Ectopic pituitary adenoma associated with an empty sella presenting with hearing loss: case report with literature review.

Ectopic pituitary adenomas are uncommon entities that may pose substantial diagnostic challenges. In the majority of these cases, patients present with endocrine and/or nasal obstruction symptoms. We report the case of an ectopic pituitary adenoma in a 76-year-old man with an empty sella who initially presented with right-sided hearing loss progressing to bilateral hearing loss over the next 4 years. Neuroimaging studies revealed a large, expansile central skull base mass replacing the clivus and sphenoid sinus, and invading the internal auditory canals and inner ear bilaterally. The tumor also involved the floor of the middle cranial fossae and bilateral medial temporal and occipital bones. Histopathologic examination, including immunohistochemical studies, revealed a sparsely granulated lactotroph adenoma. Hearing loss in a patient with ectopic pituitary adenoma constitutes an extremely unusual presentation. This case was further complicated by the presence of an empty sella and the absence of symptoms related to hyperprolactinemia.

Cellular solitary fibrous tumor (hemangiopericytoma) with anaplasia at cerebellopontine angle--a case report.

Cellular solitary fibrous tumor is currently considered a synonym for hemangiopericytoma, as it became increasingly clear that the morphological and immunohistochemical features that separate these two entities have become tenuous, and evidence for a unifying concept has emerged. Furthermore, as no evidence of pericytic differentiation is given in most cases of hemangiopericytoma, this diagnostic term is waning in popularity. We present here a case of cellular solitary fibrous tumor in a 22-year-old man. Neuroimaging revealed a right cerebellopontine angle tumor. Most of the tumor was cellular although some less cellular areas were seen. Sinusoidally dilated large vessels, including staghorn type, were seen. Nuclear pleomorphism and increased mitotic activity (5 mitosis/10 high power field) were regarded as evidence of anaplasia. Diffuse CD34 immunoreactivity and focal positivity for Factor XIIIa were seen in the tumor, which was negative for EMA and S100. The tumor also displayed rich reticulin network. Solitary fibrous tumor at cerebellopontine angle is rare, and 20 such cases (five reported as hemangiopericytoma) have been reported in the English literature.

Primary Ewing sarcoma of lumbar spine with massive intraspinal extension.

Primary vertebral Ewing sarcoma-primitive neuroectodermal tumor is uncommon. Although epidural extension has been seen in such tumors, cases with massive intraspinal involvement are decidedly rare. Here we present the case of a 4-year-old girl with back pain and difficulty walking. Magnetic resonance imaging showed a mass filling the spinal canal from T(11) to the L(3)/L(4) levels. Vertebral involvement with extension into the paraspinal soft tissue through neural foramina was seen. Histologically, a small-blue-cell tumor with strong membranous CD99 reactivity was noted. Molecular analysis revealed translocation t(11;22)(q24;q12), thus confirming the diagnosis of Ewing sarcoma-primitive neuroectodermal tumor. Our case emphasizes that vertebral Ewing sarcoma-primitive neuroectodermal tumor may present with massive intraspinal extension and should be included in the differential diagnosis of intraspinal lesions.

Molecular diagnosis of Nocardia farcinica from a cerebral abscess.

Histopathology and special stains of a brain biopsy specimen from a 42-year-old man revealed numerous gram-positive bacilli arranged in branching filaments, suggesting Nocardia infection. Antibiotic therapy with trimethoprim-sulfamethoxazole markedly decreased the abscess size, and the patient improved. DNA was analyzed from formalin-fixed sections of the cerebral abscess by a 16S ribosomal DNA polymerase chain reaction assay demonstrating the presence of either Nocardia farcinica or N otitidiscaviarum. A species-specific polymerase chain reaction assay confirmed N farcinica as the etiologic agent.

Atypical protein kinase C in neurodegenerative disease II: PKCiota/lambda in tauopathies and alpha-synucleinopathies.

To study the role of atypical protein kinase C (aPKC) in neurodegenerative disease, we investigated the distribution of PKCiota/lambda, an aPKC isoform, in a variety of tauopathies and alpha-synucleinopathies. Immunohistochemical study revealed PKCiota/lambda within tau-positive neurofibrillary inclusions in Alzheimer disease (AD), progressive supranuclear palsy, corticobasal degeneration (CBD), and Pick disease (PiD), within alpha-synuclein-positive Lewy bodies in idiopathic Parkinson disease and dementia with Lewy bodies, as well as within glial inclusions in multisystem atrophy. We also observed PKCiota/lambda label of actin-rich Hirano bodies in AD, PiD, and elderly individuals. Double immunolabeling and fluorescence resonance energy transfer demonstrated close physical association between PKCiota/lambda and phospho-tau or alpha-synuclein in some neurofibrillary tangles and Lewy bodies. Furthermore, PKCiota/lambda colocalized with p62, a chaperone protein that binds to both aPKC and ubiquitin, in most of these inclusions. PKCiota/lambda also closely associated with the inactivated form of glycogen synthase kinase-3beta, GSK-3beta[ser9]. Together, these findings suggest that PKCiota/lambda may play a role in common mechanisms involving the pathogenesis of neurodegenerative disease.

Plasma cell tumors in HIV-positive patients: report of a case and review of the literature.

Plasma cell tumors show an increased incidence in HIV-positive patients. The cases reported in the literature suggest that plasma cell tumors occur in a younger age group than that encountered in the general population. Pathologically, many of these tumors show a plasmablastic morphology. Plasma cell tumors in HIV-positive patients may present at unusual sites and progress rapidly to involve multiple sites, including the soft tissues and viscera. The prognosis is generally poor. These features may be related to a combination of factors, including immunodeficiency, oncogenic viruses, and altered cytokine milieu in these patients. A case of plasma cell tumor in an HIV-positive patient is presented.

Prolonged survival in recurrent endometrial carcinoma to the brain.

BACKGROUND: Recurrence of endometrial cancer in the brain is a rare event generally accompanied by limited life expectancy. We present an unusual case of long-term survival following surgical resection of an intracranial endometrial cancer metastasis. CASE: The present case is a patient with FIGO stage IIB, grade III endometrial cancer which recurred 2 months following completion of primary therapy with an isolated lesion in the brain. Aggressive trimodal therapy was initiated with curative intent and she has remained without clinical or radiographic evidence of disease for more than 30 months following treatment of her recurrence. CONCLUSIONS: Aggressive multi-modal therapy is warranted in the treatment of isolated intracranial recurrences of endometrial cancer in carefully selected patients. With complete surgical resection of disease, the precise nature and role of adjuvant treatment has yet to be clearly defined.