Etoposide with or without mannitol for the treatment of recurrent or primarily unresponsive brain tumors: a Children's Cancer Group Study, CCG-9881.

PURPOSE: This study was undertaken to evaluate the response of recurrent brain tumors to intravenous etoposide and to evaluate the efficacy of mannitol in augmenting etoposide's tumoricidal effect. PATIENTS AND METHODS: Ninety-nine children between one and 21 years of age with recurrent brain tumors were randomly assigned to treatment with intravenous etoposide 150 mg/M2, with or without mannitol 15 gm/M2, daily for five days every three weeks for one year or until disease progression or death. Computerized tomographic (CT) or magnetic resonance image (MRI) scans, obtained after three cycles of therapy, were compared with pre-therapy scans. Scans were centrally reviewed. RESULTS: Of 87 evaluable patients, 12 (13.8%) were determined to have had an objective response by the institutional radiologist. On central review, 7/66 (10.6%) responses were documented. Responses in centrally reviewed patients were observed in 2/12 (16.7%) low grade astrocytomas, 4/26 (15.4%) medulloblastoma or primitive neuroectodermal tumors (PNET), 1/13 (7.7%) high grade astrocytomas and 0/15 (0%) brain stem gliomas. Survival at one year was 53% (SE 12%) for low grade astrocytomas, 38% (SE 7%) for medulloblastoma or PNET, 28% (SE 10%) for high grade astrocytomas and 9% (SE 5%) for brain stem gliomas. An effect of mannitol was not observed. CONCLUSION: Intravenous etoposide has a low level of activity in the treatment of recurrent low grade astrocytomas and medulloblastoma or PNET. The efficacy of this agent was not enhanced by the coincident intravenous administration of mannitol.

Intrinsic tumors of the medulla: predicting outcome after surgery.

Twenty-four children and young adults who underwent surgery for intrinsic tumors of the medulla were studied, correlating preoperative symptoms to postoperative brain stem dysfunction. 75% harbored benign tumors with a mean survival of 3.6 years, while the remaining 25% with anaplastic tumors had a mean survival of 3.2 years. Six patients required postoperative ventilatory support for an extended period of time, and 9 required feeding gastrostomies. Those patients who experienced frequent symptoms of upper respiratory tract infection, preoperative pneumonia, or an alteration in their voice were at risk of postoperative ventilatory dependency. Those without such symptoms did not experience such a fate. Difficulty in swallowing food or liquids seems to predict the need for postoperative feeding gastrostomies, and a lack of such a history was correlated with no such need 100% of the time. There seem to be preoperative signs and symptoms which predict postoperative brain stem dysfunction for an extended period of time in those undergoing brain stem surgery for tumors intrinsic to the medulla.

Brainstem tumors in childhood: preparing patients and families for long- and short-term care.

Brainstem tumors make up only 10% of all childhood brain tumors and they carry with them a bleak prognosis. The majority of children are dead within 2 years of diagnosis from a malignant histology in a malignant location within the brain. A small group of brainstem tumors which by clinical and magnetic resonance imaging diagnosis are slow growing may be amenable to surgical debulking prior to adjunctive therapy. The morbidity associated with this treatment is high but long-term survival in this category of brainstem tumors is possible. Regardless of histopathologies, the medical, physical and emotional care of children and their families is complex and challenging. Effective patient/family education and support for children diagnosed with brainstem tumors is essential to providing medical care aimed at the cure or terminal care which allows the child to die in comfort with dignity.

Pediatric brain tumors: diagnosis and management.

Pediatric brain tumors are the most common solid tumor and the second most common neoplasm in childhood. Confirmation of diagnosis is made by visualization of the tumor on a computed tomographic or magnetic resonance imaging scan. Treatment includes surgery followed by observation or chemotherapy and/or radiation therapy, depending on the amount of tumor removed and its histological characteristics. Recent advances in neuroradiology, neurosurgical techniques, and neuro-oncology have begun to impact the length and quality of survival of children diagnosed with brain tumors. A multidisciplinary approach in the treatment of these children is necessary with the nurses and/or nurse practitioners as the consistent members of the team often coordinating the care.

Hydrocephalus and shunts in children with brain tumors.

Children with brain tumors are at risk for developing hydrocephalus necessitating placement of a permanent shunt catheter. As comprehensive, interdisciplinary, multispecialty care is required for these patients, the pediatric oncology nurse must become knowledgeable about caring for children with a shunt. This article focuses on the care of the pediatric neuro-oncology patient requiring shunting for hydrocephalus, including a review of cerebrospinal fluid flow and hydrocephalus, determinants of shunt placement, complications of ventriculoperitoneal shunts, and nursing considerations.

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.

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.

Current trends in the diagnosis and management of hydrocephalus in children.

Hydrocephalus is a clinical condition caused by an imbalance between the production and absorption of cerebrospinal fluid (CSF). This imbalance results in dilated cerebral ventricles and increased intracranial pressure (ICP). It is more common in children and results from congenital or acquired disease processes including developmental anomalies, neoplasms, or inflammatory conditions. Technological advances in neuroradiology and neurosurgery have led to earlier diagnosis and treatment. Treatment includes insertion of a shunting device that diverts the excess CSF to another resorptive body cavity. Regardless of the etiology, hydrocephalus is a chronic illness requiring frequent medical and nursing involvement. This article focuses on the diagnosis and management of hydrocephalus in children, complications associated with management, and the role of the pediatric nurse in caring for these children.

