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R A Prayson

Publications and source records attributed to R A Prayson.

At least 73 records · Page 4Linked to original sources
PubMed1998

Systemic T cell adoptive immunotherapy of malignant gliomas.

OBJECT: To determine the feasibility, toxicity, and potential therapeutic benefits of systemic adoptive immunotherapy, 10 patients with progressive primary or recurrent malignant glioma received this treatment. Adoptive immunotherapy, the transfer of immune T lymphocytes, is capable of mediating the regression of experimental brain tumors in animal models. In animal models, lymph nodes (LNs) that drain the tumor vaccine site are a rich source of tumor-immune T cells. METHODS: In this clinical study, patients were inoculated intradermally with irradiated autologous tumor cells and granulocyte macrophage-colony stimulating factor as an adjuvant. Cells from draining inguinal LNs, surgically resected 7 days after vaccination, were stimulated sequentially with staphylococcal enterotoxin A and anti-CD3, and a low dose of interleukin-2 (60 IU/ml) was used to expand the stimulated cells. The maximum cell proliferation was 350-fold over 10 days of culture. The activated cells were virtually all T cells consisting of various proportions of CD4 and CD8 cells. These cells were given to patients by intravenous infusion at doses ranging from 9 x 10(8) to 1.5 x 10(11). There were no Grade 3 or 4 toxicities associated with the treatment. Following T-cell transfer therapy, radiographic regression that lasted at least 6 months was demonstrated in two patients with recurrent tumors. One patient demonstrated stable disease that has lasted for more than 17 months. The remaining patients had progressive disease; however, four of the eight patients with recurrent tumor remain alive more than 1 year after surgery for recurrence. Three patients required intervention with corticosteroid agents or additional surgery approximately 1 month following cell transfer. CONCLUSIONS: These intriguing clinical observations warrant further trials to determine whether this approach can provide therapeutic benefits and improve survival.

Adjuvants, Immunologic↗
PubMed1998

Cerebrospinal fluid cytology: an 11-year experience with 5951 specimens.

OBJECTIVE: Cerebrospinal fluid (CSF) diagnoses encompass a wide spectrum of conditions. The authors review one institution's CSF cytology results over an 11-year period. DESIGN AND SETTING: A retrospective study of 5951 CSF specimens generated between 1985 and 1995. Specimens from pediatric patients (<19 years of age) from the same time period were separately identified. RESULTS: A total of 5561 adult and 390 pediatric CSF specimens were interpreted. A diagnosis of "negative for malignant cells" was assigned in 5171 (93%) of the adult cases and in 351 (90%) of the pediatric cases. Specific infectious organisms were identified in 26 adult specimens and one pediatric specimen. Cryptococcus was the most common infectious agent observed (n = 23 adults), and Toxoplasma was the sole pediatric infectious agent. Two hundred seventy-six (5%) adult cases and 31 (8%) pediatric cases were positive for malignant cells. Diagnoses included metastatic tumors (adult, 140 [51%]; pediatric, 0); lymphoma/leukemia (adult, 112 [41%]; pediatric, 4 [13%]); malignant unclassified neoplasms (adult, 9 [3%]; pediatric, 0); and primary central nervous system neoplasms (adult, 12 [4%]; pediatric, 27 [87%]). Medulloblastoma was the most common pediatric neoplasm (n = 21). There were 105 (2%) adult cases and 8 (2%) pediatric cases with atypical cells present. Atypical lymphoid cells were the most common type in adult cases (53%). CONCLUSIONS: In our experience, infectious agents were rarely identified in pediatric CSF specimens. In adult specimens, the most commonly identified organisms was Cryptococcus. Primary central nervous system neoplasms accounted for a higher percentage of CSF specimens in the pediatric population than in the adult population. The most commonly identified malignancy in adults was metastatic neoplasms, and in children, medulloblastoma.

Adenocarcinoma↗
PubMed1998

The neuropathology of orthotopic liver transplantation: an autopsy series of 16 patients.

