Desmoplastic fibroma of bone can imitate the pattern of a lowgrade intraosseous osteosarcoma, but df lacks osteoid matrix production, as previously discussed. Nasopharynx fibroma an overview sciencedirect topics. Desmoplastic fibroma of bone may be extremely difficult to distinguish from lowgrade. This tumour has been observed infrequently in the diaphysis like the one presented here. Desmoplastic fibroma is a rare locally aggressive benign tumour. Case reports of desmoplastic fibroma of the bone mention the presence of low to intermediate signal intensity on t2weighted mri sequences and vary in the degree of emphasis on this observation. Desmoplastic fibroma of the bone is an extremely rare, lytic, locally aggressive but nonmetastatic, primary bone tumor.
Case report successful treatment of a massive desmoplastic fibroma of the ilium without surgery. Desmoplastic fibroma recurrence associated with tuberous sclerosis in a young patient. The term fibrous dysplasia refers to a variety of bony diseases characterized by the substituion of the bone by abnormal connective tissue. Kesani reported a case of a 15yearold 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. Treatment and functional result of desmoplastic fibroma with. Only one previous example has been reported in the calvaria.
Desmoplastic fibroma of bone is a rare benign prima. It characterized also by aggressive intramedullary proliferation of myofibroblasts. Successful treatment of a massive desmoplastic fibroma of the. Desmoplastic fibroma df is a benign intraosseus neoplasia with locally aggressive and infiltrative characteristics. Histologically and biologically, desmoplastic fibroma mimics extraabdominal desmoid tumor of soft tissue. Moreover, a report of an interesting case is included localized in the mandibular corpus. A case report with longterm followup hideyuki kinoshita,1 takeshi ishii,1 hiroto kamoda,1 yoko hagiwara,1 toshinori tsukanishi,1 sumihisa orita,2 kazuhide inage,2 naoya hirosawa,2 seiji ohtori,2 and tsukasa yonemoto1 1department of orthopedic surgery, chiba cancer center, 6662 nitonacho. Desmoplastic fibroma of the mandible a rare benign tumor. A 29yearold man suffered minor pain in his right hip for 2 years. Pdf desmoplastic fibroma of the ilium researchgate. Pdf congenital desmoplastic fibroma of the cranium. Desmoplastic fibroma df is a rare benign intraosseous tumor with provincially aggressive nature. Desmoplastic fibroma of bone dfb is a rare bone tumor with an incidence rate of less than 0. Desmoplastic fibroma of the bone is an extremely rare primary benign tumor.
D esmoplastic fibroma of bone is a benign, locally aggressive tumor with varied recurrence rates found in the literature. A case of desmoplastic fibroma of bone with ctnnb1 point. Pdf desmoplastic fibroma recurrence associated with. Desmoplastic fibroma df is a rare, benign but locally aggressive, intraosseous lesion with a high tendency of local recurrence. Benign bone tumor, desmoplastic fibroma, mandible, recurrence. Desmoplastic fibroma of the temporal bone the journal of. Desmoplastic fibroma of the rib archives of pathology. Df earned its appellation as a result of its morphological resemblance to desmoidtype fibromatosis. Desmoplastic fibroma is an unusual benign tumor of connective tissue origin that has most. Although considered a benign lesion, it is locally destructive, can extend into the soft tissues, and has a high rate of local recurrences after incomplete surgical excision.
This tumor grows locally in bone in an aggressive manner and destroys the bone. In this case report, we present a desmoplastic fibroma in an unusual location, the clavicle. Desmoplastic fibroma of the rib with cystic change. It usually affects craniofacial bones, mandible most frequently, long bones metaphyseal femur, tibia, humerus. Pdf desmoplastic fibroma of the rib with cystic change. Basic science and systemic disease, part 3 of the netter collection of medical illustrations. The majority of dfs occur in the long bones, mandible and ilium. The typical clinical presentation is pain and swelling above the affected area. The radiographic and histological aspects of a case of desmoplastic fibroma involving the parietal bone are reported. In this particular case, the advantages of wide resection, including a. Although there is a considerable overlap in the mr imaging features with other bone tumors, an. Described by evans in 1995 desmoplastic fibroblastoma is an uncommon benign soft tissue tumor. Desmoplastic fibroblastoma of the left upper arm bmj. Numerous authors have subsequently attempted to clarify the histological criteria and, in 20, the who.
Fibrosarcoma, however, will have cellular atypia, occasional mitotic figures, and a herringbone arrangement of cells. Desmoplastic fibroma df is a rare, locally invasive but benign bone tumor. To our knowledge, only three cases of this lesion involving the calvaria have been described previously. Desmoplastic fibroma df is a rare, locally aggressive benign bone tumour with a reported incidence of 0. Jaffe first described it as a benign tumor characterized by the formation of abundant collagen fibers by the tumor cells. Particularly, the agressive fibromatosis which occures peripherally and that proliferates to cortical bone is very similar to this tumor. Desmoplastic fibroma df is an extremely rare locally aggressive bone tumor with an incidence of 0.
