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Objective@#To assess clinical features and treatment outcomes in immunocompetent patients with primary central nervous system lymphoma (PCNSL).@*Methods@#Sixty-two patients with PCNSL who attended Guangdong General Hospital between January 1998 and January 2012 were included. Survival curves were estimated using Kaplan-Meier survival methodology and statistical significance of continuous was assessed via the Cox proportional hazard model.@*Results@#The median age of the patient cohort was 56 years, and the male to female ratio was 1.14∶1.00. The common presentations were increased intracranial pressure symptoms and neuron damage. Performance status of 54 (54/62, 87.1%) patients were the international prognostic index (IPI) 0-2. Diffuse large B-cell lymphoma (57/62, 91.9%) was most common, and the rest were T-cell lymphoma (4/62,6.4%) and extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (1/62, 1.6%). In the series, 32 patients (32/62, 51.6%) had multiple lesions. Involvement of deep structures was found in 30 (30/62, 48.4%) patients. An elevated serum LDH level was detected in 19 (19/62, 30.6%) patients and the Ki-67 index was ≥90% in 38 (38/62, 61.3%) patients. Univariate analysis showed patients who were female, age<60 years, had WHO Eastern Cooperative Oncology Group performance status grade 0-2, single lesion, absence of deep structures involvement and normal LDH level showed better 2-year survival rate and longer median survival time. Significance was only seen in the normal LDH level group. Multivariate Cox regression analysis revealed that radical surgery only and Rituximab+ high-dose of methotrexate+ whole brain radiation therapy (WBRT) were independent prognostic indicators in PCNSL patients (P<0.05).@*Conclusions@#PCNSL is a rare but aggressive tumor with poor prognosis. Patients treated with high-dose of methotrexate combining with rituximab, followed by WBRT have a better prognosis and longer survival time, and thus these could probably be a promising treatment.
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Objective@#To investigate MAML2 gene-translocation in primary pulmonary mucoepidermoid carcinoma (PMEC) and pulmanary adenosquamous carcinoma, and the optimal diagnostic immunohistiochemical (IHC) panel in distinguishing PMEC from adenosqumous carcinoma.@*Methods@#Twenty-four cases of PMEC and 44 adenosqumous carcinoma diagnosed in the Guangdong General Hospital were tested for MAML2 translocation by fluorescent in-situ hybridization (FISH) using tissue array. An IHC panel including TTF1, Napsin A, CK5/6, p63, p40 and Ki-67 was performed on the cohort. The clinical data for all cases were collected and all PMEC patients had follow-up information.@*Results@#The patients′ age ranged form 6 to 73 years, with a median age of 32 years. The male to female ratio was 1.4∶1.0. MAML2 translocation was found in 16/24 (66.7%) cases of PMEC whereas all 44 cases adenosqumous carcinoma were negative for translocation. All the cases of the PMEC were negative for TTF1 and Napsin A but positive for CK5/6, p63 and p40 in the intermediate cells and epidermal-like cells. In most PMEC cases, the Ki-67 expression index was lower than 10%. In contrast, most cases of adenosqumous carcinomas expressed TTF1 and Napsin A in the adenomatous component and CK5/6, p63 and p40 in the squamous component, which expression pattern was different from that of PMEC. Based on IHC staining, 2 cases of highly invasive ALK-positive adenocarcinoma mimicing PMEC were also found in the study.@*Conclusions@#MAML2 gene translocation can be detected in about two-third of PMEC. Translocation of MAML2 gene and lower morphology grading are associated with good prognosis. The combined use of IHC antibodies panel is helpful to distinguish PMEC from the adenosqumous carcinoma and adenocarcinoma mimicing PMEC.
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Objective@#To investigate the clinicopathological features, treatment and prognosis of duodenal-type follicular lymphoma.@*Methods@#Four cases of duodenal-type follicular lymphoma diagnosed at Guangdong General Hospital from 2014 to 2015 with detailed clinical data were included. The histomorphology, immunophenotype, treatment and prognoses were analyzed.@*Results@#The patients′ age ranged from 51 to 57 years (mean 54 years), and there were 2 males and 2 females. The involved sites were gastric fundus in one case, second portion of the duodenum in two cases and terminal ileum in one case. All patients presented with multiple mucosal granules or nodules at endoscopy. Microscopically, there were multiple mucosal neoplastic follicles, constituting grade 1-2 disease based on nodal follicular lymphoma grading system. The tumor cells were positive for CD20, CD10, bcl-6 and bcl-2. CD21 highlighted the follicular dendritic meshwork mainly at the periphery of the follicles. Proliferation index was low. Three patients received rituximab monotherapy for 4 cycles, leading to complete remission. One patient refused therapy and the disease progressed to systemic lymphoma 15 months after the initial diagnosis.@*Conclusions@#Duodenal-type follicular lymphoma is a special variant of follicular lymphoma with indolent clinical course. The tumor exhibits morphology of low grade follicular lymphoma with characteristic dendritic meshwork at the periphery of the follicles and a low proliferation index. Prognosis is excellent. Rituximab monotherapy is treatment of choice, but a small minority of patients may progress to systemic disease.