OBJECTIVE: To examine the neuropathologic findings seen in the setting of orthotopic liver transplantation (OLT) and to asses the role, if any, that the neuropathology had in the patient's death. DESIGN: Retrospective autopsy series of 16 patients. SETTING: Tertiary referral center with a high volume of liver transplantation. PATIENTS: Sixteen OLT patients who died and in whom a complete autopsy, including examination of the brain and spinal cord, was performed. RESULTS: Sixteen patients, including 13 women and 3 men, comprised the study group. Patients ranged in age from 25 to 64 years (mean 44.8 years). Postoperative OLT survival ranged from 1 to 1962 days (mean 236 days). Reasons for the initial OLT included hepatitis (n = 6), fulminant hepatic failure (n = 4), cryptogenic cirrhosis (n = 2), methotrexate toxicity (n = 1), postoperative complication (n = 1), primary biliary cirrhosis (n = 1), and hepatocellular carcinoma (n = 1). Autopsies in 13 (81%) patients showed neuropathology; in only 2 patients, however, was the primary cause of death attributable to these findings. The most common neuropathology was related to anoxia or infarction, specifically, ischemia or focal neuronal necrosis (n = 9), infarction (n = 4), and diffuse anoxic encephalopathy (n = 3). Other central nervous system findings included infection with Aspergillus, Candida, and Toxoplasma (n = 3). The most common cause of death was infection-related in 8 patients. One patient died of pulmonary hypertension, 1 of acute rejection, and 1 of possible hyperacute rejection. Two patients died directly as a consequence of neuropathology findings; one had massive central edema with herniation, and the other had a large intracerebral hemorrhage with herniation. The exact cause of death was unclear in 3 patients. CONCLUSIONS: The most common neuropathology findings in this series were related to ischemia and infarction. Neuropathology findings are a significant cause of morbidity, but were only rarely the main cause of death (n = 2) in the OLT patients in this study.

Adult↗
PubMed1998

Mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes (MELAS) syndrome: an autopsy report.

Autopsy reports of patients with mitochondrial encephalopathy with lactic acidosis and strokelike episode (MELAS) are rare. This report documents the clinical and autopsy findings of a 47-year-old woman with MELAS syndrome. The diagnosis was corroborated by documenting a mitochondrial DNA mutation tRNA-Leu (UUR) at position 3243. The patient's clinical history was marked by schizophrenia, peptic ulcer disease, constipation requiring hemicolectomy, migraine headaches, deafness, and a left temporal lobe infarct. At autopsy, a muscle biopsy demonstrated numerous ragged red fibers and a partial cytochrome C oxidase deficiency. By electron microscopy, increased numbers of slightly hypertrophic mitochondria were observed focally within myocytes and vessel walls; paracrystalline mitochondrial inclusions were not seen. The brain at autopsy showed mild cerebral atrophy and diffuse cortical gliosis. Prominent bilateral basal ganglia calcifications and vascular sclerosis were present, and a small remote left temporal lobe infarct was seen.

DNA Mutational Analysis↗
PubMed1998

Anaplastic pleomorphic xanthoastrocytoma.

Well-documented cases of malignant degeneration in pleomorphic xanthoastrocytoma and of anaplastic pleomorphic xanthoastrocytoma are rare in the literature. We report 2 cases of pleomorphic xanthoastrocytoma, 1 of which demonstrated clear evidence of malignant degeneration in the absence of prior radiation therapy over an 18-year period. Both anaplastic tumors were characterized by foci of necrosis and increased mitotic activity (3 and 2 mitotic figures/10 high-power fields). Both tumors demonstrated focal positive staining for glial fibrillary acidic protein and showed marked reticulin deposition. An MIB-1 labeling index (marker of cell proliferation) in the initial low-grade-appearing tumor in case 1 was 0.1%. The recurrent tumor in case 1 had an MIB-1 labeling index of 4.9%, and the anaplastic tumor in case 2 had an MIB-1 labeling index of 5.4%. Significant cyclin D1 immunoreactivity was not observed in either anaplastic tumor. Two percent to 3% of tumor cells stained positive with p53 protein antibody in the recurrent anaplastic tumor in case 1. Although histology may not reliably predict aggressive behavior in pleomorphic xanthoastrocytomas, the presence of increased mitosis, necrosis, and increased cell proliferation labeling indices may be indicative of a higher grade tumor.

Adolescent↗
PubMed1997

Cytologic specimens from the eye: a clinicopathologic study of 33 patients.

