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Cytomorphology of Pleomorphic Fibroma of Skin: a Diagnostic Enigma

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Cytomorphology of Pleomorphic Fibroma of Skin: a Diagnostic Enigma Case Report Cytomorphology of pleomorphic fibroma of skin: A diagnostic enigma ABSTRACT Pleomorphic fibroma (PF) is a benign, polypoid, or dome‑shaped cutaneous neoplasm with cytologically atypical fibrohistiocytic cells. We describe the cytomorphological features of PF retrospectively with histopathological diagnosis in a 38‑year‑old male who presented with 3 × 1.5 cm swelling in the soft tissues of the thigh for 6 months. This lesion is benign despite the presence of pleomorphic or bizarre cells. We review the differential diagnosis of PF with other mesenchymal tumors. To the best of our knowledge, cytomorphological features on fine needle aspiration cytology of this tumor are not yet documented in literature. Key words: Fine needle aspiration cytology; pleomorphic cells; pleomorphic fibroma. Introduction thigh. Fine needle aspiration cytology (FNAC) was done and slides were stained with Giemsa stain. The aspirate yielded Pleomorphic fibroma (PF) of the skin is a rare benign fibrous cellular smears. Background showed metachromatic stromal tumor.[1] The lesion is usually polypoid, located in the dermis, fragments. Cells were pleomorphic having very large nuclei and is formed by coarse collagen bundles with sparse cells. (monster cells) with scanty cytoplasm. Few of the nuclei It is also characterized by the presence of marked cellular showed single nucleoli [Figure 1]. Nuclear membranes atypia and pleomorphism without mitosis.[1] We describe the frequently showed notches, creases, or folds. Cells were cytomorphological features on fine needle aspiration (FNA) lying singly and occasionally forming clusters. These were smears of a histologically and immunohistochemically proven admixed with the spindle cell component along with few case of PF. Cytomorphological features on FNA of this tumor multinucleated cells. Necrosis and mitotic figures were not are not well documented in literature. seen in smears. On FNA, the swelling was diagnosed as fibrohistiocytic lesion with atypical cells and the mass was Case Report subsequently excised. A 38‑year‑old male presented with a superficial, painless, On gross examination, the mass was well circumscribed polypoidal, soft tissue mass since 6 months, measuring measuring 3 × 1.5 cm. The cut section was solid, grayish‑white, about 3 × 1.5 cm in size, and involving the back of the and fibrous. Routine processing and hematoxylin and eosin staining were done. Microscopically, tumor showed thick bundle of haphazardly arranged collagen in the dermis. Access this article online Quick Response Code Spindle‑ and irregularly shaped stellate or multinucleate Website: cells were scattered between the collagen bundles. These www.jcytol.org cells were markedly atypical showing scant cytoplasm and large, pleomorphic, enlarged hyperchromatic nuclei with DOI: small nucleoli [Figure 2]. No mitosis or necrosis was evident 10.4103/0970-9371.107525 in multiple sections examined. Based on these features, diagnosis of PF was made. YADAV YK, KUSHWAHA R1, SHARMA U2, GUPTA K2 Departments of Pathology, Integral Institute of Medical Sciences and Research, 1C.S.M. Medical University (erstwhile KGMC), Lucknow, 2VMMC and Safdarjung Hospital, New Delhi, India Address for correspondence: Dr. Rashmi Kushwaha, Department of Pathology, C.S.M. Medical University (erstwhile KGMC), Lucknow, India. E‑mail: [email protected] Journal of Cytology / January 2013 / Volume 30 / Issue 1 71 Yadav, et al.: Cytomorphology of pleomorphic fibroma of skin Immunoprofile of lesional cells revealed positive staining for Discussion vimentin [Figure 3] and CD34 and negative staining for S‑100. PF of skin was first described by Kamino et al., in 1989.[1] PF is a benign fibrous lesion which is located mainly in the lower extremities, followed by trunk, head, and neck and rarely in the subungual region. Most of these lesions occur in adults, with a peak incidence in the fifth decade of life.[1] Role of FNAC in diagnosis of soft tissue tumor has been fairly documented as well as debated but the cytomorphological features on FNAC of PF are not yet documented in literature.[2] In contrast to the histological findings (which is hypocellular), the FNA smear in our case was cellular. Majority of cells were pleomorphic with very large nuclei like monster cells of dermatofibroma and few of them possessed single Figure 1: Cellular smears containing pleomorphic cells having very large nucleolus. Nuclear membrane showed notches, creases, or nuclei (monster cells) with scanty cytoplasm. Few of the nuclei revealing nucleoli (Giemsa, ×400) 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.