Hemophilic Pseudotumors: Diagnosis and Management



Department of Orthopaedic Surgery, La Paz University Hospital, Madrid, Spain


Prevention is essential for avoiding the complications of muscle hematomas (pseudotumors, compartment syndromes
and peripheral nerve lesions) in hemophilic patients. This is achieved through early diagnosis of muscle hematomas
and proper long-term hematological treatment until they have resolved (confirmed by image studies). Ultrasound-guided
percutaneous drainage could be beneficial in terms of achieving better and faster symptom relief. When suspecting
a hemophilic pseudotumor, biopsy will help us confirm the diagnosis and rule out true tumors (chondrosarcoma,
liposarcoma, synovial sarcoma) that sometimes mimic hemophilic pseudotumor. Surgical removal of hemophilic
pseudotumor is the best solution. As alternatives, there are curettage and filling with cancellous bone and radiotherapy
(when surgery is contraindicated). Preoperative arterial embolization (ideally 2 weeks before surgery) helps control
intraoperative bleeding during surgery for giant pelvic pseudotumors.
Level of evidence: III


Main Subjects

1. Rodriguez-Merchan EC. The role of orthopaedic
surgery in haemophilia: current rationale, indications
and results. EFORT Open Rev. 2019; 4(5):165-73.
2. Rodriguez-Merchan EC. Musculo-skeletal manifestations
of haemophilia. Blood Rev. 2016; 30(5):401-9.
3. De la Corte-Rodriguez H, Rodriguez-Merchan EC.
Treatment of muscle haematomas in haemophiliacs
with special emphasis on percutaneous drainage.
Blood Coagul Fibrinolysis. 2014; 25(8):787-94.
4. Rodriguez-Merchan EC. The haemophilic
pseudotumour. Haemophilia. 2002; 8(1):12-6.
5. Rodriguez-Merchan EC. Acute compartment
syndrome in haemophilia. Blood Coagul Fibrinolysis.
2013; 24(7):677-82.
6. Donaldson J, Goddard N. Compartment syndrome
in patients with haemophilia. J Orthop. 2015;
7. Large DF, Ludlam CA, Macnicol MF. Common peroneal
nerve entrapment in a hemophiliac. Clin Orthop Relat
Res. 1983; 181:165-6.
8. Kaymak B, Ozçakar L, Cetin A, Erol K, Birsin Ozçakar
Z. Concomitant compression of median and ulnar
nerves in a hemophiliac patient: a case report. Joint
Bone Spine. 2002; 69(6):611-3.
9. Caviglia H, Candela M, Landro ME, Douglas Price AL,
Neme D, Galatro GA. Haemophilia pseudotumours
in patients with inhibitors. Haemophilia. 2015;
10. Rodriguez-Merchan EC, Jimenez-Yuste V. The role of
selective angiographic embolization of the musculoskeletal
system in haemophilia. Haemophilia. 2009;
11. Espandar R, Heidari P, Rodriguez-Merchan EC.
Management of haemophilic pseudotumours with
special emphasis on radiotherapy and arterial
embolization. Haemophilia. 2009; 15(2):448-57.
12. Rodriguez-Merchan EC. Haemophilic cysts
(pseudotumours). Haemophilia. 2002; 8(3):393-401.
13. Hampton KK, Grant PJ, Johnston D, Prentice CR. Pelvic
haemophilic pseudotumour occurring in a patient
with mild haemophilia: a brief report. Blood Coagul
Fibrinolysis. 1990; 1(6):747-8.
14. Marisavljević D, Glisić M, Elezović I, Popović A, Rolović
Z. Successfull extirpation of femoral pseudotumour in
a patient with severe haemophilia A and an inhibitor
to factor VIII. Srp Arh Celok Lek. 1991; 119(11-
15. Heim M, Horoszowski H, Schulman S, Varon D, Barzilai
A, Engelberg S, et al. Multifocal pseudotumour in a
single limb. Haemophilia. 1997; 3(1):50-3.
16. Maliekel K, Rana N, Green D. Recombinant factor VIIa
in the management of a pseudotumour in acquired
haemophilia. Haemophilia. 1997; 3(1):54-8.
17. Ishiguro N, Iwahori Y, Kato T, Ito T, Kojima T, Takamatsu
J, et al. The surgical treatment of a haemophilic
pseudotumour in an extremity: a report of three
cases with pathological fractures. Haemophilia. 1998;
18. Heeg M, Smit WM, van der Meer J, van Horn JR.
Excision of a haemophilic pseudotumour of the
ilium, complicated by fistulation. Haemophilia. 1998;
19. Sevilla J, Alvarez MT, Hernández D, Canales M, De
Bustos JG, Magallón M, et al. Therapeutic embolization
and surgical excision of haemophilic pseudotumour.