Desmoplastic fibroma is a distinctive and rare neoplasm of bone. Although classified benign, this intraosseous lesion exhibits local aggressiveness and has a high potential for recurrence. Desmoplastic fibroma of the mandible bir publications. Although classified as benign, it frequently demonstrates aggressive behavior, often causing tooth mobility, extensive bone destruction, and has a. Desmoplastic fibroma of bone may be extremely difficult to distinguish from lowgrade fibrosarcoma of bone. The diagnostic and surgical aspects of a case of desmoplastic fibroma of the skull that radiographically simulated eosinophilic granuloma are reported. Desmoplastic fibroma df is a rare, locally aggressive bone tumour, first described by jaffe in 1958. Desmoplastic fibroma is a benign but aggressive tumor composed of fibroblasts and myoblasts in heavily collagenized stroma. Desmoplastic fibroma of bone is a very rare primary bone tumor morphologically resembling desmoidtype fibromatosis, its much more common counterpart of soft tissue. Desmoplastic fibroma df of bone is a rare, nonmetastasizing but locally aggressive tumor that has been discussed infrequently in the radiology literature.
Mirzaei journal of hand surgery european volume 2017 42. Although surgical resection of df arising in the spine is commonly regarded as a recommended treatment, it is difficult to achieve satisfactory results. Desmoplastic fibroma df is a benign intraosseous neoplasm, that is, recognized as the intraosseous counterpart of soft tissue fibromatosis in both gnathic and extragnathic sites. The present study reports a case of desmoplastic fibroma of the bone with the longest published follow. Desmoplastic fibroma article about desmoplastic fibroma by. Herein, a case of massive and unusual df, with simultaneous involvement of ilium and ischium, is described. This website is intended for pathologists and laboratory personnel, who understand that medical information is imperfect and must be interpreted using reasonable medical judgment. The differential diagnosis of these both tumors is very difficult. A case report article pdf available in journal of orthopaedic surgery hong kong 111. Whitesides and ackerman 2 documented three cases in 1960, and sheer and kuhlman 3 reported an additional case in 1963.
Desmoplastic fibroblastoma is an uncommon slowgrowing benign tumour, also known as a collagenous fibroma, and occur in the subcutaneous or intramuscular tissues. Desmoplastic fibroma is a very rare primary tumor of bone, closely related to aggressive fibromatosis of soft tissue. We identified thirteen patients with desmoplastic fibroma of bone treated at our institute over a 30 year period. Desmoplastic fibroma of the mandible h shi, p wang, s wang and q yu department of radiology, ninth peoples hospital, medical school, shanghai jiao tong university, shanghai, pr china desmoplastic fibroma df of the bone, also know as desmoid tumour, is a rare nonmetastasizing but locally aggressive tumour.
Desmoplastic fibroblastoma collagenous fibroma is a benign fibrous tumor that occurs most often in patients during the fifth and sixth decades of life with a malefemale ratio of 5. Desmoplastic fibroma in the spine is extremely rare, and only a few cases have been reported. Diagnosis is not easy and is often made by excluding other tumours. Massive rare desmoplastic fibroma of the ilium and ischium i. Desmoplastic fibroma of the mandible case report springerlink. Although classified as benign, df frequently exhibits an aggressive behavior, has a moderate. This rare condition occurs predominantly in the mandible and in.
We studied two cases of rare fibrous bone tumors, namely desmoplastic fibroma df and lowgrade central osteosarcoma lgcos resembling desmoplastic fibroma dflike lgcos. Pdf desmoplastic fibroma df is an exceedingly rare benign primary tumor of. In contrast to the welldocumented radiological appearance, the literature on mr imaging features of this tumor is scarce. Desmoplastic fibroma of bone is a rare locally aggressive, but nonmetastatic tumor. Desmoplastic fibroma of the bone dfb is a notably rare, lytic, locally aggressive but nonmetastatic, primary benign bone tumor in patients less than 30 years old. Diaphyseal desmoplastic fibroma of humerus report of a. Desmoplastic fibroma df is a benign but locally aggressive tumor usually recognized as an intraosseous counterpart of soft tissue fibromatosis in both gnathic and extragnathic sites. Desmoplastic fibroma df is a benign, rare fibroblastic intraosseous neoplasm histologically resembling a desmoid soft tissue tumor. Aug 12, 2014 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. 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.
