Congenital hemangiopericytoma: An unusual vascular neoplasm of infancy

Patrick V. Bailey, Thomas R. Weber, Thomas Tracy, Dennis M. O'Connor, Cirilo Sotelo-Avila

Research output: Contribution to journalArticle

38 Citations (Scopus)

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.

Original languageEnglish (US)
Pages (from-to)936-941
Number of pages6
JournalSurgery
Volume114
Issue number5
StatePublished - Jan 1 1993

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Vascular Neoplasms
Hemangiopericytoma
Neck
Neoplasm Metastasis
Blood Vessels
Pericytes
Vascular Malformations
Axilla
Ultrasonography
Steroids
Parturition
Newborn Infant
Recurrence
Drug Therapy
Therapeutics
Neoplasms

All Science Journal Classification (ASJC) codes

  • Surgery

Cite this

Bailey, P. V., Weber, T. R., Tracy, T., O'Connor, D. M., & Sotelo-Avila, C. (1993). Congenital hemangiopericytoma: An unusual vascular neoplasm of infancy. Surgery, 114(5), 936-941.
Bailey, Patrick V. ; Weber, Thomas R. ; Tracy, Thomas ; O'Connor, Dennis M. ; Sotelo-Avila, Cirilo. / Congenital hemangiopericytoma : An unusual vascular neoplasm of infancy. In: Surgery. 1993 ; Vol. 114, No. 5. pp. 936-941.
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Bailey, PV, Weber, TR, Tracy, T, O'Connor, DM & Sotelo-Avila, C 1993, 'Congenital hemangiopericytoma: An unusual vascular neoplasm of infancy', Surgery, vol. 114, no. 5, pp. 936-941.

Congenital hemangiopericytoma : An unusual vascular neoplasm of infancy. / Bailey, Patrick V.; Weber, Thomas R.; Tracy, Thomas; O'Connor, Dennis M.; Sotelo-Avila, Cirilo.

In: Surgery, Vol. 114, No. 5, 01.01.1993, p. 936-941.

Research output: Contribution to journalArticle

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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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Bailey PV, Weber TR, Tracy T, O'Connor DM, Sotelo-Avila C. Congenital hemangiopericytoma: An unusual vascular neoplasm of infancy. Surgery. 1993 Jan 1;114(5):936-941.