Clinicopathological features of T-lymphoblastic lymphoma with Langerhans cell histiocytosis in the same lymph node / 中华病理学杂志

Objective@#To investigate the clinicopathological features, immunophenotypes, genetics and prognosis of T-lymphocyte lymphoma/myeloid sarcoma combined with Langerhans cell histiocytyosis (coexistence of T-LBL/MS and LCH).@*Methods@#Clinical and pathological data of the 6 patients with coexistence of T-LBL/MS and LCH were analyzed, who were diagnosed at the Foshan Hospital of Sun Yat-sen University and the Friendship Hospital of Capital Medical University, from December 2013 to April 2019. The hematoxylin and eosin stain, immunohitochemistry (EnVision) and in situ hybridization were used. Related literatures were reviewed.@*Results@#Four patients were T-LBL combined with LCH, 1 was T-LBL/MS combined with LCH, and 1 was MS combined with LCH. There were 2 male and 4 female patients, with age ranged from 5 to 77 years old (median, 59 years old). Three patients represented with only multiple lymph node swelling. The other 3 displayed both multiple lymph node swelling, and skin/liver or spleen lesions. Lymph node structure was destroyed in 5 cases, while 3 cases had several residual atrophic follicles. Histologically, there were two types of tumor cells: one type of the abnormal lymphoid-cells exhibited small to medium-sized blast cells, typically showing a nested distribution, and these cells were mainly identified in residual follicles and paracortical areas; the other type of histiocytoid cells had a large cell size and abundant pale or dichromatic cytoplasm. Their nuclei were irregularly shaped, showing folded appearance and nuclear grooves. These cells were mainly present in marginal sinus, medullary sinus and interstitial area between follicles. Eosinophil infiltration in the background was not evident in any of the cases. The lymphoid-cells of medium size showed TdT+/CD99+/CD7+, with variable expression of CD34/MPO/CD2/CD3. Ki-67 index was mostly 30%-50%. However, the histiocytoid cells showed phenotype of CD1a+/S-100+/Langerin+/-, while CD163/CD68 were positive in some degree. These cells did not express any T or B cell markers. The Ki-67 index mostly ranged between 10%-20%. None of the cases had Epstin-Barr viral infection. Among the 6 patients, 4 patients were followed up (6-63 months, median time, 18.5 months), of whom 1 patient died of the disease and 3 patients were alive at the end of follow-up.@*Conclusions@#T-LBL/MS combined with LCH is a rare mixed type of immature hematopoietic disease, and mainly occurs in lymph node and skin. The clinical course is overall aggressive. Therefore, it is helpful to recognize and identify the two pathologic components in the same tissue for accurate diagnosis and proper treatment.

Classification for primary cutaneous lymphoproliferative disorders: an update / 中华皮肤科杂志

Primary cutaneous lymphoproliferative disorders are a group of heterogeneous diseases that mainly involve the skin and are completely different from lymph node lymphomas. Although rare, they are still an important part of lymphoid and hematopoietic tumors. In the 2017 World Health Organization (WHO) classification for tumors of hematopoietic and lymphoid tissues and the 2018 updated version of WHO-European Organization for the Research and Treatment of Cancer (EORTC) classification for cutaneous lymphomas, classifications of most diseases do not change, but the understanding of some diseases such as Epstein-Barr virus-associated lymphoproliferative diseases and primary cutaneous CD4 positive small/medium T-cell lymphoproliferative disorder has been changed, and new diseases have been added. This article introduces updates on the classification of primary cutaneous lymphoproliferative disorders based on the 2017 WHO and 2018 WHO-EORTC classification systems.

Paediatric nodal marginal zone lymphoma: a clinicopathological study of seven cases / 中华病理学杂志

Objective@#To investigate the pathological features and clinical manifestation of pediatric nodal marginal zone lymphoma(NMZL).@*Methods@#Histological morphology and immunophenotype of 7 cases of pediatric NMZL were retrospectively reviewed at Beijing Friendship Hospital Affiliated to Medical University between January 2008 to October 2018. Clonal rearrangement analysis was performed. Clinical information including patient follow-up data were analyzed.@*Results@#All 7 patients were male with a median age of 15 years aged from 10 to 26 years. All patients presented with only lymph node enlargement without B symptoms, including cervical lymph node (5 cases), preauricular lymph node (1 case) and retroauricular lymph node (1 case). Histologically, all cases showed irregular large follicles on the edges with widened marginal areas and intervesicular areas, and lesional cells were uniform with progressive transformation of germinal center centers along with a small amount of intrinsic lymphoid tissue. All 7 cases showed diffuse CD20 positivity both follicle and interfollicular region along with 30%-40% positivity in the interfollicular region (pathological region). Markers of other B-cell lymphomas werenot expressed. All 7 cases were positive for immunoglobulin(Ig) gene rearrangement. None of the patients showed no recurrence up on after follow-up for an average of 13 months.@*Conclusions@#Pediatric NMZL is a rare type of lymphoma that has a unique morphology and occurs almost exclusively in male children and young adults and often in head and neck lymph nodes. It has an excellent prognosis. Therefore, awareness of the disease with accurate diagnosis is important.

