1/12. Multiple endocrine neoplasia syndrome--type 2b. Case report and review.The multiple endocrine neoplasia syndromes are an association of tumours of 2 or more endocrine glands. multiple endocrine neoplasia type 2b (men 2b) patients develop medullary thyroid carcinoma and pheochromocytomas as well as unique physical characteristics. Most commonly, MEN2b is inherited with an autosomal dominant pattern although sporadic cases are not uncommon. If untreated the disease may be lethal. The facial, oral and ocular characteristics are reliable markers of the disease. These patients give a history most commonly of slipped capital femoral epiphysis, hypertension and life-long diarrhoea and/or constipation. MEN2b is most commonly characterised by nodules on the anterior aspect of the tongue, thickened lips with nodules, thickened upper eyelids, broadened nasal bridge, thickened corneal nerves and dilated, symmetrical, pedunculated nodules on the cheek mucosa. The patient described has most of these characteristics. Radiographic features of the jaws which have not been previously described are reported. These include a markedly enlarged and bifurcated inferior alveolar canal and shortened roots of the lower incisor teeth. Due to the lethality of the disease, patients who present with the above physical characteristics must be further investigated to exclude MEN2b.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
2/12. Multiple endocrine neoplasia type III.The multiple endocrine neoplasia (men) syndromes are autosomally dominant inherited disorders in which hyperplastic or neoplastic changes occur in a wide variety of tissues. The specific syndromes are classified according to the endocrine glands affected. men type I consists of an aggregation of tumors of parathyroid, pancreatic, and pituitary glands. The association of medullary carcinoma of the thyroid (MCT) and pheochromocytoma is called men type II or type IIA, and if combined with mucosal neuromas, intestinal ganglioneuromatosis, and prominent corneal nerves, is named men type III or type IIB. Individuals afflicted with men type III are characterized by a marfanoid habitus, mucosal neuromas involving oral and ocular tissues, and a number of ophthalmologic findings including prominent corneal nerves, thickened eyelids, and subconjunctival neuromas. These features are easily recognized during the ocular exam, allowing the ophthalmologist to make an early diagnosis of this syndrome prior to the onset of life-threatening manifestations like medullary thyroid carcinoma and pheochromocytoma.- - - - - - - - - - ranking = 2keywords = gland (Clic here for more details about this article) |
3/12. Surgical treatment of medullary carcinoma of the thyroid.Medullary thyroid carcinoma, comprising approximately 7% of thyroid carcinoma, produces calcitonin, which can be monitored by immunoassay for diagnosis, at preclinical stages, and for persistent disease and its extent. It presents as sporadic and hereditary forms. The latter consists of the multiple endocrine neoplasia (men)-2A syndrome, which includes pheochromocytomas and hyperparathyroidism in some families, and the men-2B syndrome, which consistently includes mucosal neuromas and somatic features. The carcinoma, especially the men-2B variety, is more aggressive than well-differentiated thyroid carcinoma. After the presence and management of a possible pheochromocytoma is resolved, treatment is by total thyroidectomy, the men-2 syndromes always indicating bilateral involvement. Gross evidence of medullary thyroid carcinoma is associated with metastases to regional lymph nodes, justifying removal of lymph nodes in the central neck, anterior superior mediastinum, and lateral neck. At operation, attention is given to preservation of parathyroid glands but also to removal of hyperplastic parathyroid glands; subtotal parathyroidectomy usually is needed if clinical hyperparathyroidism is evident. Diagnosis at the preclinical stage, C-cell hyperplasia, permits total thyroidectomy. Lateral cervical lymph node dissection is determined by biopsy of midjugular lymph nodes. In this situation, serum calcitonin levels are usually normal after operation, indicating cure. However, for palpable medullary thyroid carcinoma, serum calcitonin levels are often elevated after appropriate neck surgery. In this event, scanning techniques are used to monitor patients, and reoperation is performed if localization of medullary thyroid carcinoma is achieved. The mediastinum is particularly observed for recurrence. reoperation is justified for recurrence in the neck and mediastinum. early diagnosis and monitoring permits long-term survival. In the future it is anticipated that diagnostic genetic techniques will provide definitive and early diagnosis in the hereditary form, permitting earlier treatment with assurance of cure.- - - - - - - - - - ranking = 2keywords = gland (Clic here for more details about this article) |
4/12. Multiple mucosal neuromatosis.Four children aged 3 months to 14 years presenting with multiple mucosal neuromatosis (MMN) and having medullary carcinoma of the thyroid (MCT) along with various other associated features are reported. All patient had screening test for pheochromocytoma. Thyroid gland and Neuromatous growths were subjected to histological examination for confirmatory diagnosis. All patients were subjected to total thyroidectomy with removal of regional lymph nodes. They were evaluated periodically for a variable post-operative period at 2 to 4 years. The first degree relatives were also screened. One of the four children had left sided pheochromocytoma which responded satisfactorily to the adrenalectomy. Prophylactic total thyroidectomy with annual/biannual critical review to detect recurrence is advocated. It is recommended that first degree relatives of familial MMN syndrome should also be subjected to annual screening in view of its autosomonal dominant transmission.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