Desmoplastic fibroma df of the bone, also know as desmoid tumour, is a rare non metastasizing but locally aggressive tumour. Desmoplastic fibroma, alternatively known as aggressive fibromatosis or desmoid tumors, occurs in the form of benign locally aggressive tumors. Desmoplastic fibroma versus softtissue desmoid tumour of. Download fulltext pdf download fulltext pdf desmoplastic fibroma of the ilium article pdf available in international journal of surgery case reports 410. The microscopic findings of desmoplastic fibroma of bone closely resemble that of the soft tissue desmoid tumor. Although classified as benign, it frequently demonstrates aggressive behavior, often causing tooth mobility, extensive bone destruction, and has a moderate to high recurrence rate. As the clinical presentation, imaging features and histopathology of df and dflike lgos show much overlap, the objective of this study was to investigate the value of cytogenetic analysis, molecular pathology. Desmoplastic fibroma versus softtissue desmoid tumour of forearm. The radiographs from 107 previously published cases of df and seven cases from the authors institution were analyzed to better understand and define its radiographic characteristics. Desmoplastic fibroma is a rare benign neoplasm of bone. The most common site of involvement is mandible reported incidence 22% of all desmoplastic fibroma cases followed by metaphysis of long bones. Massive rare desmoplastic fibroma of the ilium and ischium. Pdf desmoplastic fibroma is a rare, locally aggressive, benign lesion.
Df occurs in the subcutaneous or intramuscular tissue, with a wide anatomic distribution, showing a strong predilection for men between the fifth and sixth decades of life 1, 3. Five examples of this distinctive lesion of bone were described by jaffe1 in 1958. They are considered to be a bony counterpart of soft tissue desmoid tumors and are histologically identical. This is a case report on an unusual presentation of desmoplastic fibroma of bone. Musculoskeletal system, volume 6, part iii biology and systemic diseases, 2nd edition. Desmoplastic fibroblastoma of the left upper arm bmj case. Collagenous fibroma desmoplastic fibroblastoma of the. Desmoplastic fibromas are rare bone tumors that have been reported in the adult skull but rarely in that of children. Presentation in the rib is extremely rare with only six cases reported previously. Desmoplastic fibroma of bone, when found in the foot, is an exceedingly rare occurrence 1. Desmoplastic fibroma is generally classified as a benign tumor and has not been shown to metastasize. Desmoplastic fibroma is an infrequent, slowgrowing, locally aggressive intraosseous fibrous tumor that rarely is associated with tuberous sclerosis. The treatment modalities for this tumour are nonuniform and often controversial. Fibrosarcoma, however, will have cellular atypia, occasional.
As the recommended primary treatment for dfb, wide resection is preferred to curettage from the perspective of recurrence but wide resection of dfb in the pelvis such as in the acetabulum could result in greater functional loss. 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. The mr imaging characteristics in our case are compared to those previously reported. Desmoplastic fibroma is a rare benign tumour of bone. Desmoplastic fibroma of the bone is now considered the intraosseous counterpart of the common softtissue desmoid or fibromatoses. Desmoplastic fibroma of the proximal end of the left femur. An unusual case of a desmoplastic fibroma of the temporal bone is presented. Pdf desmoplastic fibroma df of the bone, also know as desmoid tumour, is a rare nonmetastasizing but locally aggressive tumour. An 18yearold female presented with progressive swelling left arm and dull aching pain for 1 year.
It represents one of the rarest bone diseases, with an incidence of only 0. Successful treatment of a massive desmoplastic fibroma of. It has a propensity for locally aggressive behavior and local recurrence. In 1958, jaffe first described desmoplastic fibroma of the bone as a distinct entity when he documented five cases of a previously unclassified osseous fibrous tumor that histologically was similar to abdominal desmoid tumor. Its a very rare tumor with slow growth and produces collagen. Desmoplastic fibroma df is a benign, yet locally aggressive, tumor of the connective tissue. Histopathological diagnosis of this tumour is also sometimes not easy. The who defines a desmoplastic fibroma as a rare, benign bone tumour composed of spindle cells with minimal atypia and abundant collagen production. Crosssectional imaging patterns of desmoplastic fibroma. It is most frequently reported in mandible, metaphysis and metadiaphysis of long bones. The most common sites of involvement are the mandible and the metaphysis of long bones.
Desmoplastic fibroma is an extremely rare tumor of bone with a reported incidence of 0. The aim of this study is to investigate the immunohistochemical profile and the. It was initially described by jaffe in 1958 who highlighted the histological resemblance to aggressive fibromatosis desmoid tumour. Desmoplastic fibromas are extremely rare bone tumors that do not metastasize but may be locally aggressive. Nov 24, 2009 desmoplastic fibroma df is a rare, benign but locally aggressive, intraosseous lesion with a high tendency of local recurrence.
Lesions of fibrous dysplasia that show little woven bone may also be confused with desmoplastic fibroma. We present a case of desmoplastic fibroma in a child. Whitesides and ackerman2 documented three cases in 1960, and sheer and kuhlman3 reported an additional case in 1963. Five examples of this distinctive lesion of bone were described by jaffe 1 in 1958. Mahuken ah, nolteernsting cc, wildberger je, wirtz dc, gunther rw. The desmoplastic fibroma df is a rare, fibroblastic lesion of bone that histologically resembles the desmoid tumor of soft tissue. Desmoplastic fibroma of bone is a very rare benign tumor, which may be locally aggressive.