Primary Pleomorphic Liposarcoma of the Orbit: A Case Report

Abstract

To our knowledge, pleomorphic liposarcoma (PL) of the orbit has only been reported in the literature four times. This rarity makes it more difficult to diagnose and to treat in this clinical setting. A 62-year-old female presented with pruritus, edema, proptosis and diplopia 5 months OS. Imaging revealed an intraorbital mass displacing the globe, with infiltration into the sinus. The tumor was removed and the histological examination revealed a highly cellular tumor with heterogenous histology, with a few vacuolated cells and many malignant features. Immunohistochemistry allowed for the differential diagnosis, resulting in a diagnosis of PL of the orbit. The cells were immuno-positive for S-100 and negative for all other relevant markers. According to the literature, prognosis for this neoplasm is quite poor, and exenteration represents the best treatment option. The patient refused exenteration and radiation therapy, however, at 2 year follow-up, she remained recurrence-free.

Carunclar mass as the presenting sign of primary orbital liposarcoma

“…The most common histological type is the myxoid form (55%). [34] Our present case is myxoid subtype. Due to compression of the orbital structures, manifest signs and symptoms generally consist of painless palpebral fullness, proptosis, reduced visual acuity and occasionally, ptosis.…”

“…Due to compression of the orbital structures, manifest signs and symptoms generally consist of painless palpebral fullness, proptosis, reduced visual acuity and occasionally, ptosis. [12345] Because of the infrequency of primary orbital liposarcoma and lack of data, it is difficult to study the behavior and prognostic factors for this kind of tumor. CT scans usually demonstrate a well-defined heterogeneous orbital mass, negative attenuation compatible with fat, and both intra- or extra-conal density.…”

“…MRI shows high signal intensity on T1 images and low signal intensity on T2. [456] Because of the lack of specific radiological signs, the tumor must be diagnosed by biopsy. Our case presented with a caruncular mass and to the best of our knowledge, it is the first reported case of caruncle mass as the presenting sign of primary orbital liposarcoma.…”

Primary pleomorphic liposarcoma of fallopian tube with recurrence: a case report and review of the literature

“…It is rare and only accounts for 5% of liposarcoma cases [ 1 ]. It has been reported that PLS originates primarily in the orbit [ 6 ] and uterus [ 7 ]. The present report may present the first case of primary fallopian tube PLS.…”

Pleomorphic liposarcoma: A cytologic study of five cases

“…PLS is a rare variant of LS with a poor prognosis. These tumors are most commonly located in the upper or lower extremity but PLS has also been reported in a variety of other sites including: the orbit, retrotonsillar fossa,parotid as well as the mediastinum, pericardium, lung,breast,and bone, dura, and subcutaneous tissues . Location of a primary lesion in a nonextremity site appears to be associated with an even more aggressive course and worse outcome than with an extremity based tumor .…”