Management of a recurrent massive abdominal haemophilic pseudotumour with adjuvant radiotherapy

Treatment of pelvic haemophilic pseudotumour: A retrospective study

“…reported in the literature include coagulation factor replacement therapy, local aspiration, radiotherapy, interventional embolization and surgical resection. [3][4][5][6] Percutaneous evacuation has been shown to result in a high risk of continuous bleeding, recurrence, infection and formation of a permanent fistula. 7 As treatment for a pelvic pseudotumour, radiotherapy and embolization alone cannot be used because it can easily lead to recurrence.…”

“…In addition, preoperative radiotherapy may not be appropriate because fibrosis increases the difficulty of performing surgical resection, although postoperative irradiation may eliminate residual tissue and help prevent recurrence. 4,5 Embolization is often used as an adjunct to surgical removal of pseudotumours, to reduce their size and infiltration of blood vessels, thereby reducing the chance of a major bleeding event during surgery. 8 Surgical resection, while undergoing coagulation factor replacement therapy, is an effective method for the treatment of a pelvic haemophilic pseudotumour.…”

Pseudotumours in haemophilia: non‐adherence, under‐reporting bleeds or bad luck?

“…This low incidence and subsequent unfamiliarity within the disease leads to diagnostic delays and a lack of consensus for treatment. The aetiology of pseudotumours is thought to come from repeated, likely undiagnosed/unreported bleeds into an extra‐articular area (often soft tissue or bone) that leads to cystic swelling and encapsulation of the blood . There are two patterns of pseudotumour development, which appears to be divided by age and anatomical location.…”

“…Younger patients more commonly present with distal pseudotumours in extremities (ankles, wrists, small bones of feet), whereas older patients are more likely to present with proximal pseudotumours. These typically involve the pelvis or abdomen and due to the anatomical space these tumours are often slowly progressive and present late in their clinical course with symptoms of compression . Treatment options for pseudotumours include conservative management with factor concentrate prophylaxis and immobilisation, embolisation, radiotherapy or surgical management.…”

“…There has also been a case report of using tranexamic acid in addition to factor prophylaxis resulting in improvement in a large inoperable proximal pseudotumour . The surgical management of these patients is usually associated with a high rate of complications and mortality, although there have been successful outcomes in case reports and retrospective studies …”