This website is intended for pathologists and laboratory personnel, who understand that medical information is imperfect and must be interpreted using reasonable medical judgment. Desmoplastic fibroma in the spine is extremely rare, and only a few cases have been reported. Only 14 examples of desmoplastic fibroma are recorded in mayo clinic files 1. Aug 12, 2014 the desmoplastic fibroma df is a rare, fibroblastic lesion of bone that histologically resembles the desmoid tumor of soft tissue. Jun 30, 2015 desmoplastic fibroma of bone, although considered benign, can be a debilitating condition leading to loss of limb 17. A ctscan and mriscan were performed and revealed a bone cyst with sclerotic borders without destruction of the cortical bone figs. In 2002, the world health organization defined df as a rare, benign bone tumor composed of spindle cells with minimal cytologic atypia and abundant collagen production. Moreover, a report of an interesting case is included localized in the mandibular corpus. Desmoplastic fibroma of the bone is an extremely rare primary benign tumor. 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. Because of its rarity, desmoplastic fibroma of bone. Multifocal desmoplastic fibromas of the mandible alan s.
Desmoplastic fibroma of bone is a very rare benign tumor, which may be locally aggressive. Desmoplastic fibroblastoma is commonly seen in middleage and older adults 4070 years. Authors report a rare case of desmoplastic fibroma of bone occurring in the distal phalanx. Although there is a considerable overlap in the mr imaging features with other bone tumors, an. A rare case of desmoplastic fibroma in the maxillary corpus is being presented with its pathology, clinical diagnostic methods, treatment and prognosis. Clinically, it is treated using surgery as the preferred choice.
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. In contrast to the welldocumented radiological appearance, the literature on mr imaging features of this tumor is scarce. Although it does not tend to metastatize, it has a high local recurrence and. This soft tissue tumor typically infiltrates into the surrounding fascia or muscle and has a predilection for recurrence after surgical removal. Desmoplastic fibroma in a toe has been extremely rarely reported. Jan 14, 2008 desmoplastic fibroma is a primary bone tumour which occurs exceedingly rarely in the cranial bones. A ctscan and mriscan were performed and revealed a bone cyst with sclerotic borders without destruction of the cortical bone. D esmoplastic fibroma of bone is a benign, locally aggressive tumor with varied recurrence rates found in the literature. Pathologic elements of this tumor include fibroblasts, collagen fibers, and elongated nuclei. Unlike most other benign lesions, a desmoplastic fibroma tends to be very aggressive at the local site.
The desmoplastic fibroma df is a rare, fibroblastic lesion of bone that histologically resembles the desmoid tumor of soft tissue. 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, when found in the foot, is an exceedingly rare occurrence 1. Desmoplastic fibroma df is a rare benign neoplasm of the bone that currently accounts for 0. Until jaffe differentiated it from other bone fibromas and established. Desmoplastic fibroma of bone may be extremely difficult to distinguish from lowgrade fibrosarcoma of bone. Desmoplastic fibroma of bone is a rare primary tumor of bone.
Desmoplastic fibroma of the jaws is most commonly discovered in the second and third decades with the mean age of 15. 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 fibroma df is a benign intraosseus neoplasia with locally aggressive and infiltrative characteristics. 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. Nichols 10 first called attention to the occurrence of desmoid tumors in extraabdominal sites 9. Pdf desmoplastic fibroma of bone is a rare benign primary bone tumor that histologically resembles the extraabdominal desmoid tumor of soft tissues find. Desmoplastic fibroma is a benign intraosseous neoplasm that is recognized as the intraosseous counterpart of soft tissue fibromatosis in both gnathic and extragnathic sites. A rare type of primary bone tumor that is characteristically composed of welldifferentiated cells that produce collagen. Although classified as benign, it frequently demonstrates aggressive behavior, often causing tooth mobility, extensive bone destruction, and has a moderate to high recurrence rate. It usually affects craniofacial bones, mandible most frequently, long bones metaphyseal femur, tibia, humerus. Fibrosarcoma, however, will have cellular atypia, occasional mitotic figures, and a herringbone arrangement of cells.
In 1958, jaffe delineated desmoplastic fibroma as a rare primary bone tumor usually found in young adults 7. Its a very rare tumor with slow growth and produces collagen. Aug 24, 2018 desmoplastic fibroblastoma is commonly seen in middleage and older adults 4070 years. Desmoplastic fibroma of the maxilla peoples journal of scientific. Desmoplastic fibroblastoma collagenous fibroma discussion. Desmoplastic fibroma article about desmoplastic fibroma.
