T-cell lymphoma: morphology, immunophenotype and clinical features

The histology, immunophenotype and clinical presentation of 43 cases of T-cell lymphoma are described. Cases were classified into nine types; T-lymphocytic lymphoma (three), mycosis fungoides (six), Sézary syndrome (two), T-zone lymphoma (13), angioimmunoblastic lymphadenopathy (AIL)-like T-cell lymphoma (five), pleomorphic medium cell (one), large cell immunoblastic (four), large cell polylobated (five) and lymphoblastic (four). The patients comprised 26 males and 17 females aged between 15 and 86 years. The majority showed disseminated disease at the time of diagnosis (18 stage IV, nine stage III, five stage II, eight stage I and three cases not staged). Thirty-one patients showed lymph node involvement. Cutaneous involvement was a common finding (18 cases, 10 cases excluding mycosis fungoides and Sézary syndrome). Details of therapy and clinical follow-up were obtained in 37 cases. With simple chemotherapy only one complete response (7%, 1/16) was obtained. With aggressive therapy 48% (13/27) of patients showed complete responses. Twenty patients died during the follow-up period. Life table analysis showed a 58% probability of surviving 1 year and 36% probability of surviving 3 years. There was a significant difference in survival probability between low/intermediate-grade (lymphocytic, Sézary syndrome, mycosis fungoides and T-zone lymphoma including AIL-type) lymphomas and high-grade (large cell immunoblastic and polylobated and lymphoblastic) lymphomas (P less than 0.025). However, when survival of T-zone and AIL-like T-cell lymphoma was compared with survival of large cell immunoblastic and polylobated lymphomas no significant difference was detected. Age (less than 50 years) and stage I or II disease were associated with significantly better survival (P less than 0.005 and P less than 0.05).     

Primary non-Hodgkin's lymphoma of the intestine: a morphological, immunohistochemical and clinical study of 31 Chinese cases

Thirty-one cases of primary non-Hodgkin's lymphoma of the intestine were investigated. Twenty-one were of B-cell and 10 of T-cell origin. The B-cell lymphomas comprised two cases of low-grade B-cell lymphoma of mucosaassociated lymphoid tissue (MALT), one of centroblastic/centrocytic type, three of high-grade B-cell lymphoma coexisting with a low-grade B-cell lymphoma of MALT, nine of centroblastic, three of immunoblastic and three of Burkitt type. Of the T-cell lymphomas, eight were of pleomorphic medium-to large-sized cell type and two of large cell anaplastic type. All the B-cell lymphomas expressed CD20 (L26) and/or Ki-B5; in six there was monotypic immunoglobulin light chain restriction. Membrane positivity for CD45RO (UCHL1) was observed in the 10 cases of T-cell lymphoma, but the tumour cells did not express monocyte-macrophage markers. Clinically, the patients with T-cell lymphomas were usually young males with constitutional symptoms and their prognosis was significantly worse than those of patients with intestinal B-cell lymphoma.     

An immunohistochemical study of non-Hodgkin''s lymphomas: correlation of morphological appearances and immunophenotype in 148 cases

An unselected series of 148 cases of non-Hodgkin's lymphoma has been studied by immunohistological methods. In each case, the morphological features displayed in paraffin sections were correlated with the immunophenotype, determined using a panel of monoclonal antibodies. Of the lymphomas 82% were B-cell type, 28% of follicle centre cell origin, 17% lymphocytic or immunocytic and 30% of large cell type. All the B-cell tumours expressed pan-B antigen, and nearly all HLA-DR. In most, light chain monoclonality was demonstrable. Nearly all had moderate to large numbers of T-cells of both subtypes interspersed amongst the B-lymphocytes. Follicle centre cell tumours expressed surface IgM and IgD and had numerous dendritic reticulum cells. Lymphocytic and immunocytic lymphoma expressed IgM but less IgD, and had fewer or no dendritic reticulum cells. Large cell lymphoma expressed either no immunoglobulin or only IgM, and contained ragged dendritic reticulum cells, giving the appearance of 'burnt-out' follicles. T-cell lymphomas usually showed a clear preponderance of either helper or suppressor subtype. Additionally, they contained residual B-cells and sometimes germinal centres. Only 3% of this series were 'non-B, non-T', and only one case HLA-DR negative, so the 'null' or 'unclassifiable' group is very small when information from antibody markers is available.     

