A 60-year-old, otherwise healthy male patient presented with a painless swelling near the lower lid of his left eye at the ophthalmology clinic of Ohud hospital in Al-Madinah Al-Munawwarah, Kingdom of Saudi Arabia. At that time, he had had the swelling for almost 3 years. He had no similar swelling elsewhere in the body and no history of any malignancy in the past ().\nOn examination, the swelling was a superficial, non-tender, pink-colored nodule located horizontally on the tarsal plate of the left lower eyelid. It measured 2 cm x 1.5 cm x 1 cm and was mobile and firm in consistency. The swelling clinically resembled a benign lesion. The rest of the ocular examination was unremarkable in both eyes.\nThe excision biopsy of the lesion revealed a 2.5 cm x 1.5 cm x 1.3 cm mass. On the cut surface, a well-circumscribed, 2 cm x 1 cm x 0.8 cm grayish-white, firm tumor was seen in the center with 5millimeter (mm) surgical margins on all sides. A histological examination of the tumor showed overlying thinned-out epidermis, beneath which were seen sheets, cords and nodules of tumor cells lying in the pool of extracellular mucin (). At places, the cells showed cords, papillae and tubule formation, the tumor cells showed mild atypia and occasional mitotic figures; no necrosis was seen (), and all the surgical margins were free from the tumor. A diagnosis of mucinous adenocarcinoma was made. A wide range of immunohistochemistry panels were run to confirm the diagnosis. The tumor cells were strongly positive for cytokeratin 7 (CK7), cytokeratin 5/6 (CK5/6), tumor protein 63 (p63), estrogen receptor (ER), progesterone receptor (PR) and were negative for cytokeratin 20 (CK20) (). To investigate the possibility that this tumor did not represent a metastatic mucinous adenocarcinoma, the patient underwent a complete metastatic work-up which included, computed tomography (CT) of chest and abdomen, Positron emission tomography (PET) scan, esophago-gastro-duodenoscopy and sigmoidoscopy; no abnormalities were detected. The final diagnosis of a Primary mucinous adenocarcinoma of the eyelid was given.\nConsidering the age of the patient and the long duration and gradually increasing size of the swelling, the patient was scheduled for a wide local excision with free surgical margins and referred to the King Fahad Hospital in Al-Madinah Al-Munawwarah.\nFor the past year, the patient has been on a regular follow-up schedule and is clinically free from the disease at present.
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.