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Year : 2022  |  Volume : 2  |  Issue : 3  |  Page : 754-756

Atypical fibroxanthoma: Case report of a rare conjunctival tumor

1 Department of Ophthalmology, Mysore Medical College and Research Institute, K.R. Hospital, Irwin Road, Mysore, Karnataka, India
2 Center for Ocular Pathology and Education (COPE), Narayana Nethralaya, NH Health City Campus, Bommsandra, B'lore-99, Karnataka, India

Date of Submission22-Feb-2022
Date of Acceptance10-May-2022
Date of Web Publication16-Jul-2022

Correspondence Address:
Dr. Sandhya Dharwadkar
Mysore Medical College and Research Institute, K.R. Hospital, Irwin Road, Mysore, Karnataka
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_511_22

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Atypical fibroxanthoma (AFX) is a rare tumor arising from conjunctival stroma, which mimics other malignancies leading to inadvertent aggressive management. We hereby report a case of an elderly male patient with a well-circumscribed mass in superior bulbar conjunctiva of 6 months duration causing corneal scarring in his left eye. He underwent excision biopsy and was suspected to have spindle cell carcinoma based on histopathological features. Immunohistochemistry (IHC) ruled out the epithelial tumors and confirmed the diagnosis of AFX, which usually follows a benign course. Thus, this case emphasizes the importance of IHC as a diagnostic tool for ocular surface tumors.

Keywords: Atypical fibroxanthoma, conjunctival tumor, immunohistochemistry, spindle cell carcinoma

How to cite this article:
Dharwadkar S, Hegde S, Kamal SS, Krishna SM. Atypical fibroxanthoma: Case report of a rare conjunctival tumor. Indian J Ophthalmol Case Rep 2022;2:754-6

How to cite this URL:
Dharwadkar S, Hegde S, Kamal SS, Krishna SM. Atypical fibroxanthoma: Case report of a rare conjunctival tumor. Indian J Ophthalmol Case Rep [serial online] 2022 [cited 2022 Oct 6];2:754-6. Available from: https://www.ijoreports.in/text.asp?2022/2/3/754/351198

Atypical fibroxanthoma (AFX) is a cutaneous tumor of mesenchymal origin, usually affecting the head and neck region. AFX of the ocular surface is a very rare tumor, reported in less than 10 cases and none from India. It is often misdiagnosed as spindle cell carcinoma, an aggressive variant of squamous cell carcinoma (SCC) due to its similar morphological appearance. We report a case of AFX arising from the superior bulbar conjunctiva in a 70-year-old male patient, which was diagnosed based on histopathology (HPE) and immunohistochemistry (IHC).

  Case Report Top

A 70-year-old, healthy male patient presented with a gradually progressive, painless mass in the left eye over the past 6 months with blurred vision. On ocular examination, the left eye had a well-circumscribed, soft to firm, pink mass in the superior bulbar conjunctiva with a smooth surface and feeder vessels. It was overhanging the cornea causing a central opacity [Figure 1]a. The best-corrected visual acuity (BCVA) was counting fingers close to the face. Fundus examination could not be performed. The right eye was normal.
Figure 1:(a) Clinical photograph of the left eye showing a pink, smooth, conjunctival mass in the superior bulbar conjunctiva overhanging the cornea and the corneal opacity. (b): Clinical photograph post excision showing the sutured wound superior to the limbus

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The patient underwent an excision biopsy of the conjunctival lesion. Gross examination of the specimen revealed a non-encapsulated, gray–black tissue mass, measuring 2.5 × 1.5 cm. On the cut section, a gray–white, ill-defined tumor was seen measuring 1.8 × 1.0 cm with no areas of necrosis or hemorrhage. On microscopy, the tumor cells were spindle-shaped with scanty cytoplasm, vesicular to hyperchromatic nuclei, and prominent nucleoli. Plenty of mitotic figures, tumor giant cells, and focal myxoid changes with pleomorphism were noted. There was absence of necrosis, deeper extension, and lymphovascular or perineural invasion. Based on this, a diagnosis of spindle cell tumor was made [Figure 2]. IHC was done to rule out different spindle cell tumors such as spindle cell carcinoma, amelanotic melanoma, malignant neurilemmoma, and undifferentiated pleomorphic sarcoma. IHC was strongly positive for CD68 [Figure 3] and negative for pan-cytokeratin, HMB45, desmin, MyoD1, and CD34. It showed Ki-67 positivity of 60% suggestive of high proliferative potential. The features of HPE and IHC combined suggested a diagnosis of AFX. The patient did not have any recurrence during the follow-up of 1 year and did not wish for any further surgery for vision improvement [Figure 1]b.
Figure 2:(a) Cut section showing well-circumscribed gray–white tumor measuring 1.8*1.0 cm. (b) Scanner view (40×) showing a spindle cell tumor. (c): Low power magnification (100×) showing spindle cells with marked nuclear pleomorphism and atypia, no necrosis was seen. (d) High power magnification (400×) showing atypical mitoses (red arrows) and one multinucleated tumor giant cell (white arrow)

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Figure 3: Immunohistochemistry staining (Dako kit) 40× magnification showing patchy CD68 cytoplasmic positivity by the tumor cells (red arrow)

