Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P10415 (Bcl-2)
 33,771 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Two cases of solitary fibrous tumor of the spinal cord are described. The patients were two men, 36 and 47 years old. Clinically, they sought medical care because of progressive paresthesias or urinary incontinence caused by a nodular, fairly circumscribed intraspinal tumor of the cervical and thoracic spinal cord, respectively. Preoperative magnetic resonance images suggested meningioma. In both cases, laminectomy with tumor resection was carried out. Microscopically, the tumors featured short spindle cells haphazardly enmeshed in a collagenized ground substance. No atypia, necrosis, or significant mitotic activity was recognized. Tumor cells were positive for CD34, bcl2, vimentin, and, in one case, CD99. Both patients recovered from symptoms and are alive and well 12 and 18 months, respectively, after surgical resection. The differential diagnosis includes meningioma, schwannoma, and hemangiopericytoma, all of which may occur in the spinal canal. A careful morphologic approach and the judicious use of immunohistochemistry may assist in distinguishing among these conditions, although some irreducible difficulties may be posed by hemangiopericytoma. Although solitary fibrous tumor of the spinal cord presents with fairly comparable clinical, instrumental, and pathologic patterns, we believe that compilation of more cases is needed to segregate it as a distinct clinicopathologic entity.
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PMID:Solitary fibrous tumor of the spinal cord: a clinicopathologic study of two cases. 1549 32

Anaplastic meningiomas (MIIIs) and meningeal hemangiopericytomas (HPCs) display significant morphologic and immunohistochemical overlap, including occasional cases of otherwise classic HPC with focal epithelial membrane antigen (EMA) positivity. The availability of several new biomarkers prompted us to examine the potential diagnostic roles of ancillary immunohistochemistry (IHC) and fluorescence in situ hybridization (FISH) studies. From the archival university neuropathology and consult files of 1 of the authors (A.P.), 19 meningeal HPCs and 19 MIIIs were retrieved for further study. IHC was performed by using EMA, CAM 5.2, CD99, Bcl-2, claudin-1 and Factor XIIIa (FXIIIa) antibodies. FISH was performed with NF2, 4.1B (DAL-1), chromosome 1p32, and 14q32 probes. HPCs showed strong CD99 (85% of cases), strong bcl-2 (86%), focal EMA (33%), focal claudin-1 (13%), and scattered individual cell FXIIIa (100%) positivity. MIIIs showed strong EMA (89%), strong claudin-1 (54%), weak or focal CD99 (15%), weak or focal bcl-2 (31%), and individual cell FXIIIa (84%) positivity. Focal CAM 5.2 expression was seen in 26% of HPCs and 15% of MIIIs. Deletions were extremely common in MIIIs: 1p (94%), 14q (67%), NF2 (100%), and 4.1B (67%). HPCs showed no 14q or 4.1B deletions, with 1 case each of 1p and NF2 deletions (6%). The sensitivities and specificities of the 3 most useful IHC markers (EMA, CD99, bcl-2) were 85%-89% and 67%-84%, respectively. The sensitivity and specificity of claudin-1 for MIII were 54% and 86%, respectively. The specificity and positive predictive value of combined CD99 and bcl-2 expression for the diagnosis of HPC was 95%. The sensitivities of individual genetic markers were 67%-100%, with specificities of 94%-100%. Our 3 conclusions were as follows: (1) EMA, CD99, bcl-2, and claudin-1 IHC and 1p, 14q, NF2, and 4.1B FISH are particularly useful for distinguishing anaplastic meningiomas from meningeal HPCs. (2) Focal EMA expression does not preclude a diagnosis of HPC. (3) The characteristic FXIIIa staining pattern reported for HPC also is encountered frequently in anaplastic meningiomas and therefore is nonspecific in this diagnostic setting.
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PMID:Anaplastic meningioma versus meningeal hemangiopericytoma: immunohistochemical and genetic markers. 1566

