Potential Sampling Error in Fine Needle Aspiration Biopsy of Dedifferentiated Chondrosarcoma

BACKGROUND: Dedifferentiated chondrosarcoma is a rare, poorly understood and often fatal sarcoma that usually manifests as a high grade, non-cartilage-producing sarcoma juxtaposed against a low grade chondrosarcoma. A correct diagnosis requires recognition of both components. In the absence of complete resection, rendering a specific diagnosis on small biopsy specimens, such as fine needle aspiration biopsy (FNAB), may be extraordinarily difficult. CASES: We retrospectively reviewed 4 cytology samples (3 primary, 1 metastatic) from 3 patients with dedifferentiated chondrosarcoma, initially analyzed by FNAB, emphasizing the potential for sampling error. Two women, aged 78 and 57 years, both of whom had prior histories of carcinoma, presented with lesions involving the right and left femur, respectively. One 27-year-old man with multiple osteochondromatosis developed a dedifferentiated chondrosarcoma of the left pelvis. Two primary cytologic specimens consisted of moderately cellular smears containing a spindled to polygonal, nonspecific, pleomorphic sarcoma unaccompanied by definite matrix material; 1 of these had a concomitant core needle biopsy (CNB), also demonstrating pleomorphic sarcoma. The third primary cytologic specimen revealed low grade chondrosarcoma, but a concomitant CNB showed only a high grade, non-matrix-producing sarcoma. The last patient developed a metastasis to the opposite femur; FNAB revealed a high grade spindle cell sarcoma. In none of the FNAB or CNB specimens were both low and high grade components of dedifferentiated chondrosarcoma recognized. However, the diagnosis was strongly suspected based on the clinical and radiographic findings. CONCLUSION: Due to sampling error, the diagnosis of dedifferentiated chondrosarcoma may be difficult to establish by cytologic examination alone. Clinical and radiographic correlation is essential for an accurate diagnosis.