Faculty Bibliography

Langerhans cell histiocytosis (LCH) encompasses a range of clinical presentations. Pure osseous involvement is referred to as eosinophilic granuloma (EG), whereas systemic involvement can either be classified as Hand-Schuller-Christian disease or Letterer-Siwe syndrome. It is estimated that of the total incidence of LCH (0.5 per 100,000 children per year in the United States), nearly 70% are categorized as EG (1). We describe a case of clinically occult calvarial eosinophilic granuloma brought to medical attention only after a traumatic event led to scalp and epidural hemorrhage at the site of the lesion. Osseous EG initially presents as a painful or tender mass, at times even mimicking osteomyelitis. Computed tomography (CT) reveals a lytic lesion with beveled edges. Magnetic resonance (MR) imaging is often non-specific, but usually demonstrates a marrow-replacing process that is T1 hypointense, T2 hyperintense, with homogenous enhancement. The complex MR imaging findings in our case provided a unique perspective regarding the presentation of EG. Once pathology established eosinophilic granuloma, skeletal survey confirmed the lesion was solitary, despite a compelling history of present illness.

The authors report the first case of a Wilms tumor (WT) with diffuse anaplasia metastatic to the brain in a 13-year-old girl with a history of neurofibromatosis Type 1. At presentation, the metastatic tumor had radiological features that suggested a meningioma. Histologically it was characterized by striking anaplasia and features similar to the patient's previously resected WT with diffuse anaplasia.

BACKGROUND: : Controversy persists regarding the optimal treatment of pediatric craniopharyngiomas. OBJECTIVE: : We performed a meta-analysis of reported series of transcranial (TC) and transsphenoidal (TS) surgery for pediatric craniopharyngiomas to determine whether comparisons between the outcomes in TS and TC approaches are valid. METHODS: : Online databases were searched for English-language articles reporting quantifiable outcome data published between 1990 and 2010 pertaining to the surgical treatment of pediatric craniopharyngiomas. Forty-eight studies describing 2955 patients having TC surgery and 13 studies describing 373 patients having TS surgery met inclusion criteria. RESULTS: : Before surgery, patients who had TC surgery had less visual loss, more frequent hydrocephalus and increased intracranial pressure, larger tumors, and more suprasellar disease. After surgery, patients in the TC group had lower rates of gross total resection (GTR), more frequent recurrence after GTR, higher neurological morbidity, more frequent diabetes insipidus, less improvement, and greater deterioration in vision. There was no difference in operative mortality, obesity/hyperphagia, or overall survival percentages. CONCLUSION: : Directly comparing outcomes after TC and TS surgery for pediatric craniopharyngiomas does not appear to be valid. Baseline differences in patients who underwent each approach create selection bias that may explain the improved rates of disease control and lower morbidity of TS resection. Although TS approaches are becoming increasingly used for smaller tumors and those primarily intrasellar, tumors more amenable to TC surgery include large tumors with significant lateral extension, those that engulf vascular structures, and those with significant peripheral calcification

BACKGROUND: Central nervous system neoplasms are the most common solid tumors in children, and more than 40% are low-grade gliomas. Variable locations, extent of resection, postoperative neurodiagnostic evaluation, and histology have confounded therapy and outcome. OBJECTIVES: To investigate disease control and survival after surgery. METHODS: A prospective natural history trial from 1991 to 1996 produced a subset of patients with low-grade gliomas managed by primary surgery and subsequent observation. Patients were evaluable if eligibility, tumor location, and extent of resection were confirmed by pathological diagnosis, preoperative and postoperative imaging, and the surgeon's report. Primary end points were overall survival (OS), progression-free survival (PFS), and postprogression survival. RESULTS: Of 726 patients enrolled, 518 were fully evaluable for analysis. The 5- and 8-year OS rates were 97% +/- 0.8% and 96% +/- 0.9%, respectively, and PFS rates were 80% +/- 1.8% and 78% +/- 2.0%. In univariate analyses, histological type, extent of residual tumor, and disease site were significantly associated with PFS and OS. In multivariate analysis, gross total resection (GTR) without residual disease was the predominant predictor of PFS. In patients with limited residual disease, 56% were free of progression at 5 years. CONCLUSION: GTR should be the goal when it can be achieved with an acceptable functional outcome. The variable rate of progression after incomplete resection highlights the need for new predictors of tumor behavior

PURPOSE: Intrathecal baclofen (ITB) therapy is an accepted treatment modality for spasticity and dystonia. Several complications related to ITB have been described, including mechanical malfunctions, infections, cerebrospinal fluid fistula, and baclofen withdrawal or overdose. In this study, we present our institutional experience with ITB therapy, emphasizing complication avoidance and lessons learned. METHODS: The charts of 87 patients treated with ITB therapy were retrospectively reviewed. The primary surgical technique, complication type and timing, method of treatment, and outcome were analyzed. RESULTS: Thirteen out of 76 (17.1%) patients primarily treated at our department had 25 complications. The first complication occurred 17.5-30.9 months (mean 24.2+/-6.7) after the pump implantation. Additional four patients with pumps placed elsewhere had six complications and were subsequently treated by our group. The main complications were: catheter fracture (11), subcutaneous fluid collection (5), lumbar wound/CSF infection (3), lumbar catheter or connector protrusion (3), pump malfunction (3), distal catheter migration outside the thecal sac (2), and baclofen withdrawal (1). Of the patients in the NYULMC group, six were treated by a single surgical procedure, six underwent multiple surgical procedures, and one was managed conservatively. In retrospect, changing the surgical technique, or adding an abdominal binder may have prevented 17 complications (54.8%). There were two deaths that were unrelated to the ITB therapy. CONCLUSION: ITB therapy is associated with complications, many of which require additional surgery. Some of these complications are avoidable by adhering to a strict surgical technique and a proper criterion for patient selection

