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Abstract：
BACKGROUND: There is currently scarcity of information on small cell neuroendocrine carcinoma of the nasopharynx (SCNEC-nasopharynx). It is believed that this type of cancer is not associated with Epstein-Barr virus (EBV) infection and is indistinguishable from classic SCNEC occurring in other organs.
METHODS: Herein we provided 3 cases of nasopharyngeal mass in our hospital, two males and one female. On admission, these patients were considered nasopharyngeal carcinoma with lymph node metastasis, and one of them had liver metastasis. The nasopharyngeal mucosal tissues were biopsied for pathological examination including immunohistochemistry and in situ hybridization. PubMed database was searched for articles about SCNEC-nasopharynx published up to April 2024 in any language.
RESULTS: The 3 cases had similar histological features of SCNEC in other organs but differed in rich- tumor-infiltrating lymphocytes (TILs). All of them stained for pancytokeratin (panCK) and epidermal growth factor receptor (EGFR). Case 1 and Case 2 diffusely expressed insulinoma-associated protein 1(INSM-1) and synaptophysin (Syn), Case 3 strongly stained for CD56 and Syn. Immunostaining of all 3 cases for p40, p63, TTF-1, CK20, S-100 and NUT showed negative. BRG-1, INI-1 and Rb were retained. And p53 all showed wild-type expression. The Ki-67 labeling indiced of case 1, 2, and 3 were 80%, 90%, and 80%, respectively. In situ hybridization showed strong and uniform nuclear positivity of EBV-encoded small RNAs (EBER) in the neoplastic cells of 3 cases.
CONCLUSIONS: EBV-positive SCNEC-nasopharynx was exactly rare. The origin of this tumor is still controversial. It may originate from EBV-infected mucosal epithelium like nasopharyngeal carcinoma. Based on our cases and relevant literature, we found EBV-positive SCNEC-nasopharynx as a probably site-specific subtype of SCNEC with differing pathogenetic mechanism. The subtype not only virus positivity but also that it was associated with TILs and did not show p53 or Rb alterations by immunohistochemistry. It may be more responsive to treatment and have a better prognosis than classic SCNEC. We will continue to follow-up these patients and collect additional cases to further understand the unique biology of this rare solid tumor.
METHODS: Herein we provided 3 cases of nasopharyngeal mass in our hospital, two males and one female. On admission, these patients were considered nasopharyngeal carcinoma with lymph node metastasis, and one of them had liver metastasis. The nasopharyngeal mucosal tissues were biopsied for pathological examination including immunohistochemistry and in situ hybridization. PubMed database was searched for articles about SCNEC-nasopharynx published up to April 2024 in any language.
RESULTS: The 3 cases had similar histological features of SCNEC in other organs but differed in rich- tumor-infiltrating lymphocytes (TILs). All of them stained for pancytokeratin (panCK) and epidermal growth factor receptor (EGFR). Case 1 and Case 2 diffusely expressed insulinoma-associated protein 1(INSM-1) and synaptophysin (Syn), Case 3 strongly stained for CD56 and Syn. Immunostaining of all 3 cases for p40, p63, TTF-1, CK20, S-100 and NUT showed negative. BRG-1, INI-1 and Rb were retained. And p53 all showed wild-type expression. The Ki-67 labeling indiced of case 1, 2, and 3 were 80%, 90%, and 80%, respectively. In situ hybridization showed strong and uniform nuclear positivity of EBV-encoded small RNAs (EBER) in the neoplastic cells of 3 cases.
CONCLUSIONS: EBV-positive SCNEC-nasopharynx was exactly rare. The origin of this tumor is still controversial. It may originate from EBV-infected mucosal epithelium like nasopharyngeal carcinoma. Based on our cases and relevant literature, we found EBV-positive SCNEC-nasopharynx as a probably site-specific subtype of SCNEC with differing pathogenetic mechanism. The subtype not only virus positivity but also that it was associated with TILs and did not show p53 or Rb alterations by immunohistochemistry. It may be more responsive to treatment and have a better prognosis than classic SCNEC. We will continue to follow-up these patients and collect additional cases to further understand the unique biology of this rare solid tumor.
