Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0026764 (multiple myeloma)
 36,148 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Neoplastic proliferation of plasma cells results in a population of immunologically homogeneous cells that can produce diffuse (multiple myeloma) or localized (extramedullary plasmacytomas and solitary plasmacytoma of bone) disease. In otorhinolaryngologic literature these neoplasms are rarely described and their nosological arrangement is often confused. The presence of a plasma cell neoplasm can be a surprise and sometimes a diagnostic challenge to the head and neck surgeon. Proper management of such lesions needs to be individualized according to their expected biologic behaviour. The recent observation of a case of maxillary sinus plasmacytoma suggested the Authors to carefully review the literature, drawing their attention mainly on the current histogenetic hypotheses and their consequences in therapeutic strategy. The correct diagnostic procedure is also explained, highlighting the difficulties due to both the protean nature of the disease and the still existing nosological confusion. The possibility of a plasma cell tumour should be never forgotten in presence of an head and neck neoplasm. Because these neoplasms may signal the presence of multiple mieloma, full evaluation is required to exclude disseminated disease. In light of recent histogenetic acquisitions it is suggested that extramedullary plasmacytomas can be classified among the so-called "mucosa-associated" lymphomas. Possible following differences in therapeutic approach and long-term follow-up are also indicated, stressing the role of surgery in managing these disorders. Surgical excision of extramedullary plasmacytomas followed by complementary radiotherapy on the site of tumour is proposed as the best treatment for these kind of neoplasms. This is in opposition with "classical" statement considering radiotherapy the only treatment for this kind of disorders.
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PMID:[Plasmacytomas of the head and neck]. 898 43

Laryngeal amyloidosis (LA) is uncommon and poorly understood, with limited long-term clinicopathologic and immunophenotypic studies in the literature. Eleven cases of LA were retrieved from the files of the Otorhinolaryngic-Head & Neck Tumor Registry from 1953 to 1990. The histology, histochemistry, immunohistochemistry, and follow-up were reviewed. All patients (three women and eight men) presented with hoarseness at an average age of 37.8 years. The lesions, polypoid or granular, measured an average of 1.6 cm and involved the true vocal cords only (n = 4), false vocal cord only (n = 1), or were transglottic (n = 6). An acellular, amorphous, eosinophilic material was present in the stroma, often accentuated around vessels and seromucous glands, which reacted positively with Congo red. A sparse lymphoplasmacytic infiltrate was present in all cases that demonstrated light chain restriction by immunohistochemistry in three cases (kappa = 2, lambda = 1). Serum and urine electrophoreses were negative in all patients. Treatment was limited to surgical excision, including a single laryngectomy. Six patients manifested either recurrent and/or multifocal/systemic disease: two patients with light chain restriction were dead with recurrent disease (mean, 11.1 years); two patients were dead with no evidence of disease (mean, 31.7 years); and two patients were alive, one with light chain restriction and recurrent and multifocal disease (41.6 years) and one with no evidence of disease after a single recurrence (43.4 years). The remaining five patients were either alive or had died with no evidence of disease an average of 32.4 years after diagnosis. No patient developed multiple myeloma or an overt B-cell lymphoma. LA is an uncommon indolent lesion that may be associated with multifocal disease (local or systemic). The presence of an associated monoclonal lymphoplasmacytic infiltrate and recurrent/multifocal disease in the respiratory or gastrointestinal tract of a few cases and the lack of development of a systemic plasma cell dyscrasia or overt systemic B-cell malignancy suggest that some LA may be the result of an immunocyte dyscrasia or tumor of mucosa-associated lymphoid tissue.
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PMID:Amyloidosis of the larynx: a clinicopathologic study of 11 cases. 1082 24