NAB2 exon 4 STAT6 exon 2 / 4: most common genotype, associated with thoracic location and older age (Hum Pathol 2015;46:347, Mod Pathol 2015;28:1324, Cancer Med 2016;5:159) NAB2 exon 6 STAT6 exon 16 / 18: associated with extrapleural location and aggressive phenotype (Hum Pathol 2015;46:347, Mod Pathol 2015;28:1324) Pathology Outlines Immunohistochemical stains for SMA and h-caldesmon are positive in tumor cells, while all of the following are negative: keratin AE1 / AE3, CD34, desmin, HMB45, S100 and STAT6. Goldblum: Rosai and Ackerman's Surgical Pathology, 11th Edition, 2017, Mills: Sternberg's Diagnostic Surgical Pathology, 6th Edition, 2015. Page views in 2021: 58,915. We have rearranged our websites menus to put the most important pages on the top line, which remains fixed as you scroll down. Table of Contents. Immunostains SMA and CD34 were focal positive and CK AE1 / AE3, caldesmon, STAT6, desmin and S100 were negative. Schwannomas are more common in the cerebellopontine angle and spinal cord but not convexity, so the main differential diagnosis in this case is meningioma versus solitary fibrous tumor. Pathology Outlines Breast nonmalignant - Myofibroblastoma. STAT6+ (specific marker) NAB2-STAT6 fusion Neurofibroma (particularly myxoid variant): Spindle cells with wavy nuclei within a collagenous to myxoid stroma (Head Neck Pathol 2020;14:374) S100+, SOX10+ Perineurial markers (EMA, GLUT1, claudin1) expressed in scattered perineurial cells CD34+ stromal cells Epithelioid fibrous Which of the following is most likely the correct diagnosis? Pathology Outlines WebSoft tissue stains: brachyury caldesmon CD99 CDK4 D2-40 desmin DOG1 DUX4 ERG FLI1 GLUT1 H3K27me3 HMB45 KIT / CD117 MDM2 MelanA MUC4 MYOD1 myogenin NKX2 S100 SMARCA4 / BRG1 SMARCB1 / IN1 SMA SOX10 STAT6 TFE3 TLE1 PubMed Search: STAT6[TI] pathology free full text[sb] Page views in 2021: 11,926. This website is intended for pathologists and laboratory personnel but not for patients. PubMed Search: STAT6[TI] pathology free full text[sb] Page views in 2021: 11,926. Endoscopy was notable for white plaques in the esophagus, longitudinal furrows and pseudotrachea. Mesenchymal chondrosarcoma [TI] free full text[sb] pathology, Malignant biphasic mesenchymal neoplasm with a well differentiated hyaline cartilage component, Malignant biphasic neoplasm that most commonly involves soft tissue, bone and intracranial sites, with tendency for late local recurrences and distant metastases, Most commonly bone, soft tissue (head and neck, thigh), intracranial sites (meninges) and visceral organs, Combination of these pathway dysregulations (including Notch signaling pathway, chromatin remodeling, apoptosis and transforming growth factor beta [TGF] signaling) results in a chimeric fusion protein that drives the biology of mesenchymal chondrosarcoma (, Clinical symptoms depend on the tumor location and can last for years prior to diagnosis, Neurologic symptoms If arising in spine / cranium (, Diagnostic diagram includes radiology, histology (biopsy, resection specimen) with immunohistochemistry and molecular analysis, Lobulated, destructive lytic lesion with granular, ring and arc or irregularly shaped calcifications (, Bone lesion: usually erodes / destroys the cortex and involves the surrounding soft tissue, Lobulated, heterogeneous lesion with low signal intensity on T1 weighted images and high signal intensity on T2 (, In one study, 5, 10 and 20 year overall survival was 55.0%, 43.5% and 15.7%, respectively (, With chemotherapy: 5 year and 10 year overall survival is 84% and 80%, respectively, Without chemotherapy, under surveillance: 5 year and 10 year overall survival is 73% and 46%, respectively (, 30 year old man with dizziness, headache and imbalance (, 31 year old man with vascular lesion near knee (, 34 year old man with expansile, erosive tumor of mandible (, 52 year old man with progressive proptosis of eye (, Wide local excision with negative margins, If tumor is not