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

Authors

  • Oduware Gloria Ugboaku (MBBS) Senior Registrar, Department of Ophthalmology, University of Benin Teaching Hospital, Benin City, Nigeria https://orcid.org/0009-0003-3651-1179
  • Edema, Temituoyo Folusho (MD) Medical Officer, JIL Specialist Eye Clinic, Benin-City, Nigeria
  • Ikhile Emmanuel Aimebelomo Consultant Radiation and Clinical Oncologist, Department of Radiation and Clinical Oncology University of Benin Teaching Hospital, Benin City, Nigeria
  • Eghobamien Mine Oghomwenoyemwen (MBBS) Senior Registrar, Department of Radiology, University of Benin Teaching Hospital, Benin City, Nigeria
  • Edema Omolabake Tolutope Professor/ Consultant Ophthalmologist, Department of Ophthalmology, University of Benin Teaching Hospital, Benin City, Nigeria

DOI:

https://doi.org/10.47672/ajhmn.1612

Keywords:

Rhabdomyosarcoma, Radiologic Imaging, External Beam Radiation Therapy, Late Presentation, Chemotherapy Remission

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.

Downloads

Download data is not yet available.

References

Wu, T. H., Huang, J. S., Wang, H. M., Wang, C. H., & Yeh, K. Y.Wu, Tsung & Huang, Jen-Seng & Wang, Hung-Ming & Wang, Cheng-Hsu & Yeh, Kun-Yun. (2010). Long-term survivors of adult rhabdomyosarcoma of maxillary sinus following multimodal therapy: Case reports and literature reviews. Chang Gung Medical Journal, 33(4), 466-471.

Egas-Bejar, D., & Huh, W. W. (2014, June 17). Rhabdomyosarcoma in adolescent and young adult patients: Current perspectives. Adolescent Health, Medicine and Therapeutics, 5, 115-125.

Jurdy, L., Merks, J. H., Pieters, B. R., Mourits, M. P., Kloos, R. J., Strackee, S. D., & Saeed, P. (2013, July). Orbital rhabdomyosarcomas: A review. Saudi Journal of Ophthalmology, 27(3), 167-175.

Mandong, B. M., & Ngbea, J. A. (2011, October-December). Childhood rhabdomyosarcoma: A review of 35 cases and literature. Nigerian Journal of Medicine, 20(4), 466-469.

Brown, B. J., & Oluwasola, A. O. (2006, December). Childhood rhabdomyosarcoma in Ibadan, Nigeria: 1984-2003. Annals of Tropical Paediatrics, 26(4), 349-355.

Raphael, S., Yusuf, I., & Imam, I. (2015, January-March). Childhood rhabdomyosarcoma in Kano, Nigeria: A retrospective analysis of 52 cases. Nigerian Journal of Medicine, 24(1), 32-36.

Linet, M. S., Ries, L. A., Smith, M. A., Tarone, R. E., & Devesa, S. S. (1999, June 16). Cancer surveillance series: Recent trends in childhood cancer incidence and mortality in the United States. Cancer Surveillance Series. Journal of the National Cancer Institute, 91(12), 1051-1058.

Ferrari, A., Dileo, P., Casanova, M., Bertulli, R., Meazza, C., Gandola, L., Navarria, P., Collini, P., Gronchi, A., Olmi, P., Fossati-Bellani, F., & Casali, P. G. (2003, August 1). Rhabdomyosarcoma in adults. A retrospective analysis of 171 patients treated at a single institution. Cancer, 98(3), 571-580.

Malu, K., Ngbea, J., Mohammand, H., Mohammand, H., Joseph A & Mohammad. (2015) Primary orbital rhabdomyosarcoma in an 11-year-old boy: A management challenge in a resource limited environment. Journal of Medicine in the Tropics, 17(1).

Fasina, O. (2013, June). Pattern of presentation and outcome of ophthalmic rhabdomyosarcoma in Ibadan. African Journal of Medicine and Medical Sciences, 42(2), 165-169.

Backhouse, O. C. E., Cove, P., & Bowen, D. I. (1997). Rhabdomyosarcoma masked by orbital trauma. International Journal of Oral and Maxillofacial Surgery, 26(5), 374-375, ISSN 0901-5027.

Andrade, A. A. M. (2017). Orbital rhabdomyosarcoma in an adult, case report. Arch. Journal of Surgery, 3(1), 11-13.

Sanz-Marco, E., España, E., Alamar, A., Prez-Rojas, J., López-Prats, M. J., & Díaz-Llopis, M. (2014, May). Rabdomiosarcoma alveolar orbitario enmascarado por sinusitis etmoidal en un adulto de 25 años [Orbital alveolar rhabdomyosarcoma masked by ethmoid sinusitis in a 25-year-old]. Archivos de la Sociedad Española de Oftalmologia, 89(5), 182-185. ISSN 2173-5794.

Machleder, D. J., Banik, R., Rosenberg, R. B., & Parikh, S. R. (2005, February). An unusual case of rhabdomyosarcoma presenting as orbital apex syndrome. International Journal of Pediatric Otorhinolaryngology, 69(2), 249-254..

Downloads

Published

2023-10-09

How to Cite

Ugboaku (MBBS), O. G. ., Folusho (MD) , E. T. ., Aimebelomo, I. E. ., Oghomwenoyemwen (MBBS), E. M. ., & Tolutope, E. O. . (2023). A Case of Orbital Rhabdomyosarcoma with Intracranial Extension in an Adult: Diagnostic and Management Challenges. American Journal of Health, Medicine and Nursing Practice, 9(3), 1–8. https://doi.org/10.47672/ajhmn.1612

Issue

Vol. 9 No. 3 (2023): Vol 9 No 3 (2023)

Section

Articles

License

Copyright (c) 2023 Oduware Gloria Ugboaku (MBBS), Edema, Temituoyo Folusho (MD) , Ikhile Emmanuel Aimebelomo, Eghobamien Mine Oghomwenoyemwen (MBBS), Edema Omolabake Tolutope

Creative Commons License

This work is licensed under a Creative Commons Attribution 4.0 International License.

Authors retain copyright and grant the journal right of first publication with the work simultaneously licensed under a Creative Commons Attribution (CC-BY) 4.0 License that allows others to share the work with an acknowledgment of the work's authorship and initial publication in this journal.