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)

The occurrence of deaf-mutism and goiter unassocaited with creatinism or mental retardation in euthyroid patients is known as Pendred's Syndrome. It is considered due to a single mutant recessive gene responsible for both the goiter and deafness. The penetrance is high, the intenseness of expressivity may vary within the same family and only one generation is affected. The extremely atypical hyperplasia seen in such goiters has been considered malignant. In 1956 the author reported a family in which 4 of 6 sibilings demonstrated Pendred's Syndrome. Three of the 4 had undergone thyroidectomy, two were considered to have carcinoma. Nineteen years later the family is again reported. The fourth sibling has recently undergone thyroidectomy. This thyroid demonstrated the same atypical hyperplasia as seen in the elder two siblings. The 19 year followup of this family has shown no evidence of recurrence or metastases, indicating that the atypical hyperplasia is probably not malignant. Pendred's Syndrome is described and certain suggestions are made for the counseling of the parents and the treatment and counseling of those children so afflicted.
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PMID:Goiter and deaf mutism. 121 96

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

An 80-year-old man was seen in the clinic with a two month history of pain, bleeding and deafness in his left ear; accompanied by a left facial palsy. Six months earlier, anaplastic carcinoma of the bladder had been diagnosed and treated with radiotherapy. On examination there was a mass in his left external auditory canal and VII, VIII, IX and X left cranial nerve palsies. Histology of a biopsy was identical with his original bladder tumour. He received a course of palliative radiotherapy but died shortly after. Post-mortem demonstrated the left temporal bone metastasis extending into the middle and posterior cranial fossae. There was no evidence of residual bladder tumour, or other primary tumour. Temporal bone metastases are probably more common than generally thought. Presentations like this may become more common as cancer therapy improves, with patients surviving longer and presenting later with metastatic disease.
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PMID:Metastasis to the temporal bone, secondary to carcinoma of the bladder. 276 28

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

The natural course of basal cell carcinoma of the middle ear is described. This tumour is extremely rarely found in the middle ear and only two cases have been reported to date. The appearance of the tumour was heralded by earache and deafness, followed years later by wide local destruction of the temporal and parietal bones, base of skull, facial palsy and multiple distant metastases. The patient refused both surgical and irradiation therapy and died 10 years after the initial diagnosis.
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PMID:Basal cell carcinoma of the middle ear--a natural history. 319 14

A case of T-prolymphocytic leukaemia (T-PLL) presenting with deafness and confusion is reported. Computerised tomography (CT) of the head showed several well-defined, rounded, high attenuation areas in the temporal, parietal and occipital regions of the brain substance that were suggestive of metastases. Treatment with weekly intravenous deoxycoformycin produced complete resolution of the CT abnormalities together with haematological evidence of disease regression 6 weeks after treatment was started.
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PMID:Regression of intracerebral lesions in T prolymphocytic leukaemia treated with intravenous deoxycoformycin. 325 29

Cisplatin was used in 14 bone and soft tissue sarcomas. Severe vomiting developed in all cases, but the duration was relatively short. Renal function was disturbed in cases with a higher total dose. This side effect was considered to be the dose-limiting factor of cisplatin. Seven cases showed high-frequency deafness but they did not complain of disturbance during conversation. In seven metastatic osteosarcomas, one was evaluated as a partial response and one as a minor response. No response was observed among three soft tissue sarcomas. Three cases of osteosarcoma receiving cisplatin in adjuvant chemotherapy have been disease-free for 4 and 46 months after resection of pulmonary metastases and for 50 months after resection of the primary tumor. We consider cisplatin to be the first-choice drug in cases resistant to adriamycin or methotrexate, but there are some problems when cisplatin is used in adjuvant chemotherapy, because of its side effects.
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PMID:[Chemotherapy using cisplatin in bone and soft tissue sarcoma]. 346 52

Supra-labyrinthic tumours usually produce lesions of the Vth and VIth cranial nerves, whereas infra-labyrinthic tumours affect the sensory-motor nerves. Both types of tumour may reveal themselves by cochleo-vestibular disorders and/or middle ear symptoms. The author has treated a series of patients with such tumours and describes the most frequent types of intrapetrosal growth: cholesteatoma (12 cases), glomic tumours (11 cases), facial nerve tumours (3 cases), metastases (2 cases). Meningiomas can also be encountered, as well as rarer tumours (4 cases), such as embryonic sarcoma, chordoma, chondroma and chondrosarcoma, cavernous angioma, eosinophilic granuloma, solitary plasmocytoma and fibrous dysplasia of the petrous bone. Some signal symptoms (sudden deafness, mucous otitis media, paralysis of vocal cords) can be particularly misleading.
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PMID:[Signal symptoms in tumours of the petrous bone (author's transl)]. 627 61

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

We report a 24-year-old man who presented unilateral multiple cranial nerve involvements followed by progressive paraplegia. The patient expired after developing DIC and pneumonia. Post-mortem examination revealed Ewing's sarcoma originated in the pubic bone with extensive metastases including the clivus which was responsible for his cranial nerve lesions. The patient was well until 24 years of age when he noted an onset of pain and a mass in the pubic region. The histology of the biopsy specimen of the tumor suggested Ewing's sarcoma. He was treated with chemotherapy and local radiation. A year after, he noted an onset of nuchal pain, difficulty in tongue movement, dysarthria, deafness in the left ear, and diplopia. On admission to our hospital in July 1990, neurological examination revealed an alert and intelligent Japanese male in no acute distress. The olfactory to the trigeminal nerves appeared intact. He showed complete abducens nerve palsy, facial weakness, mild deafness, and weakness of the soft palate, the sternocleidomastoid muscle and the tongue, all on the left side. The remainder of the neurological examination was unremarkable except for dysesthesia along the left C8 and Th1 dermatoms. Radiological examination revealed a 10 x 10 cm sclerotic mass in the public bone and a high signal mass lesion between the clivus and the pons in the T2-weighted MRI. His clinical course was complicated by acute paraplegia with anesthesia below the Th4 dermatom, DIC, and respiratory distress due to plural effusion. Post-mortem examination revealed a necrotic and hemorrhagic tumor in the pubic bone. The histology was consistent with Ewing's sarcoma.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[A 24-year-old man presenting Garcin syndrome and paraplegia]. 847 71


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