1/79. Amyloid goiter with parathyroid involvement: a case report and review of the literature.Although involvement of the thyroid gland by amyloid is a relatively common phenomenon, diffuse enlargement of the gland secondary to infiltration by amyloid is infrequent. We report the pathologic findings in a case of primary amyloid goiter with involvement of a parathyroid gland in an euthyroid patient who presented to his physician with an enlarged thyroid, upper airway obstructive symptoms, and dysphagia. The clinical features of amyloid goiter, including difficulties in preoperative diagnosis and management, are discussed.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
2/79. Familial amyloidotic polyneuropathy (ATTR Ser50Ile): the first autopsy case report.We report an autopsy case of a pedigree of familial amyloidotic polyneuropathy (FAP) with a mutation of isoleucine-50 transthyretin (ATTR Ser50Ile). A 47-year-old man started developing severe diarrhea and weight loss at age 41 years, followed by urinary incontinence, autonomic-nervous-system abnormalities and serious heart failure; the diagnosis of FAP (ATTR Ser50Ile) was made on the basis of genetic, histochemical and immunohistochemical analysis. Six years after the initial symptoms, he died of septic shock. autopsy revealed suppurative peritonitis, perforation of the sigmoid colon and marked systemic amyloid deposition. The total amount of amyloid deposited in the heart was greatly increased and was much lower in the thyroid gland and kidneys compared with amyloid deposits in ordinary FAP (ATTR Val30Met). Amyloid deposition in peripheral vessel walls was prominent, particularly in lymphatics and veins. His elder sister, 54 years old, started to develop orthostatic hypotension at age 49 years, followed by dysesthesia, diarrhea and severe congestive heart failure. Endomyocardial biopsy revealed severe TTR-amyloid deposition; ultrastructural examination demonstrated that amyloid fibrils were deposited disproportionately and extended radially around microvessels.- - - - - - - - - - ranking = 0.33333333333333keywords = gland (Clic here for more details about this article) |
3/79. The pattern of amyloid deposition in the lung.A review of routine histopathological samples and autopsies examined at the Department of pathology, University of Malaya revealed 15 cases of amyloidosis of the lung. Two were localized depositions limited to the lung while in the remainder, lung involvement was part of the picture of systemic amyloidosis. Both cases of localized amyloidosis presented with symptomatic lung/bronchial masses and a clinical diagnosis of tumour. histology revealed "amyloidomas" associated with heavy plasma cell and lymphocytic infiltration and the presence of multinucleated giant cells. In both cases, the amyloid deposits were immunopositive for lambda light chains and negative for kappa chains and AA protein. One was a known systemic lupus erythematosus patient with polyclonal hypergammaglobulinaemia. The other patient was found to have plasma cell dyscrasia with monoclonal IgG lambda gammopathy. Both patients did not develop systemic amyloidosis. In contrast, lung involvement in systemic AA amyloidosis was not obvious clinically or macroscopically but was histologically evident in 75% of cases subjected to autopsy. Amyloid was detected mainly in the walls of arterioles and small vessels, and along the alveolar septa. It was less frequently detected in the pleura, along the basement membrane of the bronchial epithelium and around bronchial glands. In one case of systemic AL amyloidosis associated with multiple myeloma, an "amyloidoma" occurred in the subpleural region reminiscent of localized amyloidosis. These cases pose questions on (1) whether localized "tumour-like" amyloidosis is a forme fruste of systemic AL amyloidosis and (2) the differing pattern of tissue deposition of different chemical types of amyloid fibrils, with the suggestion that light chain amyloid has a greater tendency to nodular deposition than AA amyloid.- - - - - - - - - - ranking = 0.33333333333333keywords = gland (Clic here for more details about this article) |
