Adrenocortical carcinoma is a rare cancer, with estimate population incidence of 0.7-2.0 cases per 1 million each year. It also carries poor prognosis with estimated 5-year survival of less than 15% of those with metastatic disease and has a poor response to cytotoxic treatment. A randomized controlled trial published in 2012 by Fassnacht et al. demonstrated improved progression-free survival with first-line etoposide-doxirubicin-cisplatin-mitotane (EDP-M) compared to first-line streptozocin-mitotane in patients with stage III-IV disease. We report a case of a 25-year-old female diagnosed with adrenocortical carcinoma with liver and lung metastases treated with adjuvant EDP-M chemotherapy. During her treatment, the patient experienced ongoing significant liver-associated burden of disease, which prompted a trial of trans-hepatic arterial chemoembolization with doxorubicin and mitomycin. The patient subsequently experienced complete remission of disease at 18 months with no fludeoxyglucose (FDG) avid lesions on PET/CT.
Purpose
Endoscopic biopsy is recommended for diagnosis of nasopharyngeal carcinoma (NPC). A proportion of lesions are hidden from endoscopic view but detected with magnetic resonance imaging (MRI). This systematic review and meta-analysis investigated the diagnostic performance of MRI for detection of NPC.
Methods
An electronic search of twelve databases and registries was performed. Studies were included if they compared the diagnostic accuracy of MRI to a reference standard (histopathology) in patients suspected of having NPC. The primary outcome was accuracy for detection of NPC. Random-effects models were used to pool outcomes for sensitivity, specificity, and positive and negative likelihood ratio (LR). Bias and applicability were assessed using the modified QUADAS-2 tool.
Results
Nine studies were included involving 1736 patients of whom 337 were diagnosed with NPC. MRI demonstrated a pooled sensitivity of 98.1% (95% CI 95.2–99.3%), specificity of 91.7% (95% CI 88.3–94.2%), negative LR of 0.02 (95% CI 0.01–0.05), and positive LR of 11.9 (95% CI 8.35–16.81) for detection of NPC. Most studies were performed in regions where NPC is endemic, and there was a risk of selection bias due to inclusion of retrospective studies and one case–control study. There was limited reporting of study randomization strategy.
Conclusion
This study demonstrates that MRI has a high pooled sensitivity, specificity, and negative predictive value for detection of NPC. MRI may be useful for lesion detection prior to endoscopic biopsy and aid the decision to avoid biopsy in patients with a low post-test probability of disease.
Background Congenital portal vein aneurysm is a rare vascular anomaly with poorly understood natural history. Whereas asymptomatic aneurysms are often managed conservatively, surgery has been used in symptomatic cases complicated by thrombosis or rupture. Surgical experience in management of portal aneurysms is restricted to case studies with limited comparative data and inconsistent reporting of outcomes. A hybrid open and endovascular approach has rarely been described in the literature. Methods We present a case of an extrahepatic portal aneurysm which demonstrated changes on surveillance imaging concerning for early asymptomatic thrombosis. Acute thrombus was identified at the time of open aneurysm repair. We review the limited literature regarding management of portal vein aneurysms in non-cirrhotic patients. Results Our case was complicated by intrahepatic thrombo-embolism, which necessitated hybrid thrombectomy and anticoagulant therapy. The patient remains asymptomatic at three-year follow-up with no recurrent aneurysm or thrombosis on surveillance Doppler and CT imaging. Conclusions Altered hemodynamic appearances on Doppler ultrasound and contrast-enhanced CT may warn of impending thrombosis in portal vein aneurysms. Hybrid open and endovascular surgical repair ensures vessel patency and a durable surgical result.
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