Lipomatous hemangiopericytoma is a rare mesenchymal tumor showing areas of lipid-containing cells admixed with a spindle-cell component. Like other hemangiopericytomas, it shows a similar vascular pattern to solitary fibrous tumor and, partly for this reason, it and other hemangiopericytomas have been subsumed into solitary fibrous tumor. The present study provides a comprehensive documentation of a single case of pulmonary lipomatous hemangiopericytoma of the lung, the first to be described at this site, and compares it with solitary fibrous tumor, in terms of clinical, histological, immunohistochemical, ultrastructural, and cytogenetic findings. Apart from the lipid-laden-cell component, pulmonary lipomatous hemangiopericytoma and solitary fibrous tumor were similar histologically. Bcl-2 was positive in both. CD34 was minimally expressed in pulmonary lipomatous hemangiopericytoma, which possessed some non-descriptive intercellular junctions, a feature shared by solitary fibrous tumor, which was CD34 positive. However, one of the latter was rich in gap junctions, a feature consistent with strong connexin (Cx) 43 staining and the existence, hitherto unappreciated, of a CD34/Cx43-positive tumor cell network. In pulmonary lipomatous hemangiopericytoma, chromosomal deletions of 43-44, X, -Y were found. In solitary fibrous tumor, 46, XY, del(13)(q?) abnormalities and abnormalities involving chromosome 10 were frequently observed. These similarities and differences are discussed in the context of the currently favored diagnostic fusion of hemangiopericytoma and solitary fibrous tumor.
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PMID:Pulmonary lipomatous hemangiopericytoma: report of a rare tumor and comparison with solitary fibrous tumor. 1745 98

We report a case of malignant solitary fibrous tumor involving the pineal region in a 49-year-old woman. The patient presented with headache, slowly progressive weakness of the right lower extremities and upgaze palsy over the past year. Histologically, the tumor was composed of moderately hypercellular proliferated spindle cells with eosinophilic collagen bands. These cells were diffusely and strongly immunoreactive with CD34, CD99, and vimentin, but were negative with epithelial membrane antigen, S-100 protein, Bcl-2, smooth muscle actin, cytokeratin and glial fibrillary antigenic protein. MIB-1 labeling indices and mitosis rates were 7.3 +/- 1.8% and 5 per 10 high power fields, respectively. Ultrastructural examination revealed that the neoplastic cells had features of fibroblastic differentiation. Differential diagnoses included fibrous meningioma and hemangiopericytoma. The present case provides one unique example of a rare entity to the already diverse spectrum of the pineal region neoplasms encountered in neuropathology.
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PMID:Malignant solitary fibrous tumor arising from the pineal region: case study and literature review. 1984 65

