1/36. E.N.T. manifestations of Von Recklinghausen's disease.Von Recklinghausen's disease (VRD) is a neurocutaneous, systemic disease characterized by CNS tumors and disorders, cafe-au-lait spots, generalized cutaneous neurofibromata, skeletal deformities, and somatic and endocrine abnormalities. It is an autosomal dominant, hereditary disorder found in approximately 1:2500 to 3300 births. There are many manifestations of this disease in the head and neck region of interest to the otolaryngologist. case reports of three patients with multiple ENT involvements are detailed. A review of the literature is presented with a brief discussion of diagnosis and treatment. The most common intracranial tumor in the adult is the acoustic neuroma, usually bilateral, while in the child it is the astrocytoma. A defect in the sphenoid bone is common and may produce temporal lobe herniation into the orbit causing pulsatile exophthalmos. Involvement of the facial bones usually causes radiolucent defects secondary to neurofibromata within nerve pathways, and a variety of asymmetrical changes, especially within the mandible. "elephantiasis" of the face is a hypertrophy of the soft tissues overlying a neurofibroma, often quite extensive and disfiguring. Laryngeal and neck involvement may compromise the airway and early and repeated surgical intervention is required. The over-all malignancy rate approaches 30%, indicating that the patient with VRD may be predisposed to developing a malignancy. There appears to be an increased surgical risk in these patients, with some demonstrating abnormal responses to neuromuscular blockade.- - - - - - - - - - ranking = 1keywords = neuroma (Clic here for more details about this article) |
2/36. Plexiform schwannoma of the cheek.We present a case of subcutaneous plexiform schwannoma, a rare benign peripheral nerve sheath tumour characterized by a multinodular and plexiform growth pattern. A review of the literature was performed to identify the relationship between plexiform schwannoma and neurofibromatosis types 1 and 2, and schwannomatosis. It is also important to distinguish plexiform schwannoma from plexiform neurofibroma, a particular type 1 neurofibromatosis lesion, because of the propensity of the latter for malignant degeneration.- - - - - - - - - - ranking = 13.222585761784keywords = schwannoma (Clic here for more details about this article) |
3/36. Primary malignant melanoma of the oral cavity: case report.An 80-year-old-female patient had a pigmented lesion on: the hard palate, the soft palate, the alveolar mucosa and the vestibular mucosa of the maxillary gingiva. Pigmented macules and patches had been persistent and asymptomatic for many years (Fig. 1). The lesion exhibited irregularities of pigmentation, border and surface contour. About 1 year later the patient had noticed an extension of the pigmented macules and plaques; there was also the appearance of nodules of the maxillary gingiva accompanied by swelling. Loosening of teeth as a result of extensive destruction of bone was further noted (Fig. 2). The histological examination showed a downward streaming in the dermis of the tumor cells and a disintegration and ulceration of the epidermis (Fig. 3). An increased number of large round or polygonal cells resembling atypical epithelioid cells were found on the submucosa. The atypical cells had enlarged, pleomorphic nuclei with prominent and sometimes multiple nucleoli. Mitoses were observed at various tissue levels (Fig. 4). Abundant pigmented melanin was present in the tumor cells (Fig. 5). Many cells had fine, dusty melanin particles. The tumor cells showed great variations in size. Immunohistochemical staining, with S100 protein and HMB45 antibodies, stained many of the spindleshaped cells, indicating that they were melanocytic cells. An inflammatory infiltrate of lymphocytes was seen in a band beneath the invading tumor cells.- - - - - - - - - - ranking = 0.32718864072861keywords = melanocytic (Clic here for more details about this article) |
4/36. Cellular schwannoma in the oral mucosa.A case of cellular schwannoma of the oral mucosa in a 34-year-old Japanese man is described. Cellular schwannoma commonly affects soft tissues such as the retroperitoneum and posterior mediastinum, and also bone, but is extremely rare in the oral region. To our knowledge, this is only the second report of oral cellular schwannoma. Histologically, the tumor parenchyma consisted of hypercellular spindle cells with nuclear and cytoplasmic pleomorphism and nuclear palisading resembling Antoni A-type conventional schwannoma, without evidence of Verocay bodies. These features were indicative of cellular schwannoma. Immunohistochemically, the tumor cells were positive for S-100, S-100alpha, S-100beta and vimentin, suggesting that they were of peripheral nervous origin. Furthermore, it is speculated that the tumor was intermediate between a benign and a malignant state, based on the histological features and positivity for S-100alpha.- - - - - - - - - - ranking = 14.875408982007keywords = schwannoma (Clic here for more details about this article) |
5/36. Epithelioid blue nevus of the oral mucosa: a rare histologic variant.Epithelioid blue nevus (EBN) is an extremely rare histologic variant of blue nevus that has only recently been identified. Unlike other variants of blue nevus, which primarily are composed of pigmented, spindle-shaped melanocytes, EBN is characterized by large, well-defined, heavily-pigmented polygonal or epithelioid-shaped melanocytes intermixed with less densely pigmented epithelioid- and fusiform-shaped melanocytes. Furthermore, in contrast to other benign melanocytic proliferations, the lesional cells in EBN exhibit little or no maturation as they extend deeper into the underlying tissue. Blue nevi are the second most common form of nevus in the oral cavity. However, to our knowledge, the epithelioid variant has not been previously identified in the mouth. Only 6 examples of EBN have been identified in the skin of the head and neck. We now report the first documented case of EBN involving the oral mucosa. A brief review of the clinical and histopathologic features of EBN is also presented.- - - - - - - - - - ranking = 0.32718864072861keywords = melanocytic (Clic here for more details about this article) |
