Cases reported "Ear Neoplasms"

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1/27. Acoustic intrameatal meningiomas.

    BACKGROUND: The sporadic finding of an acoustic intrameatal meningioma stimulated the authors to the present study. An analysis of the cases previously reported in the literature aimed to outline a preliminary account about biological, radiological and surgical specific hallmarks of these tumours. methods: Eight previous cases of meningiomas, meeting the prerequisite of origin and situation within the internal acoustic canal, have been discovered in the known literature since 1975. A further case was recently observed in our experience. The cases in the series showed no sex prevalence and in most of them the age of incidence was comprised between the fifth and sixth decade of life. hearing loss was the prevalent symptom, lasting 1 month to 7 years before presentation. Myelocisternography, myelo-CT or high resolution CT/MR revealed no specific radiological features to distinguish small intrameatal meningiomas from the more frequently occurring vestibular schwannomas, while CT scan with bone algorithm could point out valuable indirect details for differential diagnosis. Various surgical approaches, i.e. middle fossa, translabyrinthine and retromastoid, were utilized by the different authors. RESULTS: Basing on apparent individual surgical preference, one of three different surgical routes (translabyrinthine, middle fossa, retromastoid) was chosen for 10 procedures in 9 patients. In all, except two cases the impression at surgery was of complete tumour removal. CONCLUSIONS: The possibility for meningiomas to recur and invade the surrounding bone requires a differential diagnosis from vestibular schwannomas. In the absence of intrinsic distinctive signs, radiological evaluation of peritumoral bone alterations could help diagnosis. Although the various surgical routes have often proved effective, temporal bone invasion justifies more extensive approach even in small tumours.
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2/27. Ceruminous gland tumours: a reappraisal.

    Ceruminous glands should no longer be regarded as purely apocrine glands, but as apoeccrine glands with both apocrine and eccrine modes of secretion. We present two cases of pleomorphic adenoma of ceruminous glands, among the rarest of such tumours. The use of such terms as 'ceruminoma' and 'hidradenoma' should finally be abandoned, and 'ceruminous gland tumour' used instead as a generic term. classification should be based on Wetli's prototype (adenoma, pleomorphic adenoma, adenoid cystic carcinoma and adenocarcinoma), with the addition of benign eccrine cylindroma and syringocystadenoma papilliferum; the inclusion of mucoepidermoid carcinoma should await full validation. Wide local excision is necessary for all tumours, with only follow-up for histologically benign neoplasms. Malignant tumours need early aggressive surgery and radiotherapy. If marginal invasion cannot be assessed histologically, then adenoma and adenocarcinoma cannot be distinguished and we suggest that the tumour be reported as 'of uncertain malignant potential'. Long-term studies are needed to confirm or refute the view that all ceruminous gland tumours are potentially malignant.
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3/27. Syringocystadenocarcinoma papilliferum: successfully treated with Mohs micrographic surgery.

    BACKGROUND: Syringocystadenocarcinoma papilliferum, a rare sweat gland carcinoma, is the malignant counterpart of syringocystadenoma papilliferum. OBJECTIVE: To demonstrate a rare case of syringocystadenocarcinoma papilliferum successfully treated with Mohs micrographic surgery. methods: A 60-year-old male presented with two verrucous plaques on his right auricle since childhood. These two plaques became ulcerated, more painful, and pruritic in 1 year. Histopathologic examination revealed syringocystadenocarcinoma papilliferum. RESULTS: Mohs micrographic surgery with reconstruction of right auricle was performed subsequently. There are no signs of recurrence or metastasis 6 years after operation. CONCLUSION: Syringocystadenocarcinoma papilliferum can be successfully treated with Mohs micrographic surgery.
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4/27. A solitary fibrous tumor in the external auditory meatus.

    We herein describe a rare case of a spindle-cell neoplasm arising in the external auditory canal. A 38-year-old man presented with a progressive swelling of the entrance of left external auditory meatus. The patient underwent a surgical removal of the tumor. A light microscopic study showed a spindle-cell proliferation with a collagenous stroma and a staghorn-like vascular pattern. The tumor cells were immunohistochemically positive for vimentin and CD34. The tumor was thereafter diagnosed to be a solitary fibrous tumor (SFT). SFTs most commonly occur in the pleura and are supposed to originate from submesothelial connective tissue. Although, several cases of extrapleural SFT have been reported, no SFT arising in the entrance of left external auditory canal have ever been reported in the literature. As a basically benign tumor, it occasionally relapses or metastasizes after excision, and therefore a careful follow up is necessary.
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5/27. Recurrent adult myofibromatosis. A case report.

    myofibromatosis generally is regarded as a fibrous proliferative disease of neonates and children that, even when multicentric, has an excellent prognosis and a propensity for spontaneous involution. Reported is a case of solitary, locally recurrent myofibromatosis in the ear canal of an adult patient. It was initially thought to be a leiomyoma. The diagnosis of myofibromatosis was made on the basis of its histologic appearance and corroborated by immunohistochemical stains. Clinicians and pathologists should be aware that myofibromatosis may occur in adults in unusual locations, with a potential for local recurrence. Furthermore, its histologic appearance may mimic that of other mesenchymal neoplasms.
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6/27. angiolymphoid hyperplasia with eosinophilia associated with pregnancy: a case report and review of the literature.

