Biomedical subjects

R A Prayson

Publications and source records attributed to R A Prayson.

At least 55 records · Page 3Linked to original sources

PubMed1999

Bcl-2 and Bcl-X expression in gangliogliomas.

Bcl-2 and bcl-xL are proteins known to inhibit cell death (apoptosis). Expression of these proteins in gangliogliomas has not been extensively examined. This study retrospectively evaluates bcl-2 and bcl-x immunostaining in paraffin-embedded materials in gangliogliomas. Twenty-nine gangliogliomas in 17 males and 12 females, age 2.5 to 47 years (mean, 20.7 years), were studied. Nineteen tumors were situated primarily in the temporal lobe. All but three patients presented with seizures ranging from 3 months to 28 years' duration (mean, 11.1 years) before surgery. All tumors histologically were comprised of an atypical neuronal component and a glioma component, which most frequently resembled a low-grade astrocytoma. Cortical dysplasia was observed adjacent to eight tumors. MIB-1 (marker of cell proliferation) labeling indices (percentage of positively staining tumor cell nuclei) ranged from 0 to 7.7 (mean, 0.8). bcl-2 staining was observed in 25 tumors (86%); neuronal staining was present in 24 cases (83%), and glial cell staining in 21 tumors (72%). Bcl-xL staining was only observed in eight gangliogliomas (28%); in all eight tumors (28%), neuronal staining was seen, and focal glial cell staining was present in two cases (7%). Four tumors (14%) did not stain with either bcl-2 or bcl-xL. There appeared to be no relationship between MIB-1 immunostaining and staining with bcl-2 or bcl-xL. bcl-2 expression by immunohistochemistry was observed more frequently than bcl-xL in gangliogliomas. Expression of these proteins may reflect abnormalities of apoptosis, which could play a role in the survival of cells that may be involved in the development of gangliogliomas.

Adolescent↗

PubMed1999

Clinicopathologic study of 61 patients with ependymoma including MIB-1 immunohistochemistry.

Predicting behavior based on histologic appearance has been problematic in ependymomas. Sixty-one patients with ependymoma (excluding subependymoma and myxopapillary ependymoma) were studied. The patients included 36 men and ranged in age from 1.5 to 74 years (median, 33 years). The most common clinical presentations included headache (n = 19), weakness (n = 18), nausea/vomiting (n = 12), and gait disturbance (n = 10). Location included spinal cord (n = 24), fourth ventricle (n = 21), lateral ventricle (n = 8), and third ventricle (n = 5). Initial surgery included a gross total resection of tumor in 22 patients and subtotal resection or biopsy in the remaining patients. Thirty-five patients were known to have been treated with adjuvant radiation therapy and 13 patients received adjuvant chemotherapy. At last known follow-up, 20 patients were alive with no evidence of tumor (median, 66.5 months), 17 patients were alive with residual tumor (median, 14 months), and 12 patients died of tumor (median, 27.5 months). Two additional patients are alive with tumor status not known, two cases are current, and two patients were lost to follow-up. The additional six patients died either shortly after surgery or of surgical complications. Sixteen of 18 patients had at least one tumor recurrence at median 28.5 months. Fifty-one tumors had a predominantly glial pattern and 10 had a mixed glial-epithelial pattern. Of histologic features examined, patients with tumor recurrence or who died of tumor more frequently had observable mitotic figures, vascular proliferation, necrosis, and foci of increased cellularity. Eight of 18 recurrent tumors were classified as high grade ependymomas (anaplastic/malignant). Of patients who died of tumor, 4 of 12 had histologically high grade tumors versus 5 of 39 of the remaining tumors. MIB-1 immunostaining (marker of cell proliferation) was performed on 50 tumors. MIB-1 labeling indices (% positive tumor cell nuclei) ranged from 0.1 to 34.0 (median, 1.1). A higher percentage of patients with recurrent tumor (6 of 13, 46%) or who died of tumor (3 of 10, 30%) had MIB-1 indices >/= 4.0 versus the remaining patients (8 of 33, 24%). The conclusions are as follows: (1) histologic appearance and MIB-1 indices were not reliably predictive of tumor behavior, probably due in part to tumor heterogeneity; (2) tumors with two or more of the following features: identifiable mitotic figures, hypercellularity, vascular proliferation, and necrosis were more likely to behave in an aggressive manner; and (3) elevated MIB-1 labeling indices (>/=4.0 in this study) were encountered in a higher percentage of fatal and recurrent tumors than in nonfatal or nonrecurrent tumors.

