Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0241908 (BFH)
 20 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

p53 mutation is one of the major results of ultraviolet (UV) radiation. UV photoproducts of cyclobutane pyrimidine dimers (CPDs) and pyrimidine-pyrimidone (6-4) photoproducts (64PPs) also play an important role in skin cancer development. Atypical fibroxanthoma (AFX), which mimics malignant fibrous histiocytoma (MFH) histologically, occurs in the sun-exposed skin of the elderly, and therefore, an association with UV has long been suspected. Eighteen fibrohistiocytic skin lesions comprising AFX (n = 7), storiform-pleomorphic type MFH centered in the subcutis (superficial MFH; S-MFH; n = 4) and benign fibrous histiocytoma (BFH; n = 7) were used for immunohistochemical and molecular analysis. Eight cases of deep MFH (D-MFH) were also analyzed for UV photoproduct expression for the purposes of comparison. Immunohistochemically, the CPD scores of AFX (3.6 +/- 0.4) were significantly higher than those of S-MFH (1.3 +/- 0.8), D-MFH (0.8 +/- 0.5), or BHF (1.4 +/- 0.7); however, the 64PP scores were extremely low in all these tumors (AFX, 0.1 +/- 0.1; S-MFH, 0.0 +/- 0.0; D-MFH, 0.0 +/- 0.0; and BHF, 0.0 +/- 0.0). AFX, S-MFH, and BFH showed immunoexpression for p53 (2/7, 2/4, and 0/7), respectively. p53 mutations were detected in AFX (4/6; 67%) and S-MFH (1/4; 25%), but not in BFH (0/5; 0%) using polymerase chain reaction-single-strand conformation polymorphism, and all of the mutations in AFX were either C-T transitions or at dipyrimidine sites. In conclusion, AFX and S-MFH are both similar fibrohistocytic lesions; however, AFX has high immunoreactivity for CPDs compared with S-MFH, D-MFH, or BFH. These data suggest that CPDs may play an important role in the pathogenesis of AFX.
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PMID:Immunoexpression of ultraviolet photoproducts and p53 mutation analysis in atypical fibroxanthoma and superficial malignant fibrous histiocytoma. 1140 60

Fibrohistiocytic tumors are characterized by the presence of fibroblast like spindle cells and histiocytes. The benign fibrous histiocytoma (dermatofibroma, BFH) as well as the malignant dermatofibrosarcoma protuberans (DFSP) and the malignant fibrous histiocytoma (MFH) belong to this group. A recurrent painful, hard 2 cm tumor on the left hallux of a 54-year-old woman led to an erosion of the underlying phalanx. The patient had suffered from ingrown toenails for more than 10 years. Histologically there was a deep penetrating fibrohistiocytic tumor that grew in a storiform pattern with interspersed foam cells. The tumor was CD34 negative and mitoses were scarce. The diagnosis was benign cellular fibrous histiocytoma (BZFH). BZFH belong to the group of BFH with a high recurrence rate especially after incomplete removal. Damage to the underlying bone has not been reported so far.
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PMID:[Benign cellular fibrous histiocytoma with erosion of the phalanx]. 1271 66

A purely Sub-cutaneous benign fibrous histiocytoma (BFH; dermatofibroma) is rarely reported, as it is usually a dermally located mesenchymal tumour and in absence of supportive immunohistochemical (IHC) studies, it is often misdiagnosed. We are describing a case of a 19-year-old female who presented to the skin outpatient department with a painful swelling on the medial side of her thigh. Fine needle aspiration (FNA) revealed a sub-cutaneous spindle cell (mesenchymal) lesion which was corroborated on histopathology, with differentials of BFH and dermatofibrosarcoma protruberans (DFSP). BFH constitutes a diagnostic dilemma for both clinicians and pathologists, because the lesions share common clinical symptoms, radiological characteristics and histological features with many varied entities. For its subtyping and confirmation; immunohistochemical (IHC) studies were undertaken. In the present case, positivity of IHC markers, vimentin and smooth muscle actin emphatically proved that BFH arose exclusively from the subcutaneous region, with no dermal origin. Also, a negative CD34 immunostaining, along with low B-cell lymphoma 2 (Bcl-2) expression ruled out DFSP (both are strongly expressed in DFSP), MFH and other malignant mesenchymal lesions. Negative CD 68 staining ruled out giant cell lesions and their congeners. This case is worth reporting, as it not only describes a rare case presentation of BFH, but as it also highlights the importance of IHC, thus helping to comprehensively clinch the diagnosis by systematically ruling out other differentials.
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PMID:Subcutaneous dermatofibroma: a rare case report with review of literature. 2495 53