OBJECTIVETo study the clinical and pathologic features of primary cardiac inflammatory myofibroblastic tumor.

METHODSA total of 4 patients with primary cardiac inflammatory myofibroblastic tumor were encountered during the period from 1993 to 2013 in National Center for Cardiovascular Disease. The clinical features, imaging findings and outcomes of the 4 patients were evaluated. ALK protein expression and ALK gene status were studied using the archival tumor tissues.

RESULTSThere were 1 female and 3 male patients. The age of patients ranged from 5 months to 30 years (mean = 16 years). The tumor was located in right ventricle (n = 2), right atrium (n = 1) or pericardium (n = 1). Histologic patterns included 2 cases of fibrous histiocytoma type, 1 case of granulomatous type and 1 case of sclerosing type. Immunohistochemical study showed that 2 cases expressed ALK protein. Fluorescence in-situ hybridization however did not reveal any ALK gene rearrangement.

CONCLUSIONSInflammatory myofibroblastic tumor of the heart is rarely encountered and easily misdiagnosed. It carries distinctive clinical and pathologic features. ALK protein expression is helpful in arriving at the correct diagnosis.

Adolescent ; Adult ; Biomarkers, Tumor ; genetics ; metabolism ; Child ; Diagnosis, Differential ; Female ; Granuloma, Plasma Cell ; enzymology ; pathology ; Heart Neoplasms ; enzymology ; pathology ; Histiocytoma, Benign Fibrous ; enzymology ; pathology ; Humans ; Immunohistochemistry ; In Situ Hybridization, Fluorescence ; Infant ; Male ; Receptor Protein-Tyrosine Kinases ; genetics ; metabolism

OBJECTIVETo study the prevalence of IgG4-positive plasma cells in inflammatory disease of nasal cavity and paranasal sinuses and its association with IgG4-related sclerosing disease (IgG4-SD).

METHODSThe expression of IgG4 and IgG in plasma cells of 103 cases diagnosed as inflammatory disease of nasal cavity and paranasal sinuses with dense lymphoplasmacytic infiltrate was studied by immunohistochemistry (EnVision) and quantitatively analyzed by medical image analysis system.

RESULTSImmunohistochemical study showed marked infiltration by IgG4-positive plasma cells (>50 per high-power field) in 28 cases, moderate infiltration (30 to 50 per high-power field) in 23 cases, mild (10 to 29 per high-power field) in 30 cases and negative (<10 per high-power field) in 22 cases (P < 0.05). Twenty-two cases studied fulfilled the diagnostic criteria of IgG4-SD (>50 IgG4-positive plasma cells per high-power field and IgG4-to-IgG ratio > 40%), including 3 cases of chronic sinusitis (3/20), 3 cases of nasal polyps (3/18), 3 cases of inflammatory pseudotumor (3/17), 4 cases of fungal sinusitis (4/20), 1 case of rhinoscleroma (1/12), 7 cases of Wegener's granulomatosis (7/11) and 1 case of Rosai-Dorfman disease (1/2).

CONCLUSIONInflammatory disease of nasal cavity and paranasal sinuses fulfilling the diagnostic criteria IgG4-SD is not uncommon. Definitive diagnosis of IgG4-SD requires correlation with other clinical and laboratory findings. Some cases of unexplained inflammatory disease of nasal cavity and paranasal sinus may represent a member of the IgG4-SD spectrum. IgG4 carries diagnostic value in differential diagnosis of inflammatory disease occurring in nasal cavity and paranasal sinuses.

Adolescent ; Adult ; Aged ; Aged, 80 and over ; Diagnosis, Differential ; Female ; Granuloma, Plasma Cell ; metabolism ; pathology ; Granulomatosis with Polyangiitis ; metabolism ; pathology ; Histiocytosis, Sinus ; metabolism ; pathology ; Humans ; Immunoglobulin G ; metabolism ; Immunohistochemistry ; Male ; Middle Aged ; Nasal Cavity ; immunology ; pathology ; Nasal Polyps ; metabolism ; pathology ; Nose Diseases ; immunology ; pathology ; Paranasal Sinus Diseases ; immunology ; pathology ; Paranasal Sinuses ; immunology ; pathology ; Plasma Cells ; immunology ; Rhinoscleroma ; metabolism ; pathology ; Sinusitis ; metabolism ; pathology ; Young Adult