[1] This also reveals the importance of mitosis and necrosis in making a diagnosis of malignant soft tissue tumor on cytology, as both these features were not seen in this tumor. Histological examination of PF of the skin reveals a dermal Figure 2: Section showing pleomorphic cells scattered between the collagen tumor with striking nuclear atypia. The neoplastic cells show bundles (H and E, ×200) 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.[1,3] Degeneration, ischemia, or the paracrine influence of mast cells may create the cytological atypia in PF.[1,4,5] Two variants have been described: Myxoid and sclerotic.[6,7] In fact, some investigators have postulated that the PF of the skin is actually a variant of the sclerotic fibroma.[4,8] Other researchers have called these as pleomorphic sclerotic fibromas.[9] Lesional cells in PF are always positive for vimentin and negative for S‑100 and cytokeratin. Positive expression of muscle‑specific actin, CD34 and rarely alpha‑1 Figure 3: Lesional cells revealed positive staining for vimentin (IHC,× 200) antichymotrypsin, has also been observed.[1,3,4,8‑10] 72 Journal of Cytology / January 2013 / Volume 30 / Issue 1 Yadav, et al.: Cytomorphology of pleomorphic fibroma of skin The cytological differential diagnosis of PF of skin has not References been discussed in literature. However, histological differential diagnosis includes: Atypical fibroxanthoma, dermatofibroma 1. Kamino H, Lee JY, Berke A. Pleomorphic fibroma of the skin: A benign neoplasm with cytologic atypia. A clinicopathologic study of eight cases. with monster cells, giant cell fibroblastoma, desmoplastic Am J Surg Pathol 1989;13:107-13. Spitz nevus, and desmoplastic melanoma.[1,3,9,10] 2. Willén H, Akerman M, Carlén B. Fine needle aspiration (FNA) in the diagnosis of soft tissue tumours; a review of 22 years experience. Cytopathology 1995;6:236-47. In our case, bundles of collagen were present with 3. Hsieh YJ, Lin YC, Wu YH, Su HY, Billings SD, Hood AF. Subungual low cellularity, thus ruling out atypical fibroxanthoma pleomorphic fibroma. J Cutan Pathol 2003;30:569‑71. and dermatofibromas with atypical or monster cells. 4. García‑Doval I, Casas L, Toribio J. Pleomorphic fibroma of the skin, Dermatofibromas with atypical or monster cells also a form of sclerotic fibroma: an immunohistochemical study. Clin Exp Dermatol 1998;23:22-4. contains foam cells and hemosiderin‑laden macrophages 5. Pitt MA, Roberts IS. Myxoid cutaneous pleomorphic fibroma. which were not seen in our case. Giant cell fibroblastoma Histopathology 1994;25:300. resembles PF as both contain atypical fibroblastic cells. 6. Miliauskas JR. Myxoid cutaneous pleomorphic fibroma. Histopathology 1994;24:179-81. Giant cell fibroblastoma is usually seen in childhood and is 7. Martín-López R, Feal-Cortizas C, Fraga J. Pleomorphic sclerotic characterized by infiltrative growth and features sinusoidal fibroma. Dermatology 1999;198:69‑72. structures lined by atypical cells, whereas our case was an 8. Mahmood MN, Salama ME, Chaffins M, Ormsby AH, Ma CK, Linden MD, et al. Solitary sclerotic fibroma of skin: A possible link with adult without infiltrative growth and sinusoids. Desmoplastic pleomorphic fibroma with immunophenotypic expression for O13 Spitz nevus and desmoplastic melanoma show some areas of (CD99) and CD34. J Cutan Pathol 2003;30:631 - 6 melanocytic differentiation and cells are positive for S‑100 9. Chen TM, Purohit SK, Wang AR. Pleomorphic sclerotic fibroma: A case report and literature review. Am J Dermatopathol 2002;24:54-8. protein, both these features were absent in our case. Thus, 10. Rudolph P, Schubert C, Zelger BG, Zelger B, Parwaresch R. Differential the final diagnosis of PF was made. expression of CD34 and Ki-M1p in pleomorphic fibroma and dermatofibroma with monster cells. Am J Dermatopathol 1999;21:414‑9. Thus, from a clinical as well as pathological standpoint, How to cite this article: Yadav YK, Kushwaha R, Sharma U, Gupta K. recognition of a benign lesion of this type is very important Cytomorphology of pleomorphic fibroma of skin: A diagnostic enigma. since an incorrect cytological interpretation could result in J Cytol 2013;30:71‑3. inappropriate treatment. Source of Support: Nil, Conflict of Interest: None declared. Journal of Cytology / January 2013 / Volume 30 / Issue 1 73 Copyright of Journal of Cytology is the property of Medknow Publications & Media Pvt. Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use..
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