Clinicopathological features of dedifferentiated liposarcomas with meningothelial-like whorls: report of six cases / 中华病理学杂志

To investigate the histopathologic, immunohistochemical, molecular genetic characteristics of dedifferentiated liposarcomas with meningothelial-like whorls(DDLMW). Six cases of DDLMW diagnosed at Jiangsu Province Hospital(the First Affiliated Hospital of Nanjing Medical University) from March 2012 to August 2018 were enrolled. The cases were analyzed by routine HE staining, immunohistochemistry(MDM2, CDK4 and p16) and fluorescent in-situ hybridization(FISH) on MDM2 gene. Related literatures were also reviewed. Three of the 6 patients were male.The patient ages ranged from 40 to 77 years (mean, 58 years). Four tumors occurred in the retroperitoneum and two in the mediastinum. Histologically, the tumors showed, in addition to foci of well-differentied liposarcoma, characteristic, scattered meningothelial-like concentrical whorls. The whorls were composed of tightly, concentrically arranged, spindle to ovoid cells with mild to mederate cytological atypia. Metaplastic bone was present within or in their immediate vicinity in four case. The tumors cell also showed strong and diffuse immunoreactivity to MDM2, CDK4 and p16, but no immunoreactivity to S-100 protein, SMA, SOX10, EMA, CD21, CD23 or CD35. The Ki-67 labeling indexes were low, while FISH showed high levels of MDM2 amplification in all cases. DDLMW is a rare morphologic variant of dedifferentiated liposarcoma. The whorls in DDLMW do not represent perineurial or follicular dendritic differentiation. Recognition and familiarity with its existence, as well as combined application of immunohistochemical staining and MDM FISH, are important to avoid confusion with other lesions.

Clinicopathologic study of pimary paget disease in esophagus / 临床与实验病理学杂志

Purpose To investigate the clinicopathological and diagnostic characteristics of primary Paget disease (PD ) in esophagus. Methods The clinical presentation, histological observation and immunohistochemical staining were analyzed in four cases of primary PD involved esophagus and related literatures were reviewed. Results The patients were all male, aged from 61 to 74 years old. All the tumors were originated from the mucosa of the esophagus. Histologically, the Paget cells showed a single or small nesting and acinar distribution in the esophage-al mucosa. Adenocarcinoma in situ were seen in 2 cases and squamous cell carcinoma was seen in one of them. Immunohisto-chemically, the Paget cells were typically strongly positive for Ckpan, CKL, and CK7, while negative for CKH, CK5/6, CK14, p63. Conclusion Primary esophagus PD is rare. It can develop alone in esophagus or accompanied with adenocarcinoma in situ, invasive squamous cell carcinoma. The correct diagnosis need detailed pathological observation, immunohistochemical ev-idence and medical history.

Solid variant of angiomatoid fibrous histocytoma:report of 3 cases / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the clinicopathologic features, immunophenotype, molecular genetics and differential diagnosis of solid variant of angiomatoid fibrous histocytoma.</p><p><b>METHODS</b>The clinicopathologic features of 3 cases of solid variant of angiomatoid fibrous histocytoma were analyzed and the literature was reviewed.</p><p><b>RESULTS</b>There were a total of 2 males and 1 female. The age of patients ranged from 9 to 12 years. The patients presented with a painless mass located in left forearm, left knee or back. The lesions were treated by complete surgical resection. On gross examination, the tumors varied from 1.6 cm to 4.5 cm in greatest dimension. They were well-circumscribed and had pale yellow to grayish-red solid cut surface. Histologically, the tumor was composed of histocytoid cells arranged in sheet-like pattern. A fibrous pseudocapsule surrounded by lymphocytes and plasma cells was identified. Immunohistochemical study showed that the tumor cells in all cases were positive for vimentin and CD68. They were negative for S100 protein, cytokeratin, CD34, CD31, smooth muscle actin, CD35, CD21 and CD30. Two cases also expressed CD99 and one of them was positive for desmin and epithelial membrane antigen. Fluorescence in-situ hybridization was positive for EWSR1 gene.</p><p><b>CONCLUSIONS</b>Solid type represents a variant of angiomatoid fibrous histocytoma and is considered as tumor of borderline malignant potential. Definitive diagnosis requires thorough histologic examination and clinical correlation. Immunohistochemistry and EWSR1 gene study are helpful in further delineation and differential diagnosis. Complete resection or wide local excision with post-operative follow up is the main modality of treatment.</p>

