Rhabdomyoblasts in Pediatric Tumors: A Review with Emphasis on their Diagnostic Utility
Published on: 9th March, 2017
OCLC Number/Unique Identifier: 7317653969
Rhabdomyosarcoma is a soft tissue pediatric sarcoma composed of cells which show morphological, immunohistochemical and ultrastructural evidence of skeletal muscle differentiation. To date four major subtypes have been recognized: embryonal, alveolar, spindle cell/sclerosing and pleomorphic. All these subtypes are defined, at least in part, by the presence of rhabdomyoblasts, i.e. cells with variable shape, densely eosinophilic cytoplasm with occasional cytoplasmic cross-striations and eccentric round nuclei. It must be remembered, however, that several benign and malignant pediatric tumours other than rhabdomyosarcoma may exhibit rhabdomyoblaststic and skeletal muscle differentiation. This review focuses on the most common malignant pediatric neoplasm that may exhibit rhabdomyoblastic differentiation, with an emphasis on the most important clinicopathological and differential diagnostic considerations.
Vaginal embryonal rhabdomyosarcoma in young woman: A case report and literature review
Published on: 25th May, 2020
OCLC Number/Unique Identifier: 8605988997
Rhabdomyosarcomas are the most common soft tissue tumors of childhood. They are characterized by their poor prognosis. Vaginal location is very rare after puberty and exceptional in the post menopause. Treatment is based on several therapeutic measures combining neoadjuvant chemotherapy followed by surgery and/or external beam radiation therapy. We report herein the case of a 25 years-old woman, presented with vaginal embryonal RMS revealed by metrorrhagia and pelvic pain. The diagnosis was confirmed by biopsy and histopathological study. Pre-treatment workup was negative for metastatic disease. She has received chemotherapy based on vincristine, doxorubicin, and cyclophosphamide. The clinical evolution was marked by improvement of symptoms, unfortunately the patient died following febrile neutropenia after the third cycle of chemotherapy.
Congenital alveolar rhabdomyosarcoma - case report
Published on: 27th September, 2022
Rhabdomyosarcoma is the most common soft tissue sarcoma of childhood and is very rare in the neonatal period. At this age, the alveolar type is a remarkably uncommon variety.We report a 56 days old female with alveolar RMS of the right eye noted since the age of 7 days with fast progression and unfavorable prognosis. Congenital alveolar RMS is an important cause of neonatal onset rapidly progressive proptosis. Early onset, alveolar type, and late diagnosis were poor prognostic factor.
Sinonasal Myxoma Extending into the Orbit in a 4-Year Old: A Case Presentation
Published on: 30th July, 2024
Background: Sinonasal myxomas are exceptionally rare benign tumors in pediatric patients. This report presents the case of a 4-year-old boy diagnosed with a sinonasal myxoma extending into the right orbit.Case presentation: The patient’s clinical presentation included moderate-angle esotropia and ocular torticollis. Advanced imaging revealed an expansile lesion in the right posterior ethmoid cavity with orbital involvement. The differential diagnosis considered included malignancies such as rhabdomyosarcoma and lymphoma, as well as benign neoplasms and inflammatory changes. A biopsy confirmed the diagnosis of sinonasal myxoma. The patient underwent a wide local resection performed by a multidisciplinary team, leading to a confirmed histopathological diagnosis of sinonasal myxoma.Conclusion: This case highlights the diagnostic challenges and the importance of thorough clinical and radiologic evaluation in pediatric patients with unusual ocular symptoms. The report underscores the need for a multidisciplinary approach in managing rare neoplasms such as sinonasal myxomas.
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