5/12. Importance of early diagnosis and follow-up in multiple endocrine neoplasia (men II B).Three patients with a sporadic form of multiple endocrine neoplasia (men) IIb are presented. men IIb is the association of medullary carcinoma of the thyroid (MTC), pheochromocytoma and multiple mucosal neuromata. The age at diagnosis and primary therapy of MTC was relatively late (8, 16, 18 years) although thyroid enlargement (two cases), typical marfanoid habitus (two cases) or multiple mucosal neuromata (one case) had been evident since early childhood. The diagnosis was confirmed by measurements of tumour markers like calcitonin (CT) and carcinoembryonic antigen (CEA) for MTC, measurements of vanillylmandelic acid for pheochromocytoma, evaluation of the thyroid gland by sonography, scintigraphy and fine needle biopsy, and investigation of the adrenals by computer tomography and meta-(I-131) iodobenzylguanidine (I-131-MIBG) scintigraphy. After surgical treatment determinations of tumour markers confirmed relapses of MTC in all cases. early diagnosis of men IIb, which could be made by recognition of the typical clinical appearance, is of special importance because of the poor prognosis of MTC.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
6/12. A microscopic focus of traumatic neuroma with intralesional glandular structures: an incidental finding.A 50-year-old man received radiation therapy and chemotherapy for squamous cell carcinoma of the right floor of the mouth. Histologic examinations of surgical materials revealed the presence of benign ductal epithelial structures containing mucinous material within a small traumatic neuroma of the mandibular alveolar mucosa. Presumably, the residual salivary ducts of the sublingual glands may become incorporated during the development of a traumatic neuroma secondary to the destruction of tissue. Our search of the literature has failed to reveal a similar recorded occurrence.- - - - - - - - - - ranking = 5keywords = gland (Clic here for more details about this article) |
7/12. Tumor involvement of the facial nerve.Tumors involving the facial nerve are rare and challenging in both diagnosis and treatment. In this paper we report 18 cases of benign and malignant neoplasms involving the temporal portion of the facial nerve. The selection of those patients with facial paralysis who require detailed evaluation is discussed. Often, despite thorough evaluation of these patients, a preoperative diagnosis is unavailable or erroneous. An occasional patient may require surgical exposure of the nerve from the middle cranial fossa to the parotid gland.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
8/12. Metastatic medullary thyroid carcinoma in young children with mucosal neuroma syndrome.Multiple endocrine neoplasia, type 2b (men 2b) is a disorder characterized by a distinct phenotype and a predisposition for medullary thyroid carcinoma (MTC) and pheochromocytoma. Two siblings aged 3 and 6 years with men 2b, who had elevated plasma calcitonin levels suggesting the presence of MTC are described. Microscopic foci of MTC were found in their thyroid glands and the glands were removed. In the younger child, a metastatic focus was present in a cervical lymph node. Of 12 previously reported children with men 2b and MTC who were less than 10 years of age at diagnosis, five had metastases. One of the present patients is the youngest described with this complication. The high frequency of metastases in very young patients with this syndrome has not been emphasized previously. These findings indicate the need for early diagnosis of men 2b and the importance of thyroidectomy at the earliest possible age when MTC is suspected by calcitonin screening tests.- - - - - - - - - - ranking = 2keywords = gland (Clic here for more details about this article) |
9/12. A case of multiple endocrine neoplasia type II b: endocrinological evaluation and family screening.The case of a 27 year-old woman with typical manifestations of multiple endocrine neoplasia type II b is reported. Medullary carcinoma of the thyroid was detected on the occasion of an operation for goiter when she was 22 years of age. constipation due to megacolon caused by intestinal neuroma had persisted since childhood. neuroma of the tongue and lips, hypertrophic corneal nerve and Marfanoid habitus were also found. The presence of pheochromocytoma was suggested in view of the positive cold pressor and results of glucagon loading tests, but this remains inconclusive. There was prolonged and exaggerated response of growth hormone and luteinizing hormone after provocative tests for anterior pituitary gland, in spite of normal basal levels. Screening of her family members for medullary carcinoma of the thyroid was carried out by measurement of immunoreactive calcitonin. Two siblings were shown to be hypercalcitoninemic, presumably due to occult medullary carcinoma of the thyroid. This case appeared to be the first in japan showing multiple endocrine neoplasia type II b accompanied by familial hypercalcitoninemia.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
10/12. Ocular histopathology in multiple endocrine neoplasia type 2b.multiple endocrine neoplasia type 2b is a syndrome, often familial, characterized by medullary carcinoma of the thyroid gland, pheochromocytoma, and many mucosal neuromas in patients who frequently have a marfanoid habitus. The ophthalmic findings are distinctive and include conjunctival and eyelid neuromas, keratoconjunctivitis sicca, and, most characteristically, greatly thickened corneal nerves. Ocular histopathologic study of a 30-year-old man demonstrated thickened nerves in the ciliary body, iris, and corneoscleral limbal regions. In these regions, the nerves were a mixture of myelinated and unmyelinated fibers. Sections through the thickened corneal nerves, 1.5 mm centrally from the corneoscleral limbus, showed only nonmyelinated nerves associated with schwann cells. The many axons were normal in appearance but varied in diameter; some were between 0.1 and 0.2 ng and others between 1.0 and 1.4 ng. An unexpected finding was numerous ganglion cells in the ciliary body. The subconjunctival tissue showed neuromas with a minimum of fibrous structure. Sections of the lacrimal gland showed normal glandular tissue.- - - - - - - - - - ranking = 3keywords = gland (Clic here for more details about this article) |
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