angiomatosis is a complex vascular malformation of infancy and childhood consisting of proliferating blood vessels with accompanying mature fat , fibrous tissue , lymphatics and nerves which may involve skin , subcutaneous tissue , skeletal muscle and occasionally bone .
it is extremely rare and benign but a clinically extensive vascular lesion of soft tissue which usually becomes symptomatic during childhood or adolescence . according to rao and weiss ,
angiomatosis has been defined as a histologically benign vascular lesion that affects a large segment of the body in a contiguous fashion , either by vertically involving multiple tissue types ( e.g. subcutis , muscle , bone ) or by involving similar types of tissues ( e.g. multiple muscle ) .
it has no malignant potential , but because of its infiltrative nature , it could be misdiagnosed as a malignancy using imaging techniques . for these reasons diagnosis based on clinical and radiographic findings in correlation with histologic features would be desirable .
its diffuse infiltrative nature makes surgical resection difficult ; hence , recurrence is a common feature in angiomatosis [ 2 , 3 ] .
the aim of this paper is to present three cases of angiomatosis which were clinically mimicking hemangioma , lipoma and arteriovenous ( av ) malformation at rare sites such as within the masseter , malar region and in the mandible , respectively .
a 13-year - old female presented with the complaint of swelling in the right side of face since 2  years .
the swelling had slowly progressed in size during the period . on examination , a diffuse swelling in the right side of the face extending from the ala - tragal plane superiorly to the lower border of mandible , from the anterior to posterior border of masseter , was seen .
intraorally , bluish discolouration of the right buccal mucosa with telengiectasia or spider nevi appearance was evident ( fig . 
the swelling was non tender , collapsible on digital pressure and filled up on releasing the pressure .
1a lesion seen on buccal mucosa , b small opacities depicting phleboliths , c proliferating vessels infiltrating in between the muscle bundles , d dilated thick walled vessel between the muscle bundles a lesion seen on buccal mucosa , b small opacities depicting phleboliths , c proliferating vessels infiltrating in between the muscle bundles , d dilated thick walled vessel between the muscle bundles the orthopantamogram ( opg ) showed calcified scattered masses lateral to the right ramus of the mandible ( fig . 
ultrasonography revealed the presence of hypoechoic areas showing the distorted fibers of the masseter muscle and hyperechoic areas showing phleboliths .
excision was done under general anesthesia and operative findings presented the buccal pad of fat infiltrated with vascular tissues and involving the right masseter muscle .
gross specimen showed soft tissues which were black in color with a few calcified masses ( possibly phleboliths ) .
the microscopic findings revealed disorganized proliferation and randomly arranged blood vessels of varying sizes in between muscle bundles ; endothelial proliferation and canalization was also seen ( fig . 
an extensive area of hemorrhage was evident and so were calcification depicting phleboliths . based on clinical , radiological and histopathological findings
, a diagnosis of angiomatosis was derived . a 19-year - old female presented with a chief complaint of swelling in left side of the face since 34  years . on examination , a well circumscribed solitary swelling of the left malar region
was seen which measured approximately 3    3  cm ( fig . 
radiographically , no significant findings were seen and a provisional diagnosis of lipoma was considered.fig . 
2a circumscribed swelling on left malar region , b gross specimen with nodular surface and irregular borders , c blood vessels of varying caliber , infiltrating into the stroma rich in adipose tissue , d proliferating capillaries around feeder vessel , e canalization and new blood vessel formation , f nerve bundle infiltrated by proliferating capillaries a circumscribed swelling on left malar region , b gross specimen with nodular surface and irregular borders , c blood vessels of varying caliber , infiltrating into the stroma rich in adipose tissue , d proliferating capillaries around feeder vessel , e canalization and new blood vessel formation , f nerve bundle infiltrated by proliferating capillaries excision was done under general anesthesia .
gross specimen showed soft tissue mass floating in formalin , with irregular borders and white to brownish in color ( fig . 
microscopically the excised specimen revealed blood vessels of varying caliber infiltrating into to the stroma rich in adipose tissue ( fig .  2c ) .
2d ) and areas of canalization and new blood vessel formation were also evident ( fig . 
2f ) , prompting a final diagnosis of angiomatosis . a 16-year - old male presented with the chief complaint of swelling on lower right region of the jaw since 2  months .
the lesion presented with facial asymmetry at the lower third of the face , with bony hard swelling extending from the symphysis to angle of the mandible ( fig . 
intraoral examination revealed diffuse bony swelling extending from the mandibular right central incisor to second molar and presented with egg shell crackling .
