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The tumour occurs on the trunk, particularly on the chest and shoulders, in 40%–50% of cases; on the proximal parts of the limbs in 30%–40% of cases; and on the scalp, cheeks, and supraclavicular area in 10%–15% of patients.
This report describes the clinical, imaging, and histopathological characteristics of 2 distinct DFSP developing on the forehead and lower eyelid in a middle-aged female.
This study adhered to the tenets of the Declaration of Helsinki. The patient subject to this study gave informed consent for the use of all medical information documented in the hospital chart and all imaging studies pertaining to the ocular disease, for scientific purposes including but not limited to presentations, journal articles and book chapters.
Case Report
A 58-year-old female presented with a painful left lower eyelid mass that gradually enlarged within 3 months. She had a similar lesion on her forehead for many years, for which she had undergone radiotherapy a year earlier at another institution (Fig. 1A). On ocular examination, her visual acuity was 20/30 in each eye. There was an immobile, indurated, and tender nodular lesion measuring approximately 3 cm × 2 cm × 2 cm on an erythematous base on her left lower eyelid (Fig. 1B). The rest of ocular examination was noncontributory. Orbital magnetic resonance imaging (MRI) study disclosed a cutaneous lesion on the left lower eyelid with heterogeneous signal intensity and diffuse contrast enhancement without orbital extension (Fig. 1C). Incisional biopsy was performed from both lesions and the diagnosis of DFSP was established. The patient gave consent for wide local excision only for the eyelid tumour. Histopathological evaluation of the whole-eyelid lesion showed a cellular dermal neoplasm with infiltrative borders into the muscle and fat, forming a “honeycomb” pattern (Fig. 2A). The tumour had a prominent storiform pattern at high magnification and was composed of spindle cells with scant pale cytoplasm and elongated nuclei with minimal pleomorphism (Fig. 2B). Mitotic activity was lower than 5 per 10 high-power fields. Immunohistochemically, the neoplastic cells stained positive for CD34 (Fig. 2C) and vimentin, and negative for factor XIIIA, EMA, S100, desmin, myogenin, HHV8, CD31, and bcl-2. The Ki-67 index was 5%. There were no areas of necrosis or fibrosarcomatous transformation. The surgical margins of the eyelid specimen were reported to be free of tumour cells.
Fig. 1(A) The forehead lesion at presentation, which was biopsied and then treated with radiotherapy a year earlier. (B) The eyelid tumour was densely vascular and hard on palpation.
(C) Orbital T1-weighted axial magnetic resonance imaging scan showed diffuse enhancement of the relatively well-delineated tumour after contrast agent administration.
Fig. 2(A) The cellular spindle cell neoplasm involved the whole dermis extending into the subcutaneous fat and sparing the skin appendages (H&E, ×40). (B) The monomorphic tumour cells formed prominent storiform patterns and had scant eosinophilic cytoplasm and hyperchromatic nuclei. The entrapped fat cells displayed honeycomb pattern (H&E, ×100). (C) Neoplastic cells were CD34 positive (CD34, ×100).
The forehead tumour received adjunct radiotherapy at 60 Gy in fractionated doses, and at 20 months of follow-up, the eyelid remained recurrence-free and the forehead tumour was stable.
Discussion
DFSP is usually a slow-growing fibroblastic/myofibroblastic tumour with variable clinical presentations and growth patterns.
Although the 2 lesions in our patient were histologically identical, their clinical courses were distinctly different; the forehead lesion developed over 20 years and the eyelid tumour emerged only in 3 months.
The MRI findings are not specific and demonstrate a well-delineated tumour that is isointense or mildly hyperintense on T1-weighted images and hyperintense on T2-weighted scans.
However, because DFSP is histologically poorly circumscribed and often extends into the dermis, subcutis, and muscles, it is advisable to resect deeper and wider from the margins that are appreciated clinically or on MRI studies.
Documented predisposing factors favouring the development of DFSP include surgical scars, radiodermatitis, sites of multiple immunizations, old burns, and sites of central venous lines.
The genetic background of DFSP has recently been elucidated. In more than 90% of patients, the t(17;22) (q22;q13) translocation is present, which leads to the fusion of COL1A1 and PDGFB genes.
The gene product acts as an autocrine factor involved in Ras-MAPK, PI3K-AKT-mTOR signalling pathways and is a potent mitogen-stimulating proliferation, differentiation, and migration.
Imatinib mesylate, a tyrosine kinase inhibitor, has been shown to be effective in locally inoperable or metastatic DFSP, and identifying this genetic rearrangement may become mandatory in selected patients.
We did not determine the genetic status of the tumour in our patient.
Securing microscopically negative surgical margins is the cornerstone of treatment in DFSP because recurrence rates after simple excisional biopsy varies between 20% and 70%.
showed that a margin of ≤2 cm was associated with a recurrence of 60%, whereas a margin of >2 cm was associated with a recurrence rate of 20%. A study on 29 patients suggested that the use of Mohs’ micrographic surgery lowered the recurrence rate to 1% and this may become the treatment of choice in most cases.
Radiotherapy has re-emerged as a useful adjunct tool in large or incompletely resected tumours, and a recent study on 14 patients with a median follow-up of 10.5 years found that with 55.8–66 Gy radiation dose, 86% of patients became disease-free.
Our patient is a rare example of DFSP developing consecutively as 2 separate tumours on the forehead and eyelid with identical histology but disparate clinical courses.
Disclosure
The authors have no proprietary or commercial interest in any materials discussed in this article.
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