Clinical and pathological study on 10 cases of cerebral lobe hemorrhage related with cerebral amyloid angiopathy / 解放军医学杂志

Objective To summarize the clinical data and pathological features of 10 cases of cerebral lobar hemorrhage related with cerebral amyloid angiopathy (CAA) diagnosed pathologically, thereby to improve the knowledge and diagnosis of the disease. Methods The clinical data of 10 cases of cerebral lobar hemorrhage related with CAA, collected in the General Hospital of Shenyang Command from 1983 up to now, were retrospectively analyzed, and the clinical and neuropathological features of these cases were summarized. Results Of the 10 patients, 2 suffered from single lobar hemorrhage and 8 multiple lobar hemorrhage, all of them were confirmed pathologically to have ruptured into the subarachnoid space. Pathological examination revealed microaneurysm in 2 cases, "double barrel" change in 4 cases, multiple arteriolar clusters in 5 cases, obliterative onion-liked intima change in 4 cases, and fibrinoid necrosis of vessel wall in 7 cases. In addition, neurofibrillary tangles were found in 8 cases, and senile plaque was observed in 5 cases. Conclusions Cerebral lobar hemorrhage related with CAA is mainly located in the parietal, temporal and occipital lobes, readily breaking into the subarachnoid space, and it is often multiple and recurrent. The CAA associated microvasculopathy was found frequently in the autopsy sample of CAA related cerebral lobar hemorrhage, and it may contribute to the pathogenesis of cerebral hemorrhage.

Clinical and radiological features of hypertensive brainstem encephalopathy / 解放军医学杂志

Objective To discuss the diagnosis and treatment of hypertensive brainstem encephalopathy. Methods The clinical and imaging data of 3 cases of hypertensive brainstem encephalopathy were summarized and analyzed for the purpose of improving the acumen in diagnosis and treatment. Results All the 3 patients showed relatively mild clinical symptoms, and they were misdiagnosed in different degrees during the treatment, but their clinical symptoms were improved by rapid and effective antihypertensive therapy. Cerebral CT and MRI scans revealed extensive abnormal signals in brain stem, with or without supratentorial lesions and brain stem hemorrhage. The lesions as revealed by imaging were improved significantly after treatment. Conclusions Clinical-radiographic dissociation is the classic feature of hypertensive brainstem encephalopathy. The clinical symptoms and lesions as shown by imaging could be improved after active treatment.

Clinical Neuropathological Analysis of 10 Cases of Cerebral Amyloid Angiopathy-Related Cerebral Lobar Hemorrhage

OBJECTIVE: The clinical and pathological characteristics of 10 cases of cerebral amyloid angiopathy (CAA)-related cerebral lobar hemorrhage (CLH) that was diagnosed at autopsy were investigated to facilitate the diagnosis of this condition. METHODS: The clinical characteristics of 10 cases of CAA-related CLH were retrospectively reviewed, and a neuropathological examination was performed on autopsy samples. RESULTS: The 10 cases included two with a single lobar hemorrhage and eight with multifocal lobar hemorrhages. In all of the cases, the hemorrhage bled into the subarachnoid space. Pathological examinations of the 10 cases revealed microaneurysms in two, double barrel-like changes in four, multifocal arteriolar clusters in five, obliterative onion skin-like intimal changes in four, fibrinoid necrosis of the vessels in seven, neurofibrillary tangles in eight, and senile plaques in five cases. CONCLUSION: CAA-related CLHs were located primarily in the parietal, temporal, and occipital lobes. These hemorrhages normally consisted of multiple repeated CLHs that frequently bled into the subarachnoid space. CAA-associated microvascular lesions may be the pathological factor underlying CLH.

