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| Year : 2014 | Volume
: 57
| Issue : 4 | Page : 617-619 |
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| Primary liposarcoma of the orbit
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Saurbhi Khurana1, Anoop K Gupta1, Seema Sen2, Seema Kashyap2
1 Departments of Oculoplasty and Paediatric Ophthalmology Services, Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, India
2 Department of Ocular Pathology, Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, India
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| Date of Web Publication | 11-Oct-2014 |
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 Abstract | | |
Liposarcoma is the most common sarcoma in adults, but is an extremely rare tumor of the orbit. We report 4 cases of primary orbital liposarcoma treated at our center. All cases had tumor localized to the orbit and underwent exenteration followed by radiotherapy in one patient. None of the patients had a recurrence until a follow-up of 1-5 years (mean: 4 years).
Keywords: Orbital liposarcoma, primary sarcoma of orbit, rare orbital tumors
How to cite this article:
Khurana S, Gupta AK, Sen S, Kashyap S. Primary liposarcoma of the orbit
. Indian J Pathol Microbiol 2014;57:617-9 |
| Introduction | |  |
Liposarcoma is the most common soft tissue sarcoma in adults, retroperitoneum being the most common site of involvement. [1] Primary orbital liposarcoma is extremely rare with around 40 cases reported in English literature. [2] We report 4 cases of primary orbital liposarcoma.
An informed consent was obtained from all patients and the study adhered to the principles outlined in Declaration of Helsinki.
| Case reports | | |
Case 1
A 50-year-old female patient presented with painless proptosis and limitation of movements in the right eye since 2 months. There was loss of perception of light in the involved eye. Left eye examination was within the normal limits. Contrast enhanced computed tomography (CT) showed an ill-defined, heterogeneous intraconal mass in the right orbit. Investigations, including blood biochemistry, chest radiography and ultrasonography of the whole abdomen revealed no abnormality. An incisional biopsy was done and histopathology showed a myxoid liposarcoma with delicate vascular channels, characteristic lipoblasts, very few mitoses, and some giant and pleomorphic cells. Immunohistochemical staining was positive for S-100. After taking an informed consent, exenteration of the right orbit was done [Figure 1]. There has been no evidence of recurrence for 2 years since surgery. | Figure 1: Gross exenterated specimen showing cut section of a myxoid liposarcoma
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Case 2
A 40-year-old male presented with recurrent painful proptosis of the left eye since 4 months. His best corrected visual acuity (BCVA) was 20/20 in right eye and 20/200 in left eye. Examination showed a swelling in the upper quadrant of the left orbit, with downward and medial displacement of the eyeball. On Hertel's exophthalmometer the amount of proptosis was 9 mm. Right eye examination was normal. The patient had undergone anterior orbitotomy 1 year back for a well-defined orbital mass that had been diagnosed as myxoid liposarcoma on histopathology. A repeat incisional biopsy showed characteristic lipoblasts in a delicate capillary network, confirming the diagnosis of a myxoid liposarcoma [Figure 2]a.
Ultrasonography of the orbit showed an ill-defined mass with high internal reflectivity indenting the globe. CT scan revealed an ill-defined mass in the left superolateral orbit with moderate enhancement. Ultrasonography of the abdomen and whole body bone scan were normal. The patient underwent an exenteration and was on a follow-up for 4 years with no recurrence or metastasis.
Case 3
A 65-year-old female presented with painless proptosis and limitation of movements in left eye since 1 year. Her BCVA was 20/20 in right eye and no perception of light in left eye. On Hertel's exophthalmometer there was 7 mm of proptosis of the left eye. Other eye examination was normal. CT scan showed a large, well-defined intraconal mass with areas of low density in the left orbit. An incisional biopsy was taken and on microscopic examination, predominantly mature fat cells intersected by fibrous bands and spindle cells with mild to moderate pleomorphism along with few multinucleate giant cells and lipoblast like cells were seen [Figure 2]b. This was consistent with the diagnosis of a well-differentiated liposarcoma and the patient was taken up for an orbital exenteration. Systemic workup was found to be normal. There was no recurrence until a follow-up of 5 years.
