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Biomedical subjects

D A Bruce

Publications and source records attributed to D A Bruce.

At least 37 records · Page 2Linked to original sources
PubMed1989

Suprasellar germinomas in childhood. A reappraisal.

Germinomas in childhood may arise in both the suprasellar and pineal region, and outcome has been reported to be worse for suprasellar germinomas with a 5-year survival rate of 20% as compared to 60% for pineal germinoma. To determine the factors impacting on outcome, the results of a uniform treatment approach were evaluated and included primary surgical debulking (PSD) and systemic craniospinal axis radiation (CSRT) for suprasellar germinomas. Between 1976 and 1985 ten consecutive patients (seven females, three males) with the pathologically confirmed diagnosis of suprasellar germinoma were treated. Outcome was compared to four male patients with pineal germinoma treated over the same time period and series of patients reported in the literature. At diagnosis the mean age of patients with suprasellar germinoma was 13.9 years (range, 8.9 to 9.4 years). Symptoms were present for a mean of 18 months (range, 2 to 72 months) prior to diagnosis and included diabetes insipidus, anterior pituitary dysfunction, decreased vision, headache, vomiting, and diplopia. Staging studies, including myelography (n = 4) and cerebrospinal fluid cytology (n = 7), disclosed dissemination in only one child. Surgical treatment included biopsy in three cases, partial resection in five, and total resection in two; no permanent postsurgical complications were noted. The mean radiation therapy dose to the tumor site was 4953 cGy (range, 4400 to 5250 cGy) and to the spine 3354 cGy (range, 3000 to 4000 cGy). Patients were followed for a mean period of 5.1 years (range, 1.9 to 10.5 years). One patient with SG who did not receive treatment initially developed a pineal tumor after diagnosis; she was treated with PSD and CSRT and is asymptomatic 5 years later. All the remaining patients are alive and remain disease-free. Surgical resection and CSRT results in excellent disease control for children with suprasellar germinomas, and outcome is similar to those patients with pineal germinoma.

Adolescent↗
PubMed1988

Results of the treatment of children with recurrent gliomas with lomustine and vincristine.

Gliomas comprise over 50% of all childhood brain tumors. Treatment of recurrent childhood gliomas has been disappointing and the effectiveness of therapy has been difficult to judge because of the variable natural history of the disease. Information gathered recently has suggested that treatment with [1-(2-chloroethyl)-3-cyclohexyl-1-nitrosourea)] (CCNU) and vincristine (VCR) after radiotherapy is effective in prolonging survival in children with newly diagnosed anaplastic gliomas. The authors have used these same drugs--CCNU (100 mg/m2) and VCR (1.5 mg/m2 up to a maximum dose of 2 mg)--in 6-week cycles for a maximum of eight cycles in children with recurrent gliomas. To date, 15 patients have been treated; five patients had malignant gliomas and ten low-grade gliomas. Three children showed improvement, five had stable disease, and seven had progressive disease. Of the five patients with malignant gliomas, four progressed within two cycles of treatment and one had stable disease for 7 months on treatment and then relapsed. Seven of ten children with low-grade gliomas benefitted from treatment and six remain in continuous remission a median of 16 months after initiation of therapy. Three of these children are off all therapy 21, 30, and 30 months after treatment, respectively. Therapy was well tolerated and toxicity consisted primarily of reversible bone marrow suppression. The authors conclude that CCNU and VCR chemotherapy is effective in children with recurrent low-grade gliomas and can result in relatively long-term disease stabilization. In limited experience of the authors, it is not of benefit in children with recurrent anaplastic lesions.

Adolescent↗
PubMed1988

Treatment of chiasmatic/hypothalamic gliomas of childhood with chemotherapy: an update.

