Extrapulmonary Inflammatory Myofibroblastic Tumor (Inflammatory Pseudotumor) A Clinicopathologic and Immunohistochemical Study of 84 Cases

Coffin, Cheryl M.; Watterson, Jan; Priest, John R.; Dehner, Louis P.

doi:10.1097/00000478-199508000-00001

Cited by 1,390 publications

(1,504 citation statements)

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“…1). The histologic features were identical to previous detailed descriptions (1,6). Histologic criteria for malignant transformation included increased cellularity, increased numbers of ganglion-like plump polygonal or round cells associated with necrosis, large prominent nucleoli, and variable numbers of mitoses, including atypical mitotic figures (1,6).…”

Section: Pathologic Findingssupporting

confidence: 76%

“…Twenty-six of the cases were previously published (1). Flow cytometric ploidy analysis was previously published for 24 cases (6).…”

Section: Case Selectionmentioning

confidence: 99%

“…It occurs in pulmonary and extrapulmonary sites, is associated with recurrences and systemic symptoms, rarely undergoes malignant transformation, and is composed of differentiated myofibroblastic spindle cell accompanied by numerous plasma cells and/or lymphocytes and eosinophils (1,(3)(4)(5). Some of the persistent uncertainties about IMT are whether it is a benign, indeterminate, or malignant neoplasm, whether it represents a neoplastic or reactive process, what is the significance of multifocal or metastatic disease, and whether histologic-prognostic features can be identified (2,7,8).…”

Section: Imt Is Classified With Intermediate Neoplasms In the Currentmentioning

confidence: 99%

“…Inflammatory myofibroblastic tumor (IMT), a tumor of myofibroblastic spindle cells accompanied by a variable inflammatory infiltrate of plasma cells, eosinophils, and lymphocytes, occurs in pulmonary and extrapulmonary sites, recurs locally, manifests systemic symptoms, and rarely undergoes malignant transformation (1)(2)(3)(4). The search for histologic prognostic features has proven elusive (5,6).…”

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confidence: 99%

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ALK1 and p80 Expression and Chromosomal Rearrangements Involving 2p23 in Inflammatory Myofibroblastic Tumor

et al. 2001

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Fluorescence in situ hybridization showed ALK rearrangements in nine cases (47%), aneuploidy in three cases (16%), and no rearrangement in seven cases (37%). IMTs with ALK abnormalities by immunohistochemistry and/or fluorescence in situ hybridization originated in the abdomen/pelvis/retroperitoneum, chest, and extremities. The mean age was 6.6 years, with a male/female ratio of 1.3. 64% of patients had no evidence of disease at last followup, 45% had one or more recurrences, and 18% displayed histologic evidence of malignant transformation. The IMTs without ALK abnormalities occurred in older children, were more frequent in females, and had fewer recurrences. However, in

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Section: Pathologic Findingssupporting

confidence: 76%

“…Twenty-six of the cases were previously published (1). Flow cytometric ploidy analysis was previously published for 24 cases (6).…”

Section: Case Selectionmentioning

confidence: 99%

Section: Imt Is Classified With Intermediate Neoplasms In the Currentmentioning

confidence: 99%

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confidence: 99%

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ALK1 and p80 Expression and Chromosomal Rearrangements Involving 2p23 in Inflammatory Myofibroblastic Tumor

et al. 2001

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“…A total of 26 cases had been published previously. 18,19 All available surgical pathology reports, consultation reports, microscopic slides, and cytogenetic results were reviewed. Medical records and follow-up information were reviewed when available.…”

Section: Methodsmentioning

confidence: 99%

IgG4 plasma cells in inflammatory myofibroblastic tumor: inflammatory marker or pathogenic link?

