Bailey P V, Weber T R, Tracy T F, O'Connor D M, Sotelo-Avila C
Cardinal Glennon Children's Hospital, St. Louis University Medical Center, Mo.
Surgery. 1993 Nov;114(5):936-41.
Hemangiopericytoma is an uncommon tumor of infants, which originates from the vascular pericytes. Although generally considered to benign, metastases can occur.
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.
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.
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.
血管外皮细胞瘤是一种罕见的婴儿肿瘤，起源于血管周细胞。虽然通常被认为是良性的，但也可能发生转移。
我们观察到5例婴儿先天性血管外皮细胞瘤；均为女性。诊断时的平均年龄为7周（范围，出生至10周）。病变位于颈部、腮腺、腋窝和腹膜后。1例颈部病变在产前通过超声检查发现。每个病变均行切除。仅在组织学检查后才确立先天性血管外皮细胞瘤的诊断。
4名儿童未发现复发迹象。然而，1名患有颈部病变的儿童在初次切除后28个月出现胸内和颅内转移，该儿童接受了化疗，但最终死于疾病进展。
对于有血管性肿块的新生儿，我们主张考虑先天性血管外皮细胞瘤。由于先天性血管外皮细胞瘤与其他血管畸形不同，对类固醇治疗无反应，因此手术切除是首选治疗方法。术后长期随访对于早期发现转移至关重要。