3a large diffuse swelling on the right side of the mandible , b panoramic radiograph showing large ill - defined radiolucency extending from 41 to 47 , c angiography showing mild increase in vascularity of right external carotid artery , d gross specimen showing large vascular channels inside the body of the mandible , e vascular channels infiltrating into the bone , f periosteal infiltration a large diffuse swelling on the right side of the mandible , b panoramic radiograph showing large ill - defined radiolucency extending from 41 to 47 , c angiography showing mild increase in vascularity of right external carotid artery , d gross specimen showing large vascular channels inside the body of the mandible , e vascular channels infiltrating into the bone , f periosteal infiltration opg revealed radiolucency extending from mandibular right central incisor to second molar ( fig . 
magnetic resonance imaging ( mri ) hard tissue window showed lytic lesion in the medulla of the mandible , with erosion of buccal and lingual cortical plates , thus giving a sun - ray appearance ( reactive periostitis ) .
angiography presented with mild increase in vascularity of the right external carotid artery ( fig . 
the gross specimen showed areas of dark brown to bluish lesion on both medial and lateral aspect of the mandible . on opening ,
histopathological findings revealed numerous vascular channels of capillary and cavernous caliber infiltrating into the bone ( fig . 
areas of periosteal infiltration with reactive new bone formation were also evident ( fig . 
a 13-year - old female presented with the complaint of swelling in the right side of face since 2  years .
the swelling had slowly progressed in size during the period . on examination , a diffuse swelling in the right side of the face extending from the ala - tragal plane superiorly to the lower border of mandible , from the anterior to posterior border of masseter , was seen .
intraorally , bluish discolouration of the right buccal mucosa with telengiectasia or spider nevi appearance was evident ( fig . 
the swelling was non tender , collapsible on digital pressure and filled up on releasing the pressure .
1a lesion seen on buccal mucosa , b small opacities depicting phleboliths , c proliferating vessels infiltrating in between the muscle bundles , d dilated thick walled vessel between the muscle bundles a lesion seen on buccal mucosa , b small opacities depicting phleboliths , c proliferating vessels infiltrating in between the muscle bundles , d dilated thick walled vessel between the muscle bundles the orthopantamogram ( opg ) showed calcified scattered masses lateral to the right ramus of the mandible ( fig . 
ultrasonography revealed the presence of hypoechoic areas showing the distorted fibers of the masseter muscle and hyperechoic areas showing phleboliths .
excision was done under general anesthesia and operative findings presented the buccal pad of fat infiltrated with vascular tissues and involving the right masseter muscle .
gross specimen showed soft tissues which were black in color with a few calcified masses ( possibly phleboliths ) .
the microscopic findings revealed disorganized proliferation and randomly arranged blood vessels of varying sizes in between muscle bundles ; endothelial proliferation and canalization was also seen ( fig . 
an extensive area of hemorrhage was evident and so were calcification depicting phleboliths . based on clinical , radiological and histopathological findings
a 19-year - old female presented with a chief complaint of swelling in left side of the face since 34  years . on examination , a well circumscribed solitary swelling of the left malar region was seen which measured approximately 3    3  cm ( fig . 
radiographically , no significant findings were seen and a provisional diagnosis of lipoma was considered.fig . 
2a circumscribed swelling on left malar region , b gross specimen with nodular surface and irregular borders , c blood vessels of varying caliber , infiltrating into the stroma rich in adipose tissue , d proliferating capillaries around feeder vessel , e canalization and new blood vessel formation , f nerve bundle infiltrated by proliferating capillaries a circumscribed swelling on left malar region , b gross specimen with nodular surface and irregular borders , c blood vessels of varying caliber , infiltrating into the stroma rich in adipose tissue , d proliferating capillaries around feeder vessel , e canalization and new blood vessel formation , f nerve bundle infiltrated by proliferating capillaries excision was done under general anesthesia .
gross specimen showed soft tissue mass floating in formalin , with irregular borders and white to brownish in color ( fig . 
microscopically the excised specimen revealed blood vessels of varying caliber infiltrating into to the stroma rich in adipose tissue ( fig .  2c ) .
2d ) and areas of canalization and new blood vessel formation were also evident ( fig . 
2e ) . nerve bundles too , were infiltrated by proliferating blood vessels ( fig . 
a 16-year - old male presented with the chief complaint of swelling on lower right region of the jaw since 2  months .
the lesion presented with facial asymmetry at the lower third of the face , with bony hard swelling extending from the symphysis to angle of the mandible ( fig . 
intraoral examination revealed diffuse bony swelling extending from the mandibular right central incisor to second molar and presented with egg shell crackling .
