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1、WHO 2004版胸腺瘤的分类,Histology,Capsule Septum Cortex Medulla Hassalls corpuscles,CK19,Ki67,CD1a,Normal Thymus,Immature T Cells,High Proliferative Activity,胸腺的免疫染色特点,胸腺瘤通常由两部分组成：上皮性和背景的未成熟小T淋巴细胞。 上皮性：CK19,CK7,CK14,CK5/6,P63; 但CK20-,CD5-,CD70-,CD117-。CK19阳性为网状分布。 T细胞：未成熟T: CD3,CD1a,TDT,CD99,Ki67有时高比例，可有少许B
2、细胞。,胸腺肿瘤的概论,胸腺肿瘤是构成胸腺的细胞分化而来的肿瘤，包括了上皮（胸腺瘤、胸腺癌、内分泌肿瘤），生殖细胞肿瘤，淋巴和造血系统肿瘤，以及间叶来源肿瘤。 组织起源和分化： 胸腺瘤：胸腺上皮（内胚层胸腺上皮干细胞，定向干细胞） 胸腺内分泌肿瘤（神经嵴和胸腺上皮） 淋巴瘤：T细胞、NK细胞发育场所，树突状细胞和B细胞。组织细胞和粒细胞肿瘤与畸胎瘤有关。 *麻雀虽小五脏俱全，取活检不易，不同疾病治疗方式不同，为极大的挑战。,A. 儿童时发病率 B. 成年时发病率,Thymoma,Definition: neoplasms arising from or exhibiting different
3、iateion towards thymic epithelial cells. Rare(15/million/year), but are the most frequent thymic tumors in adults. Exceedingly rare in children and adolescents. Etiology is largely unknown. Clinical features: local complication, some autoimmune diseases, myasthenia gravis(MG) is the most frequent as
4、sociated with thymoma especially type AB,B2,B3 thymoma.,Comparison of major histologic classifications of thymoma,Najamul Sahar Azad, Zubair Ahmad, et al. Thymoma : A Clinicopathologic association of World Health Organization histologic subtype and invasive behavior. JCPSP 2007, Vol. 17 (11): 658-66
5、1. *Suster and Moran: 仅以形态方式分类，thymoma, atypical thymoma, and thymic carcinoma.,临床Masaoka分类,T1：包膜完整 T2：侵犯包膜至周围结缔组织 T3：肿瘤侵犯周围脏器 T31：手术可切除组 T32：手术不易切除组 T4：肿瘤伴有心包或胸膜扩散 *注：良性胸腺瘤： 恶性胸腺瘤： I型恶性胸腺瘤 II型恶性胸腺瘤（胸腺癌）,胸腺瘤的分类,胸腺肿瘤的分类一直以来有争论和混乱不清。其焦点为以组织形态为主？临床分期为主？ 2004版的WHO分类，将胸腺瘤分为A、AB、B1、B2、B3型。原则上尽量使该分类与临床分期和预
6、后相一致。 *但仍有争议，临床的治疗依赖于临床分期，组织学分型仅起了小的作用。,Thymoma1,2 1/：为交界性或生物学行为未定肿瘤。 2 ：对于胸腺而言，设定为恶性。 行为编码/1-/3。,2004年胸腺瘤分类的原则,1. 主要分为两型： A型胸腺瘤细胞梭形或椭圆形，形态一致温和 B型胸腺瘤：细胞圆形或多边形为主。 2. B型根据淋巴细胞的多少和上皮细胞的异型程度分为三个亚型：B1(富于淋巴细胞型), B2 and B3(富于上皮细胞型); 3. 将伴有B1样或（罕见）B2 样表现混合性A型胸腺瘤分为AB型胸腺瘤。 4. 胸腺癌根据其分化而命名（如：鳞状细胞癌，粘液表皮样癌，肉瘤样癌，神
7、经内分泌癌等），将原有的C型胸腺瘤删除。 5. 混合型胸腺瘤 ，按其WHO组织形态每种成分相近为分类。 6. 传统的恶性胸腺瘤为两型晚期的胸腺瘤，临床T2期，与组织分型无关。胸腺癌，无论临床分期。将恶性胸腺瘤删除，强调A型或AB型预后好。,Prognosis,Type A and AB thymomasfavorable clinical course; Type B1very low malignant potential; Type B2 and B3 thymomas and thymic carcinomamalignant.,Alberto M. Marchevsky, Ruta G
8、upta, Robert J. McKenna, et al. Evidence-based Pathology and the Pathologic Evaluation of Thymomas: The World Health Organization Classification Can Be Simplified Into Only 3 Categories Other Than Thymic Carcinoma. Cancer 2008;112:27808.,Type A,A tumor comprised of a homogenous population of neoplas
9、tic epithelial cells with spindle/oval shape, lacking nuclear atypia, and accompanied by few or no non-neoplastic lymphocytes The tumor cells can form a variety of histologic structures. Synonyms: Spindle thymoma; medullary thymoma.,Fig 3.05 A well encapsulated type A thymoma with vaguely lobulated
10、appearance and thin white firous bands. 镜下：小叶不清，纤维分隔不明显。,Type A thymoma. A. Spindle cells. B. Oval cells,Type A thymoma. A. Type A thymoma cells can form cysts of various size. B.Type A thymoma cells with haemangiopericytoma-like appearance. C.Rosettes without a lumen. D.Anaplastic malignant cells a
11、rising in type A thymoma.,Different histological patterns in type A thymoma. A. Glandular structures. B. Glomeruloid bodies. C. Rosettes with lumens. D. Perivascular spaces.,Type AB,An organotypical thymic epithelial neoplasm composed of a mixture of a lymphocyte-poor type A thymoma component and a
12、more lymphocyte-rich type B-like component. B area: small polygonal epithelial cells; medullary differnetiation is rare, Hassalls corpuscles are absent. Synonyms: Mixed thymoma.,A. Macroscopic appearance of a type AB thymoma showing multiple nodules separated by fibrous bands. B. Type AB thymoma com
13、posed of lymphocyte-associated type B nodules and diffuse lymphocyte-poor type A areas.,Type AB thymoma. A. The lymphocyte-associated type B component and the lymphocyte-poor type A component intermix together. The type A component cells appear as elongated fibroblast-like spindle cells. B. The type
14、 B component of predominantly small polygonal cells with small round or oval pale nuclei with pale chromatin and inconspicuous nucleoli. C. Type A component is poor in lymphcytes(left), while type B component is lymphocyte-rich (right). 同一肿瘤中二者的混合不同部位，表现不同，可互相交织或截然分开。,Type B1,A tumor composed predom
15、inantly of areas resembling cortex with epithelial cells scantered in a prominent population of immature lymphocytes, With area of medullary differentiation, with or without Hassalls corpuscles, simliar to normal thymic medulla. Synonyms: Lymphocyte-rich thymoma; lymphocytic thymoma; organoid thymom
16、a; predominantly cortical thymoma. *小叶大小不一，由薄或厚的纤维分隔。,Type B1 thymoma. A. Medullary island(MI) showing Hassalls corpuscles. The abnormal localization of MI adjacent to septa or the tumor capsule is wery typical. B. High-power of cortex-like areas in B1 thymoma showing a vast majority of lymphoid cells compared to few inconspicuous epithelial cells characterized by vesicular, clear nuclei and distinct but small nucleoli. C. Small MI, that is devoid of Hassalls corpuscles is surro
