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Pelvic hemophilic pseudotumor presenting as severe sciatic pain in a patient with no history of hemophilic symptoms
J Orthop Sci (2012) 17:490–494 DOI 10.1007/s00776-011-0094-7
CASE REPORT
Pelvic hemophilic pseudotumor presenting as severe sciatic pain in a patient with no history of hemophilic symptoms Szu Han Ying • Wei Ming Chen • Po Kuei Wu Cheng Fong Chen • Chien Lin Liu • Tain Hsiung Chen
•
Received: 26 August 2010 / Accepted: 31 March 2011 / Published online: 4 June 2011 Ó The Japanese Orthopaedic Association 2011
Introduction Hemophilic pseudotumor is a rare, but well-known complication of hemophilia occurring in approximately 1–2% of patients with severe forms of the disease [1, 2]. A pseudotumor is a collection of encapsulated blood caused by recurrent hemorrhage in bone or soft tissue. Pseudotumors are classified as soft-tissue or osseous based on their location of occurrence [3]. The pseudotumor itself is painless; however, as it grows it causes increasing compression of adjacent structures, leading to necrosis [2]. Nerve compression or compartment syndrome can result in severe pain. Though the diagnosis is straightforward in individuals with known hemophilia, the occurrence of a pseudotumor in a patient without a known history of hemophilia can be a diagnostic challenge [4]. Although the treatment of bleeding episodes in patients with hemophilia is well established, the treatment of hemophilic pseudotumors is difficult, partly because of their rarity. Surgical resection or drainage, chronic factor replacement therapy, external beam irradiation, and other non-surgical measures have been used to treat hemophilic pseudotumors [5, 6]. We herein report a hemophilic pseudotumor in a patient without a history of hemophilia or bleeding tendency who
S. H. Ying W. M. Chen (&) P. K. Wu C. F. Chen C. L. Liu T. H. Chen Department of Orthopaedics, Taipei Veterans General Hospital, No. 201, Sec. 2, Shipai Rd., Beitou District, Taipei 11217, Taiwan, ROC e-mail: [email protected] S. H. Ying W. M. Chen P. K. Wu C. F. Chen C. L. Liu T. H. Chen Department of Surgery, School of Medicine, National Yang-Ming University, Taipei, Taiwan, ROC
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presented with severe right leg pain initially diagnosed as sciatica.
Case report A 34-year-old male presented with a 1-month history of pain from the lateral side of his right hip to his right knee. The patient had been seen at another hospital and was told he had sciatica caused by a tumor in his pelvis. He denied any history of trauma, fever, local heat, night sweats, or weight loss. He also denied any history of medical conditions or systemic diseases. Physical examination revealed the patient to be in distress from right leg pain, his right hip and knee were in flexion, and he could not walk. Decreased range of motion (ROM) of the right hip and knee were noted, and a large soft tissue mass with a well-defined border was noted in the right iliac region. The patient was admitted for further evaluation. Initial blood tests including blood count, electrolytes, and tests of liver and kidney function were normal. Chest X-ray, abdominal sonography, and computed tomography (CT) of the abdomen were normal. Bone scan showed localized changes in the right pelvic wall. Magnetic resonance imaging (MRI) of the pelvis revealed a 10 9 10 9 15-cm lobulated soft tissue mass, possibly arising from the right psoas muscle (Fig. 1). A soft tissue tumor was suspected, likely malignant in nature, and the differential diagnosis included synovial sarcoma, malignant fibrous histiocytoma (MFH), neurogenic tumor such as malignant peripheral nerve sheath tumor (MPNST), or other types of sarcomas. Tumor marker studies, i.e., carcinoembryonic antigen (CEA), CA-125, and CA-199 were negative. Electromyography was performed; the result suggested a right lumbosacral plexus lesion, but was
