Atypical Lipomatous Tumor/Well-Differentiated Liposarcoma of the Orbit: Three Cases and Review of the Literature

Abstract

The authors present 3 patients from this retrospective case series to review the clinical findings, imaging, pathology, and treatment of orbital atypical lipomatous tumor/well-differentiated liposarcoma. Pathology of biopsy specimens ranged from spindle cell proliferations mimicking neurofibroma to proliferations of well-differentiated adipocytes. Immunohistochemical stains were positive for murine double minute 2 in 1 case, and fluorescent in situ hybridization showed amplification of murine double minute 2 in 2 cases. Treatments ranged from serial debulking, proton beam irradiation, and exenteration. None of the patients developed metastases. A literature review supported the low-grade nature of this lesion. Orbital atypical lipomatous tumor/well-differentiated liposarcoma is a low-grade, indolent liposarcoma that may be locally invasive. The histologic diagnosis is enhanced with immunohistochemical staining for murine double minute 2 and fluorescent in situ hybridization analysis for amplification of murine double minute 2. Although treatment may vary according to the individual, conservative therapies may be attempted prior to radical surgery.

Recurrent primary orbital well-differentiated liposarcoma /atypical lipomatous tumor: A rare case report with six-year follow-up

“… 2 So far, less than 70 primary orbital liposarcoma cases have been reported and less than 30 of them have been the WDL/ALT subtype, slightly more than myxoid/round cell type. 5 , 6 , 7 , 8 , 9 , 10 , 11 Although WDL/ALT is considered to have the least potential to metastasize among all subtypes of liposarcomas, local invasion or recurrence have been reported. 2 , 5 , 6 , 11 , 12 Only four of 29 cases in the literature have been followed up for at least six years as in our current case.…”

“… 5 , 6 , 7 , 8 , 9 , 10 , 11 Although WDL/ALT is considered to have the least potential to metastasize among all subtypes of liposarcomas, local invasion or recurrence have been reported. 2 , 5 , 6 , 11 , 12 Only four of 29 cases in the literature have been followed up for at least six years as in our current case. Three out of four of these cases had local recurrence.…”

Orbital Liposarcoma: A Surveillance, Epidemiology and End Results Database Study

“…While liposarcoma is the most common soft-tissue sarcoma in adulthood, in the orbit, this tumor is exceedingly rare, with only 70 reported cases to date. 1–27 In part due to its rarity, orbital liposarcoma remains a challenge to treat. Surgery is the mainstay of management; however, local recurrence is common and most patients eventually require exenteration.…”