Clinicopathological features of long-term tumor-free survival of patients with untreated primary tonsillar diffuse large B-cell lymphoma / 中华病理学杂志

Objective@#To investigate the clinicopathological features of long-term tumor-free survival in patients with untreated primary diffuse large B-cell lymphoma (DLBCL) of the tonsil.@*Methods@#The study included 80 consultation cases of primary tonsillar DLBCL from April 2006 to July 2017 in the Department of Pathology, Beijing Friendship Hospital, Capital Medical University. The patients were divided into two groups: experimental groups of 10 untreated patients with long-term tumor-free survival, and 70 patients who had been treated (control group). The clinical data, histopathological features, immunohistochemical staining, and molecular biology test results of the patients were analyzed retrospectively.@*Results@#Patients who had long-term tumor-free survival with untreated primary diffuse large B-cell lymphoma had the disease mostly confined to the tonsil. Biopsy showed that the tonsil structure was only partially effaced and the lesions were relatively "fresh". EBER and FISH test for t (14;18) results were negative. Gene rearrangement detection showed monoclonality. There was statistically significant difference between the age, bcl-2 expression, CMYC protein expression and co-expression of CMYC and bcl-2 between the untreated group and the treated group(P<0.05). Patient gender, tumor site, histological type and clinical stage showed no difference between the untreated group and the treated group (P>0.05); The median overall survival of the untreated group and treated group was 81 months and 20 months, respectively, and the difference was not statistically significant (P>0.05).In patients younger than 40 years of age, the untreated group had a statistically significant difference in primary site and CMYC protein expression compared with the treated group (P<0.05), and there was no statistical significance in other aspects.@*Conclusions@#Long-term tumor-free survival patients with untreated tonsillar primary DLBCL have relatively unique clinical characteristics. There is no significant difference in the prognosis between the untreated and treated groups, indicating radiotherapy and chemotherapy may not be required and therefore, avoiding related side effects.

Clinicopathological features of follicular lymphoma in children / 中华病理学杂志

Objective@#To investigate the clinicopathologic features of follicular lymphoma (FL) in children.@*Methods@#One female and one male patients with FL diagnosed in the First College of Clinical Medical Science, China Three Gorges University and Beijing Friendship Hospital of the Capital University of Medical Science in February 2016 and June 2015 were studied by HE immunohistochemistry, EBER in situ hybridization, IgH and IgK gene rearrangement analysis and IRF4 fusion gene detection.@*Results@#The two patients′ age were 6.3 and 12 years, respectively. The lesions involved head and neck lymph nodes with duration of more than 2 months. Histopathologically, the lesions consisted of nodular proliferation of lymphoid follicles with diffuse distribution of large cells. Starry sky phenomenon was seen in one of the two cases. Immunohistochemistry showed that one case was positive for bcl-2 and MUM1, but negative for bcl-6 and CD10. Ki-67 index was>50% and oligoclonal IgK rearrangement was observed. The second case showed positivity for bcl-6, and CD10 but negative for bcl-2. Ki-67 index was>50% and clonal IgH FR1-JH and IgH FR2-JH rearrangements were detected. Both cases showed no evidence of IRF4 gene fusion.@*Conclusions@#Childhood FL is a rare B-cell lymphoma with characteristic features and high-grade histomorphology. However, its immunophenotype and molecular genetic characteristics are divergent.

Classification for primary cutaneous lymphoproliferative disorders: an update / 中华皮肤科杂志

Primary cutaneous lymphoproliferative disorders are a group of heterogeneous diseases that mainly involve the skin and are completely different from lymph node lymphomas.Although rare,they are still an important part of lymphoid and hematopoietic tumors.In the 2017 World Health Organization (WHO) classification for tumors of hematopoietic and lymphoid tissues and the 2018 updated version of WHO-European Organization for the Research and Treatment of Cancer (EORTC) classification for cutaneous lymphomas,classifications of most diseases do not change,but the understanding of some diseases such as Epstein-Barr virus-associated lymphoproliferative diseases and primary cutaneous CD4 positive small/medium T-cell lymphoproliferative disorder has been changed,and new diseases have been added.This article introduces updates on the classification of primary cutaneous lymphoproliferative disorders based on the 2017 WHO and 2018 WHO-EORTC classification systems.

