scholarly journals Dedifferentiated Liposarcoma With Myofibroblastic Differentiation

2018 ◽  
Vol 142 (10) ◽  
pp. 1159-1163 ◽  
Author(s):  
Grace Y. Wang ◽  
David R. Lucas
Keyword(s):  
Dedifferentiated Liposarcoma ◽  
Pertinent Literature ◽  
The Past ◽  
Molecular Features ◽  
Major Subset ◽  
Retroperitoneal Tumors ◽  
Well Differentiated ◽  
Distinguishing Features ◽  
Myofibroblastic Differentiation ◽  
Malignant Soft Tissue

Context.— Liposarcoma is divided into myxoid, pleomorphic, well-differentiated, and dedifferentiated subtypes. Dedifferentiated liposarcoma displays the greatest histomorphologic diversity, including a subset with myofibroblastic differentiation that shares similarities with a spectrum of reactive, benign, and malignant soft tissue lesions. Misdiagnosis may lead to deleterious consequences, as dedifferentiated liposarcoma differs significantly in its prognosis and treatment from its mimics. Objective.— To review the clinicopathologic, immunohistochemical, and molecular features of the myofibroblastic variant of dedifferentiated liposarcoma as well as the key distinguishing features from its mimics. Data Sources.— Review of pertinent literature on major features and current understanding of dedifferentiated liposarcoma with myofibroblastic differentiation. Conclusions.— The myofibroblastic variant of dedifferentiated liposarcoma is an uncommon and underrecognized sarcoma with several important differential diagnoses, and likely represents the major subset of aggressive retroperitoneal tumors that may have been misdiagnosed as desmoid-type fibromatosis, inflammatory myofibroblastic tumor, or another type of sarcoma in the past.

scholarly journals Biology and Management of Dedifferentiated Liposarcoma: State of the Art and Perspectives

2021 ◽  
Vol 10 (15) ◽  
pp. 3230
Author(s):  
Jun Nishio ◽  
Shizuhide Nakayama ◽  
Kazuki Nabeshima ◽  
Takuaki Yamamoto
Keyword(s):  
Standard Treatment ◽  
Clinical Presentation ◽  
Current Knowledge ◽  
Single Agent ◽  
Dedifferentiated Liposarcoma ◽  
Cyclin Dependent Kinase ◽  
First Line ◽  
First Line Therapy ◽  
Well Differentiated ◽  
Genetic Events

Dedifferentiated liposarcoma (DDL) is defined as the transition from well-differentiated liposarcoma (WDL)/atypical lipomatous tumor (ALT) to non-lipogenic sarcoma, which arises mostly in the retroperitoneum and deep soft tissue of proximal extremities. It is characterized by a supernumerary ring and giant marker chromosomes, both of which contain amplified sequences of 12q13-15 including murinedouble minute 2 (MDM2) and cyclin-dependent kinase 4 (CDK4) cell cycle oncogenes. Detection of MDM2 (and/or CDK4) amplification serves to distinguish DDL from other undifferentiated sarcomas. Recently, CTDSP1/2-DNM3OS fusion genes have been identified in a subset of DDL. However, the genetic events associated with dedifferentiation of WDL/ALT remain to be clarified. The standard treatment for localized DDL is surgery, with or without radiotherapy. In advanced disease, the standard first-line therapy is an anthracycline-based regimen, with either single-agent anthracycline or anthracycline in combination with the alkylating agent ifosfamide. Unfortunately, this regimen has not necessarily led to a satisfactory clinical outcome. Recent advances in the understanding of the pathogenesis of DDL may allow for the development of more-effective innovative therapeutic strategies. This review provides an overview of the current knowledge on the clinical presentation, pathogenesis, histopathology and treatment of DDL.

Nasal Chondromesenchymal Hamartoma in Children

2001 ◽  
Vol 125 (3) ◽  
pp. 400-403 ◽  
Author(s):  
Chuen Hsueh ◽  
Swei Hsueh ◽  
Frank Gonzalez-Crussi ◽  
Ta-jen Lee ◽  
Jen-liang Su
Keyword(s):  
Immunohistochemical Study ◽  
Correct Diagnosis ◽  
S100 Protein ◽  
Soft Tissue Tumors ◽  
Biological Behavior ◽  
Ultrastructural Examination ◽  
Spindle Cells ◽  
Nasal Chondromesenchymal Hamartoma ◽  
Myofibroblastic Differentiation ◽  
Malignant Soft Tissue

Abstract Hamartoma in the nasal cavity of children is especially rare. Most documented cases occurred in infants, with characteristic histologic features of a mixture of various mesenchymal tissues. McDermott et al designated it nasal chondromesenchymal hamartoma in 1998, and it has since been considered a distinct clinicopathological entity. We report 2 such examples in a full-term male newborn and a 9-month-old boy, respectively. Histologically, both cases were characterized by a mixture of various mesenchymal elements, including spindle cells, collagen fibers, and irregular islands of osseous and chondroid tissue. Immunohistochemical study showed positivity to vimentin and S100 protein. Ultrastructural examination of case 1 demonstrated fibroblastic and myofibroblastic differentiation in tumor cells. There were 11 cases of nasal chondromesenchymal hamartoma in children published to date. The tumor has a benign biological behavior, and complete resection is the treatment of choice. It is apt to be misdiagnosed because of overlapping histologic features shared with a number of benign and malignant soft tissue tumors. Awareness of this entity is essential for correct diagnosis and adequate therapy.

