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Low-grade papillary schneiderian carcinoma, a unique and deceptively bland malignant neoplasm: report of a case.

低级别乳头状Schneiderian癌,一种独特的具有欺骗性温和表现的恶性肿瘤:附一例报告

Lewis JS,Chernock RD,Haynes W,El-Mofty SK

Abstract

The sinonasal tract harbors several different types of papillomas, some of which can progress to carcinoma. The most frequent among these are inverted and oncocytic Schneiderian papillomas. The rates of progression are somewhat controversial but are approximately 5% to 10% and are almost invariably described in the literature as in situ or invasive squamous cell carcinoma. Other carcinoma types, such as mucoepidermoid and sinonasal undifferentiated carcinoma, have also been described. Almost all of the described patterns of malignancy involve frank carcinoma with overtly dysplastic nuclear features, lack of cell maturation, and increased mitotic activity. Some squamous cell carcinomas, particularly nonkeratinizing, can grow in a papillary pattern, appearing to only line the surface epithelium, but they are cytologically overtly malignant throughout. In this case report, however, we describe a novel, human papillomavirus-negative, papillary carcinoma, which presented as a left nasal and maxillary sinus exophytic and inverted-appearing, papillomatous mass with very bland cytomorphology. The initial features were not typical for any defined Schneiderian papilloma but were also not clearly diagnostic of papillary carcinoma. The tumor recurred >10 times over 18 years despite extensive surgical resection including orbital exenteration. The tumor retained a bland appearance throughout the patient's entire clinical course, but did develop a pushing pattern of stromal invasion, increased mitotic activity, vesicular nuclei with prominent nucleoli, lymph node metastases, and eventually overwhelming local recurrence and nodal metastases, resulting in death. This tumor seems best characterized as a low-grade papillary Schneiderian carcinoma and appears to represent a novel type of sinonasal carcinoma.

摘要

鼻窦可发生几种不同类型的乳头状瘤,其中一些可以进展为癌。其中最常见的是内翻性乳头状瘤和嗜酸细胞性Schneiderian乳头状瘤。进展的发生率虽有争议,但大致为5%至10%,文献中几乎都描述进展为原位或浸润性鳞状细胞癌。其他类型的癌,如粘液表皮样癌和鼻窦未分化癌,也有所报道。几乎所有报道恶变模式都包括显著的核异型特征、缺乏细胞成熟和分裂活性增加。有些鳞状细胞癌,特别是非角化性,可以有乳头状生长方式,仅在上皮表面生长,但细胞学上也具有显著恶性特征。本病例报告中,我们描述了一种独特的、人类乳头状瘤病毒阴性的乳头状癌,表现为左鼻腔和上颌窦外生性和内翻性乳头瘤样肿块,伴有温和的细胞形态。初始特征不是任何明确的Schneiderian乳头状瘤的典型表现,也不能明确诊断为乳头状癌。尽管采取了广泛手术切除包括眶内容摘除术,肿瘤在18年内复发> 10次。患者整个临床过程中,肿瘤保持了温和的形态,但进展有推挤性间质浸润、核分裂活性增加、出现空泡状细胞核和显著核仁、淋巴结转移,并且最终出现势不可挡的局部复发和淋巴结转移,导致死亡。这种肿瘤最好诊断为低级别乳头状Schneiderian癌,似乎代表了鼻腔鼻窦癌一种新的类型。
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