Nasal Duplication: A Review of Literature and Case Report

Introduction: Nasal duplication is a rare congenital deformity with many subtypes including supernumerary nostril. The challenge of surgical correction is to achieve nasal symmetry and restore nasal airflow. However, there is no defined protocol for treatment, especially with regard to presurgical therapy. Methods: We performed a review of literature of studies reporting on patients with supernumerary nostril to complete this review. We then report on a patient with supernumerary nostril who was treated with nostril expansion therapy prior to surgical intervention. Results: We identified 59 cases of nostril duplication. Because of the rarity of the condition, treatment protocols varied greatly. For our patient, preoperative nasal appliance therapy was implemented for 3 months prior to surgical intervention. Patient was followed-up regularly for 1 year. Discussion: Although literature on nasal duplication is scarce, there is a general agreement that early intervention has psychological, anatomic, and functional benefits to the patient. In our case report, nostril expansion therapy was easy to implement and facilitated surgical reconstruction, resulting in aesthetic outcome and expanded airway 1 year postoperatively.

Randomized phase II trial of presurgical androgen deprivation therapy (ADT) with or without axitinib in prostate cancer (PCa) presenting with lymph node (LN) metastasis.

5078 Background: Strategies integrating androgen targeted and locoregional therapies are being increasingly used in PCa with regional spread, but are rarely curative. Angiogenesis inhibitors delay progression in castration resistant PCa and may synergize with ADT through endothelial cell apoptosis in hormone naïve patients (pts). We hypothesized that the frontline combination of ADT with the potent VEGF inhibitor Axi would improve PCa control relative to ADT alone and allow for meaningful time off systemic therapy after surgical consolidation. Methods: Pts with either clinically detected LN+ (TxN1M0 or TxNxM1a) or very high risk for LN PCa were treated with ADT for 2 mos and then randomized 2:1 to respectively add open label Axi (5 mg PO bid) vs. continue ADT alone for 4 mos until surgery. Those responding with PSA ≤5 ng/mL were offered prostatectomy and extended pelvic lymphadenectomy (RP). ADT +/- Axi was withheld postoperatively and PSA measured q3 mos until 1 y. Primary objective: proportion of pts progression free (FFP) 12 mos after RP, defined as PSA ≤1.0 ng/mL and no radiation or ADT, aiming to detect a 35% difference favoring ADT+Axi. Results: 72 pts completed accrual. We report on the 54 pts with LN+ disease: median age 62 y (range 42-76), pretreatment PSA 22.9 ng/mL (range 3.6 - 404.4), 38 N1 / 16 M1a. Table shows presurgical therapy outcomes. Path responses in the prostate were similar between arms, but in 5 ADT+Axi vs. 0 ADT LN+ pts there was no residual nodal disease. Testosterone recovery: 24/26 ADT+Axi and 9/9 ADT RP pts by 6 mos. 21/36 ADT+Axi and 15/18 ADT pts have failed; 1 y FFP estimates 48.0% (SE 8.6%) and 16.7% (SE 8.3%), respectively (p = 0.02). 1 y undetectable PSA 9 pts (6 ADT+Axi). No grade 4 toxicities or unexpected side-effects were observed. Conclusions: 1 year after RP, ADT+Axi resulted in proportionally greater number of LN+ PCa pts off treatment and progression free than ADT alone. Tissue analysis is evaluating predictors of benefit to further develop angiogenesis inhibition as part of combination strategies for hormone naïve PCa. Clinical trial information: NCT01409200. [Table: see text]

The impact of contrast enhancement reduction on tumor response in patients with advanced renal cell carcinoma that treated with presurgical targeting therapy.

510 Background: RECIST is a standard tool for tumor response assessment. However, evaluation for targeted therapy by RECIST is insensitive because tumors often showed a modest change in size despite of significant necrosis. Therefore, we investigated an impact of contrast enhancement reduction for tumor shrinkage in RCC patients with presurgical targeting therapy. Methods: From March 2011 to July 2015, 17 patients (18 tumors) with RCC who underwent presurgical targeting therapy were included. Indications for presurgical targeting therapy were cT3-4, bilateral tumors, poor performance status, or small distant metastasis. Contrast enhancement reduction was evaluated by contrast medium enhanced area (CMEA) before and after targeting therapy. CMEA measurements were performed by OsiriX and Adobe Photoshop. Eighteen tumors were evaluated by RECIST and CMEA. Differences between two methods were compared. Results: Median age and followup after initiation of targeted therapy were 68 years old and 15.3 months. IVC thrombus and small distant metastasis existed in seven (41%) and five (29%) patents, respectively. Fifteen patients (88%) received TKI based (sunitinib and/or axitinib), and three patients (12%) received mTOR inhibitor based (everolimus or temsirolimus) presurgical therapy. The median treatment period of presurgical targeted therapy was 3.3 months. Median tumor responses in RECIST and CMEA were 10.5% and 49.0%, that was significantly correlated by liner regression analysis (P = 0.002). TKI based presurgical therapy showed significant tumor reduction in CMEA than RECIST (P = 0.0062). In addition, tumor reduction rate was significantly higher in TKI based presurgical therapy than mTOR inhibitor (P = 0.014). No patient experienced tumor recurrence. Conclusions: Contrast enhancement reduction might be useful tool for tumor shrinkage in patients with RCC who underwent presurgical targeting therapy.