A phase II trial of all-trans retinoic acid (ATRA) in advanced adenoid cystic carcinoma.

BACKGROUND: Effective therapies are lacking for recurrent, metastatic adenoid cystic carcinoma (R/M ACC) and preclinical models suggest retinoic acid agonists inhibit ACC growth. This phase II trial evaluated all-trans retinoic acid (ATRA) as a novel therapy for ACC. METHODS: Patients with R/M ACC (any site) with clinical and/or radiographic progression ≤12 months prior to study entry were eligible. Cohort 1 (CH1) received ATRA 45 mg/m2 split oral daily dosing on days 1-14 of a 28-day cycle; Cohort 2 (CH2) received the same dosing continuously. Primary endpoint was best overall response rate (CR + PR) (RECIST v1.1). Secondary endpoints: safety and progression-free survival (PFS). Exploratory analyses: ATRA impact on MYB expression and genomic predictors of response. RESULTS: Eighteen patients enrolled. There were no responses, but 61% (11/18) had stable disease (SD) and 28% (5/18) progression as best response; 11% (2/18) unevaluable. Median duration of stability: 3.7 months (95%CI, 1.9-3.9). One patient (CH1) remains on drug with SD approaching 1 year. Half of those who received prior VEGFR therapy achieved SD (4/8). At median follow up of 7.9 months, median PFS was 3.2 months (95%CI, 1.8-3.9). N = 1 required dose adjustment; N = 1 came off drug for toxicity. There were no grade 3-4 adverse events. NOTCH1 and PI3K pathway alterations were most frequent. Low MYB protein expression was associated with longer duration of stability on ATRA (P < 0.01). CONCLUSION(S): While the trial did not meet its prespecified response endpoint, ATRA alone or in combination may be a low toxicity treatment for disease growth stabilization in R/M ACC.

Massive Hemorrhage From Multiple Hepatic Artery Aneurysms.

A 66-year-old man, with an abnormal porta hepatis, consistent with tumor or inflammation, developed massive bleeding from one of numerous hepatic artery aneurysms, and coil embolization achieved control of bleeding. He died of subsequent multisystem organ failure, and the most likely diagnosis was either polyarteritis nodosa or segmental arterial mediolysis. Although the dual hepatic blood supply allows a degree of arterial embolization, this case demonstrates the risks associated with large territory hepatic arterial embolization in the presence of hemodynamic instability. We discuss the management issues related to massive hepatic bleeding when no surgical approach is possible.

Extravascular Revascularization of a Chronic Total Occlusion Using the Dual Bull's-Eye Technique.

PURPOSE: To describe the dual bull's-eye technique to achieve successful extravascular revascularization of an occlusion after vessel perforation when the antegrade and retrograde catheters are too far apart for salvage with a reentry catheter. TECHNIQUE: The dual bull's-eye technique, which is an adaptation of the "gun-sight" method for transjugular portosystemic shunt procedures, is demonstrated in a popliteal artery occlusion in which multiple recanalization attempts have produced vessel perforation. Via antegrade femoral and retrograde anterior tibial artery accesses, 10-mm Amplatz GooseNeck snares were advanced in plane from each access to rendezvous in the popliteal fossa to obtain through-and-through wire access. A 15-cm Chiba needle was advanced percutaneously in plane through both snares, and a hydrophilic guidewire was maneuvered across an extravascular tract parallel to the popliteal artery. The tract was carefully predilated, and a 5×100-mm Viabahn stent-graft was deployed across the extravascular tract. CONCLUSION: Owing to potential complications (limited patency, injury to adjacent structures) and only single-case use, this new technique should be considered a bailout strategy exclusively employed for limb salvage when traditional methods have failed and there are no viable surgical options.

In vivo construction of a multilayer bare-metal stent for the treatment of an aortic mycotic pseudoaneurysm.

Although not currently available in the United States, multilayer stents have been used successfully to treat a variety of aneurysms. These stents promote laminar flow and depressurize the aneurysmal portion of the vessel, while preserving side branch vessel flow. A conceivable benefit of the multilayer stent is in the treatment of infected pseudoaneurysms, given the absence of a fabric covering, a potential nidus for colonization. Here we present the case of a 64-year-old woman with symptomatic, enlarging infrarenal mycotic pseudoaneurysms who was successfully treated with an in vivo multilayer stent created by the layering of three concentric bare-metal Wallstents (Boston Scientific, Natick, Mass).

Case report: Avoidance of palpable corporal fibrosis due to priapism with upregulators of nitric oxide.

INTRODUCTION: Recent evidence suggests that blocking inducible nitric oxide (NO) synthase in the penis may exacerbate fibrotic processes and that application of medications known to increase NO in tissues may prevent fibrosis. AIM: To report the use of an antifibrotic regimen consisting of medications known to upregulate NO production in two patients with refractory priapism. METHODS: Two patients presented with priapism of greater than 48-hour duration. After corporal aspiration/irrigation and shunting procedures failed, both were prescribed a daily antifibrotic regimen comprising the phosphodiesterase inhibitors pentoxifylline and sildenafil, and the NO precursor, L-arginine. RESULTS: At 1 year, both patients were found to have supple corpora without evidence of corporal fibrosis. CONCLUSIONS: An antifibrotic regimen consisting of upregulators of NO production may ameliorate the corporal fibrosis associated with recalcitrant priapism.