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.
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.
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.
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