Epithelioid hemangioma: a clinicopathologic analysis of 7 cases / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the clinicopathologic features, diagnosis and differential diagnosis of epithelioid hemangioma.</p><p><b>METHODS</b>The morphologic features of 7 cases of epithelioid hemangioma of skin, bone and venous vessels were studied.</p><p><b>RESULTS</b>There were altogether 4 male and 3 female patients (median age = 34 years; age range from 14 to 54 years). The 3 skin cases presented as single or multiple erythematous to bluish nodules or papules, with or without itchiness. The 2 bone cases appeared as osteolytic expansile lesions on radiologic examination. The remaining 2 cases involved medium-sized venous structures and presented as small isolated nodules in soft tissue. Histologically, the lesions were characterized by the presence of exuberant endothelial proliferations with various degree of inflammatory reaction. The neoplastic endothelial cells were plump, eosinophilic and polygonal, forming vascular channels. Occasional solid sheet-like arrangement was demonstrated. Intracytoplasmic vacuoles were commonly identified, indicating formation of primary lumen. The surrounding stroma contained various number of eosinophils and lymphoplasmacytic cells. Immunohistochemical study showed that the tumor cells were positive for endothelial markers (CD31 and CD34) and negative for epithelial marker (cytokeratin). Follow-up information was available in 6 cases. The duration of follow-up ranged from 5 to 36 months (median = 14 months). There was no evidence of recurrence or distant metastasis.</p><p><b>CONCLUSIONS</b>Epithelioid hemangioma is a rare benign curable lesion which can be multifocal, involving skin, soft tissue and bone. It needs to be distinguished from Kimura's disease and epithelioid hemangioendothelioma.</p>

Primary intermediate hemangioendothelioma of bone: a study of 5 cases / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the radiologic and pathologic features of primary intermediate hemangioendothelioma of the bone.</p><p><b>METHODS</b>Five cases of primary intermediate hemangioendothelioma of bone encountered in the past three years were enrolled into the study. The clinical, radiologic, pathologic and immunohistochemical features of the tumors were reviewed.</p><p><b>RESULTS</b>The patients included 3 children with Kaposiform hemangioendothelioma and 2 elderly with retiform hemangioendothelioma. Four of the cases affected long bones and the remaining case affected the clavicle. One case showed multifocal involvement of the humerus. Radiologically, the tumors showed borderline to low-grade bony destruction, with various degrees of cortical defect. Intralesional or perilesional bone formation was demonstrated in 4 cases and radial spicules were seen in 1 case. The histopathologic features of primary intermediate hemangioendothelioma of bone were similar to those of soft tissue, except for the presence of reactive bone formation. Immunohistochemically, the tumor cells were positive for CD31 (5/5), CD34 (5/5), vimentin (5/5) and smooth muscle actin (3/5) but negative for cytokeratin and epithelial membrane antigen.</p><p><b>CONCLUSIONS</b>Primary intermediate hemangioendothelioma of bone is a distinct entity and similar histologic classification applies as in its soft tissue counterparts. Comparison of the biologic behavior requires long-term follow-up studies.</p>

Adult prostate sarcoma: a clinicopathologic study of 15 cases / 中华病理学杂志

<p><b>OBJECTIVE</b>To clarify the clinical and morphological features of adult prostate sarcoma (APS) and to further improve the knowledge and diagnostic accuracy for APS.</p><p><b>METHODS</b>Fifteen cases of APS were observed and analyzed on the clinical symptom, pathological features, treatment and prognosis.</p><p><b>RESULTS</b>Age of onset ranged from 22 to 77 years (mean 46.3 years). The majority of cases were presented with dysuresia. By digital rectal examination and imaging of the prostate, APS was often identified as a large tumor mass. There were 6 cases of leiomyosarcomas, 6 embryonal rhabdomyosarcomas, and 3 fibrosarcomas in this series. Follow-up data were available for 12 cases: 7 cases died of the disease between 9 days and 360 days after surgery. Among 5 survived patients, 3 cases had recurrence after 2 to 24 months follow-up.</p><p><b>CONCLUSIONS</b>APS is a rare tumor that typically has clinical features: earlier age of onset, fast-appeared urinary tract symptoms, significant mass effects, and poor outcome. Level of prostate specific antigen (PSA) is usually normal or lower. Final diagnosis relies on the features of histology and immunohistochemistry expression profile.</p>

