1/113. Neuromas and prominent corneal nerves without men 2B.PURPOSE: We studied a family composed of 2 members with the characteristic phenotype of the men 2B and without RET protooncogene mutations in order to determine whether they had multiple endocrine neoplasia associated with men 2B in the 5-year follow-up. SUBJECTS AND methods: The family consisted of a 15 year old female complaining of burning eyes, examined ophthalmologically in 1992 and her mother and sister, who were examined later on in 1992. The proband and the mother were affected with multiple mucosal neuromas and visible corneal nerves. pentagastrin-stimulated serum calcitonin levels, catecholamines, serum calcium and phosphate levels were measured. Molecular genetic studies were performed on the 2 affected members to look for the specific RET mutation seen in men 2B. RESULTS: Endocrine neoplasia of the syndrome men 2B, medullary thyroid carcinoma, pheochromocytoma and hyperparathyroidism, were ruled out in the first examination and after 5-year follow-up. In the 2 cases no mutation at codon 918 for the RET proto-oncogene was found. CONCLUSIONS: We consider that familial multiple mucosal neuromas are a highly distinctive entity of men 2B.- - - - - - - - - - ranking = 1keywords = multiple endocrine, multiple endocrine neoplasia, endocrine neoplasia, endocrine, neoplasia (Clic here for more details about this article) |
2/113. The blastic variant of mantle cell lymphoma arising in Waldeyer's tonsillar ring.We present three cases of blastic mantle cell lymphoma with an unusual initial manifestation in Waldeyer's ring with methods for differentiating it from other blastic neoplasms of the head and neck. All cases presented with a feeling of fullness in the area of the mass. Morphologically, the tumours were blastic with a high mitotic rate (three to nine per high power field). All were B-cell phenotype with coexpression of CD43. In all cases cyclin d1 and bcl-2 were positive and CD23 negative. Blastic mantle cell lymphoma occurring in Waldeyer's tonsillar ring may be mistaken for other high grade haematopoietic neoplasms. immunohistochemistry and awareness of this type of lymphoma are helpful in differentiating it from other neoplasms.- - - - - - - - - - ranking = 0.033704394843937keywords = neoplasm (Clic here for more details about this article) |
3/113. Evaluation and management of benign, non-congenital tongue masses in children.Lingual tumors are rare, primarily benign, lesions in the pediatric population. Congenital lesions, such as hemangiomas, lymphatic malformations, dermoids, hamartomas and thyroglossal ducts cysts, are seen more commonly. Primary, non-congenital lingual neoplasms are less common in children. We present three patients with benign lingual neoplasms. Evaluation, management, pathology and follow-up are discussed.- - - - - - - - - - ranking = 0.022469596562625keywords = neoplasm (Clic here for more details about this article) |
4/113. Dedifferentiation occurring in adenoid cystic carcinoma of the tongue.A 61-year-old man came to the University of Granada School of dentistry complaining of a mass involving his ventral tongue. Histopathologic examination of the excised specimen showed adenoid cystic carcinoma in which cribriform and tubular patterns were observed, juxtaposed with an undifferentiated carcinoma, large-cell type. No cervical lymph node metastasis was present, and the patient is alive and free of disease 5 years after treatment. To our knowledge, no similar cases have been reported thus far, though other salivary gland malignancies have been described in association with undifferentiated carcinoma, especially in the parotid gland. These neoplasms have been highly aggressive, and the adequacy of the primary surgical resection may be critical in determining the ultimate prognosis and survival.- - - - - - - - - - ranking = 0.011234798281312keywords = neoplasm (Clic here for more details about this article) |
5/113. paraganglioma of the tongue.Paragangliomas are neuroendocrine tumors arising from extra-adrenal autonomic ganglia. We present what is to our knowledge the first immunohistochemically documented case of a paraganglioma of the tongue, a 2.5-cm benign tumor growing in the position of the foramen cecum. The patient was an elderly woman who presented with throat irritation. The histologic profile and ultrastructural appearance of this lesion were classic for paraganglioma, and cells stained strongly for neurosecretory granules by immunohistochemistry. Carcinoid, ectopic thyroid neoplasm, and other tumors that mimic paraganglioma were ruled out on immunohistochemical grounds. To our knowledge, paraganglia have not been previously documented in the tongue; this tumor may have arisen from a branch of cranial nerve VII or IX, or from an embryologic remnant of the thyroid or thyroid capsule.- - - - - - - - - - ranking = 0.024036392788604keywords = endocrine, neoplasm (Clic here for more details about this article) |