Pelvic hemophilic pseudotumor
Fig. 1 Magnetic resonance imaging (MRI) of the pelvis. a Axial T2weighted imaging (WI), b axial T1 WI post-gadolinium contrast enhancement, and c coronal T2 WI with fat saturation revealed a 10 9 10 9 15-cm lobulated soft tissue mass (arrows) in the right lower abdominal/pelvic region, which appeared to be arising from the right psoas muscle. The mass appeared to be attached to the right iliac wing, with possible periosteal invasion and compression of the pelvic organs to the left. Internal cystic changes can be seen (arrowheads). There is mild post-contrast enhancement suggestive of high vascularity (arrowheads in b)
491
inconclusive because the patient could not cooperate because of pain. Since a malignant tumor with nerve compression was suspected, surgical intervention was planned. Preoperative testing of coagulation function revealed an increased activated partial thromboplastin time (aPTT) of 65.0 s (normal range 23.9–35.5 s) and normal prothrombin time (PT). A full workup of coagulation dysfunction revealed a bleeding time [10 min (normal \7 min), factor VIII activity of 8% (normal 50–150%), and epinephrine platelet aggregation test of 8% (normal [50%). The results of other factor and platelet aggregation assays were within normal limits. Antinuclear antibody and lupus anticoagulant studies were negative. An ultrasound-guided biopsy of the lesion was performed 2 days after admission, and histological examination revealed a fibrinous exudate without remarkable skeletal muscle tissue. Fibrosis and focal chronic inflammatory cell infiltration were noted, and no evidence of malignancy was observed. Based on these findings, the patient’s diagnosis was revised from malignant tumor to hemophilic pseudotumor. A hematologist was consulted, and the patient was diagnosed with hemophilia A (acquired factor VIII deficiency). NovoSevenÒ (Novo Nordisk), which is indicated for the treatment of bleeding episodes in hemophilia A or B patients with inhibitors to factor VIII or factor IX, was administered for 6 days before surgery. NovoSeven was used rather than factor VIII replacement because the patient had acquired factor VIII deficiency, and cryoprecipitate may have been ineffective because of an immune response. Transaortic embolization of the pseudotumor was performed 4 days before surgery (Fig. 2). Surgery was performed 30 days after admission. Intraoperatively, the lesion appeared similar to a hematoma without an identifiable border or capsule-liked margin. Thus, we performed an intralesional excision, and 400 g of lobulated hematoma was removed (Fig. 3). The procedure was performed without complications. Physical rehabilitation consisting of passive and active flexion and extension was initiated immediately postoperatively, and EMG showed improvement compared with the prior study. The patient was discharged on postoperative day 61 (91 days after admission). At discharge he was able to walk without assistance, and at final follow-up motor and sensory functions of the lower extremities were intact. No recurrence of the pseudotumor has been noted. He is currently being followed in the hematology department every 6 months and is asymptomatic without treatment of the factor VIII deficiency. As of his last follow-up in May 2010, he has full function of his right lower limb. The patient and his family were informed that his data would be submitted for publication and gave their consent.
