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Biomedical subjects

Richard A Prayson

Publications and source records attributed to Richard A Prayson.

At least 73 records · Page 4Linked to original sources
PubMed2003

Intracranial sinus thrombosis in a patient with Crohn disease and factor V Leiden mutation.

There is a well-known risk of thrombosis in patients with inflammatory bowel disease. Documented cases of intracranial sinus thrombosis in this setting are rare. We present the case of a 30-year-old man with Crohn disease who spontaneously developed a superior sagittal sinus thrombosis and bifrontal infarcts that resulted in death. The patient was heterozygous for factor V Leiden mutation. The literature was searched to assess the frequency of cerebral venous infarcts in inflammatory bowel disease and the role that factor V Leiden plays in thrombosis in such patients.

Adult↗
PubMed2002

Gliomatosis cerebri: treatment results with radiotherapy alone.

BACKGROUND: Gliomatosis cerebri (GC) is a rare primary brain tumor characterized by proliferation of neoplastic glial cells that typically involve multiple brain areas, with preservation of brain structures and sparing of neurons. The optimal therapeutic strategy is not well established. The impact of radiotherapy on survival in patients with GC remains undefined. METHODS: Between 1980 and 2001, 12 patients with GC were identified, representing less than 1% of all patients with primary brain neoplasms treated at the Cleveland Clinic. RESULTS: All 12 patients had brain biopsies between March 1986 and July 2001 (seven males, five females, with a median age of 53 years [range, 13-85 years]). Median Karnofsky performance status at the time of presentation was 70 (range, 40-90). Eight patients had low-grade gliomas and four patients had anaplastic astrocytomas. Eight patients received radiation treatment to the brain as the only treatment, and four received neither radiation nor chemotherapy. The median dose of megavoltage radiation was 55.4 Gy (range, 45-61.2 Gy). Of the eight patients who received brain radiotherapy, the clinical and radiologic follow-up findings improved in three patients, stabilized in three patients, and worsened in two patients. Median follow-up was 10.3 months (range, 1-55 months). The median survival for the eight patients who received brain radiotherapy was 11.4 months. The one- and two-year survival rates were 45% and 30%, respectively. Two of the eight patients who received radiotherapy were alive at the time of writing. The four patients who did not receive radiotherapy died of the disease at 0.6, 1.0, 1.9, and 2.4 months after diagnosis. CONCLUSIONS: Gliomatosis cerebri is associated with poor survival. Although brain radiotherapy controlled or stabilized disease progression in most patients, the overall survival after brain radiotherapy alone was not satisfactory. More aggressive therapy may be needed.

Adolescent↗
PubMed2002

Pathologic characteristics of the cortical dysplasias.

The gross and microscopic features of cortical dysplasia (malformations caused by abnormalities of cortical development) are reviewed and illustrated in this article. The pathologic associations of neurocutaneous disorders, neoplasms, and hippocampal sclerosis with cortical dysplasia also are discussed.

Brain↗
PubMed2002

Collision of a syncytial meningioma and malignant astrocytoma.

The coexistence of two or more primary brain tumors is a relatively rare occurrence. We report an 87-year-old woman with a 5-cm cystic-solid, peripherally enhancing, right frontal lobe mass. The woman presented with progressive hemiparesis, difficulty ambulating, and urinary incontinence. Histologic examination of the surgically excised lesion showed geographically distinct areas of syncytial meningioma (World Health Organization grade I) and malignant astrocytoma (World Health Organization grade III). Focally, infiltration of the meningioma by astrocytoma was observed. Differential diagnostic issues are considered and the literature reviewed.

Aged↗
PubMed2002

Cyclooxygenase-2 (COX-2) expression by immunohistochemistry in glioblastoma multiforme.

