A 51-year-old male presented to MDACC for evaluation of a left upper eyelid mass. On review of the outside needle core biopsy sections, scant histiocytoid cells (CK7 positive) were noted percolating among normal lacrimal gland tissues. The microscopic assessment was a poorly differentiated adenocarcinoma with indeterminate origin due to the limited biopsy sample. An MRI of orbit and face showed an infiltrative lesion involving pre- and postseptal regions of the left upper and lower eyelids, the lacrimal gland, and the focal intraconal extension (). The patient's previous PET/CT scan was negative for a second malignancy. Exenteration of the left orbit with intraoperative evaluation of the margins was performed. Macroscopically, the tumor measured 5.5 × 2.5 cm. Histopathologically, clusters of atypical histiocytoid cells involving the dermis, tarsus, palpebral conjunctival, and periorbital soft tissues were present (). Despite wide excision beyond the clinically visible mass, tumor cells were seen along the cauterized resection margins. Postoperative adjuvant chemoradiation therapy was completed, and at 12-month follow-up, there was no evidence of recurrence.
A 40-year-old male consulted MDACC with a history of thickening of the left medial lower eyelid and enlargement of a caruncle. A CT scan of orbits and head and neck soft tissue demonstrated an enhancing and infiltrative mass along the inferomedial aspect of the left anterior orbit () and no evidence of facial or cervical lymphadenopathy. PET/CT scan performed in an outside facility did not detect second primary malignancy or metastatic disease. Radical surgical resection of the left orbital, eyelid, and lacrimal sac mass with intraoperative assessment of margins was performed. Grossly, the specimen measured 4.0 × 3.5 cm. Histopathologically, neoplastic cells forming a mass lesion adjacent to the punctum and infiltrating caruncle was seen (). Cords of pleomorphic cells with histiocytoid appearance infiltrating connective tissue and depicting vesicular nuclei, large nucleoli, and eosinophilic cytoplasm with intracytoplasmic vacuolization were identified ( inset). Perineural invasion was present. The patient underwent proton therapy and chemotherapy. Thirteen months after treatment, the patient had a left neck dissection with metastatic adenocarcinoma to two of six lymph nodes at level III.