We describe the clinicopathologic features in a series of 32 cases. Dedifferentiated leiomyosarcoma was defined as showing. It is typically a large, bulky tumor, and tends to have multiple smaller satellites that extend beyond the main confines of the tumor. Welldifferentiated liposarcoma and dedifferentiated liposarcoma. And a dedifferentiated liposarcoma is a highgrade tumor that occurs when a lowergrade tumor changes and creates new highgrade cells.
Dedifferentiated liposarcoma is defined as the transition from a welldifferentiated liposarcoma to nonlipogenic sarcoma of variable histological grade, usually measuring at least several millimeters in diameter. The feature of dedifferentiated liposarcoma is the histological coexistence of well to poorly differentiated liposarcoma and nonlipomatous differentiated areas. Less frequently, poorly differentiated fibrosarcoma. These are typically deep seated tumours encountered in the skin subcutis by direct invasion.
Sarcoma and cancer treatment options and prognosis chance of recovery depend on the following. The dedifferentiation occurs in 10% of welldifferentiated liposarcomas, a risk apparently higher in deepseated lesions. Dedifferentiated liposarcoma can be readily diagnosed by the juxtaposition of a welldifferentiated liposarcoma to a nonlipogenic sarcoma. Liposarcoma differential diagnoses medscape reference.
The most common dedifferentiated component is undifferentiated. Dedifferentiated liposarcoma is uncommon, and only a small number of cases have been documented. We present the case of a dedifferentiated paratesticular liposarcoma with osseous metaplasia of the spermatic cord, in a male presented with acute scrotum. Immunoreactivity for the human hematopoietic progenitor cell antigen cd34 in lipomatous tumors. Liposarcoma cancer treatment prognosis sarcoma oncology.
Liposarcoma is the second most common type of softtissue sarcoma, accounting for 10%35% of these lesions. The world health organization has categorized softtissue liposarcomas into five distinct histologic subtypes. To describe the pattern of dedifferentiated liposarcoma ddlps metastases and to analyze their predictors and outcome. Despite dedifferentiated liposarcoma having a highgrade morphology, the prognosis is better than a highgrade pleomorphic sarcoma. Rare dermal and subcutaneous tumours have also been described histology of liposarcoma. Genomic amplification of the mouse double minute 2 homolog mdm2 locus is. Pdf histopathological subtype of liposarcoma is one of the factors to assess.
Overlapping features between dedifferentiated liposarcoma and undifferentiated highgrade pleomorphic sarcoma article in the american journal of surgical pathology 3311. Alt and welldifferentiated lps have a predilection for the extremities, retroperitoneum, and paratesticular and inguinal. Mdm2 and cdk4 immunostainings are useful adjuncts in diagnosing welldifferentiated and dedifferentiated liposarcoma subtypes. Liposarcoma is a cancer that arises in fat cells in soft tissue, such as that inside the thigh or in the retroperitoneum. Pdf the dedifferentiated component of dedifferentiated liposarcoma shows wide. Patients must also have evidence of disease progression by recist 1. The occurrence of endobronchial or peripheral lipomas reflects the capacity of. 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. Overlapping features between dedifferentiated liposarcoma. Dedifferentiated component is a cellular and typically a nonlipogenic sarcoma with significant pleomorphism. Liposarcoma is one of the most common softtissue sarcomas.
Dedifferentiated liposarcoma and pleomorphic liposarcoma. In general, liposarcomas form approximately 5% of all sarcomas. All patients were male and were aged 66, 70 and 76. Paratesticular liposarcoma is a rare tumour of the genitourinary track but the most common of all sarcomas in adults. Liposarcoma are fatproducing tumours that have a spectrum of morphologies welldifferentiated liposarcoma shows features similar to lipoma, with a. We present the case of a dedifferentiated paratesticular liposarcoma with osseous metaplasia of the. A case of undifferentiated pleomorphic sarcoma originally. Well differentiated liposarcoma wdl with transition, either in the primary tumor or as a recurrence, to a sarcoma that is typically nonlipogenic. A minority additionally features myogenic, osteo or chondrosarcomatous heterologous differentiation. Primary dedifferentiated liposarcoma of the lung primary dedifferentiated liposarcoma of the lung loddenkemper, c. The fusddit3 fusion transcript type does not appear to have a significant impact on clinical outcome. Primary colonic welldifferentiated dedifferentiated. Critical histopathological analysis of 25 dedifferentiated.
Although some propose a mitotic rate of 5 mitoses10 high power fields, this isnt uniformly adopted. As the mass grows, there is usually no initial subjective symptoms, and making the diagnosis is often difficult until the tumor reaches a great size to cause symptoms of pressure. Dedifferentiated liposarcoma of retroperitoneum and mesentery. Well differentiated liposarcoma may progress into dedifferentiated liposarcoma with pleomorphic histology. Liposarcoma are fatproducing tumours that have a spectrum of morphologies welldifferentiated liposarcoma shows. Welldifferentiated liposarcomas wdlps, especially those located in the. A poorlydifferentiated pleomorphic sarcoma which accounts for 10% of liposarcomas. Dedifferentiated liposarcoma with myxoid liposarcomalike features. Dedifferentiated liposarcoma ofretroperitoneum and.