Cyclooxygenase-2 (COX-2) (an enzyme responsible for the conversion of arachidonic acid to prostaglandin) upregulation has been described in association with a variety of tumors including gliomas. This study reviews the immunohistochemical profile of 47 glioblastoma multiforme using COX-2 antibody and correlates the results with MIB-1 (marker of cell proliferation) immunostaining. Positive staining with COX-2 was observed in 35 tumors (74.5%); more than 10% tumor cell positivity was seen in 10 tumors (21.3%). Immunostaining results were as follows: no staining, N = 12 (25.5%); 0% to 5% of tumor cells, N = 20 (42.6%); 5% to 10%, N = 5 (10.6%); 10% to 20%, N = 4 (8.5%); 20% to 50%, N = 4 (8.5%); and greater than 50%, N = 2 (4.3%). The mean MIB-1 labeling index for all tumors ranged from 3.0 to 76.4 (mean, 19.6). Mean MIB-1 labeling indices were higher in tumors with greater than 5% COX-2 immunostaining (mean MIB-1 labeling index, 23.5) versus tumors with 0% to 5% COX-2 immunostaining (mean MIB-1 labeling index, 17.7). There is evidence of COX-2 expression by immunohistochemistry in the majority of glioblastoma multiforme. As a group, tumors with a higher rate of cell proliferation tended to have increased expression of COX-2. These findings are significant in that therapeutic agents, which inhibit COX-2, are currently available and may play a role in the management of glioblastoma multiforme.

Adolescent↗
PubMed2002

Apoptotic and proliferative markers in chordomas: a study of 26 tumors.

Few studies have examined the role of cell proliferation and apoptotic markers in chordomas. This study retrospectively reviews the clinicopathologic features of 26 chordomas and examines MIB-1, p53, bcl-2, and cyclin D1 immunoreactivity in these neoplasms. Patients ranged in age from 34 to 78 years (mean, 60.7 years) and included 14 females. The most common presentations included lower back pain (N = 15) and headaches (N = 10). Sixteen tumors arose in the lumbosacral region and 10 in the clivus. Initial surgery included biopsy (N = 17), subtotal resection (N = 4), and gross total resection (N = 5). The single highest mitosis count per 10 high power fields ranged from 0 to 6 (mean, 1). Marked nuclear pleomorphism was identified in seven tumors. Marked hypercellularity was seen in two tumors. Focal necrosis was identified in seven tumors. MIB-1 labeling indices (LI) in 22 tumors ranged from 0 to 3.8 (mean, 0.5). Cyclin D1 LI ranged from 0 to 82.4 (mean, 35.6). Seven tumors had positive p53 immunostaining and three demonstrated focal positive staining with bcl-2 antibody. Five tumors locally recurred; two patients developed metastatic disease. Thirteen patients received adjuvant chemotherapy and/or radiation therapy. At last known follow-up, seven patients died with tumor (12 to 132 months follow-up). Five additional patients died, two without tumor at 36 and 72 months follow-up and three patients in whom the tumor status at death was not known. Seven patients were alive with evidence of tumor (1 to 120 months) and five patients were alive without evidence of tumor (12 to 84 months). Clinical follow-up was not available in one patient. In conclusion, the low MIB-1 LIs and the lack of p53 and bcl-2 staining is in keeping with the low-grade nature of most chordomas. High cyclin D1 LIs may be reflective of a tendency to accumulate cyclin D1 protein; however, there appears to be a block in the effect of cyclin D1 on cell proliferation in these tumors. Cyclin D1, MIB-1, p53, and bcl-2 immunostaining does not appear to improve one's ability to predict behavior versus routine light microscopy.

Adult↗
PubMed2002

Metastatic epithelioid sarcoma to the brain: palisaded necrosis mimicking glioblastoma multiforme.

