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Year : 2022  |  Volume : 65  |  Issue : 3  |  Page : 726-728
Primary orbital dermatofibrosarcoma protuberans without dermal involvement---A rare presentation

1 Oculoplasty and Paediatric Ophthalmology Services, Dr Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences (AIIMS), New Delhi, India
2 Department of Ocular Pathology, Dr Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences (AIIMS), New Delhi, India
3 Department of Ophthalmology, Northern Railway Central Hospital, New Delhi, India

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Date of Submission27-Jun-2021
Date of Decision30-Oct-2021
Date of Acceptance25-Nov-2021
Date of Web Publication21-Jul-2022

How to cite this article:
Das D, Arnav K, Sen S, Bajaj MS, Gupta S, Agrawal S. Primary orbital dermatofibrosarcoma protuberans without dermal involvement---A rare presentation. Indian J Pathol Microbiol 2022;65:726-8

How to cite this URL:
Das D, Arnav K, Sen S, Bajaj MS, Gupta S, Agrawal S. Primary orbital dermatofibrosarcoma protuberans without dermal involvement---A rare presentation. Indian J Pathol Microbiol [serial online] 2022 [cited 2022 Aug 15];65:726-8. Available from: https://www.ijpmonline.org/text.asp?2022/65/3/726/351613

Dear Editor,

Dermatofibrosarcoma protuberans is a rare mesenchymal tumor of cutaneous origin. It can be primarily originating from mesenchymal tissues of the orbit, invasion from adjacent structures like eyelid, forehead or scalp or in rare cases, a distant metastasis. The tumor rarely involves the periorbital area.

A 34-year-old male reported with abaxial proptosis and inferior dystopia in the right eye, which was painless and gradually progressed over 1.5 years [Figure 1]a. Ophthalmic examination was within normal limits. On Hertle's exopthalmometry, there was a proptosis in right eye of 6 mm. The mass, on palpation, was firm, non-tender, immobile and non-pulsatile. There were no surrounding skin lesions/changes or lymphadenopathy.
Figure 1: (a) Clinical picture of the patient shows abaxial proptosis with inferior dystopia. (b and c) Coronal and saggitalsection of contrast-enhanced computed tomography of the orbit, showing homogenously enhancing extraconal lesion in the superior aspect of right orbit with bony destruction and resultant proptosis. No intracranial extension noted (d and e) Clinical picture of the patient at 3 months of follow up

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On ultrasonography, the mass lesion was heteroechoic with low flow on color doppler. CECT head and orbit showed homogenously enhancing extraconal lesion in the superior aspect of right orbit with bony destruction [Figure 1]b and [Figure 1]c. There was no sinuses involvement and no intracranial extension.

The lesion was removed en mass with clear margins through anterior orbitotomy. The tumor was 22 mm in the largest dimension [Figure 2]d, and microscopic examination showed a spindle cell tumor arranged in a storiform pattern [Figure 2]a. There was no pallisading characteristic of nerve sheath tumors, presence of lipoblasts, presence of inflammatory cells, multinucleate giant cells or foamy histiocytes. The edge of the tumor was invading the surrounding dermis. There was no pleomorphism and occasional mitosis [Figure 2]b; immunostains were positive for CD 34 and negative for S-100, SMA and desmin [Figure 2]c. Ki67 proliferative index was intermediate <10%. Based on the above features, a diagnosis of dermato fibrosacrcoma protuberans was made. The patient was referred to the department of oncology. A full metastatic workup was done, and no other abnormality could be detected. The postoperative period was uneventful, and he has been on regular follow-up for the last 12 months [Figure 1]d and [Figure 2]e.
Figure 2: (a) Light microscopy shows spindle-shaped tumor cells, arranged in a storiform pattern (Hematoxylin- eosin, ×200). (b) Higher magnification demonstrates interlacing tumor fascicles with occasional mitotic figure (Hematoxylin-eosin, x400). (c) Cytoplasmic CD-34 positivity is seen in the tumor cells (Avidin-Biotin × 400) with the inset showing Ki-67 labelling index was <10% (Avidin Biotin × 400) (d) Gross specimen of the resected tumor

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Dermatofibrosarcoma Protruberans, a rare fibrohistiocytic tumor of cutaneous origin, is characterised by a pattern of slow infiltrative growth and a tendency to recur locally after surgical excision.[1] With an incidence of nearly 1:100,000, it represents about 1--2% of all sarcomas.[2] The mean age of presentation is 20--39 years, with a slight female predominance (53.1%). It classically presents as an area of cutaneous thickening/plaque, usually associated with telangiectasia of the surrounding skin, which gradually enlarges to produce a nodular mass.[3]

