TY - JOUR
T1 - Congenital hemangiopericytoma
T2 - An unusual vascular neoplasm of infancy
AU - Bailey, Patrick V.
AU - Weber, Thomas R.
AU - Tracy, Thomas F.
AU - O'Connor, Dennis M.
AU - Sotelo-Avila, Cirilo
PY - 1993/11
Y1 - 1993/11
N2 - 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.
AB - 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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M3 - Article
C2 - 8236018
AN - SCOPUS:0027362231
SN - 0039-6060
VL - 114
SP - 936
EP - 941
JO - Surgery
JF - Surgery
IS - 5
ER -