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Biomedical subjects

J H Wisoff

Publications and source records attributed to J H Wisoff.

At least 37 records · Page 2Linked to original sources
PubMed1995

Prognostic factors in children with supratentorial (nonpineal) primitive neuroectodermal tumors. A neurosurgical perspective from the Children's Cancer Group.

Supratentorial primitive neuroectodermal tumors (S-PNETs), which have also been called cerebral neuroblastomas, have been considered to be the hemispheric equivalent of posterior fossa medulloblastomas. Twenty-seven children with S-PNETs (excluding pineoblastomas) which were confirmed by central pathology review were treated on the CCG-921 protocol from 1986 to 1992. After operation, all patients were staged with CSF cytology and spinal myelography or magnetic resonance scans and were treated with craniospinal irradiation and chemotherapy. Data from these 27 patients have been reviewed to evaluate neurosurgical treatment, survival, and prognostic variables that correlate with survival. Overall survival at 5 years was 34% (SE 20%) and progression-free survival (PFS) was 31% (SE 18%), which is lower than the survival of patients with posterior fossa PNETs (medulloblastomas). PFS was significantly worse in children 1.5-3 years of age at diagnosis and in those with evidence of tumor dissemination at the time of diagnosis. Large preoperative tumors were more likely to be associated with greater than 1.5 cm2 residual tumor postoperatively. Neurosurgeons estimated that less than 1.5 cm2 of residual tumor was present in 52% of the cases; postoperative scans confirmed that in 58%. For children with less than 1.5 cm2 residual tumor, postoperative survival at 4.0 years was 40% (SE 22%); for those with greater than 1.5 cm2 residual tumor, survival was 13% (SE 8%). The difference did not reach statistical significance, due to small numbers in this series, though a trend did exist (p = 0.19). Large series will be required to clarify the effects of extent of resection on survival.

Adolescent↗
PubMed1995

Randomized phase III trial in childhood high-grade astrocytoma comparing vincristine, lomustine, and prednisone with the eight-drugs-in-1-day regimen. Childrens Cancer Group.

PURPOSE: In a previous randomized trial, the addition of adjuvant chemotherapy to postoperative radiotherapy proved beneficial in the treatment of childhood high-grade astrocytomas. The present study tests the hypothesis that an eight-drug adjuvant chemotherapy regimen would improve survival in such children compared with the three-drug regimen of the prior study. PATIENTS AND METHODS: Between April 1985 and May 1990, patients between the ages of 18 months and 21 years with newly diagnosed high-grade astrocytomas were eligible for this study, as determined by the treating institution's histopathologic diagnosis. Treatment consisted of postoperative local-field radiotherapy and adjuvant chemotherapy, either lomustine (CCNU), vincristine, and prednisone (control regimen) or eight-drugs-in-1-day chemotherapy (experimental regimen). Two cycles of postoperative preirradiation chemotherapy were administered in the experimental regimen. Patients were evaluated radiographically every 3 months after irradiation. RESULTS: Eighty-five eligible patients were randomized to the control regimen and 87 to the experimental regimen. The progression-free survival (PFS) and overall survival (OS) at 5 years were 33% (SE = 5%) and 36% (SE = 6%), respectively. There was no statistical difference in outcome between the two chemotherapy regimens. In patients with confirmed diagnoses of anaplastic astrocytoma (AA) or glioblastoma multiforme (GBM), anaplastic astrocytoma, greater than 90% resection, and nonmidline tumor location were characteristics predictive of an improved PFS. There was a difference in toxicity between the two chemotherapeutic regimens, with greater myelosuppression and hearing loss in the experimental regimen. Tumor recurrence occurred primarily within the primary tumor site. CONCLUSIONS: There is no benefit to the treatment of high-grade astrocytomas in children with eight-drugs-in-1-day chemotherapy compared with CCNU, vincristine, and prednisone. Extent of tumor resection and histopathologic diagnosis are significant prognostic variables. The overall outcome for children with high-grade astrocytomas remains poor.

Adolescent↗
PubMed1995

Current treatment of normal-pressure hydrocephalus: comparison of flow-regulated and differential-pressure shunt valves.

