Fibrohistiocytic Tumors Containing Zebra Body-like Inclusions and Fibripositors

Plexiform fibrohistiocytic tumor (PFH) and cellular neurothekeoma (CN) are dermal neoplasms that present many histological and immunophenotype similarities as well as unknown histogenesis. Recently, the ultrastructural detection of zebra body-like inclusions in lesional histiocytes has been consider...

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Bibliographic Details
Published in:Ultrastructural pathology 2010-12, Vol.34 (6), p.366-370
Main Author: Pasquinelli, G.
Format: Article
Language:English
Subjects:
Adolescent
Adult
Cell Surface Extensions - ultrastructure
cellular neurothekeoma
Extracellular Matrix
Face
Female
fibripositors
Fibroblasts
Histiocytoma, Malignant Fibrous - metabolism
Histiocytoma, Malignant Fibrous - pathology
Humans
Inclusion Bodies - ultrastructure
Male
Microscopy, Electron, Transmission
Multivesicular Bodies - ultrastructure
Myelin Sheath - ultrastructure
Neurothekeoma - metabolism
Neurothekeoma - pathology
plexiform fibrohistiocytic tumor
Scalp
Skin Neoplasms - metabolism
Skin Neoplasms - pathology
ultrastructure
zebra body-like inclusions
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Summary:Plexiform fibrohistiocytic tumor (PFH) and cellular neurothekeoma (CN) are dermal neoplasms that present many histological and immunophenotype similarities as well as unknown histogenesis. Recently, the ultrastructural detection of zebra body-like inclusions in lesional histiocytes has been considered crucial to favor the diagnosis of PFH over that of CN. Here we report two examples of dermal tumors that were diagnosed as CN. Electron microscopy revealed cytoplasmic collections of myelin and zebra body-like inclusions in tumor cells; these inclusions focally merged together or with multivesicular bodies; tumor cells also showed collagen secretion granules and fibripositors, i.e., channels containing single, double or multiple copies of collagen fibrils. These observations support the view that PFH and CN have a common histogenesis and consists of cells sharing phagocityc and fibrillogenic abilities.
ISSN:0191-3123
1521-0758
DOI:10.3109/01913123.2010.513079