Papers by Patricia Duffner
CRC Press eBooks, Dec 26, 2003
The New England Journal of Medicine, Jun 17, 1993
Journal of Neurosurgery, Sep 1, 1981
✓ Primitive neuroectodermal tumors are found in the cerebrum of children and young adults. They a... more ✓ Primitive neuroectodermal tumors are found in the cerebrum of children and young adults. They are clinically highly malignant and have a rapid course from diagnosis to death. Their microscopic pathology reveals 90% to 95% nondifferentiation, frequent mitoses, and small dark cells with no observable cytoplasm. This paper discusses the treatment of three children with this tumor with a combination of surgery, irradiation, and combinaton chemotherapy. The results of this approach are compared with previous reports in the literature. The average survival in this series is 24 months versus approximately 8 months reported in the literature.
Neurology, Feb 1, 1984
Seventeen children with CNS leukemia treated with chemotherapy and 5 children treated with both c... more Seventeen children with CNS leukemia treated with chemotherapy and 5 children treated with both cranial radiation (CRT) and chemotherapy were evaluated. Eighty-eight percent of patients treated with chemotherapy alone had CT abnormalities, and all treated with CRT and chemotherapy had abnormal CT. The severity of CT abnormality paralleled intraventricular methotrexate levels and clinical signs of leukoencephalopathy. Children who receive chemotherapy for CNS leukemia, even without cranial irradiation, are more likely to have leukoencephalopathy than children without CNS leukemia. Moreover, patients with CNS leukemia may have abnormalities of CSF clearance of intraventricularly administered drugs.
Neurology, Apr 1, 1979
Central nervous system neoplasms are the most common solid tumours that develop in children, with... more Central nervous system neoplasms are the most common solid tumours that develop in children, with the greatest proportion located in the infratentorium. The 2016 World Health Organization Central Nervous System tumour classification evolved from the 2007 edition with the integration of molecular and genetic profiling into the diagnosis, the addition of new entities and the removal of others. Radiology can assist with the subtyping of tumours from certain characteristics described below to provide prognostic information and guide further management. The latest insights into the radiologic characteristics of these posterior fossa tumours are presented below: medulloblastoma, ependymoma, pilocytic astrocytoma, embryonal tumours with multilayered rosettes, atypical teratoid rhabdoid tumours, diffuse midline glioma and the new entity of diffuse leptomeningeal glioneuronal tumours.
A case of central core disease in one of identical twins is presented. The light and electron mic... more A case of central core disease in one of identical twins is presented. The light and electron microscopic pathology is reviewed. We believe that these entities are not genetic in this sibship and may be an example of isolated maturational arrest of non-specific aetiology. Central core disease was first described by Shy and Magee in 1956. The disease was seen in five members from three generations of the same family, suggesting a dominant mode of inheritance. The clinical features are hypotonia, delayed motor milestones, and a mild, non-progressive proximal myopathy. We wish to report a case of central core disease in one of identical twins. To our knowledge this occurrence has not previously been described. An associated finding in this child is the presence of arthrogryposis multiplex congenita. Although there have been cases of congenital arthrogryposis occurring in either both or one of identical twins (Hillman and Johnson, 1952; Lipton and Morgenstern, 1955) in none of the cases of arthrogryposis multiplex congenita associated with monozygotic twinning have muscle biopsies been done to delineate specific pathology. This report will document the existence of central core disease in one of monozygotic twins, establish the identical nature of the twinning, and speculate on the aetiology of the arthrogryposis associated with the central core disease. Case report SD, a one year old twin boy, was the product of an eight month pregnancy and breech delivery. He was the second born of identical twins. There was a single placenta. Birthweight was 19 kg (four pounds, five ounces). Twin A weighed 2 kg (four pounds, 10 ounces) and was a vertex presentation. After delivery it was noted that the baby was
CA: A Cancer Journal for Clinicians, Sep 1, 1985
States is 2.4 per 100,000, which means that brain tumors are diagnosed in approx imately 1,200 to... more States is 2.4 per 100,000, which means that brain tumors are diagnosed in approx imately 1,200 to 1,500 children each year.' The yearly accrual of the various histologic subtypes of brain tumors is, therefore, cor respondingly small. In addition, because only about 50 percent of children with brain tumors are referred to university or cancer treatment centers,2 therapeutic trials, by default rather than design, have been re stricted to small numbers of patients. Another complication is the lack of a universally accepted histologic classifica tion or grading system for brain tumors. The World Health Organization (WHO) classification has been inadequate for a substantial proportion of brain tumors in children. The lack of uniformity in clas sification results in the inability to accu rately evaluate prognosis based on histology from one center to another. Similarly, re ported responses to treatment are often dif ficult to assess. In addition, there are neither well established clinical and surgical staging criteria, nor universally accepted defini tions of response and relapse. As with other tumorsof childhood, staging systemsfor braintumorsmay establish guidelines that permit prognosis based on extent of disease at diagnosis and postoperatively. Medul loblastoma has been the only CNS tumor for which an effort has been made to cor relate surgical staging with prognosis. Subsequent evaluations have indicated that staging in this tumor may have prognostic significance.
