inflammatory pseudotumor of nerve€¦ · epithelioid sarcoma •multinodular •epitheloid cells...
TRANSCRIPT
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Inflammatory pseudotumor
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Inflammatory pseudotumor (IPT)
• Heterogeneous group of lesions of obscure etiology
• On physical and radiographic examination often confused with malignancy
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Synonyms
• Plasma cell granuloma
• Inflammatory myofibroblastic tumor (IMT)
• Inflammatory myofibrohistiocytic proliferation
• Omental-mesenteric myxoid hamartoma
• Inflammatory fibrosarcoma
• Inflammatory myofibroblastic sarcoma
• Xanthofibroma/-granuloma
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Location
Soft tissue and viscera
1. Mesentery, omentum, retroperitoneum, pelvis, abdominal soft tissue
2. Lung, mediastinum, head and neck
3. Unuasual: Liver, GIT, uterus, bladder, pancreas, spleen, kidney, lymph nodes, CNS, Nerve
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Epidemiology
• Primary children and young adults
• Most frequent in the first 3 decades of life
• Slight female predominance
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Exact pathogenesis unknown:
• Autoimmune?
• Acute infectious?
• Postinflammatory reparative?
• Trauma?
• Neoplasia?
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ALK, Virus
• Rearrangement (Chromosome 2p23) of the anaplastic lymphoma kinase (ALK) gene in 50-70%
-> Suggests neoplasia in a subset of cases
• Rare association with Epstein-Barr virus (EBV) and HHV8 positivity
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Symptoms
• Chest pain, dyspnoea
• Gastrointestinal obstruction
• Fever, malaise, weight loss, anaemia, thrombocytosis, elevated erythrocyte sedimentation, elevated CRP
->Cytokine mediated ???
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Macroscopy
Nodular circumscribed or multinodular mass. Fleshy or myxoid cut surface. Hemorrhagia, necrosis, calcification
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Histology
• Fibroblastic-myofibroblastic cells
• Inflammatory cells
• Collagen
• Vascular proliferation
• Few mitoses, should not be marked
• Necrosis uncommon
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3 histological patterns
1. Myxoid vascular pattern
• Loosely arranged myofibroblasts in oedematous or myxoid background
• Abundant blood vessels
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3 histological patterns
2. Compact spindled cell pattern
• Compact fascicular spindle cell proliferation
• Pronounced inflammatory infiltrate, aggregates
• Ganglion - like myofibroblasts
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3 histological patterns
3. Hypocellular fibrous pattern
• Lower cellularity
• Sparse inflammatory infiltrate
• Calcification, osseous metaplasia
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Fibrohistiocytic variant
• Round epitheloid or histiocytoid cells
• Associated with RANBP2-ALK gene rearrangement
• More agressive clinical behaviour
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Immunphenotype
• SMA, Desmin+
• Fokal keratin expression (30%)
• CD68+ (histocytic-appearing cells)
• ALK+: 50-70%
• CD34-
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Differential diagnosis
Inflammatory fibroid polyp
• Submucosa of Gastric antrum
• Proliferating cells around prominent capillary vessels
• Eosinophils
• CD34+, SMA, PDGFRA
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Differential diagnosis
GIST
• Usually muscularis propria of stomach
• Spindle or epitheloid cells
• Sparse inflammation
• CD34+, CD117+, Dog1+, S100-
• KIT or PDGFRA mutations
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Differential diagnosis
Inflammatory liposarcoma
• Retroperitoneum of older adults
• Prominent inflammatory infiltrate
• Atypical hyperchromatic nuclei
• Zones of well differentiated liposarcoma
• MDM2 and CDK4+
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Differential diagnosis
Inflammatory Leiomyosarcoma
• Deep soft tissues of older adults
• Dense inflammation
• Nuclear atypia
• Areas of classic leyomyosarcoma
• Actin+, desmin+, ALK-
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Differential diagnosis
Desmoid tumor/Fibromatosis
• Usually in small bowel mesentery
• Sparse inflammation
• Fascicles of myofibroblasts with interspersed collagen
• Beta-catenin+
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Inflammatory Pseudotumor of the nerve
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Inflammatory pseudotumor of the nerve
• 9 cases have been reported
