Two patients, with non-small cell lung carcinoma treated with pembrolizumab, developed bilateral diffuse uveal melanocytic proliferation (BDUMP) with interesting histopathological features. The first patient developed a right ciliary body mass concurrently with BDUMP. The globe was enucleated. The ciliary body mass was a mitotically active epithelioid uveal melanoma, invading the trabecular meshwork and peripheral corneal stroma, with over 90% of the cells expressing Cyclin D1 protein. The melanoma showed no chromosome 3 or 8 changes. The background uvea showed diffuse, bland spindle cell melanocytic proliferation with much lower Cyclin D1 expression (around 10%). In the choroid, this population was punctuated by islands of pigmented epithelioid cells, some of which were necrotic. All these islands expressed a high level of Cyclin D1, and some islands expressed nuclear preferentially expressed antigen in melanoma (PRAME). The ciliary body mass, epithelioid cell islands, and the BDUMP all expressed c-Met (the receptor for hepatocyte growth factor [HGF]). The features were those of ciliary body melanoma and choroidal melanoma “tumorlets,” developing on a background of BDUMP. The second patient developed bilateral periocular skin pigmentation following a diagnosis of BDUMP, which when biopsied, showed dermal islands of paraneoplastic perivascular melanocytic cell proliferation. These cells also expressed c-Met protein. These observations implicate the HGF/c-Met axis in the pathogenesis of BDUMP, the uveal melanomas in the ciliary body and choroid in the first patient and the paraneoplastic dermal melanocytic proliferation in the second patient.
<b><i>Purpose:</i></b> To describe our experience in performing biopsy of post-septal orbital masses with core needle under computerized tomography guidance (CT-CNB). <b><i>Methods:</i></b> The medical records of all patients who underwent this procedure were reviewed. The procedure was performed under local anesthesia on a day case basis under a peribulbar block. A planning non-contrast computerized tomography (CT) scan of the orbits was performed to localise the mass. A 6-cm 18-G Temno Evolution® semi-automated biopsy needle was inserted through the skin into the orbit. Prior to further advancement of the needle, a low-dose CT limited to the previously determined plane was performed to confirm its position. The needle was then advanced, and the cutting needle was deployed to obtain the biopsy. <b><i>Results:</i></b> Five patients who underwent CT-CNB were identified. The CNB was successful in 4 patients and revealed a metastatic prostate adenocarcinoma, diffuse large B-cell lymphoma, a metastatic neuroendocrine tumour, and orbital inflammatory disease. The biopsy failed in the fifth patient when the needle failed to penetrate the tumour despite good localisation on CT. He was eventually diagnosed with fibrous meningioma of the greater wing of sphenoid on open biopsy. None of the patients had any complications other than peri-ocular bruising which was present in all of them. <b><i>Conclusion:</i></b> CT-CNB of mass lesions located in the lateral aspect of the orbit can be an alternative to open biopsy in selected cases. It avoids major surgery and allows the use of radiotherapy, if required, without any delay.
Purpose: To describe the clinical and histopathological features of a conjunctival melanoma (CM) during early pregnancy. Procedures: A 37-year-old, 20-week pregnant primigravida was referred to the Sheffield Ocular Oncology Service with a rapidly growing lesion arising from the right superior conjunctival fornix, noted from the first trimester of pregnancy. This was associated with pain and bloody discharge. Incisional biopsy confirmed the clinical suspicion of invasive CM. She was treated by primary surgical excision and cryotherapy under local anaesthesia. Results: Histology of the excised specimen showed an invasive malignant melanoma with surrounding in situ conjunctival changes arising from a naevus. The melanoma was 10.5 mm thick, focally necrotic, and had a mitotic count of 11/mm2 focally. The patient responded well to surgical treatment. She gave birth to a healthy boy, and the placenta showed no evidence of metastatic melanoma. There has been no recurrence or distant metastasis during 5 years of follow-up. Conclusion: CM during pregnancy is extremely rare. Because of possible transformation to malignant melanoma, we recommend close monitoring of females known to have pigmented conjunctival lesions of the conjunctiva during pregnancy.
<b><i>Introduction:</i></b> The aim of the study was to report our experience in the use of Gamma Knife Stereotactic Radiosurgery (GKSTRS) for conjunctival squamous cell carcinoma (SCC) invading the orbit, as an alternative to exenteration surgery. <b><i>Patients and Methods:</i></b> Patients who had GKSTRS for conjunctival SCC invading the orbit but sparing the bone (AJCC eighth ed. T4a) with a minimum of 1-year follow-up were included. Treatment failure was defined as no decrease in tumour size 3 months post-treatment, or further growth during the follow-up period. Patients were followed up 3-monthly for 2 years and 6-monthly afterwards with a minimum of yearly MR Imaging of orbit. <b><i>Results:</i></b> Six patients met the inclusion criteria. MR imaging was used to identify the extent of orbital involvement of SCC. Stereotactic radiosurgery utilizing the Leksell Gamma Knife® Perfexion was delivered in a single session in which patients received 18–20 Gy to the 45–50% isodose. The median follow-up was 29 months. Four patients responded to the treatment and had no evidence of recurrence at their most recent follow-up. The treatment failed in 2 patients, in 1 of whom the tumour was larger and extended deeper into the orbit. The other patient developed a recurrence away from the treated area at 9 months, suggesting a multifocal disease. Both patients had poorly differentiated SCC. <b><i>Conclusion:</i></b> GKSTRS is a potential alternative to orbital exenteration surgery for conjunctival SCC extending into the anterior orbit in a selected group of patients refusing orbital exenteration or has a second blind eye. Nevertheless, the disease is more likely to recur than with exenteration surgery, hence lifelong monitoring and low threshold for surgical intervention or retreatment is prudent.
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