Cytologic specimens from the eye (CSE) (fine-needle aspirates and intraocular [vitrectomy] washings) are uncommon in most cytology practices. We reviewed our experience and diagnostic accuracy in lesions from this site over a 13-yr period. Thirty-three patients (12 males, 21 females, age 7-85 yr; mean 56 yr) were identified. Eighteen specimens were submitted to rule out malignancy. Of these, four CSE were positive, including melanoma (n = 1), large-cell lymphoma (n = 2), and carcinoma (n = 1). None of the 14 patients with negative cytology showed clinical evidence of malignancy over a 1-28-mo follow-up period (mean, 8.4 mo). Fourteen specimens were submitted to rule out infectious agents. Cytology material contained culture-proven organisms in one case each of Candida and Aspergillus. Of serology-confirmed specimens, four CSE failed to reveal toxoplasma, and three lacked herpes viral inclusions. One case each of culture-positive P. acnes and C. albicans lacked the organisms in the CSE. Twelve specimens were submitted to rule out inflammatory lesions. CSE showed chronic inflammation (n = 5), acute and chronic inflammation (n = 1), macrophages (n = 5), and nonnecrotizing granulomas (n = 1). Of 3 patients with sarcoidosis, CSE contained mature lymphocytes in all 3 cases, but no granulomas. Although the numbers are small, there is greater sensitivity in the detection of malignancy than of infectious agents.

Adolescent↗
PubMed1997

Ubiquitin immunostaining and inclusion body myositis: study of 30 patients with inclusion body myositis.

Distinction of inclusion body myositis (IBM) from other forms of inflammatory myopathy is significant from prognostic and therapeutic standpoints. This study retrospectively examines ubiquitin expression by paraffin immunohistochemistry in muscle biopsy material from 30 patients with IBM. Patients included 19 men and 11 women (ages 29 to 80 years; mean, 64 years). All biopsies were characterized by endomysial chronic inflammation, muscle fiber degeneration and regeneration, rimmed vacuoles, and angular atrophic esterase-positive muscle fibers. Ragged red fibers were identified in biopsies of five patients and a partial cytochrome C-oxidase deficiency by enzyme histochemistry in biopsies of 10 patients. Evidence of intranuclear or cytoplasmic tubulofilamentous structures confirming a diagnosis of IBM was observed in all 30 cases. Paracrystalline mitochondrial inclusions were noted in five patients. Discrete myocyte intranuclear ubiquitin-positive inclusions were noted in 14 patients (47%). Discrete intracytoplasmic ubiquitin-positive inclusions were noted in 24 (80%) patients. Positive staining of rimmed vacuoles by ubiquitin was observed in 25 (83%) patients. Diffuse staining of scattered muscle fibers was observed in 21 (70%) patients. In a control group including patients with polymyositis (n = 3), dermatomyositis (n = 3), necrotizing vasculitis (n = 1), and granulomatous myositis (n = 1), discrete intranuclear or cytoplasmic ubiquitin-positive inclusions were not observed. Rimmed vacuoles were not seen either by light microscopy or ubiquitin immunostaining in any of the eight cases. Occasional myofibers from all eight cases showed diffuse, positive muscle fiber staining. Although not present in all cases, evidence of ubiquitin-positive myocytic intranuclear or cytoplasmic inclusions or positive-staining rimmed vacuoles in the setting of an inflammatory myopathy may be suggestive of a diagnosis of inclusion body myositis. Use of ubiquitin immunohistochemistry may be useful in cases in which frozen tissue or tissue processed for electron microscopy is not available, and IBM is suspected. Light or electron microscopic evidence of mitochondrial abnormalities were noted in a significant subset of patients (13 of 30; 43%) of patients with IBM.

Adult↗
PubMed1997

Hamartomas in the setting of chronic epilepsy: a clinicopathologic study of 13 cases.

