Carunclar Mass as the Presenting Sign of Primary Orbital Liposarcoma

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.

Introduction

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

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 3a and 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

Figure 2.

Axial computed tomography scan

Figure 3.

(a and b) Hematoxylin and eosin staining revealed myxoid background

Discussion

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.