1/15. Inflammatory myofibroblastic tumor with HMGIC rearrangement.Inflammatory pseudotumors or inflammatory myofibroblastic tumors (IMT) are lesions of extreme heterogeneity showing a highly variable mixture of bland-looking spindle cells, inflammatory cells, and collagen fibers. We describe our results of molecular cytogenetic and rapid amplification of cDNA ends (RACE-PCR) studies on an IMT characterized by a translocation involving 12q15. Chromosomal aberrations involving this region are very frequent among other benign tumors, such as lipomas, uterine leiomyomas, or pulmonary chondroid hamartomas. Recently, we have shown that, by these structural chromosomal aberrations, the HMGIC gene is affected. fluorescence in situ hybridization (FISH) analysis and 3' RACE-PCR on cells of the present case of an inflammatory myofibroblastic tumor indicated an intragenic rearrangement of HMGIC, resulting in an aberrant transcript of that gene. Clonal cytogenetic aberrations have been described in very few cases of IMT. The results presented herein indicate that this case of IMT represents a true benign mesenchymal neoplasm associated with, or due to, a rearrangement of HMGIC.- - - - - - - - - - ranking = 1keywords = hybridization (Clic here for more details about this article) |
2/15. Specific detection of epstein-barr virus in inflammatory pseudotumor of the spleen in a patient with a high serum level of soluble IL-2 receptor.A case of inflammatory pseudotumor of the spleen is described in a 63-year-old woman who presented with an intrasplenic tumor and an elevated serum level of soluble interleukin 2 receptor (sIL-2R). Microscopic examination after removal of the spleen revealed that the tumor was composed of mixed cellular infiltrates, mainly lymphocytes and plasma cells, and spindle-cell proliferation. Epstein-Barr virus (EBV) was specifically detected in the tumor by in situ hybridization for EBV rna. The serum level of sIL-2R level was normalized after splenectomy. EBV infection may play a role in the development of splenic inflammatory pseudotumor and the elevation of sIL-2R level.- - - - - - - - - - ranking = 1keywords = hybridization (Clic here for more details about this article) |
3/15. ALK probe rearrangement in a t(2;11;2)(p23;p15;q31) translocation found in a prenatal myofibroblastic fibrous lesion: toward a molecular definition of an inflammatory myofibroblastic tumor family?A prenatal tumor located in the lumbar paravertebral area was discovered during a routine ultrasound examination at 32 weeks of pregnancy and surgically removed at 4 months of life. The histopathological diagnosis was first suggested to be an infantile desmoid fibromatosis. The tumor karyotype showed a three-way translocation involving both chromosomes 2 and a chromosome 11, t(2;11;2)(p23;p15;q31). fluorescence in situ hybridization with a probe flanking the ALK gene at 2p23 demonstrated a rearrangement, as previously described in inflammatory myofibroblastic tumors (IMTs). In light of the genetic analysis, the histopathological diagnosis was revised to IMT, although inflammatory cells were scarce. IMTs are pseudosarcomatous inflammatory lesions that primarily occur in the soft tissue and viscera of children and young adults. Our report describes for the first time the occurrence of IMT during prenatal life. The ALK rearrangement may represent the molecular definition of a subgroup of mesenchymal tumors, not always with complete morphological features of IMT, similar to the model of EWS rearrangement in the Ewing sarcoma family of tumors.- - - - - - - - - - ranking = 1keywords = hybridization (Clic here for more details about this article) |
4/15. Plasma cell granuloma--an enigmatic lesion: description of an extensive intracranial case and review of the literature.We present an unusual case of intracranial plasma cell granuloma in a 70-year-old man with a 6-month history of progressive visual disturbance. The lesion extensively involved the cranial base, extended into the frontal region, and reached the floor of the third ventricle in the suprasellar area. Microscopic examination of multiple diagnostic transsphenoidal biopsies showed an inflammatory proliferation with a predominance of cells that were immunohistochemically determined to be polyclonal plasma cells. Ultrastructural analysis confirmed the presence of numerous mature plasma cells in a mixed inflammatory proliferation. in situ hybridization for Epstein-Barr viral rna revealed no evidence of viral expression. The patient was treated with steroid therapy and radiotherapy, without any appreciable reduction of the lesion's size. He is alive with persistent severe visual disturbance 14 months after the diagnosis. We discuss the etiopathogenetic, diagnostic, and therapeutic issues related to this entity, and review the literature.- - - - - - - - - - ranking = 1keywords = hybridization (Clic here for more details about this article) |
5/15. Inflammatory pseudotumor of the spleen associated with a clonal Epstein-Barr virus genome. Case report and review of the literature.We report a case of an inflammatory pseudotumor (IPT) of the spleen occurring in an 81-year-old woman with a history of a monoclonal gammopathy of undetermined significance. Eighteen-month follow-up after splenectomy demonstrated no tumor recurrence or progression of underlying plasma cell disease. Histologic examination of the tumor demonstrated a polymorphic population of inflammatory and epithelioid and spindle cells. immunophenotyping showed large numbers of T cells, B cells, and polyclonal plasma cells. The epithelioid and spindle cells were positive for vimentin and CD68 but lacked expression of follicular dendritic cell markers and actin. Epstein-Barr virus (EBV) genome was identified in the epithelioid and spindle cell population by in situ hybridization using probes specific for EBV-encoded RNAs (EBER1 and EBER2). Southern blot analysis of digested dna extracted from the tumor using an EBV-specific probe (XhoI) demonstrated the presence of a single high-intensity band, indicative of EBV monoclonality. While there have been 2 previous reports of hepatic IPTs containing a monoclonal population of EBV-infected tumor cells, this is the first report of such an association occurring in the spleen. The presence of clonal EBV dna suggests some splenic IPTs may be true neoplasms.- - - - - - - - - - ranking = 1keywords = hybridization (Clic here for more details about this article) |