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Fig. 2 Transaortic embolization of the aneurysm (TAE). a Right pelvic angiography done via the left femoral artery approach revealed a fusiform and saccular aneurysm, which appeared to be a pseudoaneurysm at the right deep circumflex iliac artery (arrow). Active bleeding was also noticed (double arrow). b Pulsatile bleeding was noted with blood pooling (arrow). c Plain film of the pelvis revealed pooling of contrast medium. The pooling area was compatible with the location of the pseudotumor shown in MRI studies (Fig. 1). d TAE was done after superselection into the artery and near the aneurysm with a Renegade coaxial catheter. The embolization agent was N-butyl-2cyanoacrylate (NBCA):lipiodol in a 1:2 ratio. Angiography immediately after embolization revealed complete obliteration of the aneurysm
Discussion Hemophilic pseudotumor affects 1–2% of individuals with severe hemophilia, and because of the genetic association of hemophilia with the X chromosome, they are predominately seen in men [1, 2]. Pseudotumors are more common in parts of the world where the diagnosis and management of bleeding disorders is difficult [7]. The lesions typically occur in soft tissues. Intramuscular locations are common, but they can also occur in bone or subperiosteal locations, and are generally classified based on location, i.e., softtissue or osseous [3, 4]. The bones most commonly affected by pseudotumors are the femur, pelvis, tibia, and bones of the hand [4]. In adult patients, pseudotumors are typically slowly expanding and can be present for years before enlargement results in symptoms prompting evaluation and diagnosis [5, 7, 8]. A pseudotumor is composed of blood in various stages of coagulation and is encapsulated by a fibrous capsule [7]. As a pseudotumors progressively enlarges, increased pressure on adjacent structures is exerted. Prolonged pressure can cause necrosis of compressed tissues, and compartment syndrome and joint contracture can occur. Pseudotumors themselves are generally painless, but
123
compression of nerves may produce pain or neurological deficits [9]. Pathological fractures can cause pain, and skin or bowel infection or fistula formation can occur [9]. Enlargement of a pseudotumor within soft tissue or muscle can result in loss of function of the extremity. Extraspinal compression of the sciatic nerve is not common, and can be difficult to diagnose because the symptoms are essentially the same as the more common causes of sciatic pain [10]. In our patient, compression of surrounding tissue resulted in neurological involvement and symptoms consistent with severe sciatica and the loss of function of his lower extremities. Since this condition is unique to hemophilia, diagnosis is usually straightforward. However, in our case a mild form was present, and no history of hemophilia or bleeding tendency was apparent. Thus, determination of the nature and cause of the mass was difficult. Percutaneous drainage or diagnostic biopsy was not recommended because of the high risk of complications, including bleeding, infection, and fistula formation [9]. A biopsy was performed in our case because a diagnosis of pseudotumor was not considered because of the negative history of bleeding, and fortunately the outcome was favorable. CT and MRI are the most useful modalities for the diagnosis of hemophilic
Pelvic hemophilic pseudotumor
493
for 6 weeks before percutaneous drainage or surgical excision. In some cases, pseudotumor have been noted to resolve with long-term factor replacement [7]. Special concern should be exerted regarding pseudotumor in the ileum, since operative management is exceedingly challenging because it is difficult to close the dead space after excision of the lesion, and hematoma formation and infection are likely [13]. Radiation therapy has been used successfully in patients with pseudotumors in a number of locations, but has generally been reserved for patients who do not respond to conservative treatment or those in whom surgery is contraindicated [6, 14–16]. Espandar et al. [17] suggest that radiotherapy in conjunction with replacement therapy is the management of choice for pseudotumors located in the skull or distal part of the limbs. Therapeutic emoblization has also been recommended preoperatively, especially for pseudotumors in the pelvis, to decrease the size of the tumor and decrease bleeding during surgery [17]. In summary, we have reported the case of a patient with pelvic hemophilic pseudotumor who presented with severe sciatic pain in a patient with no history, signs, or symptoms of hemophilia. Diagnosis was made after finding coagulation abnormalities on routine preoperative testing. This case highlights a very rare cause of sciatic pain, and the importance of thorough evaluation of clinical data to achieve a correct diagnosis and avoid invasive diagnostic testing when contraindicated. Fig. 3 a Surgical excision of the pseudotumor. b Grossly, blood clots were found without any remarkable architecture
pseudotumor, and for determining the extent of the pseudotumor invasion in bone and soft tissues as well as the degree of neurovascular involvement [4, 11]. CT is useful for the evaluation of trabeculae, cortical change, and periosteal reaction, while MRI is useful for assessment of intramedullary bone and adjacent soft tissue as well as monitoring the therapeutic response [4]. Prevention of pseudotumors is paramount, as treatment is difficult and complications, including infection, fistulization, pathological fractures, and death, can occur [12]. There is no standard therapy for hemophilic pseudotumors, and the primarily goals of management are minimizing complications and preserving function of the affected tissues [8, 9]. Conservative therapy with immobilization and clotting factor replacement is recommended by some authors for pseudotumors due to recent hemorrhage, and surgical management for those that have failed conservative management or that have been present for years [2, 9]. Other authors, however, suggest surgery should be performed in all cases [9]. Caviglia et al. [7] have described daily preoperative treatment with factor VIII or factor IX
Conflict of interest The authors declare that they have no conflict of interest regarding this work.
