A Case of Orbital Rhabdomyosarcoma with Intracranial Extension in an Adult: Diagnostic and Management Challenges

Abstract

Purpose: To report a rare case of orbital rhabdomyosarcoma with intracranial extension in an adult and the challenges in managing this case.
 Methodology: Research design was through a case report, while data was collected through observation, analysis of medical records and individual interviews. Interpretation of data was through the qualitative method of data analysis.
 Findings: A 34-year-old female meat seller was referred to our center with a rapidly progressive protrusion of the right eye of one month duration. She had a previous history of a penetrating injury to the right globe with bone fragment retention. She had had evisceration. Two weeks post-surgery, she noticed pain and a progressively worsening swelling around the right orbit. In our center, an initial diagnosis of orbital cellulitis post-evisceration was entertained. Orbito-cranial CT scan and MRI done showed a large homogenous irregular soft tissue mass occupying the right orbital cavity with extension to the right lateral rectus, lacrimal gland, cavernous sinus, optic nerve and brain parenchyma; findings were suggestive of orbital rhabdomyosarcoma with intracranial extension. The patient was discharged against medical advice. She re-presented 7 weeks later with a much larger mass which had multiple friable bleeding sites. With much assurance by the oncologists, she had several blood transfusions and 6 courses of chemotherapy. Tumour regression was very impressive. Incisional biopsy and histology confirmed rhabdomyosarcoma with chemotherapeutic changes. The patient was stable and so referred to another tertiary center for External Beam Radiation Therapy (EBRT), but this was delayed due to financial constraints. Two months from her referral for EBRT, colleagues reported she died of intractable seizures
 Recommendations: This study is based on suggestions that therapeutic success for rhabdomyosarcoma is better in children with survival rates reaching 75% unlike in adults where prognosis is poor.1 In this report, remission was observed soon after commencement of treatment. Therefore, it is recommended that early diagnosis be done to improve outcomes. Secondly, combination drugs should be used in all rhabdomyosarcoma cases. Lastly, government policies geared towards universal health coverage should be made and implemented.