Epithelioid sarcomas are rare, morphologically distinct tumors that have a propensity to arise in the extremities. Brain metastasis from epithelioid sarcoma are a relatively rare occurrence. We report a case of brain metastasis in a 50-year-old man who was previously diagnosed with an epithelioid sarcoma arising in the elbow. Before the diagnosis of brain metastasis, he had developed an axillary lymph node metastasis. He presented with neurologic symptoms of progressively worsening headache and loss of vision on the right side. He underwent gross total resection of an occipital lobe mass. Histologically, the tumor was focally characterized by prominent perinecrotic pseudopalisading and demonstrated immunoreactivity with antibodies to cytokeratin AE1/3 and CAM5.2; the tumor did not stain with glial fibrillary acidic protein antibody. The literature is reviewed and the morphologic distinction between metastatic epithelioid sarcoma and other central nervous system neoplasms is discussed.

Brain Neoplasms↗
PubMed2002

Skeletal muscle vasculitis exclusive of inflammatory myopathic conditions: a clinicopathologic study of 40 patients.

Vasculitis is an infrequently encountered pathology in skeletal muscle biopsy specimens, the diagnosis of which has significant therapeutic implications. This study retrospectively reviewed the clinicopathologic features of 40 patients with vasculitis (infiltration of vessel walls by inflammatory cells) diagnosed via skeletal muscle biopsy during a 27-year period of time. Cases of vasculitis associated with inflammatory myopathic conditions, such as polymyositis or dermatomyositis, were excluded from study. Forty patients, including 21 women and 19 men ranging in age from 19 to 83 years (mean of 52 years), formed the study group. The most common clinical presentations included muscle pain (n = 22, 55%), paresthesias (n = 16, 40%), and weight loss (n = 15, 38%). Westergren erythrocyte sedimentation rate was known in 31 patients and ranged from 12 to >150 mm/h (mean, 61 mm/h). Necrotizing vasculitis was identified in 35 patients (88%). The vasculitic process involved multiple vessels in 30 cases (75%). Vessel wall scarring and/or fibrosis were identified in 11 patients (28%), thrombi in 3 patients (8%), eosinophils in 2 patients (5%), and granulomas in 1 patient (3%). Neurogenic changes were observed in the majority of biopsy specimens as follows: angular atrophic esterase-positive muscle fibers (n = 35, 88%), grouped atrophy (n = 14, 35%), fiber type grouping (n = 17, 43%), and target fibers (n = 6, 15%). Other commonly identified pathologies included scattered degenerating muscle fibers (n = 15, 38%), regenerating muscle fibers (n = 17, 43%), and type II muscle fiber atrophy (n = 10, 25%). Thirty-three patients also underwent sural nerve biopsy. Vasculitis was identified in the nerve in 26 of 33 of these patients (79%). Peripheral nerve vasculitis was classified as necrotizing in 15 cases (57.7%) and as nonnecrotizing in 11 cases (42.5%). Seventeen of 33 patients (52%) with known follow-up showed clinical improvement with steroid/immunosuppressive therapy. The following 2 conclusions were made: the majority of vasculitis cases arising outside the setting of inflammatory myopathy in skeletal muscle are necrotizing, and the predominant muscle pathology in the setting of vasculitis is neurogenic atrophy, likely caused by concomitant involvement of the peripheral nervous system.

Adult↗
PubMed2002

Skull base neurocytoma: case report and review of the literature of extraventricular neurocytomas.

We report a rare skull base neurocytoma. A 44-year-old female with a history of focal seizure and progressive right-sided weakness sought treatment at an outside institution, where she underwent total resection of a "left medial sphenoid wing paraganglioma" in 1984. In 1995 after experiencing intense left-sided headaches for 3 weeks, the patient presented to our institution. Magnetic resonance imaging revealed a large local recurrence. She had deficits dating to her initial surgery, including moderate right-sided hemiparesis, complete left ophthalmoplegia, and left facial numbness.The patient underwent a craniotomy with extensive removal of the involved sphenoid bone and a subtotal resection of the tumor. Neurocytoma was diagnosed based on strong immunohistochemical staining for synaptophysin and no reactivity for glial fibrillary acidic protein. Postoperatively, her headaches resolved completely and her neurologic status remained at baseline. The residual tumor was treated with radiation therapy. After 5 years, she remains clinically and radiographically stable.Although typically located adjacent to the foramen of Monro, neurocytomas have now been reported in almost every subcompartment of the craniospinal axis. Finding neurocytomas in extraventricular locations may require revisiting the current theory that subependymal progenitor cells are the cells of origin for these tumors.

