Infantile haemangiopericytoma: a rare congenital cervical tumour.

OBJECTIVE: Infantile haemangiopericytoma is a rare childhood vascular tumour of borderline malignancy and unpredictable clinical course. It can present a diagnostic challenge due to indeterminate clinical, radiological and pathological features. This report presents the case of a large congenital haemangiopericytoma of the neck in a neonate, and discusses diagnosis, imaging, pathology and surgical management. CLINICAL PRESENTATION: A full-term neonate presented with a large posterior neck mass at birth. Pre-operative radiological appearances were suggestive of teratoma, but following surgical excision the diagnosis of infantile haemangiopericytoma was confirmed on histological analysis. There were no signs of recurrence at 12-month follow up. CONCLUSION: 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.

[Orbital tumor--hemangiopericytoma--in a neonate]. / Guz oczodolu--haemangiopericytoma--u noworodka.

PURPOSE: To present a case of Haemangiopericytoma (HPC), a rare neopalsm which originates from the vascular pericytes. HPC occurs most commonly in adults. Only 5-10% of cases occur in children. Congenital orbital HPC is generally unknown in the field of ophthalmology. MATERIAL AND METHODS: A case of congenital, large exophthalmus is reported in a 1 day old male neonate. Imaging studies demonstrated a vascular, orbital mass involving skull base and cranial fossa. RESULTS: The diagnosis of HPC was established after histological exmination. Lesion did not qualify to surgical resection. The child was treated with chemotherapy for 10 months and a great regression of tumor was noted. There was no tumor recurrence during 4 years of a follow up. CONCLUSIONS: Chemotherapy may have a significant role in the treatment of infants with nonoperative malignant hemangiopericytoma.

Congenital hemangiopericytoma presenting as rectal bleeding in a neonate: a case report.

Hemangiopericytoma is a rare soft tissue lesion originating from capillary pericytes that can occur anywhere vascular capillaries are found. It is an uncommon tumor found typically in adults but rarely in children. We describe a previously unreported case of an intraluminal lesion in a neonate.

Role of neoadjuvant chemotherapy in congenital intracranial haemangiopericytoma.

Infantile haemangiopericytoma of the CNS is a rare entity. We report the first case of a congenital haemangiopericytoma successfully treated by preoperative chemotherapy. The patient presented shortly after birth with the diagnosis of a haemangiopericytoma. As neurosurgery was too risky due to size, location and age of the patient an anthracycline-based chemotherapeutic regimen was applied and resulted in a significant decrease in tumour size, making a postchemotherapy complete surgical resection possible. Chemotherapy may benefit patients with congenital haemangiopericytoma especially if the tumour cannot initially be treated by complete neurosurgical resection.

Auriculotemporal nerve syndrome in association with congenital haemangiopericytoma: a case report.

BACKGROUND: Auriculotemporal nerve syndrome is characterised by recurrent episodes of facial gustatory flushing and/or sweating along the cutaneous distribution of the auriculotemporal nerve. The condition is rare in children and is normally a sequel of perinatal birth trauma. We report a case of a sixteen-month-old boy referred by paediatric oncology with recurrent, unilateral facial flushing of the left cheek which had been present for 2 months. The flushing only occurred during mastication. The patient had also received treatment for a rare vascular tumour, congenital haemangiopericytoma, of the left cheek and parotid region. The possible association between auriculotemporal nerve syndrome and congenital haemangiopericytoma is discussed. Knowledge of the presentation, aetiology and management of Auriculotemporal Nerve Syndrome can provide much needed reassurance to those suffering with this condition.

A newborn with infantile fibrosarcoma of foot: treatment with chemotherapy and extremity-sparing surgery.

Infantile fibrosarcoma represents less than 1% of all childhood cancers, but it is the most common soft-tissue sarcoma in those under 1 year of age. We report an infant with congenital infantile fibrosarcoma diagnosed as hemangiopericytoma. He was treated with chemotherapy and extremity-sparing surgery. Amputation was avoided.

