Abstract：
OBJECTIVE: To explore the clinicopathologic features, treatment, and survival outcomes of angioimmunoblastic T-cell lymphoma (AITL) involving the nasopharynx.
METHODS: We retrospectively analyzed 73 cases of AITL. Among them, 64 cases with complete pre-treatment 18F-FDG positron emission tomography/computed tomography (PET/CT) images were integrated into the analysis of clinical characteristics and PET/CT findings of AITL involving the nasopharynx; 14 cases with both biopsies from lymph node and nasopharynx were included in the comparison of pathological characteristics of AITL in the two areas. Forty-six of the 73 patients who received first-line systemic treatment at our institute were included in the treatment efficacy and survival analyses.
RESULTS: Nasopharyngeal involvement was seen in 44/64 (68.8%) patients. Histologically, lymph node and nasopharyngeal biopsies in 14 patients both showed small to medium-sized tumor cells, complex inflammatory infiltration, and Reed-Sternberg-like cells or B immunoblasts. However, tumor cells with clear cytoplasm, significant high endothelial venule (HEV) hyperplasia, and perivascular infiltration were observed in 5/14, 3/14, and 2/14 nasopharyngeal biopsies, respectively, but in all fourteen lymph node biopsies (P < 0.05). Immunophenotypic profiles and gene rearrangements were highly concordant. Treatment efficacy and survival were similar between patients with nasopharyngeal involvement and those without (P > 0.05), indicating nasopharyngeal involvement is not a prognostic factor for AITL patients.
CONCLUSIONS: Nasopharyngeal involvement is common in AITL but can be easily misdiagnosed because of its atypical pathologic pattern, especially when a lymph node biopsy is unavailable. However, the patient\'s clinical presentation, PET/CT manifestations, the typical immunophenotype, and gene rearrangements help the diagnosis.
METHODS: We retrospectively analyzed 73 cases of AITL. Among them, 64 cases with complete pre-treatment 18F-FDG positron emission tomography/computed tomography (PET/CT) images were integrated into the analysis of clinical characteristics and PET/CT findings of AITL involving the nasopharynx; 14 cases with both biopsies from lymph node and nasopharynx were included in the comparison of pathological characteristics of AITL in the two areas. Forty-six of the 73 patients who received first-line systemic treatment at our institute were included in the treatment efficacy and survival analyses.
RESULTS: Nasopharyngeal involvement was seen in 44/64 (68.8%) patients. Histologically, lymph node and nasopharyngeal biopsies in 14 patients both showed small to medium-sized tumor cells, complex inflammatory infiltration, and Reed-Sternberg-like cells or B immunoblasts. However, tumor cells with clear cytoplasm, significant high endothelial venule (HEV) hyperplasia, and perivascular infiltration were observed in 5/14, 3/14, and 2/14 nasopharyngeal biopsies, respectively, but in all fourteen lymph node biopsies (P < 0.05). Immunophenotypic profiles and gene rearrangements were highly concordant. Treatment efficacy and survival were similar between patients with nasopharyngeal involvement and those without (P > 0.05), indicating nasopharyngeal involvement is not a prognostic factor for AITL patients.
CONCLUSIONS: Nasopharyngeal involvement is common in AITL but can be easily misdiagnosed because of its atypical pathologic pattern, especially when a lymph node biopsy is unavailable. However, the patient\'s clinical presentation, PET/CT manifestations, the typical immunophenotype, and gene rearrangements help the diagnosis.
摘要：
目的：探讨临床病理特征，治疗,涉及鼻咽部的血管免疫母细胞性T细胞淋巴瘤（AITL）的生存结局。
方法：对73例AITL患者进行回顾性分析。其中,将64例治疗前完整的18F-FDG正电子发射断层扫描/计算机断层扫描（PET/CT）图像纳入AITL累及鼻咽的临床特征和PET/CT检查结果分析；14例同时进行淋巴结和鼻咽活检，纳入AITL两区病理特征比较。在我们研究所接受一线全身治疗的73例患者中有46例被纳入治疗效果和生存分析。
结果：44/64（68.8%）的患者出现鼻咽受累。组织学上,14例患者的淋巴结和鼻咽活检均显示小到中等大小的肿瘤细胞，复杂的炎症浸润，和Reed-Sternberg样细胞或B免疫母细胞。然而,具有清晰细胞质的肿瘤细胞，显著的高内皮小静脉（HEV）增生，在5/14、3/14和2/14鼻咽活检中观察到血管周围浸润，分别,但在所有14个淋巴结活检中（P<0.05）。免疫表型谱和基因重排高度一致。鼻咽受累患者和无鼻咽受累患者的治疗效果和生存率相似(P>0.05)。提示鼻咽受累不是AITL患者的预后因素。
结论：鼻咽受累在AITL中很常见，但由于其不典型的病理模式，很容易被误诊。尤其是在无法进行淋巴结活检时。然而,患者的临床表现，PET/CT表现,典型的免疫表型，基因重排有助于诊断.
方法：对73例AITL患者进行回顾性分析。其中,将64例治疗前完整的18F-FDG正电子发射断层扫描/计算机断层扫描（PET/CT）图像纳入AITL累及鼻咽的临床特征和PET/CT检查结果分析；14例同时进行淋巴结和鼻咽活检，纳入AITL两区病理特征比较。在我们研究所接受一线全身治疗的73例患者中有46例被纳入治疗效果和生存分析。
结果：44/64（68.8%）的患者出现鼻咽受累。组织学上,14例患者的淋巴结和鼻咽活检均显示小到中等大小的肿瘤细胞，复杂的炎症浸润，和Reed-Sternberg样细胞或B免疫母细胞。然而,具有清晰细胞质的肿瘤细胞，显著的高内皮小静脉（HEV）增生，在5/14、3/14和2/14鼻咽活检中观察到血管周围浸润，分别,但在所有14个淋巴结活检中（P<0.05）。免疫表型谱和基因重排高度一致。鼻咽受累患者和无鼻咽受累患者的治疗效果和生存率相似(P>0.05)。提示鼻咽受累不是AITL患者的预后因素。
结论：鼻咽受累在AITL中很常见，但由于其不典型的病理模式，很容易被误诊。尤其是在无法进行淋巴结活检时。然而,患者的临床表现，PET/CT表现,典型的免疫表型，基因重排有助于诊断.
