Recurring digital fibrous tumor of childhood

Mehregan, Amir H.

doi:10.1001/archderm.106.3.375

Cited by 12 publications

(6 citation statements)

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“…While some authors Burry et al) support the possibility that the dense bodies are of viral nature, others (McKenzie et al, 1970;Mehregan et al, 1972;Grunnet et al, 1973;Stiller and Katenkamp, 1975) agree with our observations and assume that these inclusions are products of a disturbed metabolism of fibroblasts. Stiller and Katenkamp (1975) suggested a defect in intracellular transport of collagen precursor with subsequent deposition of collagenous proteins.…”

Section: M Zardawi and M J Earleysupporting

confidence: 84%

Inclusion body fibromatosis

Zardawi

Earley

1982

The Journal of Pathology

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The clinical and pathological features of two further cases of the condition previously described as Recurring Digital Fibrous Tumour of Childhood are presented. The ultrastructural appearance of the characteristic cell inclusions bears some resemblance to Mallory's hyaline and suggest increased functional activities in the fibroblasts carrying these unusual dense bodies. Retention of abnormal metabolic products in fibroblasts is the most likely explanation. The term Inclusion Body Fibromatosis is proposed for this entity.

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“…The tumor cell nuclei are stellate or elongated, Mitotic figures are rare. The characteristic histologic findings are numerous L5 to 10 micrones round eosinophilic inclusion bodies in the cytoplasm of fibroblasts which may be visualized in the H&E sections under high magnification (Battifora & Hines 1971), These round bodies are, however, well demonstrated by Mallory's trichome stain in red color against the blue background of collagen and in a deep purple color in sections stained with the Phosphotungstic acid hematoxylin technique (Mehregan et al, 1972), In electron microscopy the dense non-membrane-bound spherical bodies are composed of densely packed microfilaments measuring 5-7 nm, Bhawan & Bacchetta (1979) and in their recent studies have identified tail-like bundles of myofilaments extending from the round inclusion bodies running longitudinally along the body of cells. Distributed along each bundle are small dense bodies such as seen in the contractile apparatus of smooth muscle cells and myofibroblasts.…”

Section: Recurring Digital Fibrous Tutnor Of Childhoodmentioning

confidence: 99%

“…Smooth muscles cells, however, show myofilaments scattered throughout the cell cytoplasm (Bhawan 1981). Electron microscopy and tissue culture findings have elimi-nated the possibility of viral origin of the round inclusion bodies (Mehregan et al 1972, Burry et al, 1970, Miyazono et al, (1980), utilizing tissue cultures, demonstrated that round inclusion body formation by the tumor fibroblasts persists for as much as 30 passages. Recurring digital fibrous tumor is benign, and no metastatic lesions have been reported.…”

Section: Recurring Digital Fibrous Tutnor Of Childhoodmentioning

confidence: 99%

Superficial fibrous tumors in childhood

Mehregan¹

1981

J Cutan Pathol

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A review is made of superficial fibrous tumors of childhood and differences between the pattern of fibrous tumor proliferation in children and adults are discussed. The fibrous tumors that occur specifically in childhood and infancy include recurring digital fibrous tumor, juvenile aponeurotic fibroma (cartilage analogue of fibromatosis), juvenile hyalin fibromatosis, fibrous hamartoma of infancy, congenital fibromatosis and fibromatosis colli (Torticollis). Palmoplantar fibromatosis and fibrosarcoma may appear in all ages. Dupuytren contracture, however, is not usually observed early in life. Similarly, the histiocytomas and dermatofibromas which are the most common fibrous tumors af adults are extremely rare in childhood.

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“…[1] These rare tumors are also known as fibrous tumors in babyhood, digital fibrous tumors in childhood, IDF, juvenile dermatofibroma, and digital fibrous tumor of childhood. [2–4]…”

Section: Introductionmentioning

confidence: 99%

Infantile digital fibromatosis (inclusion body fibromatosis) observed in a baby without finger involvement

et al. 2013

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A 9-day-old male baby was hospitalized after his birth due to some swells under the skin. The hard consistency nodules observed under the skin all over the body of the patient were of different size, and presented lesions, among which the biggest was 1 × 1 cm. No lesions were observed on the fingers. By superficial ultrasonography, multiple isoechoic hypoechoic lesions were observed among the muscle plan. In thoracolumbar magnetic resonance imaging, multiple massif lesions retaining peripheral contrast (the biggest was 1.7 × 1.4 cm large) had been observed under the skin muscle plans, between the muscles of the extremities. The biopsy was positive for smooth muscle actin, but negative for desmin, S100, and CD34. These findings were diagnosed as infantile digital fibromatosis (IDF) (inclusion body fibromatosis). The case was presented with an objective to illustrate and remind that IDF can be observed in babies without finger involvement.

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“…A diagnostic morphologic feature is an eosinophilic inclusion body in the cytoplasm of the tumor cells. Some authors have suggested that the inclusion bodies could be derived from abnormal products of disturbed fibroblast metabolism (8,9). Bhawan et al (10) have shown by electron microscopy that these tumor cells are compatible with myofibroblasts.…”

mentioning

confidence: 99%

Infantile digital fibromatosis. Immunohistochemical and immunoelectron microscopic studies

et al. 1990

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A typical case of infantile digital fibromatosis (IDF) was studied with antibodies raised against actin, vimentin, desmin and several species of cytokeratin. Strong reactions were observed for desmin, cytokeratin and CK-5, and moderate reactions for vimentin and actin. The diagnostic intracytoplasmic inclusion bodies within the tumor cells were ultrastructurally composed of aggregations of dense microfilaments. Immunoelectron microscopy showed that these filament aggregations are positively stained with anti-actin antibody. From these results, we suggest that the large tumor cell of IDF is a myofibroblast and may originate from or differentiate toward vascular smooth muscle cells, because only this type of smooth muscle can coexpress desmin, vimentin and cytokeratin.

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Recurring digital fibrous tumor of childhood

Cited by 12 publications

References 0 publications

Inclusion body fibromatosis

Superficial fibrous tumors in childhood

Infantile digital fibromatosis (inclusion body fibromatosis) observed in a baby without finger involvement

Infantile digital fibromatosis. Immunohistochemical and immunoelectron microscopic studies

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