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J M Bilbao

Publications and source records attributed to J M Bilbao.

At least 37 records · Page 2Linked to original sources

Postirradiated and nonirradiated gliosarcoma: immunophenotypical profile.

BACKGROUND: Thirty-one gliosarcomas (25 nonirradiated and 6 postirradiated tumors) were selected based on the presence of two distinctive areas: a malignant gliomatous and a sarcomatous component. In all cases, the sarcomatous component appears like fibrosarcoma or malignant fibrous histiocytoma. Two tumors showed additional areas consisting of osteochondroid differentiation. METHODS: All tumors were examined using antibodies against Ulex europaeus agglutinin I (UEA), glial fibrillary acidic protein (GFAP), vimentin (VM), epithelial membrane antigen (EMA), desmin, collagen IV, alpha-1-antitrypsin (alpha-1-AT) and smooth muscle actin (SMA). RESULTS: While the VM highlighted the sarcomatous areas of all tumors there were only scattered spindle cells that were GFAP-positive in the same areas. The alpha-1-AT was diffusely reactive in the sarcomatous areas in 20 cases. Focal immunoreactivity was seen with SMA (20 tumors), UEA (8 tumors), EMA (5 tumors), collagen IV (5 tumors) and desmin (4 tumors) in the nonvascular sarcomatous cells. CONCLUSIONS: The range of immunophenotypical expression is likely to be a reflection of the capacity of a multipotential progenitor to undergo divergent differentiation. There is a very little morphological difference between the postirradiated and nonirradiated tumors except that a higher proportion of postirradiated tumors are immunoreactive to SMA and desmin.

Brain Neoplasms↗

Paraneoplastic limbic encephalitis in Hodgkin's disease.

BACKGROUND: Oat cell carcinoma of the lung is the most common cause of paraneoplastic limbic encephalitis. Association with other malignancies, in particular Hodgkin's disease, is very rare. CASE REPORT: This 23-year-old male presented with a six month history of progressive alteration in mental status, which consisted of insomnia, short-term memory loss, depression and cognitive impairment. Gadolinium MRI of the head showed intense bilateral contrast enhancement affecting the medial aspects of the temporal lobes in the region of the amygdala and hippocampus. The brain biopsy showed minimal neuronal loss with intense perivascular lymphocytic cuffing and microglial nodules. Polymerase chain reaction for herpes simplex and cytomegalovirus were negative. With prednisone treatment, the patient's neurologic status stabilized but did not improve. Four months later, he presented with left axillary lymphadenopathy. Lymph node biopsy was diagnostic of Hodgkin's disease. During the chemotherapy, his lymphadenopathy subsided and his neurologic and mental status improved. When seen last after completion of his chemotherapy, one year after presentation, he had resumed normal social activities and was enrolled in a university language course. CONCLUSION: This is the first reported case in the English literature of a biopsy proven paraneoplastic limbic encephalitis associated with Hodgkin's disease. Hodgkin's disease should be thought of as a possible cause of paraneoplastic limbic encephalitis in the appropriate clinical setting.

Adult↗

Clinicopathologic features of primary and postirradiation cerebral gliosarcoma.

BACKGROUND: Gliosarcoma is an uncommon malignant brain tumor with mixed glial and mesenchymal elements. Experience is limited to case series, and pathologic data are disparate, leading to uncertainty about clinical features, management, and histogenesis. METHODS: A clinicopathologic review of 32 patients with survival analysis and immunohistochemical studies was performed including glial fibrillary acidic protein analysis, alpha-1-antitrypsin (alpha-1-AT) analysis, and smooth muscle actin (SMA) analysis. RESULTS: Twenty-five patients had primary gliosarcoma, whereas 7 developed gliosarcoma after irradiation for glioblastoma multiforme (GBM). Clinical features were similar to those of GBM. Most tumors were intraaxial and diffusely infiltrating by radiologic studies and at surgery. Median survival for primary gliosarcoma was 25 weeks overall, with patients who received irradiation surviving longer (46 vs. 13 weeks, P < 0.025). Gliosarcoma occurring after irradiation appeared hyperdense by computed tomography in five of seven cases, and median survival was 53 weeks. Primary gliosarcoma was a dimorphic tumor with malignant glial elements and features of malignant fibrous histiocytoma (MFH) or fibrosarcoma and one osteosarcoma. Smooth muscle actin labeled tumor vessels heavily, but in 15/25 primary cases, it extended to the surrounding spindle cells. The remaining cases appeared morphologically like MFH and tended to be positive for alpha-1-AT. Postirradiation gliosarcoma was fibrosarcomatous with positive SMA in 75% of the cases examined. CONCLUSIONS: Gliosarcoma behaves clinically like GBM, and survival may be improved by cranial irradiation of selected patients. Smooth muscle actin reactivity in sarcomatous areas suggests histogenesis in some tumors from the smooth muscle within GBM, whereas others may arise via different mechanisms including differentiation from a pluripotential precursor. Transformation of the smooth muscle within GBM may have therapeutic implications for antiangiogenesis agents.