resected in toto, chemotherapy can be used (however, better survival is published in only one study) (, Lobulated, solid, firm, gray-tan, fleshy mass with scattered gritty white calcifications, Bone lesions arise in the medullary cavity or on the bone surface, causing cortical destruction and extension into the soft tissue (, Tumor composed of islands of well differentiated hyaline cartilage and primitive mesenchymal cells / undifferentiated small blue cells, Solid sheets of undifferentiated small blue cells with a hemangiopericytoma-like vascular pattern mixed with islands of mature appearing, well differentiated hyaline cartilage, Usually mitotic activity or necrosis present (, Highly cellular smears composed of small round blue cells with high nuclear to cytoplasmic ratio infiltrating the fibrillary matrix (, Extraskeletal mesenchymal chondrosarcoma (see comment). Janeway's Immunobiology - 9th Edition Goldblum: Rosai and Ackerman's Surgical Pathology, 11th Edition, 2017, Mills: Sternberg's Diagnostic Surgical Pathology, 6th Edition, 2015. Pathology Outlines Pathology Outlines Janeway's Immunobiology - 9th Edition STAT6+ (specific marker) NAB2-STAT6 fusion Neurofibroma (particularly myxoid variant): Spindle cells with wavy nuclei within a collagenous to myxoid stroma (Head Neck Pathol 2020;14:374) S100+, SOX10+ Perineurial markers (EMA, GLUT1, claudin1) expressed in scattered perineurial cells CD34+ stromal cells Epithelioid fibrous Inflammatory myofibroblastic tumor: Prominent inflammatory infiltrate ALK positive and negative for DOG1 and CD117. Pathology Outlines Immunohistochemical stains for SMA and h-caldesmon are positive in tumor cells, while all of the following are negative: keratin AE1 / AE3, CD34, desmin, HMB45, S100 and STAT6. Pathology Outlines JAK/STAT signaling pathway: from bench to Among the choices, SSTR2a is the most sensitive and specific marker for meningioma and STAT6 is the most sensitive and specific marker for solitary fibrous Immunohistochemically, the tumor cells are immunoreactive for CD99, EMA (subset) and TLE1, while all of the following are negative: h-caldesmon, myogenin, MyoD1, STAT6, SOX10 and WT1 (C terminus). May be beta catenin+ but is typically diffuse for CD34 and STAT6. WebMacrophage polarization refers to how macrophages have been activated at a given point in space and time. Malignant biphasic mesenchymal neoplasm with a well differentiated hyaline cartilage component Most cases have HEY-NCOA2 fusions; however IRF2BP2-CDX1 fusion has also been described (PLoS One 2012;7:e49705) Your IP address is listed in our blacklist and blocked from completing this request. However, we cannot answer medical or research questions or give advice. Immunostains SMA and CD34 were focal positive and CK AE1 / AE3, caldesmon, STAT6, desmin and S100 were negative. WebSoft tissue stains: brachyury caldesmon CD99 CDK4 D2-40 desmin DOG1 DUX4 ERG FLI1 GLUT1 H3K27me3 HMB45 KIT / CD117 MDM2 MelanA MUC4 MYOD1 myogenin NKX2 S100 SMARCA4 / BRG1 SMARCB1 / IN1 SMA SOX10 STAT6 TFE3 TLE1 maximum count) (see comment). Features favoring primary mediastinal large B cell lymphoma: Localized to mediastinum, absent systemic involvement on staging imaging, Intermediate sized cells with clear cytoplasm in a sclerotic stroma, Unlike classic Hodgkin lymphoma, primary mediastinal large B cell lymphoma expresses, Polymorphic background that may be seen in classic Hodgkin lymphoma is absent in primary mediastinal large B cell lymphoma, B cell lymphoma, unclassifiable, with features intermediate between diffuse large B cell lymphoma and classic Hodgkin lymphoma, Most frequent histologic finding is a variable microscopic appearance in different areas (some areas look like classic Hodgkin lymphoma, some areas look like primary mediastinal large B cell lymphoma), Mediastinal gray zone lymphoma is characterized by a heterogenous immunophenotype among lymphoma cells in an individual case OR the immunophenotype of the lymphoma cells