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Objectives@#To investigate the clinicopathological features, therapy and prognosis of primary cardiac CD5-positive diffuse large B-cell lymphoma with C-MYC and bcl-2 double expression.@*Methods@#Two cases diagnosed at Guangdong Provincial People′s Hospital were included, the clinical data were collected; the tumor morphology, immunophenotypic profiles, therapy and prognosis were analyzed.@*Results@#Case 1 was a 55-year-old man and case 2 was a 61-year-old women. Intraoperatively, both cases showed large masses in the right atrium or ventricle, involving adjacent tissue. Pathologically, the tumors were composed of diffusely infiltrating large lymphoid cells with high mitotic activity and apoptosis. The tumor cells were positive for CD20, CD5, bcl-6, MUM1, C-MYC and bcl-2, and the Ki-67 index was equal or greater than 90%. Case 1 had bcl-6, but not bcl-2 or MYC gene rearrangements. No MYC, bcl-2 or bcl-6 gene rearrangements were detected in case 2. Case 1 defaulted chemotherapy after operation and died 1 month after diagnosis. Case 2 was treated with 4 cycles of rituximab, cyclophosphamide, doxorubicin, vincristine and prednisone (R-CHOP) therapy after surgery and attained partial remission, and was then treated with apatinib and ibrutinib, and remained stable 18 months after initial diagnosis.@*Conclusion@#Primary cardiac CD5-positive diffuse large B-cell lymphoma with C-MYC and bcl-2 double expression usually shows large infiltrative mass in the right atrium or ventricle, non-germinal center like immunophenotype and high proliferation index, and this may contribute to the aggressiveness of primary cardiac lymphoma.
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Objective@#To evaluate the expression of βF1 and T cell receptor (TCR)γ in T lymphoblastic lymphoma/leukemia(T-LBL/ALL), and investigate the clinicopathological features.@*Methods@#Fifty-one cases of T-LBL/ALL were collected at Guangdong General Hospital from 2010 to 2016, the expression of βF1 and TCRγ was assessed by immunohistochemistry.@*Results@#There were 13 cases of children and adolescents, and 38 cases of adults. The expression rates of βF1 and TCRγ were 27.5%(14/51) and 15.7%(8/51) respectively. The proportion of adults in αβ T-LBL/ALL, TCR-silent T-LBL/ALL and γδ T-LBL/ALL was 7/14, 79.3%(23/29)and 8/8 respectively, and the difference was significant (P=0.023). There was no statistical difference in sex, LDH, bone marrow involvement and Ann arbor stage among these three groups(P>0.05). γδ T-LBL/ALLs included 6 cases of CD4-/CD8- phenotype, whereas αβ T-LBL/ALL included 7 cases of CD4+ /CD8+ phenotype. There was significant difference in CD4/CD8 expression among these three groups(P<0.01).@*Conclusions@#γδ T-LBL/ALL occurred only in adults, with predominantly CD4-/CD8- phenotype. αβ T-LBL/ALL occurred more common in children and adolescents, with predominantly CD4+ /CD8+ phenotype.