FROM THE RECORDS of approximately 1500 shunt operations performed between 1987 and 1992, we identified 37 adults between ages 38 and 86 years (mean, 70 yr) with the normal-pressure hydrocephalus (NPH) syndrome who underwent surgery by a single surgeon. Since 1990, we have routinely used a flow-regulated shunt system (Orbis-Sigma valve [OSV]; Cordis Corporation, Miami, FL) in these patients. In this study, we compared the OSV system with conventional differential-pressure (DP) shunt systems uniformly used before 1990. This series (n = 37) consisted of 62% men (n = 23) and 38% women (n = 14). We excluded all patients with hydrocephalus associated with central nervous system neoplasms, intracerebral hemorrhage, or trauma as well those with radiographically documented late-onset aqueductal stenosis. All patients presented with the NPH clinical syndrome, chiefly with magnetic gait. In addition, 75% of patients experienced cognitive loss and 59% experienced urinary incontinence. The mean duration of preoperative symptoms was 35 months (range, 7-120 mo). Eight patients (22%) had undergone previous shunting procedures before referral to our service. A total of 89 shunt operations were performed in the 37 patients. Using actuarial methods and controlling for a history of prior shunt surgery, we found no significant difference in the time to initial malfunction (shunt survival) between the OSV and the DP shunts. There were three subdural hematomas and one infection in the OSV group compared with no complications in the DP valve group (P = 0.11). Thirty-six patients were available for follow-up, at a mean of 14 months after surgery. Nearly 90% of all patients experienced improvement in gait after shunting, regardless of the valve system that was used. There was one unrelated death. Realizing the limitations of a retrospective analysis and on the basis of the limited number of patients in this study, we conclude that using actuarial methods, we found no significant difference in shunt survival when comparing the OSV with the standard DP valve shunt systems with antisiphon devices in patients with NPH. Contrary to previous reports, the OSV is not free of overdrainage complications. Most patients (89%) with the NPH syndrome who primarily presented with gait disorder experienced significant improvement in gait after either OSV or DP shunting procedures when selected for surgery on the basis of the clinical syndrome and confirmatory radiographic data.

Adult↗
PubMed1995

Pediatric brain tumors.

Pediatric brain tumors are the most common solid tumor and the second most common neoplasm in childhood. Diagnosis is made by CT scan and MR imaging. Treatment includes surgery followed by observation, chemotherapy, or radiation therapy. A multidisciplinary approach in the treatment of these children is necessary with nurses and nurse practitioners as the consistent members of the team coordinating care.

Brain Neoplasms↗
PubMed1994

Management of hydrocephalus in children with medulloblastoma: prognostic factors for shunting.

Patients with medulloblastoma frequently present with hydrocephalus. While not all patients with medulloblastoma will continue to suffer from hydrocephalus after tumor resection, there is a significant proportion who will require a permanent shunt. We have retrospectively reviewed a group of children with medulloblastoma not shunted preoperatively, and have identified several characteristics which are associated with a requirement for permanent shunt. We studied 42 patients and found that 17 patients (40%) required permanent shunts within 4 weeks of craniotomy. The shunted patients were younger (5.4 +/- 2.9 vs. 10.0 +/- 5.7 years; p < 0.01), had larger ventricles (p < 0.05), and had more extensive tumors (Chang's stage T3 and T4; p < 0.01). These variables were all independently significant. In addition, we found that the patients requiring postcraniotomy shunts had a much higher rate of morbidity including a postoperative pseudobulbar syndrome. Of interest, we found that none of our patients without the above characteristics required a shunt. In particular, we found that no patient older than 10 years required a shunt. Thus, we conclude that younger patients with moderate to severe preoperative hydrocephalus and extensive tumors may benefit from perioperative CSF diversion. Other patients not meeting the above criteria can probably be safely managed with perioperative corticosteroids alone.

Adolescent↗
PubMed1994

Surgical management of recurrent craniopharyngiomas.

