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.

Expression of myocyte enhancer factor 2B in mantle cell lymphoma and its clinical significance / 中华病理学杂志

Objective@#To investigate the expression of myocyte enhancer factor 2B (MEF2B) in mantle cell lymphomas (MCL), and to analyze the correlation between the expression of MEF2B and pathological subtypes, structural subtypes, SOX11 expression and its clinical significance.@*Methods@#Paraffin-embedded tissues were stained with HE, immunohistochemistry (EnVision method) and fluorescence in situ hybridization (FISH) , in addition, the clinical and pathological data of 60 cases of MCL were collected at Sun Yat-sen University Foshan Hospital and Sun Yat-sen University Cancer Center from January,2002 to May, 2019 for analysis.@*Results@#Of the 60 MCLs, males is predominant (M∶F=3∶1). Histologically, the typical MCL is the majority (classical MCL: variant type MCL=48 cases:12 cases) . Fifty cases were classified into non-complete FDC meshwork type MCL, and the remaining 10 cases were classified into the complete-FDC meshwork type MCL group. Patients with classical MCL were more than 60 years old. The coexistent lesion sites both node and extranode in pathological subtype or structural subtype was the most common lesion sites. SOX11(+) MCL was common in classical MCL (P=0.040) and tended to be complete-FDC meshwork type MCL (P=0.086). The expression rate of MEF2B in MCL was 60.0%(36/60). This rate of MEF2B in classical type, complete-FDC meshwork type and SOX11(+) MCL was significantly higher than that variant type, no complete-FDC meshwork type, SOX11(-)MCL (P<0.05), respectively. There was no difference in clinical characteristics of MCL between MEF2B positive and negative groups. Compared with SOX11(-)MCL, the percentage of MEF2B expressed in tumor cells of SOX11(+)MCL was significantly higher (P=0.027). The expression of MEF2B was not related to the proliferation of tumor cells (P=0.341). There was no significant difference in the survival rate between different expression groups of MEF2B and SOX11 (P=0.304 and P=0.819, respectively). Only the mortality of variant type (blastoid/pleomorphic) MCL within 2 years was significantly higher than that of classical type MCL (P<0.05).@*Conclusions@#The expression of MEF2B in MCL is related to the pathological subtypes, structural subtypes and the expression of SOX11, but not to the proliferation and prognosis. The high mortality rate within 2 years is only found in variant MCL. However, the role of MEF2B in MCL needs to be further studied.

Clinicopathologic features of primary mucosal CD30-positive T-cell lymphoproliferative disorders in head and neck region / 中华病理学杂志

Objective@#To study clinicopathologic features, prognosis and differential diagnoses of primary mucosal CD30-positive T-cell lymphoproliferative disorders of the head and neck(mCD30+ TLPD-head and neck).@*Methods@#Three cases of mCD30+ TLPD-head and neck were collected from January 2014 to April 2017 at Sun Yat-Sen University Foshan Hospital. A literature review of mCD30+ TLPD of head and neck was provided.@*Results@#All three cases presented with either bulging/exophytic nodule or mucosal ulcer/erosion. Morphologically, the tumor consisted of diffuse proliferation of uniform, large atypical mononuclear lymphoid cells that showed irregular or polymorphic nuclei with small nucleoli, and abundant pale or amphophilic cytoplasm. Hallmark cells with eccentric, horseshoe, kidney-like, or doughnut-shaped nuclei were present. While mitotic figures were present, no tumor necrosis was found. Eosinophilc infiltration was obvious in the background. The atypical large lymphoid cells had a immunophenotype of CD30+ /CD3+ /CD4+ /CD56- along with positive cytotoxic molecule. While being negative for EBER/ALK/CD20/CD8, TCR rearrangement was found in 2 out of 3 cases. Three patients were cured after excision without relapse and metastasis.The two patients with TCR rearrangement didn′t show aggressive clinical course.@*Conclusions@#mCD30+ TLPD-head and neck is a rare benign lymphoproliferative disorder with spontaneous regression. It should be differentiated from cutaneous CD30+ anaplstic large cell lymphoma, lymphomatoid papulosis, and EBV-related mucocutaneous ulcer. Correct recognition of mCD30+ TLPD of head and neck is important to avoid overtreatment.

Analysis of mature T-cell and NK-cell lymphoma with CD30 expression based on latest WHO classification / 中华病理学杂志