Prenatal diagnosis of a giant congenital primary cerebral hemangiopericytoma.

Congenital primary intracranial hemangiopericytomas are exceptionally rare tumors. We present a case of a fetus, with the prenatal sonogram at 33 weeks of gestation revealing a large cerebral tumor. Because of the enlarged head, a cesarean section was performed. The tumor was confirmed by postnatal ultrasound, magnetic resonance imaging (MRI) and biopsy. Elevated intracranial pressure and hemorrhage led to death on the 11th day. Autopsy revealed a 10x9 cm large inhomogeneous tumor located centrally, mainly in the posterior fossa. Histology showed a hypercellular and hypervascular tumor with extended necrosis and high mitotic rate. The tumor cells were positive for vimentin and CD34 antigens and negative for several neurological markers, desmin and CD31. The diagnosis of a congenital primary cerebral hemangiopericytoma was confirmed.

Congenital hemangiopericytoma in a neonate.

Hemangiopericytoma is a rare malignant vascular tumor that usually occurs in adults. The occurrence of these tumors in infants, known as congenital or infantile hemangiopericytoma, is even rarer and their behavior may be more benign than the adult type. We describe a 1-day-old female neonate with congenital hemangiopericytoma, presenting with a right inguinal mass at birth. At the time of surgery, lymphangioma was suspected because of its appearance, fluid-filled multicystic content, and the high incidence of this disease in pediatric patients. Tumor excision was performed and hemangiopericytoma was diagnosed by histology. There was no tumor recurrence during 12 months of follow-up.

Recurrent congenital haemangiopericytoma in a child.

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.

Case report of a newborn with a posterior thoracic midline congenital hemangiopericytoma of the back.

The posterior thoracic midline location is an unusual site for a congenital hemangiopericytoma. The authors report such a case that caused near fatal exsanguination of a newborn after vaginal delivery. Magnetic resonance imaging (MRI) studies of the mass were completed after hemostasis. These studies showed a well-defined border between the tumor and underlying trapezius muscle. The mass was removed successfully surgically and presumed initially to be a teratoma. Pathological diagnosis of the tumor was hemangiopericytoma with low malignant potential. After a 9-day hospital course, the patient was discharged with recovering hepatic and renal function.

Congenital hemangiopericytoma: report of a case.

We report a case of congenital hemangiopericytoma arising in the lower right leg of a 4-day-old male neonate. Despite the generally good prognosis associated with this neoplasm, a complete surgical excision has so far been recommended to avoid recurrences, because no definite criteria for determining whether or not the tumor will regress spontaneously have been established to date.

A case report: malignant haemangiopericytoma in infancy.

A case of malignant haemangiopericytoma of the right foot in an infant is reported. Nodules were noticed on the foot at birth and these gradually increased in size to involve the whole foot on presentation at the age of ten months. With a working diagnosis of haemangioma, tumour excision and foot reconstruction was done. After histological diagnosis, ablation was offered but refused.

Congenital nasal hemangiopericytoma: intrauterine, intraoperative, and histologic findings.

Hemangiopericytoma is a rare tumor of mesenchymal origin. To date, 91 cases of nasal or paranasal hemangiopericytoma and 59 congenital hemangiopericytomas have been reported in the literature. A congenital hemangiopericytoma arising from the nasal cavity and skull base has not yet been described. We report a case of a male newborn with a highly vascular nasal tumor diagnosed by in utero sonography with three-dimensional surface reconstruction. The tumor extended to the right anterior skull base, the right nasal cavity, and the right side of the nasal pyramid. A complete resection by neodymium:yttrium-aluminum-garnet-potassium titanyl phosphate ("Nd:YAG-KTP") laser was performed on the day of cesarean section at 33 weeks' gestation. The tumor was diagnosed as hemangiopericytoma by histologic and immunohistochemical findings. Postoperative nasal flow, feeding, and sight were unimpaired. At the 9-month follow-up, the infant remained free of disease.