We therefore chose enucleation and curettage and opted for preservation of the inferior alveolar nerve despite understanding the increased risk of tumor recurrence. Original article clinical application of mpr nerve display. Desmoplastic fibroma df is a rare, locally invasive but benign bone tumor. It characterized also by aggressive intramedullary proliferation of myofibroblasts.
Desmoplastic fibroma article about desmoplastic fibroma. The who defines a desmoplastic fibroma as a rare, benign bone tumour composed of spindle cells with minimal atypia and abundant collagen production. Although there is a considerable overlap in the mr imaging features with other bone tumors, an. Desmoplastic fibroblastoma collagenous fibroma is a benign fibrous tumor that presents as a slowgrowing, painless mass. Desmoplastic fibroma of bone, when found in the foot, is an exceedingly rare occurrence 1.
A rare case of desmoplastic fibroma in the maxillary corpus is being presented with its pathology, clinical diagnostic methods, treatment and prognosis. Aug 12, 2014 the desmoplastic fibroma df is a rare, fibroblastic lesion of bone that histologically resembles the desmoid tumor of soft tissue. Most patients are adults and it is more common in males m. 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. Desmoplastic fibromalike tumor of maxillofacial region. Desmoplastic fibroma the open orthopaedics journal. Whitesides and ackerman 2 documented three cases in 1960, and sheer and kuhlman 3 reported an additional case in 1963. The desmoplastic fibroma df is a rare, fibroblastic lesion of bone that histologically resembles the desmoid tumor of soft tissue. Histologically, desmoplastic fibroma of the bone is formed of wavy fibroblasts and abundant collagenous tissue, and bears a close resemblance to a desmoid tumor of the soft tissues.
Pathologic elements of this tumor include fibroblasts, collagen fibers, and elongated nuclei. Desmoplastic fibroma of bone is a very rare primary bone tumor morphologically resembling desmoidtype fibromatosis, its much more common counterpart of soft tissue. Figure 4 photomicrograph showing monomorphic, wavy, spindled cells with. Desmoplastic fibromas are rare, benign, yet aggressive fibrous lesions of bone, with high recurrence rates. Desmoplastic fibromas are discovered most often in the first three decades of life, in the mandible the femur and pelvis are also favored sites. Cryoablation, image processing, computerassisted, desmoplastic fibroma introduction desmoplastic fibroma is a rare tumor between benign and malignant, and surrounds adjacent vital structures such as nerves and blood vessels with invasive growth pattern. It is usually seen radiographically as a welldemarcated, radiolucent lesion. Desmoplastic fibroma is a benign but aggressive tumor composed of fibroblasts and myoblasts in heavily collagenized stroma. The second represents a softtissue desmoid invading bone secondarily. 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.
It usually affects craniofacial bones, mandible most frequently, long bones metaphyseal femur, tibia, humerus. Five examples of this distinctive lesion of bone were described by jaffe 1 in 1958. Fibroma treatment questions about fibroma on justanswer. The present study reports a case of desmoplastic fibroma of the bone with the longest published followup. Desmoplastic fibroma of the mandible a rare benign tumor. In contrast to the welldocumented radiological appearance, the literature on mr imaging features of this.
Desmoplastic fibroma of the jaws is most commonly discovered in the second and third decades with the mean age of 15. Desmoplastic fibroma is generally classified as a benign tumor and has not been shown to metastasize. A 21yearold female presented to the first hospital of jilin university changchun, jilin, china with thigh pain. Desmoplastic fibroma df of bone is a rare, lytic, locally aggressive but nonmetastatic tumor that was first described by jaffe in 1958. When there are any issues with fibroma tumors, questions regarding treatment and cure may arise. A desmoplastic fibroma is a type of benign bone tumor. Desmoplastic fibroma an overview sciencedirect topics. May 05, 2020 a desmoplastic fibroma is a type of benign bone tumor. We report a case of desmoplastic fibroma of the frontal bone in a young male. Desmoplastic fibroma of bone is a rare primary tumor of bone. Desmoplastic fibroma of bone can imitate the pattern of a lowgrade intraosseous osteosarcoma, but df lacks osteoid matrix production, as previously discussed. The first would be considered a desmoplastic fibroma belonging with the group in table i by the pathologic criteria of some authorities 4, 16, but a periosteal desmoid by others 12. 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.
