Faculty Bibliography

Conjunctival amyloid is usually of the immunoglobulin light chain (AL) variety. The authors report an exceptional case of bilateral limbal amyloid deposits that were identified by mass spectrometry as keratin-related specifically regarding to basal keratinocyte keratins 5 and 14. Cytokeratin-related amyloid has been described by immunohistochemical analysis of formalin-fixed cutaneous and mucous membrane lesions but not ocular tissues. The conjunctival lesion in the right eye contained intraepithelial dyskeratotic cells that extended to the surface, causing a lacy leukoplakia on clinical examination. The authors excluded the diagnosis of hereditary benign intraepithelial dyskeratosis in this patient. Collection of patient data and all protected patient health information was compliant with the Health Insurance Portability and Accountability Act.

The authors report 2 cases of apocrine cystadenoma of the eyelid, 1 of which was studied with immunohistochemical and other special stains. While a previous report describes the tumor in a palpebro-orbital location, no other detailed descriptions of a purely eyelid location are present in the literature. Apocrine cystadenoma occupies an unusual portion of the spectrum of Moll gland tumors of the eyelid. It has no definitive clinical characteristics and may be diagnosed pathologically by hematoxylin-eosin-stained sections.

PURPOSE: To evaluate the clinical behavior of spheno-orbital meningiomas with regard to World Health Organization (WHO) tumor grade and Ki-67, a cellular marker of proliferation. METHODS: A retrospective review over a 16-year period of the demographic, clinical, radiographic, and surgical data of all patients with spheno-orbital meningioma who underwent surgical resection. Tumor specimens were examined histologically using the current WHO 2016 classification and immunohistochemically using Ki-67/MIB-1 monoclonal antibody. RESULTS: Thirty-eight patients met all inclusion criteria: 78.9% of tumors were WHO grade I with a mean Ki-67 of 3.76, and 93% of patients were clinically stable at last follow up; 10.5% of lesions were WHO grade II (atypical) with a mean Ki-67 of 14.93, and 10.5% of lesions were WHO grade III (anaplastic) with a mean Ki-67 of 58.3. All grade II and III meningiomas exhibited an aggressive clinical course. There were statistically significant correlations between disease clinical progression and WHO tumor grade (p < 0.001), between disease clinical progression and Ki-67 (p < 0.001), and between increasing Ki-67 index and higher WHO grade (p < 0.001). For WHO grade I lesions, a Ki-67 of >/=3.3 correlated with recurrence (p = 0.0256). Overall, disease-specific mortality occurred in 5 (13%) patients. CONCLUSIONS: Ki-67 index is a valuable marker to use in conjunction with WHO grade to predict meningioma behavior, particularly in histologically borderline lesions, and possibly to identify a subset of WHO grade I tumors at risk of recurrence. This combination of methods can aid in tailoring treatment and surveillance strategies.

OBJECTIVE: To assess outcomes after endoscopic endonasal surgery for recurrent or residual pituitary adenomas. METHODS: We retrospectively analyzed 61 patients from 2009 - 2016 that underwent endoscopic endonasal surgery for recurrent or residual pituitary adenomas after prior microscopic or endoscopic transphenoidal operation. RESULTS: Prior surgical approach was endoscopic endonasal in 55.7% and microscopic in 44.2%. The mean preoperative maximal tumor diameter was 2.3cm. Tumor commonly invaded the suprasellar cistern (63.9%). Gross total resection (GTR) was achieved in 31 (51.7%) patients. GTR rate was 68.4% and 21.7% for Knosp grade 0-2 and grade 3-4 tumors, respectively (p<0.001). GTR was 73.1% and 35.3% for patients with previous microscopic and endoscopic transphenoidal surgery, respectively (p=0.002). On multivariate analysis, smaller tumor size (odds ratio [OR] 1.1 per cm, p=0.007), Knosp grade 0-2 (OR 9.7, p=0.002), and prior microscopic approach (OR 12.7, p=0.007) were independent predictors of GTR. Preoperative visual deficit outcome was improvement in 32.5%, unchanged in 62.5%, and worse in 5.0%. New postoperative endocrinopathies included adrenal insufficiency (6.5%), hypothyroidism (8.1%), hypogonadism (6.5%), and diabetes insipidus (4.9%). Complications included postoperative CSF leak (4.9%), meningitis (1.6%), medical complications (4.9%), and postoperative hematoma requiring re-exploration (3.2%). CONCLUSION: The endoscopic endonasal approach provides a safe and effective option for recurrent pituitary adenomas. Smaller tumor size, absence of cavernous sinus invasion, and prior microscopic approach were independent predictors of GTR. This might suggest inadequate exposure or limited viewing angle may adversely affect extent of resection in primary microscopic surgeries.

The authors describe a rare case of a primary extradural ectopic meningioma occurring in a 9-year-old female. A review of the literature with respect to clinical presentation, radiographic findings, management, and outcome among similar cases is discussed. Common features that may assist with diagnosing this unusual tumor include absence of bone or optic nerve sheath involvement, presentation at a young age, occurrence in the medial orbit, and sinus asymmetry on radiographic imaging. Preferred method of treatment is complete surgical excision.

The authors describe a spindle cell lipoma that occurred in the anterior lamellae of the eyelid in a 47-year-old man. This benign tumor was reported previously only once within that location and poses histologic challenges when a "low fat" or "fat free" variant is present.

PURPOSE: To describe two cases of choroidal hemorrhage caused by optic disk drusen-induced choroidal neovascularization simulating uveal melanoma. METHODS: Observational case reports of two patients and brief review of the literature. RESULTS: Two patients were referred with pigmented juxtapapillary lesions concerning for choroidal melanoma. Multimodal imaging revealed the presence of optic disk drusen with overlying choroidal neovascular membranes and peripapillary choroidal hemorrhage. Both patients were treated with antivascular endothelial growth factor and the lesions resolved. CONCLUSION: In the setting of diagnostic uncertainty, careful multimodal imaging can assist in distinguishing between malignant choroidal melanoma and a benign simulating lesion. Optic disk drusen with associated neovascularization and hemorrhage should be included in the list of pseudomelanomas.