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 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.