EZH2 expression in human prostate cancer and its clinicopathologic significance / 中华男科学杂志

<p><b>OBJECTIVE</b>To investigate the expressions of the EZH2 protein and EZH2 mRNA in human prostate cancer (PCa) and their correlation with the clinicopathologic parameters.</p><p><b>METHODS</b>A tissue microarray (TMA) was constructed, which contained 48 dots of formalin-fixed paraffin-embedded tissue samples of human PCa. The expressions of the EZH2 protein and EZH2 mRNA in the samples were detected by immunohistochemistry (EnVision) and in situ hybridization (ISH). Another 15 cases of human benign prostate hyperplasia (BPH) and 12 cases of human prostate intraepithelial neoplasia (HGPIN) were taken as controls.</p><p><b>RESULTS</b>The positive rates of the EZH2 protein and mRNA were significantly higher in PCa than in BPH and HGPIN (87.5% vs 13.33% and 16.67%, 81.25% vs 6.67% and 16.67%, P < 0.05). The positive expression of the EZH2 protein was 96.67% and 72.22% in the Gleason score > or = 7 and Gleason score < or = 6 groups, respectively, with significant differences between the two groups (P < 0.05). The positivity of the EZH2 protein was significantly related to the TNM stage, increasing with tumor progression (P < 0.05), but not to age and serum PSA (P > 0.05), and so was that of EZH2 mRNA to TNM stage (P < 0.05), but not to age, serum PSA and Gleason score (P > 0.05). When the above characteristics were regarded as two-level discrete variables, both the EZH2 protein and EZH2 mRNA showed statistically significant differences in the positive expression rate (P < 0.05).</p><p><b>CONCLUSION</b>The over-expressions of the EZH2 protein and EZH2 mRNA may play an important role in the pathogenesis and progression of PCa and provide some reference indexes for estimating the malignancy, progression and prognosis of PCa.</p>

Roles of immunohistochemistry in prognostic assessment of basal-like breast cancer / 中华病理学杂志

<p><b>OBJECTIVES</b>Basal cell-like breast cancer is one of the subtypes using molecular typing, and this subtype attracted a wide spread attention. Currently, no uniform diagnostic criteria are available. Most studies demonstrated poor outcomes, but contradictory conclusions appeared recently. The prognosis of basal cell-like breast cancer using different immunohistochemical criteria were analyzed.</p><p><b>METHODS</b>Two hundred and eighty-four invasive breast cancers with a follow-up information over 5 years were evaluated for ER, PR, HER2, CK5/6, CK14, EGFR expression on tissue microarray immunohistochemically. Based on the results, these cases using four different diagnostic criteria were categorized, namely: Nielsen (ER-/HER2-, CK5/6+ and/or EGFR+), Kim (ER-/PR-/HER2-, CK5/6+ and/or CK14+ and/or EGFR+), Triple-negative (ER-/PR-/HER2-), and basal-CK (CK5/6+ and/or CK14+). 5-year survival information was compared between groups.</p><p><b>RESULTS</b>The prevalence of basal cell-like breast cancer by Nielsen, Kim, Triple-negative and basal-CK were 15.5% (44/284), 14.8% (42/284), 43.3% (123/284) and 21.1% (60/284) respectively; the recurrence rates were 18.2% (8/44), 21.4% (9/42), 10.6% (13/123) and 11.7% (7/60) respectively. These were higher than recurrence rates for other subtypes, but only the differences by Nielsen's and Kim's criteria were significant. Using Nielsen's and Triple-negative's criteria, basal-like tumors showed shorter 5-year disease-free survival (both P < 0. 01) and overall survival (P < 0.05 and 0.01) than luminal A subtype, using Kim's criteria, basal-like tumors showed a lower 5-year disease-free but not overall survival than luminal A subtype (P < 0.01); no significant difference was found on 5-year survival between basal-like and non-basal-like tumors when typed by basal-CK.</p><p><b>CONCLUSION</b>Basal cell-like breast cancers are more likely to show more recurrence and worse outcome, but different immunohistochemical diagnostic criteria have an influence on their prognostic analysis, so a uniform diagnostic criteria is essential for the further study of basal-like breast cancers.</p>

