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)

Twenty-six patients with far-advanced epidermoid carcinoma of the head and neck were treated with cis-dichlorodiammineplatinum(II) (DDP) in high doses, utilizing the technique of concurrent mannitol-induced diuresis. All 26 patients had received prior radical radiotherapy to local disease, 14 had had major ablative surgical procedures, and all but five had been previously treated with chemotherapy. Responses were as follows (duration in months): two complete (2+, 6+), six partial (1, 2, 3, 4, 5+, 8+), ten minor (all but one less than 1.5), and eight no change or progression. Responses were seen in primary tumors, neck nodes, and pulmonary metastases. Nausea and vomiting were seen consistently in all patients but were never dose-limiting. Myelosuppression was mild, with only three patients developing platelet counts less than 50,000/mm3 and no patient with a wbc count less than 2000/mm3. Transient elevations of serum creatinine were common but no patient developed peak levels greater than 2 mg/dl. Transient tinnitus was also common although only four patients developed clinically significant hearing loss during the trial and DDP could be definitely implicated in only one instance. DDP in this dose and schedule is thus effective and safe therapy in a very heavily pretreated group of patients with advanced head and neck cancer and is now being used in combination with other agents in the initial treatment of these patients.
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PMID:CIS-Dichlorodiammineplatinum(II) in the treatment of epidermoid carcinoma of the head and neck. 87 36

Progressive unilateral sensorineural deafness and tinnitus developed in a 59-year-old woman over a 1-year period. Clinical examination showed a tumor mass which almost completely filled the tympanic cavity, and grew around the auditory ossicular chain. Histological findings revealed the tumor to be a primary carcinoid of the middle ear. Neuro-endocrine differentiation was confirmed immunohistochemically by positivity for neuron-specific enolase, chromogranin, pancreatic polypeptide and synaptophysin. Using electron microscopy, neuroendocrine granules could be visualized. In addition, both light and electron microscopy revealed that cells had an epithelial differentiation with mucin granules while immunohistochemistry showed a positivity for cytokeratins. The detection of intermediary filaments (immunohistochemically with vimentin and under electron microscopy) was unique to this neoplasm and has to be considered in distinguishing the carcinoid tumor from the papillary adenoma of the middle ear. Tumor prognosis is excellent with radical extirpation from the middle ear. In the case presented, there has been no evidence for either recurrence or metastases 10 months after surgical resection.
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PMID:[Carcinoid of the middle ear: a rare tumor with biphasic differentiation. Case report with special reference to immunohistochemistry and electron microscopy]. 142 31

The authors review the clinical, radiological and pathological features of 6 cases of glomus tumors of the temporal bone. Out of the 6 patients, 5 were female; age was distributed between 22 and 76 years (mean 48 years). The main clinical features were hypoacusia, tinnitus and otoscopic findings suggestive of the diagnosis. In one case was noted the concomitant presence of a neurinoma of the VIII cranial nerve with a ipsilateral glomus tumor, and in another case there was a concomitancy of carotid body tumor with temporal glomus jugularis tumor. Metastases were not observed in any case. Tumoral lesions were successfully ressected employing microsurgical techniques and a multidisciplinary staff involving neurosurgeons, head and neck surgeons and otolaryngologists. Radioteraphy was not employed, neither pre-operative embolization. Some aspects related to the nosology, embriology, pathophysiology, diagnosis and treatment of this interesting type of neoplasms are discussed.
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PMID:[Glomus tumors of the temporal bone: a report of 6 cases]. 254 64

