Two Cases of Intraocular Undifferentiated Pleomorphic Sarcoma

Abstract

Introduction: Undifferentiated pleomorphic sarcoma (UPS), formerly known as malignant fibrous histiocytoma, is a high-grade soft tissue sarcoma arising from mesenchymal stem cells. UPSs are rare and account for about 5% of all soft tissue sarcoma. UPSs arising in the head and neck are especially rare, comprising 1-3% of all UPSs.

Case presentation: In this report, we describe 2 cases of intraocular UPS. Both cases concern 68-year-old males: one developing a UPS in an eviscerated socket after a chronic fibrinous inflammation and the other years after ocular trauma.

Conclusion: Our cases may support the hypothesis of chronic inflammation playing a role in sarcoma formation as they are characterized by a longstanding history of (surgical) trauma with signs of chronic inflammation and phthisis bulbi.

Keywords: Adult ocular oncology; Adult ocular tumours; Intraocular tumours; Ocular oncology; Orbital tumour; Sarcoma.

Fig. 1.

Clinical photos of patient 1, left eye. a Pre-pupillary fibrotic plaque and posterior synechiae. b One year later, rubeosis of the iris and edema of the peripheral cornea. c Left socket: 6 months after evisceration, protrusion of the implant is noted with redness and swelling of the conjunctiva.

Fig. 2.

Patient 1, MRI: mass in the left orbit, located anteriorly to the implant. The lesion demonstrates low intensity on T1-weighted images, intermediate intensity on T2-weighted images, and high contrast enhancement.

Fig. 3.

Patient 1, histopathology of the exenterated tissue. a Cytological and nuclear pleomorphism, spindle cells, epithelioid cells, and histiocytes. SMA (b) and caldesmon (c) show moderate positive staining. d Keratin stain was interpreted as aspecific.

Fig. 4.

Patient 2, left eye: opacity in the anterior chamber and conjunctival lesions and hyperemia.

Fig. 5.

Patient 2, MRI: tumor located at the posterior aspect inside the left globe (a) and the extension into the optic nerve (b) (arrow).

Fig. 6.

Patient 2, histopathology of the exenterated tissue. a Cells with large nuclei, vacuolization, and inconspicuous nucleoli in ample eosinophilic cytoplasm. b, d Pankeratin shows weak positive staining; keratin 7 and other keratins were negative. c The CNV plot of the methylation profiling shows losses of 1p, 9p, and 13q and gains of 5p and 7p, a pattern that can typically be seen with pleiomorphic undifferentiated sarcoma.