Our case highlights a 73-year-old male who was referred for surgical correction of right eye ptosis that was present for 2 years. He complained that his ptosis was progressively worsening. On assessment of his driver’s license from 5 years prior, the right sided ptosis was present. His past medical history was significant for diabetes mellitus, dyslipidemia and remote head trauma resulting in a basal skull fracture. His past ocular history was significant for prior cataract extraction and primary open angle glaucoma which was controlled medically.\nOn initial examination, his visual acuity was 20/25–1 and 20/20–1 in the right and left eye, respectively. His intraocular pressures were 12 mmHg bilaterally. His anterior segment examination was unremarkable. His posterior examination was unremarkable apart from glaucomatous disc damage. His ocular movements were full and there was no enophthalmos or exophthalmos. He was noted to have right-sided ptosis (Fig. ). Upon palpation of his right orbit, he was noted to have an upper lateral orbital rim mass. The patient was otherwise asymptomatic; he did not report pain, pressure or right-sided visual deficits. Computed tomography (CT) noted ill-defined soft tissue thickening anterior to the right globe, predominantly pre-septal but with slight post-septal extension (Fig. ).\nAn orbitotomy was performed for biopsy of the right orbicularis muscle and anterior orbit through an eyelid crease incision. The pathology revealed diffusely and deeply infiltrating tumour cells extending through the dermis, subcutis, orbicularis muscle bundles and nerve fibers (Fig. a-c); the tumour cells were noted to have a monotonous histiocytoid appearance with foamy granular eosinophilic cytoplasm (Fig. d). There was diffuse infiltration of the dermis with sparing of the epidermis and lack of surrounding desmoplastic reaction. In specific areas, lesional cells showed single filing. Sebaceous differentiation was not seen, and the epidermis did not show a cutaneous Paget’s disease-like change. At high magnification, intracytoplasmic vacuoles and occasional intermixed signet ring cells were identified (Fig. e). They were characterized by a crescent-like nuclei and a main, centrally located cytoplasmic vacuole. Rare scattered mitoses were noted. Immunohistochemical staining revealed the tumour cells to be AE1/AE3, CK7, GCDFP-15, E-cadherin, androgen receptor (AR) stain and GATA3 positive (Fig. f). No appreciable staining was noted with CK20, CDX2, TTF-1, S100, mammaglobin, p63, uroplakin-II, PSAP, PAX8, tryptase, CD20, CD117, CD43, WT1, calretinin and PSA stains. MIB-1 showed a low proliferative index (about 10%). Progesterone receptor stain was negative; however, estrogen receptor stain did show patchy staining (about 15–20%). Final pathology report confirmed the diagnosis of primary cutaneous signet-ring cell/histiocytoid carcinoma of the right eyelid. Systemic workup was performed with positron emission tomography (PET) and full body CT which failed to identify a distant primary malignancy or metastatic disease.\nThe decision was made to attempt surgical excision of the tumor. After the bulk of the grossly apparent tumor was removed, intraoperative frozen sections were sent. Unfortunately, frozen pathology was unable to differentiate tumor cells from normal inflammatory histiocytes. Thus, numerous superficial biopsies of the right periorbital soft tissue were performed, which revealed extension significantly further than the gross disease. Thereafter, the patient underwent a wide orbital exenteration with reconstruction using a temporary split-thickness skin graft. On final permanent sections, there still remained positive margins.\nLater, the patient underwent further resection by the head and neck oncology team, in which further extensive resections were performed around the original rim of the exenteration site. The previous skin graft was resected, the orbital apex was further dissected, the maxillary sinus was removed and further resection into the skull base was performed. Neck dissection was performed in preparation for free muscle-skin flap reconstruction after all skin and deep margins were found to be negative.\nTwo rounds of adjuvant post-operative radiation were administered (5040 cGy in 28 fractions) 3 months after his reconstruction. Post-radiation PET and CT scans were negative for recurrence 6 months after his initial surgery. There was no evidence of recurrence after 4 years of follow-up.
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.