Background Juvenile nasopharyngeal angiofibroma (JNA) is a rare but potentially lifethreatening fibrovascular tumor that is seen almost exclusively in adolescent males and usually presents with symptoms of nasal obstruction or severe epistaxis. The current gold standard of treatment consists of complete surgical resection; however, this is inherently challenging because of the tumor's invasive nature and a substantial risk of intraoperative hemorrhage. Flutamide, an anti-androgen antineoplastic agent, has been used preoperatively in attempts to reduce tumor volume allowing for surgical resection with more conservative procedural techniques and reduce intraoperative blood loss.Methods A literature review of PubMed and CINAHL was used to identify and analyze 29 male patients with JNA to determine the efficacy of the preoperative use of flutamide.
ResultsOur analyses indicate that flutamide may be effective as a neoadjuvant agent by reducing tumor volume prior to resection in some patients but seemed to be more effective in the early stages of JNA without advanced tumor invasion. However, individual tumor response to flutamide was variable. Additionally, postpubertal patients seemed to demonstrate a greater reduction in tumor volume with flutamide compared to their prepubertal counterparts. Dosing regimen and side effects associated with flutamide therapy are also discussed.
ConclusionFlutamide may be an effective neoadjuvant therapy in some cases of juvenile nasopharyngeal angiofibroma, but larger scale, case-control studies are likely needed to further expand on this conclusion. Postpubertal males with early-stage disease seemed to be the population that may benefit most from this treatment protocol.
Invasive atypical fibroxanthomas and undifferentiated pleomorphic sarcomas of the scalp are relatively rare spindle cell tumors that recur at high rates. Wide local excision and Mohs surgery alone are not feasible for the definitive management of lesions adhering to the underlying pericranium and/or calvarium. This brief clinical study presents 2 patient cases and includes a systematic review of the literature. In our literature review, 2 of 4 patients treated with outer table resection had no disease recurrence, 1 died due to an unrelated cause, and 1 died due to disease progression. Three of 9 patients treated with full-thickness craniectomy had no disease recurrence, 5 patient outcomes were not specified, and 1 died from disease progression. There are currently no recommendations for the management of these invasive scalp lesions. We believe invasive atypical fibroxanthomas/undifferentiated pleomorphic sarcomas of the scalp without frank calvarial involvement can be effectively treated with outer table resection.
SUMMARY
This article describes a unique approach for reconstruction of large helical rim defects. By raising bilateral chondrocutaneous flaps of the helical rim while including a semicircular chondrocutaneous excision of the adjacent scapha and antihelix, large defects can be repaired with minimal loss to the overall external ear circumference. This is a technically simple and reliable method that has resulted in excellent cosmetic outcomes and minimal morbidity in our practice.
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