Uveal melanoma (UM) is the second most frequent type of melanoma. Therapeutic options for UM favor minimally invasive techniques such as irradiation for vision preservation. As a consequence, no tumor material is obtained. Without available tissue, molecular analyses for gene expression, mutation or copy number analysis cannot be performed. Thus, proper patient stratification is impossible and patients’ uncertainty about their prognosis rises. Minimally invasive techniques have been studied for prognostication in UM. Blood-based biomarker analysis has become more common in recent years; however, no clinically standardized protocol exists. This review summarizes insights in biomarker analysis, addressing new insights in circulating tumor cells, circulating tumor DNA, extracellular vesicles, proteomics, and metabolomics. Additionally, medical imaging can play a significant role in staging, surveillance, and prognostication of UM and is addressed in this review. We propose that combining multiple minimally invasive modalities using tumor biomarkers should be the way forward and warrant more attention in the coming years.
Background: Sebaceous carcinoma (SC) is a rare malignant tumour whereby, comprehensive long-term data are scarce. This study aimed to assess the outcome of patients treated with resection for SC.Methods: Patients treated at four tertiary centres were included. Cumulative incidence curves were calculated for recurrences.Results: A total of 100 patients (57 males, 57%) were included with 103 SCs. The median age was 72 (range, 15-95) years with a median follow-up of 52 (interquartile range [IQR], 24-93) months. Most SCs were located (peri)ocular (49.5%). Of all SCs, 17 locally recurred (16.5%) with a median time to recurrence of 19 (IQR, months. The cumulative incidence probability for recurrence was statistically higher for (peri)ocular tumours (p = 0.005), and for positive resection margins (p = 0.001).Two patients presented with lymph node metastases and additional seven patients (8.7%) developed lymph node metastases during follow-up with a median time to metastases of 8 (IQR, 0.5-28) months. Three patients had concurrent in-transit metastases and one patient also developed liver and bone metastases during follow-up. Conclusion:SC is a rare, yet locally aggressive tumour. Positive resection margins and (peri)ocular SCs are more frequently associated with local recurrence. SC infrequently presents with locoregional or distant metastases.
To demonstrate that long-circulating PEGylated liposomal prednisolone is a safe and effective therapy in patients with active moderate-to-severe Graves' orbitopathy.Methods: Open-label, proof-of-concept, multicentre pilot study. Ten patients with moderate-to-severe Graves's orbitopathy, who were euthyroid for at least three months. Long-circulating PEGylated liposomal prednisolone 150 mg was administered intravenously twice, with 2-week interval. Total follow-up was 12 months, with visits at baseline, week 2, 6, 13, 26 and 52. Physical, laboratory and ophthalmological examinations were performed. Response to treatment was defined as a reduction in Clinical Activity Score by ≥2 points; palpebral aperture by ≥3 mm; soft tissue signs by ≥2 grades; exophthalmos by ≥2 mm; and motility by >8 degrees or improvement in diplopia score. A response was sustained when equally observed at weeks 6 and 13.Results: One patient achieved a sustained response according to the predetermined definition. All patients showed a decrease in Clinical Activity Score after one infusion, with a mean decrease of two points. The Clinical Activity Score was ≤1 at week 52 for all patients. Improvement was also observed in the soft tissue signs. Most of the adverse events were mild and of a transient nature. Two patients required further treatment with intravenous methylprednisolone. Conclusion:This pilot study showed a positive effect of long-circulating PEGylated liposomal prednisolone on the Clinical Activity Score in patients with moderate-to-severe Graves's orbitopathy, resulting in fewer hospital visits and possibly less glucocorticoidrelated side-effects.
Introduction Anterior levator disinsertion/reinsertion and Müllerectomy (ALDM) is generally performed to correct upper eyelid retraction in patients with Graves’ orbitopathy (GO). We studied the outcome of this procedure and its correlation with clinical parameters. Patients and Methods Retrospective analysis of results of ALDM in 305 consecutive euthyroid patients with burnt‐out GO treated in our hospital between 1 January 2000 and 1 January 2016. From the patient files, we recorded data on age, gender, laterality of surgery, smoking history and previous interventions. For outcome analysis, we used a qualitative scoring system with subdivision into three categories (good (MRD‐1 of 4–5 mm, smooth upper eyelid contour and left‐right difference of < 1 mm); acceptable (MRD‐1 of 3–<4 or> 5–6 mm), smooth upper eyelid contour and left‐right difference of < 2 mm; poor: if none of the above criteria was met). We analysed the outcome per eyelid as well as per patient. Results Of the 305 patients (471 eyelids), 166 underwent bilateral and 139 unilateral surgery. Regarding eyelids, the outcome of surgery was good in 71.6% (337/471) acceptable in 15.7% (74/471) and poor in 12.7% (60/471). Regarding patients, the outcome was good in 64.3% (196/305), acceptable in 15.7% (48/305) and poor in 20% (61/305). Concerning bilateral and unilateral surgery, the outcome was good in 60.8% (101/166) and acceptable in 16.9% (28/166) of patients after bilateral surgery, and good in 68.3% (95/139) and acceptable in 14.4% (20/139) of the patients after unilateral surgery (p = 0.17). Reoperation was performed in 16% (75 of 471) of eyelids and in 22 % (66 of 305) of patients. After secondary surgery, the cumulative success percentage was good in 78.6% (370/471) of eyelids and in 79% (241/305) of patients. We found no relation between surgical outcome and any other studied parameter, such as age, gender, smoking history and previous intervention such as extraocular muscle surgery and/or orbital decompression. Conclusion Correction of upper eyelid retraction yields a good result in 64.3% of patients and 71.6% of eyelids. With one additional procedure, the procedure proved was successful in 79% of patients and eyelids. We found no relation between the outcome of surgery and any other parameter, such as previous disease severity, previous interventions or smoking history. For comparative analysis, we recommend to report the outcome per patient rather than per eyelid.
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