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Middle East African Journal of Ophthalmology Middle East African Journal of Ophthalmology
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  Table of Contents 
CASE REPORT
Year : 2019  |  Volume : 26  |  Issue : 3  |  Page : 172-174  

Carunclar mass as the presenting sign of primary orbital liposarcoma


1 Nikookari Eye Center, Tabriz, Iran
2 Department of Pathology, Imam Reza Hospital, Tabriz University of Medical Sciences, Tabriz, Iran

Date of Submission15-Mar-2018
Date of Decision27-Feb-2019
Date of Acceptance22-Aug-2019
Date of Web Publication30-Sep-2019

Correspondence Address:
Reza Nabie
Nikookari Eye Center, Tabriz
Iran
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/meajo.MEAJO_69_18

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   Abstract 


This study aims to report a case of primary orbital liposarcoma which presented as caruncle mass. A 45-year-old woman presented to our hospital to evaluate the bumpiness of the caruncle in her left eye. A yellowish nodule , which was firmly attached to the sclera , was seen in the caruncle area and seemed to extend into the orbit. Incisional biopsy revealed myxoid liposarcoma She denied further evaluation and came back with enlarged mass after one year .She underwent lid-sparing exentration and was free of recurrence for 5 years.

Keywords: Exentration, liposarcoma, orbit


How to cite this article:
Nabie R, Halimi M, Bahramani E, Ghadim BK. Carunclar mass as the presenting sign of primary orbital liposarcoma. Middle East Afr J Ophthalmol 2019;26:172-4

How to cite this URL:
Nabie R, Halimi M, Bahramani E, Ghadim BK. Carunclar mass as the presenting sign of primary orbital liposarcoma. Middle East Afr J Ophthalmol [serial online] 2019 [cited 2019 Dec 10];26:172-4. Available from: http://www.meajo.org/text.asp?2019/26/3/172/268252




   Introduction Top


Although liposarcoma is considered the most common soft-tissue sarcomas in adults, primary orbital liposarcoma is rare. To date, fewer than 50 cases of primary orbital liposarcoma have been reported.[1],[2] We report a new case of primary orbital liposarcoma.


   Case Report Top


A 45-year-old woman presented to our hospital to evaluate the bumpiness of the caruncle in her left eye. On slit lamp examination, a yellowish nodule was seen in the carunclar area, which was firmly attached to the sclera and seemed to extend into the orbit. Other slit lamp examinations were unremarkable. No pain was present, and proptosis and ocular motility were normal except for the very mild limitation of adduction. The patient underwent incisional biopsy, and the pathologic report was myxoid liposarcoma. The patient was informed about the diagnosis and treatment modality, but she resisted further evaluation. After 1 year, she was referred because of exacerbation of symptoms. On examination, the visual acuity of both eyes was 10/10. Anterior and posterior segments were normal. A yellowish dense mass was visible in the caruncular area, and there was 6-mm proptosis and 2-mm hypoglobus in the left eye [Figure 1]. Adduction was severely limited. A computed tomography (CT) scan revealed a relatively well-defined and dense mass observed in the medial aspect of the orbit, which extended from the caruncular area toward the apex of orbit [Figure 2]. Since the report of prior biopsy was definite and considering the expenses, no further imaging such as magnetic resonance imaging (MRI) was done. A systemic evaluation was performed. There was no distant metastasis. The patient underwent lid-sparing exenteration. Although temporal flap covering of the exenterated cavity might be preferable, we allowed the cavity to be repaired by granulation tissue. The patient preferred to use dressing instead of prosthesis. Pathologic examination of the specimen confirmed a myxoid-type liposarcoma [Figure 3]a and [Figure 3]b. She was free of disease and recurrence for more than 5 years. Unfortunately, after that the patient failed to follow-up.
Figure 1: Left eye proptosis and lateral displacement

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Figure 2: Axial computed tomography scan

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Figure 3: (a and b) Hematoxylin and eosin staining revealed myxoid background

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   Discussion Top


Liposarcoma is considered the most common soft-tissue malignancy in adults and frequently occurs in the retroperitoneum and deep soft tissues of the extremities such as thighs/upper legs and the inguinal area, but it is rare in orbit. The first description of orbital liposarcoma was reported by Strauss in 1911. This neoplasm affects men slightly more often than women, and the average patient age is 53 years.[1],[2]

According to the World Health Organization classification, there are five histologic subtypes of liposarcoma: well-differentiated, myxoid, round cell, pleomorphic, and dedifferentiated. The most common histological type is the myxoid form (55%).[3],[4] 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.[1],[2],[3],[4],[5] 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.[4],[5],[6] 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. In this case, the most prominent sign was proptosis and globe displacement and the CT scan showed a dense mass without any specific signs.

Liposarcomas usually progress slowly, but in some cases, symptoms may appear abruptly and progressively. Several factors determine prognosis: size and location, histological type, and grade of differentiation; myxoid liposarcomas progress relatively slowly, whereas the round cell and pleomorphic subtypes are aggressive and usually have distant metastasis.[7]

Exenteration is generally regarded as the treatment of choice for liposarcomas. Some surgeons advocate radiotherapy after exenteration.[5] In the present case, the patient underwent an exenteration and has been followed up for 5 years postoperatively and showed no signs of tumor recurrence. We speculated that although the tumor had extensive involvement of orbit, due to the myxoid subtype the prognosis was good.

In conclusion, as in previously reported cases, our case did not have specific imaging, and clinical findings and liposarcoma should be borne in mind as one of the less common, but important causes of caruncular masses.

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.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
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.  Back to cited text no. 1
  [Full text]  
2.
Chebbi A, Hachicha F, Ben Hssine L, Feki O, Bouayed E, Malek I, et al. Primary orbital liposarcoma: A case report. J Fr Ophtalmol 2016;39:e45-7.  Back to cited text no. 2
    
3.
Khurana S, Gupta AK, Sen S, Kashyap S. Primary liposarcoma of the orbit. Indian J Pathol Microbiol 2014;57:617-9.  Back to cited text no. 3
[PUBMED]  [Full text]  
4.
Doyle M, Odashiro AN, Pereira PR, Odashiro M, Kobayashi F, Shinzato I, et al. Primary pleomorphic liposarcoma of the orbit: A case report. Orbit 2012;31:168-70.  Back to cited text no. 4
    
5.
Mridha AR, Sharma MC, Sarkar C, Suri V, Garg A, Suri A, et al. Primary liposarcoma of the orbit: A report of two cases. Can J Ophthalmol 2007;42:481-3.  Back to cited text no. 5
    
6.
Madge SN, Tumuluri K, Strianese D, Bonavolonta P, Wilcsek G, Dodd TJ, et al. Primary orbital liposarcoma. Ophthalmology 2010;117:606-14.  Back to cited text no. 6
    
7.
Cai YC, McMenamin ME, Rose G, Sandy CJ, Cree IA, Fletcher CD, et al. Primary liposarcoma of the orbit: A clinicopathologic study of seven cases. Ann Diagn Pathol 2001;5:255-66.  Back to cited text no. 7
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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