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Skin
Published in Pritam S. Sahota, James A. Popp, Jerry F. Hardisty, Chirukandath Gopinath, Page R. Bouchard, Toxicologic Pathology, 2018
Zbigniew W. Wojcinski, Lydia Andrews-Jones, Daher Ibrahim Aibo, Rie Kikkawa, Robert Dunstan
Mastocytomas (mast cell tumors) are benign or malignant tumors of mast cells often forming nodules in the subcutis (Frith et al. 2001). Mastocytomas are composed of well-differentiated mast cells containing characteristic metachromatic granules (i.e., toluidine blue or Giemsa stained). Tumors may contain varying amounts of collagen and are characteristically infiltrated by eosinophils. The principal differential diagnosis is histiocytic sarcoma (Frith et al. 2001). Canine mastocytomas often occur in the dermis and exhibit collagen necrosis (Hottendorf and Nielsen 1966). Two distinct histologic subtypes have been identified in cats: one consisting of solitary, discrete tumors in the dermis consisting of slightly atypical mast cells, and the other characterized by multiple, discrete subcutaneous nodules of histiocytic-like cells with few toluidine blue cytoplasmic granules (Wilcock et al. 1986). Spontaneous regression was observed in some cats within a 2-year period.
Histiocytic and Dendritic Cell Neoplasms
Published in Dongyou Liu, Tumors and Cancers, 2017
Classified along with mature B-cell neoplasms, mature T and NK neoplasms, Hodgkin lymphoma, and post-transplant lymphoproliferative disorders (PTLD) under the mature lymphoid, histiocytic, and dendritic neoplasms category, histiocytic and dendritic cell neoplasms consist of (i) histiocytic sarcoma (HS), (ii) Langerhans cell histiocytosis (LCH), (iii) Langerhans cell sarcoma (LCS), (iv) indeterminate dendritic cell tumor (IDCT), (v) interdigitating dendritic cell sarcoma (IDCS), (vi) follicular dendritic cell sarcoma (FDCS), (vii) disseminated juvenile xanthogranuloma (DJX), and (viii) Erdheim–Chester disease (ECD) (Table 27.1) [1].
Update on the classification of T-cell lymphomas, Hodgkin lymphomas, and histiocytic/dendritic cell neoplasms
Published in Expert Review of Hematology, 2019
Akira Satou, N. Nora Bennani, Andrew L. Feldman
HS is a rare neoplasm showing morphologic and immunophenotypic evidence of histiocytic differentiation [118]. There is a wide age range, from infant to elderly, showing a peak at 50–69 years. HS mostly affects extranodal sites, particularly the gastrointestinal tract, skin, and soft tissue. Some cases present with nodal lesions. Cases can be localized or disseminated. Most patients present with advanced clinical stage, respond poorly to therapy, and die of progressive disease [118,119]. BRAF V600E mutations occur in nearly two-thirds of HS [120]. A targeted next-generation sequencing study recently demonstrated recurrent mutations activating the Ras- mitogen-activated protein kinase (MAPK) pathway (MAP2K1, KRAS, NRAS, BRAF, PTPN11, NF1, CBL) and the phosphoinositide 3-kinase (PI3K) pathway (PTEN, MTOR, PIK3R1, PIK3CA) in 57% and 21% of HS cases, respectively [121]. The tumor-suppressor gene CDKN2A was the most frequently altered gene (46%). These findings suggest that MAPK, PI3K, and cyclin-CDK4/6-INK4 signaling may cooperatively contribute to the pathogenesis of histiocytic sarcoma. Another recent study found that microsatellite instability and tumor mutational burden were generally low among histiocytic neoplasms, although the patient with the highest mutational burden had HS [122]; these findings may have implications for the application of checkpoint inhibitor therapies to patients with histiocytic neoplasms.
A chronic eyelid lesion in a child: multi-disciplinary approach to diagnosis, treatment and management of a highly atypical histiocytic lesion
Published in Pediatric Hematology and Oncology, 2022
Archana Ramgopal, Julia Segal, Sabrina Mukhtar, Jenny Yu, Jennifer Picarsic, Jean M. Tersak, Steven W. Allen
Malignant histiocytic neoplasm with histiocytic sarcoma (HS) phenotype is an extraordinarily rare malignant neoplasm arising from myeloid precursor derived hematopoietic tissue, accounting for less than one percent of all hematolymphoid neoplasms. It is distinguished cytologically by cells with phenotypic characteristics of mature tissue histiocytes.1,2 However, there are no defined or well-established criteria for an “atypical” juvenile xanthogranuloma (JXG) family that bears an increased proliferation index and atypia outside of what is expected for a typical benign JXG family lesion. JXG is often identified as a solitary lesion on the head and neck region, seen with characteristic Touton giant cells, and is typically positive for vimentin, CD68, and factor XIIIa, and negative for S-100 and CD1a.3 The Ki-67/MIB1 proliferation index is typically less than 20%; however, one described case that had an elevated Ki-67 (up to 40%) went on to have an aggressive clinical behavior and harbored a BRAF fusion.4,5 We present the case of a teenager presenting with a histiocytic eyelid lesion with a monomorphic infiltrate without Touton giant cells, an unusually high Ki-67 index of > 50%, lack of Factor XIIIa expression, but with a quiescent molecular profile. The differential diagnosis for such a lesion in a child includes a low-grade histiocytic lesion with atypical pathologic features versus a high-grade malignant histiocytic neoplasm with an unusual site and age.6,7 This report highlights the importance of an integrated team approach to the diagnosis of pediatric histiocytic neoplasms, especially those that hover between low-grade and high-grade neoplasms.
Wiskott-Aldrich syndrome gene mutations modulate cancer susceptibility in the p53± murine model
Published in OncoImmunology, 2018
Marton Keszei, Joanna S. Kritikou, Deborah Sandfort, Minghui He, Mariana M.S. Oliveira, Hannah Wurzer, Raoul V. Kuiper, Lisa S. Westerberg
Immune phenotyping showed that a large proportion (more than 50%) of spindle cells in sarcomas were positive for F4/80 (macrophage, myeloid lineage) (Figure 3A). This phenotype is consistent with histiocytic sarcoma, a frequent tumor in C57BL/6 mice that can be triggered with chronic low-dose irradiation.27 FACS analysis of the tumor cell suspension indicated a large proportion of hematopoietic (CD45+) cells in the tumor mass, which mostly came from the myeloid lineage and contained neutrophils when the tumor was ulcerating (Figure 3B and Table 1A).