An approach to pleomorphic sarcomas: can we subclassify, and does it matter?

Goldblum, John R.

doi:10.1038/modpathol.2013.174

Cited by 101 publications

(101 citation statements)

References 36 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…The pleomorphic variant (undifferentiated pleomorphic sarcoma, UPS) is particularly common among adults, and most frequently arises in the lower extremities (2). It is typically a high-grade lesion with a local recurrence rate ranging between 19% and 31%, a metastatic rate of 31% to 35%, and a five-year survival of 65% to 70% (3). UPS have a highly variable morphology, all sharing a marked pleomorphism often admixed with spindle cells and bizarre multinucleated giant cells.…”

Section: Introductionmentioning

confidence: 99%

Recurrent PRDM10 Gene Fusions in Undifferentiated Pleomorphic Sarcoma

Hofvander

Tayebwa

Nilsson

et al. 2015

Clinical Cancer Research

View full text Add to dashboard Cite

Purpose: Undifferentiated pleomorphic sarcoma (UPS) is defined as a sarcoma with cellular pleomorphism and no identifiable line of differentiation. It is typically a high-grade lesion with a metastatic rate of about one third. No tumor-specific rearrangement has been identified, and genetic markers that could be used for treatment stratification are lacking. We performed transcriptome sequencing (RNA-Seq) to search for novel gene fusions.Experimental design: RNA-Seq, FISH, and/or various PCR methodologies were used to search for gene fusions and rearrangements of the PRDM10 gene in 84 soft tissue sarcomas.Results: Using RNA-Seq, two cases of UPS were found to display novel gene fusions, both involving the transcription factor PRDM10 as the 3 0 partner and either MED12 or CITED2 as the 5 0 partner gene. Further screening of 82 soft tissue sarcomas for rearrangements of the PRDM10 locus revealed one more UPS with a MED12/PRDM10 fusion. None of these genes has been implicated in neoplasia-associated gene fusions before. Conclusions: Our results suggest that PRDM10 fusions are present in around 5% of UPS. Although the fusion-positive cases in our series showed the same nuclear pleomorphism and lack of differentiation as other UPS, it is noteworthy that all three were morphologically low grade and that none of the patients developed metastases. Thus, PRDM10 fusion-positive sarcomas may constitute a clinically important subset of UPS.

show abstract

Section: Introductionmentioning

confidence: 99%

Recurrent PRDM10 Gene Fusions in Undifferentiated Pleomorphic Sarcoma

Hofvander

Tayebwa

Nilsson

et al. 2015

Clinical Cancer Research

View full text Add to dashboard Cite

show abstract

“…Overall, the morphologic and immunohistochemical results described are consistent with a conclusive diagnosis of this tumor type. 25 Protein profiling describes differences in pathological status between tissues Currently, little information about the proteome of common hippos exists, but MS/MS analysis enables the observation of the proteomic changes in the different tissues, which can be compared to differentiate the physiopathological status among them. Of the proteins identified, 25 were found in healthy skeletal muscle, 27 in primary tumors, and 15 in metastatic tumors (Supplementary Information); uncommon characteristics were shown between normal and tumor tissues based on protein class annotated in GO terms.…”

Section: Resultsmentioning

confidence: 99%

Morpho/Proteomic Comparative between High Grade Pleomorphic Sarcoma and Metastasis Diagnosed in an Old Captive Common Hippo

