen (CEA), human chorionic gonadotropin (HCG), glial fibrillary acidic protein (GFAP), neuron specific enolase (NSE), epithelial membrane antigen (EMA), hepatitis B surface antigen (HbSA), lactoferrin (LF), desmin (DES), vimentin (VIM), and keratin (KER). Except for HbSA, the antigen staining pattern was unable to differentiate between hepatoblastoma and hepatocellular carcinoma. Both neoplasms where positive for AFP, AAT, CEA, EMA, and KER; however, neither stained for GFAP, NSE, LYS, LF, HCG, or DES. Vimentin was weakly positive in those hepatoblastomas where mesenchymal tissue was present in the tumor. Only the tissue adjacent to hepatocellular carcinomas stained positively for HbSA and correlated with the elevated serum levels of HbSA.
Adolescent↗
Acute B-lymphocytic leukemia with L1 morphology: a report of two pediatric cases.
Two pediatric cases of acute lymphoblastic leukemia with L1 morphology who also demonstrated immunoglobulin on the leukemic cell surface are presented. The first patient's disease progressed on standard induction therapy, despite the presence of good prognostic features at presentation. The second patient has achieved a sustained remission with an aggressive lymphoma regimen. These two cases represent the first report on the outcome of patients with this rare phenotype. They indicate the importance of assessing cell surface immunoglobulin in children with acute leukemia, even in the absence of the L3 phenotype, in addition to the currently utilized batteries of monoclonal antibodies. Failure to recognize this phenotype may have significant therapeutic implications.
Antineoplastic Combined Chemotherapy Protocols↗
Medulloblastoma: presentation, diagnosis and management.
Primitive neuroectodermal tumor or medulloblastoma (PNET/MB) is the most common malignant tumor of the brain in children. Recent progress in the management of this once almost uniformly fatal disease is illustrative of both the advances which have been made in the management of childhood brain tumors and the gaps in our understanding of childhood central nervous system malignancies. Using aggressive surgery and radiotherapy, more than 50% of children with this tumor can be expected to be alive and free of disease five years later. With detailed postoperative evaluation, children with PNET/MB can be stratified into two major subgroups: Those with an average prognosis and those with a poor prognosis. The addition of chemotherapy for children at the highest risk of relapse after treatment with radiotherapy results in an improved duration and rate of survival. However, treatment often results in significant endocrinological and intellectual sequelae.
Adolescent↗
Liver transplantation for primary hepatic cancer in childhood.
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Antineoplastic Combined Chemotherapy Protocols↗
Fatal cardiac toxicity in bone marrow transplant patients receiving cytosine arabinoside, cyclophosphamide, and total body irradiation.
Three patients developed fatal cardiac toxicity from the combination of cytosine arabinoside, cyclophosphamide, and total body irradiation while undergoing preparation for a bone marrow transplant. The pattern of the toxicity was unique for this combination of ablative chemotherapy. All three patients had autopsies demonstrating characteristic myocardial and pericardial toxicity. The cardiotoxic effects of this combination may be averted by lowering the dose of the cyclophosphamide.
Adolescent↗
The Drash syndrome revisited: diagnosis and follow-up.
The Drash syndrome of Wilms tumor, glomerulonephritis and male pseudohermaphroditism (XY gonadal dysgenesis), was first reported in 1967 [Drash et al, 1970; Denys et al, 1967]. Since then reports have pointed to the delayed appearance of some manifestations of the syndrome with specific attention paid to the late development of renal failure, or Wilms tumor, in patients with XY gonadal dysgenesis. Therefore, early accurate diagnosis of this syndrome is important. We report on a phenotypically and genotypically normal girl with Wilms tumor and glomerulonephritis. This report and others, which indicate that the syndrome may be present in patients without ambiguous genitalia, lead us to suggest that all girls with Wilms tumor should be considered at risk for the Drash syndrome. If possible, a careful evaluation of the ovaries at surgery is advisable. Furthermore, vigilant follow-up of renal function in Wilms tumor patients is warranted.
Disorders of Sex Development↗
Haploidentical bone marrow transplantation for osteopetrosis.
HLA-matched bone marrow transplantation is an effective form of treatment for some patients with malignant osteopetrosis, a defect of osteoclast function. Following transplant, normal osteoclasts differentiate from donor-derived marrow stem cells and can function normally in some of these patients. For patients without an HLA-matched marrow donor, pharmacologic treatments have not yet proved effective. This article demonstrates that normal osteoclast function can be obtained following the transplantation of HLA-nonidentical marrow that has been purged of T lymphocytes in vitro.
