ABSTRACT
Objective: To investigate the clinicopathological characteristics, pathological diagnosis of Ewing's sarcoma of the central nervous system. Methods: Six cases of Ewing's sarcoma of the central nervous system diagnosed at the First Affiliated Hospital of Nanjing Medical University, Nanjing, China from 2015 to 2022 were collected. The clinical manifestations, histological morphology, immunophenotype and molecular genetics of these cases were analyzed. The related literature was reviewed. Results: There were four males and two females, with a male to female ratio of 2∶1. The onset age was 17-40 years, with a median age of 23 years. All 6 tumors were located in the spinal cord (2 cases of cervical vertebra, 1 case of thoracic vertebra, 2 cases of lumbar vertebra, and 1 case of sacral vertebra). The patients' clinical manifestations were mostly lumbago, weakness and numbness of lower limbs/limbs. In 1 case, the tumor recurred and metastasized to the suprasellar region and the third ventricle. Microscopically, the tumor showed diffuse infiltrative growth. In some cases, the tumor was closely related to the spinal meninges. The tumor cells were arranged in sheet, lobular, thin-rope, and nest-like patterns. Homer-Wright rosette was visible. The tumor cells were small to medium in size, and most of them had scant cytoplasm. A few cells had clear cytoplasm. Some areas were rhabdoid. The tumor cell nuclei showed focal mild pleomorphism. The chromatin was uniform and delicate while the nucleoli were not obvious. Mitosis was commonly seen. The tumor was separated by fibrous connective tissue and may be accompanied by mucinous degeneration. Immunohistochemistry showed that all tumors were positive for CD99, NKX2.2, Fli1, ERG. ATRX, H3K27me3, INI1 and BRG1 were all retained. Immunohistochemical stains for EMA, GFAP and Olig2 were negative. The Ki-67 proliferation index was 30%-70%. EWSR1 break-apart FISH test was positive. Conclusions: Ewing's sarcoma is rare in the central nervous system and needs to be distinguished from a variety of neoplasms with primitive undifferentiated small cell morphology. Immunohistochemistry and molecular genetics may be required for a proper diagnosis.
Subject(s)
Humans , Male , Female , Young Adult , Adult , Adolescent , Sarcoma, Ewing/pathology , Proto-Oncogene Protein c-fli-1 , Immunohistochemistry , Biomarkers, Tumor/genetics , Central Nervous System/pathologyABSTRACT
Objective: To investigate the clinical manifestations, histomorphology, and differential diagnosis of primary hepatic angiosarcoma. Methods: Nine cases of primary hepatic angiosarcoma diagnosed in the Department of Pathology, the First Affiliated Hospital of Nanjing Medical University from January 2014 to December 2021 were collected, including biopsy and surgical specimens. The histomorphology, clinical, and radiologic findings were analyzed. The relevant literature was also reviewed. Results: There were six males and three females, aged 30 to 73 years (mean 57 years). Grossly, the growth pattern of the tumor was classified as either mass formation or non-mass formation (sinusoidal). Microscopically, the mass-forming primary hepatic angiosarcoma were further subdivided into vasoformative or non-vasoformative growth patterns; and those non-vasoformative tumors had either epithelioid, spindled, or undifferentiated sarcomatoid features. Sinusoidal primary hepatic angiosarcoma on the other hand presented with markedly dilated and congested blood vessels of varying sizes, with mild to moderately atypical endothelial cells. Follow-up in all nine cases revealed 8 mortality ranging from 1 to 18 months (mean 5 months) from initial diagnosis. One patient was alive with disease within a period of 48 months. Conclusions: Primary hepatic angiosarcoma is a rare entity with a wide spectrum of histomorphology, and often misdiagnosed. It should be considered when there are dilated and congested sinusoids, with overt nuclear atypia. The overall biological behavior is aggressive, and the prognosis is worse.
