Desmoplastic fibroma definition of desmoplastic fibroma. A 21yearold female presented to the first hospital of jilin university changchun, jilin, china with thigh pain. Desmoplastic fibroma is a benign intraosseous neoplasm that is recognized as the intraosseous counterpart of soft tissue fibromatosis in both gnathic and extragnathic sites. Moreover, a report of an interesting case is included localized in the mandibular corpus. A rare case of desmoplastic fibroma in the maxillary corpus is being presented with its pathology, clinical diagnostic methods, treatment and prognosis. Desmoplastic fibromas are discovered most often in the first three decades of life, in the mandible the femur and pelvis are also favored sites. Desmoplastic fibromas histologically are identical to soft tissue desmoid tumors, with abundant collagenous stroma and little cellularity or pleomorphism. Desmoplastic fibroma of bone has been described as a rare, locally aggressive, benign lesion that histologically resembles a desmoid tumour of the soft tissue. Surgical resection with minimal margins is recommended.
Desmoplastic fibroma an overview sciencedirect topics. Primary osteosarcoma of the skull is a rare finding. It characterized also by aggressive intramedullary proliferation of myofibroblasts. Desmoplastic fibroblastoma collagenous fibroma of the. They have been described involving a wide range of anatomic sites but most commonly arise on the upper extremity. This tumor grows locally in bone in an aggressive manner and destroys the bone.
Desmoplastic fibroma, alternatively known as aggressive fibromatosis or desmoid tumors, was initially described by macfarlane in 1832. It was initially described by jaffe in 1958 who highlighted the histological resemblance to aggressive fibromatosis desmoid tumour. Otherwise known as collagenous fibroma, desmoplastic fibroblastoma is a benign tumour that presents as slowgrowing masses in the subcutis and mainly occur in middle age. The imaging features of df have been characterized in a few studies 11 stefanidis k, benakis s, tsatalou e, et al. Although there is a considerable overlap in the mr imaging features with other. Desmoplastic fibroma df is a rare, benign but locally aggressive, intraosseous lesion with a high tendency of local recurrence. Desmoplastic fibroma of bone may be extremely difficult to distinguish from lowgrade fibrosarcoma of bone. Although classified as benign, it frequently demonstrates aggressive behavior, often causing tooth mobility, extensive bone destruction, and has a. Lesions of fibrous dysplasia that show little woven bone may also be confused with desmoplastic fibroma. The main cell types that are seen include fibroblasts, myofibroblasts, and undifferentiated mesenchymal cells 9. Desmoplastic fibroma is a rare primary bone tumor which morphologically resembles its soft tissue counterpart the desmoidtype fibromatosis commonly seen in the abdominal wall. Pdf desmoplastic fibroma of the ilium researchgate. Desmoplastic fibroma of bone is a rare primary tumor of bone. The aggressive nature of this tumor was emphasized by inwards et al.
We report here on a pathologically proven case of osteosarcoma that presented as a painless mass in the frontal bone of a 7yearold boy. In contrast to the welldocumented radiological appearance, the literature on mr imaging features of this tumor is scarce. This unusual form of osteosarcoma had features of desmoplastic fibroma in a large por. Jaffe first described it as a benign tumor characterized by the formation of abundant collagen fibers by the tumor cells. Desmoplastic fibroma is a benign but locally aggressive tumor arising usually from the mandible, pelvis and long bones with a potential for recurrence. Desmoplastic fibroma df is a benign intraosseus neoplasia with locally aggressive and infiltrative characteristics. We report a case of desmoplastic fibroma of the frontal bone in a young male.
It is an extremely rare tumor with less than 200 cases in the published literature and with a reported incidence of 0. In 2002 df was defined by the who as a rare benign bone tumor composed of spindleshaped cells with minimal cytological atypia and abundant collagen production 2. Fibrosarcoma, however, will have cellular atypia, occasional mitotic figures, and a herringbone arrangement of cells. Peripheral ossifying fibroma pof is a reactive gingival overgrowth which is relatively uncommon in nature. With some extraabdominal desmoids, invasion of contiguous bone has been described 2, 8, 15. Desmoplastic fibroblastoma is commonly seen in middleage and older adults 4070 years. Computed tomography and magnetic resonance imaging of. Desmoplastic fibroma of the bone is an extremely rare primary benign tumor. Randelli 7 described a patient who had four recurrences of a desmoplastic. The incidence of desmoplastic fibroma is reported to be 0.
