Aggressive orbital rhabdomyosarcoma in adulthood: A case report in a public hospital in Damascus, Syria

Ahmad, Tagrid Younes; Houri, Hasan Nabil Al; Houri, Abdullah Nabil Al; Ahmad, Nagham Younes

doi:10.4103/ajm.ajm_62_18

Cited by 6 publications

(4 citation statements)

References 9 publications

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“…RMS has also been reported as lid masses and as masses in other areas of orbit 8 . Most of the data derived from retrospective case series state that the typical first presentation is unilateral proptosis, while other cases describe atypical presentation such as an eyelid nodule 4 . Although superonasal orbit is the most common location, in our case the tumor was located in the medial and temporal wall of the right orbit with nonaxial, inferonasal proptosis.…”

Section: Clinical Discussionmentioning

confidence: 99%

“…A biopsy is important in establishing the diagnosis and determining the prognosis. Recurrence or metastatic spread usually occurs within the first 3 years after treatment, and the most common site of metastasis is the lung 4 . Treatment options include debulking in addition to chemotherapy, additional external beam radiation therapy, brachytherapy, and if all fails exenteration is the remaining option.…”

Section: Introductionmentioning

confidence: 99%

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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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Section: Clinical Discussionmentioning

confidence: 99%

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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“…The symptoms of focal damage of the nervous system appear in the case of RMS of the perimeningeal area. Within the eye socket, it may cause exophthalmos, perturbations of eyeball mobility, or vision perturbations [117][118][119]. Localized within the head and neck it may give symptoms of chronic or acute sinusitis, purulent or bloody discharge from the nasal cavity or ear canal, their obturation, or swallowing difficulti es [55,120].…”

Section: Clinical Picturementioning

confidence: 99%

Diagnosis and treatment of rhabdomyosarcomas

et al. 2023

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Rhabdomyosarcoma (RMS) is a soft tissue sarcoma. The primary tumor is most commonly localized in the head and neck, the urogenital system, or the limbs. Classification by the World Health Organization has distinguished four histopathological RMS subtypes: embryonal, alveolar, pleomorphic, and spindle cell/sclerosing. Differential diagnosis of RMS includes melanoma, malignant neoplasm of peripheral nerve sheaths, liposarcoma, and PE-Coma. Among typical cytogenetic changes in RMS are chromosomal translocations t(2;13)(q35;q14) and t(1;13) (p36;q14). They lead to the formation of fusion genes that have a prognostic value. In the course of RMS, changes may also be present in signaling pathways, including RAS-PI3K, Wnt/b-catenin, receptor tyrosine kinase pathways, and myogenesis regulation. In 30% of patients at the time of diagnosis of RMS, distant metastases are present, most commonly to lungs, lymph nodes, bones, and bone marrow. Treatment of patients with RMS requires a multidisciplinary approach, and steadily perfected diagnostic techniques contribute to the individualization of therapeutic strategies. Optimal treatment of localized RMS is based on surgery combined with radiotherapy and chemotherapy. If distant metastases are present, the basic therapeutic method is multidrug chemotherapy, most frequently based on vincristine, dactinomycin, ifosfamide/cyclophosphamide, and etoposide. Despite intensive treatment, the 5-year survival index for RMS is not greater than 50%. There are still no unequivocal guidelines concerning the treatment in patients with local or distant recurrences.

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“…I read with interest the case report by Ahmad et al . [ 1 ] on the orbital rhabdomyosarcoma (RMS) in a 36-year-old Syrian patient. The authors interestingly described the clinical presentation, imaging and histopathologic findings, and treatment protocol in the studied patient.…”

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confidence: 99%