Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027651 (tumor)
 685,946 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Middle ear adenoma (MEA) is a rare tumor postulated to take origin from the lining epithelium of the middle ear cavity. The authors report on a case of MEA arising in a 53-year old woman suffering from a sensation of fullness in her left ear, otalgia, and light left-sided hearing loss. Histopathologically, the lesion was composed of cuboidal and polygonal cells displaying a trabecular, tubulo-glandular, and solid pattern of growth. Immunohistochemically, neoplastic cells diffusely stained with anti-vimentin antibodies and were focally positive for chromogranin A, neuron-specific enolase, lysozyme, and cytokeratins AE1/AE3. The majority of tumor cells showed weak and diffuse staining with both anti-PP and anti-ACTH antibodies and intense positivity with anti-glucagon and anti Leu-7 antibodies. Ultrastructural investigation revealed both mucinous-glandular and neuroendocrine differentiation. The authors suggest that the appropriate terminology would be adeno-carcinoid or amphicrine tumor of the middle ear rather than "adenoma," a term that does not reflect its dual nature.
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PMID:Middle ear adenoma is an amphicrine tumor: why call it adenoma? 1129 23

Primary adenocarcinoma is a rare tumor of the middle ear and temporal bone; its most frequent symptoms are hearing loss, otalgia, and facial paralysis. Otoscopic examination of a 27-year-old man revealed purulent discharge in the ear canal, diffuse edema, and hypertrophy of the right tympanic membrane. He presented with a grade III (House-Brachman) facial paralysis and right conductive hearing loss with a history of aural discharge for 6 months, otalgia, and facial weakness for 2 days. Computed tomography of the temporal bone showed an opacity filling the tympanic cavity, antrum, and aditus. Tympanotomy revealed diffuse edema of the middle ear mucosa, and granulation tissue was encountered during mastoidectomy filling the antrum and periantral cells and eroding the fallopian canal at the level of the oval window. After the histopathological examination revealed papillary adenocarcinoma, a subtotal temporal bone resection, facial nerve segmenter resection, and end-to-end anastomosis of the facial with the hypoglossal nerves were performed. The importance of histopathological examination in all cases of chronic otitis media with granulation tissue is stressed.
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PMID:Primary papillary adenocarcinoma confined to the middle ear and mastoid. 1130 13

We report an extremely rare case of bilateral primary carcinoma of the external auditory meatus. A 50-year-old man suffered 1 month from left-ear discharge and otalgia. Examination revealed a tumor of the left ear canal and a biopsy showed well-differentiated squamous cell carcinoma. The patient underwent 60 Gy radiotherapy and left subtotal temporal bone resection. A tumor with irregular swelling of the right external canal was found 8 months after the first diagnosis. Biopsy of the right external canal confirmed the same squamous cell carcinoma. Genetic examination that the carcinoma of the right ear was probably not a metastatic from the carcinoma of the left ear.
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PMID:[Bilateral primary carcinoma of the external auditory meatus]. 1143 42

This report describes a patient with Von Hippel-Lindau disease revealed by an endolymphatic sac tumor. Endolymphatic sac tumor (EST) was only recently recognized as a manifestation of Von Hippel-Lindau (VHL) disease. EST are vascular lesions that destroy and expand bone. We report a recently treated case of an EST. A 30-year-old woman presented with otalgia and hearing loss. Computed tomography and magnetic resonance imaging showed typical features of an EST. We checked for VHL and found this disease in the patient. VHL disease is a hereditary cancer syndrome caused by germline mutations of the VHL tumor suppressor gene. A molecular diagnosis of VHL is nowadays available, and this has change the clinical management of patients and their families. Diagnosis of VHL has to be suspected in patients with a VHL-related tumor without familial history and especially in those cases of hemangioblastoma or endolymphatic sac tumors. Such patients should be systematically investigated for clinical and molecular evidence of VHL disease.
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PMID:[Endolymphatic sac tumor and von Hippel-Lindau disease. Review of the literature]. 1248 74

Garcin syndrome is characterized by a progressive ipsilateral involvement of cranial nerves, culminating in paralysis of all or at least seven of them, without sensory or motor long-tract disturbance, with no intracranial hypertension, and with osteoclastic involvement in the skull base on radiographic computed tomography. Giant cell tumor is a primary bone tumor rarely affecting the skull base. An 8-year-old female presented with a 3-month history of increasingly worsening right otalgia, tinnitus, hearing loss, right facial numbness, and diplopia. She was admitted with a 2-week history of swallowing difficulties, voice change, and right shoulder pain. Neurologic examination disclosed unilateral paralysis of the right fifth through twelfth cranial nerves, with no other abnormal neurologic findings. Skull radiographic computed tomography revealed lytic lesions in the right temporal petrous portion. Computed tomographic scan indicated a destructive mass involving the right greater wing of the sphenoid bone and temporal petrous apex. Magnetic resonance imaging demonstrated a tumor arising from the temporosphenoidal region, infiltrating neither the brain nor the brainstem. No hydrocephalus was observed. Biopsy revealed giant cell tumor. Posterior treatment consisted of radiotherapy. At an 8-year follow-up, the patient was well but with functional sequelae. There is no magnetic resonance imaging evidence of tumor growth. No other giant cell tumor presenting as Garcin syndrome is known to have been reported.
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PMID:Garcin syndrome resulting from a giant cell tumor of the skull base in a child. 1287 3