Actins↗

"Unexplained" delayed death from fungal meningitis after meningioma resection.

BACKGROUND: Progressive deterioration and ensuing death following a neurosurgical procedure often represents a diagnostic challenge to the team responsible for patient care. Many, but not all, causes are treatable if a diagnosis is made early. METHODS: A 69-year-old woman who died 6 weeks post-operatively following a meningioma resection is reported. An initial routine post-operative course became complicated by progressive neurological deterioration 3-4 weeks later. Despite extensive investigation she died 6 weeks post-operatively without a diagnosis. RESULTS: Autopsy demonstrated extensive Candida meningitis. A review of the literature demonstrates this to be a reported complication in high risk patients, difficult to diagnose, but treatable when identified. CONCLUSIONS: Fungal meningitis should be high in the differential diagnosis in the post-operative patient with delayed, unexplained neurological deterioration, especially when associated with negative CSF cultures.

Aged↗

Cerebral and cerebellar gangliocytomas: a morphological study of nine cases.

Hypothalamic gangliocytomas have been shown to contain immunoreactivity for hypophysiotropic peptides and some have been associated with endocrine dysfunction. Extrahypothalamic gangliocytomas are usually not associated with endocrine abnormalities. We studied nine cerebral or cerebellar gangliocytomas from six men and three women; none of the patients had detectable alterations of endocrine homeostasis. On histological examination, the tumor cells resembled hypothalamic neurons. Electron microscopy disclosed the presence of dense-core vesicles in neuronal cytoplasm and processes resembling Herring bodies, and there were synaptic contacts between tumor cells. All but two tumors contained immunocytochemical positivity for at least one peptide hormone or amine; these included somatostatin, corticotropin-releasing hormone, beta-endorphin, galanin, vasoactive intestinal peptide, calcitonin, serotonin, catecholamines or met-enkephalin. These tumors have been thought to represent neoplasms arising in ectopic autonomic neural tissue. Their morphological features, their similarity to hypothalamic gangliocytomas and the multiple immunoreactivities shown here suggest that they can be regarded as tumors of peptidergic neurons that are widely distributed throughout the central nervous system.

Adolescent↗

Nevus of Ota.

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Adult↗

Metastatic alveolar soft part sarcoma presenting as a dural-based cerebral mass.

Sarcoma metastatic to the brain is uncommon and rarely occurs as the initial manifestation of tumor. Alveolar soft-part sarcoma, a rare but well studied subtype of a soft tissue sarcoma with a propensity for central nervous system invasion, presenting with brain metastases, has been reported only once previously. We report the case of a 28-year-old man who presented with partial seizures and who was found to have a homogeneously enhancing frontal lesion on a broad dural base disclosed by computed tomography. preoperatively, the lesion was thought to be a meningioma. The tumor was excised easily and had features typical of an alveolar soft-part sarcoma, which were revealed by light and electron microscopy as well as immunohistochemical analysis. Multiple lung nodules compatible with metastases were found on a chest film. Meningeal dissemination has been reported in a variety of sarcoma types, including rhabdomyosarcoma, fibrosarcoma, and leiomyosarcoma. We add alveolar soft-part sarcoma to this list and suggest that increased recognition of the propensity for these tumors to exhibit metastatic spread to the dura should eliminate diagnostic confusion and provide an earlier diagnosis of these rare lesions. The patterns of spread in metastatic sarcoma deserve further study.

Adult↗

The significance of spinal cord compression as the initial manifestation of lymphoma.

