Rare Diseases Symptoms Automatic Extraction

Dedifferentiated liposarcoma with lipoma-like well-differentiated liposarcoma: clinicopathological study of 30 cases, with particular attention to the comingling pattern of well- and dedifferentiated components: a proposal for regrouping of the present subclassification of well-differentiated liposarcoma and dedifferentiated liposarcoma.

[well-differentiated liposarcoma]

Dedifferentiated liposarcoma (DDL) is defined as nonlipogenic sarcoma, with an abrupt transition from coexisting well-differentiated liposarcoma (WDL). However, intermingled transition in a mosaic pattern between WDL and DDL is not infrequently encountered. Here, the authors review clinicopathological features of 30 cases of DDL associated with lipoma-like WDL. Histological examination revealed 20 tumors that showed an abrupt transition between WDL and DDL. Among these, 13 tumors showed high-grade spindle-cell sarcoma having histological features of unclassified malignant fibrous histiocytoma (MFH)-like sarcoma (high-grade DDL [HDDL]). The remaining 7 tumors showed moderate cellular spindle-cell proliferation with mild nuclear atypia and scant mitotic figures (low-grade DDL [LDDL]). The other 10 tumors showed intermingled transition between WDL and DDL. The interface between these 2 components overlapped, resulting in frequent occurrence of a lipogenic spindle-cell component (comingling DDL). Based on the cellularity and nuclear atypia of the spindle-cell components, there were 7 comingling HDDLs and 3 comingling LDDLs. The histology of comingling LDDL simulated an admixture of spindle-cell liposarcoma and LDDL, and distinction from each other was practically difficult. The histology of comingling HDDL simulated pleomorphic liposarcoma. Follow-up data, available for 23 patients (median, 39 months), showed that 2 patients died of tumor (both had HDDL), and 1 patient died of unrelated disease; 8 patients were alive with recurrent or metastatic diseases (3 HDDLs, 3 LDDLs, and 2 comingling HDDLs). Statistical analysis by Fisher's exact test showed no correlation between histological subtypes (HDDL and LDDL, and typical DDL and comingling DDL).

Diseases presenting "lipoma-like wdl" symptom

  • dedifferentiated liposarcoma
  • pleomorphic liposarcoma
  • well-differentiated liposarcoma

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