Impact of perioperative chemotherapy on survival in patients with cholangiocarcinoma undergoing curative resection.

BACKGROUND: Most patients with localized cholangiocarcinoma (CCA) endure cancer relapse after curative resection underscoring the importance of systemic therapy. The current study attempts to determine the impact of perioperative chemotherapy (PC) on survival in patients with CCA undergoing resection. METHODS: Patients diagnosed with CCA undergoing curative-intent resection between January 1, 2000, and December 31, 2019, in a tertiary care center were included. Cox proportional hazard modeling was used to determine the impact of PC on disease-free survival (DFS) and overall survival (OS). In addition, a nomogram was constructed to estimate 3-year DFS. RESULTS: Among the 182 patients included in the analysis, 102 underwent surgery alone, and 80 received surgery plus PC. Forty-two patients received neoadjuvant therapy, and 38 patients received adjuvant therapy. On multivariate analysis, PC was significantly associated with an improved DFS (HR, 95% CI: 0.63, 0.41-0.98; p = 0.04) and OS (HR, 95% CI: 0.46, 0.27-0.78; p < 0.01). In the interaction analysis, the survival benefit was especially seen in patients with positive resection margins and tumor size > 5 cm. CONCLUSION: In patients with CCA undergoing curative resection, receipt of PC was associated with improved DFS and OS. The nomogram constructed from this database provides an estimate of 3-year DFS after surgical resection. Randomized trials are needed to define the optimal regimen and sequence.

Epstein-Barr Virus Hepatitis Masquerading as Painless Jaundice.

Epstein-Barr virus (EBV) infection typically presents with pharyngeal symptoms and subclinical transaminitis. We present a case of a 27-year-old woman with no known past medical history who presented with painless jaundice and dark-colored urine for three days. Her review of systems was negative for fever, sore throat, nausea, vomiting, pruritus, or rash. Her last sexual contact was six months ago with a male partner, and she only drank alcohol socially. Family and surgical history were non-significant. Physical examination revealed 3+ bilateral conjunctival icterus without abdominal tenderness or organomegaly. She had elevated transaminases: alanine transaminase (ALT) of 1287U/L and aspartate aminotransferase of (AST) 1057U/L but her alkaline phosphatase (ALP) was only slightly above normal at 109U/L (normal range 35-104U/L), with a direct hyperbilirubinemia - total bilirubin 9.5mg/dl, direct bilirubin 6.8mg/dl; the abdominal ultrasound revealed non-dilated bile ducts. Hepatitis A, B, and C serology was negative, but her EBV serology showed an infection. She had incidental thalassemia minor without splenomegaly or asterixis. She was managed conservatively, and her liver enzymes trended down with supportive management. Although EBV is an uncommon cause of painless jaundice, this diagnosis should be considered, especially when other more common causes of jaundice have been ruled out. A high index of suspicion should be maintained to detect EBV hepatitis as it can easily be diagnosed through serological testing.

RESPONSE OF UVEAL METASTASES TO ALK INHIBITORS IN ALK-POSITIVE NON-SMALL-CELL LUNG CANCER.

PURPOSE: To present a case of uveal metastases in a patient with anaplastic lymphoma kinase (ALK)-positive non-small-cell lung cancer and the response to systemic ALK inhibitors. METHODS: A retrospective case report. A 75-year-old nonsmoker who has ALK-positive left upper bronchus adenocarcinoma-developed uveal metastases during his course of treatment. RESULTS: Initially, the patient's disseminated malignancy showed a significant response to crizotinib. However, because the bone metastases started progressing again and he developed bilateral ocular metastases, he was switched to ceritinib. After initiation of ceritinib therapy, the uveal melanomas have shown a significant decrease in thickness and no new lesions have developed. CONCLUSION: ALK inhibitors are an effective first-line treatment in patients with ALK-positive uveal metastases secondary to ALK-positive non-small-cell lung cancer. Despite the fact that crizotinib, a first-generation ALK inhibitor, is initially effective in dealing with non-small-cell lung cancer and its metastases, resistance to it seems to develop on a regular basis. A second-generation ALK inhibitor, such as ceritinib, is also effective in overcoming this resistance in treating those with ALK-positive uveal metastases.

UVEAL MELANOMA EXTENSION TO THE OPTIC CHIASM.

PURPOSE: Case report describing a patient who developed intracranial extension of a uveal melanoma through the optic nerve. METHODS: We reviewed the patient's medical history and images. A 41-year-old woman who was blind in one eye had a uveal melanoma that extended through the optic nerve into the optic chiasm and involved the hypophysis. The patient then developed metastasis. RESULTS: The patient developed uveal melanoma extension into the optic chiasm through the optic nerve resulting in a visual field defect in the fellow eye. CONCLUSION: Uveal melanoma extension through the optic nerve is a devastating complication, which occurs anywhere from 0.6% to 3.7% in patients with uveal melanoma. If enucleation of the affected eye is performed, a representative portion of the optic nerve should be excised to decrease the risk of extension. Patients with phthisical eyes should undergo appropriate imaging techniques to prevent a missed diagnosis of optic nerve involvement.

Choroidal Involvement in Erdheim-Chester Disease.

Erdheim-Chester disease is a rare infiltrative disorder of non-Langerhans cell histiocytes, which affects multiple organs, including bone, heart, lungs, orbits, central nervous system (CNS), pituitary gland, and retroperitoneum. Bone affliction is almost universal in these patients and, to a lesser degree, the CNS, retroperitoneum, and other tissues. Eye involvement is typically retro-orbital or periorbital. This case report describes a 48-year-old patient who presented with reduced vision in the left eye and no other complaints. Fundus examination revealed a choroidal mass in the macula. The authors believe that this might be the second case of choroidal involvement in Erdheim-Chester disease.

Indocyanine green angiographic evidence of choroiditis in scleroderma.

PURPOSE: The purpose of this case report was to demonstrate evidence of indocyanine green angiography leakage consistent with choroiditis in a patient with scleroderma. METHODS: In this case report, the patient underwent a variety of tests and examinations, including systemic evaluation, full ocular examination, skin biopsy, indocyanine green, and fluorescein angiography testing. A 52-year-old man had blurred vision centrally in both eyes. Vision was 20/25 and 20/20. Posterior examination showed cotton-wool spots in both eyes. The patient met European League against Rheumatism (EULAR) criteria for scleroderma. RESULTS: Fluorescein angiography confirmed the presence of leakage from the retinal vessels. More importantly, indocyanine green angiography revealed choroidal vessel leakage in both eyes. This provided evidence of choroiditis before vessel obliteration. Previous studies have shown evidence of choriocapillaris obliteration. Choroidal vessel leakage, however, has not been reported in patients with scleroderma. CONCLUSION: The results of this case demonstrate the usefulness of indocyanine green angiography in detecting the presence of choroiditis in scleroderma.