Haemophilia. 1999; 5(5):360-3.
20. Raj P, Wilde JT, Oliff J, Drake-Lee AB. Nasal haemophilic
pseudotumour. J Laryngol Otol. 1999; 113(10):924-7.
21. Heaton DC, Robertson RW, Rothwell AG. Iliopsoas
haemophiliac pseudotumours with bowel fistulation.
Haemophilia. 2000; 6(1):41-3.
22. Sagarra M, Lucas M, De la Torre E, Almagro D, González
R, Garcí􀆴a T, et al. Successful surgical treatment of
haemophilic pseudotumour, filling the defect with
hydroxyapatite. Haemophilia. 2000; 6(1):55-6.
23. Bellinazzo P, Silvello L, Caimi TM, Mostarda G,
Decataldo F, Baudo F. Long-term evaluation of a novel
surgical approach to the pseudotumour of the ilium
in haemophilia: exeresis and transposition of the
omentum in the residual cavity. Haemophilia. 2000;
24. Kale HA, Rathod KR, Prasad SR, Madiwale CM, Sheth RJ.
Mandibular haemophilic pseudotumour containing a
fluid-fluid level. Br J Radiol. 2001; 74(878):186-8.
25. Wexler S, Edgar M, Thomas A, Learmonth I, Scott
G. Pseudotumour in von Willebrand disease.
Haemophilia. 2001; 7(6):592-4.
26. Gupta S, Mohapatra BB, Ghai S, Seith A, Kashyap R,
Sharma R, et al. Haemophilic pseudotumour of the
paranasal sinuses: management with radiotherapy
and factor replacement therapy. Haemophilia. 2001;
27. O’Connell N, Chen J, Byrne M, O’Shea E, Smyth H,
Smith OP. Massive pseudotumour resection with
recombinant factor VIIa (NovoSeven) cover. Br J
Haematol. 2002; 116(3):645-8.
28. Stevenson DS, Keast AT. An unusual cause of epistaxis:
a haemophilic pseudotumour in a non-haemophiliac,
arising in a paranasal sinus. J Laryngol Otol. 2002;
29. Eby CS, Caracioni AA, Badar S, Joist JH. Massive
retroperitoneal pseudotumour in a patient with
type 3 von Willebrand disease. Haemophilia. 2002;
30. Keller A, Terrier F, Schneider PA, Bianchi S, Howarth
N, De Moerloose P. Pelvic haemophilic pseudotumour:
management of a patient with high level of inhibitors.
Skeletal Radiol. 2002; 31(9):550-3.
31. Takedani H, Mikami S, Kawasaki N, Abe Y, Arai
M, Naka H, et al. Excision of pseudotumour in a
patient with haemophilia A and inhibitor managed 
with recombinant factor VIIa. Haemophilia. 2004;
32. Libby EN, White GC. Cranial pseudotumour in
haemophilia. Haemophilia. 2004; 10(2):186-8.
33. O’Dowd M, Geoghegan T, Munk PL, McAuley G,
Torreggiani WC. Haemophilic pseudotumour
presenting with large bowel obstruction. Australas
Radiol. 2006; 50(4):386-8.
34. Ahuja SP, Sidonio R Jr, Raj AB, Bertolone SJ, Silverman
C, Antekeier DP, et al. Successful combination therapy
of a proximal haemophilic pseudotumour with
surgery, radiation and embolization in a child with
mild haemophilia A. Haemophilia. 2007; 13(2):209-
35. Rey EA, Puia S, Bianco RP, Pinto MT. Haemophilic
pseudotumour of the mandible: report of three cases.
Int J Oral Maxillofac Surg. 2007; 36(6):552-5.
36. Al Saadi AS, Al Wadan AH, El Hamarneh SA, Emad ME.
Life-threatening biopsy of an iliopsoas pseudotumour
in a patient with haemophilia: a case report. J Med
Case Rep. 2008; 2:135.
37. D’Young AI. Conservative physiotherapeutic
management of chronic haematomata and
haemophilic pseudotumours: case study and
comparison to historical management. Haemophilia.
2009; 15(1):253-60.
38. Toepfer A, Diehl P, Gradinger R, Rudert M. Haemophilic
pseudotumour of the distal femur - a case report and
characterisation of this entity. Z Orthop Unfall. 2008;
39. Shi H, Wang S, Wang P, Yu Q. Haemophilic
pseudotumour of the mandible. Dentomaxillofac
Radiol. 2009; 38(3):182-4.