Eighteen patients with spinal cord compression caused by previously undiagnosed lymphoma were treated at our institution between 1976 and 1991. There were 14 male and 4 female patients (mean age, 58.2 years). The absence of bony involvement on radiographic images was a feature in 16 of the cases. All patients underwent laminectomy for decompression and tissue diagnosis, after which 5 underwent radiotherapy, 3 underwent chemotherapy, and 10 underwent combined-modality treatment. The functional outcome was improvement in 8 patients and no change in 10; no patient worsened after surgery. Eleven had advanced disease at diagnosis, while seven had limited disease, including three patients with localized extradural lymphoma. There were 16 cases of non-Hodgkin's lymphoma and 2 of Hodgkin's disease. Two patients had T-cell lymphoma and were among the longest survivors. DNA flow cytometry identified the low-grade tumors as diploid with very low proliferative indices, while the high-grade tumors all had high indices. At a mean observation time of 41.7 months, five patients have died of their disease, and seven remain in complete remission. Survival is markedly better than that reported for other malignant extradural tumors; however, even limited stage lymphoma can behave aggressively. Similarities in age, sex distribution, histological features, and the results of flow cytometry suggest behavior similar to extranodal lymphoma at other sites. Surgery to provide a tissue diagnosis, followed by combined radiotherapy and chemotherapy, is indicated for all cases.

Adult↗

Dural cavernous hemangioma: an under-recognized lesion mimicking meningioma.

We report a 77-year-old woman who presented with partial seizures and was found to have an enhancing dural-based parietal convexity mass. The lesion enlarged on serial examination by computed tomography (CT) over a one year period. The clinical features and radiologic appearance were compatible with a pre-operative diagnosis of meningioma; however, pathologic findings were typical of a dural cavernous hemangioma. Accumulating evidence suggests that these lesions are an uncommon but distinct type of vascular malformation most often arising from the cavernous sinus, tentorium, or cerebello-pontine angle. With CT, magnetic resonance imaging and angiography, these lesions can closely resemble meningioma in terms of signal characteristics, enhancement pattern, and location. This is of importance both in the practical management of meningiomas where the diagnosis is often based on radiologic studies alone, and in clinical trials where incorrect entry diagnosis should be avoided.

Aged↗

Ubiquitin expression in inclusion body myositis. An immunohistochemical study.

Ubiquitin has been shown by immunohistochemical studies to be a component of many of the filamentous inclusion bodies that are known in neuropathology. In the current study, we examined the expression of ubiquitin in 14 cases of typical inclusion body myositis, in skeletal muscle specimens from four cases of typical amyotrophic lateral sclerosis, and in muscle specimens from three normal controls. In the cases of inclusion body myositis, rimmed vacuoles were ubiquitin immunoreactive in all cases. Intrasarcoplasmic inclusions were positive in the nine cases that had them. In four cases, there were positive intranuclear inclusions, and in seven, there was homogeneous staining of nuclei. Atrophic fibers and necrotic fibers were positive in 11 and nine cases, respectively. In the cases of amyotrophic lateral sclerosis, atrophic fibers were positive in three cases, and focal nuclear staining was seen in two. In one of the three control cases, a few atrophic fibers had faint sarcoplasmic positivity; no other staining was seen. We conclude that ubiquitin is a component of the inclusions that characterize inclusion body myositis. However, ubiquitin expression in skeletal muscle disease is not pathognomonic of inclusion body myositis.

Adult↗

Fatal basilar vasculopathy complicating bacterial meningitis.

BACKGROUND: Bacterial meningitis complicated by thrombosis, vasculitis, and aneurysm formation affecting both small and distal branches of cerebral vessels has been well described. Involvement of major cerebral vessels is rare and has only been documented late in the course of disease. CASE DESCRIPTION: We describe the clinical and pathological findings in a young man who presented with pontine infarction as an early manifestation of bacterial meningitis. Streptococcus milleri, an unusual organism in this setting, was cultured. Despite improvement with antibiotic therapy, the patient experienced fatal subarachnoid hemorrhage. A ruptured inflammatory aneurysm of the basilar artery, evidence of residual meningitis and vasculitis, and basilar thrombosis associated with pontine infarction were found. CONCLUSIONS: Bacterial meningitis, which may be associated with severe vasculopathy of the basilar artery and lead to cerebral infarction, aneurysm formation, and subarachnoid hemorrhage, should be considered in the differential diagnosis of these conditions. The role of S. milleri in meningitis and its vascular complications merits further study.