Subject(s)
Male , Female , Humans , Hemangiosarcoma/diagnosis , Endothelial Cells/pathology , Liver Neoplasms/surgery , Prognosis , BiopsyABSTRACT
Objective: To investigate the clinicopathological, immunophenotypic, and genetic features of malignant peripheral nerve sheath tumor (MPNST). Methods: Twenty-three cases of MPNST were diagnosed at the Jiangsu Province Hospital (the First Affiliated Hospital of Nanjing Medical University), China, between January 2012 and December 2022 and thus included in the study. EnVision immunostaining and next-generation sequencing (NGS) were used to examine their immunophenotypical characteristics and genomic aberrations, respectively. Results: There were 10 males and 13 females, with an age range of 11 to 79 years (median 36 years), including 14 cases of neurofibromatosis type I-associated MPNST and 9 cases of sporadic MPNST. The tumors were located in extremities (7 cases), trunk (4 cases), neck and shoulder (3 cases), chest cavity (3 cases), paraspinal area (2 cases), abdominal cavity (2 cases), retroperitoneum (1 case), and pelvic cavity (1 case). Morphologically, the tumors were composed of dense spindle cells arranged in fascicles. Periphery neurofibroma-like pattern was found in 73.9% (17/23) of the cases. Under low magnification, alternating hypercellular and hypocellular areas resembled marbled appearance. Under high power, the tumor cell nuclei were irregular, presenting with oval, conical, comma-like, bullet-like or wavy contour. In 7 cases, the tumor cells demonstrated marked cytological pleomorphism and rare giant tumor cells. The mitotic figures were commonly not less than 3/10 HPF, and geographic necrosis was often noted. Immunohistochemically, tumor cells were positive for S-100 (14/23, 60.9%) and SOX10 (11/23, 47.8%). The loss of the CD34-positive fibroblastic network encountered in neurofibromas was observed in 14/17 of the MPNST cases. The loss of H3K27me3 expression was observed in 82.6% (19/23) of the cases. Moreover, SDHA and SDHB losses were presented in one case. NGS revealed that NF1 gene loss of function (germline or somatic) were found in all 5 cases tested. Furthermore, four cases accompanied with somatic mutations of SUZ12 gene and half of them had somatic mutations of TP53 gene, while one case with germline mutation in SDHA gene and somatic mutations in FAT1, BRAF, and KRAS genes. Available clinical follow-up was obtained in 19 cases and ranged from 1 to 67 months. Four patients died of the disease, all of whom had the clinical history of neurofibromatosis type Ⅰ. Conclusions: MPNST is difficult to be differentiated from a variety of spindle cell tumors due to its wide spectrum of histological morphology and complex genetic changes. H3K27me3 is a useful diagnostic marker, while the loss of CD34 positive fibroblastic network can also be a diagnostic feature of MPNST. NF1 gene inactivation mutations and complete loss of PRC2 activity are the common molecular diagnostic features, but other less commonly recurred genomic aberrations might also contribute to the MPNST pathogenesis.
Subject(s)
Female , Male , Humans , Child , Adolescent , Young Adult , Adult , Middle Aged , Aged , Neurofibrosarcoma , Neurofibromatosis 1 , Histones , Genes, p53 , Nerve Sheath NeoplasmsABSTRACT
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.
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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.
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<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>
Subject(s)
Child , Female , Humans , Male , Antigens, CD , Metabolism , Antigens, Differentiation, Myelomonocytic , Metabolism , Back , Calmodulin-Binding Proteins , Genetics , Dendritic Cell Sarcoma, Follicular , Metabolism , Pathology , Diagnosis, Differential , Forearm , Histiocytoma, Malignant Fibrous , Genetics , Metabolism , Pathology , General Surgery , Knee , Neoplasms, Muscle Tissue , Pathology , Neurilemmoma , Metabolism , Pathology , RNA-Binding Protein EWS , RNA-Binding Proteins , Genetics , Soft Tissue Neoplasms , Genetics , Metabolism , Pathology , General Surgery , Vimentin , MetabolismABSTRACT
<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>
Subject(s)
Adolescent , Adult , Female , Humans , Male , Middle Aged , Angiolymphoid Hyperplasia with Eosinophilia , Pathology , Antigens, CD34 , Metabolism , Bone Neoplasms , Metabolism , Pathology , General Surgery , Diagnosis, Differential , Follow-Up Studies , Hemangioendothelioma, Epithelioid , Pathology , Hemangioma , Metabolism , Pathology , General Surgery , Platelet Endothelial Cell Adhesion Molecule-1 , Metabolism , Skin Neoplasms , Metabolism , Pathology , General SurgeryABSTRACT
<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>
