Tania Shiminski-Maher

Multiagent Chemotherapy and Deferred Radiotherapy in Infants With Malignant Brain Tumors: A Report From the Children’s Cancer Group

PURPOSE To evaluate response rate, event-free survival (EFS), and toxicity of two chemotherapeutic regimens for treatment of children younger than 36 months with malignant brain tumors and to estimate control intervals without irradiation in children with no residual tumor after initial surgery and induction chemotherapy and with delayed irradiation in patients with residual tumor or metastatic disease at diagnosis. PATIENTS AND METHODS Patients were randomly assigned to one of two regimens of induction chemotherapy (vincristine, cisplatin, cyclophosphamide, and etoposide v vincristine, carboplatin, ifosfamide, and etoposide). Maintenance chemotherapy began after induction in children without progressive disease. Children with no residual tumors after induction therapy and no metastatic disease at diagnosis were not to receive radiation therapy unless their tumors progressed. RESULTS Two hundred ninety-nine infants were enrolled. Forty-two percent of patients responded to induction chemotherapy. At 5 years from study entry, the EFS rate was 27% +/- 3%, and the survival rate was 43% +/- 3%. There was no significant difference between the two arms in terms of response rate or EFS. For medulloblastoma, supratentorial primitive neuroectodermal tumor, ependymoma, and rhabdoid tumors, 5-year EFS rates were 32% +/- 5%, 17% +/- 6%, and 32% +/- 6%, and 14% +/- 7%, respectively. Fifty-eight percent of patients who were alive 5 years after study entry had not received radiation therapy. CONCLUSION Intensified induction chemotherapy resulted in a high response rate of malignant brain tumors in infants. Survival was comparable to that of previous studies, and most patients who survived did not receive radiation therapy.

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.

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.

Late Effects Associated with Treatment of Craniopharyngiomas in Childhood

&NA; 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.

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.

Recovery of Lower Cranial Nerve Function after Surgery for Medullary Brainstem Tumors

OBJECTIVE:Although optimal treatment for intrinsic focal tumors of the medulla remains controversial, many surgeons advocate radical surgery for patients with these tumors. Postoperative surgical morbidity may include loss of lower cranial nerve function and significant motor deficits. Recovery of lower cranial nerve dysfunction after radical surgery has not been reported previously. METHODS:Forty-one children and adolescents with tumors involving the medulla underwent operations between 1986 and 1997. Nineteen (46%) of these patients experienced loss of lower cranial nerve function requiring tracheostomy, ventilator support, and feeding gastrostomy. A retrospective analysis of this patient population and the time to cranial nerve recovery was undertaken. RESULTS:Thirteen (68%) of the 19 patients with loss of lower cranial nerve function had full recovery of lower cranial nerve function. Two patients (11%) have had significant improvement in their lower cranial nerve function, and four patients (21%) have remained without lower cranial nerve function. CONCLUSION:Lower cranial dysfunction is common after surgery for intrinsic medullary tumors. However, the majority of patients who require tracheostomy or gastrostomy tubes will recover cranial nerve function.

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.

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.

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 vasopressins 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.

Current trends in the management of brainstem tumors in childhood.

&NA; 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 cervicomedullary 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.