Dedifferentiated Liposarcoma

Thway, Khin; Jones, Robin L.; Noujaim, Jonathan; Zaidi, Shane; Miah, Aisha; Fisher, Cyril

doi:10.1097/pap.0000000000000101

Cited by 104 publications

(58 citation statements)

References 88 publications

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“…This was exclusively seen co-existing with a CDK4 amplification. This alteration has been reported most commonly in dedifferentiated/well-differentiated liposarcoma [33] and rarely in osteosarcoma [34]. In addition to the aforementioned subtypes our study detected this co-amplification in rhabdomyosarcoma, Ewing sarcoma (EWSR1 fusion positive), and an unclassified sarcoma.…”

Section: Discussionsupporting

confidence: 74%

Clinical genomic profiling to identify actionable alterations for investigational therapies in patients with diverse sarcomas

et al. 2017

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BackgroundThere are currently no United States Food and Drug Administration approved molecularly matched therapies for sarcomas except gastrointestinal stromal tumors. Complicating this is the extreme diversity, heterogeneity, and rarity of these neoplasms. Few therapeutic options exist for relapsed and refractory sarcomas. In clinical practice many oncologists refer patients for genomic profiling hoping for guidance on treatment options after standard therapy. However, a systematic analysis of actionable mutations has yet to be completed. We analyzed genomic profiling results in patients referred to MD Anderson Cancer Center with advanced sarcomas to elucidate the frequency of potentially actionable genomic alterations in this population.MethodsWe reviewed charts of patients with advanced sarcoma who were referred to investigational cancer therapeutics department and had CLIA certified comprehensive genomic profiling (CGP) of 236 or 315 cancer genes in at least 50ng of DNA. Actionable alterations were defined as those identifying anti-cancer drugs on the market, in registered clinical trials, or in the Drug-Gene Interaction Database.ResultsAmong the 102 patients analyzed median age was 45.5 years (range 8-76), M: F ratio 48:54. The most common subtypes seen in our study were leiomyosarcoma (18.6%), dedifferentiated liposarcoma (11%), osteosarcoma (11%), well-differentiated liposarcoma (7%), carcinosarcoma (6%), and rhabdomyosarcoma (6%). Ninety-five out of 102 patients (93%) had at least one genomic alteration identified with a mean of six mutations per patient. Of the 95 biopsy samples with identifiable genomic alterations, the most commonly affected genes were TP53 (31.4%), CDK4 (23.5%), MDM2 (21.6%), RB1 (18.6%), and CDKN2A/B (13.7%). Notable co-segregating amplifications included MDM2-CDK4 and FRS2-FGF. Sixteen percent of patients received targeted therapy based on CGP of which 50% had at least stable disease.ConclusionsIncorporating CGP into sarcoma management may allow for more precise diagnosis and sub-classification of this diverse and rare disease, as well as personalized matching of patients to targeted therapies such as those available in basket clinical trials.

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Section: Discussionsupporting

confidence: 74%

Clinical genomic profiling to identify actionable alterations for investigational therapies in patients with diverse sarcomas

et al. 2017

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“…Despite common genetic features, WD and DD liposarcoma can be thought of as a spectrum of disease with clear differences between WD and DD ( 5 ). By histology, WD liposarcoma (low grade) consists of adipocytes of varying size, separated by fibrous bands with hyperchromatic cells (HCs); in contrast, in DD liposarcoma (high grade), the adipocytic areas are replaced by cellular areas, frequently with mitotic figures (Figure 1 A).…”

Section: Wd and Dd: “A Good Brother And A Bad Brother”mentioning

confidence: 99%

“…In fact, tumors may be entirely WD, WD with a DD component, or rarely DD without a clear WD component. The true relationship between the WD and DD components of tumor is unclear; however, presence of DD dramatically changes the disease biology and clinical outcome ( 1 , 2 , 5 ).…”

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confidence: 99%

Hypothesis: The Intratumoral Immune Response against a Cancer Progenitor Cell Impacts the Development of Well-Differentiated versus Dedifferentiated Disease in Liposarcoma

et al. 2016

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Well-differentiated/dedifferentiated (WD/DD) liposarcoma is a rare malignancy of adipocyte origin (“fat cancer”). Tumors may be entirely WD, WD with a DD component, or rarely DD without a clear WD component. WD tumors are low grade and generally indolent, while tumors with a DD component are high grade and behave much more aggressively, with a modest potential for distant metastasis. The presence of cancer progenitor cells in WD/DD liposarcoma is suggested by clinical evidence and reported research findings. In addition, there are emerging data to support the existence of a naturally occurring, antigen-driven, and adaptive immune response within the tumor microenvironment. We hypothesize that the intratumoral immune response is directed against a cancer progenitor cell and that the outcome of this response impacts the development of WD versus DD disease. Further study will likely provide interesting insights into the disease biology of WD/DD liposarcoma that may be readily translated to other more common cancers.

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“…Soft tissue sarcomas are an extremely rare malignancy accounting only 1% of all tumors in adult patients [ 1 ]. Liposarcomas are the most common type of sarcomas arising in the retroperitoneum that could become life-threatening [ 2 , 3 ].…”

Section: Introductionmentioning

confidence: 99%

Massive retroperitoneal dedifferentiated liposarcoma in a young patient

Moyon

Moyón

Tufiño

et al. 2018

Journal of Surgical Case Reports

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Liposarcomas are rare malignant tumors that mostly develop in the retroperitoneum. They have a broad behavioral spectrum, from small masses of tissue to highly aggressive tumors. The dedifferentiation process occurs in up to 10% and it’s most likely to occur in the retroperitoneum, a process that not only changes its components but also its prognosis. These tumors can grow to a massive size since most of them do not give any symptoms until they invade the adjacent structures. Timely detection and surgery could avoid all these potentially lethal scenarios. We present a case of a 34-year-old patient, who reported a growing mass in her abdomen that reached massive proportions but remained untreated due to lack of sufficient access to healthcare facilities in her geographic location. After complete removal of the mass the patient underwent complete recovery, dedifferentiated liposarcoma was the final diagnosis.

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Dedifferentiated Liposarcoma

Cited by 104 publications

References 88 publications

Clinical genomic profiling to identify actionable alterations for investigational therapies in patients with diverse sarcomas

Clinical genomic profiling to identify actionable alterations for investigational therapies in patients with diverse sarcomas

Hypothesis: The Intratumoral Immune Response against a Cancer Progenitor Cell Impacts the Development of Well-Differentiated versus Dedifferentiated Disease in Liposarcoma

Massive retroperitoneal dedifferentiated liposarcoma in a young patient

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