Clinicopathologic features and prognosis of gastrointestinal mantle cell lymphoma / 中华病理学杂志

Objective@#To investigate the clinicopathologic features and prognosis of gastrointestinal mantle cell lymphoma (GI-MCL).@*Methods@#Clinical data of 38 GI-MCL patients diagnosed at Beijing Friendship Hospital from January 2002 to January 2016 were retrospectively reviewed morphologically and immunophenotypically. IgH/CCND1 gene fusion was assessed by fluorescent in situ hybridization (FISH). For comparison, 60 cases of non-GI-MCL were randomly selected to extract the differences inclinicopathological features and patient survival between the two groups.@*Results@#Of 38 patients with GI-MCL, the median age was 62 years (range: 35-78 years, 23 males and 15 females), of which patients of 60 years of age or older accounted for 55.3%. Patients with clinical course of less than 6 months accounted for 81.1%(30/37). The main symptoms included abdominal pain, diarrhea, anorexia and hematochezia. Those with B symptoms accounted for 32.4%(12/37). The tumor most often involved lleocecal region (57.9%, 22/38), followed by rectum (36.8%, 14/38) and sigmoid colon (28.9%, 11/37), and the stomach accounted for 18.4%(14/38). Endoscopic polypoid lesions were found in 33 cases (86.8%, 33/38), of which 22 cases (66.7%, 22/33) were multiple. Five cases (13.2%, 5/38) presented with local protuberant neoplasm. According to Ann Arbor staging, 3 cases (7.9%, 3/38) were at stage Ⅰ, 4 cases (10.5%, 4/38) were at stage Ⅱ, and 31 cases (81.6%, 31/38) were at stage Ⅳ. The number of patients with tumor involvement of abdominal and retroperitoneal lymph nodes accounted for 45.7%(16/35), including 41.7%(15/36) involving the superficial lymph node, 17.1%(6/35) involving extranodal sites, and 23.5%(8/34) having splenomegaly. All of the 38 cases were classic MCL, and the tumor was composed of uniform lymphoid cells and effacing normal mucosal structure. All tumors were positive for CD20 and CD5. 97.4% (37/38) tumors were positive for cyclin D1, and 92.0% (23/25) tumors were positive for SOX11. FISH test was positive in 1 case of cyclin D1 negative tumor. Twenty-eight patients (73.7%) had a median follow-up of 25.0 months (range: 3-79 months). The 3-year survival rate for stage Ⅰ-Ⅱ and stage Ⅲ-Ⅳ of patients were 80.0% and 69.1%, respectively (P> 0.05). The 3-year survival rate for GI-MCL and non-GI-MCL patients were 71.7% and 72.5%, respectively (P>0.05). Single factor analysis showed that age of >60 years and splenomegaly were correlated with a worse overall survival rate (P<0.05).@*Conclusions@#Gastrointestinal malaise is the most common presenting symptom in GI-MCL patients. GI-MCL more commonly involves colorectum with more frequent multiple polypoid lesions. Patients of age >60 years and with splenomegaly have poor prognosis. There is no difference in the prognosis between GI-MCL and non-GI-MCL patients.

Cut-off value of Ki-67 labeling index in the pathologic grading of follicular lymphoma / 中华病理学杂志

Objective@#To determine the cut-off values of Ki-67 labeling index (LI) in the histological grading of follicular lymphoma (FL).@*Methods@#Clinicopathological data of 350 FL patients diagnosed at Beijing Friendship Hospital from June 2014 to January 2016 were analyzed retrospectively by quantitative evaluation and statistical analysis of Ki-67 LI.@*Results@#Of the 350 patients with FL, the male and female ratio was 1.1 and the average age was (50.2±14.0) years with a median age of 51 years (range 4 to 82 years). The tumors were graded as grade Ⅰ-Ⅱ in 215 cases (61.4%), grade Ⅲ A in 105 cases (30.0%), and grade Ⅲ B in 30 cases (8.6%). The average Ki-67 values were (22.8%±8.3%) for the FL low (grade Ⅰ-Ⅱ) and (50.4%±10.7%) for high grade (Ⅲ A and Ⅲ B) and were statistically significant by Mann Whitney U test (P<0.01). Receiver operated characteristic curve analysis showed that the best diagnostic cut-off value of low grade FL was 35% (sensitivity of 96.3% and specificity of 93.3%) with the largest area under curve (AUC=0.990, P<0.01, 95%CI for 0.982-0.998). According to the analysis of four lattice diagnostic tests, Ki-67 LI >40% was an important factor (χ2=230.733, P<0.01) in predicting high grade FL. When the cut-off value of Ki-67 LI was set at 40%, high grade LF could be diagnosed with the greatest sensitivity (98.1%) and specificity (87.7%). Moreover, a significant correlation between the Ki-67 LI and the pathological grade of FL (r=0.836, P<0.01) was observed.@*Conclusions@#Ki-67 LI of below a cut-off value of 35% is a reliable indicator of low grade FL.Ki-67 over 40% is consistent with high grade FL. These Ki-67 cut-off values may serveas an important auxiliary indicator in the grading of FL.