Adolescent↗

PubMed1999

Extracerebellar primitive neuroectodermal tumors: A clinicopathologic study with bcl-2 and CD99 immunohistochemistry.

Supratentorial primitive neuroectodermal tumors (S-PNETs) are uncommon lesions that occur predominantly in children and are histologically identical to cerebellar medulloblastomas. Like their cerebellar counterparts, S-PNETs often show divergent differentiation along neuronal, glial, and mesenchymal lines. The relationship of S-PNETs to medulloblastoma and other embryonal neoplasms remains controversial, largely because the cell of origin and histogenesis of these lesions are incompletely understood. To clarify these issues, we examined eight S-PNETs with antibodies to bcl-2 (an antiapoptosis protooncogene that has been postulated to be a marker of neuronal differentiation) and CD99 (a glycoprotein present in most peripheral embryonal tumors). S-PNETs in eight patients (seven males and one female; age range, 2 months to 40 years) were studied. All lesions were composed predominantly of small round cells with deeply basophilic nuclei and minimal surrounding cytoplasm. Tumors in two patients demonstrated no evidence of differentiation, two tumors neuronal differentiation only, and four tumors both neuronal and glial differentiation. No tumors stained with CD99. Three tumors showed focal, strong cytoplasmic staining with bcl-2. The positive lesions included one tumor showing neuronal differentiation only and two tumors with both neuronal and astrocytic differentiation. Patients were treated with various combinations of radiation and chemotherapy; five patients died from their tumor a mean of 1.7 years after diagnosis, two patients were alive with residual disease at 3 months and 3 years, and one patient was alive without disease at 17 months. Our findings suggest that bcl-2 positivity may identify a subgroup of patients having inhibition of apoptosis as a pathogenetic mechanism; we were unable to show any definite relationship between bcl-2 staining and neuronal differentiation. Despite their morphologic similarity, supratentorial and peripheral embryonal tumors appear to be distinct pathogenetic lesions, as evidenced by their different staining patterns with CD99.

12E7 Antigen↗

PubMed1999

Histopathologic findings in 37 cases of functional hemispherectomy.

Hemispherectomy procedures are performed in patients for whom focal cortical resection would be predicted to produce a significant reduction in seizures. The functional hemispherectomy procedure consists of disconnecting the hemispheres while attempting, in some cases, to preserve parenchyma. This study retrospectively reviews the histopathologic findings in 37 cases of functional hemispherectomy performed between 1990 and 1998 at a major epilepsy center. Procedures were performed in 20 males and 17 females who ranged in age from 3 months to 37 years (mean age, 9.6 years). In all but two cases, more than half or all the material submitted for pathologic testing was examined histologically. Cortical dysplasias or hemimegalencephaly were identified in 14 patients. The most common patterns of dysplasia observed included architectural disorganization (n = 13), increased molecular layer neurons (n = 11), and neuronal cytomegaly (n = 11). One patient was known to have epidermal nevus syndrome. Six patients had Sturge-Weber syndrome. Remote infarct/ischemic damage was identified as the etiology of seizures in six patients; four of these patients had mild associated secondary cortical architectural abnormalities. Three patients demonstrated pathology consistent with Rasmussen's encephalitis; one additional patient had chronic encephalitis changes, not otherwise specified. In two cases, changes consistent with hippocampal sclerosis were identified; additionally, hippocampal neuronal loss and gliosis was focally identified in three patients. Most of these patients had coexistent cortical dysplasia or radiographic evidence of remote infarct. One specimen demonstrated areas of infarct following resection of an arteriovenous malformation. In two specimens, significant histopathologic findings were not identified; both of these patients had radiographic evidence of remote infarct. The spectrum of pathologic conditions that may be encountered in the setting of a functional hemispherectomy is varied and in this study most frequently included cortical dysplasia, Sturge-Weber syndrome remote infarct, and Rasmussen's encephalitis.

Adolescent↗

PubMed1999

Granulomatous myositis. Clinicopathologic study of 12 cases.