To our knowledge, no case of perineural extension of a desmoplastic fibroma has been described. The main cell types that are seen include fibroblasts, myofibroblasts, and undifferentiated mesenchymal cells 9. Histologically and biologically, desmoplastic fibroma mimics extraabdominal desmoid tumor of soft tissue. Desmoplastic fibroma df, a benign locally aggressive lesion of the bone is recognized as an intraosseous counterpart of soft tissue fibromatosis and is usually. The aim of this study is to investigate the immunohistochemical profile and the involvement of the. In contrast to the welldocumented radiological appearance, the literature on mr imaging features of this tumor is scarce. Desmoplastic fibroma of bone is a very rare benign tumor, which may be locally aggressive. They have been described involving a wide range of anatomic sites but most commonly arise on the upper extremity. In many cases, this condition may be treated depending on where the tumor is located.
Dfb can occur in any bone, but is predominant in the jaw and the femur bones. Desmoplastic fibroma of the bone is an extremely rare primary benign tumor. Although it does not tend to metastatize, it has a high local recurrence and. Desmoplastic fibroma of the skull in an infant request pdf. In this setting, the clinical presentation is usually represented by a bulging tumor of the face. Desmoplastic fibroma, of which an example is seen in fig.
Frontal there is a welldefined, highly expansile, purely lytic lesion within the proximal ulna which extends to the distal third of the diaphysis. Computed tomography and magnetic resonance imaging of. Although classified as benign, it frequently demonstrates aggressive behavior, often causing tooth mobility, extensive bone destruction, and has a moderate to high recurrence rate. Desmoplastic fibroma df is a benign intraosseus neoplasia with locally aggressive and infiltrative characteristics. Desmoplastic fibroma definition of desmoplastic fibroma by.
Other differential diagnosis includes squamous cell carcinoma and ameloblastic fibroma. The usual sites of involvement are subcutaneous tissue or skeletal muscles of upper or lower extremities. Desmoplastic fibroma of the frontal bone lath r, ranjan a. Desmoplastic fibroma of the mandible in young childrena case. A 29yearold man suffered minor pain in his right hip for 2 years. Jun 30, 2015 desmoplastic fibroma of bone, although considered benign, can be a debilitating condition leading to loss of limb 17. Jaffe first described it as a benign tumor characterized by the formation of abundant collagen fibers by the tumor cells. This tumor grows locally in bone in an aggressive manner and destroys the bone. Desmoplastic fibroma is a benign but locally aggressive tumor arising usually from the mandible, pelvis and long bones with a potential for recurrence. The aim of this study is to investigate the immunohistochemical profile and the.
Lesions of fibrous dysplasia that show little woven bone may also be confused with desmoplastic fibroma. Each of the two tumors presented in this communication shows a softtissue mass with an accompanying bony lesion. Desmoplastic fibroma df is a rare, benign but locally aggressive, intraosseous lesion with a high tendency of local recurrence. Desmoplastic fibroma definition of desmoplastic fibroma. 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. Desmoplastic fibroma is a benign intraosseous neoplasm that is recognized as the intraosseous counterpart of soft tissue fibromatosis in both gnathic and extragnathic sites. Desmoplastic fibroma of proximal femur surgical reconstruction. Desmoplastic fibroma of the maxilla peoples journal of scientific. Desmoplastic fibroma is a rare primary tumor of bone that histologically and biologically mimics the extraabdominal desmoid tumor of soft tissue. Clinically, it is treated using surgery as the preferred choice. Typically, dfb is more common in the distal femoral metaphysis when it occurs in the femur. Although classified as benign, it frequently demonstrates aggressive behavior, often causing tooth mobility, extensive bone destruction, and has a. This website is intended for pathologists and laboratory personnel, who understand that medical information is imperfect and must be interpreted using reasonable medical judgment.
Grossly, it is firm, wellcircumscribed with a white or gray homogenous surface without. The mr imaging characteristics in our case are compared to those previously reported. Desmoplastic fibroma is a rare benign neoplasm of the bone characterized by aggressive local infiltration. Desmoplastic fibroma occurs most commonly in persons under the age of 30,4 but has a wide reported age range from 15 months to 75 years of age. Benign intraosseous fibroma desmoplastic fibroma associated with paget disease of the iliac bone skeletal radiol 1988. Desmoplastic fibroma article about desmoplastic fibroma by. Fibroma is a kind of tumor that is made by fibers masses that have grown together and caused a tumor. Its a very rare tumor with slow growth and produces collagen.