Actins ; metabolism ; Diagnosis, Differential ; Granuloma, Plasma Cell ; diagnostic imaging ; drug therapy ; metabolism ; pathology ; surgery ; Head and Neck Neoplasms ; diagnostic imaging ; drug therapy ; metabolism ; pathology ; surgery ; Humans ; Neoplasm Recurrence, Local ; Protein Kinase Inhibitors ; therapeutic use ; Pyrazoles ; therapeutic use ; Pyridines ; therapeutic use ; Tomography, X-Ray Computed ; Vimentin ; metabolism

The term inflammatory pseudotumor (IPT) has been used to describe inflammatory and fibrosing tumoral processes of an undetermined cause that may involve a variety of organ system. IgG4-related disease is a newly recognized fibroinflammatory condition characterized by IgG4-producing plasma cell expansion in affected organs and, often but not always, elevated serum IgG4 concentrations. IgG4-related IPTs, a subtype of IPT, are characterized by dense infiltration of IgG4-positive plasma cells and stromal fibrosis. The association between inflammatory pseudotumor and IgG4 was first reported with a regard to sclerosing pancreatitis. Despite there are many reports on intraperitoneal IPTs including both cellular and lymphoplasmacytic type, only a few cases have been confirmed to be IgG4-related. We experienced a case of intraperitoneal IgG4-related inflammatory pseudotumor in an 83-year-old woman presenting with epigastric pain and malaise. Surgical specimens revealed an IgG4-related inflammatory pseudotumor.
Aged, 80 and over ; C-Reactive Protein/analysis ; Female ; Granuloma, Plasma Cell/*diagnosis/pathology/surgery ; Humans ; Immunoglobulin G/*blood ; Plasma Cells/metabolism ; Positron-Emission Tomography ; Tomography, X-Ray Computed

The term inflammatory pseudotumor (IPT) has been used to describe inflammatory and fibrosing tumoral processes of an undetermined cause that may involve a variety of organ system. IgG4-related disease is a newly recognized fibroinflammatory condition characterized by IgG4-producing plasma cell expansion in affected organs and, often but not always, elevated serum IgG4 concentrations. IgG4-related IPTs, a subtype of IPT, are characterized by dense infiltration of IgG4-positive plasma cells and stromal fibrosis. The association between inflammatory pseudotumor and IgG4 was first reported with a regard to sclerosing pancreatitis. Despite there are many reports on intraperitoneal IPTs including both cellular and lymphoplasmacytic type, only a few cases have been confirmed to be IgG4-related. We experienced a case of intraperitoneal IgG4-related inflammatory pseudotumor in an 83-year-old woman presenting with epigastric pain and malaise. Surgical specimens revealed an IgG4-related inflammatory pseudotumor.
Aged, 80 and over ; C-Reactive Protein/analysis ; Female ; Granuloma, Plasma Cell/*diagnosis/pathology/surgery ; Humans ; Immunoglobulin G/*blood ; Plasma Cells/metabolism ; Positron-Emission Tomography ; Tomography, X-Ray Computed

BACKGROUND/AIMS: Hepatic angiomyolipoma (AML) is a rare mesenchymal tumor of the liver and demonstrates a marked histologic diversity. HMB-45 is a promising immunomarker for this tumor and especially helpful to diagnosis of some AMLs with unusual morphology. The purpose of this study was to better define the variable histologic feature of hepatic AML. METHODS: Eight hepatic AMLs were examined, and all of that were resection specimens. The diagnosis was confirmed by the presence of HMB-45 positive cells. Median age was 41.5 years old, and mean size of tumor was 8.94 cm. RESULTS: Conventional mixed type was 5 cases which showed myomatous, angiomatous and lipomatous component, and 3 cases were myomatous predominant. Variable patterns including spider web cell morphology, solid sheet-like and trabecular pattern were identified on myomatous component and variable amount and patterns of inflammatory cell infiltration was identified. CONCLUSIONS: With only histologic features, it is difficult to distinguish hepatic AML from other hepatic tumor including hepatocellular carcinoma or inflammatory pseudotumor. A correct diagnosis of hepatic AML is possible by a close histologic examination with immunohistochemical stainings such as HMB-45 which is important to patient's prognosis.
Adult ; Aged ; Angiomyolipoma/metabolism/*pathology/surgery ; Carcinoma, Hepatocellular/metabolism/*pathology/surgery ; Female ; Granuloma, Plasma Cell/metabolism/pathology ; Humans ; Liver Neoplasms/metabolism/*pathology/surgery ; Male ; Melanoma-Specific Antigens/*metabolism ; Middle Aged ; Tomography, X-Ray Computed

