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Biomedical subjects

R J Packer

Publications and source records attributed to R J Packer.

At least 181 records · Page 10Linked to original sources
PubMed1984

Intracranial embryonal cell carcinoma.

Intracranial embryonal cell carcinoma (ECC) is a germ cell tumor most frequently found in the pineal region. However, little is known about the incidence, pattern of growth, or response to treatment of ECC. Between 1975 and 1983, 16 consecutive patients younger than age 18 with tumors of the pineal region have had biopsies performed prior to treatment, and 5 (31%) have had primary intracranial ECC. One other child had ECC in the suprasellar region. The clinical, radiographic, and histologic features of the six patients with ECC are presented and contrasted with the findings in children with other pineal region neoplasms. Pathologic confirmation is necessary to distinguish ECC from other intracranial tumors. Three patients were treated with radiation therapy (RT) plus adjuvant chemotherapy; two patients were treated with chemotherapy followed by RT; and one was treated with RT only. All patients initially responded to therapy, but only one has survived for longer than 1 year. It is concluded that biopsy is necessary for the diagnosis of ECC; that ECC is more common than currently believed; and that current treatment for intracranial ECC is unsatisfactory. Biopsy of pineal region tumors is necessary if progress is to be made in their management.

Adolescent↗
PubMed1984

Neurological complications of bone marrow transplantation in childhood.

Bone marrow transplantation, used in the treatment of cancer, aplastic anemia, and metabolic diseases, involves the use of potentially neurotoxic agents to suppress immunity and eradicate malignancy. Fifty-seven patients with a median age of 11 years (age range, 6 months to 24 years) underwent bone marrow transplantation at the Children's Hospital of Philadelphia. Fifty-nine percent developed neurological abnormalities. Twenty-six patients (46%) had central nervous system (CNS) dysfunction, including infection (8), cerebrovascular accident (5), CNS leukemia (7), metabolic encephalopathy (5), and paraparesis with CNS toxoplasmosis (1). Neuropsychological dysfunction was present in 4 of 5 long-term survivors who were tested. Fourteen of 19 patients (74%) on whom postmortem examination was performed were found to have CNS abnormalities, including cerebral atrophy (10), focal cerebral injury (6), leukemia (5), and infection (3). Fourteen patients (24%) had peripheral nervous system dysfunction. CNS dysfunction was more common in patients with lymphoreticular malignancies. Cerebrovascular accidents (in patients with lymphoreticular malignancies) and infections (in our general population and in patients with lymphoreticular malignancies) occurred more often in our patients than in patients with similar illnesses who did not undergo bone marrow transplantation. The combination of prior treatment and preparative therapy for bone marrow transplantation predisposes patients to neurological and neuropsychological sequelae.

Adolescent↗
PubMed1984

Prognostic importance of cellular differentiation in medulloblastoma of childhood.

Medulloblastoma is the most common intracranial primitive neuroectodermal malignancy of childhood. Certain parameters are predictive of survival in children with medulloblastoma; however, tumor histology is of unclear prognostic value. A classification system, proposed by Rorke for all central nervous system (CNS) neoplasms composed of primitive neuroepithelial cells, was utilized in a review of 38 consecutive patients with newly diagnosed medulloblastoma. The classification is based on the concept that medulloblastoma is not unique to the cerebellum but is similar to tumors that may arise elsewhere in the CNS consequent to neoplastic transformation of primitive neuroepithelial cells. Cells forming the tumors may remain in the undifferentiated state or they may exhibit differentiation along glial, and/or ependymal, and/or neuronal lines. For purposes of simplification, the cases were divided into two major groups: those primitive neuroectodermal tumors (PNET's) which showed no evidence of cellular differentiation (PNET-U) and those that were differentiated (PNET-D). There were 20 cases in the PNET-U group and 18 in the PNET-D group. The 4-year survival rate was 70% for PNET-U, compared to 32% for PNET-D (p = 0.004). Only one of 10 children with PNET-D with differentiation along more than one cell line survived. Other factors, including age at diagnosis, tumor metastasis (TM) stage, and extent of surgical resection, were analyzed and were of prognostic importance; but histological features remained statistically significant within each subgroup.

Adolescent↗
PubMed1984

Pineal region tumors of childhood.

