Clinicopathologic features and prognosis of T lymphoblastic lymphoma associated with Langerhans cell histiocytosis / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the clinicopathologic features, immunophenotype and molecular genetic changes of T lymphoblastic lymphoma (T-LBL) associated with Langerhans cell histiocytosis (LCH).</p><p><b>METHODS</b>Three cases of T-LBL associated with LCH were included. The morphologic characteristics were reviewed along with immunohistochemical profiling using EnVision method and TCR gene rearrangement by PCR. A review of composite lymphoma previously reported in the literature was performed.</p><p><b>RESULTS</b>All three patients were male with the mean age of 61.7 years. One was Hans and the other 2 were Uyguers. All presented with superficial lymph node enlargement. Biopsy of lymph node showed two abnormal cell populations: distended sinus by large, pale histiocytes with nuclear grooves, and the interfollicular region containing immature-appearing cells with irregular nuclei slightly larger than that of small lymphocyte, dispersed chromatin, inconspicuous nucleoli, scant cytoplasm, and scattered mitotic figures. These cells presented in aggregates and small sheets interspersed with normal-appearing lymphocyte. The histiocytes were positive for CD1a, S-100 protein and CD68. The lymphoma cells were positive for CD3, CD7, TdT and CD34. TCR-γ gene rearrangement was detected in one case by PCR technology. One case involved bone marrow with double phenotype acute leukemia. Amongst the 8 including 5 reported cases, there were 4 males and 4 females. The mean age of the patients and the median age were 54 years. Lymphoadenopathy was the most common presentation. Bone marrow was involved in 4 cases. The time of follow-up was 2 to 27 months. The median survival was 5.5 months and the one-year survival rate was 33.3%.</p><p><b>CONCLUSIONS</b>Diagnosis of T-LBL and LCH should be based on typical morphology, immunophenotype and molecular genetic findings, with differential diagnoses including Langerhans cell hyperplasia originated from dermatopathic lymphadenopathy. When involving lymph node, extensive sampling supplemented by immunohistochemical staining is important to reach a correct diagnosis. Although coexistent T-LBL and LCH is clonally related, the understanding of its pathogenesis requires further investigation.</p>

Clinicopathological significance of Sox17 and β-catenin protein of Wnt in oligodendroglioma / 中华病理学杂志

<p><b>OBJECTIVE</b>To investigate the expression of Sox17 and β-catenin proteins in oligodendroglioma, and its clinical significance.</p><p><b>METHODS</b>One hundred cases of oligodendroglioma of different grades and 10 cases of surrounding benign tissue from First Affiliated Hospital of Xinjiang Medical University from 2003 to 2013 were assessed by immunohistochemistry for Sox17 and β-catenin protein expression. The clinicopathologic characteristics and outcome of patients with oligodendroglioma were evaluated by Kaplan-Meien and Cox regression analyses.</p><p><b>RESULTS</b>Sox17 was expressed in 10/10, 82% (41/50) and 62% (31/50) of normal control, oligodendroglioma and anaplastic oligodendroglioma, respectively. β-catenin was expressed in 2/10, 22% (11/50), and 52% (26/50) of normal control, oligodendroglioma and anaplastic oligodendroglioma, respectively. The differences of Sox17 and β-catenin expression between normal control and different types of oligodendroglioma were statistically significant. Univariate analysis showed that the expression of Sox17 protein (P = 0.000), β-catenin protein (P = 0.033), tumor position (P = 0.001), radiotherapy (P = 0.077), and chemotherapy (P = 0.000) were significant prognostic factors.</p><p><b>CONCLUSIONS</b>Oligodendrogliomas with expression of Sox17 protein, but not β-catenin, have better prognosis. Evaluation of Sox17 and β-catenin protein expression is important for accurate pathological diagnosis, prognostication and guiding treatment.</p>

Clinicopathologic analysis of 52 cases of thymic epithelial tumor / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the clinicopathologic characteristics of thymic epithelial tumors and to evaluate the diagnostic reproducibility and clinical relevance of the 2004 WHO histologic classification system.</p><p><b>METHODS</b>The morphology and immunophenotype of 52 cases of thymic epithelial tumor were reviewed. The tumors were classified according to the new WHO classification system and the clinical data were analyzed.</p><p><b>RESULTS</b>Of the 52 cases studied, 45 were thymomas and 7 were thymic carcinomas. Amongst the 45 cases of thymoma, 6 (13.4%) were type A, 15 (33.3%) were type AB, 4 (8.9%) were type B1, 9 (20.0%) were type B2, 9 (20.0%) were type B3 and 2 (4.4%) were metaplastic thymoma. Amongst the 7 cases of thymic carcinoma, 6 were squamous cell carcinomas and 1 was neuroendocrine carcinoma. The commonest presentations were cough and chest pain. Some cases were incidentally discovered by routine physical examination. Thirteen cases (25.0%) of thymoma were associated with myasthenia gravis. CT scan showed that 49 cases (94.2%) were located in the anterior mediastinum. All cases of type A, AB and B1 thymoma and most cases of B2 thymoma appeared as well-defined homogeneous mass, whereas a few cases of type B2 thymoma and most cases of type B3 thymoma and thymic carcinoma were poorly demarcated and heterogeneous. According to Masaoka staging system, 20 cases (41.7%) belonged to stage I, 15 cases (31.3%) stage II, 11 cases (22.9%) stage III and 2 cases (4.1%) stage IV. The histologic subtypes of thymic epithelial tumors significantly correlated with the clinical stages (chi(2) = 32.5, P < 0.01).</p><p><b>CONCLUSIONS</b>The 2004 revision of WHO histologic classification system for thymic epithelial tumors shows a high degree of reproducibility. Correlation with the radiologic, clinical and prognostic parameters is helpful in determining the management strategy for individual patients.</p>