Dedifferentiated liposarcoma: a report of nine cases with a peculiar neurallike whorling pattern associated with...

Nine cases of dedifferentiated liposarcoma with both a peculiar neurallike or meningeallike whorling pattern and metaplastic bone formation are reported. The tumors predominated in the retroperitoneum of elderly adults. All nine tumors were resected, and five of seven that were followed-up recurred locally, but none metastasized after a follow-up of 2 to 7 years. Grossly, most of the tumors were huge masses, ranging from 2.5 cm to 60 cm. Histologically, the tumors revealed, in addition to areas of well-differentiated liposarcoma, discrete nodules consisting of hypercellular, spindled to ovoid cellular proliferation arranged in tight, concentric whorls resembling neural tumors or meningiomas. Metaplastic, heterotopic ossification was present in seven of nine tumors and consisted of variable amounts of osteoblast-rimmed bone trabeculae situated at the periphery of the whorled areas or intimately mixed with the whorled cellular component. Immunohistochemical studies were inconclusive in determining the nature of the dedifferentiated, whorled element. Ultrastructural evaluation of one tumor disclosed neoplastic cells featuring thin, interdigitating cytoplasmic processes connected by desmosomes, similar to those described in follicular dendritic cell neoplasms. Although suggested by the light and electron microscopic features, the follicular dendritic cell differentiation of the dedifferentiated component could not be confirmed on immunohistochemical grounds, and the histogenesis of the intriguing neurallike or meningiomalike component in these dedifferentiated liposarcomas is unknown.