Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C1762617 (weakness)
 37,932 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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.
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PMID:Clinicopathologic study of 61 patients with ependymoma including MIB-1 immunohistochemistry. 999 Jan 8

We present a 54-yr-old woman with ectopic corticotropin syndrome caused by a neuroendocrine tumor of the pancreas. At initial presentation, the patient suffered from diarrhea, heartburn, and nonspecific abdominal pain. There was no evidence of Cushing's syndrome. A neuroendocrine tumor in the head of the pancreas with metastases into peripancreatic lymph nodes was diagnosed and completely resected. Fourteen months later, abdominal computed tomography and scintigraphy with (111)In-labeled octreotide suggested relapse of the tumor. The patient again had no evidence of Cushing's syndrome. A second in toto tumor resection was performed. Another 8 months later, the patient developed forgetfulness, depressive episodes, muscle weakness, new-onset hypertension, hypokalemia, plethora, diabetes mellitus, polyuria, and weight loss. Endocrine testing suggested a source of ectopic ACTH production. An octreotide scan showed an intense uptake ventromedial of the left kidney, an area that showed a mass lateral of the superior mesenteric artery on abdominal magnetic resonance imaging. A complete pancreatectomy with splenectomy and left-sided adrenalectomy were performed. At this second relapse, this neuroendocrine tumor clinically had changed its hormonal profile. Immunohistochemically, in contrast to primary tumor and first relapse, we found strong immunostaining for ACTH in tumor cells of the second relapse and a MIB-1 index greater than 20%. To our knowledge, this is the first report describing a pancreatic neuroendocrine tumor that started to secrete ACTH de novo at the time of the second relapse after two former complete tumor resections. This case underscores the pluripotency of neuroendocrine tumor cells and the importance of keeping in mind a possible shift in hormone production during tumor evolution and progression.
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PMID:Pancreatic neuroendocrine tumor with ectopic adrenocorticotropin production upon second recurrence. 1529 97

June 2004: Over the past year, this man in late-60s had complained about progressive weakness of concentration and memory disturbances, associated with word finding difficulties. MRI examination revealed an extra-axial, parasagittal tumor 3 cm in diameter located in the left frontoparietal region. Five years ago, a meningioma in the same region, with radiographic appearance comparable to the present tumor had been totally removed. The histological picture of the current tumor was dominated by sheets of large rounded pleomorphic tumor cells with abundant eosinophilic cytoplasm and eccentric nuclei (rhabdoid cells). Cytoplasmic inclusions were frequent; occasionally,multinucleatedtumorcellswereseen. Mitoticfigures were absent and the MIB was 3%. Meningothelial lobules were scarce, and regions with fibroblastic appearance were absent. There were no psammoma bodies, necrosis or brain invasion. Moderate immunoreactivity for EMA was found. Additionally, strong cytoplasmic immunoreaction for vimentin within the rhabdoid cells was observed. Review of the previous material showed small islets of rhabdoid cells. Rhabdoid meningioma is an uncommon meningioma variant. It has been suggested that rhabdoid meningiomas are highly aggressive tumors (WHO grade III)and that the rhabdoid phenotype represents a marker of malignant transformation in meningiomas. Histologically, rhabdoid meningiomas usually exhibit signs of anaplasia, a high mitotic activity, and a markedly increased MIB-1 labeling index. Extracranial metastases may occur in the course of the disease. However, not all rhabdoid tumors appear to have anaplastic features (as this case illustrates). Another interesting feature of rhabdoid meningiomas is that in a significant number of cases, the rhabdoid cells appear only at the time of recurrence. Alternatively, as seen in this case, the rhabdoid cells may be already present in the primary meningioma, but not as the predominating histological feature.
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PMID:June 2004: a male in his late 60s with recurrent extracerebral tumor. 1560 95

