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Miofibromatosis infantil: reporte de un caso y revisión de la literatura

Keywords: infantile myofibromatosis, neck tumors.

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Abstract:

background. infantile myofibromatosis (im) is a disease characterized by solitary or multiple benign tumors. the etiology is unknown. im isa benign mesenchymal disorder of early infancy and is more frequent in males. im may present in two manners: asa solitary lesion most commonly in skin, bone, muscle, subcutaneous tissue, in head, neck and trunk, with good prognosis, or a multicentric form of im with or without visceral involvement (heart, lung, gastrointestinal tract, kidney) with a poor prognosis. the definitive diagnosis of im is confirmed by pathology. treatment may be conservative (observation with close follow-up) or surgery (solitary form) or chemotherapy (visceral form). case report. we reporta case of a 16-months-old male patient with left neck tumor diagnosed as im. he was admitted into intensive care unit because of respiratory distress with stridor caused by tumor compression. mechanical ventilation was required by the patient who underwent surgery to resect the tumor. the patient had a favorable postoperative evolution. conclusions. im must be considered in the differential diagnosis of tumors in early infancy, despite its low frequency. treatment and prognosis depend on location, clinical form (solitary or multicentric), with or without visceral involvement.

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