Diffuse large B-cell lymphoma with aberrant expression of CD56: a clinicopathologic and immunohistochemical study / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the clinicopathologic features and significance of aberrant CD56 expression in diffuse large B-cell lymphoma (DLBCL).</p><p><b>METHODS</b>The clinical and pathologic profiles of 10 cases of DLBCL with aberrant expression of CD56 were investigated. Immunohistochemical staining, in-situ hybridization for Epstein-Barr virus encoded RNA and gene rearrangement for IgH and Igκ were carried out.</p><p><b>RESULTS</b>There were 6 male and 4 female patients. The medium age of patients was 46 years. All of them presented with extranodal lymphoma involvement, with gastrointestinal tract being the commonest site (5/10). Histologic examination showed that most of the atypical lymphoid cells were centroblast-like and demonstrated a diffuse growth pattern. Apoptosis and necrosis were identified in some cases. Immunohistochemical study showed that the tumor cells were positive for CD20 or CD79α and aberrantly expressed CD56. Five cases had the GCB phenotype while the remaining cases had the non-GCB phenotype, according to Hans classification. Bcl-6 was positive in most cases (9/10). All cases showed a high proliferation index by Ki-67. The tumor cells were negative for CD3ε, CD138 and granzyme B. In-situ hybridization for Epstein-Barr virus encoded RNA was performed in 7 cases and none of them showed positive signals. IgH gene rearranged bands were detected in 4 cases (4/6) and Igκ was detected in 3 cases (3/6). Follow-up data were available in 8 patients. Two patients died of disease progression within 5 to 13 months after diagnosis and the other 6 patients were alive 8 to 60 months after therapy.</p><p><b>CONCLUSIONS</b>DLBCL with aberrant expression of CD56 is rare. Most of them present with extranodal involvement, show high frequency of bcl-6 expression and high proliferation index. The patients often have good response to chemotherapy.</p>

Correlation of immunoglobulin variable heavy chain gene mutation status with prognosis in patients with mantle cell lymphoma / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the relationship between immunoglobulin variable heavy chain (IgVH) gene mutation status and clinical features, pathologic findings and biologic behavior of mantle cell lymphoma (MCL).</p><p><b>METHODS</b>IgVH gene was amplified in 60 cases of MCL with FR1-JH and FR2-JH primers in BIOMED-2. The sequence was determined by cloning. The IgVH somatic mutational status was analyzed using NCBI's Ig-Blast tool. The relationship between IgVH gene mutation status and clinicopathologic features was also analyzed.</p><p><b>RESULTS</b>Forty percent (24 cases, 28 functional Ig genes) of the MCL cases displayed somatically mutated VH genes (defined as > 2% mutated), whereas 60.0% (36 cases, 40 functional Ig genes) showed unmutated VH genes. The most widely used genes were VH3-21 (27.9%) and VH4-34 (19.1%). The former were mainly used by unmutated cases, while the later mainly by mutated cases.Intraclonal heterogeneity was noted in 19 cases. There was no correlation of VH mutation status and specific VH gene with survival (P > 0.05).</p><p><b>CONCLUSIONS</b>MCL comprises at least two subsets that do not correlate with morphology: one with unmutated VH genes and one with mutated VH genes. The biased use of VH3-21 and VH4-34 is noted. The nonrandom usage of IgVH segments suggests specific antigens may play a role in the pathogenesis and progression of MCL subsets. There is no correlation of IgVH mutation status and specific VH gene with survival.</p>

A clinicopathological analysis of 160 cases of adult Langerhans cell histiocytosis / 中华血液学杂志

<p><b>OBJECTIVE</b>To observe the clinical features of Langerhans cell histiocytosis (LCH), and to improve its early diagnosis and treatment.</p><p><b>METHODS</b>Retrospective analysis of 160 cases of adult LCH from pathology department, West China Hospital of Sichuan University and Union Hospital of Tongji Medical College of Huazhong University of Science and Technology from January 1992 to December 2013 were performed, and their clinical features were analyzed.</p><p><b>RESULTS</b>Of 160 cases, there were 110 male and 50 female, the male to female ratio was 2.2:1. The mean age was 35(18-73) years. There were total 222 lesion sites, including 172(77.5%) osteal lesions, followed by 13(5.8%) lymph nodes and 8(3.6%) oral cavity lesions. The other involved organs were skin(5, 2.2%), liver(5, 2.2%), fossa orbitalis(4, 1.8%), lungs(4, 1.8%), sternoclavicular joint(3, 1.4%), gastrointestinal(2, 0.9%), ear(2, 0.9%), and thyroid (2, 0.9%), adrenal gland (1, 0.5%) and sublingual gland (1, 0.5%). Of 160 cases, 150 (93.8%) had one organ involved while 10 (6.2%) had two or more organs involved. Clinically, 77 cases (48.1%) were misdiagnosed as bone tumors (28 cases, including giant cell tumor, fibrous dysplasia, chondroblastoma, osteoblastoma and osteosarcoma), bone tuberculosis (13 cases), meningioma(9 cases), bone cysts (5 cases), chronic osteomyelitis (5 cases) and diabetes insipidus (5 cases) , skin (4 cases) diseases malignant lymphoma (4 cases), chronic skin ulcers (4 cases), chronic otitis media (1 case), lung (1 case) and oral cancer (1 case).</p><p><b>CONCLUSION</b>In this group of the adult cases, the ratio of the male patients is higher. Adult LCH occurs predominantly in bone and presents mainly as unisystem single-focal disease, but multi-organ lesion and skin involvement are lower than that reported in the literatures. Just as LCH in children, adult LCH is also easy to be misdiagnosed. We should raise awareness of the disease and pathological examination is helpful for early diagnosis.</p>