Clinical features of parathyroid space-occupying lesions / 中华耳鼻咽喉头颈外科杂志

<p><b>OBJECTIVE</b>To investigate the clinical manifestation, diagnosis, treatment of parathyroid occupying lesions.</p><p><b>METHODS</b>The clinical data of 42 patients with parathyroid occupying lesions were retrospectively analyzed, including the clinical symptoms and signs, laboratory results, pathologic and imaging results and treatment.</p><p><b>RESULTS</b>The number of males and females were 8 and 34, with females: males ratio being 1:5.25. The median age was 39 years. There were 2 cases of parathyroid cancer, 29 cases of parathyroid adenoma, 11 cases of parathyroid cysts in this study. The symptoms were as follows: 40 cases of neck lump, 34 cases of osteoporosis/osteitis fibrosa cystica, 29 cases of urinary symptom, 7 cases of voice hoarseness, 4 cases of peptic ulcer, 3 cases of dyspnoea and dysphagia, 3 cases of thoracic cavity lump, 2 cases of enhanced amylase activity. Serum calcium ion level and serum parathyroid hormone (PTH) level were examined qualitatively before operation. Ultrasonography, ECT-99mTc-MIBI, CT, MRI were used in diagnosing and locating parathyroid occupying lesions before operation. Twenty nine cases of parathyroid adenoma were treated with operation, 28 patients achieved complete remission, 1 suffered relapse after 23 months postoperative follow up. Eleven cases of parathyroid cysts were treated with operation and the outcome was no recurrence. Two cases of parathyroid cancer survived with out recurrence during follow up for 28 months and 50 months after operation.</p><p><b>CONCLUSIONS</b>Examination of serum calcium and PTH level together with ultrasonography, ECT-99mTc-MIBI, CT, MRI is helpful to diagnose parathyroid occupying lesions. Surgery should be done as primary treatment. Tumor resection can be performed for parathyroid cysts, intraoperative exploration of bilateral neck is indicated for parathyroid adenoma, and a radical resection should be performed primarily for the parathyroid cancer.</p>

Clinicopathologic characteristics of hemangiopericytoma/solitary fibrous tumor with giant cells / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the pathological characteristics, diagnosis and differential diagnoses of hemangiopericytoma-solitary fibrous tumor with giant cells.</p><p><b>METHODS</b>Pathological characteristics of seven cases of orbital and extraorbital hemangiopericytoma-solitary fibrous tumors with giant cells were evaluated by HE and immunohistochemistry (EnVision method).</p><p><b>RESULTS</b>Two cases were located in the orbit, one of which had recurred. Five cases were located in the extraorbital regions. Histologically, the tumors were well-circumscribed and composed of non-atypical, round to spindle cells with collagen deposition in the stroma. The tumors had prominent vasculatures and in areas, pseudovascular spaces lined by multinucleated giant cells lining which were also present in the stroma. Immunohistochemically, both neoplastic cells and multinucleate giant cells expressed CD34. Seven patients underwent tumor excision and were well and without tumor recurrence upon the clinical follow-up.</p><p><b>CONCLUSIONS</b>Hemangiopericytoma-solitary fibrous tumor with giant cells is an intermediate soft tissue tumor. It typically involves the orbital or extraorbital regions. Histologically, the tumor should be distinguished from giant cell fibroblastoma, pleomorphic hyalinzing angiectatic tumor of soft part and angiomatoid fibrous histiocytoma.</p>

Clinicopathologic features and prognostic significance of basal-like breast cancer / 中华病理学杂志

<p><b>OBJECTIVE</b>To analyzed a large group of invasive breast cancers with long-term follow-up information to evaluate the clinicopathologic, morphological and prognostic features of basal-like breast cancers in Chinese population.</p><p><b>METHODS</b>Immunohistochemistry was used to detect the expression of ER, HER2, CK5/6, EGFR on tissue microarray with 1311 invasive breast cancers. Based on the results, these cases were categorized into luminal A, luminal B, basal-like, HER2-overexpressing and null subtypes. Clinicopathological features and survival rates were compared between these groups.</p><p><b>RESULTS</b>Basal-like breast cancers constituted 17.0% of 1311 invasive breast cancers with a significantly larger size, higher grade and higher incidence of the medullary carcinoma, frequent recurrence and infrequent node metastasis. Morphologically, basal-like breast cancers showed a significantly more solid architecture and ribbon-like architecture associated with necrosis (more geographic necrosis) and central scar, a more pushing margin, lymphocytic infiltration and a higher mitosis score, more syncytial growth, presence of basaloid cells, spindle cells and squamous metaplasia. The disease-free survival and overall survival of basal-like breast cancers were significantly poorer than that of luminal A subtype, but similar to the other ER-negative subtypes. Basal markers were not independent prognostic factors.</p><p><b>CONCLUSIONS</b>Basal-like breast cancers in Chinese population has a similar prevalence to that of the western populations. They have distinct clinicopathologic features compared to other non-basal breast cancers, but overlapping with other ER-negative breast cancers. Morphological features are strongly associated with basal-like breast cancers although they are not very specific. The survival of basal-like breast cancers is poorer than luminal A, but similar to the other ER-negative breast cancers, and basal markers are not independent prognostic factors of breast cancers.</p>