Hamartomas are a poorly defined group of lesions and a rare cause of chronic epilepsy. We studied 13 patients, nine males and four females, whose cause of seizures was attributed to a hamartoma. The patients ranged in age from 6 to 33 years (mean, 21 years). Seizure duration before surgery ranged from 1.5 to 22 years (mean, 10 years). Seven hamartomas were located on the right side and six on the left. Six were located in the frontal lobe, five in the temporal lobe, and two in the occipital lobe. Twelve patients underwent gross total resection of the lesion and one a partial resection. All consisted of a circumscribed, disorganized collection of glial cells, primarily astrocytes. Rarely a neuronal component was admixed. One lesion contained an increased number of small blood vessels. Eight (62%) hamartomas contained eosinophilic granular bodies, and focal microcalcification was observed in three lesions (23%). Adjacent cortical architectural abnormalities (cortical dysplasia) were identified in eight (62%) resection specimens. Necrosis, mitoses, and prominent cytological atypia were absent in all lesions. Differential diagnostic considerations include low grade astrocytoma, ganglioglioma, dysembryoplastic neuroepithelial tumor, and cortical dysplasia. Postoperatively, 10 patients (77%) had complete resolution or greater than 90% reduction of seizure frequency. Two patients (16%) developed recurrent seizures 8 and 13 months postoperatively. One patient who underwent a partial resection showed no decrease in seizure frequency. No lesion recurrence on imaging studies has been observed in the 12 patients who underwent gross total resection of their hamartoma during 1 to 51 months (mean, 14 months) follow-up. We conclude that hamartomas seen in the setting of chronic epilepsy are generally low-grade lesions that respond well to gross total resection. Circumscription and lack of significant cytological atypia help distinguish these lesions from other neoplastic causes of epilepsy. Hamartomas that arise in the setting of chronic epilepsy appear to be associated with increased incidence of cortical architectural abnormalities (cortical dysplasia) and represent maldevelopmental lesions.

Adolescent↗
PubMed1997

A clinicopathologic study of 30 cases of oligoastrocytoma including p53 immunohistochemistry.

Mixed gliomas have been difficult to define and subsequently diagnose due to the paucity of literature specifically examining this group of tumors. Thirty mixed gliomas in which the minor glial component comprised at least 20% of the total tumor were studied: 20 oligoastrocytomas (OA) and ten malignant oligoastrocytomas (MOA). Nineteen patients were male (mean age 36 years) and 19 patients (63%) presented with seizures. The tumor was located in the frontal lobe in 17 patients (57%) and the temporal lobe in nine patients (30%). The duration of preoperative symptoms in 25 patients ranged from five days to 14 years (mean 2.6 years). A mean follow-up of four years was available in 29 patients. Fourteen patients, seven with OA and seven with MOA, had recurrent tumor. One patient with MOA and four patients with OA (three with tumor progression and one with extensive leptomeningeal spread) died as a result of their tumor one to five years after diagnosis. Eighteen patients received chemotherapy and/or radiation therapy. Twenty-five tumors were immunostained with antibody to p53 protein. p53 nuclear staining was seen in 5/16 OA (31%) and 3/9 MOA (33%). Positive staining was observed only in astrocytic appearing cells. One of the four patients who died with OA was p53 positive. Three recurrent MOAs were p53 positive. Our study indicates that: 1. mixed gliomas most frequently occur in the frontal lobe and the majority of patients present with seizures; 2. there is no obvious association of p53 detection in mixed gliomas with tumor grade or behavior; and 3. similar to pure fibrillary astrocytomas, a subset of OA and MOA may be associated with p53 alterations.

Adolescent↗
PubMed1997

Epithelioid smooth-muscle tumors of the uterus: a clinicopathologic study of 18 patients.

Epithelioid smooth-muscle tumors of the uterus are uncommon neoplasms for which prognostic factors have not been well established. A retrospective follow-up study of 18 uterine epithelioid smooth-muscle tumors was performed. Patients ranged in age from 27 to 83 years (mean, 45 years) and were separated into three groups based on the nuclear grade of the epithelioid tumor cells. Two tumors had grade 1 nuclei; both were examples of intravenous leiomyomatosis. They had highest mitosis counts of 1 and 3 mitotic figures (MF)/10 high-power fields (HPF), no tumor cell necrosis was found, and both patients were alive with no evidence of disease at 64 and 5 months' follow-up. Ten tumors had grade 2 nuclei. All had highest mitosis counts of 0 to 3 MF/10 HPF, except one (5 MF/10 HPF). Tumor cell necrosis was absent in nine and only one had an infiltrative border. Tumor size ranged from 1.5 to 14 cm. Two tumors contained pleomorphic ("symplastic") multinucleated giant cells similar to those seen in bizarre leiomyomas. All nine patients with follow-up were alive with no evidence of disease 5 to 203 months postoperatively (median, 74 months). One patient had also received adjuvant radiation therapy. Six tumors had grade 3 nuclei. Highest mitosis counts of 4 to 9 MF/10 HPF were found in five; one had 1 MF/10 HPF. Maximum tumor size ranged from 4.5 to 13 cm. Two had tumor cell necrosis, and two had an infiltrative border. Two of these patients died of tumor 11 and 132 months postoperatively. The former had widespread metastases at initial surgery (stage IVb); the latter patient (stage I) developed the first of seven tumor recurrences 3 years postoperatively. Both patients had also received adjuvant chemotherapy. Of the remaining four patients, two were alive with no evidence of disease at 48 and 83 months, one was alive (tumor status unknown) at 28 months, and one was lost to follow-up. Based on our findings and those in the literature, we conclude that uterine smooth-muscle tumors with a predominance of epithelioid cells are extremely uncommon and metastasize infrequently. No single histologic feature is predictive of metastatic potential. Clinically malignant tumors (i.e., epithelioid leiomyosarcomas) typically have the combination of significant nuclear atypia (either grade 2 or grade 3 nuclei) and some mitotic activity (usually at least 3 to 4 MF/10 HPF); most also have tumor cell necrosis.

