Department of Radiation Oncology, Georgetown University Hospital , Washington, DC , USA.
Department of Radiology, Georgetown University Hospital , Washington, DC , USA.
Front Oncol. 2014 Mar 14;4:44. doi: 10.3389/fonc.2014.00044. eCollection 2014.
Inverted papilloma (IP) of the nasal cavity is a benign tumor that represents 0.5-4% of all nasal tumors and have been known to rarely undergo malignant transformation to squamous carcinoma and even more rarely adenocarcinoma. Synchronous association with low-grade esthesioneuroblastoma (ENB) has been reported in only one case report where a small-sized lesion was treated with surgery alone. Here we report the first case of invasion of IP by high-grade ENB with nodal metastasis that was treated with combined modality therapy.
A case of a 64-year-old African American gentleman presented to the otolaryngology with a 3-month history of recurrent epistaxis. Imaging revealed a large right nasal cavity mass extending into the right sphenoid sinus but without intracranial extension. Surgical pathology revealed high-grade ENB invading IP. An orbitofrontal craniotomy approach was used to achieve complete resection of the mass but with positive margins. Post-operative positron emission tomography/computed tomography showed nodal metastasis. The patient was then treated with adjuvant chemoradiation and remains without evidence of disease at 42 months post-treatment. We discuss the disease presentation, histopathologic features, and disease management with literature support.
In this very rare disease presentation where two extremely rare malignancies collide, we show that aggressive management with trimodality therapy of surgery, adjuvant radiation with stereotactic radiosurgical boost, and adjuvant chemotherapy gives excellent results. Given the natural history of the disease, however, long follow-up is needed to declare complete freedom from the disease.
鼻腔内翻性乳头状瘤（IP）是一种良性肿瘤，占所有鼻腔肿瘤的 0.5-4%，已知其很少发生恶性转化为鳞状细胞癌，甚至更罕见的是腺癌。仅在一例报告中报道了与低级别嗅神经母细胞瘤（ENB）的同步关联，其中一个小病变仅通过手术治疗。在这里，我们报告了首例由高级别 ENB 侵犯 IP 并伴有淋巴结转移的病例，该病例采用联合治疗。
一名 64 岁非裔美国男性因反复发作性鼻出血 3 个月就诊耳鼻喉科。影像学显示右侧鼻腔有一个大肿块，延伸至右侧蝶窦，但没有颅内延伸。手术病理显示高级别 ENB 侵犯 IP。采用眶额额骨开颅术实现肿块的完全切除，但边缘阳性。术后正电子发射断层扫描/计算机断层扫描显示淋巴结转移。然后，患者接受了辅助放化疗，在治疗后 42 个月仍无疾病迹象。我们结合文献支持讨论了疾病表现、组织病理学特征和疾病管理。
在这种非常罕见的疾病表现中，两种极为罕见的恶性肿瘤同时发生，我们表明通过手术、辅助放疗加立体定向放射外科加量和辅助化疗的三联疗法进行积极治疗可获得良好的结果。然而，鉴于疾病的自然史，需要长期随访才能宣布完全无病。