Primary gastric lymphomas: Morphologic, immunohistochemical and immunogenetic analyses

Morphologic, lmmunohistochemical and lmmunogenetic studies were performed on 28 cases of primary gastric lymphoma from fresh frozen tissue. Eight cases were diagnosed as diffuse large B-cell lymphoma, four as follicular center lymphoma (follicular), five as mucosa-associated lymphoid tissue (MALT) lymphoma, three as plasmacytoma, and three as T-cell lymphoma, two as mantle cell lymphoma, one as follicular center lymphoma (diffuse, predominantly small cell), and one as lymphoplasmacytoid lymphoma, and one as Hodgkin's disease.
From lmmunohistochemical studies, four types of morphologically similar low-grade lymphomas can be differentiated by a combination of various monoclonal antibodies. Cases of diffuse large B-cell lymphoma may have a germinal center origin. We observed lympho-epithelial lesions in cases of non-MALT lymphomas. We therefore consider that the current diagnostic criterion for MALT lymphoma may not always be valid.
Except for cases of T-cell lymphoma and Hodgkin's disease, 17 out of 22 cases revealed clonal rearrangement bands of the JH gene. In situ hybridization (ISH) and polymerase chain reaction (PCR) studies revealed the presence of Epstein-Barr (EB) virus genomes in two and three cases, respectively. Epstein-Barr virus may play a role in lympho-magenesis, although on relatively rare occasions.     

Primary lymphoma of the thyroid: a clinical, histological and immunohistochemical study of 20 cases

Twenty cases of malignant lymphoma arising in the thyroid gland were studied clinically, histologically and immunohistochemically. Nineteen cases were non-Hodgkin's lymphoma (15 diffuse and four follicular lymphoma) and one was a plasmacytoma. Immunohistochemical analysis of the lymphomas using paraffin-embedded sections disclosed that 17 lymphomas were B-cell type and two were T-cell type. The plasmacytoma was of IgG kappa type. The large majority of the lymphomas were associated with an underlying chronic thyroiditis. The 5-year survival rate of the patients was 70%. An unfavourable diagnosis was more likely when the tumour was diffuse rather than follicular, when it was of diffuse large cell type or of immunoblastic type and when there was cervical lymph node involvement.     

A morphological and immunohistochemical study of 25 cases of so-called sclerosing haemangioma of the lung

The so-called sclerosing haemangioma of the lung is an uncommon tumour with predilection for Chinese women. Twenty-five cases showed a predominantly solid/cellular pattern in three, a haemorrhagic/angiomatoid pattern in three, a papillary pattern in one, a sclerotic pattern in one, and in seventeen, a mixed pattern was observed. Immunostaining revealed the expression of epithelial membrane antigen, vimentin (V9), and CD68 (KP1) in all tumours, cytokeratin in 23 (Cam 5.2 and MNF116), S-100 protein in 22 and alpha-smooth muscle actin in 23 tumours. All tumours were negative for surfactant, factor VIII-related antigen, CD34 and MAC387. There was strong labelling for progesterone receptor protein in 20 tumours, and oestrogen receptor protein was weakly stainable in 13 tumours. The histogenesis of sclerosing haemangioma of the lung is controversial and origin from pneumocyte, histiocyte, mesothelial, endothelial and Clara cell has been suggested. Our immunohistochemical findings, in the largest series studied to date, indicated a cytokeratin+, vimentin+, epithelial membrane antigen+, alpha-smooth muscle actin+, CD68+, oestrogen+, progesterone+, phenotype, confirming an epithelial lineage but which does not clearly correspond to any recognized cell in the respiratory system. The distinct expression of progesterone and oestrogen receptor proteins correlates with the predilection of this tumour for women.     

小B细胞恶性淋巴瘤形态学和免疫组织化学研究

目的：探讨各种小B细胞恶性淋巴瘤的形态学、免疫表型特征及其鉴别诊断。方法：对15例小淋巴细胞性淋巴瘤（SLL）、3例淋巴浆细胞性淋巴瘤（LPL）、36例滤泡性淋巴瘤（FL）、25例套细胞淋巴瘤（MCL）、7例淋巴结边缘区B细胞淋巴瘤（MZL）和30例黏膜相关淋巴细胞型结外边缘区B细胞淋巴瘤（MALT－MZL）的石蜡切片进行HE形态学观察和CD5、CD10、CD23和cyclinD1等抗体的免疫组织化学分析。结果：各种小B细胞恶性淋巴瘤在组成细胞和组织结构上各具特征；免疫表型：SLL表达CD5（82％）和CD23（80％），FL表达CD10（87％），MCL表达cyclinD1（84％）和CD5（80％），MZL／MALT－MZL和LPL均不表达CD5、CD10、CD23和cyclinD1。结论：各种小B细胞恶性淋巴瘤均是独立疾病，各具形态学和免疫表型特征，结合HE形态学观察和CD5、CD10、CD23、cyclinD1等免疫组化分析有助于正确诊断和鉴别诊断。     

Morphological and immunohistochemical characteristics of T-cell malignant lymphomas in the west of Scotland

Sixteen cases of T-cell malignant lymphoma are described. They represent the experience of a single pathology department in recent years and serve to illustrate several of the reasons why recognition of T-cell differentiation is important in the classification of lymphomas.     

