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 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.