4/79. Amyloid goiter: the first evidence in secondary amyloidosis. Report of five cases and review of literature.Amyloid deposition in secondary amyloidosis frequently involves thyroid gland, but rarely is responsible of a goiter. Amyloid goiter in secondary amyloidosis is characterized by deposition of amyloid A protein (AA) in the gland, associated to atrophic follicles. We identified cases of amyloid goiter in the files of our department in the period from 1985 to 1998. Five cases of amyloid goiter with ingravescent symptomatology, characterized by dyspnea, dysphagia and hoarseness were selected. In four cases of five we observed predisposing conditions as, for example, tuberculosis, Crohn's disease, or rheumatoid arthritis. In all cases the symptoms relative to thyroid enlargement preceded or, anyway, predominated over other clinical evidence of systemic amyloidosis. In one case a symptomatology of systemic amyloidosis was not evident. We would like to underline that in all cases the immunoreactivity for amyloid A in the amorphous material present in the gland permitted the diagnosis of secondary amyloidosis even in the absence of systemic symptoms.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
5/79. Papillary carcinoma in amyloid goitre.Amyloid goitre is a rare lesion characterized by a diffuse and bilateral enlargement of the thyroid gland due to amyloid deposition. It is uncommon that a massive and widespread amount of adipose tissue deposition is found within these lesions and only in exceptional cases a differentiated carcinoma can develop. We describe the third example of thyroid carcinoma, arising in a 74-year old female who had also massive adipose thyroidal metaplasia, within amyloid goitre. The congo red stain confirmed the diagnosis of amyloid goitre. immunohistochemistry showed reactivity with MoAb against amyloid fibril protein A. The patient suffered from renal failure of undetermined aetiology for three years, but neither systemic amyloidosis nor risk factors for its development were found. It is important to correctly diagnose amyloid goitre both to rule out the presence of a differentiated thyroidal carcinoma and to search for amyloid infiltration in other organs in view of an early appropriate therapy.- - - - - - - - - - ranking = 0.33333333333333keywords = gland (Clic here for more details about this article) |
6/79. Amyloid goiter: report of two cases and review of the literature.OBJECTIVE: To describe two recent cases of amyloid goiter that occurred in two patients with a history of diffuse enlargement of the thyroid gland, progressing over several years' time. methods: We reviewed the medical histories as well as the histologic, immunohistochemical, and electron microscopic findings in these patients. RESULTS: Assessment of the clinical records of a 59-year-old man and a 64-year-old woman revealed histories that included chronic psoriasiform arthritis and asthma in conjunction with chronic obstructive pneumonia and bronchiectasis. In both cases, total thyroidectomy was performed. On histologic examination of both thyroid glands, the appearance was characterized by moderate to severe distortion of the normal thyroid architecture by amyloid in a perifollicular distribution and focally abundant interfollicular adipose tissue. In both cases, the amyloid stained intensely positive with congo red, which bleached after treatment with potassium permanganate. Immunohistochemical staining patterns were consistent with AA amyloid, and electron microscopy showed nonbranching 9-nm fibrils consistent with amyloid. CONCLUSION: The diagnosis of amyloid goiter should be suspected in patients with a diffusely enlarging thyroid gland and an appropriate clinical history.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
7/79. Amyloid goiter as the initial manifestation of systemic amyloidosis due to familial mediterranean fever with homozygous MEFV mutation.We describe a case of amyloid goiter revealing a systemic amyloidosis secondary to familial mediterranean fever (FMF) with homozygous MEFV mutation, and we review the literature. A 45-year-old euthyroid Sephardic man, known to suffer from FMF, developed a goiter with cold nodule, after which a subtotal thyroidectomy was performed. Histologic evaluation revealed diffuse AA amyloid deposition without any associated thyroid neoplasia. At that time, no other organ was found to be affected by amyloidosis. colchicine and levothyroxine were prescribed. Eight years later, the patient presented with a rapidly growing neck enlargement. He reported that he had discontinued colchicine therapy 2 years earlier. The serum thyrotropin (TSH) and calcitonin levels were normal. Renal, digestive, and salivary gland biopsies confirmed the presence of systemic AA amyloidosis. Despite the reintroduction of colchicine, the onset of compressive symptoms led to the completion of the total thyroidectomy. The histopathology again demonstrated amyloid deposition, and excluded a malignant neoplasm. Nine cases of amyloid goiter associated with FMF have been reported in the literature; none of them had an amyloid goiter as the first manifestation of systemic amyloidosis. To our knowledge, this is the first case of FMF in which an amyloid goiter preceded the development of secondary systemic amyloidosis. The cessation of colchicine therapy may have played a role in local relapse and the secondary spread of amyloid deposits.