Detection of chromosomal translocations involving ALK gene in anaplastic large cell lymphoma by fluorescence in-situ hybridization and its clinical significance / 中华病理学杂志

<p><b>OBJECTIVE</b>To investigate clinicopathologic features and clinical value of the chromosomal translocation involving anaplastic lymphoma kinase (ALK) in anaplastic large cell lymphoma (ALCL) by fluorescence in situ hybridization (FISH).</p><p><b>METHODS</b>A total of 55 cases, including 45 cases of ALCL and 10 reactive lymphoid hyperplasia, were collected during 1999 to 2006 in the Department of Pathology, Fudan University Shanghai Cancer Center, and Xinhua Hospital Affiliated to Shanghai Jiaotong University. All cases were studied by FISH using dual color break apart probes of ALK for detection of chromosomal translocation, compared with the previous results of immunohistochemistry (IHC) and reverse-transcriptase polymerase chain reaction (RT-PCR) for the detection of ALK aberrations.</p><p><b>RESULTS</b>The result of FISH showed that the clear red and green fluorescence signals were detected in 38 cases of ALCL, in which conspicuous split signals were observed in tumor cells in 24 cases (63.2%), suggesting the rearrangement of the ALK locus, with multiple copies of ALK gene in one case. In addition, the rearrangement of the ALK locus was not identified in 14 of 38 cases (36.8%); and the FISH results were unable to be evaluated in 7 cases, because no fluorescent signals involving ALK gene were found or signals were too weak to be analyzed. The concordance for the detection ALK aberrations in ALCL between FISH and RT-PCR, FISH and IHC were both statistically significant (P < 0.01). Chromosomal translocation involving ALK gene was not found in all 10 cases of reactive lymphoid hyperplasia.</p><p><b>CONCLUSIONS</b>ALCL is an entity of lymphoma characterized by special clinical presentation, morphology, and ALK aberrations. FISH is helpful for detection of the chromosomal translocations involving ALK in ALCL, however, the detection efficiency by FISH may be affected by storage time of the paraffin-embedded tissue; and therefore combined detection with IHC and RT-PCR could complement each other and help for differential diagnosis of ALK(+)ALCL from ALK(-)ALCL.</p>

Follicular variant of peripheral T-cell lymphoma: a clinicopathologic and genetic study of 2 cases / 中华病理学杂志

<p><b>OBJECTIVE</b>To observe the clinicopathologic and genetic features of follicular variant of peripheral T-cell lymphoma (FV-PTCL), with particular attention to the relationship of this type of lymphoma with angioimmunoblastic T-cell lymphoma (AITL).</p><p><b>METHODS</b>The clinical data, hematoxylin and eosin-stained sections of lymph node biopsies from 2 FV-PTCL cases were reviewed. Immunohistochemical phenotyping and detection of EBV-encoded RNAs (EBER) through in situ hybridization (ISH) were performed. The EnVision two-step method was used for all antibodies except CXCL13 (by using three-step streptavidin immunoperoxidase method). Analysis of clonality and ITK/SYK gene rearrangement was conducted using PCR and RT-PCR assays, respectively.</p><p><b>RESULTS</b>Clinically, the two patients presented with superficial lymphadenopathy similarly. Histologically, case 1 showed a follicular/nodular lymphoid proliferation without marked germinal centers. The neoplastic cells comprised mainly medium sized cells with abundant, sometimes clear cytoplasms. Similar histologic findings were seen in case 2 in addition to a concurrent component mimicking typical AITL noticed. Of both cases, the neoplastic cells showed positive reactivity to CD3, CD4, CD10, PD1, and CXCL13. Positive hybridization signals for EBER were only seen in case 2, and double stains demonstrated that those EBV-positive cells were mostly the reactive transformed B-cells. Monoclonal T-cell proliferation was proved by the rearranged TCR gene detection in both cases. Neither of the current cases expressed ITK/SYK fusion transcripts.</p><p><b>CONCLUSION</b>FV-PTCL shows the similar or overlapped morphological and immunophenotypic features to those of AITL, possibly suggesting the presence of a potential relationship between these two types of lymphomas.</p>

Immunoglobulin heavy chain gene rearrangement study in difficult cases of B-cell lymphoproliferative disorder / 中华病理学杂志