Recurrence is the most common complication of all forms of treatment of craniopharyngioma. Reoperation is the preferred treatment for unsuspected residual tumor after primary radical resection. Surgical resection can be curative in the majority of recurrences, especially those with solid tumor. Scarring from previous surgery, particularly following primary radical removal, may increase the technical difficulty of surgery. Although most patients will have a reasonable functional outcome, the morbidity following operation for recurrence is greater than primary surgery with some degree of new deficit being common. Adjuvant irradiation, interstitial irradiation or bleomycin, and serial aspiration of cystic tumors have lower cure rates than surgical resection but may have lower morbidity in selected patients. They are potential adjuvant or second-line treatments for subsequent recurrence.

Child↗
PubMed1994

Survival of infants with primitive neuroectodermal tumors or malignant ependymomas of the CNS treated with eight drugs in 1 day: a report from the Childrens Cancer Group.

PURPOSE: Very young children with CNS primitive neuroectodermal tumors (PNETs) and ependymomas have a poor prognosis and commonly have impairment of growth and cognitive abilities, in part resulting from radiotherapy. Thus, an intensive chemotherapeutic regimen was used to treat children less than 18 months of age at diagnosis. PATIENTS AND METHODS: Children were treated on a Childrens Cancer Group (CCG) protocol with an eight-drug chemotherapeutic regimen (vincristine, carmustine, procarbazine, hydroxyurea, cisplatin, cytarabine, prednisone, and cyclophosphamide) following surgery and postoperative staging. Delayed or reduced-volume radiotherapy was to be administered to all patients, but, in fact, was omitted in most cases. RESULTS: On central review of pathology, 82 children had diagnosis concordant with study entry criteria. Of these, 46 (56%) had posterior fossa (PF) PNET, eight (10%) had pineal PNET, 11 (12%) had nonpineal supratentorial PNET, 15 (18%) had ependymoma, and two had rhabdoid tumors. Fifty percent of tumor resections were complete, as verified by postoperative computed tomographic (CT) scan, and 23% of patients had metastatic disease at the time of diagnosis. Objective tumor response was documented following two cycles of chemotherapy in 28% of assessable patients. Toxicity of chemotherapy was primarily hematopoietic. Five children died of chemotherapy-related complications. Radiotherapy was administered to only nine patients before tumor progression. The 3-year progression-free survival (PFS) rates for PF PNET, pineal PNET, supratentorial nonpineal PNET, and ependymoma are 22% (SE = 6%), 0%, 55% (16%), and 26% (11%), respectively. The 3-year PFS rate for those children without metastatic disease was 29% (6%), as compared with 11% (6%) for those with metastatic disease. The only independent predictors of PFS were metastasis stage and location of the tumor within the pineal region. The median time to progression was 6 months. Twenty-four children completed the chemotherapeutic regimen without tumor progression; 19 are event-free survivors more than 2 years from diagnosis, only three of whom received radiation therapy. CONCLUSION: While overall survival in this group of very young patients is poor, a subset of children who have received only chemotherapy as adjuvant treatment remain free from tumor recurrence.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1994

Craniopharyngiomas: a clinicopathological analysis of factors predictive of recurrence and functional outcome.

Pathological and clinical data from 56 patients operated on for craniopharyngioma since 1981 were analyzed to determine the utility of dividing patients with this tumor into distinct clinical groups based on recognized pathological type and to determine the prognostic import of brain invasion. Of the tumors in the 30 adult patients, 66% were adamantinomatous, 28% were squamous papillary, and the remainder were mixed. However, of the tumors in the 26 children, 96% were adamantinomatous and none were pure squamous papillary (P < 0.01). Forty-six percent of the children compared with 17% of the adults had brain invasion (P < 0.01). Brain invasion was present in 37% of the adamantinomatous but in only 13% of the squamous papillary tumors. Seventy-seven percent of the children underwent gross total resection (GTR) compared with 27% of the adults (P < 0.01). Sixty-three percent of the squamous papillary tumors underwent GTR compared with 54% of the adamantinomatous and mixed tumors. Follow-up ranged from 7 to 187 months (mean, 49 mo). After subtotal resection, with or without radiation therapy, 58% of the tumors recurred compared with 17% recurrence after GTR (P < 0.01), with a mean time to recurrence of 34 months. In both tumor histological types, subtotal resection was associated with a higher rate of tumor recurrence compared with gross total resection. Among the subtotally resected craniopharyngiomas, 2 of the 3 (67%) squamous papillary and 11 of the 21 (52%) adamantinomatous and mixed tumors recurred. In contrast, among the totally resected tumors, none of the 5 squamous papillary and only 5 of the 25 (20%) adamantinomatous and mixed tumors recurred. There were no significant differences in Karnofsky performance status score, mortality rate, or visual and endocrine outcomes when comparing patients based on histological tumor type. When controlling for age and extent of resection, we found that brain invasion had no significant effect on recurrence rate in totally resected tumors. Based on the limited number of patients in this series, we conclude as follows. 1) Contrary to previous reports, squamous papillary craniopharyngiomas, like adamantinomatous tumors, may recur when subtotally resected. 2) For both tumor variants, the most significant factor associated with craniopharyngioma recurrence is the extent of surgical resection rather than histopathological subtype. 3) Contrary to prior hypotheses, brain invasion in totally resected tumors does not predict higher recurrence. 4) GTR is associated with a significantly lower recurrence rate and can be achieved without sacrificing functional outcome.

