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Infantile digital fibromatosis. Ultrastructural, histochemical, and tissue culture observations
Abstract:
Three cases of infantile digital fibromatosis were studied by electron microscopy, enzyme histochemistry, and tissue culture. The tumors were made up equally of myofibroblasts containing electron-dense inclusions which were composed chiefly of microfilaments measuring about 5 to 7 nm. Dense bodies usually observable in the smooth muscle cells were found in the bundles of these microfilaments and in the process of the inclusions, suggesting that these inclusions may represent an abnormal accumulation of contractile protein in the cytoplasm of tumor cells. Two cell lines were established from culture of the tumor cells, and the cultured cells also contained inclusion bodies showing the same morphologic characteristics as those of the original tumor cells. Lysosomal enzymes were abundant in the cultured cells, but they were scant in the cells of the fresh tissue specimens. Cocultivation of the cultured cells with human embryonic lung cells yielded no cytopathic effect.
Insights
Infantile digital fibromatosis tumors contain myofibroblasts with abnormal microfilamentous inclusions, possibly contractile protein. Cultured cells showed similar inclusions and abundant lysosomal enzymes.
Area of Science:
- Pathology
- Cell Biology
- Dermatology
Background:
- Infantile digital fibromatosis (IDF) is a rare benign soft tissue tumor.
- Understanding the cellular and molecular basis of IDF is crucial for diagnosis and management.
Purpose of the Study:
- To investigate the ultrastructural and cellular characteristics of infantile digital fibromatosis.
- To analyze the composition and origin of the characteristic inclusion bodies in IDF.
Main Methods:
- Electron microscopy was used to examine tumor tissue.
- Enzyme histochemistry and tissue culture were employed to study cellular properties.
- Two cell lines were established from cultured tumor cells.
Main Results:
- Tumors comprised myofibroblasts with electron-dense inclusions of 5-7 nm microfilaments.
- Inclusions contained dense bodies, suggesting abnormal contractile protein accumulation.
- Cultured cells retained inclusion bodies and showed increased lysosomal enzymes compared to fresh tissue.
Conclusions:
- The findings suggest IDF inclusions represent an abnormal accumulation of contractile proteins.
- Cultured IDF cells exhibit distinct enzymatic profiles and retain ultrastructural features.
- Further research into contractile protein dynamics in IDF is warranted.