Granulomatous inflammation is infrequently encountered in skeletal muscle biopsy material. Of 2,985 muscle biopsy specimens reviewed over 12 years, 12 (0.4%) with granulomatous inflammation were identified. The patients included 9 women who ranged in age from 24 to 76 years (mean 50.3 years). The most common clinical findings included decreased strength or weakness in the extremities (n = 8), muscle pain (n = 5), and weight loss (n = 3). All muscles exhibited nonnecrotizing granulomas; an associated vasculitic process was identified in 2. Endomysial chronic inflammation consisting primarily of lymphocytes and plasma cells was present in 10 muscles, and perivascular chronic inflammation in 8. Degenerating muscle fibers were noted in 10 cases, and regenerating fibers in 11. Evidence of neurogenic atrophy was seen in 8 muscles. Increased endomysial fibrosis was observed in 5 muscles, and type II muscle fiber atrophy in 5 muscles. Stains for acid-fast bacilli and Gomori methenamine silver stain were performed in all but 2 cases and failed to demonstrate organisms. In 3 cases, concomitant sural nerve biopsies were performed, and granulomas were identified in 2 of those cases. Clinicopathologic diagnoses included sarcoidosis (n = 6), vasculitis (n = 2), and granulomatous myositis not otherwise specified (n = 2). In 2 cases, there was insufficient clinical information or follow-up data to determine a cause. In conclusion, granulomatous myositis is infrequently found in muscle biopsy specimens (0.5% of all biopsies in this series); most muscles demonstrate evidence of chronic endomysial or perivascular inflammation accompanied by muscle fiber degeneration and regeneration; and the most common cause for granulomatous myositis was sarcoidosis in this series.

Adult↗

PubMed1999

Primary pulmonary malignant meningioma.

Fewer than 20 cases of primary pulmonary meningioma have been reported. Most of these cases have been histologically and clinically benign. We report an unusual case of primary pulmonary malignant meningioma with atypical histologic features and malignant behavior. A computed tomography scan of the head did not show evidence of tumor. The right upper lobe mass was resected and showed features of an atypical meningioma with loss of architectural pattern, mild nuclear pleomorphism, increased mitotic counts (up to 15 mitotic figures per 10 high power fields), and focally prominent nucleoli. Focally, cells with rhabdoid features were identified. The tumor's immunohistochemical and ultrastructural profiles were consistent with a meningioma. The tumor stained negative for estrogen and focally positive for progesterone receptors and had a MIB-1 labeling index (marker of cell proliferation) of 9.2%. Approximately 5 months after the initial resection, the patient experienced a tumor recurrence with multiple lymph node metastases, spread to the middle and lower lobes of the right lung, and metastasis to the diaphragm. Rarely, primary pulmonary meningiomas may present as high-grade malignant lesions.

Antineoplastic Combined Chemotherapy Protocols↗

PubMed1999

Composite ganglioglioma and dysembryoplastic neuroepithelial tumor.

Both ganglioglioma and dysembryoplastic neuroepithelial tumors are well-recognized glial-neuronal neoplasms associated with chronic epilepsy and cortical dysplasia (neuronal migration abnormalities). The exact relationship between these 2 glial-neuronal tumors continues to be debated. This article reports a case of a composite ganglioglioma and dysembryoplastic neuroepithelial tumor occurring in a 36-year-old woman in the left temporal lobe region. The resection histologically demonstrated distinct areas of ganglioglioma and dysembryoplastic neuroepithelial tumor. A focal area of cortical dysplasia is also identified. The MIB-1 labeling indexes in both components were low (<1% of tumor cell nuclei). The coexistence of these 2 lesions and cortical dysplasia suggest a possible etiologic relationship between these 2 tumors.

Adult↗

PubMed1999

Linear epidermal nevus and nevus sebaceus syndromes: a clinicopathologic study of 3 patients.