et al. 2011

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Inflammatory myofibroblastic tumor is a rare mesenchymal neoplasm that harbors an anaplastic lymphoma kinase (ALK) gene rearrangement in the majority of cases. It is composed of fibroblastic-myofibroblastic cells with a characteristic inflammatory infiltrate that consists predominantly of plasma cells. In contrast, IgG4-related sclerosing disease is a recently described multisystem disorder with a histological appearance similar to inflammatory myofibroblastic tumor. The plasma cell infiltrate is characteristic in IgG4-related sclerosing disease and has been studied as a tool to render this diagnosis. Histologically, the two disorders overlap, although there are significant clinical differences. This study analyzes the histological appearance of 36 inflammatory myofibroblastic tumors, compares them with IgG4-related sclerosing disease, and assesses the plasma cell profile using immunohistochemistry to determine the range and proportion of IgG4 plasma cells. The majority of patients were children and young adults, mainly with solitary masses and no clinical manifestations of IgG4-related sclerosing disease. ALK-1 positivity was present in 23 cases (64%). None showed obliterative phlebitis or prominent lymphoid aggregates. Of 36 inflammatory myofibroblastic tumors, 15 cases showed an IgG4/IgG ratio Z0.10, a cutoff described in the literature as supportive of IgG4-related sclerosing disease and up to 33 IgG4-positive plasma cells per high-power field indicating a mild-to-moderate increase as compared with IgG4-related sclerosing disease. Currently, the diagnostic recognition of inflammatory myofibroblastic tumor is based on clinicopathological features and diagnostic adjuncts, such as ALK-1 reactivity and genetic tests. Although inflammatory myofibroblastic tumor and IgG4-related sclerosing disease are distinct entities, a subset of inflammatory myofibroblastic tumors exhibit an IgG4/IgG ratio that is within the range for IgG4-related sclerosing disease. Therefore, the ratio alone cannot be used as a reliable discriminator between these two entities and other clinical and pathologic features must always be taken into account.

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Immunoapheresis in Paraneoplastic Pemphigus

Schoen

Foedinger²,

Derfler³

et al. 1998

Arch Dermatol

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Background: Paraneoplastic pemphigus was first described in 1990 in 5 patients with extensive mucocutaneous erosions, a distinct set of autoantibodies, and underlying neoplasia. Since then, patients described have been middle-aged, have suffered from prognostically unfavorable malignant neoplasms, and have responded poorly to immunosuppressive agents.Observation: A 16-year-old boy was examined with extensive oral erosions, halitosis that interfered with his quality of life, and rapid weight loss. The suspected clinical diagnosis of paraneoplastic pemphigus was confirmed by histopathological, immunofluorescence, and biochemical (eg, immunoblotting and immunoprecipitation) findings as well as by the demonstration of an inflammatory myofibroblastic tumor of the left retroclavicular region.Despite administration of corticosteroids, followed by excision of the neoplasm, clinical symptoms improved only slightly, and autoantibody titers decreased only marginally. We therefore initiated an immunoapheresis regimen with the use of sheep anti-human-IgG beadformed agarose gel (Sepharose; Pharmacia Biotech Comp, Vienna, Austria), which led to the disappearance of circulating autoantibodies and the patient's recovery. Conclusion:Immunoapheresis may represent a novel therapeutic option for patients with paraneoplastic pemphigus who show little improvement after curative treatment of their neoplasms.

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Extrapulmonary Inflammatory Myofibroblastic Tumor (Inflammatory Pseudotumor) A Clinicopathologic and Immunohistochemical Study of 84 Cases

Cited by 1,390 publications

References 0 publications

ALK1 and p80 Expression and Chromosomal Rearrangements Involving 2p23 in Inflammatory Myofibroblastic Tumor

ALK1 and p80 Expression and Chromosomal Rearrangements Involving 2p23 in Inflammatory Myofibroblastic Tumor

IgG4 plasma cells in inflammatory myofibroblastic tumor: inflammatory marker or pathogenic link?

Immunoapheresis in Paraneoplastic Pemphigus

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