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 62-year old male presented to the oncology center for evaluation of a painless, superficial nodular lesion over his left lower eyelid that had slowly grown over the course of approximately 18 months, to measure 4.0 × 2.0 cm. Patient gave history of a similar swelling at the same site which had appeared in July 2005 and after a similar slow, painless progressive increase in size, it had been excised in February 2006. The histopathology report from the previous surgery was not available for review. The swelling recurred within six months and displayed a painless, gradual, progressive increase in size till he presented at our center. On examination, he had a well-defined, irregularly marginated nodular lesion over the left lower eyelid []. The overlying skin was normal in appearance and freely mobile over the underlying nodular lesion except for an area of 1 × 1 cm at the lateral end, where the scar of the previous surgery was tethered to the mass. There was no regional lymphadenopathy. The lesion appeared free from the underlying orbital ridge. Fine needle aspiration cytology from this lesion reported a benign adenexal tumor. The lesion was excised with 5 mm margins under general anesthesia. The resultant defect involved the entire lower eyelid and was reconstructed by a Mustarde's cheek rotation flap. This was a large skin flap which was rotated from the cheek. Incision began at the lateral canthal angle, extending upward onto the temple, and swinging posteriorly just anterior to the ear and then inferiorly across the mandible []. Eight weeks postoperatively, there was marginal ectropion and lateral tissue sag []. Gross pathology revealed a subcutaneous nodule of tan, gelatinous tissue measuring 4 × 2 × 1.0 cm. Microscopic examination revealed a dermal tumor composed of epithelial cell islands surrounded by lakes of mucin consistent with the diagnosis of mucinous carcinoma []. The lateral margins were tumor cell-free but the deep resected margin was involved. A thorough search for other possible sources of mucinous adenocarcinoma was made. Upper and lower gastrointestinal study, contrast enhanced computer tomography study of the chest and abdomen, as well as a whole body Positron Emission Tomography scan were negative for any other primary.