Hemophilic Pseudotumor: Radiologic-Pathologic Correlation

Stafford, James M.; James, Tina T.; Allen, Anton M.; Dixon, Lisa R.

doi:10.1148/rg.234025154

Cited by 68 publications

(86 citation statements)

References 11 publications

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“…HPs are found almost exclusively in men between 20 and 70 years of age [3], whereas our three male patients were aged under 3 years. HP may cause no symptoms and remain unchanged for decades.…”

Section: Discussionmentioning

confidence: 51%

“…Conclusion: A high index of suspicion for HP and a familiarity with imaging findings may help to accurately diagnose this rare entity. Haemophilic pseudotumour (HP) is a rare and serious complication that occurs in 1-2% of patients with severe haemophilia, and most frequently develops in the femur, tibia, pelvic bones, small bones of the hand and, rarely, in the craniofacial bones [1][2][3][4][5][6]. It is essentially an encapsulated haematoma resulting from repetitive bleeding and is surrounded by a thick, fibrous capsule.…”

mentioning

confidence: 99%

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Imaging features of paediatric haemophilic pseudotumour of the maxillary bone: report of three cases and review of the literature

Yang

Wang²,

Wang³

et al. 2012

The British Journal of Radiology

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Objectives: Haemophilic pseudotumour (HP) is an extremely rare lesion. The purpose of this study was to describe the CT and MRI features of maxillary bone HPs and introduce the key points to differentiate HP from the mimicking entities in the region. Methods: We retrospectively reviewed three paediatric patients with histologyproven HPs arising from the maxillary bone. All three patients underwent CT and/or MRI. Combined with six previously reported cases in the literature, the imaging features were comprehensively analysed. Results: All HPs showed a well-demarcated, multilobulated expansile osteolytic lesion in the maxillary bone. On non-enhanced CT, HPs appeared of mixed density relative to grey matter. The lesions appeared to have markedly heterogeneous signal intensity on both T 1 and T 2 weighted images, with septa-like enhancement following the administration of contrast material, which corresponded to blood products in various stages of evolution. The lesions caused cortical thinning and even focal disappearance and multiple bone septa were identified within the involved maxillary bone. Some HPs were associated with radiated periosteal proliferation, which can easily be misdiagnosed as a malignant bone tumour. Conclusion: A high index of suspicion for HP and a familiarity with imaging findings may help to accurately diagnose this rare entity. Haemophilic pseudotumour (HP) is a rare and serious complication that occurs in 1-2% of patients with severe haemophilia, and most frequently develops in the femur, tibia, pelvic bones, small bones of the hand and, rarely, in the craniofacial bones [1][2][3][4][5][6]. It is essentially an encapsulated haematoma resulting from repetitive bleeding and is surrounded by a thick, fibrous capsule. To the best of our knowledge, a total of six cases of maxillary bone HPs have been reported in the literature to date [1,2,[7][8][9][10]; unfortunately, the imaging findings were not described effectively. During the past 10 years, HPs in the maxillary bone have been confirmed by histopathology in three children at our hospital. The imaging findings of these three HPs were retrospectively reviewed and, combined with the literature, the value of using CT and MR imaging to diagnose, treat and follow up patients with HP in the maxillary bone was also discussed. Methods and materialsThis study was approved by the institutional review board. We retrospectively reviewed the CT and MRI findings of three patients with histology-proven HPs over a 10-year period

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Section: Discussionmentioning

confidence: 51%

mentioning

confidence: 99%

Imaging features of paediatric haemophilic pseudotumour of the maxillary bone: report of three cases and review of the literature

Yang

Wang²,

Wang³

et al. 2012

The British Journal of Radiology

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“…The large size of the hematoma limits the body's ability to reabsorb the blood products, and a capsule develops around the hematoma creating a pseudotumor [2,4,5,[9][10][11]13]. There may be continued bleeding into the hematoma, resulting in progressive enlargement of the mass and various signal patterns on MRI [3,5,9,11]. Pseudotumors typically are found in the vastus lateralis, soleus, or iliopsoas muscles [10].…”