BACKGROUND: Linear epidermal nevus syndrome and linear sebaceus nevus syndrome are rare neurocutaneous syndromes characterized by epidermal nevi, epilepsy, and mental retardation. Pathologic descriptions of the central nervous system findings in such patients are rare. DESIGN: We examined the clinicopathologic features of 2 patients with linear epidermal nevus syndrome and 1 with nevus sebaceus syndrome who underwent surgical resections for chronic epilepsy in a tertiary referral center with a high volume of epilepsy surgery. RESULTS: Patients included 3 females, aged 11 months (patient 1), 8 years (patient 2), and 2 1/2 years (patient 3) at the time of surgery. The duration of seizures prior to surgery was 11 months, 6 years, and 28 months, respectively. Two patients had epidermal nevi involving the head region (patients 1 and 3), and 1 had a nevus sebaceus of Jadassohn (patient 2); patient 2 had a choristoma, and patient 3 had a dermoid cyst in the eye region. Patient 1 demonstrated hemimegalencephaly radiographically. Histologic examination of resected cortical tissue in patients 1 and 2 demonstrated severe diffuse cortical dysplasia characterized by a disorganized cortical architectural pattern, a haphazard orientation of cortical neurons, and increased molecular layer neurons. Gyral fusion was seen in patient 1. Pial glioneuronal hamartomas were observed in patient 1. Prominent cortical astrocytosis was seen in patients 1 and 2, and foci of microcalcification were evident in patient 1. Cortical dysplasia was milder in patient 3 and consisted of an increased number of molecular layer neurons. Neuronal heterotopia was observed in all 3 patients. CONCLUSION: The spectrum of neuronal migration abnormalities in the setting of these syndromes may be variable in terms of its histologic phenotypic manifestations.

Child↗

PubMed1999

Subependymomas: clinicopathologic study of 14 tumors, including comparative MIB-1 immunohistochemical analysis with other ependymal neoplasms.

BACKGROUND: Subependymomas are uncommonly encountered ependymal tumors, which are important to distinguish from ordinary ependymomas because of their generally better prognosis. OBJECTIVE: To review the clinicopathologic features and MIB-1 labeling indices (marker of cell proliferation) of 14 subependymomas. DESIGN: Retrospective review of 14 subependymomas encountered in a tertiary care setting. RESULTS: Fourteen ependymomas presenting in 8 men and 6 women between the ages of 18 and 78 years (mean, 53.6 years) comprise the study group. The most common clinical presentations included ataxia (n = 4), dizziness/vertigo (n = 3), nausea/vomiting (n = 3), headaches (n = 3), and incidental finding at autopsy (n = 2). Tumor locations included fourth ventricle (n = 7), lateral ventricle (n = 4), third ventricle (n = 2), and thoracic spinal cord (n = 1). Eight patients underwent gross total resection, and 4 had subtotal resection. Tumors were characterized by clustering of cell nuclei arranged against a fibrillary background. Focal cystic degeneration was seen in 10 tumors, hemosiderin deposition in 8 tumors, sclerotic vessels in 8 tumors, calcifications in 5 tumors, and focal nuclear pleomorphism in 2 tumors. Mitotic figures, vascular endothelial proliferation, and necrosis were not seen in any of these tumors. Cell proliferation marker MIB-1 labeling indices (percentage of positive staining tumor cells) ranged from 0 to 1.4 (mean, 0.3). In comparison, 13 myxopapillary ependymomas had labeling indices ranging from 0 to 5.5 (mean, 1.1). Thirty-nine low-grade ependymomas had MIB-1 labeling indices of 0.1 to 5.4 (mean, 1.1). Fourteen anaplastic/malignant ependymomas had MIB-1 labeling indices ranging from 0.4 to 34.0 (mean, 12.8). One subependymoma was treated with radiation therapy. Six patients were alive with no evidence of tumor at a mean follow-up of 94.4 months. Two patients were alive with residual tumor (follow-up of 4 and 53 months). Two patients died with tumor at 0.67 and 43.4 months. One patient was lost to follow-up, 1 is a recent case, and 2 were incidental findings at autopsy. None of the patients developed tumor recurrence. CONCLUSIONS: Subependymomas are generally low-grade lesions, as evidenced by their benign clinical course and low MIB-1 labeling indices. Compared with other ependymal tumors, subependymomas have the lowest rate of cell proliferation as evidenced by MIB-1 immunostaining.

Adult↗

PubMed1999

Histopathologic features and MIB-1 labeling indices in recurrent and nonrecurrent meningiomas.