<p><b>OBJECTIVE</b>To investigate the frequency of different types of mature T- and NK-cell lymphomas diagnosed in a 4-year period at Sun Yat-sen University Cancer Center, and to study baseline CD30 for potential anti-CD30 targeted therapy in mature T- and NK-cell lymphoma.</p><p><b>METHODS</b>All cases of mature T- and NK-cell lymphoma diagnosed at Sun Yat-sen University Cancer Center from September 1, 2009 to August 31, 2013, were reviewed. Paraffin-blocks of available 164 consecutive cases were stained for CD30 immunohistochemistry using EnVision protocol.</p><p><b>RESULTS</b>A total of 625 cases of mature T- and NK-cell lymphomas were diagnosed and the most common type was extranodal NK/T cell lymphoma (ENKTL), nasal type 319 (51.0%) cases, followed by angioimmunoblastic T-cell lymphoma (AITL) (119 cases, 19.0%), peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS) (81 cases, 13.0%), and anaplastic large-cell lymphoma (ALCL), including 48 cases (7.7%) of systematic ALCL and 11 cases (1.8%) of primary cutaneous ALCL. Besides ALCL, ENKTL had the highest expression rate of CD30 among the 164 cases, with positivity observed in 41 cases (62.1%, 41/66). Only 1 case of PTCL-NOS was CD30 positive. CD30 was not expressed in all 28 cases of AITL and other rare types of mature T- and NK-cell lymphoma.</p><p><b>CONCLUSIONS</b>The frequency of different types of mature T- and NK-cell lymphoma encountered at Sun Yat-sen University Cancer Center was similar to that seen in other areas of China and other Asia countries. CD30 expression is different among several types of mature T- and NK-cell lymphoma. In addition to ALCL, ENKTL has the highest expression rate of CD30, which may be a candidate disease for anti-CD30 targeted therapy.</p>

Retrospective study of clinicopathological characteristics in bronchopulmonary carcinoid / 中国肺癌杂志

<p><b>BACKGROUND AND OBJECTIVE</b>Bronchopulmonary carcinoid (BPC) account for less than 2% of all primary lung malignant tumors, but few related studies were reported. The aim of this study is to analyze this rare disease's clinicopathological characteristics.</p><p><b>METHODS</b>The clinical data of 28 patients with BPC in Cancer Center of Sun Yat-sen University, from January 1994 to June 2009, were enrolled into retrospective analysis. First, the corresponding paraffin blocks reexamined, slice up and stained, multiple pathologists re-consulted, and its subsets (typical carcinoid, TC; atypical carcinoid, AC) defined. Second, the clinical characteristics and immunohistochemical markers and its relationship with prognosis were analyzed.</p><p><b>RESULTS</b>First, the 5-year survival for overall and TC, AC was 56% and 70%, 41% respectively in 28 cases. The markers CD99, Bcl-2 and Ki-67 expression correlated significantly with the BPC subsets (P = 0.017, P = 0.043, and P = 0.033 respectively). Further univariate analysis revealed that advanced TNM staging (P = 0.037), lymph node metastasis (P = 0.001) and Ki-67 nucleolus's positive expression (P = 0.009) are poor prognostic factors. Second, the overall, TC, AC 5-year survival rate was 73%, 83%, 57% respectively in 20 cases underwent the radically surgical resection. Further univariate analysis revealed that AC subset (P = 0.013), lymph node metastasis (P = 0.004) and Ki-67 nucleolus's positive expression (P = 0.006), advanced TNM staging (P = 0.047) are poor prognostic factors in this 20 cases. Third, as univariate analysis, local recurrence and metastasis (n = 4) correlate significantly with Ki-67 nucleolus's and Bcl-2 positive expression (P = 0.027, 0.045, respectively).</p><p><b>CONCLUSION</b>The prognosis of BPC was better than other types of primary lung cancer. Ki-67, Bcl-2 high expression and advanced TNM staging are the poor recurrence and prognostic factors of BPC. The radical surgery remains the treatment of choice for resectable candidates in BPC as NSCLC.</p>

Association of Epstein-Barr virus with lymphoepithelioma-like carcinoma of the parotid gland / 临床与实验病理学杂志

Purpose To investigate the relationship between Epstein-Barr virus (EBV) and lymphoepithelioma-like carcinoma (LELC) of the parotid gland and detect the gene expression products of EBV harbouring in LELC cells. Methods Thirty-two parotid LELCs were collected from the Departments of Pathology, Sun Yat-sen University of Medical Sciences, Guangzhou, China during the period of January 1986 and December 1995. All the 32 formalin-fixed paraffin-embedded blocks had been consecutively re-sectioned again. Immunohistochemical and in situ nucleic acid hybridization methods for detection of EBV gene encoded products were performed. Results (1) 32 LELCs were found out of 125 parotid gland carcinomas, the frequency was 25.6% (32/125). (2) All of the 32 specimens contained a variable number of EBNA-1 and EBERs positive neoplastic cells. (3) Twenty-seven out of 32 specimens (27/32, 84.4%) had a portion of carcinoma cells expressing LMP-1. (4) No ZEBRA positive cell could be found. (5) EA-D, VCA and MA positivity rates for these 32 parotid LELCs reached to 71.9%(23/32), 68.8%(22/32), and 12.5%(4/32), respectively. Conclusions (1) The parotid gland LELC is frequently to be seen in Guangzhou locale of China, where is a high-incidence area of nasopharyngeal carcinoma (NPC). The parotid gland LELC and NPC are co-prevalent in Guangzhou locale. (2) This disease is also consistently associated with EBV infection. (3) The EBV infection of the parotid gland LELCs is essentially the type of latency Ⅱ, expressing EBNA-1, EBERs and LMP-1. (4) The latent infected EBV harbouring in LELC cells could in part be switched over to lytic cycle, producing EA-D, VCA or/and MA.