Hemangiopericytomas and solitary fibrous tumors are uncommon neoplasms found in many locations, including the orbit. Both mesenchymal neoplasms share several clinicopathologic features, thus prompting intense debate as to whether they are variants of the same entity or merit separate designations in the orbit. These 2 entities, with the addition of giant cell angiofibroma of orbit, are of benign- to uncertain-behavior, CD34-positive, collagen-rich, specialized fibroblastic tumors, which may have overlapping or histologically identical features. In addition, so-called fibrous histiocytoma of orbit, a previous designation, has overlapping morphologic features with these tumors. To date, a large series of these collagen-rich fibroblastic tumors of the orbit has not been fully explored. Forty-one fibroblastic orbital tumors, originally diagnosed as hemangiopericytomas (n = 16), fibrous histiocytomas (n = 9), mixed tumors (hemangiopericytomas/fibrous histiocytoma) (n = 14), and giant cell angiofibromas of orbit (n = 2) between 1970 and 2009, were retrieved from our consultation files, the Ophthalmic Registry, at the Armed Forces Institute of Pathology. Slides and clinical records were reviewed, analyzed, and compared. Immunochemistry was performed for CD34, CD99, Bcl-2, Ki-67, and p53. Upon histologic review, all cases were reclassified as solitary fibrous tumor (41/41). The patients included 23 (56%) males, 17 (41%) females, and 1 unknown, with a mean age at presentation of 40.7 years (range, 16-70 years). The sites of involvement were the right orbit in 18 (44%) cases and the left in 16 (39%) cases. Tumors ranged in size from 0.4 to 5.0 cm (mean, 2.2 cm). Seventeen (41%) patients presented with an orbital mass, 8 (20%) with proptosis, 2 (5%) with painful mass, and 2 (5%) with painless mass. Duration of symptoms ranged from 3 to 96 months, with a mean of 23 months (median, 9 months). Microscopically, all lesions showed considerable similarity, varying in degree of cellularity, stromal collagen, and the presence of giant cells. Overlapping features with soft tissue giant cell fibroblastoma were observed. Immunochemistry revealed positivity for CD34 in all cases (100%), p53 in 85%, CD99 in 67.5%, and Bcl-2 in 47.5%. Although Ki-67 labeling was seen in all cases, it ranged from less than 1% in 54.3% of cases to 5% to 10% in 20% of cases. Taken together, the findings of this study suggest that orbital hemangiopericytoma and some cases previously designated as fibrous histiocytoma, giant cell angiofibroma of orbit, and solitary fibrous tumor have overlapping morphologic and immunohistochemical features and should be designated as solitary fibrous tumor. Adipocytes and unusual multivacuolated adipocytic cells may be present in these tumors, as well stromal myxoid change; and even stromal intramembranous ossification can be observed. There are overlapping features of orbital solitary fibrous tumor with another CD34-positive specialized fibroblastic tumor of soft tissue, giant cell fibroblastoma. Morphologic criteria for uncertain behavior to low-grade malignant ocular solitary fibrous tumors can be made by cytologic atypia and increased mitotic activity, but overall outcome for malignant solitary fibrous tumors of the eye should be further explored.
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PMID:Orbital solitary fibrous tumor: encompassing terminology for hemangiopericytoma, giant cell angiofibroma, and fibrous histiocytoma of the orbit: reappraisal of 41 cases. 2105 98

Myopericytoma is a recently described hemangiopericytoma-like neoplasm with myoid differentiation. These tumors are typically located in the subcutaneous and soft tissues of the extremities. The authors report a rare pleural-based pulmonary myopericytoma in a 58-year-old woman. The lesion was grossly homogeneous and well circumscribed. Microscopically, it was composed of densely packed spindle cells organized as whorls and short interlacing fascicles with a concentric perivascular distribution. Immunohistochemical reactions were positive for vimentin, smooth muscle actin (SMA), muscle-specific actin, and Bcl-2 and negative for desmin, h-caldesmon, cytokeratin, and CD34. Atypically, increased mitotic activity was noted, but no other malignant features were identified. The differential diagnoses are discussed with specific emphasis on solitary fibrous tumor of the pleura, which is the most common benign pleural-based spindle cell neoplasm and may be a diagnostic pitfall with potentially harmful consequences.
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PMID:Myopericytoma: a pleural-based spindle cell neoplasm off the beaten path. 2147 7

Aims. We report on the unusual case of a 43-year-old man who developed recurrent meningeal hemangiopericytoma and presented with hypoglycemia 6 years after excision of the tumor. Methods and Results. We utilized computed tomography to assure multiple tumor metastasis and cranial recurrence of previous meningeal hemangiopericytoma and clinical laboratory tests and immunohistochemical staining to characterize this case. Magnetic resonance imaging and computed tomography showed the recurrent tumor at original torcular site was increased in size. Abnormal low levels of growth hormone, insulin, and insulin-like growth factor-I except insulin-like growth factor-II were detected in the serum. By immunohistochemistry, the neoplastic cells characteristically express diffusely CD99, bcl2, and variable CD34. After radio- and chemotherapy, serum glucose level of the patient returned to normal. Conclusions. Comparing other brain tumors, meningeal hemangiopericytoma has a higher recurrent and metastatic rate, but this tumor with hypoglycemia is very rare.
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PMID:Recurrent meningeal hemangiopericytoma with multiple metastasis and hypoglycemia: a case report. 2302 57