BACKGROUND: Predicting the behavior of meningiomas based on histopathologic features alone has remained problematic. DESIGN: This study retrospectively compared several histopathologic features and MIB-1 labeling indices (LIs) in recurrent meningiomas with those of nonrecurrent meningiomas. Six histopathologic features, including mitoses, necrosis, loss of architectural pattern, hypervascularity/hemosiderin deposition, prominent nucleoli, and nuclear pleomorphism, were compared between 32 recurrent and 27 nonrecurrent meningiomas using Fisher exact tests. MIB-1 LIs (% positive tumor cell nuclei) were compared using the Wilcoxon rank sum test. RESULTS: The patients in the recurrent group included 26 women (mean age, 55 years), who developed 1 to 5 recurrences. Time intervals to the first recurrence ranged from 5 to 183 months (mean, 55 months). The nonrecurrent group included 21 women (mean age, 56 years), with follow-up ranging from 88 to 124 months (mean, 109 months). Of the histopathologic features evaluated, statistically significant differences between the recurrent and nonrecurrent groups were found only with respect to prominent nucleoli (P =.024) and nuclear pleomorphism (P <.001), both of which were more common in the recurrent group. In the recurrent group, 9 tumors were considered malignant (defined by brain invasion or metastasis) versus 2 of the nonrecurrent meningiomas. Nineteen percent of nonrecurrent tumors versus 41% of recurrent tumors had 2 or more of the 6 histopathologic features. MIB-1 LIs in the nonrecurrent group ranged from 0 to 8.3 (mean, 1.5) and were generally lower than those in the recurrent group (range, 0-32.5; mean, 5.4); no statistical difference was identified between these groups. No statistically significant difference with regard to histology or MIB-1 LIs was noted between the initially excised recurrent tumor and the most recently resected recurrence. CONCLUSIONS: Of the histopathologic features examined, only prominent nucleoli and nuclear pleomorphism were found to be statistically more common in recurrent than nonrecurrent meningiomas. The mean MIB-1 LI was higher in the recurrent than in the nonrecurrent group, although there was no statistical difference between means and there was clear overlap with regard to MIB-1 LI ranges.

Adult↗

PubMed1999

Evaluation of epithelial and keratin markers in glioblastoma multiforme: an immunohistochemical study.

OBJECTIVE: Poorly differentiated metastatic carcinoma may be difficult to distinguish histologically from high-grade astrocytic malignant neoplasms, particularly on small open or stereotactic biopsy specimens. Previous authors have reported that a subset of glioblastoma multiforme (GBM) variably stains with cytokeratin immunomarkers. The authors examined a panel of epithelial and keratin antibodies by paraffin immunohistochemistry to evaluate the immunophenotype of GBM for these markers and to determine what combination of immunostains would be optimal in distinguishing GBM from metastatic carcinoma. METHODS: Twenty-three patients with GBM (age range, 19-86 years; mean, 63.4 years; 14 men and 9 women) and 22 patients with metastatic carcinoma (age range, 26-77 years; mean, 58.1 years; 7 men and 15 women) to the brain were studied with a panel of immunostains, including glial fibrillary acid protein (GFAP), Ber-EP4, antikeratin monoclonal antibodies AE1/3, and antibodies to CAM 5.2 and cytokeratins 7 (CK7) and 20 (CK20). Sites of origin for the metastatic tumors included lung (n = 11), breast (n = 5), endometrium (n = 1), prostate (n = 1), colon (n = 1), presumed kidney (n = 1), and unknown (n = 2). RESULTS: All GBMs stained positive for GFAP (100%), and all but 1 (95.7%) stained positive for cytokeratins AE1/3. Only rare focal immunoreactivity was observed in a single case of GBM with CAM 5.2 (4.3%), CK7 (4.3%), and CK20 (4.3%). Immunoreactivity with Ber-EP4 was not observed in any of the GBMs (0.0%). All cases of metastatic carcinoma stained positive with cytokeratins AE1/3 (100%) and CAM 5.2 (100%). Variable staining was observed in carcinomas with CK7 (17 of 22, 77.3%), Ber-EP4 (11 of 22, 50.0%), and CK20 (9 of 22, 40.9%). Three metastatic carcinomas showed rare GFAP-positive staining cells (13.6%). CONCLUSIONS: Based on the aforementioned results, a combination of immunostains, including GFAP and cytokeratin CAM5.2, may be the most useful in differentiating poorly differentiated metastatic carcinoma from GBM. A significant number of GBMs stain with some cytokeratin markers, in particular cytokeratins AE1/3. Because of the poor specificity of cytokeratins AE1/3 in distinguishing metastatic carcinoma from GBM, it should not be used to differentiate the 2 entities.

Adult↗

PubMed1998

MIB-1 labeling indices in benign, aggressive, and malignant meningiomas: a study of 90 tumors.

