Adult Rhabdomyosarcoma: A Rare Ophthalmic Case Report
Journal of Clinical and Experimental Ophthalmology is a journal which provides rapid peer-review process with 6 issues per year (Bi Monthly). Journal of Clinical and Experimental Ophthalmology accepts all types of articles including case-reports, research, review, case-series, mini-review, opinion articles, short communications, commentary, perspective and video articles.
Journal of Clinical and Experimental Ophthalmology has recently release its 3rd issue in 2020 with 6 articles and 3 Research articles, 2 case reports and a short communication. Here we are mentioning about the case report entitled “Adult Rhabdomyosarcoma: A Rare Case Report and the Associated Challenges” written and submitted by Shipra Sharada whose abstract was as follows:
Rhabdomyosarcoma is a malignant tumour of mesenchymal origin with an aggressive pattern of growth. Its occurrence is quite rare in children and adolescents, and even rarer in adults. We discuss the case of a young adult female diagnosed with embryonal rhabdomyosarcoma.
More information includes: Rhabdomyosarcoma is a rare soft tissue malignant neoplasm which arises from the undifferentiated mesenchyme and histologically resembles the normal fetal skeletal muscle before innervation . Four histological varities of rhabdomyosarcoma are: - embryonic, alveolar, botryoidal and pleomorphic. Of these, 60% are embryonal type, which have predilection for young children . Rhabdomyosarcoma is a rarely occurring neoplasm of the childhood and therefore its occurrence in an adult makes it further rare tumour accounting for 3% of soft tissue sarcomas, which themselves have a prevalence of less than 1% .According to the literature reports, Rhabdomyosarcoma has anestimated incidence of 4.3 million cases per million occurring inthe head and neck region and of these 10% occur in the orbit[4,5].The prevalence is more in males as compared to females .The main Head and Neck sites include-orbit, parameningeal sites (nasopharynx, nasal fossa, para- nasal sinuses, infra-temporal fossa, pterygoid fossa, middle ear and mastoid) and non-parameningeal sites (scalp, face, parotid, oral cavity, oropharynx, larynx and neck). Tumours which are arising from the parameningeal sites tend to invade the cranial cavity via basalskull foramina and are therefore having the worst prognosis .
A 26 years old female presented to our ophthalmic OPD with sudden onset, paraxial proptosis of the right eye (RE) with lid swelling since 5 days associated with ipsilateral periorbital pain of 15 days duration. Patient denied history of ocular trauma, surgery or any other systemic illness. On further probing, patient mentioned about the symptoms of intermittent episodes of nasal blockage along with occasional nasal bleed for the past 4-5months aggravated since last 2 weeks for which no previous consultation was taken. Ocular examination of the affected eye(RE) revealed best corrected visual acuity of finger counting at 4metres with total ophthalmoplegia. Anterior segment findings revealed conjunctival congestion with chemosis along with sluggish pupillary reaction. Fundus examination revealed disco edema with tortuous vessels. In the Left eye (LE), the unaided visual acuity was 6/6. The anterior segment findings and fundus examination were within normal limits (Figure 1).
MRI revealed a multi-lobulated sino-nasal mass which was iso-intense on T1and hyperintense on T2 weighted images. It wasinvading the surrounding structures like the frontal sinus,ethmoidal sinus and the olfactory recess. Intracranial extension was seen through base of the skull (Figures 2 and 3).Soft tissue punch biopsy specimen taken from the right nostril showed round hyperchromatic cells with oedematous stroma which favoured the diagnosis of rhabdomyosarcoma-embryonal type. Diagnosis was confirmed with immunohistochemistry. Patient was referred to the oncology department of atertiary care center for further management. Unfortunately, the patient could not be saved.
Journal of Clinical and Experimental Ophthalmology
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