hemophilia a is a congenital disease transmitted by the x chromosome with a recessive trait , characterized by a deficiency in the production of factor viii .
the hemophilic pseudotumor affects 12% of patients with a severe disease , frequently associated with a traumatic injury .
the hemophilic pseudotumor develops from repeated episodes of bleeding , either from fracture sites or bleeding or subperiosteal hemorrhage of any soft tissue .
the interior of the pseudotumor consists of blood products at different stages of development , surrounded by a fibrous capsule containing hemosiderin - laden macrophages .
it appears as a painless tumor of slow growth that can compress key organs causing bone destruction , muscle and skin necrosis .
the objective of case presentation is to describe a patient with a hemophilic pseudotumor of the abdomen , and to review the literature on hemophilic pseudotumor .
this is a case of a 31 year old male , diagnosed with severe hemophilia a at 2 years of age , treated with factor vii 2000  iu weekly since , whit a family history of 2 cousins with hemophilia ( no other specified ) . in september 2011 , he underwent drainage of a hematoma of the left pelvic limb . in october 2011
blood transfusion on multiple occasions . in august 2012 had a hematoma in the left thigh surgically removed . in september 2012 presented upper gastrointestinal bleeding requiring hospitalization with remission of the symptoms .
an abdominal tumor diagnosed 10 years ago during routine screening that progressively grew to encompass the entire abdominal area , with no associated symptoms . during his last hospital admission , hematology referred him to general surgery due to bowel obstruction symptoms , and to evaluate surgical treatment of a pelvic tumor referred as a possible teratoma . a ct scan of the abdomen ( figs . 1 and 2 )
was performed , showing a tumor in the pelvis with clear borders , 241  mm    192  mm of diameter , from the pelvis and projected into the bladder , rectum and adjacent structures , including the kidneys .
the lesion had irregular wall thickness , with a density of 195 houndsfield units in its interior , with heterogeneous enhancement and vascularity present within ; with tomographic diagnosis of abdominal pelvic teratoma .
the patient underwent surgery with surgical findings that included a cyst - like tumor of 40  cm    30  cm displacing loops of small bowel , colon and bladder , with adhesions to the great omentum , small intestine , descending colon and bladder .
macroscopically appeared as an old , organized , and calcified clots with a volume of 5000  ml .
an abdominal tumor diagnosed 10 years ago during routine screening that progressively grew to encompass the entire abdominal area , with no associated symptoms . during his last hospital admission , hematology referred him to general surgery due to bowel obstruction symptoms , and to evaluate surgical treatment of a pelvic tumor referred as a possible teratoma . a ct scan of the abdomen ( figs . 1 and 2 )
was performed , showing a tumor in the pelvis with clear borders , 241  mm    192  mm of diameter , from the pelvis and projected into the bladder , rectum and adjacent structures , including the kidneys .
the lesion had irregular wall thickness , with a density of 195 houndsfield units in its interior , with heterogeneous enhancement and vascularity present within ; with tomographic diagnosis of abdominal pelvic teratoma .
the patient underwent surgery with surgical findings that included a cyst - like tumor of 40  cm    30  cm displacing loops of small bowel , colon and bladder , with adhesions to the great omentum , small intestine , descending colon and bladder .
macroscopically appeared as an old , organized , and calcified clots with a volume of 5000  ml .
the hemophilic pseudotumor of the abdomen is a rare but often disabling condition , potentially fatal in patients with severe hemophilia . diagnosing a hemophilic pseudotumor with invasive techniques such as , aspiration and biopsy
imaging techniques of which mri is preferred , allows recognition of blood products in various stages of evolution .
ultrasonography ( usg ) shows a central anechoic region with increased echoes behind the lesion due to enclosed fluid in the pseudotumor . computed tomography ( ct )
identifies the thick pseudocapsule , but can not differentiate a hematoma from a chronic abscess .
ct is particularly helpful in the evaluation of bone , whereas mri is superior to ct for delineating soft tissue . at the moment ,
however , there are instances where surgical extraction of the lesion is not feasible . in such situations ,
the decision to operate on this patient was made based on the gradual increase in the tumor size , the symptoms that the displayed by the patient . in this patient
this type of procedure is associated with complications such as bleeding , bowel perforation , and damage to nearby structures due to firm vascular , and nervous adhesions . surgical resection after performing arterial embolization to reduce the vascularization of the pseudotumor is a good alternative , thereby reducing the size of the pseudotumor and the risk of bleeding complications during surgery , at best about 2  weeks prior to surgery .
it is a rare pathological entity , but it must be considered in the hemophilic patient with a long - standing abdominal tumor and trauma history , to plan appropriate treatment .
the abdominal hemophilic pseudotumor is a rare pathological entity , with few reports worldwide , but must be considered in hemophilic patients with a well documented abdominal tumor , as a complication of the hematologic disease .
signs and symptoms of compression should be evaluated by ct or mri because its early diagnosis is crucial for evaluation and proper surgical planning , and treatment in conjunction with other specialties .
written informed consent was obtained from the patient for publication of this case report and case series and accompanying images .
a copy of the written consent is available for review by the editor - in - chief of this journal on request .
the study conception and design , as well as writing the manuscript works were all equally distributed among all the authors.key learning pointshemophilic pseudotumor etiology.hemophilic pseudotumor management .
 introductionhemophilic pseudotumor is a rare complication that occurs in patients with severe hemophilia . 
 results from multiple episodes of bleeding into the bones and soft tissues.presentation of casea 31 years old male patient , with severe hemophilia a. diagnosed with an abdominal tumor 10 years ago during routine screening , that progressively grew to encompass the entire abdominal area , with symptoms of intestinal obstruction.discussionhemophilic pseudotumor appears as a painless tumor of slow growth that can compress vital organs producing bone destruction , muscle and skin necrosis . 
 the tumor may have fistulas or break spontaneously.conclusionthe abdominal hemophilic pseudotumor is a rare pathological entity , with few reports worldwide , but must be considered in hemophilic patients with a well documented abdominal tumor . 