A 65-year-old male with a right lower eyelid mass had history of adenocarcinoma of the left orbit, status post radiation therapy, followed by left orbital exenteration, 12 years prior to presentation at MDACC. An MRI of the orbit, face, and neck performed at MDACC demonstrated a thickened enhancing lesion adjacent to the right nasolacrimal duct measuring 4.1 × 1.0 cm () which extends from the medial canthus to the lateral inferior periorbital soft tissue. In addition, mildly thickened soft tissue enhancement along the left premolar soft tissues suspicious for recurrent disease was reported. Prior positron emission tomography (PET)/CT scan and MRI of the brain performed in an outside facility failed to demonstrate second primary or metastatic disease. Wide local excision of the right lower eyelid and cheek mass was performed with intraoperative margin assessment showing positive margins. Microscopically, cords of histiocytoid cells arising from eccrine glands were identified infiltrating skeletal muscle and orbit with extensive perineural invasion (). Surgical margins were positive. Five months after completion of radiation therapy, the patient noted a new right upper eyelid nodule. A CT scan of head and neck reveals local right retroorbital recurrence and left neck adenopathy. A more radical surgery for the right orbit was avoided due to the cancer demonstrating a very slow growth pattern, and it would render him totally blind.\nFour years of documented follow-up (until January 2019, when the patient was last contacted at MDACC) after the first surgery done for the lower eyelid mass, he was still alive. Currently, the patient is being seen at an outside facility with his only eye globe intact. He has had slowly progressive distant lymph node metastatic disease for which standard treatments have not been offered, and he is not keen to enroll in clinical trials.
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.