Abstract
Embryonal rhabdomyosarcoma (eRMS) is one of the most common soft tissue sarcomas in children, accounting for 4.5% of all childhood tumors. Half of the eRMS occuring in the head and neck are parameningeal. About 40% of patients with eRMS can develop distant metastases. In patients with intracranial tumors, metastatic spread can occur along the central nervous system (CNS) meninges. The literature describes only 4 clinical cases of eRMS with distant metastases in the spinal cord and along the meninges. Only in two out of these four cases, CSF cytology was positive (meaning that tumor cells were detected in cerebrospinal fluid). Magnetic resonance imaging (MRI) of the central nervous system with contrast enhancement can be used to detect distant metastases in the CNS and meninges. We present a clinical case of a 4-year old girl with parameningeal eRMS. MRI of the CNS performed as part of a diagnostic check-up revealed nodal metastatic foci along the meninges of the spinal cord. In accordance with the treatment protocol, the patient was diagnosed with stage 4 disease and received intensive polychemotherapy resulting in the disappearance of the nodal lesions in the spinal cord and a good prognosis. The parents gave their consent to the use of their child's data, including photographs, for research purposes and in publications.
Highlights
ФГБУ «Национальный медицинский исследовательский центр детской гематологии, онкологии и иммунологии им
Half of the Embryonal rhabdomyosarcoma (eRMS) occuring in the head and neck are parameningeal
In patients with intracranial tumors, metastatic spread can occur along the central nervous system (CNS) meninges
Summary
Эмбриональная рабдомиосаркома с метастатическим поражением оболочек спинного мозга: диагностический случай и МР-картина. Эмбриональная рабдомиосаркома (эРМС) является одной из распространенных сарком мягких тканей в детском возрасте и составляет 4,5% всех опухолей. Нами представлен клинический случай пациентки 4 лет с эРМС параменингеальной локализации, у которой на этапе обследования при проведении МРТ ЦНС были выявлены узловые очаги метастазирования по оболочкам спинного мозга. The literature describes only 4 clinical cases of eRMS with distant metastases in the spinal cord and along the meninges. In accordance with the treatment protocol, the patient was diagnosed with stage 4 disease and received intensive polychemotherapy resulting in the disappearance of the nodal lesions in the spinal cord and a good prognosis The parents gave their consent to the use of their child's data, including photographs, for research purposes and in publications. РМС является одной из наиболее часто встречающихся детских сарком и составляет 3–5% всех опухолей [1].
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