Matrix-producing metaplastic carcinoma: a clinicopathologic and prognostic analysis of 16 cases with review of literature / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the clinicopathologic features, immunophenotype and prognosis of matrix-producing metaplastic carcinoma (MPC).</p><p><b>METHODS</b>Sixteen cases of MPC diagnosed between 2002 and 2012 in West China Hospital were identified. The clinicopathologic features were analyzed. Immunohistochemistry for E-cadherin, S-100 protein, CK5/6, HCK, PCK, CK7, CK8, p63, SMA, EMA, CD99, MSA, CK14, EGFR, ER, PR, HER2 and Ki-67 was performed with EnVision method. The clinical outcome was evaluated and compared to matched controls of invasive ductal carcinoma.</p><p><b>RESULTS</b>All patients were women and ranged in age from 29 to 69 years (median age 48 years). The median size of primary tumor was 4 cm. Most of the tumors were well-circumscribed with expansile and multinodular appearance. Histology showed invasive carcinoma with a direct transition from carcinoma to cartilaginous/chondromyxoid matrix without an intervening spindle cell component. Tumor distribution was either nodular or diffuse. The matrix component accounted for 10%-80% of the tumor volume. All the tumors were strongly positive for S-100 protein and basal-like cytokeratin with triple negative phenotype (ER, PR and HER2 negative). Alcian blue stain was positive for the cartilaginous/chondromyxoid matrix. Compared with invasive ductal carcinoma, patients with MPC had increased locoregional recurrence (P = 0.010), increased distant recurrence (P = 0.011) and shorter disease-free survival (P = 0.017).</p><p><b>CONCLUSIONS</b>MPC is a rare variant of mammary metaplastic carcinoma with unique characteristics of morphology and immunohistochemical staining pattern. This subtype seems to have aggressive biologic behavior.</p>

The roles of VEGF and C-myc in occurrence, development and metastasis of gallbladder carcinoma / 生物医学工程学杂志

To investigate the relationship of the expression of vascular endothelial growth factor (VEGF) and C-myc with the occurrence, development and metastasis of gallbladder carcinoma, the expression levels of VEGF and C-myc in gallbladder carcinoma tissue (n = 30) and in normal gallbladder tissue (n = 20) were examined by immunochemistry. Results show that the positive rates of VEGF and C-myc in gallbladder carcinoma tissue were 80% and 63.3% respectively, and 45% and 25% respectively in normal gallbladder tissue. The positive rates of VEGF and C-myc were significantly higher in gallbladder carcinoma than in normal gallbladder tissue. The expression of VEGF and C-myc in gallbladder carcinoma related to the metastasis of gallbladder carcinoma. VEGF and C-myc play important roles in the occurrence, development and metastasis of gallbladder carcinoma.

Clinicopathologic features of mantle cell lymphoma and the significance of cyclin D1 in diagnosis / 中华病理学杂志

<p><b>OBJECTIVE</b>To investigate the clinicopathologic features of mantle cell lymphoma (MCL) and the significance of immunostaining for cyclin D1 in diagnosis.</p><p><b>METHODS</b>Clinicopathologic observation and immunohistochemical staining for CD20, CD45RO, cyclin D1, bcl-2, Ki-67, CD5 for 8 cases of mantle cell lymphoma were performed.</p><p><b>RESULTS</b>The 8 cases of mantle cell lymphoma consisted of 6 males and 2 females, aged from 43 to 78 years (mean 57 years). Histopathologically, MCL demonstrated architectural destruction by a vaguely nodular monomorphic lymphoid proliferation with vaguely nodular, diffuse or mantle zone growth patterns. Analogous to centrocytes, the lymphoma cells with slightly to markedly irregular nuclear contours showed moderately dispersed chromatin and a low mitotic figure. Three cases were transformed into highly aggressive blastoid variants. The tumor cells were positive for CD20, CD5, bcl-2 and cyclinD1 in all 8 cases and negative for CD45RO.</p><p><b>CONCLUSIONS</b>The clinicopathological features and special immunophenotypes were present in mantle cell lymphoma. This tumor can be differentiated from other small B-cell lymphomas on the basis of histopathologic features and positive cyclin D1 immunophenotype. The blastoid variant should also be differentiated from other variants.</p>