Pediatric primary orbital rhabdomyosarcoma

Thien, Ho Huu; Hoa, Nguyễn Thị Kim; Duy, Phan Canh; Carlos, Rodgiuez-Galindo; Son, Nguyen Huu

doi:10.1016/j.epsc.2020.101475

Cited by 8 publications

(8 citation statements)

References 9 publications

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“…Metastatic orbital RMS carries an unfavorable prognosis compared to localized disease. [1,12] Embryonal and alveolar are the two major histological subtypes which affect the orbit, embryonal being more common (89%). [3] Tumor location within the orbit correlates with histology to a certain extent.…”

Section: Discussionmentioning

confidence: 99%

Unilateral proptosis in a child-need for prompt diagnosis

Kalantri

Balasubramanian

Sneha

et al. 2021

SRJHS

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Proptosis in a child often presents as a diagnostic dilemma. Proptosis can be secondary to infection or childhood malignancies. It warrants urgent and relevant investigations to facilitate correct diagnosis and treatment. The common causes of proptosis include infection and malignant lesions. Any delay in intervention in either of the causes can lead to significant morbidity or can impair the vision of the child. An orbital neoplasm in the pediatric age group is an uncommon clinical finding which can initially manifest as proptosis. Here, we report a case of a 2-year-old girl, presenting with progressive swelling of the right eye. An incisional biopsy confirmed the diagnosis of embryonal rhabdomyosarcoma (RMS). RMS is an aggressive tumor; hence, early diagnosis and prompt treatment are highly essential to prevent significant morbidity and can save the vision of the child.

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Section: Discussionmentioning

confidence: 99%

Unilateral proptosis in a child-need for prompt diagnosis

Kalantri

Balasubramanian

Sneha

et al. 2021

SRJHS

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“…In addition, the prognosis was considered good after continued chemotherapy and radiation, regardless of the amount of tissue excised. [10] Suppose a diagnosis of rhabdomyosarcoma has been established, treatment can be given based on the Intergroup Rhabdomyosarcoma Study (IRS) by postsurgical staging system using a combination of intensive chemotherapy and radiotherapy. [8] Pathological diagnosis assisting the selection of appropriate management.…”

Section: Discussionmentioning

confidence: 99%

Difficulty in Management of Advanced Pediatric Orbital Tumor

Sudrajat

Lutfi

2021

VSEHJ

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Introduction: Tertiary care centers often manage pediatric orbital tumors, especially in advanced lesions and complex management. We report a case of a young boy with a malignant orbital tumor involving intracranial infiltration. Case presentation: A four-year-old boy had proptosis on the left eye for two months accompanied with blurred vision, pain, and oftenly bleeds with ipsilateral nasal congestion. There was no history of trauma, eye disorders, systemic diseases, or growth disorders. The physical examination showed stable vital signs, however a weak general condition and no enlarged regional lymph nodes. Visual acuity of the left eye was no light perception. There was a mass with protrusion of the left eye, swelling of the eyelid with an irregular surface, and a tendency to bleeds. The cornea was hazy with partly scarring, so we could not evaluate the posterior segment. MRI of the head presented a malignant soft tissue mass of the left orbital region with intracranial infiltration. The patient was given adjuvant chemotherapy; however, he could not survive due to systemic complications. Conclusions: The definitive diagnosis for the orbital tumor is obtained by histopathological examination. The investigation with CT scan or MRI imaging could be considered if not possible. However, since the definitive diagnosis still not assessed, the management can be affected. It is essential to have a definitive diagnosis to provide adequate treatment for the patients. Delayed and inadequate management can make malignant orbital tumors potentially life-threatening.

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“…Primary orbital RMS is mainly a disease of young children with 90% occurring before the age of 16 years old with a mean age of onset of 5–7 years. The ratio of male to female is 5 : 34 2 . Orbital RMS is extremely rare in our part of the world with very few cases being clinically evaluated every year, which makes this case exceptional.…”

Section: Introductionmentioning

confidence: 99%

“…Primary orbital RMS involves the orbit, eyelid, conjunctiva, and rarely uveal tract. Typical presentation is rapid onset unilateral proptosis, eyelid edema, and ptosis 2 . A detailed history of pain, visual loss, signs of sinusitis, and any rapidly progressive alteration of lid, conjunctiva or caruncle should be taken to exclude orbital cellulitis, lymphangioma, hemangioma, Langerhans cell histiocytosis, and dermoid cyst.…”

Section: Introductionmentioning

confidence: 99%

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

Shrestha

Mainali

Poudel

et al. 2023

Annals of Medicine &Amp; Surgery

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Introduction and Importance: 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.

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Pediatric primary orbital rhabdomyosarcoma

Cited by 8 publications

References 9 publications

Unilateral proptosis in a child-need for prompt diagnosis

Unilateral proptosis in a child-need for prompt diagnosis

Difficulty in Management of Advanced Pediatric Orbital Tumor

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

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