References 1. Brant EE, Jordan HH. Radiologic aspects of hemophilic pseudotumor in bone. AJR Am J Roentgenol. 1972;115:525–39. 2. Ahlberg AK. On the natural history of hemophilic pseudotumor. J Bone Joint Surg. 1975;57A:1133–6. 3. Resnick D. Diagnosis of bone and joint disorders. 4th ed, vol 2002. Philadelphia: WB Saunders. p. 2346–73. 4. Park JS, Ryu KN. Hemophilic pseudotumor involving the musculoskeletal system: spectrum of radiologic findings. AJR Am J Roentgenol. 2004;183:55–61. 5. Lewis TR, Webb HR, Bell JP, Beall DP, Fish JR. Hemophilic pseudotumor. J Okla State Med Assoc. 2005;98:485–7. 6. Subasi M, Dirier A, Kapukaya A, Uludag˘ A, Karadayi B, Cebesoy O. Successful treatment of hemophilic hand pseudotumors by only radiotherapy. Ann Plast Surg. 2007;59:338–40. 7. Caviglia HA, FernAndez-Palazzi F, Gilbert MS. Haemophilic pseudotumours of the limbs and their percutaneous treatment. Haemophilia. 2002;8:402–6. 8. Castro-Boix S, Pradell-Teigell J, Boque´-Genovard R, Zano´nNavarro V, Nadal-Guinard A, Altisent-Roca C, Armengol-Carrasco M. Hemophilic pelvic pseudotumor. Circ Esp. 2007;81: 102–4; Article in Spanish. 9. Magallo´n M, Monteagudo J, Altisent C, Iba´n˜ez A, Rodrı´guezPe´rez A, Riba J, Tusell J, Martı´n-Villar J. Hemophilic
123
494
10.
11.
12. 13.
S. H. Ying et al. pseudotumor: multicenter experience over a 25-year period. Am J Hematol. 1994;45:103–8; Article in Spanish. Kulcu DG, Naderi S. Differential diagnosis of intraspinal and extraspinal non-discogenic sciatica. J Clin Neurosci. 2008;15: 1246–52. Geyskens W, Vanhoenacker FM, Van der Zijden T, Peerlinck K. MR imaging of intra-osseous hemophilic pseudotumor: case report and review of the literature. JBR-BTR. 2004;87:289–93. Rodriguez-Merchan EC. Haemophilic cysts (pseudotumours). Haemophilia. 2002;8:393–401. Iwata H, Oishi Y, Itoh A, Ishiguro N, Yamaga H, Miyamoto N, Kamiya T. Surgical excision of hemophilic pseudotumor of the ilium. Clin Orthop Relat Res. 1992;284:234–8; Article in Japanese.
123
14. Kang JO, Cho YJ, Yoo MC, Hong SE. Hemophilic pseudotumor of the ulna treated with low dose radiation therapy: a case report. J Korean Med Sci. 2000;15:601–3. 15. Nongrum B, Srinivasan R, Pandian DG, Gupta A, Krishnan M. Role of radiotherapy in hemophilic pseudotumor of the orbit. Orbit. 2008;27:377–9. 16. Issaivanan M, Shrikande MP, Mahapatra M, Choudhry VP. Management of hemophilic pseudotumor of thumb in a child. J Pediatr Hematol Oncol. 2004;26:128–32. 17. Espandar R, Heidari P, Rodriguez-Merchan EC. Management of haemophilic pseudotumours with special emphasis on radiotherapy and arterial embolization. Haemophilia. 2009;15:448–57.
