elastin and Fibroma

Elastofibromatous change in the gastrointestinal tract is a rarely reported, usually polypoid lesion of unknown etiology with submucosal stromal change that may mimic amyloid deposition. The constituent amorphous material of the polyp stroma has distinctive features that permit an accurate assessment and diagnosis including: distribution of the material predominantly in the submucosa; distinctive fibrillar and granular appearance of the deposits; thick, irregular, haphazardly arranged bundles of elastic fibres positive for Verhoeff's elastic stain; ultrastructural fibres with an electron dense curvilinear or beaded appearance; lack of amyloid type vascular wall deposits; and lack of amyloid congophilia or crystal violet metachromasia. The clinical, light microscopic, histochemical and ultrastructural characteristics of this deposited material are reviewed in detail in the present report of a patient who presented with an asymptomatic polypoid lesion of the sigmoid colon. Other reported cases are summarized, and their clinical and pathological features are compared with the current case.

Topics: Aged; Amyloid; Colonic Polyps; Diagnosis, Differential; Elastin; Fibroma; Humans; Intestinal Mucosa; Lipoma; Male; Sigmoid Neoplasms

The recent progress made in understanding the normal biology and biochemistry of the extracellular matrix of human skin has allowed us to identify several different levels at which errors could be introduced into the structure and metabolism of collagen or elastin, the two major fibrillar components of the dermis. Currently, several heritable cutaneous diseases are known to display distinct collagen or elastin abnormalities. This article reviews some of the heritable cutaneous diseases and highlights those entities in which definite information on molecular alterations in collagen or elastin is available.

Topics: Adult; Collagen; Collagen Diseases; Cutis Laxa; Ehlers-Danlos Syndrome; Elastin; Fibroma; Humans; Male; Osteopoikilosis; Osteosclerosis; Pedigree; Skin Diseases; Skin Neoplasms

Topics: Animals; Arteriosclerosis; Chemical Phenomena; Chemistry; Chick Embryo; Copper; Deficiency Diseases; Elastic Tissue; Elastin; Fibroma; Guinea Pigs; Haplorhini; Histocytochemistry; Microscopy; Microscopy, Electron; Pseudoxanthoma Elasticum; Rats; Skin Neoplasms; Staining and Labeling; Swine

The heterotrimeric G protein alpha-subunit G(s)alpha links receptors to stimulation of cAMP/protein kinase A signaling, which inhibits skin fibroblast proliferation and collagen synthesis. We now describe the development of fibrous tumors in mice with heterozygous disruption of the Gnas gene, which encodes G(s)alpha and other gene products.. Disruption of Gnas exon 2 on either the maternal or paternal allele (Gnas(E2-/+)) results in fibromas or angiofibromas on the ears, paws and tail beginning at 4 months of age. The tumors were composed of fibroblastic cell proliferation with collagen and elastin deposition and calcification, and seemed to be associated with mechanical skin damage. The presence of calcification was associated with greater amounts of matrix metalloproteinase-2, suggesting an association between calcium deposition and extracellular matrix degradation. Osteoblast-specific markers were absent, consistent with the calcification not being secondary to ossification. Molecular studies showed that the tumors were not associated with deletion of the wild-type allele, making it unlikely that these tumors resulted from homozygous loss of G(s)alpha.. These findings provide in vivo evidence that G(s)alpha pathways inhibit fibroblast and endothelial proliferation and matrix deposition.

Topics: Angiofibroma; Animals; Calcinosis; Chromogranins; Collagen; Elastin; Fibroma; GTP-Binding Protein alpha Subunits, Gs; Mice; Skin Neoplasms

Topics: Biopsy, Fine-Needle; Coloring Agents; Elastic Tissue; Elastin; Female; Fibroma; Humans; Middle Aged; Oxazines; Soft Tissue Neoplasms

Elastofibroma dorsi (EFD) is a relatively rare soft tissue mass, probably of reactive nature. The lesion is typically located near the inferior margin of the scapula or between the inferior part of scapula and the chest wall in elderly women. Although location of the tumor together with the age/sex of the patients and radiologic findings is often suggestive of the diagnosis, tissue examination has been considered necessary to confirm the diagnosis. Although the histologic features of EFD are well known, there are only four single case reports of the cytologic findings in the English language literature. We describe the cytologic features of EFD in five patients with correlations to clinical, radiologic, histologic, and electron microscopic findings. The current study suggests that the fine-needle aspiration (FNA) features are highly diagnostic, permitting a firm diagnosis of EFD in a typical clinical setting and eliminating the need for preoperative histologic examination.

