Faculty Bibliography

Extraneural ependymomas are rare tumors that occur in sacrococcygeal, pelvic and extra pelvic regions. While sacrococcygeal extraneural ependymomas are equally distributed among males and females, pelvic and extra pelvic ependymomas have been exclusively reported in women, mainly of child bearing age. We present a case of extraneural, pelvic ependymoma that showed clinical response to GnRH therapy with its immunohistochemical and electron microscopic analysis, and an overview of primary extraneural ependymomas based on a review of all such cases published in English literature.

Sarcoidosis is a granulomatous disorder of unknown etiology involving multiple organ systems. Isolated neurosarcoidosis is exceedingly rare. This case report presents isolated trigeminal nerve sarcoidosis mimicking schwannoma in a patient presenting with symptoms of trigeminal neuralgia. Neuroimaging revealed a mass associated with trigeminal nerve which prospectively thought to represent schwannoma. However, surgical pathology was consistent with sarcoidosis. Given great overlap in imaging characteristic of tumors in the Meckel's cave intraoperative frozen section biopsy may be considered to rule out an inflammatory lesion.

Systemic Lupus erythematosus (SLE) is an autoimmune dis- ease with multiple clinical presentations and manifestations. Here, we report an intriguing case of a 30-year-old female with full-blown SLE, associated with longitudinal extensive transverse myelitis (LETM) on Magnetic Resonance Imaging (MRI) manifested by lower extremity weakness, neurogenic bladder and bowel, and central nervous system (CNS) lupus clinically manifested by changes in mood and behavior as well as neutrophilic vasculitis and cerebritis on pathology. LETM is a rare complication of SLE; however, what makes this case even more intriguing is that it additionally had cerebral lesions consistent with neutrophilic vasculitis and cerebritis, and that it may all have started at least 10 years prior with nonspecific musculoskeletal manifestations sub - sequently followed by a rash as well as intractable fevers of unknown etiology-much later attributed to her lupus. Although she had a most concerning and dramatic pre- sentation, she, so far, had responded very well to therapy including pulse dose steroids, plasmapheresis, intravenous immunoglobulins (IVIG), cyclophosphamide, and related medications.

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.

We report 10 cases of a non-neurocytic, purely neuronal tumor affecting adults. Situated in the cerebral hemispheres, with 7 of 10 confined to the temporal lobes, most presented with seizures as their principal clinical manifestations. On magnetic resosnance imaging (MRI), the tumors generally appeared solid and non-contrast enhancing with minimal diffuse infiltration, edema, or mass effect. Six examples demonstrated internal nodularity. Microscopically, the tumor cells were largely distributed into discrete and coalescent nodules exhibiting varying degrees of matrix vacuolization, principally within the deep cortical ribbon and superficial subcortical white matter. Populating elements ranged from morphologically ambiguous to recognizably neuronal, with only two cases manifesting overt ganglion cell cytology. In all cases, tumor cells exhibited widespread nuclear immunolabeling for the HuC/HuD neuronal antigens, although expression of other neuronal markers, including synaptophysin, neurofilament and chromogranin was variable to absent. Tumor cells also failed to express GFAP, p53, IDH1 R132H, or CD34, although CD34-labeling ramified neural elements were present in the adjoining cortex of seven cases. Molecular analysis in a subset of cases failed to reveal DNA copy number abnormalities or BRAF V600E mutation. Follow-up data indicate that this unusual neuronal lesion behaves in benign, World Health Organization (WHO) grade I fashion and is amenable to surgical control.

Paraganglioma (PGL) of the filum terminale is a rare tumor of extra-adrenal paraganglia. The reported cases of filum terminale and cauda equina PGLs often present with low-back pain and sciatica. While sensory or motor deficits and paraplegia may occur, the incidence is relatively low. We present a case of a 51-year old male with hemorrhagic paraganglioma of the filum terminale. He presented with acute paraplegia and was treated via emergent laminectomy, evacuation of hematoma, and resection of tumor. The patient had a significant but incomplete neurological recovery. The clinical, radiologic, and histopathological characteristics of the condition are described

Comitant infantile exotropia is a rare entity and is far less common than infantile esotropia. It has been shown to be highly correlated with intracranial pathology. We report a case of a medulloblastoma in a child in whom it presented as an incomitant esotropia after surgical correction for comitant infantile exotropia