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

Abstract

Purpose: Orbital liposarcoma is a challenging tumor to treat due to its rarity and high rate of local recurrence, and the role of radiotherapy and chemotherapy remain unclear. Analysis of big data may improve our overall understanding of orbital disease and role of adjuvant therapies. Methods: Data were extracted from the Surveillance, Epidemiology and End Results (SEER) Research Plus database from 1975 to 2017. All patients with a diagnosis of liposarcoma (ICD-O3 codes 8850-8858, 8869-8862, 8870, 8880, 8881) were included. Cases were divided into 4 groups by primary site: orbit, retroperitoneum, soft tissue, and other. Results: A total of 16,958 patients were included. Patients with orbital involvement were younger and more likely to be female (p < 0.05). Among orbital lesions, myxoid liposarcoma was the most common histologic subtype (6/19; 31.6%) followed by well differentiated (5/19; 26.3%). This differed from the distribution of histologic subtypes encountered elsewhere, for which well-differentiated liposarcoma was the most common (retroperitoneum 979/3,136; 31%, soft tissue 3,493/11,671; 30%, and other sites 497/2,132; 23%, p < 0.05). Dedifferentiated histologic subtype was the second most common subtype found in the retroperitoneum (946/3,136; 30%), whereas it was less common in the orbit (2/19; 11%) and soft tissue (1,396/11,671; 12%) (p < 0.001). Patients with orbital liposarcoma had similar disease-specific mortality compared with soft-tissue location (p = 0.825) and lower disease-specific mortality compared with retroperitoneal location (p < 0.001). When all locations were combined, patients with well-differentiated liposarcoma had the lowest disease-specific mortality. Conclusions: Patients with orbital liposarcoma tend to be younger, female, and have a better prognosis than those with retroperitoneal disease, likely due to the lower incidence of dedifferentiated histologic subtype.

Primary Orbital Myxoid Liposarcoma

“…Interestingly, this particular type of tumor tends to manifest in individuals at an earlier age (most cases occurring between the third and sixth decade of life) compared to its counterparts located in the retroperitoneum, soft tissues or other areas of the body. It also shows a tendency to occur more frequently in women [5,9]. Although some cases have been reported suggesting a possible link between orbital liposarcoma and Li-Fraumeni syndrome (a genetic disorder that predisposes individuals to cancer due to germline mutations in the p53 tumor suppressor gene) [5,13], it is important to note that the available data are not yet conclusive to confirm this claim.…”

“…Although liposarcoma is the most common soft tissue sarcoma in adulthood [1], it is exceptionally rare to occur in the orbit [9]. The first case is attributed to Strauss in 1911, and surprisingly, fewer than 100 cases have been documented since then [4,5].…”

“…The first case is attributed to Strauss in 1911, and surprisingly, fewer than 100 cases have been documented since then [4,5]. According to a recent study conducted in the United States, orbital liposarcoma represents only 0.12% of all cases of liposarcoma in that country [9]. Interestingly, this particular type of tumor tends to manifest in individuals at an earlier age (most cases occurring between the third and sixth decade of life) compared to its counterparts located in the retroperitoneum, soft tissues or other areas of the body.…”

“…This condition is categorically divided into four primary subtypes: welldifferentiated liposarcoma (WDLPS, also known as atypical lipomatous tumor), dedifferentiated liposarcoma (DDLPS), myxoid liposarcoma (MLPS) and pleomorphic liposarcoma [1]. Although liposarcoma is most commonly seen in the muscles of the extremities or abdomen, it can also occur in the orbit, although this is rare [2][3][4][5][6][7][8][9]. The first case of orbital liposarcoma was documented by Strauss in 1911 [5,9], and to date (2023), the scientific literature has reported fewer than 100 documented cases of this rare condition.…”

“…Although liposarcoma is most commonly seen in the muscles of the extremities or abdomen, it can also occur in the orbit, although this is rare [2][3][4][5][6][7][8][9]. The first case of orbital liposarcoma was documented by Strauss in 1911 [5,9], and to date (2023), the scientific literature has reported fewer than 100 documented cases of this rare condition. Interestingly, it is believed that the origin of the cells in ocular liposarcoma is not the mature adipocyte, but rather primitive stromal cells associated with intermuscular fascial planes or perivascular mesenchymal pluripotent mesenchymal cells [5,6,10].…”