1.First Department of Orthopaedics, Athens University Medical School, ATTIKON University Hospital, Athens, Greece
Well-differentiated liposarcomas are most commonly found in the extremities and the retroperitoneum, and, less often, in the head and neck area. The tumors usually arise from deep-seated, well-vascularized structures, and rarely from submucosal or subcutaneous fat. Patients usually note a deep seated, slow growing mass in their soft tissue. Only when the tumor is very large do symptoms of pain or functional disturbances occur. Retroperitoneal tumors may present with signs of weight loss and abdominal pain, or mass effect to the kidney or ureter leading to kidney failure.
Imaging- Radiography: May show a faint soft tissue mass or swelling; areas of calcification or bony erosion may be related to dedifferentiation or higher tumor grade. Chest radiography and computed tomography may be used as screening for pulmonary metastases.- Sonography: Ultrasonography shows a well-circumscribed uniformly hyperechoic mass.- Computed tomography: Usually shows a lobulated, well-circumscribed mass with multiple septations and variable contrast enhancement. Computed tomography is superior to MR imaging in detailing cortical bone erosion and tumor mineralization.- MR imaging: Well-differentiated liposarcomas have well-defined and mostly lobulated margins. The tumors are encapsulated and composed of mainly fat with septa or nodules. Their signal intensity is intermediate between subcutaneous fat and muscle; well-differentiated liposarcomas are hyperintense on T2-weighted images, and they demonstrate faint enhancement or no enhancement after the intravenous administration of contrast material, and linear septations. Diffuse enhancement is usually observed following gadolinium administration. There may be incomplete fat suppression because of the nature and amount of lipids.- Scintigraphy: An early-phase bone scan may show a marked increase of radioisotopic uptake.
Microscopically: Light microscopy typically shows mature adipocytic cells, atypical stromal cells and limited number of scattered lipoblasts. Four sybtypes have been distinguished:- Adipocytic or Lipoma-like: the most common form; it is composed of a relatively mature adipocytic proliferation in which, in contrast to benign lipoma, significant variation in cell size, focal adipocytic nuclear atypia and hyperchromasia, and scattered hyperchromatic as well as multinucleate stromal cells are often identified (see figure 3 and 4).- Sclerosing: the second in frequency, most often seen in retroperitoneal or paratesticular lesions. It is characterized by the presence of scattered bizarre stromal cells, marked nuclear hyperchromasia and rare multivacuolated lipoblasts set in an extensive fibrillary collagenous stroma. Occasionally, the fibrous component may represent the majority of the neoplasm.- Inflammatory: rare variant, most often seen in the retroperitoneum. A chronic inflammatory infiltrate predominates to the extent that the adipocytic nature of the neoplasm can be obscured.- Spindle cell: it is composed of a fairly bland neural-like spindle cell proliferation set in a fibrous and/or myxoid stroma, and an atypical lipomatous component which usually includes lipoblasts.
Interpretative difficulties arise when atypical lipomatous tumors contain fibrous or myxoid areas whose cellularity and mitotic rate are higher than in the typical tumors but lower than in the typical dedifferentiated component of dedifferentiated liposarcoma. Many studies pointed out that the cut-off portion of myxoid and fibrous areas with higher mitotic rate and cellularity is uncertain. This portion has been regarded 1 cm (up to 3 cm in large tumors) and considered as a sign of low-grade dedifferentiation from the very beginning.Similarity to large deep-seated lipomas is also a diagnostic problem. Several authors proposed a positivity in over-expression of MDM2, HMGA2 and CDK4 as a reliable hallmark to distinguish between these two entities, and genetic differences between low and higher grade liposarcomas. Others reported that CDK4 in ALT/WD liposarcomas is expressed inconsistently and is associated with adverse prognosis compared to the MDM2/CDK4 genotype.
Differential diagnosis: Lipoma; lipoblastoma; hibernoma; neurofibroma. Cytogenetics may be of value when diagnosing lipomatous tumors because different tumor types have different more or less specific chromosomal abnormalities.
Andreas F Mavrogenis ; Panayiotis J Papagelopoulos
Soft Tissues: Well-differentiated liposarcoma
Atlas Genet Cytogenet Oncol Haematol. 2013-02-01
Online version: http://atlasgeneticsoncology.org/solid-tumor/5167/soft-tissues-well-differentiated-liposarcoma