Adolescent↗
PubMed1993

Recent improvement in outcome using transcatheter embolization techniques for neonatal aneurysmal malformations of the vein of Galen.

Vein of Galen malformations may present as congestive heart failure in the neonate and are usually fatal if untreated. Early studies using transcatheter embolization techniques, in a series of 22 patients, had a 50% mortality rate and a 37% incidence of severe mental retardation in survivors. Modifications of embolization techniques and neonatal care have improved the outlook in a more recent series of 11 patients. The diagnosis was established within 3 days of life in 91% of the cases. No mortality occurred, and 6 of the patients were functionally normal at up to 30 months' follow-up. Although two patients had severe neurologic deficits and/or a seizure disorder, only one case was possibly temporally associated with the embolization procedure. Some developmental delay was noted in one other patient. These improvements result, in part, from modifications of the treatment protocol, including earlier diagnosis, avoidance of digoxin, improvement in the application of newer microcatheters and acrylic polymers (n-butylcyanoacrylate), avoidance of overly aggressive neurosurgical procedures, and the use of stable central vascular access for total parenteral nutrition accompanying other general improvements in neonatal care.

Catheterization↗
PubMed1992

Osteoplastic laminotomy in children.

The article reviews 180 cases of osteoplastic laminotomies, 1 year after operation. The patients were predominantly children undergoing selective dorsal rhizotomies. Also included were a few pediatric patients who received laminotomies for spinal cord tumors. Spinal X-rays were reviewed at 1 and 3 years of follow-up. There was complete to partial bridging of the laminar roof in all cases. This experience confirms that the procedure is well tolerated and is associated with reossification of the replaced bony segment.

Adolescent↗
PubMed1992

Surgical management of symptomatic pineal cysts.

The authors present a series of six patients with large symptomatic benign pineal cysts and review the 27 patients previously reported in the literature. Patients with symptomatic pineal cysts most often present with one of three syndromes: 1) paroxysmal headache with gaze paresis; 2) chronic headache, gaze paresis, papilledema, and hydrocephalus; or 3) pineal apoplexy with acute hydrocephalus. Surgical intervention with radical cyst removal is the treatment of choice for all symptomatic pineal cysts. Complete cyst removal is desirable; however, radical subtotal resection is appropriate if the cyst cannot be easily separated from the quadrigeminal plate. Ventricular shunting should be reserved for patients with persistent hydrocephalus after cyst resection.

Adolescent↗
PubMed1991

Chronic headache associated with a functioning shunt: usefulness of pressure monitoring.

Chronic headaches in a shunt-dependent patient with small ventricles has long been treated with little or no regard to intracranial pressure. In this study, pressure monitoring on 12 such patients demonstrated that they fell into three distinct categories: 3 had headaches caused by intracranial hypertension, 2 had headaches from hypotension, and 7 showed no relation of symptoms to pressure. As therapeutic procedures for treating these three categories are entirely different and sometimes opposing, it is clear that intracranial pressure monitoring is essential to successful management of this complaint.

Adolescent↗
PubMed1991

Current trends in the management of brainstem tumors in childhood.