Chiasmatic/hypothalamic gliomas (CHG) of childhood may cause progressive neurological and visual deterioration. Radiotherapy results in at least transient stabilization of tumor growth in most patients but may also have adverse long-term effects, especially in young children. Since 1977, children with progressive CHG under 5 years of age at diagnosis have been treated with combination chemotherapy (actinomycin D and vincristine) without radiotherapy. Twenty-four patients, a median of 1.6 years of age at diagnosis, have been treated and followed for a median of 4.3 years (range, 0.3-10 years). All patients are alive. Nine have developed radiographic or clinical progression, occurring a median of 3 years (range, 2-6.5 years) after initiation of treatment. Fifteen of 24 (62.5%) have remained free of progressive disease and have received no other therapy. Tumor shrinkage was documented in 9 of 24 patients but did not clearly relate to long-term outcome. Full-scale intelligence quotient (IQ) obtained a median of 3.5 years after diagnosis in patients who received only chemotherapy was a mean of 103 (range 84-133). We conclude that chemotherapy can significantly delay the need for radiotherapy in children with CHG and such a delay may be beneficial regarding long-term outcome.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1988

Leptomeningeal spread of intramedullary spinal cord tumors. Report of three cases.

Three patients with intramedullary spinal cord tumors and secondary leptomeningeal spread of their tumors are presented. Two patients had astrocytomas and one had a ganglioglioma. Two tumors were located in the cervical spinal cord and one within the thoracic spinal cord. Review of the past and recent literature shows leptomeningeal dissemination of spinal cord tumors to be relatively rare, but it should be suspected and investigated in any patient whose condition deteriorates following removal of a spinal cord neoplasm.

Adolescent↗
PubMed1987

Radioimmunodetection of human glioma xenografts by monoclonal antibody to epidermal growth factor receptor.

Murine IgG2a monoclonal antibody (MAb) 425 specifically detects epidermal growth factor receptor, which is expressed on human gliomas and tumors of other tissue origin but rarely on normal brain tissues, and not at all on bone marrow and peripheral blood cells. 131I-labeled F(ab')2 fragments of this MAb injected into nude mice grafted with U-87 MG glioma cells preferentially localized in tumor tissue compared to normal mouse tissues, as determined by differential tissue counting of radioactivity. The mean tumor-to-tissue ratios of radioactivity ranged between 8.2 (blood) and 55.8 (muscle) at 2 days after the injection of 15 muCi of 131I-425 F(ab')2/mouse. Radiolabeled fragments of an anti-hepatitis virus IgG2a MAb did not localize in tumors. The localization index derived from the ratios of specific antibody to indifferent antibody in tumor tissue relative to blood was 9.94 at 2 days following the MAb injection. The labeled MAb did not localize in a xenograft of colorectal cancer tumor, which does not express the epidermal growth factor receptor. Tumors could be located by whole-body gamma-scintigraphy without background subtraction following the injection of 100 muCi of radiolabeled MAb 425 F(ab')2 fragments. The data suggest that MAb 425 is a likely candidate for clinical diagnostic and radioimmunotherapy trials.

Animals↗
PubMed1987

Pineocytomas of childhood. A reappraisal of natural history and response to therapy.

Pineocytomas are pineal parenchymal tumors composed of differentiated cells histologically similar to those of the mature pineal gland. Little is known about the incidence, pattern of growth, or response to treatment of pineocytomas. Between 1975 and 1985, six children with pineocytomas were treated at our institution, and pineocytomas constituted 11% of all newly diagnosed pineal region tumors. The clinical, radiographic, and pathologic features of these six patients with pineocytomas are presented. Initial treatment for these children included craniospinal plus supplemental local radiotherapy (three), local radiotherapy alone (one), or radiation therapy plus adjuvant chemotherapy (two). Four of six patients had tumor recurrence a median of two years after diagnosis. Three patients had leptomeningeal dissemination, one at the time of diagnosis and two following therapy. Our findings suggest that: biopsy is necessary to distinguish pineocytomas from other pineal region tumors; radiation therapy alone is inadequate; and these tumors are aggressive in the pediatric population, with a high propensity for leptomeningeal dissemination.