Predicting tumor behavior in meningiomas based on histology alone has been problematic. This study retrospectively compares histology and MIB-1 (cell proliferation marker) labeling indices (LI) in benign, aggressive, and malignant meningiomas. Six histological features, including mitoses, necrosis, loss of pattern, hypervascularity/hemosiderin deposition, prominent nucleoli, and nuclear pleomorphism, were compared in 90 meningiomas (Fisher's exact test). Tumors with two or more of the above features were designated as aggressive meningiomas. Malignant meningiomas were characterized by brain invasion or metastasis. The MIB-1 LIs (% positive tumor cell nuclei) were compared between the three groups (Kruskal-Wallis test, Wilcoxon two-sample test). Of the benign meningiomas (n=37; mean age, 54 years), 41% had one of the six histological features, with nuclear pleomorphism (n=10) being the most frequent. The aggressive tumors (n=29; mean age, 61 years) were characterized by nuclear pleomorphism (n=28), mitoses (n=20), necrosis (n=16), loss of pattern (n=16), prominent nucleoli (n=6), and hypervascularity/hemosiderin deposition (n=5). Malignant tumors (n=24; mean age, 59 years) were characterized by nuclear pleomorphism (n=22), mitoses (n=21), loss of pattern (n=21), necrosis (n=21), nucleoli (n=17), and hypervascularity/hemosiderin deposition (n=3). Significant differences were found between the aggressive and malignant groups with regard to loss of pattern, necrosis, and nucleoli (P=.0043, .011, and .00029, respectively). Mean MIB-1 LIs for the benign, aggressive, and malignant groups were 1.0% (range, 0 to 5.5%),5.5% (range, 0.1 to 32.5%), and 12.0% (range, 0.3 to 32.5%), respectively. Differences in the mean MIB-1 LI between groups were statistically significant, with P values of <.0001 (benign v aggressive) and .0012 (aggressive v malignant). Mean MIB-1 LIs for recurrent versus nonrecurrent tumors were 7.1% (range, 0 to 32.5%) versus 3.8% (range, 0 to 20.9%) (P=.32). The mean MIB-1 LI for patients who were alive with or without tumor was 6.2% (range, 0 to 32.5%) versus a mean MIB-1 LI of 14.2% (range, 2.8% to 32.5%) for patients who died of or with tumor (P=.0013). In conclusion, (1) There is a statistically significant difference in the increasing MIB-1 LI means between benign, aggressive, and malignant meningiomas and between patients who were alive versus those who died; (2) there is some overlap in MIB-1 LI ranges between groups, which warrants caution in interpreting an individual MIB-1 LI in a given tumor.

Adolescent↗

PubMed1998

Cyclin D1 and MIB-1 immunohistochemistry in pilocytic astrocytomas: a study of 48 cases.

Pilocytic astrocytoma is an infrequently encountered, generally low-grade neoplasm. No study has extensively looked at both cyclin D1 and MIB-1 labeling indices in pilocytic astrocytoma and their relation to clinical outcome. This study retrospectively examines the clinicopathologic features of 48 patients with pilocytic astrocytoma including MIB-1 (cell proliferation marker) and cyclin D1 (protein that regulates progression from G1 to S phase of the cell cycle) immunohistochemistry. Of 48 patients (27 females and 21 males; mean age, 12.7 years; age range, 2 to 57 years), 26 initially underwent gross total resection; 17, subtotal resection; four, biopsy alone; in one patient, the extent of tumor resection was unknown. Histological features observed included Rosenthal fibers (83.3%), granular bodies (75%), vascular sclerosis (56.2%), vascular proliferation (56.2%), prominent nuclear pleomorphism (14.6%), necrosis (10.4%), and identifiable mitotic figures (2.1%). MIB-1 labeling indices (n=45) (positive staining tumor nuclei per 1,000 nuclei evaluated) ranged from 0 to 3.5% (mean, 0.6%); seven tumors had a labeling index greater than 1.0%. Cyclin D1 labeling indices (n=45) ranged from 0 to 0.8% (mean, 0.1%). Most tumors (N=29, 66.7%) had no immunostaining. At last known follow-up, 27 patients were alive with no evidence of disease (mean, 49.2 months), 17 patients were alive with evidence of disease (mean, 36.8 months), three died with tumor at 2, 22, and 156 months, and one patient was lost to follow-up. Eight patients had at least one tumor recurrence requiring additional surgery; seven of these patients had an initial subtotal resection. In summary, MIB-1 labeling indices were generally low (mean, 0.6%) and are reflective of the slow growth of the tumors. Cyclin D1 immunostaining does not appear to be significantly increased in pilocytic astrocytoma. Adverse outcome in patients with pilocytic astrocytoma may be related to extent of surgical resection and does not seem to correlate with histology, MIB-1 labeling indices, or cyclin D1 immunoreactivity.