The incidence, response to treatment, and outcome of children with pineal region neoplasms is poorly characterized. Since 1975, in one institution, 25 consecutive patients with pineal tumors have undergone biopsy prior to further treatment. This constituted 11% (25/234) of all brain neoplasms seen over this time period. Specific tumors diagnosed included pineal parenchymal tumors ( pineoblastomas , pineocytomas ) in eight patients (32%); germ cell tumors (embryonal cell carcinomas, teratomas, germinomas) in eight patients (32%); glial tumors (astrocytoma, ganglioglioma ) in eight patients (32%); and ganglioneuroblastoma in one patient (4%). Clinical parameters, computed tomographic findings and CSF markers (alphafetoprotein and human chorionic gonadotropin) were unreliable in discriminating between specific tumor types. Response to treatment and patterns of disease relapse were dependent on the type of tumor present. Five of eight children with pineal parenchymal tumors had disease recurrence, and in all leptomeningeal dissemination occurred prior to or concurrent with local relapse. Three of eight children with germ cell tumors and two of eight patients with glial tumors suffered a relapse; in all five children recurrence was initially local. Findings suggest that pineal region neoplasms are not infrequent in childhood; that these tumors vary greatly in histologic type; that contrary to other reports germinomas do not constitute the majority of pineal tumors; and that histologic confirmation is necessary prior to treatment for appropriate management.

Adolescent↗
PubMed1983

Brainstem glioma: clinical manifestations of meningeal gliomatosis.

Brainstem gliomas of children are variably malignant tumors that rarely have been reported to produce subarachnoid dissemination. Nevertheless, during a two-year period, 5 of 15 such patients treated by us developed symptoms of leptomeningeal metastases. The diagnosis of an anaplastic astrocytoma with meningeal gliomatosis was confirmed postmortem in all 5. In 3 children, meningeal symptoms preceded other signs of posterior fossa recurrence. Symptoms of meningeal gliomatosis included local or radiating back pain (5 patients), segmental weakness (3), paresthesia (2), and incontinence (2). Myelography, performed in 4 patients, was the most useful diagnostic technique, disclosing multiple intradural filling defects or a high degree of block in 3 patients. Although the cerebrospinal fluid was abnormal in all 4 examined patients, in only 1 were malignant cells detected. Prolonged survival, which appears to predispose to dissemination of adult malignant gliomas, was not an apparent factor in our patients.

Adult↗
PubMed1983

High dose systemic methotrexate-associated acute neurologic dysfunction.

Two patients treated with high dose methotrexate otrexate with citrovorum rescue (HDMTX-CF) for osteogenic sarcoma developed the acute onset of neurologic deficits. Prior to the onset of symptoms, one child suffered brief episodes of altered consciousness. Both patients developed hemiparesis and then steadily improved over 72 hours. Laboratory evaluations disclosed normal coagulation parameters and nontoxic serum methotrexate levels at onset of symptoms. Computed tomography in one child disclosed a large low absorption abnormality. This patient represents the first reported case of positive radiologic findings associated with this syndrome. The two patients recovered completely and received further HDMTX-CF without sequelae. This condition may result from transient demyelination or embolic cerebrovascular disease.

Adolescent↗
PubMed1983

Enhanced cis-platinum neurotoxicity in pediatric patients with brain tumors.

Eight children were treated with cis-platinum for recurrent brain tumor. Six of these children were fully evaluable. All received cis-platinum (total dose per course 60 to 120 mg/m2; up to three courses) by infusion with mannitol and hydration. All had received one or more courses of cranial irradiation. Five of the six patients had evidence of significant hearing loss after only one cycle of treatment. All six patients demonstrated significant audiometric or symptomatic hearing loss after a total dose of 110-120 mg/m2. In patients further challenged with cis-platinum hearing loss progressed significantly. Two patients developed profound deterioration in neurologic status within 72 h after infusion. These experiences suggest that Ototoxicity is accentuated in patients who have received cranial irradiation. Further treatment may lead to prohibitive hearing loss. Severe neurologic deterioration may occur secondary to drug infusion.

Adolescent↗
PubMed1983

Cerebral gangliogliomas during childhood.