6/15. Intraocular inflammatory myofibroblastic tumor with ALK overexpression.We report a case of an intraocular inflammatory myofibroblastic tumor nearly filling the vitreous cavity of the eye of a 50-year-old man. The tumor was composed of a mixture of spindle cells and mixed inflammatory elements, including numerous plasma cells. The differential diagnosis included inflammatory pseudotumor and neoplastic mimics of this condition. Further investigation with immunohistochemistry revealed the mass to be composed of myofibroblasts, positive for smooth muscle actin stains and with weak anaplastic lymphoma kinase (ALK) expression in some tumor cells. Evaluation by fluorescence in situ hybridization revealed the tumor cells to have multiple copies of chromosome 2 and ALK but no rearrangement of the ALK gene. The authors propose that multiple copies of the ALK gene may be involved in inflammatory myofibroblastic tumor tumorigenesis, in addition to ALK gene rearrangements.- - - - - - - - - - ranking = 1keywords = hybridization (Clic here for more details about this article) |
7/15. Oligoclonal T-cell populations in an inflammatory pseudotumor of the pancreas possibly related to autoimmune pancreatitis: an immunohistochemical and molecular analysis.Inflammatory pseudotumors (IPT), also known as inflammatory myofibroblastic tumors (IMT), are benign inflammatory processes that may have an infectious etiology and are very rare in the pancreatico-biliary region. Recent studies suggest a biological distinction between IPT and IMT, the latter being a true neoplastic process. We describe a case of pancreatic IPT, originally diagnosed as malignancy, which presumably recurred 4 months after the operation. Histologically, the tumor consisted of a smooth muscle actin and CD68-positive spindle cell population and a more abundant mononuclear inflammatory cell population, primarily composed of macrophages and t-lymphocytes. Inflammatory cells were the source of connective tissue growth factor and transforming growth factor-beta1 and tended to accumulate around nerves and blood vessels, as well as around residual pancreatic parenchymal elements, where an intense angiogenetic response was detected. comparative genomic hybridization analysis of the tumor showed no chromosomal imbalances. polymerase chain reaction-based analysis of T-cell receptor gamma gene rearrangement revealed an oligoclonal pattern. These findings suggest that the pathogenesis of aggressive cases of IPT could be related to the development of an intense and self-maintaining immune response, with the emergence of clonal populations of t-lymphocytes. The relation of the pancreatic IPT to autoimmune pancreatitis is emphasized.- - - - - - - - - - ranking = 1keywords = hybridization (Clic here for more details about this article) |
8/15. Inflammatory pseudotumor of the spleen with EBV positivity: report of a case.Inflammatory pseudotumor (IPT) of the spleen is a rare benign tumor with unknown etiology. It causes problems in the diagnosis because of mimicking some hematopoetic malignancies. Here we report the case of a 36-yr-old woman complaining of nausea and insomnia. Laboratory investigations were limited to increase of leukocyte and thrombocyte count. ultrasonography and magnetic resonance (MR) imaging showed circumscribed solid lobulated mass, measuring about 6.5 cm in diameter, located in the dorsal region of the spleen. splenectomy was performed with the differential diagnosis including hamartoma and lymphoma of the spleen. Histological examination of the sharply demarcated splenic mass consisted of myofibroblasts and admixture of inflammatory cells. immunohistochemistry and in situ hybridization were performed. IPT of the spleen was diagnosed. Epstein-Barr virus (EBV) was detected in the tumor by in situ hybridization. This rare entity is presented because of its clinical, radiological and pathological difficulties in the differential diagnosis.- - - - - - - - - - ranking = 2keywords = hybridization (Clic here for more details about this article) |
9/15. Inflammatory myofibroblastic tumor with ALK/TPM3 fusion presenting as ileocolic intussusception: an unusual presentation of an unusual neoplasm.Inflammatory myofibroblastic tumor is a rare spindle cell lesion of indeterminate malignant potential occurring in both pulmonary and extrapulmonary tissues. This report describes an unusual presentation of an unusual tumor at an unusual location: an intramural ileal case of inflammatory myofibroblastic tumor presenting with intussusception in a 29-year-old woman. We characterize this tumor through microscopic and ultrastructural analysis, extensive immunohistochemical analysis, ploidy analysis, and Epstein-Barr virus in situ hybridization, and we report the finding of an ALK/TPM3 fusion using fluorescence in situ hybridization.- - - - - - - - - - ranking = 2keywords = hybridization (Clic here for more details about this article) |
10/15. In situ expression of T-helper type 2 immunoregulatory response in myofibroblast of a pediatric bladder inflammatory pseudotumor.Inflammatory pseudotumor (IP) is generally considered to be a non-neoplastic, inflammatory response despite its gross and microscopic features of spindle cell neoplasm. Reported herein is a case arising from the muscularis propria of the urinary bladder in a 14-year-old girl who presented with gross hematuria and impending hypovolemic shock. The tumor consisted of proliferating myofibroblastic cells and various types of inflammatory cells. The immunoregulatory responses and reactions were investigated with regard to infiltration of inflammatory mononuclear cells being a documented finding in IP. Immunhistochemical and in situ hybridization studies for cytokines showed that the myofibroblastic cells were positive for interleukin (IL)-4, IL-5 and negative for interferon (IFN)-gamma, and tumor necrosis factor (TNF)-alpha expression. The patient has been free of disease since excision of the tumor 34 months ago. Thus, cytokine production in IP suggests that it is reactive in nature and myofibroblasts may be the source of cytokines in the pathogenic process.- - - - - - - - - - ranking = 1keywords = hybridization (Clic here for more details about this article) |
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