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RESIDENT
CASE
STUDIES
Week 11 June 28 - July 4, 2003: Case
3
Table
of Contents
| List of Diagnoses |
Case 1 | Case 2
| Case 3
75
year old male with left posterior neck mass and a previous history of
recurrences of the same tumor in the head and neck region, and in the
level C2 of the spine
Discussion
Chordoma is a low to intermediate grade malignant tumor, that
recapitulates the notochord.1 It accounts for 1-4% of all
primary malignant bone tumors and most commonly presents after the age of
30, usually after the 6th decade.1 Chordoma
usually occurs in the axial skeleton, especially at the sacral (60%)
and spheno-occipital/nasal (25%).1 The clinical features are
dependent on location and spread of the tumor and ranges from pain,
headache, bleeding to neurological deficits.1
Radiologically,
chordomas are typically solitary, central, lytic and destructive lesion of
bone, usually axial skeleton.1 Macroscopically, it is
usually greyish tan, glistening, lobulated tumor with mucogelatinous to
friable and hemorrhagic areas. There is usually extension beyond the bone
into the sorrounding soft tissue.1,2
Histologically, chordomas are lobulated
with individual lobules being separated by fibrous bands. The tumor cells
may be in cords, sheets or float singly within an abundant myxoid stroma.
The cells typically have an abundant pale vacuolated cytoplasm (physaliferous
cells). There is usually mild to moderate nuclear atypia and mitoses
are infrequent.1 Dedifferentiated chordomas (chordomas
associated with high grade sarcoma) are uncommon.1 Chordomas usually have positive immunoreactivity with S100, pan keratin,
LMK and EMA. 1 There may be metastases to lung, bone, soft
tissue, lympn nodes and skin in advanced disease.1
References: 1Fletcher
CDM, Unni KK, Mertens F. (Eds.): World Health Organization Classification
of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone.
IARC Press: Lyon 2002.
2D’Haen
B, De Jaegere T, Goffin J, et al. Chordoma of the lower cervical spine.
Clin Neurol Neurosurg 1995;97:245-248.
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