CAJ | 학술논문

目的：探讨鼻咽部低级别乳头状腺癌（ LGNPPA）的临床病理学特征、免疫表型特点及鉴别诊断。方法收集9例鼻咽部低级别乳头状腺癌的临床及病理检查标本资料，观察其组织学形态，采用免疫组织化学（ En Vision法）染色对5例行CKpan、波形蛋白、CK7、CK19、甲状腺转录因子（TTF）-1及甲状腺球蛋白（ TG）等多种标志物染色，以原位杂交方法检测 Epstein-Barr 病毒小mRNA1/2（EBER），以导流杂交方法检测人乳头状瘤病毒（HPV）。结果患者年龄23～62岁，平均年龄45.3岁。男8例，女1例。镜下肿瘤组织结构可见分叶状、乳头状、腺管状和成片的梭形细胞区，乳头间质可伴有水肿、黏液样变或玻璃样变，肿瘤呈浸润性生长，无包膜；4例可见肿瘤细胞与鼻咽部黏膜被覆柱状上皮有移行，1例肿瘤间质内可见沙砾体结构。5例免疫组织化学染色显示CKpan、波形蛋白、CK7、CK19、TTF-1弥漫强阳性，上皮细胞膜抗原（ EMA）局灶阳性，CD117部分区域呈阳性，TG、CK5/6、CK20、S-100蛋白、p63、Calponin及平滑肌肌动蛋白（ SMA）均阴性。 EBER原位杂交及HPV导流检测结果均为阴性。术后随访结果均无复发。结论鼻咽部低级别乳头状腺癌是一种罕见的、具有明显形态学特征的低级别上皮性恶性肿瘤，以发生部位、组织学特点为主要诊断依据，免疫组织化学染色标志物可辅助诊断及鉴别诊断；肿瘤可能起源于鼻咽部被覆上皮，完全切除预后良好。
목적：탐토비인부저급별유두상선암（ LGNPPA）적림상병이학특정、면역표형특점급감별진단。방법수집9례비인부저급별유두상선암적림상급병리검사표본자료，관찰기조직학형태，채용면역조직화학（ En Vision법）염색대5례행CKpan、파형단백、CK7、CK19、갑상선전록인자（TTF）-1급갑상선구단백（ TG）등다충표지물염색，이원위잡교방법검측 Epstein-Barr 병독소mRNA1/2（EBER），이도류잡교방법검측인유두상류병독（HPV）。결과환자년령23～62세，평균년령45.3세。남8례，녀1례。경하종류조직결구가견분협상、유두상、선관상화성편적사형세포구，유두간질가반유수종、점액양변혹파리양변，종류정침윤성생장，무포막；4례가견종류세포여비인부점막피복주상상피유이행，1례종류간질내가견사력체결구。5례면역조직화학염색현시CKpan、파형단백、CK7、CK19、TTF-1미만강양성，상피세포막항원（ EMA）국조양성，CD117부분구역정양성，TG、CK5/6、CK20、S-100단백、p63、Calponin급평활기기동단백（ SMA）균음성。 EBER원위잡교급HPV도류검측결과균위음성。술후수방결과균무복발。결론비인부저급별유두상선암시일충한견적、구유명현형태학특정적저급별상피성악성종류，이발생부위、조직학특점위주요진단의거，면역조직화학염색표지물가보조진단급감별진단；종류가능기원우비인부피복상피，완전절제예후량호。
Objective To study the clinicopathological features , immunophenotype , differential diagnosis and prognosis of low-grade nasopharyngeal papillary adenocarcinoma ( LGNPPA).Methods The histopathological features and clinical and pathological data of nine cases of LGNPPA were retrospectively analyzed.Immunohistochemistry ( Two-step EnVision methods ) was used to evaluate the expression of CKpan, vimentin, CK7, CK19, TTF-1 and TG;in situ hybridization was used to detect Epstein-Barr virus mRNA ( EBER);and flow-through hybridization was used to evaluate the presence of human papilloma virus (HPV).Results The mean age for the nine patients (eight males, one female) was 45.3 years (range 23 to 62 years).Microscopically the tumors were characterized by lobulated , papillary and glandular structures with patchy distribution of spindle cells.The papillary interstitial tissue was edematous , myxoid or hyalinized.The tumors were unencapsulated and infiltrated into the surrounding stroma.Four cases displayed transition between normal nasopharyngeal epithelium to neoplastic cells ;and one case contained psammoma bodies.Five cases were strongly positive for CKpan , vimentin, CK7, CK19, TTF-1, and were focally positive for EMA and CD117.These five cases were all negative for TG , CK5/6, CK20, S-100 protein, p63, Calponin and SMA.In situ hybridization for EBER and flow-through hybridization for HPV were negative in all five cases.Follow-up data showed no post-operative recurrence of the LGNPPA.Conclusions LGNPPA is a rare low-grade neoplasm with distinct morphological characteristics.Its diagnosis is primarily based on the site of lesions and the histological features.The diagnosis and differential diagnosis of LGNPPA could be aided by immunohistochemical staining.LGNPPA may originate from nasopharyngeal epithelium;and the prognosis is good with simple and complete resection.