scholarly journals Effect of premedication with oral clonidine on intraoperative blood loss during rhinoplasty surgery - A systematic review

2020 ◽  
Vol 11 (4) ◽  
pp. 5206-5213
Author(s):  
Sudarssan Subramaniam Gouthaman ◽  
Janani Kandamani ◽  
Divya Sanjeevi Ramakrishnan ◽  
P. U. Abdul Wahab
Keyword(s):  
Systematic Review ◽  
Blood Loss ◽  
Surgical Procedure ◽  
Intraoperative Blood Loss ◽  
Pertinent Literature ◽  
Intraoperative Bleeding ◽  
Oral Clonidine ◽  
Hypotensive Anaesthesia ◽  
The Past ◽  
Manual Search

Rhinoplasty is one frequent surgical procedure of many technical variations that only a few surgeons are considered to have mastered its broad scope. Operative site bleeding is considered to be an exasperating issue in the surgical procedure of rhinoplasty. Over the past few decades, the strategy of lowering patient's blood pressure during anaesthesia or "Hypotensive anaesthesia" has been practised to reduce the blood loss during surgeries. Clonidine is an antihypertensive drug and is suggested to have advantageous effects in controlling the intraoperative blood loss. The objective of this systematic review was to explore and study the existing literature and determine the efficacy of oral clonidine as a premedication in reducing the intraoperative blood loss in rhinoplasty surgeries. Data was gathered from electronic databases like PubMed, Medline and Cochrane central library. An additional manual search was performed with various journals to look for available articles to include in the systematic review. Only those studies which met the criteria for inclusion were selected. All studies and reports that evaluated oral clonidine with placebo in reducing bleeding during rhinoplasty surgery were included. Pertinent literature abstracts and full-text articles pertaining to the query were analysed. Two articles in total were taken in for qualitative analysis, both of which were randomised clinical trials. Oral clonidine shows significantly more efficient in reducing intraoperative bleeding than the placebo group. Premedication with oral clonidine is significantly effective in controlling blood loss during the surgical procedure of rhinoplasty.

scholarly journals Retroperitoneal Liposarcoma Presenting as Bilateral Inguinal Hernias

2021 ◽  
Author(s):  
DO Haley S. Lehman ◽  
DO Ryan N. Qasawa ◽  
John J. Lim
Keyword(s):  
Case Report ◽  
Soft Tissue ◽  
Soft Tissue Sarcomas ◽  
Mass Effect ◽  
Potential Space ◽  
Retroperitoneal Liposarcoma ◽  
Median Size ◽  
Inguinal Hernias ◽  
Retroperitoneal Tumors ◽  
Well Differentiated

Abstract Liposarcoma is one of the most common soft tissue sarcomas and has multiple subtypes, including atypical, well-differentiated, and dedifferentiated liposarcoma1. These tumors most commonly occur in the extremities and the retroperitoneum2, and account for 20% of all retroperitoneal tumors3. Retroperitoneal liposarcoma is very rare overall, occurring in 2.5 per one million people4. Patients will present from symptoms of mass effect due to the uncontrolled growth in the large potential space of the retroperitoneum, with its median size being around 30 cm5. The mainstay of treatment for this type of tumor is resection to a negative margin6. This is a case report describing a retroperitoneal liposarcoma presenting with bilateral inguinal hernias containing intraperitoneal fat from mass effect.

scholarly journals Cdk5 drives formation of heterogeneous pancreatic neuroendocrine tumors

Oncogenesis ◽  
2021 ◽  
Vol 10 (12) ◽  
Author(s):  
Angela M. Carter ◽  
Nilesh Kumar ◽  
Brendon Herring ◽  
Chunfeng Tan ◽  
Rachael Guenter ◽  
...  
Keyword(s):  
Neuroendocrine Tumors ◽  
Islet Cells ◽  
Tumor Development ◽  
Heterogeneous Population ◽  
Targeted Therapeutics ◽  
Pancreatic Neuroendocrine Tumors ◽  
The Past ◽  
Regulated Expression ◽  
Well Differentiated ◽  
Conditional Mouse Model

