Menon Santosh, Pai Prathamesh, Sengar Manju, Aggarwal J P, Kane Shubhada V
Department of Pathology, Tata Memorial Hospital, Parel, Mumbai 400 012, India.
Indian J Pathol Microbiol. 2010 Jan-Mar;53(1):28-34. doi: 10.4103/0377-4929.59179.
Primary sinonasal tumors with neuroendocrine differentiation (SCND) are uncommon tumors with considerable overlap of histological features. Based on their neuroendocrine differentiation they can be sub categorized into sinonasal undifferentiated carcinoma (SNUC), sinonasal neuroendocrine carcinoma (SNEC), esthesioneuroblastoma (ENB) and small cell carcinoma (SmCC). The natural history and biological behavior varies in this group of tumors. Hence the histo-morphological diagnosis coupled with grading/staging is important for the prognostication of these tumors. Aim : To study the clinicopathological characteristics of sinonasal neuroendocrine malignancies at our institute. Material and Methods : We searched our institute's pathology database for the period from 2002 to 2007, for the four subcategories of sinonasal tumors with neuroendocrine differentiation. Morphological and immunohistochemical features were studied and, grading, staging was done in accordance with standard criteria. The clinical treatment and follow- up data were retrieved from the case files in available cases. Results : A total of 37 cases were retrieved from our database which include 14 cases of SNUC, 14 cases of ENB and nine cases of SNEC. The cases of SNUC were immunopositive for cytokeratin, epithelial membrane antigen and weakly for neuron-specific enolase. SNEC showed strong reactivity with epithelial and neuroendocrine markers whereas ENB demonstrated immunoreactivity to synaptophysisn and chromogranin strongly, with weak to negative expression of epithelial markers. All cases of SNUC and SNEC were of high grade and stage whereas 50% of ENB cases were of grade II but high stage tumors. Most of the SNUC and SNEC patients had been treated with multimodality treatment regimens including upfront chemotherapy followed by surgery and loco- regional radiation. In contrast, ENB patients had undergone surgical extirpation followed by radiation therapy in majority of cases. With limited follow-up data, it was observed that four out of five SNUC patients and three out of four SNEC patients developed either loco-regional (three of SNUC and two of SNEC) or distant metastasis (one patient each of SNUC and SNEC). ENB patients also had loco-regional recurrences (five out of seven patients) with a more protracted course but no distant metastases were observed during the follow up in available cases. Conclusion : Sino nasal tumors with neuroendocrine differentiation are a heterogenous group of tumors with overlapping histo-morphological features. They can be distinguished based on immunohistochemical characteristics. Pathological sub categorization is imperative for management and prognostication of these aggressive tumors.
具有神经内分泌分化的原发性鼻窦肿瘤（SCND）是一类罕见肿瘤，其组织学特征有相当大的重叠。基于其神经内分泌分化，可将它们细分为鼻窦未分化癌（SNUC）、鼻窦神经内分泌癌（SNEC）、嗅神经母细胞瘤（ENB）和小细胞癌（SmCC）。这组肿瘤的自然病史和生物学行为各不相同。因此，组织形态学诊断以及分级/分期对于这些肿瘤的预后评估很重要。目的：研究我院鼻窦神经内分泌恶性肿瘤的临床病理特征。材料与方法：我们在我院病理数据库中检索了2002年至2007年期间具有神经内分泌分化的四类鼻窦肿瘤。研究了其形态学和免疫组化特征，并按照标准标准进行分级和分期。从现有病例的病历中检索临床治疗和随访数据。结果：从我们的数据库中总共检索到37例病例，其中包括14例SNUC、14例ENB和9例SNEC。SNUC病例对细胞角蛋白、上皮膜抗原免疫阳性，对神经元特异性烯醇化酶弱阳性。SNEC对上皮和神经内分泌标志物显示强反应性，而ENB对突触素和嗜铬粒蛋白免疫反应强烈，上皮标志物呈弱至阴性表达。所有SNUC和SNEC病例均为高级别和高分期，而50%的ENB病例为II级但为高分期肿瘤。大多数SNUC和SNEC患者接受了多模式治疗方案，包括 upfront化疗，随后进行手术和局部区域放疗。相比之下，大多数ENB患者接受了手术切除，随后进行放疗。由于随访数据有限，观察到五分之四的SNUC患者和四分之三的SNEC患者发生了局部区域转移（SNUC患者中有3例，SNEC患者中有2例）或远处转移（SNUC和SNEC各有1例患者）。ENB患者也有局部区域复发（7例患者中有5例），病程较长，但在现有病例的随访期间未观察到远处转移。结论：具有神经内分泌分化的鼻窦肿瘤是一组组织形态学特征重叠的异质性肿瘤。它们可以根据免疫组化特征进行区分。病理细分对于这些侵袭性肿瘤的管理和预后评估至关重要。