Silva¹,

Lacerda²,

Faria³

et al. 2017

J. Braz. Chem. Soc.

View full text Add to dashboard Cite

Old age is a risk factor for cancer development in humans and animals, and studies have shown that tumors in animals are acceptable models for studying human cancers, considering the similarities between their factors. This work was conducted in a 53-year-old captive female common hippo (Hippopotamus amphibious) with a left leg tumor and metastatic mass. Histopathological and immunohistochemical analyses were carried out with a final diagnosis of a high grade pleomorphic sarcoma. A proteomic study using mass spectrometry was added in order to identify further aspects of the primary tumor and metastasis which could improve our understanding, and each tissue showed a proteomic profile indicative of its pathologic state with significant differences between healthy tissue, primary and metastatic tumors. Low levels of β-actin in primary tumors were identified, and this may be associated with a possible consequence of cytoskeleton dynamic modification. In metastatic tissue, these dynamics may be affected by the presence of HSP chaperone 60. Keywords:Hippopotamus amphibious, high grade pleomorphic sarcoma, cytoskeleton, β-actin, mass spectrometry IntroductionOncogenic phenomena in animals include a wide variety of benign and malignant tumors that develop during the life of the animal and have several health consequences. 1 Species with large bodies tend to have a lower cancer rate, which seems to indicate the presence of complex tumor detection systems (Peto's paradox).2 To illustrate this paradox, in contrast with humans, who have only two copies of the TP53 tumor suppressor gene, elephants, the largest of the land mammmals, have 20 copies of the TP53 gene. This allows for extra efficiency in their systems, enabling the correction of DNA damage or inducing apoptosis in mutated cells. As a result, only 3% of elephants develop cancer. 3,4 Despite some comparative cancer and lifespan analyses between wild and zoo animals showing that captive animals were susceptible to cancer and decreased longevity. 5,6 Tidière et al. 7 revealed that mammals from zoo populations generally lived longer than their wild counterparts (84% of species). Of particular interest, the common hippopotamus (Hippopotamus amphibius), a robust mammal with an estimated 40-year lifespan in nature and with slow pace of life, has increased longevity in captivity. [7][8][9] The relationship between life expectancy and environment may be associated with the low caloric diet and reduced metabolism present in captive animals, which slows ageing and the appearance of cancer cases. This is also true for humans. 10,11 Paradoxically, the exceptional longevity these captive animals exhibit can be associated with cancer due to several factors such as telomere shortening, increased cellular senescence, accumulation mutation of DNA in stem and progenitor cells required for active replenishment during the lifespan of the organism, mutations in tumor suppressor genes and metabolic changes. 12-14Based on available database information and proteomics approach, it is poss...

show abstract

“…Nowadays this term has been supplanted by undifferentiated pleiomorphic sarcoma (UPS) [14]. Further in the diagnosis, frequency of UPS may arise by using array-comparative genomic hybridization [Kelleher].…”

Section: Discussionmentioning

confidence: 99%

Orbital Rhabdomyosarcoma with a good life prognosis after surgical treatment in a 14-year-old patient

Nicula

Nicula²,

Blidaru

et al. 2014

Open Medicine

View full text Add to dashboard Cite

AbstractThe orbital rhabdomyosarcoma is one of the most frequent malignant orbital tumours in children. At this age, the common histological types are the embryonal and alveolar type. The onset is mainly under the age of 16. Without a recent and correct treatment it can give metastasis in lung and bone. The hereby paper presents one clinical case of a teenager presented at the ophthalmological consultation for a small tumor located in the superomedial part of the orbit. Computed tomography (CT) and magnetic resonance imaging (MRI) supported the diagnosis revealing the location and extension of the tumor. During the surgery, we discovered two small tumors and the histological examination revealed an embryonal type of orbital rhabdomyosarcoma. After the surgery, the patient followed an oncological treatment consisting of chemotherapy and local radiotherapy. The prognosis for life was favorable, linked with the recent diagnosis and treatment, the histological type and the good response at the oncological treatment.

show abstract

An approach to pleomorphic sarcomas: can we subclassify, and does it matter?

Cited by 101 publications

References 36 publications

Recurrent PRDM10 Gene Fusions in Undifferentiated Pleomorphic Sarcoma

Recurrent PRDM10 Gene Fusions in Undifferentiated Pleomorphic Sarcoma

Morpho/Proteomic Comparative between High Grade Pleomorphic Sarcoma and Metastasis Diagnosed in an Old Captive Common Hippo

Orbital Rhabdomyosarcoma with a good life prognosis after surgical treatment in a 14-year-old patient

Contact Info

Product

Resources

About