Adolescent↗

PubMed1998

Amyloid myopathy: clinicopathologic study of 16 cases.

Amyloid deposition in skeletal muscle is a well-recognized but rare occurrence. Sixteen such cases seen in a 17-year period (1979 to 1996) out of a total of 3,937 muscle biopsy specimens (0.004%) form this study group. Either Congo red or sulfated alcian blue stains were routinely performed in each biopsy to screen for amyloid. Patients in this study (eight men, eight women) ranged in age from 42 to 90 years (mean, 61 years) at initial presentation. The most common symptoms at presentation included weakness/fatigue (n = 10), autonomic symptoms (n = 8), and weight loss/decreased appetite (n = 7). Five patients had a concomitant malignancy (myeloma, n = 3; malignant carcinoid tumor, n = 1; melanoma, n = 1). Two patients had known hereditary forms of amyloidosis. Five patients had amyloid diagnosed on another organ biopsy (excluding peripheral nerve). Histologically, amyloid was deposited in the interstitium or perivascular region in 14 muscles and endomysial region in seven muscles. All cases were confirmed with Congo red staining (apple green birefringence) or by electron microscopic identification of fibrillary amyloid material. Scattered angular atrophic esterase-positive muscle myofibers indicative of acute denervation atrophy were seen in 14 muscles. Eight muscles showed small group atrophy, and seven showed myofiber type grouping. Scattered regenerating muscle fibers were seen in nine cases, degenerating myofibers in six, and foci of chronic endomysial and perivascular inflammation in two. Four muscles showed type II muscle fiber atrophy. A concomitant sural nerve biopsy specimen was evaluated in seven patients; all seven contained amyloid, confirmed either by Congo red staining or electron microscopic examination. In two nerves, there was a mild loss of myelinated axons; four had a moderate loss, and one, severe loss. Six of seven nerves showed predominantly axonopathic changes. In conclusion, (1) the prevalence rate of amyloid myopathy in muscle biopsy specimens was low (in this series, 0.004%); (2) only a minority of patients had multiple myeloma, and most presented with muscle weakness/fatigue or autonomic symptoms; (3) most of the muscles showed neurogenic features histologically; (4) all concomitant sural nerve biopsy specimens contained amyloid, and most showed a predominance of axonopathic changes.

Adult↗

PubMed1998

Cryptococcal myositis: a case report and review of the literature.

Only rare cases of cryptococcal myositis have been previously reported in the literature. All of these cases have occurred in the setting of human immunodeficiency virus (HIV) infection. We report a case of cryptococcal myositis diagnosed premortem on a needle biopsy in a heart transplant patient undergoing immunosuppressive therapy.

Aged↗

PubMed1998

Cyclin D1 and MIB-1 immunohistochemistry in ependymomas: a study of 41 cases.

The molecular genetic events involved in the development of ependymal neoplasms are not well understood. This study retrospectively examines 41 ependymomas; in all tumors the cyclin D1 labeling index (LI) (percent of positive immunostaining tumor cells) was correlated with MIB-1 LI and outcome. The study included 41 patients (25 males and 16 females) ranging in age from 1.5 to 70 years (mean, 30.7 years). Twenty-five patients underwent a subtotal resection or biopsy, and 16 underwent a gross total resection. Thirty-two patents had an ordinary or tanycytic ependymoma, and 9 had anaplastic/malignant tumors. The cyclin D1 LI ranged from 0 to 8.2 (mean, 0.7); 21 tumors had an LI of 0, and 8 had an LI of >1.0. The MIB-1 LI ranged from 0.1 to 34 (mean, 4.6); 16 tumors had an LI >2.0. All 8 patients with a cyclin D1 LI of >1.0 had an MIB-1 LI of >2.0. The tumors in the 6 of 8 patients with a cyclin D1 LI of >1.0 were classified as anaplastic/malignant tumors. The findings at the most recent follow-up were as follows: alive with no evidence of tumor (n = 11; mean, 64.1 months); died with evidence of tumor (n = 11; mean, 45.9 months); alive with tumor (n = 10; mean, 39.0 months). Two patients were alive with evidence of residual disease at follow-up intervals of 7 and 16 months but were lost to further follow-up. The remaining 6 patients either were lost to follow-up or else died in the immediate postoperative period. Cyclin D1 and MIB-1 LI did not reliably correlate with clinical outcome or recurrence. All tumors with an elevated cyclin D1 LI also had an elevated MIB-1 LI; however, the converse was not true. An elevated cyclin D1 LI (>1.0 in this study) appeared to be associated with anaplastic/malignant histology; however, cyclin D1 LI along with MIB-1 LI and histology did not always reliably correlate with clinical outcome.

