glycogen and Lipoma

Cowden's disease is a rare autosomal dominant condition characterized by multiple hamartomas of ectodermal, endodermal, and mesodermal origin affecting many organ systems. Gastrointestinal manifestation includes the formation of multiple polyps of various benign histopathological types throughout the alimentary tract. Recent literature suggests that the frequency of gastrointestinal involvement is approximately 70-85%. The diagnosis of Cowden's disease, however, relies mainly on subtle dermatologic findings, which may not be obvious to the gastroenterologist. We describe a patient with Cowden's disease and review the English literature on the topic of gastrointestinal polyposis and Cowden's disease. These studies suggest that gastrointestinal polyposis is commonly found in this disease, and that diffuse esophageal glycogenic acanthosis is a characteristic feature of Cowden's disease. We propose that the finding of extensive glycogenic acanthosis in the presence of other benign gastrointestinal polyposis should be considered pathognomonic for the diagnosis of Cowden's disease.

Topics: Colonic Polyps; Duodenal Neoplasms; Esophageal Diseases; Esophagoscopy; Ganglioneuroma; Glycogen; Hamartoma Syndrome, Multiple; Humans; Hyperplasia; Intestinal Polyps; Jejunal Neoplasms; Lipoma; Male; Middle Aged

An angiomyolipoma from a twenty-six-year-old woman was studied by electron microscopy. The tumor was composed of a mixture of mature smooth muscle cells, fat cells, and abnormal blood vessels. MAny of the smooth muscle cells showed excessive intracytoplasmic accumulation of glycogen, while the abnormal blood vessels lacked a normal media.

Topics: Adipose Tissue; Adult; Female; Glycogen; Hemangioma; Humans; Kidney Neoplasms; Lipoma; Microscopy, Electron; Muscle, Smooth

Topics: Adipose Tissue; Cartilage; Chondrosarcoma; Chordoma; Connective Tissue; Female; Fibroma; Fibrosarcoma; Glycogen; Hemangioma; Humans; Leiomyoma; Leiomyosarcoma; Lipoma; Liposarcoma; Muscle, Smooth; Neoplasms, Connective Tissue; Neoplasms, Muscle Tissue; Neoplasms, Nerve Tissue; Neurilemmoma; Neurofibroma; Sarcoma; Sarcoma, Synovial; Synovial Membrane; Uterus

Topics: Glycogen; Glycoproteins; Glycosaminoglycans; Histocytochemistry; Humans; Hyaluronic Acid; Lipoma; Lipoproteins; Malate Dehydrogenase; Methods; Mucoproteins; Succinate Dehydrogenase