We have reviewed our experience with gangliogliomas treated in the post-computed tomography (CT) era at the Children's Hospital of Philadelphia. Of 234 newly histologically verified neoplasms seen at our institution since 1975, 10 (4.3%) were gangliogliomas of the cerebral hemispheres. The presenting complaint was seizures in 9 of 10 patients, and in 8 the seizures were poorly controlled despite increasing doses of anticonvulsant medication. At the time of diagnosis, only 2 patients had a focal neurological deficit and none had signs or symptoms of increased intracranial pressure. Learning disability and behavioral disturbances were common in this group of children. The CT appearance of these lesions was characteristic: most appeared as a cerebrospinal fluid density area that was located peripherally and often indented the skull. There was little contrast enhancement, and a few were diagnosed initially as arachnoid or porencephalic cysts. Despite the CT appearance, all but 2 of the lesions were found to be solid at operation. In one patient, the lesion appeared as an enhancing lesion of the thalamus, and this patient died. Operation resulted in symptomatic improvement. Eight of 9 children are alive, with a follow-up of 1 to 80 months (median, 19.5 months), and are free of progressive disease. Five are seizure-free while receiving anticonvulsant therapy and an additional 3 have improved seizure control as a result of operation. Two of the 3 children with intellectual difficulties preoperatively have shown improvement on testing after operation, probably the result of improved seizure control. One patient developed a disseminating malignancy and died. It is concluded that worsening seizures in the pediatric age group should warrant CT examination and that ganglioglioma should be included in the differential diagnosis of low density areas on CT.

Adolescent↗
PubMed1983

Chiasmatic gliomas of childhood. A reappraisal of natural history and effectiveness of cranial irradiation.

Disagreement exists concerning the natural history and treatment of chiasmatic gliomas (CG) of childhood. We reviewed our experience in 21 cases of surgically verified CG with a median age of 4 years at time of diagnosis, followed for a median of 5.3 years (range 3-14.8 years). Initial treatment included radiation therapy (RT) in 18 patients, chemotherapy in 2, and observation in 1. Disease recurrence, defined as progressive visual or neurological deterioration, was documented in 10 children (48%), occurring at a median of 6 years after diagnosis. 5-year actuarial survival was 89%, but fell to 60% by 10 years. Visual improvement after RT was uncommon, occurring once. Intellectual deficits were noted in 5 of 17 survivors. We compared our results to that of other patients reported and concluded that: (1) CG may act aggressively independent of their location in the visual pathway at time of diagnosis; (2) the beneficial effects of RT are difficult to document; (3) progressive disease may occur late in the course of illness, and (4) intellectual sequelae are common in long-term survivors.

Astrocytoma↗
PubMed1982

The diagnostic value of electromyography in infantile hypotonia.

There is controversy over the usefulness of electromyography (EMG) in the examination of hypotonic infants with suspected neuromuscular disease. We compared the initial EMG findings of 51 such children under 1 year of age with their final clinical diagnoses determined by independent means. The EMG predicted the final diagnosis in 82% of infants less than 4 months of age and 85% of those over 4 months of age. Botulism was correctly identified by EMG in nine of 11 cases. The EMG diagnosis was identical to that obtained by muscle biopsy in 64% of cases in which biopsy was done, and diagnosis obtained by the two methods were inconsistent in only 14%. Electromyography is a sensitive and noninvasive diagnostic tool for the diagnosis of neuromuscular disease in infantile hypotonia.

Electromyography↗
PubMed1982

CT parenchymal abnormalities in bacterial meningitis: clinical significance.

Over a 5 year period, 80 children with acute bacterial meningitis underwent computed tomography (CT) because of neurologic complications. In nine children, CT disclosed parenchymal abnormalities. The type of parenchymal abnormality was classified as mild, moderate, or severe on the basis of the severity and location of brain injury. All children had neurologic deficits at the time they underwent CT. The seven surviving children were followed for a mean of 32 months. The four children with either mild or moderate CT changes recovered without neurologic sequelae. The five children with severe CT abnormalities either died (two) or survived with major neurologic (two mentally retarded; three major motor deficits) sequelae. Computed tomography is of prognostic value in children with bacterial meningitis.

Cerebral Cortex↗
PubMed1981

Focal encephalopathy following methotrexate therapy. Administration via a misplaced intraventricular catheter.

Two patients with lymphoreticular malignant neoplasms and leptomeningeal tumor spread were treated with intraventricular administration of methotrexate via an Ommaya reservoir. The intraventricular instillation of methotrexate resulted in focal CNS damage in brain parenchyma inadvertently pierced by the shunt. Disorientation, aphasia, and right hemiparesis developed in both patients. Computed tomography disclosed a contrast-enhancing, low-density mass in the left cerebral cortex where the distal shunt tip was lodged. In one case the lesion was pathologically consistent with chronic inflammation. A lesion occurred in one patient despite repositioning of the shunt prior to methotrexate instillation. Shunt removal and administration of systemic corticosteroids resulted in resolution of symptoms in one patient.

Adolescent↗
PubMed1980

Creutzfeldt-Jakob disease in a 20-year-old woman.