Case 4
A 60-year-old male presented with painless axial proptosis of right eye since 1 year with a significant increase in the last 5 months. His BCVA was perception of light in right eye and 20/20 in left eye. There was exposure keratopathy with limitation of movements at presentation. On Hertel's exophthalmometer, the amount of proptosis was 10 mm. CT scan revealed a large heterogenous mass with areas of negative attenuation compatible with fat seen in the intraconal space of right orbit. Biopsy was done and histopathology showed predominantly mature lipocytes with a variable number of spindle cells having hyperchromatic nuclei and multi-vacuolated lipoblasts compatible with the diagnosis of a well-differentiated liposarcoma. Metastatic workup did not reveal any other site of involvement. The orbit was exenterated and no recurrence was found until a follow up of 5 years.
| Discussion | | |
Liposarcoma is the most common soft tissue sarcoma in adults most commonly arising from the thigh and retroperitoneum. [1],[2],[3],[4],[5] In spite of large amount of fat present in the orbit; it is a rare site for a primary liposarcoma. Metastatic liposarcoma of the orbit is even rarer. [6] Liposarcoma in the orbit is believed to arise from pluripotent mesenchymal cells. The median age of presentation is 31.5 years (17-71 years). [7]
Clinical presentation is of a malignant orbital mass as was found in all our patients as well as previously reported cases. CT findings can vary from a homogenous lesion of soft tissue density to a heterogeneous mass containing fat. [4],[5],[8] In two of our cases the mass was well-defined with areas of fat density. However, the diagnosis in all 4 cases was established on histopathology after an incisional biopsy.
On gross examination the tumor is greyish white, multilobulated and soft in consistency. Histologically, liposarcomas are subtyped into well-differentiated, myxoid, round cell, dedifferentiated, and pleomorphic variants. [1] The most common type is myxoid followed by well-differentiated. [8] The link between the various types of liposarcomas is the lipoblast. As observed in cases 1 and 2, the myxoid liposarcoma showed a myxoid matrix with lipoblasts (a characteristic "signet ring" type,) and a delicate capillary network. The other two cases were well-differentiated liposarcomas, which showed predominantly mature lipocytes with a variable number of spindle cells having hyperchromatic nuclei and multi-vacuolated lipoblasts.
The overall survival in primary orbital liposarcoma is not known due to its rare occurrence and variable morphological and histopathological subtypes. The consensus on treatment elsewhere in the body is wide local excision. [8] Local resections of orbital liposarcomas have been associated with recurrences as also seen in one of our cases. [3] Exenteration was done in all our cases with no evidence of distant metastasis or local recurrence on follow-up. There is a variable response to radiotherapy depending on the histopathological subtype. It has been recommended for recurrent tumors. [9]
| Conclusion | | |
Liposarcoma of the orbit is rare and the diagnosis is essentially on the basis of histopathology. Treatment is wide excision, which may necessitate exenteration in most cases. Long-term prognosis is not well-known.
| References | | |
| 1. | Enzinger FM, Weiss SW. Soft Tissue Tumors. 2 nd ed. St. Louis, Mo: CV Mosby; 1988. p. 346-82.  |
| 2. | Al-Qahtani AA, Al-Hussain H, Chaudhry I, El-Khamary S, Alkatan HM. Primary orbital liposarcoma: Histopathologic report of two cases. Middle East Afr J Ophthalmol 2011;18:314-6. [ PUBMED]  |
| 3. | Wagle AM, Biswas J, Subramaniam N, Mahesh L. Primary liposarcoma of the orbit: A clinicopathological study. Orbit 1999;18:33-36. |
| 4. | Lane CM, Wright JE, Garner A. Primary myxoid liposarcoma of the orbit. Br J Ophthalmol 1988;72:912-7. |
| 5. | Naeser P, Moström U. Liposarcoma of the orbit: A clinicopathological case report. Br J Ophthalmol 1982;66:190-3. |
| 6. | Fezza J, Sinard J. Metastatic liposarcoma to the orbit. Am J Ophthalmol 1997;123:271-2. |
| 7. | Garity JA, Henderson JW, Cameron JD. Henderson's; Tumors of Primitive Mesoderm, Striated muscle, Adipose Tissue and Smooth muscle. In: Garity JA, Henderson JW, Cameron JD, editors. Henderson's Orbital Tumors. 4 th ed. Philadelphia: Lippincott Williams and Wilkins; 2007. p. 123-41. |
| 8. | McNab AA, Moseley I. Primary orbital liposarcoma: Clinical and computed tomographic features. Br J Ophthalmol 1990;74:437-9. |
| 9. | Mridha AR, Sharma MC, Sarkar C, Suri V, Garg A, Suri A. Primary liposarcoma of the orbit: A report of two cases. Can J Ophthalmol 2007;42:481-3. |
Correspondence Address:
Seema Kashyap
Department of Department of Ocular Pathology, Room No. 732, 7th Floor, Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, Ansari Nagar, New Delhi - 110 029
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/0377-4929.142700
[Figure 1], [Figure 2] |
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