Rekhi Bharat, Karmarkar Srushti
Department of Surgical Pathology, Tata Memorial Hospital, Mumbai, India.
Cytopathology. 2019 Mar;30(2):229-235. doi: 10.1111/cyt.12631. Epub 2018 Nov 8.
To present clinical and cytopathological features of nine cases of chordomas, diagnosed over 9 years and confirmed by brachyury (T) immunostaining.
Conventional cytological smears, stained with Papanicolaou and May-Grünwald Giemsa, along with corresponding histopathological (n = 8) and immunostained sections (n = 8) were reviewed. Immunohistochemical staining was performed on tissue sections by polymer detection technique.
Nine tumours occurred in seven males and two females, with age ranging from 36 to 72 years (average = 58.7), in the sacrum (seven) and spine (two). On fine needle aspiration cytology, five cases were either diagnosed with or diagnosed with a suggestion of a chordoma, while three cases were diagnosed with chordoma as a differential diagnosis. On review, smears were moderately cellular, comprising myxoid stroma (9/9), epithelioid cells (9/9), physaliphorous cells (8/9), including binucleation (7/9), prominent nucleolisation (2/9), pleomorphic cells (2/9) and intranuclear inclusions (3/9). Immunohistochemically, tumour cells expressed cytokeratin (4/4), pan cytokeratin (4/4), epithelial membrane antigen (8/8), S100 protein (6/8) and brachyury (8/8). Five patients underwent surgical excision, including two who underwent adjuvant radiotherapy (RT) and four patients who underwent RT. During follow-up (n = 8), a single patient developed recurrence and another presented with metastatic lesions. Finally, five patients were alive with disease (7-53 months); a single patient was free of disease (4 months), and two patients died of disease; the latter cases displayed pleomorphic cells and intranuclear inclusions.
Chordomas can be primarily diagnosed by fine needle aspiration cytology in a typical clinicoradiological setting with a combination of key cytomorphological features. Pleomorphic cells and intranuclear inclusions are associated with a relatively aggressive subtype. An exact diagnosis has treatment implications and requires confirmation by brachyury immunostaining.
介绍9例脊索瘤的临床和细胞病理学特征，这些病例在9年多的时间里被诊断出来，并通过brachyury（T）免疫染色得以确诊。
回顾了用巴氏染色法和May-Grünwald Giemsa染色法染色的传统细胞学涂片，以及相应的组织病理学切片（n = 8）和免疫染色切片（n = 8）。采用聚合物检测技术对组织切片进行免疫组织化学染色。
9例肿瘤发生于7例男性和2例女性，年龄在36至72岁之间（平均58.7岁），部位在骶骨（7例）和脊柱（2例）。细针穿刺细胞学检查时，5例被诊断为脊索瘤或疑似脊索瘤，3例将脊索瘤作为鉴别诊断。复查时，涂片细胞量中等，包括黏液样基质（9/9）、上皮样细胞（9/9）、泡状核细胞（8/9），包括双核（7/9）、明显核仁化（2/9）、多形性细胞（2/9）和核内包涵体（3/9）。免疫组织化学方面，肿瘤细胞表达细胞角蛋白（4/4）、全细胞角蛋白（4/4）、上皮膜抗原（8/8）、S100蛋白（6/8）和brachyury（8/8）。5例患者接受了手术切除，其中2例接受了辅助放疗（RT），4例患者接受了放疗。随访期间（n = 8），1例患者复发，另1例出现转移病灶。最后，5例患者带瘤生存（7 - 53个月）；1例患者无病生存（4个月），2例患者死于疾病；后两例显示有多形性细胞和核内包涵体。
在典型的临床放射学背景下，结合关键的细胞形态学特征，脊索瘤可通过细针穿刺细胞学进行初步诊断。多形性细胞和核内包涵体与侵袭性较强的亚型相关。准确的诊断对治疗有指导意义，且需要通过brachyury免疫染色来确诊。
