1. Introduction
Chordomas are rare tumors of the axial skeleton originating from
remnants of primitive notochord and occurring in the midline from clivus
to sacrum, anterior to the spinal cord. Historically 50% cases were
estimated to originate in the sacrococcygeal region, 35% in skull base,
and 15% in mobile spine1. Surveillance, Epidemiology,
and End Results (SEER) database analysis of 400 patients from 1973-1995
suggested an almost equal distribution in the skull base (32%), mobile
spine (32.8%), and sacrum (29.2%). The incidence is 0.08 per 1,00,000,
with the prevalence being twice as high in males compared to females.
The peak incidence is seen between 40 to 60 years of age, and proportion
of cases in first two decades has been reported as low as 2.6%-5% of
all2-3. No risk factors as such have been identified,
however, patients of tuberous sclerosis are at higher risk for the
same4. These slow-growing malignancies are locally
aggressive, but have recurrence rate upto 66% by eight to ten years,
depending on the treatment delivered5. Distant
metastases to lung, bone, soft tissues and liver have been reported in
upto 43% patients on prolonged follow-up of 16 years and as low as 5%
at presentation or on shorter follow-up duration of five
years6-7. These figures imply that metastases occur
mostly late in the course of disease. Multiple studies have attributed
the risk of developing distant metastases to local recurrences, with the
risk being much lesser in the absence of local
recurrences8. We present a rare case of rapidly
progressive, both locally and distally, case of poorly differentiated
sacral chordoma with histologically proven lung metastases at
presentation in a child. Being a case report, this article was not
required to be reviewed by the ethics committee. Also, informed consent
from the patient’s (minor) father has been taken regarding the same.