Rabdomiossarcoma alveolar cutâneo primário em paciente pediátrico

A young boy with mild developmental delay and autism presented with a 1-week history of progressive left periorbital swelling. His parents reported that he complained of left orbital pain just before they noticed the swelling, and he was treated with cold compresses with only slight improvement. He had never experienced these symptoms before. The patient had not experienced diplopia, change in visual acuity, nasal obstruction, change in oral intake, weight loss, recent upper respiratory infection, sick contacts, fevers, or chills. At the time of presentation, the patient was breathing comfortably and was afebrile. Findings from his head and neck examination were significant for a firm mass over the left zygoma and lateral infraorbital rim, 2 × 3 cm in diameter. The mass was nontender, nonerythematous, and fixed to the underlying zygoma and lateral infraorbital rim. There was no cervical lymphadenopathy. Ophthalmology examination revealed intact extraocular movements, bilateral visual acuity of 20/50, and no evidence of afferent papillary defect. Computed tomographic (CT) images showed a round, soft-tissue mass with osseous destruction and erosion into the zygoma, infratemporal fossa, inferolateral orbit, and maxillary sinus ( Figure, A and B). Concern for orbital involvement prompted magnetic resonance imaging (MRI) of the maxillofacial region ( Figure, C and D). He was taken to the operating room the following day for open biopsy.

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