Patient/family preparation and education for complications and late sequelae of craniopharyngiomas.

Craniopharyngiomas are the most common pediatric suprasellar tumor. While they are benign by histology, their location in the brain predisposes craniopharyngiomas to be associated with both acute and chronic neurologic, endocrine, visual, neuropsychologic and psychosocial problems. Because of the complexity of problems it is difficult to really prepare a patient and family for every complication or late effect that may occur with a craniopharyngioma diagnosis. Patient/family preparation for potential complications and late sequelae is an ongoing and labor-intensive process for all members of the multidisciplinary health care team. This paper will discuss this process and identify strategies for effective patient/family education for children with the diagnosis of craniopharyngioma.

Selective dorsal rhizotomy: outcome and complications in treating spastic cerebral palsy.

Selective dorsal rhizotomy has shown great promise as a treatment for the functional disabilities and deforming hypertonia of spastic cerebral palsy. At New York University Medical Center, 200 children underwent this procedure between 1986 and 1990. All groups, whether walkers, crawlers, or nonlocomotors, showed improvement in the tone and range of most muscles tested. Half of these patients experienced complications. Thirty-five of these were serious and included bronchospasm (5.5%), aspiration pneumonia (3.5%), urinary retention (7%), and sensory loss (2%). There are, however, clear indications that warn of these complications; monitoring and prophylactic treatment can minimize their effects, and the possibility of such problems is more than offset by the proven benefits of this operative procedure.

Physician-patient-parent communication problems.

The physician-patient relationship has been addressed frequently in the literature. It is well documented that a positive physician-patient relationship is influential in clinical outcome. This concept is even more complex in pediatrics (and pediatric neurosurgery) where the physician must 'relate' or 'communicate' with the entire family. Complications in physician-parent-child communication occur for a variety of reasons. Most commonly, they occur as a result of lack of communication, miscommunication and/or information overload. The intent of this paper is to discuss the pediatric neurosurgeon-parent-child communication complications and to identify strategies for their prevention.

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.

Diabetes insipidus and syndrome of inappropriate secretion of antidiuretic hormone in children with midline suprasellar brain tumors.

Salt and water balance within the body is controlled by the hormonal influence of vasopressin. Vasopressin is produced in the hypothalamus, stored and released from the posterior lobe of the pituitary, and travels via the blood to the kidneys to regulate the amount and concentration of urine excreted. Oversecretion or undersecretion of vasopressin, eg, diabetes insipidus (DI) or syndrome of inappropriate secretion of antidiuretic hormone (SIADH) results in an imbalance of the salt-to-water ratio. In children this hormonal imbalance may occur secondary to a suprasellar brain tumor and/or the treatment of such. Approximately 50% to 75% of children with suprasellar tumors will develop permanent DI and the remainder will experience transient postoperative DI or SIADH. Pathophysiology of vasopressin's control on salt and water balance and its relationship to suprasellar brain tumors in children are presented. Nursing assessment and intervention parameters for management of DI and SIADH in children with brain tumors are also discussed.

Intrinsic tumors of the medulla: surgical complications.

Intrinsic focal tumor of the medulla oblongata is an uncommon entity. Of the 51 cases presenting for treatment of a brainstem glioma between 1984 and 1990, only 7 were confined to the medulla. Radical excision was performed on each patient. Four of the first 5 patients who were extubated in the recovery room had CO2 retention, with associated respiratory arrest and hypoxia. Three suffered permanent cranial nerve deficits as a result. It is the purpose of this report to describe the serious complications of medullary surgery and to make recommendations as to how they may be avoided.

Late effects associated with treatment of craniopharyngiomas in childhood.

Craniopharyngiomas are the most common pediatric suprasellar tumor. They are clinically slow growing tumors whose primary treatment is surgical resection. Because of their location in the brain, craniopharyngiomas are associated with both acute and chronic problems including endocrinopathies and visual, neuropsychological and psychosocial dysfunctions. Fifteen patients and families of children operated upon at New York University Medical Center over the past twenty years indicated through interviews that all had significant sequelae associated with their illness. Nursing management of these problems during the acute and chronic phases of the illness will be discussed.

Brain tumors in childhood: implications for nursing practice.

Pediatric brain tumors are the most common solid tumor and the second most common neoplasm in childhood. Confirmation of diagnosis includes visualization of the tumor on a computed tomographic or magnetic resonance imaging scan. Treatment includes surgical resection or biopsy for confirmation of tissue diagnosis, with observation, radiation, and/or chemotherapy, depending on the tumor's pathology. The care of the child with a brain tumor involves a large multidisciplinary health care team. Nurses and nurse practitioners/clinicians are consistent members of the team that cares for the child and family throughout the course of the illness. Their intervention as advocates and educators in a liasion role between the patient/family and various members of the medical team is critical for the patient and family throughout the course of the illness.