3a large diffuse swelling on the right side of the mandible , b panoramic radiograph showing large ill - defined radiolucency extending from 41 to 47 , c angiography showing mild increase in vascularity of right external carotid artery , d gross specimen showing large vascular channels inside the body of the mandible , e vascular channels infiltrating into the bone , f periosteal infiltration a large diffuse swelling on the right side of the mandible , b panoramic radiograph showing large ill - defined radiolucency extending from 41 to 47 , c angiography showing mild increase in vascularity of right external carotid artery , d gross specimen showing large vascular channels inside the body of the mandible , e vascular channels infiltrating into the bone , f periosteal infiltration opg revealed radiolucency extending from mandibular right central incisor to second molar ( fig . 
hard tissue window showed lytic lesion in the medulla of the mandible , with erosion of buccal and lingual cortical plates , thus giving a sun - ray appearance ( reactive periostitis ) .
angiography presented with mild increase in vascularity of the right external carotid artery ( fig . 
the gross specimen showed areas of dark brown to bluish lesion on both medial and lateral aspect of the mandible . on opening ,
histopathological findings revealed numerous vascular channels of capillary and cavernous caliber infiltrating into the bone ( fig . 
areas of periosteal infiltration with reactive new bone formation were also evident ( fig . 
the co - existence of vascular proliferation and abundant fat in this infiltrating lesion previously inspired the now obsolete term 
if only muscles are predominantly involved , it may also be termed as  intra - muscular hemangioma , which is once again a subjective diagnosis .
arteriovenous malformation , a term well founded in the radiologic literature , implies functional arteriovenous communications and may correspond in some instances to what pathologists term angiomatosis .
because of nomenclature issues , it has been very difficult to gain a clear understanding of this lesion and in particular to compare the experience derived from one speciality to another . to promote a more standardized approach to diagnosis
angiomatosis be used to connote a histologically benign vascular lesion that extensively involves a region of the body or several different tissue types in a contiguous fashion .
the congenital form may be seen sporadically or accompanying certain types of syndromes such as klippel  trenaunay 
rao and weiss s study   bears similarities to our reports in that the lesions commonly occurs in the second decade of life , there is a female predilection to its occurrence and it presents as a diffuse persistent swelling having two histological patterns .
these lesions probably begin during early intra - uterine life , grow proportionately with the fetus and consequently affect large areas of the body [ 2 , 4 ] .
the most common pattern consists of a haphazard proliferation of varying sized vessels , particularly large veins .
however , the most distinctive feature is the clusters of capillary vessels residing within or adjacent to the vein walls .
a second but uncommon pattern is the clusters of capillary - sized vessels arranged in nodules around a large vessel in the center .
both types are typically associated with large amounts of adipose tissue and hence these lesions have lead previous authors to use the term  infiltrating angiolipoma , suggesting that these are more generalized mesenchymal proliferations rather than exclusive vascular pathology .
because of its infiltrative nature , angiomatosis may resemble many of the vascular tumors and malformations .
angiolipoma can be considered as an important differential diagnosis , but it is well circumscribed and proliferating vessels are usually concentrated at the periphery of intratumoral lobules of adipocytes .
angiomyolipoma can be distinguished from angiomatosis as they are composed of varying amounts of blood vessels , smooth muscle and fat .
infiltrating lipoma consist of lesional fat tissue infiltrating in the form of long thin streaks radiating from the intratumoral mass .
angiomyxolipoma usually resembles angiomatosis due to presence of adipose tissue component and blood vessels with thick and thin walls .
it can be distinguished by the presence of myxoid change in adipose tissue and absence of haphazard blood vessel proliferation .
liposarcoma can also be considered as a differential diagnosis ; however , mitotic figures , cellular atypia and lipoblasts ( signet ring appearance ) are not seen in angiomatosis .
the treatment of choice in extensive angiomatosis is either radiotherapy or interferon -2a treatment . in localized cases ,
angiomatosis has no tendency to progress or evolve histologically to a more aggressive lesion . on
follow up , nearly 90% of patients experienced local recurrence and approximately 40% encountered more than one local recurrence within a period of 5  years .
the possibility of angiomatosis should be considered whenever a persistent swelling in the head and neck region in early childhood is seen .
 angiomatosis is a diffuse vascular lesion which involves a large segment of the body in a contiguous fashion involving multiple tissues ( e.g. subcutis , muscle , bone , adipose tissue etc . ) in different planes . 
 such lesions usually present in the first two decades of life with female predilection and are commonly seen in lower extremities . 
 it clinically mimics hemangioma or vascular malformation and its surgical removal is difficult because of its infiltrative nature and thus has high recurrence rate ( 90% ) . 
 therefore a precise histopathological diagnosis of angiomatosis is important to achieve a curative resection . 
 histopathologically it consists of proliferating blood vessels of varying caliber , infiltrating into the soft tissues . 
 proliferating capillaries are seen within or adjacent to major vessels . 
 few cases are reported in head and neck region . 
 this article highlights three unusual cases of angiomatosis reported as benign lesions , in rare sites such as the malar region ( predominantly infiltrating the adipose tissue ) , within the masseter ( predominantly infiltrating the muscle ) and in the mandible ( infiltrating the bone ) . 
 histopathological differential diagnosis is also discussed . 
Introduction
Case Reports
Case 1
Case 2
Case 3
Discussion
Conclusion