• 18 - 44 years
• Men: women: 1:1
Fibrosis with large epineurial perivascular inflammatory infiltrates near nerve fascicles
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IPT of the nerve
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Involved peripheral nerves
Facial, greater auricular, trigeminal, mandibular nerve
Sciatic nerve
Radial, ulnar, median nerve
Peroneal nerve
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Clinical presentation
• Weakness, paresis
• Paresthesia, sensory loss
• Neuropathic pain
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Serological markers
• Sedimentation rate, angiotensin-converting enzyme (ACE), anti-neutrophil cytoplasmic antibodies, antinuclear antibody, rheumatoid factor, hepatitis panel, HIV, and paraneoplastic antibodies
....normal
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IPT of sciatic nerve 61 year old women
Irregular, mass lesion that involves and surrounds the nerve with heterogeneous signal characteristics
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Intraoperative, sciatic nerve
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Histology
Chronic inflammatory, with histiocytes, plasma cells, eosinophils and fibroblasts, hemosiderin-laden macrophages
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Histology
Increased microvessels
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Histology
Compressed and atrophic nerve fibers with edema
Focal atrophy, axonal degeneration, edema, decreased density of myelin
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Histology
Fibrosis and lipocytes
Increased collagen of the perineurium and epineurium Increase in epineurial lipocytes
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Histology
Rare non-caseating granulomas and multi-nucleated giant cells in the epineurium
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Immunhistochemistry
• CD68+
• Ham 56+
• CD45+
• S100, CD1a and EMA- Histiocytes expressing CD68
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Differential diagnosis
Leprosy
• M. leprae and M. lepromatosis
• Fite positive acid fast organisms
• Linear Perivascular, periadnexal and neurotropic lymphohistiocytic infiltrate
• Granulomas
• Foamy macrophages
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Differential diagnosis
Neurofibroma
• Angulated, wavy nuclei
• Myxoid or collagenous
matrix
• Collagen bundels
• No inflammation
• S100+
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Differential diagnosis
Perineurioma
• Neoplastic perineural cells surround nerve fibers
• Onion skin formation
• No atypia
• Rare mitoses
• EMA+, S100-
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Differential diagnosis
Malignant peripheral nerve sheath tumor
• Mostly high grade sarcoma
• Hypercellular, storiform
• Spindle cell
• Necrosis, high mitotic rate
• Heterologous differentiation
• S100+
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Differential diagnosis
Epithelioid sarcoma
• Multinodular • Epitheloid cells, spindled at periphery • Mildly atypical nuclei • Central necrosis • Epithelial markers+ • Vimentin+ • INI1- • S100-
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Therapy
• Nerve sparing resection
• Improvement in pain, weakness, reduction in lesion size with intravenous steroids
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Immune component? Systemic process?
• One patient with history of excessive lacrimation which required monthly intraocular steroid injections
-> with removal of the IPT symptoms resolved
• Response to treatment with steroids
• Further studies are needed
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AJNR September 2011
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Case report
59-year-old woman with IPT of the trigeminal nerve with abundant IgG4 plasma cell infiltrates
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IgG4 related IPT
• In recent years, the relationship between some populations of IPT and IgG4 has been suggested
• Some IPTs of the liver and lung have been reported with IgG4-related sclerosing disease
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Prognosis
• Approximately 25% recurrence
• Metastasis <2%
• Metastasis may be associated with specific ALK fusion partners (RANBP2) and round cell morphology (fibrohistiocytic variant)
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Conclusions
• Increasingly recognized
• Should be considered in the differential diagnosis of peripheral nerve nodular mass lesions
• Can mimic neoplastic nerve sheath tumors
• Recognition is important to avoid overaggressive surgery