|Year : 2023 | Volume
| Issue : 2 | Page : 526-528
Saga of primary myxoid liposarcoma of the orbit
Chatura K Ramakantha1, Aditi Bhattacharya1, Shivayogi Kusagur2
1 Department of Pathology, Jagadguru Jayadeva Murugarajendra Medical College, Davangere, Karnataka, India
2 Department of Opthalmology, Jagadguru Jayadeva Murugarajendra Medical College, Davangere, Karnataka, India
|Date of Submission||07-Oct-2022|
|Date of Acceptance||06-Jan-2023|
|Date of Web Publication||28-Apr-2023|
Chatura K Ramakantha
Professor, Department of Pathology, 2nd Floor, New Block, Jagadguru Jayadeva Murugarajendra Medical College, Davangere - 577 004, Karnataka
Source of Support: None, Conflict of Interest: None
A 24-year-old female presented with proptosis and limitation of eye movements and was investigated radiologically with a tentative diagnosis of lymphangioma. Initial biopsy was considered to be in favor of optic glioma on histopathology, but immunohistochemistry was negative for Glial fibrillary acidic protein (GFAP). A year later, the patient reported back with worsening proptosis. She underwent exenteration of left eye for a posterior mass encasing the optic nerve. The final diagnosis of myxoid liposarcoma was rendered on excision biopsy. The difficulties experienced in establishing diagnosis are shared in this case report.
Keywords: Myxoid liposarcoma, orbit, proptosis
|How to cite this article:|
Ramakantha CK, Bhattacharya A, Kusagur S. Saga of primary myxoid liposarcoma of the orbit. Indian J Ophthalmol Case Rep 2023;3:526-8
Liposarcoma is a malignant tumor of the adipose tissue. It is the most common soft tissue sarcoma in adults. The orbit is a rare site for primary liposarcoma, which usually originates in deep connective tissue planes and is most common in the thigh and retroperitoneum.
Myxoid liposarcoma is the most common subtype; it occurs predominantly in the extremities of adults and has a tendency to either recur locally or metastasize to unusual soft tissue locations. It is an intermediate- to high-grade tumor.
The first description of liposarcoma in the orbit was given by Strauss in 1911, but since then, there have been only sparse case reports and only two major case series. We have documented the clinical, radiological findings, and histopathologic features of the case, with a discussion on the difficulties which may be experienced in establishing diagnosis.
| Case Report|| |
A 24-year-old female with proptosis and limitation of left eye movements since 6 months presented to the outpatient clinic with sudden increase in pain and size in 2019 [Figure 1]a. High-resolution computerized tomography (HRCT) of the orbital plane showed a well-defined cystic lesion seen encasing the optic nerve as well as displacing the optic nerve and ocular globe within the left intraconal space, stretching the right medial rectus muscle as well as causing proptosis of the left ocular globe [Figure 1]b. On the post-contrast magnetic resonance imaging (MRI) of orbit, there was peripheral enhancement with a few septations and predominantly nonenhancing, thick fluid-like content within the lesion [Figure 1]c. A tentative diagnosis of lymphangioma was suggested.
|Figure 1: (a) Proptosis left eye at presentation. (b) CT image: Well-defined cystic lesion encasing the optic nerve (arrow). (c) MRI post-contrast imaging: Nonenhancing, thick fluid-like content within the cystic lesion (arrow). (d) Severe proptosis after 1 year. (e) Gross of myxoid tumor around the optic nerve. (f) Spindle cells in a myxoid background with “chicken-wire” vessels and multivacuolated lipoblasts showing indented nuclei (H and E, ×100). CT = computed tomography, H and E = hematoxylin and eosin, MRI = magnetic resonance imaging|
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The left orbital mass excision biopsy on initial histopathologic examination showed spindle cells with mild pleomorphism and numerous capillaries in a background of edematous stroma, suggestive of optic nerve glioma. On immunohistochemistry (IHC), it was positive for S-100 and negative for GFAP. Patient was lost to follow-up with no treatment received. A year later in 2020, patient presented once again to the outpatient clinic, with worsening proptosis [Figure 1]d for which exenteration was performed of the left eye posterior mass encasing the optic nerve [Figure 1]e. The left globe with upper and lower eyelids on the cut section showed a gelatinous, gray-yellow mass in the posterior aspect of the globe, surrounding the optic nerve and extending to the resected margin. Intraoperative squash smears were prepared, and the histopathologic examination revealed cellular tumor composed of spindle to oval cells with mild pleomorphism in a myxoid background with numerous “chicken-wire” vessels. Many multivacuolated lipoblasts with indented nuclei were seen [Figure 1]f.