6/36. Sporadic sclerotic fibroma of the oral soft tissues.Sclerotic fibroma (SF) is an uncommon, benign fibrous neoplasm that may present either as a sporadic, small, solitary cutaneous mass, in otherwise healthy individuals, or as solitary or multiple, discrete skin nodules in patients with Cowden syndrome. Oral SF has been reported in patients with Cowden syndrome; however we now report the first documented series of sporadic SF originating within the oral mucosa. We describe 5 cases of SF arising in 3 women and 2 men with an age range of 43 to 66 years. The buccal mucosa was the site of involvement in 4 patients and the lower lip in 1 patient. Microscopically, each of the tumors was characterized by an unencapsulated, well-circumscribed, hypocellular submucosal nodule that was sharply demarcated from the surrounding tissues. The neoplasms were primarily composed of thick collagen bundles that were occasionally arranged in a storiform pattern. Prominent clefts separated many of the collagen bundles. In all cases, spindle and stellate-shaped cells containing fusiform or stellate-shaped nuclei and inconspicuous nucleoli were found scattered throughout the lesion. Occasional stellate-shaped, multinucleated cells were also seen. Many of the cells also exhibited long dendritic cytoplasmic processes. The tumor cells strongly expressed CD34 and vimentin, and occasionally factor xiiia, but were negative for markers of myofibroblastic, neural or melanocytic differentiation. These findings confirm that oral SF represents a unique entity and should be differentiated from more commonly occurring benign fibrous lesions of the oral soft tissues.- - - - - - - - - - ranking = 0.32718864072861keywords = melanocytic (Clic here for more details about this article) |
7/36. Solitary mucosal neuroma of the hard palate: a case report.Mucosal neuroma is rare neurogenic tumour that arises in the oral cavity as a component of multiple endocrine neoplasia type 2b (MEN 2b). We present the case of a histologically confirmed solitary mucosal neuroma of the hard palate in a 49-year-old woman with no other components of MEN 2b.- - - - - - - - - - ranking = 6keywords = neuroma (Clic here for more details about this article) |
8/36. Schwannoma in the sublingual space.In this report, we present CT and MRI findings of a case of a schwannoma that developed in the floor of the oral cavity. A 49-year-old woman visited our hospital with a painless swelling in the oral floor. CT and MRI revealed a well circumscribed oval mass in the sublingual space, which showed cystic degeneration in most of the lesion. In addition, a thickened wall that strongly enhanced after injection of contrast medium and formation of fluid level were observed in the mass. The mass was removed and was histopathologically diagnosed as schwannoma. Only a few cases of schwannoma in the oral floor have been reported. However, when the characteristic findings are observed on CT and MRI, schwannoma should be added to the differential diagnosis.- - - - - - - - - - ranking = 6.6112928808918keywords = schwannoma (Clic here for more details about this article) |
9/36. Oral melanoacanthosis (melanoachantoma): report of a case and review of the literature.Oral melanoacanthosis (MA) is a rare pigmented mucosal lesion that is considered the counterpart of cutaneous melanoacanthoma. Microscopically the superficial epithelium shows mild to moderate acanthosis, spongiosis and prominent dendritic melanin producing melanocytes, which are present throughout the spinous keratinocytes. Reported cases show predilection for black females and the most common locations in decreasing frequency are buccal mucosa, lip, palate and gingiva. Although its pathogenesis remains uncertain, its clinical behavior is suggestive of a reactive origin. The clinical appearance of oral MA is non diagnostic and therefore biopsy is mandatory to differentiate from other melanocytic lesions, including melanoma.- - - - - - - - - - ranking = 0.32718864072861keywords = melanocytic (Clic here for more details about this article) |
10/36. Pigmented mucoepidermoid carcinoma of the oral cavity: a case report.melanocytes are normally present in the oral mucosa, but unlike the skin, melanocytic colonization and pigmentation of nonmelanocytic tumors rarely occur. A case of pigmented mucoepidermoid carcinoma (MEC) in a 36-year-old woman is reported. On H&E-stained sections, brownish granules were seen in intermediate and epidermoid neoplastic elements and in elongated or dendritic cells showing histologic and immunohistochemical features of melanocytes. Ultrastructurally numerous epithelial cells contained large aggregates of melanin granules, a feature suggestive of phagocytosis. In addition small individual melanin granules were observed at the external border of the epithelial cell membrane. These features suggest that melanocytes colonized the MEC and that neoplastic cells may phagocytose the granules, similarly to what occurs with epidermal melanocytes. The phenomenon of melanocyte colonization of nonmelanocytic tumors should be taken into consideration in dealing with pigmented lesions of the oral cavity.- - - - - - - - - - ranking = 0.98156592218583keywords = melanocytic (Clic here for more details about this article) |
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