Section: Discussion and Treatmentmentioning

confidence: 99%

Thigh Pain in a 73-year-old Man

Dixon

Kransdorf

Krishna

et al. 2009

Clinical Orthopaedics &Amp; Related Research

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A 73-year-old man with hemophilia was referred for evaluation of acute on chronic left hip and thigh pain. The patient had mild chronic discomfort in his proximal left thigh for several years, and although no prior records were available, he reported an MRI was performed 7 years earlier for evaluation of his pain and a soft tissue mass was identified. He did not recall any specific treatment given at that time. One month before his current presentation, he awoke with acute pain in his thigh, although he had no history of injury. His pain was exacerbated with weightbearing and improved with rest. He was evaluated by his local physician, and the radiographs showed a bone abnormality with loss of lateral cortical bone in his femur.He was instructed to walk with crutches and was referred for further evaluation and treatment. His medical history revealed Type A hemophilia and a left TKA 23 years earlier that required perioperative clotting factor products.Physical examination revealed a soft tissue fullness in the proximal left thigh that was tender to palpation and a well-healed anterior left knee incision from his TKA. The skin was normal and range of motion in his lower extremity was satisfactory. The patient was intact from a neurovascular standpoint, but he had weakness and pain associated with left knee extension.Imaging studies were performed and included radiographs, MRI, and CT of the femur. The radiograph of the chest was unremarkable.Based on clinical history, physical examination, and imaging studies, what is the differential diagnosis?Imaging Interpretation

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“…Perdarahan berulang dengan pembentukan hematom dan hemartrosis merupakan manifestasi paling sering dari hemofilia. Otot yang paling sering terlibat adalah iliopsoas dan gastrocnemeus, dan sendi yang paling sering terkena adalah sendi lutut, angkle, siku, bahu dan panggul (7,8).…”

Section: Pendahuluanunclassified

Pseudotumor Hemofilia, Suatu Komplikasi Hemofilia yang Jarang

Nurshanty

Hermanto

2016

JKB

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ABSTRAKPseudotumor adalah kista berisi darah pada jaringan lunak atau tulang. Satu sampai dua persen hemofilia berat menyebabkan pseudotumor, yang bisa berasal dari jaringan lunak atau pada subperiosteal atau area intraosseus. Pseudotumor jarang terjadi namun merupakan komplikasi hemofilia yang berbahaya. Kami laporkan kasus pseudotumor hemofilia pada seorang laki-laki 27 tahun dengan keluhan bengkak pada paha kanan yang muncul tiba-tiba, merata dari pangkal paha sampai lutut. Keluhan hilang timbul, bergantian pada kaki kanan kiri sejak 2002. Riwayat perdarahan yang sulit berhenti 15 tahun sebelumnya. Pemeriksaan MRI (magnetic resonansi imaging) menunjukkan massa intra muskular regio femur dekstra 1/3 proksimal hingga 1/3 tengah dan massa intra muskular regio femur sinistra 1/3 proksimal melibatkan kompartemen anterior dan bone marrow replacement. Didapatkan Hemofilia A sedang dengan APTT memanjang (faktor VIII 7%) pada pemeriksaan darah. Pasien membaik dengan terapi konservatif, yaitu pemberian faktor VIII. Kata Kunci: Hemofilia A, pseudotumor hemofilia ABSTRACT Pseudotumor is a blood-filled cysts in

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Hemophilic Pseudotumor: Radiologic-Pathologic Correlation

Cited by 68 publications

References 11 publications

Imaging features of paediatric haemophilic pseudotumour of the maxillary bone: report of three cases and review of the literature

Imaging features of paediatric haemophilic pseudotumour of the maxillary bone: report of three cases and review of the literature

Thigh Pain in a 73-year-old Man

Pseudotumor Hemofilia, Suatu Komplikasi Hemofilia yang Jarang

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