Infantile haemangiopericytoma: a rare congenital cervical tumour

Abstract

Haemangiopericytoma can follow an aggressive course in adults, including local recurrence and metastasis. The infantile variant is rare but typically follows a distinct clinical course, and is associated with more benign behaviour compared with similar tumours in adults and children over one year. Congenital haemangiopericytoma can be effectively treated with surgery, without requiring adjuvant therapy.

Multifocal Congenital Hemangiopericytoma

“…Eighty percent are malignant and 10% to 20% are metastatic at presentation . It is estimated that 5% to 10% of children may have this evolution , predominantly boys, although malignant evolution has been described only in children 12 months of age and older and not in those with congenital HPC. Histologic features such as necrosis, mitoses, vascular invasion, and cellular pleomorphism are associated with more aggressive tumor behavior in adult‐type HPC .…”

“…The diagnosis is confirmed by biopsy . The standard histopathology of the lesion is a cellular fusiform neoplasm with some atypical cytology and a prominent “staghorn‐like” vascular pattern with fine branching blood vessels.…”

“…Despite the presence of histologic findings associated with aggressive tumor behavior, such as focal necrosis, high mitotic index, vascular invasion, and cellular pleomorphism, infantile‐type HPC has a better prognosis with chemotherapy than the adult type .…”

“…It can be difficult to distinguish HPC from other tumors with prominent vascularization , and its rarity can delay the diagnosis, especially in newborns. Most authors have reported that congenital HPC is associated with good prognosis and has a more benign behavior than tumors in adults and children 1 year of age and older , although despite the possibility of spontaneous regression, surgical intervention followed by chemotherapy may be indicated, depending on tumor size and its propensity to hemorrhage.…”

Infantile hemangiopericytoma leading to hypovolemic shock in a neonate

“…Infantile HPCs (5–10%) in infants <1 year have a better prognosis, lower metastasis, and recurrence rates . The most common location is the head or the neck . We report an unusual case of a neonate with congenital parietal HPC and secondary hypovolemic shock at the delivery room.…”