Levinzon L. · Madigan M. · Nguyen V. · Hasic E. · Conway M. · Cherepanoff S.
Purpose: To determine the expression of histone deacetylase enzymes in uveal melanoma tumour cells. Procedures: This is an observational immunohistochemical study of 16 formalin-fixed, paraffin-embedded eyes enucleated for uveal melanoma between January 2001 and March 2002. Haematoxylin and eosin paraffin sections were reviewed for histopathological parameters according to the American Joint Committee on Cancer 7th edition. Sections were then immunohistochemically stained for histone deacetylases 1, 2, 3, 4 and 6 and sirtuin 2 using an automated Leica Bond II platform and Fast Red chromogen, then digitally scanned using Aperio software before assessment of staining. Results: Nuclear expression of histone deacetylases 1, 2, 3, 4 and 6 and of sirtuin 2 was confirmed in uveal melanoma tumour cells. In addition, the tumour cells showed cytoplasmic expression of histone deacetylases 4 and 6 and sirtuin 2. Nuclear and cytoplasmic immunostaining was also seen in intraocular tissues uninvolved by the tumour. Conclusion: Uveal melanoma tumour cells express histone deacetylases 1, 2, 3, 4 and 6 and sirtuin 2, confirming potential tissue targets for histone deacetylase inhibitors.
Honig S.E. · Srinivasan A. · Shields C.L.
Background: We report a case of suprachoroidal hemorrhage simulating choroidal melanoma in a patient with idiopathic thrombocytopenic purpura (ITP). Methods: Case report. Results: A 79-year-old white male with a history of lung adenocarcinoma, ITP, and mild anemia noted blurred vision with photopsia and floaters in the right eye (OD) that worsened over 2 months. He was found to have a pigmented choroidal mass and was referred for evaluation of possible choroidal melanoma. Visual acuity was 20/30 in the affected right eye and 20/25 in the left eye (OS). There was mild vitreous hemorrhage and 2 pigmented peripheral choroidal tumors in the temporal and nasal fundus OD. B-scan ultrasonography confirmed 2 acoustically hollow tumors, and transillumination demonstrated no shadowing. Optical coherence tomography over the lesions revealed choroidal elevation with suprachoroidal scalloped surface. Fluorescein and indocyanine green angiography revealed normal choroidal fluorescence and cyanescence with no “double circulation” of melanoma and no dye blockage, suggesting a suprachoroidal location. These findings were suggestive of suprachoroidal hemorrhage rather than melanoma. The patient was managed conservatively, and the hemorrhages demonstrated spontaneous resolution. Conclusion: Ocular manifestations of ITP are uncommon. Rarely, spontaneous suprachoroidal hemorrhage simulating melanoma can occur. A thorough clinical evaluation with multimodal imaging is critical to establishing the appropriate diagnosis.
McGrath L.A. · Bacon J.F. · Rundle P. · Rennie I.G. · Mudhar H.S.
Malignant melanoma is the most common primary malignant tumour of the iris, but represents a small proportion of all uveal melanomas. The authors describe a 34-year-old male with a pigmented lesion of the iris. The lesion remained stable for 7 years, but the patient re-presented after this time with sudden enlargement of the mass and hyphaema. Excisional biopsy confirmed cavitary melanoma of the iris. This is the first reported case of cavitation in a primary iris melanoma. The patient has not had any further adjuvant treatment and remains metastasis free at 5 years of follow-up.
Kasaee A. · Rafizadeh S.M. · Ghadimi H. · Shahraki K. · Asadi Amoli F. · Jamshidian-Tehrani M.
Neuroblastoma, a relatively common tumor in infants, is rarely present at birth. A solid mass is the typical appearance of this neoplasm, while cystic formation is uncommon. Congenital cystic neuroblastoma is extremely rare and no previous report exists of its occurrence in the orbit. A newborn girl was referred with an extremely large mass on the right side of her face on the third day of her life. Prenatal ultrasonography had revealed a large mass (80 × 80 mm) in the frontal area. MRI revealed a heterogeneous mass composed of cystic and solid, contrast-enhancing components. Excisional biopsy was successfully performed and histopathologic diagnosis of neuroblastoma was confirmed. Systemic workup was negative for involvement of other organs and levels of catecholamine metabolites of serum and urine (homovanillic acid and vanillylmandelic acid) were normal. Postoperative course was uneventful with complete healing of the anophthalmic socket and normal physical and mental growth and development of the infant. To our knowledge, this is the first report of congenital cystic neuroblastoma primarily arising in the orbit.