<p><b>OBJECTIVE</b>To evaluate the ancillary diagnostic value of IgH gene rearrangements in those B-cell lymphoproliferative disorder cases whom are difficult in making a final diagnosis.</p><p><b>METHODS</b>IgH gene clonal rearrangements were retrospectively analyzed in a total of 77 diagnostically difficult B-cell lympho-proliferative patients. Standardized BIOMED-2 system IgH gene clonality assay kit targeting FR1, FR2, FR3 was used, followed by heteroduplex-polyacrylamide gel electrophoresis (PAGE) and silver nitrate staining.</p><p><b>RESULTS</b>The final diagnoses of the 77 cases were: 12 cases of reactive lymphoid hyperplasia, 20 cases of atypical lymphoid hyperplasia or suspicious lymphoma, and 45 cases of B-cell lymphoma. Detection rates of at least one positive reaction were 2/12, 11/20 (55%), 36/45 (80%) in the three groups, respectively. In B-cell lymphomas, the clonality detection rate of FR1, FR2 and FR3 was 60% (27/45), 60% (27/45) and 56% (25/45), respectively. The type distribution were: 20 marginal zone lymphomas, including 18 extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue, 7 diffuse large B-cell lymphomas, 7 follicular lymphomas, 1 mantle-cell lymphoma, 1 Burkitt's lymphoma, 4 plasma cell neoplasms and 5 unclassified B-cell lymphomas. Rearrangements of FR1, FR2 or FR3 were not detected in 9 (20%) of the B cell lymphoma cases, nevertheless, one of them had developed liver lesion later, and was confirmed finally to be B cell lymphoma. Fourteen patients of reactive lymphoid hyperplasia with positive IgH gene clonal rearrangements, and atypical lymphoid hyperplasia had follow-up history available. Four of them were diagnosed as lymphoid malignancies upon further biopsy, and in three of them, clonal IgH gene rearrangements were detected.</p><p><b>CONCLUSIONS</b>B-cell lymphoproliferative disorder requiring a detection of clonal IgH gene rearrangement for making a final diagnosis. Combined detections of three IgH FR1, FR2 and FR3 rearrangements provide important ancillary diagnostic value in confirming suspected B-cell lympho-proliferative disorders. It is important to take an additional biopsy or to follow-up those patients who that have a detectable IgH gene clonal rearrangement but without apparent morphological evidence of lymphoma. For cases with a negative IgH gene rearrangements, it might be necessary to perform clonality analysis for other forms of gene rearrangements including IgH or IgK and IgL in order to further improve the detection sensitivity.</p>

Distinction between lymphoma-like lesions and lymphoma of uterine cervix: a clinicopathologic study of 26 cases / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the clinicopathologic features and differential diagnosis of lymphoma-like lesions and lymphoma of uterine cervix.</p><p><b>METHODS</b>Clinical data and hematoxylin and eosin-stained slides of 10 cases of lymphoma-like lesion and 16 cases of lymphoma of uterine cervix were reviewed. Immunohistochemical study for B- and T-cell markers and light chains (kappa, lambda) were performed on paraffin sections. The rearrangement status of immunoglobulin heavy chain (IgH) gene was analyzed with semi-nested polymerase chain reaction in 4 cases lymphoma-like lesion and 4 cases of lymphoma of uterine cervix.</p><p><b>RESULTS</b>The age of patients with lymphoma-like lesion ranged from 24 to 54 years (medium = 43 years). The lesion generally presented with cervical erosion or polyp. Microscopically, it is characterized by focal or diffuse superficial infiltration of immunoblast-like large B cells intermingled with a polymorphic population of inflammatory cells, including plasma cells, eosinophils and neutrophils. Maturation of the transformed large B cells was also noticed. On the other hand, the age of the patients with lymphoma of uterine cervix varied from 28 to 78 years (medium = 58 years). Cervical mass or diffuse enlargement of cervix were the commonest clinical findings. The cases included 12 examples of diffuse large B-cell lymphoma and 4 examples of follicular lymphoma. The former was characterized by a diffuse monomorphic population of large atypical lymphoid cells, while neoplastic follicles were identified in the latter. Neither polymorphic inflammatory infiltrates nor maturation phenomenon was found. The immunostaining for kappa and lambda light chains was inconclusive. Molecular study showed clonal rearrangement of IgH gene in all cases of cervical lymphoma, as well as 2 cases of lymphoma-like lesion.</p><p><b>CONCLUSIONS</b>The distinction between lymphoma-like lesion and lymphoma of uterine cervix depends primarily on the clinical and histopathologic features. Assay for rearrangement of IgH gene may be helpful in differential diagnosis, though monoclonality can be detected in some benign lesions as well.</p>