摘要：
背景：目前缺乏关于鼻咽小细胞神经内分泌癌（SCNEC-鼻咽）的信息。据信,这种类型的癌症与EB病毒(EBV)感染无关,并且与在其他器官中发生的经典SCNEC难以区分。
方法：本文提供了我院3例鼻咽肿块,两名男性和一名女性。一入场,这些患者被认为是鼻咽癌伴淋巴结转移，其中一人有肝转移。对鼻咽粘膜组织进行活检以进行病理检查，包括免疫组织化学和原位杂交。在PubMed数据库中搜索了截至2024年4月以任何语言发表的有关SCNEC-鼻咽的文章。
结果：3例患者在其他器官中具有相似的SCNEC组织学特征，但富含肿瘤浸润淋巴细胞（TIL）不同。所有这些都对全细胞角蛋白（panCK）和表皮生长因子受体（EGFR）进行了染色。病例1和病例2弥漫性表达胰岛素瘤相关蛋白1（INSM-1）和突触素（Syn），病例3对CD56和Syn强烈染色。3例p40、p63、TTF-1、CK20、S-100和NUT的免疫染色均为阴性。保留BRG-1、INI-1和Rb。p53均呈野生型表达。病例1、2和3的Ki-67标记为80%，90%,80%,分别。原位杂交显示3例肿瘤细胞中EBV编码的小RNA（EBER）的核阳性强且均匀。
结论：EBV阳性的SCNEC鼻咽部非常罕见。这种肿瘤的起源仍然存在争议。它可能起源于EBV感染的粘膜上皮，如鼻咽癌。根据我们的案例和相关文献，我们发现EBV阳性的SCNEC鼻咽部可能是SCNEC的一个位点特异性亚型,具有不同的发病机制.该亚型不仅病毒阳性，而且与TIL相关，并且通过免疫组织化学未显示p53或Rb改变。它可能比经典的SCNEC对治疗更敏感，预后更好。我们将继续对这些患者进行随访，并收集更多病例，以进一步了解这种罕见实体瘤的独特生物学特性。
方法：本文提供了我院3例鼻咽肿块,两名男性和一名女性。一入场,这些患者被认为是鼻咽癌伴淋巴结转移，其中一人有肝转移。对鼻咽粘膜组织进行活检以进行病理检查，包括免疫组织化学和原位杂交。在PubMed数据库中搜索了截至2024年4月以任何语言发表的有关SCNEC-鼻咽的文章。
结果：3例患者在其他器官中具有相似的SCNEC组织学特征，但富含肿瘤浸润淋巴细胞（TIL）不同。所有这些都对全细胞角蛋白（panCK）和表皮生长因子受体（EGFR）进行了染色。病例1和病例2弥漫性表达胰岛素瘤相关蛋白1（INSM-1）和突触素（Syn），病例3对CD56和Syn强烈染色。3例p40、p63、TTF-1、CK20、S-100和NUT的免疫染色均为阴性。保留BRG-1、INI-1和Rb。p53均呈野生型表达。病例1、2和3的Ki-67标记为80%，90%,80%,分别。原位杂交显示3例肿瘤细胞中EBV编码的小RNA（EBER）的核阳性强且均匀。
结论：EBV阳性的SCNEC鼻咽部非常罕见。这种肿瘤的起源仍然存在争议。它可能起源于EBV感染的粘膜上皮，如鼻咽癌。根据我们的案例和相关文献，我们发现EBV阳性的SCNEC鼻咽部可能是SCNEC的一个位点特异性亚型,具有不同的发病机制.该亚型不仅病毒阳性，而且与TIL相关，并且通过免疫组织化学未显示p53或Rb改变。它可能比经典的SCNEC对治疗更敏感，预后更好。我们将继续对这些患者进行随访，并收集更多病例，以进一步了解这种罕见实体瘤的独特生物学特性。