does not match the morphologic appearance (for example, cells look like classic Hodgkin lymphoma but have preserved B cell program; cells do not look like classic Hodgkin lymphoma but show classic Hodgkin lymphoma immunophenotype), Variable expression of B cell antigens (primary mediastinal large B cell lymphoma consistently express the B cell antigens in homogenous pattern), Although both mediastinal gray zone lymphomas and primary mediastinal large B cell lymphoma usually express. Pathology Outlines Has NAB2-STAT6 gene fusions (diffuse STAT6 and CD34 IHC positivity) and negative for 12q13-15 amplification Additional references. We welcome suggestions or questions about using the website. STAT6 (useful to distinguish meningioma [negative] from solitary fibrous tumor / hemangiopericytoma [positive]) (Clin Neuropathol 2017;36:56) Molecular / cytogenetics description Majority of atypical meningiomas have loss of NF2 combined with either genome instability (large scale chromosomal alterations) or loss of SMARCB1 ( Nat STAT6 positive (strong diffuse) (Hum Pathol 2016;54:184) Gastrointestinal stromal tumor (GIST): secondarily from rectum (Am J Surg Pathol 2006;30:1389) Additional references. We welcome suggestions or questions about using the website. Pathology Outlines Spindle cell lipoma is diffusely positive for CD34 and demonstrates loss of expression of RB1. Pathology Outlines www.pathologyoutlines.com/topic/softtissuemyopericytoma.html, Mozilla/5.0 (Macintosh; Intel Mac OS X 10_15_6) AppleWebKit/537.36 (KHTML, like Gecko) Chrome/92.0.4515.159 Safari/537.36. However, we cannot answer medical or research questions or give advice. NAB2 exon 4 STAT6 exon 2 / 4: most common genotype, associated with thoracic location and older age (Hum Pathol 2015;46:347, Mod Pathol 2015;28:1324, Cancer Med 2016;5:159) NAB2 exon 6 STAT6 exon 16 / 18: associated with extrapleural location and aggressive phenotype (Hum Pathol 2015;46:347, Mod Pathol 2015;28:1324) Lower urinary tract symptoms (LUTS), including: Obstructive symptoms (hesitancy, intermittent stream and straining), Urinary bladder irritation symptoms (frequency, urgency and urge incontinence), Urinary retention (whether acute or chronic), Severity assessed through self administered questionnaire (, Physical examination (including digital rectal exam, assessment of bladder distention, motor and sensory deficits of the lower extremities and perineum and decreased anal sphincter tone to identify neurogenic bladder), Serum prostate specific antigen (PSA) measurement not recommended routinely (, Urinalysis may reveal hematuria, proteinuria or evidence of urinary tract infection, Prostate specific antigen (PSA) may be elevated (, Imaging typically not specific for benign prostatic hyperplasia, Often performed to rule out other causes of lower urinary tract symptoms, Hypoechogenic nodules or variable echogenicity on ultrasound, Nodules ranging from hypointense to hyperintense on MRI T2 weighted images, depending on stroma / gland ratio (, 7 subtypes of benign prostatic hyperplasia on MRI based on location of the hyperplasia (, Not a precursor or risk factor for prostate cancer (, Side effects of medications may increase with increasing age (, Immediate and long term complications of surgical treatment are relatively rare (, Risk of recurrence varies with type of treatment (, 17 year old boy with acute urinary retention due to benign prostatic hyperplasia (, 61 year old man with spontaneous prostatic hematoma in a case of benign prostatic hyperplasia (, 71 year old man with primary prostate intravascular large B cell lymphoma presenting as prostatic hyperplasia (, 78 year old man with giant benign prostatic hyperplasia (, Watchful waiting for patients with mild symptoms or moderate to severe symptoms with minimal impairment in quality of life (, Medical treatment based on alpha blockers (nonselective; alpha-1A selective), 5-alpha-reductase inhibitors, phosphodiesterase type 5 inhibitors and