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Objective To analyze the pathologically confirmed pulmonary Actinomycosis in the 11 patients in focusing on clinical features and mis-diagnostic reasons so as to improve physicians' awareness of this rare disease and reduce the misdiagnosis.Methods We retrospectively reviewed the medical records of 11 cases with pathologically confirmed pulmonary Actinomycosis during January 2003-August 2015.The clinical data and main causes of misdiagnosis in these cases were collected and analyzed.Results The study included 11 patients with a mean age of(53.0 ± 11.6.0)years.Among the 11 cases,8 (72.7 ％) patients had complications,6 (54.5 ％) were current or ex-smokers.Main clinical manifestations of 11 cases were cough(11/11,100.0 ％),sputum(11/11,100.0 ％),hemoptysis (7/11,63.6％),chest pain(6/11,54.5％)and fever(3/11,27.3％).Ten patients presented with one lobe of lung lesions,including 4 patients in the lower lobe and 3 in the upper lobe of the left lung,2 in the upper lobe and 1 in the lower lobe of the right lung.While,the remained one case presented with lesion locating in right main bronchus.Iconography often presented as pulmonary mass shadow,consolidation shadow,spicule sign,lobulation sign,hilar and/or mediastinal lymphadenopathy and pleural effusion.Vacuolar lesions were observed in some of the focuses.Flexible bronchoscopy was performed in 8 (72.7％)patients.Among them,7 patients showed mucosal swelling and congestion,luminal occlusion with purulence secretion,2 cases with polypoid neoplasm.Initial misdiagnosis rate were 100％ (11/11),among which 7 cases were misdiagnosed as lung cancer,2 cases as fungus infection,and 1 case as pulmonary tuberculosis and 1 case as pneumonia,respectively.All patients were definitely diagnosed by biopsy finding an evidence of hyphae of Actinomycosis in lung tissue specimens.The definitive diagnosis was made by CT-guided percutaneous lung biopsy in 4 cases,by transbronchial lung biopsy (TBLB)in 5 cases and by thoracotomy or video-assisted thoracoscopic surgery(VATS) in 1 case respectively.Actinomycosis in most patients was cured with high-dose penicillin administration over a prolonged period.Conclusions The diagnosis of pulmonary Actinomycosis remains challenging via its non-specific clinical symptoms and iconography features,and the presence of comorbidity may further increase the difficulty and complexity of diagnosis,leading to delaying-or mistaking-diagnosis.Obtaining positively pathological specimens is diagnostic key.Transbronchial lung biopsy through a bronchoscope and CT-guided percutaneous needle biopsy are the priority methods.
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<p><b>OBJECTIVE</b>To evaluate the diagnostic value of HGAL and LMO2 expression and compare with CD10 and bcl-6 in follicular lymphoma (FL).</p><p><b>METHODS</b>63 cases of FL were collected from Guangdong General Hospital. The expression of HGAL, LMO2, CD10 and bcl-6 was assessed by immunohistochemistry.</p><p><b>RESULTS</b>The expression rates of HGAL, LMO2, CD10 and bcl-6 were 98.4% (62/63), 82.5% (52/63), 82.5% (52/63) and 87.3% (55/63), respectively. The expression rate of HGAL was higher than those of LMO2, CD10 and bcl-6, but the differences were not significant (P>0.05). There was no significant difference in HGAL, LMO2 and bcl-6 expression among FL1, FL2 and FL3 cases. The CD10 expression rate of FL1-3A cases was significantly higher than that of FL3B cases(P<0.01).</p><p><b>CONCLUSIONS</b>HGAL and LMO2, especially HGAL, can be used in FL particularly high grade FL as useful germinal center marker.</p>
Subject(s)
Humans , Adaptor Proteins, Signal Transducing , Metabolism , Biomarkers, Tumor , Metabolism , Germinal Center , Immunohistochemistry , LIM Domain Proteins , Metabolism , Lymphoma, Follicular , Metabolism , Neoplasm Proteins , Metabolism , Neprilysin , Metabolism , Proto-Oncogene Proteins , Metabolism , Proto-Oncogene Proteins c-bcl-6 , MetabolismABSTRACT
<p><b>OBJECTIVE</b>To study the clinical, pathologic, immunophenotype, molecular characteristics and prognosis of HIV-negative plasmablastic lymphoma (PBL).</p><p><b>METHODS</b>Twelve cases of HIV-negative PBLs diagnosed between 2005 and 2014 in Guangdong General Hospital were identified according to WHO classification of tumors of haematopoietic and lymphoid tissues (2008). The clinicopathologic features and outcome were analyzed and the relevant literatures were reviewed.</p><p><b>RESULTS</b>The patients were predominantly male (11/12) with a median age of 55.5 years. The tumor cells showed the characteristic combination of immunoblastic/plasmablastic morphology, plasma cell phenotype and high proliferation, no expression of mature B cell markers. 7/10 of the cases were EBER positive. Two cases were positive for C-myc translocation. Four of twelve patients were died.</p><p><b>CONCLUSIONS</b>PBL is a rare, aggressive B-cell lymphoma. HIV-negative PBL has lower rate of oral involvement and EBER expression than HIV-positive patients, the differential diagnosis is very challenging, and the prognosis is worse.</p>
Subject(s)
Female , Humans , Male , Middle Aged , Diagnosis, Differential , HIV Seronegativity , Immunophenotyping , Lymphoma, B-Cell , Diagnosis , Plasma Cells , Classification , Plasmablastic Lymphoma , Diagnosis , Pathology , Prognosis , Translocation, GeneticABSTRACT
Objective To investigate the clinical characteristics of pulmonary actinomycosis and to improve the understanding of the clinical misdiagnosis of pulmonary actinomycosis. Method Clinical data of 4 pulmonary actinomycosis patients were analized and related literatures were reviewed. Results All the 4 pulmonary actinomy-eosis cases were misdiaguosed as lung cancer. Pulmonary actinomycosis can be correctly diagnosed through lung bi-opsy by CT-guided percutaneous paracentesis,fiberoptic,bronchoscopy,or pathological examination of the resected pulmonary lobe. Conclusions Pulmonary actinomycosis is a rare lung infection caused by fungus,and is liable to be misdiagnosed as peripheral lung cancer. The capacity of distinguishing pulmonary actinomycosis from peripheral lung cancer needs to be improved.
