| 본문 : Purpose: We report a case of a 2-month-old infant with a rare phakomatous choristoma presenting as an orbital tumor with involvement of the inferior oblique muscle.
Method: A 2-month-old male was referred to ophthalmology for a congenital mass of the medial aspect of right lower eyelid. Magnetic resonance imaging and excision of a congenital inferonasal orbital mass was conducted. An immunohistochemical testing was performed with antibodies against CK,S100, Desmin, P63, MyoD1, Ki67, CD31, CD34.
Result: Ophthalmic examination showed a subcutaneous mass in the inferomedial aspect of the right lower eyelid. Magnetic resonance imaging demonstrated a homogenous, 10 x 8 x 7mm well defined intermediate to mild
hyperechoic mass inferomedial the right globe. There was no bone erosion. With these radiographic findings and the age of presentation, the tumor was assumed to be a rhabdomyosarcoma or lymphoma. During the surgery, the lesion was discovered involving the inferior oblique muscle. On gross examination, a dark red soft mass was observed. The immunohistochemical staining was positive for CK,S-100, but was negative for desmin, P63, MyoD1, CD31,CD34.
Concluison: The pathologic diagnosis was consistent with phakomatous choristoma. We present this rare case of phakomatous choristoma, a rare congenital tumor involving the inferior oblique muscle. |