combination products (, Surgical treatment, including ablative (such as transurethral resection, simple prostatectomy, transurethral laser vaporization, ablation, enucleation) and other procedures (such as transurethral incision, prostatic urethral lift device) reserved for patients who either have failed medical management or have complications (, Variably sized nodules with a gray to yellow color and a granular appearance bulge above the cut surface of a prostate section. Copyright PathologyOutlines.com, Inc. Click, 30100 Telegraph Road, Suite 408, Bingham Farms, Michigan 48025 (USA). Leads to constitutive phosphorylation of the receptor tyrosine kinase and activation of downstream pathways The 12q13-q15 region contains multiple genes implicated in the pathogenesis of liposarcoma, including MDM2, CDK4, HMGA2, TSPAN31, OS1, OS9, CHOP, STAT6 and GLI (Virchows Arch 2010;456:277, Genes Chromosomes Cancer 1999;24:30, Genes Chromosomes Cancer 1995;14:8, Cancer Genet Cytogenet 1997;99:14, Mod Pathol Pathology Outlines Three broad pathways control polarization: epigenetic and cell survival Neither necrosis nor mitotic activity is identified. Page views in 2022 to date: 10,507. Pathology Outlines Pathology Outlines Pathology Outlines Table of Contents. Pathology Outlines NAB2 exon 4 STAT6 exon 2 / 4: most common genotype, associated with thoracic location and older age (Hum Pathol 2015;46:347, Mod Pathol 2015;28:1324, Cancer Med 2016;5:159) NAB2 exon 6 STAT6 exon 16 / 18: associated with extrapleural location and aggressive phenotype (Hum Pathol 2015;46:347, Mod Pathol 2015;28:1324) The overall findings are those of diffuse large B cell lymphoma with histologic, immunophenotypic and clinical features compatible with designation as primary mediastinal (thymic) large B cell lymphoma. Pathology Outlines Stomach - Gastrointestinal stromal tumor (GIST) Activating mutations in the proto-oncogene KIT (~75%) (Science 1998;279:577) or platelet derived growth factor receptor- (PDGFR-) (~10%) (Science 2003;299:708) . STAT6 positive (strong diffuse) (Hum Pathol 2016;54:184) Gastrointestinal stromal tumor (GIST): secondarily from rectum (Am J Surg Pathol 2006;30:1389) Additional references. Macrophage Polarization | Annual Review of Physiology Mitotic figures and cellular apoptosis are increased. Pathology Outlines Brisk mitoses and necrosis were present. Pathology Outlines Lymphoma cells are present in a diffuse distribution and consist of intermediate to large sized neoplastic cells with round to ovoid nuclei, vesicular chromatin and occasionally prominent nucleoli. Breast nonmalignant - Myofibroblastoma. Gastrointestinal stromal tumor (GIST): Leiomyoma Myofibroblastoma Myopericytoma PEComa Neither necrosis nor mitotic activity is identified. We have rearranged our websites menus to put the most important pages on the top line, which remains fixed as you scroll down. As an introductory text, students will appreciate the book's clear writing and informative illustrations, while advanced students and working immunologists will appreciate its comprehensive scope and depth. JAK/STAT signaling pathway: from bench to By immunohistochemistry, lymphoma cells are positive for CD20 (strong and diffuse), BCL2, CD30 (subset, heterogeneous intensity) and CD23 (subset) and are negative for CD10, BCL6 and CD3. Pathology Outlines Brisk mitoses and necrosis were present. Pathology Outlines Histologic sections consist of endobronchial biopsies of the lung mass and show respiratory mucosa that is involved by lymphoma. The eosinophils have a top heavy distribution and few eosinophilic microabscesses are identified. We have rearranged our websites menus to put the most important pages on the top line, which remains fixed as you scroll down. Inflammatory myofibroblastic tumor: Prominent inflammatory infiltrate ALK positive and negative for DOG1 and CD117. Pathology Outlines As an introductory text, students will appreciate the book's clear writing and informative