Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027627 (metastases)
 103,950 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

During a 6 month period, 50 patients with signs and symptoms referable to the cervical spine were studied with a 0.6 T superconducting magnetic resonance (MR) imaging unit. The last 23 of these 50 patients were studied with combined multislice and multiecho techniques. In 38 of the 50 patients, abnormalities were demonstrated on MR images. Intramedullary lesions included syringomyelia (three cases), primary tumors (two), metastatic neoplasm (one), cord atrophy secondary to trauma (one), and multiple sclerosis (one). Intradural, extramedullary lesions included two neurofibromas and two Chiari malformations. The rest of the lesions were extradural: degenerative changes (10), spinal stenosis with cord compression (five), disk degeneration and/or herniation (five), postoperative changes (four), metastases to bone/epidural disease (three), and neurofibromatosis (one). Two patients had more than one abnormality. The MR findings were compared with available routine radiographs, computed tomographic (CT) scans with and without metrizamide, and myelograms. MR imaging was consistently better than routine CT scanning in the detection of lesions of the spinal cord and in directly imaging the effects on the spinal cord of extrinsic abnormalities such as spinal stenosis. Metrizamide-enhanced CT scanning detected all cases of syringomyelia, but it involved an invasive procedure. Myelography alone was slightly less sensitive and considerably less specific than MR in detecting intramedullary lesions and in distinguishing cord neoplasms from syringomyelia. Multislice, multiecho techniques with up to 240 msec echo times (TEs) were particularly helpful in the detection and characterization of extradural processes.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:0.6 T MR imaging of the cervical spine: multislice and multiecho techniques. 392 Aug 81

A 39-year-old female patient with familiar neurofibromatosis developed a sarcoma in the hollow of the knee that appeared clinically and sonographically as a Baker cyst. Following an operation to remove the tumour and subsequent local radiation treatment multiple metastases appeared distant to the primary tumour from which the patient died. On the observation of a Baker cyst in a patient with neurofibromatosis the possibility of malignant degeneration must be kept in mind and an operation carried out immediately.
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PMID:[Neurofibromatosis and the development of neurofibrosarcoma simulating Baker's cyst]. 392 48

Twenty -six adrenal pheochromocytomas and four normal adrenal medullae were studied immunohistochemically. These included four malignant tumors with proven metastases, six tumors in patients with neurofibromatosis, nine tumors in patients with multiple endocrine neoplasia type 2, and seven sporadic nonfamilial pheochromocytomas. Immunohistochemical localization of neuron-specific enolase (NSE) was seen in all tumors and in the four normal adrenals. Methionine enkephalin-like immunoreactivity was present in tumors from all four groups and in the normal adrenals. Corticotropinlike immunoreactivity was found focally in two normal adrenal medullae and in four benign pheochromocytomas. Our results indicated that NSE was present in all four major groups of pheochromocytomas, including benign and malignant tumors. This marker aided in distinguishing between adrenal cortical and medullary tumors in unusually difficult cases, since all normal adrenal cortex and adrenal cortical tumors had negative test results for NSE.
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PMID:An immunohistochemical study of pheochromocytomas. 620 98

Twenty-six cases of enophthalmos were reviewed. The causes in order of frequency were: orbital asymmetry (8); trauma (5); orbital metastasis (4); microphthalmos (2); orbital varix (2); maxillary mucocele (2); localized scleroderma (1); absence of sphenoid wing (neurofibromatosis) (1); post irradiation atrophy (1). Only six of the patients (23%) were referred with the diagnosis of enophthalmos, suggesting the sign maybe subtle and is frequently missed or misdiagnosed. The nature of the causes underscore the need for careful and thorough diagnosis. In particular, the therapeutic implications of diagnosing metastatic disease, maxillary mucocele, and orbital varices is noted. A review of etiology and mechanisms of enophthalmos point to the diversity, importance and conditions causing this sign.
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PMID:Enophthalmos: a clinical review. 671 10

Case histories of 10 patients with neurofibrosarcoma, including 14 (70%) with neurofibromatosis, evaluated over 10 years were reviewed to determine the incidence of local and systemic recurrence and the most effective means of therapy for this rare neoplasm. Initial therapy resulted in complete local disease control in only 11 (55%) patients. Local excision, or local excision plus radiation or chemotherapy resulted in local recurrence in 8 of 12 patients. Radical surgery alone, or radical surgery combined with radiation and chemotherapy resulted in local recurrence in 1 of 6. Even with complete local disease control, 7 of 16 (44%) patients died of metastases. Both A.J.C. Clinical Stage II and III patients had a similar poor prognosis. Associated neurofibromatosis did not worsen prognosis. These data suggest that both moderate and high-grade primary neurofibrosarcoma are highly malignant neoplasms and should be treated by radical resection. Preoperative intraarterial Adriamycin and radiation--found to be successful for other highly malignant sarcomas--may be of benefit. Since distant disease occurs despite local control, postoperative adjuvant chemotherapy trials are warranted.
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PMID:Neurofibrosarcoma. 676 81