- - - - - - - - - - ranking = 0.33333333333333keywords = gland (Clic here for more details about this article) |
8/79. Extramedullary plasmacytoma of the parotid gland. Report of a case with extensive amyloid deposition masking the cytologic and histopathologic picture.BACKGROUND: Extramedullary plasmacytomas are uncommon. Although approximately 90% occur in the head and neck region, parotid gland localization is extremely rare. CASE: This report describes fine needle aspiration and histopathologic findings in an extramedullary plasmacytoma arising in the left parotid gland of a 62-year-old man. Aspiration smears showed multiple amorphous clumps of material admixed with ductal epithelial cells, multinucleated giant cells and inflammatory cells rich in plasma cells, suggestive of pleomorphic adenoma. In surgical material, excessive amyloid deposition was observed. Six months later the tumor recurred, and in the second surgical specimen clusters of atypical plasma cells among amyloid deposits was noted. Clinical and laboratory examination excluded multiple myeloma. After local recurrence, radiotherapy was applied. Ten months later the patient was well and without systemic involvement. CONCLUSION: Two points are important. First, in the salivary gland region, because of the focal metachromasia of amyloid with Giemsa stain, amyloid can be confused with the chondromyxoid matrix of pleomorphic adenoma. Second, although localized forms of amyloid tumor exist, one should keep in mind that amyloid may be so abundant that it may mask an underlying plasma cell neoplasm, as in our case.- - - - - - - - - - ranking = 2.3333333333333keywords = gland (Clic here for more details about this article) |
9/79. Concomitant diagnosis of primary sjogren's syndrome and systemic AL amyloidosis.A 48 year old woman was referred to hospital for buccal discomfort. physical examination showed a macroglossia and features of xerostomia. She was diagnosed as having primary sjogren's syndrome according to the criteria proposed by the European Community study group in 1993. Furthermore, a lower lip salivary gland biopsy showed amyloid deposits that were also seen in the stomach and in the bone marrow. echocardiography was consistent with cardiac amyloidosis. serum immunofixation identified a monoclonal IgGlambda. As far as is known, this is the first report of systemic primary amyloidosis associated with sjogren's syndrome. The relation between these two disorders is discussed.- - - - - - - - - - ranking = 0.33333333333333keywords = gland (Clic here for more details about this article) |
10/79. Painless thyroiditis associated with severe inflammatory reactions in amyloid goiter: a case report.We report the case of a 64-year-old woman with rheumatoid arthritis (RA) associated with high grade fever, malaise, and painless swelling of thyroid gland. Laboratory findings showed severe systemic inflammatory reactions, including increases in various cytokines such as IL-6. gallium-67 citrate imaging revealed intense uptake in the painlessly enlarged thyroid gland. Histologically, biopsied specimens of thyroid showed diffuse amyloid infiltrations, which included amyloid A (AA) protein. Biopsies of rectum and stomach revealed similar amyloid depositions, indicating that the amyloid had a secondary origin, potentially due to RA. All clinical symptoms were relieved by intravenous pulsatile administration of methylprednisolone followed by oral prednisone, resulting in prolonged hypothyroid status. To our knowledge, this is the first case report in japan describing painless thyroiditis with severe inflammatory reactions in amyloid goiter.- - - - - - - - - - ranking = 0.66666666666667keywords = gland (Clic here for more details about this article) |
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