illustrations, while advanced students and working immunologists will appreciate its comprehensive scope and depth. Malignant biphasic mesenchymal neoplasm with a well differentiated hyaline cartilage component Most cases have HEY-NCOA2 fusions; however IRF2BP2-CDX1 fusion has also been described (PLoS One 2012;7:e49705) The STAT6 gene encodes 850 amino acids, and the tyrosine phosphorylation site in the STAT6 protein at position 641 marks the activation of STAT6. Goldblum: Rosai and Ackerman's Surgical Pathology, 11th Edition, 2017, Mills: Sternberg's Diagnostic Surgical Pathology, 6th Edition, 2015. Immunostains SMA and CD34 were focal positive and CK AE1 / AE3, caldesmon, STAT6, desmin and S100 were negative. WebJaneway's Immunobiology is a textbook for students studying immunology at the undergraduate, graduate, and medical school levels. Board review The stroma contains strands of wiry collagen. Pathology Outlines Pathology Outlines We welcome suggestions or questions about using the website. Mitoses are sparse (2 mitoses/10 high power fields). Inflammatory myofibroblastic tumor: Prominent inflammatory infiltrate ALK positive and negative for DOG1 and CD117. Positive for nuclear STAT6 (J Neuropathol Exp Neurol 2017;76:289) Negative for inhibin alpha (J Neuropathol Exp Neurol 2017;76:289) Glioma: Smear shows fibrillary processes, less cohesion Ependymoma: Perivascular pseudorosettes, ependymal rosettes and canals Positive for GFAP (Appl Immunohistochem Mol Morphol 2000;8:25) Pathology Outlines This website is intended for pathologists and laboratory personnel but not for patients. Among the choices, SSTR2a is the most sensitive and specific marker for meningioma and STAT6 is the most sensitive and specific marker for solitary fibrous Pathology Outlines Might have morphologic similarities with type A thymoma; expresses STAT6, CD34 Additional references J Thorac Oncol 2015;10:1383 , Am J Surg Pathol 2015;39:427 , Arch Pathol Lab Med 2017;141:232 , J Thorac Oncol 2017;12:354 , Histopathology 2017;70:522 , Eur J Cardiothorac Surg 2019;56:2 , Histopathology Comment: The biopsy demonstrates marked reactive basal zone hyperplasia with increased eosinophils. Associated granulation tissue is seen. STAT6 positive and negative for DOG1 and CD117. Perforated esophagus with eosinophilic esophagitis, Endoscopic features of eosinophilic esophagitis. Pathology Outlines Spindle cell lipoma is diffusely positive for CD34 and demonstrates loss of expression of RB1. Immunohistochemically, the tumor cells are immunoreactive for CD99, EMA (subset) and TLE1, while all of the following are negative: h-caldesmon, myogenin, MyoD1, STAT6, SOX10 and WT1 (C terminus). Page views in 2021: 58,915. Multifactorial, chronic eosinophilic inflammatory condition affecting the esophagus in both adult and pediatric patients that occurs in the absence of identifiable secondary causes (Med Clin North Am 2019;103:29) Characterized by a chronic immune reaction to environmental and food allergens leading to a deficient esophageal mucosal The STAT6 gene encodes 850 amino acids, and the tyrosine phosphorylation site in the STAT6 protein at position 641 marks the activation of STAT6. Soft tissue: brachyury caldesmon CD99 CDK4 D2-40 desmin DOG1 DUX4 ERG FLI1 GLUT1 H3K27me3 HMB45 c-kit / CD117 MDM2 MelanA MUC4 MYOD1 myogenin NKX2 S100 SMARCA4 / BRG1 SMARCB1 / IN1 SMA SOX10 STAT6 TFE3 TLE1 Breast Pathology Outlines 37 year old woman with superficial myofibroblastoma in the vulva (Case Rep Pathol 2019;2019:1582714) 53 year old woman with epithelioid myofibroblastoma mimicking invasive lobular carcinoma (Int J Surg Pathol 2015;23:284) 55 year old postmenopausal woman with 2 cm mass (J Midlife Copyright PathologyOutlines.com, Inc. Click, 30100 Telegraph Road, Suite 408, Bingham Farms, Michigan 48025 (USA). Pathology Outlines and Joshua A. Hanson, M.D. Best Pract Res Clin Endocrinol Metab 2010;24:957, J Neurol Neurosurg Psychiatry 2001;70:644, J Neurol Neurosurg