A 54-year-old man initially complained of frontal headache, right ear pain and tinnitus in May, 1985. This was followed by right facial palsy and hearing loss, and he was admitted to our hospital. Physical findings revealed right trigeminal nerve disturbance, left facial nerve palsy and bulbar palsy. The spinal fluid showed pleocytosis, increased protein, decreased glucose, markedly increased carcinoembryonic antigen and adenocarcinoma cells. Gastric carcinoma was revealed by an upper GI series. He was treated with chemotherapy. However, he die in August, 1985. Nodular metastases were discovered at the right internal acoustic meatus and other areas. Microscopically, signet-ring cell carcinoma had diffusely infiltrated at the subarachnoid space.
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PMID:[An autopsy case of meningeal carcinomatosis with vestibulocochlear nerve disturbance as the first manifestation]. 302 29

A 42-year-old man presented with a two-week history of right-sided otalgia, deafness and nocturnal tinnitus. Examination revealed a black mass on the posterior meatal wall. Two years before presentation, he had a malignant melanoma removed from the left hip region. Biopsy of the lesion in the posterior canal wall and another which had developed within two weeks on the anterior wall, confirmed metastatic disease. Treatment with radiotherapy and later chemotherapy were unsuccessful and the patient died 15 months later.
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PMID:Melanoma metastatic to the head and neck. 317 81

Renal cell carcinoma (Grawitz tumor, hypernephroma), may metastasize to the head and neck region at different stages of its evolution. The authors present a case of a 79 year old woman who consulted for otalgia, deafness, and tinnitus. A polyp in the external auditory canal proved to be a metastasis of a renal carcinoma. The patient underwent a nephrectomy eight years previously and had no problems since. The data on otolaryngological metastasis of this tumor as well as its pathogenesis are reviewed.
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PMID:[Metastasis of a hypernephrome to the ear]. 713 34

Malignant melanoma is a common cause of central nervous system metastases. This report describes an extremely rare case of metastatic melanoma presenting as an isolated cerebellopontine angle tumor. Clinically and radiographically, the lesion mimicked an acoustic neuroma. The patient had neuro-otological symptoms, including tinnitus, vertigo, sensorineural hearing loss, facial nerve dysfunction, and prominent cerebellar dysfunction. Magnetic resonance images showed a lesion of the internal auditory canal and cerebellopontine angle that was hypointense on T1-weighted images, hyperintense on T2-weighted images, and enhanced after the administration of gadolinium. T2-weighted images showed significant cerebellar edema. Subtotal resection of the tumor through a suboccipital craniotomy palliated the symptoms, but the patient died of tumor progression 6 months later. In contrast to other metastatic tumors of the temporal bone, melanoma initially metastasizes to the internal auditory canal and is characterized by early neurovascular infiltration. This report highlights the pathophysiological characteristics, radiological findings, differential diagnosis, and treatment of metastatic melanoma of the internal auditory canal and cerebellopontine angle.
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PMID:Isolated metastatic melanoma of the cerebellopontine angle: case report. 835 32

Metastasis in the inner auditory canal (IAC), or more precisely in the pontocerebellar angle (PCA) is extremely rare. Indeed acoustic neurinoma (AN), meningioma and other types of benign neoformations, in decreasing order, account for nearly all expansive pathologies in this anatomical region. The present paper reports a clinical case of a patient with the sudden onset of an hearing loss in the right ear. This hearing loss was promptly followed by paralysis of the homolateral facial nerve. The function of both cranial nerves worsened progressively and rapidly. Cerebral radiology, performed with MRI and the administration of gadolinium, showed a AN-compatible neoformation fundamentally affecting the IAC. However, histological tests performed after surgery indicated an adenocarcinoma. Post-operative instrumental tests identified the primary neoplasm in the lower lobe of the right lung. The purpose of this work was to highlight, in view of the literature on the topic, the main clinical aspects to consider when a malignant expansion is suspected in the PCA. Naturally there is greater basis for such suspicion when a primary malignant neoplasm has previously been diagnosed. Generally AN progresses quite gradually, in line with an extremely slow growth rate. Therefore it is normally seen in a progressive worsening of hearing, tinnitus and equilibrium disorders. Appearance of a facial nerve palsy is strictly limited to relatively large neoplasms and is encountered at a later stage. This is why a rapid progression of hearing damage accompanied, or promptly followed, by complete facial nerve paralysis must alert one to the possibility of a PCA malignancy. Imaging does not permit easy pre-operative diagnosis since the radiological signs and morphologies are often quite similar to those produced by AN. Therefore, post-operative histological examination can hold some surprises.
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PMID:[Metastasis in the inner auditory canal]. 1020 26