Pediatrics, 2004
We present a case in which human coronavirus was detected in the cerebrospinal fluid of a child p... more We present a case in which human coronavirus was detected in the cerebrospinal fluid of a child presumed to have acute disseminated encephalomyelitis. In murine models, coronavirus has been found to cause a chronic demyelinating condition that resembles multiple sclerosis. Additionally, there is in vitro evidence of human coronavirus's ability to infect neural cells. This case report provides additional support for the hypothesis that coronavirus may be an important etiologic factor in the pathogenesis of demyelinating disease in humans.
Journal of Neuropathology and Experimental Neurology, Nov 1, 1984
The American Journal of Surgical Pathology, Sep 1, 1998
Fifty-five patients with atypical teratoid/rhabdoid tumors of the central nervous system were stu... more Fifty-five patients with atypical teratoid/rhabdoid tumors of the central nervous system were studied to define the clinical and pathologic features of this newly described neoplasm. The lesion occurred primarily in children younger than 2 (mean age at diagnosis, 17 months). The neoplasms were located in the posterior fossa (36 patients) and the supratentorial compartment (17 patients) or were multifocal in both compartments (2 patients) at presentation. Histologically, the tumors were composed of small cells and large, pale cells in a jumbled architectural arrangement. The small cell component resembled medulloblastoma and occasionally had cords of cells in a mucinous background, simulating chordoma. The cytoplasm of the larger cells was conspicuous with a somewhat "rhabdoid" appearance, although rhabdoid features were not always prominent. Epithelioid features in the form of poorly formed glands or Flexner-Wintersteiner rosettes were noted in a minority of lesions. The neoplasms showed striking polyphenotypic immunoreactivity, including that for vimentin, glial fibrillary acidic protein, epithelial membrane antigen, cytokeratins, synaptophysin, chromogranin, and smooth muscle actin. Using a probe for chromosome 22, seven of eight scorable cases showed a solitary signal by fluorescence in situ hybridization (FISH) consistent with monosomy 22. The eighth scorable case showed three signals by fluorescence in situ hybridization and had a translocation involving chromosome 22 reported by conventional cytogenetics. In contrast to patients with medulloblastoma, the neoplasm with which these lesions are often confused, the outcome of the patients was uniformly poor. The mean postoperative survival of patients with atypical teratoid/rhabdoid tumors was only 11 months. Local recurrence, seeding of the cerebrospinal fluid pathways, or both, were common terminal events. This study underscores the distinctive clinical, histopathologic, immunohistochemical, and cytogenetic character of this unusually aggressive tumor.
Eight patients with recurrent medulloblastoma were treated with a chemotherapy regimen consisting... more Eight patients with recurrent medulloblastoma were treated with a chemotherapy regimen consisting of vincristine, BCNU, dexamethasone and intrathecal and intermediate dose intravenous methotrexate (500 mg/m/sup 2/). Five also received local low dose radiotherapy (RT). All 8 patients responded to treatment; 6 completely and 2 partially. These latter 2 were in their second and third recurrences. Three remain in remission. The median duration of response was 18.8 months, and median time from start of chemotherapy to death was 32 months using the Kaplan-Meier technique. In addition, 9 other patients with newly diagnosed medulloblastoma were treated with craniospinal radiation and the same adjuvant chemotherapy as above. The first 5 patients also received intraventricular methotrexate and/or intravenous BCNU during radiotherapy. The toxicity in the 5 patients was very severe. There were three toxic deaths, one death from cancer; one patient survives disease-free, but he is demented. With...
