Management of haemophilic pseudotumours with special emphasis on radiotherapy and arterial embolization

Abstract

The haemophilic pseudotumour is an expanding destructive haematoma, which is associated with a considerable amount of morbidity in haemophilic patients. Its prevention is paramount. In fact, this goal can be achieved by primary prophylaxis to avoid muscle haematomas and by adequate and long-term haematological treatment of muscle haematomas in case they appear. At the moment, surgical excision of pseudotumour is the preferred treatment by many authors. However, there are instances that surgical extraction of the lesion is not feasible. In such situations, radiotherapy and arterial embolization should be considered either alone or as an adjunct to surgery. Conservative management using a combination of radiotherapy and replacement therapy should be considered for treating haemophilic bone pseudotumours, which are located in the skull or in the distal parts of the limbs, especially in conditions where some impediments to surgical excision exist. In fact, the radiation should be delivered to the lesion site in small fragments of 2 Gy or less to a total dosage of 6-23.5 Gy, which is the most recommended radiation dosage, at the moment. Therapeutic arterial embolization of haemophilic pseudotumours should be considered in lesions of large size, especially in pseudotumours of pelvic region, as it may effectively reduce its size and decrease the risk of bleeding complications during surgery. Nevertheless, in view of its temporary effect, embolization may better be performed, as a preparatory procedure, at best about 2 weeks prior to surgery. This time lapse will allow for mass shrinkage but is insufficient for vessel restoration.

Hemophilic Pseudotumor of the Temporal Bone With Conductive Hearing Loss

“…The effects of this procedure are temporary, so it should be performed as a preparatory procedure, so treatment of hemophilic pseudotumors is mainly based on conservative measures and surgical resection. 2) In our case, as the neuromeningeal branch of the ascending pharyngeal artery was the main feeder, there was the risk of caudal nerve ischemia. Hemophilic pseudotumors are rarely encountered by neurosurgeons, but early-stage diagnosis is very important since neurological deficits in this stage may be reversed.…”

“…On the other hand, surgical removal is considered the treatment of choice in most reports. [2][3][4][5]9,10) Surgery is recommended for hemophilic pseudotumors that manifest as neurological symptoms or cosmetic problems or continue to expand and destroy the surrounding tissue despite coagulation factor replacement. Surgical resection was performed with good results in 5 previous cases of hemophilic pseudotumors in cranial bones.…”

“…Hemophilic pseudotumor usually occurs in the soft tissues, muscles, and long bones, most frequently in the femur, tibia, and pelvic bones. 2,8,9) Hemophilic pseudotumors in cranial bones are extremely rare. The tumor enlarges slowly and develops a fibrous capsule, and can destroy underlying tissues by its progressive expansion.…”

Surgical treatment of the haemophilic pseudotumour: A single centre experience

“…Different therapy options have been described in the treatment of the haemophilic pseudotumour including surgical resection, arterial embolisation, radiotherapy, percutaneous curettage, and filling with fibrin and/or bone graft or hydroxyapatite [2,3,[10][11][12][13][14][15][16][17]. However, treatment of the haemophilic pseudotumour poses a challenge, and extensive clinical experience is essential to appropriately address this serious complication in patients with haemophilia.…”

Giant Hemophilic Pseudotumor of the Iliopsoas: Case Report

“…Many inoperable cases that were managed by radiotherapy have been reported. 2,4 The mechanism of the response of the hemophilic pseudotumor to radiation therapy is not yet known. Brant and Jordan suggested that 1000-2000 cGy radiotherapy leads to an endarteritis in an acutely bleeding hematoma.…”