CASE REPORT
Pelvic hemophilic pseudotumor presenting as severe sciatic pain in a patient with no history of hemophilic symptoms Szu Han Ying • Wei Ming Chen • Po Kuei Wu Cheng Fong Chen • Chien Lin Liu • Tain Hsiung Chen
•
Received: 26 August 2010 / Accepted: 31 March 2011 / Published online: 4 June 2011 Ó The Japanese Orthopaedic Association 2011
Introduction Hemophilic pseudotumor is a rare, but well-known complication of hemophilia occurring in approximately 1–2% of patients with severe forms of the disease [1, 2]. A pseudotumor is a collection of encapsulated blood caused by recurrent hemorrhage in bone or soft tissue. Pseudotumors are classified as soft-tissue or osseous based on their location of occurrence [3]. The pseudotumor itself is painless; however, as it grows it causes increasing compression of adjacent structures, leading to necrosis [2]. Nerve compression or compartment syndrome can result in severe pain. Though the diagnosis is straightforward in individuals with known hemophilia, the occurrence of a pseudotumor in a patient without a known history of hemophilia can be a diagnostic challenge [4]. Although the treatment of bleeding episodes in patients with hemophilia is well established, the treatment of hemophilic pseudotumors is difficult, partly because of their rarity. Surgical resection or drainage, chronic factor replacement therapy, external beam irradiation, and other non-surgical measures have been used to treat hemophilic pseudotumors [5, 6]. We herein report a hemophilic pseudotumor in a patient without a history of hemophilia or bleeding tendency who
S. H. Ying W. M. Chen (&) P. K. Wu C. F. Chen C. L. Liu T. H. Chen Department of Orthopaedics, Taipei Veterans General Hospital, No. 201, Sec. 2, Shipai Rd., Beitou District, Taipei 11217, Taiwan, ROC e-mail: [email protected] S. H. Ying W. M. Chen P. K. Wu C. F. Chen C. L. Liu T. H. Chen Department of Surgery, School of Medicine, National Yang-Ming University, Taipei, Taiwan, ROC
123
presented with severe right leg pain initially diagnosed as sciatica.
Case report A 34-year-old male presented with a 1-month history of pain from the lateral side of his right hip to his right knee. The patient had been seen at another hospital and was told he had sciatica caused by a tumor in his pelvis. He denied any history of trauma, fever, local heat, night sweats, or weight loss. He also denied any history of medical conditions or systemic diseases. Physical examination revealed the patient to be in distress from right leg pain, his right hip and knee were in flexion, and he could not walk. Decreased range of motion (ROM) of the right hip and knee were noted, and a large soft tissue mass with a well-defined border was noted in the right iliac region. The patient was admitted for further evaluation. Initial blood tests including blood count, electrolytes, and tests of liver and kidney function were normal. Chest X-ray, abdominal sonography, and computed tomography (CT) of the abdomen were normal. Bone scan showed localized changes in the right pelvic wall. Magnetic resonance imaging (MRI) of the pelvis revealed a 10 9 10 9 15-cm lobulated soft tissue mass, possibly arising from the right psoas muscle (Fig. 1). A soft tissue tumor was suspected, likely malignant in nature, and the differential diagnosis included synovial sarcoma, malignant fibrous histiocytoma (MFH), neurogenic tumor such as malignant peripheral nerve sheath tumor (MPNST), or other types of sarcomas. Tumor marker studies, i.e., carcinoembryonic antigen (CEA), CA-125, and CA-199 were negative. Electromyography was performed; the result suggested a right lumbosacral plexus lesion, but was