Subject(s)
Child , Female , Humans , Infant , Male , Middle Aged , Actins , Metabolism , Antigens, CD34 , Metabolism , Bone Neoplasms , Diagnostic Imaging , Metabolism , Pathology , Clavicle , Pathology , Diagnosis, Differential , Femur , Pathology , Hemangioendothelioma , Diagnostic Imaging , Metabolism , Pathology , Hemangiosarcoma , Pathology , Humerus , Pathology , Immunohistochemistry , Kasabach-Merritt Syndrome , Diagnostic Imaging , Metabolism , Pathology , Platelet Endothelial Cell Adhesion Molecule-1 , Metabolism , Radiography , Sarcoma, Kaposi , Diagnostic Imaging , Metabolism , Pathology , Vimentin , MetabolismABSTRACT
<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>
Subject(s)
Adult , Aged , Humans , Male , Middle Aged , Young Adult , Actins , Metabolism , Desmin , Metabolism , Diagnosis, Differential , Digital Rectal Examination , Fibronectins , Metabolism , Fibrosarcoma , Diagnosis , Metabolism , Pathology , General Surgery , Follow-Up Studies , Immunohistochemistry , Leiomyosarcoma , Diagnosis , Metabolism , Pathology , General Surgery , Myogenin , Metabolism , Myosins , Metabolism , Neoplasm Recurrence, Local , Prostate-Specific Antigen , Metabolism , Prostatectomy , Methods , Prostatic Neoplasms , Diagnosis , Metabolism , Pathology , General Surgery , Rhabdomyosarcoma, Embryonal , Diagnosis , Metabolism , Pathology , General Surgery , Sarcoma , Diagnosis , Metabolism , Pathology , General Surgery , Survival Rate , Vimentin , MetabolismABSTRACT
<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>
Subject(s)
Aged , Aged, 80 and over , Humans , Male , Middle Aged , DNA-Binding Proteins , Genetics , Metabolism , Enhancer of Zeste Homolog 2 Protein , Polycomb Repressive Complex 2 , Prognosis , Prostatic Hyperplasia , Metabolism , Pathology , Prostatic Neoplasms , Metabolism , Pathology , RNA, Messenger , Genetics , Transcription Factors , Genetics , MetabolismABSTRACT
<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>
Subject(s)
Adolescent , Adult , Aged , Child , Female , Humans , Male , Middle Aged , Young Adult , Calcium , Blood , Parathyroid Hormone , Blood , Parathyroid Neoplasms , Diagnosis , General Surgery , Parathyroidectomy , Retrospective StudiesABSTRACT
<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>
Subject(s)
Adult , Aged , Aged, 80 and over , Female , Humans , Middle Aged , Young Adult , Bone Neoplasms , Breast Neoplasms , Classification , Metabolism , Pathology , Carcinoma, Ductal, Breast , Metabolism , Pathology , Disease-Free Survival , Follow-Up Studies , Immunohistochemistry , Keratin-5 , Metabolism , Keratin-6 , Metabolism , Lung Neoplasms , Neoplasm Recurrence, Local , Neoplasms, Basal Cell , Metabolism , Pathology , Prognosis , ErbB Receptors , Metabolism , Receptor, ErbB-2 , Metabolism , Receptors, Estrogen , Metabolism , Receptors, Progesterone , Metabolism , Survival RateABSTRACT
<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>
Subject(s)
Adult , Female , Humans , Male , Middle Aged , Young Adult , 12E7 Antigen , Antigens, CD , Metabolism , Antigens, CD34 , Metabolism , Cell Adhesion Molecules , Metabolism , Dermatofibrosarcoma , Pathology , Diagnosis, Differential , Follow-Up Studies , Hemangiopericytoma , Metabolism , Pathology , General Surgery , Histiocytoma, Benign Fibrous , Pathology , Immunohistochemistry , Neoplasm Recurrence, Local , Orbital Neoplasms , Metabolism , Pathology , General Surgery , Proto-Oncogene Proteins c-bcl-2 , Metabolism , Soft Tissue Neoplasms , Pathology , Solitary Fibrous Tumor, Pleural , Metabolism , Pathology , General Surgery , Solitary Fibrous Tumors , Metabolism , Pathology , General SurgeryABSTRACT
<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>
Subject(s)
Adult , Aged , Aged, 80 and over , Female , Humans , Male , Middle Aged , Young Adult , Breast Neoplasms , Epidemiology , Metabolism , Pathology , General Surgery , Breast Neoplasms, Male , Epidemiology , Metabolism , Pathology , General Surgery , Carcinoma, Ductal, Breast , Epidemiology , Metabolism , Pathology , General Surgery , China , Epidemiology , Disease-Free Survival , Follow-Up Studies , Keratin-5 , Metabolism , Neoplasm Metastasis , Neoplasm Recurrence, Local , ErbB Receptors , Metabolism , Receptor, ErbB-2 , Metabolism , Receptors, Estrogen , Metabolism , Survival RateABSTRACT
<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>
Subject(s)
Adult , Female , Humans , Antigens, CD , Antigens, Differentiation, Myelomonocytic , Dendritic Cell Sarcoma, Follicular , Pathology , Dendritic Cells, Follicular , Pathology , Granuloma, Plasma Cell , Herpesvirus 4, Human , Genetics , Allergy and Immunology , Splenic Neoplasms , PathologyABSTRACT
<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>