肺纤毛性黏液结节性乳头状肿瘤2例临床病理观察
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摘要
目的探讨肺纤毛性黏液结节性乳头状肿瘤(ciliated muconodular papillary tumor of the lung,CMPT)临床病理学特点。方法回顾性分析2例肺纤毛性黏液结节性乳头状肿瘤,分析其病理形态、免疫表型并复习相关文献。结果患者年龄50岁和70岁,男女各1例,均无吸烟史和临床症状。肺部CT均提示肺外周型小结节。1例发生于左下肺,1例发生于右上肺,均行楔形肺切除术。肿瘤最大径分别为8 mm(伴薄壁空洞)和17 mm;镜下见肿瘤为假复层柱状上皮乳头样结构,被覆3种细胞即纤毛细胞、杯状细胞及基底细胞,伴有大量细胞外黏液,细胞无异型性,无核分裂象及坏死。免疫组化:纤毛细胞、杯状细胞及基底细胞均CK7强(+),TTF-1弱(+),CK20和MUC2(-);基底细胞p40、p63和CK5/6强(+);纤毛细胞MUC5AC(+),Ki-67阳性指数分别为10%和2%。术后随访5个月及11个月,均无复发或转移。分子检测:EGFR和BRAF均无基因突变。结论肺纤毛性黏液结节性乳头状肿瘤是一种少见的良性肿瘤,依据其典型病理组织学特点、免疫表型可做出诊断,需要与肺黏液腺癌等恶性肿瘤相鉴别,尤其在术中冷冻快速诊断时。
Objective To investigate the pathological features of ciliated muconodular papillary tumor of the lung. Methods The morphology and immunoprofiles of ciliated muconodular papillary tumor of the lung, and the relevant literatures were reviewed. Results A seventy-year-old female had a CT scan showing a nodule with a thin-wall cavity in her left lower lobe of lung and another fifty-year-old male showed a nodule in his right upper lobe of lung. Neither of them had history of smoking or symptoms. Video-assisted thoracoscopic partial lung resection was performed. Grossly, the peripheral soft nodules were detected with greyish cut-surface and the maximum diameters of the tumor were 8 mm and 17 mm, respectively. Microscopically, the tumor was comprised of papillary fronds lined by pseudostratified columnar epithelial cells. The pseudostratified columnar epithelial cells included ciliated columnar cells, goblet cells and basal cells, which were accompanied with abundant extracellular mucin. No cytological atypia and mitosis were found in the tumor. Immunohistochemically, all the types of cells were strongly positive for CK7, weakly positive for TTF-1 while negative for CK20 and MUC2. The basal cells were immunoreactive for P40, P63 and CK5/6, and the ciliated columnar cells were immunoreactive for MUC5 AC. The Ki-67 labeling index was about 10% and 2%, respectively. There was no EGFR or BRAF mutation detected in the tumor. The two patients were disease-free after operation for 5 months and 11 months, respectively. Conclusion Ciliated muconodular papillary tumor of the lung is a rare benign tumor, which can be diagnosed according to its typical pathological features and immunophenotype. It should be distinguished from mucinous adenocarcinoma of the lung, especially in the intraoperative consultation.
Objective To investigate the pathological features of ciliated muconodular papillary tumor of the lung. Methods The morphology and immunoprofiles of ciliated muconodular papillary tumor of the lung, and the relevant literatures were reviewed. Results A seventy-year-old female had a CT scan showing a nodule with a thin-wall cavity in her left lower lobe of lung and another fifty-year-old male showed a nodule in his right upper lobe of lung. Neither of them had history of smoking or symptoms. Video-assisted thoracoscopic partial lung resection was performed. Grossly, the peripheral soft nodules were detected with greyish cut-surface and the maximum diameters of the tumor were 8 mm and 17 mm, respectively. Microscopically, the tumor was comprised of papillary fronds lined by pseudostratified columnar epithelial cells. The pseudostratified columnar epithelial cells included ciliated columnar cells, goblet cells and basal cells, which were accompanied with abundant extracellular mucin. No cytological atypia and mitosis were found in the tumor. Immunohistochemically, all the types of cells were strongly positive for CK7, weakly positive for TTF-1 while negative for CK20 and MUC2. The basal cells were immunoreactive for P40, P63 and CK5/6, and the ciliated columnar cells were immunoreactive for MUC5 AC. The Ki-67 labeling index was about 10% and 2%, respectively. There was no EGFR or BRAF mutation detected in the tumor. The two patients were disease-free after operation for 5 months and 11 months, respectively. Conclusion Ciliated muconodular papillary tumor of the lung is a rare benign tumor, which can be diagnosed according to its typical pathological features and immunophenotype. It should be distinguished from mucinous adenocarcinoma of the lung, especially in the intraoperative consultation.
引文
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[15] Kataoka T,Okudela K,Matsumura M,et al.A molecular pathological study of four cases of ciliated muconodular papillary tumors of the lung [J].Pathol Int,2018,68(6):353-358.
[16] Kamata T,Sunami K,Yoshida A,et al.Frequent BRAF or EGFR mutations in ciliated muconodular papillary tumors of the lung [J].J Thorac Oncol,2016,11(2):261-265.
[17] Nakamura S,Koshikawa T,Sato T,et al.Extremely well differentiated papillary adenocarcinoma of the lung with prominent cilia formation [J].Acta Pathol J,1992,42(10):745-750.
[18] Arai Y,Shimizu S,Eimoto T,et al.Peripheral pulmonary papillary/glandular neoplasms with ciliated cells and a component of well-differentiated adenocarcinoma:report of three tumours[J].Histopathology,2010,56(2):265-269.