Sivalingam M.D. · Dalvin L.A. · Shields C.L. · Mashayekhi A. · Shields J.A.
To report a case of amelanotic ciliochoroidal melanoma in a patient with oculocutaneous albinism. A 76-year-old Caucasian male with a past medical history of oculocutaneous albinism and recurrent urothelial carcinoma was found to have a mass in the left eye, suspicious for ciliochoroidal melanoma. On examination, visual acuity was 20/400 in both eyes (OU). External examination showed iris transillumination defects. Funduscopic examination OU revealed blonde fundus, optic nerve hypoplasia, and foveal hypoplasia, confirmed on optical coherence tomography. Funduscopic examination of the left eye revealed an inferonasal amelanotic ciliochoroidal mass, measuring 12.0 mm × 13.0 mm × 8.8 mm. There was visible intrinsic tumor vasculature and overlying subretinal fluid. B-scan ultrasonography demonstrated a hypoechoic, dome-shaped mass. The clinical and imaging features were consistent with amelanotic ciliochoroidal melanoma. The patient was treated with iodine-125 plaque radiotherapy. At the 4-month follow-up, the tumor demonstrated regression from 8.8 mm to 3.2 mm in thickness. Despite the apparent lack of uveal pigmentation, patients with oculocutaneous albinism can develop uveal melanoma.
Francis J.H. · Barker C.A. · Yin V.T. · Carvajal R.D. · Chapman P. · Abramson D.H. · Gobin Y.P.
Background/Aims: The treatment of orbital melanoma poses a management challenge. This case explores the delivery of high-dose melphalan to an orbital recurrence of uveal melanoma via intra-arterial delivery of melphalan to the orbit. A 62-year-old man developed recurrent orbital disease 7 months after enucleation for a large uveal melanoma. He received 6 monthly intra-arterial infusions of melphalan to the orbit, ranging in dose from 20 to 30 mg per infusion. Following the last infusion, mild temporary erythema was noted on the forehead along the distribution of the supratrochlear artery. The orbital recurrence was reduced in size by 66% in the longest dimension as measured by magnetic resonance imaging (MRI). However, 9 months following intra-arterial melphalan, tumor regrowth was detected on MRI, and additional treatment options were pursued. Conclusion: This case demonstrates that intra-arterial melphalan can result in nonsustained tumor regression of recurrent orbital uveal melanoma. It suggests that local delivery of high-dose melphalan may be helpful as a neoadjuvant treatment for uveal melanoma, and future studies will be useful to confirm the value of this approach in additional cases of recurrent and possibly in primary uveal melanoma.
Ancona-Lezama D.A. · Dalvin L.A. · Lucio-Alvarez J.A. · Jabbour P. · Shields C.L.
Purpose: Intra-arterial chemotherapy (IAC) has become a mainstay in the management of retinoblastoma, especially in advanced or refractory disease. However, IAC is not without complications, and chemotherapy toxic effects can lead to partial or complete choroidal ischemia, often causing vision loss. Methods: This is a case report. Results: A 4-month-old girl with bilateral retinoblastoma was treated with secondary IAC (melphalan 5 mg) for recurrent tumor following intravenous chemotherapy. One month later, complete tumor control was achieved. However, she demonstrated broad choroidal ischemia in the nasal and temporal quadrants but sparing of the watershed zone superior and inferior to the optic disc and in the papillomacular region. Fluorescein angiography revealed poor perfusion of the choriocapillaris with visibility of the large choroidal vessels in the nasal and temporal areas but preserved perfusion of the watershed zone. The watershed zone remained intact on the 10-month follow-up, and the final visual acuity was fix and follow without strabismus. Conclusion: The pathophysiology of choroidal ischemia is not well understood, but the fortuitous watershed zone preservation in this case could represent uneven distribution of the chemotherapeutic drug, resulting in partial chemo-dilution of the medication in the watershed region, which represents the final downstream overlapping choroidal perfusion from both medial and lateral posterior ciliary arteries.
Baily C. · O’Neill V. · Dunne M. · Cunningham M. · Gullo G. · Kennedy S. · Walsh P.M. · Deady S. · Horgan N.
