The patient was a 62-year-old female of African American ethnicity, with a past medical history of type 2 diabetes mellitus, chronic kidney disease secondary to diabetes, glaucoma, macrocytic anemia, chronic back pain and hypertension, who initially presented to her ophthalmologist with a small growth over her left upper eyelid. The patient had the swelling for more than four years that had gradually increased in size. She did not have it evaluated as it very occasionally caused symptoms like on and off tearing, itching, and irritation. She decided to seek medical care when she noticed some tenderness over the lesion. On initial evaluation, she was noted to have a 0.6 mm, elevated, cystic-appearing subcutaneous nodule in the central medial left upper lid margin and lash row. It was noninflamed and tender to palpation. The lesion was in close proximity to the cornea. All other eyelid and orbital functions were normal.\nThe lesion was initially thought to be a benign cyst. As it was symptomatic, it was excised and reconstruction was done. Histopathological analysis revealed positivity for carcinoma cells and suggested that it was a mucinous colloid carcinoma. As the margins were positive, a repeat excision with wider margins and reconstruction via advancement of her lateral full thickness upper lip into the central upper lid was done following a superior cantholysis of the lateral canthal tendon. The surgeons felt that there was no evidence of regional extension beyond what was excised. This was further confirmed by CT imaging of the orbits that revealed no locoregional extension or residual tumor (Figure ). Histopathological exam showed that the tumor was present within the dermis abutting the orbicularis muscle and was composed of lobules of epithelial cells floating in pools of mucin. Small ductal structures were observed within the lobules. A dual population of epithelial cells was identified with mild to moderate pleomorphism admixed with some atypical mitotic figures. Adjacent to the tumor, there were distended ductal structures with atypical proliferation of epithelial cells with a cribriform architecture and extracellular mucin. The tumor extended into the deep tissue edges, but perineural invasion was not identified. Immunohistochemical analysis revealed that the tumor was positive for tumor protein p63 (P63) and cytokeratin 7 (CK7) and negative for cytokeratin 20 (CK20), thyroid transcription factor-1 (TTF-1), and human epidermal growth factor receptor 2 (HER-2). These findings confirmed the diagnosis of PMA of the eyelid.\nGiven that an underlying occult malignancy of the gastrointestinal tract or breast may present similarly, she was evaluated extensively with CT scans of the thorax, abdomen, and soft tissues of the neck. She also had a mammogram and a colonoscopy. All the tests were unremarkable and negative for malignancy. Given the rarity of the disease, Radiation Oncology and Medical Oncology services were integrated to her care. She is being followed up in regular intervals and has not had any recurrence of her tumor thus far.
A 79-year-old male visited our clinic for the evaluation of left ocular pain that had recently worsened. His left eye was phthitic due to glaucoma. The patient had also previously undergone an endoscopic rectal polypectomy. Evisceration and silicone ball (18 mm) implantation were performed. He returned six weeks later with symptoms of mild fever, cough, and chills, as well as left facial erythema, swelling, and tenderness (). We diagnosed the symptoms as orbital cellulitis induced by recent evisceration surgery and the common cold. However, despite two weeks of empirical antibiotic therapy, the patient's symptoms worsened. An orbital computed tomography (CT) scan revealed enhanced soft tissue infiltrations in his left extraconal and intraconal spaces and a thickening of the left eyelid (). An incisional biopsy of the orbital tissue was performed through the superomedial portion of the conjunctiva. The tumor was gray-white colored and was not separated from the surrounding tissue on gross examination. Histopathology demonstrated cells with round to oblong nuclei, small distinct nucleoli, and relatively abundant granular amphophilic cytoplasm. Some cells had signet ring cytology, with a dominant cytoplasmic vacuole and an eccentrically placed, crescent-shaped nucleus (). Immunohistochemistry revealed that the tumor tissue was positive for cytokeratin and cytokeratin 7 but negative for cytokeratin 20.\nResults of an abdominal ultrasound, abdominal-pelvic CT, and a whole body bone scan suggested no systemic involvement. Additionally, tumor marker tests were all within the normal ranges. However, brain magnetic resonance imaging and positron emission tomography revealed metastatic lesions on the left frontal and temporal lobes of the skull; nasal bone; left basal skull; right mandibular ramus and sternum; vertebra in the cervical, thoracic, lumbar and sacral regions; and the left ischium (). To rule out a secondary tumor from the previous rectal lesion, rectal biopsy was performed and revealed a benign adenoma confined to the mucosal layer. Consequently, the lesion in question was diagnosed as a primary malignant eccrine sweat gland carcinoma of the eye (a histiocytoid variant of eccrine sweat gland carcinoma) with multiple bone metastases. Although the patient was fully functional on the first visit, his general condition declined during the subsequent three months. The patient became bedridden, refused all recommended treatments, and received only conservative treatment.