A 20-year-old woman developed ataxia, extrapyramidal movements, myoclonus, and progressive dementia. Brain biopsy disclosed status spongiosus, diagnostic of Creutzfeldt-Jakob disease; this is the youngest spontaneous case ever reported. Creutzfeldt-Jakob disease can occur in young adulthood.

Adult↗
PubMedDate not supplied

An integrated approach to the treatment of chiasmatic-hypothalamic gliomas.

The treatment of visual pathway gliomas is controversial. The many retrospective studies reporting outcome data for patients with chiasmatic/hypothalamic gliomas are difficult to interpret for several reasons. First the natural history of these tumors is erratic with some reports suggesting that most visual pathway gliomas are hamartomas and follow an indolent course, and others reporting 10-year survival rates of close to 60%. Second, earlier studies did not clearly indicate which patients had neurofibromatosis type 1 (NF1) and recent evidence suggests that the natural history of optic gliomas is more favorable in patients with NF1. Third the methods and accuracy of diagnosis have changed dramatically and patients diagnosed before and after the advent of CT/MR imaging have often been included in the same series. While surgical resection is usually not a viable option for definitive treatment of these tumors, recent studies have shown favorable results after subtotal resection in selected patients. The efficacy of radiotherapy has not been unequivocally demonstrated and treatment-related morbidity has become a major concern, in particular, adverse effects on cognition and growth. Chemotherapy has been advanced as an viable alternative to avoid or delay the adverse affects of RT, but the long-term outcome benefits and adverse effects of treatment are just being defined. Despite the limitations of currently available information, sufficient data are now available to rational management quotelines for the majority of children with chiasmatic/hypothalamic gliomas.

Antineoplastic Agents↗
PubMedDate not supplied

Isolated angiitis of CNS: treatment with pulse cyclophosphamide.

A 12-year-old boy with a history of "migraine" headache presented with an increasingly severe headache accompanied by emesis and unsteadiness. Evaluation revealed an acute cerebral hemorrhage with subsequent angiographic studies demonstrating multiple areas of segmental narrowing in both the anterior and posterior cerebral circulations. He was diagnosed with isolated angiitis of the central nervous system and high-dose steroid therapy was administered to which he responded for 6 months. Symptoms recurred and repeat angiography demonstrated persistent segmental narrowing. Pulse cyclophosphamide therapy was begun with resolution of symptoms and normalization of angiography following 6 treatments. Although rare in children, isolated angiitis of the central nervous system can occur in children and aggressive immunosuppression should be considered as the mode of therapy.

Cerebral Angiography↗
PubMedDate not supplied

Management of children with primitive neuroectodermal tumors of the posterior fossa/medulloblastoma.

Primitive neuroectodermal tumors of the posterior fossa/medulloblastoma (PNET-MB) are the most common malignant primary central nervous system tumors of childhood. Current approaches to therapy for children with PNET-MB are illustrative of both the advances which have been made in management of childhood brain tumors and the areas where increased understanding is needed. With the optimal use of surgery and radiotherapy, the majority of children with PNET-MB can be expected to be alive and free of disease 5 years after diagnosis. Based on various factors, including the age of patient, the extent of the disease at the time of diagnosis and histological parameters, it is possible to stratify patients with PNET-MB into 'risk' groups. It is unclear whether preoperative or postoperative factors are most predictive of outcome. Patients with factors predictive of a higher likelihood of disease relapse seem to benefit from treatment with chemotherapy. The amount of radiotherapy and the type and amount of chemotherapy which is most efficacious in controlling the disease has yet to be determined. Children surviving PNET-MB are at high risk for endocrinologic and neuropsychologic sequelae. Multiple prospective treatment protocols are now underway, attempting to determine which therapy is best at controlling disease without causing severe long-term damage. A multidisciplinary approach is needed for the treatment of children with PNET-MB.

Brain Neoplasms↗
PubMedDate not supplied

Management of intracranial neoplasms in children with neurofibromatosis type 1 and 2. The Children's Cancer Study Group.

The association of central nervous system neoplasms in neurofibromatosis is common and children are at particular risk. Recent advances in neurodiagnostic techniques allow for non-invasive detection of these tumors, and often these tumors are found in asymptomatic patients. Due to the diversity of neoplasms, varying grades of malignancy, variable growth patterns, and the occupancy of non-neoplastic hamartomatous lesions, the clinician often has difficulty determining which patient should have a diagnostic biopsy or resection, or if therapy should be started without pathologic confirmation. Following a review of the neuroradiology and more common intracranial tumors, recommendations regarding management are proposed.

Brain↗