Treatment and functional result of desmoplastic fibroma. Desmoplastic fibroma of proximal femur surgical reconstruction. The roentgenogram of left knee showed welldefined osteolytic lesion with surrounding sclerotic rim at left distal femoral metaphysis. Desmoplastic fibroma of bone can imitate the pattern of a lowgrade intraosseous osteosarcoma, but df lacks osteoid matrix production, as previously discussed. Benign fibroaosseous lesions of the mandible and maxilla. In 1958, jaffe delineated desmoplastic fibroma as a rare. Desmoplastic fibroblastoma of the oral cavity e89 journal section. Osteosarcoma arising in a desmoplastic fibroma of the. A desmoplastic fibroma often behaves in an aggressive manner and macroscopically has a firm consistency with welldefined advancing surfaces that may extend into surrounding soft tissue. Summary of published cases of desmoplastic fibroma in the jawbones. Pdf desmoplastic fibroma df is a rare bone tumor, which is known to involve. Desmoplastic fibroma df of the bone, also know as desmoid tumour, is a rare nonmetastasizing but locally aggressive tumour.
Terminology currently in use nonossifying fibroma jaffe and. Because of its rarity, desmoplastic fibroma of bone. The microscopic findings of desmoplastic fibroma of bone closely resemble that of the soft tissue desmoid tumor. Desmoplastic fibroma is generally classified as a benign tumor and has not been shown to metastasize.
Unlike most other benign lesions, a desmoplastic fibroma tends to be very aggressive at the local site. Desmoplastic fibroma was first recognized as a distinct kind of tumor in 1958 by henry l. Bohm p, krober s, greschniok a, laniado m, kaiserling f. Oral and maxillofacial radiology desmoplastic fibroma of. It occurs most often in the first three decades of life and is found equally in men and women. We report a case of desmoplastic fibroma of the mandible with a 5 year followup without any signs of recurrence and discuss the utilization of resorbable hardware in the surgical. The annual incidence of desmoplastic fibroma is 24 per 1 million individuals, worldwide 1. Its a very rare tumor with slow growth and produces collagen. Desmoplastic fibroma histologically and biologically mimics the soft tissue desmoid tumor. This website is intended for pathologists and laboratory personnel, who understand that medical information is imperfect and must be interpreted using reasonable medical judgment. Pofs comprises of, one or more mineralized tissues, including bone, cementum like material or dystrophic calcification, within a matrix of cellular fibroblastic tissue 2. In this case report, we present imaging findings of a histopathologically proven desmoplastic fibroma involving the. Particularly, the agressive fibromatosis which occures peripherally and that proliferates to cortical bone is very similar to this tumor. Hence, desmoplastic fibroma of bone is also referred to as desmoid tumor of bone 2.
It is usually seen radiographically as a welldemarcated, radiolucent lesion. The present study reports a case of desmoplastic fibroma of the bone with the longest published follow. Desmoplastic fibroma df is a rare, locally aggressive benign bone tumour with a reported incidence of 0. Desmoplastic fibroma of the bone is rare, and the most common site of occurrence is the jaw, followed by the pelvis and long bones. Computed tomography and magnetic resonance imaging of desmoplastic fibroma with simultaneous manifestation in two unusual locations. A 15 yearold boy was referred with chronic left thigh pain. Desmoplastic fibroma of the jaws is most commonly discovered in the second and third decades with the mean age of 15. Approximately, 180 cases have been reported in the english medical literature, most of which were case reports. A desmoplastic fibroma is a type of benign bone tumor. The differential diagnosis of these both tumors is very difficult. Desmoplastic fibroma is a benign but aggressive tumor composed of fibroblasts and myoblasts in heavily collagenized stroma. Numerous authors have subsequently attempted to clarify the histological criteria and, in 20, the who. Desmoplastic fibroma is a very rare bone tumor which histopathologically and biologically very much similar to extraabdominal desmoid tumor of the soft tissue.
