Lj. Layfield et al., DEDIFFERENTIATED CHORDOMA - A CASE-REPORT OF THE CYTOMORPHOLOGIC FINDINGS ON FINE-NEEDLE ASPIRATION, Diagnostic cytopathology, 19(5), 1998, pp. 378-381
Chordomas are relatively rare neoplasms occurring at both ends of a ne
uro axis. The majority follow an indolent course of multiple local rec
urrences, ultimately leading to the patient's death. Rare examples hav
e been associated with sarcomatous components, usually resembling mali
gnant fibrous histiocytoma, fibrosarcoma, or osteosarcoma. These tumor
s have followed a more aggressive course, with poor response to treatm
ent and death following a relatively short time course. Cytologic mate
rial from a single case obtained by fine-needle aspiration revealed a
high-grade malignancy composed of short atypical spindle cells contain
ing modest amounts of granular cytoplasm. Physaliphorous cells were ab
sent, and myxoid material was not a significant component of the smear
s. Rare polygonal cells with foamy cytoplasm were identified. Histolog
ic study revealed a high-grade spindle-cell sarcoma in which were scat
tered small aggregates of vacuolated cells resembling physaliphorous c
ells. Anaplastic giant cells were present, and the overall appearance
of the neoplasm resembled a malignant fibrous histiocytoma. Despite th
e radiographic appearance of a conventional chordoma in this case, the
cytologic findings were indicative of a high-grade sarcoma consistent
with a dedifferentiated chordoma. (C) 1998 Wiley-Liss, Inc.