Adolescent↗

PubMed1998

Histopathological correlates of epileptogenicity as expressed by electrocorticographic spiking and seizure frequency.

PURPOSE: To study the correlation between histopathology and epileptogenicity, as measured by seizure frequency and electrocorticography (EcoG), in patients with cortical dysplasia (CD) as compared with control patients with gangliogliomas or gliomas. METHODS: The influence of the histopathological classification and the presence of balloon cells in CD on the frequency and extension of five predefined patterns of ECoG spiking, seizure frequency, age of seizure onset and 6-month postoperative outcome were analyzed in 32 patients with focal epilepsy undergoing presurgical evaluation with chronically implanted subdural electrodes. RESULTS: Comparison of patients with CD, gangliogliomas, and gliomas showed that the seizure frequency was greatest in patients with CD and ECoG spiking and was most extensive in patients with gangliogliomas. The onset of epilepsy was earlier in patients with CD and with gangliogliomas. None of these differences was significant. However, in patients with CD, the presence of balloon cells was associated with significantly greater seizure frequency (p=0.009), and a significantly greater number of electrodes recording continuous frequent spiking (p=0.03). The presence of continuous very frequent spiking correlated with the duration of the epilepsy and the number of seizures recorded during monitoring. No significant correlation was detected between histopathology, seizure frequency, or ECoG activity and postoperative outcome, which was relatively favorable in patients with balloon cells. CONCLUSIONS: CD refers to a variety of histopathological patterns associated with different epileptogenicity. In CD, increased clinical and ECoG epileptogenicity correlates with the presence of balloon cells. This finding confirms that balloon cells should be considered in the histopathological classification of CD. The predefined ECoG were not specific for any of the histopathologies investigated.

Adolescent↗

PubMed1998

Ganglioglioma and intractable epilepsy: clinical and neurophysiologic features and predictors of outcome after surgery.

PURPOSE: To review the clinical, neurophysiologic, and radiological data of patients with ganglioglioma who had undergone evaluation and surgery in our Epilepsy Program. METHODS: The medical and neurophysiologic records of 38 patients with intractable epilepsy and ganglioglioma were reviewed. Data underwent statistical analysis. RESULTS: There were 28 temporal and 10 extratemporal resections, with a mean age at seizure onset of 10.5 years and mean age at surgery of 22 years. Five tumor resections performed earlier were recorded. Twenty-nine patients had auras and 20 had secondarily generalized seizures. All 28 patients with temporal tumor had complex partial seizures. Preoperative MRI demonstrated the tumor in 36 of 36 patients: 17 of 29 demonstrated gadolinium enhancement, and 17 of 36 had mass effect. Scalp interictal sharp waves were present in 32 patients, and in 15 they were multiregional. In two patients, scalp EEG seizure onset was from the hemisphere contralateral to the tumor. Postoperatively, 79% of patients (30 of 38) were seizure-free (Engel's class I) at 6 months, 72% at 1 year (26 of 36), and 63% at 2 years (20 of 32). Excellent outcome was associated with a lower age at operation (p = 0.008), shorter duration of epilepsy (p = <0.01), absence of generalized seizures (p = <0.01), and no epileptiform discharges on a postoperative EEG (p = 0.01). CONCLUSIONS: Good surgical outcome is expected in patients with ganglioglioma despite years of medically resistant seizures. Good outcome may be achieved despite EEG findings that may conflict with tumor location, and is more likely when surgery is performed relatively soon after epilepsy onset.

Adult↗