The spindle cells had bland nuclear chromatin and indistinct cell borders, which appeared to merge with the surrounding fibrous stroma figure. The desmoplastic fibroma df is a rare, fibroblastic lesion of bone that histologically resembles the desmoid tumor of soft tissue. The lesion most frequently occurs in the mandibular posterior area in children or young adults. We describe a case of an osteosarcoma arising in a desmoplastic fibroma in the proximal tibia. Frontal there is a welldefined, highly expansile, purely lytic lesion within the proximal ulna which extends to the distal third of the diaphysis. A case report abstract desmoplastic fibroma of bone is an uncommon myofibroblastic tumor characterized by local aggressive behavior that is associated with a highrecurrence rate. In medicine, a desmoplastic fibroma is a benign, but locally aggressive, fibrous and rare tumor of the bone, affecting children and young adults, potentially resulting in cortical bone destruction.
It was first described by jaffe in 1958 and there are around 270 reported cases in literature accounting for less than 0. Desmoplastic fibroma can be treated with surgery, radiotherapy or pharmacological agents. En bloc resection is the treatment of choice in view of. Desmoplastic fibroma of bone is a rare myofibroblastic tumor, locally aggressive, benign lesion that histologically resembles a desmoid tumor of the softtissue. Although it does not tend to metastatize, it has a high local recurrence and. However, ct and mri offer the best imaging of the lesion.
Desmoplastic fibroma of the frontal bone lath r, ranjan a. There is no metastasis but beside of their destructive growth they show a high frequent recurrence after local resection. The disease occurs in the form of benign locally aggressive tumors that possess a high rate of recurrence. Desmoplastic fibroma df of bone is a rare, lytic, locally aggressive but nonmetastatic tumor that was first described by jaffe in 1958.
Desmoplastic fibroma of the mandible dentomaxillofacial. Desmoplastic fibroma article about desmoplastic fibroma. Desmoplastic fibroma of bone is a very rare benign tumor, which may be locally aggressive. In 1838 the german physiologist and anatomist johannes muller characterised the term. Desmoplastic fibroma is a rare bone tumor, accounting for only 0. The mr imaging characteristics in our case are compared to those previously reported. Desmoplastic fibroma df is a benign but locally aggressive neoplasm of the bones 1,2 and it is very rare in the mandible like some other intraoral tumours. Desmoplastic fibroma is a benign intraosseous neoplasm that is recognized as the intraosseous counterpart of. Desmoplastic fibroma, skull introduction desmoplastic fibroma is a rare benign but locally aggressive. The present study reports a case of desmoplastic fibroma of the bone with the longest published followup. It usually affects craniofacial bones, mandible most frequently, long bones metaphyseal femur, tibia, humerus.
A rare type of primary bone tumor that is characteristically composed of welldifferentiated cells that produce collagen. Pdf desmoplastic fibroma of bone is a very rare primary bone tumor. Desmoplastic fibroma is a rare, nonmetastasizing but locally aggressive tumor. Desmoplastic fibroma df is an unusual bone tumor having a myofibroblastic origin, akin to the desmoid tumor seen in the soft tissue. It was initially described in 1958 by jaffe, who separated it as a distinct entity from other intraosseous. As per the who classification of soft tissue and bone tumors of 20.
Desmoplastic fibroma of bone, when found in the foot, is an exceedingly rare occurrence 1. The desmoplastic fibroma is a rare locally invasive bone tumour. Desmoplastic fibroblastoma also known as collagenous fibroma is a benign relatively paucicellular, soft tissue tumor characterized by spindle to stellateshaped fibroblasts and myofibroblasts embedded in a prominent collagenous background. D esmoplastic fibroma of bone is a benign, locally aggressive tumor with varied recurrence rates found in the literature.
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