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)

Nineteen temporal bones were examined from 11 patients who had metastatic temporal bone disease from a distant primary. The salient clinical features were: the high incidence of occult temporal bone involvement (7 of the 10 clinically documented cases), the considerable incidence of melanoma (3 of 10) and the variable correlation between clinical findings and pathologic localization of tumor in the temporal bone. Pathologic examination revealed two distinct modes of tumor spread within the temporal bone: 1) vascularosseous (petrous apex, mastoid, middle ear, external canal); and 2) perineural (nerves in IAC branches, labyrinthine endorgans). Every case was involved by one or both or these routes and no case of CSF-borne metastasis to the perilymphatic space was seen. The external canal was involved extensively in spite of an intact tympanic membrane. Since the presence of symptomatic or occult metastases in the temporal bone affects treatment and prognosis, they must be actively sought by the clinician.
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PMID:Metastatic tumors in the temporal bone--a pathophysiologic study. 37 58

Two patients with primary adenocarcinoma of the middle ear displayed symptoms in the affected ear many years before their condition was diagnosed as adenocarcinoma. Treatment included simple extirpation of the tumor, radical mastoidectomy with or without radiation. A review of the literature shows 11 cases reported previously. Follow-up of the patients for more than four years revealed four deaths in the reported cases-one from causes unrelated to the tumor and the others showing only local invasion without distant metastases. These tumors grow slowly and have a low-grade of malignancy.
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PMID:Primary adenocarcinoma of the middle ear. 114 28

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

Carcinoid tumors are rare in the middle ear. To our knowledge, only 17 cases could be found in the literature, the first of which was described in 1980. In addition to enlarging on a previous observation we present three new cases. The neoplasms showed a striking, heterogeneous aspect ranging from solid trabecular to tubuloglandular growth patterns resembling the classic carcinoid tumor and adenomatous middle ear tumor, respectively. Based on immunohistochemistry and electron microscopy, three cell types were found. A review was made of our four patients and the cases described in the literature. The medical histories ranged from 1 month to 9 years. Presenting symptoms and signs were not characteristics, but hearing loss predominated. In two patients, the eardrums were perforated, in all the others it was intact and often bulging. Surgery, usually radical mastoidectomy, was performed in all cases. Often the tumor encased the ossicular chain, without infiltration. In two patients, local recurrence occurred that was treated successfully with surgery. All the cases showed an indolent biological course and the tumors were clinically nonfunctional, despite the recognition of biogenically active products by immunohistochemistry. To our knowledge, regional or distant metastases have never been reported. Conservative surgery with radical removal of the primary or recurrent tumor is the treatment of choice.
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PMID:Primary carcinoid tumors of the middle ear. Report on four cases and a review of the literature. 144 96

A case is described of embryonal rhabdomyosarcoma (E.R.) of the middle ear in a 4-year-old child; survival has been over 9 years. R.E. is the most common malignant tumor of the auricular region in children and is most often fatal due to locoregional extensions or secondary metastases carried through the bloodstream and lymphatic systems. The basis for treatment is a multidisciplinary approach to the disease: surgery with as broad an exeresis as possible; radiotherapy with tumor-killing doses of 5,500/6,000 rads; and polychemotherapy (Vincristina, Endoxan, Methotrexate). Such "aggressive" treatment often results in a high rate of morbidity with complications involving the blood, bones, eyes and meninx often requiring temporary suspension of treatment and prolonged hospitalization.
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PMID:[Embryonal rhabdomyosarcoma of the middle ear: description of a case with long-term survival]. 226 Apr 43

One case of primary adenocarcinoma of the middle ear is reported, which developed from a chronic otitis media. Histological examination established the diagnosis of adenocarcinoma. Its clinical appearance was benign, being restricted to the middle ear and tympanic membrane and with no bone destruction or metastases. Two years after local surgery there was no evidence of recurrence.
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PMID:[Primary adenocarcinoma of the middle ear]. 232 60

From 1962-1984, ten children were referred to St. Jude Children's Hospital with a metastatic poorly differentiated malignancy; extensive diagnostic workup had failed to disclose the site of the primary tumor. Multiple skeletal metastases as well as bone marrow involvement were common findings. Erythrocytes were detected in the cytoplasm of tumor cells in several cases, and cytochemical stains confirmed that these phagocytic cells did not have features of mononuclear phagocytes. Establishing a pathologic diagnosis in these cases was difficult, and most special studies including cytochemistry and electron microscopy were not helpful in elucidating the diagnosis. A diagnosis of rhabdomyosarcoma was made at presentation in six cases. In the remaining cases, the diagnosis of rhabdomyosarcoma was subsequently made after rebiopsy of new tumor masses during the course of the illness, by ultrastructural examination of a cell line derived from the tumor or at postmortem examination. Based on initial symptoms, clinical features, and postmortem findings, the primary tumor sites were assumed to be in the middle ear, paravertebral area, base of skull, retrobulbar space, chest wall, and retropancreatic area. In four patients the disease was confined to bone marrow, lymph nodes, and meninges so that a primary site could not be assigned. The approach to pediatric patients presenting with disseminated malignancy from an occult primary site should consist of an aggressive pursuit of a specific diagnosis and establishment of a primary site to better direct therapy, particularly for those children whose tumors may be responsive to specific therapy.
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PMID:Rhabdomyosarcoma, presenting as disseminated malignancy from an unknown primary site: a retrospective study of ten pediatric cases. 291 73

Carcinoid tumours belong to the rarer neoplasms of the middle ear. In 1980, Murphy et al. described the first case of a carcinoid tumour of the middle ear. Only five cases could be traced in the literature. The light and electron microscope features of a primary atypical carcinoid tumour of the middle ear are presented and compared with those described in the literature. Primary carcinoid of the middle ear appears to be derived from the epithelial lining of the middle ear. Distant metastases have not been reported. The duration and nature of the symptoms are of little diagnostic value. An atypical carcinoid tumour in the left ear of a 33-year-old woman is described.
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PMID:Carcinoid tumour of the middle ear. 292 60

MR imaging of 15 paragangliomas in 10 patients was compared with CT of 13 of the lesions in eight patients. All lesions were confirmed with angiography. All lesions were detected by MR and CT with the exception of one small glomus tympanicum tumor that was seen only in retrospect with MR. CT better demonstrated subtle osseous changes of the skull base and the relation of the tumor to the middle ear structures. MR better demonstrated the relation of the tumor to the adjacent internal jugular vein and carotid artery. The paragangliomas had a characteristic MR appearance based on their vascularity. Serpiginous areas of signal void representing high vascular flow were interspersed among areas of high signal intensity caused by slowly flowing blood and tumor cells. This "salt-and-pepper" pattern was seen in all lesions greater than 2 cm in maximal dimension. MR was therefore able to accurately characterize the tumors as highly vascular. Multiplanar imaging and good tissue contrast and anatomic detail permitted display of the relations of these neoplasms to surrounding carotid sheath vessels and to intracranial structures better than did CT. In this experience, the MR appearance of paragangliomas was quite characteristic and differed markedly from meningiomas, neuromas, and metastatic disease of the skull base.
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PMID:MR imaging of paragangliomas. 302 73

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


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