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Abstract
Background. Hemangiopericytoma is an uncommon tumor of infants, which originates from the vascular
pericytes. Although generally considered to be benign, metastases can occur.
Methods. Five cases of congenital hemangiopericytoma were seen in infants; all were found
in females. The mean age at diagnosis was 7 weeks (range, birth to 10 weeks). The
lesions were located in the neck, the parotid, the axilla, and the retroperitoneum.
One neck lesion was detected prenatally by ultrasonography. Each lesion was resected.
The diagnosis of congenital hemangiopericytoma was established only after histologic
examination.
Results. No evidence of recurrence has been found in four of the children. However, intrathoracic
and intracranial metastases developed in one child with a neck lesion 28 months after
the original resection, and the child was treated with chemotherapy, but she died
of progressive disease.
Conclusions. We advocate the consideration of congenital hemangiopericytoma in the newborn infant
with a vascular mass. Because congenital hemangiopericytoma is unresponsive to steroid
therapy, unlike other vascular malformations, resection is the treatment of choice.
Long-term postoperative follow-up is essential for the early detection of metastases.
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Article info
Publication history
Accepted:
September 2,
1992
Identification
Copyright
© 1993 Published by Elsevier Inc.
