Inflammatory myofibroblastic tumor is a rare tumor of mesenchymal origin. A case of intratracheal inflammatory myofibroblastic tumor in a male child was reported. The clinical characteristics, diagnosis, treatment and prognosis of the disease were reviewed based on the literature, and a differential diagnosis between inflammatory myofibroblastic tumor and hamartoma was performed to ultimately confirm the nature of the tumor in the child.
Humans ; Child ; Male ; Trachea/pathology* ; Granuloma, Plasma Cell/diagnosis* ; Prognosis ; Diagnosis, Differential ; Tomography, X-Ray Computed

Inflammatory myofibroblastic tumor （IMT） is a rare mesenchymal soft tissue tumor characterized by borderline or low-grade malignancy. It is rare childhood tumor with an average age of onset of 10 years old. It is even rarer in infants and toddlers, and the etiology and pathogenesis of this tumor are still unclear. The clinical presentation of IMT is non-specific and are related to the location of the tumor. When the tumor compresses adjacent organs, it can cause pain and functional impairment. According to the current literature, IMT is most commonly found in the digestive and respiratory systems, but also occasionally occur in the genitourinary system, head and neck, and limbs. At present, there have been no reports of nasopharyngeal IMT involving nasal cavity of infants and toddlers at home and abroad.This article reports a case of a massive inflammatory myofibroblastic tumor involving the nasal cavity and nasopharynx in an infant. Plasma-assisted minimally invasive surgery was performed through multiple surgical approaches and achieved satisfactory therapeutic results. This case report may provide valuable reference for the treatment of similar diseases.
Humans ; Infant ; Granuloma, Plasma Cell/pathology* ; Nasopharynx/pathology* ; Neoplasms, Muscle Tissue ; Soft Tissue Neoplasms

Carcinoma ; diagnosis ; Granuloma, Plasma Cell ; diagnosis ; Humans ; Laryngeal Neoplasms ; diagnosis ; Larynx ; pathology

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 investigate the clinicopathologic features, immunohistochemic phenotypes and genetic alterations of extrapulmonary inflammatory myofibroblastic tumor (IMT) and the correlation with prognosis.

METHODSThirty cases of IMT with follow-up were analyzed morphologically and immunohistochemically. ALK FISH was also performed to determine the ALK gene status.

RESULTSPatients ranged in age from 12 to 73 years (mean 43.4 years). The male-to-female ratio was 1.0: 1.1. The tumors were located in various anatomical sites including gastrointestinal tract, liver, spleen, kidney, pelvic, retroperitoneum, mediastinum etc. Histologically, the majority of cases were composed of spindled fibroblastic and myofibroblastic cells accompanied by an inflammatory infiltrate of plasma cells, lymphocytes, and eosinophils. Most cases with aggressive behavior had features including prominent nucleoli and edematous myxoid background. Lymphohistiocytic reactions were usually absent. Some cases showed multinucleation, nuclear pleomorphism and mitoses. One case demonstrated epithelioid morphology with round-to-epithelioid cells. The immunohistochemical study showed vimentin, SMA, CK, desmin, and ALK were expressed in 100% (30/30), 70% (21/30), 13% (4/30), 27% (8/30), and 27% (8/30) of IMT, respectively. Diffuse cytoplasmic ALK staining was detected in seven cases. One case (containing round-to-epithelioid cells) demonstrated ALK nuclear membrane staining, coupled with positive reaction for CD30 and negative reaction for LCA. EMA, CD34, CD117 and S-100 protein, and MyoD1 were negative for all cases. Six ALK protein positive cases harbored ALK gene rearrangement, but not the remaining 22 cases. Follow-up data were available in 21 patients. After initial resection, 14 patients were alive with no evidence of disease, while 4 patients were alive with tumor recurrence and 3 patients died of the disease.

CONCLUSIONSMost IMT with aggressive behavior have features including prominent nucleoli, edematous myxoid background, and positive expression of ALK. Lymphohistiocytic reaction is usually absent. ALK may be a potential novel therapeutic target for IMT.

Adolescent ; Adult ; Aged ; Child ; Female ; Fibroblasts ; pathology ; Granuloma, Plasma Cell ; genetics ; pathology ; Humans ; Inflammation ; pathology ; Male ; Middle Aged ; Myofibroblasts ; pathology ; Prognosis ; Receptor Protein-Tyrosine Kinases ; genetics ; Young Adult

Bile Duct Neoplasms ; diagnosis ; Bile Ducts, Intrahepatic ; Cholangiocarcinoma ; diagnosis ; Diagnosis, Differential ; Female ; Granuloma, Plasma Cell ; diagnosis ; surgery ; Hepatectomy ; Humans ; Liver ; pathology ; Middle Aged

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

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

No abstract available.
Adult ; Carcinoma, Hepatocellular/radiography/radionuclide imaging/ultrasonography ; Granuloma, Plasma Cell/pathology/*radiography/radionuclide imaging ; Humans ; Liver Diseases/pathology/*radiography/radionuclide imaging ; Liver Neoplasms/radiography/radionuclide imaging/ultrasonography ; Magnetic Resonance Imaging ; Male ; 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