Clinicopathologic characteristics of Burkitt-like lymphoma with chromosome 11q aberration / 中华病理学杂志

Objective@#To analyze clinical, pathological, molecular and genetic characteristics of Burkitt-like lymphoma with chromosome 11q aberration.@*Methods@#A case of Burkitt-like lymphoma with 11q aberration was presented at Beijing Friendship Hospital in November 2016 with detailed clinicopathological features, immunophenotypes, Epstein-Barr virus(EBV) status and molecular genetic characteristics.@*Results@#The patient was a 38-year-old man presenting with the cervical lymphadenopathy. In morphology, the tumor had the similar characteristics of Burkitt lymphoma, including diffuse infiltration of medium to large lymphoid cells, and presence of"starry sky"phenomenon. Immunophenotypically, the tumor cells were positive for CD20, CD10, bcl-6, but negative for bcl-2. MUM-1 showed weak and patchy positivity. Ki-67 index was more than 95%. C-MYC expression was seen in about 50% of tumor cells. EBV in situ hybridization was negative. IgH and IgK genes were clonally rearranged.Fluorescence in situ hybrization detection using MYC break probe was negative but ATM gene amplification on chromosome 11q was detected. The patient did not receive any chemotherapy or radiotherapy and had not recurrence during the 10 months follow-up.@*Conclusion@#Burkitt-like lymphoma with chromosome 11q aberration has similar clinical, morphological and immunological characteristics to classic Burkitt′s lymphoma.

Primary testicular NK/T cell lymphoma: a clinicopathologic analysis of six cases / 中华病理学杂志

Objective@#To evaluate the clinicopathological features, diagnosis and management of primary testicular NK/T cell lymphoma (NKTCL).@*Methods@#Six cases of primary testicular NKTCL at Beijing Friendship Hospital, Capital Medical University from January 2007 to December 2016 were retrospectively analyzed for the morphology, immunephenotype and outcome, and relevant literature was reviewed.@*Results@#The median age of patients at diagnosis was 45 years(range 32-65 years). All patients presented with testicular masses as initial symptoms (6/6), five cases (5/6) were on the right. The lesions were confined to the testis. All patients were classified as Ann Arbor stage Ⅰ but the tumors exhibited aggressive clinical behavior. Two patients died of the disease within two months, three (3/6) had clinical remission, and one (1/6) was lost to follow-up. Morphologically, the lymphoma cells showed a diffuse growth pattern that largely effaced the interstitial tissues, and surrounded seminiferous tubules in all cases. There was also a prominent angioinvasive pattern, with focal necrosis and karyorrhexis(4/6). Cytologically, the medium-sized neoplastic cells showed scanty to moderate amount of cytoplasm and irregular folded nuclei. The immunophenotype was similar to that of nasal NKTCL: the neoplastic cells were positive for cytoplasmic CD3, CD56, cytotoxic molecules and EBV-encoded small RNA, the loss of CD5 antigen was seen in all cases.@*Conclusions@#Primary testicular NKTCL is extremely rare, highly aggressive and is associated with a poor prognosis. There is no unified standard of treatment. Thus, at the time of diagnosis of testicular lymphoma, NKTCL should be included in the differential diagnosis.