A 35-year-old woman presented with one month's history of progressive bilateral leg weakness and altered sensation. There had been no pain. She had noted urinary frequency and constipation in the previous two weeks. On examination, the patient had diffuse lower extremity weakness (2-3/5), with a T6 sensory level to pain and temperature sensation. MRI demonstrated a T4-5 intradural mass ventral to the spinal cord, with an enhancing dural tail, consistent with meningioma. At surgery an intradural, extramedullary, firm, black neoplasm was encountered, which invaded the ventral dura and elevated and distorted the spinal cord. The mass was removed, leaving only microscopic invasion of the ventral dura. There was no bone invasion. Serial sections revealed a homogeneous black tumor without necrosis. H&E stained sections showed an occasionally fascicular tumor of melanocytes and small round blue tumor spindle cells with melanin pigmentation and 1-2 mitotic figures per 10 high-powered fields. The nuclei are generally oval-shaped and elongated, with prominent nucleoli. Necrosis, hemorrhage, and nuclear and cellular pleomorphism are not present and mitotic figures are rare. Immunohistochemical staining was positive for S-100 and HMB-45. MIB-1 labeling averaged 1-2%. A diagnosis of primary meningeal melanocytic tumor was made. Primary meningeal melanocytic tumors (PMMTs) are rare; fewer than 100 cases have been described. PMMTs of the CNS consist of a spectrum of tumors ranging from well-differentiated melanocytoma to its overtly malignant counterpart, melanoma. Intermediate grade melanocytomas (IMGs) are the least common variant, comprising about 10% of PMMTs reported. IGMS occur in the spinal leptomeninges and intracranially in approximately equal proportions. IGMs are more cellular than the well-differentiated variant, with 1-3 mitotic figures per 10 HPFs and MIB-1 labeling of <6%. By contrast, melanomas contain more mitotic figures (3-15 per 10 HPF) and MIB-1 labeling rates up to 15%. Once metastasis, including drop metastasis from pigmented medulloblastomas, have been excluded, the differential includes pigmented meningiomas and schwannomas (solitary or as part of Carney complex), as well as other pigmented CNS tumors such as ependymoma and pineoblastoma and systemic diseases such as lymphoma . . . For primary CNS melanocytic neoplasms, complete tumor resection is preferred, as it leads to cure of well-differentiated and intermediate-grade melanocytomas and most melanomas. Radiotherapy is recommended for incomplete resection of IMGs and melanomas; the recurrence potential of low-grade melanocytomas is less clear and watchful waiting may be employed, since recurrent tumors may be treated surgically prior to radiation. Two months after surgery, the patient had normal sensation and strength. She was given focused radiotherapy to the region of the ventral thecal sac to 40 cGy. At one year following surgery, the patient's neurological examination is normal and she remains free of residual disease by MR examination.
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PMID:35-year-old woman with progressive bilateral leg weakness. 1676 59

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

Primary meningeal gliomas are uncommon tumors in the subarachnoid space, their primary characteristic being the absence of any obvious connection to the brain parenchyma. Rarely, they are quite malignant and assume a bulky, well circumscribed appearance rendering the differential diagnosis from other CNS neoplasms difficult. A 53-year-old man presented with a history of persistent headaches and left sided weakness. Magnetic resonance imaging revealed a temporoparietal mass attached to the dura that strongly resembled a meningioma. At surgery, the outer layer of the dura mater was intact and there was a clear brain-tumor interface without obvious pial disruption. Histological examination showed a biphasic pattern consisting of benign connective tissue intermingled with bundles of what seemed to be a glioblastoma. The mass demonstrated strong positivity for GFAP and the MIB labeling index focally exceeded 20%. The tumor was identified as a primary meningeal glioblastoma. The patient was disease-free for 42 months, after which he developed a recurrence for which he was re-operated. This time, the pathological findings of the tumor were those of a typical glioblastoma multiforme. We discuss the origin of the initial neoplasm and also the differential diagnosis that needs to include meningioma, aggressive glioblastoma infiltrating the dura and a recently recognized bimorphic CNS tumor: the desmoplastic glioblastoma.
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PMID:Primary extracerebral meningeal glioblastoma: clinical and pathological analysis. 2017 27