原发性骨恶性淋巴瘤临床病理及免疫表型观察

目的：观察原发性骨恶性淋巴瘤（PLB）的临床病理及免疫表型特征。方法：对8例PLB进行临床病理学观察及免疫组织化学染色（ABC法）。结果：8例PLB平均年龄51岁。X线片示溶骨性破坏或伴有骨质疏松;组织学观察均为弥漫性非霍奇金淋巴瘤（NHL）,大细胞性4例,中心细胞性2例、混合细胞性（中心细胞－中心母细胞）和淋巴交细胞样各1例;免疫化显示B细胞6例、T细胞2例。结论：PLB好于50岁左右,X线片主要表现为溶骨性破坏和骨质疏松。病理类型为弥漫性NHL,细胞形态主要是大细胞,免疫表型则以B细胞多见,免疫化有助于PLB的鉴别诊断。     

乳腺叶状肿瘤的形态学和免疫组织化学研究

目的观察乳腺叶状肿瘤（phyllodes tumours,PTs）的形态学和免疫组织化学特征,并探讨其诊断标准。方法对21例PTs进行组织学观察和免疫组化SP法检测,并选取5例乳腺纤维腺瘤和5例乳腺腺病标本作为对照组。使用一抗包括CK-pan、EMA、SMA、p53、S-100蛋白、CD117、CD34、CD99、bc l-2、ER、PR、K i-67、CD10。结果21例PTs大体上均表现为界限清楚的肿块,且呈分叶状。肿瘤由具有双层排列的上皮成分以及过度生长的间质成分组成。根据间质的过度生长程度、细胞的异型程度、核分裂数、肿瘤边缘情况、有无异源性成分以及肿瘤性坏死等继发性改变将其分为良性、交界性和恶性3个级别。间质细胞免疫组化表达情况：CKpan：0/21、EMA：0/21、SMA：17/21、CD117：6/21、CD34：18/21、S-100蛋白：2/21、CD99：13/21、CD10：8/21、PR：5/21、ER：4/21、p53：18/21、K i-67：3%~10%、bc l-2：15/21。结论PTs的诊断主要依据组织学观察,免疫组化CD117、CD34、CD99、CD10、bc l-2的检测可以起到一定的辅助作用。     

Peripheral T-cell lymphomas: a clinicopathological and immunological study of 10 cases

Diagnosis and classification of T-cell lymphomas is notoriously difficult. Existing classification schemes are insufficient. Some clinicopathologically well defined T-cell lymphomas exist (mycosis fungoides, Sézary's syndrome, and T-lymphoblastic lymphomas) but the remaining tumours, frequently called peripheral T-cell lymphomas, are a heterogeneous group, clinically, morphologically and immunologically. The data on 10 peripheral T-cell lymphomas are presented and compared to data from the literature. Patients were elderly, had a high frequency of extranodal localizations (notably the skin 75%) and had a poor prognosis: five of 10 patients have died, median survival 22 months. Morphologically and immunophenotypically the group is very heterogeneous. The variety of blast cell morphology is emphasized. No correlations were found between immunophenotype and prognosis, or immunophenotype and morphology.     

Histiocyte-rich, T-cell-rich B-cell lymphoma: a distinct diffuse large B-cell lymphoma subtype showing characteristic morphologic and immunophenotypic features

AIMS: The clinicopathological features of histiocyte-rich, T-cell-rich B-cell lymphoma (HRTR-BCL) were first recognized in 1992. In this study, 60 cases of HRTR-BCL were analysed in order to provide a detailed morphological and immunophenotypical profile of the disorder. METHODS AND RESULTS: HRTR-BCL is easily distinguished from other B-cell lymphomas rich in stromal T-cells by (i) a diffuse or vaguely nodular growth pattern, (ii) the presence of a minority population of CD15-, CD20+ large neoplastic B-cells, (iii) a prominent stromal component composed of both T-cells and non-epithelioid histiocytes, and (iv) the scarcity of small reactive B-cells. These criteria also enable a reliable distinction from lymphocyte-rich classical Hodgkin's lymphoma (CHL), from lymphocyte-predominant Hodgkin's lymphoma (LPHL), paragranuloma type and from peripheral T-cell lymphoma. Based on the morphology of the neoplastic cells and on their frequent bcl-6 immunoreactivity, we speculate that HRTR-BCL may be derived from a progenitor cell of germinal centre origin. CONCLUSIONS: HRTR-BCL presents characteristic clinical features, affecting predominantly middle-aged men who present with advanced stage disease and are at high risk of treatment failure. Considering these distinctive clinicopathological features, recognizing HRTR-BCL as a lymphoma entity may be justified.     