Clinicopathologic characteristics and prognositic indicators of tonsillar mantle cell lymphoma / 中华病理学杂志

Objective@#To investigate clinicopathological features and prognosis of tonsillar mantle cell lymphoma(TMCL).@*Methods@#Clinical data of 25 patients with TMCL at Beijing Friendship Hospital, Capital Medical University from 2002 to 2016 were included. All the cases were reviewed microscopically. Various immunohistochemical stains were performed using the MaxVision two-step method. IgH/CCND1 gene fusion was detected by fluorescent in situ hybridization(FISH). Additionally, randomly selected 40 cases of non-tonsil MCL of the same period were compared.@*Results@#Among all mantle cell lymphomas (MCL), TMCL accounted for 5.6%(25/449). The median age of the patients was 60 years(range: 44-82 years) with a M∶F ratio of 5.3 to 1.0. The main symptoms were sore throat and foreign body sensation and patients usually presented with enlargement or mass of tonsil. At the early stage of the disease, 18 cases(72.0%) were clinically misdiagnosed as tonsillitis. Lymph node involvement was present in 76.0%(19/25) of the patients. There were 4 cases(16.0%)with current splenic involvement, 11 cases(44.0%) with pharyngeal focal recidivism, and 3 cases(12.0%) with involvement of other non-lymphoid organs. Morphologically, tonsillar architectures were effaced at various degrees. Eighteen MCL cases showed classical type and 7 cases were blastoid variant. All tumors were positive for CD20 and cyclin D1. 92.0%(23/25) tumors showed weakly positive or positive expression for CD5. FISH test that IgH/CCND1 gene fusion was positive in two CD5 negative classical cases. 18 patients(72.0%) had a median follow-up time of 26 months(range: 6-81 months). The difference of survival rate between stage Ⅰ-Ⅱ and stage Ⅲ-Ⅳ patients was not statistically significant(P>0.05). Compared with NTMCL, TMCL was found to have higher proportion of stage Ⅰ-Ⅱ disease (χ2=12.789, P<0.01), lower the proportion of non-lymphatic organ involvement (χ2=8.125, P<0.01), and better prognosis (χ2=4.351, P=0.037).@*Conclusion@#The incidence of TMCL is low and prone to be misdiagnosed as tonsillitis. Patients with TMCL are more likely at stage Ⅰ-Ⅱ at presentation and the prognosis is better than that of NTMCL.

Clinicopathologic features and prognosis of mantle cell lymphoma: an analysis of 349 cases / 中华病理学杂志

Objective@#To investigate clinicopathologic features and prognostic factors of mantle cell lymphoma(MCL).@*Methods@#The clinical data of 349 MCL patients diagnosed at Beijing Friendship Hospital from January 2004 to January 2016 were retrospectively collected. Corresponding histological sections were reviewed. Additional studies included immunohistochemical staining using the MaxVision two-step method, IgH/CCND1 fusion gene detection by fluorescent in situ hybridization (FISH), and correlative statistical analysis.@*Results@#Of 349 patients with MCL, the median patient age was 61 years (range: 25-83 years, M∶F=2.7∶1.0) and the age of 243 patients ranged from 51 to 70 years (69.6%). Those with B symptoms accounted for 22.4% (70/313). Most of the patients presented with superficial lymphadenopathy and the clinical stage Ⅲ-Ⅳ accounted for 76.1% (235/309). Extranodal involvement was seen in 47.9% (148/309), among which the gastrointestinal tract accounted for 31.8% (47/148) and splenic involvement accounted for 15.4% (47/305). Three hundred and nine (88.5%) cases were of classical type and 40 (11.5%) cases were of aggressive variant type, and all were composed of proliferating lymphoid cells. All the tumors were positive for CD20 and cyclin D1, and 98.6% (344/349) tumors were weakly positive or positive for CD5. FISH test was positive in 12 cases that were CD5 negative and with cyclin D1 partial expression.Two hundred and forty-three (69.6%) patients had a median follow-up of 26 months (range: 3-108 months). The 3- and 5-year overall survival rates for patients were 63.0% and 34.8%, respectively. Single factor analysis showed that age of >60 years, splenic involvement, aggressive variant type, incompletely overlapping type [Based on the degree of overlap ≥90% and <90% between the follicular dendritic cell (FDC) meshwork and tumor cells, the tumors were divided into the completely overlapped type and incompletely overlapped type] and Ki-67 index >40% had poor prognosis (P<0.05). Multiple factor Cox proportional risk regression analysis after removing the aggressive variant type showed that age, splenic involvement, the degree of overlap between the FDC meshwork and tumor cells and Ki-67 index were independent prognostic factors for overall survival rate of MCL patients (P<0.05).@*Conclusions@#MCL is more commonly found among middle-aged and elderly men. Patient age, splenic involvement, degree of overlap between FDC meshwork and tumor cells and Ki-67 index are the independent prognostic indicators for MCL.