Adolescent↗
PubMed1997

Prevention of chronic cerebral vasospasm in dogs with milrinone.

OBJECTIVE: Delayed cerebral ischemia resulting from vasospasm is a major cause of morbidity and death in patients with aneurysmal subarachnoid hemorrhage. Milrinone, because it inhibits Type IV cyclic adenosine monophosphate-specific phosphodiesterase enzyme in both cardiac and vascular smooth muscle, is a powerful inotrope and vasodilator, but it has little effect on heart rate or blood pressure. Because of these properties, milrinone is an attractive potential therapy after subarachnoid hemorrhage. The purpose of the present study was to investigate the effect of milrinone on chronic experimental cerebral vasospasm. METHODS: A double-hemorrhage canine model of vasospasm was used to study the efficacy of milrinone. Angiographic vasospasm and systemic hemodynamics were compared in a treatment group of animals that received a loading dose of milrinone (0.05 mg/kg, intravenously) and then slow-release (0.05 microgram/kg/min) milrinone pellets (n = 10) and a control group that received placebo pellets (n = 9), over an 8-day period after the initial subarachnoid hemorrhage. The hemorrhage was created by injection of 4 ml of autologous, nonheparinized, arterial blood into the cisterna magna on Days 1 and 3. Hemodynamic measurements, including cardiac output determinations, were made on Days 0, 1, 3, 6, and 8 with a pulmonary artery catheter, and angiographic vasospasm was assessed on Day 8 by comparison with baseline angiograms. RESULTS: Treatment with milrinone caused no significant changes in systemic hemodynamics. Angiographic vasospasm, however, was significantly reduced in the Day 8 angiograms for the treated group, compared with the control group (98.28 +/- 14.06 and 67.89 +/- 13.06% of original vessel cross-sectional area, respectively; P < 0.001). CONCLUSION: Milrinone is effective in preventing chronic cerebral vasospasm in a canine model of experimental chronic cerebral vasospasm. This effect is independent of changes in systemic hemodynamics. Milrinone and related drugs warrant further investigation for the treatment of cerebral vasospasm.

Animals↗
PubMed1997

Solitary fibrous tumor of the meninges. Case report and review of the literature.

The authors present the case of a left frontal solitary fibrous tumor of the meninges. The gross appearance of the tumor was very similar to that of a fibroblastic meningioma. Histological examination showed a mixture of spindle-shaped and round cells arranged in a collagen matrix. Immunohistochemical staining of the tumor demonstrated diffuse positive staining for CD34 and vimentin. The tumor displayed no positive staining for markers of muscle, epithelial, glial, or neurocrest differentiation or for estrogen and progesterone receptors. The MIB-1 labeling index (the percentage of positive staining tumor cell nuclei), a marker of cellular proliferation, was 1.1%. Ultrastructural studies support attributing a mesenchymal, rather than meningothelial, nature to the tumor. A differential diagnosis is discussed and a review of the literature on these rare tumors is presented.

Adult↗
PubMed1997

Myxopapillary ependymomas: a clinicopathologic study of 14 cases including MIB-1 and p53 immunoreactivity.