Low-grade B-cell lymphomas of the splenic marginal zone: a clinicopathological and immunohistochemical study of 14 cases

We have studied 14 cases of low-grade, splenic marginal zone, B-cell non-Hodgkin's lymphoma. The clinical presentation in all cases was with splenomegaly and, in 10 cases, circulating neoplastic lymphoid cells in the peripheral blood with involvement of bone marrow. In all cases the splenic white pulp was hyperplastic with expansion of marginal zones and varying degrees of infiltration of germinal centres by neoplastic cells. The cells were a mixture of medium sized lymphocytes with moderate amounts of cytoplasm and finely dispersed nuclear chromatin, together with occasional blast cells with small nucleoli. Satellite red pulp aggregates of tumour cells centred on small epithelioid cell clusters were seen in all cases. These appear to be a characteristic and diagnostically important feature of splenic marginal zone lymphoma. The tumour cells expressed CD20, CD45RA, bcl-2 and the antigens detected by MB2. All cases expressed IgM with light chain restriction. In addition, IgD was expressed in four cases. The follicular dendritic cell network was disrupted in those follicles which were infiltrated by tumour cells. A network of stromal myoid cells, at the periphery of the marginal zone, identified by expression of α-smooth muscle actin, was preserved. Alpha-smooth muscle actin positive dendritic cells were also seen within and around satellite tumour nodules in the red pulp.     

原发性卵巢Burkitt淋巴瘤病理分析1例并文献复习

目的:为了更好地认识原发性卵巢Burkitt淋巴瘤(Burkitt's lymphoma,BL)的临床表现、病理特征及其预后.方法:收集1例原发性卵巢BL的病例及其参考文献资料,观察其临床表现、临床病理学特征、免疫组织化学染色及1年的预后.结果:原发性卵巢BL临床表现主要为腹部或盆腔肿块,镜下可见肿瘤细胞弥漫增生,主要由淋巴样细胞组成,细胞中等大小,弥漫生长,细胞核圆形或卵圆形,染色质粗块状,核仁中等大小,嗜碱性,其中散在巨噬细胞,成"星空现象",免疫组织化学结果显示:CD79a(+),CD20(+),CD3(-),CD10(+),BCL-6(+),BCL2(-)和Ki-67(>90%).结论:诊断原发性卵巢BL需结合组织学及免疫组织化学结果,并对其鉴别诊断加以区分.     

Retiform Sertoli-Leydig cell tumours: clinical,morphological and immunohistochemical findings

AIMS: To determine the morphological and immunohistochemical profile of retiform Sertoli-Leydig cell tumours and to compare the observed profile with that of adult rete ovarii. METHODS AND RESULTS: Nineteen retiform Sertoli-Leydig cell tumours were studied, eight by immunohistochemistry, and five examples of rete ovarii from adult females were also evaluated immunohistochemically. The patients ranged in age from 3 to 74 years with a mean age of 31 years. Four patients were virilized and had an abdominal mass; two were virilized with amenorrhoea and two had amenorrhoea alone. Eight presented with an abdominal mass and one patient was pregnant. Two tumours were incidental findings. Information on stage was available in 16 patients: 14 tumours were stage 1, one was stage 2, and one was stage 3. Fifteen tumours were of intermediate differentiation and four were poorly differentiated. Papillary structures were evident grossly in four cases. Microscopically, all cases had a retiform pattern in addition to varying quantities of sex cord, gonadal stromal and heterologous elements. Heterologous elements were present in 13 cases and consisted of hepatocytes (n = 7), mucinous epithelium (n = 7) and skeletal muscle (n = 2). Immunohistochemical evaluation of eight tumours showed a more intense positivity for keratin in the retiform areas, whereas the gonadal stromal component had a more intense expression of inhibin. Inhibin stains Leydig cells strongly and hepatocytes moderately. Rete ovarii epithelium was positive for keratin and vimentin in the five cases studied, and for inhibin in one case. Follow-up was available on 13 patients. Three tumours behaved in a malignant fashion: one each was stage 1, 2, and 3 at diagnosis. CONCLUSIONS: Immunohistochemistry is useful in distinguishing retiform Sertoli-Leydig cell tumours from other tumours that they may resemble. Inclusion of inhibin is essential in a panel of antibodies to evaluate these tumours. The clinical behaviour of these neoplasms cannot always be predicted from their morphology or clinical stage.     

