Plurihormonal PIT1-lineage pituitary neuroendocrine tumors: a clinicopathological study / 中华病理学杂志

Objective: To investigate the clinicopathological characteristics of plurihormonal PIT1-lineage pituitary neuroendocrine tumors. Methods: Forty-eight plurihormonal PIT1-lineage tumors were collected between January 2018 and April 2022 from the pathological database of Sanbo Brain Hospital, Capital Medical University. The related clinical and imaging data were retrieved. H&E, immunohistochemical and special stains were performed. Results: Out of the 48 plurihormonal PIT1-lineage tumors included, 13 cases were mature PIT1-lineage tumors and 35 cases were immature PIT1-lineage tumors. There were some obvious clinicopathological differences between the two groups. Clinically, the mature plurihormonal PIT1-lineage tumor mostly had endocrine symptoms due to increased hormone production, while a small number of immature PIT1-lineage tumors had endocrine symptoms accompanied by low-level increased serum pituitary hormone; patients with the immature PIT1-lineage tumors were younger than the mature PIT1-lineage tumors; the immature PIT1-lineage tumors were larger in size and more likely invasive in imaging. Histopathologically, the mature PIT1-lineage tumors were composed of large eosinophilic cells with high proportion of growth hormone expression, while the immature PIT1-lineage tumors consisted of chromophobe cells with a relatively higher expression of prolactin; the mature PIT1-lineage tumors had consistently diffuse cytoplasmic positive staining for keratin, while the immature PIT1-lineage tumors had various expression for keratin; the immature PIT1-lineage tumors showed more mitotic figures and higher Ki-67 proliferation index; in addition, 25.0% (12/48) of PIT1-positive plurihormonal tumors showed abnormal positive staining for gonadotropin hormones. There was no significant difference in the progression-free survival between the two groups (P=0.648) by Kaplan-Meier analysis. Conclusions: Plurihormonal PIT1-lineage tumor belongs to a rare type of PIT1-lineage pituitary neuroendocrine tumors, most of which are of immature lineage. Clinically increased symptoms owing to pituitary hormone secretion, histopathologically increased number of eosinophilic tumor cells with high proportion of growth hormone expression, diffusely cytoplasmic keratin staining and low proliferative activity can help differentiate the mature plurihormonal PIT1-lineage tumors from the immature PIT1-lineage tumors. The immature PIT1-lineage tumors have more complicated clinicopathological characteristics.

Pediatric SMARCB1/INI1-deficient poorly differentiated chordoma of the skull base: report of five cases and review of literature / 中华病理学杂志

Objective: To investigate the clinicopathological characteristics and differential diagnosis of pediatric SMARCB1/INI1-deficient poorly differentiated chordoma (PDC) of the skull base. Methods: Five cases of SMARCB1/INI1-deficient PDC were identified in 139 cases of chordoma diagnosed in Sanbo Brain Institute, Capital Medical University, Beijing, China from March 2017 to March 2021. The clinical and imaging data of the 5 PDCs were collected. H&E and immunohistochemical staining, and DNA methylation array were used, and the relevant literatures were reviewed. Results: All 5 PDCs were located at the clivus. The average age of the patients was 6.4 years, ranging from 3 to 16 years. Three patients were female and two were male. Morphologically, in contrast with classical chordomas, they presented as epithelioid or spindle tumor cells organized in sheets or nests, with necrosis, active mitoses, and infiltration into surrounding tissue. All cases showed positivity of CKpan, EMA, vimentin and brachyury (nuclear stain), and loss of nuclear SMARCB1/INI1 expression. S-100 protein expression was not frequent (2/5). Ki-67 proliferative index was high (20%-50%). All cases had over-expressed p53. It was necessary to differentiate SMARCB1/INI1-dificient PDC from SMARCB1/INI1-dificient tumors occurring at skull base of children or the tumors with epithelial and spindle cell morphological features. The 3 PDCs with DNA methylation testing showed the methylation profiles different from the pediatric atypical teratoid/rhabdoid tumors. They formed an independent methylation profile cluster. The clinical prognosis of the 5 patients was poor, and the overall survival time was 2-17 months. Conclusions: PDC is a special subtype of chordoma, which often affects children and occurs in the clivus. The PDC shares epithelioid or spindle cell morphologic features which are different from the classic chordoma. Besides the typical immunohistochemical profile of chordoma, PDC also has loss of nuclear SMARCB1/INI1 expression and distinct epigenetic characteristics.