Purpose: To report the clinical features and epidemiology of uveal melanoma in Ireland. Methods: This was an observational study of 253 patients with a new diagnosis of uveal melanoma between June 2010 and December 2015. Main outcome measures included demographics, clinical features, age-adjusted incidence, relative survival, overall survival, and distant metastases-free survival. Results: The mean patient age was 61.7 years. Tumour location was choroidal in 82%, ciliochoroidal in 9%, iridociliary in 2%, and iris in 7%. Treatment modalities included brachytherapy (ruthenium-106 and iodine-125 [64%]), enucleation (27%), and proton beam radiation (8%). The mean age-adjusted incidence of uveal melanoma in Ireland from 2010 to 2015 was 9.5 per million of the population (95% confidence interval [CI]: 8.4–10.7). Four-year relative survival was 81.3% (95% CI: 72.8–87.3). Four-year overall survival was 84% (95% CI: 78–90) and 4-year distant metastases-free survival was 79% (95% CI: 73–86). Conclusion: Based on this data, the incidence of uveal melanoma in Ireland is high when compared with other reported incidence rates in Europe and worldwide. Relative and observed survival were in keeping with other reported European survival rates.
India, with a population of 1.35 billion, nearly 20% of the world’s population, is establishing itself as an economic and intellectual powerhouse, not unlike China. The once yawning differences between developed and developing countries continue to shrink. Yet, accurate epidemiological data on some cancers, like malignant eyelid tumors, have never been obtained on a national level in India, unlike in many other (developed and developing) countries. Indeed, the first reports on malignant eyelid tumors were only published as recently as 1996, albeit small institution-based reports. Kaliki et al.  from Hyderabad, India, have gone some way in rectifying this deficiency: they have compiled an important analysis of 536 malignant eyelid tumors seen at their institution over a 22-year period. Histopathology-proven diagnoses of these patients included sebaceous gland carcinoma (SGC; n = 285, 53%), basal cell carcinoma (BCC; n = 128, 24%), squamous cell carcinoma (SCC; n = 99, 18%), and miscellaneous tumors (n = 24, 4%). The young oculoplastic resident or fellow would do well to scrutinize this paper carefully and be asked which of these statements is true. SGCs have a higher incidence in Asian Indians (hereafter referred to as Indians) than in Caucasians. SGC is much more common (higher incidence) than basal cell carcinoma in Indians (or twice as common, as they found). The commonest eyelid malignancy in Indians is SGC. In Indians, SGC is three times more common than SCC. BCC is more common in females across all age groups, with a male to female ratio of 1: 1.5. Of course, none of these statements is necessarily true, based upon the findings of this study……
Kaliki S. · Bothra N. · Bejjanki K.M. · Nayak A. · Ramappa G. · Mohamed A. · Dave T.V. · Ali M.J. · Naik M.N.
Purpose: To study the proportion of eyelid malignant tumors in an Asian Indian population and to review their clinical features and outcomes. Methods: This is a retrospective study of 536 patients. Results: The mean age at presentation with eyelid malignancy was 58 years. Histopathology-proven diagnoses of these patients included sebaceous gland carcinoma (SGC) (n = 285, 53%), basal cell carcinoma (BCC) (n = 128, 24%), squamous cell carcinoma (SCC) (n = 99, 18%), and miscellaneous tumors (n = 24, 4%). The statistically significant differences between eyelid malignant tumors included age at presentation, tumor location, and tumor extent. The clinicopathological correlation of SGC, BCC, SCC, and miscellaneous tumors was 91, 86, 46, and 38% (p = 0.001), respectively. Comparing SGC with BCC, SCC, and miscellaneous tumors, SGC was more commonly associated with tumor recurrence (21 vs. 3, 8, and 13%; p = 0.001), systemic metastasis (13 vs. 0, 4, and 13%; p = 0.001), and death (9 vs. 0, 4, and 0%; p = 0.004). Compared to SGC, BCC, and SCC, locoregional lymph node metastasis was more common with miscellaneous tumors (26 vs. 16, < 1, and 8%; p = 0.001) over a mean follow-up period of 19 months. Conclusion: In Asian Indians, SGC is twice as common as BCC and 3 times more common than SCC. SGC is associated with poorer prognosis compared to other eyelid malignant tumors.