Ovarian hepatoid yolk sac tumours: morphological, immunohistochemical and ultrastructural features

AIM: The clinicopathological, immunohistochemical and ultrastructural features of two ovarian hepatoid yolk sac tumours (H-YST) from our files are reviewed. METHODS AND RESULTS: Using avidin-biotin-peroxidase complex technique, the immunoprofile of these tumours was compared to that of a classic yolk sac tumour and to that previously reported for hepatocellular carcinomas. The clinicopathological and morphological features of our cases are similar to the seven previously reported ovarian cases. This rare germ cell tumour occurs in young females (mean age = 17.6 years) and presents most commonly with abdominal pain and a large ovarian mass (average size = 140 mm). Histologically, the tumours display a striking resemblance to hepatocellular carcinoma. The absence of an associated typical pattern of yolk sac tumour or other germ cell neoplasm may make it difficult to recognize the germ cell origin of this lesion. Our cases demonstrated positive staining for alpha-fetoprotein and alpha-1-antitrypsin. In addition, there was immunoreactivity with polyclonal carcinoembryonic antigen (CEA) antiserum in a canalicular pattern, focal staining for inhibin, oestrogen and progesterone receptors and absence of immunoreactivity for CK7 that contrasts with the immunophenotype of a usual yolk sac tumour. CONCLUSIONS: Ovarian H-YST and hepatocellular carcinoma share a similar immunoprofile. Ovarian H-YST is a highly aggressive tumour, most patients exhibit recurrence or die of disease within 2 years of diagnosis.     

Non-small cell lung carcinomas with neuroendocrine features. A light microscopic, immunohistochemical and ultrastructural study of 11 cases

Eleven resected primary lung carcinomas classified as large cell carcinomas or squamous cell carcinomas, but showing some microscopic resemblances to bronchial carcinoid and small cell carcinoma, were studied. All cases were neurone-specific enolase and protein gene product 9.5 positive, indicating neuroendocrine differentiation. Staining for bombesin, C-terminal peptide of human pro-bombesin and chromogranin was positive in some cases. Electron microscopy showed dense-core granules in six of seven cases investigated, the remaining case showing small granules of uncertain nature. All but one patient died within 15 months after operation. These data indicate that neuroendocrine differentiation in non-small cell carcinomas of the lung may in some cases be suspected on routine histology. The follow-up data suggest that the identification of these cases might have implications for prognosis and therapy, and consequently for diagnostic lung tumour classification.     

Clinicopathological features of primary hepatic diffuse large B-cell lymphoma: a report of seven cases and a literature review

We studied the imaging and histopathological features of primary hepatic diffuse large B-cell lymphoma in order to explore the clinicopathological features, diagnosis, differential diagnoses, and treatment. Immunolabelling was performed in seven cases of primary hepatic diffuse large B-cell lymphoma using histological and immunohistochemical techniques. The clinical manifestations; imaging, histopathological, and immunohistochemical features; treatment; and prognosis of primary hepatic diffuse large B-cell lymphoma were observed and analyzed in light of the relevant literature. The average age of the seven patients was 63.4 years. Moreover, bulge of the upper right abdomen and progressive athrepsia and anemia were observed in all seven patients. Computed tomography (CT) revealed the presence of multiple solid hypodense lesions. Further, CT also revealed an enhanced irregular focus. Histopathological analysis revealed the following characteristics: heavy infiltration composed mainly of medium-sized round cells with a lightly stained cytoplasm, prominent nucleoli and vesicular nuclei, nuclear fission and visible sky star phenomena. The tumor cells showed diffuse expression of CD19, CD20, and CD79a, with the percentage of Ki67-positive cells being 75%-80%. All these findings indicated that primary hepatic diffuse large B-cell lymphoma is rare and generally has a poor prognosis. Biopsy and immunohistochemical staining are helpful in its diagnosis. Further, the differential diagnoses include secondary liver diffuse large B-cell lymphoma, low/undifferentiated cancer of the liver, hepatoblastoma, leukemia of the liver, and other tumors. Early surgery and chemotherapy can have a good curative effect.