Prognosis of diffuse intrinsic pontine gliomas (DIPGs) remains poor. Failure has been predominantly local, with leptomeningeal dissemination (LD) occurring in 4-33% of patients in pre-MRI era series. Routine craniospinal imaging after initial treatment may reveal other relapse patterns relapse. Sixteen consecutive pediatric patients with DIPG treated between 2006 and 2009 were retrospectively reviewed. Treatment regimens, recurrence patterns, survival, and pathologic diagnosis were recorded. Fourteen patients received involved-field radiotherapy to 54 Gy, and two patients received craniospinal irradiation for LD at presentation. Neuraxis MRI was performed at diagnosis and at 4 month intervals following radiotherapy. Fifteen patients have had progression of disease (median progression-free survival 5.0 +/- 1.2 months), and 13 patients have died (median survival 9.0 +/- 1.4 months). Local failure occurred in 12 patients (75%). LD occurred in nine patients (56%). LD was present at diagnosis in three patients, after initial staging and treatment in six patients, and during autopsy in two patients. Median overall survival was 12.0 +/- 3.3 months without LD and 8.0 +/- 2.1 months with LD (P = 0.059, log rank test). Median progression-free survival was 9.5 +/- 3.9 months without LD and 3.0 +/- 2.1 months with LD (P = 0.012, log rank test). The high incidence of LD probably reflects liberal use of spine MRI surveillance. All patients should undergo routine craniospinal imaging at diagnosis and follow-up. Central nervous system prophylaxis should be considered in future clinical trials

BACKGROUND: Endosopic septum pellucidotomy is used for treating patients with unilateral and specific types of bilateral hydrocephalus. The ideal location for septostomy is controversial; however, an avascular region is preferred. OBJECTIVE: As the septal veins (SVs) are viewed only from one side, we studied the symmetry of the SVs in an attempt to define a safe area for septostomy. METHODS: Sixteen cadaver brains were dissected. The septum pellucidum was exposed bilaterally and divided into 3 regions. SVs of both sides were evaluated according to number, size, distribution, and location relative to common markers on both sides. RESULTS: Each side included 1 to 7 large veins (mean +/- standard deviation, 2.3 +/- 1.4), 0 to 3 small veins (2.05 +/- 1.73), and a total of 2 to 7 veins (4.35 +/- 1.53). Of the large veins, 88% were located in the anterior septal region (anterior to the foramen of Monro). Among the 10 brains that were extensively dissected, 90% had asymmetric SVs (either in the number of large veins or in the existence of any veins) in at least 1 of the septal regions, and 50% of brains had asymmetric SVs in the anterior region. CONCLUSION: Distribution of the SVs is asymmetric in most cases. We recommend septostomy be performed at the anterior area of the middle septal region, at the level of the foramen of Monro, mid-height between the corpus callosum and fornix. Careful evaluation of preoperative images and thorough coagulation at the septostomy site are essential to avoid injury to a contralateral large SV

OBJECT: Prior work by the authors' group and reports of other authors suggest worse functional outcomes and decreased survival in children with larger craniopharyngiomas. The purpose of this study was to assess the oncological, endocrinological, and functional outcomes in children who underwent radical resection of giant craniopharyngiomas (defined as 5 cm or greater in largest diameter). METHODS: Between 1986 and 2006, 26 children under the age of 18 (14 boys, 12 girls; mean age 10.5 years) underwent radical resection of giant craniopharyngiomas performed by the senior author. Data were retrospectively collected to assess the outcome of surgical treatment. RESULTS: Twenty (77%) of 26 patients underwent gross-total resection (GTR) confirmed by intraoperative inspection and postoperative imaging. All primary tumors (17 of 17) and 3 (33%) of 9 recurrent tumors were treated with GTR. There was no operative mortality, and 18 of 26 patients (69%) were alive at a mean follow-up of 8.9 years (median 9.3 years). Disease control was achieved in 21 (84%) of the 25 patients followed up for more than 6 months and was more successful in patients who underwent GTR (95%) than in those who underwent STR (50%, p = 0.03). New-onset diabetes insipidus (DI) occurred in 63.2% of patients (73% of patients had DI postoperatively). New or worsened deficits in visual acuity and visual fields occurred in 16% and 28%, respectively, of the 25 patients for whom postoperative visual data were available. Five patients (19%) experienced significant, permanent neurological deficits, and 5 (19%) had mild to moderate deficits. New or worsened hypothalamic disturbance occurred in 35% and 22% of patients, respectively, but obesity developed in only 15%. CONCLUSIONS: In this retrospective series, radical resection of giant craniopharyngiomas in children was found to lead to excellent rates of disease control with acceptable or good functional outcomes but slightly higher rates of neurological complications compared with rates in patients with smaller tumors. Radical resection is less successful in recurrent tumors that reach very large sizes, especially previously irradiated tumors, with resultant diminished survival