Adolescent↗
PubMed1987

Outcome of children with primary intramedullary spinal cord tumors.

The influence of clinical and treatment factors on the outcome of children with primary intramedullary spinal cord tumors (PST) was evaluated by reviewing the records of 26 children diagnosed during the 15-year period 1970-1984. Five-year survival was 39%, but 5-year event-free survival (EFS) was only 14%. Eighteen-month EFS was 53% (9/17) among children with low-grade astrocytoma. 100% (2/2) with ependymoma, and 0 of 7 with anaplastic astrocytoma or ganglioglioma. There was no significant difference in the 18-month EFS by location of tumor, duration of symptoms, or extent of surgical removal. Five of 9 children with locally recurrent PST had a second operation, and 4 were alive a median of 56 months later. PST disseminated to the leptomeninges or the III ventricle in 5 children: 2 at diagnosis, 2 as the first sign of disease relapse, and 1 after local recurrence. Given the poor outcome of our children, different methods of treatment for children with tumors in this location should be evaluated.

Adolescent↗
PubMed1987

Results of a pilot study of hyperfractionated radiation therapy for children with brain stem gliomas.

The majority of children with brain stem gliomas develop progressive disease within 18 months of diagnosis and treatment. Radiotherapy (RT) is of transient benefit in most patients and higher total doses of RT have been related to improved survival. The amount of RT which can be given is limited by the tolerance of the surrounding brain. Hyperfractionated RT theoretically allows higher doses of RT to be tolerated by the brain. Sixteen children with brain stem gliomas were treated on a hyperfractionated RT schedule, receiving 120 cGy of RT twice daily, to a total dose of 6480 cGy. All patients tolerated treatment well. Eleven of 15 (73%) evaluable patients had a response to treatment and two (13%) others had stable disease. One patient developed progressive disease during treatment. All patients were tapered off steroids by the completion of treatment. Thirteen of 16 (81%) patients developed progressive disease at a median of 7 months after diagnosis and three remain in remission 8, 12, and 15 months following diagnosis. These results were similar to those of historical controls. Two patients were surgically explored at time of relapse and 5 have had an autopsy. No acute or subacute neurologic toxicity was seen; but long-term detrimental effects on brain could not be assessed. The implications of this study are that escalations of the dose of hyperfractionated RT can be entertained for children with brain stem gliomas.

Adolescent↗
PubMed1987

Dandy-Walker syndrome and associated anomalies.

This is a retrospective study of 28 infants and children with Dandy-Walker syndrome: 16 had a complete postmortem examination and 12 are currently being followed clinically. Eleven of sixteen in the first category had hydrocephalus; 10 of those 11 had other central nervous system malformations, and 6 also had visceral anomalies. Four of the five without hydrocephalus had complex cardiac malformations and other visceral anomalies whereas 1 died as a consequence of acute myelogenous leukemia. The 12 children still under surveillance manifest no clinical evidence of significant visceral or central nervous system malformation, although 1 has CT scan evidence of agenesis of the corpus callosum. The majority (9/12) of these children have normal intelligence although 40% have focal neurologic deficits or gait abnormalities and 60% have an abnormal EEG. Frequent association of other malformations and/or visceral anomalies in children with Dandy-Walker syndrome make it difficult to propose a unifying hypothesis relative to embryogenesis of the syndrome.

Abnormalities, Multiple↗
PubMed1987

Quality of life in children with primitive neuroectodermal tumors (medulloblastoma) of the posterior fossa.