Case Reports↗
PubMed2002

Cell proliferation and tumors of the central nervous system. Part 1: Evaluation of mitotic activity.

Evaluation of cell proliferation has been long recognized in pathology as a mainstay of diagnosis and important in the prognostication of a variety of neoplasms. Routine light microscopic evaluation of mitotic activity has long served as a reasonable assessment of cell proliferation. Counting mitotic figures has the advantage of being inexpensive and relatively quick. The main objections leveled against utilization of mitosis counts in diagnostic decision making are related to the instability of mitotic figures due to prefixation and fixation issues and problems with interobserver reproducibility of counts. This paper reviews factors that affect the identification of mitotic figures and the determination of mitosis counts. The role mitosis evaluation plays in the evaluation of certain neoplasms of the central nervous system is discussed.

Brain Neoplasms↗
PubMed2002

Cell proliferation and tumors of the central nervous system, part II: radiolabeling, cytometric, and immunohistochemical techniques.

A variety of techniques have been developed to evaluate cell proliferation. Many of these methods provide a more accurate means of assessing the true proliferation rate of a given neoplasm, as compared with the simple assessment of mitotic activity. Similar to the evaluation of mitotic activity, these methods are also subject to limitations associated with tumor heterogeneity and interobserver variability. This paper reviews a variety of methodologies including radiolabeling, flow cytometric, and immunohistochemical that have been used in recent years to evaluate cell proliferation in brain neoplasms. Factors that affect these methodologies and their practical application to routine practice of diagnostic neuropathology will be explored.

Cell Division↗
PubMed2002

Necrotizing neurosarcoidosis of the cranial base resembling an en plaque sphenoid wing meningioma: case report.

OBJECTIVE AND IMPORTANCE: Necrotizing sarcoid granuloma (NSG) has been recognized as a histological variant of sarcoidosis. Two cases of neurosarcoidosis (NS) with NSG with concomitant systemic disease have been described previously. We present an unusual case of primary NS-NSG that resembled an en plaque cranial base meningioma. CLINICAL PRESENTATION: A 51-year-old man presented with a 3-month history of progressive left visual deterioration and proptosis. Brain magnetic resonance imaging demonstrated a large cranial base lesion occupying the left anterior clinoid process and sphenoid wing, extending to the left frontotemporal convexity. A second dura-based lesion was observed in the right parietal convexity. Both lesions enhanced homogeneously after administration of intravenous contrast medium. Magnetic resonance imaging characteristics were consistent with a typical clinoidal meningioma with an en plaque extension laterally. INTERVENTION: A left frontotemporal craniotomy with extradural removal of the anterior clinoid process was performed. When the dura was opened, a red fibrous mass was identified. Intraoperative histological analysis revealed the presence of necrotizing and noncaseating granulomas. Postoperatively, cerebrospinal fluid, erythrocyte sedimentation rate, C-reactive protein, and serum and cerebrospinal fluid angiotensin-converting enzyme values were normal. The search for acid-fast bacilli or fungi was negative. A diagnosis of primary NS-NSG was made. The patient began long-term high-dose corticosteroid therapy. One and a half years after surgery, his vision had improved significantly and the lesions were stable as revealed by magnetic resonance imaging. CONCLUSION: The first case of primary NS-NSG in the absence of systemic sarcoidosis is reported. NS should be included in the differential diagnosis of dura-based lesions resembling meningioma.

Diagnosis, Differential↗