A 56-year-old male with a history of lung cancer presented with isolated metastases of adenocarcinoma in the bilateral internal auditory meatuses (IAMs), mimicking the bilateral acoustic schwannomas of neurofibromatosis type 2, and manifesting as rapidly worsening tinnitus and bilateral hearing loss. Magnetic resonance imaging showed small tumors in both IAMs with no sign of leptomeningeal metastasis. The preoperative diagnosis was neurofibromatosis type 2. Both tumors were removed and the histological diagnoses were adenocarcinoma. Neuroimaging differentiation of a solitary metastatic IAM tumor from a benign tumor is difficult, although rapidly progressive eighth cranial nerve dysfunction suggests a malignant process. Metastases should be considered as a rare diagnostic possibility in a patient with small tumors in both IAMs.
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PMID:Isolated metastases of adenocarcinoma in the bilateral internal auditory meatuses mimicking neurofibromatosis type 2--case report. 1059 43

Von Hippel-Lindau (VHL) syndrome (OMIM 193300) is an autosomal dominant disorder caused by deletions or mutations in a tumor suppressor gene mapped to human chromosome 3p25. It is characterized clinically by vascular tumors, including retinal and central nervous system hemangioblastomas (cerebellar, spinal, and brain stem). Hemangioblastomas are benign and do not metastasize. Other features include cysts of the kidneys, liver, and pancreas. Clear cell renal cell carcinoma occurs in up to 70% of patients with VHL and is a frequent cause of death. Pheochromocytomas occur in association with specific alleles of the VHL gene; therefore, a family history of pheochromocytoma in association with VHL is an indication for thorough surveillance for pheochromocytoma in affected family members. Recently, it has been appreciated that patients with VHL may develop endolymphatic sac tumors, which can cause tinnitus or deafness. The diagnosis of VHL may be made in a patient with a family history of VHL based on a single retinal or cerebellar hemangioblastoma, renal cell carcinoma or pheochromocytoma, and, possibly, multiple pancreatic cysts. Renal and epididymal cysts are not sufficient to make the diagnosis of VHL. In the absence of a family history of VHL the presence of two or more retinal or cerebellar hemangioblastomas, or one hemangioblastoma with one visceral tumor, is required for diagnosis. Studies of the natural history of VHL showed a life expectancy less than 50 years before surveillance protocols were developed. Annual assessments (physical and ophthalmologic examinations) should begin in infancy. Imaging of abdominal organs and the brain and spine should be added in teenagers and adults. Renal cysts and tumors should be monitored by computed tomography every 6 months. Mutation analysis has allowed presymptomatic identification of affected family members; those found not to have inherited the gene do not need to be monitored. The VHL gene coding sequence contains three exons, and two isoforms of mRNA exist, reflecting the presence or absence of exon 2. Tumors arise after the loss or inactivation of the wild type allele in a cell. About 20% of patients have large germline mutations detectable by Southern blot analysis, 27% have missense mutations, and 27% have nonsense or frameshift mutations. In about 20% of VHL families no deletion or mutation can be detected. Families may be characterized by the presence (type 2; 7% to 20% of families) or absence (type 1) of pheochromocytomas. Most type 2 families have missense mutations, whereas most type 1 families are affected by deletions or premature termination mutations. Prognostic counseling regarding the lifetime risk of pheochromocytoma can be aided by determination of the underlying mutation in patients without family histories of VHL.
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PMID:Von Hippel-Lindau syndrome. A pleomorphic condition. 1063 Jan 73


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