Nodal versus extranodal diffuse large B-cell lymphoma: comparison of clinicopathologic features, immunophenotype and prognosis / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the clinicopathologic features and outcome of patients with diffuse large B-cell lymphoma (DLBCL), and to compare the differences between DLBCL of nodal and extranodal origins.</p><p><b>METHODS</b>One hundred and forty-two cases of de novo DLBCL collected during a 10-year period were reviewed. The clinicopathologic features and follow-up (2 - 108 months) data were analyzed. Tissue microarray blocks were performed and immunohistochemical studies using antibodies against CD10, bcl-6 and MUM1 were carried out. The cases were then further categorized into germinal center B cell-like (GCB) and non-GCB subtypes.</p><p><b>RESULTS</b>Primary gastrointestinal DLBCL often presented as early-stage disease (stage I or II) and was associated with low international prognostic index. They showed better prognosis than DLBCL of nodal and other extranodal origins. The positivity rates of CD10, bcl-6 and MUM1 were 19%, 51% and 58%, respectively. 36% of the cases belonged to GCB, while the remaining 64% were non-GCB. In general, DLBCL of extranodal origin showed more frequent bcl-6 expression than nodal DLBCL. As for extranodal DLBCL, GCB immunophenotype was often seen in thyroid and breast tumors, while testicular DLBCL usually carried a non-GCB immunophenotype.</p><p><b>CONCLUSIONS</b>DLBCL of various origins show a diversified GCB and non-GCB differentiation. Nodal and extranodal DLBCL, as well as extranodal DLBCL from different primary sites, carry different biologic characteristics and prognostic implications.</p>

Clinicopathologic and genetic studies of desmoid-type fibromatosis / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the clinicopathological and genetic features of desmoid-type fibromatosis, and to investigate the feasibility of detecting trisomy 8 in formalin fixed, paraffin embedded (FFPE) tissue by fluorescence in-situ hybridization (FISH).</p><p><b>METHODS</b>A total of 96 cases were included in this study. All patients had clinical information. Histopathologic and immunohistochemical evaluations were available in 69 cases, and ultrastructural evaluation was done in 2 cases of desmoid-type fibromatosis. FFPE tissue sections were available in 20 tumors for the trisomy 8 detection by FISH.</p><p><b>RESULTS</b>There were 20 male and 76 female patients with ages ranging from 8 to 86 years (mean 35.3 years). Clinically, there were 44 extra-abdominal tumors, 28 abdominal wall tumors and 23 intra-abdominal lesions mostly involving the mesentery. Most cases presented with nodular or funicular masses with white firm cut surfaces, measuring 0.6 to 24.0 cm (mean 8.4 cm) in size. Histologically, desmoid-type fibromatoses showed longitudinal fascicles of spindle fibroblasts and myofibroblasts in a predominantly collagenous background. The tumor cells stained positive for vimentin, alpha-smooth muscle actin, desmin, and beta-catenin (47.8%, 33/69). Ultrastructurally, most tumor cells had features of fibroblasts, including rich endoplasmic reticulum and Golgi apparatus. Some tumor cells were myofibroblast-like cells exhibiting intercellular junctions, fibronexous junctions and stress fibers. Trisomy 8 was detected in 6 of 20 cases of desmoid-type fibromatosis including 5 of the 8 recurrent tumors but only one of the 12 primary tumors. The latter tumor also recurred three years later.</p><p><b>CONCLUSIONS</b>Desmoid-type fibromatosis is an intermediate (locally aggressive) tumor that occurs predominantly in young females. The lesion consists of fibroblasts and myofibroblasts with the latter showing characteristic features including stress fibers and fibronexous junctions. Trisomy 8 can be detected in FFPE tissue by FISH, and its presence serves as a useful predictor of tumor recurrence and may define a subtype of desmoid-type fibromatosis with high recurrence rate.</p>