EB virus-positive T/NK lymphoproliferative diseases: an analysis of 156 patients / 中华病理学杂志

Objective@#To investigate the clinicopathological features of EBV-positive T/NK cell lymphoproliferative diseases (EBV+ T/NK-LPD).@*Methods@#The clinical characteristics of 156 cases of EBV+ T/NK-LPD were collected from August 2002 to March 2015 at Beijing Friendship Hospital, Capital Medical University. Immunohistochemical staining, EBER in situ hybridization and clonal analysis of TCR gene were performed. All patients were followed up.@*Results@#There were 106 male and 50 female patients; patients′ age ranged from 1 to 75 years (median 20 years). The course of the diseases before diagnosis ranged from 2 to 540 months (median 20 months). Fever was noted in 122 patients (78.2%), 108 patients had lymphadenopathy (69.2%), and 75 patients had hepatosplenomegaly (48.1%). Thirty-three cases were grade 1, 68 cases were grade 2, and 55 cases were grade 3. TCR gene arrangement analysis was performed in 45 cases, and 33 cases (73.3%) showed clonal rearrangement. The follow-up period ranged from 1-134 months, and 44 patients (28.2%) died. There was a trend of increased death rate associated with increasing grade (P>0.05).@*Conclusions@#There are many types of EBV+ T/NK-LPD, and they can be classified as systemic, relatively localized and localized. The prognosis should be based on a comprehensive analysis of pathology and clinical data. There is no significant correlation between morphological grade and mortality. An important goal of therapy is to prevent serious complications.

Clinicopathologic characteristics and prognosis of neoplastic cell-rich mixed cellularity classic Hodgkin lymphoma / 中华病理学杂志

Objective@#To investigate the clinicopathologic characteristics of neoplastic cell-rich mixed cellularity classical Hodgkin lymphoma(MCCHL-R) and to compare the prognosis with typical mixed cellularity classic Hodgkin lymphoma(MCCHL).@*Methods@#Fifty-four patients with MCCHL-R(the tumor cells >10%) and 65 patients with typical MCCHL identified from 1 721 Hodgkin lymphomas were reviewed to compare the clinicopathological characteristics including morphologic and immunophenotypic features, EBV infection status, clinical therapy and overall survival.@*Results@#The median age of the patients of MCCHL-R was 28.5 years(range: 9-76 years, male∶female=1.6∶1.0). Twenty-seven patients(50.0%) had B symptoms. Most patients had cervical lymph node involvement(81.5%, 44/54). Mediastinum and spleen involvement were seen in 69.2%(36/54) and 24.1%(13/54), respectively. Extranodal non-lymphoid organ involvement was seen in 41.3%(19/46) cases. Morphologically, lymph node architectures were effaced at various degree with large neoplastic cells of variable morphology, including Hodgkin/Reed-Sternberg(H/RS) cells and anaplastic large cells. There were abundant background heterogeneous admixtures of non-neoplastic inflammatory and accessory cells that were predominant mature small lymphocytes. All tumors were positive for CD30 and weakly positive for PAX5. Epstein-Barr encoded RNA(EBER)detectable by in situ hybridization was seen in 39.0% cases. Forty-six patients had a median follow-up time of 32.5 months(range: 5-128 months) and the 5-year survival rate for stage Ⅰ-Ⅱ and stage Ⅲ-Ⅳ patients were 91.7% and 50.1%, respectively(P<0.05). The 5-year survival rate for MCCHL-R was lower than typical MCCHL patients. Single factor analysis showed that age of >45 years, extranodal involvement and stage Ⅲ-Ⅳ were correlated with poorer 5-year survival rate(P<0.05). Multiple factors Cox proportional hazards regression showed that extranodal involvement was the independent prognostic factor(RR: 4.352, 95%CI: 1.122-16.879, P<0.05).@*Conclusions@#MCCHL-R is more common in young people. The tumor has pathological features of classic Hodgkin lymphoma enriched with the tumor cells(>10%) and similar immunophenotype to classical Hodgkin lymphoma. Compared with typical MCCHL, extranodal disease is an independent prognostic factor of MCCHL-R.