At the 1year followup visit, no relapse in the tumor was observed. Moreover, a report of an interesting case is included localized in the mandibular corpus. Five examples of this distinctive lesion of bone were described by jaffe1 in 1958. A rare type of primary bone tumor that is characteristically composed of welldifferentiated cells that produce collagen. Desmoplastic fibroma is a very rare bone tumor which histopathologically and biologically very much similar to extraabdominal desmoid tumor of the soft tissue. Physical examination showed a limitation when the right hip was flexed and a percussion pain on the hip region. Imaging examination revealed a spaceoccupying lesion in the right femur. Desmoplastic fibroma of the femur with atypical image findin. Massive rare desmoplastic fibroma of the ilium and ischium i. Herein, a case of massive and unusual df, with simultaneous involvement of ilium and ischium, is described. Whitesides and ackerman2 documented three cases in 1960, and sheer and kuhlman3 reported an additional case in 1963. Printable desmoplastic fibroblastoma surgical pathology. However, ct and mri offer the best imaging of the lesion.
Desmoplastic fibroma of the bone is an extremely rare, lytic, locally aggressive but nonmetastatic, primary bone tumor. Pdf desmoplastic fibroma of bone is a rare benign primary bone tumor that histologically resembles the extraabdominal desmoid tumor of soft tissues find. Pdf desmoplastic fibroma df is an exceedingly rare benign primary tumor of bone, first described in 1958, which can be locally aggressive. Desmoplastic fibroma of the bone, the intraosseous counterpart of softtissue fibromatoses, also is known as desmoid tumor of bone. En bloc resection is the treatment of choice in view of.
It is an extremely rare tumor with less than 200 cases in the published literature and with a reported incidence of 0. Desmoplastic fibroma of bone has been described as a rare, locally aggressive, benign lesion that histologically resembles a desmoid tumour of the soft tissue. The first would be considered a desmoplastic fibroma belonging with the group in table i by the pathologic criteria of some authorities 4. Desmoplastic fibroma was first recognized as a distinct kind of tumor in 1958 by henry l. The incidence of desmoplastic fibroma is reported to be 0. Desmoplastic fibroma of bone may be extremely difficult to distinguish from lowgrade fibrosarcoma of bone. It usually presents in patients during the first three decades of life and often involves the mandible or long bones of the skeleton. It worsened after sudden movements, which prevented him from walking normally. Massive rare desmoplastic fibroma of the ilium and ischium. In this case report, we described and emphasized the particularity of the. We describe a case of desmoplastic fibroma with a local recurrence and perineural spread along the third division of the trigeminal nerve. Because of its rarity, desmoplastic fibroma of bone. 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.
Desmoplastic fibroma of bone dfb is an extremely rare benign bone tumor, which accounts for approximately 0. It is considered to be the bony equivalent of the desmoidtype fibromatosis aggressive fibromatosis of soft tissue, based on its comparable histological and clinical features. Desmoplastic fibroma df is a rare bone tumor, which is known to involve mostly the gnathic bones. The incidence of desmoplastic fibroma of bone in all patients with benign bone tumours in our population is 0. 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. A medical imaging examination showed that the right ilium and ischium had a massive bone lesion. Left foramen ovale arrow is enlarged compared with normal right foramen ovale arrowhead, suggesting perineural spread of tumor. Request pdf desmoplastic fibroma of the skull in an infant case report a 1yearold girl presented with a 10month history of progressive protuberance of the left frontal skull. Approximately, 180 cases have been reported in the english medical literature, most of which were case reports. Desmoplastic fibroma of the femur with atypical image. Fibrosarcoma, however, will have cellular atypia, occasional mitotic figures, and a herringbone arrangement of cells. It represents one of the rarest bone diseases, with an incidence of only 0.
Bohm p, krober s, greschniok a, laniado m, kaiserling f. Desmoplastic fibroma df is a rare benign intraosseous tumor with provincially aggressive nature. Desmoplastic fibroma of bone is a rare locally aggressive, but nonmetastatic tumor. Recognition of desmoplastic fibrom a is important because on radiology and histology, the lesion may be mistaken. Unlike most other benign lesions, a desmoplastic fibroma tends to be very aggressive at the local site. Nov 24, 2009 desmoplastic fibroma df is a rare, benign but locally aggressive, intraosseous lesion with a high tendency of local recurrence. Desmoplastic fibromas histologically are identical to soft tissue desmoid tumors, with abundant collagenous stroma and little cellularity or pleomorphism.
Although there is a considerable overlap in the mr imaging features with other. Desmoplastic fibroma is an aggressive fibrous lesion that may rarely be encountered in the jaw fig. D esmoplastic fibroma of bone is a benign, locally aggressive tumor with varied recurrence rates found in the literature. It occurs most often in the first three decades of life and is found equally in men and women.
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