Pelvic hemophilic pseudotumor
Fig. 1 Magnetic resonance imaging (MRI) of the pelvis. a Axial T2weighted imaging (WI), b axial T1 WI post-gadolinium contrast enhancement, and c coronal T2 WI with fat saturation revealed a 10 9 10 9 15-cm lobulated soft tissue mass (arrows) in the right lower abdominal/pelvic region, which appeared to be arising from the right psoas muscle. The mass appeared to be attached to the right iliac wing, with possible periosteal invasion and compression of the pelvic organs to the left. Internal cystic changes can be seen (arrowheads). There is mild post-contrast enhancement suggestive of high vascularity (arrowheads in b)
491
inconclusive because the patient could not cooperate because of pain. Since a malignant tumor with nerve compression was suspected, surgical intervention was planned. Preoperative testing of coagulation function revealed an increased activated partial thromboplastin time (aPTT) of 65.0 s (normal range 23.9–35.5 s) and normal prothrombin time (PT). A full workup of coagulation dysfunction revealed a bleeding time [10 min (normal \7 min), factor VIII activity of 8% (normal 50–150%), and epinephrine platelet aggregation test of 8% (normal [50%). The results of other factor and platelet aggregation assays were within normal limits. Antinuclear antibody and lupus anticoagulant studies were negative. An ultrasound-guided biopsy of the lesion was performed 2 days after admission, and histological examination revealed a fibrinous exudate without remarkable skeletal muscle tissue. Fibrosis and focal chronic inflammatory cell infiltration were noted, and no evidence of malignancy was observed. Based on these findings, the patient’s diagnosis was revised from malignant tumor to hemophilic pseudotumor. A hematologist was consulted, and the patient was diagnosed with hemophilia A (acquired factor VIII deficiency). NovoSevenÒ (Novo Nordisk), which is indicated for the treatment of bleeding episodes in hemophilia A or B patients with inhibitors to factor VIII or factor IX, was administered for 6 days before surgery. NovoSeven was used rather than factor VIII replacement because the patient had acquired factor VIII deficiency, and cryoprecipitate may have been ineffective because of an immune response. Transaortic embolization of the pseudotumor was performed 4 days before surgery (Fig. 2). Surgery was performed 30 days after admission. Intraoperatively, the lesion appeared similar to a hematoma without an identifiable border or capsule-liked margin. Thus, we performed an intralesional excision, and 400 g of lobulated hematoma was removed (Fig. 3). The procedure was performed without complications. Physical rehabilitation consisting of passive and active flexion and extension was initiated immediately postoperatively, and EMG showed improvement compared with the prior study. The patient was discharged on postoperative day 61 (91 days after admission). At discharge he was able to walk without assistance, and at final follow-up motor and sensory functions of the lower extremities were intact. No recurrence of the pseudotumor has been noted. He is currently being followed in the hematology department every 6 months and is asymptomatic without treatment of the factor VIII deficiency. As of his last follow-up in May 2010, he has full function of his right lower limb. The patient and his family were informed that his data would be submitted for publication and gave their consent.
123
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S. H. Ying et al.
