Recurrent congenital haemangiopericytoma in a child

Abstract

A five-day-old boy was referred with a soft-tissue mass in his right upper arm. Plain radiographs and ultrasound demonstrated a lesion extending from the axilla to the elbow on the posterolateral aspect of the humerus. Open biopsy confirmed the diagnosis of congenital haemangiopericytoma. After MRI and selective angiography, excision biopsy was carried out, but no adjuvant therapy was administered. At further examination, four years and ten months later, he was noted to have three small nodules at the site of the original tumour. Excision biopsy confirmed this to be a local recurrence, although the lesion was less cellular with no appreciable mitotic activity. Congenital haemangiopericytoma is a rare cause of a soft-tissue mass in children. Most tumours are benign, and recurrence is uncommon. The treatment is controversial, but most centres recommend the use of adjuvant chemotherapy, combined with complete excision. We recommend treatment with doxorubicin. Orthopaedic surgeons should be familiar with this tumour since 30% to 50% of cases occur in the limbs.

Infantile Hemangiopericytoma: Two Cases Report and Literature Review

“…The diagnosis is confirmed by excisional biopsy [9]. The gross appearance of this lesion is usually transparent or white-grey [6].…”

“…There is seldom discolouration of the lesions in the limb in contrast to those in the head and neck [6]. Infantile hemangiopericytoma tends to be located more superficially than that in adults [9].…”

“…Ultrasound determines the size of the lesion, vascular loops, the echogenic content and whether or not a capsule is present, and angiography shows the vascular pattern and the presence of feeder vessels. CT and MRI can provide information about the extent of the tumour and help in preoperative planning [9].…”

“…Mitotic figures are usually easily identified. The tumour cells stain positively with vimentin [6,9].…”

“…Some tumours have similar histological features, such as infantile myofibromatosis, synovial sarcoma, fibrosarcoma, malignant fibrous histiocytoma, mesenchymal chondrosarcoma, and leiomyosarcoma [10][11][12][13]. It is therefore important to examine multiple areas of the specimen to confirm the diagnosis [9].…”