round cell liposarcoma

round cell liposarcoma

A high-grade radiosensitive sarcoma which most commonly involves the limbs (2/3 thigh) and deep soft tissue. Round cell liposarcoma is at the aggressive end of a spectrum of liposarcomas, at the “benign” end of which is myxoid liposarcoma. 

DiffDx
Mxofibrosarcoma, metastatic carcinoma, melanoma, B- or T-cell lymphoma, Ewing sarcoma/PNET.

Prognosis
Poor outcome if ≥ 5% of tumour is composed of round cells—it metastasises to the spine, retroperitoneum, pleural cavity, soft tissue, pelvis, lung. Median survival is < 5 years.
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References in periodicals archive ?
Goldblum, "Myxoid/round cell liposarcoma of the extremities: A clinicopathologic study of 29 cases with particular attention to extent of round cell liposarcoma," The American Journal of Surgical Pathology, vol.
ALT/WDLS is low to intermediate grade tumors/malignancy and dedifferentiated liposarcoma, myxoid round cell liposarcoma, and pleomorphic liposarcoma are high grade tumors/malignancy.
According to the expanded deal, the firms will accelerate the development of NY-ESO therapy into pivotal synovial sarcoma studies and also explore development in myxoid round cell liposarcoma. The companies are also likely to start around eight proof-of-principle studies evaluating combinations with other therapies, including checkpoint inhibitors.
- Round Cell Liposarcoma Global Clinical Trials Review, H1, 2015
[USPRwire, Mon Apr 27 2015] GlobalData's clinical trial report, "Round Cell Liposarcoma Global Clinical Trials Review, H1, 2015" provides data on the Round Cell Liposarcoma clinical trial scenario.
In some areas of the tumour, a proportion of cells had morphological features compatible with round cell liposarcoma (good differentiation and low malignancy).
Histopathologically most of the tumours were of myxoid type (3 cases), followed by well differentiated (2 cases), dedifferentiated (1 case) and round cell liposarcoma (1 case).
Coakley et al., "PPARy agonists enhance ET-743 - Induced adipogenic differentiation in a transgenic mouse model of myxoid round cell liposarcoma," Journal of Clinical Investigation, vol.
Liposarcoma can be subclassified into three groups: (a) well-differentiated liposarcoma with and without dedifferentiated components, (b) myxoid and round cell liposarcoma, and (c) pleomorphic liposarcoma.
Most of myxoid/round cell liposarcomas of are found primarily in the limb and their severity may vary from low grade to high-grade tumor as seen in pure myxoid and pure round cell liposarcoma respectively; but in some cases transitional features may also be seen which behavior may depend upon the presence of round cell quantity.
Myxoid-round cell liposarcoma is a common subtype of liposarcoma characterized by the t(12;16)(q13;p11) reciprocal translocation, which results in the formation of the FUS-DDIT3 chimeric gene (128,129) found in about 95% of myxoid and round cell liposarcoma cases (Table 3).