It characterized also by aggressive intramedullary proliferation of myofibroblasts. Desmoplastic fibroma is a benign but aggressive tumor composed of fibroblasts and myoblasts in heavily collagenized stroma. Because of its intraosseus location the working diagnosis of desmoplastic fibroma was made. The operation was performed with the patient under general anaesthesia. With some extraabdominal desmoids, invasion of contiguous bone has been described 2, 8, 15. Desmoplastic fibroma was first recognized as a distinct kind of tumor in 1958 by henry l. 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. Collagenous fibroma desmoplastic fibroma of the palate. A rare case of desmoplastic fibroma in the maxillary corpus is being presented with its pathology. Desmoplastic fibroblastoma df, also known as collagenous fibroma is a distinct yet uncommon benign fibrous soft tissue tumor that typically occurs in the subcutaneous tissue or skeletal muscle in adults. Desmoplastic fibroma is generally classified as a benign tumor and has not been shown to metastasize.
The present study reports a case of desmoplastic fibroma of the bone with the longest published follow. Desmoplastic fibroma the open orthopaedics journal, 20, volume 7 41 pain was attributable to his physically demanding job. Hence, desmoplastic fibroma of bone is also referred to as desmoid tumor of bone 2. Bohm p, krober s, greschniok a, laniado m, kaiserling f. Left foramen ovale arrow is enlarged compared with normal right foramen ovale arrowhead, suggesting perineural spread of tumor.
It most often involves the mandible, large long bone or iliac bone. The tissue is described as being poorly cellular with nuclei that are ovoid or elongated. Desmoplastic fibroma is a rare benign primary bone tumor that is histologically similar to the soft tissue desmoid tumor. Although surgical resection of df arising in the spine is commonly regarded as a recommended treatment, it is difficult to achieve satisfactory results. Figure 4 photomicrograph showing monomorphic, wavy, spindled cells with. Desmo plastic fibroma of the extremities was first reported by jaffel in 1958. The mr imaging characteristics in our case are compared to those previously reported. Whitesides and ackerman 2 documented three cases in 1960, and sheer and kuhlman 3 reported an additional case in 1963. Biopsy confirmed a chondromyxoid fibroma which was removed surgically.
Sabo, dpm, facfas2 the foot and ankle online journal 8 2. Desmoplastic fibroma is a benign but locally aggressive tumor arising usually from the mandible, pelvis and long bones with a potential for recurrence. Frontal there is a welldefined, highly expansile, purely lytic lesion within the proximal ulna which extends to the distal third of the diaphysis. Desmoplastic fibroma df is a benign, yet locally aggressive, tumor of the connective tissue. They have been described involving a wide range of anatomic sites but most commonly arise on the upper extremity. Wed like to understand how you use our websites in order to improve them. Desmoplastic fibroma article about desmoplastic fibroma by. Desmoplastic fibroma df is a rare, benign but locally aggressive, intraosseous lesion with a high tendency of local recurrence. Desmoplastic fibroma is a primary bone tumour which occurs exceedingly rarely in the cranial bones. Paucicellular, bland spindled stellate and reactive appearing fibroblasts and myofibroblasts separated by. Lesions of fibrous dysplasia that show little woven bone may also be confused with desmoplastic fibroma. It is usually seen radiographically as a welldemarcated, radiolucent lesion.
This tumor grows locally in bone in an aggressive manner and destroys the bone. Desmoplastic fibroma of the frontal bone lath r, ranjan a. Desmoplastic fibroma of bone inwards 1991 cancer wiley. Five examples of this distinctive lesion of bone were described by jaffe 1 in 1958. Although classified as benign, it frequently demonstrates aggressive behavior, often causing tooth mobility, extensive bone destruction, and has a. A desmoplastic fibroma is a type of benign bone tumor. Printable desmoplastic fibroblastoma surgical pathology. Desmoplastic fibroma of bone can imitate the pattern of a lowgrade intraosseous osteosarcoma, but df lacks osteoid matrix production, as previously discussed. Original article clinical application of mpr nerve display. The main cell types that are seen include fibroblasts, myofibroblasts, and undifferentiated mesenchymal cells 9. Approximately, 180 cases have been reported in the english medical literature, most of which were case reports.
Accordingly the indication for operative excision via curettage and grafting was established. 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 definition of desmoplastic fibroma. The second represents a softtissue desmoid invading bone secondarily. The incidence of desmoplastic fibroma is reported to be 0. Benign intraosseous fibroma desmoplastic fibroma associated with paget disease of the iliac bone skeletal radiol 1988. Desmoplastic fibroma of the bone is now considered the intraosseous counterpart of the common softtissue desmoid or fibromatoses. Desmoplastic fibroma an overview sciencedirect topics. We report a case of desmoplastic fibroma of the frontal bone in a young male. 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.
There was a male predominance, and 74% of the patients were in. 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 df, a benign locally aggressive lesion of the bone is recognized as an intraosseous counterpart of soft tissue fibromatosis and is usually. Herford, dds, md, paul reder, md,t and yadranko ducic, md, desmoplastic fibroma is a locally aggressive benign fibrous tumor of bone that has been reported in both the maxillofacial skeleton and the long bones. Desmoplastic fibroma definition of desmoplastic fibroma by. However, ct and mri offer the best imaging of the lesion. Although there is a considerable overlap in the mr imaging features with other. 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. Desmoplastic fibroma, skull introduction desmoplastic fibroma is a rare benign but locally aggressive.
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