[19] Nair AR,Samvedam S,Madathipat U,et al.Moderately differentiated peripheral adenocarcinoma of the lung with cilia [J].Int J Surg Pathol,2014,22(6):540-543.
[20] Kaseda K,Horio H,Harada M,et al.Solitary glandular papilloma of the peripheral lung:a report of two cases [J].World J Surg Oncol,2014,12(1):149.
[21] 李雪,金木兰,王跃,等.气管黏液腺腺瘤1例临床病理观察[J].临床与实验病理学杂志,2012,28(9):1019-1022.
[22] Haruki T,Nakamura H,Taniguchi Y,et al.Pulmonary mucinous cystadenoma:a rare benign tumor of the lung [J].Gen Thorac Cardiovasc Surg,2010,58(6):287-290.
[23] Sugano M,Nagasaka T,Sasaki E,et al.HNF4α as a marker for invasive mucinous adenocarcinoma of the lung [J].Am J Surg Pathol,2013,37(2):211-218.
[2] Aida S,Ohara I,Shimazaki H,et al.Solitary peripheral ciliated glandular papillomas of the lung:a report of 3 cases [J].Am J Surg Pathol,2008,32(10):1489-1494.
[3] Harada T,Akiyama Y,Ogasawara H,et al.Ciliated muconodular papillary tumor of the peripheral lung:a newly defined rare tumor [J].Res Med Cme,2008,1(2):176-178.
[4] Sato S,Koike T,Homma K,et al.Ciliated muconodular papillary tumour of the lung:a newly defined low-grade malignant tumour[J].Int Cardiovasc Thorac Surg,2010,11(5):685-687.
[5] Lau KW,Aubry MC,Tan GS,et al.Ciliated muconodular papillary tumor:a solitary peripheral lung nodule in a teenage girl[J].Hum Pathol,2016,49:22-26.
[6] Kamata T,Yoshida A,Kosuge T,et al.Ciliated muconodular papillary tumors of the lung:a clinicopathologic analysis of 10 cases [J].Am J Surg Pathol,2015,39(6):753-760.
[7] Liu L,Aesif SW,Kipp BR,et al.Ciliated muconodular papillary tumors of the lung can occur in western patients and show mutations in BRAF and AKT1 [J].Am J Surg Pathol,2016,40(12):1631.
[8] Ishikawa M,Sumitomo S,Imamura N,et al.Ciliated muconodular papillary tumor of the lung:report of five cases [J].J Surg Case Rep,2016,2016(8):144.
[9] Kon T,Baba Y,Fukai I,et al.Ciliated muconodular papillary tumor of the lung:a report of five cases [J].Pathol Int,2016,66(11):633-639.
[10] Taguchi R,Higuchi K,Sudo M,et al.A case of anaplastic lymphoma kinase (ALK)-positive ciliated muconodular papillary tumor (CMPT) of the lung [J].Pathol Int,2017,67(2):99.
[11] Jin Y,Shen X,Shen L,et al.Ciliated muconodular papillary tumor of the lung harboring ALK gene rearrangement:case report and review of the literature [J].Pathol Int,2017,67(3):171-175.
[12] Matsuoka S,Kondo R,Ishii K.Differential diagnosis of a rare papillary tumor and mucinous adenocarcinoma[J].Asian Cardiovasc Thorac Ann,2017,25(5):218492317713100.
[13] Udo E,Furusato B,Sakai K,et al.Ciliated muconodular papillary tumors of the lung with KRAS/BRAF/AKT1 mutation[J].Diagn Pathol,2017,12(1):62.
[14] Kim L,Kim YS,Lee JS,et al.Ciliated muconodular papillary tumor of the lung harboring BRAF V600E mutation and p16INK4a overexpression without proliferative activity may represent an example of oncogene-induced senescence[J].J Thorac Dis,2017,9(12):E1039-1044.
[15] Kataoka T,Okudela K,Matsumura M,et al.A molecular pathological study of four cases of ciliated muconodular papillary tumors of the lung [J].Pathol Int,2018,68(6):353-358.
[16] Kamata T,Sunami K,Yoshida A,et al.Frequent BRAF or EGFR mutations in ciliated muconodular papillary tumors of the lung [J].J Thorac Oncol,2016,11(2):261-265.
[17] Nakamura S,Koshikawa T,Sato T,et al.Extremely well differentiated papillary adenocarcinoma of the lung with prominent cilia formation [J].Acta Pathol J,1992,42(10):745-750.
[18] Arai Y,Shimizu S,Eimoto T,et al.Peripheral pulmonary papillary/glandular neoplasms with ciliated cells and a component of well-differentiated adenocarcinoma:report of three tumours[J].Histopathology,2010,56(2):265-269.
[19] Nair AR,Samvedam S,Madathipat U,et al.Moderately differentiated peripheral adenocarcinoma of the lung with cilia [J].Int J Surg Pathol,2014,22(6):540-543.
[20] Kaseda K,Horio H,Harada M,et al.Solitary glandular papilloma of the peripheral lung:a report of two cases [J].World J Surg Oncol,2014,12(1):149.
[21] 李雪,金木兰,王跃,等.气管黏液腺腺瘤1例临床病理观察[J].临床与实验病理学杂志,2012,28(9):1019-1022.
[22] Haruki T,Nakamura H,Taniguchi Y,et al.Pulmonary mucinous cystadenoma:a rare benign tumor of the lung [J].Gen Thorac Cardiovasc Surg,2010,58(6):287-290.
[23] Sugano M,Nagasaka T,Sasaki E,et al.HNF4α as a marker for invasive mucinous adenocarcinoma of the lung [J].Am J Surg Pathol,2013,37(2):211-218.