Journal of Clinical Oncology, 1996
PURPOSE The outcome for patients with recurrent medulloblastoma has historically been poor, with ... more PURPOSE The outcome for patients with recurrent medulloblastoma has historically been poor, with most patients dying of disseminated disease. Here, we report on seven patients with recurrent medulloblastoma, most heavily pretreated with a variety of chemotherapeutic agents, including parenteral etoposide (VP-16), who showed responses to the administration of repeated courses of low-dose oral VP-16. PATIENTS AND METHODS Seven patients age 4 to 16 years were treated with VP-16 after neuroradiographic and clinical evidence of tumor progression. Six had received prior irradiation. All seven had been pretreated with a variety of chemotherapeutic agents and schedules, including parenteral VP-16. VP-16 was administered orally as repeated 21-day courses at 50 mg/m2/d with a 7-day interval between courses. Evaluation consisted of neuroradiographic and clinical examination after completion of every two courses of therapy. Complete blood cell counts were performed weekly. RESULTS The major tox...
The neurologist, 2004
As the number of long-term survivors of childhood cancer has grown, it has become increasingly cl... more As the number of long-term survivors of childhood cancer has grown, it has become increasingly clear that central nervous system therapy may have serious long-term effects on cognition and endocrine function. These complications have been studied most extensively in children with brain tumors and leukemia. Children with acute lymphoblastic leukemia previously treated with cranial irradiation are at risk for cognitive decline. Chemotherapy-only regimens, which rely on high-dose frequently administered methotrexate, are also associated with producing cognitive dysfunction. Children irradiated for brain tumors are even more vulnerable. Risk factors include perioperative morbidity, young age, large-volume high-dose cranial irradiation, supra-tentorial location of tumor, moyamoya syndrome, and leukoencephalopathy. Cognitive decline is progressive over at least a decade. The most common radiation-induced endocrinopathies are hypothyroidism and growth hormone deficiency. Treatment effects ...
Pediatric pharmacology (New York, N.Y.), 1982
The absorption and disposition of rectally administered secobarbital was studied in ten epileptic... more The absorption and disposition of rectally administered secobarbital was studied in ten epileptic children, ages 2-13 yrs. Five subjects received secobarbital rectally in solution, and the other five received secobarbital suppositories. Concentration of secobarbital in serum was serially determined during 48 hrs after a single rectal dose of about 5 mg/kg. The rate of absorption of secobarbital, as measured by the time to reach peak serum concentration, was much more rapid from the solution than the suppository (0.92 +/- 0.47 hr vs 4.60 +/ 2.30 hr). The peak serum concentration of secobarbital in the solution group was consistently higher than in the suppository group (2.26 +/- 0.37 micrograms/ml vs 1.35 +/- 0.24 microgram/ml). None of the individual peak serum concentrations exceeded 3 micrograms/ml, which is well below the previously reported minimum toxic concentration of secobarbital (ie, 6 microgram/ml). The elimination half-life of secobarbital varied over a wide range, from 2...
Handbook of Brain Tumor Chemotherapy, 2006
ABSTRACT: Pediatric gliomas are a heterogeneous group of brain tumors that include low-grade and ... more ABSTRACT: Pediatric gliomas are a heterogeneous group of brain tumors that include low-grade and high-grade malignancies. Historically, treatment for these tumors has been a combination of surgery and radiation therapy. In certain situations, addition of chemotherapy to treatment regimens has proven beneficial in the treatment of pediatric gliomas. This chapter will address the uses of chemotherapy in the treatment of pediatric gliomas, including low-grade gliomas, optic pathway/hypothalamic gliomas, high-grade gliomas, and diffuse intrinsic pontine gliomas.
Uploads
Papers by Patricia Duffner