glycogen and Hamartoma

Indications for liver biopsy in children are often specific to this age group, especially in young children for the diagnosis of cholestasis. Since liver biopsies are quite unfrequent in the children population and concern rare but various diseases, it is recommended to entrust the analysis to a specialized liver pathologist, in a laboratory where cryoconservation, specific immuno-stainings, enzymatic studies, and electron microscopy can be performed. Histology is complementary to other methods for the diagnosis, and is valuable for the evaluation of the prognosis, especially the staging of fibrosis and the grading of inflammatory diseases. In cases of co-morbidity or difficult differential diagnosis, histology can also be of great value. For metabolic disorders, the liver tissue can also be used for enzyme detection or evaluation of iron or copper overload. Biopsy is also a key element in the management after liver transplantation. The microscopic images shown here are representative of the most frequent liver diseases in childhood and illustrate the data outlined during the conference.

Topics: Biliary Tract; Biliary Tract Diseases; Biopsy; Child; Glycogen; Hamartoma; Hepatoblastoma; Hepatocytes; Humans; Hyperplasia; Liver; Liver Diseases; Metabolism, Inborn Errors

Apoeccrine glands were found in 13 of 57 specimens of nevus sebaceus. The glands showed the basic structure of eccrine glands, but differed in that a part of the secretory duct was lined by characteristic apocrine epithelium. The apocrine alteration varied from slight apocrine changes in a few secretory cells to an almost complete alteration of the secretory epithelium in other glands. The association with changes in the excretory ducts of rare glands suggested a transformation of eccrine into apocrine glands. It was nevertheless considered as most likely that a majority of the apocrine glands in nevus sebaceus had developed in the "classic" way in association with pilosebaceus structures. In nevus sebaceus, apoeccrine glands appeared to represent eccrine glands with a variable degree of apocrine metaplasia that could result in complete transformation rather than the formation of a separate category of specialized sweat glands. The capacity of eccrine epithelium to transform into apocrine epithelium is why difficulties are experienced in the precise categorization of sweat gland neoplasms into eccrine and apocrine tumors.

Topics: Adipose Tissue; Adolescent; Adult; Antigens; Apocrine Glands; Apolipoproteins; Apolipoproteins D; Carrier Proteins; Child; Child, Preschool; Eccrine Glands; Epidermis; Female; Glycogen; Glycoproteins; Hair; Hamartoma; Humans; Infant; Male; Membrane Glycoproteins; Membrane Transport Proteins; Middle Aged; Mucin-1; Sebaceous Glands; Sweat Gland Diseases

Topics: Colon; Colonic Neoplasms; Esophagus; Female; Ganglioneuroma; Glycogen; Hamartoma; Humans; Hyperplasia; Middle Aged; Neoplasms, Multiple Primary; Syndrome

Multiple hamartomatous polyps from two patients with Peutz-Jeghers syndrome were studied using histochemical, electron-microscopic, and light-microscopic means. Using normal intestinal mucosa as control, mucosubstances containing sulfate, hexose and glycogen, as well as neutral and acid mucins, were detected. Electron-microscopic findings in different cellular populations of hamartomatous and normal mucosa were also evaluated. The results indicated the hamartomatous nature of such polypoid formations in Peutz-Jeghers syndrome. Review of the literature supports the possibility of genetic predisposition of these patients to develop unusual types of neoplasms in unexpected localizations.

Topics: Adult; Child; Colonic Polyps; Female; Glycogen; Hamartoma; Hexoses; Histocytochemistry; Humans; Intestinal Mucosa; Male; Mucins; Peutz-Jeghers Syndrome; Sulfates

Ultrastructural and histochemical examination of chondromatous pulmonary hamartomas revealed the epithelial component to be comprised elements similar to those lining the distal bronchioles and the alveoli of adult lung. The stromal cells nearest the epithelium include a population resembling mature fibroblasts and a population of glycogen-containing, primitive appearing cells. More deeply situated stromal cells showed features of chrondroid differentiation.

Topics: Epithelium; Glycogen; Hamartoma; Humans; Lung Neoplasms; Microscopy, Electron