Actins ; metabolism ; Antigens, CD ; metabolism ; Antigens, Differentiation, Myelomonocytic ; metabolism ; Diagnosis, Differential ; Female ; Granuloma, Plasma Cell ; metabolism ; pathology ; Histiocytoma, Benign Fibrous ; diagnostic imaging ; metabolism ; pathology ; surgery ; Humans ; Lung Neoplasms ; diagnostic imaging ; metabolism ; pathology ; surgery ; Middle Aged ; Neprilysin ; metabolism ; Pneumonectomy ; methods ; Radiography ; Sarcoma ; metabolism ; pathology ; Solitary Fibrous Tumors ; metabolism ; pathology ; Vimentin ; metabolism ; Xanthomatosis ; metabolism ; pathology

The inflammatory myofibroblastic tumor (IMT), also knowns as inflammatory pseuduotumor, is a soft tissue lesion of unknown etiology. In the urogenital tract, IMT mainly affects the urinary bladder or prostate, but rarely the kidney. It has been considered as a nonneoplastic reactive inflammatory lesion, but nowadays, it is regarded as a neoplasm due to its high recurrence rate and metastasis. We describe a case of a 61-yr-old woman that had originally been misdiagnosed as renal cell carcinoma, which was pathologically revealed to be an IMT.
Actins/metabolism ; Carcinoma, Renal Cell/diagnosis/pathology ; Diagnosis, Differential ; *Diagnostic Errors ; Female ; Granuloma, Plasma Cell/*diagnosis/pathology/surgery ; Humans ; Kidney Neoplasms/*diagnosis/pathology ; Magnetic Resonance Imaging ; Middle Aged ; Tomography, X-Ray Computed ; Vimentin/metabolism

OBJECTIVETo investigate the clinicopathologic features and differential diagnostic methods for follicular dendritic cell sarcoma.

METHODSHistological and immunohistochemical examinations and EBER in situ hybridization were used to investigate the pathological features of 5 cases of follicular dendritic cell sarcoma, and related literature was reviewed.

RESULTSThere were 3 males and 2 females with a median age of 54 years (range, 28 - 75 years). The location of lesions included lymph node (2 cases), tonsil (1 case), stomach (1 case), and liver (1 case). The growth patterns were fascicular or whorls and/or diffuse. The neoplastic cells were spindle or ovoid in shape with indistinct border and slightly eosinophilic cytoplasm. The nuclei were round, oval or spindle in shape with small distinct nucleoli. Warthin-Finkeldey-like multinucleated giant cells were detected in two cases. Mitotic figures were found in 1-22/10 HPF. Immunohistochemical staining showed that CD21 and CD23 (3 of 5), CD35 (4 of 5), D2-40 (4 of 4), and CXCL13 (3 of 4) were positive in neoplastic cells. EBER was detected in one of five cases by in situ hybridization. Four cases were followed-up for 6 approximately 25 months and no recurrence or death was observed yet.

CONCLUSIONFollicular dendritic cell sarcoma is an extremely rare and should be considered as a moderately malignant tumor, and may present histological polymorphism with certain distinctive features. Immunohistochemistry is necessary in differential diagnosis to distinguish from other tumors.

Adult ; Aged ; Antibodies, Monoclonal ; metabolism ; Antibodies, Monoclonal, Murine-Derived ; Chemokine CXCL13 ; metabolism ; Dendritic Cell Sarcoma, Follicular ; metabolism ; pathology ; surgery ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Gastrointestinal Stromal Tumors ; metabolism ; pathology ; Granuloma, Plasma Cell ; metabolism ; pathology ; Humans ; Liver Neoplasms ; metabolism ; pathology ; surgery ; Lymph Nodes ; metabolism ; pathology ; Male ; Membrane Glycoproteins ; metabolism ; Middle Aged ; RNA-Binding Proteins ; metabolism ; Receptors, Complement 3b ; metabolism ; Receptors, Complement 3d ; metabolism ; Receptors, IgE ; metabolism ; Ribosomal Proteins ; metabolism ; Stomach Neoplasms ; metabolism ; pathology ; surgery ; Tonsillar Neoplasms ; metabolism ; pathology ; surgery

Adult ; Antigens, CD ; metabolism ; Antigens, Differentiation, Myelomonocytic ; metabolism ; Carcinoma, Giant Cell ; metabolism ; pathology ; Diagnosis, Differential ; Granuloma, Plasma Cell ; metabolism ; pathology ; Histiocytosis, Sinus ; metabolism ; pathology ; surgery ; Humans ; Immunohistochemistry ; Lung Diseases ; metabolism ; pathology ; surgery ; Lung Neoplasms ; metabolism ; pathology ; Male ; Pneumonectomy ; methods ; S100 Proteins ; metabolism ; Vimentin ; metabolism