Cristina Miyamoto

Current and emerging treatment options for uveal melanoma

Uveal melanoma (UM) is the most common primary malignant intraocular tumor in adults, with a 10-year cumulative metastatic rate of 34%. The most common site of metastasis is the liver (95%). Unfortunately, the current treatment of metastatic UM is limited by the lack of effective systemic therapy. Options for the management of the primary intraocular tumor include radical surgery as well as conservative treatments in order to preserve visual acuity. For metastatic disease, several approaches have been described with no standard method. Nevertheless, median survival after liver metastasis is poor, being around 4–6 months, with a 1-year survival of 10%–15%. In this review, the authors summarize current and promising new treatments for UM.

Uveal melanoma dormancy: an acceptable clinical endpoint?

Uveal melanoma is a rare but life-threatening malignancy. Over the past decades, the morbidity of uveal melanoma has been markedly reduced as a result of advances in the diagnostic ability to detect smaller tumors at an earlier stage. This has allowed for the use of more conservative treatments, avoiding enucleation. Mortality, however, has remained unchanged. This indicates that life expectancy is independent of local tumor control. Metastatic disease, the leading cause of death, is usually diagnosed many years later, despite successful treatment of the primary tumor, and at a late stage, when no effective therapy is available. These observations suggest that the disease was already disseminated at the time of tumor diagnosis. The detection of circulating malignant cells in the bloodstream of patients at different time points in the course of the disease supports this observation. Tumor dormancy has been considered as the leading theory for this intriguing delayed appearance of metastasis. Recent knowledge gained about the biological behavior of uveal melanoma as well as novel potential therapeutic targets are presented in this review.

Rapid growth of an orbital hemangiopericytoma with atypical histopathological findings.

Hemangiopericytoma is a rare vascular tumor that originates from pericytes. The orbit is a rare location for this particular tumor, and corresponds to 0.8% to 3% of all primary orbital tumors. We report a case of a hemangiopericytoma in a 45-year-old man that had an unusual presentation, as a rapidly growing mass in the anterior right inferior orbit. Given that there are no clinical or radiological signs pathognomonic of this tumor, a careful histopathological examination is necessary to confirm the diagnosis. In our case, it presented also with unusual histopathological findings. The clinical features, radiological findings, differential diagnosis and treatment of this challenging entity are reviewed in this case report.

Use of CD25 as an immunohistochemical marker for acquired ocular toxoplasmosis.

PURPOSE Toxoplasmosis is the most common cause of posterior infectious uveitis worldwide. It is often impossible to determine its congenital or acquired nature. Interleukin-2 (IL-2) in peripheral blood has been described as a possible marker for acquired toxoplasmosis. The purpose of this study is to evaluate the histopathological characteristics of ocular toxoplasmosis cases using CD25 as a marker for the expression of interleukin-2. METHODS Ten formalin-fixed, paraffin-embedded enucleated globes from ten immunocompetent patients with clinical diagnosis of toxoplasmosis were evaluated. Four patients had the acquired form of ocular toxoplasmosis (positive IgM) while six were IgM negative and IgG positive for toxoplasmosis. Histopathological slides were reviewed for the extension of the retinal necrosis, number of toxo cysts, the granulomatous inflammatory reaction, the presence of T and B cells within the choroid and the IL-2 expression. Immunohistochemistry using monoclonal antibodies was performed to observe the expression of CD4, CD8, CD20, CD25, and CD68. RESULTS The histopathological evaluation disclosed no differences between acquired and the other ocular toxoplasmosis cases regarding the characteristics studied. However, CD25 showed a higher expression of IL-2 on the 4 acquired cases of ocular toxoplasmosis compared to the remainders. CONCLUSIONS To the best of our knowledge, this is the first report showing that the use of CD25 as a marker for interleukin-2 could differentiate acquired ocular toxoplasmosis.

Uveal melanoma: Ocular and systemic disease

Although rare, uveal melanoma is the most common intraocular tumor in adults. Most cases arise from the choroidal layer of the uvea, displaying a discoid, collar-button, or mushroom shaped growth. Histopathologically, neoplasms are classified by the dominant cell type: spindle, epithelioid or mixed spindle cell type. The most important prognostic factors are cell type, nucleolar size, largest tumor dimension, and mitotic figures. Patient prognosis is poor when metastases occur in the liver, one of the main reasons that despite advances in the diagnosis and treatment of uveal melanoma, the mortality rate has not change significantly since 1973.

Case report: an atypical peripapillary uveal melanoma

BackgroundThe treatment of uveal melanoma has seen a shift towards eye conserving treatments. Efforts have been made towards the identification of patients at high risk of metastatic disease with the use of prognostic fine needle biopsy, Monosomy 3 a risk factor for metastatic death thought to occur early in the development of uveal melanoma.Case presentationWe report a case in which an atypical optic nerve lesion was found to be a peripapillary primary uveal melanoma with distinct non-pigmented and pigmented halves on gross dissection and corresponding disomy 3 and monosomy 3 halves. The tumour demonstrated rapid growth with apparent transformation from disomy 3 to monosomy 3.ConclusionsThese are clinical features that challenge the current concepts of the cytogenetic pathogenesis of uveal melanoma and demonstrate the potential problems and limitations of prognostic fine needle biopsy and molecular classifications.

Sebaceous Cell Carcinoma: A Persistent Challenge in Clinical andHistopathological Diagnosis

Sebaceous cell carcinoma continues to defy clinicians and pathologists in terms of early diagnosis. The tumor may be mistaken as benign lesions such as chalazion and blepharitis, and also as malignant neoplasms, mainly basal cell carcinoma and squamous cell carcinoma. Despite advances in immunohistochemical analysis and treatment options during the last decades, morbidity and metastasis rates remain high. Prognosis is strongly related to the length of time between diagnosis and initiation of treatment, which reinforces the importance of early recognition of this condition. This article reviews key features of sebaceous cell carcinoma, from epidemiology to treatment, and new strategies to improve outcome.