Nasal hemophilic pseudotumor: Favorable response to radiotherapy

Purkait, Radheshyam; Mukherjee, A.S.; Naskar, Suptotthitaa; Bhadra, Ramchandra

doi:10.1007/s13312-013-0071-7

Cited by 9 publications

(13 citation statements)

References 5 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…12,27,[34][35][36][37][38][39][40][41][42] None of these underwent surgery. 12,27,[34][35][36][37][38][39][40][41][42] None of these underwent surgery.…”

Section: Radiotherapymentioning

confidence: 99%

Characteristics and management of the haemophilia‐associated pseudotumours

2019

View full text Add to dashboard Cite

| INTRODUC TI ONHaemophilia-associated pseudotumours (HPT) were first described by Starker in 1918 and are a continuing cause of severe morbidity and mortality for patients with congenital haemophilia. 1 The prevalence of HPT has been described between 1% and 2% of patients with haemophilia with similar levels persisting despite the use of recombinant factor replacement over the last decades. 2,3 The clinical course of HPT can be heterogenous due to a variety of factors including age of onset, disease severity, presence of an inhibitor and inadequate factor replacement. 4 They can result in a variety of problems including bleeding, organ compression and loss of function, bone destruction and fistula formation.HPT typically develops at a site of injury such as a bone fracture, or from spontaneous bleeding in subperiosteal and soft tissues. A preceding traumatic event has been described in 57%-75% of patients with HPT. 3,5 Subsequent bleeding, inadequate blood reabsorption and capsular formation around the haematoma lead to progressive enlargement of the HPT. This can ultimately lead to the eventual mass effect on local structures or bone and skin AbstractThe management of haemophilia-associated pseudotumours presents an ongoing challenge to the haematologist, surgeon and interventional radiologist alike. There is a range of therapeutic approaches including factor replacement, embolization, radiotherapy and a variety of surgical interventions. However, there remains little evidence regarding the most appropriate treatment. We aimed to evaluate the available options of management for the haemophilia-associated pseudotumour. A literature review was performed using relevant terminology and reviewed for treatment approaches and outcomes. The results demonstrated that most of the data is from single case reports with a small number of single-and multicentre case series. In total, 133 patients with 134 described pseudotumours were identified. Adequate haemostatic control with factor replacement was a key component to successful treatment. Surgical excision was the most commonly reported surgical intervention with various composites used for filling of the surgical cavity. The use of radiotherapy has been described particularly in the paediatric population and sites of difficult surgical access. Embolization can be considered as a method of presurgical optimization.Patients with both factor inhibitors and pseudotumours have poorer postoperative outcomes. This review demonstrates that although a lack of large-centre, randomized studies, timely surgical intervention with adequate haemostatic support and the consideration adjuvant therapies in selected cases can achieve acceptable outcomes in this cohort of patients. K E Y W O R D Scomplication, haemophilia A, haemophilia B, pseudotumour, surgery S U PP O RTI N G I N FO R M ATI O NAdditional supporting information may be found online in the Supporting Information section.

show abstract

“…12,27,[34][35][36][37][38][39][40][41][42] None of these underwent surgery. 12,27,[34][35][36][37][38][39][40][41][42] None of these underwent surgery.…”

Section: Radiotherapymentioning

confidence: 99%

Characteristics and management of the haemophilia‐associated pseudotumours

2019

View full text Add to dashboard Cite

show abstract

“…Many patients recall sustaining an injury prior to the development of pseudotumor. When left untreated, such a mass may induce compression and pressure necrosis of adjacent structures [1,9]. The exact pathogenesis of pseudotumor of bone is not well understood.…”

Section: Discussionmentioning

confidence: 99%

“…High quality CT scan and/or magnetic resonance imaging (MRI) is an excellent tool for preoperative visualization of the extent of the lesion, its mass effect on vital surrounding structures and possible invasion of joints. CT is also best suited for detection of intralesional calcifications, internal gas bubbles due to superinfection, cortical expansion and perforation, whereas MRI is superior to CT for delineating soft tissue and intramedullary spaces [2,9].…”

Section: Discussionmentioning

confidence: 99%

“…In general, operative removal of the entire mass is a reliable treatment because the pseudotumor likely will reform if it is not completely removed. Non-surgical mode of treatment in form of radiotherapy alone or combined with replacement therapy has shown promising results as an alternative to a more mutilating surgery or where surgery is contraindicated, or resistant to conservative treatment [9,10].…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Intraosseous Pseudotumour in a Child with Mild Hemophilia B: Report of a Rare Case and Brief Review of Literature

Purkait

Mukherji

Dolai

et al. 2014

Indian J Hematol Blood Transfus

Self Cite

View full text Add to dashboard Cite

Hemophilic pseudotumor is a rare, but wellknown, complication of hemophilia occurring in 1-2 % of individuals with a severe factor VIII or IX deficiency. The hemophilic pseudotumor is defined as an encapsulated hematoma that increases of volume progressively by episodes of recurrent hemorrhage; usually originate in soft tissues or in subperiosteal or intraosseous areas. Very seldom, patient with mild form of hemophilia present with intraosseous pseudotumor. This report describe an 11-yearold boy with mild factor IX deficiency (17 % of normal factor IX activity), who developed a pseudotumor of the femur.

show abstract

“…Various conservative modalities of treatment of pseudotumours have been advocated such as embolization of the vessel [12,13] and radiotherapy [14]. Surgical removal has been shown to be the best option especially in the case of recurrence [2,5,15], as in this case where incomplete removal of the lesion during the first attempt resulted in the recurrence of the lesion.…”

Section: Discussionmentioning

confidence: 99%