Historically, the prognosis for a child diagnosed with a brainstem tumor has been bleak. The development of magnetic resonance imaging (MRI) has allowed for detailed visualization of tumors within the brainstem. This knowledge combined with the clinical history and neurological examination allows subcategorization of such tumors and delineation of children who might benefit from surgery. Surgical candidates include children with long clinical histories, focal neurological deficits and focal, cystic, exophytic or cervico-medullary tumors on the MRI scan. All children have the potential for multiple cranial nerve abnormalities, extremity weakness and swallowing and breathing difficulties. Nursing care of patients with brainstem tumors is complex and challenging.

Astrocytoma↗
PubMed1991

Neonatal vein of Galen malformations: experience in developing a multidisciplinary approach using an embolization treatment protocol.

A multidisciplinary team approach using a staged transcatheter embolization and neurosurgical protocol was applied to 22 patients with neonatal presentation of vein of Galen malformations over a 12 year period. Aggressive medical therapy was combined with interventions including: ventricular shunting, transcatheter embolization, retrograde transtorcular embolization, and neurosurgical obliteration. There was a high frequency of high output cardiac failure, multiple organ system dysfunction, seizures, hydrocephalus, visual, developmental and neurological disability. Of the first 11 patients, five survived; four with seizures and three with marked retardation. Of the last 11 patients, six survived; five with seizures but only one with retardation. Despite persistently high morbidity and mortality, our continuously evolving protocol offers these otherwise hopeless patients some chance of survival.

Cerebral Veins↗
PubMed1991

Pregnancy in patients with cerebrospinal fluid shunts: report of a series and review of the literature.

Hydrocephalic women with cerebrospinal fluid shunts are now surviving to reproductive age. Twenty-one pregnancies in 18 patients with shunts, including 11 from the present series and 10 from previous series, were analyzed for neurological, obstetrical, and perinatal outcome. Fourteen women had preexisting shunts, and 4 had the onset of symptomatic hydrocephalus and the placement of shunts during pregnancy. Neurological complications occurred in 13 of 17 (76%) pregnancies in patients with preexisting shunts, including symptoms of increased intracranial pressure (ICP) in 10 of 17 (59%) pregnancies, exacerbation of seizure disorder in 2 of 17 (12%) pregnancies, and severe headaches without increased ICD in 1 patient. In 7 of 11 (66%) of the symptomatic patients, symptoms spontaneously resolved postpartum. Four of 17 (23%) of these pregnancies were associated with shunt obstruction requiring antepartum or postpartum surgery. Four patients had a primary shunt placement, and one had a shunt revision during pregnancy without complications. There were no unusual obstetrical or perinatal complications in the series. The clinical management of pregnant patients with hydrocephalus should include preconception counseling and magnetic resonance imaging, as well as the use of serial antenatal magnetic resonance images, ICP monitoring, or the judicious use of radioisotope studies of shunt patency if signs of increased ICP appear. A cesarean section is recommended for the delivery of the neurologically unstable patient. For asymptomatic mothers, a vaginal delivery with a shortened second stage and prophylactic antibiotics are advised.

Adolescent↗
PubMed1990

The association of hydrocephalus with intramedullary spinal cord tumors: a series of 25 patients.

171 patients with intramedullary spinal cord tumors were operated on, of which 25 patients (15%), mostly children, developed symptomatic hydrocephalus. Twenty patients (12%) had malignant tumors, with 13 of the 20 cases (63%) complicated by increased intracranial pressure and ventriculomegaly. Of the remaining 151 patients with benign tumors (89%), only 12 (8%) developed symptomatic hydrocephalus. In an effort to understand the relationship between hydrocephalus and intramedullary spinal cord tumor, the authors analyze the level and histology of the neoplasm, as well as its association with spinal cysts. A review of the neurosurgical literature reveals that 34 similar cases of hydrocephalus associated with intramedullary spinal cord tumors have been reported to date. The authors note that the presence of hydrocephalus in patients with malignant intramedullary astrocytomas is associated with a shorter rate of survival than in those patients with high-grade lesions but without hydrocephalus, apparently due to rapid tumor progression. The ventriculomegaly seen with benign spinal cord gliomas has no statistically significant effect upon long-term prognosis.

Adolescent↗