Few studies have examined cell proliferation or p53 immunoreactivity in myxopapillary ependymomas. This study retrospectively examines tumor MIB-1 and p53 immunohistochemical features in 14 patients (eight women, six men; age range, 12-69 yr; median, 32 yr) with myxopapillary ependymoma. Their preoperative symptoms lasted from 2 months to 18 years (median, 12 mo) and most commonly involved lower back pain. The tumor was in the lumbar spinal cord region in 12 patients, the sacral cord in 1, and both the lower thoracic and upper lumbar cord in 1. In three patients, cerebrospinal fluid protein levels were markedly elevated, with negative cytologic results. Thirteen patients underwent a gross total resection. All of the tumors demonstrated histologic features diagnostic of myxopapillary ependymoma. Four cases had focal, prominent, nuclear pleomorphism. From 1 to 5 mitotic figures per 10 high power fields were identified in four tumors. There was no vascular proliferation or necrosis. Nine patients are alive at last known follow-up with no evidence of tumor (median, 36 mo); four are alive with residual tumor (median, 40 mo); and one died after 74 months (tumor status unknown). Eleven patients received adjuvant radiation and/or chemotherapy. Six experienced at least one tumor recurrence at intervals of 20 to 132 months. MIB-1 indices on the initial tumor resection ranged from 0 to 5.5 (median, 0.9) in 12 cases. In three patients with recurrent tumor, MIB-1 indices were higher in the initial tumor in two cases and lower in one. p53 Immunostaining of 13 tumors showed rare positive-staining tumor cell nuclei. The conclusions are that myxopapillary ependymomas grow slowly; that MIB-1 labeling indices are unreliable predictors of tumor recurrence; and that the lack of p53 immunostaining in most myxopapillary ependymomas in this series suggests that this gene might not play a significant role in the pathogenesis of these tumors.

Adolescent↗
PubMed1997

Subependymal giant cell astrocytoma: a clinicopathologic study with HMB45 and MIB-1 immunohistochemical analysis.

Subependymal giant cell astrocytomas (SEGAs) occur in approximately 6% of patients with tuberous sclerosis (TS) and are often considered to be a forme fruste or partial expression of this disorder. Recently, a number of other TS-associated lesions, including pulmonary and uterine lymphangiomyomatosis, renal angiomyolipoma, and cardiac rhabdomyoma, were shown to stain with the monoclonal antibody HMB45, suggesting a pathogenetic relationship among these lesions. Seven SEGAs in six patients (three men and three women; age range, 2-55 yr; mean, 24 yr) were studied. Cutaneous and other central nervous system features of TS were present in two patients. All of the SEGAs were intraventricular in location and were characterized by large, round, eosinophilic and spindle-shaped cells, with nuclear pseudoinclusions and mild nuclear pleomorphism, arranged in a vaguely perivascular pattern. All of the SEGAs were negative with HMB45 immunostaining. MIB-1 immunostaining was also performed in all of the cases, and MIB-1 indices (the percentage of MIB-1-positive nuclei) ranged from 0.1 to 3.8 (mean, 1.1). Two patients required an additional surgical procedure for residual/recurrent tumor; three patients are alive without evidence of disease 5 to 11 years after diagnosis; and one patient was lost to follow-up. SEGAs are generally slowly growing intraventricular neoplasms, as evidenced by their low MIB-1 labeling. Unlike other TS-associated lesions, SEGAs do not stain with HMB45.

Adolescent↗
PubMed1997

Gliosarcoma. A study of 13 tumors, including p53 and CD34 immunohistochemistry.

OBJECTIVE: Gliosarcoma is a biphasic neoplasm composed of a glioblastoma multiforme admixed with a sarcomatous component that is presumed to arise from a malignant transformation of the hyperplastic vasculature elements. We studied 13 cases of gliosarcoma to describe the clinical and pathologic features of this tumor. In addition, immunohistochemical analysis using p53 and CD34 was performed in an attempt to examine the histogenesis of gliosarcomas. DESIGN AND SETTING: A retrospective study of 13 patients with gliosarcoma from a tertiary care center. RESULTS: The 13 patients (7 women, 6 men) had a median age of 62 years (range, 33 to 79 years). All patients were initially treated with surgical excision followed by adjuvant radiation therapy, and two received adjuvant chemotherapy. Nine patients died of disease (median, 9 months; range, 1 to 17 months). Three patients were alive with evidence of residual tumor at most recent follow-up (median, 18 months; range, 6 to 41 months). A single patient was lost to follow-up. Histologically, all neoplasms consisted of an admixture of glioblastoma multiforme and sarcomatous elements. The sarcomatous component resembled fibrosarcoma in 12 cases and a malignant fibrous histiocytoma in one case. The sarcoma appeared to arise from hyperplastic vasculature in five tumors. CD34 staining in all five of these cases was negative. Immunostaining for p53 was observed in eight tumors (61.5%). p53 positivity was definitely present in both glial and sarcomatous components in all but three positive cases. The degree of staining was comparable between the two elements in five cases, present in only the glial component in two cases, and not able to be reliably assigned to one or the other component in one case. CONCLUSION: Gliosarcoma is an aggressive-behaving neoplasm, as previously reported. The presence of p53-positive immunostaining, similar to glioblastoma multiforme, may be indicative of a role for the p53 gene in these neoplasms. A lack of CD34 staining does not support an endothelial cell origin for gliosarcoma.

