Pseudotumor of hemophilia in the orbit: The role of radiotherapy in management

Meyers, Lee; Hakami, Nasrollah

doi:10.1002/ajh.2830190113

Cited by 32 publications

(25 citation statements)

References 11 publications

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“…Radiation may injure the feeder vessels and thereby prevent tumor progression, 7) or may affect the tumor cells by causing fibrosis. 6) No cases of radiotherapy for hemophilic pseudotumors in cranial bones have been reported.…”

Section: Discussionmentioning

confidence: 99%

Hemophilic Pseudotumor of the Temporal Bone With Conductive Hearing Loss

Kashiwazaki

Terasaka

Kamoshima

et al. 2012

Neurol. Med. Chir.(Tokyo)

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A 46-year-old man with factor VIII deficiency presented with a rare case of hemophilic pseudotumor in the temporal bone manifesting as severe conductive hearing loss and external ear bleeding. The pseudotumor expanded and destroyed the temporal bone and skin of the external ear over the course of 8 years. The pseudotumor was surgically excised, and the patient's symptoms improved. Histological examination of a specimen collected from inside the pseudotumor demonstrated blood products in various stages of evolution and showed that the outer membrane consisted of a collagen layer. Hemophilic pseudotumors are rare complications occurring in 1-2% of patients with mild or severe hemophilia. Pseudotumors are chronic, slowly expanding, encapsulated cystic masses, and most are located in the long bones and pelvis. The present case suggests that cranial pseudotumor should be considered in the differential diagnosis of cranial lesion in a patient with hemophilia.

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Section: Discussionmentioning

confidence: 99%

Hemophilic Pseudotumor of the Temporal Bone With Conductive Hearing Loss

Kashiwazaki

Terasaka

Kamoshima

et al. 2012

Neurol. Med. Chir.(Tokyo)

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“…Even though this latter treatment has been the subject of great changes throughout the period studied, making comparisons difficult, the doses of either factor VIII or factor IX transfused were closely similar in terms of units/kg/day. Reported patients initially given only factor VIII transfusion have also frequently shown poor results [8,14,. Hence the choice of a conservative measure at first does not appear to be useful for most patients, as a general overview, obviously keeping in mind that an individual evaluation of each case cannot be overlooked.…”

Section: Discussionmentioning

confidence: 99%

“…Several therapy approaches therefore have been used to date in managing hemophilic pseudotumors [2]. Surgery [3,4], replacement therapy [5,6], and local irradiation [5,7], alone or combined [5, 6,8], as well as conservative measures [9], mainly have been described.…”

Section: Introductionmentioning

confidence: 99%

Hemophilic pseudotumor: Multicenter experience over 25‐year period

et al. 1994

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Pseudotumor is an infrequent manifestation of bleeding diathesis seen in hemophiliacs, the management of which is still controversial. To ascertain the effectiveness of the main therapies of choice, we have reviewed 1,831 patients affected by hemophilias A (1,108) and B (172), von Willebrand's disease (329), and other miscellaneous coagulopathies (222) diagnosed between 1965 and 1990 in a multicentric, retrospective study. Pseudotumor was proven in 21 patients. Replacement therapy was given in 15 cases as the first therapeutic approach, with complete success attained in only two, whereas surgery, which was carried out in 14 patients, completely resolved the process in eight (P = 0.017). Patients over 40 years of age demonstrated worse prognosis than younger patients (P = 0.02), but no other clinical parameters were shown to have influenced the patients' evolution. Surgical management was the most effective treatment for pseudotumor, although more conservative therapies cannot be overlooked in selected cases.

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“…Radiotherapy with 7.5 Gy external beam radiotherapy was reported by Meyers et al . [7] There has been considerable variation in the literature in radiotherapy dose. Also, the doses, as low as 600 cGy to as high as 2350 cGy, with or without factor VIII replacement, have shown good response with complete resolution of lesions.…”

Section: Discussionmentioning

confidence: 99%

Hemophilia of orbit

Karunanithi¹,

Sethi²,

Reddy³

et al. 2009

Oman J Ophthalmol

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Hemophilic pseudotumor is an uncommon complication of factor VIII and IX deficiencies in the coagulation cascade and occurs in a wide spectrum of bones and soft tissues. We present a six-year-old boy with hemophilic pseudotumor localized in the right orbit. He showed a favorable response to radiation therapy after unsuccessful treatment with factor VIII replacement therapy with no recurrence till eight months. Radiotherapy in the treatment of pseudotumors in hemophiliacs should be strongly considered, particularly in severely affected patients who do not respond to conservative therapy.

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Pseudotumor of hemophilia in the orbit: The role of radiotherapy in management

Cited by 32 publications

References 11 publications

Hemophilic Pseudotumor of the Temporal Bone With Conductive Hearing Loss

Hemophilic Pseudotumor of the Temporal Bone With Conductive Hearing Loss

Hemophilic pseudotumor: Multicenter experience over 25‐year period

Hemophilia of orbit

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