Hemangiopericytoma (HPC) and meningioma, for their morphology immunohistochemical markers similarity, were usually confused especially before surgery. This study aimed to develop a panel of biomarkers to differentiate HPC from meningioma. Real-time PCR and immunohistochemical staining were employed to determine the levels of p53, bcl-2, c-myc, vimentin, CD34, FVIIIa, MGMT and reticular fiber in 15 meningiomas, HPCs and their normal controls. We found that, in the mRNA expression level, both Bcl-2 and c-myc were high in HPC and meningiomas, but bcl-2 was higher in HPC than in meningiomas, c-myc was lower in HPC than in meningiomas. In protein expression level, reticular fibers were around most HPC tumor cells but observed null in meningiomas; CD34 and FVIIIa were both found positive in HPCs but negative in meningiomas; MGMT was weak focal in HPC but strong diffuse in meningiomas. In conclusion, bcl-2, c-myc, and MGMT could be employed as the new panels of biomarkers for distinguishing HPC from meningiomas.
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PMID:Immunohistochemical and genetic markers to distinguish hemangiopericytoma and meningioma. 2606 18

Using whole exome and transcriptome sequencing, NAB2-STAT6 gene fusions have recently been identified in patients with solitary fibrous tumors (SFT). We report two cases of SFT in which NAB2-STAT6 fusions were identified. The patients were a 32-year-old man with a parasagittal tumor involving the superior sagittal sinus, and a 40-year-old man with a cerebellar convexity tumor partially involving the transverse sinus. Their tumors were gross totally resected and diagnosed to be SFT according to the following pathological findings:the tumors were composed of spindle cells with a patternless architecture, with prominent stromal collagen and staghorn vessels. An immunohistochemical study yielded positive results for CD34, CD99, and Bcl-2 and negative results for EMA, GFAP, and S100. The MIB-1 indexes were 13 and 7%, respectively. NAB2-STAT6 fusions were detected in both cases with a common fusion variant, NAB2ex6-STAT6ex16/17. We also identified NAB2-STAT6 fusions in two hemangiopericytomas diagnosed in the past with a common variant of NAB2ex6-STAT6ex16/17. These findings suggest that solitary fibrous tumor and hemangiopericytoma may be diagnosed based on the presence of NAB2-STAT6 fusion, and not classified separately because of the same genetic background.
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PMID:[Two Cases of Primary Intracranial Solitary Fibrous Tumor:Genetic Examination of <i>NAB2-STAT6</i> Fusion and Its Association with Hemangiopericytoma]. 2613 29

Solitary fibrous tumor/hemangiopericytoma with primary tumor location in the central nervous system accounts for less than 1% of all central nervous system tumors. Despite the relatively indolent clinical course, extracranial metastases are reported in 28% of cases. In recent years, NAB2-STAT6 gene fusion has been recognized as the pathognomonic molecular feature of solitary fibrous tumor/hemangiopericytoma and STAT6 immunohistochemistry has been shown to be a sensitive and specific surrogate for the identification of the gene fusion in these patients. Here we report two cases of patients who experienced occurrence of diffuse extracranial metastases several years after successful surgery for an intracranial solitary fibrous tumor/hemangiopericytoma. In the first patient, the metastases had maintained similar histological features to the primary tumor; in contrast, in the second case, a dedifferentiation occurred with loss of expression of CD34 and Bcl-2. These different histological features were associated with radically different behaviors. Whereas the first case experienced an indolent course of the disease, the second patient had a rapid disease progression and deterioration of clinical conditions. The molecular imaging findings in these two cases and the role of functional imaging for tumor detection, disease staging and monitoring in this rare cancer are also discussed. Recurrences and metastases maintained high expression of somatostatin receptors confirmed by somatostatin receptor imaging in the first case. In contrast, in the second patient, the abrupt transition into a highly aggressive form was associated with the absence of somatostatin receptors at 111In Pentetreotide scan and intense hypermetabolism at 18F-FDG PET.
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PMID:Metastatic intracranial solitary fibrous tumors/hemangiopericytomas: description of two cases with radically different behaviors and review of the literature. 3197 91


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