    A case of angiolymphoid hyperplasia with eosinophilia (ALH) is reported in a 33-year-old woman who developed an auricular nodule during the second trimester of her pregnancy. angiolymphoid hyperplasia with eosinophilia usually occurs on the head and neck of young adults and is more common in women than in men. Characteristic histologic features of ALH present in this case included proliferation of thick-walled blood vessels lined by prominent endothelial cells, infiltration of the interstitium by chronic inflammatory cells (mainly eosinophils), and presence of lymphoid follicles with germinal centers. The auricular tumor was completely excised. Thirteen months after excision, the patient remains tumor free. Although there are not many case reports on ALH during pregnancy or involving use of oral contraceptive pills, sex hormones may play a role in the pathogenesis of ALH. This hypothesis, in the context of cases previously described in the literature, and the differential diagnosis of ALH are discussed.
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7/27. magnetic resonance imaging of syringocystadenoma papilliferum of the external auditory canal.

    Syringocystadenoma papilliferum (SCAP) usually occurs on the face or the scalp and is very rare in the external auditory canal (EAC). There has been no information on magnetic resonance (MR) imaging of this tumor irrespective of its site. We report here a case of 57-year-old man having this tumor, which was surgically removed and its histopathology was confirmed. MR imaging demonstrated a lobulated 4-cm mass with clearly defined margins in the EAC. Although the tumor was bulky, these MR findings were different from the malignancies. The mass lesion showed intermediate signal intensity both on T1- and T2-weighted MR images and showed slight enhancement on gadolinium-enhanced T1-weighted images. Signal intensities on T2-weighted images of this tumor were low compared to those of pleomorphic adenoma. All ceruminous gland tumors including SCAP are thought to be potentially malignant; therefore, pre-operative biopsy should not be performed. Even though incisional biopsy is sometimes needed as in our case, the current MR features would be helpful for differential diagnosis of this rare condition and assessing the extension of the tumor.
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8/27. Tubular apocrine adenoma with syringocystadenoma papilliferum arising from the external auditory canal.

    Tubular apocrine adenoma (TAA) is a very rare sweat gland tumour. Comprehensive review of the literature reveals that TAA in the external auditory canal (EAC) has not yet been reported. We report a case of TAA in the EAC, together with characteristic histopathological findings.
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9/27. Cancer patients with large defects. Reconstructional options: a case study.

    We report a case of a seventy-five years old male patient with a squamous cell carcinoma (SCC) originated from the right external ear four years ago. He was undergone surgical removal of the lesion with a combination of modified neck dissection and reconstruction with the use of pectoralis major flap. Furthermore, he had radiotherapy with 6000 rads of the right temporal region. Two months ago the patient showed an extended recurrence concerning the temporal muscle and bone, the lithoid bone, the masseter and the pterygoid muscles, the right part of the mandible, the parotid gland with the facial nerve, and the superior bulb of the internal jugular vein. He had a surgical removal of the lesion in extended healthy margins and functional and esthetic reconstruction of the defect with a combination of metal fixed prosthesis of the condyle and the right mandible and the use of myocutaneous trapezius flap. This is a case report of the reconstruction options we have nowadays to provide quality of life in cancer patients.
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10/27. Tissue culture, electron microscopic and enzyme histochemical investigations of extraadrenal paragangliomas.

    light and electromicroscopical as well as histochemical investigations were performed on three cases of extraadrenal paragangliomas. They were localized in the carotid body, tympanicum and cauda equina region. Tissue of two cases was cultivated in vitro in nutrient medium TCM 199. The tumours were classified as paragangliomas of the paraganglionic type with typical cell clusters, of the adenomatous and angiomatous type. The enzyme histochemistry showed a very high dehydrogenase activity. Ultrastructurally numerous typical osmiophilic granules could be observed in the cytoplasm of the tumour cells. In tissue culture only a minimal cellular proliferative activity could be detected. The few proliferating cell colonies showed mostly characteristics of epithelial tissue and sometimes a similar behaviour to cells of a ganglioneuroblastoma. The minimal proliferative activity in vitro is in good agreement with the proliferative behaviour of the extraadrenal paragangliomas in vivo.
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