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Central Nervous System
Published in Pat Price, Karol Sikora, Treatment of Cancer, 2020
Previously thought of as a variant of meningioma, this tumor is now believed to be indistinguishable from hemangiopericytoma in other sites, where it is usually designated as solitary fibrous tumor. It has been thought to derive from the meningeal capillary pericyte, although the true histogenesis remains uncertain. It is a rare tumor that tends to arise in a younger age group than meningioma and is more common in men. These tumors arise in the dura as highly vascular, lobulated masses. They are densely cellular and often highly mitotic and may be graded WHO 2 or 3 on this basis. These tumors carry fusions of NAB2 and STAT6, and staining for STAT6 to identify the fusion event is recommended to confirm diagnosis. Hemangiopericytomas have a marked tendency to recur after surgery alone and to metastasize within and outside the CNS, particularly to bone. Maximal surgery is essential treatment, but post-operative high-dose radiotherapy (55–60 Gy) is often recommended to reduce local recurrence and metastasis. Median survival is around 5 years.
The respiratory system
Published in C. Simon Herrington, Muir's Textbook of Pathology, 2020
This is a rare tumour of submesothelial fibroblasts. It is often asymptomatic but some patients have cough, dyspnoea, finger clubbing, and hypoglycaemia. They are greyish/white, nodular, or lobulated tumours often attached to the lung by a pedicle. They are composed of spindle cells and harbour a characteristic NAB2-STAT6 fusion gene which leads to the overexpression of STAT6 which can aid in diagnosis. They are usually benign but some tumours can behave in a more aggressive fashion, invading the underlying lung. However, both are generally cured by complete surgical excision.
Malignant Solitary Fibrous Tumor of the Pleura Associated with a Paraneoplastic Hypoglycemia
Published in Wickii T. Vigneswaran, Thoracic Surgery, 2019
Evgeny V. Arshava, Adnan Al Ayoubi, Kalpaj R. Parekh
While several histologic subtypes exist, intersecting fascicles composed of bland spindle cells and thick bands of collagen, in a so-called randomly distributed “patternless pattern,” are characteristic features of SFTs (a review of several slides may be required to establish this pattern). Immunohistochemistry markers CD34, Bcl2, CD99, and vimentin may be inconsistently expressed, which may lead to some difficulties in establishing the diagnosis in cases with non-classical histology. Actin, desmin, S100 protein, and epithelial markers are negative. Genetically, SFTs are characterized by a translocation and resultant fusion of the NGFI-A binding protein 2 gene (NAB2) to the signal transducer and activator of transcription 6 gene (STAT6) [4]. Thus, nuclear STAT6 expression is generally specific for SFTs.
Tumor suppression by the EGR1, DMP1, ARF, p53, and PTEN Network
Published in Cancer Investigation, 2018
Kazushi Inoue, Elizabeth A. Fry
EGR1 expression in the primary tumor correlates with radiation response in terms of control of the local tumor (71, 72). Therefore, it should be an indicator for favorable therapeutic response. However, in the post-irradiated biopsies, EGR1 expression correlates with residual tumor and treatment failure (69, 71, 72). In this case, NAB2, the regulatory protein that represses the transcriptional activity of EGR1, is downregulated in human primary prostate carcinomas (8, 9, 73). Interestingly, EGR1 is overexpressed in the tumors of CR2-TAg mice where T antigen is expressed under the control of the cryptidine 2 (CR2) promoter and NAB2 expression is decreased (65, 69). Thus, both upregulation of EGR1 and loss of its repressor, NAB2, contribute to increased EGR1 activity in prostate cancer. It is hypothesized that when these two things happen together in cells with p53 dysfunction, the EGR1 expression behaves as an invasive factor rather than tumor suppressor explaining why it is associated with radiation treatment failure. Overexpression of EGR1 in prostate cancer should be reevaluated from the viewpoint of p53 because EGR1 (and also other p53-binding proteins) may stimulate mutant p53 associated with increased stem cell-ness, invasiveness/metastasis, and therapy resistance (74).
Different approaches to advanced soft tissue sarcomas depending on treatment line, goal of therapy and histological subtype
Published in Expert Review of Anticancer Therapy, 2020
Javier Martín-Broto, Peter Reichardt, Robin L Jones, Silvia Stacchiotti
Solitary fibrous tumors (SFT) were first described in 1942 [75]. Discovery of the NAB2/STAT6 gene fusion as the oncogenic mutation in SFT [76] improved diagnosis and enabled refinement of the natural history of these tumors.