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

Humans ; Dendritic Cell Sarcoma, Follicular/diagnosis* ; Granuloma, Plasma Cell/diagnosis* ; Biopsy, Needle ; Gastrointestinal Neoplasms ; Hepatitis

Diagnosis, Differential ; Endoscopy ; Granuloma, Plasma Cell/diagnosis* ; Humans ; Orbital Pseudotumor ; Skull Base

Systemic lupus erythematosus (SLE) is a systemic autoimmune disease affecting various organs. Among its manifestations, inflammatory pseudotumor (IPT) is an extremely rare disease about which no case has been reported of it occurring in the liver. We present a case of a SLE patient with hepatic IPT (hIPT) successfully treated with immunosuppressants. A 16-year-old male with elevated liver enzymes visited our clinic and was diagnosed as SLE. Although no lesion was observed in the initial abdomen ultrasonography, the abdominal CT on hospital day 7 revealed a new hepatic mass resembling an abscess. Despite 5 weeks of antibiotics treatment, the hepatic mass remained, and was re-diagnosed as hIPT secondary to SLE with an abdominal MRI. After high dose prednisolone and mycophenolate mofetil treatment, lupus activity subsided and hIPT disappeared in the follow-up CT. This case suggests that hIPT should be considered as a differential diagnosis among hepatic mass in SLE patients.
Abdomen ; Abscess ; Adolescent ; Anti-Bacterial Agents ; Autoimmune Diseases ; Diagnosis, Differential ; Follow-Up Studies ; Granuloma ; Granuloma, Plasma Cell ; Humans ; Immunosuppressive Agents ; Liver ; Liver Neoplasms ; Lupus Erythematosus, Systemic ; Lupus Nephritis ; Magnetic Resonance Imaging ; Male ; Plasma Cells ; Prednisolone ; Rare Diseases ; Tomography, X-Ray Computed ; Ultrasonography

Isolated gastric IgG4-related disease (IgG4-RD) is a very rare tumefactive inflammatory condition, with only a few cases reported to date. A 48-year-old woman was incidentally found to have a subepithelial tumor in the stomach. Given a presumptive diagnosis of gastrointestinal stromal tumor or neuroendocrine tumor, she underwent wedge resection. The lesion was vaguely nodular and mainly involved the submucosa and proper muscle layer. Microscopically, all classical features of type I autoimmune pancreatitis including lymphoplasmacytic infiltration, storiform fibrosis, obliterative phlebitis, and numerous IgG4-positive plasma cells were seen. She had no evidence of IgG4-RD in other organs. Although very rare, IgG4-RD should be considered one of the differential diagnoses in the setting of gastric wall thickening or subepithelial mass-like lesion. Deep biopsy with awareness of this entity might avoid unnecessary surgical intervention.
Autoimmune Diseases ; Biopsy ; Diagnosis ; Diagnosis, Differential ; Female ; Fibrosis ; Gastrointestinal Stromal Tumors ; Granuloma, Plasma Cell ; Humans ; Middle Aged ; Neuroendocrine Tumors ; Pancreatitis ; Phlebitis ; Plasma Cells ; Stomach*

IgG4-related disease is a relatively new disease entity characterized by elevated serum IgG4 levels and marked infiltration of IgG4-positive plasma cells in lesions. Organ enlargement or nodular lesions consisting of abundant infiltration of lymphocytes and IgG4-positive plasma cells and fibrosis are seen in various organs throughout. We encountered a patient with an inflammatory pseudotumor of the rectum, which was histopathologically confirmed to be an IgG4-related disease. The patient was a 28-year-old woman who had constipation for 3 months. The endoluminal ultrasonography showed a lesion that was heterogeneous and low echogenic in lower rectum. The result of colonoscopic biopsy findings was of chronic proctitis with lymphoid aggregates. For a confirmative diagnosis, excision was performed. Histopathological examination represented plasma cell infiltration and fibrosis. Immunohistochemistry revealed prominence of IgG4-positive plasma cells and confirmed the diagnosis of IgG4-related disease. The patient is currently under observation on low-dose oral prednisolone without relapse.
Adult ; Biopsy ; Colonoscopy ; Constipation ; Diagnosis ; Female ; Fibrosis ; Granuloma, Plasma Cell ; Humans ; Immunoglobulin G ; Immunohistochemistry ; Lymphocytes ; Plasma Cells ; Prednisolone ; Proctitis ; Rectum* ; Recurrence ; Ultrasonography

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

Immunoglobulin G4 (IgG4)-related disease is a newly named fibroinflammatory condition characterized by tumefactive lesions that contain dense lymphoplasmacytic infiltrates rich in IgG4-positive cells. Elevated serum IgG4 concentrations also often accompany IgG4-related disease. Many medical conditions that were long viewed as diseases confined to single organs (e.g., Mikulicz disease, type 1 autoimmune pancreatitis, Riedel's thyroiditis, multifocal fibrosclerosis, inflammatory pseudotumor, mediastinal fibrosis, retroperitoneal fibrosis, and etc.) are now designated as part of the spectrum of IgG4-related disease. The preferred nomenclature suggested by a committee of international experts uses the prefix "IgG4-related-" for individual organ involvement, regardless of the organ system affected. One exception is type 1 autoimmune pancreatitis (IgG4-related pancreatitis). Comprehensive diagnostic criteria for IgG4-related disease and organ-specific diagnostic criteria (e.g., IgG4-related dacryoadenitis and sialadenitis, type 1 autoimmune pancreatitis, IgG4-related kidney disease and IgG4-related sclerosing cholangitis) can aid clinicians in the diagnosis of this erratic condition.
Autoimmune Diseases ; Dacryocystitis ; Diagnosis ; Fibrosis ; Granuloma, Plasma Cell ; Immunoglobulin G ; Immunoglobulins* ; Kidney Diseases ; Mikulicz' Disease ; Pancreatitis ; Retroperitoneal Fibrosis ; Sialadenitis ; Thyroid Gland ; Thyroiditis

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

BACKGROUND/AIMS: Inflammatory pseudotumor (IPT) of the liver is a rare disease characterized by chronic infiltration of inflammatory cells. However, the clinical characteristics and outcomes of IPT remain uncertain. METHODS: Clinical features, image findings, and outcomes of 55 patients with histologically proven IPT were evaluated. RESULTS: They consisted of 26 men and 19 women with median age of 65 years. Serum carcinoembryonal antigen and carbohydrate antigen 19-9 levels were normal in 42 patients (93.3%). Enhanced CT scans indicated poorly defined peripheral enhancement (82.5%) at the arterial phase and poorly defined hyperattenuating lesions with internal hypoattenuating areas at the equilibrium phase (77.0%). Gadolinium-enhancement MRI revealed poorly defined peripheral rim-like enhancement (77.8%). Ten patients underwent surgical resection and 35 were treated conservatively with or without antibiotics. No recurrence was noted after surgical resection during follow-up (1 to 48 months). In all patients who received conservative treatment, complete resolution or size reduction was noted during follow-up (1 to 192 months). CONCLUSIONS: CT and MRI provide clues to the diagnosis of IPT in patients with liver masses and normal tumor markers. However, due to the lack of pathognomonic findings, the clinician's suspicion and histological diagnosis are necessary to make an accurate diagnosis of IPT.
Adult ; Aged ; Aged, 80 and over ; Contrast Media/diagnostic use ; Female ; Granuloma, Plasma Cell/*diagnosis/therapy ; Humans ; Liver Diseases/*diagnosis/therapy ; Magnetic Resonance Imaging/methods ; Male ; Middle Aged ; Prognosis ; Tomography, X-Ray Computed/methods