Faculty Bibliography

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: Pituitary adenoma may present with neuro-ophthalmic manifestations and, typically, rapid tumor expansion is the result of apoplexy. Herein, we present the first case of an isolated sixth cranial nerve palsy as initial feature of a rapidly expanding ACTH positive silent tumor without apoplexy. CASE PRESENTATION: A 44 year old female with a history of sarcoidosis presented with an isolated sixth cranial nerve palsy as the initial clinical feature of a rapidly expanding ACTH positive silent pituitary adenoma. The patient underwent emergent transsphenoidal hypophysectomy for this rapidly progressive tumor and subsequently regained complete vision and ocular motility. Despite tumor extension into the cavernous sinus, the other cranial nerves were spared during the initial presentation. CONCLUSIONS: This case illustrates the need to consider a rapidly growing pituitary tumor as a possibility when presented with a rapidly progressive ophthalmoplegia

Neurocysticercosis (NCC) is the most common parasitic infection of the CNS. Surgical intervention is often required to treat for hydrocephalus with neuroendoscopy emerging as a logical alternative to craniotomies. While cranial NCC cases have increased in the United States, spinal NCC incidences have remained relatively low. We report a case of trapped fourth ventricle and myelopathy from both concomitant cranial and spinal NCC. This patient exhibited a subacute thoracic myelopathy and acute deterioration from a trapped fourth ventricle (TFV) from aqueductal and fourth ventricular outflow occlusion. She underwent urgent posterior fossa exploration. A fourth ventricular lesion consistent with NCC was removed from the distal aqueduct and the aqueduct was opened via aqueductoplasty with a ventricular catheter and balloon dilatation. She improved following surgery without extraoccular dysfunction. Intraoperative visualization and postoperative MRI flow-studies confirmed patency of the aqueduct and there was complete resolution of her fourth ventricle dilatation. With antiparasitic and corticosteroid therapy, her myelopathy has slowly improved over 9 months of follow-up

Epilepsy associated with preterm birth is often refractory to anticonvulsants. Children who are born preterm are also prone to cognitive delay and behavioral problems. Brains from these children often show diffuse abnormalities in cerebral circuitry that is likely caused by disrupted development during critical stages of cortical formation. To test the hypothesis that prenatal injury impairs the developmental switch of gamma-amino butyric acid (GABA)ergic synapses from excitatory to inhibitory, thereby disrupting cortical circuit formation and predisposing to epilepsy, we used immunohistochemistry to compare the expression of cation-chloride transporters that developmentally regulate postsynaptic GABAergic discharges in postmortem cerebral samples from infants born preterm with known white matter injury (n = 11) with that of controls with minimal white matter gliosis (n = 7). Controls showed the expected developmental expression of cation-chloride transporters NKCC1 and KCC2 and ofcalretinin, a marker of a GABAergic neuronal subpopulation. Samples from infants with white matter damage showed a significant loss of expression of both NKCC1 and KCC2 in subplate and white matter. By contrast, there were no significant differences in total cell number or glutamate transporter VGLUT1 expression. Together, these novel findings suggest a molecular mechanism involved in the disruption of a critical stage of cerebral circuit development after brain injury from preterm birth that may predispose to epilepsy.

Metameric lesions of the spine are rare. The authors present a case of patient with a complex metameric vascular lesion of the thoracic spine and describe a management strategy for this entity

Giant cell tumors (GCTs) and aneurysmal bone cysts (ABCs) are locally aggressive but benign lesions typically of the long bones of the extremities or spine and rarely involve the cranial vault or skull base. Although they are distinct entities pathologically and cytogenetically, GCTs can occur with secondary ABCs, which are likely reactive in nature. The reported cases have primarily involved bones of the extremities and, to our knowledge, there has been only one case of coexisting GCT and ABC of the calvarium or skull base. We describe a 27-year old woman who underwent complete resection of a GCT with secondary ABC of the left occipital bone and condyle. We discuss the significance of the co-occurrence of these lesions and their optimal management

A choroid plexus papilloma is a rare CNS neoplasm arising from the neuroepithelial lining of the choroid plexus. A third ventricular location of a choroid plexus papilloma is rare compared with the more common sites in the lateral and fourth ventricles. Cystic choroid plexus papilloma represents an infrequent subtype that may present diagnostic ambiguity. The authors present a case of cystic choroid plexus papilloma within a cavum septum pellucidum that radiographically mimicked neurocysticercosis

We report a case of extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) in a 75-year-old woman with a neuropathy related to high levels of serum immunoglobulin M and a history of rheumatoid arthritis and polymyositis. The patient developed a mass in the right submandibular salivary gland, and this mass demonstrated histopathologic features that are typical of MALT lymphoma, including infiltrates of small monocytoid B cells in the epithelium (forming "lymphoepithelial lesions"), a reactive background of florid germinal center hyperplasia, and follicular colonization by the monocytoid B cells. Many plasma cells in the background expressed cytoplasmic immunoglobulin M lambda, matching the serum spike. Flow cytometric analysis confirmed the presence of clonal mature B cells; however, unlike most MALT lymphomas, these cells coexpressed dim CD5. Clinical staging revealed evidence of systemic distribution with documented disease involving the bone marrow, the lung, and a paratracheal lymph node. Analysis of this unusual systemic MALT lymphoma, and a comparison with similar examples from the literature, illuminates relationships among MALT lymphoma, chronic lymphocytic leukemia/small lymphocytic lymphoma, and Waldenstrom macroglobulinemia.

BACKGROUND: Traditionally, snare polypectomy is performed using blended, coagulation, or pure cutting electrical current (EC). The aim of this study was to assess and compare the diagnostic quality of polyps obtained by snare polypectomy using two different electrosurgical currents. METHODS: Consecutive patients undergoing colonoscopy underwent polypectomy using either blended EC with a conventional electrosurgical generator (ESG) or using an ESG with a microprocessor that automatically controls cutting and coagulation (Endocut). An experienced blinded gastrointestinal (GI) pathologist evaluated the specimens for diameter, cautery damage (amount and degree), margin evaluability, architecture, and general histologic diagnostic quality. RESULTS: One hundred sixteen patients (69% men, mean age 63.8 +/- 15 yr) underwent 148 polypectomies (78 using blended current and 70 using Endocut). We found that the cautery degree was less with the Endocut than with the blended current (p < 0.02). Cautery amount was also higher in polyps resected using blended current (56%) than Endocut (51%) but this difference did not reach statistical significance (p= 0.1). Polyps resected using Endocut had better margin evaluability (75.7% to 60.3%, p= 0.046). The overall tissue architecture was similar in both groups. Polyps removed with blended current had less overall quality as compared to polyps removed by Endocut (p= 0.024). CONCLUSIONS: More extensive tissue damage occurred using blended EC with the conventional ESG than when using Endocut. The quality of the polypectomy specimens was overall better using Endocut. Finally, the ability to evaluate resected polyp margins and overall tissue histology was better with the microprocessor-controlled ESG than with the conventional ESG