40. Bernstaedt M, Kunze C, Brandt J, Hasenmueller
S, Oldenburg J, Koerholz D, et al. Haemophiliac
pseudotumour: two case reports of patients with
moderate haemophilia A. Klin Padiatr. 2009;
41. Petratos DV, Pergantou H, Matsinos GS, Anastasopoulos
JN, Platokouki H. Intraosseous pseudotumour of the
talus in a child with severe haemophilia. J Pediatr
Orthop B. 2009; 18(6):357-61.
42. Yoshitake Y, Nakayama H, Takamune Y, Yasunaga M,
Hiraki A, Shinohara M. Haemophilic pseudotumour
of the mandible in a 5-year-old patient. Int J Oral
Maxillofac Surg. 2011; 40(1):120-3.
43. Liu B, Liu L, Feng Y, Li L. A case report on the surgical
treatment of the huge inflammatory pseudotumor in
the AIDS patient with hemophilic. Case Rep Pathol.
2011; 2011:798649.
44. Yang BT, Wang YZ, Wang XY, Wang ZC. Imaging
features of paediatric haemophilic pseudotumour of
the maxillary bone: report of three cases and review
of the literature. Br J Radiol. 2012; 85(1016):1107-11.
45. Panotopoulos J, Ay C, Trieb K, Funovics PT,
Stockhammer V, Lang S, et al. Surgical treatment
of the haemophilic pseudotumour: a single centre
experience. Int Orthop. 2012; 36(10):2157-62.
46. Serban M, Mihailov D, Savescu D, Bordos D, Talpos
Niculescu S, Jinca C, et al. Long-term outcome
of an unusual haemophilic pseudotumour.
Hamostaseologie. 2012; 32(Suppl 1):S43-4.
47. Lim MY, Nielsen B, Ma A, Key NS. Clinical features
and management of haemophilic pseudotumours: a
single US centre experience over a 30-year period.
Haemophilia. 2014; 20(1):e58-62.
48. Low SF, Sridharan R, Ngiu CS, Haflah NH. Osseous
haemophilic pseudotumour and concurrent primary
hyperparathyroidism: a diagnostic conundrum. BMJ
Case Rep. 2014; 2014:bcr2013203282.
49. Kamal AF, Sukrisman L, Dilogo IH, Priyamurti
H, Qomaruzzaman MN. Pelvic haemophilic
pseudotumour: a case report. J Orthop Surg (Hong
Kong). 2014; 22(2):263-8.
50. Purkait R, Mukherji A, Dolai TK, Bhadra R.
Intraosseous pseudotumour in a child with mild
hemophilia B: report of a rare case and brief review
of literature. Indian J Hematol Blood Transfus. 2014;
30(Suppl 1):366-8.
51. Dutt K, Agarwal PN, Singh R, Tomar VS. Haemophilic
pseudotumour: surgical management of a rare case.
Indian J Surg. 2015; 77(1):62-4.
52. Kwon AY, Huh KH, Yi WJ, Symkhampha K, Heo MS,
Lee SS, et al. Haemophilic pseudotumour in two parts
of the maxilla: case report. Dentomaxillofac Radiol.
2016; 45(6):20150440.
53. Kumar S, Charllu AP, Chacko R, Porinchu J.
Spontaneous bone regeneration in a large haemophilic
pseudotumour of mandible. BMJ Case Rep. 2018;
54. Rodriguez Merchan EC. The haemophilic
pseudotumour. Int Orthop. 1995; 19(4):255-60.
55. Mann HA, Hilton A, Goddard NJ, Smith MA, Holloway
B, Lee CA. Synovial sarcoma mimicking haemophilic
pseudotumour. Sarcoma. 2006; 2006:27212.
56. Allen DJ, Goddard NJ, Mann HA, Rodriguez-
Merchan EC. Primary malignancies mistaken
for pseudotumours in haemophilic patients.
Haemophilia. 2007; 13(4):383-6.
57. Rodriguez-Merchan EC, Gomez-Cardero P. Pathological
fracture of a true tumour mimicking a haemophilic
pseudotumor. Haemophilia. 2005; 11(2):188-90.
58. Fernandez-Delgado J, Rodriguez-Merchan EC,
Martinez-Mendez JR. First giant gluteal neurofibroma
reported in the literature in a person with haemophilia
and its high risk of massive bleeding to death.
Haemophilia. 2005; 11(4):419-21.