Pathologic subtyping of primary lymphoma of breast and prognostic analysis / 中华病理学杂志

Objective@#To investigate the pathological types and prognostic factors of primary lymphoma of breast (PLB).@*Methods@#The clinical pathological data of 115 cases of PLB during October 2006 to October 2016 were retrospectively analyzed, and the basic clinical and pathological data, pathology types and the immunohistochemical slides by EliVision two-step method for staining were summarized.@*Results@#Almost all the patients were women (113/115), and the median age was 52 years old (range: 27 to 81 years old). The main symptom was painless progressive mass in breast. Ten cases (8.7%) showed B symptoms. The masses were mainly confined to the unilateral breast (80.9%, 93/115), of which 22 cases showed axillary lymph nodes enlargement in the same side. The average diameter of masses was 3.0 cm (range from 0.5 to 9.0 cm). There is no differences between the sides (left or right). Pathologically, 106 cases (92.2%) were mature non-Hodgkin′s B-cell lymphomas, of which there were mainly diffuse large B cell lymphoma (DLBCL, 64.3%) and mucosa associated lymphoid tissue (MALT) extranodal marginal lymphoma (17.4%). Five cases (4.4%) were mature T/NK cell lymphomas, including extranodal nasal NK/T cell lymphoma (1.7%), peripheral T-cell lymphoma non-specific type (0.9%), subcutaneous panniculitis-like T cells lymphoma (0.9%) and undivided (0.9%). Four cases were lymphoblastic lymphoma. According to Ann Arbor staging criteria, 93 cases were stage ⅠE (6 cases were stage ⅠEB), 22 cases were stage ⅡE (4 cases were stage ⅡEB). Ninety-two cases were followed 1 to 122 months (median: 36 months). The five-year overall survival rate was 85.3%, and 13 patients dead. B symptom was one of the factors that affect the prognosis (P<0.05), but the pathological type has no relationship with the prognosis (P>0.05).@*Conclusions@#PLB is relatively rare, the main clinical manifestation is painless mass, which is difficult to distinguish with breast carcinoma. The most common type is DLBCL, followed by MALT lymphoma, while T cell lymphoma is rarely seen. PLB is early stage tumor with good prognosis, while patients with B symptom turn out to suffer worse prognosis.

Mucosa-associated lymphoid tissue lymphoma in children and adolescents: a clinicopathologic study of 5 cases / 中华病理学杂志

Objective@#To investigate the pathological features and clinical manifestations of mucosa-associated lymphoid tissue (MALT) lymphoma in children and adolescents.@*Methods@#Five cases of MALT lymphoma in children were investigated by morphology and immunophenotyping along with clinical follow-up.@*Results@#Five cases of MALT lymphoma occurred in the antrum, orbit, parotid gland and nasopharynx. All patients had no B symptoms and only one patient showed a local mass with ulcer. One case presented with cervical lymph node enlargement, and 4 cases showed no evidence of lymphadenopathy.All cases had pathological features similar to those of adult MALT lymphoma, with proliferation of monocytoid B cells orcentralcyte-like tumor cells, with plasma cell differentiation and lymphoid epithelial lesions.No specific immunophenotypic marker was found. Clonal Ig gene rearrangement was detected in two cases.One patient was treated with rituximab treatment, 1 patient was given anti-Helicobacter pylori therapy, and 2 patients had no additional treatment.The follow-up data showed that 4 patients survived without tumor recurrence.@*Conclusions@#Similar to adult patients, MALT lymphoma in children and adolescents has similar pathological features with indolent clinical course and good prognosis. It is important to note that misdiagnosis and incorrect diagnosis mightoccur because of the young age of the patients.

Discrepancies in pathologic evaluation of dyaplasia/early cancer in 60 gastric endoscopic submucosal dissection specimens / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the reasons for the discrepancies in pathologic diagnosis of gastric dysplasia/early cancer in endoscopic submucosal dissection (ESD) specimens, and how to cope with the discrepancies.</p><p><b>METHODS</b>The pathologic diagnoses in 60 cases of ESD specimens according to the three currently used classification systems (namely Western criteria, Japanese criteria and Vienna classification) were compared. The diagnostic discrepancies were analyzed.</p><p><b>RESULTS</b>Fifteen of the 17 cases diagnosed as low-grade intraepithelial neoplasia according to the Western criteria were revised as adenoma by the Japanese criteria. Amongst the 43 cases of gastric intramucosal adenocarcinoma diagnosed according to the Japanese criteria, 23 cases had concordant diagnosis by the Western criteria. While the diagnosis of low-grade intraepithelial neoplasia/adenoma was basically similar irrespective of classification system used, there were significant differences in the interpretation of gastric early cancer.</p><p><b>CONCLUSIONS</b>The diagnostic discrepancies in the gastric dysplasia/early cancer are mainly related to the morphologic criteria applied in different classifications. In order to facilitate clinical and pathologic communication, a consensus using Vienna/WHO classifications, supplemented with Japanese system, is desirable.</p>