Orbital DFSP is a rare presentation of this rare entity. A few of those are primary orbital DFSP with involvement of immediate periorbital area like periocular skin, orbital rim, lid margin, brow, and lacrimal sac, with others being metastasis to the orbit.[4] On radiologic imaging, DFSP appears as a nodular soft tissue mass involving surrounding skin and subcutaneous tissue. The signal intensity of lesion in MRI is non-specific, as is the tissue attenuation on CT scan. Hence, radiological imaging is useful to assess location and extent of the mass, its relation to underlying tissue and assessment of its nodular/lobular architecture.[5] The diagnosis is confirmed by histopathology and immunohistochemistry. Histologically, it is highly cellular and composed of monomorphic, fusiform cells with elongated nuclei arranged in irregular interwoven fascicles forming a storiform pattern, with these cells intersecting at tight right angles around central vessels.[6] Invasion of underlying dermis in a honey comb pattern is characteristic of DFSP. Lack of pallisading and lipoblasts ruled out other soft tissue tumors including schwannoma and liposarcoma. Occasional mitotic figures may be seen (0-3MF/10 HPF). Immunohistochemically, the tumour is positive for CD34, vimentin and negative for S-100, although S-100 positivity has been reported in some extremely rare cases.

The differential diagnosis includes benign tumors like dermoid cyst, venolymphatic malformations, tumors of lacrimal gland, orbital tumor, nerve sheath tumor, metastatic mass and non-specific orbital inflammation, resulting in a pseudotumor. Dermoid cyst and vascular malformations were ruled out because of the age of presentation and disease progression.[7] Nerve sheath tumor and orbital pseudotumor remained unlikely owing to its association with axial proptosis.[8] Based on the location and presence of bony destruction, as determined by radiological imaging, the primary differential remained an orbital tumor of unspecified origin, for which en bloc removal and subsequent biopsy was done. The classical histopathological and immunohistochemical patterns, combined with the low mitotic count typical of a low-grade tumour, helped us reach the diagnosis of orbital DFSP in our case. Metastatic disease was later ruled out with metastatic work up.

Surgical resection with wide margins of 2–5 cm, adjuvant RT to decrease local recurrence, and Imatinib in cases of surgically unresectable, recurrent and metastatic DFSP, is the mainstay of treatment.

To conclude, DFSP is a rare tumor of fibrohistiocytic lineage, originating primarily from the dermis, with extension into deeper tissues. The storiform arrangement on histopathology, CD34 positivity and S-100 negativity on immunohistochemistry usually clinch the diagnosis. The tumour has metastatic potential, with a high risk of local recurrence. Surgical resection, with a wide tumor free margin, is the mainstay of treatment.

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

Jeremic J, Stefanovic A, Jeremic K, Jovic M, Pilic I, Cvetkovic A, et al. Giant dermatofibrosarcoma protuberans vulvae: Rare clinical presentation and literature review. J BUON 2019;24:1289-95.  Back to cited text no. 1
Schittkowski MP, Wrede A. Dermatofibrosarcoma protuberans with primary orbital manifestation. Orbit 2013;32:117-9.  Back to cited text no. 2
Rahman T, Bhattacharjee K, Sarma JD, Dey D, Kuri G. Primary dermatofibrosarcoma protuberans of orbit--A rare entity. Orbit 2013;32:127-9.  Back to cited text no. 3
Nakra T, Cook T, Douglas RS, Goldberg RA. Dermatofibrosarcoma protuberans metastatic to the orbit. Arch Ophthalmol 2004;122:1240-1.  Back to cited text no. 4
Zhang L, Liu QY, Cao Y, Zhong JS, Zhang WD. Dermatofibrosarcoma protuberans: Computed tomography and magnetic resonance imaging findings. Medicine (Baltimore) 2015;94:e1001.  Back to cited text no. 5
Larbcharoensub N, Kayankarnnavee J, Sanpaphant S, Kiranantawat K, Wirojtananugoon C, Sirikulchayanonta V. Clinicopathological features of dermatofibrosarcoma protuberans. Oncol Lett 2016;11:661-7.  Back to cited text no. 6
Choi JS, Bae YC, Lee JW, Kang GB. Dermoid cysts: Epidemiology and diagnostic approach based on clinical experiences. Arch Plast Surg 2018;45:512-6.  Back to cited text no. 7
Sharma A, Singh D, Saran R. Primary optic nerve sheath schwannoma: A case report. Br J Neurosurg 2021:1-3. doi: 10.1080/02688697.2020.1869181.  Back to cited text no. 8

Correspondence Address:
Sahil Agrawal
Oculoplasty and Pediatric Ophthalmology Services, Dr Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi - 110 029
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijpm.ijpm_666_21

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