Cargando…

A rare case of pediatric orbital rhabdomyosarcoma in Nepal: a case report

Orbital rhabdomyosarcoma (RMS) is a highly malignant, mesenchymal orbital tumor of childhood with a predilection in children less than 20 years of age. It presents as a space-occupying lesion in the orbit, most commonly over the superior nasal quadrant of the orbit. The patient usually presents with...

Descripción completa

Detalles Bibliográficos
Autores principales: Shrestha, Tina, Mainali, Sumina, Poudel, Sandhya, K.C, Narendra, Dulal, Aliza
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Lippincott Williams & Wilkins 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10129160/
https://www.ncbi.nlm.nih.gov/pubmed/37113866
http://dx.doi.org/10.1097/MS9.0000000000000501
_version_ 1785030670327742464
author Shrestha, Tina
Mainali, Sumina
Poudel, Sandhya
K.C, Narendra
Dulal, Aliza
author_facet Shrestha, Tina
Mainali, Sumina
Poudel, Sandhya
K.C, Narendra
Dulal, Aliza
author_sort Shrestha, Tina
collection PubMed
description Orbital rhabdomyosarcoma (RMS) is a highly malignant, mesenchymal orbital tumor of childhood with a predilection in children less than 20 years of age. It presents as a space-occupying lesion in the orbit, most commonly over the superior nasal quadrant of the orbit. The patient usually presents with rapid onset unilateral proptosis and eyelid edema. CASE PPRESENTATION: In this article, a 14-year-old male presented with rapidly progressive swelling of the right orbit. On ocular examination, there was nonaxial inferolateral proptosis of the right eye. Computed tomography revealed a large soft tissue density tissue lesion in the right nasal cavity and meati measuring at least 3.2×2.7×5.4 cm in size with the erosion of the right orbit along with extension of the lesion in the extraconal compartment of the orbit. An MRI of the brain with contrast showed a heterogeneously enhancing altered signal intensity lesion. Debulking was planned, and a biopsy of the mass was sent that gave an impression of alveolar RMS. He also received radiotherapy and chemotherapy at one of the cancer hospitals in Nepal. Postsurgical follow-up showed gradual improvement in the visual acuity of the right eye. No evidence of metastasis and recurrence was found upon subsequent follow-up. CONCLUSION: Thus, early diagnosis and prompt treatment is most for a favorable prognosis in the case of RMS. The main aim of this article was to briefly overview a rare case of RMS, its clinical presentation, diagnosis, treatment modalities, and its prognosis.
format Online
Article
Text
id pubmed-10129160
institution National Center for Biotechnology Information
language English
publishDate 2023
publisher Lippincott Williams & Wilkins
record_format MEDLINE/PubMed
spelling pubmed-101291602023-04-26 A rare case of pediatric orbital rhabdomyosarcoma in Nepal: a case report Shrestha, Tina Mainali, Sumina Poudel, Sandhya K.C, Narendra Dulal, Aliza Ann Med Surg (Lond) Case Reports Orbital rhabdomyosarcoma (RMS) is a highly malignant, mesenchymal orbital tumor of childhood with a predilection in children less than 20 years of age. It presents as a space-occupying lesion in the orbit, most commonly over the superior nasal quadrant of the orbit. The patient usually presents with rapid onset unilateral proptosis and eyelid edema. CASE PPRESENTATION: In this article, a 14-year-old male presented with rapidly progressive swelling of the right orbit. On ocular examination, there was nonaxial inferolateral proptosis of the right eye. Computed tomography revealed a large soft tissue density tissue lesion in the right nasal cavity and meati measuring at least 3.2×2.7×5.4 cm in size with the erosion of the right orbit along with extension of the lesion in the extraconal compartment of the orbit. An MRI of the brain with contrast showed a heterogeneously enhancing altered signal intensity lesion. Debulking was planned, and a biopsy of the mass was sent that gave an impression of alveolar RMS. He also received radiotherapy and chemotherapy at one of the cancer hospitals in Nepal. Postsurgical follow-up showed gradual improvement in the visual acuity of the right eye. No evidence of metastasis and recurrence was found upon subsequent follow-up. CONCLUSION: Thus, early diagnosis and prompt treatment is most for a favorable prognosis in the case of RMS. The main aim of this article was to briefly overview a rare case of RMS, its clinical presentation, diagnosis, treatment modalities, and its prognosis. Lippincott Williams & Wilkins 2023-04-10 /pmc/articles/PMC10129160/ /pubmed/37113866 http://dx.doi.org/10.1097/MS9.0000000000000501 Text en Copyright © 2023 The Author(s). Published by Wolters Kluwer Health, Inc. https://creativecommons.org/licenses/by-nc-nd/4.0/This is an open access article distributed under the terms of the Creative Commons Attribution-Non Commercial-No Derivatives License 4.0 (https://creativecommons.org/licenses/by-nc-nd/4.0/) (CCBY-NC-ND), where it is permissible to download and share the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal. http://creativecommons.org/licenses/by-nc-nd/4.0/ (https://creativecommons.org/licenses/by-nc-nd/4.0/)
spellingShingle Case Reports
Shrestha, Tina
Mainali, Sumina
Poudel, Sandhya
K.C, Narendra
Dulal, Aliza
A rare case of pediatric orbital rhabdomyosarcoma in Nepal: a case report
title A rare case of pediatric orbital rhabdomyosarcoma in Nepal: a case report
title_full A rare case of pediatric orbital rhabdomyosarcoma in Nepal: a case report
title_fullStr A rare case of pediatric orbital rhabdomyosarcoma in Nepal: a case report
title_full_unstemmed A rare case of pediatric orbital rhabdomyosarcoma in Nepal: a case report
title_short A rare case of pediatric orbital rhabdomyosarcoma in Nepal: a case report
title_sort rare case of pediatric orbital rhabdomyosarcoma in nepal: a case report
topic Case Reports
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10129160/
https://www.ncbi.nlm.nih.gov/pubmed/37113866
http://dx.doi.org/10.1097/MS9.0000000000000501
work_keys_str_mv AT shresthatina ararecaseofpediatricorbitalrhabdomyosarcomainnepalacasereport
AT mainalisumina ararecaseofpediatricorbitalrhabdomyosarcomainnepalacasereport
AT poudelsandhya ararecaseofpediatricorbitalrhabdomyosarcomainnepalacasereport
AT kcnarendra ararecaseofpediatricorbitalrhabdomyosarcomainnepalacasereport
AT dulalaliza ararecaseofpediatricorbitalrhabdomyosarcomainnepalacasereport
AT shresthatina rarecaseofpediatricorbitalrhabdomyosarcomainnepalacasereport
AT mainalisumina rarecaseofpediatricorbitalrhabdomyosarcomainnepalacasereport
AT poudelsandhya rarecaseofpediatricorbitalrhabdomyosarcomainnepalacasereport
AT kcnarendra rarecaseofpediatricorbitalrhabdomyosarcomainnepalacasereport
AT dulalaliza rarecaseofpediatricorbitalrhabdomyosarcomainnepalacasereport