6/113. Nerve sheath myxoma (neurothekeoma) in the tongue of a newborn.Nerve sheath myxoma is a benign peripheral nerve sheath tumor that rarely occurs in the oral cavity; experience with these lesions is therefore limited. The lesion described in this report appeared clinically as a gradually enlarging, painless growth arising on the tongue of a newborn girl. Microscopically, the lesion was characterized by nodules of spindle-shaped cells with abundant myxoid stroma. Immunohistochemical studies were consistent with a nerve sheath neoplasm.- - - - - - - - - - ranking = 0.011234798281312keywords = neoplasm (Clic here for more details about this article) |
7/113. Cowden's syndrome (multiple hamartoma and neoplasia syndrome): diagnostic dilemmas in three cases.Cowden's syndrome is a multisystem disease inherited as an autosomal dominant trait with incomplete penetrance and variable expression. The disease has typical oral manifestations which often precede more systemic involvement, and the dental professional is therefore well placed to institute a regime of regular checks to ensure early treatment of any neoplasms which may occur. However, since not all of the classical signs are present in all patients, diagnosis may be difficult. The case report of a patient with most of the features of Cowden's syndrome is presented and features compared with two other possible cases.- - - - - - - - - - ranking = 0.058038573004645keywords = neoplasia, neoplasm (Clic here for more details about this article) |
8/113. Subglottic hemangioma associated with cutaneous and cerebellar hemangiomas detected by MRI: report of one case.Subglottic hemangioma (SGH) is a benign neoplasm that may cause severe and life-threatening respiratory obstruction in infants. However, patients usually present with inspiratory stridor in the first few months of life and may be mistakenly diagnosed as recurrent or persistent croup. Definitive diagnosis is made by image studies, endoscopic examination and biopsy or all. We report a 2-month-old female infant of SGH with initial clinical manifestations of dyspnea and inspiratory stridor co-existing with cutaneous and cerebellar hemangiomas. Clinicians must be alert the possibility of SGH when associated with cutaneous hemangioma. This patient has received oral steroid treatment for more than two months with improvement of the airway obstruction. Although purplish patch lesions over left side of face, eyelid, cheek, and peri-oral regions regressed, the size of the SGH on the followed MRI was slightly enlarged. The diagnosis and various treatments of SGH are discussed and reviewed in this paper.- - - - - - - - - - ranking = 0.011234798281312keywords = neoplasm (Clic here for more details about this article) |
9/113. From eyelid bumps to thyroid lumps: report of a men type IIb family and review of the literature.PURPOSE: We present a two-generation family with multiple endocrine neoplasia (men) type IIb diagnosed by their ophthalmologists based on characteristic ophthalmic findings. methods: A family consisting of a 33-year-old female proband and her 8- and 7-year-old children had prominent corneal nerves; eyelid, lip, and tongue nodules; and a characteristic facies. A polymerase chain reaction-based genetic assay was obtained to detect the genetic mutation most commonly associated with men type IIb. serum calcitonin and urine catecholamine studies were obtained. RESULTS: Molecular genetic studies detected in all 3 patients a mutation at codon 918 of the RET proto-oncogene known to be present in 95% of the cases of men type IIb. serum calcitonin was elevated in the proband and her son. urine catecholamine levels were elevated in the proband. Surgical treatment and histologic analysis confirmed pheochromocytoma and medullary thyroid carcinoma (MTC) in the proband. Surgical exploration revealed the MTC to be metastatic to the liver. CONCLUSIONS: This family demonstrates the characteristic findings of men type IIb: prominent corneal nerves in a clear stroma and multiple submucosal neuromas of the conjunctiva, eyelids, lips, and tongue. Ophthalmologists have a critical role to play in recognizing these signs, because the early diagnosis of medullary thyroid carcinoma and pheochromocytoma may be life saving.- - - - - - - - - - ranking = 0.98829905631917keywords = multiple endocrine, multiple endocrine neoplasia, endocrine neoplasia, endocrine, neoplasia (Clic here for more details about this article) |
10/113. myoepithelioma presenting as a midline cystic tongue lesion: cytology, histology, ancillary studies, and differential diagnosis.Salivary gland myoepithelioma (ME) is a neoplasm derived from myoepithelial cells that lacks the ductal and broad mesenchymal differentiation seen in the vast majority of mixed tumors. This report describes the cytologic findings of a cystic ME presenting in the midline of the dorsal tongue, a site where no salivary glands are generally present. The tumor was well circumscribed and composed of sheets of monotonous epithelioid cells without ductal cells. The cells were positive for S-100 protein and ultrastructurally had features of myoepithelial cells. The fine needle aspiration (FNA) biopsy findings, differential diagnosis, histology, immunohistochemistry, and electron microscopic features of this interesting and uncommon neoplasm are presented. To the best of our knowledge, there have been no cytologic reports of ME of the tongue.- - - - - - - - - - ranking = 0.022469596562625keywords = neoplasm (Clic here for more details about this article) |
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