Prognostic predictors of nasal NK/T cell lymphoma detected by immunohistochemical staining / 中华肿瘤杂志

<p><b>OBJECTIVE</b>To investigate the prognostic predictors of nasal NK/T cell lymphoma.</p><p><b>METHODS</b>The clinicopathologic feature data of 61 patients with nasal NK/T cell lymphoma proven by pathological examination from Jan. 1997 to Jan. 2005 were collected. Expression of survivin, CD44, nm23, p53, Ki-67, MDR-1 and CD95 was detected by immunohistochemical staining in 30 patients with available histologic specimens. The correlation between these factors and prognosis were analyzed.</p><p><b>RESULTS</b>In univariate analysis, performance status, LDH level, clinical stage, initial treatment response, CD56, Ki-67 and CD95 were found to be the prognostic factors associated with time to progression (TTP) in nasal NK/T cell lymphoma, while the performance status, B symptoms, LDH level, initial treatment response, Ki-67 and CD95 were demonstrated as prognostic factors related to overall survival. In multivariate analysis, clinical stage, initial treatment response and performance status were independent prognostic factors for TTP, while the latter two factors were independent prognostic factors of overall survival.</p><p><b>CONCLUSION</b>Clinical stage and initial treatment response, and performance status are found to be independent prognostic factors for TTP, whereas the latter two factors are demonstrated as independent prognostic factors of the overall survival. Overexpression of Ki-67 may be an unfavorable prognostic factor, but overexpression of CD95 may be a favorable one.</p>

Kimura disease and epithelioid hemangioma: a comparative study of 12 cases / 中华病理学杂志

<p><b>OBJECTIVE</b>To investigate the clinicopathologic features, immunophenotypes and differential diagnosis of Kimura's disease (KD) and epithelioid hemangioma (EH).</p><p><b>METHODS</b>Nine cases of KD and three cases of EH were evaluated by light microscopy and immunohistochemistry.</p><p><b>RESULTS</b>In this series, KD occurred predominantly in males, whereas EH had a female predilection. Both KD and EH arose most frequently in the head and neck region. However, KD usually presented as multiple subcutaneous nodules or swellings and was accompanied by lymphadenopathy in some cases. On the other hand, EH appeared only as a small skin nodule or red plaque. Histologically, both lesions may involve the dermis or subcutis. All the 9 KD cases displayed florid hyperplasia of lymphoid tissue, of which, 7 cases exhibited formation of lymphoid follicles and active germinal centers. Proliferation of post-capillary venules were seen between follicles. They were lined by plump or attenuated endothelial cells. Large number of eosinophils aggregated around the vessels or adjacent to the follicles, formation of microabscesses were observed in 3 cases. All the 3 EH cases showed prominent proliferation of vessels (capillary-sized in 2 cases and small to medium-sized in 1 case). The vessels were lined by epithelioid endothelial cells with abundant eosinophilic cytoplasm. The endothelial cells also proliferated within the lumen in 1 case and grew in sheets or cords adjacent to the vessel walls in 2 cases. Some endothelial cells contained intracytoplasmic vacuoles, suggesting formation of primitive vessels. Associated inflammatory component was noted in 2 cases. Lymphoid follicles however were not present and eosinophil infiltration was not as prominent as in KD. Immunohistochemical study in KD revealed B cells in the lymphoid follicles and mostly T cells in the interfollicular regions. In EH, the epithelioid endothelial cells showed strong reactivity to CD31 and factor VIII-related antigen.</p><p><b>CONCLUSIONS</b>KD and EH are two distinctive entities. The former represents a lymphoid hyperplasia and the latter represents a benign vascular tumor. Recognition of the clinical characteristics and morphologic features of KD and EH is very important in making this distinction.</p>