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 [1]. Four histological varities of rhabdomyosarcoma are: - embryonic, alveolar, botryoidal and pleomorphic. Of these, 60% are  embryonal  type,  which  have  predilection  for  young children [2]. 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% [3].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 [2].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 [2].

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.

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