Fig. 2 Transaortic embolization of the aneurysm (TAE). a Right pelvic angiography done via the left femoral artery approach revealed a fusiform and saccular aneurysm, which appeared to be a pseudoaneurysm at the right deep circumflex iliac artery (arrow). Active bleeding was also noticed (double arrow). b Pulsatile bleeding was noted with blood pooling (arrow). c Plain film of the pelvis revealed pooling of contrast medium. The pooling area was compatible with the location of the pseudotumor shown in MRI studies (Fig. 1). d TAE was done after superselection into the artery and near the aneurysm with a Renegade coaxial catheter. The embolization agent was N-butyl-2cyanoacrylate (NBCA):lipiodol in a 1:2 ratio. Angiography immediately after embolization revealed complete obliteration of the aneurysm
Discussion Hemophilic pseudotumor affects 1–2% of individuals with severe hemophilia, and because of the genetic association of hemophilia with the X chromosome, they are predominately seen in men [1, 2]. Pseudotumors are more common in parts of the world where the diagnosis and management of bleeding disorders is difficult [7]. The lesions typically occur in soft tissues. Intramuscular locations are common, but they can also occur in bone or subperiosteal locations, and are generally classified based on location, i.e., softtissue or osseous [3, 4]. The bones most commonly affected by pseudotumors are the femur, pelvis, tibia, and bones of the hand [4]. In adult patients, pseudotumors are typically slowly expanding and can be present for years before enlargement results in symptoms prompting evaluation and diagnosis [5, 7, 8]. A pseudotumor is composed of blood in various stages of coagulation and is encapsulated by a fibrous capsule [7]. As a pseudotumors progressively enlarges, increased pressure on adjacent structures is exerted. Prolonged pressure can cause necrosis of compressed tissues, and compartment syndrome and joint contracture can occur. Pseudotumors themselves are generally painless, but
123
compression of nerves may produce pain or neurological deficits [9]. Pathological fractures can cause pain, and skin or bowel infection or fistula formation can occur [9]. Enlargement of a pseudotumor within soft tissue or muscle can result in loss of function of the extremity. Extraspinal compression of the sciatic nerve is not common, and can be difficult to diagnose because the symptoms are essentially the same as the more common causes of sciatic pain [10]. In our patient, compression of surrounding tissue resulted in neurological involvement and symptoms consistent with severe sciatica and the loss of function of his lower extremities. Since this condition is unique to hemophilia, diagnosis is usually straightforward. However, in our case a mild form was present, and no history of hemophilia or bleeding tendency was apparent. Thus, determination of the nature and cause of the mass was difficult. Percutaneous drainage or diagnostic biopsy was not recommended because of the high risk of complications, including bleeding, infection, and fistula formation [9]. A biopsy was performed in our case because a diagnosis of pseudotumor was not considered because of the negative history of bleeding, and fortunately the outcome was favorable. CT and MRI are the most useful modalities for the diagnosis of hemophilic
Pelvic hemophilic pseudotumor
493
for 6 weeks before percutaneous drainage or surgical excision. In some cases, pseudotumor have been noted to resolve with long-term factor replacement [7]. Special concern should be exerted regarding pseudotumor in the ileum, since operative management is exceedingly challenging because it is difficult to close the dead space after excision of the lesion, and hematoma formation and infection are likely [13]. Radiation therapy has been used successfully in patients with pseudotumors in a number of locations, but has generally been reserved for patients who do not respond to conservative treatment or those in whom surgery is contraindicated [6, 14–16]. Espandar et al. [17] suggest that radiotherapy in conjunction with replacement therapy is the management of choice for pseudotumors located in the skull or distal part of the limbs. Therapeutic emoblization has also been recommended preoperatively, especially for pseudotumors in the pelvis, to decrease the size of the tumor and decrease bleeding during surgery [17]. In summary, we have reported the case of a patient with pelvic hemophilic pseudotumor who presented with severe sciatic pain in a patient with no history, signs, or symptoms of hemophilia. Diagnosis was made after finding coagulation abnormalities on routine preoperative testing. This case highlights a very rare cause of sciatic pain, and the importance of thorough evaluation of clinical data to achieve a correct diagnosis and avoid invasive diagnostic testing when contraindicated. Fig. 3 a Surgical excision of the pseudotumor. b Grossly, blood clots were found without any remarkable architecture
pseudotumor, and for determining the extent of the pseudotumor invasion in bone and soft tissues as well as the degree of neurovascular involvement [4, 11]. CT is useful for the evaluation of trabeculae, cortical change, and periosteal reaction, while MRI is useful for assessment of intramedullary bone and adjacent soft tissue as well as monitoring the therapeutic response [4]. Prevention of pseudotumors is paramount, as treatment is difficult and complications, including infection, fistulization, pathological fractures, and death, can occur [12]. There is no standard therapy for hemophilic pseudotumors, and the primarily goals of management are minimizing complications and preserving function of the affected tissues [8, 9]. Conservative therapy with immobilization and clotting factor replacement is recommended by some authors for pseudotumors due to recent hemorrhage, and surgical management for those that have failed conservative management or that have been present for years [2, 9]. Other authors, however, suggest surgery should be performed in all cases [9]. Caviglia et al. [7] have described daily preoperative treatment with factor VIII or factor IX
Conflict of interest The authors declare that they have no conflict of interest regarding this work.