AbstractPancreatic neuroendocrine tumors (PanNETs) are a heterogeneous population of neoplasms that arise from hormone-secreting islet cells of the pancreas and have increased markedly in incidence over the past four decades. Non-functional PanNETs, which occur more frequently than hormone-secreting tumors, are often not diagnosed until later stages of tumor development and have poorer prognoses. Development of successful therapeutics for PanNETs has been slow, partially due to a lack of diverse animal models for pre-clinical testing. Here, we report development of an inducible, conditional mouse model of PanNETs by using a bi-transgenic system for regulated expression of the aberrant activator of Cdk5, p25, specifically in β-islet cells. This model produces a heterogeneous population of PanNETs that includes a subgroup of well-differentiated, non-functional tumors. Production of these tumors demonstrates the causative potential of aberrantly active Cdk5 for generation of PanNETs. Further, we show that human PanNETs express Cdk5 pathway components, are dependent on Cdk5 for growth, and share genetic and transcriptional overlap with the INS-p25OE model. The utility of this model is enhanced by the ability to form tumor-derived allografts. This new model of PanNETs will facilitate molecular delineation of Cdk5-dependent PanNETs and the development of new targeted therapeutics.

scholarly journals Genomic profiling of dedifferentiated liposarcoma compared to matched well-differentiated liposarcoma reveals higher genomic complexity and a common origin

2018 ◽  
Vol 4 (2) ◽  
pp. a002386 ◽  
Author(s):  
Hannah C. Beird ◽  
Chia-Chin Wu ◽  
Davis R. Ingram ◽  
Wei-Lien Wang ◽  
Asrar Alimohamed ◽  
...  
Keyword(s):  
Common Origin ◽  
Dedifferentiated Liposarcoma ◽  
Genomic Profiling ◽  
Genomic Complexity ◽  
Well Differentiated

Dedifferentiated Liposarcoma With a Paraganglioma-like Histologic Pattern: A Case Report and Review of the Literature

2004 ◽  
Vol 128 (7) ◽  
pp. 788-791
Author(s):  
Dating Liu ◽  
Guillermo Quinonez ◽  
Steven Latosinsky
Keyword(s):  
Neuron Specific Enolase ◽  
Malignant Neoplasm ◽  
Neuroendocrine Differentiation ◽  
Growth Patterns ◽  
Dedifferentiated Liposarcoma ◽  
Tissue Mass ◽  
Histologic Pattern ◽  
History Of ◽  
Poorly Differentiated ◽  
Well Differentiated

Abstract A 53-year-old man presented with a 4-month history of increasing abdominal discomfort and distension. A large retroperitoneal mass was found on imaging. Image-guided needle core biopsy demonstrated a poorly differentiated malignant neoplasm. A 30 × 32 × 33-cm soft tissue mass was removed. Microscopically, the tumor consisted of predominantly epithelioid malignant cells arranged in a paraganglioma-like growth pattern. Immunohistochemically, these cells were strongly positive for neuron-specific enolase. Stains for synaptophysin and chromogranin, however, were negative. There was no ultrastructural evidence of neuroendocrine differentiation. Adjacent sarcomatous areas were composed of spindled cells arranged in storiform and fibrosarcoma-like growth patterns. A small area of well-differentiated liposarcoma was identified, and a diagnosis of dedifferentiated liposarcoma was established. To the best of our knowledge, this represents the first reported case of dedifferentiated liposarcoma with a paraganglioma-like histologic pattern. A brief review focusing on the morphologic variations of dedifferentiated liposarcoma is also presented.

scholarly journals PET/CT Imaging as a Diagnostic Tool in Distinguishing Well-Differentiated versus Dedifferentiated Liposarcoma

Sarcoma ◽  
2020 ◽  
Vol 2020 ◽  
pp. 1-6
Author(s):  
Amanda Parkes ◽  
Elizabeth Urquiola ◽  
Priya Bhosale ◽  
Heather Lin ◽  
Kelsey Watson ◽  
...  
Keyword(s):  
Diagnostic Tool ◽  
Standardized Uptake Value ◽  
Percutaneous Biopsy ◽  
Cutoff Point ◽  
Dedifferentiated Liposarcoma ◽  
Positron Emission ◽  
Pet Ct ◽  
Well Differentiated ◽  
Low Sensitivity ◽  
Cart Algorithm

Distinguishing well-differentiated liposarcoma (WDLPS) from dedifferentiated liposarcoma (DDLPS) is essential given distinct treatment paradigms and chemosensitivity. Percutaneous biopsy has a low sensitivity for detecting DDLPS. We sought to identify the diagnostic utility of positron emission tomography/computed tomography (PET/CT) in identifying WDLPS versus DDLPS. An independent radiologist reviewed PET/CT images to identify target lesions and determine the maximum standardized uptake value (SUVmax). An independent pathologist review confirmed WDLPS or DDLPS histology. A binary cutoff point of SUVmax was identified using a classification and regression trees (CART) algorithm. We identified 20 patients with WDLPS or DDLPS with 26 PET/CTs performed for separate recurrences that were followed by surgical sampling. Of the 26 records, 12 were DDLPS (46%) and 14 were WDLPS (54%). Patients with DDLPS had significantly higher SUVmax than those with WDLPS (p value = 0.0035). A SUVmax of 4 was identified as the cutoff point. Using this cutoff, the sensitivity of SUVmax identifying a case as DDLPS was 83.3% (95% CI: 51.6%, 97.9%) and the specificity was 85.7% (95% CI: 57.2%, 98.2%). PET/CT is a sensitive and specific diagnostic tool to identify the presence of dedifferentiation within the tumor.

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