Faculty Bibliography

OBJECTIVE: The differential diagnosis for suprasellar masses includes a variety of pathologies, ranging from stable and benign lesions to aggressive and malignant ones. We report a case of a suprasellar hamartoma associated with an arachnoid cyst and review the literature surrounding the topic. CASE DESCRIPTION: A 32-year-old man who presented with headaches and nonspecific vision loss was found to have a cystic, calcified, and minimally contrast enhancing lesion of the suprasellar region. Intraoperative inspection revealed a discrete mass in the right side of suprasellar region that resembled normal brain completely enveloped by the basal arachnoid membranes including the membrane of Lillequist and was not connected to the brain. Fenestration and exploration of the cystic portion demonstrated a simple arachnoid cyst filled with what appeared to be cerebrospinal fluid. Given the adherence of the lesion to numerous perforating arteries arising from the posterior communicating artery, a biopsy with intraoperative confirmation was taken. Pathology was consistent with neuroglial tissue without evidence of neoplasia. CONCLUSIONS: To our knowledge this is only the third case of an isolated suprasellar hamartoma described in the literature and the first of its kind to be associated with an arachnoid cyst.

PURPOSE Surgery is curative therapy for pediatric low-grade gliomas (LGGs) in areas of the brain amenable to complete resection. However, LGGs located in areas where complete resection is not possible can threaten both function and life. The purpose of this study was to compare two chemotherapy regimens for LGGs in children younger than age 10 years for whom radiotherapy was felt by the practitioner to pose a high risk of neurodevelopmental injury. PATIENTS AND METHODS Previously untreated children younger than age 10 years with progressive or residual LGGs were eligible. Children were randomly assigned to receive carboplatin and vincristine (CV) or thioguanine, procarbazine, lomustine, and vincristine (TPCV). Children with neurofibromatosis are reported separately. Results Of 274 randomly assigned patients who met eligibility requirements, 137 received CV and 137 received TPCV. The 5-year event-free survival (EFS) and overall survival (OS) rates for all eligible patients were 45% +/- 3.2% and 86% +/- 2.2%, respectively. The 5-year EFS rates were 39% +/- 4% for CV and 52% +/- 5% for TPCV (stratified log-rank test P = .10; cure model analysis P = .007). On multivariate analysis, factors independently predictive of worse EFS and OS were younger age and tumor size greater than 3 cm(2). Tumor location in the thalamus was also associated with poor OS. CONCLUSION The difference in EFS between the regimens did not reach significance on the basis of the stratified log-rank test. The 5-year EFS was higher for TPCV on the basis of the cure model analysis. Differences in toxicity may influence physician choice of regimens.

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.