Clinicopathologic analysis of angioimmunoblastic T-cell lymphoma with Hodgkin/Reed-Sternberg-like cells / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the clinicopathologic features and pathologic diagnosis and differential diagnosis of angioimmunoblastic T-cell lymphoma with HRS-like cells.</p><p><b>METHODS</b>Six cases of angioimmunoblastic T-cell lymphoma with HRS-like cells were examined histologically and immunohistochemically (EliVision method) and in-situ hybridization for Epstein-Barr virus-encoded RNA (EBER), and the literature was reviewed.</p><p><b>RESULTS</b>The cytologic and microscopic features of these imprints and lymph node samples showed a heterogeneous population of hematolymphoid cells, including small to intermediate lymphoid cells, immunoblasts, plasma cells, dendritic cells, and eosinophils, as well as small vessels that were surrounded by some of the abnormal cells. The neoplastic T-cells expressed CD3 and CD5 and partly positive for CD10 and bcl-6, CD21 showed expanded and irregular follicular dendritic cell (FDC) meshworks that surrounding the high HEV. The HRS-like cells were positive for MUM-1 and Ki-67, variable intensity positive for CD30, CD20, and PAX-5, but negative for CD15. EBV-positive cells included HRS-like cells and small to large-sized neoplastic T-cells, which formed small clusters or scattering in the background of the disease.</p><p><b>CONCLUSIONS</b>The clinical course of angioimmunoblastic T-cell lymphoma with HRS-like cells is aggressive. Which present with histomorphology overlap with classical Hodgkin lymphoma (CHL), similar to CHL in EBER and immunophenotype, however, it is easy to misdiagnosis as HL. Thus, angioimmunoblastic T-cell lymphoma pathology diagnosis should comprehensive analysis of different kinds of materials, including clinical features, and histological structure, and EBER, and immunophenotype, and gene rearrangement.</p>

Significance of CD123 in histiocytic necrotizing lymphadenitis / 中华病理学杂志

<p><b>OBJECTIVE</b>To investigate the mode of presentation, cytologic features of the plasmacytoid dendritic cells (pDC), and the expression of CD123 and its significance in Kikuchi's disease.</p><p><b>METHODS</b>CD123 expression was evaluated by EliVision immunohistochemical staining in formalin-fixed and paraffin-embedded tissues from 30 cases of Kikuchi's disease, 5 cases of T cell lymphoma, 10 cases of reactive lymphoid hyperplasia and 10 cases of chronic tonsillitis.</p><p><b>RESULTS</b>Clusters of CD123 positive PDC were observed in Kikuchi's disease (28 of 30 cases, 93.3%) and the staining intensity was more prominent in the PDC at the periphery of the lesion and around the high endothelial venule-like vessels. CD123 showed three staining patterns: membranous (10 cases, 33.3%), cytoplasmic (10 cases, 33.3%), and membranous and cytoplasmic (8 cases, 26.7%). In the control group, CD123 showed cytoplasmic staining in reactive hyperplasia and chronic tonsillitis. Regarding the staining intensity, 12 of 28 cases (42.9%) were 3+ for CD123, 8 of 28 cases (28.6%) were 2+, and 8 of 28 cases (28.6%) were 1+. In contrast, PDC clusters with 1+ staining intensity were observed in 1 of 10 cases of reactive lymphoid hyperplasia; 2 of 10 chronic tonsillitis diseases; and much less in T cell lymphoma.</p><p><b>CONCLUSIONS</b>Large cluster of PDC is detected in both proliferative and necrotizing types of Kikuchi's disease, making this a useful adjunctive diagnostic marker.</p>

A20 and TNF genes aberration in ocular adnexal MALT lymphomas / 白血病·淋巴瘤

Objective To investigate the copy number changes of A20 and TNF genes,and determine the contribution of the two genes in the development of ocular adnexal MALT lymphoma.Methods Forty-one cases of archive paraffin-embedded ocular adnexal MALT lymphoma tissues were detected by interphase fluorescence in situ hybridization (FISH) using the commercial chromosome 6 centromere probe (CEP6),and house-made site-specific probe of A20 and TNF. Results Of the 41 ocular adnexal MALT lymphoma cases,loss of heterozygosity (LOH)in A20 locus was detected in 2 cases (4.88 ％).TNF extra copies were found in 5 cases (12.20 ％),of which three cases simultaneously had extra CEP6 signals.No A20 deletion were found coexistence with TNF extra copies in any case.Conclusion A20 gene deletion is present in the small part of ocular adnexal MALT lymphoma, and might contribute to the development of Chinese ocular adnexal MALT lymphoma.A20 deletion is not associated with extra copies of TNF locus.