Meningioangiomatosis is a rare hamartomatous lesion or meningiovascular malformation in brain. In extremely rare condition, meningioma may occur together with meningioangiomatosis, and only 19 cases have been described in English literature until now. We now report a case of meningioangiomatosis-associated meningioma with atypical and clear cell variant. A 34-year-old man presented a 3-month history of progressive numbness and weakness of his left lower extremity. He had no stigmata of neurofibromatosis type 2. Magnetic resonance imaging (MRI) revealed multifocal lesions in the right frontoparietal lobe. The lesions were totally removed. Microscopically, parts of lesions were atypical and clear cell meningioma corresponding to WHO grade II. The adjacent brain parenchyma showed the histological features of meningioangiomatosis. Neoplastic cells in atypical meningioma area were immunoreactive to epithelial membrane antigen (EMA) with high MIB-1 index of up to 20%. However, the spindle cells in meningioangiomatosis area were negative for EMA with low MIB-1 index of up to 1%. The diagnosis of atypical meningioma associated with sporadic meningioangiomatosis was made. To our knowledge, this is the first case of a meningioangiomatosis-associated meningioma with atypical and clear cell variant component to be described. The patient had been followed-up for 11 months without adjuvant radiotherapy or chemotherapy. No tumor recurrence was found during this period. Meningioangiomatosis-associated meningioma is more likely to occur in younger patients and histologically to mimic parenchymal invasion of brain. We suggest that postoperative radiotherapy or chemotherapy should be given careful consideration to avoid over-treatment due to erroneously interpret as malignant meningioma.
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PMID:Sporadic meningioangiomatosis-associated atypical meningioma mimicking parenchymal invasion of brain: a case report and review of the literature. 2056 69

Epithelial-myoepithelial carcinoma (EMC) of the lung is rare and is considered to be low-grade malignancy. Intracranial metastasis of pulmonary EMC has not previously been reported according to our search of the literature. We report a case of skull metastasis as the initial manifestation of pulmonary EMC. An 81-year-old man complained of left leg motor weakness. Neurological examination showed left hemiparesis. Computed tomography and magnetic resonance imaging revealed an osteolytic tumor in the right frontal bone with invasion to the dura and subdural space, attached to the superior sagittal sinus. Subtotal removal of the tumor was performed, and the left hemiparesis showed improvement. Histopathological study revealed the tumor to consist of epithelial and myoepithelial cells. Pulmonary EMC was diagnosed. The MIB-1 index in primary lesion was approximately 10%. The skull and dura are possible sites for metastasis from pulmonary EMC. The MIB-1 index is a predictive marker of malignant potential.
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PMID:Skull metastasis as initial manifestation of pulmonary epithelial-myoepithelial carcinoma: a case report of an unusual case. 2262 83

The patient with a history of bone pain and muscle weakness, was thought to have oncogenic osteomalacia as a result of biochemical investigations and directed to Nuclear Medicine Department for a whole-body bone scintigraphy and 111In-octreotide scintigraphy. There was no focal pathologic tracer uptake, but generalized marked increase in skeletal uptake on bone scintigraphy. Octreotide scintigraphy showed accumulation of octreotide in the region of the left lobe of the thyroid gland in the neck. Thereafter, parathyroid scintigraphy was performed with technetium-99m labeled metroxy-isobutyl-isonitryl (99mTc-MIB) and MIBI scan demonstrated radiotracer uptake at the same location with octreotide scintigraphy. The patient underwent left inferior parathyroidectomy and histopathology confirmed a parathyroid adenoma. Somatostatin receptor positive parathyroid adenoma may show octreotide uptake. Octreotide scintigraphy may be promising and indicate a possibility of using somatostatin analogues for the medical treatment of somatostatin receptor positive Conflict of interest:None declared.
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PMID:Octreotide uptake in parathyroid adenoma. 2348 97

Medulloepithelioma is an extremely rare PNET in late adolescence and adults with only two cases noted in literature. These are WHO grade IV tumors with dismal prognosis. Only few cases survived beyond 5 months. We report a rare case of supratentorial medulloepithelioma in a 17 year old girl. She had presented with right sided weakness, headache and vomiting. Imaging showed an enhancing mass lesion in left parietal region which undergone gross total resection. After surgery, her headache, vomiting and right sided weakness improved. On histopathology, the tumor had characteristic trabecular, ribbon and palisaded arrangement with brisk mitotic activity, necrosis and calcification. Immuno-histochemistry revealed positivity for Synaptophysin, Vimentin and EMA while GFAP was negative. MIB-1 labeling was very high. Patient received postoperative radiotherapy. On follow up after 14 months, she was clinically asymptomatic with no recurrence on imaging.
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PMID:Unusual occurrence of supratentorial medulloepithelioma in a young female. 2500 66


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