Surgical Treatment of Recurrent Orbital Hemangiopericytoma

Abstract

Hemangiopericytoma is a rare vascular tumor featuring the uncontrolled proliferation of pericytes. This tumor tends to develop in subcutaneous tissue and skeletal muscle. The localization of hemangiopericytoma in the orbit is particularly rare. Clinical signs and symptoms are, in order of frequency, proptosis, perception of an intraorbital mass, pain, diplopia, reduction of visus, tumescence, and ecchymoses of the eyelids. Hemangiopericytomas have higher potential of relapse, local invasiveness, and the possibility of producing distant metastases. Surgery must be as radical as possible to avoid incomplete tumor resection and high frequency of relapse together with increased duration of the disease and a higher risk of malignant development. The current report is a study of two cases of hemangiopericytoma in the orbital cavity treated at the Unit of Maxillo-Facial Surgery of the University of "La Sapienza" in Rome. The present data demonstrate that complete en bloc excision of the mass with its capsule is important to avoid the risk of relapse. To this end, an appropriate first surgical treatment should be chosen so as to obtain a wide "surgical light" and complete view of the mass. In the reported cases, the use of a combined anterolateral approach was preferred to attack the tumor from different sides. Indeed, such an approach allows one to obtain optimal exposition of the orbital region and temporal and infratemporal fossae contemporaneously.

Hemangiopericytoma of the Hand

“…Such tumours have a wide anatomical distribution but tend to develop in subcutaneous tissue and skeletal muscle. 34.9 % of all hemangiopericytomas affect the lower limbs, whereas 15 % affect the head and neck region [2]. Hemangiopericytoma of upper extremity is rare [1,2].…”

“…34.9 % of all hemangiopericytomas affect the lower limbs, whereas 15 % affect the head and neck region [2]. Hemangiopericytoma of upper extremity is rare [1,2]. There are 83 cases reported in English literature, of these only 14 are documented as malignant [4].…”

“…It occurs most commonly in the soft tissues of the upper and lower extremities, pelvis, and retroperitoneal space and seldom in visceral organs [5]. Due to its vascular origin, it can be found anywhere in the body [2]. The etiopathogenesis of hemangiopericytomas is not yet defined.…”

“…Surgery should be as radical as possible to avoid incomplete tumour resection, the high frequency of relapse and a higher risk of malignancy. The present treatment includes complete en bloc excision of the mass with its capsule to avoid the risk of relapse [2]. Although unusual, hemangiopericytoma can be considered benign or malignant depending on histological features.…”

“…They are mesenchymal tumours that account for 3 to 5 % of all soft tissue sarcomas and 1 % of all vascular tumours [1,2]. These tumours have a wide anatomical distribution but tend to develop in subcutaneous tissue and skeletal muscle.…”