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<p><b>OBJECTIVE</b>To study the immunophenotype and differential diagnosis of Hodgkin's lymphoma (HL).</p><p><b>METHOD</b>Fifty six cases originally diagnosed as HL were re-evaluated according to lymphoma classification of WHO 2000 on paraffin sections using SP immunohistochemistry.</p><p><b>RESULTS</b>Among the 56 cases, 47 met the WHO criteria for HL, 8 were NHL and 1 metastatic tumor. Of the 47 HL cases, 2 were nodular lymphocyte predominant HL (NLPHL), 43 classical Hodgkin's lymphoma (CHL) and 2 unclassified HL, and of the 8 cases reclassified as NHL, 6 were T-cell rich B-cell lymphoma (TCRBCL) and 2 anaplastic large cell lymphoma (ALCL). In NLPHL cases, L&H cells were CD(20)(+), CD(15)(-) and CD(30)(-); CD(57)(+) cells and small B-lymphocytes predominated the background infiltration. Diagnostic R-S cells and other tumor cells in 43 cases of CHL were positive for CD(30) (100%), CD(15) (81%) and CD(20) (7%). Six cases of TCRBCL were negative for CD(15) and CD(30). Two cases of ALCL were positive for CD(30), ALK-1 and CD(3), and negative for CD(15) and CD(20). The reactive inflammatory infiltration in CHL and TCRBCL was rich in TIA-1 positive cytotoxic lymphocytes, and CD(57)(+) cells were rarely encountered.</p><p><b>CONCLUSION</b>Combining the immunophenotype of tumor cells and background cells with morphologic criteria are more helpful for classification of HL, and discrimination between NLPHL, CHL and TCRBCL.</p>
Subject(s)
Humans , Antigens, CD , Diagnosis, Differential , Hodgkin Disease , Allergy and Immunology , Metabolism , Pathology , Immunohistochemistry , Immunophenotyping , Membrane Proteins , Mucin-1 , Poly(A)-Binding Proteins , Proteins , RNA-Binding Proteins , T-Cell Intracellular Antigen-1ABSTRACT
<p><b>OBJECTIVE</b>To study the diagnosis and the differential diagnosis of nodular lymphocyte-predominant Hodgkin's lymphoma (NLPHL).</p><p><b>METHODS</b>245 cases of Hodgkin's lymphoma (HL) diagnosed between 1980 and 2000 from 3 hospitals in Guangzhou were reviewed. Four cases of NLPHL were confirmed according to the WHO classification of lymphoid neoplasms. Among the other 3 cases of NLPHL, 2 collected from other clinical centers and 1 from Fudan University Cancer Hospital. Immunohistochemistry (IHC) were performed on paraffin sections through SP technique using a panel of markers to define the large neoplastic cells (CD45, CD20, CD15, CD30 and vimentin) as well as the non-neoplastic background cells (CD3, CD20, CD45RO, CD57, CD68 and TIA-1).</p><p><b>RESULTS</b>Seven patients with NLPHL were 4 males and 3 females, age 29 to 70 years, average 43.8 years. All patients had lymphadenopathy. Histologically, in NLPHL, instead of the structure of normal lymph nodes, the tumor tissue became nodular in architecture. Characteristic lymphocytic and histiocytic (L&H) cells with scant cytoplasm and large multilobulated nuclei distributed among a predominant population of small lymphoid cells. The large cells exhibited a CD45+, CD20+, but CD15-, CD30- and vimentin-phenotype. The background cellularity was relatively rich in B cells and the majority of T-cells infiltrated were CD57(+) cells. TIA-1+ cells were few.</p><p><b>CONCLUSIONS</b>NLPHL can be diagnosed according to the morphologic and immunophenotypic features rather than by morphology alone. It is important to distinguish this tumor from its morphologic mimics, such as lymphocyte-rich classical Hodgkin's lymphoma (LRCHL) and T-cell rich B-cell lymphoma (TCRBCL). The immunophenotype of neoplastic cells and background cells are the helpful criteria for the differential diagnosis.</p>