Adult↗
PubMed1997

Rare lesions of the posterior fossa with initial retrocochlear auditory and vestibular complaints.

OBJECTIVE: To catalog a series of rare lesions of the posterior fossa that appeared with unusual initial retrocochlear symptoms and signs and to make the reader more aware of these unusual lesions with a view to improving initial assessment and treatment planning. STUDY DESIGN: The study was a retrospective case review of seven patients. SETTING: Multidisciplinary team evaluation in a tertiary hospital referral center. PATIENTS: Patients with unusual lesions of the cerebellopontine angle and posterior fossa with initial retrocochlear symptoms and signs were included. INTERVENTIONS: Diagnostic and therapeutic. MAIN OUTCOME MEASURES: Hearing preservation and balance function. RESULTS: The rare lesions presented include two aneurysms of the anterior inferior cerebellar artery, one giant basilar artery aneurysm, and one each of the following neoplasms: endodermal cyst, choroid plexus papilloma, cavernous angioma, and ependymoma. CONCLUSIONS: A close working relationship among the otolaryngologist, neurotologist, neurosurgeon, and neuroradiologist is necessary to accurately evaluate these unusual cerebellopontine angle lesions and effect the best treatment outcome.

Adult↗
PubMed1997

Frozen section evaluation of stereotactic brain biopsies: diagnostic yield at the stereotactic target position in 188 cases.

OBJECTIVE: Use of the image-guided stereotactic brain biopsy has facilitated the diagnosis of previously inaccessible lesions with both safety and reliability. However, few studies have assessed the diagnostic yield of frozen section evaluation of the initial stereotactic target (FS-0). We describe our experience with 188 stereotactic brain biopsies in order to evaluate the diagnostic yield of FS-0. DESIGN: Retrospective study of 188 stereotactic brain biopsies from 185 patients. SETTING: Tertiary referral center with a high volume of neurosurgical cases including image-guided stereotactic brain biopsies. PATIENTS: One hundred eighty-five patients who underwent imaged-guided stereotactic brain biopsy over a 58-month period. RESULTS: The patients studied included 107 males and 78 females (mean age 48 years). Eleven (6%) biopsies were nondiagnostic. Diagnoses from FS-0 included a neoplastic condition in 96 (73%) of 131 cases and a nonneoplastic condition in 23 (50%) of 46 cases. In 119 (67%) of 177 cases, a diagnosis was reached at FS-0. A correct diagnosis was made on subsequent frozen section in 28 (16%) of cases, including 21 (16%) of 131 neoplasms and 7 (15%) of nonneoplastic conditions. In 15 (54%) of 28 cases, the correct diagnosis was made on the second frozen section; in 25 (89%) of 28, the correct diagnosis was made by the fourth frozen section. In 14 (11%) of 131 neoplastic cases, a sampling error relative to the lesion resulted in an inaccurate diagnosis at FS-0. A significant error in diagnosis occurred in three cases (1.7%). CONCLUSIONS: We conclude that (1) because 58 (33%) of 177 diagnosed cases in our series would have been potentially misdiagnosed if only one biopsy had been taken at the stereotactic target, frozen section evaluation or cytologic examination of material at the time of surgery should be performed routinely to ensure that adequate tissue has been obtained for purposes of diagnosis; (2) taking up to four biopsies increases the diagnostic yield (from 67% to 89% in this series); and (3) neoplastic lesions are more likely to be definitively diagnosed at FS-0 than non-neoplastic lesions.

Adolescent↗