Metastatic andor locally advanced or locally recurrent disease that is not surgically resectable. Dedifferentiated liposarcoma ddlps results from the progression of a well. Liposarcoma primary in the lung are extremely rare with less than 10 cases having been reported. It is a rare type of cancer that bears a resemblance to fat cells when examined under a microscope. Dedifferentiation of a welldifferentiated liposarcoma to a highly. Classically, pleomorphic liposarcoma was not permitted as the dedifferentiated component, but it appears that it rarely occurs in this guise, associated with atypical lipomatous tumor and expressing mdm2 see boland 2010.
Challenging dedifferentiated liposarcoma identified by. Dedifferentiated liposarcoma ddlps demonstrates a variety of growth patterns, and their histologic resemblance to other spindle cell mesenchymal tumors has been widely recognized. Pleomorphic liposarcoma is the rarest but sometimes is a very aggressive disease type. Histology driven systemic therapy of liposarcomaready for. Dedifferentiated liposarcoma of the retroperitoneum. Liposarcoma is divided into myxoid, pleomorphic, welldifferentiated, and dedifferentiated subtypes. Differential diagnosis dedifferentiated liposarcoma. Pdf dedifferentiated liposarcoma with inflammatory. Patients with welldifferentiated liposarcoma can survive for decades, but recurrence is a problem. Liposarcoma is the most frequent soft tissue sarcoma. To clinicopathologically characterize the dedifferentiated variant of leiomyosarcoma in a series of 18 cases. Lead article dedifferentiation of a welldifferentiated. Welldifferentiated and dedifferentiated liposarcomas rreps.
Dedifferentiated liposarcoma has also been defined as an atypical. Dedifferentiated liposarcoma with epithelioidepithelial. Imaging of musculoskeletal liposarcoma with radiologic. All patients must have measurable disease as defined by recist 1. Welldifferentiated liposarcoma wdl atypical lipomatous tumor and dedifferentiated liposarcoma ddl together comprise. The dedifferentiated variation occurs only in 10% of liposarcoma cases.
Additional and relevant useful information for dedifferentiated liposarcoma. A diagnosis of dedifferentiated liposarcoma confirmed at mskcc. Generally, the myxoid and well differentiated behave as lowgrade tumors, with the pleomorphic and dedifferentiated demonstrating more aggressive, highgrade behaviors. Retroperitoneal dedifferentiated liposarcoma has higher rates of local recurrence and disease specific death curr opin oncol 2011. Atypical lipomatous tumor is preferred by who classi. The definition of dedifferentiated liposarcoma has been modified slightly from its prior definition as a nonlipogenic sarcoma arising in association with well. It is a mixture of welldifferentiated liposarcoma and a highgrade nonlipogenic sarcoma, an aggressive component. Dedifferentiated dedifferentiated liposarcoma is characterised by abrupt transition from low grade to high grade non lipogenic morphology within a well differentiated liposarcoma. At the time of presentation, most of the dedifferentiated liposarcomas. Dedifferentiated liposarcoma with myofibroblastic differentiation. Primary colonic welldifferentiated dedifferentiated liposarcoma of the ascending colon. Dedifferentiated liposarcoma is the least common subtype of liposarcoma and usually arises from a welldifferentiated liposarcoma wdlps. Liposarcoma, and dedifferentiated liposarcoma this category of lps, which includes alt, welldifferentiated lps, and dedifferentiated lps, is the most common subtype of lps, accounting for approximately 50% of all cases. The typical clinical presentation is similar to an inguinal hernia or a benign lipoma.
Liposarcoma is the most common malignant soft tissue sarcoma. Dedifferentiated liposarcoma a more aggressive version of welldifferentiated liposarcoma, a dedifferentiated liposarcoma is less aggressive than sarcomas identified as highgrade. Memorial sloan kettering cancer center has reported the largest series. Progression occurs in 17 % of patients when wdlps is located in the retroperitoneum and 6% of cases when wdlps is located in the extremities. However, if the lipogenic component is not abundant due. Study of abemaciclib in dedifferentiated liposarcoma. Dedifferentiated liposarcoma, liposarcoma, mdm2, myxofibrosarcoma, soft tissue tumors, well differentiated liposarcoma how to cite this article. About 50% of retroperitoneal liposarcomas are dedifferentiated. Dedifferentiated liposarcoma misdiagnosed as dermatofibrosarcoma protuberans. Pdf liposarcomas of the head and neck region are rare. Rarely myxoidround cell liposarcoma is associated with areas of well. In more than twothirds of the cases, the poorly differentiated component of the tumor resembles malignant fibrous histiocytoma mfh. Casereport dedifferentiated paratesticular liposarcoma with osseous metaplasia kostaschondros,1 ioannisheretis,1 michaelpapadakis,2 vasilikibozionelou,3.
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