Adult↗

Arteriovenous malformation of the pyriformis muscle manifesting as a sciatic nerve tumor.

We present a patient with an intramuscular pelvic arteriovenous malformation that was initially diagnosed as a tumor of the sciatic nerve sheath. Endovascular embolization, attempted as a preoperative maneuver, was complicated by diffuse pulmonary emboli and death. To our knowledge, this is the first such reported case. Its presentation here broadens the differential diagnosis of sciatic nerve sheath tumors, emphasizes the diagnostic and management issues of masses presenting as peripheral nerve tumors, and provides clinicopathological evidence regarding the cause of neurological symptoms.

Angiography↗

Adult peripheral neuroepithelioma in Meckel's cave.

A case of peripheral neuroepithelioma arising from the trigeminal nerve in Meckel's cave is presented. The discussion emphasizes the pathological criteria for the diagnosis of a peripheral neuroepithelioma and the current controversy about the classification of this and related tumors.

Humans↗

An immunohistochemical study of neuroepithelial (colloid) cysts.

Monoclonal and polyclonal antisera were used against 12 cases of neuroepithelial (colloid) cysts to determine the specific antigenic profile of the cyst epithelium. Intermediate filament markers (cytokeratin, vimentin, neurofilament, and glial fibrillary acidic protein) and epithelial markers (epithelial membrane antigen and monoclonal antibody lu-5) demonstrated that colloid cyst epithelium has a unique antigenic profile in contrast to that of choroid plexus or glial tissue. Theories raised to explain the etiology of colloid cysts have included derivation from the embryonic paraphysis, detachments of developing neuroepithelium from the tela choroidea, and remnants of respiratory epithelium; a more recent theory suggests that these cysts are products of developing choroid plexus or ependyma. The present study shows that colloid cyst epithelium is distinct from that of choroid plexus or ependyma and therefore does not represent a product of their formation, nor does it represent a form of immature glia. This finding supports the conclusion that colloid cysts in any ventricular location represent a developmental anomaly of primitive neuroectoderm in the embryo, which remains unique from other products of neuroectodermal derivation.

Brain Diseases↗

Fat embolism in acute pancreatitis.

A patient who developed progressive hypoxemia and multiple system failure during the course of acute pancreatitis is described. Autopsy showed fat emboli to the lungs, kidneys, and heart, as well as multiple petechial hemorrhages in the brain. We conclude that fat embolism should be considered in the differential diagnosis of progressive hypoxemia in patients with acute pancreatitis.

Acute Disease↗

Hypertrophic brachial plexus neuritis: a pathological study of two cases.

Two patients with brachial plexus neuropathy had recurrent symptoms for years that culminated in the development of a painful lower cervical mass. On macroscopic examination the affected nerves had a fusiform segmental enlargement, suggestive of a nerve sheath tumor, that was excised. Histologically, marked endoneurial edema, florid focal chronic inflammation, extensive onion bulb formation, and perineurial sparing were seen. Electron microscopic studies on one patient revealed microvasculitis; frequent tubuloreticular inclusions in endothelial cells, histiocytes, and lymphocytes; and cylindrical confronting cisternae in lymphocytes. We conclude that some cases of recurrent brachial plexus neuropathy are due to a localized chronic inflammation that may be related to autoimmunity or to a viral infection.

Adult↗

Mixed ependymoma-astrocytoma (subependymoma?) of the cerebral cortex.

Tumors of mixed glial origin may present as low-grade neoplasms with distinct cell populations or as polymorphic malignant gliomas. Such cell populations are usually appropriate to the location of the tumor. A mixed tumor, with a cell population inappropriate to tumor location, is presented to discuss theories of glial differentiation and cellular heterogeneity. This mixed ependymoma-astrocytoma of the parietal cortex, ultrastructurally similar to subependymoma but anatomically separate from the subependymal cell matrix or ventricular surface, gives evidence for neoplastic differentiation into ependymal and astrocytic cell lines. This supports the idea of a common progenitor cell, the ependymoglia or tanycyte, in human cerebral cortex.

Adult↗