The clinical significance of a new classification algorithm in Chinese DLBCL cases / 中华血液学杂志

<p><b>OBJECTIVE</b>To investigate the clinicopathologic features, pathogenesis, diagnostic criteria and the relationship between different classification models and prognosis in Chinese patients with DLBCL, and try to look for the most appropriate classification model to predict clinical prognosis and therapeutic responses for Chinese patients with DLBCL.</p><p><b>METHODS</b>181 cases of Chinese DLBCLs diagnosed according to the WHO 2008 classification were collected. Standard two-step Envision method of immunohistochemical staining was used to assess the expressions of CD20, CD3ε, CD79a, CD10, Mum-1, Bcl-6, GCET-1, FOXP1 and Ki-67. The phenotypic classifications were assessed according to the standard of Hans model and Chan model. Data were analyzed by χ(2) test and Life Table survival analysis with the SPSS14.0 statistical package.</p><p><b>RESULTS</b>The ratio of male to female in this cohort was 1.26:1. The median age of all patients was 57 yrs with the average age of 53.5 yrs. Of 61 cases (33.7%) primarily showed lymph node involvement. Gastrointestinal tract as the most involved extra-nodal organ was observed in 43 cases (35.8%). All patients with complete clinical follow-up materials survived from 1 - 120 months. The patients showed a high risk for death in the initial one and half years. Three year survival rate was 49.7% (90/181). Three year survival of 44 cases received R-CHOP (Rituximab, cyclophosphamide, doxorubicin, vincristine, bolus) was 76.9% (20/26), whereas 61.9% (60/97) in 119 cases received CHOP alone, R-CHOP group showed better prognosis (P = 0.017). All cases expressed one or more pan B cell markers, such as CD20 (176/179, 98.3%) and CD79a (62/77, 80.5%). For Hans model, 78 cases were classified as GCB group, while 103 cases as Non-GCB group. The ratio of Non-GCB to GCB was 1.32 without difference on the survival (P > 0.05). For the Chan's algorithm, 68 cases belonged to GCB subgroup, while 113 cases non-GCB subgroup. The ratio of non-GCB to GCB was 1.66. GCB subtype showed much better prognosis than non-GCB subtype according to Life Table survival analysis (P < 0.05).</p><p><b>CONCLUSION</b>The epidemiology and clinicopathologic features of Chinese DLBCLs were similarly with the western cases. Chan's algorithm was a significant tool to predict the cell origin and clinical biology of Chinese DLBCLs.</p>

Clinicopathological analysis of cutaneous natural killer/T cell lymphoma: 36 case report / 中华皮肤科杂志

ObjectiveTo investigate the clinicopathological features and prognosis of natural killer (NK)/T cell lymphoma and to analyze its relationship with Epstein-barr virus(EBV). MethodsTotally, 36 cases of cutaneous NK/T cell lymphoma were collected from 2000 to 2010 at the Department of Pathology, Peking University Health Science Center, and classified into primary and secondary groups according to whether there is evidence of extracutaneous involvement within 6 months after diagnosis. Clinicopathological features were analyzed and Epstein-barr virus (EBV) was detected. ResultsOf these 36 cases, 13 (36.1％) were classified as primary cutaneous NK/T cell lymphoma, 20 (55.6％) as secondary, and 3 (8.3％) remained unclassified because of the lack of clinical data. Males were more likely to develop both primary and secondary cutaneous NK/T cell lymphoma than females, but there was no striking difference in sex ratio between the patients with primary and secondary lymphoma (P ＞ 0.05 ). Compared with the patients with primary cutaneous NK/T cell lymphoma, those with secondary cutaneous NK/T cell lymphoma showed a younger median age at onset(43.5 vs. 54 years, P ＜ 0.05), higher prevalence of B symptoms(including fever, night sweat, body weight loss) and multiple skin lesions (P ＜ 0.05 and 0.01, respectively). EBV was positive in 92.3％ (12/13) of the primary lymphoma cases and 85％(17/20) of the secondary lymphoma cases. Moreover, the median survival was 8 months in all the cutaneous NK/T cell lymphoma cases, and was significantly shorter in secondary cases than in the primary cases(6 vs. 18 months, x2 = 6.074, P ＜ 0.05). ConclusionsCutaneous NK/T cell lymphoma is an EBV-associated, clinica]ly aggressive disease entity. Patients with primary cutaneous NI/T cell lymphoma seem to have an older age at onset and a better prognosis as compared with those with secondary cutaneous NK/T cell lymphoma.