The Billings Enigma

Tess Livingstone
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These cells were markedly atypical showing scant cytoplasm and large, pleomorphic, enlarged hyperchromatic nuclei with small nucleoli [ Figure 2 ]. No mitosis or necrosis was evident in multiple sections examined.

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Based on these features, diagnosis of PF was made. Immunoprofile of lesional cells revealed positive staining for vimentin [ Figure 3 ] and CD34 and negative staining for S PF of skin was first described by Kamino et al. Most of these lesions occur in adults, with a peak incidence in the fifth decade of life.

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Majority of cells were pleomorphic with very large nuclei like monster cells of dermatofibroma and few of them possessed single nucleolus. Nuclear membrane showed notches, creases, or folds. Few cells were multinucleated. Cells were having scanty cytoplasm or were devoid of cytoplasm. Small fragments of metachromatic stroma were present in the background representing collagen bundles. Necrosis and mitotic figures were not seen in the smears. Thus, on FNA, PF can easily be misdiagnosed as malignant soft tissue tumor because it contains bizarre pleomorphic cells showing considerable atypia.

This lesion has been shown to be benign despite the presence of pleomorphic or bizarre cells, although lesion may locally recur when incompletely excised. Histological examination of PF of the skin reveals a dermal tumor with striking nuclear atypia. The neoplastic cells show large pleomorphic and hyperchromatic nuclei with small nucleoli. In addition to these mononuclear cells, the atypical nuclear features can also be observed in multinucleated giant cells.

Lesional cells in PF are always positive for vimentin and negative for S and cytokeratin. Positive expression of muscle-specific actin, CD34 and rarely alpha-1 antichymotrypsin, has also been observed. The cytological differential diagnosis of PF of skin has not been discussed in literature.

However, histological differential diagnosis includes: Atypical fibroxanthoma, dermatofibroma with monster cells, giant cell fibroblastoma, desmoplastic Spitz nevus, and desmoplastic melanoma. In our case, bundles of collagen were present with low cellularity, thus ruling out atypical fibroxanthoma and dermatofibromas with atypical or monster cells.

Dermatofibromas with atypical or monster cells also contains foam cells and hemosiderin-laden macrophages which were not seen in our case. Giant cell fibroblastoma resembles PF as both contain atypical fibroblastic cells. Giant cell fibroblastoma is usually seen in childhood and is characterized by infiltrative growth and features sinusoidal structures lined by atypical cells, whereas our case was an adult without infiltrative growth and sinusoids.

Desmoplastic Spitz nevus and desmoplastic melanoma show some areas of melanocytic differentiation and cells are positive for S protein, both these features were absent in our case. Thus, the final diagnosis of PF was made.

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Thus, from a clinical as well as pathological standpoint, recognition of a benign lesion of this type is very important since an incorrect cytological interpretation could result in inappropriate treatment. Source of Support: Nil. Conflict of Interest: None declared.

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Journal List J Cytol v. J Cytol. R Kushwaha 1 Department of Pathology, C. Author information Copyright and License information Disclaimer. Address for correspondence: Dr. Rashmi Kushwaha, Department of Pathology, C. E-mail: ni.

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This is an open-access article distributed under the terms of the Creative Commons Attribution-Noncommercial-Share Alike 3. This article has been cited by other articles in PMC. Abstract Pleomorphic fibroma PF is a benign, polypoid, or dome-shaped cutaneous neoplasm with cytologically atypical fibrohistiocytic cells. Keywords: Fine needle aspiration cytology, pleomorphic cells, pleomorphic fibroma. Introduction Pleomorphic fibroma PF of the skin is a rare benign fibrous tumor. Open in a separate window. Maryland Online Classifieds.

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