Psychiatry 1999;67:758, Metastatic clear cell renal cell carcinoma, Appl Immunohistochem Mol Morphol 2000;8:25, Louis: WHO Classification of Tumours of the Central Nervous System, 4th Edition, 2016, Perry: Practical Surgical Neuropathology - A Diagnostic Approach, 2nd Edition, 2017, Burger: Smears and Frozen Sections in Surgical Neuropathology, 1st Edition, 2009, Benign, slowly growing, highly vascular neoplasm containing neoplastic stromal cells which usually involves the cerebellum, brainstem or spinal cord, Generally associated with loss or inactivation of the, Circumscribed, enhancing solid cystic mass, most frequently located in the cerebellum and observed as a cyst with enhancing mural nodule (, Composed of neoplastic stromal cells with foamy cytoplasm within a background network of numerous small vessels, Other neoplasms associated with VHL may be histologic mimics (e.g. Three broad pathways control polarization: epigenetic and cell survival MYC expression by immunohistochemistry is detected in 30% of tumor cell nuclei. vascular endothelial factor, platelet derived growth factor, transforming growth factor alpha) (, Autosomal dominant tumor predisposition syndrome resulting from inactivating germline mutation in the, Sporadic: up to 78% of sporadic cases with, Familial (VHL syndrome): ~60% of VHL disease patients develop hemangioblastoma (, Increased intracranial pressure / hydrocephalus (, Secondary polycythemia due to erythropoietin production (, Kidney: clear cell renal cell carcinoma, cysts, Pancreas: neuroendocrine islet cell tumor, cysts, Based on histologic and immunophenotypic features (, Clinical history of VHL syndrome or evidence of genetic alterations leading to, Secondary polycythemia (~5 - 40% of cases) (, Well demarcated enhancing mass ranging from solid to cystic, frequently presenting as a cyst with an enhancing mural nodule (, Spinal cord tumors often associated with syrinx (, Nodule: T1 hypointense to isointense, T2 hyperintense (, Serpentine flow voids in the nodular portion (, Mural nodule is isodense to the brain on noncontrast scans (, Excellent prognosis with complete surgical resection (, Outcome better in sporadic cases than in VHL syndrome cases (, 41 year old man with a family history of VHL syndrome and a synonymous, 42 year old man with cerebellopontine angle tumor (, 54 year old woman with a past medical history of VHL syndrome and bilateral clear cell renal cell carcinoma found to have an enhancing intramedullary mass at C6 / C7 (, 63 year old man with suspected posterior inferior cerebellar artery aneurysm (, Stereotactic radiosurgery may provide some control for unresectable tumors but data on safety and long term efficacy is limited (, Well circumscribed and pseudoencapsulated, Red with yellow / orange cut surface regions attributed to lipid content, Composed of neoplastic stromal cells arranged between numerous small vessels, Compact, generally noninfiltrative growth with variable lobularity, Mild nuclear pleomorphism with degenerative atypia, Clear, foamy cytoplasm with lipid containing vacuoles, Adjacent parenchyma may have piloid gliosis with Rosenthal fibers, Generally resistant to smear (squash preparation), resulting in larger tissue clumps, Successful cytologic smear preparations include the following (, Clumping of cohesive cells often obscures cytologic features, Lipid droplet containing stromal cells seen between capillaries (, Up to 78% of cases show loss / inactivation of, Distinctive epigenetic classifying signature can be determined by genome wide DNA methylation array profiling (, MicroRNA (miRNA) patterns include elevated miRNA-9 and decreased miRNA-200a (, Usually lacks foamy / vacuolated cytoplasm, Smear shows fibrillary processes, less cohesion, Perivascular pseudorosettes, ependymal rosettes and canals, Compact fibrillar portions with Rosenthal fibers, Loose microcystic portions with eosinophilic granular bodies. 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