Undifferentiated pleomorphic sarcoma

Paiva, Ana Carolina Gonçalves de; Abreu, Marilda Aparecida Milanêz Morgado de; Souza, Marcela Pesce de

doi:10.1590/abd1806-4841.20186613

Cited by 5 publications

(3 citation statements)

References 5 publications

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“…A nodular tumefaction at the nail base can be associated as well. Many variants of OM are described; especially pigmented forms creating concern for melanoma of the nail matrix and prompting a biopsy [7], but also giant forms and forms associated with pterygium or with a cutaneous horn [8-10]. …”

Section: Discussionmentioning

confidence: 99%

Magnetic Resonance Imaging in the Diagnosis of Onychomatricoma: A Case Report

Charfi¹,

Jaber²,

Khammouma³

et al. 2019

Skin Appendage Disord

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Onychomatricoma (OM) is a rare benign tumour of the nail matrix characterized by specific clinical and histologic features. The main clinical signs are thickening of the nail plate, xanthonychia, overcurvature of the nail plate, and multiple splinter haemorrhages. The diagnosis is based on clinical, radiological and histopathological findings. Histologically, the tumour is characterized by filiform epithelial projections. The objective of this study is to present the first reported Tunisian case of OM, focusing on the contribution of magnetic resonance imaging to the diagnosis of OM. A review on the subject is also presented.

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Section: Discussionmentioning

confidence: 99%

Magnetic Resonance Imaging in the Diagnosis of Onychomatricoma: A Case Report

Charfi¹,

Jaber²,

Khammouma³

et al. 2019

Skin Appendage Disord

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“…Previously referred to as malignant fibrocystic histiocytomas, the undifferentiated pleomorphic sarcomas (UPS) encompass neoplasms that can arise in soft tissue and the dermis [ 184 ]. No true cell origin is known for UPS, and the histogenesis of UPS remains poorly defined [ 185 ]. UPS has thus been used as a wastebasket diagnostic term for tumors progressing toward undifferentiation [ 186 ].…”

Section: Alt Positive Soft Tissue Tumorsmentioning

confidence: 99%

ALT Positivity in Human Cancers: Prevalence and Clinical Insights

MacKenzie

Watters

et al. 2021

Cancers

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Since it was first described over two decades ago, the Alternative Lengthening of Telomeres (ALT) pathway has been well accepted to hold clinical significance in cancer development, cancer diagnosis and cancer treatment. In this review, we first discuss how the activation of this pathway is determined. We then provide up-to-date statistics on the cancers ALT activity is detected. Additionally, we discussed the relationship between ALT positivity and prognosis as well as the pathogenetics of the ALT positive cancers. Finally, we evaluated the pre-clinical and clinical investigation of potential therapy for ALT cancers.Abstract: Many exciting advances in cancer-related telomere biology have been made in the past decade. Of these recent advances, great progress has also been made with respect to the Alternative Lengthening of Telomeres (ALT) pathway. Along with a better understanding of the molecular mechanism of this unique telomere maintenance pathway, many studies have also evaluated ALT activity in various cancer subtypes. We first briefly review and assess a variety of commonly used ALT biomarkers. Then, we provide both an update on ALT-positive (ALT+) tumor prevalence as well as a systematic clinical assessment of the presently studied ALT+ malignancies. Additionally, we discuss the pathogenetic alterations in ALT+ cancers, for example, the mutation status of ATRX and DAXX, and their correlations with the activation of the ALT pathway. Finally, we highlight important ALT+ clinical associations within each cancer subtype and subdivisions within, as well as their prognoses. We hope this alternative perspective will allow scientists, clinicians, and drug developers to have greater insight into the ALT cancers so that together, we may develop more efficacious treatments and improved management strategies to meet the urgent needs of cancer patients.

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“…UPS is one of the most common sarcomas in older people; it is most common in patients between 50 and 70 years old, and rare in children. In general, tumours are located deeply, although recently a case of UPS with a skin location was also described [71]. The spectrum of mutations found in UPS has not been fully characterised; however, these tumour cells are quite similar to mesenchymal stem cells [72].…”

Section: Undifferentiated Pleomorphic Sarcoma and Myxofibrosarcomamentioning

confidence: 99%

Molecular biology of sarcoma

et al. 2019

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Soft tissue sarcomas are a large group of heterogenous neoplasms, many of them are highly aggressive. Most of the cases are sporadic, without any well-defined pathogenetic factor. Potential risk factors are ionizing radiation, lymphatic oedema (secondary angiosarcoma of the breast), viral infections (HHV8 and Kaposi sarcoma), exposure to chemical factors (vinyl chloride and hepatic angiosarcoma). Genetic susceptibility plays a role in a minority of cases. However, mutations in TP53, ATM and ATR genes are associated with enhanced susceptibility to radiation. Li-Fraumeni syndrome (autosomal dominant TP53 mutation) predisposes to development of malignancies, one third of them are sarcomas. Genetic alterations observed in sarcomas could be divided into three major groups characterized by: (1) chromosome translocations; (2) simple karyotype and mutations; (3) variably complex karyotypes. A large part of sarcomas belong to the first group and the specific chromosal translocations could be utilized in the diagnostic process. A smaller number of sarcomas could be assigned to the second group, e.g. desmoid fibromatosis (CTNNB1 or APC mutations) and GIST (KIT, PDGFRA, or less frequently BRAF, SDH, NF1). A large number of sarcomas are characterized by complex and variable karyotypes. Gene copy number alterations are frequent in this group, e.g. in well-differentiated liposarcoma there is an amplification of MDM2, CDK4 and HMGA2 genes or sarcoma-specific chromosomal break regions present in the CHOP gene in myxoid liposarcoma and FKHR in alveolar rhabdomyosarcoma.

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Undifferentiated pleomorphic sarcoma

Cited by 5 publications

References 5 publications

Magnetic Resonance Imaging in the Diagnosis of Onychomatricoma: A Case Report

Magnetic Resonance Imaging in the Diagnosis of Onychomatricoma: A Case Report

ALT Positivity in Human Cancers: Prevalence and Clinical Insights

Molecular biology of sarcoma

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