A 40-year-old man presented with right eye axial proptosis and ophthalmoplegia for 3 months. Imaging study showed a right intraconal mass with the erosion of the orbital floor. Incisional biopsy revealed mucoepidermoid carcinoma. Nasal endoscopy was normal and systemic tumor screening was negative for a primary source. The patient underwent right orbital exenteration, uncinectomy, nasal and maxillary mucosal biopsy. Malignant cells were found present in the mucosa of maxillary sinus roof and uncinate bone. The postoperative positron emission tomography scan showed residual active lesion in right orbital apex and maxilla but no primary lesion elsewhere. The patient subsequently underwent 35 cycles of postoperative radiotherapy. Primary mucoepidermoid carcinoma of the orbit is rare and typically arises from the lacrimal gland or sac. Those tumors not arising from lacrimal apparatus should be presumed metastatic in origin, and the thorough systemic survey should be undertaken in the search for the primary tumor.

A 25-year-old Chinese woman presented with recurrent painless swelling over the left medial canthus region for 3 months and intranasal mass for an indeterminate duration. Initial incision biopsy of the mass was reported as nodular fasciitis but the lesion recurred 3 weeks later. Intraoperative findings during repeat biopsy showed a mass extending from the deep dermal tissue into the anterior orbit and polyp-like nasal mass. Histopathology findings were that of dermatofibrosarcoma protuberans (DFSP). The mass recurred 4 months later without orbital or intranasal recurrence. Wide excision biopsy under frozen section guidance was attempted however; clear surgical margins could not be achieved despite extensive resection. She was subsequently referred for adjuvant radiotherapy. We report an exceptionally rare case of local recurrence of DFSP in an unusual anatomic location. This case was surgically challenging in achieving negative margins, and thus neoadjuvant therapy may improve overall outcome to prevent local relapse.