Antilymphocyte Serum↗
Brain tumors in children. III. Advances in chemotherapy.
In parts I and II of this article, we touched on some of the newer innovative therapeutic approaches that are presently being evaluated in brain tumors in children, such as monoclonal antibody-directed therapy of brain tumors, implantation irradiation techniques, and related techniques. Nevertheless, the potential uses of the many conventional chemotherapeutic agents now available in our treatment of brain tumors in children are far from exhausted. In some senses, pediatric neuro-oncologists have achieved the same level of sophistication, and similar outcome, that our pediatric oncologists achieved 25 years ago in the treatment of Wilms' tumor. By 1960, children with Wilms' tumor were achieving 50% cure rates following early attempts at incorporating chemotherapy into the therapeutic armamentarium. Did researchers anticipate that their coordinated efforts, through multicenter cooperative group trials, would lead to more than 80% cure rates in the 1980s? Of course, there is no preordained guarantee that children with brain tumors will achieve the same therapeutic successes that children with Wilms' tumor have achieved. Nevertheless, our modest successes with chemotherapy over the past 10 years should offer encouragement to our colleagues as well as to our patients. These successes may finally dispel the pessimism and trepidation with which too many physicians have approached the problem of the child with a brain tumor for so long.
Antineoplastic Combined Chemotherapy Protocols↗
Brain tumors in children. I. Advances in diagnosis.
This article emphasizes the contributions that new diagnostic techniques have made toward the management of children with brain tumors. The development of computerized tomographic (CT) scanning has revolutionized both the diagnosis and management of patients with brain tumors and has obviated the previously inevitable delays in diagnosis. The development of magnetic resonance imaging (MRI) has certainly facilitated diagnosis of brain tumors in certain locations with the brain, but it remains unproven in other locations. It is clear that at least some of the early promise of MRI scanning has not been realized. Neither CT nor MRI are able to provide functional detail within the brain, nor are they able to differentiate tumor from peritumoral edema to better delineate the tumor margins. It is hoped that the currently experimental techniques of Positron Emission Tomography (PET) scanning and contrast-enhanced MRI scanning will provide such information in the near future. Neurophysiologic methods of assessing brain tumors merit greater consideration than has been afforded to date. Sensory evoked-potential monitoring provides information about nervous system function. This information is useful both in diagnosis and in monitoring of brain tumors, since the functional information can be localized to discrete regions within the brain. The value of cerebrospinal fluid (CSF) evaluation, both for cytology and tumor markers, cannot be overstated. A significant proportion of childhood brain tumors tend to seed throughout the neuraxis by the CSF pathways. Thus, evaluation of CSF cytology prior to surgical perturbation of the primary tumor should be undertaken whenever safely feasible, in order to avoid the dilemma of postoperative positive CSF cytology and its questionable significance.
Brain Neoplasms↗
Brain tumors in children. II. Advances in neurosurgery and radiation oncology.
In this article, the contributions of neurosurgery and radiation oncology to the management of childhood brain tumors are described. Progress in a particular discipline rarely occurs in an isolated fashion, and it is clear that neurosurgical advances owe much to similar advances in anesthesiology, neuroradiology, and intensive care management. These advances in various disciplines have all permitted bolder yet safer attempts at radical resection by the neurosurgeon. The goal of radical surgical resection is cure in the case of low-grade brain tumors. However, even in those situations where surgery alone cannot be curative, the reduction of tumor bulk facilitates the task of both radiation therapy and chemotherapy, with improvement in progression-free survival as well as overall survival. For those tumors that cannot be cured by surgical resection alone, the standard of therapy thereafter still remains irradiation of the residual tumor. It is clear that the single major advance in the treatment of childhood medulloblastoma has been Cushing's recognition of the value of craniospinal irradiation. Refinements in radiation machines and treatment planning have permitted more accurate delivery of radiation therapy with a slight reduction in toxicity. Newer approaches, such as hyperfractionated irradiation and interstitial irradiation, attempt to improve therapeutic efficacy while minimizing toxicity. Nevertheless, as more children are surviving their brain tumors following surgery and radiation therapy, the price of the successful therapy is being increasingly realized in terms of developmental deficits, particularly in the very young child. It is the desire of all those involved in the management of children with primary brain tumors to seek alternative approaches to wide-field irradiation of the brain in children with high-grade tumors.
Brain Neoplasms↗