desmoplastic fibromas are benign but locally aggressive bone tumors first described by jaffe in 1958 .
desmoplastic fibromas are quite rare , accounting for 0.06% of all bone tumors and 0.3% of benign bone tumors , typically in patients younger than 30 years , involving the metaphysis or diametaphysis of long bones in > 50% of cases , with no reported cases of metastases in the literature [ 24 ] .
radiography of desmoplastic fibromas is typically lytic , expansile and well demarcated , with low signal intensity on both t1- and t2-weighted magnetic resonance imaging ( mri ) .
en bloc or wide resection of the tumors to prevent local recurrence has historically been the method of choice for treatment .
some recent case reports with limited follow - up duration have advocated use of curettage with adjunctive therapy and bone grafting rather than wide resection to minimize functional loss [ 6 , 7 ] .
however , recurrence rates following curettage , excision and wide resection have been shown to be 55 , 72 and 17% , respectively .
the authors have obtained the guardian or patient 's informed written consent for print and electronic publication of the case report .
a 10-year - old male was referred to our office after being seen by a pediatric orthopedic surgeon for a distal left forearm mass with block in pronation and supination .
the patient 's past medical history is significant for a distal radius fracture 2 years prior to his aforementioned office visit .
follow - up radiographs after fracture union at that time showed no evidence of disease ( fig .  1 ) .
radiographs taken at the time of presentation 2 years after fracture healing revealed a 6.5 cm long lesion in the distal syndesmosis arising from the radius .
the lesion resulted in pressure erosions upon the distal radius and ulna , causing a slight splaying of the bones ( fig .  2 ) . 

figure  1:lateral , oblique and anteroposterior ( ap ) views of distal radius fracture 2 years prior to presentation of desmoplastic fibroma . 
 figure  2:ap and lateral radiographs of the left forearm showing 6.5 cm long lesion in the region of the distal syndesmosis between the radius and ulna .
lateral , oblique and anteroposterior ( ap ) views of distal radius fracture 2 years prior to presentation of desmoplastic fibroma .
ap and lateral radiographs of the left forearm showing 6.5 cm long lesion in the region of the distal syndesmosis between the radius and ulna .
the differential diagnosis included primary benign and malignant bone tumors , and thus preoperative staging studies were performed .
a triple - phase bone scan was administered and demonstrated increased uptake in the distal shaft of the left radius and left ulna , as well as in the distal physis of the left radius ( fig . 
figure  3:triple - phase bone scan revealing moderately increased radiotracer uptake in the distal shaft of the left radius and left ulna , as well as in the distal physis of the left radius .
triple - phase bone scan revealing moderately increased radiotracer uptake in the distal shaft of the left radius and left ulna , as well as in the distal physis of the left radius .
a mri images revealed a large mass at the level of the distal radial and ulnar metadiaphyses , measuring 6 cm craniocaudad , 3.7 cm ap and 3 cm transverse ( fig . 
figure  4:(a and b ) t1 mri showing mass iso - intense to muscle .
( c ) t2 mri axial view with overall increased signal intensity , but with a central area of decreased signal .
( c ) t2 mri axial view with overall increased signal intensity , but with a central area of decreased signal .
positive b - catenin nuclear immunostaining , along with histology showing fibrous tissue , supported a diagnosis of desmoplastic fibroma . upon confirmation of desmoplastic fibroma ,
a discussion with the patient 's mother was held , explaining the rarity of the tumor , and the likelihood that the patient would require radical resection to decrease risk of local recurrence , which would lead to a vast reduction in wrist motion .
additionally , as the tumor extended to the distal radius , resection of the joint surface would be necessary .
subsequently , a conversation about reconstructive options was held , including options for use of the fibula , allograft and allo - arthrodesis of the wrist joint . ultimately , the patient 's mother decided to pursue allo - arthrodesis with allograft , with plans to pursue future reconstruction with use of the fibula if this surgery was unsuccessful . upon removal of the mass
dorsal plate fixation was used to stabilize the allograft , which extended from the native proximal radius to the proximal carpal row , effectively creating a single - bone forearm ( fig .  5 ) . 

figure  5:(a and b ) post - operative radiographs of allo - arthrodesis with dorsal plate fixation .
( c and d ) follow - up radiographs at 7 months post - op .
( a and b ) post - operative radiographs of allo - arthrodesis with dorsal plate fixation .
( c and d ) follow - up radiographs at 7 months post - op .
post - operatively , the patient began occupational therapy 1 week after surgery , completing 13 sessions over several months , with a progressive increase in strength , range of motion and coordination of the left upper extremity . at 8 weeks
, the patient was able to employ pincer grasp to pick up small marbles and make a fist at 10 lbs of force , with minimal pain on palpation as well as during therapy exercises . at the request of the patient 's family
at 12 months follow - up , the patient has exhibited excellent recovery , including normal range of motion at the shoulder and elbow and ability to perform all activities of daily livings despite reduced grip strength .
on all follow - up imaging , the allograft has fully incorporated , with no hardware failure or loosening .
although some recent literature has explored the possibility of utilizing intralesional curettage with adjuvant therapy with the intention of sparing maximum limb function , the level of invasion of the tumor and thus increased risk of recurrence in this patient made radical resection a more favorable surgical option . in the largest review of desmoplastic fibroma cases to date ,
recurrence rates were 38% higher following curettage than radical resection , further strengthening a decision to pursue radical resection .
muramatsu reported a case of a young man with desmoplastic fibroma of the distal radius treated successfully with excision of the radius and fibular graft , free of tumor for 7 years as per last reported follow - up , with good range of motion at elbow and wrist .
kesani reported a case of a 15-year - old male with recurrent desmoplastic fibroma of the radius and ulna treated successfully with distal radius allograft with radiocarpal , partial intercarpal and third metacarpal capitate arthrodesis osteosynthesis to the proximal ulna . in this particular case ,
the advantages of wide resection , including a substantial decrease in the rate of local recurrence , outweighed the drawbacks of the procedure , including reduced limb function .
factors such as tumor size and location , as well as age and activity of the patient , must be considered in each case .
previously , the senior author had a patient with a similar case which was treated with more conservative curettage several times , and ultimately required an above the elbow amputation to control extensive recurrent disease , fracture and hardware failure .
this experience encouraged the author to advocate for more aggressive treatment on this patient 's behalf .
this case highlights the importance of extensive discussion with parents of the child and detailed follow - up with the surgeon and physical therapists to ensure maximal limb function recovery and monitoring for recurrence of disease .
 desmoplastic fibromas are rare , benign , locally aggressive bone tumors , which arise primarily in patients younger than 30 years old . 
 historically , even with greater functional loss , en bloc or wide resection of the tumors to prevent local recurrence has been the method of choice in treatment . 
 this article discusses the presentation of a 10-year - old male who presented with a mass in the distal forearm , after reporting difficulty in pronation and supination . 
 the patient was ultimately treated with wide resection and allo - arthrodesis with allograft . 
 post - operatively , the patient has exhibited excellent recovery , including normal range of motion at the shoulder and elbow , and ability to perform all activities of daily livings despite reduced grip strength compared with the contralateral side . 
INTRODUCTION
CASE REPORT
DISCUSSION
CONFLICT OF INTEREST STATEMENT