Topics: Aged; Biopsy; Collagen; Elastic Tissue; Elastin; Female; Fibroblasts; Fibroma; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Soft Tissue Neoplasms; Tomography, X-Ray Computed

Eight specimens each of the following groups were investigated: gingival pyogenic granuloma, fibrous epulis, calcifying fibrous epulis, peripheral giant cell granuloma, giant cell fibroma (four gingival, four non-gingival), denture-irritation hyperplasia and fibroepithelial polyp. These lesions have diverse histopathological appearances but the composition of their connective tissue is poorly defined. The elastic system consists of a complex mixture of glycoproteins that in normal oral mucosa form three differentially distributed fibre types; oxytalan, elaunin and elastic. The elastic system was investigated by Verhoeff's haematoxylin stain, aldehyde fuchsin staining and an anti-elastin monoclonal antibody. Elastin was identified in all fibroepithelial polyps and denture-irritation hyperplasias, but in none of the other lesions. In particular, this identified a distinct difference in the extracellular matrix between the giant cell fibroma and fibroepithelial polyp. Many of the epulides included only oxytalan fibres, but the presence of oxytalan fibres did not follow any pattern within either a single lesion group, or between different lesions. However, the presence of oxytalan fibres in the absence of elastin does not necessarily support a periodontal ligament origin for reactive epulides.

Topics: Antibodies, Monoclonal; Diagnosis, Differential; Elastic Tissue; Elastin; Fibroma; Gingival Diseases; Gingival Hyperplasia; Gingival Neoplasms; Gingival Overgrowth; Granuloma, Giant Cell; Granuloma, Pyogenic; Hematoxylin; Humans; Immunohistochemistry; Mouth Mucosa; Polyps; Rosaniline Dyes; Stomatitis, Denture

Topics: Amyloidosis; Bronchial Diseases; Elastin; Fibroma; Humans; Tracheal Diseases

Monospecific antibodies to elastic tissue components have been used for immunoelectron microscopy of two examples of elastofibroma. The elastic-staining fibers typically seen in these lesions exhibited a variety of morphologies with differing ratios of the amorphous and microfibrillar components usually seen in elastic fibers. The amorphous elastic material in these fibers had variable affinity for ionic stains and exhibited several substructural morphologies. Despite this, each form reacted specifically with anti-elastin antibodies. Most of the elastic fibers were associated with relatively large numbers of 12-nm diameter microfibrils that were typical of those associated with normal elastic fibers, and were specifically reactive with monospecific antibodies to microfibril-associated glycoprotein. In situ hybridization studies with a cRNA probe for human elastin confirmed that active elastin biosynthesis was occurring patchily within the lesions. The appearances and staining characteristics of the elastic tissue elements, the morphology of the cells, and the structure of the collagen fibers in these lesions were shown to have many features in common with those of normal periosteum. It is proposed that elastofibromas arise from the periosteum as a result of chronic irritation and that the different elastic fiber morphologies represent disturbances of elastic fibrillogenesis by periosteal-derived cells.

Topics: Aged; Amino Acid Sequence; Biomarkers; Elastin; Female; Fibroma; Humans; In Situ Hybridization; Microscopy, Immunoelectron; Molecular Sequence Data; Periosteum; RNA, Messenger; RNA, Neoplasm; Scapula

Topics: Diagnosis, Differential; Elastin; Female; Fibroma; Humans; Hyperplasia; Male; Middle Aged

Topics: Elastin; Fibroma; Humans; Male; Middle Aged; Muscular Diseases; Scapula

On the occasion of a case report on histiocytoma associated with elastofibroma-like parts on the shoulder, the extreme rarity of elastofibroma was doubted. This thesis was confirmed by post-mortem reports from Turku and Prague as well as by clinical experiences from Japan. Its possible relationship to other subcutaneous tumors rich in collagen and elastin was mentioned, and the abundant production of elastin was discussed as a variation of a one-sided metabolic mistake of the cells producing parenchyma. The symmetric or multiple, but principally the extracutaneous localization of elastofibromas may also direct our attention to developing factors other than traumatic ones, especially those being hereditary or ethnical.