A diagnosis of orbital myxoid liposarcoma was made considering proptosis, pain and increase in size, computed tomography (CT) and MRI suggestive of intraconal lesion encasing the optic nerve, IHC on biopsy specimen showing GFAP-negative and S100-positive staining and subsequent intraoperative squash smears, and gross and histopathologic sections giving the definitive diagnosis. In our case, after surgery, patient was referred to a higher center for further management and hence lost to follow-up.
| Discussion|| |
Liposarcoma accounts for at least 20% of all sarcomas in adulthood. Liposarcoma in the orbit is said to arise from pluripotent mesenchymal cells. There are three principal forms: well differentiated, myxoid, and pleomorphic.
Liposarcoma produces a clinical picture that resembles other orbital tumors, including signs such as proptosis, eye displacement with or without diplopia in addition to optic nerve compression with pain, and impairment of vision if the tumor is located at the orbital apex. There are no characteristic clinical findings or diagnostic tests that are specific for liposarcoma.
CT findings can vary from a homogenous lesion of soft tissue density to a heterogeneous mass containing fat. There is only one imaging feature common to all scans, that is, the intimate association of the tumor with extraocular muscle.
It is difficult to establish diagnosis of myxoid liposarcoma on MRI due to lack of fat signal intensity. A spectrum of imaging abnormalities occurs, and most appear to be cystic in nonenhanced MRI, which represents the accumulated myxoid material. In our case, other benign cystic tumors were, therefore, considered in diagnosis.
On gross examination, the tumor was grayish white, multilobulated, and soft in consistency. Histologically, the most common type is myxoid followed by well-differentiated type. The common link is the lipoblast between various types of liposarcomas. Lipoblasts have sharply outlined cytoplasmic vacuoles that indent or scallop atypical hyperchromatic nuclei. The presence of lipoblast is essential for the pathologic diagnosis. All subtypes tend to have some lobular configuration and are either poorly circumscribed or obviously invasive of extraocular muscle. Sections of myxoid liposarcoma also show chicken-wire vasculature and myxoid background. The above features were seen in the exenteration specimen, and hence, the diagnosis was established in our case.
Literature review identified 34 other cases of primary orbital liposarcoma, apart from six cases in a study, which, partly because of its rarity is frequently initially misdiagnosed. The most common subtype is myxoid (56.8%); other types are pleomorphic (10.8%) and well differentiated (29.7%). Well-differentiated tumors have the best prognosis. Myxoid liposarcoma subtype in the orbit is rare with very few case reports like two out of four cases, two out of six cases, and three out of five cases.
The overall survival in primary liposarcoma is not known due to its rare occurrence and variable morphologic and histopathologic subtypes. The consensus on treatment elsewhere in the body is wide local excision. Local resections of orbital liposarcomas have been associated with recurrences.
Non-exenterating surgery is associated with recurrence, although recurrence also occurs post-exenteration. Although radiotherapy has an established role in the treatment of nonorbital liposarcoma, the role of both radiotherapy and chemotherapy in the management of primary orbital liposarcoma is still unclear.
| Conclusion|| |
Histopathologic diagnosis of liposarcoma frequently proves difficult, as the appearance may be confused with that of pseudotumor, lipoma, lymphangioma, or myxoma. Because of the rarity of orbital liposarcoma and the consequent lack of experience of clinician and pathologist alike, a high index of suspicion is required.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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