We reviewed our experience in 43 consecutive patients with primitive neuroectodermal tumors (medulloblastoma), PNET (MB), treated between 1975 and 1984, to characterize their quality of life and identify factors which impacted on long-term function. Twenty-four of forty-three (56%) of children are alive and free of disease, a median of 4.5 years after diagnosis. The quality of life was analyzed for the 24 long-term survivors. 79% (19 of 24) were functioning well in everyday activities. The median full-scale intelligence quotient (FSIQ), obtained a median of 3.5 years after diagnosis for those tested (n = 17) was 97, with all but 3 (12%) of the patients functioning in the normal range. Specific learning, memory and fine-motor disabilities were found in over one half of patients. Factors associated with poorer performance and lower FSIQ included preoperative obtundation, the need for a permanent shunt, younger age at diagnosis, and a complicated postoperative course. It is concluded that (1) the majority of long-term survivors have 'normal' intellectual function, but may have specific intellectual and academic disabilities, and (2) preoperative and postoperative factors strongly impact on the quality of life of survivors.

Adolescent↗
PubMed1987

The shaken baby syndrome. A clinical, pathological, and biomechanical study.

Because a history of shaking is often lacking in the so-called "shaken baby syndrome," diagnosis is usually based on a constellation of clinical and radiographic findings. Forty-eight cases of infants and young children with this diagnosis seen between 1978 and 1985 at the Children's Hospital of Philadelphia were reviewed. All patients had a presenting history thought to be suspicious for child abuse, and either retinal hemorrhages with subdural or subarachnoid hemorrhages or a computerized tomography scan showing subdural or subarachnoid hemorrhages with interhemispheric blood. The physical examination and presence of associated trauma were analyzed; autopsy findings for the 13 fatalities were reviewed. All fatal cases had signs of blunt impact to the head, although in more than half of them these findings were noted only at autopsy. All deaths were associated with uncontrollably increased intracranial pressure. Models of 1-month-old infants with various neck and skull parameters were instrumented with accelerometers and shaken and impacted against padded or unpadded surfaces. Angular accelerations for shakes were smaller than those for impacts by a factor of 50. All shakes fell below injury thresholds established for subhuman primates scaled for the same brain mass, while impacts spanned concussion, subdural hematoma, and diffuse axonal injury ranges. It was concluded that severe head injuries commonly diagnosed as shaking injuries require impact to occur and that shaking alone in an otherwise normal baby is unlikely to cause the shaken baby syndrome.

Battered Child Syndrome↗
PubMed1987

Symptomatic Arnold-Chiari malformation: review of experience with 22 cases.

Twenty-two patients with closed myelomeningoceles, shunted hydrocephalus, and symptomatic Arnold-Chiari malformations were studied retrospectively. Seventeen of the 22 patients were aged 6 months or younger; five patients were aged 3 to 23 years. Patients in the younger group presented with stridor, apnea, and/or feeding difficulty; those in the older group presented with hemiparesis, quadriparesis, oscillopsia, nystagmus, or opisthotonos. Fourteen of the 17 younger patients underwent surgical decompression of the Arnold-Chiari malformation: 10 within 18 days following the onset of symptoms and four on Day 19 or later. Of the 10 infants with early treatment, five eventually died secondary to continued symptoms and five survived. Of those surviving, three were asymptomatic and two had continuing symptoms but were improved over their preoperative state. Among the four patients undergoing surgery later, two died, one had lessening of stridor, and one had complete relief of symptoms. Of the three infants not undergoing decompression, two died and one eventually became asymptomatic. All five of the older patients underwent decompression and all had complete resolution of their preoperative symptoms and signs. The authors conclude that while decompression of a symptomatic Arnold-Chiari malformation may be effective in children and adults, this treatment does not always improve the clinical condition of infants. This may be the result of ischemic/hypoxic effects on the infant's brain stem, which may not be organized normally at birth.

Adult↗
PubMed1987

Neurosurgical considerations in cranio-orbital surgery.

The neurosurgeon is an integral member of the craniofacial team. The role of the neurosurgeon in the preoperative planning, intraoperative management, and postoperative follow-up of patients with craniofacial malformations is reviewed.

Brain↗