Topics: Collagen; Diagnosis, Differential; Elastin; Female; Fibroma; Histiocytoma, Benign Fibrous; Humans; Middle Aged; Shoulder; Skin; Skin Neoplasms

Electron microscopy and both light and electron microscopic immunohistochemical tests for elastin were employed to study the morphogenesis of the unique elastinophilic fibers of an elastofibroma removed from the subscapular region of a 62-year-old woman. Ultrastructurally, as shown by tannic acid stain, elastinophilic fibers of the elastofibroma consisted of central cores and outer zones. The latter were composed of various sizes of vaguely demarcated, irregularly shaped amorphous components and compactly and randomly arranged large amounts of microfibrils. The electron microscopic immunohistochemical results showed that the small-sized amorphous components and microfibrils in the outer zones of the elastinophilic fibers were stained evenly and of granular texture, but the vaguely outlined large amorphous components were not stained. These findings were interpreted as indicating that the amorphous components of the outer zones of elastinophilic fibers were less compact and allowed the penetration of antielastin antibody. The unique elastinophilic fibers of elastofibromas appear not to be formed by the degeneration of the fibers but by abnormal elastogenesis, including an abnormal arrangement of microfibrils.

Topics: Bone Neoplasms; Elastic Tissue; Elastin; Female; Fibroma; Humans; Immunoenzyme Techniques; Microscopy, Electron; Middle Aged; Scapula

Elastin from both elastofibroma and control skin samples was analyzed by means of pancreatic elastase digestion and subsequent biochemical studies, such as for elastin content and amino acid composition. Elastin of elastofibroma was more resistant to elastase digestion than that of controls. Elastin content was increased almost twice (wet weight) or three times (dry weight) in elastofibroma. The amino acid composition had the characteristics of elastin; however, the cross-linking amino acids such as desmosine, isodesmosine, and lysinonorleucine were increased in elastofibroma when compared with controls. An electron microscopic study showed that the interspersed cells had prominent intermediate filaments without any periodicity, pinocytotic vesicles, rough endoplasmic reticulum, and other organelles. These cells were considered to be fibroblasts not myofibroblasts. Therefore, it could be supposed that fibroblasts newly form an elastin that has a slightly different amino acid composition from that of controls.

Topics: Aged; Amino Acids; Desmosine; Dipeptides; Elastin; Female; Fibroma; Humans; Isodesmosine; Microscopy, Electron; Pancreas; Pancreatic Elastase; Shoulder; Skin Neoplasms

A fibroproliferative lesion arising anterior to the lower end of the scapula and containing polymorphic often fiberlike deposits of weakly elastinophilic fibrillary material is described. It is compared on the light and ultrastructural level to a typical elastofibroma. The fibrillary material seen in this tumor strongly resembles that which surrounds the strongly elastinophilic deposits in the typical elastofibroma and from which the elastin is thought to be derived. It is concluded that elastin-producing tumors form a continuum; at one end of which lies the tumor we have designated pre-elastofibroma and at the other, the typical elastofibroma.

Topics: Bone Neoplasms; Elastin; Female; Fibroma; Humans; Middle Aged; Precancerous Conditions; Scapula

Four cases of elastofibroma located in the subscapular region of 3 men aged 66, 74, and 83 years, and a woman 70 years old are reported. A correlated light and electron microscopic study including ultrastructural examination of Verhoeff's iron-hematoxylin (VIH)-stained sections was performed. Light microscopically, the elastofibromas were characterized by connective tissue built up by collagen fibers and sclerotic masses mingled with numerous fibers and globules of elastin material. In one micron thick Epon sections these elastin fibers often revealed a central axis surrounded by a mantle composed of periodic segments giving them a necklace-like appearance. The ultrastructural findings of these elastin structures, stained with VIH, and the appearance of the stroma cells and their relation to the elastin indicate that elastofibroma is a non-neoplastic reactive lesion in which elastin material is synthesized by the stromal cells and predominantly laid down around preexisting elastic fibers.

Topics: Aged; Back; Collagen; Cytoskeleton; Elastin; Female; Fibroma; Histocytochemistry; Humans; Male; Scapula; Shoulder

A patient with dermatofibrosis lenticularis disseminata (Buschke-Ollendorff syndrome) was found to have a distinctive abnormality of the cutaneous elastic tissue. This abnormality, studied by histochemical and ultrastructural techniques, was characterized by the presence of hyalinized, thick fibers that entrapped normal bundles of collagen. Histochemically, these fibers stained like elastin but showed certain tinctorial characteristics of pre-elastin. Ultrastructurally, this elastin-like substance was composed of large clumps of electron-dense material with a fine fibrillar coating. The changes were sufficiently distinctive to be diagnosed by light microscopy.