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  Discussion Top

The term AFX was first proposed by Helwig[1] in 1961 and then described by Lund and Kraus.[2] The name was derived from the large xanthomatous-looking cells with fibrocystic cells and marked as cellular atypia.[3] Patients with xeroderma pigmentosa are considered at high risk for developing various skin malignancies; however, only two cases of conjunctival AFX have been reported in such patients.[4] Prolonged sun exposure is considered a predisposing factor for these tumors affecting the head and neck region, although rarely reported in ocular cases[5] Our patient was an elderly farmer and prolonged sunlight exposure could have been a causative factor in his case. AFX is a mesenchymal tumor arising from the stromal layer of conjunctiva sparing the epithelium and hence is seen as pale pink, well-delineated, and pedunculated mass with some reports of overlying ulceration. It has been reported as a single or multiple lesions along the limbus[5],[6] or inferior[6] bulbar conjunctiva and two cases from nasal[7],[8] bulbar conjunctiva. Ours is the first reported case arising from superior bulbar conjunctiva away from the limbus. Histopathology shows pleomorphic spindle cells and multiple atypical mitotic figures with high proliferative potential.[9] IHC is usually positive for CD-68, CD-10, CD-99, and more importantly negative for pan-cytokeratin, desmin, S-100, HMB-45, and CD-34 amongst others.[9] In our case, negative pan-cytokeratin ruled out the commonest spindle cell carcinoma. Strong CD-68 positivity with HMB-45 negative helped to rule out amelanotic melanoma and favor AFX. IHC thus helped to differentiate AFX from most of the spindle cell tumors. Koch et al.[9] in their review paper state various other markers seen with AFX such as positive vimentin, pro-collagen 1, CD1a, fascin, and negative p53, nerve growth factor receptor, CD15, CD-31, and EMA. The lack of invasive features on HPE ruled out undifferentiated pleomorphic sarcoma, which is highly proliferative and has a poor prognosis and potential to recur.[9] AFX, despite its malignant histopathologic features usually follows a benign course with a good prognosis. The mainstay of management is surgical excision with cryotherapy of the edges and reconstruction as required and diligent follow-up. In our case, the overlying epithelium was healthy and hence the tumor could be clearly removed in toto without many defects thus allowing for primary closure [Figure 1]b. Local recurrences have been reported only in a few cases so far.[10] Magnetic resonance imaging (MRI) orbit helps to rule out the deeper spread and can be added in the diagnostic workup. We report a rare case of AFX of the conjunctiva, which is easily misdiagnosed on routine histopathology as spindle cell carcinoma that almost always presents with recurrence and scleral and extraocular spread even after wide excision and cryoradiotherapy.

  Conclusion Top

With this case report, we highlight the role of IHC in guiding the diagnosis of spindle cell tumors of the conjunctiva when combined with histopathological features. IHC in spite of being an expensive investigation can add a lot of information and should be included in the diagnosis of ocular surface tumors. It can help to prognosticate such cases and avoid unnecessary exenteration and anxiety.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Helwig EB. Atypical fibroxanthoma: Proceedings of the 18th Annual Tumor Seminar San Antonio Society of Pathologists; 1961.  Back to cited text no. 1
Lund HZ, Kraus JM. Melanotic Tumors of the Skin. Atypical Fibroxanthoma; Comparison with Malignant Melanoma. Armed Forces Institute of Pathology; 1962.  Back to cited text no. 2
Helwig EB. Atypical fibroxanthoma. Tex State J Med 1963;664.  Back to cited text no. 3
Shalabi N, Galor A, Dubovy SR, Thompson J, Bermudez-Magner JA, Karp CL. Atypical fibroxanthoma of the conjunctiva in xeroderma pigmentosum. Ocul Oncol Pathol 2015;1:254-8.  Back to cited text no. 4
Kuiper J, Shah A, Kuennen R, Schoenfield L. Atypical fibroxanthoma of the bulbar conjunctiva: A unique case describing the pathology, clinical presentation, and management. Ocul Oncol Pathol 2018;4:152-6.  Back to cited text no. 5
Monsalve P, Garcia AL, Zhou XY, Elgart GW, Karp CL, Johnson TE, et al. Atypical fibroxanthoma affecting the ocular surface and adnexa: A case series.Invest Ophthalmol Vis Sci2019;60:3574.  Back to cited text no. 6
Shieh C, Daluvoy MC, Ellington KS, Praia AD. Atypical fibroxanthoma of the bulbar conjunctiva. Cornea 2015;34:1620-2.  Back to cited text no. 7
Rathore D, Mohyudin MN, Mehta P, AhluShieh C, Ahluwalia HS. Atypical fibroxanthoma of the medial canthus: A rare presentation. Orbit 2013;23:194-6.  Back to cited text no. 8
Koch M, Freundl AJ, Agaimy A, Kiesewetter F, Künzel J, Cicha I, et al. Atypical fibroxanthoma-histological diagnosis, immunohistochemical markers and concepts of therapy. Anticancer Res 2015;35:5717-35.  Back to cited text no. 9
Ullrich K, Wells J, Brennan C, Craig J. Recurrent atypical fibroxanthoma of the limbus. BMJ Case Rep 2013;2013:bcr2013008762.doi: 10.1136/bcr-2013-008762.  Back to cited text no. 10


  [Figure 1], [Figure 2], [Figure 3]


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