Recurrent dermatofibrosarcoma protuberans involving the lacrimal sac: A case report

Table 1 Reported cases of isolated dermatofibrosarcoma protuberans involving lacrimal sac and orbit, their clinical manifestations, and their management

Ref.	Patient demographics	Clinical presentation	Imaging findings	Histopathology & IHC	Treatment	Outcome/follow-up
Erickson et al[5], 2015	71/male	Indolent growth above left medial canthus	CT: 2.1 cm × 2.2 cm × 2.5 cm mass extending into medial orbit and proximal nasolacrimal duct	Spindle cells in storiform pattern; CD34-positive; t(17;22) translocation absent	Surgical excision; partial intensity-modulated radiation therapy	No recurrence at 11 months
Schittkowski and Wrede[4], 2013	45/female	Mild epiphora; palpable mass in lacrimal sac	Not specified	Not specified	Surgical excision	Not specified
Sharma et al[14], 2017	50/female	Swelling in the medial aspect of the right eye associated with pain and diminution of vision	MRI: 5.4 cm × 3.8 cm × 4.1 cm in right orbit with extension to the ethmoid and frontal sinus, similar lesion in left medial canthus	Spindle tumor cells are uniform in appearance, with elongated nuclei, little or no pleomorphism, mitotic figures arranged in a storiform manner, CD34-positive and vimentin-positive	Palliative radiotherapy 20 Gy in five fractions, followed by tab Imatinib 400 mg per day increased to 800 mg per day after 2 years	Stable disease on tablet imatinib 800 mg per day on last follow-up
Rahman et al[15], 2013	70/female	Painless progressive swelling of left eye for 4 years	MRI: Well-defined lobulated soft tissue mass in the superolateral aspect of the left orbit measuring 58 mm × 42 mm × 38 mm, occupying the retro-orbital compartment with expansion of the bony wall and stretching of the optic nerve	Spindle cell tumor composed of slender to plump spindle cells arranged in storiform and focal pericytoma pattern, CD34-positive, S100-negative	Total orbital exenteration of the left eye with split-thickness skin graft from the thigh	No recurrence at 24 months
Present case	32/female	Recurrent swelling inferior to right medial canthus for 6 years	CT: Soft tissue mass extending from medial canthus to nasolacrimal duct, causing canal widening	Storiform spindle cell proliferation; CD34-positive; STAT6-negative	Wide local excision with Mustarde flap reconstruction	No recurrence at 2-year follow-up

CD34: Cluster of differentiation 34; CT: Computed tomography; IHC: Immunohistochemistry; MRI: Magnetic resonance imaging; STAT6: Signal transducer and activator of transcription 6.