Topics: Collagen; Elastic Tissue; Elastin; Female; Fibroma; Humans; Microscopy, Electron; Middle Aged; Osteopoikilosis; Skin; Skin Neoplasms; Syndrome

The Buschke-Ollendorff syndrome is an association of cutaneous lesions, dermatofibrosis lenticularis disseminata, with osteopoikilosis. This condition is inherited in an autosomal dominant pattern. In order to clarify the biochemical nature of the skin lesions, we have examined 12 patients with the Buschke-Ollendorf syndrome, representing 2 unrelated kindreds. Histologically, the lesions were characterized by excessive amounts of unusually broad, interlacing elastic fibers in the dermis. Digestion of skin secretions with pancreatic elastase completely removed these fibers. Electron microscopy of the dermis further revealed markedly branched elastic fibers without fragmentation. The accumulation of elastin in the skin was also demonstrated by measurements of desmosine employing a radioimmunoassay. The desmosine content of the skin lesions increased 3- to 7-fold when compared to the skin either from healthy controls or from uninvolved skin adjacent to a lesion. The results indicate that the skin lesions of the Buschke-Ollendorff syndrome are connective tissue nevi of the elastin type. Cell cultures from these patients may provide a convenient model to study the control mechanisms involved in elastin metabolism.

Topics: Desmosine; Elastic Tissue; Elastin; Fibroma; Humans; Osteopoikilosis; Osteosclerosis; Skin; Skin Neoplasms; Syndrome

Two cases of elastofibroma were studied by light and electron microscopy. In both cases the lesion was located in the subscapular area; in one case the lesions were bilateral. By light microscopy the masses were seen to be unencapsulated and composed of dense collagen and numerous elastinophilic globules and fibers. Ultrastructurally the elastinophilic material frequently contained a central core of mature elastic tissue and appeared to be secreted by active fibroblasts. Unique dense granular bodies within the fibroblast cytoplasm are described, which are thought to represent elastin or elastin precursors. This study further substantiates the thesis that the elastic material in elastofibroma is derived from excessive production by fibroblasts rather than from elastotic degeneration of collagen as suggested by previous studies.

Topics: Elastin; Fibroblasts; Fibroma; Humans; Male; Middle Aged; Organoids; Scapula

Topics: Elastin; Female; Fibroma; Humans; Middle Aged; Shoulder; Staining and Labeling

Topics: Adult; Elastin; Female; Fibroma; Humans; Scapula; Staining and Labeling; Thoracic Neoplasms

Topics: Aged; Elastic Tissue; Elastin; Female; Fibroma; Histocytochemistry; Humans; Male; Microbial Collagenase; Microscopy, Electron; Middle Aged; Neoplasms, Connective Tissue; Pancreatic Elastase

Topics: Elastic Tissue; Elastin; Fibroblasts; Fibroma; Histocytochemistry; Humans; Lipids; Male; Microscopy, Electron; Middle Aged; Polysaccharides; Thoracic Neoplasms

Topics: Aged; Back; Collagen; Connective Tissue; Elastic Tissue; Elastin; Female; Fibroma; Humans; Microscopy, Electron

Two cases of elastofibroma dorsi are reported and the light and electron microscopic features analysed. The appearances suggest that the characteristic fibres and globules that stain as for elastin arise by denaturation of collagen. The fibres differ from the elastin of arterial wall, elastotic degeneration of skin, and pseudoxanthoma elasticum. The morphology and clinical behaviour indicate that elastofibroma dorsi is a reactive fibromatosis rather than a true neoplasm.

Topics: Arteries; Back; Collagen; Elastic Tissue; Elastin; Female; Fibroma; Humans; Microscopy, Electron; Middle Aged; Pseudoxanthoma Elasticum; Scapula; Skin

Three cases of elastofibroma are described and the literature is reviewed. It is suggested that they are non-neoplastic lesions resulting from trauma. Their staining reactions and the response of the elastic type fibres to enzyme digestion suggest that these fibres are either true elastin or very closely related to it.

Topics: Aged; Back; Elastic Tissue; Elastin; Fibroma; Humans; Male; Middle Aged; Scapula