Inflammatory pseudotumor-like follicular dendritic cell tumor of spleen / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the clinicopathologic features of inflammatory pseudotumor-like follicular dendritic cell tumor of spleen.</p><p><b>METHODS</b>One case of inflammatory pseudotumor-like follicular dendritic cell tumor of spleen was examined macroscopically and microscopically. Immunohistochemical study for CD21, CD23, CD35, clusterin, S-100 protein, vimentin, smooth muscle actin, CD1a, CD68, ALK protein, CD30, CD31, CD34, CD3 and CD20 was performed on formalin-fixed, paraffin-embedded sections by standard EnVision method. In-situ hybridization for Epstein-Barr virus (EBV)-encoded RNA was also carried out.</p><p><b>RESULTS</b>Macroscopically, inflammatory pseudotumor-like follicular dendritic cell tumor was large in size, tan-colored, soft to rubbery in consistance and associated with central hemorrhage and necrosis. Histological examination showed scattered follicular dendritic cells admixed with abundant lymphocytes and plasma cells in the background, simulating inflammatory pseudotumor. On high-power magnification, the follicular dendritic cells possessed a moderate amount of pale to lightly eosinophilic cytoplasm, with indistinct cell borders. The nuclei were ovoid or spindly, with vesicular or stippled chromatin and small distinct, often centrally located, nucleoli. Some of the tumor cells showed nuclear pleomorphism and contained irregular foldings of nuclear membrane, coarse chromatin and prominent eosinophilic nucleoli. Mitotic figures were rarely identified. Immunohistochemical study showed that the tumor cells were positive for vimentin, clusterin, smooth muscle actin and CD68. They were weakly and focally positive for CD35 and S-100 protein, but negative for CD21, CD23, CD1a, ALK protein, CD30, CD31 and CD34. Most of the background lymphocytes were of T-lineage (CD3-positive) ,some were CD20 (B-cell marker)-positive. EBV RNA was demonstrated in the tumor cells by in-situ hybridization analysis.</p><p><b>CONCLUSIONS</b>Inflammatory pseudotumor-like follicular dendritic cell tumor is a rarely encountered low-grade malignancy with distinctive morphologic pattern. It is associated with EBV infection.</p>

Clinicopathologic and immunohistochemical study of atypical teratoid/rhabdoid tumor of central nervous system / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the clinicopathologic features and differential diagnosis of atypical teratoid/rhabdoid tumor (AT/RT) occurring in the central nervous system.</p><p><b>METHODS</b>Two cases of AT/RT were studied by hematoxylin-eosin, reticulin and immunohistochemical staining. The clinical and pathologic features were analyzed and the literatures reviewed.</p><p><b>RESULTS</b>Histologically, AT/RT was characterized by the presence of rhabdoid cells associated with various degrees of primitive neuroectodermal, epithelial or mesenchymal differentiation. Abundant reticulin fibers and a complex immunophenotype were observed. The tumor cells were positive for vimentin, CD99, epithelial membrane antigen, cytokeratin, glial fibrillary acidic protein, S-100 protein, neurofilament, desmin and smooth muscle actin. They were negative for synaptophysin, MyoD1, placental alkaline phosphatase and HMB45.</p><p><b>CONCLUSIONS</b>AT/RT is a highly malignant tumor occurring in the central nervous system. It manifests mainly in children and occasionally in adults. The tumor is characterized by a heterogeneous histologic and immunohistochemical phenotype. It needs to be distinguished from a number of central nervous system tumors, including medulloblastoma, primitive neuroectodermal tumor, germ cell neoplasm and rhabdoid meningioma.</p>