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Aims: MYC rearrangements are the main cytogenetic alterations in plasmablastic lymphoma (PBL). We aimed to investigate the relationship between MYC rearrangement and the clinicopathological features of PBL. Methods and results: MYC rearrangements assessed in 13 unpublished single‐centre PBL cases, and an additional 85 cases from the literature, with reported MYC rearrangement information individualised by patient, were reviewed. In Asia, PBL was much less commonly diagnosed in human immunodeficiency virus (HIV)‐positive patients (27% versus 84%, P = 0.000), with older age (median age at diagnosis: 52 years versus 44 years, P = 0.046) and a lower EBV infection rate (56.8% versus 81.8%, P = 0.049), than in non‐Asian regions. Overall, MYC rearrangements were identified in 44 of 98 (44.9%) PBL cases, and IGH was the partner in almost all available cases (30/31, 96.8%), as confirmed with a MYC–IGH fusion probe. The MYC rearrangement rate in HIV‐positive cases (33/55, 60.0%) was significantly higher than that in HIV‐negative cases (11/38, 28.9%, P = 0.003). Patients with MYC rearrangement showed a trend towards an inferior median survival time (9.6 months versus 15.7 months, P = 0.122) and 2‐year overall survival (17% versus 32%, P = 0.238). Conclusions: MYC rearrangement was frequently identified in PBL patients, and IGH was the partner gene in an overwhelming majority of MYC rearrangements. In addition, the MYC rearrangement rate was significantly higher in HIV‐positive PBL patients than that in HIV‐negative patients. MYC rearrangement may play an important role in the pathogenesis of HIV‐positive PBL, but further studies are required to understand the underlying mechanisms. [ABSTRACT FROM AUTHOR]

Castleman disease (CD) is a rare lymphoproliferative disorder. To assess the clinical features, outcomes, and prognostic factors of this disease, we retrospectively analyzed 185 HIV‐negative CD patients from four medical centers in southern China. The median age was 37 years. One hundred and twenty‐one patients (65.4%) were classified as unicentric CD (UCD) and 64 patients (34.6%) were classified as multicentric CD (MCD). The histology subtype was hyaline‐vascular for 132 patients (71.4%), plasma cell for 50 patients (27%), and mixed type for 3 patients (1.6%). The 5‐year overall survival (OS) of 185 CD cases was 80.3%. All UCD patients underwent surgical excision, whereas the treatment strategies of MCD patients were heterogeneous. The outcome for UCD patients was better than MCD patients, with 5‐year OS rates of 93.6% and 51.2%, respectively. In further analysis of the MCD subgroup, a multivariate analysis using a Cox regression model revealed that age, splenomegaly and pretreatment serum albumin level were independent prognostic factors for OS. This multicenter study comprising the largest sample size to date suggested that MCD is a distinct entity from UCD with a significantly worse outcome. Older age (≥40 years), splenomegaly, and hypoalbuminemia were risk factors for poorer MCD prognosis. [ABSTRACT FROM AUTHOR]

Objectives: Primary intestinal T-cell and natural killer-cell lymphomas (PITNKLs) are aggressive and make pathologic diagnoses in biopsy specimens challenging. We analyzed different subtypes' clinicopathologic features and treatment outcomes., Methods: Seventy-nine PITNKL cases were characterized by clinical, morphologic, and immunohistochemical features., Results: Among 79 cases of PITNKLs from 2008 to 2017 in our institution, 40 (50.63%) were extranodal NK/T-cell lymphoma, nasal type (ENKTL); 32 (40.51%) monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL); 6 (7.59%) intestinal T-cell lymphoma, not otherwise specified; and 1 (1.27%) indolent T-cell lymphoma of the gastrointestinal tract. Small intestine (n = 47) was the most common site. Monomorphic epitheliotropic intestinal T-cell lymphoma showed distinctive clinicopathologic features from other subtypes with high expression (96.88%) of spleen tyrosine kinase (SYK) and PD-L1 (87.5%) and the poorest prognosis (P < .001). CD30 was highly expressed in ENKTL (9/17, 57.94%) and irrelevant to prognosis (P > .05)., Conclusions: Cases of PITNKL are biologically heterogeneous; most have a dismal prognosis. SYK and PD-L1 expression might be a significant marker for MEITL and helps differential diagnosis., (© American Society for Clinical Pathology, 2024.)

Objective: 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., Methods: 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., Results: 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., Conclusions: 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.

Background and Objective: 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., Methods: 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., Results: 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)., Conclusion: 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.

In order to determine the prevalence of the Epstein-Barr virus (EBV) infection in salivary gland lymphoepithelial carcinomas (LEC), we have collected 160 cases from Asian countries and Russia. All the cases examined by PCR for EBV DNA BamHI fragment and in-situ hybridization for EBER-1, EBV encoded small RNA, showed positivity for EBV infection in LEC cells, while no positive signals were found in any other salivary neoplasm examined. The incidence of LEC was highest in Guanzhou, followed by Shanghai and Chengdu and lowest in the northern parts of China, Seoul, Niigata, and Moscow. The mean age of the patients with LEC was 43.9 years with no sex predilection. The Chinese patients were of the Han race, only including minor races. There were ninety-five cases found with LEC in the parotid gland (75%), 20 in the submandibular gland (5%), and 28 in the minor salivary gland (20%). Histologically, the LECs were classified into two types: small nest type and large nest type. The latter type consisted of large-sized tumor cell nests and dense lymphocytic stromata and more frequently occurred in the minor salivary gland. The former consisted of small-sized tumor cell nests with fibrous and lymphocyte-depleted stromata, which were more frequently found in the parotid gland. The results indicated that EBV infection and certain geographic factors play important roles in the pathogenesis of the salivary LEC.