References 1. Brant EE, Jordan HH. Radiologic aspects of hemophilic pseudotumor in bone. AJR Am J Roentgenol. 1972;115:525–39. 2. Ahlberg AK. On the natural history of hemophilic pseudotumor. J Bone Joint Surg. 1975;57A:1133–6. 3. Resnick D. Diagnosis of bone and joint disorders. 4th ed, vol 2002. Philadelphia: WB Saunders. p. 2346–73. 4. Park JS, Ryu KN. Hemophilic pseudotumor involving the musculoskeletal system: spectrum of radiologic findings. AJR Am J Roentgenol. 2004;183:55–61. 5. Lewis TR, Webb HR, Bell JP, Beall DP, Fish JR. Hemophilic pseudotumor. J Okla State Med Assoc. 2005;98:485–7. 6. Subasi M, Dirier A, Kapukaya A, Uludag˘ A, Karadayi B, Cebesoy O. Successful treatment of hemophilic hand pseudotumors by only radiotherapy. Ann Plast Surg. 2007;59:338–40. 7. Caviglia HA, FernAndez-Palazzi F, Gilbert MS. Haemophilic pseudotumours of the limbs and their percutaneous treatment. Haemophilia. 2002;8:402–6. 8. Castro-Boix S, Pradell-Teigell J, Boque´-Genovard R, Zano´nNavarro V, Nadal-Guinard A, Altisent-Roca C, Armengol-Carrasco M. Hemophilic pelvic pseudotumor. Circ Esp. 2007;81: 102–4; Article in Spanish. 9. Magallo´n M, Monteagudo J, Altisent C, Iba´n˜ez A, Rodrı´guezPe´rez A, Riba J, Tusell J, Martı´n-Villar J. Hemophilic
123
494
10.
11.
12. 13.
S. H. Ying et al. pseudotumor: multicenter experience over a 25-year period. Am J Hematol. 1994;45:103–8; Article in Spanish. Kulcu DG, Naderi S. Differential diagnosis of intraspinal and extraspinal non-discogenic sciatica. J Clin Neurosci. 2008;15: 1246–52. Geyskens W, Vanhoenacker FM, Van der Zijden T, Peerlinck K. MR imaging of intra-osseous hemophilic pseudotumor: case report and review of the literature. JBR-BTR. 2004;87:289–93. Rodriguez-Merchan EC. Haemophilic cysts (pseudotumours). Haemophilia. 2002;8:393–401. Iwata H, Oishi Y, Itoh A, Ishiguro N, Yamaga H, Miyamoto N, Kamiya T. Surgical excision of hemophilic pseudotumor of the ilium. Clin Orthop Relat Res. 1992;284:234–8; Article in Japanese.
123
14. Kang JO, Cho YJ, Yoo MC, Hong SE. Hemophilic pseudotumor of the ulna treated with low dose radiation therapy: a case report. J Korean Med Sci. 2000;15:601–3. 15. Nongrum B, Srinivasan R, Pandian DG, Gupta A, Krishnan M. Role of radiotherapy in hemophilic pseudotumor of the orbit. Orbit. 2008;27:377–9. 16. Issaivanan M, Shrikande MP, Mahapatra M, Choudhry VP. Management of hemophilic pseudotumor of thumb in a child. J Pediatr Hematol Oncol. 2004;26:128–32. 17. Espandar R, Heidari P, Rodriguez-Merchan EC. Management of haemophilic pseudotumours with special emphasis on radiotherapy and arterial embolization. Haemophilia. 2009;15:448–57.