Kakkar Aanchal, Rajeshwari Madhu, Sakthivel Pirabu, Sharma Mehar C, Sharma Suresh C
Department of Pathology, All India Institute of Medical Sciences, New Delhi 110029, India.
Department of Otolaryngology and Head and Neck Surgery, All India Institute of Medical Sciences, New Delhi 110029, India.
Ann Diagn Pathol. 2018 Apr;33:6-10. doi: 10.1016/j.anndiagpath.2017.11.005. Epub 2017 Nov 8.
Biphenotypic sinonasal sarcoma (BSNS) is a recently described mesenchymal tumor exclusive to the sinonasal region. It is a low grade sarcoma, displaying evidence of myogenic and neural differentiation. Role of β-catenin immunohistochemistry in distinguishing it from its morphological mimics is not well-established. We conducted this study to identify cases of BSNS from our archives, and to examine immunopositivity for β-catenin in them as well as in its close differential diagnosis.
All cases of nasal cavity and paranasal sinus mesenchymal neoplasms were identified. Histopathological features were reviewed. Cases showing smooth muscle actin (SMA) and S-100 immunopositivity, and typical morphology were reclassified as BSNS. β-catenin immunoexpression was assessed.
Twenty-one mesenchymal tumors, including 12 sinonasal hemangiopericytoma (SNHPC), five solitary fibrous tumors (SFT), three BSNS, and one synovial sarcoma were identified. Three SNHPC cases were reclassified as BSNS. BSNS patients included one male and five females, with mean age of 51years. Five BSNS cases (83.3%) showed nuclear β-catenin immunopositivity. SNHPC cases also were β-catenin positive (60%).
BSNS is a rare sinonasal neoplasm, frequently misdiagnosed as SNHPC and SFT. β-catenin immunopositivity is seen in majority of cases, indicating a role in pathogenesis. However, due to positivity in other tumors like SNHPC, it has limited role in differential diagnosis.
双表型鼻窦肉瘤(BSNS)是一种最近描述的仅发生于鼻窦区域的间叶性肿瘤。它是一种低级别肉瘤,具有肌源性和神经分化的证据。β-连环蛋白免疫组化在将其与形态学相似的肿瘤区分开来中的作用尚未明确。我们进行这项研究以从我们的存档病例中识别出BSNS病例,并检查其以及与之密切相关的鉴别诊断病例中β-连环蛋白的免疫阳性情况。
识别出所有鼻腔和鼻窦间叶性肿瘤病例。回顾组织病理学特征。将显示平滑肌肌动蛋白(SMA)和S-100免疫阳性且具有典型形态的病例重新分类为BSNS。评估β-连环蛋白的免疫表达。
共识别出21例间叶性肿瘤,包括12例鼻窦血管外皮细胞瘤(SNHPC)、5例孤立性纤维瘤(SFT)、3例BSNS和1例滑膜肉瘤。3例SNHPC病例被重新分类为BSNS。BSNS患者包括1名男性和5名女性,平均年龄51岁。5例BSNS病例(83.3%)显示核β-连环蛋白免疫阳性。SNHPC病例也呈β-连环蛋白阳性(60%)。
BSNS是一种罕见的鼻窦肿瘤,常被误诊为SNHPC和SFT。大多数病例可见β-连环蛋白免疫阳性,表明其在发病机制中起作用。然而,由于在SNHPC等其他肿瘤中也呈阳性,其在鉴别诊断中的作用有限。