Plexiform Fibrohistiocytic Tumor: A Rare Low‐Grade Myofibroblastic Tumor of Children and Young Adults
Identifieur interne : 001495 ( Istex/Curation ); précédent : 001494; suivant : 001496Plexiform Fibrohistiocytic Tumor: A Rare Low‐Grade Myofibroblastic Tumor of Children and Young Adults
Auteurs : Nneka I. Comfere ; Iftikhar Ahmed [États-Unis]Source :
- Journal of Cutaneous Pathology [ 0303-6987 ] ; 2005-01.
Abstract
We describe a 7‐year old male with a 7‐month history of a plexiform fibrohistiocytic tumor (PFT) presenting as an innocuous 0.5 × 0.5 cm nodule on the nasal dorsum. Following diagnostic sampling, Mohs’ micrographic surgery was performed to ensure adequate removal and patient continues to do well 1 year post‐surgery. PFT is a rare neoplasm described initially by Enzinger and Zhang in 1988. It afflicts children and young adults (median age: 14.5 years) and has a strong female predisposition. It typically occurs on the upper extremity. Histologically, the tumor has a bland appearance consisting of a biphasic population of varying numbers of fibroblast and histiocyte‐like cells. Cellular atypia and pleomorphism are lacking and mitotic activity is low. Immunohistochemically and ultrastructurally, the tumor demonstrates myofibroblastic differentiation. The constituent cells have a diploid DNA content. Unique cytogenetic aberrations have been anecdotally identified. PFT behaves in a locally aggressive fashion and regional nodal disease can occur despite adequate excision. In rare instances, metastasis to the lungs has been described. No association between specific clinicopathologic features and outcome has been identified. In summary, PFT is a rare but distinct low‐grade myofibroblastic tumor of children and young adults with a misleading clinical and histological appearance.
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DOI: 10.1111/j.0303-6987.2005.320ay.x
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Nneka I. Comfere<affiliation><mods:affiliation>Departments of Dermatology and Laboratory Medicine and Pathology</mods:affiliation><wicri:noCountry code="no comma">Departments of Dermatology and Laboratory Medicine and Pathology</wicri:noCountry></affiliation>Le document en format XML
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Comfere</name><affiliation><mods:affiliation>Departments of Dermatology and Laboratory Medicine and Pathology</mods:affiliation><wicri:noCountry code="no comma">Departments of Dermatology and Laboratory Medicine and Pathology</wicri:noCountry></affiliation></author><author><name sortKey="Ahmed, Iftikhar" sort="Ahmed, Iftikhar" uniqKey="Ahmed I" first="Iftikhar" last="Ahmed">Iftikhar Ahmed</name><affiliation><mods:affiliation>Departments of Dermatology and Laboratory Medicine and Pathology</mods:affiliation></affiliation><affiliation wicri:level="1"><mods:affiliation>, Mayo Clinic and Mayo Foundation, Rochester, Minnesota USA.</mods:affiliation><country xml:lang="fr" wicri:curation="lc">États-Unis</country><wicri:regionArea>, Mayo Clinic and Mayo Foundation, Rochester</wicri:regionArea></affiliation></author></analytic><monogr></monogr><series><title level="j" type="main">Journal of Cutaneous Pathology</title><title level="j" type="alt">JOURNAL OF CUTANEOUS PATHOLOGY</title><idno type="ISSN">0303-6987</idno><idno type="eISSN">1600-0560</idno><imprint><biblScope unit="vol">32</biblScope><biblScope unit="issue">1</biblScope><biblScope unit="page" from="82">82</biblScope><biblScope unit="page" to="83">83</biblScope><biblScope unit="page-count">2</biblScope><publisher>Blackwell Publishing Ltd/Inc.</publisher><pubPlace>Oxford, UK; Malden, USA</pubPlace><date type="published" when="2005-01">2005-01</date></imprint><idno type="ISSN">0303-6987</idno></series></biblStruct></sourceDesc><seriesStmt><idno type="ISSN">0303-6987</idno></seriesStmt></fileDesc><profileDesc><textClass></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">We describe a 7‐year old male with a 7‐month history of a plexiform fibrohistiocytic tumor (PFT) presenting as an innocuous 0.5 × 0.5 cm nodule on the nasal dorsum. Following diagnostic sampling, Mohs’ micrographic surgery was performed to ensure adequate removal and patient continues to do well 1 year post‐surgery. PFT is a rare neoplasm described initially by Enzinger and Zhang in 1988. It afflicts children and young adults (median age: 14.5 years) and has a strong female predisposition. It typically occurs on the upper extremity. Histologically, the tumor has a bland appearance consisting of a biphasic population of varying numbers of fibroblast and histiocyte‐like cells. Cellular atypia and pleomorphism are lacking and mitotic activity is low. Immunohistochemically and ultrastructurally, the tumor demonstrates myofibroblastic differentiation. The constituent cells have a diploid DNA content. Unique cytogenetic aberrations have been anecdotally identified. PFT behaves in a locally aggressive fashion and regional nodal disease can occur despite adequate excision. In rare instances, metastasis to the lungs has been described. No association between specific clinicopathologic features and outcome has been identified. In summary, PFT is a rare but distinct low‐grade myofibroblastic tumor of children and young adults with a misleading clinical and histological appearance.</div></front></TEI></record>Pour manipuler ce document sous Unix (Dilib)
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