Middle ear (ME) carcinoid tumor is exceedingly rare. We present a case of ME carcinoid tumor in a 29-year-old woman whose chief complaints were right otalgia, aural fullness, hearing loss, and facial palsy of 5 days duration. A reddish bulging mass behind the right eardrum and right facial palsy staged as grade IV on the House-Brackmann scale were noted. The high-resolution computed tomography of the temporal bone showed a soft-tissue density mass in the right middle ear and antrum without bony invasion. Although the initial diagnosis was ME adenoma made by the intraoperative frozen section report, the tumor was resected completely by a modified conservative ossicles-preservation method. The patient's initial symptoms of otalgia, aural fullness, and hearing loss immediately improved after surgery, and facial palsy diminished in the following months. No recurrence was noted after 12 months of follow-up.
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PMID:Carcinoid tumor of the middle ear: a case report. 1563 84

The adenoid cystic carcinoma arising in the external auditory canal is a rare tumor, as other malignant neoplasias of the EAC and middle ear. The early diagnosis of these malignant tumors of the ear is very important to improve the prognosis. In this sense, the clinic experience is frequently the most useful diagnosis instrument to suspect this pathology when we have a confrontation with chronic otorrea and otalgia, common presentation form of this disease. In this report we show a new case of adenoid cystic carcinoma of external auditory canal and review the most significant medical literature existing in relationship with this pathology.
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PMID:[The adenoid cystic carcinoma]. 1600 90

We present a rare case of temporal bone angiosarcoma diagnosed in a 26-year-old female patient at 36 week of pregnancy. The patient was referred with a 2 months history of left otalgia and tinnitus with a tender swelling above the mastoid. Cranial imaging studies showed a 7 x 5 x 4 cm hypervascularized mass located in the left middle fossa with lysis of the temporal bone and extension to the subcutis. After the baby was delivered by caesarean section, the patient entered the oncology protocol. Selective embolization of the feeding vessels was followed by gross total surgical resection using a combined supra- and infra-tentorial approach. Pathological findings were those of a poorly differentiated, highly malignant sarcoma with a large epitheloid component and immunohistochemical evidence of endothelial differentiation (CD31, Factor VIII related antigen, CD34), consistent with an angiosarcoma with epitheloid features. No extra-cranial tumor was found after extensive staging. The patient received adjuvant radiotherapy followed by a course of chemotherapy consisting of 6 cycles of paclitaxel. At 15 months follow-up, she developed multiple distant metastasis to a left postauricular lymph node and to the lungs and ribs. The patient was given a second line chemotherapy using doxorubicine and ifosfamide. Despite an initial good response, she died with metastatic disease 26 months after diagnosis. We present a rare case of primary temporal bone angiosarcoma and report our experience with a multimode therapeutic approach combining surgery, radiotherapy and chemotherapy.
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PMID:Primary temporal bone angiosarcoma: a case report. 1613 18

We report a case of capillary hemangioma of the tympanic membrane in a 51-year-old man with conductive hearing loss, otalgia, and otorrhea. On examination, the external auditory canal was obstructed with a hard, dark red tumor, and purulent discharge was observed. The infection was controlled with antibiotics, but hearing loss persisted. Therefore, transcanal resection was performed. The tumor was attached to the postero-superior quadrant, and arose from the lamina propria. Histologically, the tumor was composed of multiple capillary-sized vessels surrounded by fibrous connective tissue and diagnosed as capillary hemangioma. After surgical treatment, hearing loss was diminished, and there has not been any sign of recurrence for 3 years despite the positive surgical margin.
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PMID:Capillary hemangioma of the tympanic membrane. 1613 37

Pleomorphic adenoma (PA) rarely occurs in the external auditory canal (EAC). A case of PA complicated with chronic otitis media is reported. The patient was a 36-year-old male who began to experience a hearing loss in the left ear in 1996. He sought medical attention only after ear pain developed in April 2003. At the initial examination, a tumor covered by smooth skin was seen filling the left EAC. Pure-tone audiometry showed residual hearing at low frequencies and scale-out across middle to high frequency range. The CT and MRI findings led to a diagnosis of a tumor of the left EAC complicated with an inflammatory middle-ear lesion. The tumor was excised en bloc with the overlying skin, and tympanoplasty was performed. The pathological diagnosis was PA of the left EAC and inflammatory granuloma of the middle ear.
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PMID:Pleomorphic adenoma of the external auditory canal complicated by hearing loss secondary to chronic otitis media. 1630 67


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