A case of intrathoracic ancient neurilemoma undergoing malignant change in a 48-year-old woman is reported. The lesion was first discovered incidentally during routine roentgenologic examination of the chest 15 years before its surgical removal. The gross and histologic examination of the tumor showed the features of ancient neurilemoma with extensive regressive changes as well as small preserved areas composed of Antoni type A and B tissues. In addition, obviously histologically malignant foci manifested by the presence of increased cellularity, active mitotic activity, and gradual loss of orginary neurilemomatous differentiation were present. The patient died 16 months after operation of recurrent and distant metastatic disease. At autopsy, there was no evidence of generalized neurofibromatosis or other primary cancer. A review of the available literature revealed only two other similar cases documenting malignant transformation of solitary benign neurilemomas in patienets without von Recklinghausen's disease.
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PMID:Malignant transformation of intrathoracic ancient neurilemoma in a patient without von Recklinghausen's disease. 680 2

A neurofibroma, a fibroma, a primary neurofibrosarcoma, and four neurofibrosarcoma metastases from a woman with hereditary neurofibromatosis who was heterozygous (GdB/GdA-) for the X-linked enzyme glucose-6-phosphate dehydrogenase were studied to determine the number of cells from which the tumors developed. Both enzyme types were observed in the benign tumors in proportions similar to those present in seven different normal tissues studied. These findings indicated that the benign tumors arose from many cells. In marked contrast, only type A activity was detected in the primary neurofibrosarcoma and in all of the metastases. Two or more steps probably were involved in the development of neurofibrosarcoma in this patient: the inherited genetic mutation producing neurofibromatosis and a rare event or combination of events that permitted a single cell to undergo malignant proliferation.
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PMID:Probable clonal origin of neurofibrosarcoma in a patient with hereditary neurofibromatosis. 681 62

A 58-year-old man presented with a solitary, asymptomatic, firm nodular lesion on his glans. A simple excision was carried out. Follow-up for 1 year failed to reveal either recurrence or metastases. Histologically, there was intradermal proliferation of atypical oval and spindle-shaped cells arranged in fascicles simulating the pattern of neural tumours. Mitotic figures were abundant. Stains for melanin, HMB-45 antigen were negative. Immunohistochemically, tumour cells stained for S-100 protein and vimentin. Malignant schwannoma usually occurs in patients with neurofibromatosis and is located in the subcutaneous tissues. Solitary malignant schwannoma of superficial soft tissue is a rare entity, and there are problems of differential diagnosis against other spindle cell tumours, especially malignant spindle cell melanoma. Their appearance on the glans is rare.
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PMID:[Malignant schwannoma of the glans penis]. 759 73

We report a case of femoral osteosarcoma with brain metastases in a female patient with longstanding von Recklinghausen disease. Osteosarcoma of a long bone has not yet, to our best knowledge, been reported in association with type I Neurofibromatosis. Central nervous system metastases occur very rarely in osteosarcoma.
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PMID:[The rare metastasis of osteosarcoma of a long bone associated with von Recklinghausen's neurofibromatosis]. 807 7

One hundred and thirty patients with soft tissue sarcoma of the head and neck were treated at the Royal Marsden Hospital between 1944 and 1988. Pathological review was possible in 103 of these cases; only pathologically reviewed cases have been analysed. The median age at presentation was 36 years, and 53% were male. Four had neurofibromatosis type I, and one previous bilateral retinoblastoma. Six had undergone previous radiotherapy, 12 to 45 years prior to developing sarcoma. The tumours were < or = 5 cm in 78% of cases and high grade in 48%. Only one patient presented with lymph node metastases and only one with distant metastases (to lung). Malignant fibrous histiocytoma was the commonest histological type, occurring in 30 cases. The overall 5 year survival was 50% (95% CI 39-60). Local tumour was the cause of death in 63% of cases and 5 year local control was only 47% (95% CI 36-58) with local recurrence occurring as late as 15 years after treatment. The only favourable independent prognostic factor for survival was the ability to perform surgery (other than biopsy), with or without radiotherapy, as opposed to radiotherapy alone (hazard ratio 0.39; P = 0.003). Only one patient had a biopsy with no further treatment. Favourable independent prognostic factors for local control at 5 years were site (tumours of the head as opposed to the neck, hazard ratio 0.42; P = 0.02) and modality of treatment (combined surgery and radiotherapy compared to either alone, hazard ratio 0.31; P = 0.002). Patients in the combined modality and single treatment modality groups were well balanced for T stage, grade and tumour site. The patients in the combined treatment group had less extensive surgery, yet their local recurrence-free survival was longer. Unlike soft tissue sarcomas at other sites, those in the head and neck region more often cause death by local recurrence. The addition of radiotherapy to surgery may result in longer local recurrence-free survival.
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PMID:Head and neck sarcomas: prognostic factors and implications for treatment. 831 14


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